Prune belly syndrome: Early management outcome of nine consecutive cases

Abstract

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 2nd week, 6th week, 3rd year and 10th year of life. Seven orchiopexies were done. Four were done by Fowler.Stephenfs 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.

Key words: Abdominoplasty, congenital, Nigeria, prune belly syndrome, undescended testis