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

• Published in: Acta neurochirurgica 2020-07, Vol.162 (7), p.1585-1595
• Main Authors: Liu, Bolin, Wang, Yuan, Liu, Shujuan, Zhang, Yufu, Lu, Dan, Chen, Lei, Zheng, Tao, Zhao, Tianzhi, Zhao, Lanfu, Sankey, Eric W., Gao, Guodong, Qu, Yan, He, Shiming
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Arnold-Chiari Malformation - surgery; Brain; Cerebrospinal fluid; Cisterna Magna - surgery; Congenital defects; Craniotomy - adverse effects; Craniotomy - methods; Decompression; Decompression, Surgical - adverse effects; Decompression, Surgical - methods; Female; Fluid flow; Humans; Interventional Radiology; Male; Medicine; Medicine & Public Health; Middle Aged; Minimally Invasive Surgery; Neural tube defects; Neurology; Neuroradiology; Neurosurgery; Original Article - Spine - Other; Patients; Postoperative Complications - epidemiology; Skull; Spinal cord; Spine - Other; Surgical Orthopedics; Syringomyelia - surgery; Tonsillectomy - adverse effects; Tonsillectomy - methods
• ISSN: 0001-6268; 0942-0940
• DOI: 10.1007/s00701-019-04177-9

Background 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.