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Anesthesia in a pediatric patient with ROHADD syndrome
E Esparza Isasa1, M A Palomero Rodríguez1, I Acebedo Bambaren1
1Servicio de Anestesiología y Reanimación, Hospital Universitario Ramón y Cajal, Madrid, España.
Revista Espanola De Anestesiologia Y Reanimacion
|May 6, 2018
Summary
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome presents unique anesthetic challenges. Careful peri-operative management is crucial for patients with this rare condition.
Area of Science:
- Pediatric Endocrinology
- Anesthesiology
- Rare Diseases
Background:
- Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare condition affecting children aged 2-4 years.
- It involves rapid weight gain, hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation.
Observation:
- A case report details a 10-year-old boy with ROHHAD syndrome experiencing anesthetic complications during port-a-cath insertion.
- The patient's condition presented significant challenges during the general anesthesia procedure.
Findings:
- Peri-operative management of ROHHAD syndrome patients is complex due to multi-system involvement.
- Respiratory comorbidities are frequent and require careful consideration.
Implications:
- Anesthetists must be aware of the specific implications and considerations for managing ROHHAD syndrome patients.
- Tailored anesthetic strategies are essential for optimizing patient safety and outcomes.
Keywords:
Anestesia generalAutonomic dysregulationCentral hypoventilation syndromeDisfunción autonómicaDisfunción hipotalámicaGeneral anaesthesiaHypothalamic dysfunctionObesidadObesityROHHAD syndromeSleep apnoeaSleep disorder breathingSíndrome de ROHHADSíndrome de apnea del sueñoSíndrome de hipoventilación centralTrastorno del sueño
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