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In a rare surgery, a four–year–old Nigerian boy was cured of Shone's Complex — a congenital heart disease, with experts claiming that there have been only 20 such cases reported across the world since 1993.

Wilson Falohun has been suffering from Shone's Complex since birth. However, he started showing evident symptoms only this year. "He started coughing, vomiting and lost appetite since March. He has always been fragile, but when he started losing weight quickly, we consulted several doctors in Nigeria. The diagnosis was wrong there. In August, we decided to seek medical advice in India," said Agha B, Falohun's uncle.

Fulohan had a hole in his heart and was supposed to undergo an open–heart surgery. However, doctors later realised that this case was different. "Wilson's weight was 11.5 kg, which is 7 kg less than that of a normal child his age. He also had a mitral valve defect. We knew we were dealing with a very rare case," said Dr Fazal Nabi, consultant pediatrician at Jaslok hospital.

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Shone's Complex is a rare heart disease consisting of four defects in the heart which leads to obstruction in blood flow to and from the heart. "This disease first causes supravalvular mitral membrane defect which triggers other defects in the heart. As a result, the blood vessels get constricted," said Nabi. The symptoms comprise breathlessness, perspiration, cough and chest infection.

According to him, Fulohun's surgery, which continued for five hours on September 9, was a complicated one.

"Since 1993, only 20 cases have been reported across the world," Nabi said.

According to Dr Suresh Joshi, director of cardiovascular and thoracic surgery in the hospital, early detection can help save the patient. "This disease can be detected during pregnancy through sonography and can be cured immediately after birth. However, delay in detecting it can prove to be fatal. It is surprising that the boy survived this long," Joshi said. He added that this is probably the first such surgery of its kind in the country.

Post–surgery, Fulohun was under observation in the ICU for three days. He has been in India for more than a month now. "Now that his surgery is complete and there are no more complications, we can leave for Nigeria. His parents are very anxious," Agha said.


Source
Indian Express
24 Sep 2013

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