Global hydrocephalus epidemiology and incidence: systematic review and meta-analysis

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OBJECTIVE

Hydrocephalus is one of the most common brain disorders, yet a reliable assessment of the global burden of disease is lacking. The authors sought a reliable estimate of the prevalence and annual incidence of hydrocephalus worldwide.

METHODS

The authors performed a systematic literature review and meta-analysis to estimate the incidence of congenital hydrocephalus by WHO region and World Bank income level using the MEDLINE/PubMed and Cochrane Database of Systematic Reviews databases. A global estimate of pediatric hydrocephalus was obtained by adding acquired forms of childhood hydrocephalus to the baseline congenital figures using neural tube defect (NTD) registry data and known proportions of posthemorrhagic and postinfectious cases. Adult forms of hydrocephalus were also examined qualitatively.

RESULTS

Seventy-eight articles were included from the systematic review, representative of all WHO regions and each income level. The pooled incidence of congenital hydrocephalus was highest in Africa and Latin America (145 and 316 per 100,000 births, respectively) and lowest in the United States/Canada (68 per 100,000 births) (p for interaction < 0.1). The incidence was higher in low- and middle-income countries (123 per 100,000 births; 95% CI 98–152 births) than in high-income countries (79 per 100,000 births; 95% CI 68–90 births) (p for interaction < 0.01). While likely representing an underestimate, this model predicts that each year, nearly 400,000 new cases of pediatric hydrocephalus will develop worldwide. The greatest burden of disease falls on the African, Latin American, and Southeast Asian regions, accounting for three-quarters of the total volume of new cases. The high crude birth rate, greater proportion of patients with postinfectious etiology, and higher incidence of NTDs all contribute to a case volume in low- and middle-income countries that outweighs that in high-income countries by more than 20-fold. Global estimates of adult and other forms of acquired hydrocephalus are lacking.

CONCLUSIONS

For the first time in a global model, the annual incidence of pediatric hydrocephalus is estimated. Low- and middle-income countries incur the greatest burden of disease, particularly those within the African and Latin American regions. Reliable incidence and burden figures for adult forms of hydrocephalus are absent in the literature and warrant specific investigation. A global effort to address hydrocephalus in regions with the greatest demand is imperative to reduce disease incidence, morbidity, mortality, and disparities of access to treatment.

ABBREVIATIONS AFR = African Region; AMR-L = Region of the Americas, Latin America; AMR-US/Can = Region of the Americas, United States/Canada; EMR = Eastern Mediterranean Region; EUR = European Region; HIC = high-income country; IHME = Institute for Health Metrics and Evaluation; IQR = interquartile range; LMIC = low- and middle-income country; NPH = normal pressure hydrocephalus; NTD = neural tube defect; PHH = posthemorrhagic hydrocephalus; PIH = postinfectious hydrocephalus; SEAR = South-East Asia Region; WPR = Western Pacific Region.

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Article Information

Correspondence Michael C. Dewan: Vanderbilt University Medical Center, Nashville, TN. dewan.michael@gmail.com.

INCLUDE WHEN CITING Published online April 27, 2018; DOI: 10.3171/2017.10.JNS17439.

Disclosures Dr. Glancz: financial assistance provided by Codman and Hospital Corporation of America for volunteering in the training of third world neurosurgical units.

© AANS, except where prohibited by US copyright law.

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Figures

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    PRISMA flow diagram. Seventy-eight articles were incorporated into the review from a total of 1711 titles.

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    Incidence of hydrocephalus by WHO region. WHO regions are shaded according to annual incidence of hydrocephalus (per 100,000 births). © OpenStreetMap contributors. Figure is available in color online only.

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    Forest plot of the incidence of congenital hydrocephalus by WHO region; random-effects model. Effect size (ES) values represent the number of cases of hydrocephalus per 100,000 births (95% CI). Diamonds represent the pooled estimate of the incidence for each subgroup (width denotes 95% CI). Weights are from the random-effects analysis using the method of DerSimonian and Laird. Heterogeneity by WHO region: AFR (I2 = 96.9%, p for heterogeneity < 0.01; 9 studies); AMR-L (I2 = not applicable; 1 study); AMR-US/Can (I2 = 95.8%, p < 0.01; 4 studies); EMR (I2 = 94.8%, p < 0.01; 7 studies); EUR (I2 = 98.0%, p < 0.01; 15 studies); SEAR (I2 = not applicable; 1 study); and WPR (I2 = 97.7%, p < 0.01; 7 studies); p for interaction comparing the different subgroups < 0.01. Figure is available in color online only.

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    Geographic representation of source data. The global map indicates countries from which hydrocephalus data were derived. Countries are shaded according to study quality along a spectrum, where 0/5 is shaded light purple and 5/5 is shaded dark purple. In countries with more than a single paper, the highest study quality was used for shading purposes. © OpenStreetMap contributors. Figure is available in color online only.

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    Forest plot of congenital hydrocephalus incidence by World Bank income level (LMICs vs HICs); random-effects model. Effect size values represent cases of hydrocephalus per 100,000 live births (95% CI). Diamonds represent the pooled estimate of the incidence for each subgroup (width denotes 95% CI). Weights are from the random-effects analysis using the method of DerSimonian and Laird. Heterogeneity by income level: LMICs (I2 = 97.1%, p < 0.01; 21 studies); HICs (I2 = 97.5%, p for heterogeneity < 0.01; 21 studies); p for interaction comparing the different subgroups < 0.01. Figure is available in color online only.

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