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Cranio-molar dysplasia, also known in colloquial language as the lion’s face, is a genetic condition that affects and distorts the bones of the skull and the shafts of long bones. It is a rare disease with only 20 diagnosed cases worldwide. It was probably discovered in 1949.
Lion’s face is a rare disease. So far, medicine has not found a clear cause of this condition, but doctors believe that recessive genes are responsible for the disease. However, no specific gene has yet been found to cause this disorder. Cases were also diagnosed in which the lion’s face was the result of dominant inherited genes. The lion’s face is diagnosed on the basis of the clinical picture. Biopsy of the mastoid process may also help in the diagnosis, as it enables the diagnosis of hyperostosis, which is manifested by aeration of the bone tissue and loss of air cells.
Lion face – symptoms
A characteristic feature of cranio-shaft dysplasia is the aforementioned hyperostosis, which leads to a permanent deformation of the skull. The lion’s face is characterized by a change in the nose that makes it wider, a change in facial features, deformation of the auditory canal, an increase in the distance between the eyeballs and an increase in the circumference of the head. Progressive hyperostosis closes the openings of the skull with a bone tank. This process gradually destroys the nerves. The optic nerves are also damaged, leading to complete blindness over time. The vestibulocochlear nerve is also damaged, which in turn leads to complete deafness. A late symptom of craniocortical dysplasia may be total tetraplegia, which is a consequence of narrowing of the spinal canal and damage to the roots of the spinal nerves. Epilepsy and mental retardation can also be another symptom. As a result of the occurrence of craniofacial dysplasia, the nasolacrimal ducts and the posterior nostrils are damaged and disabled. The progressive thickening and enlargement of the bone tissue leads to increased pressure and the resulting symptoms. In patients diagnosed with lion’s face, changes in the epiphysis, enlargement of the shafts and changes in the collarbones, ribs and bones of the pelvis may also be found.
Lion face – differentiation
There are three diseases that the lion’s face can be mistaken for
- Van Buchem’s team;
- Camurati-Engelmann disease;
- cranio-epiphyseal dysplasia.
Lion face – treatment
The medicine of the XNUMXst century does not in any way make it possible to effectively treat craniofacial dysplasia, so the lion’s face still remains a medical mystery. Despite this, the treatment is symptomatic and supportive. MRI scans of the head are essential to check the degree of nerve compression. There are also treatments aimed at eliminating symptoms. These treatments include, for example, craniofacial remodeling, nostril widening and opening of the nasolacrimal duct.
Often times, a craniectomy is required in which part of the skull bone is surgically removed. Cranio-molar dysplasia, or lion’s face, is an incurable disease, and these treatments can, at best, eliminate most of the symptoms or slow down the course of the disease. People with craniofacial dysplasia require constant psychological care, as do their families, and awareness of genetic diseases should be increased both by the media and the public.