Bartsocas-papas syndrome and anesthesia

We report the anesthetic management of an 8-year-old girl, followed up with the diagnosis of BPS since 1.5 months of age, scheduled to undergo a surgery for enlarging the oral cavity.

📌 3 Key Takeaways

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Objective & Hypothesis:Bartsocas-Papas Syndrome (BSP), known to be lethal form of pterygial syndromes, first described in 1972 (1).

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Key Findings & Data:It is a rare autosomal recessive disorder, characterized with multiple congenital anomalies including microcephaly, micrognatia, cleft lip-palate, multiple pterygia, ankyloblepharon, filiform bands between the jaws, syndactyly and other ectodermal and non-ectodermal anomalies (2,3).

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Conclusion & Impact:We report the anesthetic management of an 8-year-old girl, followed up with the diagnosis of BPS since 1.5 months of age, scheduled to undergo a surgery for enlarging the oral cavity.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

Bartsocas-Papas Syndrome (BSP), known to be lethal form of pterygial syndromes, first described in 1972 (1). It is a rare autosomal recessive disorder, characterized with multiple congenital anomalies including microcephaly, micrognatia, cleft lip-palate, multiple pterygia, ankyloblepharon, filiform bands between the jaws, syndactyly and other ectodermal and non-ectodermal anomalies (2,3). We report the anesthetic management of an 8-year-old girl, followed up with the diagnosis of BPS since 1.5 months of age, scheduled to undergo a surgery for enlarging the oral cavity.

Özet

Pterijiyal sendromların ölümcül formu olarak bilinen Bartsocas-Papas Sendromu (BPS) ilk kez 1972’de tanımlanmıştır (1). Mikrosefali, mikrognati, yarık damak-dudak, multipl pterijiyum, ankiloblefaron, çeneler arasında filiform bantlar, sindaktili ve diğer ektodermal ve non ektodermal anomaliler gibi multipl konjenital anomalilerle karakterize, ender görülen otozomal resesif bir hastalıktır (2,3). Bu raporda, 1,5 aylıktan itibaren BPS tanısı ile izlenen sekiz yaşındaki bir kız çocuğunun oral kavite genişletilmesi operasyonundaki anestezi yaklaşımını ele aldık.

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Cite This Article

ŞAHİN, A., ÖZGEN, S., CANBAY, Ö., KARAGÖZ, H. A., ÇELEBİ, N., ÜZÜMCÜGİL, F. (2022). Bartsocas-papas syndrome and anesthesia. Türk Anestezi ve Reanimasyon Dergisi, Cilt: 36 - Sayı: 1, 61–64. https://ajindex.com/articles/620009

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