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.

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.

CONGENITAL METACARPAL SYNOSTOSIS TREATED BY DOUBLE BONE BLOCKS TECHNIQUE: A CASE REPORT FROM THAILAND

Hand Surgery ◽  
2005 ◽  
Vol 10 (01) ◽  
pp. 131-134 ◽  
Author(s):  
Surut Jianmongkol ◽  
Tala Thammaroj ◽  
Kitiwan Vipulakorn
Keyword(s):  
Congenital Anomaly ◽  
Case Report ◽  
Surgical Techniques ◽  
Rare Condition ◽  
Rare Congenital Anomaly

Congenital metacarpal synostosis is a rare congenital anomaly in the hand, especially in our area. There were several reports of surgical techniques for correction deformities. We report this rare condition in our hospital and treatment with the metacarpal osteotomy and double bone blocks technique of grafting.

scholarly journals Living-related kidney transplantation with catheterizable urinary conduit in prune belly syndrome: A case report

2018 ◽  
Vol 51 ◽  
pp. 150-153 ◽  
Author(s):  
Taro Banno ◽  
Yoichi Kakuta ◽  
Kohei Unagami ◽  
Akiko Sakoda ◽  
Masayoshi Okumi ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Case Report ◽  
Prune Belly Syndrome ◽  
Prune Belly ◽  
Living Related

Total Abdominal Wall Reconstruction in the Prune Belly Syndrome

1986 ◽  
Vol 136 (1 Part 2) ◽  
pp. 282-285 ◽  
Author(s):  
Richard M. Ehrlich ◽  
Malcolm A. Lesavoy ◽  
Richard N. Fine
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Reconstruction ◽  
Prune Belly Syndrome ◽  
Prune Belly

scholarly journals PRUNE-BELLY SYNDROME IN 2 CASES SEEN IN A TERTIARY MEDICAL INSTITUTION SOUTHEAST NIGERIA – A CASE REPORT

2019 ◽  
Vol 6 (6) ◽  
pp. 331-334
Author(s):  
Muoneke Vivian Uzoamaka ◽  
Nwokoye Ikenna ◽  
Ezeanosike Obumneme ◽  
Onyire Nnamdi Benson ◽  
Nwokeji-Onwe Linda ◽  
...  
Keyword(s):  
Case Report ◽  
Medical Institution ◽  
Prune Belly Syndrome ◽  
Prune Belly

scholarly journals Anesthetic Management of Prune-Belly Syndrome: Case Report

2015 ◽  
Vol 23 (3) ◽  
pp. 215-219
Author(s):  
Tuğçe BARÇA ŞEKER ◽  
Ayşe Çiğdem TÜTÜNCÜ
Keyword(s):  
Case Report ◽  
Anesthetic Management ◽  
Prune Belly Syndrome ◽  
Prune Belly

scholarly journals Rare presentation of incomplete male urethral duplication: A case report and review of literature

2021 ◽  
Vol 9 (1) ◽  
pp. 292-296
Author(s):  
Orgeness J Mbwambo ◽  
Alex Mremi ◽  
Mohamed Mbarouk ◽  
Jasper Mbwambo ◽  
Frank Bright ◽  
...  
Keyword(s):  
Congenital Anomaly ◽  
Case Report ◽  
Postoperative Period ◽  
Surgical Removal ◽  
Urethral Duplication ◽  
Review Of Literature ◽  
Rare Presentation ◽  
Rare Congenital Anomaly ◽  
Accessory Urethra

Urethral duplication is a rare congenital anomaly affecting mainly males. Here, we report a case of urethral duplication in a 19 years old male presented as a scrotal sinus discharging pus for 1 year. Surgical removal of accessory urethra was done and postoperative period was uneventful.

scholarly journals Incidental Finding of Annular Pancreas in a Routine Cadaveric Dissection: Case Report

2019 ◽  
Vol 36 (04) ◽  
pp. 299-302
Author(s):  
Mythraeyee Prasad ◽  
Theresa Susan Kuriakose ◽  
Sipra Rout
Keyword(s):  
Congenital Anomaly ◽  
Case Report ◽  
Duodenal Obstruction ◽  
Clinical Symptoms ◽  
Incidental Finding ◽  
Age Groups ◽  
Annular Pancreas ◽  
Present Case Report ◽  
Rare Congenital Anomaly ◽  
Variable Presentation

AbstractAnnular pancreas is a rare congenital anomaly that results from the malrotation of the ventral pancreatic bud. The presentation of annular pancreas varies: it can be asymptomatic or present clinical symptoms of duodenal obstruction that can affect all age groups, from newborns to adults. In the present case report, we describe a complete type of annular pancreas at the level of the second part of the duodenum, which was an incidental finding in a prosected specimen. This anomaly has significant clinical relevance to clinicians and radiologists due to its variable presentation. The embryological, clinical and radiological aspects of this congenital anomaly are discussed in detail in the present article.

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.

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