scholarly journals The impact of scoliosis surgery on pulmonary function in spinal muscular atrophy: a systematic review

2021 ◽  
Author(s):  
Abduljabber Alhammoud ◽  
Yahya Othman ◽  
Ron El-Hawary ◽  
William G. Mackenzie ◽  
Jason J. Howard
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Primary Outcome ◽  
Muscular Atrophy ◽  
Scoliosis Surgery ◽  
Inclusion Criteria ◽  
Rate Of Decline ◽  
The Impact ◽  
Function Testing

AbstractScoliosis often occurs coincident with pulmonary function deterioration in spinal muscular atrophy but a causal relationship has not yet been reliably established. A systematic literature review was performed, with pulmonary function testing being the primary outcome pre- and post-scoliosis surgery. Levels of evidence were determined and GRADE recommendations made. Ninety studies were identified with only 14 meeting inclusion criteria. Four studies were level III and the rest were level IV evidence. The average age at surgical intervention was 11.8 years (follow-up 6.1 years). Post-operative pulmonary function progressively declined for the majority of studies. Otherwise, pulmonary function: improved (two studies), were unchanged (two studies), had a decreased rate of decline (three studies), declined initially then returned to baseline (two studies). Respiratory and spine-based complications were common. Given the available evidence, the following GRADE C recommendations were made: (1) surgery is most often associated with decreases in pulmonary function; (2) the impact of surgery on pulmonary function is variable, but does not improve over pre-operative baseline; (3) surgery may result in a decreased rate of decline in pulmonary function post-operatively. Given this lack of evidence-based support, the risk–benefit balance should be taken into consideration when contemplating scoliosis surgery.

The effect of scoliosis surgery on pulmonary function in spinal muscular atrophy type II patients

2016 ◽  
Vol 26 (6) ◽  
pp. 1721-1731 ◽  
Author(s):  
Shih-Hsiang Chou ◽  
Gau-Tyan Lin ◽  
Po-Chih Shen ◽  
Yi-Jing Lue ◽  
Cheng-Chang Lu ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Scoliosis Surgery ◽  
Type Ii ◽  
Spinal Muscular Atrophy Type

Impact of scoliosis surgery on pulmonary function in patients with muscular dystrophies and spinal muscular atrophy

2020 ◽  
Vol 55 (4) ◽  
pp. 1037-1042
Author(s):  
Harold J. Farber ◽  
William A. Phillips ◽  
Kenneth L. Kocab ◽  
Darrell S. Hanson ◽  
John A. Heydemann ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Muscular Dystrophies ◽  
Scoliosis Surgery

scholarly journals Newborn screening for spinal muscular atrophy in Germany: clinical results after 2 years

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Katharina Vill ◽  
Oliver Schwartz ◽  
Astrid Blaschek ◽  
Dieter Gläser ◽  
Uta Nennstiel ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Newborn Screening ◽  
Muscular Atrophy ◽  
Neurodevelopmental Outcome ◽  
Specific Treatment ◽  
Time Interval ◽  
Short Time Interval ◽  
Relevant Parameter ◽  
The Impact

Abstract Background Spinal muscular atrophy (SMA) is the most common neurodegenerative disease in childhood. Since motor neuron injury is usually not reversible, early diagnosis and treatment are essential to prevent major disability. Our objective was to assess the impact of genetic newborn screening for SMA on outcome. Methods We provided clinical data from 43 SMA patients, identified via polymerase chain reaction of the SMN1 gene from dried blood spots between January 2018 and January 2020 in Germany. Follow-up included neurophysiological examinations and standardized physiotherapeutic testing. Results Detection of SMA with newborn screening was consistent with known incidence in Germany. Birth prevalence was 1:6910; 39.5% had 2 SMN2 copies, 23% had 3 SMN2 copies, 32.5% had 4 copies, and 4.5% had 5 copies of the SMN2 gene. Treatment with SMA-specific medication could be started at the age of 14–39 days in 21 patients. Pre-symptomatically treated patients remained throughout asymptomatic within the observation period. 47% of patients with 2 SMN2 copies showed early, presumably intrauterine onset of disease. These patients reached motor milestones with delay; none of them developed respiratory symptoms. Untreated children with 2 SMN2 copies died. Untreated children with 3 SMN2 copies developed proximal weakness in their first year. In patients with ≥ 4 SMN2 copies, a follow-up strategy of “watchful waiting” was applied despite the fact that one of them was treated from the age of 6 months. Two infant siblings with 4 SMN2 copies were identified with a missed diagnosis of SMA type 3. Conclusion Identification of newborns with infantile SMA and prompt SMA-specific treatment substantially improves neurodevelopmental outcome, and we recommend implementation in the public newborn screening in countries where therapy is available. Electrophysiology is a relevant parameter to support the urgency of therapy. There has to be a short time interval between a positive screening result and referral to a therapy-ready specialized treatment center.

The impact of statin therapy after surgical or endovascular treatment of cerebral aneurysms

2020 ◽  
Vol 133 (1) ◽  
pp. 182-189
Author(s):  
Tae-Jin Song ◽  
Seung-Hun Oh ◽  
Jinkwon Kim
Keyword(s):  
Statin Therapy ◽  
Coil Embolization ◽  
Primary Outcome ◽  
Cox Regression ◽  
Cerebral Aneurysms ◽  
Time Dependent ◽  
Lipid Lowering ◽  
Surgical Clipping ◽  
The Impact

OBJECTIVECerebral aneurysms represent the most common cause of spontaneous subarachnoid hemorrhage. Statins are lipid-lowering agents that may expert multiple pleiotropic vascular protective effects. The authors hypothesized that statin therapy after coil embolization or surgical clipping of cerebral aneurysms might improve clinical outcomes.METHODSThis was a retrospective cohort study using the National Health Insurance Service–National Sample Cohort Database in Korea. Patients who underwent coil embolization or surgical clipping for cerebral aneurysm between 2002 and 2013 were included. Based on prescription claims, the authors calculated the proportion of days covered (PDC) by statins during follow-up as a marker of statin therapy. The primary outcome was a composite of the development of stroke, myocardial infarction, and all-cause death. Multivariate time-dependent Cox regression analyses were performed.RESULTSA total of 1381 patients who underwent coil embolization (n = 542) or surgical clipping (n = 839) of cerebral aneurysms were included in this study. During the mean (± SD) follow-up period of 3.83 ± 3.35 years, 335 (24.3%) patients experienced the primary outcome. Adjustments were performed for sex, age (as a continuous variable), treatment modality, aneurysm rupture status (ruptured or unruptured aneurysm), hypertension, diabetes mellitus, household income level, and prior history of ischemic stroke or intracerebral hemorrhage as time-independent variables and statin therapy during follow-up as a time-dependent variable. Consistent statin therapy (PDC > 80%) was significantly associated with a lower risk of the primary outcome (adjusted hazard ratio 0.34, 95% CI 0.14–0.85).CONCLUSIONSConsistent statin therapy was significantly associated with better prognosis after coil embolization or surgical clipping of cerebral aneurysms.

scholarly journals Hybrid versus Total Sublaminar Wires in Patients With Spinal Muscular Atrophy Undergoing Scoliosis Surgery

2021 ◽  
Author(s):  
Shih-Hsiang Chou ◽  
Wen-Wei Li ◽  
Cheng-Chang Lu ◽  
Kun-Ling Lin ◽  
Sung-Yen Lin ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Pedicle Screw ◽  
Muscular Atrophy ◽  
Deformity Correction ◽  
Vertebral Rotation ◽  
Major Curve ◽  
Apical Vertebral Rotation ◽  
Hybrid Instrumentation ◽  
Hybrid Construct

Abstract BackgroundEarly versions of spinal muscular atrophy (SMA) scoliosis correction surgeries often involved sublaminar devices. Recently the utilization of pedicle screw is gaining much popularity. Pedicle screw generally believed to provide additional deformity correction, but pedicle size and rotational deformity limit the application of pedicle screw in the thoracic spine, resulting in a hybrid construct of the pedicle screw and sublaminar wire. Studies of the efficacy of hybrid instrumentation in SMA scoliosis is often limited by the scarcity of the disease itself. In this study, we aimed to compare the surgical outcome of using hybrid constructs of the pedicle screw and sublaminar wire and that of sublaminar wire alone in patients with SMA scoliosis.MethodsWe retrospectively reviewed the clinical records and radiographic assessments of patients with SMA scoliosis who underwent corrective surgery between 1993 and 2015. The radiographic assessments included the deformity correction and the progressive change of major curve angle, pelvic tilt (PT) and coronal balance (CB). The correction of deformities was observed postoperatively and at the patient’s 2-year follow-up to test the efficacy of each type of constructs.ResultsThirty-three patients were included in this study. There were 14 and 19 patients in the wiring and the hybrid construct groups, respectively. The hybrid construct demonstrated a higher major curve angle correction (50.5° ± 11.2° vs. 36.4° ± 8.4°, p < 0.001), a higher apical vertebral rotation correction (10.6° ± 3.9° vs. 4.8° ± 2.6°, p < 0.001), and reduced the progression of major curve angle after the 2-year follow-up (5.1° ± 2.9° vs. 8.7° ± 4.8°, p < 0.001). A moderate correlation was observed between the magnitude of correction of apical vertebral rotation angle and major curve (r = 0.528, p = 0.002).ConclusionThis study demonstrated that hybrid instrumentation can provide a greater magnitude of correction in major curve and apical rotation, as well as less major curve progression in comparison with sublaminar wire in patients with SMA scoliosis.Level of evidence III

Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III

2003 ◽  
Vol 39 (9) ◽  
pp. 673-676 ◽  
Author(s):  
SY Chng ◽  
YQ Wong ◽  
JH Hui ◽  
HK Wong ◽  
HT Ong ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy

Abstract 17492: Extent and Pattern of Late Gadolinium Enhancement Predict Arrhythmic Events in Patients With Nonischemic Dilated Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Dong Geum Shin ◽  
Hye-Jeong Lee ◽  
Junbeom Park ◽  
Young Jin Kim ◽  
Jae-Sun Uhm ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Ventricular Arrhythmia ◽  
Dilated Cardiomyopathy ◽  
Cardiac Death ◽  
Primary Outcome ◽  
Multivariable Analysis ◽  
Gadolinium Enhancement ◽  
Nonischemic Dilated Cardiomyopathy ◽  
The Impact

Background: Late gadolinium enhancement (LGE) by cardiac MR (CMR) has been related to adverse clinical outcomes in patients with nonischemic dilated cardiomyopathy (NIDC). But, a statistically significant association between LGE and arrhythmic risk in NIDC has not been demonstrated consistently. This study evaluated the impact of the presence, location and pattern of LGE on arrhythmic risk prediction in NICM. Methods: This study included 365 patients (54±15years) with NICM who underwent CMR. The extent, location and pattern of LGE were categorized. We analyzed for the primary outcome of ventricular arrhythmia (VA) including sustained or nonsustained ventricular tachycardia (VT), appropriate implantable cardioverter-defibrillator (ICD) intervention and ventricular fibrillation (VF). Cardiac death and hospitalization for heart failure (HF) were evaluated as secondary outcomes. Results: LGE was seen in 267 (73 %) patients. During median follow-up of 44±36 months, patients with LGE had higher incidence of cardiac death (15 % vs. 2 %, p<0.001), hospitalization for HF (40 % vs. 15 %, p<0.001) and VA (14% vs. 6%, p=0.03). In multivariable analysis, the presence of LGE (HR 2.78; 95% CI 1.10-7.02; p=0.03) was the independent predictor of arrhythmias. Patients with extensive LGE had higher VA (32% vs. 10%, p<0.001) with lower cumulative survival free of VA than those without extensive LGE (p=0.001). The frequent LGE location was as follows: LV septum 64%, LV-RV junction 42% and inferior 10%. VA was lower in patients with than without localized LGE limited to LV-RV junction (21% vs. 46%, p=0.005). Interestingly, while the incidence of ventricular arrhythmia was higher in patients with transmural LGE (29% vs. 10%, p=0.003), it was lower in those with patch LGE (2% vs. 16%, p=0.02) than the other patients. Conclusions: In patients with NICM, the LGE was an independent prognostic predictor of VA. Extensive LGE and specific location of LGE was related with the arrhythmic events.

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