Gurkan Erkula

Spine Deformity Correction in Marfan Syndrome

Study Design. Retrospective review of records and radiographs with postoperative questionnaire of patients with Marfan syndrome receiving surgical correction for spinal deformity. Objective. To characterize and analyze the challenges of surgical correction for spinal deformity in Marfan syndrome and to present recommendations that may reduce morbidity and increase success for these procedures. Summary of Background Data. Spinal deformities are common in Marfan syndrome and usually refractory to conservative management. The few, smaller studies of surgical intervention reported on earlier surgical techniques but suggested increased complication rates. Methods. Records and radiographs of 39 patients with confirmed Marfan syndrome who underwent surgical management at either of two institutions for primary scoliosis (n = 26), kyphosis (n = 7), or deformity secondary to previous surgery elsewhere (n = 6) were reviewed. Presentation features, complications, and therapeutic results were analyzed. Low back outcome scores were generated from a questionnaire given to patients after surgery. Results. Increased blood loss and rates of infection (10%), dural tear (8%), instrumentation fixation failure (21%), pseudarthrosis (10%), and coronal (8%) and sagittal (21%) curve decompensation were noted. Infection was often associated with dural tear and decompensation with extreme correction. One patient died of valvular insufficiency 11 weeks after surgery. Conclusion. The cardiopulmonary condition of patients with Marfan syndrome should be evaluated and planned for before surgery. Preoperative computed tomograph to assess bony adequacy for fixation and magnetic resonance imaging to evaluate dural ectasia are indicated. Attention paid to the sagittal profile, extension of fusion to vertebrae neutral and stable in both planes, minimization of soft tissue dissection, and avoidance of extreme correction may prevent curve decompensation.

Patterns of Pediatric Supracondylar Humerus Fractures

Purpose: The Wilkins-modified Gartland classification of pediatric supracondylar humerus fractures does not consider coronal or sagittal obliquity. The purposes of our study were (1) to identify and describe fracture characteristics with unique properties and (2) to propose a fracture classification system that can be reproduced reliably. Methods: We retrospectively studied 203 consecutive displaced pediatric extension-type supracondylar humerus fractures treated operatively from January 1998 to January 2003. Fracture characteristics (eg, coronal and sagittal obliquity, postoperative alignment), type of surgical treatment, outcome, and complications were assessed and analyzed statistically with Student t test and a receiver operating characteristic curve. Significance was defined as P < 0.05. We incorporated significant cutoff values for fracture obliquity into our classification scheme and tested the classifications interobserver and intraobserver reliability. Results: We identified 4 coronal (typical transverse, medial oblique, lateral oblique, and high fractures) and 2 sagittal (low sagittal and high sagittal) subtypes with significantly different characteristics and outcome. Compared with fractures with coronal obliquity of less than 10 degrees, fractures with coronal obliquity of 10 degrees or greater were associated with significantly more comminution and rotational malunion. Compared with fractures with sagittal obliquity of less than 20 degrees, fractures with sagittal obliquity of 20 degrees or greater were associated with a significantly higher incidence of additional injuries and were more likely to result in extension malunion. Analysis of the interobserver and intraobserver reliability for our system identified correlation coefficients ranging from 0.772 to 0.907 and 0.860 to 0.899, respectively. Conclusions: Because pediatric extension-type supracondylar humerus fractures vary significantly in terms of characteristics, identification of sagittal oblique and coronal oblique angles may have an important role in surgical decision making and may impact outcomes. Level of Evidence: Level 3 (retrospective study).

Hamstring shortening in healthy adults

Hamstring shortening (HS) causes changes in the posture and walking ability in spastic children, however, there are no studies defining the abnormalities in patellar alignment in individuals with HS.Twenty-five patients with a primary complaint of anterior knee pain, having HS detected at physical examination, and 11 healthy individuals without any HS or knee discomfort as a control group are included in this study. Serial x-rays of the spine, pelvis and knee are taken and knee x-rays are evaluated for the position of the patella in both groups.In patients with Knee Extension Deficit (KED) ≥ 60°, the Blackburne-Peel ratio is lower and the Insall-Salvati ratio is higher than the other groups, denoting a cephalic location of the patella (p < 0.05). There are no changes in the congruency and sulcus angles between the HS and control groups. Vertebral and pelvic changes correlate well with the literature, confirming that significant alterations occur after 60° of KED in adults.The extensor mechanism of the knee is affected and patella is located higher than normal in patients with severe HS, which may be a cause for knee discomfort. In the light of these findings, a routine knee extension deficit examination can be suggested in the initial evaluation of knee discomfort.

Protrusio acetabuli in Marfan syndrome: Age-related prevalence and associated hip function

BACKGROUND Protrusio acetabuli is known to occur in patients with Marfan syndrome, but its prevalence, its effects on hip function, and its possible association with the subsequent development of degenerative hip disease have not been studied in a large population. Nevertheless, some clinicians have recommended prophylactic hip surgery for preadolescents with Marfan syndrome and protrusio acetabuli. METHODS We performed a cross-sectional study of 173 patients (346 hips) with Marfan syndrome who were interviewed and examined for calculation of the Iowa hip score. Anteroposterior radiographs of the pelvis were made, and two radiographic indices of acetabular depth were measured: (1) the center-edge angle of Wiberg and (2) the acetabular-ilioischial distance. The presence of protrusio was defined with use of two extant definitions: (1) a center-edge angle of >50 degrees or (2) an acetabular-ilioischial distance of >/=3 mm in male patients or >/=6 mm in female patients. Linear regression analyses were performed between these radiographic indices of acetabular depth and patient age, Iowa hip scores, the magnitude of the radiographic joint space, and range of motion. RESULTS The prevalence of protrusio acetabuli was 27% according to the center-edge angle criterion and 16% according to the acetabular-ilioischial distance criterion. The prevalence of protrusio increased until the age of twenty years and remained stable after the age of twenty years. Slight but significant negative correlations were detected between the two radiographic indices of acetabular depth and both the Iowa hip score and the summed range of motion (p < 0.02 for all). No significant relationship was found between the two radiographic indices and pain scores. In patients with Marfan syndrome who were more than forty years old, the Iowa hip scores for hips with protrusio were not significantly lower than those for hips without protrusio. CONCLUSIONS In patients with Marfan syndrome, the prevalence of protrusio acetabuli increases during the first two decades of life and then plateaus in terms of both population-wide prevalence and radiographic severity. In this population, protrusio generally is not associated with severely problematic hip function but it is associated with slightly decreased range of motion of the hip. We concluded that prophylactic surgical intervention is not indicated for most patients with Marfan syndrome who have a radiographic diagnosis of protrusio.

Lateral shelf acetabuloplasty in the treatment of Legg-Calvé-Perthes disease.

Although head containment is extremely important for the development of the acetabulum and femoral head, there are debates about conservative and surgical treatment. Shelf acetabuloplasty is an effective means of treatment as regards the coverage of the femoral head within the acetabulum, which is the most important issue for the normal development of the hip joint. Nineteen hips of 18 patients were evaluated radiographically using the acetabulum–head index of Heyman and Herndon and the center–edge angle for containment. It was shown that postoperatively both indices improve to increase the containment, thus demonstrating the effectiveness of shelf acetabuloplasty.

Plain radiography of the lumbosacral spine in Marfan syndrome

BACKGROUND CONTEXT Marfan syndrome is a connective tissue disorder that results from a defect in the production of fibrillin. These patients tend to have several osseous anomalies of the lumbosacral spine. PURPOSE This study examines the effectiveness of plain radiographic findings in predicting Marfan syndrome. STUDY DESIGN/SETTING Case-control study. PATIENT SAMPLE Fourteen height-matched controls and 33 patients with Marfan syndrome were obtained from our genetics clinic or through the National Marfan Foundation. OUTCOME MEASURES Determined using measurements taken on plain radiographs. METHODS Five measurements were acquired of the lumbosacral spine from the radiographs of both groups: interpedicular distance, scalloping value, sagittal canal diameter, vertebral body width and transverse process width. RESULTS The following measurements were significantly larger in patients with Marfan syndrome: interpedicular distance at L1-L5 (p<.0001); sagittal diameters of the vertebral canal at L4-S2 (p<.01); transverse process to vertebral body width ratio at L2-L5 (p<.01). There was no significant difference in the scalloping values from L1-L5 between the patients with Marfan syndrome and the controls. A multivariate regression analysis generated the following criteria for plain film diagnosis of Marfan syndrome (two criteria need to be met for diagnosis): interpedicular distance at L5 greater than or equal to 36.0 mm, sagittal diameter at L5 greater than or equal to 13.5 mm or transverse process to vertebral width ratio at L3 greater than or equal to 2.25. CONCLUSION Based on this criteria, patients can be diagnosed with Marfan syndrome with a high sensitivity (81.8%) but a low specificity (58.3%). Thus, plain radiography can be a useful means of screening patients with Marfan syndrome.

Pin size influences sagittal alignment in percutaneously pinned pediatric supracondylar humerus fractures.

Background No study examining pin constructs has adequately addressed pin size and its role in fracture fixation. Our goal was to review our experience with Wilkins-modified Gartland type-III pediatric supracondylar humerus fractures treated with closed reduction and percutaneous pinning to evaluate the effects of pin size within 2 different pin constructs on maintenance of reduction and on the risk of surgical complications. Methods We retrospectively reviewed the medical records of pediatric patients with Wilkins-modified Gartland type-III supracondylar humerus fractures that were closed reduced and percutaneously pinned at our institution from March 1999 through December 2008. We grouped those 159 patients by fracture stabilization method (lateral-entry-pin or crossed-pin constructs), by pin size ratio (ie, ratio of pin diameter to the humeral midshaft cortical thickness: small ⩽0.9; large >0.9), and then by 4 combinations of pin construct and pin size ratio. For each group, we evaluated radiographs for immediate postoperative reduction (coronal and sagittal alignment), maintenance of reduction at last follow-up, and the number of surgical complications. We used the Student t test, &khgr;2 test, Mann-Whitney U test, and Wilcoxon Signed Rank test to examine for significance, which was set at P<0.05. Results Although we found no significant differences between the groups immediately after surgery, final follow-up sagittal alignment was significantly more likely to be maintained in the large pin size ratio group than in the small pin size ratio group. For 2 types of surgical complications, infection and nerve palsy, we found no statistically significant differences in these complications between the pin construct or pin size ratio groups. Conclusions Large pin sizes improved radiographic sagittal alignment at final follow-up without an increased rate of infection or ulnar nerve palsy. Level of Evidence Level III Therapeutic Study.

The relation of joint laxity and trunk rotation.

A study was designed to evaluate the joint laxity during scoliosis screening, and to show if there is a relation of joint laxity values to the trunk rotation. One thousand, two hundred and seventy-three children (598 females, 675 males) with an average age of 10.4 years were screened with a scoliometer and forward bending for trunk rotations. Scapular and shoulder elevations, flexible pes planus were recorded and joint laxity was evaluated with the Beighton score. There was high inter-observer and intra-observer reliability for both scoliometer and Beighton scores. In 41 children (3.2%) with Beighton score 7 or higher, trunk rotation measurements were higher than for the rest of the children. Trunk rotation measurements of 7° or higher were found in 30 children, who were more lax than the rest of the group and were invited for radiography, with a detection of curves between 11 and 18° in 10 of them. The Beighton score is a practical and reliable method for defining joint laxity. Although the number of patients with scoliosis was limited, there are findings supporting the relation between joint laxity and scoliosis. Moreover, there was increased laxity in children with increased trunk rotations. Ligamentous laxity may be one of the causes changing the contour of the back.

Implant-related fractures in children: a 15-year review.

Background: To our knowledge, there are no comprehensive clinical studies of implant-related fractures in children. Our goal was to identify the incidence, skeletal location, and associated diagnoses of implant-related fractures. Methods: We reviewed our institutional database to identify cases of implant insertion (7584 cases) in patients less than 18 years old from January 1, 1995 through December 31, 2009. We calculated the overall incidence of these fractures and stratified the incidence by skeletal location and preoperative diagnoses. Fisher exact test was used to ascertain differences in fracture incidence. Risk ratios were calculated when appropriate. Significance was set at P<0.05. Results: There were 25 cases of implant-related fractures: 22 in the femur, 2 in the tibia, and 1 in the radius. The overall incidence of implant-related fracture was 0.33%; the incidence by skeletal location was: femur, 0.89%; tibia, 0.1%; and radius, 0.14%. Associated diagnoses were cerebral palsy (9 cases), hip dysplasia (3 cases), spina bifida (2 cases), and avascular necrosis (1 case); 10 cases were associated with “other diagnoses,” which included various skeletal syndromes (5 cases) and traumatic fractures (5 cases). The incidences of implant-related fractures by diagnoses were: cerebral palsy, 1.1%; hip dysplasia, 1.1%; spina bifida, 1.3%; and avascular necrosis, 0.35%. The incidence of implant-related fracture in the “other diagnoses” group was 0.16%, and the incidence of fracture in otherwise healthy patients was 0.084%. The femur was 15.2 times more likely to fracture than other bones (P<0.001). Diagnoses of cerebral palsy, hip dysplasia, spina bifida, and avascular necrosis were 6.1 times more likely to be associated with implant-related fractures than the “other diagnoses” (P<0.001). The mean time to fracture in the study was 2.8 years. The overall implant removal rate at our institution was 24.3%, and it varied significantly by patient diagnosis (P<0.01). Conclusions: Skeletal location and preoperative diagnosis should be factors of consideration in a surgeon’s decision about removing implants to prevent implant-related fractures. Level of Evidence: Prognostic Level III.

The acetabular teardrop and ultrasonography of the hip.

The acetabular teardrop is an important tool for the diagnosis of acetabular dysplasia. Since it was first described by Köhler its widespread use has not become established due to the relative subjectivity in classification of the types. The acetabular teardrop angle (ATA) is proposed for this purpose, and to show the teardrops direct relation to acetabular development by correlating it with hip ultrasonography results. Patients (51 male, 53 female) included in this study received hip ultrasonography and concomitant pelvic radiography. In the 296 hips, &agr; and &bgr; angles were measured from ultrasonography, whilst the acetabular index (AI), ATA, teardrop distance and the transverse diameter of the most upper, most widened part of the teardrop were measured from the radiographs. The enlarged, and triangle-shaped teardrop is seen later than normal ones, and the triangle-shaped-teardrop hips have a higher AI than the normal hips. The ATA is in good correlation with the widest diameter, showing this to be a reliable tool for describing the teardrop. The ATA also has a positive correlation with the &bgr; angle denoting its significance to acetabular development. High-risk babies should be followed-up primarily with ultrasonography wherever possible. The AI from radiographs and the acetabular teardrop may be used as a helper tools in decision-making, with the guides of the ATA and the transverse diameter of the most upper, most widened part of the teardrop, as proposed in this study.