Bart’s sendromunda anestezi yönetimi: Bir olgu sunumu

Year 2022, Volume: 6 Issue: 1, 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

References

• 1. Bart BJ, Gorlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa: a new syndrome. Arch Dermatol. 1966;93(3):296-304. doi: 10.1001/archderm.1966.01600210032005
• 2. Smith SZ, Cram DL. A mechanobullous disease of the newborn. Arch Dermatol. 1978;114(1):81-4. doi: 10.1001/archderm.1978.01640130045013
• 3. Fine JD, Bruckner-Tuderman L, Eady RAJ, Bauer EA, Bauer JW, Has C, Heagerty A, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1103-26. doi: 10.1016/j.jaad.2014.01.903
• 4. Rajpal A, Mishra R, Hajirnis K, Shah M, Nagpur N. Bart’s syndrome. Indian J Dermatol. 2008;53(2):88-90. doi: 10.4103/0019-5154.41655
• 5. Duran-McKinster C, Rivera-Franco A, Tamayo L, De La Luz Orozco-Covarrubias M, Ruiz-Maldonado R. Bart syndrome: the congenital localized absence of skin may follow the lines of Blaschko. Report of six cases. Pediatr Dermatol. 2000;17(3):179-82. doi: 10.1046/j.1525-1470.2000.01747.x
• 6. Zelickson B, Matsumura K, Kist D, Epstein Jr EH, Bart BJ. Bart’s syndrome: ultrastructure and genetic linkage. Arch Dermatol. 1995;131(6):663-8. doi: 10.1001/archderm.131.6.663.
• 7. Chiaverini C, Charlesworth A, Fernandez, Barbarot S, Bessis D, Bodemer C A et al. Aplasia cutis congenita with dystrophic epidermolysis bullosa: clinical and mutational study. Br J Dermatol. 2014;170(4);901-6. doi: 10.1111/bjd.12741 8. Bart BJ, Lussky RC. Bart syndrome with associated anomalies. Am J Perinatol. 2005;22(7):365-9. doi: 10.1055/s-2005-871657
• 9. Lesteringant GG, Akel SR, Qayed K. The pyloric atresia-junctional epidermolysis bullosa syndrome: Report of a case and review of literature. Arch Dermatol 1992;128(8):1083-6. doi: 10.1001/archderm.128.8.1083.
• 10. Mittal RR, Singh SP, Gill SS; Dimple. Bart syndrome. Indian J Dermatol Venereol Leprol. 1996 Jul-Aug;62(4):266-7. PMID: 20948079.
• 11. Aygun AD, Yilmaz E, Citak AN, Elkiran O, Okur I, Oercan I, et al. Aplasia cutis congenita and epidemolysis bullosa: bart syndrome. Int J Dermatol. 2010;49(3);343–5. doi: 10.1111/j.1365-4632.2009.04182.x