THE SILVER SYNDROME: CONGENITAL ASYMMETRY, SHORT STATURE AND VARIATIONS IN SEXUAL DEVELOPMENT

ROENTGEN FEATURES

¹ From the Departments of Radiology and Pediatrics, The Mt. Sinai Hospital, New York, New York, and the Department of Radiology, The Hospital for Special Surgery, New York, New York

1. Two cases of the Silver syndrome are reported. The major characteristics of this syndrome are shortness of stature, significant asymmetry, small size at birth despite being born at term and variations in the pattern of sexual development. Minor manifestations include short incurved 5th fingers, triangular shape to the face, turned-down corners of the mouth, cafe-au-lait areas of pigmentation of the skin and syndactylism.

2. The most consistent roentgen features are congenital hemihypertrophy and hypoplasia of the middle phalanx of the 5th fingers. One case is reported in which there was a Kirner's deformity of the 5th fingers. Other less frequent skeletal abnormalities which have been noted in some cases are mentioned.

3. The syndrome may be considered one category of low birth weight dwarfism with special congenital stigmata which set it apart from other varieties of intrauterine dwarfism.

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