Metabolic Brain Disease

, Volume 33, Issue 3, pp 837–842 | Cite as

Prevalence and trend of isolated and complicated congenital hydrocephalus and preventive effect of folic acid in northern China, 2005–2015

  • Jufen Liu
  • Lei Jin
  • Zhiwen Li
  • Yali Zhang
  • Le Zhang
  • Linlin Wang
  • Aiguo RenEmail author
Original Article


Congenital hydrocephalus (CH) was a major birth defect of the central nervous system besides neural tube defects (NTDs). Few studies have focused on both the prevalence and trend of isolated and complicated CH in China. Data were drawn from a population-based birth defects surveillance program in five rural counties in northern China from 2005 to 2015. All livebirths and pregnancy terminations at any gestational age affected with CH were recorded. The prevalence and trend of isolated and complicated CH were examined. During the 11-year period, a total of 176,223 births and 357 CH cases were recorded, resulting in a prevalence rate of 20.3 CH cases per 10,000 births. Of the CH cases, 146 were isolated CH, resulting in a prevalence rate of 8.3 per 10,000 births. The pre-perinatal prevalence (<28 gestational weeks) was higher than the perinatal prevalence for both isolated and total CH. The prevalence rates of total and isolated CH showed a similar downward trend during the 11–year period. This downward trend was statistically significant after 2009 (p < 0.05), when a massive folic acid supplementation program was introduced. Although it decreased over time, the prevalence of CH remains high in this population which has a high prevalence of neural tube defects.


Congenital hydrocephalus High prevalence Neural tube defects Folic acid supplementation 



This work was supported in part by the National Key Research and Development Program, Ministry of Science and Technology, P.R. China (grant No. 2016YFC1000500) and the National Natural Science Foundation of China (Grant Numbers: 81202265).

Compliance with ethical standards

Conflict of interest

None declared.


  1. Adeloye A, Warkany J (1976) Experimental congenital hydrocephalus. A review with special consideration of hydrocephalus produced by zinc deficiency. Childs Brain 2:325–360PubMedGoogle Scholar
  2. Cains S, Shepherd A, Nabiuni M, Owen-Lynch PJ, Miyan J (2009) Addressing a folate imbalance in fetal cerebrospinal fluid can decrease the incidence of congenital hydrocephalus. J Neuropathol Exp Neurol 68:404–416. CrossRefPubMedGoogle Scholar
  3. Dai L, Zhou GX, Miao L, Zhu J, Wang YP, Liang J (2006) Prevalence analysis on congenital hydrocephalus in Chinese perinatal from 1996 to 2004. Zhonghua Yu Fang Yi Xue Za Zhi 40:180–183PubMedGoogle Scholar
  4. Dreha-Kulaczewski S, Joseph AA, Merboldt KD, Ludwig HC, Gartner J, Frahm J (2017) Identification of the Upward Movement of Human CSF In Vivo and its Relation to the Brain Venous System. J Neurosci 37:2395–2402. CrossRefPubMedGoogle Scholar
  5. Eke CB, Uche EO, Chinawa JM, Obi IE, Obu HA, Ibekwe RC (2016) Epidemiology of congenital anomalies of the central nervous system in children in Enugu, Nigeria: A retrospective study. Ann Afr Med 15:126–132. CrossRefPubMedPubMedCentralGoogle Scholar
  6. Fan L, Gong T, Cao X, Du Y (2013) Epidemiologic characteristics of birth defects in the Hainan Province from 2000 to 2010, China. Birth Defects Res A Clin Mol Teratol 97:750–754. CrossRefPubMedGoogle Scholar
  7. Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H (2010) Congenital hydrocephalus--prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol 14:150–155. CrossRefPubMedGoogle Scholar
  8. Goh YI, Bollano E, Einarson TR, Koren G (2006) Prenatal multivitamin supplementation and rates of congenital anomalies: a meta-analysis. J Obstet Gynaecol Can 28:680–689CrossRefGoogle Scholar
  9. Gu X et al (2007) High prevalence of NTDs in Shanxi Province: a combined epidemiological approach. Birth Defects Res A Clin Mol Teratol 79:702–707. CrossRefPubMedGoogle Scholar
  10. Jeng S, Gupta N, Wrensch M, Zhao S, Wu YW (2011) Prevalence of congenital hydrocephalus in California, 1991-2000. Pediatr Neurol 45:67–71. CrossRefPubMedGoogle Scholar
  11. Jin L, Jin L, Yu J, Xu Y, Liu H, Ren A (2017) Prevalence of Neural Tube Defects and the Impact of Prenatal Diagnosis in Three Districts of Beijing, China. Paediatr Perinat Epidemiol.
  12. Li Z et al (2006) Extremely high prevalence of neural tube defects in a 4-county area in Shanxi Province, China. Birth Defects Res A Clin Mol Teratol 76:237–240. CrossRefPubMedGoogle Scholar
  13. Lim H, Agopian AJ, Whitehead LW, Beasley CW, Langlois PH, Emery RJ, Waller DK (2015) Maternal occupational exposure to ionizing radiation and major structural birth defects. Birth Defects Res A Clin Mol Teratol 103:243–254. CrossRefPubMedPubMedCentralGoogle Scholar
  14. Lindquist B, Persson EK, Fernell E, Uvebrant P (2011) Very long-term follow-up of cognitive function in adults treated in infancy for hydrocephalus. Childs Nerv Syst 27:597–601. CrossRefPubMedGoogle Scholar
  15. Liu J et al (2016a) Prevalence and trend of neural tube defects in five counties in Shanxi province of Northern China, 2000 to 2014. Birth Defects Res A Clin Mol Teratol 106:267–274. CrossRefPubMedGoogle Scholar
  16. Liu QG, Sun J, Xiao XW, Song GR (2016b) Birth defects data from surveillance hospitals in Dalian city, China, 2006-2010. J Matern Fetal Neonatal Med 29:3615–3621. CrossRefPubMedGoogle Scholar
  17. Ma R, Wang L (2017) Plasma folate levels and associated factors in women planning to become pregnant in a population with high prevalence of neural tube defects. Birth Defects Res A Clin Mol Teratol.
  18. Ministry of Health C (2012) China birth defects prevention report (2012). Available from: Accessed 4 Dec 2017
  19. Moritake K, Nagai H, Miyazaki T, Nagasako N, Yamasaki M, Tamakoshi A (2007) Nationwide survey of the etiology and associated conditions of prenatally and postnatally diagnosed congenital hydrocephalus in Japan. Neurol Med Chir (Tokyo) 47:448–452 discussion 452CrossRefGoogle Scholar
  20. Munch TN, Rostgaard K, Rasmussen ML, Wohlfahrt J, Juhler M, Melbye M (2012) Familial aggregation of congenital hydrocephalus in a nationwide cohort. Brain 135:2409–2415. CrossRefPubMedGoogle Scholar
  21. Naz N, Jimenez AR, Sanjuan-Vilaplana A, Gurney M, Miyan J (2016) Neonatal hydrocephalus is a result of a block in folate handling and metabolism involving 10-formyltetrahydrofolate dehydrogenase. J Neurochem 138:610–623. CrossRefPubMedGoogle Scholar
  22. O'Dell BL, Whitley JR, Hogan AG (1948) Relation of folic acid and vitamin A to incidence of hydrocephalus in infant rats. Proc Soc Exp Biol Med 69:272–275CrossRefPubMedGoogle Scholar
  23. Qian L et al (2017) Analysis of epidemiological trends in human papillomavirus infection among gynaecological outpatients in Hangzhou, China, 2011-2015. BMC Infect Dis 17:393. CrossRefPubMedPubMedCentralGoogle Scholar
  24. Ren AG (2015) Prevention of neural tube defects with folic acid: The Chinese experience. World J Clin Pediatr 4:41–44. CrossRefPubMedPubMedCentralGoogle Scholar
  25. Santos MM et al (2017) Infant hydrocephalus in sub-Saharan Africa: the reality on the Tanzanian side of the lake. J Neurosurg Pediatr 20:423–431. CrossRefPubMedGoogle Scholar
  26. Sun G, Xu ZM, Liang JF, Li L, Tang DX (2011) Twelve-year prevalence of common neonatal congenital malformations in Zhejiang Province, China. World J Pediatr 7:331–336. CrossRefPubMedGoogle Scholar
  27. Walsh S, Donnan J, Morrissey A, Sikora L, Bowen S, Collins K, MacDonald D (2016) A systematic review of the risks factors associated with the onset and natural progression of hydrocephalus. Neurotoxicology.
  28. Wilson RD et al (2007) Pre-conceptional vitamin/folic acid supplementation 2007: the use of folic acid in combination with a multivitamin supplement for the prevention of neural tube defects and other congenital anomalies. J Obstet Gynaecol Can 29:1003–1026CrossRefPubMedGoogle Scholar
  29. Woodard JC, Newberne PM (1966) Relation of vitamin B12 and one-carbon metabolism to hydrocephalus in the rat. J Nutr 88:375–381CrossRefPubMedGoogle Scholar
  30. Woodward JC, Newberne PM (1967) The pathogenesis of hydrocephalus in newborn rats deficient in vitamin B12. J Embryol Exp Morpholog 17:177–187Google Scholar
  31. Xie D, Yang T, Liu Z, Wang H (2016) Epidemiology of Birth Defects Based on a Birth Defect Surveillance System from 2005 to 2014 in Hunan Province, China. PLoS One 11:e0147280. CrossRefPubMedPubMedCentralGoogle Scholar
  32. Yang W, Zeng L, Cheng Y, Chen Z, Wang X, Li X, Yan H (2012) The effects of periconceptional risk factor exposure and micronutrient supplementation on birth defects in Shaanxi Province in Western China. PLoS One 7:e53429. CrossRefPubMedPubMedCentralGoogle Scholar
  33. Zhang X et al (2012) Prevalence of birth defects and risk-factor analysis from a population-based survey in Inner Mongolia, China. BMC Pediatr 12:125. CrossRefPubMedPubMedCentralGoogle Scholar
  34. Zhang TN et al (2017) Time trends in the prevalence and epidemiological characteristics of neural tube defects in Liaoning Province, China, 2006-2015: A population-based study. Oncotarget 8:17092–17104. PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Jufen Liu
    • 1
    • 2
  • Lei Jin
    • 1
    • 2
  • Zhiwen Li
    • 1
    • 2
  • Yali Zhang
    • 1
    • 2
  • Le Zhang
    • 1
    • 2
  • Linlin Wang
    • 1
    • 2
  • Aiguo Ren
    • 1
    • 2
    • 3
    Email author
  1. 1.Institute of Reproductive and Child Health, Key Laboratory of Reproductive Health, National Health and Family Planning Commission of the People’s Republic of ChinaPeking UniversityBeijingChina
  2. 2.Department of Epidemiology and Biostatistics, School of Public HealthPeking UniversityBeijingChina
  3. 3.Institute of Reproductive and Child HealthPeking University Health Science CenterBeijingChina

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