Akut peritonit sırasında tesadüfen fark edilen konjenital izole asplenia

Öz

Çok nadir görülen bir durum olan konjenital aspleninin 2 farklı tipi vardır: heterotaksi sendromları ve izole konjenital aspleni (ICA). Ivemark sendromu, asplenia, kalbin malformasyonları ve göğüs ve karındaki iç organların malpozisyonu ile karakterize heterotaksi sendromlarından biridir. ICA vakaları çocuklukta da ölümcüldür, ancak bildirilen canlı yetişkin vakaları mevcuttur. Etkilenenler tipik olarak fulminan sepsis için yüksek risk altındadır ve trombositoz ve mezenterik tromboz gibi bulaşıcı olmayan komplikasyon riski daha yüksektir. Burada ülser perforasyonuna bağlı peritonit için acil bir laparotomi sırasında tesadüfen saptanan doğuştan asplenisi olan bir hastanın olağandışı olgusunu sunuyoruz.

Anahtar Kelimeler

• Konjenital izole asplenia,Yetişkin peritoniti

Congenital isolated asplenia accidentally discovered during acute peritonitis

Abstract

An extremely rare condition, congenital asplenia has 2 distinct types: heterotaxy syndromes and isolated congenital asplenia (ICA). Ivemark syndrome is one of the heterotaxy syndromes characterized by asplenia, malformations of the heart, and malposition of internal organs in the chest and abdomen. ICA cases are also fatal in childhood, but there are reported living adult cases. Those affected are typically at increased risk for fulminant sepsis and carry a higher risk of noninfectious complications, such as thrombocytosis and mesenteric thrombosis. We herein report the unusual case of a patient with congenital asplenia, which was discovered fortuitously during an emergency laparotomy for peritonitis due to ulcer perforation.

Keywords

• Congenital isolated asplenia,Adult peritonitis

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