Prenatal Tanı Alan Bir Gollop-Wolfgang Kompleksi Olgusu

Öz

Gollop-Wolfgang kompleksi (GWC) , el ektrodaktilisi ile ilişkili olan veya olmayan distal femoral duplikasyon ve tibial agenezi ile karakterize nadir bir konjenital ortopedik malformasyondur (1/106 canlı doğum). GWC etiyolojisi hala bilinmiyor ve cerrahi ile düzeltme günümüzde hala zordur. Gallop Wolfgang Kompleksi ile ilişkili diğer anomaliler: ulna hipoplazisi, kardiyak anomaliler, omurga ve toraks segmentasyon defektleri, kloaka ekstrofisi, imferfore anus, trakea-özofageal fistül, özefagus atrezisi, proksimal fokal femoral yetmezlik, yarık damak ve dudaktır. Prenatal tanı, gebeliğin ikinci trimesterinde ultrason taramaları ile yapılabilir. Olgu sunumumuzda prenatal tanı almış, oldukça nadir görülen Gallop Wolfgang kompleksi tartışıldı.

Anahtar Kelimeler

• Gollop Wolfgang kompleks
• tibial agenezi
• prenatal tanı

A Case of Gollop-Wolfgang Complex with Prenatal Diagnosis

Abstract

Gollop-Wolfgang complex (GWC) is a rare congenital orthopedic malformation (1/106 live births) characterized by a distal femoral duplication and tibial agenesis associated or not with hand ectrodactyly. The etiology of GWC is unknown and the surgical correction is still difficult. Other abnormalities related to GWC: ulna hypoplasia, cardiac abnormalities, spine and thorax segmentation defects, kloaca extropy, imperforated anus, trachea-esophageal fistule, esophagus atresia, proximal focal femoral failure, cleft palate, and lip. Prenatal diagnosis can be performed by ultrasound scans in the second trimester of pregnancy. Object in our case, very rarely seen Gallop Wolfgang Complex with prenatal diagnosis has been discussed.

Keywords

• Gollop Wolfgang complex
• tibial agenesis
• prenatal diagnosis

Kaynakça

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