Structural changes of collagen components and diminution of nerves in congenital ureteropelvic junction obstruction

Purpose: Three-dimensional arrangements of smooth muscle cells, collagenous component and peripheral nerves of congenital ureteropelvic junction (UPJ) obstruction were studied in order to clarify the pathogenetic mechanism of interaction among these neuro-myo-stromal components. Materials and Methods: The UPJ and upper ureters were obtained from 14 patients with congenital hydronephrosis (7 intrinsic and 4 extrinsic obstruction) and 7 normal controls. Three-dimensional arrangement of each structural component was observed by scanning electron microscopy, and the nerve distribution was analyzed with immunohistochemistry for protein gene product 9.5. Results: The UPJ of intrinsic obstruction had structural features as follows. Muscle fascicles were sparse and thin. Each muscle cell was thin in diameter. Intercellular spaces were six to seven times wider than controls. Collagen fibrillar sheaths of smooth muscle cells (pericellular collagen fibrils attached to the basement membrane) were interwoven to form a dense felt-like structure against thin lace-like sheaths in controls. Interstitial collagenous component showed dense and compact structure against loose network of wavy collagen bundles in controls. In the muscular layer, nerve distribution was decreased to about one-third of controls. In contrast, non-stenotic portion of intrinsic UPJ obstruction as well as materials from extrinsic UPJ obstruction showed no structural difference as compared with controls. Conclusions: In the intrinsic obstruction, nerve fibers were depleted in the muscular layers in the ureteric walls, resulting in dysfunction and atrophy of muscle fibers and an increase of collagen fibers in the muscle layers with abnormal accumulation of intercellular and interstitial collagen. These changes may disrupt the mobility of UPJ and lead to both mechanical and functional obstruction.