Introduction

Hirschsprung disease is a common cause of lower intestinal obstruction in neonates and infants. It is a functional rather than mechanical obstruction caused by maldevelopment of the enteric nervous system and surgery is usually required to alleviate the obstruction. It was first recognized by ancient Hindu surgeons but then not again until the 17th century[1][2]. It was for the most part lethal due to malnutrition and enterocolitis. In 1887, Harald Hirschsprung described two patients with congenital megacolon and the disease was subsequently named for him[3]. At the time, the dilated colon was thought to be the pathologic specimen and was resected with subsequent attempts at anastomosis uniformly meeting with failure.

The absence of ganglion cells in the distal colon was first recognized by Karl von Tittel in 1901 and described many times subsequently although clinicians taking care of such patients took several decades to become aware of this finding [4]. In 1946, Theodor Ehrenpreis was the first surgeon to recognize the problem and in 1947, Orvar Swenson described the first successful reconstructive operation for the disease [5][6]. Swenson originally described the technique without a protecting ostomy but subsequent problems led most surgeons to do the reconstruction in stages. More recently however, advances in technique and surgical care have allowed surgeons to revert to a one stage technique with excellent results.

see also Total Colon Aganglionosis, Colostomy for Hirschsprung Disease, Myectomy for Hirschsprung Disease and Pull Through for Hirschsprung Disease

Content in this topic is referenced in SCORE Hirschsprung Disease