1/26. Prune-belly syndrome diagnosed at 14 weeks' gestation with severe urethral obstruction but normal kidneys.A fetus was diagnosed with prune-belly syndrome (PBS) found by ultrasound demonstration of cystomegaly at 12 weeks' gestation. Fetal urinary electrolyte and beta2-microglobulin concentrations at 14 weeks' gestation indicated a poor renal prognosis. The pregnancy was therefore terminated. An autopsy showed defects of the abdominal wall muscles and megalocystis caused by severe urethral obstruction. However, the kidneys were histologically normal. Together with previous reports, the present case suggests that renal dysplasia in PBS with severe uretheral obstruction may develop after 14 weeks' gestation. We discuss the possibility of preventing the development of renal dysplasia in PBS by early prenatal decompression of the obstructed fetal urinary tract.- - - - - - - - - - ranking = 1keywords = gestation, pregnancy (Clic here for more details about this article) |
2/26. prenatal diagnosis of cystic bladder distension secondary to obstructive uropathy.We report the perinatal findings of a huge midline posterior cystic bladder distension secondary to lower urinary tract obstruction and prune-belly syndrome in a male fetus. A 40-year-old woman, gravida 3, para 0, was referred at 21 gestation weeks with sonographic findings of anhydramnios and a fetus with a 9.5 x 6.0 cm intra-abdominal cystic mass containing two chambers. The in utero ultrasound-guided fetal bladder drainage using a single needle aspiration and the ultrasound follow-ups of fetal bladder filling provided a diagnostic aid. This method helped to show the position of the bladder and the cystic bladder mass as well as the status of communication in response to decompression or filling of the fetal bladder. cytogenetic analysis revealed a 46,XY karyotype. autopsy showed agenesis of the posterior urethra, prominent megacystis, a cystically distended mass arising from the lower posterior bladder, hydronephrosis, megaureters, and anorectal agenesis with an intestinal blind end adherent to the posterior wall of the uterus. There were no urogenital duplication, hindgut duplication, or urachal abnormalities. The contracted bladder had a full-thickness muscular wall with a trigone and two ureteral orifices while the cystically distended bladder did not have any opening and was lined by a very thin wall. histology of the cystic bladder wall demonstrated typical urothelium, lamina propria and muscularis propria. The pathogenesis and differential diagnosis of cystic bladder distension are discussed.- - - - - - - - - - ranking = 0.14228481762959keywords = gestation (Clic here for more details about this article) |
3/26. Complications of intrauterine intervention for treatment of fetal obstructive uropathy.The intrauterine surgical placement of vesicoamniotic shunts in the treatment of fetal obstructive uropathy associated with prune-belly syndrome to avoid such complications as renal damage and oligohydramnios remains controversial. We present a case of an infant born with prune-belly syndrome at 33 weeks and 5 days of estimated gestational age to a mother of two by vaginal delivery after a pregnancy complicated by fetal obstructive uropathy with attempted intrauterine intervention. After sonographic and laboratory diagnostic and prognostic evaluations, an intrauterine procedure was performed in which a vesicoamniotic shunt was placed under ultrasound guidance. Complications included dislodgment of the initial shunt, with a failed subsequent attempt at placement, oligohydramnios, preterm labor and delivery, and traumatic gastroschisis through the surgical abdominal wall defect. His hospital stay was further complicated by chronic renal insufficiency, prematurity, respiratory distress, bowel malrotation, an episode of gram-negative sepsis with enterobacter cloacae, signs of liver failure, an exploratory laparotomy for severe enterocolitis, and orchiopexy for bilateral undescended testes. At present, it is unclear whether vesicoamniotic shunt placement can provide any significant improvement in the morbidity or mortality for patients with prune-belly syndrome. A large, prospective, randomized trial is needed to determine its efficacy.- - - - - - - - - - ranking = 0.14629109422243keywords = gestation, pregnancy (Clic here for more details about this article) |
4/26. Prune-belly syndrome associated with extra-abdominal abnormalities in a 7-year-old boy.Prune-belly syndrome (PBS) is an association of abdominal wall deficiency, genitourinary anomalies, and in males, cryptorchidism. Other congenital anomalies are associated with PBS, particularly musculoskeletal deformities and gastrointestinal tract anomalies. In this report, a seven-year-old boy with PBS had mega cisterna magna variant, microcornea, aortic stenosis with bicuspid aortic valves, cholelithiasis, and Hirschsprung's disease. Coexistence of these abnormalities with PBS supports the concept of PBS being caused by an early disturbance of not only mesodermal development but also of the other germ layers. There was maternal ingestion of drugs in the 1st month of gestation. All cases with PBS should be evaluated thoroughly for extra-abdominal abnormalities resulting from disturbances of ectodermal and endodermal development. Even though disturbances related to ectodermal and endodermal development may be asymptomatic, early diagnosis of the disturbances may help in preventing possible future problems.- - - - - - - - - - ranking = 0.14228481762959keywords = gestation (Clic here for more details about this article) |
5/26. Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt.The prune-belly syndrome (PBS) consists of abdominal wall distention with deficiency of the abdominal wall musculature, urinary tract abnormalities, and cryptorchidism. The impaired drainage of the bladder leads to oligohydramnios and pulmonary hypoplasia. We present 4 cases of PBS diagnosed by prenatal sonography. In 2 cases, vesicoamniotic shunt therapy was not indicated because of a poor prognosis based on sonographic and laboratory findings; the pregnancies were terminated. In another case, treatment was not performed because of a twin pregnancy, and the neonate with PBS died the day of delivery by cesarean section at 31 weeks' menstrual age. In the other case, vesicoamniotic shunt therapy was successfully performed, and a healthy child was delivered. Several conditions must be met for vesicoamniotic shunt therapy to have a good chance of success: the karyotype must be normal, other malformations must be excluded by careful sonographic examination, and renal function must be normal, as determined by serial analyses of fetal urine. Generally, the shunt should be inserted as early as possible.- - - - - - - - - - ranking = 0.0040062765928362keywords = pregnancy (Clic here for more details about this article) |
6/26. Antenatal diagnosis of prune belly syndrome at 11 weeks of gestation.We present a case of prune belly syndrome diagnosed by ultrasonography at 11 weeks of gestation. We believe this case to be the earliest detection of this syndrome.- - - - - - - - - - ranking = 0.71142408814797keywords = gestation (Clic here for more details about this article) |
7/26. Single vaginal ectopic ureter and renal hypoplasia associated with urogenital sinus and abdominal muscular hypoplasia-a novel subtype of prune-belly syndrome in a female child?We report a rare case that can be classified as prune-belly syndrome in a female. This patient came to our attention during the 19th week of gestation when massive ascites was found by ultrasound and the mother was transferred to our hospital. After birth, it was demonstrated that the infant had complicated anomalies, including abdominal muscular hypoplasia, urogenital sinus, a single, right-sided vaginal ectopic ureter, and hypoplasia of the right kidney. A sibling had a right hypoplastic kidney and right vesicoureteral reflux. Considering the combination of anomalies and the family history of urinary tract disease, her anomalies could be attributed to a genetic mutation.- - - - - - - - - - ranking = 0.14228481762959keywords = gestation (Clic here for more details about this article) |
8/26. In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia.Prenatal ultrasound scanning of a 20-year-old woman at 17 weeks of gestation revealed findings suggestive of bladder outlet obstruction, including bladder distension, dilated bilateral ureters, urinary ascites, and oligohydramnios. Vesicoamniotic shunts were placed with decompression of the bladder and correction of the amniotic fluid levels. Labor was induced at 36 weeks' gestation. At birth, the infant was noted to have prune-belly syndrome with severe urethral hypoplasia, a variant usually associated with a poor prognosis, necessitating vesicostomy for bladder drainage. We present a case of a patient with prune-belly syndrome and bladder outlet obstruction in whom early intervention resulted in an excellent outcome with preservation of renal and pulmonary function.- - - - - - - - - - ranking = 0.28456963525919keywords = gestation (Clic here for more details about this article) |
9/26. phimosis as a cause of the prune belly syndrome: comparison to a more common pattern of proximal penile urethra obstruction.The pathogenesis of the prune belly syndrome (PBS) remains controversial, but two theories predominate. The first theory supports an obstructive phenomenon early in gestation leading to irreversible damage to the genitourinary tract and abdominal wall. The second theory suggests mesodermal injury between the 6th and 10th weeks of gestation as the primary abnormality. This paper reports of two fetuses with the PBS phenotype that were examined postmortem at our institution. Thorough examination of the lower urinary tract allowed demonstration of anatomic obstruction of the urethra in both cases. One case illustrated a relatively common pattern of proximal penile urethral obstruction, a flap-like obstruction between the prostatic and penile urethra. The other case provided what we believe to be the first description of PBS caused by severe phimosis.- - - - - - - - - - ranking = 0.28456963525919keywords = gestation (Clic here for more details about this article) |
10/26. Vertically transmitted hypoplasia of the abdominal wall musculature.The prune belly syndrome (OMIM 100100) is an association of bladder dilation with hypoplasia of the abdominal wall muscles. This malformation sequence is due to early urethral obstruction. We report a family with abdominal wall muscular hypoplasia as an isolated defect, not associated with the urethral obstruction sequence. The proband is a q3-year-old male who presented with abdominal wall laxity and severe constipation. His mother, maternal grandmother and younger brother had varying degrees of abdominal wall muscular deficiency and constipation. His mother's condition was aggravated by her 2 pregnancies. This family shows vertical transmission (compatible with autosomal dominant or mitochondrial inheritance) of the abdominal phenotype of prune belly sequence without any evidence of urinary tract or renal pathology. The expression in the sons may remain incomplete because abdominal distention due to pregnancy will not occur.- - - - - - - - - - ranking = 0.0040062765928362keywords = pregnancy (Clic here for more details about this article) |
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