Table of Contents  
LETTER TO THE EDITOR
Year : 2015  |  Volume : 8  |  Issue : 5  |  Page : 688-690  

Neurological image of the child with neural tube defect (meningomyelocele) with Arnold-Chiari malformation type 1 with syringomyelia with obstructive hydrocephalus: Observation of folic acid supplementation failure in eliminating the risk of NTD in neonate of the mother taking anti-epileptic medicine


1 Department of Neonatology, Sunrise Hospital, Hyderabad, Andhra Pradesh, India
2 Department of Neonatology, Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh, India
3 Division of Neurology, Ottawa Civic Hospital, 1053 Carling Avenue, Ottawa, ON K1Y 4E9, Canada

Date of Web Publication10-Sep-2015

Correspondence Address:
Deepak Sharma
Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh - 500 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.164972

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How to cite this article:
Parakh H, Kapoor MS, Sharma D, Deshpande A. Neurological image of the child with neural tube defect (meningomyelocele) with Arnold-Chiari malformation type 1 with syringomyelia with obstructive hydrocephalus: Observation of folic acid supplementation failure in eliminating the risk of NTD in neonate of the mother taking anti-epileptic medicine. Med J DY Patil Univ 2015;8:688-90

How to cite this URL:
Parakh H, Kapoor MS, Sharma D, Deshpande A. Neurological image of the child with neural tube defect (meningomyelocele) with Arnold-Chiari malformation type 1 with syringomyelia with obstructive hydrocephalus: Observation of folic acid supplementation failure in eliminating the risk of NTD in neonate of the mother taking anti-epileptic medicine. Med J DY Patil Univ [serial online] 2015 [cited 2024 Mar 28];8:688-90. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2015/8/5/688/164972

Sir,

Folic acid (Vitamin B 9 ) is a water-soluble B Vitamin that is vital for DNA repair, cell division, and normal cellular growth. Folic acid deficiency during pregnancy has been associated with various neural tube defects (NTDs), such as spina bifida, meningocele or meningomyelocele in neonates. Certain antiepileptic drugs can dramatically decrease folate levels, either via hepatic enzyme-induction and/or decreased absorption. The usual recommendation is to supplement 0.4-0.8 mg of folic acid per day in all women planning a pregnancy. Ideally, this should be started at least 1-month prior to pregnancy if possible. These guidelines recommend higher daily folic acid doses (4 mg/day) in women with a history of NTDs. In addition, enzyme-inducing anticonvulsants, such as phenytoin, carbamazepine, primidone, and phenobarbital, are known to decrease folate levels, and valproic acid (VPA) may interfere with folate metabolism. We would like to bring in attention an interesting case in which baby had NTDs in-spite of mother getting adequate folic acid supplementation.

We report a term male baby with birth weight of 3.2 kg was born to 26-year-old Gravida 4 mother. Baby had normal Apgar score of 8/9/9. At birth, baby was diagnosed to have meningomyelocele with dermal sinus in the lumbar region [Figure 1]. There was no lower limb weakness and with intact bladder and bowel continence. Mother of the patient had a previous pregnancy with the child having cervical myelomeningocele with hydrocephalous. Mother had history of epilepsy for which she was on valproate 600 mg total for the day since her teenage. Mother was started on folic acid supplementation 5 mg twice daily prior 3 months of conception. Magnetic resonance imaging of the baby was done which showed Inferior herniation of cerebellar tonsils into upper cervical canal with medullary kink and suggestion of syrinx in lower cervical spinal cord consistent with  Arnold- Chiari malformation More Details More Details type 1 with marked dilatation of atria, occipital horn of lateral ventricles and myelomeningocele of lumber region [Figure 2], [Figure 3], [Figure 4]. There were no other facial or internal malformations. Echocardiography (ECHO) done was normal.
Figure 1: Swelling in the lower back of the patient, 10 cm × 10 cm and reddish in colour, with violeceous colour irregular border suggestive of myelomeningocele

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Figure 2: Depicts the T2-weighted sagittal magnetic resonance imaging of whole spine suggestive of hyperintense bulge arising from the sacral vertebral level suggestive of myelomeningocele

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Figure 3: Suggestive of axial T2-weighted magnetic resonance imaging brain suggestive of dilated ventricular system (lateral, third ventricle and normal fourth ventricle)

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Figure 4: Suggestive of T2-weighted sagittal magnetic resonance imaging brain and spine suggestive of Arnold Chiari type 1 malformation and intramedullary hyperintensity (syrinx) to about mid-thoracic level

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Mother on anticonvulsant have an increased incidence of major and minor congenital abnormalities in newborn (6-7% compared with 2-3% in the general population).[1] VPA is a broad spectrum anticonvulsant with drug of choice of almost all type of seizures. Fetal valproate syndrome occurs as results from prenatal exposure to VPA and is characterized by a distinctive facial appearance, a cluster of minor and major anomalies and central nervous system dysfunction. Major malformations include NTD, congenital heart disease, oral clefts, genital abnormalities and limb defects.[2] Folic acid deficiency is considered as a possible mechanism of teratogenicity for phenytoin, carbamazepine, phenobarbital, and VPA.[3] American Congress of Obstetricians and Gynecology and the Canadian Society of Medical Geneticists recommend that all women of childbearing age taking AEDs receive folic acid supplementation at 0.4-5.0 mg/day for prevention of NTD.[4],[5] Less than 1000 mg/day is not known to cause NTD and can be easily given.[4] In our case, mother was taking only 600 mg of valproate daily with supplementation of folic acid 10 mg/day that is double the recommended doses. This case image tells about observation of the folic acid failure in prevention of the NTD in few cases.


  Learning Points/Take Home Message Top


  • There can still be the folic acid failure in prevention of NTD in fetus/newborn, in mothers who are taking anticonvulsant drugs, although this rarely happens.
  • Pregnant mothers must be explained about the risk of malformations with intake of anticonvulsant, and the risk-benefit ratio must be seen.
  • There is a need to find very safe antiepileptic drug in the near future because all the present drugs are teratogenic.






 
  References Top

1.
Dieterich E, Steveling A, Lukas A, Seyfeddinipur N, Spranger J. Congenital anomalies in children of epileptic mothers and fathers. Neuropediatrics 1980;11:274-83.  Back to cited text no. 1
    
2.
Clayton-Smith J, Donnai D. Fetal valproate syndrome. J Med Genet 1995;32:724-7.  Back to cited text no. 2
    
3.
Tomson T, Lindbom U, Sundqvist A, Berg A. Red cell folate levels in pregnant epileptic women. Eur J Clin Pharmacol 1995;48:305-8.  Back to cited text no. 3
    
4.
American College of Obstetric and Gynecologic Physicians Educational Bulletin. Seizure disorders in pregnancy. Am Coll Obstet Gynecol Physicians Educ Bull 1996;231:1-13.  Back to cited text no. 4
    
5.
Van Allen MI, Fraser FC, Dallaire L, Allanson J, McLeod DR, Andermann E, et al. Recommendations on the use of folic acid supplementation to prevent the recurrence of neural tube defects. Clinical Teratology Committee, Canadian College of Medical Geneticists. CMAJ 1993;149: 1239-43.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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