Adult outcome of pediatric hydrocephalus

Abstract

Introduction: The outlook of pediatric hydrocephalus has spectacularly improved over the past decades; however, the adult outcome is still poorly documented. Determining the healthcare profile of these patients is important in order to organize the management of this growing population. We decided to review our pediatric hydrocephalus database for pediatric patients treated for hydrocephalus and followed up into adulthood.

Methods: Our institution has a virtual monopoly for pediatric hydrocephalus, serving a four-million-plus population; the transition to adult care is also managed in the same institution. We retrospectively reviewed patients younger than 18 treated for hydrocephalus since 1980 and followed up beyond the age of 20.

Results: We reviewed 456 patients, with a mean initial age of 55.6 months, and followed up for a mean of 24.2 years. In 81 patients (17.8%), the last shunt operation occurred after 20 years; 22 of these (4.9% of the total) having never been revised earlier. Sixteen patients (3.5%) could be weaned of their shunt. Thirteen patients died in adult age, 5 of these dying of shunt-related causes. The most prominent sequels were motor (46.5%) and cognitive (47.6%); only 82 patients (18.0%) had no sequel at all. Intelligence quotient values were ≥80 in 54.5% of tested patients; however, schooling was normal in only 41.4%, and only 33.7% was employed in the competitive labor market.

Conclusion: Adults treated for hydrocephalus in childhood require a life-long follow-up. Late mortality is low but not null, morbidity is high, and many patients require shunt surgery during adulthood. The transition from child to adult neurosurgery needs to be organized for these vulnerable patients.

Fig. 1

Actuarial event-free survival after the first surgery. This curve shows the characteristic biphasic curve initially, and then the curve shows a shoulder around 15 years, becoming almost parallel to the baseline. However, several patients had their first shunt revision after 20 years, showing that patients who have never been revised before cannot be considered as shunt-independent

Fig. 2

Number of reoperations per patient. Most patients had at least one reoperation, although 91 (20.0%) had not required any reoperation at last control

Fig. 3

Mortality after the age of 20. Thirteen patients died between 20 and 27 years, the actuarial mortality was 13%

Fig. 4

Distribution of Karnofsky independence score; 53.8% had a KNK score at or above 80 (independent life)

Fig. 5

Prevalence of the different sequels among the patients of the series. Only 82 patients (18.0%) were free of all of these ailments

Fig. 6

Overall outcome. The outcome was evaluated with the GOS (GOS 1 = normal life, GOS 5 = dead) according to the year of initial treatment; this figure suggests that mortality and severe morbidity (GOS 3 to 5) were higher for patients treated in the 1980s and early 1990s. However, patients treated in the 1990s must have been older than the average at the time of initial treatment to meet the inclusion criteria, and their follow-up was also shorter, introducing important biases

Fig. 7

Comparison between IQ testing, schooling, and social integration. Normal IQ was defined as 80 or higher. The discrepancy points at underestimation of handicap by formal testing and academic achievements, representing “the invisible handicap”