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Sotos syndrome and scoliosis surgical treatment: a 10-year follow-up.

ABSTRACT: Sotos syndrome is caused by a gene deletion with an autosomal dominant pattern of inheritance. Cerebral gigantism, hypotonia and joint hyperextensibility are characteristic features of this syndrome. A percentage of these patients develop progressive scoliosis early in life. In the literature, few studies on the evolution of scoliosis in Sotos syndrome have been published. We retrospectively evaluated eight patients diagnosed with Sotos syndrome and scoliosis treated at the Garrahan Children Hospital between 1988 and March 2009. Clinical charts and imaging studies were assessed. Eight patients (19%) presented with scoliosis and seven of them (87.5%) required surgical treatment. The mean follow-up was 9.5 years (range 3-18). Mean age at first consultation was 5.2 years (range 1.1-11.2). Mean Cobb angle for scoliosis at first consultation was 34.3° (range 20°-42°) and the mean Cobb angle for kyphosis was 45.6° (range 30°-90°). Mean age at surgery was 11.2 years (range 3.7-18.10). The surgical procedures performed were instrumented posterior arthrodesis, alone or combined with anterior arthrodesis, instrumented anterior arthrodesis, while one patient is currently in treatment with growing rods. Preoperative mean Cobb angle for scoliosis was 72.3° (range 54°-130°) and for kyphosis was 59.8° (range 30°-108°); postoperative mean Cobb angle for scoliosis was 45.5° (range 6°-90°) and for kyphosis was 40.2° (range 30°-80°). There were three early complications (pleural effusion in two cases and death due to sepsis in one) and two late complications (kyphosis above the instrumentation area and dislodgement of the proximal hooks). Incidence of scoliosis in Sotos syndrome is high and thus close monitoring of patients with Sotos syndrome during growth is important for early detection of this entity. Joint hyperextensibility and hypotonia that are characteristic of the syndrome should be considered at the moment of surgery to avoid short fusions.

SUBMITTER: Corrado R

PROVIDER: S-EPMC3111518 | biostudies-literature | 2011 Jul

REPOSITORIES: biostudies-literature

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Sotos syndrome and scoliosis surgical treatment: a 10-year follow-up.

Corrado Romina R Wilson Alejandra Francheri AF Tello Carlos C Noel Mariano M Galaretto Eduardo E Bersusky Ernesto E

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 20110112

Sotos syndrome is caused by a gene deletion with an autosomal dominant pattern of inheritance. Cerebral gigantism, hypotonia and joint hyperextensibility are characteristic features of this syndrome. A percentage of these patients develop progressive scoliosis early in life. In the literature, few studies on the evolution of scoliosis in Sotos syndrome have been published. We retrospectively evaluated eight patients diagnosed with Sotos syndrome and scoliosis treated at the Garrahan Children Hos ...[more]

PMID: 21225435

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Project description:Study designA retrospective analysis of prospective, multicenter National Institute of Health clinical trial.ObjectiveThe aim of this study was to assess the rate of neurologic complications and impact of new neurologic deficits on 1-year postoperative patient-reported outcomes (PROs).Summary of background dataThere are limited studies evaluating the impact of new neurologic deficits on PROs following surgery for primary presentation adult lumbar scoliosis.MethodsPatients were divided into two groups: new postoperative neurological deficit (Def) or no deficit (NoDef). Preoperative and 1-year follow-up PROs were analyzed [Scoliosis Research Society (SRS) Questionnaire, Oswestry Disability Index (ODI), Short Form-12 Physical/Mental Health Composite Scores (PCS/MCS), and back/leg pain Numerical Rating Scale (NRS)].ResultsOne hundred forty-one patients: 14 Def (9.9%), 127 NoDef (90.1%). No differences were observed in demographic, radiographic, or PRO data between groups preoperatively. Def group had longer surgical procedures (8.3 vs. 6.9 hours, P = 0.030), greater blood loss (2832 vs. 2606 mL, P = 0.022), and longer hospitalizations (10.6 vs. 7.8 days, P = 0.004). NoDef group reported significant improvement in all PROs from preop to 1-year postoperative. Def group only had improvement in SRS Pain (2.7 preop to 3.4 postop, P = 0.037) and self-image domains (2.7 to 3.6, p = 0.004), and NRS back pain (6.6 to 3.2, P = 0.004) scores with significant worsening of NRS leg pain (4.1 to 6.1, P = 0.045). Group comparisons of 1-year postop PROs found that Def group reported more NRS leg pain (6.1 vs. 1.7, P < 0.001) and worse outcomes than NoDef group for ODI (35.7 vs. 23.1, P = 0.016) and PCS (32.6 vs. 41.9, P = 0.007).ConclusionWe found a 9.9% rate of new neurologic deficits following surgery for symptomatic primary presentation adult lumbar scoliosis, much higher than previous studies. Most neurologic deficits improved by 1-year follow-up, but appeared to have a dramatic negative impact on PROs, with increased postoperative leg pain and greater patient-perceived pathology reported in patients experiencing neurological deficits compared with those who did not.Level of evidence3.

Project description:ObjectiveThe demand for treating degenerative lumbar spinal disease has been increasing, leading to increased utilization of medical resources. Thus, we need to understand how the budget of insurance is currently used. The objective of the present study is to overview the utilization of the National Health Insurance Service (NHIS) by providing the direct insured cost between patients receiving surgery and patients receiving nonsurgical treatment for degenerative lumbar disease.MethodsThe NHIS-National Sample Cohort was utilized to select patients with lumbar disc herniation, spinal stenosis, spondylolisthesis or spondylolysis. A matched cohort study design was used to show direct medical costs of surgery (n = 2,698) and nonsurgical (n = 2,698) cohorts. Non-surgical treatment included medication, physiotherapy, injection, and chiropractic. The monthly costs of the surgery cohort and nonsurgical cohort were presented at initial treatment, posttreatment 1, 3, 6, 9, and 12 months and yearly thereafter for 10 years.ResultsThe characteristics and matching factors were well-balanced between the matched cohorts. Overall, surgery cohort spent $50.84/patient/month, while the nonsurgical cohort spent $29.34/patient/month (p<0.01). Initially, surgery treatment led to more charge to NHIS ($2,762) than nonsurgical treatment ($180.4) (p<0.01). Compared with the non-surgical cohort, the surgery cohort charged $33/month more for the first 3 months, charged less at 12 months, and charged approximately the same over the course of 10 years.ConclusionSurgical treatment initially led to more government reimbursement than nonsurgical treatment, but the charges during follow-up period were not different. The results of the present study should be interpreted in light of the costs of medical services, indirect costs, societal cost, quality of life and societal willingness to pay in each country. The monetary figures are implied to be actual economic costs but those in the reimbursement system instead reflect reimbursement charges from the government.

Project description:BackgroundThe long-term clinical and radiographic outcomes following coracoclavicular (CC) ligament reconstruction for the operative treatment of acute acromioclavicular (AC) joint dislocation remain uncertain. The purpose of the present study was to determine the long-term clinical and radiographic outcomes of CC ligament reconstruction and to identify risk factors for unfavorable outcomes.MethodsWe reviewed 20 cases of AC joint dislocation in 19 patients (18 male and 1 female; mean age, 32.3 years) that were treated with single-bundle reconstruction. The mean duration of follow-up was 12.7 years. We measured the CC vertical distance (CCD) on the anteroposterior view and compared the affected and unaffected sides (CCD ratio). We divided the patients into those with a CCD ratio of <25% (Group 1) and those with a CCD ratio of ≥25% (Group 2). We radiographically investigated the clavicular tunnel anteroposterior (CTAP) angle, clavicular tunnel ratio, and coracoid tunnel orientation on the basis of the entry and exit points at the base of the coracoid. For the coracoid tunnel orientation, we compared center-center orientation and noncenter-center orientation.ResultsGroup 1 comprised 17 cases (85%), and Group 2 comprised 3 cases (15%). At the time of the latest follow-up, Group 1 had a significantly higher mean Constant score than Group 2 (98.2 compared with 90.7; p = 0.038). Of the 3 radiographic parameters, only the CTAP angle was significantly different between the 2 groups (p < 0.0001). Two (67%) of the 3 cases in Group 2 were associated with posterior AC joint displacement.ConclusionsCC ligament reconstruction for the treatment of acute AC joint dislocation resulted in successful long-term clinical and radiographic outcomes. It is important to decrease the CTAP angle and to ensure proper anatomic placement of the clavicular and coracoid tunnels at the time of surgery.Level of evidenceTherapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Dataset Information

Sotos syndrome and scoliosis surgical treatment: a 10-year follow-up.

Publications

Sotos syndrome and scoliosis surgical treatment: a 10-year follow-up.

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