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A 17-year-old female with a history of untreated hypothyroidism, familial pseudohypoparathyroidism and morbid obesity presented to the emergency room with nausea, vomiting and severe diffuse abdominal pain. After evaluation by the pediatric surgical team, she was scheduled for emergency exploratory laparotomy.

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Preoperative Evaluation

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The patient’s longstanding hypothyroidism was secondary to familial pseudohypoparathyroidism that was also present in her mother, grandmother and maternal uncle. She was diagnosed with these endocrine disorders at age 8 and, due to noncompliance on the part of her family, was never effectively treated or followed. Her past medical history was also significant for developmental delay and morbid obesity (height 60”, weight 117 kg, BMI 50). She reportedly snored, but did not have apneic episodes while sleeping. Of note, she was actively vomiting while being evaluated by the anesthesia team.

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The patient was not cooperative with the physical examination and was unable to lie flat due to dyspnea. She was tachycardia (118/min) and hypotensive (77/36). Her respiratory rate was 18 and her oxygen saturation was 98% on room air. Her airway examination revealed a large tongue, Mallampati Class III airway, and reduced thyromental distance and neck extension. She had very poor dentition, including multiple missing teeth. A large abdominal mass extended above the umbilicus. She had no obvious peripheral veins and her only intravenous access was via a 22-gauge peripheral IV.

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Preoperative laboratory studies revealed hyponatremia (132 mEql/L), hyperkalemia (5.5 mEql/L), hypocalcemia (ionized calcium 3.0 mg/dL) and hypothyroidism (TSH 15.1 mIU/L). The bicarbonate concentration was 21 mmol/L and the anion gap was 12. The hematocrit was 44%. A CT scan of the abdomen revealed a massive intraabdominal cyst.

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Given the combination of a full stomach and a possibly difficult airway, we considered performing an awake fiberoptic intubation. Her developmental delay and uncooperative demeanor, however, precluded this option. We decided to proceed with an inhalational induction with maintenance of spontaneous ventilation.

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The patient was given a bolus of crystalloid in the holding area and her hemodynamics improved. She was also given glycopyrrolate 0.2 mg IV, metoclopramide 10 mg IV, famotidine 20 mg IV and midazolam 2 mg IV and was brought to the operating room.

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Intraoperative Course

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The patient was positioned in a semi-upright position and standard ASA monitors were placed. Induction was first attempted by placing a mask with 8% sevoflurane in oxygen over her face, but she was combative and would not allow a tight seal. She was then given ketamine 110 mg IV in divided doses that enabled her to tolerate the mask. Cricoid pressure was maintained throughout the induction. A slow, smooth induction with maintenance of spontaneous respiration was accomplished, though she did become hypotensive requiring several bolus doses of phenylephrine. After insertion of a second peripheral IV (20-gauge), direct laryngoscopy was performed with a Macintosh 4 blade that revealed a grade II view. A 7.0 mm endotracheal tube was inserted atraumatically on the first attempt and its proper location was confirmed with auscultation and capnography. Rocuronium 50 mg IV was then given and cricoid pressure was released. Anesthesia was subsequently maintained with isoflurane 0.8 ET% in oxygen and air, supplemented by periodic bolus doses ...

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