Tuesday, December 30, 2003

Dera Ghazi Khan Child Born with an exposed heart

Dera Ghazi Khan Child Born with an exposed heart

By Nadeem Saeed

MULTAN, Dec 29: Aleena, aged 3, is a lively little girl from Dera Ghazi Khan, who is as joyful as any child of her age can be. But, she is living with the rare congenital anomaly of cleft sternum that has left her little heart and great vessels exposed to external trauma.

Pulsation of the heart and the great vessels can easily be seen through the gap, which is covered only by a thin layer of skin.

Her father, Farooq Laskani told Dawn that initially they thought that the gap at the middle of the rib cage would close with the passage of time. "But our worries increased with her age as the bulge of the heart became so conspicuous that I and her mother simply couldn't bear the sight of her bare chest."

They decided to consult the specialists at the Nishter Medical College and Hospital. They got Aleena examined by a thoracic surgeon of the NMC&H at a private hospital reportedly run by an official of the same institute.

After a complete round of tests, ultrasound, X-rays and echocardiography, she was diagnosed with cleft sternum (or fissure sterni). But, they were advised to get opinion of some consultants in Lahore also.

The paediatric cardiologists and thoracic surgeons of the provincial capital also confirmed the absence of sternum and advised immediate repair of the cleft. The NMC&H thoracic surgeon however told her parents that he would only do the job at the private hospital.

"We had exhausted our resources in diagnosis of the problem, and her treatment at a private hospital was out of our reach," Farooq said, adding: "Moreover, we learned that the NMC&H surgeon had no previous experience of handling such a rare congenital anomaly."

Recently, a couple of children from Dera with some heart ailments went to India for treatment. Their parents told Aleena's family that her treatment in the neighbouring country including the travel expenses would cost much less than the expenses to be incurred on the operation at a private hospital of Multan by an official doctor. "We are now exploring this avenue," Farooq said.

Cleft sternum is a rare congenital defect of the anterior chest wall and is the result of midline fusion failure. Prenatal diagnosis by ultra-sonography is possible.

Surgical correction should be performed during the neonatal period when direct suturing of the sternal halves is possible. At an older age, surgical repair is feasible, but due to the increased rigidity of the chest wall and physiologic accommodation of the thoracic organs to the accustomed circumference of the chest, it may require additional measures, such as sliding chondrotomies of the adjacent costal cartilage and notching of the sternal bars to facilitate approximation.

A number of methods for repair of cleft sternum have been reported, including primary approximation, sliding or rotating chondrotomies, and use of prosthetic grafts or flaps of bone, cartilage, autogenous tissue or Pectoralis Major muscle.

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