Journals / Türk Patoloji Dergisi / 2020 / Cilt: 36 - Sayı: 1

An Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence

Pages
64–67
Publication Date
2022-11-06
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

This case report aims to remind the obstetricians aboutthe concurrent occurrence of PBS with URSM and its poor prognosis.

📌 3 Key Takeaways

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Objective & Hypothesis:Prune belly syndrome (PBS) is characterized by a classical triad of congenitally absent abdominal muscles, bilateral cryptorchidism, and amalformed urinary tract.
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Key Findings & Data:Thiscase report describes a 15-week-old female fetus with megacystis, ascites and oligohydramnios in a 19-year-old nulliparous woman.
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Conclusion & Impact:This case report aims to remind the obstetricians aboutthe concurrent occurrence of PBS with URSM and its poor prognosis.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Prune belly syndrome (PBS) is characterized by a classical triad of congenitally absent abdominal muscles, bilateral cryptorchidism, and amalformed urinary tract. Urorectal septum malformation sequence (URSM) is identified with the absence of urogenital and anal openings. Thiscase report describes a 15-week-old female fetus with megacystis, ascites and oligohydramnios in a 19-year-old nulliparous woman. The patientunderwent preterm labor at the 33rd gestational week and delivered a female newborn weighing 2250 grams who died three days later due toprogressive respiratory insufficiency. To the best of our knowledge, this is the third case of an overlap between PBS and URSM in literature. Suchan overlap refers to the existence of left renal agenesis, right renal cystic dysplasia, bilateral club foot and lumbar scoliosis as well as the absenceof abdominal wall muscles, internal genital organs, urethral, vaginal and anal openings. This case report aims to remind the obstetricians aboutthe concurrent occurrence of PBS with URSM and its poor prognosis.

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Cite This Article

KÖSEOĞLU, D., PEKTAŞ, M. K., TAKÇI, Ş., İŞGÜDER, Ç. K. (2022). An Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence. Türk Patoloji Dergisi, Cilt: 36 - Sayı: 1, 64–67. https://ajindex.com/articles/640390

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References (8)

  1. Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 2010;76: 44-8
  2. Grimsby GM, Harrison SM, Granberg CF, Bernstein IH, Baker LA. Impact and frequency of extra-genitourinary manifestations of prune belly syndrome. J Pediatr Urol. 2015;11:280. e1-6
  3. Seidel N, Arlen AM, Smith EA, Kirsch AJ. Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 2015; 85:211-5
  4. Escobar LF, Heiman M, Zimmer D, Careskey H. Urorectal septum malformation sequence: prenatal progression, clinical report, and embryology review. Am J Med Genet A. 2007;143A:2722-6
  5. Tennant PW, Glinianaia SV, Wellesley D, Draper ES, Kurinczuk JJ, Tonks AM, Tucker DF, Wreyford B, Rankin J. Epidemiology of partial urorectal septum malformation sequence (or ‘persistent cloaca’): a population-based study in seven regions of England and Wales 1985-2010. Arch Dis Child Fetal Neonatal Ed. 2014; 99: F413-8
  6. Shah K, Nayak SS, Shukla A, Girisha KM. Spectrum of urorectal septum malformation sequence. Congenit Anom (Kyoto). 2016;56:119-26
  7. Goswami D, Kusre G, Kumar Dutta H, Sarma A. The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association. J Clin Diagn Res. 2013;7:1727-9
  8. Farooqui A, Al Aqeel A, Habib Z. Prune belly syndrome with overlapping presentation of partial urorectal septum malformation sequence in a female newborn with absent perineal openings. Case Rep Surg. 2014;2014:746323.