Otomandibular syndrome

A rare, otomandibular dysplasia characterized by asymmetric, predominantly unilateral hypoplasia of facial structures, typically involving the external ear, mandible, and ipsilateral facial soft tissues. It manifests with mild to severe facial asymmetry, micrognathia, microtia, slanted lip commissure and occlusal canting.

Micrognathia

Developmental hypoplasia of the mandible.


Total: 5

                       


(per page)
PMID (PMCID)
21184723
 FEMALE
[Holt-Oram syndrome associated with facial anomalies. A case report].
Avina-Fierro JA, Colonnelli-Barba G.
Rev Med Inst Mex Seguro Soc. 2010;48(6):657-9.
This is an unusual case of a patient with Holt-Oram syndrome associated with facial anomalies: hemifacial microsomia on the right side, forehead prominent and tall, hypertelorism, depressed nasal bridge, low set ears and micrognathia.
16532152
 FEMALE Infant, Newborn
Monozygotic twins discordant for Goldenhar syndrome.
Verona LL, Damian NG, Pavarina LP, Ferreira CH, Melo DG.
J Pediatr (Rio J). 2006;82(1):75-8.
The affected child's dysmorphic features included left hemifacial microsomia, severe micrognathia, abnormal ears, bilateral preauricular tags and epibulbar dermoid in the right eye.
16573158
 MIXED_SAMPLE Child
[Analysis of the complications following mandibular distraction using internal distractors].
Wu GP, Teng L, Gui L, Sun XM, Zhang ZY, Liu JF, Yu B, Xia DL, Luo JC.
Zhonghua Zheng Xing Wai Ke Za Zhi. 2006;22(1):18-21.
The diseases included hemifacial microsomia in 27 cases, congenital or acquired mandibular hypoplasia and micrognathia bilateral in 8 cases and unilateral in 4 cases, electronical injury or postoperative mandibular defects in 3 cases, Treacher Collins syndrome in 2 cases, obstructive sleep apnea syndrome in 2 cases.
11770457
 MIXED_SAMPLE Child
[Mandibular elongation and remodeling by gradual distraction. An experience of 277 cases].
Molina F.
Ann Chir Plast Esthet. 2001;46(5):507-15.
After ten years, we have performed 221 patients with hemifacial microsomia and 56 patients with micrognathia using external mandibular distraction.
8669439
 MIXED_SAMPLE Infant, Newborn
Three-generation family with resemblance to Townes-Brocks syndrome and Goldenhar/oculoauriculovertebral spectrum.
Johnson JP, Poskanzer LS, Sherman S.
Am J Med Genet. 1996;61(2):134-9.
The family manifests a variety of Goldenhar-like findings, including epibulbar dermoids, hemifacial microsomia, preauricular tags, macrostomia, and micrognathia in addition to classical ear, radial, and anal findings of TBS.