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Fetal hydantoin syndrome(FHS)

MedGen UID:
75569
Concept ID:
C0265372
Disease or Syndrome
Synonyms: Dilantin Embryopathy; Phenytoin Embryopathy
SNOMED CT: Fetal hydantoin syndrome (70065001); Meadow's syndrome (70065001); Foetal hydantoin syndrome (70065001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016008
OMIM®: 617955
Orphanet: ORPHA1912

Definition

A drug-related embryofetopathy that can occur when an embryo/fetus is exposed to the anticonvulsant drug phenytoin, characterized by distinct craniofacial anomalies (hypertelorism and epicanthal folds, short nose and deep nasal bridge, malformed and low set ears, short neck) as well as hypoplastic distal phalanges and underdevelopment of nails of fingers and toes, prenatal and postnatal growth retardation, and neurological impairment (at a 2-3 times higher risk than that of the general population) including cognitive deficits and motor developmental delay. Less commonly, microcephaly, ocular defects, oral clefts, umbilical and inguinal hernias, hypospadias and cardiac anomalies have also been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFetal hydantoin syndrome
Follow this link to review classifications for Fetal hydantoin syndrome in Orphanet.

Recent clinical studies

Etiology

Oguni M, Osawa M
Epilepsia 2004;45 Suppl 8:37-41. doi: 10.1111/j.0013-9580.2004.458008.x. PMID: 15610193
Graeter LJ, Mortensen ME
Toxicology 1996 Jul 17;111(1-3):15-20. doi: 10.1016/0300-483x(96)03389-6. PMID: 8711731
Buehler BA, Rao V, Finnell RH
Neurol Clin 1994 Nov;12(4):741-8. PMID: 7845340
Jones KL, Lacro RV, Johnson KA, Adams J
N Engl J Med 1989 Jun 22;320(25):1661-6. doi: 10.1056/NEJM198906223202505. PMID: 2725616
Koren G, Demitrakoudis D, Weksberg R, Rieder M, Shear NH, Sonely M, Shandling B, Spielberg SP
Teratology 1989 Aug;40(2):157-62. doi: 10.1002/tera.1420400209. PMID: 2672404

Diagnosis

Hegde A, Kaur A, Sood A, Dhanorkar M, Varma HT, Singh G, Saini AG, Kumar P
J Pediatr 2017 Sep;188:304. Epub 2017 May 31 doi: 10.1016/j.jpeds.2017.05.018. PMID: 28578158
Singh A, Bhatia HP, Mohan A, Sharma N
J Indian Soc Pedod Prev Dent 2016 Jan-Mar;34(1):92-5. doi: 10.4103/0970-4388.175526. PMID: 26838156
Koren G, Demitrakoudis D, Weksberg R, Rieder M, Shear NH, Sonely M, Shandling B, Spielberg SP
Teratology 1989 Aug;40(2):157-62. doi: 10.1002/tera.1420400209. PMID: 2672404
Johnson RB, Goldsmith LA
J Am Acad Dermatol 1981 Aug;5(2):191-6. doi: 10.1016/s0190-9622(81)70087-2. PMID: 7263964
Hanson JW, Smith DW
J Pediatr 1975 Aug;87(2):285-90. doi: 10.1016/s0022-3476(75)80604-4. PMID: 50428

Therapy

Hegde A, Kaur A, Sood A, Dhanorkar M, Varma HT, Singh G, Saini AG, Kumar P
J Pediatr 2017 Sep;188:304. Epub 2017 May 31 doi: 10.1016/j.jpeds.2017.05.018. PMID: 28578158
Scheinfeld N
Dermatol Online J 2003 Aug;9(3):6. PMID: 12952753
Buehler BA, Rao V, Finnell RH
Neurol Clin 1994 Nov;12(4):741-8. PMID: 7845340
Adams J, Vorhees CV, Middaugh LD
Neurotoxicol Teratol 1990 May-Jun;12(3):203-14. doi: 10.1016/0892-0362(90)90092-q. PMID: 2196420
Kogutt MS
South Med J 1984 May;77(5):657-8. doi: 10.1097/00007611-198405000-00031. PMID: 6719170

Prognosis

Buehler BA, Rao V, Finnell RH
Neurol Clin 1994 Nov;12(4):741-8. PMID: 7845340
Finnell RH, Buehler BA, Kerr BM, Ager PL, Levy RH
Neurology 1992 Apr;42(4 Suppl 5):25-31. PMID: 1574173
Buehler BA, Delimont D, van Waes M, Finnell RH
N Engl J Med 1990 May 31;322(22):1567-72. doi: 10.1056/NEJM199005313222204. PMID: 2336087

Clinical prediction guides

Buehler BA, Rao V, Finnell RH
Neurol Clin 1994 Nov;12(4):741-8. PMID: 7845340
Buehler BA, Delimont D, van Waes M, Finnell RH
N Engl J Med 1990 May 31;322(22):1567-72. doi: 10.1056/NEJM199005313222204. PMID: 2336087
Gaily E, Granström ML, Hiilesmaa V, Bardy A
J Pediatr 1988 Apr;112(4):520-9. doi: 10.1016/s0022-3476(88)80162-8. PMID: 3351676
Nau H, Rating D, Koch S, Häuser I, Helge H
J Pharmacol Exp Ther 1981 Dec;219(3):768-77. PMID: 6795343
Seeler RA, Israel JN, Royal JE, Kaye CI, Rao S, Abulaban M
Pediatrics 1979 Apr;63(4):524-7. PMID: 440860

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