Aylar POYRAZ, Kemali BAYKANER, Ebru ERGENEKON, Zafer TÜRKYILMAZ, Ramazan KARABULUT, İbrahim Murat HİRFANOĞLU, Pınar BOZDEMİR, Özden TURAN

A rare congenital anomaly: Split notochord syndrome with rectal duplication and uterus didelphus

Split notokord sendromu (SNS) nadir bir spinal anomali olup, vertebra, santral sinir sistemi ve gastrointestinal sistem anomalilerine eşlik eder. Literatürde 30'dan az yenidoğan olgusu yayınlanmıştır. Gastrointestinal sistemin duplikasyonları nadirdir ve rektal duplikasyonlar tüm duplikasyonların %5'ini oluşturur. Split notokord sendromlu bu olguda, rektal duplikasyon, posterior stenotik anüs, meningomyelosel, Arnold Chiari malformasyonu, hemivertebra, kısa boyun ve pes ekinovarus bulunmaktadır. Postmortem incelemeler ile uterus didelfîs ve bilateral hipoplastik böbrekler saptandı. Bu olgu, Split Notokord sendromuna nadir anomalilerin eşlik etmesi nedeniyle tüm literatür gözden geçirilerek sunuldu.

Nadir bir konjenital anomali: Split notokord sendromu, rektal dublikasyon ve uterus didelfis birlikteliği

Split notochord syndrome (SNS) is an extremely rare spinal anomaly, associated with anomalies of the vertebrae, central nervous system, and gastrointestinal tract. So far less than 30 neonatal cases with SNS have been published in the literature. Duplications of the gastrointestinal tract are rarely seen and rectal duplications (RDs) account for 5% of alimentary tract duplications. This case of SNS was associated with RD, posterior stenotic anus, meningomyelocele, Arnold Chiari malformation, hemiver-tebrae, short neck, and pes equinovarus. Postmortem investigations have also revealed uterus didelphus and bilateral hypoplastic kidneys. This case was presented to highlight the fact that there are rare associations with SNS, together with a literature review.

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