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Neuromuscular Scoliosis
Published in Kelechi Eseonu, Nicolas Beresford-Cleary, Spine Surgery Vivas for the FRCS (Tr & Orth), 2022
Kelechi Eseonu, Nicolas Beresford-Cleary
Neuromuscular scoliosis may be caused by a number of disorders of the brain, spinal cord and muscular system (e.g. CP, spinal muscular atrophy). These curves tend to be long c-shaped curves, are associated with pelvic obliquity and progress more quickly than adolescent idiopathic curves. They are at higher risk of operative and perioperative complications.
Weaver Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Management of Weaver syndrome requires multidisciplinary approaches (including pediatric, orthopedic, neurological, and cardiological care). Learning/behavior/speech assessment and support are indicated for affected patients showing developmental delay and/or learning disability. Surgical intervention may be required for those with toe camptodactyly. Physiotherapy may benefit those experiencing joint pain secondary to ligamentous laxity or joint contractures, or those with abnormal muscle tone. Treatment is routinely prescribed for those with scoliosis. Other measures may be undertaken when appropriate [5].
Klippel–Trenaunay syndrome and complex venous malformations: Should multimodality approach be standard of care?
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
All patients with KTS should receive a comprehensive orthopedic assessment. Limb length and circumference measurements will be required and should be repeated periodically. The spine needs to be assessed for scoliosis.
Does exercise therapy improve pulmonary function in patients with Adolescent Idiopathic Scoliosis?
Published in Physiotherapy Theory and Practice, 2023
Anthony Rafferty, Neil Fleming, Patrick Kiely, David Mockler, Sara Dockrell
Physiotherapeutic scoliosis-specific exercise programs are frequently used in the management of spinal curve progression in scoliosis (Berdishevsky et al., 2016; Negrini et al., 2018). Despite the methodological variability, all 12 studies in this review that included a PSSE intervention reported significant improvements in FVC or VC. A number of studies postulated that improvement in pulmonary function following a PSSE program was related to addressing the abnormal breathing patterns that occur in patients with scoliosis because of reduced chest expansion and restricted rib mobility (Borysov and Borysov, 2012; Moramarco et al., 2016). Weiss (1991) also proposed the concept of abnormal chest cage mechanics leading to dysfunctional breathing. Interestingly, Xavier, Avanzi, de Carvalho, and Alves (2020) who included resistance exercises in their intervention group found that participants with severe AIS had better outcomes when aerobic exercises were combined with anaerobic workouts. Similar findings have also been found in other non-AIS-based cohorts (Khosravi, Tayebi, and Safari, 2013).
Locoregional lung ventilation distribution in girls with adolescent idiopathic scoliosis and healthy adolescents. The immediate effect of Schroth ‘derotational breathing’ exercise in a controlled-trial
Published in Physiotherapy Theory and Practice, 2023
Mercedes David, Maxime Raison, Stéphanie Paul, Olivier Cartiaux, Christine Detrembleur, Philippe Mahaudens
Some authors have advanced the interdependency between the severity of scoliosis and ventilation parameters (Tsiligiannis and Grivas, 2012). To others, a relationship exists between these latter ventilation parameters and rib cage deformations (Leong, Lu, Luk, and Karlberg, 1999). These considerations do not rule out the possibility of asymmetric locoregional lung ventilation distribution in patients with more severe AIS. As our results focused on mild-to-moderate scoliosis, future studies should be conducted in patients with more severe AIS to assess the effect of derotational breathing on the severity of scoliosis. Additionally, considering that the greater the vertebral rotation is, the more pronounced the rib cage rotation (Closkey and Schultz, 1993), one avenue for future research may also be whether the effect of derotational breathing on locoregional lung ventilation distribution may be inherent to the relevance of the parameter considered to evaluate scoliosis severity (i.e. Cobb angle versus the apical vertebral rotation) (Perdriolle and Vidal, 1985).
Assessing diagnosis and managing respiratory and cardiac complications of sarcoglycanopathy
Published in Expert Opinion on Orphan Drugs, 2020
Corrado Angelini, Valentina Pegoraro
Respiratory involvement was found in 26% of the cases that required ventilatory support, with the treatment recommended at a mean age of 29.1 years. Between the different subgroups, it was not identified a difference in the frequency of patients requiring ventilator support nor in the age at which it was recommended. A practical indication of nocturnal mechanical ventilation is represented by the presence of clinical signs of hypoxemia, such as morning headache, sleep abnormality, dyspnea lying flat, and FVC below 50%. However, patients requiring ventilator support had a significant longer duration of the disease. When a specific analysis was conducted on the potential influence of scoliosis in the need of ventilator support: 69.1% of the patients requiring ventilation had scoliosis, while only 31.9% that did not require ventilation support had scoliosis [16]. These differences were statistically significant. Additionally, it was observed a nonsignificant trend of starting the ventilation earlier for patients with scoliosis compared to those patients without scoliosis. When the upper airway musculature is affected, speech and swallowing difficulties and dysphagia start to develop. Snoring, apneic episodes, and daytime somnolence point to the possibility of obstructive sleep apnea. If patients under ventilation at night, the resultant hypercapnia may cause early‐morning headache, reduced attention, and concentration with clouded consciousness [17,18].