Longitudinal scoliosis behavior in Chiari malformation with and without syringomyelia

Author(s):  
Silky Chotai ◽  
Jeffrey L. Nadel ◽  
Katherine G. Holste ◽  
James M. Mossner ◽  
Brandon W. Smith ◽  
...  

OBJECTIVE The objective of this study was to understand the natural history of scoliosis in patients with Chiari malformation type I (CM-I) with and without syringomyelia. METHODS A retrospective review of data was conducted. Patients with CM-I were identified from a cohort of 14,118 individuals age 18 years or younger who had undergone MRI over an 11-year period at the University of Michigan. Patients eligible for study inclusion had a coronal curve ≥ 10° on radiography, associated CM-I with or without syringomyelia, and at least 1 year of clinical follow-up prior to any surgery. Curve magnitude at initial diagnosis, prior to posterior fossa decompression (PFD; if applicable), and at the last follow-up (prior to any surgical correction of scoliosis) was recorded, and clinical and radiographic characteristics were noted. The change in curve magnitude by 10° was defined as curve progression (increase by 10°) or regression (decrease by 10°). RESULTS Forty-three patients met the study inclusion criteria and were analyzed. About one-third (35%) of the patients presented with symptoms attributed to their CM-I. The mean degree of scoliosis at presentation was 32.6° ± 17.7°. Twenty-one patients (49%) had an associated syrinx. The mean tonsil position below the level of the foramen magnum was 9.8 ± 5.8 mm. Patients with a syrinx were more likely to have a curve > 20° (86% vs 41%, p = 0.002). Curve magnitude remained stable (≤ ±10°) in 77% of patients (33/43), progressed in 16% (7/43), and regressed in 7% (3/43). Mean age was higher (14.8 ± 0.59 years) among patients with regressed curves (p = 0.026). All regressed curves initially measured ≤ 20° (mean 14° ± 5.3°), and none of the patients with regressed curves had a syrinx. The change in curve magnitude was statistically similar in patients with (7.32° ± 17.7°) and without (5.32° ± 15.8°) a syrinx (p = 0.67). After a mean follow-up of 3.13 ± 2.04 years prior to surgery, 27 patients (63%) ultimately underwent posterior fossa or scoliosis correction surgery. For those who eventually underwent PFD only, the rate of change in curve magnitude prior to surgery was 0.054° ± 0.79°. The rate of change in curve magnitude was statistically similar before (0.054° ± 0.79°) and after (0.042° ± 0.33°) surgery (p = 0.45) for patients who underwent PFD surgery only. CONCLUSIONS The natural history of scoliosis in the presence of CM-I is variable, though most curves remained stable. All curves that regressed were ≤ 20° at initial diagnosis, and most patients in such cases were older at scoliosis diagnosis. Patients who underwent no surgery or PFD only had similar profiles for the change in curve magnitude, which remained relatively stable overall, as compared to patients who underwent PFD and subsequent fusion, who demonstrated curve progression. Among the patients with a syrinx, no curves regressed, most remained stable, and some progressed. Understanding this variability is a first step toward building a prediction model for outcomes for these patients.

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.


2010 ◽  
Vol 5 (2) ◽  
pp. 162-166 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Cormac O. Maher ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton

Object The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding. Methods The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval. Results The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics. Conclusions Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.


2014 ◽  
Vol 80 (3) ◽  
pp. 245-252 ◽  
Author(s):  
Charles F. Bellows ◽  
Celia Robinson ◽  
Robert J. Fitzgibbons ◽  
Larry S. Webber ◽  
David H. Berger

Ventral hernias are a common clinical problem. Immediate repair is recommended for most ventral hernias despite significant recurrence rates. This practice may be related to a lack of understanding of the natural history of ventral hernias. The purpose of this study was to determine the natural history of ventral hernias and to determine if watchful waiting is an acceptable and safe option. Forty-one patients with ventral hernias were enrolled in a longitudinal cohort study of watchful waiting. Primary outcomes were functional impairment resulting from hernia disease as measured by the Activities Assessment Scale (AAS) and changes from baseline to two years in the physical and mental component score of the SF-36 Health Survey. Secondary outcomes included complications such as incarceration. Mixed-effects model for repeated measures and Student's t tests were used to evaluate scale performance. The mean age of enrollees was 64 years, and the mean hernia size was 239 cm2. Eleven patients were lost to follow-up, and seven patients died of other causes. All remaining patients were followed for two years. There was one incarceration during the follow-up period. There was no deterioration in the AAS score (baseline vs 24 months = 28 vs 25, P = 0.60). There was deterioration of the physical functioning dimension of the SF-36 (baseline vs 24 months = 40 vs 32, P < 0.01), but the mental functioning dimension was improved (45 vs 51; P = 0.01). Watchful waiting was a safe option for patients in this study with ventral hernias.


Neurosurgery ◽  
2007 ◽  
Vol 60 (5) ◽  
pp. 865-872 ◽  
Author(s):  
Siddharth Kharkar ◽  
John Shuck ◽  
James Conway ◽  
Daniele Rigamonti

Abstract OBJECTIVE The presentation and natural history of untreated, symptomatic intramedullary spinal cavernomas at our institution were analyzed. The objective is to provide additional information regarding the natural history of conservatively managed, symptomatic, intramedullary spinal cord cavernous malformations. METHODS The medical records of patients treated in our institution between 1989 and 2002 were reviewed to identify those with intramedullary cavernomas. The medical, radiological, surgical, and pathological records from these patients were retrospectively reviewed and analyzed. RESULTS Fourteen patients were included in the study. The mean age at presentation was 42 years. Four lesions (29%) were located in the cervical region and 10 lesions (71%) were present in the thoracolumbar spinal cord. All patients were symptomatic at the time of presentation. In this cohort of 14 patients, 10 patients (71%) were conservatively managed. For these patients, the mean duration of symptoms before presentation was 10 months. The mean duration of follow-up from the time of presentation was 80 months. The median McCormick grade for conservatively treated patients at presentation was II. During this period, none of the conservatively managed patients had an acute intramedullary bleed. In nine patients, the McCormick grade at the last follow-up evaluation was the same as or better than their score at presentation. Four patients (29%) were treated surgically. The mean duration of symptoms before presentation was 33 months. The mean duration of follow-up from the time of presentation was 42 months. In two surgical patients, the McCormick grade at the last follow-up evaluation remained unchanged compared with their score at presentation, whereas the McCormick grade improved in one patient and deteriorated in another patient. CONCLUSION This cohort of conservatively managed patients with symptomatic, intramedullary spinal cord cavernomas was clinically stable throughout the follow-up period. In this series, patients harboring symptomatic spinal cord cavernous malformation did not have significant, permanent neurological decline during the follow-up period when treated with the conservative approach of observation. This data provides additional information for determining the appropriate treatment strategy for patients with intramedullary spinal cavernomas.


2013 ◽  
Vol 118 (1) ◽  
pp. 43-46 ◽  
Author(s):  
Kelly D. Flemming ◽  
Michael J. Link ◽  
Teresa J. H. Christianson ◽  
Robert D. Brown

Object The goal of this study was to determine the risk of using antithrombotic agents in patients with established intracerebral cavernous malformations (ICMs). Methods From a previously described cohort of 292 patients with radiographically defined ICMs, 40 required an antithrombotic after the ICM was diagnosed. Patients underwent follow-up to determine the incidence of hemorrhage. Results The mean age of these 40 patients was 62.4 years; there were 21 male and 19 female patients. Five (12.5%) of the 40 patients initially presented with hemorrhage and 4 (10%) had multiple ICMs. Of these patients, 32 were placed on an antiplatelet agent alone, 6 on an anticoagulant alone, and 2 were placed on both. In patients necessitating any antithrombotic agent, 1 patient developed a prospective hemorrhage over the 258 person-years of follow-up (prospective hemorrhage rate 0.41% per person-year). Conclusions Antithrombotics likely do not precipitate hemorrhage in patients with known ICMs. However, caution should be exercised in the use of antithrombotics in patients with ICMs at high risk for hemorrhage. The risks and benefits of antithrombotics in each situation should be carefully weighed against the natural history of ICM.


1999 ◽  
Vol 91 (3) ◽  
pp. 364-369 ◽  
Author(s):  
Bruce E. Pollock ◽  
John Huston

Object. To determine the natural history of colloid cysts of the third ventricle in patients in whom the cysts were incidentally discovered, the authors retrospectively reviewed cases observed during the modern neuroimaging era (1974–1998).Methods. During this 25-year interval, 162 patients with colloid cysts were examined and cared for at our center. Sixty-eight patients (42%) were thought to be asymptomatic with regard to their colloid cyst and observation with serial neuroimaging was recommended. The mean patient age was 57 years at the time of diagnosis (range 7–88 years) and the mean cyst size was 8 mm (range 4–18 mm). Computerized tomography scanning revealed a hyperdense cyst in 49 (84%) of 58 patients. Three patients were excluded from the study because they died of unrelated causes within 6 months of scanning and seven patients were lost to follow-up review. Clinical follow-up evaluation was available at a mean of 79 months (range 7–268 months) in the remaining 58 patients. The numbers of patients who participated in follow-up review at 2, 5, and 10 years after diagnosis were 40, 28, and 14, respectively. The incidences of symptomatic progression related to the cyst were 0%, 0%, and 8% at 2, 5, and 10 years, respectively. No patient died suddenly during the follow-up interval. Two (6%) of 34 patients in whom follow-up imaging was performed either exhibited cyst growth (one patient) or experienced hydrocephalus (one patient) at a mean of 41 months after diagnosis (range 4–160 months).Conclusions. Patients in whom asymptomatic colloid cysts are diagnosed can be cared for safely with observation and serial neuroimaging. If a patient becomes symptomatic, the cyst enlarges, or hydrocephalus develops, prompt neurosurgical intervention is necessary to prevent the occurrence of neurological decline from these benign tumors.


1996 ◽  
Vol 2 (2) ◽  
pp. 127-135 ◽  
Author(s):  
R. Willinsky ◽  
W. Harper ◽  
M.C. Wallace ◽  
W. Kucharczyk ◽  
W. Montanera ◽  
...  

The purpose of this review was to determine whether a classification scheme for cavernomas based on the MR appearance is predictive of the natural history of the lesions with particular reference to the risk of haemorrhage. We reviewed the clinical and MR findings in 156 patients with intracranial cavernomas. The dominant or symptomatic cavernoma was graded into three groups according to the appearance of the blood products. The presence of haemorrhage, mass effect and oedema was evaluated. Sixty patients had serial MRs with a mean follow-up of 1.9 years. Subacute haemorrhage with mass effect and oedema was evident on the initial MR in 30 patients (19%). In seven patients follow-up MR showed haemorrhage with mass effect and oedema. Six of these bleeds were in the posterior fossa. The haemorrhages seen on follow-up MR occurred in all three morphological types. Regression was found on follow-up MR in five of the seven patients who initially showed a bleed. In 35% of patients, the dominant or symptomatic cavernoma changed on serial MR. Intracranial cavernomas are dynamic lesions with both active and regressive changes seen on follow-up MR. A classification system based on morphology was not found to be useful in predicting future bleeds. Bleeding rates, based on MR follow-up, were 10.9% in the posterior fossa and 1.7% in the supratentorial compartment.


2001 ◽  
Vol 120 (5) ◽  
pp. A128-A128 ◽  
Author(s):  
H MALATY ◽  
D GRAHAM ◽  
A ELKASABANY ◽  
S REDDY ◽  
S SRINIVASAN ◽  
...  

Author(s):  
J. Terrence Jose Jerome

Abstract Background The natural history of scaphoid nonunion is the development of degenerative arthritis. A lot of information is still unclear about this progression. The purpose of this study is to analyze patients with scaphoid nonunions who had not received any kind of treatment and to assess the functional outcome. Materials and Methods This is a retrospective study that analyzed the patients with chronic scaphoid nonunions between 2009 and 2019. None of the patients received any treatment. The age at the time of injury, examination, pattern of fracture, types of scaphoid nonunion, symptoms, and duration of nonunion were noted. Diagnosis was confirmed by radiographs, computed tomography (CT) scan, and magnetic resonance imaging (MRI). Scapholunate and radiolunate angles were recorded. Pain score, modified mayo wrist score, grip strength, range of movement, and the functional outcome of these scaphoid nonunions were analyzed. A statistical correlation between the scaphoid nonunion presentations and the functional outcome was assessed. Results The mean age of the patients was 62 years (range: 35–82 years.). There were 17 male and 3 female patients. There were 9 waist and 11 proximal pole scaphoid nonunions. The mean duration of scaphoid nonunion was 34 years (range: 10–62 years). None of the patients had avascular necrosis (AVN) of the proximal scaphoid. The age at examination, gender, side of injury, fracture pattern (waist/proximal pole), fracture displacement ≤ 1 mm or > 1 mm, nonunion duration, and radiographic arthritic parameters had no significant impact on the functional outcome. Conclusions Untreated chronic scaphoid nonunion leads to the development of degenerative arthritis over a period of years, which is still unpredictable. Most of the patients become aware of the nonunion following a precedent injury or other reasons. Most of the patients have fair/good functional outcome despite reduced range of movements and grip strength. Many do not favor surgical intervention in the course of nonunion. Chronic nonunions open a lot of unanswered questions. Clinical relevance There have been numerous studies on the treatment aspects of scaphoid nonunion, with little knowledge about certain people with nonunion who did not have any kind of treatment. The demographics, clinical findings, and radiological parameters do confirm the progression of these nonunion to arthritis, but most of them had fair-to-good outcome throughout their life. It opens our thinking about the real need of treatment in such nonunions and raises numerous questions about the disease. Level of evidence This is a Level IV study.


2019 ◽  
Author(s):  
Ayesha Shaikh ◽  
Natasha Shrikrishnapalasuriyar ◽  
Giselle Sharaf ◽  
David Price ◽  
Maneesh Udiawar ◽  
...  

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