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2020, Volume 36, Number 1, Page(s) 064-067
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DOI: 10.5146/tjpath.2018.01440 |
An Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence |
Çiğdem KUNT İŞGÜDER1, Mine KANAT PEKTAŞ2, Doğan KÖSEOĞLU3, Şahin TAKÇI4 |
1Department of Obstetrics and Gynecology, Istanbul University, School of Medicine Istanbul, ISTANBUL, TURKEY
2Department of Kocatepe University, School of Medicine, AFYON, TURKEY
3Department of Pathology, Gaziosmanpasa University, School of Medicine, TOKAT, TURKEY
4Department of Pediatrics, Gaziosmanpasa University, School of Medicine, TOKAT, TURKEY |
Keywords:
Congenital anomaly, Newborn, Prune belly syndrome, Urorectal septum malformation sequence |
Prune belly syndrome (PBS) is characterized by a classical triad of congenitally absent abdominal muscles, bilateral cryptorchidism, and a
malformed urinary tract. Urorectal septum malformation sequence (URSM) is identified with the absence of urogenital and anal openings. This
case report describes a 15-week-old female fetus with megacystis, ascites and oligohydramnios in a 19-year-old nulliparous woman. The patient
underwent preterm labor at the 33rd gestational week and delivered a female newborn weighing 2250 grams who died three days later due to
progressive respiratory insufficiency. To the best of our knowledge, this is the third case of an overlap between PBS and URSM in literature. Such
an overlap refers to the existence of left renal agenesis, right renal cystic dysplasia, bilateral club foot and lumbar scoliosis as well as the absence
of abdominal wall muscles, internal genital organs, urethral, vaginal and anal openings. This case report aims to remind the obstetricians about
the concurrent occurrence of PBS with URSM and its poor prognosis.
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