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2023

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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General anesthesia in pediatric mastocytosis: how safe it is?

Margarida Gonçalves1, Ana F. Carvalheiro2, Teresa Rocha1

1 Hospital Dona Estefânia, CHLC, EPE, Lisbon, Portugal;
2 Hospitalar do Algarve, Portimão, Portugal.

- 67th Annual Postgraduate Assembly in Anesthesiology (PGA), NYC, EUA, 17/12/2013 (Poster)

Introduction: Mastocytosis is a rare disease with a prevalence of approximately 1:25-30000.It consists of a group of disorders characterized by a pathologic increase in mast cells in tissues. General anesthesia is considered as a high risk procedure in mastocytosis, by the mast cell degranulation hazard on exposure to various stimuli and drugs, in spite of the exact incidence of complications being not known and appearing to have a great variability in patient response.In this report, we contribute to this experience by reviewing the anesthetic management of a pediatric patient with mastocytosis.

Case report: We report a 4-year-old male, ASA III, with cutaneous mastocytosis, admitted for an elective amygdalectomy and tonsillectomy, under general anesthesia (GA). He did never have any symptoms or episodes of anaphylaxis, and no history of prior anesthetic care. He was given methylprednisolone and ranitidine as premedication, and epinephrine was prepared for use in emergency. Orotracheal intubation was performed under GA with sevoflurane, diazepam and cisatracurium. Paracetamol and ketamine iv were used for analgesia. The perioperative period remained uneventful.

Discussion: During the perioperative period, surgical stress and medications may lead to mast cell degranulation with severe systemic involvement. There is no consensus in the literature as to which medications are truly contraindicated. Overall, medications that result in histamine release should be avoided and epinephrine should be prepared.

Keywords: systemic mastocytosis, pediatric, general anesthesia