Risk factors, presentation and outcome of meningomyelocele repair.

Lal Rehman,Ali Afzal,Munwar Sheikh,Raza Rizvi

doi:10.12669/pjms.36.3.1237

Lal Rehman, Ali Afzal + Show 2 more

Open Access

https://doi.org/10.12669/pjms.36.3.1237

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Abstract

Objective:To determine the risk factors, presentation and outcome of meningomyelocele repairMethods:We reviewed 150 cases operated for meningomyelocele (MMC) at Jinnah Postgraduate Medical Centre Karachi between May 2015 and May 2018. Data of infants operated for MMC repair was extracted including socioeconomic status, maternal folate intake during pregnancy, head circumference, location and width of the defect, accompanying bladder and limb anomalies and treatments administered. Patients were followed up for a mean period of six months.Results:A total of 150 children were evaluated, out of which there were 83(55.3%) males and 67(44.7%) females. All belonged to low socio economic group and prenatal maternal folate intake as risk factor was positive in 103(68.7%) cases. Mean head circumference was 37.4 cm (range, 30.7 to 50 cm). Based on their location, 83(55%) of the defects were lumbosacral, 38(25.4%) were lumbar, 16(10.7%) were thoraco lumbar, 10(6.7%) were thoracic and three (2%) were cervical. Mean size of the meningomyelocele sac was 4.3 cm×5.6 cm (range, 1cm×2 cm to 11cm×8.4cm) and 21(14%) of the babies had a skin defect requiring flap. According to accompanying anomalies, 98(65.3%) of the babies had hydrocephalus, 13(9%) had club foot, four (2.7%) had diastematomyelia and three (2%) had tethered cord. Eighty seven (58%) patients had neurological deficit pre operatively and eight (5.4%) patients with normal power deteriorated after surgery out of which five (3.3%) developed paraplegia and three (2%) developed paraparesis. CSF leak was the major complication encountered in 16(11%) followed by meningitis in seven (5%), while the overall mortality was four (2.6%).Conclusion:The practice of periconceptional folic acid supplementation is essential to reduce the prevalence of neural tube defects (NTDs) in the developing world. Improved maternal nutrition with access to quality antenatal care is vital to decrease the prevalence and health burden.

Highlights

A potentially preventable cause of perinatal morbidity and mortality, neural tube defects (NTDs) are one of the most common congenital malformations
Based on whether the rostral or caudal neuropore fails to close, NTDs are classified into cranial dysraphism with resultant anencephaly or spinal dysraphism with or without MMC
In this study we present our institutional experience of risk factors, presentation and outcomes of MMC repair

Summary

Introduction

A potentially preventable cause of perinatal morbidity and mortality, NTDs are one of the most common congenital malformations. Studies in Pakistan estimate incidence between 38.6 and 124.1 per 10,000 births[2,3] The mortality rate in the first 6 months of life is 65%–70% in untreated patients.[4] This congenital anomaly of the central nervous system (CNS) affects the neural tube in the. It is important to close MMC defects in the early postnatal period to decrease mortality rates by providing protection for neural elements and preventing CSF leakage and related central nervous system infections.[6] MMC repair is a relatively uncommon procedure in the Western world secondary to better nutrition and early detection but it still poses a significant health burden in the developing world with devastating outcomes for the affected child and family

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