A note on the history of Hirschsprung’s disease, and an over 120 years apology


Celayir A., Çetiner H., Ağartan C. A., Günaydın R., CELAYİR A.

Pediatric Surgery International, vol.41, no.1, 2025 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 41 Issue: 1
  • Publication Date: 2025
  • Doi Number: 10.1007/s00383-025-06003-z
  • Journal Name: Pediatric Surgery International
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, CINAHL, MEDLINE, Veterinary Science Database
  • Keywords: Ganglion cell, Hirschsprung disease, History of medicine, Idiopathic colon dilatation, K. Tittel, Medical publications
  • Çanakkale Onsekiz Mart University Affiliated: Yes

Abstract

In 1886, Harald Hirschsprung presented what he believed to be a new and rare condition at the Berlin Congress for Children’s Diseases. In 1888, still unaware of earlier reports, he published a paper titled “Constipation in Newborns as a Consequence of Dilation and Hypertrophy of the Colon,” suggesting that the enlargement of the colon was congenital. Although he noted a narrowing of the rectum in his initial case, Hirschsprung attributed this condition to a dilated intestine rather than a narrowed rectum. In fact, F. Ruysch had described a case of over-enlarged colon as early as 1691, and in 1800, D. Battini published another case of severe constipation posthumously. Between 1825 and 1888, around 20 similar cases had been documented in medical literature. K. Tittel was the first person to draw attention to the narrow rectum and the absence or scarcity of ganglion cells in this region in 1901. However, he did not identify this as the exact cause of the disease, This was evident not only in his own article but also in references to his work in other publications. Despite this fact in the literature, the disease is known as “Hirschsprung Disease”. Given the historical and scientific context, it may be time to reconsider the attribution of K. Tittel’s discovery and to recognize the significant contributions of him in understanding of this disease.