Shunted Infantile Hydrocephalus Linked to Early Puberty in Girls

Girls who received a ventriculoperitoneal shunt for infantile hydrocephalus had an increased risk of early puberty, particularly those with myelomeningocele and repeated shunt revisions, a population-based cohort study showed.

Among 82 girls with infantile hydrocephalus, 21% had precocious puberty before age 8 years and another 29% had early puberty with signs appearing before 8 years and 9 months, found researchers led by Margareta Dahl, MD, PhD, of Uppsala University in Sweden.

Early or precocious puberty was more common among girls who had undergone three or more shunt revisions (33% vs 8% among those with fewer revisions, P=0.01), the researchers reported in Acta Paediatrica.

The number of shunt revisions was negatively correlated with age at the start of puberty in the 39 girls with myelomeningocele (Spearman’s correlation coefficient = -0.512, P=0.001); whereas no risk factors were associated with precocious or early puberty in the children without myelomeningocele.

“Since patients with hydrocephalus are already afflicted by their condition, early pubertal maturation may present an additional burden,” both in practical and psychological terms, the researchers noted. “Girls with a shunted hydrocephalus should therefore be routinely monitored for linear growth and physical development, in order to identify signs of precocious or early puberty.”

Early detection, by finding a breast bud palpable under the areola (Tanner stage 2B) or a young age at the intersection of the prepubertal and pubertal linear growth trajectories, is important and actionable, they explained.

“When indicated, this condition may be alleviated by postponing the pubertal development, by treatment with gonadotropin-releasing hormone analogues,” which halt physical as well as skeletal maturation and postpone menarche, Dahl’s group wrote.

Precocious and early puberty have been documented in girls with hydrocephalus, but little has been known about their occurrence or potential associated risk factors in children with hydrocephalus due to causes other than myelomeningocele, Dahl’s group noted. Thus, the group aimed to define these in a population-based cohort of unselected female patients with shunted infantile hydrocephalus.

For the study, researchers analyzed clinical data and timing of pubertal signs of all 82 girls born between 1980 and 2002 who were admitted to the Department of Pediatric Surgery at Uppsala University Children’s Hospital for insertion of a ventriculoperitoneal shunt across a catchment area of about 2 million inhabitants.

The study population included 39 girls with and 42 without myelomeningocele. Median age at last admission was 15.8 years (range 10.0-18.0).

The group assessed puberty according to Tanner stages at regular follow-up visits to pediatric outpatient clinics and collected data on weight and length at birth and up to 15 years of age. Gestational age, weight, height, and BMI up to 6 years of age were similar between the two groups. There were no significant differences between groups in terms of intracranial pressure or timing of shunt insertion.

Almost half (45%) of the girls received treatment with gonadotropin-releasing hormone analogue; this included 22 with myelomeningocele and 11 without myelomeningocele. The majority of the patients with precocious or early puberty, with or without hydrocephalus, received hormone treatment. Five of the 22 with myelomeningocele received it despite not meeting criteria for early puberty.

The researchers cautioned that adverse effects of long-term treatment with gonadotropin-releasing hormone analogues include weight gain and impaired bone mineralization. “These effects are particularly disadvantageous for non-ambulant girls and should be kept in mind when treatment is considered, as well as during follow-up of treatment,” they wrote.

More children with early versus normal age at puberty were unable to walk (72% vs 43%, P=0.018).

Dahl’s group acknowledged that study limitations included the lack of pubertal data for nine patients without myelomeningocele and difficulty measuring height in patients who were unable to ambulate.

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