Puberty in children with CP and other chronic conditions

Children with cerebral palsy and other chronic conditions should be expected to undergo pubertal development at the same or at a slightly earlier age than typically developing children, although data suggest that it may take children with CP slightly longer to reach full pubertal development. Thus, evaluation for early or late development of secondary sexual characteristics should be initiated as in typically developing children.

Early Pubertal Development: (Before age 8 in girls and 9 in boys tempered by family history of maturation patterns). If present, consider an evaluation by endocrinology (see below).

Premature Adrenarche: Anecdotal experience suggests precocious adrenarche is common in children with CP (e.g., the development of sparse pubic hair sometimes with body odor). This does not require evaluation or treatment if it is unaccompanied by other signs of virilization or pubertal development. If accompanied by other signs of development (e.g., growth spurt, axillary hair, signs of estrogen effects in girls, or clitoral/phallic enlargement), then evaluation would be appropriate.

Late Pubertal Development: (No sign by age 14, tempered by family history of maturation patterns.) Delayed pubertal development is often assumed of little importance because of the child's severe impairments. However, the lack of pubertal development may be a sign of underlying pathology that needs treatment (e.g., malnutrition, thyroid dysfunction, pituitary adenoma). In cases of hypogonadism, intervention will depend on the particular situation. For example, given the bone density impacts of estrogen and testosterone deficiency, intervention may be critical for the young adult who has had pathologic fractures. Initial evaluation for the child with delayed puberty should include:

• family history of age of maturation;
• medical history of CNS or gonadal insults, including trauma, infection, irradiation, or chemotherapy;
• physical exam to document current status and identify any syndromes that cause delayed puberty such as: Klinefelter, Prader-Will, Lawrence-Moon-Biedl, Noonan, Turner, and Kallman;
• a nutritional evaluation focusing on energy stored as fat (measurements of skin folds); and
• an initial laboratory evaluation might include:
  • a bone-age x-ray (left hand and wrist);
  • TSH, FSH, LH;
  • testosterone levels in boys;
  • estradiol level in girls; and
  • karyotyping, or directed genetic testing if a specific genetic syndrome is suspected as the cause for the delay.