Pediatric Idiopathic Intervertebral Disc Calcification of the Cervical Spine

Pediatric idiopathic intervertebral disc calcification (PIIVDC) is a rare childhood condition characterized by the calcification of the intervertebral disc, which can progress to inflammation or extrusion. The etiology of intervertebral disc calcification in children remains unclear, but it is generally believed to involve genetic and/or metabolic deficits, inflammation triggered by infection, reduced nutrient supply, or trauma. This condition is often self-limited, with favorable outcomes even when treated conservatively. However, there are no gold standard guidelines for its treatment, making it essential to understand its clinical presentation, management, and outcomes.

Clinical Presentation and Diagnosis

PIIVDC most commonly affects the cervical spine, with about 70% of cases located in this region. Patients often present with neck pain, and some may experience neurological symptoms such as muscle weakness, numbness, or radiating pain in the upper limbs. Torticollis, or a twisted neck, can also be a presenting symptom. In some cases, the condition is asymptomatic and discovered incidentally during imaging studies.

Diagnosis of PIIVDC typically involves imaging techniques such as anteroposterior and lateral radiographs of the cervical spine, computed tomography (CT), and magnetic resonance imaging (MRI). Radiographs can reveal increased density consistent with calcification at the affected intervertebral levels. CT scans are useful for confirming the calcified density of the nucleus pulposus, while MRI can assess the extent of spinal cord compression. In some cases, focal dense areas of enhancement are detected in the respective disc spaces.

Patient Demographics and Symptoms

In a study of 12 children diagnosed with cervical disc calcification, the age range was between 5 and 18 years, with a male predominance (nine boys and three girls). All patients were symptomatic at the time of presentation. The most common symptom was neck pain, reported in 10 out of 12 children. Torticollis was observed in three children, and neurological symptoms involving the upper limbs were present in four children, including one with muscle weakness in the hand and four with numbness or radiating pain in the hands. No bladder or anal sphincter dysfunction was observed. Three children had a history of neck injury, and two had fever prior to the onset of other symptoms.

Imaging Findings

All 12 patients underwent anteroposterior and lateral radiographs of the cervical spine. Seven patients underwent CT to confirm the calcified density of the nucleus pulposus, and five patients underwent MRI to assess spinal cord compression. Most children had one-level involvement of the cervical spine, with the C4/5 level being the most commonly affected (41.7%). Only one child had involvement of two calcified intervertebral discs at C2–C3 and C3–C4, with flattening of the C3 vertebrae.

Treatment and Management

Given the benign and self-limiting nature of PIIVDC, most patients were treated conservatively. Nine out of the 12 patients (75%) received non-interventional strategies, including rest, intensive follow-up observation, and avoidance of strenuous activities. The remaining three patients (25%) were treated with analgesics or neck braces for persistent neck pain or stiffness. All patients were followed up at 3 months, 6 months, and 12 months, and annually thereafter until both the symptoms and calcification had resolved.

Outcomes

All patients achieved good therapeutic outcomes with no worsening of symptoms after treatment. Five patients (5/12) achieved complete symptom relief in less than 3 months. The nine patients who received non-interventional treatment did not experience any exacerbation or recurrence of symptoms. Follow-up imaging showed that 8 out of the 12 patients (66.7%) achieved complete resolution of disc space calcification within 2 years, and four patients had a reduction in the volume of the calcifications during the follow-up period but did not achieve complete resolution.

Case Study

A 5-year-old boy was admitted to the hospital with a sudden onset of neck pain, torticollis, and focal neurological deficiency. The symptoms started 2 months earlier with neck pain and torticollis due to a trauma-induced neck strain. Initial X-ray examination did not reveal any abnormalities, and the symptoms improved on day 5 after the onset. A week before admission, the boy started to experience weakness in his left upper limbs. Physical examination revealed limited activity of the neck and a muscular deficit of the left musculus deltoideus. Cervical spine radiographs showed C4–C5 intervertebral disc calcification. CT scan demonstrated calcification of the nucleus pulposus with lateral herniated calcification at the C4–C5 level. MRI revealed decreased signal intensity of the involved disc on T1- and T2-weighted images, causing serious spinal cord compression. The patient was treated conservatively under clinical supervision and advised to avoid strenuous exercise. After 2 weeks of treatment, there was a marked improvement in the left upper limb weakness. A neurological examination in 3 months showed complete resolution of the symptoms. A CT scan of the cervical spine performed 3 months after the initial presentation showed continued disappearance of the calcified disc and extrusion.

Discussion

PIIVDC is a rare condition with a generally benign course. The exact etiology remains unclear, but it is believed to involve genetic and/or metabolic deficits, inflammation triggered by infection, reduced nutrient supply, or trauma. The condition is most commonly located in the cervical spine and often presents with neck pain and occasional neurological dysfunction. Diagnosis is primarily based on imaging studies, including radiographs, CT, and MRI.

Treatment of PIIVDC is primarily conservative, with non-interventional strategies such as rest, intensive follow-up observation, and avoidance of strenuous activities being the mainstay of management. Analgesics and neck braces may be used for persistent symptoms. Surgical intervention is rarely required and is only considered in cases of progressive neurological deterioration.

Principles for Treatment

Based on the experience from the study, the following principles for the treatment of PIIVDC have been summarized:

Non-Interventional Measures: For asymptomatic or mildly symptomatic patients, especially those under 10 years of age, non-interventional measures including rest and intensive follow-up observation are recommended. Patients should be advised to avoid strenuous activities.
Conservative Treatment: For patients with persistent and intolerable symptoms such as neck pain, stiffness, torticollis, and back pain, conservative treatment including analgesics, cervical collars, and traction should be recommended. Symptoms usually resolve spontaneously within 6 months with conservative treatment.
Surgical Treatment: Surgical intervention should be considered for patients with progressive neurological deterioration, regardless of whether they have undergone an adequate course of conservative treatment. Critical attention should be paid to the aggravation of symptoms, which usually manifest as weakness of the extremities. Surgical options include anterior cervical corpectomy/discectomy or posterior laminoplasty/laminectomy, depending on individual circumstances.

Conclusion

PIIVDC is a rare and generally benign condition that most commonly affects the cervical spine in children. The exact etiology remains unclear, but it is believed to involve genetic and/or metabolic deficits, inflammation triggered by infection, reduced nutrient supply, or trauma. Diagnosis is primarily based on imaging studies, and treatment is primarily conservative, with non-interventional strategies being the mainstay of management. Surgical intervention is rarely required and is only considered in cases of progressive neurological deterioration. The condition has a favorable prognosis, with most patients achieving complete resolution of symptoms and calcification with conservative treatment.

doi.org/10.1097/CM9.0000000000001927

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