Current concepts in tall stature and overgrowth syndromes

In this overview an update is given on the pathogenesis, classification and differential diagnosis of overgrowth syndromes. In addition, height progno sis and therapeutic modalities available for managing mainly constitutional tall stature are discussed. Constitutional tall stature comprises normal v ariants in which one or both parents are tall. Primary disorders may have a prenatal onset and may be of chromosomal or genetic origin. Secondary over growth syndromes are most often the result of hormonal disturbances. Height prediction plays a key role in the management of tall children. Prediction equation models have been developed based on the growth data of healthy ta ll children. There is general agreement that a favourable effect on reducin g ultimate height is obtained using high doses of sex steroids (girls 100-3 00 mug ethinyl-oestradiol; boys testosterone (T) ester depot preparations 2 50-1000 mg/month), the height reduction being greater when the treatment is started at a lower chronological and/or bone age. An alternative is the in duction of puberty with low doses of sex steroids (girls 5-50 mug ethinyloe stradiol; boys T esters 25-50 mg/m(2)/3 wk). In addition orthopaedic proced ures have been suggested, but there is limited experience. Although psychos ocial factors constitute the main reason for treating tall stature, extensi ve psychological investigations before or during height limiting therapy ar e lacking. Moreover, there are no objective data indicating lifelong psycho social damage resulting from being tall.