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Mietens syndrome (mietens-weber syndrome)


Mietens Syndrome (Mietens-Weber Syndrome)

General: Etiology unknown; unclassifiable familial condition. 616d39g

Ocular: Bilateral corneal opacities; horizontal and rotational nystagmus; strabismus; bushy eyebrows; ptosis.

Clinical: Growth failure; flexion contracture of the elbows; dislocation of the head of the radii; mental retardation; small pointed nose with a depressed root; low hairline; external ear defects; digital defects; hypertrichosis.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981:548.

Mietens C, Weber H. A syndrome characterized by corneal opacity, nystagmus, flexion contracture of the elbows, growth failure and mental retardation. J Pediatr 1966; 69:624.

Waring GO, Rodrigues MM. Ultrastructural and successful keratoplasty of sclerocornea in Mietens' syndrome. Am J Ophthalmol 1980; 90:469-475.




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