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Inicio Revista Española de Anestesiología y Reanimación (English Edition) Anesthesia in a pediatric patient with ROHADD syndrome
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Vol. 65. Issue 9.
Pages 525-529 (November 2018)
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Vol. 65. Issue 9.
Pages 525-529 (November 2018)
Case report
Anesthesia in a pediatric patient with ROHADD syndrome
Anestesia en paciente pediátrico con síndrome de Rohhad
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2
E. Esparza Isasa, M.A. Palomero Rodríguez
Corresponding author
mapalomero@gmail.com

Corresponding author.
, I. Acebedo Bambaren, C. Medrano Viñas, D. Gil Mayo, F. Domínguez Pérez, D. Pestaña Lagunas
Servicio de Anestesiología y Reanimación, Hospital Universitario Ramón y Cajal, Madrid, Spain
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Table 1. Summary of possible complications to be considered in the management of this type of patient.
Abstract

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.

Keywords:
Central hypoventilation syndrome
ROHHAD syndrome
Obesity
Autonomic dysregulation
Sleep apnoea
General anesthesia
Hypothalamic dysfunction
Sleep disorder breathing
Resumen

El síndrome obesidad de rápida progresión, hipoventilación alveolar, disfunción hipotalámica y disregulación autonómica (ROHHAD) es una entidad infrecuente caracterizada por un comienzo en niños sanos a los 2-4 años. Se trata de un síndrome complejo caracterizado por una rápida ganancia de peso con hiperfagia, disfunción hipotalámica, hipoventilación central y disregulación autonómica, entre otros síntomas. Presentamos el caso de un niño de 10 años con diagnóstico de síndrome de ROHHAD a quien se colocó un porth-a-cath bajo anestesia general y que desarrolló complicaciones durante el procedimiento anestésico relacionadas con su enfermedad. El manejo perioperatorio de estos pacientes supone todo un reto para el anestesista dada la afectación de múltiples sistemas y las frecuentes comorbilidades respiratorias que asocian. Se resumen algunas de las implicaciones y consideraciones anestésicas que hay que tener en cuenta en el manejo de estos pacientes.

Palabras clave:
Síndrome de hipoventilación central
Síndrome de ROHHAD
Obesidad
Disfunción autonómica
Síndrome de apnea del sueño
Anestesia general
Disfunción hipotalámica
Trastorno del sueño

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