- Nager syndrome is assoiciated with facial findings very much like Franceschetti-Zwahlen-Klein syndrome patients, but also involves hypoplastic or absent thumbs and radioulnar synostosis. Some cases suggesting autosomal dominant inheritance have been described, but there evidence of genetic heterogeneity.
- Miller syndrome patient also have similar facial anomalies observed in Franceschetti-Zwahlen-Klein Syndrome, but present postaxial limb anomalies such as hypoplastic or absent fifth digits and absent toes. The inheritence is persumably autosmal recessive.
- Dolichofacial pattern.
- Hypoplasticsupraorbital rims.
- Hypoplastic zygomas.
- Down turned corners ofmouth.
- Process of hair thatextends toward thecheek-25%.
- Retrusivemandible andmaxilla.
- High mandibularplane angle.
- Deficient malarprojection.
- Dorsal nasal hump.
- Cleft palate - 35%.
- Cleft lip-palate - rare.
- Macrostomia - 15%.
- Abnormal molar morphology.
- Abnormal condylar andcoronoid process.
- Absent or hypoplasticparotid glands
- Eyes:Hypertelorism, down slanted palpebralfissures, Coloboma of lower eyelid, decrease or absence ofcilia on lower eyelid, hypoplastic lacrimal glands.
- Ears:Symmetric external earmalformations, middle ear abnormalities, hypoplastic or absent cavitiesand ossicles, most patients have moderate-to-severe conductive hearingloss.
- Airway may becompromised insignificantlyretrognathic patients, tracheotomy and sleep apnea.
- Intelligence is usually normal.
- Mild mental retardation is present in fewcases.
- Second, an early childhood program for speech and language stimulation may be recommended.
- Third, if a cleft palate is present, the craniofacial team will advise you on the optimum timing for surgical closure of the cleft.
- Fourth, reconstructive surgery is available to improve the appearance of the face. The craniofacial center will advise you on what to expect from such surgery and on optimum timing. Since not all children are affected to the same degree, both the necessity and the outcome of reconstructive surgery vary from child to child. Surgical procedures vary among surgical treatment centers and according to the severity of the conditions. Surgery to rebuild the cheek bones usually begins before the child reaches the age of five. It is believed that the best approach is to use the child's own bone and to avoid placing artificial material beneath the skin. At the time of this surgery, the outer corners of the eyes are usually raised to tighter the lower eyelids.
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