scholarly journals Prune Belly syndrome: a case report

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.

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

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
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.

scholarly journals PRUNE BELLY SYNDROME: A CASE REPORT

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.

Aplasia of the Epiglottis: A Rare Congenital Anomaly

1998 ◽  
Vol 77 (1) ◽  
pp. 51-55 ◽  
Author(s):  
Jose A. Bonilla ◽  
Michael P. Pizzuto ◽  
Linda S. Brodsky
Keyword(s):  
Heart Failure ◽  
Computed Tomography ◽  
Congenital Anomaly ◽  
Obstructive Sleep Apnea ◽  
Case Report ◽  
Clinical Course ◽  
Embryological Development ◽  
Review Of The Literature ◽  
Rare Congenital Anomaly ◽  
Obstructive Sleep

Aplasia of the epiglottis is a rare laryngeal anomaly. We present a case of absence of the epiglottis in a child whose clinical course has been followed for nine years. She required a tracheostomy at two years of age for obstructive sleep apnea which resulted in heart failure; she was eventually decannulated at age seven. This case report highlights the clinical challenges faced in the identification and treatment of the sequelae of this defect. Both endoscopic and computed tomography (CT) documentation are provided. Embryological development and a review of the literature are also discussed.

scholarly journals Prune-belly syndrome

2009 ◽  
Vol 49 (5) ◽  
pp. 304
Author(s):  
Yenny Yenny ◽  
Kusuma P. A ◽  
Damanik M. P.
Keyword(s):  
Congenital Anomaly ◽  
Prognostic Factors ◽  
Urinary Tract ◽  
Pulmonary Hypoplasia ◽  
Clinical Findings ◽  
Prune Belly Syndrome ◽  
Live Births ◽  
Prune Belly ◽  
Clinical Variants ◽  
Urinary Tract Anomalies

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.

scholarly journals Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age – case report and review of the literature

2020 ◽  
Author(s):  
Adrian Surd ◽  
Dan Gheban ◽  
Aurel Mironescu ◽  
Cornel Aldea ◽  
Horațiu Gocan
Keyword(s):  
Congenital Anomaly ◽  
Abdominal Distension ◽  
Colonic Perforation ◽  
Prune Belly Syndrome ◽  
Feeding Intolerance ◽  
Meconium Peritonitis ◽  
Review Of The Literature ◽  
Rare Congenital Anomaly ◽  
Distended Abdomen ◽  
Hypoperistalsis Syndrome

 We present a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a four days old female infant who presented with abdominal distension, bilious vomiting, massive hematuria and feeding intolerance which was first interpreted as Prune Belly Syndrome (PBS), referred to our department after iatrogenic gastric and colonic perforation. Berdon syndrome or MMIHS is a rare congenital anomaly characterized by a massive enlarged bladder, distended abdomen, microcolon, functional obstruction of the gastrointestinal tract, and malrotation.

scholarly journals Cor triatriatum dexter, a very rare congenital anomaly presented in a complex pathological context – case presentation

2014 ◽  
Vol 60 (4) ◽  
pp. 160-162
Author(s):  
Liliana Gozar ◽  
Cristina Blesneac ◽  
Rodica Toganel
Keyword(s):  
Congenital Anomaly ◽  
Cytomegalovirus Infection ◽  
Cor Triatriatum ◽  
Anatomical Feature ◽  
Renal Anomalies ◽  
Anatomical Variant ◽  
Case Presentation ◽  
Congenital Cytomegalovirus ◽  
Rare Congenital Anomaly ◽  
Cor Triatriatum Dexter

Abstract Background Cor triatriatum dexter is an extremely rare congenital anomaly, and in most cases, without hemodynamic significance. Congenital cytomegalovirus infection is the most common viral infection, the majority of infected infants being asymptomatic and only 5-15% being symptomatic in the neonatal period. Case report We present the case of a female infant, aged 3 months. Clinical examination reveals cranial and facial anomalies. The karyotype was normal. Laboratory tests revealed the presence of anti-CMV antibodies in urine. Imaging studies showed cerebral and renal anomalies. Echocardiographic exam revealed a congenital anatomical feature without hemodynamic significance - cor triatriatum dexter. Conclusions although cytomegalovirus infection is mostly asymptomatic, it can present as a complex pathological association, in which a congenital cardiac anatomical feature without hemodynamic significance, considered by some authors as an anatomical variant, can appear

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