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Hirschsprung’s disease

Hirschsprung’s disease is a disorder of the nerve supply of the intestine, especially the large intestine, where the newborn is unable to have normal bowel movements. The feces get jammed in the large intestine, the tummy bloats and the obstruction can be disastrous, even fatal. Those who escape the newborn period have nagging constipation, poor growth and a host of infectious complications of the intestines later.

The diagnosis rests on detection of certain cells in a biopsy from the terminal part of the intestine just above the anus (rectum) and its careful interpretation. If this is abnormal and suggestive of Hirschsprung’s disease, more biopsies are taken further up from the intestine (large and small) either by a procedure through the anus or after opening the abdomen / with help of laparoscopy. Finally, the extent of normal and abnormal intestine is determined and further surgical treatment planned. The treatment can be one or many staged. Basically, the abnormal intestine is removed and normal intestine is joined at the anus so that the child can evacuate feces normally and normal growth and development is resumed.

The diagnosis is difficult in inexperienced hands and the consequent surgical misadventures can be prohibitive. Many centers across India either rely on just the x ray picture for a diagnosis and several pathologists are also unable to provide a clear diagnosis for the surgeon to decide – both of these being undesirable situation fraught with danger. At St. John’s Medical College and Hospital, Bangalore, we have devised a special, new, improvised, cost effective method of establishing the diagnosis with a rectal biopsy (rapid improvised acetyl cholinesterase staining) than what was conventionally practiced and reported in literature earlier. It provides for an accurate and conclusive diagnosis while the baby is on the operation table and allows the surgeon to proceed with the surgical management in the same sitting. The method needs pathologist’s expertise and close co ordination between the paediatric surgeon and pathologist. Using this method routinely in newborns, we have moved to one stage treatment of the common form of this disease. St.John’s is the only centre in India that has been offering this streamlined management for this disease since 1998 and we are a national referral centre for this group of diseases.

What started as a small research project under the captainship of Dr Usha Kini (Pathology) and Dr. Ashley D’Cruz (Paediatic Surgery) in 1997 has evolved into a multifaceted venture in diagnosis, teaching and research over the last 15 years. The ‘Hirschsprung Team’ at St. John’s exemplifies a robust cooperative ongoing effort from the departments Pathology and Paediatric Surgery at the academy. Besides many accolades that have come our way, several funded research projects(ICMR, VGST – Government of Karnataka,DBT Wellcome trust – Govt. of India) and indexed publications have supported and encouraged the team. Indeed the best rewards have been the smiles on our little patients and their families!

The Hirschsprung’s team @St.John’s

The Hirschsprung’s team @St.John’s

For more details, contact :

Dr Usha Kini
Email: drushakini@gmail.com
Mob : 09448270384
Professor of Pathology
St. John’s Medical College
Bangalore 560034.

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