Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia.

OBJECT: Patients with hindbrain herniation or the so-called Chiari malformation Type I (CM-I) and/or syringohydromyelia are treated with dorsal decompression alone; however, a small percentage of patients with other associated abnormalities require concomitant dorsal craniocervical junction (CCJ) fusion. The authors surveyed the indications for CCJ fusions in this population.

METHODS: A retrospective review of University of Iowa medical records and radiographs obtained between 1996 and 2005 was performed. Inclusion criteria encompassed patients with diagnoses of CM-I and/or syringohydromyelia requiring dorsal CCJ fusions, and others with CCJ abnormalities who had CM-I and/or syringohydromyelia.

RESULTS: Two hundred thirty-four patients were identified, all of whom were symptomatic at presentation. Their ages ranged from 2.5 to 86 years; 33% of the patients were < 16 years of age. Patients were categorized as follows, with some being assigned to > 1 category: Group I, congenital or acquired CCJ abnormalities with reducible bone compression (25% of patients); Group II, previous anterior CCJ/upper brainstem decompression (44%); Group III, occipitocervical complex instability with CM-I and/or syringohydromyelia but without CCJ bone abnormalities requiring adjunctive posterior fossa decompression (26%); and Group IV, musculoligamentous instability, either from pathological states or from muscle dehiscence from repeated posterior fossa procedures (14%). Instrumentation was used in 96% of patients, with all 96% receiving semirigid fixation with titanium loop and sublaminar cables; all fusion constructs incorporated autologous bone. At last follow-up evaluation, fusion was radiographically complete in 97%, and symptom improvement was seen in 92%.

CONCLUSIONS: Dorsal CCJ fusions are required in patients with CM-I and/or syringohydromyelia who have concomitant CCJ abnormalities (Groups I and II). A definite group (CM-I and/or syringohydromyelia) without bone abnormality exists (Groups III and IV). This may be due to muscle weakness secondary to a high syrinx.