Brazilian Journal of Anesthesiology
https://app.periodikos.com.br/journal/rba/article/doi/10.1590/S0034-70942010000300010
Brazilian Journal of Anesthesiology
Clinical Information

Anestesia para amigdalectomia em criança portadora de síndrome de Klippel-Feil associada à síndrome de down: Relato de caso

Anesthesia for tonsillectomy in a child with Klippel-Feil syndrome associated with down syndrome: Case report

Magda Lourenço Fernandes; Núbia Campos Faria; Thiago Ferreira Gonçalves; Bruno Holanda Santos

http://dx.doi.org/10.1590/S0034-70942010000300010 Braz J Anesthesiol, vol.60, n3, p.315-320, 2010
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Resumo

JUSTIFICATIVA E OBJETIVOS: Anormalidades craniofaciais, presentes na síndrome de Klippel-Feil (SKF) e na síndrome de Down (SD), podem dificultar o acesso à via aérea. Cirurgias na orofaringe também exigem atenção especial com a via aérea. A associação de ambas as síndromes em paciente candidato à amigdalectomia é uma condição rara, que impõe desafios ao tratamento anestésico-cirúrgico. O objetivo deste relato é discutir os cuidados para a abordagem da via aérea e os riscos da manipulação cervical em paciente portador de SKF e SD submetido à amigdalectomia. RELATO DE CASO: Criança de 5 anos com diagnóstico prévio de SKF, SD e instabilidade da articulação atlantoaxial foi submetida à amigdalectomia sob anestesia geral balanceada. A ventilação sob máscara e a intubação traqueal foram realizadas com a cabeça em posição neutra. A perfeita visualização da epiglote e das cordas vocais permitiu intubação traqueal com laringoscopia convencional. A cirurgia também foi realizada sem extensão cervical, transcorrendo sem intercorrências. CONCLUSÕES: Embora o acesso à via aérea possa ser fácil, alterações anatômicas pressupõem via aérea difícil em pacientes portadores de SKF e SD. Cuidados diferenciados e recursos adequados são obrigatórios para se evitarem complicações durante a abordagem da via aérea. A manipulação cervical deve ser evitada na presença de instabilidade da articulação atlantoccipital pelo risco de lesão neurológica.

Palavras-chave

CIRURGIA, DOENÇAS, DOENÇAS, DOENÇAS, INTUBAÇÃO TRAQUEAL

Abstract

BACKGROUND AND OBJECTIVES: Craniofacial abnormalities present in Klippel-Feil Syndrome (KFS) and Down Syndrome (DS) can hinder access to the airways. Oropharyngeal surgeries also require special attention with the airways. The association of both syndromes in a patient scheduled for tonsillectomy is a rare condition that imposes challenges to the anesthetic-surgical treatment. The objective of this report was to discuss the approach of the airways and the risks of cervical manipulation in a patient with KFS and DS undergoing tonsillectomy. CASE REPORT: This is a 5 years old child with diagnosis of KFS and DS and instability of the atlantoaxial joint who underwent tonsillectomy under general balanced anesthesia. Ventilation under face mask and tracheal intubation were done with the neck in the neutral position. The perfect visualization of the epiglottis and vocal cords allowed tracheal intubation with conventional laryngoscopy. The surgery was also performed without cervical extension and without intercurrences. CONCLUSIONS: Although access to the airways can be easy, anatomical changes presuppose the presence of difficult airways in patients with KFS and DS. Differentiated care and adequate resources are mandatory to avoid complications during approach of the airways. Cervical manipulation should be avoided in the presence of instability of the atlantoaxial joint due to the risk of neurological damage.

Keywords

DISEASES, DISEASES, DISEASES, SURGERY, TRACHEAL INTUBATION

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