Three cases of prune belly syndrome at the lagos state university teaching hospital, Ikeja

Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate p...

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Bibliographic Details
Published in:Saudi journal of kidney diseases and transplantation 2018, Vol.29 (1), p.178-184
Main Authors: Solarin, Adaobi U., Ogunnaike, Gbemisola O., Aremu, Oluwatosin E., Ogbuokiri, Eucharia, Oladimeji, Alaba, Animasahaun, Adeola B., Gbelee, Henry O., Disu, Elizabeth A.
Format: Article
Language:English
Subjects:
Abdomen
Antibiotics
Bladder
Care and treatment
Case studies
Diagnosis
Education
Electrolytes
Intubation
Kidneys
Males
Metabolism
Parents & parenting
Pediatrics
Prune belly syndrome
Teaching hospitals
Theory
Ultrasonic imaging
Urine
Urogenital system
التشخيص الطبي
دراسات الحالة
متلازمة البطن البرقوقية
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Summary:Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.
ISSN:1319-2442
2320-3838
DOI:10.4103/1319-2442.225190