Perioperative Care of a Child With ROHHADNET Syndrome

Abstract

ROHHAD syndrome is an acronym, coined in 2007, that outlines a syndrome including rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. Common signs and symptoms include obesity, electrolyte imbalance, hypoxemia, thermal dysregulation, and gastrointestinal dysmotility. The high prevalence of neural crest tumors has led to a modification of the acronym to ROHHAD-NET when a neural crest tumor is present. Many of the findings have implications for anesthetic management. We present a 6-year-old girl with ROHHAD syndrome who presented for direct laryngoscopy, bronchoscopy, and adenotonsillectomy. Previous reports of anesthetic care for these patients are reviewed, the end-organ involvement discussed, and options for anesthetic care presented.




J Med Cases. 2013;4(11):710-714
doi: https://doi.org/10.4021/jmc1445w