David O. Udoh, Abiodun L. Azeez, Emmanuel C. Obeta, Uyiosa Osazuwa, Kingsley O. Osabuohien
University of Benin Teaching Hospital
P.M.B.1111, Benin City, Edo State, Nigeria
Email: davidudoh07@gmail.com
Consultant Neurological Surgeon
ABSTRACT
Congenital anomalies of the neuraxis are an important cause of morbidity and mortality in the perinatal
period and during infancy; presentation is rare after childhood though some present for the first time in older
children and adults. There is a dearth of data from our geographically distinct population referred to as the
Niger Delta, which includes Nigeria’s major oil producing areas. A retrospective study of 213 patients
surgically managed on our service from June 2006 to May 2014. We studied demographic, clinical and
radiologic data as well as outcomes. Data was analysed using SPSS 21.0. We surgically managed 213 patients
representing 17% of all neurosurgical (and 53% of paediatric neurosurgical) operations in our centre during the
8-year period. Most patients, 70%, presented in infancy; only 8% presented in the neonatal period and 2.8%
after 15 years. Patients with neural tube defects presented earlier. Overall male to female ratio was 1.2: 1;
however, there are more males born with hydrocephalus spina bifida while the reverse is the case with the
others in this study. Age range at time of operation was 18 days to 34 years. The other anomalies managed
surgically were encephalocoeles, craniosynostosis, porencephalic cysts and Adeloye- Odeku disease. The
majority, 37.5%, were born to parents who lived in Warri, Sapele and other parts of Delta State as well as
Port-Harcourt and Bayelsa; 31%, Benin City, 17% Edo North, 7.5% from the East (Onitsha etc), 3.7% from
the West (Akure, Ondo) and 3.7% from the Middle belt (Taraba, Lokoja). Fifty-five percent, 55.5%, were from
very low income/ low socio-economic settings, 38% from lower middle class and 6.5% upper middle class.
Mean maternal age was 30.6 years (range 18 to 41 years). Likelihood of congenital anomalies increased with
both maternal age and the sum of maternal and paternal ages. All the mothers had antenatal care in various
facilities, 75.5% had pre-natal ultrasonography which detected all cases of hydrocephalus, but only 25% of
encephalocoeles and none of the others. None had exposure to irradiation or teratogenic medication during
pregnancy. Family history of previous congenital anomaly was positive in one case of spina bifida and two
siblings with encephalocoele both operated in our facility. Congenital anomalies of the CNS are common in
paediatric neurosurgical practice. Most present in early infancy while an index of suspicion, meticulous
clinical and radiological examination will detect about 2.5 to 5% presenting between the second and third
decades. Further study may be necessary to determine other factors which might be involved in congenital
anomalies in the sub region.
Keywords: Congenital Anomalies; Neuraxis; Morbidity and Mortality; Childhood; Niger Delta; Paediatric
Neurosurgical Practice
ABSTRACT
Congenital anomalies of the neuraxis are an important cause of morbidity and mortality in the perinatal period and during infancy; presentation is rare after childhood though some present for the first time in older
children and adults. There is a dearth of data from our geographically distinct population referred to as the Niger Delta, which includes Nigeria’s major oil producing areas. A retrospective study of 213 patients
surgically managed on our service from June 2006 to May 2014. We studied demographic, clinical and radiologic data as well as outcomes. Data was analysed using SPSS 21.0. We surgically managed 213 patients
representing 17% of all neurosurgical (and 53% of paediatric neurosurgical) operations in our centre during the 8-year period. Most patients, 70%, presented in infancy; only 8% presented in the neonatal period and 2.8% after 15 years. Patients with neural tube defects presented earlier. Overall male to female ratio was 1.2: 1; however, there are more males born with hydrocephalus spina bifida while the reverse is the case with the others in this study. Age range at time of operation was 18 days to 34 years. The other anomalies managed surgically were encephalocoeles, craniosynostosis, porencephalic cysts and Adeloye- Odeku disease. The
majority, 37.5%, were born to parents who lived in Warri, Sapele and other parts of Delta State as well as Port-Harcourt and Bayelsa; 31%, Benin City, 17% Edo North, 7.5% from the East (Onitsha etc), 3.7% from
the West (Akure, Ondo) and 3.7% from the Middle belt (Taraba, Lokoja). Fifty-five percent, 55.5%, were from very low income/ low socio-economic settings, 38% from lower middle class and 6.5% upper middle class.
Mean maternal age was 30.6 years (range 18 to 41 years). Likelihood of congenital anomalies increased with both maternal age and the sum of maternal and paternal ages. All the mothers had antenatal care in various
facilities, 75.5% had pre-natal ultrasonography which detected all cases of hydrocephalus, but only 25% of encephalocoeles and none of the others. None had exposure to irradiation or teratogenic medication during
pregnancy. Family history of previous congenital anomaly was positive in one case of spina bifida and two siblings with encephalocoele both operated in our facility. Congenital anomalies of the CNS are common in
paediatric neurosurgical practice. Most present in early infancy while an index of suspicion, meticulous clinical and radiological examination will detect about 2.5 to 5% presenting between the second and third
decades. Further study may be necessary to determine other factors which might be involved in congenital anomalies in the sub region.
Keywords: Congenital Anomalies; Neuraxis; Morbidity and Mortality; Childhood; Niger Delta; Paediatric Neurosurgical Practice
