Long-term morbidity in total colonic aganglionosis

Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve

“…Shandling combined the total ileo-colonic side-to-side anastomosis with an endorectal pull-through procedure according to Soave [8]. However, utilization of the total aganglionic colon does not only bear a higher surgical complication risk but is also associated with a higher incidence of enterocolitis and bowel obstruction, wherefore this technique has been abandoned in several institutions [18][19][20]. Sophisticated research investigations in animal studies and human subjects have shown that the right colon has superior efficiency compared to the transverse and left colon in terms of water and electrolyte absorptive capacities, also leading to a reduced transit time [21][22][23][24][25][26][27][28][29][30].…”

Acute diverticulitis after Duhamel–Martin procedure for total colonic Hirschsprung’s disease

“…They have higher rates of preoperative morbidity including enterocolitis, complications associated with parenteral nutrition, and overall failure to thrive [1]. A major advancement in the treatment of these patients came in 1968 when Martin reported the results of his series of five children with TCA in whom he modified the Duhamel pull-through procedure by the creation of a long jejunocolonic anastomosis [7,8].…”

“…The absence of intramural ganglion cells in the distal alimentary tract, commonly known as Hirschsprung's disease (HD), affects the entire colon and distal ileum in approximately 5-15% of children diagnosed [1,2]. Surgical treatment of these children can be technically challenging.…”

“…A variety of surgical pull-through procedures for total colonic aganglionosis (TCA) have been reported each associated with variable outcomes and complications. The majority of long-term follow-up data focuses on functionality and overall quality of life [1][2][3][4][5][6]. Common complications after definitive pullthrough procedures include recurrent constipation, irregular soiling, enterocolitis, bowel obstruction, rectal stricture, and fecaloma [1,3,5].…”

Total Colonic Aganglionosis (Zuelzer Wilson Syndrome)

“…Enterostomy alone carries a mortality rate of more than 60% [9]. Recently Hoehner et al [10] claim improved results with 97% survival in this disease. The significant improvement in survival is a result of early recognition and diagnosis, progress in parenteral and enteral nutrition with improvement in supportive care and innovation of various surgical techniques.…”