Neurosurgical Consultants: Spine Disorders: Occipitocervical Synostosis

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OCCIPITOCERVICAL SYNOSTOSIS

Occipitocervical Synostosis is a congenital malformation characterized by a partial or complete union between the Atlas (C1 Vertebra) and the Base of the Occiput of the Skull. It also is known as "Occipitalization" of the Atlas which means the assimilation of the Atlas into the Occipital Bone at the Base of Skull.

Occipitocervical Synostosis, Basilar Impression, and other Odontoid Anomalies are the most common developmental malformations of the Occipitocervical junction. The expression of these entities is up to 2.5 per 1000 children with males and females affected equally. There is no particular age range of patients presenting with this anomaly and it has been identified in patients from 8-52 years.

SYMPTOMS

Some patients remain entirely asymptomatic throughout life, while others develop symptoms following mild trauma.

The following represent the most common signs and symptoms experienced by these patients:

Weakness
Poor co-ordination (Ataxia) in the lower limbs (occasionally in the upper limbs)
Pain in the sub-occipital region and neck
Vertigo (dizziness)
Dysequilibrium (Unsteady gait)
Weakness (Paresis) of the limbs
Tingling (Paresthesia)
Speech disturbances (Dysarthria)
Hoarseness
Double vision (Diplopia)
Loss of Conscience (Syncope)

Interference with swallowing

DIAGNOSIS

Physical Examination

There are certain physical characteristics that made lead to the suspicion of this condition:

Short broad neck
Low hairline
Torticollis
High scapula

Restricted neck movements (similar to Klippel-Feil Syndrome)

Among the other associated anomalies occasionally seen with Occipitocervical Synostosis are:

C2-C3 fusion
Dwarfism,
Funnel chest
Pes cavus
Syndactylies
Jaw anomalies
Cleft palate
Congenital ear deformities
Hypospadias

Genitourinary tract defects

Neuroimaging

CT and MRI Scans are important diagnostic tools in the evaluation of anomalies in this anatomical region.

Among the bone anomalies that become apparent are: backward tilt of the Odontoid process, the presence of an Articular Facet between the anterior rim of the Occiput and the Odontoid process, asymmetry of the Atlanto-axial joints and fusion between the body and lamina of the 2^nd and 3^rd Cervical Vertebrae.

TREATMENT

Occipitocervical Synostosis (with its related anomalies) constitutes a serious risk to severe neurological impairment and death. While most patients are managed, at first, with non-operative measures in the form of traction and support of the neck by a Neck Brace, once neurological signs and symptoms develop consideration should be given to surgical management.

There are several operations that have been used in the management of these problems among which are Laminectomy alone or combined with Craniectomy and "release of posterior dural bands".

ALL PATIENTS WITH CONGENITAL CERVICAL FUSION SHOULD AVOID BODY CONTACT SPORTS AS WELL AS ANY RECREATIONAL ACTIVITIES THAT MAY PUT THEM AT RISK FOR HEAD TRAUMA.
THE POTENTIAL FOR CATASTROPHIC OUTCOMES WITH THESE STRUCTURAL ABNORMALITIES IS VERY REAL AND MUST BE KEPT IN MIND THROUGHOUT THE LIFE OF THE AFFECTED INDIVIDUAL.
LONG TERM FOLLOW UP IS HIGHLY RECOMMENDED. PATIENTS AND HIS/HER FAMILY MUST BE CAREFULLY AND FULLY COUNSELLED.