Prune belly syndrome is a rare congenital anomaly usually presented with triad characteristic feature of deficient abdominal muscles, cryptorchidism, and urinary tract anomalies. Here, we present a case with all the characteristic features of prune belly and the associated secondary features which were observed on detailed dissection and exploration of the fetus.
Abdominal Muscles ; Anus, Imperforate ; Cryptorchidism ; Fetus* ; Hydronephrosis ; Male ; Prune Belly Syndrome* ; Urinary Tract

No abstract available.
Humans ; Prune Belly Syndrome*

Prune-belly syndrome may be related to lower urinary tract obstruction (LUTO). LUTO in the early gestational age exacerbates fetal renal function and may require intrauterine intervention. If early developed LUTO causes bladder distension and abdominal musculature deficiency, it will result in prune belly syndrome. Therefore, early detection of the disease and proper treatment before the renal impairment is important. However, there are few literatures concerning the treatment of prune belly syndrome in the first trimester. We report a case of prune belly syndrome diagnosed at 11+6 weeks of gestation and the value of vesicocentesis as a modality of treatment. Ultrasound showed dilated fetal bladder and vesicocentesis was successful in reducing the volume of the bladder. However, the pregnancy was terminated upon request.
Cryptorchidism ; Female ; Gestational Age ; Humans ; Male ; Pregnancy ; Pregnancy Trimester, First ; Prune Belly Syndrome ; Urinary Bladder ; Urinary Tract

Funnel Chest ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Male ; Prune Belly Syndrome ; complications ; Situs Inversus ; complications ; Spleen ; abnormalities

Prune-belly syndrome is characterized by absent abdominal wall musculature with wrinkled overlying skin, urinary tract dilatation and cryptorchidism. Prune-belly syndrome is also associated with diseases of the respiratory, cardiovascular, skeletal, gastrointestinal and central nervous system. Because the congenital disease is uncommon, it is difficult to collect the information of anesthetic management of prune-belly syndrome. We report a case of 4 year-old-boy with prune-belly syndrome who underwent abdominoplasty and Mitrofanoff operation under general anesthesia.
Abdominal Wall ; Abdominoplasty ; Anesthesia, General ; Central Nervous System ; Cryptorchidism ; Dilatation ; Humans ; Male ; Prune Belly Syndrome ; Skin ; Urinary Tract

Humans ; Prune Belly Syndrome

Congenital megacystis with bilateral hydroureteronephrosis is most commonly associated with posterior urethral valves, prune-belly syndrome, vesicoureteral reflux, or nonrefluxing, nonobstructive megaureters. Among the other cause of congenital megacystis, megacystis-microcolon-intestinal hypoperistalsis syndrome(MMIHS), which is characterized by megacystis, microcolon and hypoperistalsis of the intestines with resultant abdominal distension, is a rare cause of functional obstruction of urinary tracts in childhood. It was first reported by Berdon, et al. in 1976, and only 89 cases have been reported until the present. There has been no report in Korea. We report a 2 month old female patient who exhibited intestinal malrotation, megacystitis, abdominal distension and hypoperistalsis. She did not show any evidence of microcolon, but her biopsy result exhibited degenerative changes of intestinal and cystic smooth muscle, pointing to a syndrome very similar to MMIHS.
Biopsy ; Female ; Humans ; Infant ; Intestines ; Korea ; Muscle, Smooth ; Prune Belly Syndrome ; Urinary Retention* ; Urinary Tract ; Vesico-Ureteral Reflux

Prune-belly syndrome is a rare compound fetal anomaly, characterized by absence or hypoplasia of abdominal wall musculature associated with urinary tract abnormalities and cryptorchidism. The prenatal ultrasound diagnosis was based on the findings of a lower abdominal cystic echo caused by abnormal dilatation of the bladder, upward compression of small intestines and decreased amniotic fluid volume. We experienced a case of Prune-belly syndrome diagnosed by ultrasound in a 12 weeks fetus following to IVF-ET pregnancy. Termination was performed at 12 weeks and autopsy confirmed the distended bladder, absence of abdominal muscles and urethra. So, we reported this case with a brief review of literature.
Abdominal Muscles ; Abdominal Wall ; Amniotic Fluid ; Autopsy ; Cryptorchidism ; Diagnosis ; Dilatation ; Female ; Fetus ; Intestine, Small ; Male ; Pregnancy ; Prenatal Diagnosis ; Prune Belly Syndrome* ; Ultrasonography* ; Urethra ; Urinary Bladder ; Urinary Tract

PURPOSE: A study was done to assess the incidence, clinical characteristics of urinary tract anomalies for decreasing urinary tract morbidity. METHODS: We review 98 cases of anomaly of urinary tract who were admitted Kyung Hee University Hospital between 1986 and 1995. We analyzed incidence and associated anomalies, associated diseases and treatment modalities. RESULTS: 1) It was composed of 45 cases (45%) of renal anomalies, 37 cases (37.7%) of ureteral anomalies, 7 cases (7.1%) of urethral anomalies, 3 cases Prune-belly syndrome, 3 cases of urachal remnants, 2 cases of bladder exstrophy, 1 cases of VATER syndrome. 2) Anomalies of the kidney were composed of 14 cases of renal agenesis, 11 cases of multicystic dysplastic kidney, 10 cases of hydronephrosis 3 cases of hypoplasia, 3 cases of polycystic kidney, 2 cases of ectopia, 1 case of malrotation and 1 case of horseshoe kidney, 20 cases (44.4%) were diagnosed before 1 month of life. 20 cases were male and 25 casses were female. Bilateral involvement were in 7 cases and 38 cases of unilateral involvement were composed of 23 cases of right side and 15 cases of left side. Operative treatment were performed in 15 cases (30%) of renal anomalies. The common chief complaint of renal anomalies were abnormal finding on urinalysis (24.4%), abnormal finding on fetal ultrasonogram (20%), gastrointestinal tract symptom (15.6%), and fever (9%). 3) Anomalies of the ureter were composed of 26 cases of ureteral duplication, 9 cases of UPJ obstruction, 2 cases of megaureter. Ureteric duplications included 8 cases of male and 18 cases of female and 19 cases were unilateral and 7 cases were bilateral. 19 cases were diagnosed before 5 years old. 19 cases (73.1%) had symptoms associated with urinary tract infection. 11 cases had abnormal finding of ipsilateral kidney on DMSA scan or IVP. Associated abnormalities were hydronephrosis, ureterocele and VUR. UPJ obstruction were mostly diagnosed before 1 month of life, 6 cases were male and 3 cases were female. 2 cases were bilateral and 3 cases were right side involvement and 4 cases were left side. CONCLUSIONS: It seems to be reasonable that we should recommend the patients with the symptoms of urinary tract diseases to do evaluate the possibility of congenital urinary tract anomalies.
Bladder Exstrophy ; Child* ; Child, Preschool ; Female ; Fever ; Gastrointestinal Tract ; Humans ; Hydronephrosis ; Incidence ; Kidney ; Male ; Multicystic Dysplastic Kidney ; Polycystic Kidney Diseases ; Prune Belly Syndrome ; Succimer ; Ultrasonography ; Ureter ; Ureterocele ; Urinalysis ; Urinary Tract Infections ; Urinary Tract* ; Urologic Diseases

No abstract available.
Prune Belly Syndrome*