Anesthesia in a pediatric patient with ROHADD syndrome.

E Esparza Isasa,M A Palomero Rodríguez,I Acebedo Bambaren, C Medrano Viñas,D Gil Mayo, F Domínguez Pérez, D Pestaña Lagunas

Revista Española de Anestesiología y Reanimación (English Edition)(2018)

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摘要
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2–4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients.
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关键词
Central hypoventilation syndrome,ROHHAD syndrome,Obesity,Autonomic dysregulation,Sleep apnoea,General anesthesia,Hypothalamic dysfunction,Sleep disorder breathing
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