Delayed Resolution of Chiari I-associated Hydromyelia after Posterior Fossa Decompression: Case Report and Review of the Literature

Doughty, Kyle E.; Tubbs, R. Shane; Webb, Daniel; Oakes, W. Jerry

doi:10.1227/01.neu.0000134548.16165.9

Cited by 21 publications

(5 citation statements)

References 33 publications

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“…11 However, it is important to note that syrinx collapse is not necessary for symptomatic improvement, 6,8,18,22,37,46 and syringes may resolve in a delayed fashion. 10 …”

Section: Discussionmentioning

confidence: 99%

Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

Kennedy

Kelly

Phan

et al. 2015

PED

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OBJECT Symptomatic pediatric Chiari malformation Type I (CM-I) is most often treated with posterior fossa decompression (PFD), but controversy exists over whether the dura needs to be opened during PFD. While dural opening as a part of PFD has been suggested to result in a higher rate of resolution of CM symptoms, it has also been shown to lead to more frequent complications. In this paper, the authors present the largest reported series of outcomes after PFD without dural opening surgery, as well as identify risk factors for recurrence. METHODS The authors performed a retrospective review of 156 consecutive pediatric patients in whom the senior authors performed PFD without dural opening from 2003 to 2013. Patient demographics, clinical symptoms and signs, radiographic findings, intraoperative ultrasound results, and neuromonitoring findings were reviewed. Univariate and multivariate regression analyses were performed to determine risk factors for recurrence of symptoms and the need for reoperation. RESULTS Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up (mean 32 months). There were no major complications. The mean length of hospital stay was 2.0 days. In a multivariate regression model, partial C-2 laminectomy was an independent risk factor associated with reoperation (p = 0.037). Motor weakness on presentation was also associated with reoperation but only with trend-level significance (p = 0.075). No patient with < 8 mm of tonsillar herniation required reoperation. CONCLUSIONS The vast majority (> 90%) of children with symptomatic CM-I will have improvement or resolution of symptoms after a PFD without dural opening. A non–dural opening approach avoids major complications. While no patient with tonsillar herniation < 8 mm required reoperation, children with tonsillar herniation at or below C-2 have a higher risk for failure when this approach is used.

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“…11 However, it is important to note that syrinx collapse is not necessary for symptomatic improvement, 6,8,18,22,37,46 and syringes may resolve in a delayed fashion. 10 …”

Section: Discussionmentioning

confidence: 99%

Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

Kennedy

Kelly

Phan

et al. 2015

PED

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“…6,9,10,46 Despite only 70% of our patients showing radiographic improvement of the syrinx, 100% had initial improvement postoperatively and 88% remained asymptomatic or with minimal symptoms at last follow-up. These results are similar to the study by Wetjen et al, where at 6 months after PFD with dural opening, 96% of the patients experienced some degree of clinical improvement while only 86% had radiographic improvement of the syrinx; after 2 years, 68% remained mildly symptomatic even though 100% had radiographic improvement of the syrinx.…”

Section: Discussionmentioning

confidence: 73%

“…13,14,26,32 Furthermore, several groups have reported symptom improvement after PFD in the absence of radiographic syrinx improvement, 6,8,15,26,37,46 and it has been shown in some cases that the syrinx may resolve in a delayed fashion. 9 The frequency and time course of syrinx resolution after PFD without dural opening has not been previously studied in a large series. This information is important for neurosurgeons when determining whether to perform a dural opening procedure and when deciding whether a prior PFD without dural opening has failed and further surgery is needed.…”

Section: Discussionmentioning

confidence: 99%

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Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation Type I

Kennedy¹,

Nelp²,

Kelly³

et al. 2015

PED

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OBJECT Chiari malformation Type I (CM-I) is associated with a syrinx in 25%–85% of patients. Although posterior fossa decompression (PFD) without dural opening is an accepted treatment option for children with symptomatic CM-I, many surgeons prefer to open the dura if a syrinx exists. The purpose of this study was to investigate the frequency and timing of syrinx resolution in children undergoing PFD without dural opening for CM-I. METHODS A retrospective review of 68 consecutive pediatric patients with CM-I and syringomyelia who underwent PFD without dural opening was conducted. Patient demographics, presenting symptoms and signs, radiographic findings, and intraoperative ultrasound and neuromonitoring findings were studied as well as the patients’ clinical and radiographic follow-up. RESULTS During the mean radiographic follow-up period of 32 months, 70% of the syringes improved. Syrinx improvement occurred at a mean of 31 months postoperatively. All patients experienced symptom improvement within the 1st year, despite only 26% of patients showing radiographic improvement during that period. Patients presenting with sensory symptoms or motor weakness had a higher likelihood of having radiographic syrinx improvement postoperatively. CONCLUSIONS In children with CM-I and a syrinx undergoing PFD without dural opening, syrinx resolution occurs in approximately 70% of patients. Radiographic improvement of the syrinx is delayed, but this does not correlate temporally with symptom improvement. Sensory symptoms or motor weakness on presentation are associated with syrinx resolution after surgery.

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“…Syringomyelia is associated with many intracranial and spinal anomalies 5) . One study postulated that syringomyelia results from obstruction of CSF flow at the foramen magnum and the outlet of the fourth ventricle 2) .…”

Section: Discussionmentioning

confidence: 99%

Syringomyelia Associated with a Huge Retrocerebellar Arachnoid Cyst: A Case Report

et al. 2015

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Occasionally, a posterior fossa arachnoid cyst can induce compression of the spinal cord and cause syringomyelia. Here, we report the case of a 29-year-old man with both progressive shoulder pain and gait disturbance, who was found to have a huge retrocerebellar arachnoid cyst associated with syringomyelia. Accordingly, posterior fossa decompression and arachnoid cyst excision were performed. Post-operative MRI showed a marked reduction in the size of the arachnoid cyst and syringomyelia. The patient's symptoms were clearly improved compared to before surgery. In our view, treatment in such patients should focus on decompressing the foramen magnum and include the removal of the coexistent arachnoid cyst walls, which appear to be the crucial factor in development of syringomyelia. In this report, we discuss the pathogenic mechanisms underlying syringomyelia-associated retrocerebellar arachnoid cyst and review the current literature on this topic.

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Delayed Resolution of Chiari I-associated Hydromyelia after Posterior Fossa Decompression: Case Report and Review of the Literature

Abstract: Unfortunately, this is an area in which the literature is lacking. With this case as a nidus, studies are now necessary to determine the range of time necessary for Chiari I malformation-related hydromyelia to resolve.

Cited by 21 publications

References 33 publications

Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation Type I

Syringomyelia Associated with a Huge Retrocerebellar Arachnoid Cyst: A Case Report

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