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

Exstrophy Anomalies: Preliminary Review of 18 Cases in Maiduguri; North-Eastern Nigeria

Background: Exstrophy anomalies; although rare; pose a tremendous challenge to both surgeons and surrogates. Management is intricate and experience with large members is restricted to a few centres worldwide. This report is a review of 18 cases of these anomalies managed in University of Maiduguri Teaching Hospital (UMTH) over 8 years; highlighting the intricacies of management; and contributes to the existing data bank on this subject matter in our environment. Materials and Methods: We reviewed the records of all cases of anomalies that were managed in the index hospital from December 2002 to December 2010. Results: Eighteen cases were studied. The youngest was a 2-day-old neonate and the oldest was 6 years at repair; with a median age of 4 months. There were 7 boys and 9 girls with bladder exstrophy; and the remaining two were girls with cloacal exstrophy; giving a male-female ratio of 7:11 for the exstrophy anomalies. Eight patients (33.5) had associated congenital anomalies; 16.7 had undescended testes (UDT); anorectal malformation (ARM) and patent processus vaginalis (PPV); and duplicate bladder occurred in 5.6 of the patients. All but one patient had bilateral posterior iliac osteotomy at bladder closure. Out of the 17 patients that had osteotomy; 76.5 had satisfactory bladder closure (no complication); 5.9 had superficial wound dehiscence; 11.8 had bladder neck dehiscence and 5.9 had complete disruption. The lone patient that did not undergo osteotomy had complete bladder dehiscence. Postoperative immobilization was done for 3 weeks for 16 cases; one was discontinued after 1 week because of complication. The longest follow-up was for 2 years and the shortest for 2 weeks. Four patients (22.2) did not turn up for follow-up. Conclusion: Exstrophy anomalies although rare remain a major challenge in paediatric surgery. Iliac osteotomy; among other technical considerations; remains a cornerstone for successful bladder closure