Russell-Silver syndrome

Russell-Silver

Syndrome

By Andrew K. Poznanski and John F. Holt

synonyms:Silver-Russell Heredity: Chief Clinical Manifestations:

Chief Roentgen Manifestations:

Other

important

Details: Variants:

Differential

dwarf

Sporadic in most cases; dwarfism evident at birth (1) Hemihypertrophy (2) Small triangular face with downturned corners of mouth (1) Clinodactyly of fifth finger; occasionally Kirner deformity instead (2) Hemihypertrophy (3) Retarded and asymmetric maturation (1) CafC au lait spots (2) Precocious puberty Russell dwarf

Diagnosis:

Pituitary dwarfism, bird-headed dwarfism, other causes of hemihypertrophy

References:

1. Mosley JE, Moloshok RE, Freiberger RH: Am J Roentgen01 97:74, 1966 2. Rimoin DL: The clinical delineation of birth defects. II. Malformation syndromes. New York, National Foundation, 1969 p 183 3. Silver HK: Am J Dis Child lO7:495, 1964

202

Seminars

in Roentgenology,

Vol. VIII, No. 2 (April),

1973

MAJOR

SYNDROMES

203

A 1-yr-old boy with right hemihypertrophy. Fig. A. Clinodactyly of both fifth fingers. The maturation is more advanced on the right which is the larger side. Fig. B. Asymmetry in maturation is well seen. The right femoral epiphysis is larger than the left. Fig. C. Growth lines in the right tibia are farther from the end of the bone than in the left.