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

Meconium peritonitis in nigerian children

Background: Meconium peritonitis is a rare disease with a fatal outcome. In Nigeria and Africa; there are only the occasional case reports on the subject matter. Methods: This is a 10-year retrospective study of all patients with meconium peritonitis treated at the University of Maiduguri Teaching Hospital; Maiduguri; Borno State; the Lagos University Teaching Hospital; Lagos State; Obafemi Awolowo University Teaching Hospitals complex; Ile-Ife; Osun State and the Federal Medical Centre Gombe; Gombe State; Nigeria. Results: There were 10 neonates comprising 6 girls and 4 boys. The median age at presentation was 4 days (range 2-6 days). Six of the mothers of the children with meconium peritonitis had a supervised antenatal care and 4 had antenatal ultrasonography but meconium peritonitis was missed. The most common clinical presentation was abdominal distension at birth in 9 of 10 patients. The abdominal X- rays showed calcification and homogenous opacity in 4 patients and pneumoperitoneum in 2 patients. At laparotomy; all the patients had inflammatory adhesion bands and matted bowel loops. The generalized type was the commonest form observed (7 patients) and giant pseudocyst was noted in 2 patients. The commonest sites of perforation were the ileum in 4 patients and jejunum in 3 patients. In one patient the perforation had sealed at laparotomy. Intestinal obstruction was the commonest cause of meconium peritonitis in 7 of 10 patients. In the remaining 3 patients the cause is unknown. The commonest procedure performed was resection and anastomosis (4 patients).The mortality rate was high (50). Conclusion: Our data revealed the rarity of meconium peritonitis and intestinal obstruction as the commonest cause. It is recommended that in patients with an unidentifiable cause a rectal biopsy should be done to rule out Hirschsprung's disease. Early diagnosis; proper operative procedure and meticulous post-operative care should improve their survival

Paediatric urolithiasis in northern Nigeria

Background. The incidence of paediatric urolithiasis varies according to geographic areas. In Nigeria, there is paucity of literature on urinary stone diseases in childhood. Materials and Methods. In the period between January 1993 and December 2003, 67 Nigerian children with urinary stones were retrospectively reviewed. Results. There were 63 (94.0%) boys and 4 (6.0%) girls. The mean age at presentation was 6.9 years. Thirty one (46.3%) were in the age group less than 5 years. The commonest presenting symptoms were dysuria 42 (62.7%) and abdominal pain in 30 (44.8%). Pulling on the penis is characteristically present in 26 (38.8%) patients. The stones were located in the lower tract in 57 (85.1%) cases. Anatomic obstruction along the urinary tract was responsible for stone formation in 12 (17.9%) patients. Sixteen (23.9%) patients had associated urinary tract infection. All the stones analyzed were mixed and 75% contained urate. In 7 (10.4%) patients the stones were passed spontaneously. Open surgical technique was employed in the remaining 60 patients. There was no death recorded. Post-operative complications occurred in 15 (25.0%) of the operated patients. The commonest complications were wound infection 3 (5.0%) and vesicocutaneous fistula in 3 (5.0%) patients. The average duration of hospital stay was 16.0 days. Conclusion. In our practice lower tract stones are common and congenital urinary tract obstruction is the commonest identifiable cause for stones. The age and sex distribution and chemical composition are in keeping with historic and endemic stones. Early detection and treatment of urinary tract obstruction will further reduce the durden of stones disease

Factors affecting outcome of emergency paediatric abdominal surgery

Background: Paediatric surgical abdominal emergencies are important causes of morbidity and mortality in children. None of the previous studies on abdominal surgical emergencies specifically addressed the problem as it affects children. Methods: An eight-year retrospective review of all children aged 0-15 years who presented with acute abdominal emergency. Results: Two-hundred and fifty one (251) children; male to female ratio of 1.5:1 and median age of presentation of 78 months (range 1 day-15 years). The median duration of symptoms before presentation to hospital was 96 hours. The median duration between admission and surgical intervention was 24 hours. Intestinal obstruction accounted for 87 (34.7) of acute abdominal emergency. This was the commonest emergency encountered overall. Typhoid perforation (TP) was responsible for 71 (28.3) of all acute abdominal emergencies and was the commonest condition encountered in children above the age of one year. Others included 55 (21.9) cases of appendicitis; 18 (7.2) of abdominal trauma; 6 (2.4) anterior abdominal wall defects and 5 (2.0) each of infantile hypertrophic pyloric stenosis and primary peritonitis. Postoperative complications were observed in 85 (33.9) of patients. The commonest complications were wound infection 31 (12.4) and wound dehiscence 23 (9.2); while mortality rate was 16 overall; and 31.8 in the neonatal period. Overwhelming sepsis was responsible for 26 (63.4) of all deaths recorded. Multiple congenital anomalies were the cause of death in 5 (12.2). Other causes of death were prolonged ileus with malnutrition in 4 patients; respiratory insufficiency in 2 patients and a patient each died from necrotising enterocolitis; acute renal failure; enterocutaneous fistula and over hydration. Conclusion: intestinal obstruction was the commonest acute abdominal emergency in children. The high rates of postoperative morbidity observed could be attributed to delayed presentation to hospital