Times of India
16 June 2010
By Risha Chitlangia
New Delhi, India

Child’s Intenstine, Liver Were Outside Body
She is one of the few babies in the world to have survived a rare syndrome –Pentalogy of Cantrell –which comprises five congenital defects mainly involving the heart and abdominal cavity. Three–month–old Chahun was diagnosed with this condition at birth. Her intestines and liver were outside the body in a sac and her heart, which had two holes, had slipped from its original location. In a rare surgery, doctors at Fortis Escorts Heart Institute (FEHI) put back her organs in the right place.

The prevalence of this condition is 5.5–7.9 cases per million births. Doctors say a majority of such children are born still or die soon after. Chahun not only survived without any proper medical care for three months, but also responded well to the surgery. “Soon after birth she developed breathing difficulty and had to be given oxygen. Doctors in Arunachal Pradesh referred her case to Guwahati Medical College. We were then told that her heart was in the abdomen,” said Chuhan’s father H Khimhun who is a school teacher in Arunachal Pradesh.

Apart from the five defects that had to be fixed, the biggest challenge before doctors was to place the organs back into her body. In a two–stage surgery, doctors first separated the heart from the abdominal organs by reconstructing the diaphragm using a plastic material. “As her heart was in the abdominal cavity, we had to fix the heart and its two holes to stabilize her condition. There was no space for the heart in the chest cavity and we couldn’t push it as it would have exerted pressure on the lung. So we had to carefully create space,” said Dr K S Iyer, director, paediatric cardiothoracic department, FEHI.

Doctors decided on a 48–hour break before the second procedure, as the heart developed swelling. “When we physically handle the heart, it often develops swelling. We gave a break between the two procedures, but didn’t close the chest after the first,” said Dr Iyer.

The liver and intestines were then put back into the abdominal cavity. This was the biggest challenge as there was no space for the organs in the abdomen. “These organs have grown outside her body. So there was no space inside the abdomen. We stretched her stomach and gradually placed the organs. The abdominal wall was reconstructed using a nylon mesh,” said Dr Meera Luthra, consultant, paediatric surgeon, FEHI.

To close the exposed area, doctors used a nylon mesh (marlex mesh) to hold the sternum and cover the abdomen. “We brought the ribcage slightly closer and attached it to this mesh. She had a cleft sternum so we couldn’t completely get the ribcage together,” said Dr Iyer.

Chahun is now on her way back home. Her parents are happy that their baby survived the rare operation.

Rare Surgery
Diagnosed with Pentalogy of Cantrell, three–month–old Chahun suffered congenital defects of heart, diaphragm, abdominal wall, pericardium and lower sternum
Prevalence | 5.5–7.9 per million births
Abnormalities

Diaphragm not formed at the centre, heart rested on the liver

Ectopia cordis: Heart was in abdomen

Two holes in heart

Cleft sternum: Only a small portion of the sternum formed

Omphalocele: Intestines and liver were outside the abdomen in a sac

Surgical Procedure
First Stage

Artificial diaphragm created using a plastic membrane to separate the heart from abdominal organs

Heart was positioned in its natural cavity after fixing the holes

Part of the sternum was recreated using a marlex (nylon) mesh

Second Stage
Performed 48 hours after first, intestines and liver were put back in the abdominal cavity
Challenges

Doctors had to ensure the heart did not exert pressure on lungs

Baby's blood pressure had to be maintained

Abdominal organs had to be put back in the newly–created cavity

Cost of Surgery
Rs 4 lakh
Hospital Stay
45 days