[Summary]
Clavicular cranial dysplasia, also known as clavicular cranial ossification anomaly (cleidocranial
It is a syndrome characterized by hereditary abnormalities in the development of the skull, maxillofacial bones and clavicle.
[Etiology]
Cleidocranial dysostosis is a congenital abnormality of the skeletal system and is often inherited in an autosomal dominant fashion.
[Clinical manifestations]
The disease is relatively rare in clinical practice. Due to the abnormal development of the skull, maxillofacial bones and clavicle, the patient develops craniofacial abnormalities, abnormalities of the hafnium dental system and abnormalities of the clavicle and even other skeletal systems throughout the body. Specifically, they are
1. Craniofacial abnormalities
The anterior and posterior fontanelles and sagittal suture are not closed, and because the facial bones do not develop, the cranial and facial size ratios are disproportionate, the face is small, the midline area from the frontal to occipital bones is obviously depressed, and the parietal, temporal and frontal bones are abnormally elevated. The orbital spacing is wide, the supraorbital ridge is obviously elevated, the infraorbital rim is receding, the nasal bridge is depressed, and the eyes appear to be protruding. The palatal lid is high arched and narrow, often with grooves or fissures, making the nasal airway narrow and poorly developed, and the lower jaw is also underdeveloped with delayed median union.
2. Abnormalities of the dental periosteal system
Insufficient development of the upper jaw and crowding of the upper teeth; due to the metabolic imbalance of bone, it cannot absorb the roots and the bone tissue of the surrounding crypt normally, so that the loss of milk teeth is delayed, the eruption of permanent teeth is abnormal, and a large number of ambiguous obstructed teeth, missing or defective teeth, and incomplete occlusion are common, which is a characteristic manifestation of this disease. In addition, there may also be congenital edentulousness, or extra teeth in the maxillary anterior and mandibular molar areas, and root malformation, enamel or dentin dysplasia may also be seen.
3. Abnormalities of clavicle and other bones
Different degrees of clavicle hypoplasia make the patient’s shoulders have abnormal dynamics, which can make the two shoulders touch each other in the midline; the upper chest is flattened and the inner angle of the scapula is posteriorly extended; some of them are accompanied by multiple bone changes in the thorax, spine, pelvis and limbs.
Such patients are usually short in stature, but do not affect intelligence.
Radiographic manifestations: showing multi-bony changes characterized by clavicle, skull and maxillary changes.
Skull: Because of bone calcification or dysplasia, it presents changes of prolonged cranial membranous state, i.e., open fontanelle and posterior fontanelle and non-closed bone sutures. The facial bones are significantly smaller than the cranial bones. The sinuses are not developed or hypoplastic. The nasal bone, lacrimal bone, and zygomatic bone are underdeveloped or calcified, and the zygomatic arch can be divided into two segments, and the mastoid is dense and uncalcified.
Maxilla: narrow, sometimes with cleft palate. The lower jaw is protruding and not united in the middle. The maxilla and mandible contain most ambulatory teeth, some with extra teeth. Various curvilinear deformities of the tooth roots are seen. Sometimes it may be accompanied by cyst formation.
Clavicle: It can be divided into two segments with fibrous connection in the middle. Some of the bone segments on both sides are absent.
The thorax is funnel-shaped, the ribs are sagging, and some of the saber processes are absent. The spine may be anteriorly convex, posteriorly concave or laterally convex.
In addition, the pelvis may have constricted deformity, narrowing of the front end of the population, asymmetry on both sides; inversion of the limbs and hip joints, and shortening of the femoral neck.
[Treatment]
Generally, only intraoral local treatment is performed. Combined with orthodontics and orthopedics to repair the erupted teeth, the morphology and functional integrity of the dental row, for the retained teeth can be seen in its deep ambulatory tooth position or permanent tooth embryo development status to decide whether to take, for the deep ambulatory teeth in the missing tooth area can be surgically opened and orthodontic implantation method to make it erupt; for non-retainable diseased teeth can be extracted under the premise of X-ray to determine the root morphology. In case of combined cysts, surgical extraction is performed. Rhinoplasty was performed for severe nasal bridge collapse. In addition, since the skull sutures and fontanelle of this patient are not closed, all kinds of sharp injuries to the head should be avoided to prevent damage to important cranial organs.