Demographics, referral patterns, and outcome of patients with neural tube defects in southwestern Uganda.

OBJECTIVE In Uganda, the burden of neural tube defects (NTDs) poses a serious neurosurgical and public health challenge; however, published data on this patient population are lacking. The authors sought to characterize the population of patients with NTDs, maternal characteristics, and referral patterns of these patients, and to quantify the burden of NTDs in southwestern Uganda. METHODS A retrospective neurosurgical database at a referral hospital was reviewed to identify all patients with NTDs treated between August 2016 and May 2022. Descriptive statistics were used to characterize the patient population and maternal risk factors. A Wilcoxon rank-sum test and chi-square test were used to determine the association between demographic variables and patient mortality. RESULTS A total of 235 patients were identified (121 male, 52%). The median age at presentation was 2 days (IQR 1-8 days). A total of 87% of patients with NTDs presented with spina bifida (n = 204) and 31 presented with encephalocele (13%). The most common location of dysraphism was lumbosacral (n = 180, 88%). Of all patients, 80% were delivered vaginally (n = 188). Overall, 67% of patients were discharged (n = 156) and 10% died (n = 23). The median length of stay was 12 days (IQR 7-19 days). The median maternal age was 26 years (IQR 22-30 years). The majority of mothers received only primary education (n = 100, 43%). The majority of mothers reported prenatal folate use (n = 158, 67%) and regular antenatal care (n = 220, 94%), although only 23% underwent an antenatal ultrasound (n = 55). Mortality was associated with younger age at presentation (p = 0.01), need for blood transfusion (p = 0.016) and oxygen supplementation (p < 0.001), and maternal education level (p = 0.001). CONCLUSIONS To the authors' knowledge, this is the first study to describe the population of patients with NTDs and their mothers in southwestern Uganda. A prospective case-control study is necessary to identify unique demographic and genetic risk factors associated with NTDs in this region.

[1]  S. Lam,et al.  The role of neurosurgeons in global public health: the case of folic acid fortification of staple foods to prevent spina bifida. , 2022, Journal of neurosurgery. Pediatrics.

[2]  A. Lekoubou,et al.  Birth prevalence of neural tube defects in eastern Africa: a systematic review and meta-analysis , 2022, BMC Neurology.

[3]  R. Berry,et al.  Preventing birth defects, saving lives, and promoting health equity: an urgent call to action for universal mandatory food fortification with folic acid. , 2022, The Lancet. Global health.

[4]  C. Moore,et al.  Prevalence of neural tube defects, maternal HIV status, and antiretroviral therapy from a hospital-based birth defect surveillance in Kampala, Uganda , 2021, Birth defects research.

[5]  K. Chalkidou,et al.  The Use of Cost-Effectiveness Thresholds for Evaluating Health Interventions in Low- and Middle-Income Countries From 2015 to 2020: A Review , 2021, Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research.

[6]  G. Oakley,et al.  Modeling shows high potential of folic acid‐fortified salt to accelerate global prevention of major neural tube defects , 2020, Birth defects research.

[7]  F. Henderson,et al.  The College of Surgeons of East, Central and Southern Africa: Successes and Challenges in Standardizing Neurosurgical Training. , 2020, World neurosurgery.

[8]  Ahmad Masum.  UGANDA: A Country Profile , 2020 .

[9]  P. Musoke,et al.  A hospital-based birth defects surveillance system in Kampala, Uganda , 2019, BMC Pregnancy and Childbirth.

[10]  S. Lockman,et al.  Neural-Tube Defects and Antiretroviral Treatment Regimens in Botswana. , 2019, The New England journal of medicine.

[11]  Paul A. Harris,et al.  The REDCap consortium: Building an international community of software platform partners , 2019, J. Biomed. Informatics.

[12]  Geoffrey A. Anderson,et al.  Patterns and treatment outcomes of anorectal malformations in Mbarara Regional Referral Hospital, Uganda. , 2019, Journal of pediatric surgery.

[13]  G. Grant,et al.  Neural tube defects in Uganda: follow-up outcomes from a national referral hospital. , 2018, Neurosurgical focus.

[14]  B. T. Tadesse,et al.  Magnitude of Neural Tube Defects and Associated Risk Factors at Three Teaching Hospitals in Addis Ababa, Ethiopia , 2018, BioMed research international.

[15]  P. Mastroiacovo,et al.  Triple surveillance: a proposal for an integrated strategy to support and accelerate birth defect prevention , 2018, Annals of the New York Academy of Sciences.

[16]  Matthew W. Darlison,et al.  Estimates of global and regional prevalence of neural tube defects for 2015: a systematic analysis , 2018, Annals of the New York Academy of Sciences.

[17]  M. Shrime,et al.  Surgical volume and postoperative mortality rate at a referral hospital in Western Uganda: Measuring the Lancet Commission on Global Surgery indicators in low‐resource settings , 2017, Surgery.

[18]  L. Park,et al.  Epidemiology and Characteristics of Neurosurgical Conditions at Mbarara Regional Referral Hospital. , 2017, World neurosurgery.

[19]  R. Berry,et al.  Retrospective Assessment of Cost Savings From Prevention: Folic Acid Fortification and Spina Bifida in the U.S. , 2016, American journal of preventive medicine.

[20]  Jennifer Williams,et al.  Describing the Prevalence of Neural Tube Defects Worldwide: A Systematic Literature Review , 2016, PloS one.

[21]  D. Polšek,et al.  Estimating the burden of neural tube defects in low– and middle–income countries , 2014, Journal of global health.

[22]  A. Elliott,et al.  Maternal recall of birthweight and birth size in Entebbe, Uganda , 2012, Tropical medicine & international health : TM & IH.

[23]  I. Munabi,et al.  Prevalence, Nature and Characteristics of External Congenital anomalies at Mulago Hospital , 2011 .

[24]  A. Kulkarni,et al.  Factors affecting survival of infants with myelomeningocele in southeastern Uganda. , 2011, Journal of neurosurgery. Pediatrics.

[25]  Ryu-ichi Katagiri,et al.  Gender‐dependent differences in the incidence of ochratoxin A‐induced neural tube defects in the Pdn/Pdn mouse , 2010, Congenital anomalies.

[26]  G. Bbosa,et al.  Aflatoxin levels in common foods of South Western Uganda: a risk factor to hepatocellular carcinoma. , 2009, European journal of cancer care.

[27]  P. Harris,et al.  Research electronic data capture (REDCap) - A metadata-driven methodology and workflow process for providing translational research informatics support , 2009, J. Biomed. Informatics.

[28]  E. Haan,et al.  Trends in state/population-based screening for neural tube defects (NTDS), south australia 1986-2004 , 2006 .

[29]  D. Weir,et al.  Maternal plasma folate and vitamin B12 are independent risk factors for neural tube defects. , 1993, The Quarterly journal of medicine.

[30]  B. Warf,et al.  Ten-year survival of Ugandan infants after myelomeningocele closure. , 2017, Journal of neurosurgery. Pediatrics.

[31]  H. Babikir,et al.  Clinical profile of neural tube defects in Sudanese children: Is malaria a risk factor? , 2017, Sudanese journal of paediatrics.

[32]  Alan D. Lopez,et al.  Health dimensions of sex and reproduction : the global burden of sexually transmitted diseases, HIV, maternal conditions, perinatal disorders, and congenital anomalies , 1998 .

[33]  From the Centers for Disease Control and Prevention. Recommendations for use of folic acid to reduce number of spina bifida cases and other neural tube defects. , 1993, Journal of the American Medical Association (JAMA).

[34]  B. Warf,et al.  Neural-tube defects. , 1978, Lancet.