Striking and Abnormal Head Shape Craniosynostosis (Premature Closure of a Suture) | |
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Incidence | |
More frequent than 1 in 1000 births. | |
Clinical significance | |
1. An abnormal head shape detracts from cosmetic appearance. | |
Etiology | |
Unknown. Some types are hereditary. | |
Pathology, anatomical types | Illustrations |
Disturbance of the regulation of normal and age-dependent maturation of one or several sutures, leading to a premature closure and an abnormal structure. Fusion of the sagittal suture, bilateral and unilateral fusion of the coronal suture, fusion of the metopic suture, bilateral and unilateral fusion of the lambdoid suture, fusion of multiple sutures, and craniofacial dysostosis. | show details |
Pathophysiology | |
The growth of the skull is disturbed in the direction perpendicular to the involved suture, and increased in all other directions, which leads to a characteristic deformity. | |
Clinical presentation (history, findings, clinical skills) | Illustrations |
It is important to look at the skull from all directions: From the front and the back, from both sides, and from the top. Due to the characteristic shape it is clinically possible to recognize the specific type of suture closure. | show details |
Natural history | Illustrations |
With advancing age there is no spontaneous correction of the deformities. With growth there is often an increase in the deformity, but rarely an involvement of other sutures, and onset or worsening of functional damage. | show details |
Differential diagnosis | Illustrations |
Differential diagnosis includes: Birth molding, deformities due to tumor-like masses or tumors, plagiocephaly following congenital muscular torticollis, postural and positional molding. | show details |
Work-up examinations | Illustrations |
Skull x-rays in two planes and CT: Confirmation of the fusion and quantification of the skull deformity and involvement of the skull base and facial bones, exclusion of combined malformations. In craniofacial dysostosis: CT with reconstruction. Opthalmological examinations, measurement of intracranial pressure during 24 to 48 hours, and genetic work-up and consulting. | show details |
Therapy | Illustrations |
Absolute indications for corrective surgery: Imminent (natural history) or present loss of function and/or increased intracranial pressure. Severe deformity. | show details |
Prognosis | |
Following one or several sessions there are good esthetic and functional results. There is only a positive influence on mental development in case of increased intracranial pressure.
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