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 Introduction
At birth the skull is made up of five bones held together by seams called sutures. The sutures remain open until age one to allow for brain growth. Craniosynostosis is a congenital anomaly caused by early closure of one or more of these sutures, resulting in limited or distorted head growth. Other names used for this condition are synostosis or cranial stenosis. In some cases, in addition to abnormal head shape, craniosynostosis can result in raised pressure inside the skull.
The infant skull consists of five plates of bone held together by clear, fibrous areas called sutures. The sutures should remain open as long as the brain continues to grow, enabling the skull to expand and properly accommodate the brain's growth.
When a suture closes, a predictable abnormality of head shape occurs. In certain children, changes around the eyes and face may occur because of fusion of the sutures in the cranial base. When this occurs, the child often has an associated craniofacial syndrome that must be diagnosed by a skilled geneticist
Symptoms
Parents and pediatricians will often note changes in the infant's head shape or premature closure of the fontanelles (soft spots).
There are several types of craniosynostosis:
 Metopic synostosis - The metopic suture is located between the soft spot and the nose. Early closure of this suture results in trigoncephaly. Infants will often have a prominent ridge down the middle of the forehead, a pointed forehead, and eyes that seem too close together.
 Sagittal synostosis - Early closure of the sagittal suture results in scaphocephaly. Infants will often have a protruding forehead, an elongated head, and a bony protruberance at the back of the skull.
 Coronal synostosis - Coronal synostosis may occur on either side or may be bilateral. Infant will often have an elevation of the eye socket, flattening of the ridge of the eye and displacement of the nose on the affected side.
 Lambdoid synostosis - Lambdoid synostosis is rare. It may occur on either side or may be bilateral. Infants will often have flattening of the back of the skull on the affected side.
Diagnosis
The diagnosis of craniosynostosis is usually made by a neurosurgeon based on physical examination of the child. Sometimes skull x-rays and 3D reconstructed CT scans are required, however this is not mandatory.
Treatment
Children with craniosynostosis need a genetic evaluation because of the strong association between this condition and several genetic syndromes, such as Apert's Syndrome and Crouzon's Syndrome. Surgical reconstruction of the cranial vault is performed by a craniofacial team that often involves neurosurgeons, plastic surgeons, and oral-maxillofacial surgeons. This usually involves removing the prematurely closed sutures and remodeling the skull bones to create a normal appearing head. Recent advancements have made this procedure less invasive with smaller incisions and less blood loss.
After surgery children will often need to be placed in a molded helmet to further guide skull development.
Prognosis
Most craniofacial centers have excellent cosmetic results. Sometimes children will need revision surgery as they grow older.
Several of these procedures are performed yearly at the University of Florida under the guidance of Dr. David Pincus.
Endoscopic craniosynostosis repair article in Neurosurgical Focus
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