Superior vesical fissure in a case of prune belly syndrome with infravesical obstruction: Beneficial pop-off with a fatal congenital anomaly
Journal Title: Pediatric Urology Case Reports - Year 2018, Vol 5, Issue 4
Abstract
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.
Authors and Affiliations
Umesh Bahadur Singh, Shrikesh Singh, Tanvir Roshan Khan
Keywords
Related Articles
- EP ID EP609716
- DOI 10.14534/j-pucr.2018440299
- Views 60
- Downloads 0
Management issues in a patient of posterior urethral valve with Renal rickets
Posterior urethral valves (PUV) are the most common cause of lower urinary tract obstruction in male neonates. The course and treatment of the disease can further be complicated if it coexists with Renal rickets. We pres...
Hair-bearing epithelium serving as a nidus for intravesical stone formation in a male who underwent exstrophy repair: A case report
Bladder calculi are rare in children; they are typically associated with congenital abnormalities of the bladder, foreign bodies, intestinal mucosa, or infection. There is a high risk of bladder calculi with bladder exst...
A rare cause of pediatric urinary incontinence: Ventriculoperitoneal shunt with bladder perforation
We present a case of 2-year-old boy with long term dysuria and intermittent incontinence, and new onset of fever and headache. Significant past medical history includes congenital hydrocephalus with a ventriculoperitonea...
Fournier's gangrene with urethral involvement in a four-year-old child with neuropathic bladder: A rare case report
A four-year-old boy presenting with neuropathic bladder and hydrocephalus developed Fournier’s gangrene of the scrotum and perineum. At first, he was in septic shock and intubated. After debridement and grafting, he was...
Bladder dysfunction and urinary tract infection caused by abdominal pseudocyst with a ventriculoperitoneal shunt: A case report with literature review
The lower urinary tract dysfunction of the neurogenic bladder often worsens owing to dysfunction of the bladder itself. We treated a patient with spina bifida who experienced bladder dysfunction that worsened owing to an...