The surgical treatment of Chiari I malformation

Menezes

2008

SPI

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.

Section: Discussionmentioning

confidence: 99%

Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia

Menezes

2008

SPI

“…4,[7][8][9] It involves removing bone in the suboccipital area along with the posterior margin of the foramen magnum Group I D A 128 66 37 20 5 Group II D+SS B 39 20 11 6 2 Group III D+T C 106 55 31 16 4 Group IV D+T+SS D 43 22 13 7 1 Abbreviations: CMI, Chiari I malformation; D, suboccipital decompression; MRI, magnetic resonance imaging; SS, syringosubarachnoid shunt; T, cerebellar tonsillectomy. Figure 1 Preoperative MRI scan of a patient with syringomyelia associated with CMI illustrating a prominent syrinx (arrow).…”

Section: Discussionmentioning

confidence: 99%

“…Some authors have concluded that surgical treatment results in the improvement or stabilization of the disease in the majority of patients. 8,15 José Dones and De Jesú s 16 found that the main benefit of surgical treatment in patients with Chiari I malformation is to arrest the progression of symptoms. This pattern is not so clearly recognizable in our results, probably due to the small number of patients.…”

Section: Discussionmentioning

confidence: 99%

Chiari I malformation associated with syringomyelia: a retrospective study of 316 surgically treated patients

Zhang

Chen

et al. 2007

Spinal Cord

Objectives: The objective of this article is to probe the surgical procedures of treatment for Chiari I malformation (CMI) associated with syringomyelia and evaluate their efficacy based on clinical and neuroradiological results. Study design: Retrospective study. Setting: Department of Neurosurgery in Qilu Hospital of Shandong University in China. Methods: We retrospectively reviewed 316 surgically treated patients with CMI. We classified our patients after surgery in four global outcome categories as follows: very good (complete remission or marked improvement), good (slight improvement), fair (stability), poor (slight or marked deterioration). We simply describe the postoperative diameter of the syrinx (collapse, decreased, unchanged or increased). Results: At the time of discharge, the result was very good in 163 cases (51.58%), good in 92 cases (29.11%), fair in 49 cases (15.51%) and poor in 12 cases (3.80%). In the follow-up period, neurological status improved and was then sustained in 94.49% of patients (206/218). At more than 2 years after surgery, we were able to compare pre-and postoperative MR images in 218 patients. A collapse of the syrinx was seen in 34.86% (76/218) of patients. The size of the syrinx was decreased in 31.65% (69/ 218) of patients, remained unchanged in 26.15% (57/218) of patients and increased in 7.34%(16/218) of patients. Conclusion: Patients with CMI should be treated using various surgical procedures according to different magnetic resonance imaging types; surgical treatments may fully decompress the medulla oblongata and ameliorate the clinical syndromes.

“…Combined series, which included both pediatric and adult patients, constituted 61 reports (42%). [5][6][7]9,11,12,[25][26][27]34,37,38,40,43,44,46,47,52,53,55,63,66,67,70,73,74,76,83,87,90,91,94,96,100,102,103,106,110,111,114,115,117,121,126,127,129,130,132,135,[138][139][140]…”

Section: General Informationmentioning

confidence: 99%

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

Arnautovic

Splavski

Boop

et al. 2015

PED

203

OBJECT Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia. METHODS The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included. RESULTS The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (> 18 years of age; 27% of the cases), pediatric (≤ 18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series. CONCLUSIONS Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.