November 10, 2011
Hollow or sunken chest, the most common congenital deformity of the chest wall affecting one in 300 to one in 400 children, is rarely life-threatening and virtually all children can have successful surgical repairs. But the condition is far from purely cosmetic and even the mildest cases require prompt evaluation upon diagnosis, say experts at the Johns Hopkins Children’s Center.
“Surgery for pectus excavatum is rarely done solely for cosmetic reasons. The main reason we do surgery is to improve heart and lung function, not looks, and any cosmetic benefits are secondary,” says Hopkins Children’s pediatric surgeon Fizan Abdullah, M.D. Ph.D.
Abdullah and his colleagues advise early check-ups that would achieve three goals: Rule out serious underlying syndromes, Assess cardio-pulmonary function and Plan surgery or RAP.
- Rule out more serious disorders. In a small subset of children, sunken chest can herald an underlying syndrome. For example, sunken chest is commonly found among children with Marfan syndrome, a genetic disorder of the connective tissues whose most feared life-threatening complications include growth of arterial aneurysms or stretching and rupture of the heart’s aorta. Experts recommend that pediatricians evaluate all children with sunken chest for other tell-tale Marfan signs, including long slender fingers, arm span exceeding height, long lean skull with downward slanted eyes and a spinal curvature (scoliosis), among others. Any child with three or more of these features should be referred to a Marfan expert, especially if the child has a family history of Marfan or unexplained heart problems.
- Assess heart and lung function. The sunken chest, especially in more severe cases, can compress the heart and lungs and affect breathing and circulation. Although serious lung and heart problems are rare, even children with milder cases often have reduced cardiovascular endurance, tire quickly, describe a feeling of something sitting on their chest and complain of neck and back pain.
- Plan the optimal time for surgery. Surgery can alleviate pulmonary and cardiac problems, reduce back and neck pain, improve posture and restore normal appearance to the chest. Minimally invasive alternatives to open-chest surgery have been developed in recent years. Depending on the patient’s age, heart/lung involvement and severity of the malformation, surgeons can choose from several approaches.
The ideal window for surgery is between ages 14 and 16, Abdullah says. If surgery is done too early, the sunken chest may re-emerge because the bones are still growing and taking their final shape. However, Abdullah says, early surgery is warranted in severe cases where lung and heart function are seriously compromised. Surgeries in older children and adults tend to be more difficult and more invasive because once the bones start to calcify they may require an open-chest surgery.
“This golden window is not a hard-and-fast rule,” Abdullah says, “but a preferred timeframe, and we can certainly do the surgery in older patients and — more rarely — in younger children with very severe symptoms.”