Hindbrain herniation syndromes: The Chiari malformations (I and II)

Cai, Christopher Y.; Oakes, W. Jerry

doi:10.1016/s1071-9091(97)80036-8

Cited by 47 publications

(42 citation statements)

References 69 publications

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“…This was relatively higher than that reported by Munshi and colleagues and Depreitere and colleagues [16,24]. This is consistent with the fact that pediatric patients experience less permanent neurologic injury and higher improvement rates after PFD, owing to their enhanced repair capability and short clinical history [5,25,26].…”

Section: Discussionsupporting

confidence: 82%

Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to Chiari malformation type I

Zhu

Jiang

et al. 2011

Eur Spine J

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Introduction Description of syrinx resolution after posterior fossa decompression (PFD) in patients with scoliosis secondary to Chiari malformation type I (CMI) and syringomyelia (SM) has been rarely reported in the literature. This study was performed to investigate the outcome of PFD in patients with scoliosis secondary to CMI and to identify potential predictive factors for better outcome after PFD. Material and methods Patients with scoliosis secondary to CMI and SM, who had undergone PFD during the period 2000 through 2009, were recruited. Inclusion criteria were (1) age B 18 years, (2) diagnosis of SM associated with CMI, (3) scoliosis as the first complaint, (4) having undergone preoperative and follow-up magnetic resonance imaging (MRI). Patients with acquired CMI anomalies or who had received syringosubarachnoid shunting were excluded. The maximal S/C ratio and syrinx length were measured to evaluate syrinx resolution after PFD. A 20% decrease in S/C ratio or length at the latest follow-up was defined as a significant radiographic improvement and complete resolution was used to describe the syrinx disappearing after PFD.Results 44 patients were recruited. Follow-up MRI was conducted for all 44 patients at 6 ± 3 months postoperatively, for 37 patients at 2 years ± 3 months, for 26 patients at 4 years ± 3 months, and for 15 patients at 6 years ± 3 months. 97.7% (43 of 44) of patients showed significant radiographic improvement by MRI. The distance of tonsillar descent (mm) was correlated significantly with the surgical outcome (r = 0.116, P = 0.013). Significant improvement was observed within 6 months postoperatively, with continued slow improvement after that. ConclusionSyringes showed significant improvement after PFD in most patients with scoliosis secondary to CMI. Resolution generally occurred within 6 months follow-up and continued at a slow rate for several years. In addition, the severity of tonsillar descent is a potential predictor for better improvement after standard PFD.

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Section: Discussionsupporting

confidence: 82%

Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to Chiari malformation type I

Zhu

Jiang

et al. 2011

Eur Spine J

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“…Of the 26 patients studied only 2 have had any symptoms referable to the Chiari malformation. In large series as many as 30% of patients with Chiari II become symptomatic [12]. This suggests that the reduction in hindbrain herniation may result in a parallel reduction in symptoms.…”

Section: Discussionmentioning

confidence: 99%

Effect of Intrauterine Myelomeningocele Repair on Central Nervous System Structure and Function

Tulipan¹,

Jp²,

Hernanz‐Schulman

et al. 1999

Pediatr Neurosurg

121

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Background: It has been postulated that intrauterine myelomeningocele repair might improve neurologic outcome in patients with myelomeningocele. A total of 59 such procedures have been performed at Vanderbilt University. Preliminary results suggested that the degree of hindbrain herniation is reduced by intrauterine repair. In an attempt to further quantify the possible benefits of this surgery, a subset of these patients was brought back to Vanderbilt for study. Methods: A group of 26 patients who had undergone intrauterine myelomeningocele repair underwent an extensive evaluation which included manual muscle testing, MR imaging and precise determination of the anatomic level of their lesions as well as multiple other tests. The results of this analysis were compared to those in 2 groups of historical controls. Results: In this group of patients intrauterine myelomeningocele repair substantially reduced the incidence of moderate to severe hindbrain herniation (4 vs. 50%). The incidence of shunt-dependent hydrocephalus was more modestly reduced (58 vs. 92%). The average level of leg function closely matched the average anatomic level of the lesion in both the fetal surgery and control groups. Conclusion: The most dramatic effect of intrauterine repair appears to be on hindbrain herniation. A less dramatic, but significant, reduction in shunt-dependent hydrocephalus is also seen. Prospective patients should be cautioned not to expect improvement in leg function as the result of this surgery. The potential benefits of surgery must be carefully weighed against the potential risks of prematurity.

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“…As a serious pathological condition described by Professor Hans Chiari in 1891 [1], Chiari Malformation type I (CM-I) is characterized by the downward displacement of cerebellar tonsils, which are rounded structures located at the bottom of each cerebellar hemisphere, into the spinal canal [2,3]. Radiologically, descent of the cerebellar tonsils more than 5 mm into the cervical canal through the foramen magnum leads to the diagnosis of CM-I [4].…”

Section: Introductionmentioning

confidence: 99%