the term ultra short is not clearly defined in ultrashort-segment hirschsprung’s disease. the limited extent of the ultrashort-segment hirschsprung’s disease allows for treatment with extended sphincteromyectomy. in anal sphincter achalasia, anal sphincter dilatation under general anesthesia may be sufficient to treat the condition; in cases with persistent constipation, sphincteromyectomy is i...

A case of peritoneal mucinous carcinomatosis in a patient who suffered a Hirschsprung disease 30 year before is presented. TH present condition caused an irreversible intestinal obstruction and the patients received home parenteral nutrition without unremarkable complications longer than two years.

Primary clinical features Retinitis pigmentosa (rod-cone dystrophy) Obesity Polydactyly Hypogonadism Intellectual disability/cognitive impairment Renal abnormalities Secondary clinical features Diabetes mellitus type II Cardiovascular problems Hearing loss Speech deficiency Behavioral problems Craniofacial dysmorphism Short stature Hepatic involvement Eye abnormalities Ataxia Dental and palatal...

MWS is a multiple congenital anomaly syndrome, first clinically delineated by Mowat et al in 1998. Over 45 cases have now been reported. All patients have typical dysmorphic features in association with severe intellectual disability, and nearly all have microcephaly and seizures. Congenital anomalies, including Hirschsprung disease (HSCR), congenital heart disease, hypospadias, genitourinary a...

  The diagnosis of functional constipation (FC) is usually straightforward. Almost 95% of childhood constipation is functional in nature. The remaining 5% can be attributed to wide variety of conditions. Many of these etiologies are obvious by history and examination and many have other specific symptoms besides constipation. It is especially important to look for presence of any symptoms or si...

This is a case report of bilateral pyosalpinx in a peripubescent female with a history of Hirschsprung's disease. Bilateral pyosalpinx is a rare condition in non-sexually active females. The presence of this disease in a patient with a history of Hirschsprung's disease is concerning for an association of the two processes.