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Volume 19, Issue 1, March 2003
Table of Contents 19-1
Editorial Comment: Sotos & Weaver Syndromes
 

Comment

Sotos syndrome and Weaver syndrome are both overgrowth syndromes beginning usually prenatally. Such overgrowth continues during childhood. These two syndromes are similar in many respects; in respect to overgrowth, mental retardation, large hands and feet, advanced bone age, and tall stature but, usually, adult height within the normal advanced percentiles. However, they do differ in certain subtle respects. The patient with Sotos syndrome (cerebral gigantism) has a head that is dolichocephalic.  The occiput tends to be flat in the patients with Weaver syndrome.  The face tends to be smaller.  There are hypoplastic facial bones and macrognathia in Weaver syndrome, but pointed chin and normal mandibular development prompts one to think more of Sotos syndrome. The joints are limited in motion often in Weaver syndrome with limited elbow, ankle, wrist, hip, and knee extension.  The long bones are widened or splayed in Weaver syndrome and camptodactyly is frequent.  Further details concerning these two syndromes can be pulled from the pediatric database, although the update listed is 1994 (http://www.icondata.com/health/pedbase/files/sotosynd.htm - or - weaversy.htm.  Comparable data can also be found on the web at http://www.nlm.nih.gov.  At this web site you will have a choice to enter “Weaver”.

Robert M. Blizzard, MD

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