Xinjiang boy with rare heart deformity undergoes surgery

Doctors in Shanghai successfully operated on a six-year-old boy from Xinjiang suffereing from a rare heart deformity.
Ti Gong

Six-year-old Abu was diagnosed with Pentalogy of Cantrell.

A 6-year-old Xinjiang boy whose heart was only covered by a layer of skin due to a rare heart deformity received a successful surgery in Shanghai on Wednesday after doctors created a "sternum."

The young patient, identified as Abu, is from Zepu County in the south of Xinjiang Uygur Autonomous Region.

Abu’s heart was only protected by a layer of skin on his chest rather than a sternum. Local doctors said he could live for no longer than two years.

Ti Gong

Abu’s heart was only protected by a layer of skin rather than a sternum.

But Abu managed to grow up to the age of six. With the help of a Shanghai-Xinjiang medical project, Abu was transferred to a hospital in Kashgar City at the beginning of last month, where he was treated by doctor Zhang Lei, from Shanghai Children’s Medical Center.

The medical center then organized a long-distance consultation, during which doctors from Zepu, Kashgar and Shanghai discussed Abu’s case and decided to transfer him to Shanghai for treatment.

Abu arrived in Shanghai on July 17, and was diagnosed with Pentalogy of Cantrell, a rare syndrome that affects fewer than five in 1 million. The deformity involved Abu’s heart, diaphragm, abdominal wall, pericardium and lower sternum.

Only 10 such cases had been reported in China. Shanghai Children’s Medical Center had received three such patients before. According to Professor Liu Jinfen of the medical center, the best age for treatment is before 2.

Liu said Abu was lucky that his heart managed all these years, but the risk of further damage was always there.

With the help of 3D technology, the hospital tailored artificial “sternum shells” with polyether ether ketone material for Abu to ensure his heart functions properly and is protected.

He faces further treatment.

Ti Gong

The artificial “sternum shells” tailored for Abu.

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