Abu Dhabi-based Sheikh Khalifa Medical City (SKMC) and UAE University in Alain have joined forces to develop new guidelines on a rare congenital disorder of the gastrointestinal tract known as Hirschsprung’s disease.

Hirschsprung disease is characterised by a partial or complete blockage of the large intestine due to improper muscle movement in the bowel, as a result of failure of the nerves to properly reside in their designated sites within the bowel.

The global incidence of the disease is one in 5,000 live births, and affects four times as many males as females, said the doctors who descended on SKMC last week to discuss updates on the rare congenital disorder.

In collaboration with UAE University in Al Ain, the workshop, targeted at pathologists, paediatric surgeons and paediatricians, aimed at developing new guidelines to improve diagnosis and management of the disease in the country.

Prevalence in the UAE is still unknown, specialised pathologists have often found that a misunderstanding of the disease, and sometimes miscommunication, has resulted in a delay in diagnosis and even misdiagnosis, said an expert.

"There are known international guidelines for more prevalent and well known diseases, like certain cancers, that are followed universally, but we don’t have anything in place for Hirschsprung disease," remarked Dr Suhaila Al Ameeri, the chief of Anatomical Pathology and Consultant Histopathologist, SKMC, who organized the workshop.

"We need to ensure that all physicians that may come across this disorder have the most updated literature available to them, and we must all work together to develop a roadmap in terms of diagnosis and management to ensure the best outcomes for patients,” she stated.

Dr Saeeda Almarzooqi, the assistant professor at UAE University, Consultant Histopathologist at Tawam Hospital, and co-organizer of the workshop said, "We’re focusing mainly on creating guidelines for diagnosis in terms of pathology, first starting across Seha hospitals, and hopefully eventually across the UAE, to minimise error and also to ensure best practice in our state of the art hospitals."

The workshop covered clinical perspectives of lower GI tract motility disorders, pathological diagnosis of Hirschsprung disease, common pitfalls of assessment of the disease, and concluded with proposed guidelines for pathological assessment in Seha hospitals, she stated.

SKMC, managed by Cleveland Clinic, is part of the Seha HealthSystem and owned and operated by Abu Dhabi Health Services Company, which is responsible for the curative activities of all the public hospitals and clinics in the emirate.-TradeArabia News Service