Peri-operative management of a child with ROHHAD-NET syndrome undergoing neural crest tumour excision.

ABSTRACT

A nine-year-old girl diagnosed with ROHHAD-NET (rapid-onset obesity, hypothalamic dysfunction, hypoventilation, autonomic dysfunction and neuroendocrine tumour) syndrome was scheduled for excision of a large paravertebral ganglioneuroma under general anaesthesia. Her comorbidities included hypothyroidism, diabetes insipidus and autonomic dysfunction. Intra- and postoperative complications included intra-operative hypotension, long surgical time and prolonged postoperative ventilation. Complete weaning from ventilation was initially unsuccessful and she was ultimately discharged on domiciliary nasal BiPAP therapy. The peri-operative care of children with this syndrome is challenging due to the involvement of multiple organ systems. In this report, we describe how pre-operative optimisation, well-planned intra-operative management and intensive postoperative care are essential for a favourable outcome.