prune belly syndrome


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prune bel·ly syn·drome

a syndrome of deficient abdominal muscle, undescended testes, large hypotonic bladder and dilated, tortuous ureters.

prune belly syndrome

Eagle-Barrett syndrome Pediatrics A condition characterized by congenital lack of abdominal muscles, which imparts a rugose, prune-like appearance to the flaccid abdominal wall; 97% occur in ♂ and are accompanied by GU anomalies–eg, bilateral cryptorchidism, hypoplastic and dysplastic kidneys; affected ♀ have uterine defects; although considered an X-linked disease, no chromosome defect has been identified, and PBS may represent a 'sequence' initiated by in utero urethral obstruction, causing urinary tract anomalies–megaureters, megabladder, patent urachus or urachal cyst; other findings include Potter's facies, talipes, hip dislocation, musculoskeletal and cardiac defects Treatment Corsets, excision of redundant tissue Prognosis Oligohydramnios may arise in utero, causing fetal pulmonary hypoplasia, 20% are stillborn, 50% die in infancy. See Oligohydramnios.

Eagle,

J.F., Jr., 20th century U.S. physician.
Eagle-Barrett syndrome - Synonym(s): prune belly syndrome

Barrett,

Norman Rupert, English surgeon, 1903-1979.
adenocarcinoma in Barrett esophagus - an adenocarcinoma arising in the lower third of the esophagus that has become columnar cell lined (Barrett mucosa) due to gastroesophageal reflux.
Barrett epithelium - columnar esophageal epithelium seen in Barrett syndrome.
Barrett esophagus - chronic peptic ulceration of the lower esophagus acquired as a result of long-standing chronic esophagitis. Synonym(s): Barrett syndrome; Barrett ulcer
Barrett syndrome - Synonym(s): Barrett esophagus
Barrett ulcer - Synonym(s): Barrett esophagus
Eagle-Barrett syndrome - Synonym(s): prune belly syndrome

Obrinsky,

William, U.S. physician, 1913–.
Obrinsky syndrome - aplasia or hypoplasia of the abdominal wall. Synonym(s): prune belly syndrome
References in periodicals archive ?
The 6 testes in fetuses with prune belly syndrome and the 14 testes in the control group had abdominal testis.
Bilateral cryptorchidism is characteristic of prune belly syndrome [1-3].
[7] showed that all patients with PBS studied had atypical germ cells with large nuclei and prominent nucleoli, and intense alkaline phosphatase staining localized in the cytoplasmic membrane, suggesting developmental arrest in undescended testes associated with prune belly syndrome. Uehling et al.
The testis structure analyzed from the fetuses with prune belly syndrome showed lower concentration of Leydig cells.
Kogan, "Testicular histology in fetuses with the prune belly syndrome and posterior urethral valves," Journal of Urology, vol.
As with posterior urethral valves, patients with Prune Belly syndrome will demonstrate a spectrum of disease manifestations.
DISCUSSION: Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 30,000 to 1 in 50,000 births and 3.8 in 100,000 live births.
Children with prune belly syndrome must be closely monitored for the development of testicular tumors.
DIAGNOSIS: Prune belly syndrome can be diagnosed antenatally by ultrasound.
Diagnosis of prune belly syndrome necessitates thorough orthopedic and cardiological evaluation and treatment because of the high prevalence of associated musculoskeletal and cardiac abnormalities.
(14) If an antenatal diagnosis of urinary obstruction is made, it may be possible to perform vesicoamniotic shunting and intrauterine surgery to prevent the development of prune belly syndrome. The results seem promising.
Contemporary epidemiology of newborn males with prune belly syndrome. Urology.