Successful Management of a Rare Case of Double Myelomeningocele in an Infant: A Case Report from Pakistan and Brief Review of Pathophysiology

Abstract

Neural tube defects (NTDs) are common congenital anomalies, with myelomeningocele (MMC) being the most severe form. Double-level MMC is exceedingly rare, with fewer than 60 cases reported worldwide and none from Southeast Asia. We report a 4-month-old male with two congenital swellings on his back, diagnosed as double MMC at the cervical and lumbar levels, along with hydrocephalus and Arnold–Chiari malformation. Surgical management, including ventriculoperitoneal shunting and MMC repair, resulted in good outcomes. This case highlights the importance of early diagnosis, timely surgical intervention, and prenatal folic acid supplementation to reduce NTD risk in resource-limited settings.

Keywords

double myelomeningocele - myelomeningocele - hydrocephalus - ventriculoperitoneal shunt - neural tube defect

• References

• 1 Idris B. Factors affecting the outcomes in children post-myelomeningocoele repair in northeastern peninsular Malaysia. Malays J Med Sci 2011; 18 (01) 52-59
• 2 Hadzagić-Catibusić F, Maksić H, Uzicanin S. et al. Congenital malformations of the central nervous system: clinical approach. Bosn J Basic Med Sci 2008; 8 (04) 356-360
• 3 Doherty D, Walker WO. Neural tube defects. In: Aminoff MJ, Daroff RB. eds. Encyclopedia of the Neurological Sciences. 2nd ed. . Oxford: Academic Press; 2014: 360-365
• 4 Shao B, Chen JS, Kozel OA. et al. Postnatal myelomeningocele repair in the United States: rates and disparities before and after the management of myelomeningocele study trial. Neurosurgery 2023; 93 (06) 1374-1382
• 5 Belfatmi N, Motawei AS, Rezkallah A. et al. Double lumbar localization of myelomeningocele: case report. Pediatr Neurosurg 2023; 58 (02) 97-104
• 6 Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers 2015; 1: 15007
• 7 Liptak GS, Dosa NP. Myelomeningocele. Pediatr Rev 2010; 31 (11) 443-450
• 8 Moeini Naghani I, Hashemi Zonouz T, Shahjouei S, Homayoun AA, Nejat F, El Khashab M. Congenital cardiac anomalies in myelomeningocele patients. Acta Med Acad 2014; 43 (02) 160-164
• 9 Yamashiro KJ, Galganski LA, Hirose S. Fetal myelomeningocele repair. Semin Pediatr Surg 2019; 28 (04) 150823
• 10 Deora H, Srinivas D, Shukla D. et al. Multiple-site neural tube defects: embryogenesis with complete review of existing literature. Neurosurg Focus 2019; 47 (04) E18
• 11 Sinha P, Kumar A, Jana M, Kandasamy D. Multiple neural tube defects: rare developmental anomaly with an elusive embryological explanation. BJR Case Rep 2023; 9 (06) 20230005
• 12 Van Allen MI, Kalousek DK, Chernoff GF. et al. Evidence for multi-site closure of the neural tube in humans. Am J Med Genet 1993; 47 (05) 723-743
• 13 Storrs BB. BB. Are dermoid and epidermoid tumors preventable complications of myelomeningocele repair?. Pediatr Neurosurg 1994; 20 (02) 160-162
• 14 Yasmin S, Siddiqa A, Rockliffe L, Miyan J. Knowledge of neural tube defects and prevention through folic acid use among women in Faisalabad, Punjab, Pakistan: a cross-sectional survey. Int J Womens Health 2022; 14: 425-434
• 15 Oncel MY, Ozdemir R, Kahilogulları G, Yurttutan S, Erdeve O, Dilmen U. The effect of surgery time on prognosis in newborns with meningomyelocele. J Korean Neurosurg Soc 2012; 51 (06) 359-362
• 16 Rogers LM, Cordero AM, Pfeiffer CM. et al. Global folate status in women of reproductive age: a systematic review with emphasis on methodological issues. Ann N Y Acad Sci 2018; 1431 (01) 35-57
• 17 Douglas Wilson R, Van Mieghem T, Langlois S, Church P. Guideline No. 410: prevention, screening, diagnosis, and pregnancy management for fetal neural tube defects. J Obstet Gynaecol Can 2021; 43 (01) 124-139.e8