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Prune belly syndrome: early management outcome of nine consecutive cases
O H Ekwunife1, J O Ugwu, V Modekwe
1Department of Surgery, Paediatric Surgery Unit, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria.
Insights
Prune Belly Syndrome (PBS) management in developing nations requires a comprehensive approach. Surgical interventions like abdominoplasty and orchiopexy improve patient outcomes and quality of life.
Area of Science:
- Pediatric Surgery
- Congenital Malformations
- Urology
Background:
- Prune Belly Syndrome (PBS) is a rare congenital disorder with unclear causes.
- Limited data exists on PBS progression and outcomes in developing countries, often relying on case reports.
Purpose of the Study:
- To review the management and outcomes of Prune Belly Syndrome (PBS) cases over five years.
- To analyze associated anomalies, treatment strategies, and quality of life improvements in PBS patients.
Main Methods:
- Retrospective review of 9 patients diagnosed with Prune Belly Syndrome (PBS).
- Analysis of patient demographics, associated anomalies, surgical interventions (abdominoplasty, orchiopexy), and conservative management.
- Evaluation of outcomes including infection rates, renal function, and quality of life.
Main Results:
- The study included 7 males and 2 females, presenting from 30 minutes to 11 days old.
- Intestinal malrotation was the most common anomaly; urinary tract issues were initially managed conservatively.
- Abdominoplasty and orchiopexy demonstrated physiological benefits and improved quality of life; however, mortality occurred due to pulmonary hypoplasia and urosepsis.
Conclusions:
- Prune Belly Syndrome (PBS) presents with diverse clinical features.
- Conservative urinary tract management and surgical interventions like abdominoplasty and orchiopexy offer significant benefits.
- Early parental education is crucial to prevent follow-up defaults and improve overall management outcomes.
Background:
Prune belly syndrome (PBS) is a rare congenital malformation of unclear etiology. The disease progress and outcome in developing countries are not clear as most reports are isolated case reports.
Materials And Methods:
A review of 9 patients managed for PBS in 5 years.
Results:
There were 7 males and 2 females, aged 30 min-11 days (median = 5 days) at the time of presentation (a child presented as neonate, defaulted from follow-up and represented at 10 years of life). Their weights on admission were 2.5-4.2 kg (median = 3 kg). Maternal age range was 26-37 years (median = 32 years), with five mothers being above 30 years. Seven mothers had febrile illness in the first trimester and took antimalarial drugs or antibiotics. Intestinal malrotation was the most common associated anomaly. The degree of the anterior abdominal wall and the urinary tract morphology varies from patient to patient. Urinary tract anomalies were initially managed conservatively. Two infants however later had cutaneous ureterostomy due to worsening renal function and recalcitrant urinary tract infection (UTI). Four infants had abdominoplasty at the 2 nd week, 6 th week, 3 rd year and 10 th year of life. Seven orchiopexies were done. Four were done by Fowler-Stephen's method while the rest were via the inguinal route. Of the former, 3 testicles have normal volume 6 months after, whereas one atrophied. Post abdominoplasty, there was a significant reduction in the frequency of respiratory tract infection (RTI), UTI and post void urine volume in three infants. In addition, there was improved peer interaction and academic performance in the 10-year-old child. One infant died of pulmonary hypoplasia and two others from worsening urosepsis and progressive renal failure.
Conclusion:
PBS presents with a spectrum of features. Initial conservative management of the urinary tract was beneficial. Abdominoplasty and orchiopexy have both physiological and improved quality of life benefits. Early Parental education helped in reducing defaults from follow-up.
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