scholarly journals Multidimensional, patient-reported outcome after posterior fossa decompression in 79 patients with Chiari malformation type I

2019 ◽  
Vol 10 ◽  
pp. 242 ◽  
Author(s):  
Jan De Vlieger ◽  
Joost Dejaegher ◽  
Frank Van Calenbergh
Keyword(s):  
Patient Satisfaction ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Patient Reported Outcome ◽  
Symptom Improvement ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Important Improvement ◽  
Patient Reported

Background: We studied patient-reported outcome among patients who underwent posterior fossa decompression (PFD) for Chiari malformation type I (CM-I). Methods: We interviewed patients who underwent PFD for CM-I from 1995 to 2016. Results: A total of 79 patients were interviewed. The median age at surgery was 30 years (range 5–72 years) with 27 pediatric patients. Forty-six patients had syringomyelia (36 adults and 10 pediatric patients). Fifty-four patients (68%) reported at least some improvement, 46 (58%) important improvement, 13 (16%) worsening, and 12 stabilization (15%). Any improvement as well as important improvement were significantly more often reported in the nonsyringomyelia group (85% vs. 57%, P = 0.01 and 76% vs. 46%, P = 0.01, respectively). Of the 47 patients reporting preoperative neck pain, 31 (66%) reported at least some improvement after surgery and 9 (19%) worsening after surgery. Of the 59 patients experiencing headaches before surgery, 45 (76%) reported at least some improvement after surgery and 4 (7%) worsening. Quality of life was mostly affected by pain and discomfort in all groups. Sixty-two patients (78%) were satisfied or very satisfied with the results of surgery and 8 (11%) were unsatisfied or very unsatisfied. Up to 71 patients (90%) would consent to surgery again. Conclusion: In CM-I patients, PFD offers symptom improvement in about two-thirds of patients with high patient satisfaction. Symptom improvement is significantly higher in patients without associated syringomyelia, but patient satisfaction is similar. Symptom worsening is more frequent in the adult than in the pediatric population, with similar rates of postoperative improvement and patient satisfaction.

Incorporating patient-centered quality-of-life measures for outcome assessment after Chiari malformation type I decompression in a pediatric population: a pilot study

2021 ◽  
pp. 1-8
Author(s):  
Solomiia Savchuk ◽  
Michael C. Jin ◽  
Stephanie Choi ◽  
Lily H. Kim ◽  
Jennifer L. Quon ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Outcome Assessment ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Patient Centered ◽  
Chiari Malformation Type I ◽  
Radiographic Findings ◽  
Patient Reported

OBJECTIVE Optimal management of pediatric Chiari malformation type I (CM-I) is much debated, chiefly due to the lack of validated tools for outcome assessment, with very few tools incorporating patient-centered measures of health-related quality of life (HRQOL). Although posterior fossa decompression (PFD) benefits a subset of patients, prediction of its impact across patients is challenging. The primary aim of this study was to investigate the role of patient-centered HRQOL measures in the assessment and prediction of outcomes after PFD. METHODS The authors collected HRQOL data from a cohort of 20 pediatric CM-I patients before and after PFD. The surveys included assessments of selected Patient-Reported Outcomes Measurement Information System (PROMIS) health domains and were used to generate the PROMIS preference (PROPr) score, which is a measure of HRQOL. PROMIS is a reliable standardized measure of HRQOL domains such as pain, fatigue, depression, and physical function, which are all relevant to CM-I. The authors then compared the PROPr scores with Chicago Chiari Outcome Scale (CCOS) scores derived from time-matched clinical documentation. Finally, the authors used the PROPr scores as an outcome measure to predict postsurgical HRQOL improvement at 1 year on the basis of patient demographic characteristics, comorbidities, and radiological and physical findings. The Wilcoxon signed-rank test, Mann-Whitney U-test, and Kendall’s correlation were used for statistical analysis. RESULTS Aggregate analysis revealed improvement of pain severity after PFD (p = 0.007) in anatomical patterns characteristic of CM-I. Most PROMIS domain scores trended toward improvement after surgery, with anxiety and pain interference reaching statistical significance (p < 0.002 and p < 0.03, respectively). PROPr scores also significantly improved after PFD (p < 0.008). Of the baseline patient characteristics, preexisting scoliosis was the most accurate negative predictor of HRQOL improvement after PFD (median −0.095 vs 0.106, p < 0.001). A correlation with modest magnitude (Kendall’s tau range 0.19–0.47) was detected between the patient-centered measures and CCOS score. CONCLUSIONS The authors observed moderate improvement of HRQOL, when measured using a modified panel of PROMIS question banks, in this pilot cohort of pediatric CM-I patients after PFD. Further investigations are necessary to validate this tool for children with CM-I and to determine whether these scores correlate with clinical and radiographic findings.

scholarly journals Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature

2018 ◽  
Vol 22 (4) ◽  
pp. 426-438 ◽  
Author(s):  
Andrew C. Vivas ◽  
Nir Shimony ◽  
Eric M. Jackson ◽  
Risheng Xu ◽  
George I. Jallo ◽  
...  
Keyword(s):  
Literature Review ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
High Dose ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Dural Graft ◽  
Csf Diversion

OBJECTIVEHydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.METHODSA retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.RESULTSOver the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.CONCLUSIONSDe novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

scholarly journals Syringobulbia in pediatric patients with Chiari malformation type I

2018 ◽  
Vol 22 (1) ◽  
pp. 52-60 ◽  
Author(s):  
Arnold H. Menezes ◽  
Jeremy D. W. Greenlee ◽  
Brian J. Dlouhy
Keyword(s):  
Posterior Fossa ◽  
Cranial Nerve ◽  
Chiari Malformation ◽  
Fourth Ventricle ◽  
Pediatric Patients ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Presenting Symptoms ◽  
Cranial Nerve Palsies

OBJECTIVESyringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I–associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition.METHODSA prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded.RESULTSThe authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients.Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases.All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13.CONCLUSIONSThe incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.

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

2015 ◽  
Vol 16 (5) ◽  
pp. 599-606 ◽  
Author(s):  
Benjamin C. Kennedy ◽  
Taylor B. Nelp ◽  
Kathleen M. Kelly ◽  
Michelle Q. Phan ◽  
Samuel S. Bruce ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Symptom Improvement ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Motor Weakness ◽  
Dural Opening ◽  
Radiographic Improvement

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.

scholarly journals Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 25 (6) ◽  
pp. 629-639
Author(s):  
Andrew T. Hale ◽  
P. David Adelson ◽  
Gregory W. Albert ◽  
Philipp R. Aldana ◽  
Tord D. Alden ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Pediatric Patients ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Factors Associated ◽  
Younger Age ◽  
Age At Surgery ◽  
Spinal Segments ◽  
Research Consortium

OBJECTIVEFactors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.METHODSUsing the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.RESULTSThe authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16–0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34–1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12–0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02–2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31–3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67–5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.CONCLUSIONSThese data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

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

2011 ◽  
Vol 21 (6) ◽  
pp. 1143-1150 ◽  
Author(s):  
Tao Wu ◽  
Zezhang Zhu ◽  
Jian Jiang ◽  
Xin Zheng ◽  
Xu Sun ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I
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