Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly
A wide spectrum of renal, ureteral, and urethral abnormalities has been reported in a patient born with Prune belly syndrome (PBS). The obstruction in the urinary tract can be found anywhere from uppermost (pelviureteral junction) to lowermost (prostatic membranous urethra) urinary tract. The mesenc...
Main Authors: | , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Ped Urol Case Rep
2018-07-01
|
Series: | Pediatric Urology Case Reports |
Subjects: | |
Online Access: | http://pediatricurologycasereports.com/ojs/index.php/pucr/article/view/413/166 |
id |
doaj-90f8998db7b647fc977f3d36e7f7bcad |
---|---|
record_format |
Article |
spelling |
doaj-90f8998db7b647fc977f3d36e7f7bcad2020-11-25T00:19:49ZengPed Urol Case RepPediatric Urology Case Reports2148-29692148-29692018-07-01549910310.14534/j-pucr.2018440299Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly Umesh Bahadur Singh0Shrikesh Singh1Tanvir Roshan Khan 2Department of Pediatric Surgery, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India Department of Pediatric Surgery, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India Department of Pediatric Surgery, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India A wide spectrum of renal, ureteral, and urethral abnormalities has been reported in a patient born with Prune belly syndrome (PBS). The obstruction in the urinary tract can be found anywhere from uppermost (pelviureteral junction) to lowermost (prostatic membranous urethra) urinary tract. The mesenchymal developmental arrest is the main embryological factor responsible for the major features of the syndrome like urethral abnormalities or gastrointestinal abnormalities. PBS patients with urethral obstruction commonly had a patent urachus, because of which such patients survived, early death usually occurred in those patients who did not had a patent urachus. We are reporting an interesting case of prune belly syndrome with superior vesical fissure (an exstrophy variant). To the best of our knowledge this was first reported case of association of two congenital anomalies having common embryological defect process. Presence of superior vesical fissure gives a new insight about the benefits gained by the child for surviving with such fatal congenital anomalies. http://pediatricurologycasereports.com/ojs/index.php/pucr/article/view/413/166Prune belly syndromeexstrophy variantsuperior vesical fissure |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Umesh Bahadur Singh Shrikesh Singh Tanvir Roshan Khan |
spellingShingle |
Umesh Bahadur Singh Shrikesh Singh Tanvir Roshan Khan Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly Pediatric Urology Case Reports Prune belly syndrome exstrophy variant superior vesical fissure |
author_facet |
Umesh Bahadur Singh Shrikesh Singh Tanvir Roshan Khan |
author_sort |
Umesh Bahadur Singh |
title |
Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly |
title_short |
Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly |
title_full |
Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly |
title_fullStr |
Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly |
title_full_unstemmed |
Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly |
title_sort |
superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: beneficial pop-off with a fatal congenital anomaly |
publisher |
Ped Urol Case Rep |
series |
Pediatric Urology Case Reports |
issn |
2148-2969 2148-2969 |
publishDate |
2018-07-01 |
description |
A wide spectrum of renal, ureteral, and urethral abnormalities has been reported in a patient born with Prune belly syndrome (PBS). The obstruction in the urinary tract can be found anywhere from uppermost (pelviureteral junction) to lowermost (prostatic membranous urethra) urinary tract. The mesenchymal developmental arrest is the main embryological factor responsible for the major features of the syndrome like urethral abnormalities or gastrointestinal abnormalities. PBS patients with urethral obstruction commonly had a patent urachus, because of which such patients survived, early death usually occurred in those patients who did not had a patent urachus. We are reporting an interesting case of prune belly syndrome with superior vesical fissure (an exstrophy variant). To the best of our knowledge this was first reported case of association of two congenital anomalies having common embryological defect process. Presence of superior vesical fissure gives a new insight about the benefits gained by the child for surviving with such fatal congenital anomalies. |
topic |
Prune belly syndrome exstrophy variant superior vesical fissure |
url |
http://pediatricurologycasereports.com/ojs/index.php/pucr/article/view/413/166 |
work_keys_str_mv |
AT umeshbahadursingh superiorvesicalfissureinacaseofprunebellysyndromewithinfravesicalobstructionbeneficialpopoffwithafatalcongenitalanomaly AT shrikeshsingh superiorvesicalfissureinacaseofprunebellysyndromewithinfravesicalobstructionbeneficialpopoffwithafatalcongenitalanomaly AT tanvirroshankhan superiorvesicalfissureinacaseofprunebellysyndromewithinfravesicalobstructionbeneficialpopoffwithafatalcongenitalanomaly |
_version_ |
1725369943531192320 |