Abstract
Prune Belly Syndrome is a rare congenital disorder with unknown aetiology, consisting of a triad of abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. We are unaware of any preceding report of Prune Belly Syndrome in Tanzania, and here we describe two cases reported in Kagera region. The first case is a 2 month old boy with the triad of Prune Belly Syndrome along with pectus carinatum who died due to septicaemia. This case posed a diagnostic challenge at birth and during the natal period. Paucity of comprehensive knowledge of congenital malformations at the peripheral health facilities may have also contributed to the diagnostic challenge in the first place.The second case is a neonate who was referred to regional referral hospital where he was diagnosed with Prune Belly Syndrome at the age of four weeks. Because of limited capacity to manage congenital malformations at the regional referral hospital, he was referred to an urologist at the zonal referral hospital. However, inadequacies in supporting systems to the parents compounded care of the neonate with Prune Belly Syndrome.High index of Prune Belly Syndrome suspicion is needed in a resource limited setting in order to timely make diagnosis. There is also a need to strengthen institutional and individual's capacity for prenatal screening to detect congenital anomalies at an early stage of foetus development. Multidisciplinary management approach is necessary in order to improve the quality of life for patients with Prune Belly Syndrome. Psychosocial and medical support systems should be put in place in order to enhance preparedness for patient care in resource limited settings including equipping the referral hospital with different specialists and ensuring availability of basic investigations for patients.
Highlights
Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by a triad of deficient abdominal wall muscles, cryptorchidism and urinary tract anomalies
Prune Belly Syndrome (PBS) is a rare congenital disorder composed of anomalies of various organ systems
The child in the first reported case was born at a health facility, his congenital malformation was not detected until 2 months when they were seen at a higher level of health facility (BRRH)
Summary
Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by a triad of deficient abdominal wall muscles, cryptorchidism and urinary tract anomalies (including hydroureteronephrosis). At a time of admission, the mother reported that the child had fever, cough and difficulty in breathing for the past 3 days He had no vomiting, no diarrhoea, and was passing urine normally. The child was treated as a case of severe pneumonia, improved after seven days of antibiotics (ampicillin and gentamicin) and was discharged with iron supplements and cod liver oil His parents were counselled about PBS and the care they can give him at home. On reviewing the patient at the paediatric clinic, his parent reported that they noted abdominal swelling on the left side since birth He had no vomiting, no diarrhoea, no history of fever, no cough and was passing urine normally. The child was treated with amoxicillin as a case of urinary tract infection, and was referred to urologist at Bugando zonal referral hospital in Mwanza for further management including management of impaired kidney function and possible orchidopexy
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