Dr Satnam Singh Chhabra,
Head of Neurosurgery department at Sir Gangaram Hospital, New Delhi.
A very young boy of four-and-a-half years, Rahul (name changed) had a severe disability of craniovertebral junction (CVJ) disorder, a complex area where the skull and upper cervical spine join together. These abnormalities can often prove to be life threatening as nerves inside the spinal cord leading to the brain, as well as the brain stem gets damaged or squeezed. “When Rahul was brought to the hospital he was totally bed ridden. The young boy was an extremely complex case of congenital CVJ disorder and his condition deteriorated over the period of time. Usually such congenital cases become worse over the years either due to some injury, accident or trauma which leads to further progression of symptoms thereby, worsening patient's condition. Also, these disorders can weaken the spine and cause neurological injuries.
When Rahul was diagnosed with CVJ disorder, he was unable to move or lift his legs and often complained of stiffness in neck, heaviness of the head along with respiratory problems. Though Rahul was way too young to be operated for such a complex decompression and fusion surgery in craniovertebral region, still that was the last resort and only hope for survival of such advanced cases,” informs Dr Chhabra. Fusion is performed by placing screws and rods in the affected bones alongwith bone graft.
“Post surgery, Rahul was shifted to ICU for a few days till his condition stabilised and was strictly observed, until the young boy was discharged from the hospital.”
Today, after almost an year of his surgery, Rahul has not just recovered well, but is able to walk around without any help/support, and is leading a normal life. His parents too are relieved to see their young boy moving around like other children of his age group.
Craniovertebral junction disorders are very common in India. The CVJ region consists of the occipital bone that forms the base of the skull and first two bones of the spine which are in neck called as the atlas and axis. Disorders that affect the large opening at the bottom of the occipital bone are a particular concern because important structures like brain stem and some nerves and blood vessels pass through this opening. “The most common form of CVJ disorders are congenital (birth defects).such as: atlas assimilation (fusion of the atlas and occipital bone), atlas hypoplasia (absence of parts of the spine and congenital fusion), spina bifida (incomplete development of the spinal cord or its coverings), etc. These disorders lead to instability between atlas and axis bone,” informs Dr Chhabra adding, “But these could be acquired later due to an injury, trauma or some kind of disorder too. And, rarely though, tumors in CVJ region may also attribute to craniovertebral junction disorders. However, if these tumors spread to the bones of the neck, the first two spinal bones may become misaligned.”
Because the lower brainstem and spinal cord are flexible and therefore, susceptible to compression at this level, It can cause symptoms that vary from patient to patient and can result in serious health problems. “Patients may experience significant disability like progressive paralysis of limbs, neck pain and headache, but if the spinal cord or lowest part of the brain, i.e., brain stem is affected people may have difficulty sensing vibration, feel pain and may have weak muscles, dizziness and impaired vision too,” cautions Dr Chhabra. However, other symptoms of the condition may include difficulty with hand and leg coordination, numbness in feet, back pain or stiffness, etc. Immediate diagnosis and treatment are necessary for CVJ disorder so as to reverse the symptoms or to prevent permanent disability. “
