To Document The Clinical Presentation and Outcome of Open Neural Tube Defect at Teaching Hospital, Dera Ghazi Khan
Objectives: To study clinical presentation and to determine outcome of open Neural Tube Defects at our
respected institution. We took a review of all cases of open neural tube defects seen at the Neurosurgery Unit of hospital in the last years to document their medical patterns, assess their neonatal outcome.
Materials and Methods: This retrospective study included 74 patients, admitted to the Department of
Neurosurgery, D. G. Khan Medical College and Hospital, Dera Ghazi Khan. The procedure for history taking
and clinical examination was completed to measure Folic Acid Intake in first trimester, ultra sound abdomen,
pre-natal diagnosis, hydrocephalous, CSF leak from swelling, sphincter involvement, weakness of lower limb,
previous baby affected, type & location of open neural tube defects along the cranio-vertebral axis. Spine X-rays,neurosonograms, MRIs were carried out to find associated irregularities and complications if exist.
Results: Out of 74 children born with open neural tube defects, 9 (12%) mothers received antenatal care only, 7
(9%) get folic acid regularly. Prenatal diagnosis was made in 11 (14.8%) mothers whose antenatal abdominal
ultrasound was done. The most common type of myelomeningocele was lumbosacral 58 (79%). Forty-four
(59.4%) babies have hydrocephalous while five babies were microcephaly. Increased risk of ruptured
myelomeningocele was linked with vaginal delivery. Sphincter disturbance and limb paralysis was present in 35
(47%) and 32 (43.7%) of babies respectively. The mortality was in 12% patients.
Conclusion: We suggest that efforts should be made in this respect to prenatal diagnosis of such lesions to get
better neonatal outcome
2. Elgamal EA. Natural history of hydrocephalus in children with spinal open neural tube defect. Surg Neurol Int 2012; 3: 112.
3. Cama A, Tortori-Donati P, Piatelli GL, Fondelli MP, Andreussi L. Chiari complex in children--neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatric Surg 1995; 5 Suppl 1: 35-38.
4. Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology 2000; 42: 471-491.
5. Copp AJ, Stanier P, Greene ND. Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol 2013; 12: 799-810.
6. Hamamy H. Epidemiological profile of neural tube defects in Arab countries. Middle East Journal of Medical Genetics 2014; 3: 1-10.
7. Bassuk AG, Kibar Z. Genetic basis of neural tube defects. Semin Pediatr Neurol 2009; 16: 101-110.
8. Copp AJ, Greene ND. Genetics and development of neural tube defects. J Pathol 2010; 220: 217-230.
9. Aguilera S, Soothill P, Denbow M, Pople I. Prognosis of spina bifida in the era of prenatal diagnosis and outcome.
10. Coran Pediatric Surgery, 7th Edition, Chapter 128, Management of neural tube defects, hydrocephalus, Pages1674-1676.
11. Bruce A. Kaufman, Neural tube defects, Pediatric Clinics of North America, 2004; 51:389 398.
12. Vademecum Pediatric Neurosurgery, Chapter 8, Spinal Dysraphism, Pages 143-149.
13. Mark S. Dias, MD; Michael Partington, MD, Embryology of Myelomeningocele and Anencephaly, Neurosurgical Focus, 2004; 16(2).
14. Toriello HV, Higgins JV. Occurrence of neural tube defects among first-, second-, and third-degree relatives of probands: results of a United States study. Am J Medical Genetics, 1983;15:601– 6.
15. P. G. SALUJA, The incidence of spina bifida occulta in a historic and a modern London population, J.Anat. ;1988; 158; 91-93,
16. Kumar R. • Singh S.N.; Spinal Dysraphism: Trends in Northern India; J. Pediatric Neurosurgery; 2003; 38:133–145.
17. Mohamed S, Ibrahim F, Kamil K, Satti SA. Meckel-Gruber syndrome: Antenatal diagnosis and ethical perspectives. Sudan J Paediatr 2012; 12: 70-72.
18. Textbook of contemporary neurosurgery, Chapter 25, Spinal and Cranial Dysraphism, Pages 338-340.
19. Hibbard BM. The role of folic acid in pregnancy. J Obstet Gynaecol Brit Commwlth 1964; 71: 529-542.
20. Smithells RW, Shephard S, Schorah CJ et al. Possible prevention of neural-tube defects by peri conceptional vitamin supplementation. Lancet 1980; i: 339 -340.Sutcliffe M, Schorah CJ, Perry A et al. Prevention of neural tube defects Lancet 1993; 342: 1174.
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