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Clinical and radiological outcome of craniocervical osteo-dural decompression for Chiari I-associated syringomyelia

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Abstract

The aim of this study was to analyze the long-term clinical and radiological outcomes of craniocervical decompression for patients affected by Chiari I-related syringomyelia. We performed a retrospective analysis of a group of patients affected by Chiari I-associated syringomyelia treated by craniocervical decompression (CCD). Surgical and technical aspects and preoperative factors predicting outcome were discussed. A total of 36 patients were reviewed. There were 17 men and 19 women (female/male ratio 1.11), and the mean age was 40.4 (range 18–68). The most important preoperative symptoms were related to myelopathy (pain, weakness, atrophy, spasticity, sensory loss, and dysesthesias). Most syrinxes were in the cervico-thoracic region (61.1%), and the majority of patients had tonsillar descent between the foramen magnum and C1. All patients underwent a craniectomy less than 3 cm in diameter followed by a duroplasty with dura substitute. No arachnoid manipulation was necessary. Three patients (8.1%) experienced cerebrospinal fluid leaks that resolved without complications. At a mean follow up of 40 months (range 16–72) 80.5% of patients exhibited improvement over their preoperative neurological examination while 11.1% stabilized. The syrinx shrank in 80.5% of patients. Chi-square test showed that preoperative syrinx extension and degree of tonsillar descent did not correlate with clinical and neuroradiological postoperative evolution. Treating syringomyelia associated in Chiari I malformation with CCD leads to a large percentage of patients with satisfying results and no irreversible complications.

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Authors and Affiliations

  1. Division of Neurosurgery, Civil Hospital, Alessandria, Italy

    Giannantonio Spena & Pietro Versari

  2. Division of Neurosurgery, Santa Croce e Carle Hospital, Cuneo, Italy

    Claudio Bernucci

  3. Department of Neurosurgery, S. Giovanni Battista Hospital, University of Turin, Turin, Italy

    Diego Garbossa

  4. Department of Neurology, S. Giovanni Battista Hospital, University of Turin, Turin, Italy

    Walter Valfrè

  5. Neurosurgery Department, via Venezia 16, 15100, Alessandria, Italy

    Giannantonio Spena

Authors
  1. Giannantonio Spena
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  2. Claudio Bernucci
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  3. Diego Garbossa
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  4. Walter Valfrè
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  5. Pietro Versari
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Correspondence to Giannantonio Spena.

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Comments

Ralf Alfons Kockro, Zurich, Switzerland

This is an interesting retrospective analysis of 36 consecutive cases summarizing the clinical and radiological outcome of decompression surgery for Chiari I associated syringomyelia. The results show first of all that straight forward posterior fossa decompression with duroplasty and without arachnoid opening results in a high percentage of clinical improvement and a low complication rate. Equally important, the radiological analysis shows that the pre-operative size of syrinx and position of tonsils do not correlate with the postoperative clinical or radiological course. In view of these results, I agree with the authors that early cranio-cervical decompression is the most appropriate first line surgical strategy. However, it would be interesting to further investigate the pre- and postoperative imaging features of those patients which did not benefit from decompression surgery in a larger series in order to define the cases in which extended surgical methods could be considered to further improve clinical outcome.

Jörg Klekamp, Quakenbrück, Germany

The authors present a series of 36 patients with Chiari I malformation and an average follow-up of 40 months. Surgical treatment involved a small decompression of the foramen magnum with a duraplasty but without arachnoid opening. The major argument for leaving the arachnoid or even the inner dura layer intact during this operation is avoidance of complications, i.e., cerebrospinal fluid (csf) fistulas in particular. In this paper, the authors claim no complications related to the surgical technique and yet they mention six accidental tears of the arachnoid (16.6%) and 3 csf fistulas (8.3%) that required either a lumbar drain or a surgical revision. In my understanding, these are complications related to the surgical technique. As with other similar papers on this subject, the technique of leaving the arachnoid intact does not automatically avoid csf fistulas. I always open the arachnoid during this operation. With appropriate closure of the wound, i.e., insertion of the duraplasty with running sutures and meticulous soft tissue closure, the rate of csf fistulas in my personal series of 194 patients is 3.1%.

The second argument is that clinical and radiological results are as good with this method as with potentially more dangerous techniques involving arachnoid dissection. This is probably a correct statement as far as short-term results are concerned. However, we have to remember, that we are dealing with a chronic disease. If we want to find out how to best treat our patients, we have to analyze long-term results applying survival statistics such as the Kaplan-Meier method and determine the rates of progression-free survival for each surgical technique. To my knowledge, none of the papers in the literature as well as this one provide such data. In this paper, the authors only mention two surgical revisions 2 and 7 years after the initial operation, i.e., a rate of 5.6%. Looking again at my series, four of 194 patients (2.1%) required surgical revisions 4–75 months after the initial operation. This low revision rate suggests that 97.9% were more or less satisfied. But that is not correct. With Kaplan-Meier statistics, the rate of patients free of progression was 82.9% for 15 years. As long as we do not have such data 5, 10, or 15 years after surgery for the different techniques advocated in the literature, this controversial discussion will continue.

Lotfi Hacein-Bey, Sacramento, USA

Although there is a wide consensus that symptomatic syringomyelia (with or without Chiari I malformation) should be generally treated with craniocervical decompression, there is some variety in the techniques advocated. The authors present a well-documented retrospective analysis of 36 patients with syringomyelia and Chiari I malformation treated during an 11-year period with a consistent surgical technique of duraplasty performed through a relatively small bone opening, and without arachnoid opening. Clinical presentation was posterior fossa overcrowding, long tract impairment or brainstem compression. Clinical and imaging follow-up are of reasonable duration and quality. Postoperative syrinx decrease rate was 80.5% and clinical improvement was observed in 92% of the patients in this series.

Although better outcomes have been reported in the literature, the main merit of this article is to show that reasonable outcomes may be obtained in patients with syringomyelia who are treated with the consistent use of a relatively minimal surgical technique.

As our understanding of the physiology of syringomyelia increases, and as newer surgical techniques become available, continued improvements in clinical outcomes can be expected.

Richard G. Fessler, Chicago, USA

Dr. Spena et al., report the retrospective results of their decompression/dura-plasty technique for the treatment of Chiari I malformation in a group of 36 patients. They state that 80.5% of their patients experienced improvement of their neurologic symptoms over their preoperative neurological examination with a mean follow-up of 40 months (range 16–72). Their conclusion, based on these results, is that treating Syringomyelia associated in Chiari I malformation leads to a large percentage of patients with satisfying results with no irreversible complications. These results are not inconsistent with previous short-term results reported for a variety of techniques of treating Chiari malformation. Important observations noted by the authors include their observation that there appeared to be no correlation between the presence of syringomyelia and clinical outcome, and that the amount of pre-operative cerebellar descent and symptoms did not correlate with post-operative clinical result.

However, several technical details do make validity of these conclusions suspect. First, of course, the data was collected retrospectively. Second, the data was collected by the operating surgeons, thus introducing potential bias. Third, it is not discernable from the text, exactly what “improved, unchanged, or worsened” actually means, given the multiple symptoms followed by the authors. Finally, although the authors report that their follow-up averages 40 months, the “patients and methods” section reports that clinical evaluation was done at “one, three, and six months and at one year.” It is difficult to know how to reconcile this difference.

It is well-known that certain aspects of Chiari syndrome, such as muscle weakness and atrophy, ataxia, dorsal column dysfunction, hypesthetic pain, and cranial neuropathies are notoriously difficult to treat. Thus, having more detail on exactly what got better, what remained unchanged, what worsened, and how these impacted the patient’s quality of life are critical to interpreting outcome. Moreover, late failures and progression of symptoms are also common following treatment of Chiari malformation. Thus, assuming that the author’s data do reflect a true, unbiased improvement in their patient’s symptoms, a more accurate conclusion could only claim short term benefit. Long-term success rate needs to be determined after at least several more years of clinical follow-up.

Ricardo Botelho, São Paulo, Brazil

Magnetic resonance imaging brought clarity to the diagnosis of Chiari malformation and has revolutionized the treatment evaluation of this disease. The adequacy of neural decompression and return of cerebrospinal fluid flow at craniocervical junction may be, now, verified. The new factor recently suggested to evaluate the adequacy of surgery is the maintenance and expansion of the cisterna magna. The presence of this cisterna has been considered important in the prevention of post-operative cerebellar ptosis, functioning as a cushion buffer for structures of cranial posterior fossa. The arachnoid preservation technique in surgery for Chiari malformation has the potential to avoid adhesions between the dura and the neural structures that would occur with arachnoid opening and neural dissection techniques, recreating the Cisterna magna.

Because of the relative rarity of Chiari malformation, several samples of patients in different series have been necessary to demonstrate efficacy of the described technique. The work of Spena and Cols. provides additional evidence of such intervention in Chiari malformation. The authors have obtained more than 80% reduction in syringomyelia cavities, high clinical improvement and low complication rates. Technique of arachnoid preservation must be considered an important option in surgery for Chiari malformation, as demonstrated by these authors, among others.

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Spena, G., Bernucci, C., Garbossa, D. et al. Clinical and radiological outcome of craniocervical osteo-dural decompression for Chiari I-associated syringomyelia. Neurosurg Rev 33, 297–304 (2010). https://doi.org/10.1007/s10143-010-0260-y

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