Comparison with an atlas of normal maturation will show the acceleration. Prolonged elevation in sex steroids. DD: precocious puberty and congenital adrenal hyperplasia, premature adrenarche, obesity, hyperthyroidism, lipodystrophy, gonadotropin-producing tumors.
Exomphalos, macroglossia, and gigantism in the neonate. Increased risk of developing adrenal carcinoma, nephroblastoma, hepatoblastoma, and rhabdomyosarcoma.
Acromegalic features. Mental retardation. Although growth may be rapid in first years and bone age advanced, final height may not be excessive.
Failure to thrive, mental retardation, blue sclera, and unusual facies with large forehead, shallow orbits, and depressed nasal bridge.
Polyostotic fibrous dysplasia (McCune-Albright syndrome)
(see Table 5.31 )
Abnormal endocrine function
Comparison with an atlas of normal maturation will show the delay.
Global delay in maturation. DD: growth hormone deficiency, hypothyroidism, Addison disease, Cushing disease, craniopharyngioma, psychosocial dwarfism.
Skeletal dysplasias with epiphyseal involvement
(see Table 5.33 )
Trisomy 18 and 21, Turner syndrome.
Involvement of all organ systems.
Only muscular, vascular, skeletal, or neurologic involvement.
Associated with a syndrome
Neurofibromatosis and Beckwith-Wiedemann (approximately 13% of patients), Klippel-Trénaunay-Weber, Proteus, McCune-Albright, and many other syndromes.
Tumors associated with hemihypertrophy
DD: Wilms tumor, adrenal carcinoma, rhabdomyosarcoma, and hepatoblastoma.62
Fig. 5.44, p. 525
Fig. 5.45, p. 525
Progressive overgrowth of all the mesenchymal elements with a disproportionate increase in the fibroadi-pose tissues.63