Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly

Prune-belly syndrome, also known as Eagle-Barretsyndrome, is a congenital anomaly comprisingthree clinical findings: deficient abdominalmusculature, urinary tract anomalies, andbilateral cryptorchidism. Other clinical findings involvingrespiratory, skeletal, digestion and cardiovascular systemmay also accompany the syndrome. The incidence isapproximately 1 : 30,000 to 40,000 live births and 95%of cases occur in boys. Pulmonary hypoplasia and kidneyfailure are important prognostic factors that contributeto 60% of mortality rate. Treatment includes surgicalcorrection of the abdominal wall and urinary tract,orchidopexy and other supportive managements.l-4 Wereport 4 cases on typical Prune-belly syndrome, togetherwith other clinical variants.

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Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence in a Female Newborn with Absent Perineal Openings

Case Reports in Surgery ◽

10.1155/2014/746323 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Azhar Farooqui ◽

Alaa AlAqeel ◽

Zakaria Habib

Keyword(s):

Congenital Anomaly ◽

Case Report ◽

Abdominal Wall ◽

Ambiguous Genitalia ◽

Prune Belly Syndrome ◽

Rare Congenital Anomaly ◽

Prune Belly ◽

Bilateral Cryptorchidism ◽

Original Case ◽

Classical Triad

Prune belly syndrome (PBS) is a rare congenital anomaly characterized in males by a triad of anomalous genitourinary tract, deficient development of abdominal wall muscles, and bilateral cryptorchidism. Although similar anomalies have been reported in females, by definition they do not full fill the classical triad. Urorectal septum malformation sequence (URSM) is a lethal condition characterized by presence of ambiguous genitalia, absent perineal openings (urogenital and anal), and lumbosacral abnormalities. In this original case report, the authors discuss the presentation and management of what would be analogous to a Woodhouse category 1 PBS in a female newborn associated with an overlapping presentation of URSM.

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Prune Belly syndrome: a case report

Modern pediatrics. Ukraine ◽

10.15574/sp.2020.110.51 ◽

2020 ◽

pp. 51-56

Author(s):

O.L. Tsymbalista ◽

◽

B.M. Ziniak ◽

O.O. Kurtash ◽

V.P. Valchyshyn ◽

...

Keyword(s):

Congenital Anomaly ◽

Conflict Of Interest ◽

Congenital Malformations ◽

Clinical Course ◽

Prune Belly Syndrome ◽

Non Profit ◽

Rare Congenital Anomaly ◽

Regional Council ◽

Clinical Hospital ◽

Prune Belly

Objective: to acquaint doctors of various specialties with clinical course, early diagnostics, and treatment strategy for a rare congenital anomaly — Prune Belly syndrome in children. Materials and methods. Inpatient medical records of municipal non-profit enterprise «Ivano-Frankivsk Regional Children's Clinical Hospital» of Ivano-Frankivsk Regional Council. Results. The paper presents the clinical course of a rare congenital anomaly — Prune Belly syndrome in two children. The disease occurs chiefly among boys manifesting itself as multiple congenital malformations including deficiency or aplasia of abdominal muscles, congenital anomalies of the kidneys and urinary tract (hydronephrosis, megaureter, hydroureter, megacystis, vesicoureteral reflux), bilateral cryptorchidism. In addition, Prune Belly syndrome can coexist with urethral pathology, as well as skeletal, cardiovascular, respiratory, gastrointestinal malformations. Conclusion. Modern views on the development, clinical course, and diagnosis of a rare congenital disease with multiple organ malformations — Prune Belly syndrome have been presented for doctors of various specialties. Prenatal diagnosis, detection of potential congenital malformations after birth allows selecting the correct algorithm for monitoring vital organ functions, syndromic treatment and making timely decision on internal organ transplantation. The study was carried out in accordance with the ethical principles of the Declaration of Helsinki. The study protocol was approved by the Local Ethics Committee of municipal non+profit enterprise «Ivano-Frankivsk Regional Children's Clinical Hospital» of Ivano-Frankivsk Regional Council. The informed consent to participate in the research study was obtained from all the children's parents. Conflict of Interest. The authors declare they have no conflict of interest. Key words: children, Prune Belly syndrome, diagnosis, therapy.

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PRUNE BELLY SYNDROME: A CASE REPORT

Ukrainian Journal of Nephrology and Dialysis ◽

10.31450/ukrjnd.1(45).2015.09 ◽

2014 ◽

pp. 65-69

Author(s):

M.I. Solodkiy ◽

K.A. Dzhuma ◽

R.V. Zhezhera ◽

O.J. Vylkov

Keyword(s):

Congenital Anomaly ◽

Case Report ◽

Urinary Tract ◽

Abdominal Muscle ◽

Prune Belly Syndrome ◽

Urinary Tract Abnormality ◽

Rare Congenital Anomaly ◽

Prune Belly ◽

Ultrasound Investigation ◽

Different Levels

Prune belly syndrome (PBS) is a rare congenital anomaly almost exclusive to males defined by the triad of abdominal muscle deficiency, severe urinary tract abnormality and cryptorchidism. The syndrome has a broad spectrum of affected anatomy with different levels of severity. We report a newborn boy with PBS. Diagnosis was confirmed by karyotyping, ultrasound investigation and intraoperative findings.

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