Anestesia en paciente pediátrico con síndrome de Rohhad / Anesthesia in a pediatric patient with ROHADD syndrome

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

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 anaesthesia, 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

Anesthesia in a pediatric patient with ROHADD syndrome. / Anestesia en paciente pediátrico con síndrome de Rohhad.

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 anaesthesia, 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.