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Hemimacrosomia syndrome (steiner syndrome; hemigigantism; hemifacial or unilateral hypertrophy)


Hemimacrosomia Syndrome (Steiner Syndrome; Hemigigantism; Hemifacial or Unilateral
Hypertrophy) 616e48g 616e48g 616e48g 616e48g 616e48g 616e48g 616e48g

General: Occasionally hereditary, although true etiology is obscure; right side affected more frequently than left side; slight male preponderance.

Ocular: Dilated pupil on the affected side; eccentric pupillary location; hypochromic heterochromia.

Clinical: Unilateral facial enlargement may be associated with enlargement of half of entire body to varied extent and degree; thickened skin over involved area with increased activity of sebaceous and sweat glands; telangiectasias and multiple nevi; polydactyly; syndactyly; macrodactyly; scoliosis.

Curtius F. Kongenitaler Partieller Riesenwuchs mit Endokrinen Storungen. Dtsch Arch Klin Med 1925; 147:310.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Stafne EC, Lovestedt SA. Congenital hemihypertrophy of the face (facial gigantism). Oral Surg 1962; 15:184.




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