Syrinx Secondary to Chiari-like Tonsillar Herniation in Spontaneous Intracranial Hypotension

Syrinx development in patients with spontaneous intracranial hypotension (SIH) has rarely been described. To better understand this entity, we compared the clinical and radiographic findings in a series of patients with SIH and acquired Chiari-like tonsillar herniation with and without syrinx formation.

Six patients with syrinx in the setting of SIH and Chiari-like tonsillar herniation were retrospectively identified. The clinical and radiographic findings and outcomes were compared with those from a control group of patients with SIH and Chiari-like tonsillar herniation without syrinx.

The patients with SIH and syrinx had had a higher opening pressure than had the control group (mean, 14.0 cm H₂O vs. 7.4 cm H₂O; P = 0.02) and a higher body mass index (mean, 33 kg/m² vs. 26 kg/m²; P = 0.01). The patients with syrinx had had an average obex displacement of 3.7 ± 2.2 mm below the plane of the foramen magnum compared with a position of 1.9 ± 3.1 mm above the plane of the foramen magnum in the control group (P = 0.004). The mean tonsillar descent was 12.7 ± 4.7 mm below the foramen magnum in those with syrinx compared with 5.9 ± 2.5 in the control group (P = 0.009). The clinical symptoms had improved in 83.3% of the patients with syrinx and 75% of the control patients after spinal cerebrospinal fluid leak closure. Three patients (50%) also had radiographic syrinx reduction.

Our results have shown that SIH can be an underrecognized cause of syrinx with key differences in body habitus and obex displacement compared with SIH without syrinx. In patients with tonsillar herniation into the foramen magnum associated with syrinx, the presence of SIH should be considered to avoid unnecessary foramen magnum decompression, even in those with a normal opening pressure.