Background

Cystic hygroma (CH), or lymphangioma, is due to benign aberrant proliferation of lymphatic vessels and is a known disease of childhood, its occurrence is rarely seen among adults.CH can occur anywhere in the body, common in the cervicofacial region, axilla, and groin. [1,2,3]. The etiology of CH is disputed, trauma and respiratory tract infections are proposed as its cause among adults. [4, 5]. Diagnosis is often aided by the use of fine needle aspiration for cytology, and radiological investigations for defining its exact location and its relations to the adjacent vital structures. A complete surgical excision with an intact capsule is considered the gold standard treatment [6], however, various conservative modalities of management are available and are preferred in certain specific conditions.

Here we present a case report of an adult cervical cystic hygroma and the literature review of the same.

Case Presentation

A 30-year-old male presented to ENT OPD with complaints of painless swelling in the left lower anterior part of the neck, insidious in onset and slowly progressed to attain the present size in 2 years’ duration.

On clinical examination, there was a single smooth cystic swelling of 8 cm x 6 cm size in the left supraclavicular fossa, which was, non-tender, fluctuant, mobile, trans-illuminant and non-pulsatile. (Fig. 1).

Fig. 1
figure 1

A single smooth cystic swelling of 8 cm × 6 cm size in the left supraclavicular fossa

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Ultrasonography of the neck showed a cystic lesion in the left supraclavicular fossa of size 7.39 cm × 3 cm with multiple septations, Contrast-enhanced Computed tomography showed an iso-intense, well-circumscribed cystic lesion deep to the left sternocleidomastoid muscle medially and subcutaneous plane laterally, suggestive of cystic hygroma. (Fig. 2A, B).

Fig. 2
figure 2

A & B Contrast-enhanced Computed tomography—axial view showed an iso-intense, well-circumscribed cystic lesion of the lower part of the left supraclavicular fossa of about 7.2 × 6.2 × 5.2 cm in size deep to the left sternocleidomastoid muscle medially and subcutaneous plane laterally suggestive of cystic hygroma

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Fine needle cytology showed a straw-colored fluid with few inflammatory infiltrates composed of lymphocytes in a thin proteinaceous background with no epithelial cells.

After obtaining consent, the patient underwent surgical excision of the cyst under general anaesthesia. A transverse cervical skin crease incision was placed, and a subplatysmal flap was raised, the greater auricular nerve was preserved, and the EJV was ligated. A single cyst of size 8.5 × 6 × 4.5 cm, translucent and multiloculated was identified and delineated from the SCM anteriorly, the trapezius, the scalenus medius, and the levator scapulae posteriorly. The transverse cervical artery was identified and preserved. The cyst was resected completely, with an intact capsule and the wound was closed in layers. (Fig. 3A & B).

Fig. 3
figure 3

A & B Intraoperative finding showing a translucent multiloculated cyst of 8.5 × 6 × 4.5 cm

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Histopathological examination revealed a multilocular cyst with a wall thickness of 0.2 mm, filled with thin serous pale-yellow fluid and lined with flat endothelial-like cells, with no solid areas, necrosis, and haemorrhage, consistent with a diagnosis of cystic hygroma. (Fig. 4A & B).

Fig. 4
figure 4

A & B Histopathological examination revealed a multilocular cavity with a cyst wall thickness of 0.2 mm, filled with thin serous pale-yellow fluid and lined with flat endothelial-like cells, with no solid areas, necrosis, and haemorrhage, consistent with a diagnosis of cystic hygroma

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Post-operatively, the patient recovered well, with no signs of any neurological dysfunction.

Discussion

CH was first described by Reden Backer in 1828 as a tumor of the lymphatic system and the term “cystic hygroma” was coined by Wernker in 1834. [7]. The word hygroma is derived from Greek which means “water tumor” or “moist tumor” because of its contents [8]. The etiology of CH among adults is not very conclusive, a delayed proliferation of lymphoid remnants secondary to neck trauma or respiratory tract infections is said to be the reason for its occurrence [9, 10].

Mulliken et al. has described CH based on their histologic appearance as either capillary lymphangiomas consisting of small lymphatic vessels, cavernous lymphangiomas consisting of larger lymphatic vessels (the most common type), or cystic lymphangiomas consisting of large macroscopic lymphatic spaces containing collagen and smooth muscle. [11] Based on histopathological findings, our case belonged to the cystic lymphangioma type.

CHs may remain asymptomatic in a patient for a long time, but if left untreated, there may be recurrent episodes of infection or haemorrhage within the cyst that can cause sudden increase in size leading to respiratory distress, oesophageal compression, and cosmetic disfigurement. In our patient, there were no compressive symptoms and the cyst was excised because the patient was concerned about cosmetic disfigurement and malignant transformation.

Surgical excision is considered the gold standard treatment for CH, complications due to surgical resection can be hematoma, postoperative seroma, chyle leak, chylous fistula owing to the location of the cyst and injuries to surrounding vital structures depending on its location. In our case we have surgically excised the lesion in-toto with an intact capsule and our patient had uneventful post op period. This patient is on a regular follow up with us and there is no recurrence for the last 1 year.

The likelihood of recurrence is approximately 15–20% with surgery, mainly due to incomplete resection or residual lesion. However, an in-toto excision is known to reduce the incidence of recurrence [12]. The risk of malignant transformation in CH or any lymphangiomas is very rare although clinical follow up is recommended for these patients. [13].

Conservative or combined management is opted in cases of inoperability where the mass involves critical structures such as airways, great vessels, and nerves of the neck that includes simple drainage or aspiration, electrocautery, cryotherapy, embolization, radiation therapy, laser surgery (using solid lasers like Nd: YAG laser or gases like CO2 laser, and semiconductor diode laser), intralesional steroid administration, intralesional sclerotherapy with OK 432 (picibanil), bleomycin, doxycycline, cyclophosphamide, alcohol, and sirolimus. However, there are concerns about the side effects of sclerosing agents [14].

Conclusion

Cystic lesions of the neck are rare among adults and there are many differential diagnosis, CH/ lymphangioma should be considered as one of them. We recommend fine needle aspiration cytology and appropriate radiological investigations to know its cytology and exact location. Although the risk of malignant transformation is unknown, they can have mass effects on vital neck structures. We recommend complete surgical excision whenever possible, and to have a regular follow up to identify recurrence.