Patients with severe prune belly syndrome rarely survive beyond the first days of life. We present a case of prune belly syndrome that initially presented with severe oligohydramnios, megacystis and associated poor urine biochemistries. Due to an anteriorly located placenta the patient was referred to three major centers, but was turned down because of the unfavorable prognostic findings. Therefore, fetal intervention was performed with 32 vesicocentesis and amnioinfusion procedures. Despite the unfavorable prenatal findings, and having undergone numerous fetal interventions, the birth resulted in a viable infant.
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Prune belly syndrome (PBS), or Eagle-Barrett syndrome, is a rare congenital disorder marked by abdominal wall muscle deficiency, urinary tract anomalies, and cryptorchidism, causing significant abdominal wall laxity and functional impairment. This case report discusses an innovative approach to abdominal wall reconstruction in a 19-year-old male patient with PBS and associated conditions, including chronic renal failure and spina bifida. Previously, he underwent distal ureterectomy and vesicoureteral reimplantation at the age of two years to correct urinary tract dilation and bilateral orchiopexy.
View Article and Find Full Text PDFOutcomes of Growth-Friendly Surgical Treatment of Early Onset Scoliosis in Children With Prune Belly Syndrome: A Preliminary Report.
J Pediatr Orthop
December 2024
Department of Orthopaedic Surgery, C.S. Mott Children's Hospital, Michigan Medicine, Ann Arbor, MI.
Background: Prune belly syndrome (PBS) is a rare condition characterized by absence of abdominal musculature, cryptorchidism, and obstructive uropathy. The most common orthopaedic problem is scoliosis, yet no reports on growth-friendly surgical treatment of early-onset scoliosis (EOS) exist. Our purpose was to evaluate outcomes of distraction-based implants in children with PBS.
View Article and Find Full Text PDFLong-term outcomes of kidney replacement therapy in Australians with prune belly syndrome.
J Paediatr Child Health
December 2024
School of Medicine, University of Tasmania, Hobart, Tasmania, Australia.
Article Synopsis
- * A total of 37 males with PBS were analyzed, with most starting KRT in their teens; half underwent kidney transplants, showing good graft survival rates at 1, 5, and 10 years.
- * The findings indicated that while there was variation in individual outcomes for PBS patients, their overall transplant and dialysis survival rates were similar or better than those of the control group.
A new spectrum of neonatal urethral pathologies in the era of early vesicoamniotic shunting?
World J Urol
October 2024
Division of Pediatric Surgery, Medical Faculty and University Hospital Cologne, University of Cologne, Cologne, Germany.
Purpose: Intrauterine vesicoamniotic shunting (VAS) was shown to affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO). Data on postnatal management are largely lacking. We aim to describe the pathologies diagnosed in children born after vesicoamniotic shunt placement in early pregnancy for megacystis.
View Article and Find Full Text PDFPrune belly syndrome in a neonate with severe bilateral hydronephrosis: A rare case report from Somalia.
Int J Surg Case Rep
October 2024
School of Postgraduate Studies and Research, Amoud University, Amoud Valley, Borama 25263, Somalia.
Article Synopsis
- Prune belly syndrome (PBS) is a rare congenital disorder marked by underdeveloped abdominal muscles, urinary system issues, and undescended testes, with an occurrence rate of approximately 1 in 30,000 to 1 in 50,000 live births; this is the first reported case in Somalia.
- A newborn presented with respiratory distress, jaundice, and abdominal issues, leading to diagnoses like bilateral hydroureteronephrosis and abdominal wall muscle underdevelopment, complicated by the limited healthcare resources in the region.
- Effective management of PBS in resource-limited settings involves addressing infections, ensuring fluid balance, and dealing with urinary abnormalities, but the lack of specialized pediatric urology services complicates care.
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