Tonsillectomy with modified reconstruction of the cisterna magna with and without craniectomy for the treatment of adult Chiari malformation type I with syringomyelia

Acta Neurochirurgica (2020) 162:1585–1595

In light of the controversies regarding the surgical treatment of adult Chiari malformation type I (CM-I) with syringomyelia, a retrospective study was conducted to evaluate the safety and efficacy of tonsillectomy followed by modified reconstruction of the cisterna magna with or without craniectomy.

Methods Between 2008 and 2017, 78 adult CM-I patients (36 males and 42 females, mean age 40.6 years old) with syringomyelia were treated with posterior fossa decompression (PFD) with tonsillectomy and modified reconstruction of the cisterna magna. Patients were divided into two study groups: group A (n = 40) underwent cranioplasty with replacement of the bone flap; group B (n=38) underwent suboccipital craniectomy. Neurological outcomes were evaluated by traditional physician assessment (improved, unchanged, and worsened) and the Chicago Chiari Outcome Scale (CCOS). Syringomyelia outcomes were assessed radiologically.

Results The procedure was successfully performed in all patients, and restoration of normal cerebrospinal fluid (CSF) flow was confirmed by intraoperative ultrasonography. The median postoperative follow-up was 20.3 months (range 18– 60 months). Clinical improvement was evident in 66 (84.6%) patients, with no significant differences between the two groups (85.0% vs. 84.2%, P = 0.897). According to the CCOS, 36 patients (90.0%) in group A were labeled as “good” outcome, compared with that of 34 (86.8%) in group B (P = 0.734). Improvement of syringomyelia was also comparable between the groups, which was observed in 35 (87.5%) vs. 33 (86.8%) patients (P = 0.887). The postoperative overall (7.5% vs. 23.7%, P = 0.048) and CSF-related (2.5% vs. 18.4%, P = 0.027) complication rates were significantly lower in group A than group B.

Conclusions Tonsillectomy with modified reconstruction of the cisterna magna without craniectomy seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia, though future well-powered prospective randomized studies are warranted to validate these findings.

Tonsillobiventral fissure approach to the lateral recess of the fourth ventricle

J Neurosurg (127): 768-774, 2017

Surgical access to the lateral recess of the fourth ventricle (LR) is suboptimal with existing transvermian and telovelar approaches because of limited lateral exposure, significant retraction of the cerebellar tonsil, and steep trajectories near brainstem perforator arteries. The goal in this study was to assess surgical exposure of the tonsillobiventral fissure approach to the LR, and to describe the relevant anatomy.

METHODS Two formaldehyde-fixed cerebella were used to study the anatomical relationships of the LR. Also, the tonsillobiventral fissure approach was simulated in 8 specimens through a lateral suboccipital craniotomy.

RESULTS The pattern of the cerebellar folia and the cortical branches of the posterior inferior cerebellar artery were key landmarks to identifying the tonsillobiventral fissure. Splitting the tonsillobiventral fissure allowed a direct and safe surgical trajectory to the LR and into the cerebellopontine cistern. The proposed approach reduces cervical flexion and optimizes the surgical angle of attack.

CONCLUSIONS The tonsillobiventral fissure approach is a feasible and effective option for exposing the LR. This approach has more favorable trajectories and positions for the patient and the surgeon, and it should be added to the armamentarium for lesions in this location.

Distribution of cerebellar tonsil position: implications for understanding Chiari malformation

Cerebellar tonsil position

J Neurosurg 119:812–819, 2013

Prior attempts to define normal cerebellar tonsil position have been limited by small numbers of patients precluding analysis of normal distribution by age group. The authors’ objective in the present study was to analyze cerebellar tonsil location in every age range.

Methods. Two thousand four hundred patients were randomly selected from a database of 62,533 consecutive patients undergoing MRI and were organized into 8 age groups. Magnetic resonance images were directly examined for tonsil location, morphology, and other features. Patients with a history or imaging findings of posterior fossa abnormalities unrelated to Chiari malformation (CM) were excluded from analysis. The caudal extent of the cerebellar tonsils was measured at the midsagittal and lowest parasagittal positions.

Results. The mean tonsil height decreased slightly with advancing age into young adulthood and increased with advancing age in the adult age range. An increasing age in the adult age range was associated with a decreased likelihood of a tonsil position 5 mm or more below the foramen magnum (p = 0.0004). In general, the lowest tonsil position in each age group was normally distributed. Patients with pegged morphology were more likely to have a tonsil location at least 5 mm below the foramen magnum (85%), as compared with those having intermediate (38%) or rounded (2%) morphology (p < 0.0001). Female sex was associated with a lower mean tonsil position (p < 0.0001). Patients with a lower tonsil position also tended to have an asymmetrical tonsil position, usually lower on the right (p < 0.0001).

Conclusions. Cerebellar tonsil position follows an essentially normal distribution and varies significantly by age. This finding has implications for advancing our understanding of CM.