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

Summary 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 h...

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Bibliographic Details
Published in:Anaesthesia reports 2022-01, Vol.10 (1), p.e12172-n/a
Main Authors: Puri, S., Yaddanapudi, S., Menon, P.
Format: Article
Language:English
Subjects:
autonomic dysreflexia
Case Report
central hypoventilation
diabetes insipidus
hypothalamic dysfunction
obesity
obstructive sleep apnoea
paediatric anaesthesia
ROHHAD syndrome
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Summary:Summary 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.
ISSN:2637-3726
2637-3726
DOI:10.1002/anr3.12172