Lokman ÜSTYOL, Hayrettin TEMEL, Erdal PEKER, Murat DOĞAN, FESİH AKTAR, Gökmen A. TAŞKIN, Hüseyin ÇAKSEN

Amniyotik band sendromu olgusu

Amniotik band sendromu (ABS), amniyondan yoksun plasenta, amniotik zar kalıntılarının fetusa yapışması ya da dolanması ile oluşan fetal deformasyon, malformas-yon veya amputasyon üçlüsü ile karakterize bir tablodur. Prevelansı 1.200 ile 15.000 canlı doğumda bir arasında değişmektedir. Altı yaşında erkek hasta doğuştan el ve ayaklarının olmaması şikayetleriyle getirildi. Fizik mua-yenede sağ diz 5 cm altı ve sol ayak bileğinin hemen altından itibaren, ayrıca sağ ve sol el ikinci, üçüncü ve dördüncü parmak metakarpofalangial eklem, sol el birinci ve beşinci parmak proksimal interfalangial eklem hizasında boğumlanma tarzında anomalileri vardı. Beyin manyetik rezonans incelemesi ve batın ultrasonografisi normal idi. Bu vaka dolayısıyla ekstremite anomalileriyle getirilen vakalarda ayırıcı tanıda ABS’nin de düşünülmesi gerektiğini vurgulamak isteriz.

A case of amniotic band syndrome

Amniotic Band Syndrome (ABS) is a clinical picture characterized by the triad of fetal deformation, malformation or amputation due to adherence to the fetus or coiling around the placenta. Its prevalence ranges in between one in 1.200 and 15.000 live births. A six-year-old male patient was admitted to the hospital with the complaint absence of hands and feets since birth. On his physical examination, there were articulation anomalies starting at 5 cm below the right knee and just below the left ankle and furthermore, at the levels of right and left hand second, third and fourth finger’s metacarpophalangeal joints, and the left hand first and fifth finger proximal interphalangeal junction. The brain magnetic resonance imaging and abdominal ultrasound were normal. With this case report, we want to emphasize that ABS should be kept in mind in the differential diagnosis of patients with extremity anomalies.

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