In a survey, mamy children are examined and were found to have urogenital abnormalities. The ones having urothelial cancers are most likely to be associated with which anomaly: (AIPG2012)
|A||Medullary sponge kidney|
|C||Unilateral renal agenesis|
a. Classic bladder exstrophy
i. The bladder is open on the lower abdomen, with mucosa fully exposed through a triangular fascial defect. The abdominal wall appears long because of a low-set umbilicus on the upper edge of the bladder plate. The distance between the umbilicus and anus is foreshortened. Rectus muscles diverge distally, attaching to the widely separated pubic bones.
ii. Indirect inguinal hernias are frequent (>80% of males, >10% of females) due to wide inguinal rings and the lack of an oblique inguinal canal.
b. Epispadias: The pubic symphysis is generally widened. The rectus muscles are divergent distally.
c. Cloacal exstrophy
i. Nearly all patients have an associated omphalocele. The bladder is open and separated into 2 halves, flanking the exposed interior of the cecum.
ii. Openings to the remainder of the hindgut and to 1 or 2 appendices are evident within the cecal plate. Terminal ileum may prolapse as a "trunk" of bowel onto the cecal plate.
d. Exstrophy variants
i. The pubic symphysis is widely separated, and rectus muscles diverge distally.
ii. The umbilicus is low or elongated. A small superior bladder opening or a patch of isolated bladder mucosa may be present.
iii. The intact bladder may be externally covered by only a thin membrane. Isolated ectopic bowel segments have been reported. Patent urachus is a differential diagnosis for the superior vesical fissure variant of exstrophy-epispadias.
iv. However, patent urachus lacks the typical musculoskeletal abnormalities of exstrophy and is open at the umbilicus. Superior vesical fissure is infraumbilical.