Unusual presentation of prune belly syndrome: a case report | Journal of Medical Case Reports

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PBS is a rare congenital disorder composed of anomalies of various organ systems. This case report describes a 12-year-old boy with the triad findings of PBS reported in previous publications [7, 8] and congenital bilateral hip dysplasia with scoliosis. In the majority of case reports the diagnosis of PBS is made during infancy. Although this child was born at a health center his congenital malformation was not detected at birth. This may indicate a low level of awareness among health professionals about PBS; however, there are very few case reports of PBS diagnosed outside the neonatal age group [9]. The embryologic characteristics of congenital musculoskeletal problems correlate well with the embryologic theory of PBS [10]. Similar to other case reports, scoliosis and hip dysplasia were noted in the current case. Orthopedic abnormalities occur in 45 to 63% of patients with PBS [11]. The most common orthopedic anomalies are hip dislocation and talipes equinovarus [12], while scoliosis is the most common spinal abnormality noted [13, 14]. Currently, children with PBS reach adulthood; however, morbidity related to the musculoskeletal system can potentially affect their quality of life. The abdominal wall defect may not have life-threatening consequences but the inefficient contraction of the abdominal wall muscles is believed to affect bladder, bowel, and pulmonary function, increasing the risk of recurrent urinary and respiratory tract infections [15]. Improvement in bladder emptying is a potential benefit of offering abdominoplasty in full-blown PBS [16]. The incidence of renal failure over time was 20 to 30%, although some case series have rates of renal insufficiency as low as 7% [17]. In the current case, despite a non-functioning left kidney, the renal function test was within normal limits for our patient’s age, which could be explained by the compensatory action of his right kidney.

Treatment of the case involved both surgical and medical teams. Removal of our patient’s left kidney and orchidectomy were performed, and he was treated for a urinary tract infection. There have been documented cases of testicular tumors developing in these patients, but overall the risk does not appear to be greater than other patients with undescended testes [1820]. Orchidopexy or removal of the testes is one of the treatment modalities available to children with PBS. Removal of the testes is done to counteract the high risk of cancer associated with intraperitoneal testes which was a potential outcome in this case.

He was discharged from our hospital with the expectation of long-term follow up, and an appointment for abdominoplasty was scheduled. After communicating with an endocrinologist, hormonal replacement therapy was considered to be started on follow up.

Our strength to the approach of this case was giving the most treatment and care that were available in our setting. In contrast, the unavailability of genetic study was our limitation.

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