Spinal Deformity Correction in Marfan Syndrome Versus Adolescent Idiopathic Scoliosis Learning From the Differences
ABSTRACT Retrospective case control study.
To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement.
Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS.
We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively.
The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate.
Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.
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ABSTRACT: The purpose of this article is to provide the pediatrician with a review of disorders that have the orthopedic manifestation of joint hypermobility. Hypermobility, also termed ligamentous laxity, may present in different parts of the body at different times throughout childhood and adolescence. It may be symptomatic or incidentally found on the physical examination. Many conditions that cause joint hypermobility resolve with nonoperative management, but occasionally operative intervention is required for the best patient outcome. In addition, hypermobility may be associated with collagen disorders that affect vital organ systems. Recognition of hypermobility combined with a thorough patient evaluation may be the initial opportunity for the pediatrician to uncover disease that may be managed promptly. Heightened awareness of subtle hypermobility or symptomatic joint laxity on physical examination facilitates optimal management and favorable outcomes in children with this condition.Current opinion in pediatrics 12/2013; 26(1). DOI:10.1097/MOP.0000000000000035 · 2.74 Impact Factor
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ABSTRACT: Scoliosis Research Society-22 (SRS-22) questionnaire was developed to evaluate health-related quality of life (HRQL) in adolescent idiopathic scoliosis (AIS) patients. Rasch analysis (RA) is a statistical procedure which turns questionnaire ordinal scores into interval measures. Measures from Rasch-compatible questionnaires can be used, similar to body temperature or blood pressure, to quantify disease severity progression and treatment efficacy. Purpose of the current work is to present Rasch analysis (RA) of the SRS-22 questionnaire and to develop an SRS-22 Rasch-approved short form. 300 SRS-22 were randomly collected from 2447 consecutive IS adolescents at their first evaluation (229 females; 13.9 ± 1.9 years; 26.9 ± 14.7 Cobb°) in a scoliosis outpatient clinic. RA showed both disordered thresholds and overall misfit of the SRS-22. Sixteen items were re-scored and two misfitting items (6 and 14) removed to obtain a Rasch-compatible questionnaire. Participants HRQL measured too high with the rearranged questionnaire, indicating a severe SRS-22 ceiling effect. RA also highlighted SRS-22 multidimensionality, with pain/function not merging with self-image/mental health items. Item 3 showed differential item functioning (DIF) for both curve and hump amplitude. A 7-item questionnaire (SRS-7) was prepared by selecting single items from the original SRS-22. SRS-7 showed fit to the model, unidimensionality and no DIF. Compared with the SRS-22, the short form scale shows better targeting of the participants’ population. RA shows that SRS-22 has poor clinimetric properties; moreover, when used with AIS at first evaluation, SRS-22 is affected by a severe ceiling effect. SRS-7, an SRS-22 7-item short form questionnaire, provides an HRQL interval measure better tailored to these participants.Research in developmental disabilities 04/2014; 35(4). DOI:10.1016/j.ridd.2014.01.020 · 4.41 Impact Factor
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ABSTRACT: Background There are a number of syndromes that have historically been associated with scoliosis e.g.: Marfan, Down, and Neurofibromatosis. These syndromes have been grouped together as one etiology of scoliosis, known as syndromic scoliosis. While multiple studies indicate that these patients are at high risk for perioperative complications, there is a paucity of literature regarding the collective complication rates and surgical needs of this population. Methods PubMed and Embase databases were searched for literature encompassing the surgical complications associated with the surgical management of patients undergoing correction of scoliosis in the syndromic scoliosis population. Following exclusion criteria, 24 articles were analyzed for data regarding these complications. Results The collective complication rates and findings of these articles were categorized based on specific syndrome. The rates and types of complications for each syndrome and the special needs of patients with each syndrome are discussed. Several complication trends of note were observed, including but not limited to the universally nearly high rate of wound infections (>5% in each group), high rate of pulmonary complications in patients with Rett syndrome (29.2%), high rate (>10%) of dural tears in Marfan and Ehlers-Danlos syndrome patients, high rate (>20%) of implant failure in Down and Prader-Willi syndrome patients, and high rate (>25%) of pseudarthrosis in Down and Ehlers-Danlos patients. Conclusions Though these syndromes have been classically grouped together under the umbrella term “syndromic,” there may be specific needs for patients with each of these ailments. Given the high rate of complications, further research is necessary to understand the unique needs for each of these patient groups in the preoperative, intraoperative, and postoperative settings.Scoliosis 04/2015; 10(1). DOI:10.1186/s13013-015-0035-x · 1.31 Impact Factor