Related Experiment Video
Updated: Jun 24, 2026
Robotic Myotomy and Partial Fundoplication for Achalasia
Published on: August 11, 2023
Myectomy-myotomy for long segment Hirschsprung's disease in a patient with Haddad syndrome
Iyore A Otabor1, Jane P Balint, Gail E Besner
1Department of Pediatric Surgery, Nationwide Children's Hospital and The Ohio State University College of Medicine, Columbus, OH 43205, USA.
Insights
Hirschsprung
Area of Science:
- Pediatric surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease is a rare congenital disorder affecting the large intestine.
- It impacts nerve cell development in the colon, leading to blockages.
Observation:
- Long segment Hirschsprung's disease involves a more extensive affected colon length.
- This specific subtype occurs in 5-10% of Hirschsprung's disease cases.
Findings:
- The abstract defines long segment Hirschsprung's disease by a transition zone proximal to the sigmoid colon.
- It highlights the association between Hirschsprung's disease and congenital central hypoventilation syndrome, known as Haddad syndrome.
Implications:
- Understanding long segment Hirschsprung's disease is crucial for surgical planning and patient outcomes.
- Recognizing Haddad syndrome is vital for comprehensive patient care, addressing both gastrointestinal and respiratory issues.
Abstract:
Hirschsprung's disease is a rare entity with an incidence of 1 in 5000 live born infants. Long segment Hirschsprung's disease occurs in approximately 5% to 10% of this patient population and is defined as a transition zone proximal to the sigmoid colon (Bodian M, Carter CO, Ward BC. Hirschsprung's disease. Lancet. 1951;1:302-309). The association of congenital central hypoventilation syndrome (also known as Ondine's curse) and Hirschsprung's disease is termed Haddad syndrome.
