Laparoscopic-Assisted Endorectal Pull-Through in Hirschsprung’s Disease and Familial Adenomatous Polyposis

Abstract

Hirschsprung’s disease (HD) is a congenital rare condition characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, and it is one of the most common causes of intestinal obstruction in the newborn. The disease occurs as a consequence of abnormal migration/differentiation of neural crest-derived neuroblasts into the developing gut that determines absence of intestinal intramural ganglia. In most of the cases, the aganglionosis involves the rectum or rectosigmoid tract, but it may extend proximally involving the whole colon or even a tract of the small bowel. The incidence of HD is approximately 1:5000 live births with male preponderance [1]. The usual presentation of a neonate affected by HD is intestinal obstruction during the first few days of life. The most feared complication is enterocolitis. The gold standard for the diagnosis of HD is rectal suction biopsy [2].