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Fetal Hydantoin Syndrome

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1. Fetal Hydantoin Syndrome

Fetal Hydantoin Syndrome Fetal Hydantoin Syndrome Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Fetal Hydantoin Syndrome Fetal (...) Hydantoin Syndrome Aka: Fetal Hydantoin Syndrome II. Definition Congenital syndrome due to in-utero exposure III. Epidemiology : <10% of exposed fetuses IV. Signs Growth delay and Craniofacial dysmorphic features Flat nasal bridge Epicanthic folds Prominent upper lip over wide mouth Distinguishing characteristics Nail and distal phalanx hypoplasia V. Differential Diagnosis VI. References Gabbe (2002) Obstetrics, Churchill, p. 225 Ellenhorn (1997) Medical Toxicology, Williams, p. 606 Images: Related

2018 FP Notebook

2. Fetal Hydantoin Syndrome

Fetal Hydantoin Syndrome Fetal Hydantoin Syndrome Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Fetal Hydantoin Syndrome Fetal (...) Hydantoin Syndrome Aka: Fetal Hydantoin Syndrome II. Definition Congenital syndrome due to in-utero exposure III. Epidemiology : <10% of exposed fetuses IV. Signs Growth delay and Craniofacial dysmorphic features Flat nasal bridge Epicanthic folds Prominent upper lip over wide mouth Distinguishing characteristics Nail and distal phalanx hypoplasia V. Differential Diagnosis VI. References Gabbe (2002) Obstetrics, Churchill, p. 225 Ellenhorn (1997) Medical Toxicology, Williams, p. 606 Images: Related

2015 FP Notebook

3. Pediatrics—Epitomes of Progress: The Fetal Hydantoin Syndrome (PubMed)

Pediatrics—Epitomes of Progress: The Fetal Hydantoin Syndrome 18748159 2010 06 30 2018 11 13 0093-0415 128 3 1978 Mar The Western journal of medicine West. J. Med. Pediatrics-epitomes of progress: the fetal hydantoin syndrome. 229 Jones K L KL eng Journal Article United States West J Med 0410504 0093-0415 1978 3 1 0 0 1978 3 1 0 1 1978 3 1 0 0 ppublish 18748159 PMC1238058 J Pediatr. 1976 Oct;89(4):662-8 957016 Am J Dis Child. 1974 May;127(5):645-53 4825584

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1978 Western Journal of Medicine

6. Acute pain management: scientific evidence (3rd Edition)

: Scientific Evidence xv CONTENTS 9. SPECIFIC CLINICAL SITUATIONS 237 9.1 Postoperative pain 237 9.1.1 Risks of acute postoperative neuropathic pain 237 9.1.2 Acute postamputation pain syndromes 238 9.1.3 Other postoperative pain syndromes 240 9.1.4 Day-stay or short-stay surgery 242 9.1.5 Cranial neurosurgery 245 9.2 Acute pain following spinal cord injury 247 9.3 Acute burn injury pain 249 9.3.1 Management of procedural pain 250 9.3.2 Non-pharmacological pain management 251 9.4 Acute back pain 252 9.5 (...) 9.7.3 Breakthrough pain 285 9.7.4 Postoperative and procedural pain 286 9.7.5 Acute cancer pain due to bone involvement 287 9.7.6 Other acute cancer pain syndromes 288 9.7.7 Interventional therapies for acute cancer pain 289 9.8 Acute pain management in intensive care 290 9.8.1 Pain assessment in the intensive care unit 291 9.8.2 Non-pharmacological measures 291 9.8.3 Pharmacological treatment 291 9.8.4 Guillain-Barre syndrome 292 9.8.5 Procedure-related pain 293 9.9 Acute pain management

2015 National Health and Medical Research Council

7. Acute Pain Management: Scientific Evidence

factors for the development of postoperative acute persistent pain. Trends Anaesth Critical Care 4: 67–70. Shipton EA (2014b) The transition of acute postoperative pain to chronic pain: Part 2 - Limiting the transition. Trends Anaesth Critical Care 4: 71–75. Vidaeff AC, Saade GR, Belfort MA (in press) Interpreting a randomized trial report: evidence-based practice for the clinician. J Matern Fetal Neonatal Med. Walsh M, Woodhouse LJ, Thomas SG, Finch E (2011) Strategies aimed at preventing chronic (...) pain management 280 8.1.3 Acute rehabilitation after surgery, “fast-track” surgery and enhanced recovery after surgery 281 8.1.4 Risks of acute postoperative neuropathic pain 282 8.1.5 Acute postamputation pain syndromes 283 8.1.6 Other postoperative pain syndromes 285 8.1.7 Day-stay or short-stay surgery 288 8.1.8 Cranial neurosurgery 294 8.1.9 Spinal surgery 297 8.2 Acute pain following spinal cord injury 298 8.2.1 Treatment of acute neuropathic pain after spinal cord injury 299 8.2.2 Treatment

2015 Clinical Practice Guidelines Portal

8. Abdominal Hernias (Diagnosis)

. Aberrant collagen states (eg, Ehlers-Danlos, fetal hydantoin, Freeman-Sheldon, Hunter-Hurler, Kniest, Marfan, and Morquio syndromes), have increased rates of hernia formation, as do osteogenesis imperfecta, pseudo-Hurler polydystrophy, and Scheie syndrome. Acquired elastase deficiency also can lead to increased hernia formation. In 1981, Cannon and Read found that the increased serum elastase and decreased alpha 1 -antitrypsin levels associated with smoking contribute to an increased rate of hernia (...) by the rectus sheath. Proof that umbilical hernias persist from childhood to present in adulthood is only hinted at by an increased incidence among black Americans. Multiparity, increased abdominal pressure, and a single midline decussation are associated with umbilical hernias. Congenital hypothyroidism, fetal hydantoin syndrome, Freeman-Sheldon syndrome, Beckwith-Wiedemann syndrome, and disorders of collagen and polysaccharide metabolism (such as Hunter-Hurler syndrome, osteogenesis imperfecta, and Ehlers

2014 eMedicine Surgery

9. Women's Health and Epilepsy (Overview)

% in women with epilepsy taking AEDs. [ ] Minor malformations such as facial dysmorphisms and digital hypoplasia occur in 6-20% of pregnancies with in utero exposure to AEDs. [ ] These malformations are often subtle and disappear with maturation through the first year of life. Evidence for medication-specific syndromes (eg, fetal hydantoin syndrome, fetal barbiturate syndrome) is lacking; because similar minor anomalies have been reported with in utero exposure to multiple different AEDs, fetal (...) remission of epilepsy. [ ] Although most epilepsy syndromes are equally or more commonly found in males than in females, childhood and the syndrome of photosensitive epilepsy are more common in females. [ ] In addition, some genetic disorders with associated epilepsy (eg, Rett syndrome and Aicardi syndrome) and eclamptic seizures in pregnancy can only occur in females. The age-adjusted incidences for simple and complex partial and generalized tonic-clonic seizures are higher for men than women

2014 eMedicine.com

10. Seizure Disorders in Pregnancy (Follow-up)

of abnormal electroencephalogram (EEG) findings, higher rates of developmentally delayed children, and lower intelligence quotient (IQ) scores. Phenytoin Fetal hydantoin syndrome was first described in 1973 by Loughnan et al. [ ] It consists of an array of anomalies, including craniofacial anomalies, distal digital hypoplasia, epicanthal folds, hypertelorism, low-set ears, and developmental delay. The early descriptions of this syndrome were noted in 12 infants, 11 of whom had been exposed to other AEDs (...) and with normal controls. Phenobarbital and primidone Infants exposed to phenobarbital have been reported to have many of the findings observed in infants with fetal hydantoin syndrome and fetal alcohol syndrome. Furthermore, many of the infants initially described as having fetal hydantoin syndrome were also exposed to phenobarbital. A drug registry reported 5 (6.5%) of 77 patients who had received phenobarbital monotherapy had fetuses with major malformations, compared with a background rate of 1.6

2014 eMedicine.com

11. Women's Health and Epilepsy (Diagnosis)

% in women with epilepsy taking AEDs. [ ] Minor malformations such as facial dysmorphisms and digital hypoplasia occur in 6-20% of pregnancies with in utero exposure to AEDs. [ ] These malformations are often subtle and disappear with maturation through the first year of life. Evidence for medication-specific syndromes (eg, fetal hydantoin syndrome, fetal barbiturate syndrome) is lacking; because similar minor anomalies have been reported with in utero exposure to multiple different AEDs, fetal (...) remission of epilepsy. [ ] Although most epilepsy syndromes are equally or more commonly found in males than in females, childhood and the syndrome of photosensitive epilepsy are more common in females. [ ] In addition, some genetic disorders with associated epilepsy (eg, Rett syndrome and Aicardi syndrome) and eclamptic seizures in pregnancy can only occur in females. The age-adjusted incidences for simple and complex partial and generalized tonic-clonic seizures are higher for men than women

2014 eMedicine.com

12. Psychosocial and Environmental Pregnancy Risks (Treatment)

the role of drug therapy. Antiepileptic drugs with the potential for fetal malformations include the following: Valproic acid: Valproic acid is associated with a 1-2% increased risk of neural tubal defects, particularly at doses above 1000 mg/d. Phenytoin: Phenytoin is known to decrease the absorption of folate and is associated with characteristic fetal hydantoin syndrome, with effects including growth deficiency, microcephaly, dysmorphic facies, and mental deficiency; this is noted in 11% of fetuses (...) an anticonvulsant is complicated by the fact that seizure itself may predispose the fetus to an anomaly. In late pregnancy, placental hypoxia can occur during prolonged seizure. In addition, many common anticonvulsants can contribute to folate deficiency. Heritable epilepsy also may be associated with other genetic abnormalities. The term fetal anticonvulsant syndrome has been used to describe an embryopathy associated with antiepileptic drugs that is variably characterized by major malformations, microcephaly

2014 eMedicine.com

13. Teratology and Drug Use During Pregnancy (Treatment)

spaced nipples, umbilical or inguinal hernia, and rib anomalies. Internal abnormalities include variable coarctation of the aorta, endocardial cushion defect, double-outlet right ventricle, ventricular septal defect, atrial septal defect, bicuspid pulmonic valve, and intestinal malrotation. Ambiguous genitalia are rarely associated with this syndrome. A patient with the dysmorphic characteristics of fetal hydantoin syndrome presented with unusual hyperpigmentation of several fingernails. Another (...) in pregnancy, phenytoin can cause a syndrome of birth defects referred to with various names, such as Dilantin congenital defects, fetal hydantoin syndrome (FHS), Meadow syndrome, congenital hydantoin syndrome, Dilantin syndrome, fetal Dilantin syndrome, fetal phenytoin syndrome, and hydantoin syndrome. Various malformations have been reported to occur because of phenytoin intake during pregnancy. Pregabalin (Lyrica) Old Categorization: Pregnancy category – C Pregnancy risk exposure: Pregnant patients

2014 eMedicine.com

14. Seizure Disorders in Pregnancy (Treatment)

of abnormal electroencephalogram (EEG) findings, higher rates of developmentally delayed children, and lower intelligence quotient (IQ) scores. Phenytoin Fetal hydantoin syndrome was first described in 1973 by Loughnan et al. [ ] It consists of an array of anomalies, including craniofacial anomalies, distal digital hypoplasia, epicanthal folds, hypertelorism, low-set ears, and developmental delay. The early descriptions of this syndrome were noted in 12 infants, 11 of whom had been exposed to other AEDs (...) and with normal controls. Phenobarbital and primidone Infants exposed to phenobarbital have been reported to have many of the findings observed in infants with fetal hydantoin syndrome and fetal alcohol syndrome. Furthermore, many of the infants initially described as having fetal hydantoin syndrome were also exposed to phenobarbital. A drug registry reported 5 (6.5%) of 77 patients who had received phenobarbital monotherapy had fetuses with major malformations, compared with a background rate of 1.6

2014 eMedicine.com

15. Women's Health and Epilepsy (Treatment)

% in women with epilepsy taking AEDs. [ ] Minor malformations such as facial dysmorphisms and digital hypoplasia occur in 6-20% of pregnancies with in utero exposure to AEDs. [ ] These malformations are often subtle and disappear with maturation through the first year of life. Evidence for medication-specific syndromes (eg, fetal hydantoin syndrome, fetal barbiturate syndrome) is lacking; because similar minor anomalies have been reported with in utero exposure to multiple different AEDs, fetal (...) remission of epilepsy. [ ] Although most epilepsy syndromes are equally or more commonly found in males than in females, childhood and the syndrome of photosensitive epilepsy are more common in females. [ ] In addition, some genetic disorders with associated epilepsy (eg, Rett syndrome and Aicardi syndrome) and eclamptic seizures in pregnancy can only occur in females. The age-adjusted incidences for simple and complex partial and generalized tonic-clonic seizures are higher for men than women

2014 eMedicine.com

16. Psychosocial and Environmental Pregnancy Risks (Overview)

the role of drug therapy. Antiepileptic drugs with the potential for fetal malformations include the following: Valproic acid: Valproic acid is associated with a 1-2% increased risk of neural tubal defects, particularly at doses above 1000 mg/d. Phenytoin: Phenytoin is known to decrease the absorption of folate and is associated with characteristic fetal hydantoin syndrome, with effects including growth deficiency, microcephaly, dysmorphic facies, and mental deficiency; this is noted in 11% of fetuses (...) an anticonvulsant is complicated by the fact that seizure itself may predispose the fetus to an anomaly. In late pregnancy, placental hypoxia can occur during prolonged seizure. In addition, many common anticonvulsants can contribute to folate deficiency. Heritable epilepsy also may be associated with other genetic abnormalities. The term fetal anticonvulsant syndrome has been used to describe an embryopathy associated with antiepileptic drugs that is variably characterized by major malformations, microcephaly

2014 eMedicine.com

17. Tetralogy of Fallot (Overview)

with fetal hydantoin syndrome or fetal carbamazepine syndrome. In addition, as one of the conotruncal malformations, tetralogy of Fallot can be associated with a spectrum of lesions known as CATCH 22 (cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia). Cytogenetic analysis may demonstrate deletions of a segment of chromosome band 22q11 (DiGeorge critical region). Ablation of cells of the neural crest has been shown to reproduce conotruncal malformations. These abnormalities (...) be associated with an increased risk for tetralogy of Fallot, whereas the risk is potentially reduced with 936C>T polymorphism. [ ] Prenatal factors associated with a higher incidence of tetralogy of Fallot include maternal rubella (or other viral illnesses) during pregnancy, poor prenatal nutrition, maternal alcohol use, maternal age older than 40 years, maternal phenylketonuria (PKU) birth defects, and diabetes. Children with Down syndrome also have a higher incidence of tetralogy of Fallot, as do infants

2014 eMedicine.com

18. Teratology and Drug Use During Pregnancy (Overview)

spaced nipples, umbilical or inguinal hernia, and rib anomalies. Internal abnormalities include variable coarctation of the aorta, endocardial cushion defect, double-outlet right ventricle, ventricular septal defect, atrial septal defect, bicuspid pulmonic valve, and intestinal malrotation. Ambiguous genitalia are rarely associated with this syndrome. A patient with the dysmorphic characteristics of fetal hydantoin syndrome presented with unusual hyperpigmentation of several fingernails. Another (...) in pregnancy, phenytoin can cause a syndrome of birth defects referred to with various names, such as Dilantin congenital defects, fetal hydantoin syndrome (FHS), Meadow syndrome, congenital hydantoin syndrome, Dilantin syndrome, fetal Dilantin syndrome, fetal phenytoin syndrome, and hydantoin syndrome. Various malformations have been reported to occur because of phenytoin intake during pregnancy. Pregabalin (Lyrica) Old Categorization: Pregnancy category – C Pregnancy risk exposure: Pregnant patients

2014 eMedicine.com

19. Seizure Disorders in Pregnancy (Overview)

of abnormal electroencephalogram (EEG) findings, higher rates of developmentally delayed children, and lower intelligence quotient (IQ) scores. Phenytoin Fetal hydantoin syndrome was first described in 1973 by Loughnan et al. [ ] It consists of an array of anomalies, including craniofacial anomalies, distal digital hypoplasia, epicanthal folds, hypertelorism, low-set ears, and developmental delay. The early descriptions of this syndrome were noted in 12 infants, 11 of whom had been exposed to other AEDs (...) and with normal controls. Phenobarbital and primidone Infants exposed to phenobarbital have been reported to have many of the findings observed in infants with fetal hydantoin syndrome and fetal alcohol syndrome. Furthermore, many of the infants initially described as having fetal hydantoin syndrome were also exposed to phenobarbital. A drug registry reported 5 (6.5%) of 77 patients who had received phenobarbital monotherapy had fetuses with major malformations, compared with a background rate of 1.6

2014 eMedicine.com

20. Psychosocial and Environmental Pregnancy Risks (Follow-up)

the role of drug therapy. Antiepileptic drugs with the potential for fetal malformations include the following: Valproic acid: Valproic acid is associated with a 1-2% increased risk of neural tubal defects, particularly at doses above 1000 mg/d. Phenytoin: Phenytoin is known to decrease the absorption of folate and is associated with characteristic fetal hydantoin syndrome, with effects including growth deficiency, microcephaly, dysmorphic facies, and mental deficiency; this is noted in 11% of fetuses (...) an anticonvulsant is complicated by the fact that seizure itself may predispose the fetus to an anomaly. In late pregnancy, placental hypoxia can occur during prolonged seizure. In addition, many common anticonvulsants can contribute to folate deficiency. Heritable epilepsy also may be associated with other genetic abnormalities. The term fetal anticonvulsant syndrome has been used to describe an embryopathy associated with antiepileptic drugs that is variably characterized by major malformations, microcephaly

2014 eMedicine.com

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