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  • Author or Editor: Alex Alfieri x
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Alex Alfieri and Giampietro Pinna

Object

There is little information about the long-term effectiveness and complications following decompressive surgery for syringomyelia related to Chiari malformation Type I (CM-I).

Methods

Examining long-term clinical and radiological follow-up, the authors studied a mixed retrospective and prospective single-institution cohort of 109 consecutive surgically treated adult patients with syringomyelia and CM-I. All patients underwent a standardized surgical protocol: decompression of the craniocervical junction, arachnoid exploration, and shrinkage of the cerebellar tonsils. Factors predicting outcome were investigated.

Results

The retrospective arm consisted of 41 cases treated between 1990 and 1994, and the prospective arm comprised 68 patients treated between 1994 and 2001. The mean overall age was 45.9 years, and 58.8% of the population was female. The median follow-up period was 12.7 years. The most frequent initial symptoms were pain and sensory and gait disturbances. There was no perioperative death or neurological deterioration. The comprehensive perioperative complication rate was approximately 11%, with 3 cases (2.7%) of CSF leakage. Regression analysis showed that the best combination of clinical and radiological outcome predictors was age and duration of symptoms. Clinical follow-up confirmed surgical result stability with clinical improvement of greater than 90% of the spinal and cranial manifestations over a long-term period. Two patients had radiological recurrences of syringomyelia without clinical signs 85 and 124 months after surgery.

Conclusions

Certain clinical predictors of poor clinical and radiological prognosis were identified—namely, age at time of surgery and symptom duration. The results of the study provide additional long-term data that support the effectiveness and safety of relieving CSF block at the craniocervical junction in CM-I–related syringomyelia.

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Giampietro Pinna, Franco Alessandrini, Alex Alfieri, Marcella Rossi and Albino Bricolo

Cerebrospinal fluid (CSF) flow abnormalities are known to be present in Chiari I malformation and to underlie the origin and progression of associated syringomyelia. The incidence of syrinx formation, however, is variable for unknown reasons. The aim of this study was to investigate whether differences in CSF flow dynamics in patients with Chiari I malformation may account for the different clinical and radiological presentation.

Presurgical and postsurgical phase-contrast magnetic resonance imaging investigations were prospectively conducted in 47 adult patients with symptomatic Chiari I malformation. Patients were divided into two groups according to the presence (32 cases) or absence (15 cases) of syrinx. Cerebrospinal fluid flow patterns were evaluated at four regions of interest: prebulbar cistern, foramen magnum, and the ventral and dorsal spinal subarachnoid spaces at the C-5 level. A temporal analysis of CSF flow waveforms was performed with measurement of cranial- and caudal-directed flow durations. All patients underwent a craniocervical decompressive procedure. Preoperatively, a prolonged caudal-directed (systolic) flow pattern was observed in patients with syringomyelia, as compared with normal control values obtained in 15 healthy volunteers. Conversely, a decreased systolic duration was observed in Chiari I patients who had malformation without syrinx. These trends were not statistically significant because of the considerable degree of overlap with the control values recorded in both groups. Additional comparison of the observed preoperative values obtained in patients with and those without syringomyelia indicated that the difference in systolic flow duration was significant at the ventral spinal subarachnoid space level (p = 0.003) and remarkable at the other levels, although not reaching statistical significance. Cerebrospinal fluid flow was minimal or absent at the foramen magnum (dorsal aspect) due to tonsillar herniation, precluding reliable quantitative measurement at this level. There was no evidence of communication between the fourth ventricle and syrinx in any case. Postoperatively, unobstructed CSF flow was recorded across the enlarged foramen magnum and into the artificial cisterna magna in all patients. A gradual restoration of near-normal flow patterns was observed in both groups. Inside the syrinx, fluid motion gradually tapered, no longer being detectable in 12 patients (37.5%) 1 year postsurgery.

In patients with Chiari I malformation and associated syringomyelia different CSF flow patterns were demonstrated as compared with patients in whom syrinx was absent. Analysis of this study's findings supports the hypothesis that in Chiari I malformation an elongated systolic flow may prolong the condition of increased spinal subarachnoid pressure caused by the junctional obstruction, thus favoring CSF penetration into the spinal cord. It may be also proposed that a shortened systolic flow may be insufficient to maintain a hypertensive condition for enough time to induce syrinx formation.