Mandibulofacial Dysostosis - Signs and Symptoms
Mandibulofacial Dysostosis is characterized by a wide variety of clinical features depending on the branchial arches involved in the developmental malformation. The first thing that is apparent on looking at a person affected by the condition is the presence of anti-mongoloid palpebral fissures and the partially absent
It is theorized that Mandibulofacial Dysostosis occurs due to the failure of the maxillary mesoderm to develop beyond what is called the 50 mm stage of the embryo. This means that the trigger that spurs the development of the head and neck region is either faulty or completely absent. This theory is supported by evidence of teeth formation by the sixth week of fetal life. The teeth in the upper jaw are especially affected in this condition and are place in quite unusual positions. Sometimes they fail to erupt and are embedded in the bone itself. Patients with Mandibulofacial Dysostosis are prone to fracture quite easily because of the fragile nature of facial bones. Even a push could bruise the face quite easily. Because of the malformation of the ears, patients can also suffer from hearing loss.
A syndrome called as the Goldenhar syndrome has similar features as the Mandibulofacial Dysostosis. It is also called as hemifacial microsomia, but occurs less frequently than MD. In Goldenhar syndrome, the defects are unilateral in distribution as against the bilateral features in MD and is postulated to occur due to the interruption in blood supply to the face.
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Dr. Reeja Tharu. "Mandibulofacial Dysostosis - Signs and Symptoms". Medindia. https://www.medindia.net/patients/patientinfo/mandibulofacialdysostosis_symptoms.htm. (accessed May 27, 2022).
Dr. Reeja Tharu. 2021. Mandibulofacial Dysostosis - Signs and Symptoms. Medindia, viewed May 27, 2022, https://www.medindia.net/patients/patientinfo/mandibulofacialdysostosis_symptoms.htm.
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