Youngest patient receives minimally invasive treatment for sunken-chest disorder
Youngest patient receives minimally invasive treatment for sunken-chest disorder
March 14, 2006
On Thursday, March 9, 2006, 17-month-old Coreon Kelly of Joliet became the youngest and, at 19 pounds, the smallest patient ever treated with the minimally invasive "Nuss" procedure to repair a common congenital chest-wall deformity called pectus excavatum. The next youngest patient on record--to the best of our knowledge--was 5 years old.
Most children with this disorder, also known as "sunken" or "funnel chest," never need surgery. Because the disorder often becomes more pronounced at puberty, and teen-aged children can be acutely sensitive about their appearance, it is commonly corrected between the ages of 8 and 12. At that age the child is mature enough to understand the operation and post-operative instructions, yet the chest wall is still malleable.
But for children with severe pectus, such as Coreon--who looked, according his mother, Tara Nobles, "like somebody stuck a fist in his chest"--the decreased chest volume can impair breathing and decrease heart function.
He needed surgical repair "as soon as possible," said genetic specialist Darrel Waggoner, MD, assistant professor of human genetics at the University of Chicago, who helped care for the child.
Coreon had a profoundly sunken chest. This indentation narrowed his chest cavity, reduced the capacity of his lungs, especially on the left side, and pushed his heart to the left, which further limited the volume of his left lung. He suffered from increasing shortness of breath, frequent respiratory infections--which delayed his surgery for a few months--and was falling behind on his developmental milestones.
"He was slow to crawl," his mother said, "because he couldn't lie on his stomach, and that made him slow to walk."
Coreon also suffers from Marfan syndrome, a genetic disorder that causes abnormalities of the heart and aorta and is often associated with an abnormal chest wall. At this young age, he was already suffering from an enlarged heart and high blood pressure requiring daily doses of adult anti-hypertensives to control. All these factors made surgical correction more urgent.
"His complex genetic syndrome and pulmonary insufficiency made a pectus repair imperative," said pediatric surgeon Donald Liu, MD, chief of surgery at Comer Children's Hospital at the University of Chicago. "But at such a tender age, the risks of major surgery, compounded by severe cardiac disease caused by his genetic syndrome, are traditionally prohibitive."
Conventional surgery for pectus excavatum includes cutting through the breastbone and several ribs on each side and repositioning them. Patients experience considerable discomfort and are left with a large chest scar. Recovery can take weeks and post-operative complications are common.
Since 2002, however, Liu has used a newer approach known as the Nuss procedure. Named for its inventor, Donald Nuss, a Norfolk, Virginia, pediatric surgeon who developed the technique in 1987, this minimally invasive surgery requires only a one-inch incision made on each side of the chest.
In the Nuss procedure, a tiny camera and a curved surgical steel bar are threaded underneath the rib cage. The gently curved bar is then rotated 180 degrees to move the chest wall into the normal position.
The procedure takes less than an hour. Patients are released from the hospital within a few days with only small scars on their chest. Within three months patients return to normal activities, even playing sports.
"The beauty of having the technology and expertise in the Nuss procedure is that it allowed us to perform major reconstructive surgery safely, effectively, and quickly with minimal blood loss in an already compromised child," said Liu.
For Coreon, the bar will be removed in about two years, after the normal curve of the chest has been established and the underlying bone structure solidified.
Only five days after surgery young Coreon already has a more normal appearance and is breathing easier. He could be dischaged from the hospital as soon as Tuesday, March 14.
"Many physicians think of pectus excavatum as a cosmetic disorder," said Liu, "but in rare cases like this it can be a significant defect, even life threatening."
Coreon will still have Marfan's, which can cause other heart complications, learning problems, and connective tissue disease, but "thanks to this novel surgical intervention," Liu said, "he should soon be living a more normal life."