Hirschsprung Disease

Mike Chen, MD, Andrew Zigman, MD, CM, Ankush Gosain, MD, PhD
Hirschsprung Disease is a topic covered in the Pediatric Surgery NaT.

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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 described as congenital megacolon and was for the most part lethal due to malnutrition and enterocolitis. It was first recognized by ancient Hindu surgeons but then not again until the 17th century [1][2]. In 1887 Harald Hirschsprung described two patients with the disease 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 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 Ehrenpreis was the first surgeon to recognize the problem and in 1947 Swenson made the first description of a successful reconstructive operation for the disease [5][6]. Swenson originally described the technique without a protecting ostomy but subsequent problems lead most surgeons to do the reconstruction in stages. More recently however, advances in technique and surgical care have allowed surgeons to revert to Swenson’s original 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

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Last updated: May 7, 2019