Perioperative Care of a Child With ROHHADNET Syndrome

Abstract

ROHHAD syndrome is an acronym, coined in 2007, that outlines a syndrome including r apid-onset o besity, h ypothalamic dysfunction, h ypoventilation, and a utonomic d ysregulation. 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. doi: http://dx.doi.org/10.4021/ jmc1445w