E-ISSN 1858-8360 | ISSN 0256-4408
 

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Prune belly syndrome: A report of 15 cases from Sudan.

Abdelmoneim E M Kheir, Eltigani M A Ali, Safaa A Medani, Huda S Maaty.

Abstract
Prune belly syndrome is a rare congenital malformation of unknown aetiology, composed of a triad of deficient abdominal wall muscle, cryptorchidism and urinary tract anomalies. The majority of patients have associated pulmonary, skeletal, cardiac, and gastrointestinal defects. This was a prospective, case finding study that was conducted in the main paediatric hospitals in Khartoum state, during the period December 2015 to September 2016. A total of 15 patients with prune belly syndrome were collected. Patients' characteristics were noted including socio-demographic data, laboratory and radiological investigations and any medical or surgical intervention. There were 12 males and 3 females with a male to female ratio of 4:1. Most of the patients (80%) had hydronephrosis and hydroureter. The study revealed that 60% of the patients had associated anomalies, there were 4 (26.6%) with cardiac defects, 3 (20%) with orthopaedic defects one patient with small bowel volvulus and one patient with cleft lip. 6 (40%) patients received medical intervention and 8 (53%) patients underwent surgical procedures. At the last follow up visit, 2 (13.4%) patients had normal renal function tests, 8 (53.3%) ended with chronic kidney disease, and 5 died with a mortality rate of 33.3%. Prune belly syndrome is a rare entity with wide variability in severity and clinical manifestations. The mortality in prune belly syndrome remains high despite medical and surgical interventions.

Key words: Abdominoplasty; Prune Belly Syndrome; Renal Anomaly; Sudan; Undescended Testis


 
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How to Cite this Article
Pubmed Style

Kheir AEM, Ali EMA, Medani SA, Maaty HS. Prune belly syndrome: A report of 15 cases from Sudan.. Sudan J Paed. 2017; 17(1): 42-48.


Web Style

Kheir AEM, Ali EMA, Medani SA, Maaty HS. Prune belly syndrome: A report of 15 cases from Sudan.. http://www.sudanjp.com/?mno=285371 [Access: August 14, 2020].


AMA (American Medical Association) Style

Kheir AEM, Ali EMA, Medani SA, Maaty HS. Prune belly syndrome: A report of 15 cases from Sudan.. Sudan J Paed. 2017; 17(1): 42-48.



Vancouver/ICMJE Style

Kheir AEM, Ali EMA, Medani SA, Maaty HS. Prune belly syndrome: A report of 15 cases from Sudan.. Sudan J Paed. (2017), [cited August 14, 2020]; 17(1): 42-48.



Harvard Style

Kheir, A. E. M., Ali, . E. M. A., Medani, . S. A. & Maaty, . H. S. (2017) Prune belly syndrome: A report of 15 cases from Sudan.. Sudan J Paed, 17 (1), 42-48.



Turabian Style

Kheir, Abdelmoneim E M, Eltigani M A Ali, Safaa A Medani, and Huda S Maaty. 2017. Prune belly syndrome: A report of 15 cases from Sudan.. Sudanese Journal of Paediatrics, 17 (1), 42-48.



Chicago Style

Kheir, Abdelmoneim E M, Eltigani M A Ali, Safaa A Medani, and Huda S Maaty. "Prune belly syndrome: A report of 15 cases from Sudan.." Sudanese Journal of Paediatrics 17 (2017), 42-48.



MLA (The Modern Language Association) Style

Kheir, Abdelmoneim E M, Eltigani M A Ali, Safaa A Medani, and Huda S Maaty. "Prune belly syndrome: A report of 15 cases from Sudan.." Sudanese Journal of Paediatrics 17.1 (2017), 42-48. Print.



APA (American Psychological Association) Style

Kheir, A. E. M., Ali, . E. M. A., Medani, . S. A. & Maaty, . H. S. (2017) Prune belly syndrome: A report of 15 cases from Sudan.. Sudanese Journal of Paediatrics, 17 (1), 42-48.





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