A review of the epidemiology, post-neurosurgical closure complications and outcomes of neonates with open spina bifida

Background. Spina bifida (SB) is a neural tube defect (NTD) that has an increased risk of fatal and disabling effects if not repaired early, i.e. within the first 24 to 48 hours of life. Its diagnosis holds an increased burden for the patient and the caregiver owing to secondary complications. The effects of the disease are detrimental even with early repair, because of the long-term disabling nature of the disease.Objective. This retrospective study aimed to assess the effects of demographics, immediate post-surgical complications and impact of time to surgical intervention on the outcome of neonates with open SB (OSB) admitted to the neonatal intensive care unit (NICU) at Inkosi Albert Luthuli Central Hospital (IALCH) in KwaZulu-Natal, South Africa (SA), between January 2011 and December 2015.Methods. A retrospective chart review was conducted at the NICU of IALCH. All neonates diagnosed with SB were identified. The study period was from 1 January 2011 to 31 December 2015. Data were collected from the IALCH electronic database. All neonates with SB admitted to the IALCH NICU were included; any patient who presented beyond the neonatal period (i.e. >28 days) was excluded from the study. Data collected included maternal demographics. Additionally, neonatal history was reviewed and post surgery complications evaluated. Outpatient management post discharge was reviewed.Results. One hundred and fifty neonates were included (58% male). The mean (standard deviation) maternal age was 26.7 (6.6) years. Only 10% had an antenatal diagnosis of OSB. Seventy-eight percent were born at term and 22% prematurely. The lumbar/sacral region was the most commonly affected. More males (14%) had thoraco/lumbar lesions than females (7.8%). Forty-eight percent presented before 3 days of life (early presentation). In the late-presentation group, there was an association with wound sepsis (p=0.003). Twenty-five percent were repaired between days 0 and 3 of life and 75% after 3 days. Postoperative complications in patients whose open SBs were repaired beyond 3 days of life were not statistically significant compared with those with early repair; all were p>0.05. There was a borderline association of prolonged hospitalisation with wound sepsis (p=0.07). Long-term outcomes showed that 68% had lower limb dysfunction, 18% urological complications, 14% limb deformity, and 11% hydrocephalus. A minority had psychomotor (7%) and developmental (15%) disorders. Ten percent required readmission secondary to shunt complications, and 7% died. Conclusion. SB remains a significant disease burden that affects outcome and survival of neonates in SA. Lack of good antenatal care, which includes early ultrasound and timely referral to centres, are barriers to good outcomes. Long-term follow-up is also necessary to prevent morbidity

Profile and management of central nervous system malformations in a tertiary hospital

Background: Central Nervous System (CNS) malformations rank among the commonest malformations. These may be identified in utero or noticed immediately after birth. Most studies showed Myelomeningocoele, a Neural Tube Defect (NTD) to be the commonest. Severe defects have a significant impact on long term disability. While some are preventable, most are surgically managed. Objective: To study the profile and management of CNS malformation in a tertiary hospital. Method: we undertook a 3-year retrospective study of the profile and surgical management, including the postoperative complications among Neonates admitted into the Special Care Baby Unit (SCBU). Data on place of delivery, Gender, Gestational age, Mode of delivery, Prenatal Diagnosis (Ultrasound Scan), types of malformations, types of surgeries, and postoperative complications were retrieved. Result: We found 71 patients with CNS anomalies (31.8% of all congenital anomalies), in delivery 10 (14.1%), out delivery 46(64.8%). Male=43, females=28. Term gestation 66 (93%), preterm 3(4.2%), post-term 2(2.8%). Delivered by Vagina (SVD) in 65(91.6%), Caesarean section 4(5.6%), and undocumented in 4(5.6%). Prenatal diagnosis in only 3(4.2%). Myelomeningocoele was 35(49.3%), meningocoele was 9(12.7%), Hydrocephalus was 18(25.3%), anencephaly was 1(1.4%), occipital Encephalocoele was 6(8.5%), Sincipital Encephalocoele was 2(2.8%). Operated on 53(75%), LAMA/lost to follow was 13(18%), preoperative death was 5(7%). Excision and closure of Neural Tube Defect were 34(64.2%), VP Shunt was 13(24.5%), Excision and closure of occipital Encephalocoele were 5(9.4%) craniofacial excision and repair of Sincipital Encephalocoele was 1(1.9%). CSF leak in 4(28.6%), wound infection in 3(21.4%), shunt infection and obstruction in 2(14.3%) each. meningitis, hypertrophied scar, and pseudo meningocoele in 1(7.1%) each. Conclusion: The commonest CNS anomaly is a Myelomeningocoele a preventable condition, Sincipital Encephalocoele is not common in our environment. Concomitant treatment of hydrocephalus averts post excision CSF Leak

Neural tube defect diagnosis and outcomes at a tertiary South African hospital with intensive case ascertainment

Background. Neural tube defects (NTDs) are an important category of birth defect, but surveillance remains inadequate in South Africa.Objectives. To assess the identification of NTDs at a tertiary hospital using a range of prenatal, perinatal and postnatal data sources, and to estimate the impact of prenatal diagnosis and birth prevalence for the referral area.Methods. Cases of anencephaly, encephalocele and spina bifida (SB) in a 6-year period were retrospectively identified from 5 data sources covering prenatal, perinatal and postnatal care. These were cross-correlated to avoid duplicate entries and to determine the contribution of different data sources. Details of prenatal diagnosis and termination of pregnancy (TOP) were obtained for 10 years, and birth prevalence over 2 years.Results. During a 6-year period 195 NTDs were identified at a Western Cape Province tertiary hospital. These included 59 (30%) cases of anencephaly, 28 (14%) of encephalocele and 108 (55%) of SB. The majority of NTDs (71%) were detected prenatally, although SB was less commonly diagnosed prenatally than cranial defects (56% v. 88%; p<0.001). Of SB cases ascertained pre- or postnatally, 57% of patients were born alive and 50% discharged alive, but 72% of survivors had not been diagnosed prenatally. Women receiving prenatal diagnosis of any type of NTD before 24 weeks' gestation were nearly always offered TOP, and the majority accepted termination after non-directive counselling. For SB, later prenatal diagnosis was associated with much lower termination rates because the option was less often offered (51% v. 100%; p<0.001), and perhaps less often accepted (57% v. 78%; p=0.06). The estimated NTD birth prevalence for the referral area was 0.76 - 0.80 per 1 000 live births, but perhaps up to 1.18 per 1 000 when considering under-referral of lethal cranial lesions from rural areas.Conclusions. A substantial number of NTDs can be ascertained from a tertiary hospital environment if multiple data sources are used, even though adding data from the Perinatal Problem Identification Program for outlying health facilities increases detection of lethal defects. Hospital-based surveillance can be considered, especially for SB. Prenatal diagnosis was fairly common and pregnancy termination was often offered and accepted if detected before 24 weeks' gestation. A regional prenatal ultrasound programme, predominantly based in primary care but with ready access to a tertiary centre, can be quite effective, although limited or delayed access to prenatal diagnosis must be addressed

Maternal serum alpha fetoprotein screening test in routine antenatal care : Is it cost effective in Cameroon?

Screening test for maternal serum alpha feoto-protein (MS-AFP) in routine antenatal care is compulsory in most developed countries to detect neural tube defect (NTD). With the cost of suck a programme; there was a need to evaluate its need in our environment. A high risk clinic was choosen for the recruitment of the subjects to maximize the risk. Incidence of NTD could be considered to be low in Cameroon compared to countries where screening for MS-AFP is compulsory. Coupled with the low socio-economic status of our population and the lack of infrastructures; we could conclude that this screening test on a routine basis is not cost-effective