Dysostosis Craniofacial

Dysostosis craniofacial (syn. Crouzon syndrome) is a disease of the craniofacial skeleton. This set of symptoms occurs due to disruption of the correct formation of bones and cartilage of the face during fetal development. Dysostoses are accompanied by disorders in a child in two directions at once: the facial part of the skull and the base of the skull

**Dysostosis** craniofacial

Disorders of the craniofacial skeleton, including the head and face, include local lesions of the facial skeleton or significant disturbances in the structure, shape and size of the head involving its structures. Usually, only the facial part of the skull is locally affected, and there are also cases where the facial part of the head is affected predominantly by pathological changes.

**Primary dysostotic craniofacial syndrome Kuku (Crouzon syndrome)** Crouzon syndrome is a genetically determined osteochondral pathology of the facial skull (dysplasia), manifested by small dysontogenetic changes in individual bones of the face and skull. The disease is characterized by a discrepancy between the normal external structure of the skull and internal dysplastic changes in the intraosseous structure of the basal skeleton. The site of primary developmental imbalance is noted in the posterior part of the palatine bone, in the sphenoid-ethmoid region or in the lingual wall of the paranasal sinuses. But the most characteristic pronounced dysontogenies for Crouzon Syndrome develop in the anterior part of the occipital bone (basal cartilage of the anterior cranial fossa), which leads to a delay in the formation of the temporo-orbital joint, zygomatic bones and frontal tubercles, nasal bones, and causes some wedge-shaped features. These deformities extend to almost all the bones of the midface and end near the midline, distinctly different from adjacent sides. The patient has a wide and flat bridge of the nose, a flat forehead, soft ears and a massive headache associated with