ESPE Abstracts (2014) 82 P-D-2-2-548

Puberty in Children with Shunted Congenital Hydrocephalus with and without Myelomeningocele

Lemm Proos, Kai Arnell, Jan Gustafsson & Margareta Dahl

Department of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden

Background: Children with myelomeningocele (MMC) run an increased risk of developing early or precocious puberty (E/PP). In previous studies of such children we found that the incidence of E/PP was 52% in girls and 21% in boys and that E/PP was strongly associated with increased intracranial pressure perinatally. It is also known that the occurrence of hydrocephalus without MMC is associated with risk of E/PP.

Objective and hypotheses: The aim was to study the influence of MMC and gender on timing of puberty in children with increased intracranial pressure perinatally.

Method: All children with congenital hydrocephalus, born between 1980 and 2002, treated with shunt and living in the county of Uppsala, were identified. The study cohort included 35 children (16 girls) with congenital hydrocephalus. Eighteen children (eight girls) had MMC whereas 17 children (eight girls) had not. Health records were examined retrospectively. E/PP was defined as pubertal signs appearing before 10:2 years for boys and 9:2 years for girls.

Results: (Table 1).

Table 1.
Without MMCWith MMCPWithout MMCWith MMCP
Age start puberty (years)8.4±2.08.3±1.4NS8.5±1.910.0±0.90.044
E/PP6/8 (75%)7/8 (88%)6/9 (67%)3/10 (30%)

Conclusion: All children with congenital shunted hydrocephalus are at high risk of developing E/PP. In the children without MMC the risk is comparable in the genders while in those with MMC it is most marked in girls. The mechanism behind the later onset of puberty in boys with MMC should be further investigated.

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