Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%), malformations of the prostate in 3 (18.8%), urethral malformations in 8 (50%) and mega-ureter in 14 patients (87.5%). A mega-bladder was observed in 13 patients (81.3%). Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8%) and hydronephrosis in 9 patients (56.3%), respectively. Abdominoplasty was performed on 4 patients (25%). Urethral surgery was performed in 10 patients (62.5%). Seven patients (43.8%) required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor.

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The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease

Prune Belly syndrome (PBS) or Eagle-Barrett syndrome is an anatomo-radiological syndrome consisting of a complex and rare malformation characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract, and bilateral cryptorchidism. The exact etiology is unknown, though PBS predominantly occurs in males. The clinical manifestations can vary widely, from stillbirth to renal and major respiratory dysplasia to almost normal children. The current study included a total of 3 patients. The findings included clinical characteristics, diagnostics, therapy, and clinical outcomes. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism and a mega-bladder were observed in 2 patients and distinctive renal malformations, such as renal dysplasia, were observed in 1 patient. Treatment varies but usually includes surgical management of symptoms. One patient required urgent urinary surgery; a vesicotomy was urgently performed due to anuria. These aspects explain the great diversity of opinions on the approach to this syndrome, but the severity of renal dysplasia is the main prognostic factor. Two newborns died a few days later due to severe renal failure. Despite these concerns, many patients with PBS report being in physical and mental health and having a good quality of life.

Prune belly syndrome: Approaches to its diagnosis and management.

Introduction
Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare.

/pdf/prune-belly-syndrome-in-an-egyptian-infant-with-down-2con2qc572.pdf

Prune belly syndrome in an Egyptian infant with Down syndrome: A case report

The Prune-Belly syndrome (PBS) is a rare pathology predominating in male infants, classically manifesting with the triad including aplasia of the abdominal wall muscles, dilatation of the urinary tract, and testicular abnormalities We report and discuss the case of a full-term male newborn, in whom clinical examination at birth revealed abdominal wall muscle hypoplasia, cryptorchidism, urinary tract dilatation and renal failure The diagnosis was made based on physical assessment, abdominal ultra-sonographic imaging, and blood sampling of urea and creatinine For such cases, the recommended surgical management usually consists in a sequential surgical intervention including urinary tract reconstruction, abdominoplasty, and orchidopexy However, these could not be practiced in due time in our patient, who died on the seventh day of life because of kidney failure The prognosis of infants with Prune-Belly syndrome may be improved by quality antenatal follow-up, to enable the early diagnosis and preparation for prompt surgical intervention

Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon

Prune Belly syndrome (PBS) is a rare congenital anomaly of uncertain etiology almost exclusive to males. We report a case of term male baby born to a 39-year-old grand multipara with previous four normal vaginal births. There was no history of genetic or congenital anomaly in her family. Examination of the baby revealed hypotonia, deficient abdominal muscle, cryptorchidism, palpable kidney, and bladder. Ultrasound examination of the abdomen revealed bilateral gross hydronephrosis and megaureter. Provisional diagnosis of PBS was made and the baby was admitted in neonatal intensive care units for further management. Routine antenatal care with ultrasonography will help in detecting renal anomalies, which can be followed postnatally. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

Prune Belly syndrome: A rare case report

Resume Le Prune Belly syndrome (PBS) est une malformation complexe et rare avec une nette predominance masculine. Le but de ce travail a ete d’analyser les aspects epidemiologiques, anatomocliniques et therapeutiques d’une serie observee a l’hopital Aristide-Le-Dantec de Dakar. Malades et methodes Il s’agit une etude retrospective portant sur 22 cas colliges dans les services d’urologie-andrologie et de chirurgie pediatrique de l’hopital Aristide-Le-Dantec. Resultats La moyenne d’âge etait de 15 mois (un jour a dix ans). L’examen somatique avait revele 20 cas d’aplasie complete de la musculature abdominale, un cas de forme partielle droite, et un cas de forme partielle gauche. Dix-neuf patients avaient eu un traitement conservateur pour les malformations urinaires et trois patients un acte chirurgical. L’intervention de Montfort a ete realisee chez deux patients âges de huit et dix ans. Un abaissement testiculaire bilateral selon la technique Fowler-Stephens a ete realise chez 13 patients. Cinq deces et neuf cas d’atrophie testiculaire ont ete observes. Conclusion L’insuffisance renale a ete la principale cause de mortalite. La chirurgie des malformations urinaires necessite une approche au cas par cas et sa realisation devra etre confiee a un operateur experimente. L’abaissement testiculaire devrait etre plus frequemment realise en periode neonatale pour augmenter les chances de paternite, de meme que l’abdominoplastie dont le benefice sur le plan esthetique et fonctionnel est reel.

Syndrome de Prune Belly : aspects épidémiologiques, cliniques et thérapeutiques

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Syndrome de Prune Belly : aspects épidémiologiques, cliniques et thérapeutiques