Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2013.10.014
Brazilian Journal of Anesthesiology
Clinical Information

Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery

Manejo anestésico de paciente pediátrico com displasia ectodérmica hipoidrótica submetido a cirurgia de emergência

Elif Oral Ahiskalioglu; Ali Ahiskalioglu; Binali Firinci; Aysenur Dostbil; Mehmet Aksoy

http://dx.doi.org/10.1016/j.bjane.2013.10.014 Braz J Anesthesiol, vol.65, n6, p.522-524, 2015
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Abstract

ABSTRACT Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

Keywords

Ectodermal dysplasia, Regional anesthesia, Acute abdomen

Resumo

As displasias ectodérmicas são condições raras, com uma tríade de hipotricose, anodontia e anidrose. Em revisão da literatura há apenas alguns relatos de manejo anestésico de pacientes com displasias ectodérmicas. Hipertermia é um risco muito sério que pode ocorrer por causa de defeito das glândulas sudoríparas. O presente caso envolve uma criança de 10 anos com displasia ectodérmica que se apresentou com abdome agudo e foi considerada para uma cirurgia de emergência. Nosso objetivo foi demonstrar o manejo bem-sucedido desse caso, com o uso de uma combinação de anestesia geral e peridural. É importante para o anestesiologista obter informações sobre essa síndrome, em caso de operações de emergência, pois pode evitar complicações graves e até salvar vidas.

Palavras-chave

Displasia ectodérmica, Anestesia regional, Abdome agudo

References

Pinheiro MF-MN. Ectodermal dysplasias: a clinical classification and a causal review. Am J Med Genet.. 1994;53:153-62.

Irvine AD. Towards a unified classification of the ectodermal dys-plasias: opportunities outweigh challenges. Am J Med Genet.. 2009;149A:1970-2.

Palit AIA. What syndrome is this?. Pediatr Dermatol.. 2006;23:396-8.

Clarke APD, Brown R, Harper PS. Clinical aspects of X-linked hypohidrotic ectodermal dysplasia. Arch Dis Child.. 1987;62:989-96.

Gordon CPLS. Multicore myopathy in a patient with anhidrotic ectodermal dysplasia. Can J Anaesth.. 1992;39:966-8.

Hotta MKT, Umemura N, Takino Y. Anesthetic manage-ment of a patient with hypohidrotic ectodermal dysplasia. Masui.. 2000;49:414-6.

Sugi YHK, Suzuki Y, Shono S. Anesthetic management of a patient with hypohidrotic ectodermal dysplasia. Masui.. 1999;48:414-6.

Docquier MAVF, Prudhomme S, Rossillon R. Anesthesia in a child presenting a anhydrotic ectodermic dysplasia associ-ated with a multiminicore myopathy. Can J Anaesth.. 2000;47:449-53.

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