Keywords

Introduction

The absence or loss of an eye due to a congenital malformation, tumor, trauma, or end-stage ocular disease can be an exceptionally difficult situation for patients, and the management of the anopthalmic socket has long been a challenge for the ophthalmologist and ocularist. Loss of binocular visual function with reduced peripheral visual field and loss of depth perception may result in difficulties with activities of daily living and have various vocational restrictions [1,2,3,4,5,6]. Individuals may experience a sense of facial disfigurement and poor self-esteem as a result of the “lost body part.” [3,4,5,6,7]. In the past three decades, there have been numerous developments and refinements in anophthalmic socket surgery with respect to implant material and design, implant wrapping, implant-prosthesis coupling, and socket volume considerations. Anophthalmic surgery is no longer simply about replacing a diseased eye with an orbital implant and delegating the procedure to junior resident staff. As with other microsurgical ophthalmic procedures, enucleation and eviscerations should be performed meticulously to attain the best functional and cosmetic result and to avoid deformities that may compound the patients’ already challenging situation. Because eye contact is such an essential part of human interaction, it is extremely important for the patient with an artificial eye to maintain a natural, normal-appearing prosthetic eye.

Characteristics of the ideal anophthalmic socket include [8, 9].

  1. 1.

    A centrally placed, well-covered, buried implant of adequate volume, fabricated from a bioinert material that transmits motility from the implant to the overlying prosthesis.

  2. 2.

    A socket lined with healthy conjunctiva and fornices deep enough to retain a prosthesis and to permit horizontal and vertical excursion of an artificial eye.

  3. 3.

    Normal eyelid and eyelash position, appearance, and eyelid tone.

  4. 4.

    A supratarsal eyelid fold that is symmetric with that of the contralateral eyelid.

  5. 5.

    A comfortable ocular prosthesis that looks similar to the sighted, contralateral globe and in the same horizontal and anterior-posterior plane.

Close collaboration between the ophthalmologist and the ocularist is essential in order to obtain the best functional and cosmetic results with an ocular prosthesis in an anophthalmic socket and to reduce the frequency of secondary periorbital procedures. Excellent cosmetic results and long-term control of socket problems are the ideal but may be difficult to achieve in some cases. Secondary procedures are often helpful but can be challenging and unsatisfactory for both the patient and surgeon. Successful anophthalmic socket surgery is achieved when the anophthalmic patient obtains a painless, non-inflamed eye socket with adequate volume restoration and an artificial eye that looks and moves almost as naturally as a normal eye. When the patient reports that their friends and colleagues “don’t know which is the prosthetic eye,” a primary goal of surgery has been achieved. Yearly follow-up examinations with the ocularist for prosthesis polishing and implant fit evaluation, as well as with the ophthalmologist to examine any socket issues that are more easily addressed when identified at an early stage (e.g., implant exposure) are important. This chapter focuses on the clinical assessment of the anophthalmic socket and some of the common problems and complications encountered following enucleation and evisceration surgery.

Changes Associated with Anophthalmic Socket

In addition to the absence of the globe, the anophthalmic socket has other anatomic differences from a normal orbit. Postoperative socket changes begin a complex sequence of interrelationships that affects both the appearance of the socket and the function of the socket with a prosthesis. Progressive intraorbital volume loss following an enucleation or evisceration procedure results in rotary displacement of the orbital contents from superior to posterior and from the posterior to the inferior orbit [10, 11]. There is retraction of the superior rectus/levator muscle complex, a downward and forward redistribution of the orbital fat, and an upward movement of the distal end of the inferior rectus muscle resulting in a shallow inferior fornix and potential tilting of the prosthesis. The superior sulcus may deepen, and upper eyelid ptosis may develop (Fig. 60.1a–d).

Fig. 60.1
figure 1

An 80-year-old female presented with difficulty wearing her prosthesis. The eye had been lost 50 years earlier. Following enucleation without a socket implant, she was fit with a stock eye that had a fairly good color match to the normal eye and felt comfortable. It fit well until the last few years when it would spontaneously fall out. (a) The right socket is enophthalmic with a very deep superior sulcus as a result of volume loss. (b) The superior edge of the prosthesis is tilted posteriorly, and the inferior edge is pushed up against the lower lid as a result of the rotary migration of the orbital tissue. (c) The superior and inferior fornices are shortened, and the inferior fornix shows a bulge as a result of the inferior rectus/oblique muscles migrating forward. (d) The stock eye does not have a custom fit and is small and lost its luster. It has not been changed or polished in over 50 years

Progressive lower eyelid laxity of senescence may be aggravated by the weight of an artificial eye. Loss of lower eyelid support and inferior fornix volume may make fitting and wearing of the custom prosthesis more difficult. Insufficient volume replacement, rotational socket changes, and tissue laxity associated with aging result in the “post-enucleation socket syndrome” or “anophthalmic socket syndrome ” (deep superior sulcus, upper eyelid ptosis, an enophthalmic appearance, lower eyelid malposition) and may require a larger than desirable prosthesis [12,13,14,15,16].

Proper implant volume at the time of enucleation or evisceration may be determined either preoperatively or intraoperatively (enucleation cases) from the axial length of the eye or by determining the volume of fluid the enucleated eye displaces in a graduated cylinder [14,15,16]. Approximately 70–80% of the volume of an individual’s normal globe ideally should be replaced with an orbital implant [14, 16]. This generally allows for a prosthetic volume that is 2.0–2.5 ml [13]. While the upper limit of prosthetic volume is approximately 4.0 ml, larger prostheses often result in progressive lower lid laxity and malposition due to the weight of the prostheses on the eyelid and the projection of the anterior surface of the artificial eye. Larger prostheses may also have limited socket excursion [14]. The preoperative or intraoperative calculated implant size is often greater than 22 mm [14,15,16]. Unfortunately, implants larger than 22 mm may have a higher exposure rate and if too large will hinder fitting of an acceptable custom prosthesis [14, 16]. In most adults, we typically use 20–22 mm spherical implants following enucleation and 18–20 mm implants in evisceration procedures. Individualization of the implant size is important in optimizing orbital volume replacement and in achieving the best possible functional and aesthetic results [13,14,15,16].

Fabrication, Care, and Maintenance of the Artificial Eye

Following enucleation, evisceration, or secondary orbital implant surgery, an acrylic (polymethyl methacrylate) conformer is placed in the conjunctival fornices to maintain the conjunctival space during the early postoperative healing phase. An anophthalmic socket without a conformer may be at risk for contraction, potentially compromising placement of an adequately sized prosthesis. The conformer is replaced with a custom-made ocular prosthesis typically fashioned in 6–8 weeks following socket surgery. Prefabricated or “stock eyes” (still used in underdeveloped countries) are unsatisfactory as they are generally not cosmetically optimal, limit motility, may trap secretions between the socket and prosthesis, and may not position well in the socket resulting in rotation and extrusion (Fig. 60.1d). The ideal prosthetic eye is custom fit to the dimensions of the conjunctival fornices of each individual patient using the “modified impression technique ” [17]. An impression of the socket is taken in a similar fashion to that used in fitting dentures. The initial impression is made of a paste-like material composed of highly refined alginate mixed with water [17]. Once the impression material sets to a firm consistency, the shape is copied into a wax mold. A prepared iris-cornea piece is polished on the front surface of the wax pattern. The wax mold is placed into the socket and reshaped for comfort and to improve cosmesis. The wax shape is then changed (using additional molds) into fine-quality acrylic (from methyl methacrylate resin) painted, cured, and polished. The patient’s remaining eye is used as a template to match the size and shape of the pupil, iris color, as well as superficial vascular network on the sclera, episcleral, and conjunctiva [17].

The time required for an individual patient to get used to the prosthetic eye is variable [18]. Some will be accustomed to it in a matter of hours; others may require days to weeks, and a few never adjust comfortably. Ideally the prosthetic eye should not be removed from the socket unless a foreign body (e.g., eyelash) is being removed or the prosthesis is being professionally cleaned or adjusted. If the artificial eye is left out while sleeping or for other reasons, it should be stored in soft contact lens saline solution. If the prosthesis is allowed to remain dry, the painted layers may separate and degrade.

Pine et al. have recently proposed a three-phase model that describes the response of the socket microenvironment to prosthetic eye wear [19,20,21,22,23]. The three phases include an “initial phase” of wear of a new (or recently polished) prosthesis when homeostasis is being established (or reestablished) within the socket (conjunctival mucous coating of the prosthesis and tear balance); a second homeostatic phase, the “equilibrium phase ” when beneficial surface deposits that facilitate wettability of the prosthesis buildup on the prosthesis and wear is comfortable; and a third phase or “breakdown phase ” where there is an increasing likelihood of irritation from continued wear. The proposed model also provides a rationale for prosthesis cleaning to help manage non-specific mucoid discharge [19].

The initial phase of wear when physiological homeostasis is being established within the socket is approximately a month [19]. The prosthetic eye then enters an equilibrium phase when a homeostatic period exists, and the artificial eye is comfortable to wear and discharge is minimal - mild. The length of time beyond this homeostatic phase (or equilibrium phase) when the microenvironment starts to break down varies between individuals but is several months (generally 6–12 months) in duration. Artificial eye patients usually know when their equilibrium phase is ending as the artificial eye starts to have increased discharge and some irritation [18]. Frequent removal and manipulation of the artificial eye (e.g., daily or weekly) never allow the artificial eye and socket microenvironment to enter the homeostatic or equilibrium phase when it’s comfortable to wear the prosthetic eye. As a result, discharge is often more of a problem [19]. Frequent removal of the artificial eye also roughens the fine polished surface of the prosthesis and may lead to microtrauma of the conjunctiva with resultant socket irritation and discharge.

If the prosthetic eye has to be removed by the patient (e.g., to remove lashes trapped behind it or other foreign bodies), proper cleaning and handling of the prosthetic eye are important. Cleaning (by the patient) approximately every 6 months is also suggested as prosthetic deposits accumulate continuously and after 6 months of wear may be thick enough to irritate the conjunctiva and cause discharge [19,20,21]. The ideal cleaning regimen for most individuals will also be influenced by medical conditions (e.g., allergies), the wearing environment (e.g., dust, dirt, wind), and the standard surface finish of the prosthesis [19]. Although numerous cleaning techniques have been suggested over the years [18, 24,25,26,27,28] (many of which are contradictory), Pine et al. have recently reported that simply wiping all surfaces of the artificial eye under cool tap water with a wet paper towel is simple and works extremely well to clean the surface deposits that build up on the artificial eye over time [19,20,21]. This can easily be done by the patient in almost any environment should he/she have to remove the prosthetic eye. Dry paper towel or tissue (e.g., Kleenex) is ineffective in removing deposits and may wear away the highly polished surface of the prosthesis creating a rougher surface with a dull appearance. Similarly, solvents such as alcohol will damage the acrylic surface. The prosthetic eye patient should also return to their ocularist at least yearly to have the artificial eye assessed for damage, reassess correct fit, and have it “professionally cleaned and repolished to optical grade contact lens standard.” [19,20,21] Repolishing by the ocularist’s buffing instruments thoroughly removes deposits, microscratches as well as providing a smoother surface that not only looks better but also allows smoother movement of the eyelids over the prosthesis, decreasing conjunctival irritation and associated mucous production. Gradual changes to the eye socket such as fat atrophy and laxity of the upper and lower eyelids may cause rotation or malposition of the prosthetic eye. Minor adjustments in the shape or thickness of the artificial eye may provide the patient with a more comfortable fit and improve cosmesis [19]. Yearly checkups with an ophthalmologist allows assessment for post-enucleation socket syndrome or other complications (e.g., implant exposure) that are easier to repair if identified early.

Living with a Prosthetic Eye

It is important for prosthetic eye patients to avoid focusing on the presence of their prosthesis. An unhealthy level of self-consciousness can lead to chronic anxiety [3,4,5,6,7]. The ocularist and ophthalmologist have a critical role in helping the patient to cope with their disability by keeping a natural-appearing prosthetic eye and comfortable socket. The patient’s primary care physician and or psychologist/psychiatrist may also play a role in helping the monocular patient adjust to life with an artificial eye [6, 7].

All ocular prostheses inherently have some limited motility. It is beneficial for patients to learn to turn their head and shoulders in the direction of gaze to maintain primary ocular gaze and minimize ocular asymmetries present with horizontal and/or vertical gaze. Facial expressions, such as smiling, animate the periorbital muscles and distract attention from the artificial eye [7].

Polycarbonate safety lenses should be worn whenever possible to protect the remaining, functioning eye. This is particularly important when the patient is involved in sports activities, using machinery, high-speed drills, etc. Spectacles with a light tint may also help minimize imperfections in the artificial eye, as well as camouflage asymmetries of the superior sulcus and eyelids. Cosmetic optics, involving plus (magnification) or minus (minification) lenses, are useful in altering the apparent size of the prosthesis and palpebral fissure. In addition, prisms in the spectacles may be used to alter the perceived position of the prosthesis.

Management of Anophthalmic Socket Problems

Socket Dryness

Tear production (basic and reflex) as well as meibomian gland secretion gradually decreases over time in the anophthalmic socket leading to burning and irritation within the socket in some individuals [29,30,31]. Mucous secretion from the conjunctival goblet cells may increase as a compensatory mechanism and not uncommonly is interpreted as an infection by the patient. Artificial tears or gel applied throughout the day is very helpful. If tear supplements do not relieve dry eye symptoms, a drop of “light” mineral oil (a laxative purchased at any pharmacy) can be used on the artificial eye surface to allow the eyelids to glide smoothly over the prosthesis. Topical silicone oil (e.g., Sil-Ophtho – Stony Brook, Inc., Davenport, Iowa) may also be used and is often available through ocularists’ offices.

Discharge and Irritation

Mucoid discharge with prosthetic eye wear often develops to some degree with time and can be a distressing condition that affects the quality of life of people who have lost an eye [19,20,21,22, 27, 28]. Alterations in tear production following enucleation or evisceration and the presence of a foreign body (the artificial eye) stimulate mucous secretion from the conjunctival goblet cells (see earlier) simulating infection. Frequent handling of the prosthesis may retard the normal prosthesis socket microenvironment development and also result in damage to the highly polished prosthetic surface [19, 20]. Similarly, a prosthesis older than 5 years may develop an irregular surface leading to further mucous production. A poorly fit prosthetic eye may also have a sizeable dead space between the posterior surface of the prosthesis and the conjunctiva where mucoid debris (i.e., stagnant tears, dust particles, eyelashes) often accumulates giving the patient the impression they have a socket infection [32,33,34].

When assessing the prosthetic eye patient with discharge, it is important for the physician to determine the age of the prosthesis and the patient’s routine of prosthetic hygiene. Minimizing handling of the prosthesis should be encouraged. Prosthesis irregularities (e.g., nicks, scratches) and poor surface quality (loss of luster) are usually obvious at the slit lamp or when the prosthesis is taken out to assess the eye socket (Fig. 60.1d). Lubrication of the prosthesis should be evaluated as socket dryness with burning is not uncommon and artificial tear gels or other lubricants can be suggested. Frequency of ocularist’s visits should be determined, and appropriate intervals of ocularist care encouraged. The ocularist will evaluate the surface of the prosthesis for nicks and scratches as well as assess the fit of the artificial eye, being sure it is the appropriate size and that there is no dead space between the prosthesis and orbital implant where mucous and other debris can accumulate. Most patients require annual polishing of the prosthesis, but some sockets require semiannual polishing of the artificial eye to smooth the surface and remove the protein deposits. The average life of an artificial eye is typically 5–7 years but varies from socket to socket and is impacted by the frequency and method of a patient’s socket hygiene routine. If discharge is still a problem after assessment by the ophthalmologist and ocularist, then treatment with a mild corticosteroid drop (i.e, fluorometholone (FML)) or a combined antibiotic/steroid drop (e.g., tobramycin- dexamethasone) once or twice daily often diminishes the discharge.

When more excessive discharge is present, a conjunctival infection (viral or bacterial) should be considered. Usually there are accompanying signs of acute or chronic conjunctivitis including edematous eyelids, conjunctival chemosis, hyperemia, and mucopurulent discharge in the conjunctival fornices. Conjunctival cultures are helpful in guiding appropriate antibiotic treatment of bacterial infections.

There is also a subset of anophthalmic patients that have an exceptionally deep superior fornix, chronic conjunctival discharge, and ptosis [34]. The deep fornix and low-grade conjunctival inflammation lead to buildup of a protein exudate that becomes colonized by bacteria leading to recurrent discharge [33, 34]. Rose described this “giant fornix syndrome” and suggested a cycle of progressively worsening symptoms that may not be responsive to frequent topical and or systemic antibiotics [33, 34]. Jones et al. proposed eliminating the potential space harboring the chronic mucopurulent coagulum by shortening the fornix and concomitantly repairing the ptosis through a posterior surgical approach [34]. The superior conjunctivoplasty-Mullerectomy procedure resulted in significant improvement in their patients’ chronic socket discharge [34].

A pyogenic granuloma may also be the cause of recurrent or chronic socket discharge. Intermittent bleeding is often reported, and these vascular lesions are typically present along the conjunctival closure site or around an implant peg (Fig. 60.17b). Pyogenic granulomas are a sign of local irritation or microtrauma and may also indicate an underlying implant exposure and/or infection [34, 35].

Recurrent and chronic socket discharge is also a distinguishing feature of giant papillary conjunctivitis or “GPC ” [36, 37]. The etiology of GPC is not fully understood but is believed to be an immunologic reaction to an antigen present on the surface of the prosthesis. Giant papillae (>1 mm) on the tarsal conjunctiva of the upper eyelid is the hallmark of GPC (Fig. 60.2a, b). In severe cases, GPC may significantly limit the amount of time the patient can wear the prosthesis. Treatment can be difficult and may involve corticosteroid eye drops in conjunction with allergy drops. Topical cyclosporine eye drops or tacrolimus 0.03% ointment may also be helpful [38,39,40]. In recalcitrant cases, carbon dioxide laser or cryoablation of the giant papillae can be attempted. Rarely, a prosthesis made of a different material (e.g., glass) may be considered.

Fig. 60.2
figure 2

(a) A 30-year-old male with a 5-year history of wearing an artificial eye presented with recurrent discharge and droopy left upper lid. (b) When the eyelid was everted, giant cobblestones typical of giant papillary conjunctivitis were seen along the tarsal plate

Lagophthalmos with Exposure of the Prosthesis Surface

Some individuals with nocturnal lagophthalmos may develop unsightly dried matter on the anterior surface of the prosthesis that results in the eyelid margin and/or lashes adhering to the anterior surface of the prosthesis. As the eyelid attempts to open upon awakening, it is pulled away from the prosthesis surface causing discomfort and irritation and contributing to conjunctival inflammation. Sometimes the debris can be cleaned without removing the prosthesis by rinsing the surface of the prosthetic eye with a soft contact lens irrigating solution or several drops of an artificial tear drop. If this is not successful, the artificial eye can be removed from the socket, and the debris wiped off the artificial eye surface under cool tap water using a wet paper towel [19,20,21]. Light mineral oil or a lubricating eye ointment (e.g., Lacri-lube) can be used at bedtime on the anterior surface of the prosthesis if debris buildup and eyelid sticking are a chronic problem.

Socket Pain

Pain and discomfort in the anophthalmic socket are uncommon but may range from mild to severe with resulting effects on daily life [37, 41,42,43,44,45,46,47,48]. A wide variety of problems can cause socket pain and discomfort, most of which can be diagnosed through a careful history and clinical examination of the prosthesis and eye socket (Table 60.1). Prosthetic care and handling should be assessed as well as the fit of the artificial eye. The socket implant should be evaluated for signs of migration and the conjunctiva for signs of inflammation, infection, or implant exposure. Migration of the implant may alter the fit of the prosthesis creating pressure on the tissue between the implant and prosthesis. Trochlear inflammation and pain (radiating along the superior orbital rim or into eye socket) secondary to the prosthetic edge hitting the trochlear area on a recurrent basis is also a consideration [42]. Palpation of the trochlea recreates the discomfort experienced by the patient. Triamcinolone injection (0.3–0.4 ml of 40 mg/ml vial) into the peritrochlear area may be very effective in resolving this type of discomfort. If an evisceration was performed, recurrent scleritis or postherpetic neuralgia is an uncommon cause of recurrent socket pain to consider. Patients with healthy-appearing sockets may be more challenging and rare etiologies (e.g., phantom eye pain, drug-seeking behavior, secondary gain, factitious) should be considered [2, 36, 48]. Persistent socket pain warrants neuroimaging (computerized tomography, magnetic resonance imaging) of the orbit, paranasal sinus, and brainstem to rule out an amputation neuroma, sinus infection or mass, or an intraorbital or central nervous system abnormality [2].

Table 60.1 Causes of socket pain

Anterior Orbital Cysts

The development of a subconjunctival/anterior orbital cyst is occasionally seen and may be responsible for socket discomfort (aching, pressure sensation) as well as problems with the fit of a patient’s prosthetic eye (Fig. 60.3) [49, 50]. These cysts arise from buried conjunctival epithelium that becomes incarcerated during wound closure. Management options include complete surgical excision of the cyst, marsupialization, absolute alcohol injection, and more recently trichloroacetic acid (TCA) injection [51,52,53,54,55,56]. Simple aspiration is not an adequate treatment on its own as recurrent cyst formation often occurs shortly after evacuation (days).

Fig. 60.3
figure 3

A 60-year-old female presented with a pressure sensation in the left eye socket. Removal of the prosthesis revealed a prominent subconjunctival cyst centrally

Eyelid Malpositions

Lower eyelid laxity is a common problem that occurs with time in many artificial eye patients and results from the aging eyelid supporting the weight of the prosthesis as well as alterations of soft tissue support associated with removal of the eye (anophthalmic socket syndrome) (Fig. 60.1a–d) [10,11,12]. Lower eyelid laxity can be corrected with a tarsal strip-type procedure [57,58,59]. Horizontal lid tightening will help maintain the natural contour of the lower eyelid and lateral canthus and provide improved support for the prosthesis without sacrificing conjunctival tissue or inferior fornix volume. Some patients will also develop an inadequate inferior fornix secondary to anterior migration of inferior orbital fat and thinning or disruption of the suspensory ligament of the inferior fornix, resulting in anterior rotation of the lower edge of the prosthesis (Fig. 60.1b) [60,61,62]. The conjunctiva is not deficient but requires repositioning with suture fixation of the conjunctiva to the orbital rim periosteum to recreate the fornix [62,63,64].

With significant lid laxity, ectropion of the lower eyelid may develop. This may initially present as an aesthetic issue but eventually leads to difficulties with prosthesis retention. A tarsal strip-type procedure and in some a posterior lamellar spacer graft (e.g., auricular cartilage) may be required [57,58,59,60]. Occasionally there is a deficiency of anterior lamella (i.e., the skin) as well, requiring a myocutaneous transposition pedicle flap from the upper eyelid to the lower eyelid. If inadequate adjacent tissue is present for a transposition flap, a full-thickness skin graft may be indicated.

Eyelid margin entropion and vertically directed lashes are common in the anophthalmic patient in both the upper and lower eyelids, often resulting from contracture of the inferior or superior fornix. Fornix contracture as a result of surgery to remove the eye, alkali−/acid-induced scarring, or other trauma to the forniceal tissues and natural contracture of the forniceal conjunctiva over time (especially in those sockets with chronic inflammation or infection) are potential causes of eyelid margin entropion and vertical lashes. Eyelid rotational sutures or a marginal tarsotomy-type procedure can be very effective to rotate the misdirected lashes (Fig. 60.4a, b) [63–67]. More advanced cases may require posterior lamellar lengthening with a spacer graft (e.g., mucous membrane, auricular or nasal cartilage, hard palate mucosa, acellular dermis) (Figs. 60.5a–d and 60.6a–d) [60,61,62].

Fig. 60.4
figure 4

(a) Vertically directed upper and lower eyelashes. (b) Three weeks post tarsal fracture surgery with lid margin rotation. The eyelashes are in a more normal position

Fig. 60.5
figure 5

(a) A 42-year-old female was seen with inability to retain her prosthetic eye due to a shortened inferior fornix. A mucous membrane graft was harvested from the lower lip with a Castroviejo handheld mucotome seen in Fig. 60.19. (b, c) The mucous membrane graft was sutured along the inferior tarsal border and remaining forniceal conjunctival edges toward the ball implant. The fornix was reformed with the aid of 4–0 slik sutures tied through a silicone band that was left in place for 4 weeks. (d) Three months postoperatively, the patient was able to retain her prosthesis without difficulty. The lower eyelid was in a normal position

Fig. 60.6
figure 6

(a, b) A 64-year-old female was seen with a retracted right lower eyelid as a result of inferior fornix shortening. (c) A hard palate graft was placed along the lower edge of tarsus and sutured in place. (d) Three months post-surgery, the right lower eyelid is in a much more normal position

Upper eyelid ptosis may result from inadequate implant size, migration of the orbital implant, a poorly fit prosthesis, laxity of the fibrous connective tissue framework in the superior orbit (including Whitnall’s ligament) with rotation of the orbital tissues posteriorly and inferiorly (post-enucleation socket syndrome), and trauma from the original injury/surgery or senile dehiscence of the levator aponeurosis [68,69,70]. The mechanisms producing anophthalmic ptosis are multifactorial and should be assessed carefully before surgical repair to achieve optimal functional and aesthetic results. Ocular prosthesis augmentation (artificial eye with a superior extension or flange) is a conservative nonsurgical method to correct mild anophthalmic upper eyelid ptosis. Levator-Mueller’s muscle involutional changes may be corrected with either an anterior or posterior approach (Figs. 60.7a, b, 60.8a, b, and 60.9a, b) [70]. A superior conjunctivoplasty-Mullerectomy has been beneficial in those with an exceptionally deep superior fornix, ptosis, and chronic discharge (giant fornix syndrome) [33, 34].

Fig. 60.7
figure 7

(a) A 40-year-old female was seen with ptosis and enophthalmos of the left socket. Since her orbital implant was put in as a child and was rather small, it was elected to put in a larger implant as a first step to restore socket volume followed by an anterior approach levator advancement ptosis correction 3 months later. (b) Six months post-surgery, there was much more symmetry present between the two sides

Fig. 60.8
figure 8

(a) A 20-year-old male is seen with a right upper eyelid ptosis. The socket volume was fine (b). Three months following an anterior approach levator advancement procedure, there is much more symmetry

Fig. 60.9
figure 9

(a) A 55-year-old male is seen with a right upper lid ptosis. The socket volume was fine. (b) Three months post anterior approach levator advancement surgery and fabrication of a new prosthetic eye, there is much more symmetry

Deep Superior Sulcus and Enophthalmos

The socket soft tissue and volume changes that occur following enucleation or evisceration surgery causing upper eyelid ptosis and lower eyelid malposition also contribute to deepening of the superior sulcus [10, 11]. Initially, following enucleation surgery and to a lesser degree after evisceration surgery, there may be an excellent cosmetic result that becomes less satisfactory over time. The implant may shift posteriorly and inferiorly due to the weight of the implant and progressive laxity of the orbital connective tissue framework. Combined with some rotary displacement of the orbital soft tissue from the superior to the posterior and from the posterior to the inferior orbit, the sulcus deepens, and the eye socket appears sunken (enophthalmic) [10, 11]. Inadequate volume replacement (too small an implant or no implant) at the time of the enucleation or evisceration procedure and postsurgical orbital fat atrophy are other important factors in the development of a deep superior sulcus [10, 11, 13,14,15,16].

Conservative management of a superior sulcus deformity includes camouflage with lightly tinted spectacles or a weak plus lens (+1 to +2) that adds some magnification and helps make a narrow palpebral fissure appear larger. Alterations in the prosthesis can provide additional eyelid support and volume and help improve eyelid position and fullness. Surgical options include correcting bone defects from old orbital fractures, socket volume restoration (e.g., orbital floor wedge implant), and superior sulcus tissue grafting (dermis fat grafting or acellular human dermis graft – AlloDerm® – Lifecell Inc., Woodlands, Texas, USA, or DermaMatrix – Synthes, West Chester, PA, USA) [71,72,73]. Acrylic floor implants (polymethyl methacrylate) are usually adequate for orbital volume augmentation (Figs. 60.10a–c and 60.11a, b), but other materials have been utilized including bone grafts, silicone beads, hydroxyapatite, silicone, teflon, supramid sheets, and dermis fat as well as a variety of injectable products such as autogenous fat, injectable hydroxylapatite (Radiesse), cross-linked collagen, silicone oil, self-inflating hydrogel pellet expanders, and hard tissue replacement polymer [74,75,76,77,78,79]. Soft tissue fillers (e.g., hyaluronic acid-derived products) are a recent alternative to correct superior sulcus volume loss in the anophthalmic socket [29]. Some anophthalmic patients with deep superior sulci, enophthalmos, as well as various eyelid malpositions will require more than one surgical procedure to restore acceptable symmetry (Figs. 60.12a, b, 60.13a–d, and 60.14a–g).

Fig. 60.10
figure 10

(a) A subperiosteal floor implant (“Coderre implant,” Oculo-Plastik, Inc., Montreal, Quebec). (b) A 35-year-old male presented 1-year post evisceration with a deep superior sulcus on the right side. (c) Three months post orbital floor implant, there was excellent symmetry

Fig. 60.11
figure 11

(a) A 49-year-old male presented 3 years postenucleation with a deep superior sulcus. (b) Three months post orbital floor implant, there was excellent symmetry

Fig. 60.12
figure 12

Socket volume restoration, ptosis surgery, and lower lid retraction surgery. (a) A 35-year-old female was seen for assessment of enophthalmos, left lower lid retraction, and left upper eyelid ptosis. (b) An orbital floor implant was put in place first (Fig. 60.10a) followed in 3 months by a hard palate graft to the left inferior fornix, followed in 23 months by an anterior approach levator advancement procedure. (b) Four months following her last surgery and an adjustment to her artificial eye was happy with the symmetry between the two sides

Fig. 60.13
figure 13

(a) Same patient as Fig. 60.7 presented 7 years later with additional enophthalmos. An orbital floor implant (Fig. 60.10a) was suggested to augment the socket volume. (b) The patient is seen 3 months following the floor implant with further improvement to the sulcus and symmetry. The lower eyelid was also slightly down, but the patient did not want to correct it. (c) She returned 5 years later to have the left lower eyelid raised. An ear cartilage graft was placed in the lower lid. (d) Three months following the last surgery as well as a new prosthesis, the left lower eyelid was higher, and there was much more symmetry to the two sides

Fig. 60.14
figure 14

(a, b) A 40-year-old male was seen with a very deep superior sulcus. A subperiosteal floor implant was considered, but the patient elected to have a dermis fat graft. (c, d) The dermis fat graft was harvested from the inside of his upper arm. The epithelium was removed, and the graft was placed just anterior to orbital septum and posterior to orbicularis. (e) Immediately after the procedure, the fullness to the sulcus is evident. The graft is being held in position with three bolster sutures coming through the skin. (f, g) Six months post-surgery, the superior sulcus is filled out nicely

Secondary Orbital Implantation

Enophthalmos may occur from fat atrophy secondary to orbital soft tissue changes following enucleation or evisceration, inadequate implant volume replacement, or implant extrusion. Insertion of a secondary intraconal orbital implant is a consideration to correct the enophthalmos and superior sulcus depression (Fig. 60.7a, b). Secondary orbital implantation may also be indicated to improve prosthesis motility or when implant exposure or migration has resulted in prosthetic fitting difficulties [80,81,82,83,84,85].

Secondary orbital implant surgery is often more complicated than enucleation or evisceration principally due to the disruption of anatomic planes with disorganization of the orbital tissues. In addition, varying degrees of fibrosis are often present throughout the orbital tissue as a result of the initial trauma or primary anophthalmic socket procedure. A variety of techniques for secondary orbital implantation have been described [80,81,82,83,84]. While secondary orbital implantation can be carried out without attempting to identify the rectus muscles, localization of the rectus muscles will typically help reposition the implant in a more anatomically correct position and improve socket motility [84].

Implant Migration

Implant migration may occur with long-standing orbital implants as a result of the progressive orbital soft tissue changes that occur with time in the anophthalmic socket [10, 11]. An early enucleation technique by Frost-Lange involved imbricating the extraocular muscles over unwrapped polymethyl methacrylate (PMMA) or silicone spheres [63]. Muscle imbrication over the anterior surface of a nonporous, non-wrapped alloplastic implant (e.g., polymethyl methacrylate, silicone) was a common practice many years ago and a frequent predisposing factor to “up and out” or “down and in” implant migration over time [85]. It is now well established that this technique leads to implant migration so it has largely been abandoned. Attaching the rectus muscles in their normal anatomic position to nonporous, wrapped spheres (e.g., PMMA, silicone) results in a very low migration rate with implants that are stable over many years. Nonporous alloplastic implants should not simply be placed within the orbit without some type of muscle fixation to stabilize their position.

Porous orbital implants were initially thought to decrease the chance of implant migration, but they also can migrate up and out or down and in. If one also considers anterior implant migration, the rate of implant migration with porous implants may be higher than nonporous implants as this type of migration manifests as implant exposure. These cases are then often grouped with other cases of implant exposure due to a variety of other factors (see below). Anterior migration is most often secondary to improper seating of the porous implant. Porous implants have a rough surface and drag tissue inward as they are placed into the orbit. The overlying tissue may be closed successfully over the implant, but with time, the tissues dragged inward may try to return to their original relaxed position. As this occurs, a gradual migration of the implant anteriorly with progressive conjunctival thinning and eventual tissue breakdown over the anterior implant surface (exposure) occurs. An implant inserter, tissue glide, and/or implant wrap may help avoid the posterior drag of anterior tissue.

A migrated implant may lead to poor motility and an uncomfortable, poorly positioned prosthesis. In mild cases, refitting the prosthesis may help socket motility and comfort. Alternatively, the migrated implant may have to be removed, and a secondary implant centrally positioned within the muscle cone with reattachment of the rectus muscles may be required [80,81,82,83,84].

Implant Exposure and Extrusion

Implant exposure may occur with any type of implant days to years following anophthalmic surgery and may lead to implant infection, extrusion, or explantation (Fig. 60.15a–c). It was anticipated that porous orbital implants would have a lower incidence of implant exposure than traditional nonporous implants because of their associated fibrovascular ingrowth. However, complications gradually became apparent with their widespread use over the past 30 years, and implant exposure is the most concerning problem with reported implant exposure rates ranging from 0% to 50%, causing some surgeons to avoid the use of porous implants [41, 42, 46, 86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111,112,113].

Fig. 60.15
figure 15

(a) Exposed polymethyl methacrylate implant. (b) Exposed aluminum oxide orbital implant with multiple hairs on the implant surface. (c) Exposed aluminum oxide orbital implant 5 years post evisceration surgery

Factors predisposing to implant exposure include closing the wound under tension, poor wound closure techniques, infection, mechanical or inflammatory irritation from the spiculated surface of the porous implant, and delayed ingrowth of fibrovascular tissue with subsequent tissue breakdown [109]. Appropriate implant sizing and placement along with proper tissue closure are of primary importance in helping to prevent porous implant exposure [48, 98, 109, 113,114,115].

A variety of techniques are available to manage implant exposure. For nonporous implants, implant removal and secondary implantation are usually required [84]. For porous implants, management may include wound reclosure early on post-surgery (first few weeks) with or without a patch graft (e.g., sclera, temporalis fascia), vaulting of the prosthesis, and watchful waiting [115]. Persistent and or large implant exposures may require additional surgery. A variety of graft and flap techniques have been advocated to repair implant exposure including Tenon’s flaps , bipedicle conjunctival flaps, tarsoconjunctival pedicle flaps, scleral patch grafts, temporalis fascia or fascia lata grafts, hard palate mucosa, or dermis fat grafts as well as other techniques [116,117,118,119,120,121].

Implant Infection

Infection of porous implants is a rare complication that may be difficult to treat without implant removal [36, 122,123,124,125,126]. Factors predisposing to infection include early conjunctival dehiscence with implant exposure, poor or delayed vascular ingrowth secondary to chronic illness (e.g., diabetes, immunosuppression, vasculopathy), chemotherapy, radiation therapy, prior socket reconstruction, or delayed fibrovascular ingrowth within a host scleral shell with no portals for vascular ingrowth [36]. Initial symptoms and signs are not always indicative of implant infection. Recurrent discharge, for example, may indicate implant infection but is also a common problem for some artificial eye patients without an infectious process. The constellation of socket findings including persistent mucopurulent discharge despite antibiotic coverage, recurrent pyogenic granuloma (indicative of implant exposure), and socket discomfort (aggravated by touching the implant) should raise suspicion for an implant infection (Fig. 60.16a, b) [122, 123]. Recurrent pyogenic granulomas are often an indicator of small conjunctival dehiscences with underlying porous implant exposure [36]. These areas of tissue breakdown may allow entry of the causative bacteria before complete implant vascularization occurs (i.e., within the first 6 months following surgery). Alternatively, bacterial colonization of the implant may occur during initial surgical implantation. The eyelid margin is the most likely source of surgical infection, despite air fluid exchange of the implant in an antibiotic solution (e.g., bacitracin 500 units per ml) prior to implant placement. As the bacteria within the implant multiply and migrate to the surface, a conjunctival dehiscence occurs as well as a pyogenic granuloma. Once the infection becomes loculated, the pyogenic granulomas are the likely sites where bacteria migrate from within the implant to the conjunctival surface, explaining the persistent conjunctival inflammatory reaction, despite the topical application of numerous types of antimicrobial drops [36].

Fig. 60.16
figure 16

(a) A 40-year-old male was seen in consultation 17 years following placement of a hydroxyapatite implant with eye socket discomfort, recurrent purulent/bloody discharge, and pyogenic granulomas. The prosthesis was difficult to retain. A peg had been in position for 15 years. (b) The socket revealed the presence of a polycarbonate peg with sleeve, gross inflammation of the entire conjunctiva, as well as a pyogenic granuloma around the peg. In addition, there was almost no superior fornix, and the superior tarsal plate was folded on itself. Implant infection was suspected. Implant removal and placement of dermis fat graft were suggested to help expand the conjunctival space

Small areas of porous implant exposures should be treated at any time with topical antibiotics and observed closely for signs of implant infection if spontaneous conjunctival wound closure does not occur. Implant infection typically requires implant explantation with the risk of rectus or levator muscle damage or oculomotor nerve injury as well as other socket tissue disruption that may limit subsequent reconstructive options [122,123,124,125,126]. Implant removal is unfortunate as it can be very destructive to the socket tissues, and it eliminates the primary advantage of the porous orbital implant, i.e., the potential for improved implant and prosthesis motility when the prosthesis is coupled to the orbital implant through a peg coupling subsystem.

Complications of Implant Pegging

One of the main advantages of porous orbital implants (e.g., hydroxyapatite, aluminum oxide, porous polyethylene) is the ability to directly integrate them with an overlying artificial eye through a pegging system (Fig. 60.17a). By coupling the orbital implant to the artificial eye via a peg, a wider range of prosthetic eye movements (as well as the fine darting eye movements seen during conversational speech) is possible. More dynamic artificial eye movement imparts a more life-like quality to the prosthetic eye. Infrared oculography has demonstrated objective and significant improvement in horizontal gaze after motility peg placement [127]. Despite the improved motility, many surgeons and patients elect to avoid peg placement due to the satisfactory results without pegging and the risk of post-pegging complications [128,129,130,131,132,133,134,135,136,137,138].

Fig. 60.17
figure 17

(a) A nice looking titanium peg sitting within an aluminum oxide orbital implant . The conjunctiva is well opposed to the peg shaft without any sign of inflammation. (b) A large pyogenic granuloma surrounds a titanium peg. (c) A smaller pyogenic granuloma sitting just off the temporal side of a titanium peg. (d) The severe angulation of this peg makes the coupling with the prosthesis difficult. (e) Exposure of the peg sleeve and adjacent aluminum oxide orbital implant. (f) Accumulation of a black deposit leached away from the titanium peg is seen in the conjunctiva surrounding the peg

Potential problems and complications associated with pegging include increased discharge, recurrent pyogenic granulomas, implant exposure around the peg, implant infection, tissue overgrowth, peg dislocation, clicking noises, and various fitting problems (Fig. 60.17b–d) [128–138]. Refitting the prosthesis, scleral patch grafting, peg removal, and at times implant removal may be required [128].

Although pegging has declined dramatically over the past 10 years or more, a precise and meticulous technique under local anesthesia with or without intravenous sedation in the appropriately selected patient can be a successful outpatient procedure [136, 137]. It is important to be selective in deciding which patients are candidates for a peg system. Proper care of the artificial eye and regular follow-up visits with the ocularist and ophthalmic plastic surgeon are important to help ensure minimal problems with the peg system. If the patient is unlikely, unable, or unwilling to maintain the postoperative visits required, then pegging should be avoided. Children under 15 years of age, the elderly (those over approximately 75 years of age), or individuals of any age with a chronic illness or vasculopathy (e.g., a collagen vascular disease, sarcoidosis, diabetes mellitus, immunosuppressive therapy, prior orbital radiation therapy, etc.) should not be considered for pegging in the author’s opinion [135].

Acquired Socket Contracture

Acquired socket contracture results from shrinkage and shortening of part or all of the orbital tissues in the anophthalmic orbit, resulting in conjunctival fornices that are inadequate to allow retention of a prosthesis [139, 134]. Socket contracture may occur as a result of several processes including scar tissue associated with the initial injury, poor surgical techniques during previous surgeries (e.g., excessive destruction of conjunctiva, traumatic dissection/cauterization within the socket), multiple socket operations (poor vascular supply), prior ischemic ocular disease, alkali/acid burns, cicatrizing conjunctival diseases, radiation therapy (plaque or external beam), chronic socket inflammation, infection, not wearing a conformer or prosthesis, and a poorly fitting prosthesis.

Prevention is always an important consideration, so during enucleation, evisceration, and orbital trauma surgeries, it is essential to preserve tissue (e.g., conjunctiva), minimize dissection into the fornices, limit cauterization, restore tissues to their anatomic position, and utilize a conformer in the postoperative healing period. It is also important to recognize that each successive surgical procedure may cause further tissue trauma to the socket, with disruption of the already compromised vasculature, resulting in further potential contracture of the socket.

The goal of treatment in socket contracture is to identify and correct the underlying cause(s) (when possible), allow the patient to comfortably wear a prosthesis, and achieve the best motility and cosmesis possible. In advanced cases of socket contracture, success may be limited to the ability of the patient to wear a prosthesis, with motility of the prosthesis and symmetry to the contralateral side secondary considerations. No single surgical technique exists for the treatment of the contracted socket, particularly in advanced cases. The management of each case must be individualized to correct the specific problem of the affected socket. While classification of socket contracture has historically been somewhat complex, it can be simplified by dividing cases into mild, moderate, and severe contracture [139, 140].

Mild Socket Contracture

Mild socket contracture involves shortening of the posterior lamella (tarsus, conjunctiva) of the upper and/or lower eyelids. It results in vertical lash orientation and marginal eyelid entropion and is generally not associated with significant loss of the fornices. Retaining a prosthesis is not usually a problem. A transverse tarsal incision (tarsotomy) with marginal rotation is the initial treatment of choice as discussed earlier (Fig. 60.6a, b) [65, 66]. If significant horizontal eyelid laxity coexists, the marginal lid rotation (tarsotomy) may be combined with horizontal lid shortening [67]. If these procedures do not correct the marginal lid entropion and vertical lashes, a spacer graft (e.g., hard palate, ear cartilage) may be required to lengthen the posterior lamella of the eyelid (Figs. 60.12b and 60.13c) [60,61,62].

In some sockets, there may be a decrease or loss of the inferior fornix space while maintaining an adequate amount of conjunctival tissue. This occurs secondary to anterior migration of the inferior orbital fat or thinning/disruption of the suspensory ligament of the inferior fornix [12,13,14,15,16, 60, 62, 67, 141]. The loss of the inferior conjunctival fornix fixation leads to a shallow inferior fornix causing prolapse of the inferior edge of the prosthesis, especially on upgaze, when the soft tissues of the socket shift anteriorly. Often these patients will also have an increase in the horizontal lid laxity of the lower eyelid. A lateral tarsal strip-type procedure combined with fornix reformation/reconstruction using large caliber sutures (2-0, 3-0 polyglactin) passed into the fornix, through the orbital rim periosteum onto the lower eyelid skin where they are either tied directly or over a bolster for 1–3 weeks may help restore adequate volume depth [57, 58, 59, 62, 67, 141].

In those patients with inferior eyelid retraction associated with lower fornix contraction, posterior lamellar lengthening by spacer graft insertion may be required. Homologous, synthetic, and autogenous materials have been advocated as spacers to lengthen the posterior lamella and deepen the inferior fornix [71,72,73, 142,143,144,145,146,147,148,149,150,151,152,153,154,155,157,158,159,160,161,162,163,164,165,166,167,168,169,170,171,172,172]. Homologous materials (e.g., donor sclera) have been used historically but potentially risk infectious disease transmission [166,167,167]. Although synthetic eyelid implants (e.g., porous polyethylene) initially looked promising, they are associated with a high risk of exposure, extrusion, and infection [144,145,146,147,148,149]. Many autogenous materials have been suggested such as spacer grafts including the fascia lata, buccal mucosa (Fig. 60.4a–d), nasal cartilage, hard palate (Fig. 60.5a–d), upper eyelid tarsus, and auricular cartilage (Figs. 60.12a, b and 60.13c, d). The fascia lata and buccal mucosa lengthen the posterior lamella but offer little support to the lower eyelid [150,151,152]. The nasal cartilage and hard palate harvesting prolong surgery time and may be associated with donor site problems [154, 156, 167]. Although upper eyelid tarsus may be useful in correcting retraction of the lower eyelid, the amount of donor tissue is limited and may be insufficient in treating patients with severe fornix contraction. Furthermore, the use of upper eyelid tarsus requires harvesting from the upper eyelid on the affected side or from a healthy eyelid on the contralateral side, potentially resulting in secondary upper eyelid malposition, tarsal kinking, and/or postoperative lagophthalmos [157, 158]. The use of auricular cartilage has several advantages over other autogenous grafts [60, 160,161,162,163,163, 168]. It is an ideal material for lifting the anophthalmic lower lid as it elevates the retracted lower eyelid, lengthens the shortened inferior conjunctival fornix, provides support for the prosthesis, and helps prevent forward migration of the artificial eye (barrier effect) (Fig. 60.13c, d) [60]. Auricular cartilage can be harvested anteriorly from the scaphoid fossa or posteriorly in the area of the helix. Donor site complications may include hemorrhage into the donor space, tenderness/pain secondary to inflammation of the transected cartilage, and occasionally a notch along the helix or a full-thickness auricular defect secondary to tissue necrosis. Unlike the cartilage obtained from the scaphoid fossa area, the cartilage obtained from the helix has a flatter surface in most patients. In either case, it is often necessary to trim the cartilage and score it with partial-thickness incisions (perpendicular to the direction of desired contour) in order to obtain an appropriately contoured piece that will fit. If an excessively ridged or curved piece is placed into the eyelid, it may be unacceptably visible and/or palpable. Two millimeters of cartilage graft for each millimeter of eyelid retraction is generally adequate for correction of the lower eyelid malposition.

Moderate Socket Contracture

Moderate socket contracture involves a more significant contracture and loss of tissue in the inferior and/or superior conjunctival fornix. Inferior fornix contracture is more common than superior fornix contracture. Superior fornix reconstruction techniques are similar to those in inferior fornix reconstruction; however, great care should be taken while dissecting superiorly to avoid injury to the levator complex. The superior fornix may undergo more contraction than the inferior fornix before a significant cosmetic deformity and prosthetic retention issue is produced. A shallow superior fornix will still retain a prosthesis in position; however, eyelid excursions and eyelid closure may be limited by the depth of the superior fornix. With moderate loss of inferior fornix depth, posterior rotation of the superior edge of the prosthetic eye with anterior rotation of the inferior edge allows the prosthesis to easily extrude, even with minimal eyelid movement or touching of the eyelid (Fig. 60.1a–d).

The goal of treatment in the moderately contracted eye socket is to enable the patient to comfortably wear a prosthesis with reasonably good cosmetic appearance. Prosthetic eye motility is often limited even with good operative results. The patient should be counseled regarding reasonable expectations, and that additional surgical procedures may be required. Significant contracture and loss of conjunctival surface area usually require a tissue grafting procedure. Amniotic membrane when tissue loss is mild or mucous membrane grafting (MMG) when more significant amounts of tissue are required are the most common tissues used for moderately contracted fornices [151, 152, 174,175,176,177,178,178]. Other suggested tissues have included dermis fat grafts [180,181,181], skin grafts [182, 183], and forearm free flaps [185,186,187,188,189,190,190]. Skin grafts are not ideal as they lack moisture, have a rough surface, and contain eccrine/apocrine/sebaceous glands and a keratinized epithelial surface that continues to turnover and slough; all contributing to a foul odor with increased socket discharge. In addition, shrinkage of the skin grafts may require repeated surgical intervention over time to maintain adequate forniceal volume needed for prosthetic retention [191].

In moderate socket contraction, mucous membrane transplantation remains the gold standard [152, 175,176,177,178,178]. Oral mucosa from the buccal area or the mucous membrane part of the lower lip is preferred and may be obtained either freehand or with a mucotome (e.g., Castroviejo dermatome) (Fig. 60.18a–c). Partial-thickness mucous membrane grafts are preferable as they are thinner and a closer match to the lost conjunctival tissue. The ideal thickness is 0.4–0.6 mm. Care is required to avoid the lip margin, gums, and opening of the parotid duct (Stenson’s duct) adjacent to the second molar tooth. The fat and muscle layers of the oral mucosa should not be violated. Any submucosal tissue is removed from the graft. Grafts should be about 40–50% larger than the anticipated host defect to allow for anticipated graft contracture following implantation. Mucosal grafts are secured to the recipient conjunctiva with absorbable sutures at the graft edges followed by placement of a large conformer to secure the graft against the underlying vascularized socket tissue. Quilting sutures applied to the center of the graft will ensure better apposition with the recipient bed [193,194,194]. The donor area on the inside of the cheek is closed with a 4–0 chromic suture, while any mucosa harvested from inside the lip is left to heal by secondary intention. Once the mucous membrane graft is sutured in place and a conformer has been inserted, it is essential to place the tissue within the socket on stretch. Two or three temporary horizontal mattress tarsorrhaphy sutures (i.e., 4–0 silk) are placed across the upper and lower eyelids using a red rubber catheter or cotton bolster to limit skin erosion. The tarsorrhaphy sutures should be left in place for 4–5 weeks or as long as they maintain adequate tension. In a socket that has undergone prior irradiation, chemical or thermal injury, or extensive trauma, the tarsorrhaphy is left in place longer than 4 weeks. If the sutures loosen or erode through the eyelid, the tarsorrhaphy may need to be replaced to ensure adequate eyelid closure and tension. With placement of large mucous membrane grafts, a silastic stent (e.g., 240 retinal band) can be positioned in the sulcus of the inferior fornix and anchored to the adjacent periosteum, with both arms of a double-armed suture passed in horizontal mattress fashion through the stent, the deepest part of the fornix, the periosteum of the inferior orbital rim, and finally through the full thickness of the eyelid (Fig. 60.4a–d). Cotton, silicone, or red rubber bolsters are used when the sutures are tied on the skin surface, anchoring the stent securely in the inferior fornix. These sutures may be removed in several weeks after adequate fibrosis has occurred between the inferior fornix and periosteum. A conformer must be in place until a prosthesis is custom fitted. The only downside to full-thickness mucous membrane grafting is that it may be thick and bulky and the produce mucous may create an annoying film over the prosthetic eye.

Fig. 60.18
figure 18

(a) Castroviejo mucotome for harvesting mucous membrane grafts . (b) The inside of the lower lip is a good donor source of mucous membrane. (c) A thin piece of mucous membrane is ready for implantation

Amniotic membrane tissue (AMT) has been used as a graft in various conjunctival and orbital reconstructive procedures including those due to chemical burns, pterygium excision, symblepharon release (e.g., cicatricial pemphigoid, Stevens-Johnson syndrome), conjunctival fornix reformation, and in the repair of exposed orbital implants [196,197,198,199,199]. Amniotic membrane is a “substrate graft ” in contrast to oral mucosa which is considered a “substitute graft.” [191] Substrate grafts require healthy conjunctival epithelial cells to differentiate and multiply over the amniotic membrane surface. Substitute grafts are replacement grafts and provide the epithelial cells. They require an underlying vascular bed to supply them. Amniotic membrane grafts do not provide any epithelial cells. Instead, they promote the migration of the adjacent conjunctival epithelial cells over the amniotic membrane graft surface by secreting various growth factors and providing a basement membrane [200]. In addition, they have an antifibrotic and anti-inflammatory effect, as well as antimicrobial activity [191, 201,202,203,203]. Immunologic rejection has not been reported after AMT grafting [201]. AMT is best suited for those anophthalmic sockets with mild to moderate socket contraction. Advantages include its availability, lack of donor site morbidity, and antifibroblastic activity [202]. Comparable and favorable outcomes have been shown with either mucous membrane graft or amniotic membrane grafts [201, 202]. Further study is needed to determine the exact amount of recipient conjunctiva that is adequate for a successful outcome.

Dermis fat grafts may also be an option in patients with moderate socket contraction as well as socket volume deficiency (enophthalmos, deep superior sulcus) [179, 204, 205]. A dermis fat graft can improve orbital volume, as well as increase the conjunctival surface area [179, 204, 205]. As with amniotic membrane grafts, some normal recipient conjunctiva is required for a successful outcome. A healthy, vascularized socket is also required as the dermis fat graft is a free graft and its viability depends upon host fibrovascular ingrowth. Following placement of a dermis fat graft, the largest conformer that can be retained and permit eyelid approximation with a temporary tarsorrhaphy (4-0 silk horizontal mattress sutures over red rubber catheter or cotton bolster) should be inserted to limit postoperative tissue contracture. The tarsorrhaphy will help keep the eyelids and socket conjunctiva on traction for at least 3–4 weeks. Conjunctival epithelium will migrate over the anterior surface of the dermis fat graft during this time and expand the conjunctival surface area. Without the tarsorrhaphy sutures, progressive tissue contraction may occur (Figs. 60.19a–j and 60.20a–k).

Fig. 60.19
figure 19figure 19

Figure 60.11 – (a, b) – A 60-year-old man was seen in consultation for enophthalmos and inability to retain his prosthesis. On examination, there was enophthalmos and shortened fornices superiorly and inferiorly. It was elected to carry out a dermis fat graft. (c, d) The donor site was marked out over the upper outer quadrant of the buttock. The epithelium was removed leaving underlying dermis. (e, f) The dermis fat graft was harvested. (g) The graft was placed into the socket with a ring-shaped conformer to maintain the fornices. External red rubber bolsters were used to suture the lid margins together. (h) The bolsters have been removed, and the dermis fat graft is visualized. (i) In 2–4 weeks, epithelium grows over the dermis fat graft. (j) The enophthalmos has improved. He was happy with the results and the residual superior sulcus defect

Fig. 60.20
figure 20figure 20

Continuation of patient seen in Fig. 60.16 with suspected infected HA orbital implant. The first step was to remove the implant followed by implantation of a dermis fat graft. (a) A horizontal incision was used to remove the orbital implant and make space for the dermis fat graft. (b, c) The dermis fat graft was harvested and sutured into the socket followed by placement of a conformer. (d) The eyelids were sutured together over red rubber bolsters for 5 weeks and then removed. (e) The dermis fat graft appears healthy and is still being epithelialized. (f) The conjunctival epithelium has grown over the central aspect of the dermis fat graft by 8 weeks. (g) The patient is fit with a new prosthesis and has some residual ptosis. (h) The tarsal plate is no longer folded, but the superior fornix remains very deficient, and the eyelid was ptotic. An amniotic membrane graft was suggested to expand the superior fornix area. (i) The amniotic membrane graft is virtually transparent and ready to be implanted. (j) After dissecting into the superior fornix with a number 15 scalpel blade and Westcott scissors to create a fornix, the graft is sutured into the superior fornix with 7–0 chromic sutures followed by placement of a conformer. The lids are again sutured together over red rubber bolsters for 4–5 weeks at which time the prosthesis is adjusted. (k) 12 weeks post amniotic membrane graft, the superior fornix has been deepened

Dermis fat grafts should be used with caution in those with a previous history of socket irradiation or in patients with severe or recurrent socket scarring as insufficient orbital vascularity will compromise success [206]. An unpredictable absorption rate is a disadvantage of the dermis fat graft in socket reconstruction.

Severe Socket Contracture

In the severely contracted socket, the conjunctival fornices are nearly absent or may be obliterated making it difficult to hold even a small prosthetic eye (Fig. 60.21a–d). In those that can still wear a prosthesis, enophthalmos with posterior displacement of the prosthesis may be evident along with poor or no prosthesis motility due to the contracted fornices. Retraction of the upper eyelid skin along the orbital roof with loss of the eyelid crease and fold may develop secondary to orbital volume loss. Contracture of the levator-superior rectus muscle complex may also contribute to the upper lid being retracted into an elevated position, even when no prosthesis is in position. Attempts to correct this type of upper eyelid contracture with a new prosthesis often result in a “staring” appearance (Fig. 60.21a, b). In addition to the discomfort and unsightly appearance of a poorly fit prosthesis, these patients often experience discharge and irritation.

Fig. 60.21
figure 21figure 21figure 21

(a) A 66-year-old female was seen in consultation for inability to wear her prosthesis. The eye had been removed following severe corneal exposure with corneal perforation as a result of an upper eyelid resection for basal cell carcinoma. Postoperative irradiation had been used following the lid surgery explaining the atrophic appearance of the upper eyelid tissue. (a, b) Severe enophthalmos is present, with loss of the right upper eyelid margin. (c, d) Loss of most of the upper eyelid and severe deficiency of the conjunctival space. (e) Example of a custom conformer that can be wired to the superior and inferior orbital rim. (f, g) a temporalis muscle transfer was performed with temporalis muscle placed into the eye socket through a tunnel in the lateral wall. (h, i) A custom conformer with a partial-thickness mucous membrane graft from the buccal mucosa was prepared and placed into the eye socket. It was not wired to the superior/inferior orbital rim. (j) The eyelids were sutured closed with 4–0 silk on red rubber catheter material. (k) A custom conformer with a champagne stem was placed in the fornices immediately following removal of the bolsters to try and maintain the fornices before an artificial eye was made. An eyepatch and tape were applied over the protruding stem. This applied pressure to the conformer sitting within the newly created fornices. (l, m) Following fabrication of the artificial eye, the right upper eyelid had a deficiency of the skin, and there was a retracted appearance to the upper eyelid. (n) A skin graft (supraclavicular) was put in place and held there with a sponge bolster. (o) Immediately following the bolster removal, the graft was healing well. (p, q) The patient is seen 12 weeks post skin graft placement and had a much more natural and symmetrical appearance. She was able to keep her prosthesis in place

Reconstruction of the severely contracted eye socket is often challenging. Patients should be counseled regarding the substantial challenges to achieve an acceptable outcome and the likelihood of several staged procedures being required. The primary goal in the treatment of severe socket contracture should be to enable the patient to retain a prosthesis with reasonable comfort and acceptable cosmetic appearance given their limited socket soft tissue. However, repair of severe socket contracture may also end in disappointment in the patient as well as the surgeon if too much surgery is attempted at once.

Complete socket reconstruction for severe contracture (loss of conjunctival fornices and orbital volume deficiency) often requires complex procedures with a multidisciplinary approach involving a craniofacial plastic or head and neck surgeon as well as an oculoplastic specialist (Fig. 60.21a–q).

If volume deficiency is not an issue, buccal mucosal donor grafts are used but may not be of sufficient size to cover the entire socket. If enough mucous membrane can be obtained, wire fixation (e.g., 30 gauge) of a conformer to the inferior and superior orbital rims may be helpful to offset the contractive forces that are often present during the healing phase in these sockets (Fig. 60.21e, h) [207]. A split-thickness mucous membrane graft sutured to itself around a custom conformer with superior and inferior holes in it is our preferred technique (Fig. 60.21i) [207]. The wires are threaded through the conformer holes and bone tunnels superiorly and inferiorly, and gentle tension is applied to them. The skin and subcutaneous tissue are closed in layers. The wires are then twisted together on top of the skin incision over silastic, cotton, or red rubber bolsters. The eyelids are approximated and also tied together with temporary tarsorrhaphy sutures on red rubber or cotton bolsters (Fig. 60.21j). These bolsters are removed in 3–6 weeks unless they erode through the eyelid sooner. Generally, the longer the bolsters are in position, the more favorable the outcome. The wired conformer is removed when the new socket tissue appears quiet (typically 6–8 weeks). A custom conformer is then placed followed by a custom-made prosthesis 1–2 weeks later.

In poorly vascularized, contracted, and volume-deficient (enophthalmic) sockets, temporalis muscle flaps based on the superficial temporal artery may be considered to bring in blood supply to the orbit which will then vascularize an overlying mucous membrane graft (or rarely a skin graft). Mucous membrane is always preferred for reasons outlined earlier (Fig. 60.21f–q) [209,210,211,212,212].

Those patients with severe socket contraction and previous irradiation are extremely challenging [185, 186]. Radial forearm free flaps are a complex reconstructive option for severely contracted sockets [185, 188,189,190,190]. The forearm flap is harvested with dimensions as large as 10 × 20 cm, making it ideally suited for fornix reconstruction. Its vasculature is anatomically consistent during dissection and robust in caliber, and the diameter of the radial artery is comparable with the diameter of the anastomosing vessels in the maxillofacial region [189]. The vascular pedicle is flexible, and its length can be altered to accommodate varying facial anatomy as needed. Because of its multiple perforating feeding vessels, the entire flap can be folded while maintaining adequate perfusion [189]. In contrast to earlier methods where a bone window was used to tunnel a vascular pedicle, the radial forearm pedicle can be tunneled subcutaneously [185, 187]. Anastomosis is made with the facial artery or superior thyroid artery and the external jugular vein [189]. Alternatively, the superficial temporal artery may be used. The disadvantages of this technique include a cosmetically unfavorable forearm donor site, forearm numbness, and a decreased arterial flow to the forearm [189, 190]. Other techniques described to revascularize severely contracted sockets with volume deficiency include a short pedicled thoracodorsal artery trilobed adiposal flap, a retroauricular island flap, and a retroauricular fasciocutaneous flap [214,215,215].

Lastly, in those patients who have undergone multiple procedures with persistent failures, total excision of the residual socket lining with permanent closure of the lids (lid-sparing exenteration) may be indicated as a last resort. Fabrication of an oculofacial prosthesis, matched to the fellow side, in some cases, although immobile, may provide a better cosmetic result than any additional attempts at socket reconstruction in some cases (Fig. 60.22a–i) [216, 217].

Fig. 60.22
figure 22figure 22

(a) An 8-year-old female presented with an inability to retain her prosthesis and enophthalmos. The right eye had been removed due to retinoblastoma at age 1. There had been radiation therapy to the eye socket postoperatively. The child had difficulty retaining the prosthesis and a very retracted upper eyelid giving her a disfigured staring appearance. The superior and inferior fornices were shortened, and the socket was enophthalmic. The child underwent a dermis fat graft procedure to augment the socket volume and improve the fornices. (b) At 1 week, the eyelids were well opposed with temporary bolster tarsorrhaphy sutures. (c) By 3 weeks, one tarsorrhaphy suture had spontaneously released. (d) At 4 weeks, the other bolster sutures were removed; the fornices appeared to be contracting. At 7 weeks, it was elected to carry out socket reconstruction with a custom-made conformer that could be wired to the orbital rim (Fig. 60.21e), in addition to a buccal mucosal graft from the mouth. (e) At 2 weeks post socket reconstruction and 9 weeks post dermis fat grafting, the eyelids were in good position, and the fornices were well formed. (f) Six weeks following the socket reconstruction, the child had a volleyball hit her in the right periocular region. Within a week, socket contraction began. The eyelids are seen retracting superiorly and shrinking in size. (g) Four weeks later, the eyelids reveal further atrophy. The custom-made conformer was removed the following week. (h) Seven months following the volleyball blow, severe socket contraction has occurred with loss of the conjunctival fornices and atrophy of most of the upper and lower eyelids. (i) An oculofacial prosthesis, often used following exenteration, was used to cover the socket

Tinted eyeglass lenses in a full frame pair of glasses may also be used to add camouflage over a reconstructed socket that has less than the desired aesthetic result. The appearance of the prosthesis and eyelids may also be modified by adding a “plus or minus” spectacle lenses to magnify a small reconstructed socket or to minimize an enlarged appearing socket. Prisms can also be used to modify the apparent horizontal or vertical position of a malpositioned prosthetic eye or eye socket.

The Congenital Anophthalmic Socket: Anophthalmos and Microphthalmos

Congenital anophthalmia is an exceedingly rare condition where the optic vesicle fails to develop (Fig. 60.23a–c). Along with cyclopia, anophthalmia is the most severe malformation of the eye. Studies report the incidence of congenital anophthalmia as approximately 0.2–0.6 per 10,000 births [215, 218]. Many cases initially diagnosed as anophthalmos contain remnants of an underdeveloped eye, or vestigial eye tissue, and are more appropriately termed microphthalmos. Congenital microphthalmos is more common than congenital anophthalmos and has an incidence of between 1.2 and 1.8 per 10,000 births [219]. Ultrasound, computerized tomography, or more preferably magnetic resonance imaging (to avoid radiation) may be helpful in determining the presence of these remnants as well as documenting any other bone deformities (Fig. 60.23c).

Fig. 60.23
figure 23

(a) A 1-week-old child is seen with a microphthalmic vs anophthalmic socket. (b) Cotton-tipped applicators are used to open the tiny underdeveloped eyelids. A poorly formed conjunctival fornix was seen superiorly and inferiorly. A small custom-made conformer was placed in the socket. (c) An MRI scan done at 4 weeks of life revealed the underdeveloped left socket, no identifiable ocular remnant, and the presence of the custom conformer. The lack of an ocular remnant confirmed congenital anophthalmos rather than microphthalmos. The size of the bony orbit is already quite a bit smaller than the opposite side

Microphthalmos is a variable condition that is separated into “simple” or “complex” types according to the appearance of the globe. Simple or pure microphthalmos is defined as an eye that is essentially normal except for its decreased axial length. The microphthalmic eye has a diameter at birth of less than 15 mm (normal range, 15–19 mm) [220]. In most cases, the size of the anterior segment is within normal limits, whereas the posterior segment length is significantly decreased. In complex microphthalmos , the anterior and posterior segments of the globe are severely malformed, and the appearance of the globe varies considerably (Fig. 60.24). There may also be an associated coloboma or cystic component (Fig. 60.25a–j) [221,222,223,223].

Fig. 60.24
figure 24

A 2-month-old male is seen with anophthalmos on the right and a microphthalmos on the left. Vision was light perception on the left eye. The palpebral fissures are both underdeveloped (R > L). The bony orbits are also underdeveloped (R > L)

Fig. 60.25
figure 25figure 25

(a) A 4-month-old male born with microphthalmos and cyst formation bilaterally as well as bilateral cleft lip and palate. (b, c) Large subconjunctival blue cystic masses seen bilaterally. (d) By 3 years of age, the eyelids and fornices were increasing in size. The prosthetic eyes were pushed superiorly by the inferior located cysts. The blue cystic masses were also helping to expand the bony orbit but gave the child a “battered” appearance, which was extremely troubling for their parents. (e, f) Axial CT view of cystic masses bilaterally, in (f), small ocular remnants were seen centrally; some calcification was present in the left remnant and to a smaller degree in the right remnant. (g) Coronal CT view of cystic masses bilaterally, the ocular remnants are also present centrally on each side. (hj) The cystic masses were removed at age 3, H, right side, I, left side, J, cysts removed. (k) 2 months post cyst removal, the lids appear near normal. (l) At 3.5 years, the ocular remnants were removed, and 16 mm polymethyl methacrylate implants were put in place. (m) The child’s prosthetic eyes fit much better, and he had a near-normal appearance (mild ptosis on the left). (n) By age 10, the child was doing well; the lid fissures and orbital volumes were symmetrical. He had a small degree of ptosis on the left

Congenital deformations of the eye and orbit such as anophthalmos and microphthalmos vary in severity, and there is a spectrum of disease that exists between these two conditions. Both anophthalmia and microphthalmia may be unilateral or bilateral, and over 50% may be associated with other systemic abnormalities [218, 224,225,226,226]. In the case of unilateral anophthalmia or microphthalmia, there may also be developmental anomalies of the contralateral eye, including coloboma, lens, retina, and optic nerve abnormalities [227,228,228].

Practically, the clinical picture is often similar in both anophthalmos and microphthalmos. The eyelids are minified with deficient palpebral and bulbar conjunctiva and an underdeveloped orbital bone skeleton (Figs. 60.23, 60.24, and 60.26) [220]. The absence of a globe not only retards growth of the socket and eyelids but retards the development of the entire hemiface, impacting the growth of the maxilla, maxillary sinus, and mandible [230,231,232,233,234,234]. The eyelids are usually short both vertically and horizontally (phimosis). The levator muscle may also be absent causing blepharoptosis, and the conjunctival fornices are extremely shortened making placement and wearing of a prosthesis difficult. Without treatment, these patients may develop a significant decrease in the dimensions of the orbital entrance and deficient orbital volume (up to 60%). Orbital soft tissue volume is an indicator of orbital bone growth, and adequate volume replacement is a critical factor in continued orbital growth as well as adjacent facial growth [236,237,238,239,240,241,241].

Fig. 60.26
figure 26

(a) A 3-week-old male child is seen with a severly contracted eye socket. (b) By 6 weeks of age, the eyelids and conjunctival space have more than doubled in size with expanding conformers. (c) Computerized tomography reveals an ophthalmic remnant confirming severe microphthalmos. The orbital bones lag behind in their growth compared to the normal side. (d) By 24 months of age, the eyelids and conjunctival space have increased to about 75% of the normal side. (e) By 48 months of age, further development of the eyelids is present. A 16 mm polymethyl methacrylate implant was put in place shortly after this photo. (f) By 11 years of age, the eyelid fissures have continued to enlarge. (g) By 13 years of age, the eyelid fissures are approximately 80–85% of the normal side

The management of a child with suspected anophthalmia or microphthalmia often involves a number of health-care professionals including a pediatric ophthalmologist, pediatrician, social worker, oculoplastic/orbital specialist, geneticist, and an ocularist [224, 226, 241, 242]. A pediatric ophthalmologist and pediatrician usually carry out the initial assessment in the neonatal period. Early examination by a pediatric ophthalmologist will include both a diagnostic and visual assessment. It is important both eyes are examined as unilateral anophthalmia/microphthalmia eye patients may have other subtler abnormalities such as coloboma, optic nerve hypoplasia, retinal dystrophic changes, or cataract in the fellow eye. An ultrasound of the eye and orbit can be useful to assess the intraocular structures and the presence of an ocular remnant or cyst (Fig. 60.25) and to determine axial length in cases of microphthalmia. Vision is assessed using pediatric vision tests and electrodiagnostic testing if necessary. A flash visual-evoked potential (VEP) will establish if any visual function remains in cases of apparent anophthalmia or severe microphthalmia; a pattern VEP will help establish both a level of visual acuity and detect any optic nerve dysfunction, and an electroretinogram will identify if there is retinal dysfunction [223]. Even children with severe microphthalmia may have some vision, and it is important to establish this as early as possible, especially in bilateral cases, as it will guide the approach to socket expansion [223].

Early assessment by a pediatrician involves a complete history and physical examination searching for clues to the etiology and any associated systemic anomalies. The history should try to establish any relevant gestational factors or family history of other ocular or systemic abnormalities. The child may already be known to have other significant medical problems requiring active management. In the physical exam, particular attention is also focused upon the face, including the ear and palate, the cardiac system, genitalia, feeding difficulties which may indicate esophageal anomalies, and metabolic disturbances which may be due to pituitary underaction. A management plan is then made depending upon any systemic abnormalities identified [223]. Since many conditions that affect ocular development also affect brain growth, imaging of the brain with attention to the midline structures is commonly performed. Magnetic resonance imaging (MRI) is preferable to computerized tomography (CT) since there is higher resolution of soft tissue structures and no radiation exposure. Other testing will depend on the pediatrician’s systemic assessment. Referral to a specialist in genetic counseling is beneficial but done at a later date once the family has adjusted to the situation and the management of the eye socket and other anomalies is under way. The development of the eye is highly complex. It is determined by a sequential and coordinated expression of eye development genes within the developing tissues. Although some individuals with anophthalmia or microphthalmia have relatives with other eye malformations, the frequent lack of clear Mendelian inheritance in these conditions has made identifying the genes for eye development challenging [223, 244].

Once the diagnosis of anophthalmos or microphthalmos (+/−cyst) has been established, it is important to discuss the diagnosis and complexity of the management with the parents. The pediatrician reviews the systemic malformations; the pediatric ophthalmologist discusses the ocular anomalies identified and the visual potential, while an oculoplastic/orbital specialist discusses the eyelid and eye socket problems. The initial meeting with the parents is often difficult. Not uncommonly, the parents have seen many individuals by the time they reach the oculoplastic/orbital specialist and have numerous concerns. Visual potential will be addressed by the pediatric ophthalmologist. Parents are also understandably interested in what can be done to improve their child’s appearance. It is important to explain the complexity of management and guarded prognosis yet remain positive and encouraging. Parents should understand that any potential aesthetic improvement with anophthalmos or microphthalmos relates to early socket expansion utilizing progressively larger-sized conformers (Fig. 60.27). This is followed by an ocular prosthesis and possibly an orbital implant which can provide acceptable results. Eyelid surgery and orbital cysts if present may be required but are usually deferred until the socket is more developed (e.g., 4–5 years of age).

Fig. 60.27
figure 27

(a) Examples of custom conformers of various sizes are used to progressively expand a congenital anophthalmic/microphthalmic socket. These are custom fit every 6–8 weeks and can be fashioned to resemble an eye when there is enough space to retain them (top row). (b) Champagne glass configuration-type conformer. Additional pressure to the fornices can sometimes be obtained by applying an eyepatch and tape over the protruding stem. The limiting factor is whether the child will tolerate the taped eye socket

The ideal management of anophthalmia and microphthalmia remains controversial, and no uniform strategy exists [223, 232, 242, 244]. Treatment of both the soft tissue hypoplasia (eyelids/conjunctival fornices) and bony hypoplasia is important to consider. Patients with phimotic lids and shortened conjunctival fornices all require early tissue expansion. Treatment of the orbital bone deficit may consist of observation only (e.g., microphthalmos with cyst – Fig. 60.25) or in severe cases may involve removal of a vestigial eye followed by placement of an orbital implant (expandable vs nonexpendable) [223, 232, 242, 243].

Treatment of these poorly developed eyelids and sockets should commence as early as possible (i.e., often within the first few weeks of life) and as previously mentioned requires the close collaboration of the ophthalmologist, ocularist, and most importantly the emotionally impacted parents [223, 224, 242, 243]. The eye and orbit develop most rapidly during the first year of life [249]. Seventy percent of the increase of the globe’s volume occurs by 4 years of age and 90% by age 7 and is completed by early adolescence (around age 14) [246, 247]. With respect to orbital volume, 80% of adult orbital volume is reached by 5 years of age in normal pediatric individuals [237]. Orbital growth is completed by 11 years in females and approximately 15 years in males [236, 248]. By contrast, the face even by 3 months is only 40% of adult face size, but by 2 years, it is 70%, and by 5–6 years, 90% of adult size is achieved [249]. Both normal facial and orbital development are affected by a reduction in ocular volume. In anophthalmia and severe microphthalmia, there is underdevelopment of the bony orbit, the eyelids, the conjunctival fornices, as well as the side of the face [229, 230, 233,234,234, 241]. Without intervention, the socket remains underdeveloped, and the ability to wear a prosthesis is compromised. The under development and facial asymmetry become more pronounced as the child grows. The cosmetic deformity that results without intervention can lead to severe difficulties with social interaction. With appropriate treatment, orbital and facial disfigurement can be minimized. The earlier that treatment is initiated, the greater the effect on influencing the growth of the orbit, the orbital soft tissues, and the ipsilateral facial development.

Reconstruction efforts in anophthalmia or microphthalmia are designed to simultaneously expand the affected tissues in several dimensions:

  1. 1.

    Expansion of the eyelids both horizontally and vertically.

  2. 2.

    Expansion of the conjunctival space and forniceal depth.

  3. 3.

    Expansion of the orbital bone volume [250].

Treatment should begin promptly after birth (within weeks) and requires a commitment of time, energy, and resources by the parents, the physician team, and the ocularist [241, 243, 252,252,253,254]. Initial efforts are directed at expansion of the lid fissure as the severe phimosis prevents access to the orbital tissue. Traditionally, the lids and conjunctiva are expanded with a series of progressively enlarging acrylic conformers.

Ocular prosthesis management is critical to minimizing orbital growth retardation and preventing periorbital asymmetries [245, 251, 253, 254]. The ocularist may use a variety of conformers to expand the tissue including “dumbbell”-shaped conformers or “champagne glass configuration” conformer that help transmit pressure to the socket by application of tape over the external component of the conformer (Fig. 60.27a, b). The ocularist may enlarge the conformers weekly for the first few months of life and then every 6–8 weeks thereafter depending on the degree of tissue growth. These progressively enlarging conformers help expand the conjunctival fornices, eyelids, and periocular soft tissues and stimulate orbital bone development [245, 251, 253]. As the conformers get large enough, they can be painted and prepared to resemble an artificial eye to improve the cosmetic appearance (Figs. 60.26 and 60.28).

Fig. 60.28
figure 28

(a) A 5-year-old girl with microphthalmos on the left had numerous expanding conformers since birth. The eyelid fissure height and horizontal length are very symmetrical to the normal side. (b) The microphthalmic left eye is approximately half the size of the normal eye. Both anterior and posterior segments were severely malformed

In recent years, another option to expand the eyelids and conjunctival fornices has involved hydrophilic expanders [223, 256,257,257]. Hydrophilic expanders are available in several sizes and can be placed into the conjunctival fornices or sutured into the underlying socket tissue. The eyelids are then closed over the expander with a temporary tarsorrhaphy, secured with sutures or Histoacryl glue [223, 256]. Approximately 2 months later, this is exchanged for an acrylic conformer, and socket expansion is considered [256].

In the case of mild to moderate microphthalmic eyes where there might be visual potential management, options vary. When the axial length is less than 16 mm, the eye is unlikely to promote normal orbital growth alone, and it is necessary to increase the socket volume early on to prevent asymmetry from becoming more pronounced as the child grows. In this situation, a custom-made cosmetic shell can be fit over the microphthalmic eye to promote orbital growth. Transparent shells should be fitted initially in the case of eyes with a positive VEP or with a near-normal size eye and cornea. Once the child grows and the vision has been established as useful or non-useful, consideration can be given to eye removal or placement of a more permanent custom-made prosthetic eye. The microphthalmic eye generally should only be removed if there are pathologic changes or symptoms that necessitate its removal. It is usually best to leave the eye in position and fit a prosthesis over it since this helps stimulate bone growth as the eye develops. A microphthalmic eye is a good template for an overlying prosthetic eye and usually has excellent motility, some of which may be transferred to a custom-made prosthesis (Fig. 60.28a, b). The question of when to change a clear prosthesis over a unilateral microphthalmic eye with some vision (e.g., light perception) for a custom-made painted prosthesis is difficult. A clear prosthesis allows maximal visual potential of the microphthalmic eye to be reached and the health of the underlying eye to be monitored. Eventually, once a stable visual development is achieved, it is unlikely to be significantly affected if the microphthalmic eye is covered with a custom prosthesis used to provide better cosmesis [223, 258]. A Gundersen conjunctival flap can also be used if the cornea shows intolerance to an acrylic shell or prosthesis. Removal of the microphthalmic eye is a consideration with some reconstructive socket procedures. Some surgeons advocate removal of a non-seeing microphthalmic eye and using a spherical implant, inflatable orbital tissue expander, or dermis fat graft at an early stage (see below). Our current preference is to try and preserve the microphthalmic eye even if there is no visual potential. The advantages of this approach are that the microphthalmic eye is likely to provide some stimulus to palpebral aperture and socket growth, particularly when the microphthalmos is mild or associated with an orbital cyst. Delay in intervention also avoids early invasive surgery with the potential risks associated with orbital implant placement (e.g., infection, migration, extrusion, ischemia).

For patients with anophthalmia or microphthalmia and an orbital cyst, gradual socket enlargement is usually achieved using increasing sized conformers for the fornices in conjunction with the natural expansion produced by the cyst (Fig. 60.25) [248]. The parents may need gentle reassurance that this is the best approach as initially the appearance may be unsatisfactory. The cysts may have a blue appearance subcutaneously which may appear to be ecchymosis and occasionally raise suspicion of child abuse (Fig. 60.28d). By the age of 3–5 years (before entering formal school education), the sockets in many of these cases have developed sufficiently for the orbital cyst to be removed [259, 260]. Once the cyst(s) is removed +/− an ocular remnant, an orbital implant is required to replace the volume deficit remaining. Alternatively, if the microphthalmic eye is left in place, a free fat graft can be placed into the socket to replace the volume loss [261, 262].

Once the eyelids of the anophthalmic or severely microphthalmic eye socket have been opened adequately and the fornices have been expanded, consideration of orbital volume augmentation and the bony skeleton is considered. This is ideally initiated within the first 2 years of life but may vary depending on the child’s eyelid and orbital growth/development as well as family considerations and willingness. Since orbital growth does not cease until around 11–15 years of age, there is still some benefit if orbital expansion begins after infancy. A variety of orbital expanders are available to clinicians to treat the congenital orbital volume deficit including solid spherical implants, inflatable soft tissue expanders, dermis fat grafts, hydrogel expanders, and integrated orbital tissue expanders [264,265,265].

Solid orbital spheres have historically been the primary implant choice; however, inadequate orbital bone growth severely limits the size of the implants that can be placed. While small implants yield marginal results, larger implants risk extrusion [266]. Repeated implant exchanges require numerous anesthetics and surgical trauma to the conjunctiva and socket until skull maturation has occurred [267]. Despite the arduous process of serial replacements, volume disparity often remains apparent, and additional expansion may be required [259]. The inability to keep pace with the bony growth of the contralateral normal orbit also results in delayed development of the ipsilateral hemiface [229, 230]. Porous orbital implants are not recommended in this group of children as it may be difficult to get a large-sized implant in without risking exposure. Porous implants are also not readily replaced without a great deal of trauma to the socket. The real advantage of a porous implant is enhanced motility when they are coupled to a peg. Children are generally not peg candidates as most are unable or unwilling to adequately care for their socket. Polymethyl methacrylate implants are recommended with reassessment of the socket volume and motility at 16–20 years old at which time implant exchange with a larger porous implant may be considered.

Inflatable soft tissue (balloon) expanders are another option to increase the orbital dimensions in children with severe microphthalmos or anophthalmos. They are implanted through a bicoronal flap or temporoparietal scalp approach in conjunction with an orbitotomy [218, 220, 251, 255, 268]. Previous animal studies have shown that the placement of a solid sphere in an orbit results in partial expansion of the bony socket and that a fully inflated serially expanded silicone implant results in growth equal to the normal side [270,271,271]. There are several techniques available, with varying results [218, 220, 251, 255, 268]. Problems with extrusion and lack of control over the direction of expansion have been experienced [218, 272,273,274,275,275]. Frequently, uncontrolled forward protrusion of the expander during inflation will displace the conformer or result in premature extrusion [246, 267, 276]. Evisceration of the microphthalmic eye and using the scleral shell as a barrier may offer improved results [220]. The balloon expanders ideally are placed within the first year of life, but good results have been obtained even at 4–6 years old [220]. The balloon is inflated on a regular basis with saline via an externalized injection/inflation port placed distally from the orbit. The age of the subject at the time of implantation was not the sole determinant of the degree of eyelid and orbital growth in one series [220]. Similarly, severity of the deformity was not a dominant factor. The variable that seemed to most influence the degree of tissue growth was the time required to complete inflation of the expander [220]. A target inflation period of 20–36 weeks, initiated 3 months after expander placement, was associated with a low risk of extrusion [220]. The volume of saline required to reach an expander diameter of 22 mm ideally is divided equally into monthly injections over this period [220]. Advantages of this technique include predictable growth of the orbit, adjacent facial skeleton, eyelids, and conjunctiva. Only two surgical procedures are needed, and the technique does not produce significant conjunctival scarring [220]. However, disadvantages may include the lack of wide availability of the expanders, the need for multiple injections over a fairly rigid time sequence to obtain good results, and the inflation pressures that can sometimes reach 150–200 mmHg which can be uncomfortable for the patient and the parents [255, 275, 277]. To minimize inflation pressure spikes, a pulsatile orbital expander that transmitted steady carotid pulsations to the orbit was suggested but did not gain wide acceptance [278]. Additional risks associated with various expandable implant devices include tissue ischemia, infection, and extrusion [255].

Mazzoli et al. have suggested that an ideal orbital expander should have several characteristics including straightforward placement through a small incision, enlargement over a relatively short time, long-term soft tissue tolerance, avoidance of uncomfortable inflation spikes, resistance to infection, low likelihood of extrusion or inflator malfunction and minimal follow-up, intervention, manipulation, or revision [266].

Dermis fat grafts may also serve as orbital expanders in children [205, 241, 250, 279]. As dermis fat grafts are vascularized structures that grow with the child, they can potentially exert enough orbital pressure and volume to expand both the lids and socket [241, 250]. Dermis fat grafts have traditionally been used most frequently after extrusion of an orbital implant or removal of a migrated implant in adults where there is some loss of conjunctival tissues and shortened fornices. Conjunctival epithelium will migrate over the anterior surface of the dermis fat graft and potentially expand the conjunctival surface area. When used in the anophthalmic or microphthalmic socket, the growth of the fat may be so exuberant in some cases that debulking is required to avoid overexpansion. Placement of dermis fat grafts before the age of 3 years is associated with higher success [241, 250]. Because of the familiarity of the procedure and low risks of complications, this technique is popular and always combined with the serial expansion of conjunctival conformers both before and after the dermis fat graft is placed. Disadvantages of dermis fat grafts include an unpredictable rate of absorption with a resulting superior sulcus deformity and orbital volume deficiency. In addition, there is little or no transfer of eye socket movement to the overlying prosthesis resulting in an artificial eye with little natural motility.

After several years of use in Europe, a self-expanding hydrogel orbital expander has become another option in the anophthalmic/microphthalmic socket [223, 256, 268, 280]. Hydrogels are highly hydrophilic polymers that expand by osmotically imbibing water or tissue fluid [266]. These implantable spheres can absorb up to 2000% of their weight in water and can increase up to 30 times their original volume [245, 255]. The amount and rate of expansion can be engineered and precisely controlled. These characteristics combined with the ability to mold any desired shape and size make it an attractive material to consider in reconstructing congenital anophthalmic fornices and orbits [256, 281,282,282]. Three ophthalmic appliances have become available: a hemispherical-designed self-expanding conformer for conjunctival fornix expansion, an orbital sphere intended to help expand orbital bone volume (when a microphthalmic eye or remnant is not present), and self-inflating pellet expanders for those orbits that have a microphthalmic eye [255, 256, 283, 284]. Each is inserted in the dry, shrunken, and anhydrous state and gradually expand, nearly ten times, over several weeks. The conjunctival hemispherical conformer is placed into the conjunctival fornices, similar to a regular conformer and held in place with temporary suture tarsorrhaphy bolsters and/or tissue glue along the eyelid margins [223]. Normal tear secretion gradually swells the conformer to a maximum size within a few weeks, as it exerts a constant hydrostatic pressure of 20–30 mmHg [255]. Maximum expansion is achieved within 3–4 weeks, although the conformer can be retained for 2–3 months. Once maximum forniceal expansion is reached, a conventional prosthesis can be placed [223, 255, 256]. The orbital expanders are either spherical or pellet shaped. The selection depends upon whether a reasonably sized microphthalmic eye is present or an eye or eye remnant is absent [283, 284]. They come in various sizes and can be placed intraorbitally through a small lateral soft tissue incision, a central conjunctival incision (e.g., post enucleation), or in the case of the pellets, via an intraorbital injection using a customized trocar [223, 255, 256]. Because the hydrogel implants are self-inflating, there is no need for subfascial tracts and remote injection ports so there is less risk of inflator-related complications [255]. The orbital expanders like the conjuctival expanders exert a constant pressure of 20–25 mm Hg. As the hydrogel implant reaches its equilibrium of water content, the expansion forces are reduced substantially, and bone stimulation ceases [245]. The spherical orbital expander may require replacement to a larger size expander if orbital expansion is inadequate with the initially placed expander [256]. Although the hydrogel expander is an appealing alternative to the conventional implant options, the enthusiasm has been tempered by concern about the biomaterial’s long-term fragility and ability to maintain sustained pressure on the orbit [266]. The requisite periodic exchanges to a larger diameter hydrogel sphere to maintain pressure holds little advantage over the conventional method of serial replacement with hard spheres of known geometry [255, 265]. In addition, although the use of spherical orbital expanders and conjunctival forniceal expanders has been described as “safe, simple and almost harmless,” potential complications do exist [256, 266, 285]. The best methods for implantation as well as the intraorbital characteristics and side effect profile have yet to be clearly delineated [285]. In the largest series to date, the authors reported 14 failures with conjunctival socket expanders and 21 failures with orbital expanders out of a total of 127 expanders placed [256]. Unfortunately, the authors did not fully define failure [256]. They did however state that all complications could be corrected with another operation, and there had not yet been an irreparable failure [256]. These implants are foreign materials subject to infection and extrusion and their expansion and may potentially cause tissue ischemia [255]. Migration within the orbit and inadequate support of a conformer or ocular prosthesis are other possible problems [285, 286]. As with any new material or technique, there is always a potential for unintended or unexpected consequences that are not well-known until one use has been analyzed [255]. One unexpected and delayed (10 + years) complication reported with previous hydrogel appliances in the orbit (retinal buckling elements made of MIRAgel – MIRA,Waltham, MA) was a delayed breakdown of the hydrogel product attributed to uncontrolled swelling of the material beyond its initial size [255]. There are several reports of buckle extrusions, intraocular erosion and migration, intraorbital fibrosis, pain, foreign body granuloma formation, and inflammatory orbital pseudotumor as a result [288,289,290,291,292,292]. Although the current hydrogel polymers are reportedly more stable than the MIRAgel implants , it remains unknown how well tolerated this new material will be over the course of a child’s life expectancy [255, 283].

In recent years, Tse et al. developed an integrated orbital tissue expander (OTE) , (distributed by Marshfield Hills, MA) to address limitations of the conventional orbital expansion options that can be implanted using standard oculoplastic surgical procedures [264,265,265]. The OTE differs from earlier injectable orbital expanders as it consists of a flexible “balloon/expander” held in place by a titanium fixation plate that is anchored to the lateral orbital walls by screws (Fig. 60.29a–c). The direction of expansion and maintenance of expander fixation within the orbit are controlled allowing sustained omnidirectional expansion pressure. A 30-gauge needle connected to a 1 cc syringe is inserted into the OTE through an injection port. The injection track seals upon the removal of the needle, and increased pressure can then be applied to the orbit. Multiple surgeries are not required to maintain the OTE, and they can be easily inflated or deflated without surgery. To date, Tse et al. have used this device in nine patients ranging in age from 9 to 108 months [265]. Six of the nine received one subsequent inflation within 6 months of the initial implantation, and none have had more than one subsequent injection [265]. Using orbital CT-determined volumetric change as the primary outcome measure, all patients had an increase in the orbital volume after expander implantation (Fig. 60.29d–g). The orbital tissue expander also induced the growth of the adjacent frontal, maxillary, and zygomatic bones, leading to external improvements of the eyebrow position, cheek fullness, forward projection of the lateral canthal angle, and horizontal eyelid plane alignment [265]. These results validate the principle that the application of sustained biomechanical force to the craniofacial skeleton can achieve bone growth [265]. Three complications were encountered in the group of nine patients [265]. The first was associated with locating the injection port under the conjunctiva during the second inflation. The silicone globe of the orbital tissue expander of the first two patients inadvertently was punctured by the needle when palpation of the rounded tip of the T-plate was misinterpreted as the metal edge of the injection port. The ruptured expander had to be replaced immediately through a transconjunctival approach. The second complication involved the tip of the T-plate which protruded forward in a hypoplastic orbit, requiring conjunctival incision, temporary deflation, and repositioning of the plate. The third complication involved spontaneous deflation of the expander. The implant was removed and replaced. Examination of the expander revealed a small tear in the silicone rubber neck in the area where it was attached to the titanium injection valve [265].

Fig. 60.29
figure 29figure 29

(a) Deflated OTE orbital expander with needle tip sitting within the injection port. (Photos with permission of Dr. D. Tse and FCI Opthalmics, Marshfield Hills, MA). (b) Side view of OTE with needle in injection port. (c) OTE being inflated with saline. (d) Deflated OTE ready for insertion into the orbit of a 3-year-old male. (e) Preop axial Ct view of orbit before expansion. (f) Preop coronal CT view of orbit before expansion. (g) 24 months post placement of OTE, the axial CT scan shows orbital expansion. (h) 24 months post placement of OTE, the coronal CT scan shows orbital expansion

Stimulation of orbital bone growth in congenital anophthalmos carries a significant biomedical and clinical burden. Management of the hypoplastic orbit requires simultaneous treatment of both the soft tissue hypoplasia and asymmetric bone growth [236]. The integrated orbital tissue expander described by Tse et al. appeared to fulfill all the essential criteria of an ideal orbital tissue expander (as described by Mazzoli and associates [266]) with the following added advantages: (1) small skin incision for implant placement, (2) ease of insertion, eliminating a lengthy implantation process, (3) collapsibility of the expander, facilitating insertion through a small opening, (4) no unpredictable implant movement or displacement, [5] sustained and uniform pressure delivered to constituent bones of the orbit without the need for serial implant exchanges, (6) reduced trauma by serial transconjunctival orbital tissue expander inflation with a needle rather than incising the conjunctiva, (7) well-tolerated long-term outcomes, (8) an implantation procedure that is familiar to orbital surgeons who routinely perform enucleation, and (9) a reduced number of procedures needed for effective orbital bone stimulation [265]. Overall, the integrated orbital tissue expander appeared safe and effective in stimulating anophthalmic socket bone growth. Validation of long-term effectiveness will require additional clinical studies with standardized protocol, pooling of data from participating clinical centers, and careful monitoring of adverse events [265]. Unfortunately, technical difficulties in the manufacturing process have yet to worked out, and it is presently unavailable for general use.

Long-Term Management of the Anophthalmic and Microphthalmic Socket

After the initial socket expansion over the first 5 years of life, the prosthesis and the socket require yearly examination. The microphthalmic eyes will also require follow-up with their pediatric ophthalmologist. Microphthalmic eyes may develop angle closure glaucoma, which may cause loss of what vision does exist and can also cause pain [291]. Children with chorioretinal coloboma and their parents should be aware of the increased risk of retinal detachment [293]. Glasses are prescribed for refractive error in the normal eye, for protection, and sometimes for providing lenses to minimize cosmetic defects (e.g., plus lenses to increase the size of a microphthalmic eye or prisms to equalize a height discrepancy).

The parents may wish to receive genetic counseling at some point regarding the risks of another child being affected. This may include chromosomal analysis and testing of particular genes [223]. Associated systemic abnormalities may have major implications for the child and often require considerable input from various pediatric specialties. It is important to reach a comprehensive diagnostic picture at some point as this helps direct future management. The parents are usually anxious to understand the nature of the condition, and coordinated counseling and management from pediatrics, ophthalmology, and genetics will help achieve this.

For those children with bilateral involvement, it is very important that they also receive assistance at an early age from low vision specialists. A social service worker should be involved from the early stages of those children bilaterally affected with anophthalmia/microphthalmia as they can help the family make connections with the various organizations for the visually impaired that will help them to obtain critical communication and survival skills. Early intervention undoubtedly makes a huge difference to the overall development of the child and the emotional well-being of the family [223].

Summary

Anophthalmic surgery is no longer simply about replacing a diseased eye with an orbital implant. There are multiple problems and complications that can occur with time. Ophthalmic surgeons working closely with ocularists must be focused on restoring a natural appearance to the patient’s prosthesis with comfort, some motility, appropriate orbital volume, and eyelids that look symmetrical so they can integrate into the society with a near-normal appearance as possible. A variety of surgical techniques exist for correcting the different problems seen with the anophthalmic socket. Reconstruction of the contracted socket can be quite challenging as tissue loss and socket ischemia are difficult problems to overcome. Surgical techniques continue to evolve in the oculoplastic, craniofacial, and maxillofacial literature. Reconstructing congenitally anophthalmic/microphthalmic sockets poses particularly difficult social and surgical challenges that may result in frustration and disappointment for all concerned. Management of the hypoplastic orbit requires simultaneous treatment of both the soft tissue component (eyelids/conjunctiva) and the adjacent bone. Serial implant exchanges have significant limitations as they require numerous surgeries and have no dynamic component to stimulate bone growth. Although dermis fat grafts have a dynamic component and are popular among ophthalmologists, inflatable tissue expanders have the potential for controlled, gentle expansion of the eyelids, conjunctiva, and orbit. Until recently, the direction of expansion and maintenance of expander fixation within the orbit have been a problem, and migration/extrusion is not uncommon. Although a study by Tse et al. was small, their results with a new integrated orbital tissue expander (OTE) appear to be a breakthrough in the management of the congenital anophthalmic socket [265]. Their integrated orbital tissue expander appears to be safe, stable, and highly effective in stimulating anophthalmic orbital bone growth. Unfortunately, it is not yet available for general use.