Bart’s sendromunda anestezi yönetimi: Bir olgu sunumu

Year 2022, , 114 - 117, 27.03.2022

Faruk Çiçekci

https://doi.org/10.30565/medalanya.975253

Abstract

Bart’s sendromu, derinin konjenital fokal yokluğu, mekanik büllöz ve tırnak distrofisi ile karakterize epidermolizis bülloza (EB) ile de ilişkili genetik bir hastalıktır. Konjenital pilorik atrofi nedeniyle ameliyat edilen doğuştan lokalize cilt yokluğu, tırnak distrofisi ve kulak atrofisi olan erkek yenidoğanın anestezi yönetimini sunuyoruz. Hastanın el ve ayaklarında zengin kırmızı soyma bölgelerindeki cilt lezyonlarına dikkat edilerek palyatif önlemler alındı ve standart genel inhalasyon anestezisi uygulandı. İngilizce literatürde araştırabildiğim kadarıyla bu olgu sunumu Bart sendromlu bir hastada anestezi yönetimini sunan ilk olgu sunumu olabilir.

Keywords

Bart's sendromu, konjenital pilorik atrezi, konjenital cilt yokluğu, epidermolizis bülloza, olgu sunumu

Anesthesia management in Bart's syndrome: A case report

Abstract

Bart's syndrome is a genetic disorder that is also associated with epidermolysis bullosa (EB), which is characterized by congenital focal absence of the skin, mechanical bullous, and nail dystrophy. We present the anesthesia management of a male neonate with congenital localized skin absence, nail dystrophy, and ear atrophy who underwent surgery due to congenital pyloric atrophy. Palliative measures were perforemed paying attention to skin lesions in areas of rich-red peeling on the hands and feet of the patient, and standard general inhalation anesthesia was administered. As far as I could research in the English literature, this case report may be the first case report to present the management of anesthesia in a patient with Bart syndrome.

Keywords

Bart's syndrome, congenital absence of skin, congenital ploric atresia, epidermolysis bullosa, case report

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