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

Outcome of management of complicated extragonadal teratoma in a resource poor setting.

BACKGROUND: Extragonadal teratomas (EXGTs) are ubiquitous in the human body; hence, they have varied presentation. In underdeveloped areas presentation and management are affected by socio-economic, cultural and health facilities factors. The aim of this study was to review the outcome of management of complicated EXGT in a tertiary health centre. MATERIALS AND METHODS: A review data of paediatric patients with EXGT was done between January 1999 and December 2012. Variables reviewed were bio-data, mode of presentation and site of tumour, comorbidity, treatments and outcome. The data was analysed with Statistical Package for Social Sciences (SPSS (R)) version 16.0. RESULTS: There were 21 complicated EXGT (77.8%) among 27 children, age ranges from 4 days to 16 years (median = 2 years). Male:Female ratio of 1:2. The complications per region of the body at presentation were cervical 4 (66.7%), mediastinal 2 (100%), abdominal 3 (75%) and sacrococcygeal 12 (75%). The complications were respiratory distress 6, intestinal obstruction 5, faecal incontinence 2, bladder outlet obstruction 3, malignant transformation 5, ruptured sacrococcygeal teratoma 2, ulcerated tumour 2, anaemia 3 and malnutrition 3. There were 5 (23.8%) progressive disease post-excision outside our facility. Excision biopsy was successful in 19 (85%) patients two of which had neoadjuvant cytotoxic therapy. Overall mortality was 5 (23.8%) (septicaemia, anaemia, respiratory distress, renal failure) and post-excision mortality was 11.8% (endotracheal tube blockage and progressive disease). CONCLUSION: Delay presentation (due to local belief, ignorance and poverty) malnutrition, sepsis, malignant transformation characterised presentation of children in this study and the lack of paediatric intensive care unit facility and intensivists compromised survival of children with EXGT.