Skeletal Maturation

10.1055/b-0034-87946

**Table 5.93 Skeletal maturation: accelerated maturation**
Diagnosis	Comments
Endocrine	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.
Idiopathic
Beckwith-Wiedemann syndrome	Exomphalos, macroglossia, and gigantism in the neonate. Increased risk of developing adrenal carcinoma, nephroblastoma, hepatoblastoma, and rhabdomyosarcoma.
Sotos syndrome	Acromegalic features. Mental retardation. Although growth may be rapid in first years and bone age advanced, final height may not be excessive.
Marshall-Smith syndrome	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 )

**Table 5.94 Skeletal maturation: delayed maturation**
Diagnosis	Findings	Comments
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.
Chronic illness
Severe malnutrition
Chromosomal abnormalities
Skeletal dysplasias with epiphyseal involvement	(see Table 5.33 )	Trisomy 18 and 21, Turner syndrome.

**Table 5.95 Skeletal maturation: classification of asymmetric maturation (hemihypertrophy)**
Diagnosis	Comments
Congenital, total	Involvement of all organ systems.
Congenital, limited	Only muscular, vascular, skeletal, or neurologic involvement.
Acquired	Localized hyperemia.
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
Macrodystrophia lipomatosa 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