Anesthesia in a pediatric patient with ROHADD syndrome

• Published in: Revista española de anestesiología y reanimación 2018-11, Vol.65 (9), p.525-529
• Main Authors: Esparza Isasa, E, Palomero Rodríguez, M A, Acebedo Bambaren, I, Medrano Viñas, C, Gil Mayo, D, Domínguez Pérez, F, Pestaña Lagunas, D
• Format: Article
• Language: eng ; spa
• ISSN: 2340-3284; 2341-1929
• DOI: 10.1016/j.redar.2018.03.005

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.