Heterotopic nephrogenic rests in the colon and multiple congenital anomalies: possibly related association.
Heterotopic renal tissue (HRT) in the wall of the colon is a very rare occurrence, with only five cases published. Our patient is only the second patient reported to have this abnormality in the absence of sirenomelia. We describe colonic HRT in a child, associated with multiple congenital anomalies. The congenital abnormalities were of the VACTERL type, accompanied by valvular cardiac anomalies that were clinically diagnosed as Shone syndrome. The HRT was not apparent clinically or grossly. Microscopically, multifocal islands of renal tissue consisting of glomeruli, cystically dilated tubules, and blastema were seen in all layers of the bowel, and simulated "cystic partially differentiated nephroblastoma." Our case provides further support to the belief that VACTERL association and sirenomelia represent related entities.
Jain
Dhanpat
D
Department of Pathology, Yale University School of Medicine, P.O. Box 208070, New Haven, CT 06520-8070, USA.
Martel
Maritza
M
Reyes-Múgica
Miguel
M
Parkash
Vinita
V
D000015:Abnormalities, Multiple; D001003:Anal Canal / Q000002:abnormalities*; D001017:Aortic Coarctation / Q000150:complications; D001024:Aortic Valve Stenosis / Q000150:complications; D002828:Choristoma / Q000150:complications* / Q000473:pathology; D003106:Colon / Q000473:pathology; D003108:Colonic Diseases / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney; D013577:Syndrome