Telocytes, c-Kit positive cells, Smooth muscles, and collagen in the ureter of pediatric patients with congenital primary obstructive megaureter: elucidation of etiopathology.

Congenital primary obstructive megaureter (POM) is an uncommon pediatric anomaly that is due to obstructive distal ureter leading to the loss of peristalsis with consequent ureterohydronephrosis causing loss of kidney function. The objectives are to elucidate the etiology of POM by demonstrating the presence of interstitial cells of Cajal (ICC), telocytes, smooth muscles, and collage in the obstructive and dilated ureteral segments. The study was carried out on 15 surgical specimens of congenital POM in pediatric patients, age range was 4 to 24 months, they were operated upon by excision of the obstructed segment, tailoring the dilated ureter, and anastomosing it to the bladder. Specimens included the obstructed ureteral segment and part of the dilated ureter. Specimens were examined with hematoxylin and eosin (H&E) stain, Modified Gomori trichrome stain, immunohistochemistry (IHC) with α-muscle actin, and c-kit (CD117), and transmission electron microscopy (TEM). Obstructed segment showed excess collagen intervening between smooth muscles, excess c-Kit positive cells, and presence of telocytes. The dilated segment of the ureteral wall is formed of smooth muscle bundles with scanty collagen. Staining with c-Kit did not demonstrate positive cells. TEM showed myofibroblasts and close adherence of smooth muscle cells to each other with absence of telocytes. The pathophysiology of POM is multifactorial. Loss of interstitial cells and rarity of collagen result in loss of elasticity of dilated segment leading to massive dilatation. While the obstructed segment had no muscle conductivity due to excess collagen irrespective of presence of telocytes.