Roentgen Differentiation of the Oculodentodigital Syndrome and the Hallermann-Streiff Syndrome in Infancy

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Abstract
The role of roentgen analysis as a complement to the clinical diagnosis of syndromes affecting chiefly the head and neck is emphasized. Two cases, both in infants, are reported, one with the oculodentodigital syndrome and the other with the Hallermann-Streiff syndrome. The roentgen features of each syndrome are listed and are sufficiently characteristic to permit differentiation in spite of the clinical similarities. The roentgen findings in the oculodentodigital syndrome are broad mandible, small orbits, broad tubular bones, hypoplasia of one or more middle phalanges of the fingers, and absence of one or more middle phalanges of the toes. The Hallermann-Streiff syndrome is characterized by hypoplasia of the rami of the mandible with anterior displacement of the temporoman-dibular joints, natal teeth, this bones of the calvaria with poor marginal ossification, and slender tubular bones. The etiology of both syndromes remains obscure, although evidence points to a dominant gene mutation for the Hallermann-Streiff syndrome, and an autosomal recessive inheritance for the oculodentodigital syndrome as the only plausible genetic hypotheses. Chromosomal anomalies have effectively been ruled out in both conditions. Teratogens and other environmental factors may play a role.

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