Optimal management of atlantoaxial rotatory subluxation entails early diagnosis with plain cervical radiographs and dynamic computed tomography. Closed reduction with cervical traction followed by rigid immobilization accomplished reduction in 15 of 16 patients (94%) and was curative in 10 of 16 patients (63%). Although reduction was achieved more rapidly and effectively with traction than with a collar, there may be a role for simple immobilization without reduction in patients with a short duration of symptoms. There does not appear to be a correlation between cause of atlantoaxial rotatory subluxation, age, or sex and the likelihood of recurrence.

The authors reviewed the records of 94 patients who underwent initial repair of a lipomyelomeningocele between 1982 and July 1996 at the Children’s Hospital of Pittsburgh to determine the incidence and time course of symptomatic retethering. In each of these patients, the initial operative goals were to microsurgically debulk as much of the lipoma as possible to allow the conus to move freely within the spinal canal, to divide any tethering arachnoidal adhesions, to close the pia if possible and to reconstitute a capacious thecal sac, using a dural graft if necessary. With a median follow-up of 58 months, 19 of these patients (20.2%) required 28 subsequent operations for symptomatic retethering. Median time between the initial procedure and reoperation for retethering was 52 months. The primary complaint of 12 patients was intractable low-back or leg pain. Other common symptoms were progressive bowel and/or bladder dysfunction, deterioration of motor function and foot deformities. The decision to reoperate was based predominantly on the clinical situation of the patient; magnetic resonance imaging was used to confirm the location and extent of tethering. Patients with transitional lipomas had a significantly higher frequency of symptomatic retethering than those with caudal or dorsal lesions (p < 0.05). No other clinical or technical feature correlated with an increased frequency of retethering. In particular, none of a variety of types of dural graft materials appeared to entirely prevent symptomatic retethering. Following reoperation, pain complaints resolved and many of the other symptoms improved partially or resolved completely. Although the long-term results were also favorable in the majority of patients, a small subgroup (n = 6) exhibited repetitive symptomatic tethering that proved increasingly difficult to treat. We concluded that symptomatic retethering is a common problem in children with lipomyelomeningoceles, even after an adequate initial operation. To date, no type of graft material has been shown to entirely prevent this problem. Close long-term surveillance of such patients is required to allow detection and treatment of symptomatic retethering.

The outcomes of 25 pediatric patients who underwent upper cervical or occipitocervical fusion at the authors' institution since 1983 were reviewed. At a mean age of 9 years, the patients presented with spinal instability that was associated with os odontoideum in 11 cases, rotatory subluxation in five cases, odontoid fracture in two cases, atlantooccipital dislocation in two cases, and congenital atlantoaxial instability in five patients, four of whom had Down's syndrome (trisomy 21). Ten children had abnormal findings on neurological examination preoperatively; however, nine experienced improvement or resolution of deficits as of their latest follow-up evaluation (mean 17 months). Fusion was achieved with the first operation in 21 of 25 patients; eventually it was attained in all but one. Four patients exhibited persistent spinal instability after an initial procedure. This was caused by erosion of a multistranded cable through the intact arch of C-2 in two cases, by pin site infection necessitating early halo removal in one case, and by slippage in a halo following a Gallie procedure, which was revised with a Brooks fusion in one case. This series, the largest yet published, shows that with appropriate surgical management, posterior upper cervical fusion in the pediatric population is highly successful. Careful attention to halo pin site care and caution in using multistranded cable in young patients may improve results.