Harald Hirschsprung - Hirschsprung's Disease

Hirschsprung's Disease

Hirschsprung published on many areas of pediatrics, including pyloric stenosis, intussusception, rickets, and rheumatic nodules, but he is most well known for his work on the disease that later came to bear his name.

At the congress of the Gesellschaft für Kinderheilkunde (Berlin Congress for Children's Diseases) in Berlin, Hirschsprung gave a lecture about what would become "his" disease. It was titled "Stuhlträgheit Neugeborener in Folge von Dilatation und Hypertrophie des Colons" and was published one year later.

He spoke of two infants who had died from the disease, constipation associated with dilatation and hypertrophy of the colon, and near the end of his lecture said that: "it appears unquestionable that the condition is caused in utero, either as a developmental abnormality or as a disease process." He published an account of the disease, which he believed to be a new condition, two years later.

Although he was the first to describe the condition, he erroneously believed the proximal, dilated bowel to be diseased. We now know that the diseased segment of intestine is the distal portion (down to the rectum), which lacks ganglion cells and can therefore not relax. This contracted state (sometimes called rectal achalasia) prevents the passage of stools and causes intestinal obstruction and constipation.