OBJECTIVE If left untreated, occipitocervical (OC) instability may lead to serious neurological injury or death. Open internal fixation is often necessary to protect the neurovascular elements. This study reviews the etiologies for pediatric OC instability, analyzes the radiographic criteria for surgical intervention, discusses surgical fixation techniques, and evaluates long-term postoperative outcomes based on a single surgeon's experience. METHODS The charts of all patients < 18 years old who underwent internal OC fixation conducted by the senior author were retrospectively reviewed. Forty consecutive patients were identified for analysis. Patient demographic data, OC junction pathology, radiological diagnostic tools, surgical indications, and outcomes are reported. RESULTS The study population consisted of 20 boys and 20 girls, with a mean age of 7.3 years. Trauma (45% [n = 18]) was the most common cause of instability, followed by congenital etiologies (37.5% [n = 15]). The condyle-C1 interval had a diagnostic sensitivity of 100% for atlantooccipital dislocation. The median number of fixated segments was 5 (occiput-C4). Structural bone grafts were used in all patients. Postsurgical neurological improvement was seen in 88.2% (15/17) of patients with chronic myelopathy and in 25% (1/4) of patients with acute myelopathy. Preoperatively, 42.5% (17/40) of patients were neurologically intact and remained unchanged at last follow-up, 42.5% (17/40) had neurological improvement, 12.5% (5/40) remained unchanged, and 2.5% (1/40) deteriorated. All patients had successful fusion at 1-year follow-up. The complication rate was 7.5% (3/40), including 1 case of vertebral artery injury. CONCLUSIONS Occipitocervical fixation is safe in children and provides immediate immobilization, with excellent survival and arthrodesis rates. Of the radiographic tools evaluated, the condyle-C1 interval was the most predictive of atlantooccipital dislocation.
Keywords: AAD = atlantoaxial dislocation; ADI = atlantodental interval; AIS = American Spinal Injury Association (ASIA) impairment scale; AOD = atlantooccipital dislocation; AVM = arteriovenous malformation; CCI = condyle-C1 interval; CVJ = craniovertebral junction; EDS = Ehlers-Danlos syndrome; NLI = neurological level of injury; OC = occipitocervical; OCF = occipitocervical fusion; fusion; long-term; mJOAS = modified Japanese Orthopaedic Association score; occipitocervical; outcome; pediatric; spine.