Barraquer-Simons syndrome, a disorder of unknown etiology, is characterized by a cephalothoracic lipodystrophy. We present 2 patients treated with a bilateral free transverse rectus abdominis myocutaneous (TRAM) flap to restore facial contour. Our technique of using a muscle component to fill the cheek defect was based on our experience with free muscle transfer in facial reanimation. In comparison with adipose tissue, muscle tissue does not show a tendency for ptosis because of its consistency and firm attachment of the muscle surface to the surrounding tissues. These cases demonstrate the possibility for the use of simultaneous dissection of the face and flaps, and the reliability of the vascular pedicle. The stability of the abdominal wall was secured by closure of the rectus sheath over Teflon mesh, which has been proven to prevent hernia. Our long-term follow-up demonstrates a stable symmetrical facial appearance.
A 9-year-old girl presented with an abnormal muscle of the upper lip, a congenital philtral deformity associated with a short columella and a bifid nose. The deformity was most clearly seen with facial expression. Congenital nasal anomalies may occur alone or in combination with clefts of the lips and face. In this case, there was no palpable facial cleft. The contribution of the medial nasal processes to the formation of the nasal septum, columella, and philtrum and the proposed embryologic errors have been well described. A threedimensional model of the philtrum and a transverse histological section from a third trimester fetus is illustrated. The smooth muscle tissues are formed by the embryonic connective tissue. A minor malformation of unilateral skin and soft tissue between columella and philtrum may be a focal fetal dysplasia and can be considered a type of facial cleft. The patient can be classified in a morphogenetic group with a slight focal fetal dysplasia.
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