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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2016  |  Volume : 34  |  Issue : 1  |  Page : 92-95

Fetal hydantoin syndrome: A case report

1 Department of Pedodontics and Preventive Dentistry, Manav Rachna Dental College, Faridabad, Haryana, India
2 Department of Oral and Maxillofacial Surgery, Manav Rachna Dental College, Faridabad, Haryana, India

Date of Web Publication2-Feb-2016

Correspondence Address:
A Singh
Department of Pedodontics and Preventive Dentistry, Manav Rachna Dental College, Manav Rachna Educational Institutions Aravalli Campus Sector - 43, Delhi Suraj kund Road, Faridabad - 121 004, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-4388.175526

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Fetal hydantoin syndrome (FHS) is a spectrum of defects caused to the developing fetus by exposure to the teratogenic effects of antiepileptic drug (AED) phenytoin during pregnancy. Its clinical manifestations include limb abnormalities, ocular defects, central nervous system anomalies, intrauterine growth restriction, and hand and phalangeal anomalies. This case report presents an 8-year-old child born to an epileptic mother with a history of AED therapy, with features suggestive of FHS.

Keywords: Anticonvulsants, crossbite, phenytoin, space maintainer

How to cite this article:
Singh A, Bhatia H P, Mohan A, Sharma N. Fetal hydantoin syndrome: A case report. J Indian Soc Pedod Prev Dent 2016;34:92-5

How to cite this URL:
Singh A, Bhatia H P, Mohan A, Sharma N. Fetal hydantoin syndrome: A case report. J Indian Soc Pedod Prev Dent [serial online] 2016 [cited 2022 Dec 2];34:92-5. Available from: http://www.jisppd.com/text.asp?2016/34/1/92/175526

   Introduction Top

Epilepsy is defined by International League Against Epilepsy (ILAE; 1993) as a condition characterized by recurrent epileptic seizures, unprovoked by any immediate identified cause. [1] It has a marked influence on patients' lifestyle. According to the World Health Organization (WHO), of the 50 million people with epilepsy worldwide, 80% reside in developing countries. [2] Its prevalence is about 1% in Indian population. [3]

Seizures form a major neurologic problem in pregnancy, and control is important to avoid complications for the mother and the potential harmful effects of maternal hypoxia on the developing fetus. Sometimes it becomes mandatory to take anticonvulsants on regular basis during pregnancy. Fetal hydantoin syndrome (FHS) is a fetopathy likely to occur when a pregnant women takes hydantoin for epileptic seizures. Hanson and Smith in 1975 coined the term FHS. [4] Its classical features include growth and developmental delay, craniofacial anomalies, varying limb abnormalities, and nail and digital hypoplasia.

The case described here is of an 8-year-old girl with features suggestive of FHS and a positive history of epilepsy therapy in her mother.

   Case Report Top

An 8-year-old girl reported with a complain of decayed teeth and pain on chewing food in the lower right back mouth region.

The child weighed 18 kg and was 48" in height. She had seven elder siblings and was eighth in order of birth. Family history revealed that the patient's mother was epileptic and was on antiepileptic drugs (AEDs; sodium hydantoin and phenobarbitone, 300 mg daily) since 12 years. She was on this medication during her seventh and eighth pregnancy for seizures. Other siblings were reportedly normal. There was no parental consanguinity.

Features suggestive of FHS were ocular defects which included watery eyes with intermittent tears, mild ptosis of right upper eyelid, strabismus and pseudohypertelorism, midface deficiency indicated by broad and flat nasal bridge, long and indistinct philtrum, limping gait, low set hairline, mild growth deficiency, and soft tissue abnormality on palate [Figure 1], [Figure 2], [Figure 3] and [Figure 4].
Figure 1: Front profi le

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Figure 2: Lateral profi le

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Figure 3: Flat nasal bridge, ptosis of right upper eyelid, strabismus, and watery eyes

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Figure 4: Limping walk

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Intraoral hard tissue examination revealed broad alveolar ridges, high arch palate, and an indistinct submucosal palatal cleft [Figure 5]. Developing crossbite was seen in relation to 11 and 21, with 21 rotated mesially [Figure 6] and [Figure 7]; and 85 was grossly decayed with sinus opening on the attached gingiva.

The body mass index of the child was 12, which was well below the normal range. Also, the parents reported that she was slow to understand and comprehend, suggestive of mental deficiency. Patient did not respond well to the commands during the treatment too.
Figure 5: High arch palate

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Figure 6: Right side in occlusion

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Figure 7: Left side in occlusion

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Radiographic findings revealed periapical-periodontal abscess in relation to 85 [Figure 8]. Extraction of 85 was done under local anesthesia. Band and loop space maintainer was delivered in the same region 1 week later [Figure 9]. Double modified Z-spring appliance was prepared and the patient was asked to wear the appliance. Z-springs were modified because of lack of space for proper placement of loops [Figure 10]. Oral hygiene and appliance care instructions were given to the patient and her family. Patient was recalled every 15 days and the springs were reactivated. The crossbite was corrected in 1.5 months [Figure 11] and [Figure 12].
Figure 8: Radiograph of 85

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Figure 9: Band and loop space maintainer (85 region)

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Figure 10: Double modifi ed Z-spring appliance

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Figure 11: Postoperative view, corrected developing crossbite

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Figure 12: Postoperative view of maxillary arch

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   Discussion Top

Hydantoin has been used since 1938 for the treatment of epilepsy. Phenytoin is a known teratogen and the Food and Drug Administration (FDA) has labeled phenytoin as a category D medication because of the risk of harm to the fetus. [5] Approximately 22% of women giving birth are epileptic and phenytoin is prescribed in 5-20% of patients. The risk that an infant exposed to hydantoin in utero will have the clinical phenotype of the full-blown FHS is approximately 5-10%. Further, the likelihood of an infant's expressing some effects of the syndrome is 33%. [6]

Classical indicators of FHS are presented in [Table 1]. However, these symptoms may vary in individual patient. [6] Since the patient's family was not willing for extensive investigations, it was assumed from the limping gait that there could be a skeletal deformity in the hip joint. Also, the bilateral soft tissue swellings present on the palate suggested some sort of deformity. Failure to thrive, learning disability, and reduction of intellectual ability in infants with FHS are the area of greatest concern. Women being treated with hydantoin anticonvulsants should be informed of the nature and the gamut of risk to the developing fetus before considering a pregnancy. Drug used in girls and young women should be chosen with respect to the future reproduction, because the use of AEDs in women with epilepsy is a balance between seizure control and adverse effects of drugs. If at all the use of medicine becomes mandatory during pregnancy, than monotherapy should be preferred over polytherapy.
Table 1: Clinical of fetal syndrome (Hanson, 1986)[6]

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Being healthcare professionals and using our contact with parents and other community members, we should promote the facts and risk factors associated with the use of AEDs and ways to prevent or avoid such conditions. Efforts are needed to reduce the stigma associated with epilepsy and to tactfully address the condition.

   Conclusion Top

FHS is a rare disorder and presents variable clinical picture, depending on the extent of damage caused to the fetus by the drug. It is a fetopathy caused by exposure of the fetus to AEDs, especially phenytoin. Tactful use of medications, proper counseling of expecting the parents, and mass awareness regarding the side effects of AEDs can help in diminution of the condition.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

   References Top

Hauser WA, Kurland LT. The epidemiology of epilepsy in Rochester, Minnesota, 1935 through 1967. Epilepsia 1975;16:1-66.  Back to cited text no. 1
World Health Organization. Neurological disorders. Public Health Challenges. Geneva: World Health Organization; 2006.  Back to cited text no. 2
Sridharan R, Murthy BN. Prevalence and pattern of epilepsy in India. Epilepsia 1999;40:631-6.  Back to cited text no. 3
Hanson JW, Smith DW. The fetal hydantoin syndrome. J Pediatr 1975;87:285-90.  Back to cited text no. 4
Singh R, Kumar N, Arora S, Bhandari R, Jain A. Fetal hydantoin syndrome and its anaesthetic implications: A case report. Case Rep Anesthesiol 2012;2012:370412.  Back to cited text no. 5
Hanson JW. Teratogen update: Fetal hydantoin effects. Teratology 1986;33:349-53.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]

  [Table 1]


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