Humpath.com - Human pathology - Photos - pictures - videos

mandibulofacial dysostosis

Mandibulofacial dysostosis or bilateral hemifacial microsomia

Association

- hearing loss
- telecanthus
- tetramelic postaxial hexadactyly
- congenital hypotonia and lymphedema with joint hypermobility
- pigmentary dysplasia

Variants

- duodenal atresia with biliary atresia associated with facial, thyroid and auditory apparatus abnormalities (#16957470#)

References

- Maegawa GH, Chitayat D, Blaser S, Whyte H, Thomas M, Kim P, Kim J, Taylor G, McNamara PJ. Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities: a new mandibulofacial dysostosis syndrome? Clin Dysmorphol. 2006 Oct;15(4):191-6. PMID: #16957470#

- Opitz JM, Reynolds JF, FitzGerald JM. Mandibulofacial dysostosis or bilateral hemifacial microsomia with hearing loss, telecanthus, tetramelic postaxial hexadactyly, congenital hypotonia and lymphedema with joint hypermobility, and pigmentary dysplasia: a new syndrome? Am J Med Genet. 1989 Aug;33(4):433-5. PMID: #2556918#