Keywords

Case Outline

Learning Objectives

  1. 1.

    Discuss risks of anesthesia in patients with Down syndrome.

  2. 2.

    Discuss pre-operative evaluation of patients with Down syndrome.

  3. 3.

    Discuss intraoperative precautions to take with Down syndrome.

Simulator Environment

  1. 1.

    Location: pre-operative area of an adult hospital

  2. 2.

    Manikin setup:

    1. (a)

      Age: adult

    2. (b)

      Lines: none at start of case

    3. (c)

      Monitors: none on patient at start of case

  3. 3.

    Medications available: normal saline, propofol, etomidate, succinylcholine, rocuronium, epinephrine, albuterol, fentanyl, midazolam, ketamine.

  4. 4.

    Equipment available

    1. (a)

      Airway equipment: ventilator, face mask, laryngoscope and cuffed and uncuffed endotracheatl tubes (ETTs) of various sizes, stylets, oral airway, nasal trumpet, laryngeal mask airway (LMA), suction.

    2. (b)

      Monitors: pulse oximeter, blood pressure cuff, 5-lead electrocardiogram (EKG).

    3. (c)

      Lines: arterial line kit, central line kit, peripheral intravenous (PIV) kits

    4. (d)

      Crash cart with defibrillator

    5. (e)

      Paperwork: pre-operative anesthesia history and physical

Actors

  1. 1.

    Scrub tech

    1. (a)

      The scrub tech is busy opening trays and making lots of noise.

  2. 2.

    Circulator nurse

    1. (a)

      The nurse is busy helping to open trays.

  3. 3.

    Surgeon

    1. (a)

      The surgeon is busy dictating the operating report from the previous patient.

Scenario Development

  1. 1.

    Background

    1. (a)

      You are the anesthesiologist starting the case of a 26-year-old boy with Down syndrome, severe developmental delay, history of large ventricular septal defect (VSD) status post repair in infancy, obesity, and obstructive sleep apnea (OSA) noncompliant with continuous positive airway pressure (CPAP) due to discomfort with face mask. He is scheduled to go to the OR for tonsillectomy and adenoidectomy.

  2. 2.

    Phase 1: patient refusal to leave the preoperative area, parent induction

    1. (a)

      In the preoperative area, the patient is calm and quiet, sitting comfortably in the gurney with warm blankets, with mom and dad at bedside. He had refused to allow the nurses to check his vital signs or to place a PIV.

    2. (b)

      The parents ask the anesthesiologist if there is any way they can do the PIV asleep, since he is scared of needles and has been a difficult PIV placement in the past.

    3. (c)

      The learner may offer to do a mask induction and place the PIV asleep, and may consider calling for difficult PIV equipment to be sent to the room ahead of time (e.g. ultrasound, vein finder).

    4. (d)

      When it is time to leave for the operating room (OR), the patient refuses, starts crying, and refuses to let go of his mother.

    5. (e)

      The parents ask the anesthesiologist if it is possible to do a parent induction. They have always done parent inductions in the past. It is their son’s first time receiving surgery in an adult hospital.

    6. (f)

      The learner may offer to do a parent induction. If they choose to do a parent induction, they should provide clear instructions for what to expect during a mask induction and make sure the parent will be calm and willing to leave when asked to do so.

    7. (g)

      With a parent present, the patient will be calm and cooperative.

  3. 3.

    Phase 2: upper airway obstruction and bradycardia during induction

    1. (a)

      During induction, the patient will develop significant upper airway obstruction making mask ventilating challenging.

    2. (b)

      The parent will become anxious and ask what’s wrong. The learner should provide reassurance to the parent that this is expected and under control. The learner should ask the parent to leave so they can focus on patient care.

    3. (c)

      The learner may cautiously place airway adjuncts such as an oral airway or nasal trumpet to assist with mask ventilation, but recognize that the patient may be in stage 2 and develop laryngospasm.

    4. (d)

      The learner may administer CPAP by closing the airway pressure release valve and holding pressure using the ventilator bag.

    5. (e)

      The learner should try to establish IV access as soon as possible but avoid triggering laryngospasm during stage 2.

    6. (f)

      The PIV placement will be challenging and require multiple attempts. During attempted PIV placement, the patient will become bradycardic.

    7. (g)

      The learner should recognize the bradycardia and discontinue the nitrous oxide, decrease the sevoflurane, and take over ventilating the patient manually via bag valve mask with 100% fraction of inspired oxygen (FiO2).

    8. (h)

      A PIV will be placed and the learner will proceed with intubation uneventfully.

Scoring Rubric

Table 15.1 Scoring rubric for case scenario on Down Syndrome
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Summary of Clinical Teaching Points

What are the chances that you will take care of a patient with Down Syndrome? [1, 2]

  • Down Syndrome is the most prevalent genetic disorder worldwide: 1 in 800 live births; >350,000 people in the United States.

  • It is associated with multiple congenital anomalies that can affect every major organ system.

  • Risk factors: maternal age >35 years old; exposure to pesticides and electromagnetic fields; smoking; drinking alcohol; thyroid autoimmunity; exposure to radiation and anesthetic agents

What are the potential challenges to airway management in Down Syndrome patients? [1, 2]

Table 15.2 Potential challenges to airway management in Down Syndrome patients by upper and lower airway anatomy
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Table 15.3 Potential airway complications and management techniques in Down Syndrome patients by intra-operative and post-operative periods
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What are cardiac considerations when caring for Down Syndrome patients? [1, 2]

40–50% of patients with Down Syndrome have some type of congenital heart disease. The following table provides a cursory overview of common heart defects.

Table 15.4 Cardiac anesthetic considerations for Down Syndrome patients
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What are the indications for spontaneous bacterial endocarditis (SBE) prophylaxis? [1, 2]

  • Dental surgeries or other “dirty” surgeries (not for all routine Urology cases).

  • Prosthetic cardiac valve or prosthetic material used for cardiac valve repair.

  • Prior infective endocarditis.

  • Congenital heart disease:

    • Unrepaired cyanotic congenital heart disease, including palliative shunts and conduits.

    • Completely repaired congenital heart disease with prosthetic material or device, whether by surgery or catheter intervention, that were repaired within the last 6 months.

    • Repaired congenital heart disease with residual defects at the site or adjacent to the site of prosthetic patch or prosthetic device (which inhibit endothelialization).

  • Cardiac transplant recipients who develop cardiac valvulopathy.

What are neurologic and musculoskeletal considerations for patients with Down Syndrome? [1, 2]

  • Atlanto-occipital instability

    • 20% incidence

    • Ligamentous laxity of atlanto-axial joint → C1-C2 subluxation → spinal cord injury

  • Minimize degree of neck flexion, extension, and rotation, especially during intubation

  • Careful intraoperative positioning

  • Review neck radiographs pre-operatively, where indicated

    • Not routinely done

    • Symptoms that should raise concern for possible cervical spine instability and prompt imaging pre-operatively: neck pain, gait disturbances, hand dysfunction, dizziness, bowel/bladder dysfunction, weakness, paresthesias, pain with neck flexion and extension, hyperactive distal tendon reflexes, ankle clonus, muscle weakness, increased muscle tone, neck discomfort, abnormal gait, difficulty walking, excessive laxity of other joints (fingers, elbows, knees)

  • Consider using cervical collar intraoperatively if there is demonstrated cervical spine instability

What are gastrointestinal considerations for patients with Down Syndrome? [1, 2]

  • High risk of gastroesophageal reflux disease (GERD)

    • Vomiting

    • Esophagitis (chest pain, anemia, irritability)

    • Respiratory: apnea, coughing, wheezing, aspiration pneumonia

  • Common associated congenital defects

    • Duodenal atresia

    • Tracheo-esophageal fistula

    • Hirschsprung disease

    • Imperforate anus

  • Consider rapid sequence induction where indicated

What are other systemic considerations for patients with Down Syndrome? [1, 2]

Table 15.5 Other anesthetic considerations for patients with Down Syndrome by organ system
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