HE management of congenital AAD is challenging. The presence of this anomaly in the pediatric age group adds to the dilemma, because children usually do not report their symptoms until the disease is quite severe. Ossification of the bones of the CVJ may not be complete until the age of 9 years, making radiological diagnosis difficult. 8 The management of IAAD or RAAD is not well delineated. The movement restriction that occurs after posterior fusion in a growing child is of concern and is usually overlooked.It remains unclear whether IAAD and RAAD represent two extremes of the same spectrum or different entities. In this study, the differences between IAAD and RAAD with regard to the clinical presentation, radiological features, and outcome in pediatric patients (children Յ 16 years of age) are analyzed. Clinical Material and Methods Patient PopulationNinety-six patients with congenital AAD (those with an atlantodental interval Ͼ 5 mm) who were 16 years of age or younger were included in this study. Fifty-seven patients had an IAAD (mean atlantodental interval 9.5 Ϯ 2.3 mm) and 39 had an RAAD (mean atlantodental interval 8.1 Ϯ 2.8 mm) calculated from dynamic (lateral, flexion, and extension views) plain x-ray films and intrathecal contrast CT scans of the CVJ. 23 The mean age at presentation for patients with IAAD and RAAD was 12.54 Ϯ 3.42 and 11.03 Ϯ 2.81 years, respectively (range 3-16 years). A definite male predominance existed in both groups (the male/ female ratio was 45:12 in the IAAD and 31:8 in the RAAD groups). Patients with associated Chiari malformation Type I and those with associated genetic syndromes (Down or Morquio syndrome, achondroplasia, and so on) were excluded from the study. Mean values are represented as the means Ϯ standard deviations.Object. Clinicoradiological differences and outcome following surgery in pediatric patients (Յ 16 years of age) with congenital irreducible atlantoaxial dislocation (IAAD) and reducible atlantoaxial dislocation (RAAD) were analyzed.Methods. Ninety-six patients (57 with IAAD and 39 with RAAD) were categorized as follows: Grade I, no deficits except hyperreflexia or neck pain (six patients); Grade II, minor deficits but independent for activities of daily living (25); Grade III, partially dependent (30); and Grade IV, totally dependent (35). Patients with RAAD underwent direct posterior fusion, and those with IAAD were treated with transoral decompression and posterior fusion. Patients with good outcomes included those who could walk unaided, with improvement in spasticity and weakness, and those who maintained Grade I status. The category of poor outcome included patients with the following conditions: postoperative deterioration or lack of improvement; inability to ambulate regardless of neurological improvement at a minimum follow-up duration of 3 months; or perioperative death.A significantly higher incidence of C-1 assimilation, C2-3 fusion, asymmetrical occiput-C2 facet joints, and basilar invagination were seen in patients with IAAD, and os odontoideum...

Radiological differences in the anatomy of patients with IAAD and those with RAAD may be due to improper segmentation of the occipital and upper cervical sclerotomes in the former and dysfunction of the transverse ligament in the latter. A significantly better outcome was noted in completely dependent patients with IAAD compared with those with RAAD. Respiratory compromise was an important prognostic factor.

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Background:Dysraphic lesions in adults, presenting clinically as tethered cord syndrome (TCS), are relatively rare, and their optimal management remains controversial.Patients and Methods:We performed a retrospective analysis of our pediatric database over a period of last 7 years to focus on the adult TCS. Our aim was to determine the clinicoradiological and etiopathological differences between adult and pediatric patients as well as to determine the results of surgery in adult TCS.Results:Adult spinal dysraphisms constituted 15.4% of our patients (20 out of 130). Motor weakness, sphincteric dysfunction, and backache (n = 13, 65.0% each) predominated in adults unlike children who presented with subcutaneous swellings (n = 74, 67.6%) followed by motor weakness (n = 40, 46.4%), backache being reported by only three patients. The different pathologic substrates underlying adult dysraphisms were lipomeningocele (n = 8), split cord malformation (total = 7; Type 1: n = 5; Type 2: n = 2), dermal sinus (n = 2), and fatty filum (n = 3). On the other hand, meningomyelocele/meningocele (61, 54.9%) followed by split cord malformation Type 1 and 2 (n = 29, 26.1%) predominated in children. The radiological differences between the two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults. At a mean follow-up of 20.5 months, the most common symptoms to improve following detethering were pain (11 out of 13, 84.6%) followed by motor weakness (six out of 13, 56.2%) and sphincteric control (7 out of 13, 53.8%).Conclusion:Most common symptoms to improve following detethering in adult TCS were pain followed by motor weakness. The major radiological differences between these two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults.

Xanthogranuloma is a rare lesion of the sellar-suprasellar region. We describe a case of suprasellar xanthogranuloma in whom serial MRI revealed features that have not been previously described--development of dural tail, vascular encasement and intra-axial lesions in posterior fossa.