J. Kotilainen et al., CRANIOFACIAL AND DENTAL CHARACTERISTICS OF SILVER-RUSSELL SYNDROME, American journal of medical genetics, 56(2), 1995, pp. 229-236
We found significant differences in a craniometric, cephalometric, and
dental study of 19 Silver-Russell syndrome patients (13 without growt
h hormone treatment) with appropriate controls, Although head circumfe
rence was normal for age, head length was increased, while cranial and
facial widths and facial heights were reduced, Posterior facial heigh
t, posterior cranial base length, cranial base height, and mandibular
body size were significantly smaller than in healthy children of the s
ame height, Articulatory speech disorders were common, Enamel defects
pointed to an early prenatal insult, Delayed dental age and small mand
ibular and cranial base dimensions support the possibility of physiolo
gical growth hormone deficiency in many Silver-Russell syndrome childr
en; however, facial soft tissue structures were strikingly different f
rom those observed in classical growth hormone deficiency. (C) 1995 Wi
ley-Liss, Inc.