Six-year-old Amaira (name changed) can now decide what she wants to eat. She is learning to understand flavours, and for the first time in years she will be joining school. You would think that she is a slow learner, but Amaira is fast catching up on life considering that she was unable to swallow food for three years. Her food pipe to the stomach was blocked and life was all about continuous vomiting, losing weight and lying listless and sick. A novel surgery, however, has changed the course of her life and she is able to reclaim every bit of normalcy in her life, not to mention the hope that it has rekindled in many other children with disorders like that of hers.
Prof (Dr) Anil Arora, chairman and head of the department of gastroenterology at Sir Ganga Ram Hospital in Delhi, who did the corrective and restorative surgery on her, said, “When Amaira came to us, she was very thin, emaciated and marasmic (protein malnutrition), and weighed 8-10 kg lower than her peers. We diagnosed her to be suffering from Achalasia Cardia (swallowing disorder) after evaluating her with endoscopy, barium swallow followed by high-resolution oesophageal manometry. In simple terms, she had repeated vomiting and regurgitation of food from the mouth and nose which resulted in marked weight loss.”
“This condition happens in one in a lakh patients. A valve connects our food pipe or oesophagus to the stomach, which flips open like sensors whenever the body temperature rises once food is ingested. You can say that the valve, in her case, lost all sensory capacity and remained shut, forcing the food out of her system,” Prof Arora explained.
Until recently, the established treatment for Achalasia Cardia in children was surgical intervention, but in Amaira’s case, doctors decided to perform a relatively new endoscopic procedure called POEM (Per Oral Endoscopic Myotomy).
POEM (Per Oral Endoscopic Myotomy) is an endoscopic procedure used to treat obstruction at the lower end of the food pipe due to failure of relaxation of the lower esophageal sphincter (musculature opening) called Achalasia Cardia. POEM is a relatively new advanced endoscopic procedure which can be performed in the endoscopic suite itself without any incision on chest or abdomen, with a minimal post-procedure hospital stay.
“This procedure is proven in adults, but had never been tried on children. The challenge for us was to do this on a six-year-old child – one of the youngest in India to the best of our knowledge – weighing just 11 kg, lowest weight to the best of our knowledge in India. There was a substantial risk of infection, aspiration and respiratory problems. Also, there were limitations of using adult endoscopic equipment and accessories used in POEM in a child. But we took a calculated risk and extreme precautions,” he said.
According to Dr Shivam Khare, consultant, department of gastroenterology, “The POEM procedure consists of four steps: mucosal entry (cutting the internal lining of the food pipe), creation of submucosal tunnel (tunnel between the muscle layers and the inside lining of the food pipe), initiation and extension of myotomy (cutting the tight muscle at the stomach and food pipe junction) and closure of mucosal entry (closing the internal lining with hemoclips after exiting the tunnel).”
Achalasia Cardia is seen rarely in the paediatric population, with less than five per cent of all cases presenting below 15 years of age globally. “The procedure took one and a half hours and the child’s obstruction and blockage at the junction of the food pipe and stomach was immediately relieved. She was able to consume a normal diet within four days and was discharged. And within two to three months, I see her doing all the things befitting her age.”
According to Dr Khare, “We believe that with increasing expertise and availability of multispecialty teams, this procedure can be safely done for long-lasting relief from dysphagia (difficulty in swallowing) and regurgitation (vomiting) of food so that these hapless children can be properly treated and achieve the full potential of physical and mental growth, especially in the formative years of early childhood and adolescence.”
Elaborating further, Prof Arora said, “With state-of-the-art advanced diagnostic procedures like high-resolution oesophageal manometry and high-definition endoscopes, disorders like Achalasia Cardia can be easily diagnosed. We have already successfully performed this procedure in 427 cases till date in our hospital… It is a no-scar, precision surgery, where patient recovery and mobilisation are faster.”
Prof Arora also pointed out how diagnosis was difficult given the symptoms of aggressive nausea and how the condition was easily confused with Gastroesophageal Reflux Disease (GERD) which affects every fifth Indian. “That’s why people report for surgical intervention rather late,” he said.