PURPOSE OF THE STUDY: Injuries to the upper cervical spine in children are rare and account for 0.6 to 9.5% of all cervical spine injuries. We present a detailed analysis of the children and adolescents with unstable upper cervical spine injuries treated at our spinal centre. MATERIAL: During 16 years of follow-up, unstable injury to the upper cervical spine was recorded in 23 children and adolescents. Two patients (8.7%) were treated conservatively and 21 (91.3%) underwent surgery. The patients were allocated by age to three groups: 0-9 year, 10-14 year and 15-18 year categories. Twenty patients were seen at the final clinical and radiographic follow-up. One patient died at 62 months after surgery and two patients unfit for transport were evaluated on the basis of mailed interviews. The interval between injury and final evaluation ranged from 6 to 137 months, with an average of 53.4 months. METHODS: The patients treated conservatively first wore a Philadelphia collar, then a custom-made brace, and eventually a soft Schanze cervical collar to finish the healing process. Application of a halo vest was considered a surgical procedure and was used only in very small children. In unstable odontoid fractures, direct osteosynthesis with two cannulated titanium screws was performed from the anterior approach in older children while, in small children, transoral or submandibular retropharyngeal decompression to treat spinal stenosis caused by bone fragments was carried out and a halo vest was applied. Hangman's fractures were treated by anterior cervical discectomy, fusion with bone graft and anterior plate fixation. The other types of unstable fractures were managed from the posterior approach by occipitocervical fixation, atlantoaxial fixation or instrumented fusion extended caudally. The patients characteristics included gender, age, mechanism of injury, type of injury, neurological findings, type of therapy or surgery, complications and treatment outcome. Neurological status was evaluated using the Frankel classification. RESULTS: The patient group comprised 14 boys (60.9%) and nine girls (39.1%), which gave a gender ratio of 3 : 2. The age of patients at injury ranged from 2 to 18 years, with an average of 11 years and 6 months. The most frequent injuries included rotational or vertical atlantoaxial dislocation in eight (34.8%) and odontoid fractures in seven (30.4%) patients; atlas fracture was recorded in three (13.0%) and hangman's fracture also in three (13.0%) patients; occipitocervical displacement was found in one (4.3%) and complex atlantoaxial fracture also in one patient (4.3%). At the time of injury, 17 patients (73.9%) had no neurological deficit (Frankel grade E), three had Frankel grade A (one paraplegic with a concomitant T5 spinal cord injury) and three had Frankel grade D neurological deficits. Of the six patients with neurological deficit, two showed improvement by one or two Frankel grades. The method of dorsal atlantoaxial fixation was used in eight patients (Magerl fixation in 2 and Harms method in 6). Direct osteosynthesis of an odontoid fracture was performed in four patients, halo fixation was applied in four, C2-C3 discectomy with tricortical bone grafting and plating was carried out in three, occipitocervical fixation was used in three patients, and direct atlas osteosynthesis, simple decompression and simple non-instrumented dorsal spondylodesis each was performed in one patient. Neither intra-operative complications nor post-operative complications related to the surgical technique were recorded. Osteoarthritis or bone non-union, as late post-operative complications, were found in two patients. All other patients showed bone healing by first intention in the desired extent. Superficial or deep wound infections were not recorded. DISCUSSION: In the first age category, the number of boys and girls with injuries to the upper cervical spine was equal while, in the third one, the boys outnumbered the girls more than twice. Of the 23 patients, 91.3% were surgically treated; the anterior approach was used in approximately one third of the patients and the posterior approach in the rest of them. The high number of surgical interventions is due to the fact that the most serious paediatric spinal injuries are referred to our centre. CONCLUSIONS: 1. Injuries to the upper cervical spine are most frequently found in the youngest children and in adolescents who, however, frequently have injury also to the lower cervical spine. 2. Neurological deficit is relatively frequent but has a better prognosis than in adults. The youngest children with mild deficits have the best prognosis. 3. The mortality rate in young children with upper cervical spine injuries is evidently high, mostly due to associated head, chest and abdomen trauma 4. Therapy, particularly in small children, is strictly individual.