Congenital

The Department of Plastic and Reconstructive Surgery evaluates and treats individuals with a diverse array of congenital deformities. Areas of specialty include (also see craniofacial section):

Hand Deformities: Next to life itself, we value our limbs. Of paramount importance to function in our society are our hands. Some anthropologists suggest that our hands are equal to the human brain for the survival of our species. It is not surprising therefore that when a child is born with a congenital hand deformity, it creates considerable stress for the parents.

Congenital hand deformities are not rare. Some deformities occur with a frequency of 1 in every 1-2,000 live births (see Syndromic Craniosyntosis). Many abnormalities are benign and require little or no treatment. For example, ulnar polydactyly, a condition where there is an extra digit (usually vestigal) attached to the little finger, may be treated with a simple suture tied at the base of the digit. Clinodactyly (a condition of angulation of the digit) often requires no treatment at all. Other conditions, such as syndactyly (webbed fingers) or congenital absence of the thumb, require surgery to optimize finger function.

Syndactyly is one of the most common congenital deformities. It is the result of the fingers and/or joints being fused together. Syndactyly is corrected through a series of zigzag incisions made through the webbing and between the fingers. By using a zigzag cut, there is a smaller chance of recurrent webbing should the scar contract. The flaps of skin are tucked between the fingers and joined toward the palm. A skin graft may also be necessary if there is not enough skin to line the entire new opening between the fingers.

In the Department of Plastic and Reconstructive Surgery, consultation for a hand deformity addresses the concerns of the parents, as well as the needs of the child. Parental education of what can be expected should begin soon after the child is born. This education helps to relieve anxiety and ensure appropriate timing of surgical or non-surgical interventions. There is usually something that can be done to help the child have a hand that allows them to live a happy and productive life. In some cases, this may require complex surgery such as pollicization of a digit (creating a thumb from another digit), and in others, less complex procedures. While parents may wish immediate correction of a congenital problem of the hand, this is often not wise. The child must be of adequate age to tolerate surgery safely and of adequate size to make the surgery technically feasible. However, to avoid learned patterns of disuse, it is recommended that most congenital problems of the hand be corrected by age three.

Nevi are congenital birthmarks or moles found on the skin. Nevi vary greatly in size, shape, surface texture, and whether or not they have hair. The color can vary from light tan to nearly black. Some have fine downy hair, while many others have long, thick, darker hair.

Treatment to remove congenital moles is often performed by simply excising the nevi. If the nevus is small enough in size, the excision can be done in one procedure with the edges sutured together. However, for larger nevi, serial excision can be done by excising the lesion in stages to remove a portion at time until the entire nevus is gone. In areas of high sun exposure and risk for cancer, the nevi can be removed and a skin graft can be utilized to cover the site. In areas where cosmesis is a concern, tissue expansion can be used to increase the amount of local tissue available to cover the deficit. Tissue expansion is done by inserting a balloon under the skin near the site. Salt-water solution is inserted into the balloon until the expansion has yielded enough skin to cover the area that will be left once the nevus is excised.

Pectus Excavatum and Pectus Carinatum are congenital anomalies of the anterior chest wall. The excavatum defect is characterized by a deep depression of the sternum, usually involving the lower half or two thirds of the sternum, with the most recessed or deepest area at the junction of the chest and the abdomen. The entire defect pushes into the underlying midline structures so that the lungs are compressed from side to side and the heart is compressed. Pectus carinatum is characterized by the overgrowth of cartilage and forward buckling onto the sternum. The carinatum deformity produces a very rigid chest so the chest is almost secured in a position near full inspiration. Respirations are inefficient and the individual needs to use the diaphragm and accessory muscles for respiration rather than normal chest muscles. Surgical correction of either of the anomalies can improve both respiratory function and appearance of the chest wall.

Poland Syndrome is a congenital disorder that is characterized by the absence or underdevelopment of certain chest muscles, and abnormally short, webbed fingers. Additionally, there may be an underdevelopment or absence of one nipple and/or patchy hair growth under the arm. In females, one breast may also be underdeveloped or absent. In some cases, affected individuals may also exhibit underdeveloped upper ribs and/or an abnormally short arm with underdeveloped forearm bones on the affected side. In most cases, physical abnormalities associated with this syndrome are confined to one side of the body. In approximately 75 percent of the cases, the right side of the body is affected.

Most patients with Poland syndrome do not require surgery to correct muscle or chest wall deformaties. Breast reconstruction and augmentation should be considered after full development for women. In the adolescent, a tissue expander may be used to facilitate maintenance of symmetry to the other growing breast. Once growth is complete, an implant may be exchanged for the tissue expander. Latissimus dorsi muscle flaps have been used in the reconstruction of absent pectoralis major muscles. Surgery for syndactyly and other hand anomalies generally improves functional capacity and cosmetic appearance.

 

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Last Updated: 04-08-2014
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