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Oculo Facio Cardio Dental Syndrome

Patients with OFCD present with congenital ocular defects (unilateral/bilateral microphthalmia with congenital cataracts or just with congenital cataracts alone), facial anomalies (narrow face with a broad nasal tip, separated nasal cartilage, cleft palate), congenital heart defects (septal defects), and skeletal anomalies. A unique and cardinal diagnostic feature is dental root radiculomegaly and other dental abnormalities. [...]

+ Mutation-specific testing   + Prenatal testing

Patients with OFCD present with congenital ocular defects (unilateral/bilateral microphthalmia with congenital cataracts or just with congenital cataracts alone), facial anomalies (narrow face with a broad nasal tip, separated nasal cartilage, cleft palate), congenital heart defects (septal defects), and skeletal anomalies. A unique and cardinal diagnostic feature is dental root radiculomegaly and other dental abnormalities. Mild mental retardation and conductive or sensorineural hearing loss are less common.

+ Mutation-specific testing   + Prenatal testing

Tests Available

BCOR Del/Dup

FORMS AND DOCUMENTS

TEST DETAILS

Genes:
BCOR
Clinical Utility:
  • Confirmation of a clinical diagnosis
  • Mutation-specific testing of family members of an affected individual with known mutation(s)
  • Prenatal diagnosis in at-risk pregnancies
Lab Method:
Exon Array CGH

ORDERING

Test Code:
906
Turnaround Time:
3-4 weeks
Preferred Specimen:
2-5 mL Blood - Lavender Top Tube

BILLING

CPT Codes:
84311x1, 88271x10, 88291x1
Billing Information:
View Billing Policy
ICD Codes:
  • 743: Anophthalmos
  • 759.7: Multiple congenital anomalies, so described Congenital: anomaly, multiple NOS deformity, multiple NOS
  • 743.1: Microphthalmos, Dysplasia of eye, Hypoplasia of eye, Rudimentary eye
  • 743.4: Coloboma and other anomalies of anterior segment

REFERENCES

  1. Martinez-Garay et al (2007) Eur J Hum Genet 15:29-34.
  2. Ng et al (2004) Nat Genet 36;4:411-416.
  3. Horn et al (2005) Eur J Hum Genet 13: 563-569.
  4. Hilton et al (2009) Eur J Hum Genet 1-11.

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