Jirásek-Zuelzer-Wilson syndrome

Classification according to ICD-10
Q43.1	Hirschsprung Disease Aganglionosis Megacolon congenitum (anganglionic)
ICD-10 online (WHO version 2019)

The Jirásek-Zuelzer-Wilson syndrome (usually also shorter Zuelzer-Wilson syndrome ) is a special form of congenital aganglionosis named after its descriptions, Wolf William Zülzer , James Leroy Wilson and Arnold Jirásek . From birth, the affected people lack the nerve cells (myenteric plexus) in the wall of the entire colon , possibly also parts of the ileum . This causes a disturbance in the passage of bowel movements in the large intestine and an expansion of the intestine ( megacolon ).

Those affected usually already show a distended stomach and defecation problems in infancy. The meconium is issued later after birth. The diagnosis is made by x-rays and examining tissue samples from the colon. After the diagnosis, an anus praeter is often created. Surgical production of adequate stool passage is difficult, e.g. Sometimes larger parts of the colon are removed.

See also

• Hirschsprung's disease
• Zuelzer-Kaplan syndrome

Individual evidence

1. ^ WW Zuelzer, JL Wilson: Functional intestinal obstruction on a congenital neurogenic basis in infancy. In: American Journal of Diseases of Children . Chicago 1948, 75, pp. 40-75.
2. ↑ A. Jirásek: On some intramural causes of spasms of the digestive tract. In: Acta Chirurgica Scandinavica . Stockholm, 1926, 59, pp. 91-99.

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !