William J. Shaughnessy

Klippel-Trénaunay syndrome : Spectrum and management

OBJECTIVE To describe a series of 252 patients with Klippel-Trénaunay syndrome (KTS), a rare congenital malformation characterized by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity. MATERIAL AND METHODS We reviewed the clinical characteristics and findings in 136 female and 116 male patients with KTS who underwent assessment at Mayo Clinic Rochester between January 1956 and January 1995. In addition, management options are discussed. RESULTS Capillary malformations (port-wine stains) were found in 246 patients (98%), varicosities or venous malformations in 182 (72%), and limb hypertrophy in 170 (67%). All three features of KTS were present in 159 patients (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, excision of vascular malformations, amputations, and debulking procedures. CONCLUSION Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2.0 cm at skeletal maturity, which can be treated with epiphysiodesis in the growing child. If a functioning deep vein system is present, removal of symptomatic varicosities or localized superficial venous malformations in selected patients can yield good results.

The Effect of Superior Placement of the Acetabular Component on the Rate of Loosening after Total Hip Arthroplasty. Long-Term Results in Patients Who Have Crowe Type-II Congenital Dysplasia of the Hip*

A method for measurement of the true acetabular region and the approximate femoral head center as well as a classification consisting of four zones for assessment of the acetabular position of the acetabular cup were used to analyze the results of primary total hip arthroplasty with cement in 117 patients (145 hips). All patients had Crowe type-II congenital dysplasia of the hip. The mean age at the time of the arthroplasty was fifty-one years (range, fifteen to seventy-six years), and the mean duration of follow-up was fourteen years (range, two to twenty-two years). The initial position of the acetabular cup outside of the true acetabular region and outside of zone 1 (inferior and medial) was associated with an increase in the rates of loosening (p < 0.05) and revision (p < 0.04) of the femoral components. Cups that initially were more than fifteen millimeters superior to the approximate femoral head center, without lateral displacement, were associated with an increased rate of loosening (p < 0.001) and of revision (p < 0.04) of the femoral components as well as with an increased rate of loosening (p < 0.002) and of revision (p < 0.01) of the acetabular components. These findings suggest that superior positioning of the acetabular component, even without lateral displacement, leads to increased rates of loosening of the femoral and acetabular components. An attempt should be made to position the acetabular component in or near the true acetabular region.

Aneurysmal Bone Cyst of the Spine: Management and Outcome

Study Design. The clinical records, radiographs, histologic sections, and operative reports of 52 consecutive patients with an aneurysmal bone cyst of the spine were reviewed to evaluate diagnostic and therapeutic options and to correlate treatment and outcome. Objectives. To define the incidence, clinical presentation, diagnostic and therapeutic options, and prognosis of patients with aneurysmal bone cyst of the spine. Summary of Background Data. There are special considerations in the management of spinal lesions: relative inaccessibility of the lesions, associated intraoperative bleeding, necessity of removing the entire lesion to avoid the possibility of recurrence, proximity of the lesion to the spinal cord and nerve roots, and potential postoperative bony spinal instability. Methods. Fifty‐two consecutive patients with an aneurysmal bone cyst of the spine were treated from 1910 to 1993. Forty patients initially treated for a primary lesion had operative treatment (19 intralesional excision and bone grafting and 21 intralesional excision); four also had adjuvant radiation therapy. Preoperative arterial embolization was performed in two. Results. There was a recurrence rate of 10% within 10 years. All recurrences were noted less than 6 months after surgery. Of 12 patients treated for a recurrent lesion, two had a subsequent recurrence (16.7%) within 9 years. At last follow‐up examination, 50 patients (96%) were free of the disease. One patient died of postradiation osteosarcoma, and one died of intraoperative bleeding. Conclusions. Current treatment recommendations involve preoperative selective arterial embolization, intralesional excision curettage, bone grafting, and fusion of the affected area if instability is present.

Evaluation and Management of Pain in Patients with Klippel-Trenaunay Syndrome: A Review

Klippel-Trenaunay syndrome (KTS) is a rare disorder that consists of a triad of capillary vascular malformation, venous malformations and/or varicose veins, and soft tissue and/or bony hypertrophy. Pain is a real and debilitating problem in these patients. We have observed 9 common causes of pain in KTS: (1) chronic venous insufficiency, (2) cellulitis, (3) superficial thrombophlebitis, (4) deep vein thrombosis, (5) calcification of vascular malformations, (6) growing pains, (7) intraosseous vascular malformation, (8) arthritis, and (9) neuropathic pain. The management of pain in patients with KTS depends on its cause. These patients are best evaluated initially in a center with an experienced multidisciplinary team that includes a primary health care provider, surgeons, and ancillary staff. The ongoing care of a patient with KTS often depends on a local provider who is more readily accessible to the patient but may not have the expertise of a large center to manage the complications of KTS. The purpose of this communication is to review the common causes of pain in these patients to provide local health care providers and patients and their families with appropriate management strategies.

Results of total hip arthroplasty for Crowe type III developmental hip dysplasia

From 1969 through 1980,90 hips in 82 patients had cemented total hip arthroplasty for Type III developmental hip dysplasia. Seventy hips were reviewed at an average of 16.6 years (range, 5–23 years) after operation. Aseptic loosening developed in 53% of acetabular cups and 40% of femoral stems. Despite attempts to place acetabular components in the anatomic center, 18 cups (25.7%) were placed outside that area. Using a measurement method to determine the true acetabular region and approximate femoral head center, final acetabular loosening strongly correlated with initial cup placement. Loosening occurred in 15 of 18 cups (83.3%) initially positioned outside of the true acetabular region compared with loosening in 22 of 52 cups (42.3%) initially positioned within the true acetabular region. Acetabular loosening also correlated with initial lateral displacement or initial superior displacement of the hip center from the approximate femoral head center. Initial cup placement medial to the approximate femoral head center was predictive of successful long term acetabular component fixation. The method of acetabular reconstruction did not affect eventual cup loosening. Placement of the hip arthroplasty center of rotation in or near the true acetabular region is recommended.

Long-Term Outcome of Lumbar Discectomy in Children and Adolescents Sixteen Years of Age or Younger*

We retrospectively reviewed the cases of seventy-two consecutive patients who had a lumbar discectomy, between 1950 and 1983, when they were sixteen years of age or younger. There were forty boys and thirty-two girls. At the time of the lumbar discectomy, twelve patients (17 per cent) also had a spinal arthrodesis. The mean duration of follow-up was 27.8 years (range, twelve to forty-five years). Twenty patients (28 per cent) had one reoperation or more, with the first reoperation performed at a mean of 9.7 years after the initial discectomy. Fourteen patients had one reoperation, four had two reoperations, one had three, and one had five. Fifty-two patients (72 per cent) did not need a reoperation. At the time of the latest follow-up, forty-eight (92 per cent) of the fifty-two patients either had no pain or had occasional pain related to strenuous activity and fifty-one (98 per cent) could participate in daily activities with no or mild limitations. Survivorship analysis showed that the overall probability that a patient would not need a reoperation was 80 per cent at ten years and 74 per cent at twenty years after the initial operation. With the numbers available for study, we could not show that age, gender, or an arthrodesis performed at the time of the initial operation were risk factors for a reoperation. We could not detect a difference, with respect to pain or the level of activity, between the patients who had had an arthrodesis at the initial operation and those who had not or between those who had a coexisting structural abnormality of the lumbar spine and those who did not.

Gait Abnormalities following Resection of Talocalcaneal Coalition

Eleven patients (fourteen feet) had resection of a coalition of the middle facet of the talocalcaneal joint because of symptoms that had been present for a mean of four years (range, 0.5 to nine years). The mean age at the time of the resection was seventeen years (range, thirteen to thirty-two years). Nine patients were male and two were female. Five feet had resection of the bone bridge with interposition of fat or tendon, and nine had resection without any interposed material. The mean duration of follow-up was six years (range, two to thirteen years). The clinical result was excellent for five feet, good for four, fair for three, and poor for two. Inversion was 8 ± 4.5 degrees (mean and standard deviation) on the involved side and 17 ± 6.2 degrees on the uninvolved side (p = 0.002). Three feet later had evidence of osteoarthrosis of the subtalar joint. Gait analysis demonstrated decreased motion of the hindfoot and the ankle, compared with that in normal subjects and with that in the contralateral (uninvolved) foot, in the sagittal and coronal planes during walking on a level surface. Motion was also reduced, particularly in the sagittal and coronal planes, during walking on a side slope. Ground-reaction forces (F8 and F9), temporal force factors (T2, T3, and T7), and the percentage of the gait cycle that consisted of the stance phase on the side-sloping walkway were different from those in normal subjects or in the uninvolved foot. These data indicate that, although most of our patients who had resection of a talocalcaneal coalition had a successful clinical result, most had a residual functional deficit.

Total Knee Arthroplasty in Young Patients with Juvenile Rheumatoid Arthritis

Background: Juvenile rheumatoid arthritis is a disabling and destructive condition that commonly affects the knee during childhood. Total knee arthroplasty occasionally may be necessary for the treatment of end-stage disabling arthritis of the knee in young patients. There is a paucity of available data on the results of total knee arthroplasty in adolescents. We report our experience with total knee arthroplasty in patients under the age of twenty years who had juvenile rheumatoid arthritis.Materials and Methods: We reviewed the results of twenty-five consecutive total knee arthroplasties that had been performed at our institution between 1982 to 1997 in thirteen patients (mean age, seventeen years) with juvenile rheumatoid arthritis. The average duration of clinical follow-up was 10.7 years, and the average duration of radiographic follow-up was 6.5 years.Results: The mean Knee Society pain score improved markedly from 27.6 to 88.3 points, and the mean Knee Society function score improved modestly from 14.8 to 39.2 points. There was a slight improvement in the range of motion. Symptomatic and progressive radiolucent lines were noted in two knees, one of which was revised. Two knees (one patient) required exchange of the polyethylene liner at thirteen years. There were four additional reoperations, including manipulation under general anesthesia (two knees in one patient), lysis of adhesions (one knee), and extensor mechanism realignment (one knee).Conclusions: Despite a substantial number of postoperative complications, total knee arthroplasty provided excellent relief of pain and improvement in function in this group of adolescent patients with juvenile rheumatoid arthritis.Level of Evidence: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.

Motocross morbidity: Economic cost and injury distribution in children

Background Motocross is a nationally organized sport that is growing in popularity. The distribution and severity of motocross injuries in the pediatric population is not known. We hypothesize a high rate of musculoskeletal injuries requiring hospitalization and/or surgical intervention. Methods All patients 17 years of age or younger with injuries sustained while using off-road 2-wheeled motorcycles were identified through surgical, diagnostic, and trauma registries at a level 1 regional trauma center. Type, severity, and mechanism of injury were assessed, as well as charges billed for medical care. Both recreational and competitive motocross activities were included. Results From 2000 to 2007, 299 cases were noted in 249 unique patients. In 141 instances, hospital admission was required, for a total of 412 inpatient days. Twenty patients required ICU admission. Surgery was performed in 91 cases (81 orthopaedic, 6 general, 1 urology, and 4 facial reconstructions). Orthopaedic surgical procedures included treatment of 29 femur fractures, 8 forearm, 6 ankle, 5 tibial shaft, 6 proximal tibia, 5 spine, 6 proximal humerus, 4 hand, 4 foot, 3 elbow fractures, and 5 other. Orthopaedic interventions also included 8 reductions under general anesthesia and 31 conscious sedations. Mean age at injury was 14.1 years (range: 5.4 to 17.9). Ninety-four percent of patients were male and 85% were White. The majority of patients were wearing helmets/safety equipment. One hundred and eighty-four injuries occurred on a track, with 150 during competition. The mean charge billed per injury was

A quantitative composite scoring tool for orthopaedic residency screening and selection.

14,947 (range: