Screenings – Get electrolytes, BUN, and creatinine. Check for hypercalciuria (which is related to perioperative renal dysfunction). If there’s a history of congenital heart disease, get a comprehensive cardiologic examination.

High risk of difficult airway – Macroglossia is one of the most prominent features of this disease. Be careful administering sedation prior to induction or extubation – especially in pediatric patients. In these cases, the large tongue can obstruct the airway, making it difficult to mask ventilate. Obstruction can become problematic even with an oral/nasal airway applied. Forward and downward traction on the tongue may improve ventilation. Where possible, have a video laryngoscope available.

Thorough airway assessment – rule out cleft palate especially if considering nasotracheal intubation. Macroglossia may require awake intubation utilizing topical anesthesia or an inhalational induction.

Cuffed tracheal airway – These patients are difficult to predict ETT size. The tracheal airway tends to be larger-than-normal. Cuffed tubes are most safe as they will provide airway protection in the event the tube is too small.

Leak Test – These patients have a higher risk for postoperative laryngeal swelling. Make sure to perform a leak test at 20-25 cm of water.

Avoid stressors – these patients are more prone to a metabolic stress response and electrolyte shifts perioperatively

Monitor for signs of hypoglycemia – Hyperinsulinism is sometimes a comorbidity. Get preoperative blood glucose levels and monitor intraoperatively.

Awake extubation – due to high risk of difficult airway and/or large tongue that will fall back and obstruct the airway in a sedated patient.

Polycythemia and hypothyroidism – these are less common comorbidities , but should be assessed preoperatively to avoid problematic bleeding or prolonged wake-ups (respectively).

This condition is a complex syndrome of tissue overgrowth believed to be caused by genetic and environmental factors. Incidence is about 1:13,700 births. Most often, this disease is detected in early infancy.

Symptoms include abnormally large abdominal organs, neonatal hypoglycemia, ear creases pits, adrenocortical cytomegaly and renal immaturity. Because of viseromegaly, these patients have an increased risk of embryonal tumor development.

Associated conditions may include congenital diaphragmatic hernia (CDH), hyperinsulinism/endocrine disorders, WIlms tumor, cardiovascular anomalies (rare)