A NEW SECKEL-LIKE SYNDROME OF PRIMORDIAL DWARFISM

Seckel syndrome is a rare, recessively inherited disorder of severe gr owth retardation and distinct craniofacial, orodental, and skeletal an omalies, Even though there are well-established minimum diagnostic cri teria for this syndrome, controversy exists about its boundaries and c riteria for exclusion, We studied 2 remarkably similar, unrelated chil dren with most of the clinical and radiographic manifestations of Seck el's original patient, Although their craniofacial and orodental anoma lies are typical of Seckel syndrome, 1 child has unusual appearance of the hands and feet that have not been previously associated with it, This patient appears to define a new Seckel-like syndrome and suggests heterogeneity in this type of primordial dwarfism. (C) 1996 Wiley-Lis s, Inc.