Manejo anestésico em lactente com rara doença: Síndrome Ectrodactilia, displasia ectodérmica e fissura labiopalatina (EEC)

Albeto Freaza Lobão Bastos; Lilian Satie Ykeizumi; Paula Cristina Leitão de Assunção; Cláudia Regina  Machado; Bruno Vítor Martins Santiago

doi:10.15448/1980-6108.2025.1.47396

Authors

Albeto Freaza Lobão Bastos Pedro Ernesto University Hospital. State University of Rio de Janeiro (UERJ), RJ, Brazil. https://orcid.org/0000-0001-6412-1221
Lilian Satie Ykeizumi Pedro Ernesto University Hospital, Rio de Janeiro State University (UERJ), RJ, Brazil https://orcid.org/0009-0003-7334-0612
Paula Cristina Leitão de Assunção Pedro Ernesto University Hospital, Rio de Janeiro State University (UERJ), RJ, Brazil https://orcid.org/0000-0002-0533-9121
Cláudia Regina Machado Faculty of Medical Sciences. Pedro Ernesto University Hospital. Rio de Janeiro State University (UERJ), RJ, Brazil https://orcid.org/0000-0002-9709-7958
Bruno Vítor Martins Santiago Faculty of Medical Sciences. Pedro Ernesto University Hospital. Rio de Janeiro State University (UERJ), RJ, Brazil https://orcid.org/0000-0002-2712-6311

DOI:

https://doi.org/10.15448/1980-6108.2025.1.47396

Keywords:

Infant, EEC syndrome, Pediatric anesthesia, Perioperative management

Abstract

Ectrodactyly, ectodermal dysplasia and cleft lip and palate syndrome (EEC), is a rare congenital genetic disease with autosomal dominant inheritance. This report is the first in the literature from the perspective of the anesthesiologist in relation to the perioperative care of a child with EEC syndrome, undergoing surgical corrections in different sites, at the same surgical time, which makes the conduction of anesthesia challenging, which must enable adequate control of pain and postoperative nausea and vomiting, without delaying hospital discharge. In these cases, pre-operative planning and interdisciplinary integration are essential to anticipate critical steps, in addition to allowing an individualized and comprehensive approach to achieve better results. The use of total intravenous anesthesia, associated with regional anesthesia, can be a systemic opioid-sparing strategy, decreasing emergency delirium and accelerating pediatric recovery.

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Author Biographies

Albeto Freaza Lobão Bastos, Pedro Ernesto University Hospital. State University of Rio de Janeiro (UERJ), RJ, Brazil.

Master of Medical Sciences and MD from the University of Lisbon. Anesthesiologist at the Pedro Ernesto University Hospital, State University of Rio de Janeiro, and the Federal University of Rio de Janeiro, RJ, Brazil

Lilian Satie Ykeizumi, Pedro Ernesto University Hospital, Rio de Janeiro State University (UERJ), RJ, Brazil

Specialist in Anesthesiology from the Brazilian Society of Anesthesiology. Anesthesiologist at the Fernandes Figueira Institute and State Children's Hospital, RJ, Brazil.

Paula Cristina Leitão de Assunção, Pedro Ernesto University Hospital, Rio de Janeiro State University (UERJ), RJ, Brazil

Specialist in Anesthesiology and Pain from the Brazilian Society of Anesthesiology. Anesthesiologist at Pedro Ernesto University Hospital, Rio de Janeiro State University, and the Federal Hospital of Andaraí, RJ, Brazil

Cláudia Regina Machado, Faculty of Medical Sciences. Pedro Ernesto University Hospital. Rio de Janeiro State University (UERJ), RJ, Brazil

PhD in Public Health from the Institute of Social Medicine of the State University of Rio de Janeiro (UERJ). Assistant Professor at the School of Medical Sciences (FCM) – UERJ, RJ, Brazil.

Bruno Vítor Martins Santiago, Faculty of Medical Sciences. Pedro Ernesto University Hospital. Rio de Janeiro State University (UERJ), RJ, Brazil

PhD in Medicine from the Postgraduate Program in Medical Sciences PGCM/UERJ. Anesthesiologist at the Clementino Fraga Filho University Hospital – Federal University of Rio de Janeiro (HUCFF-UFRJ); Professor of Anesthesiology at the School of Medical Sciences of UERJ (FCM-UERJ), RJ, Brazil.

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