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.
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:
There are certain physical characteristics that made lead
to the suspicion of this condition:
- 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)
- Double vision (Diplopia)
- Loss of Conscience (Syncope)
- Interference with swallowing
Among the other associated anomalies occasionally seen with
Occipitocervical Synostosis are:
- Short broad neck
- Low hairline
- High scapula
- Restricted neck movements (similar to Klippel-Feil Syndrome)
CT and MRI Scans are important diagnostic tools in the
evaluation of anomalies in this anatomical region.
- C2-C3 fusion
- Funnel chest
- Pes cavus
- Jaw anomalies
- Cleft palate
- Congenital ear deformities
- Genitourinary tract defects
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 2nd and
3rd Cervical Vertebrae.
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.
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This page last edited on 2/19