Progressive hemifacial atrophy is a rare disorder, affecting almost 0.30 percent of the population, characterized by progressive atrophy of some or all tissues on one side of the face, occasionally extending to other parts of the body. First described by Parry in 1825, the disorder is better known as Romberg’s syndrome or Parry- Romberg syndrome.1 The disease is unilateral in 95 percent of cases and can be located on both sides of the body. The process may be bilateral in 5 to 10 percent of cases. Tissues involved include skin, tongue, gingiva, soft palate, cartilage of the nose, ear, subcutaneous fat, larynx, muscle, and bone. Either sex may be affected. It is believed to be related to scleroderma,2 although is not congenital and the cause is not really known.3 Onset occurs most often before puberty or during adolescence but occasionally begins in middle or later years.1 Several reconstructive methods using autologous4–7 or synthetic materials8 have been proposed to restore the symmetry of the face, the treatment of choice to date remaining surgery. Soft-tissue reconstruction is mostly performed using free, muscle, or fasciocutaneous flaps.9 We report a case of a young, diabetic patient with a thin body habitus affected by Parry-Romberg syndrome in which the deepithelialized supraclavicular island fascial flap was used to obtain a symmetric contour of the involved side. The flap based on the supraclavicular artery is harvested not as a pure island flap but with a fascial pedicle, thin and resistant, which ensures a good reliability. Also, when a higher tension rate is present, it avoids risk of excessive traction or kinking of the vessels.10 Features and results of the method used are reported in the article.
Supraclavicular island fascial flap in the treatment of progressive hemifacial atrophy / DI BENEDETTO, GIOVANNI MARIA; Aquinati, A; Balercia, P; Forlini, W; Bertani, A.. - In: PLASTIC AND RECONSTRUCTIVE SURGERY. - ISSN 0032-1052. - 121(5):(2008), pp. 247-250.
Supraclavicular island fascial flap in the treatment of progressive hemifacial atrophy.
DI BENEDETTO, GIOVANNI MARIA;
2008-01-01
Abstract
Progressive hemifacial atrophy is a rare disorder, affecting almost 0.30 percent of the population, characterized by progressive atrophy of some or all tissues on one side of the face, occasionally extending to other parts of the body. First described by Parry in 1825, the disorder is better known as Romberg’s syndrome or Parry- Romberg syndrome.1 The disease is unilateral in 95 percent of cases and can be located on both sides of the body. The process may be bilateral in 5 to 10 percent of cases. Tissues involved include skin, tongue, gingiva, soft palate, cartilage of the nose, ear, subcutaneous fat, larynx, muscle, and bone. Either sex may be affected. It is believed to be related to scleroderma,2 although is not congenital and the cause is not really known.3 Onset occurs most often before puberty or during adolescence but occasionally begins in middle or later years.1 Several reconstructive methods using autologous4–7 or synthetic materials8 have been proposed to restore the symmetry of the face, the treatment of choice to date remaining surgery. Soft-tissue reconstruction is mostly performed using free, muscle, or fasciocutaneous flaps.9 We report a case of a young, diabetic patient with a thin body habitus affected by Parry-Romberg syndrome in which the deepithelialized supraclavicular island fascial flap was used to obtain a symmetric contour of the involved side. The flap based on the supraclavicular artery is harvested not as a pure island flap but with a fascial pedicle, thin and resistant, which ensures a good reliability. Also, when a higher tension rate is present, it avoids risk of excessive traction or kinking of the vessels.10 Features and results of the method used are reported in the article.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.