Radiographic and Functional Outcome After Surgical Management of Severe Scoliosis in Skeletally Immature Patients with Muscular Dystrophy

Kim, Hak-Sun; Park, Jin-Oh; Lee, Hwan Mo; Shin, Dong-Eun; Ha, Jung-Won; Shim, Dong-Jun; Lee, Kwang

doi:10.1097/01.bsd.0000133063.43337.c8

Cited by 7 publications

(3 citation statements)

References 20 publications

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“…Currently, spinal correction and fusion surgery is the mainstay of scoliosis treatment in patients with DMD. The advantages of surgery include increased comfort and sitting tolerance [ 10 12 23 ], increased upper extremity function [ 23 24 ], easier nursing care by parents [ 12 ], increased QOL [ 8 11 12 ], cosmetic improvement, and higher self-image [ 12 13 ].…”

Section: Discussionmentioning

confidence: 99%

Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy

et al. 2017

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Study DesignRetrospective cohort study.PurposeTo investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD).Overview of LiteratureSeveral studies have reported that scoliosis correction in patients with DMD does not improve pulmonary function. In these studies, pulmonary function was evaluated using the traditional spirometric values of percent vital capacity (%VC) and percent forced vital capacity (%FVC). However, traditional spirometry may not be suitable for patients with DMD because the results can be influenced by patient fatigue or level of understanding. Therefore, we evaluated respiratory function focusing on respiratory muscle strength using maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP), in addition to %VC and %FVC.MethodsWe retrospectively reviewed 16 patients with DMD who underwent spinal correction surgery between 2006 and 2011 at Kitasato University Hospital. All patients were males, and the mean age was 13.5 years. Respiratory muscle strength was evaluated using MIP, MEP, and SNIP. Measurements were obtained preoperatively and at 1 and 6 months postoperatively, and %VC and %FVC were obtained preoperatively and within 6 months postoperatively.ResultsThe mean preoperative and postoperative %VC values were 54.0% and 51.7%, whereas the mean %FVC values were 53.9% and 53.2%, respectively. The mean MIP, MEP, and SNIP values obtained preoperatively and at 1 and 6 months postoperatively were as follows: MIP, 40.5, 42.7 and 47.2 cm H2O; MEP, 26.0, 28.0, and 29.0 cm H2O; and SNIP, 33.4, 33.0, and 33.0 cm H2O; respectively. The mean MIP and MEP values significantly improved postoperatively. There were no significant differences in SNIP, %VC, or %FVC preand postoperatively.ConclusionsBy focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function.

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Section: Discussionmentioning

confidence: 99%

Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy

et al. 2017

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“…Severe pelvic obliquity follows, and is caused by general muscle weakness leading to a general imbalance of the trunk 5 . At this stage, vertebral body collapse may occur making sitting difficult and limiting the use of the upper extremities as the patient has to use both arms to maintain the body in an upright position 6 . The restrictive respiratory pattern caused by the skeletal changes leads to further respiratory muscle weakness, ineffective cough mechanisms, mucus plugging, and recurrent pneumonia 7 .…”

Section: Introductionmentioning

confidence: 99%

Radiographic Features of Golden Retriever Muscular Dystrophy

Brumitt

Essman

Kornegay

et al. 2006

Vet Radiology Ultrasound

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Golden Retriever muscular dystrophy is an inherited, degenerative myopathy due to the absence of dystrophin and is used as a model of Duchenne muscular dystrophy of young boys. This report describes the radiographic abnormalities of Golden Retriever muscular dystrophy in 26 dogs. The thoracic abnormalities included diaphragmatic asymmetry (18/26), diaphragmatic undulation (18/26), and gastro-esophageal hiatal hernia (6/26). Pelvic abnormalities included narrowing of the body of the ilia (14/19), ventral deviation and curvature of the tuber ischii (14/19), elongation of the obturator foramen with a decrease in opacity of the surrounding bone (12/19), and lateral flaring of the wings of the ilia (12/19). Abdominal abnormalities consisted of hepatomegaly (14/22) and poor serosal detail (12/22). The unique thoracic abnormalities were a consistent finding in affected Golden Retriever muscular dystrophy dogs. The diagnosis of muscular dystrophy should be included in the differential list if the combination of diaphragm undulation and asymmetry, and gastro-esophageal hiatal hernia are identified. These diaphragmatic abnormalities are related to hypertrophy and hyperplasia of the diaphragm. Additionally, the skeletal changes of pelvic tilt, elongation of the pelvis, widening of the obturator foramina and thinning of the ischiatic tables appear to be specific to Golden Retriever muscular dystrophy in dogs. These pelvic abnormalities are most likely secondary to bone remodeling associated with the progressive skeletal myopathy and subsequent contracture/fibrosis.

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“…The surgical intervention may provide good spinal curve correction, but the procedure may not be justified for routine cases when considering the risks of significant complications for these patients. 15,17 For Cobb angles less than 40°, nonsurgical methods, such as the use of spinal braces and special seating, can serve as a viable alternative. 10 These methods have focused mainly on the orthotic management of the scoliosis.…”

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confidence: 99%

Effects of Lateral Trunk Support on Scoliotic Spinal Alignment in Persons With Spinal Cord Injury: A Radiographic Study

Mao

Huang

et al. 2006

Archives of Physical Medicine and Rehabilitation

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Radiographic and Functional Outcome After Surgical Management of Severe Scoliosis in Skeletally Immature Patients with Muscular Dystrophy

Cited by 7 publications

References 20 publications

Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy

Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy

Radiographic Features of Golden Retriever Muscular Dystrophy

Effects of Lateral Trunk Support on Scoliotic Spinal Alignment in Persons With Spinal Cord Injury: A Radiographic Study

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