The prune-belly syndrome: current insights

Abstract

The prune-belly syndrome comprises a constellation of well-established physical findings, yet the cause and management remain controversial. This review focuses on the current understanding of its pathogenesis and characterizes the fetal and neonatal diagnosis and management. Other associated anomalies are discussed to understand better the factors affecting treatment and prognosis as these patients grow into childhood and beyond.

Testicular torsion in a patient with Cohen syndrome

“…[3] Hypotonia of the abdominal wall and cryptorchidism are commonly observed in diseases such as prune belly and Lowe syndrome. [4][5][6] In contrast, no testicular abnormality has been reported in Cohen Syndrome. In our case, the patient had a history of bilateral cryptorchidism up to the second year of his life.…”

Pseudo prune belly syndrome

“…The incidence varies between one in 35,000 to one in 50,000 with greater predominance in males 1,2 . Mutation is usually sporadic although familial forms are seen 3 .…”

“…C-reactive protein was 25.2 mg/L, blood urea 50mg/dl, the serum creatinine 0.52mg/dl with normal serum electrolytes and normal liver function. Urine had plenty of pus cells per high power field and urine culture revealed growth of E.coli which was sensitive to carbapenems, amikacin, nitrofurantoin and _________________________________________ 1 …”

Anesthetic Management of Prune-Belly Syndrome: Case Report

“…5 Patogenezi kesin olarak bilinmemekle birlikte, enfeksiyöz ve/veya teratojen nedenlerin multifaktöriyel etkisine bağlı inutero embriyolojik gelişimdeki değişiklikler sebebiyle meydana geldiği düşünülmektedir. 6 Etiyolojisinde yer alan oligohidroamniosun tipik yüz görünümü, abdominal duvar hipoplazisi, inmemiş testis, pulmoner hipoplazinin nedeni olduğu dü-şünülmektedir. Üriner sistem anomalileri, üreter dilatasyonu, megakist ve hidronefroza kadar değişiklik gösterebilmekte, anomaliler; vezikoüre-teral reflü ve üriner sistem enfeksiyon sıklığını arttırabilmektedir.…”