Lengthening of the ulna by callotasis in children with multiple hereditary exostoses: comparison of methods with and without internal fixation.

PURPOSE: The goal of this retrospective study was to compare the gradual lengthening of the ulna in children with multiple hereditary exostoses with and without an elastic intramedullary nail. METHODS: Between 1998 to 2018, the ulna was lengthened in 28 forearms in 21 patients (aged 7.1 to 16.6 years) using a monolateral external fixator when relative ulnar shortening exceeded 15 mm. In total, 16 forearms were lengthened with the external fixator (group I) and 12 forearms with the addition of an intramedullary elastic nail (group II). Subjective assessment of function, range of movement (ROM) of the wrist and elbow and complications were compared. Ulnar shortening, radial head dislocation, radial articular angle (RAA) and percentage of carpal slip and radial bowing were followed radiographically. The difference between the groups has been evaluated statistically. RESULTS: The function of the extremity improved partially in 81% of patients in group I and in 83% of patients in group II. ROM was not improved except for radial deviation. Radial head position did not change. The values in group II in comparison with group I are higher for gain of length and lower for bone lengthening index and for bone healing index. Carpal slip decreased insignificantly. The RAA and radial bowing decreased, the comparison of values between groups and age under and over ten years were not statistically significant. Complications were more common in group I. No permanent complications were noted. CONCLUSION: The addition of an intramedullary nail during the gradual ulnar lengthening improves the gain, bone healing index and rate of complications. LEVEL OF EVIDENCE: III.

Range of motion after total knee arthroplasty in hemophilic arthropathy.

BACKGROUND: Outcomes of total knee replacement in cases of hemophilic patients are worse than in patients who undergo operations due to osteoarthritis. Previous publications have reported varying rates of complications in hemophilic patients, such as infection and an unsatisfactory range of motion, which have influenced the survival of prostheses. Our retrospective study evaluated the data of hemophilic patients regarding changes in the development of the range of motion. METHODS: The data and clinical outcomes of 72 total knee replacements in 45 patients with hemophilia types A and B were reviewed retrospectively. Patients were operated between 1998 and 2013. All of the patients were systematically followed up to record the range of motion and other parameters before and after surgery. RESULTS: The mean preoperative flexion contracture was 17° ± 11° (range, 0°-40°), and it was 7° ± 12° (range, 0°-60°) postoperatively. The mean flexion of the knee was 73° ± 30° (range, 5°-135°) before the operation and 80° ± 19° (range, 30°-110°) at the last follow-up. The mean range of motion was 56° ± 34° (range, 0°-130°) before the operation and 73° ± 24° (range, 10°-110°) at the last follow-up. CONCLUSIONS: Statistical analysis suggested that the range of motion could be improved until the 9th postoperative week. The patient should be operated on until the flexion contracture reaches 22° to obtain a contracture < 15° postoperatively or until the contracture reaches 12° to obtain less than 5°. The operation generally does not change the flexion of the knee in cases of hemophilic patients, but it reduces the flexion contracture and therefore improves the range.

Proteomic analysis of the extracellular matrix in idiopathic pes equinovarus.

Idiopathic pes equinovarus is a congenital deformity of the foot and lower leg defined as a fixation of the foot in adduction, supination, and varus. Although the pathogenesis of clubfoot remains unclear, it has been suggested that fibroblasts and growth factors are involved. To directly analyze the protein composition of the extracellular matrix in contracted tissue of patients with clubfoot. A total of 13 infants with idiopathic clubfoot treated with the Ponseti method were included in the present study. Tissue samples were obtained from patients undergoing surgery for relapsed clubfeet. Contracted tissues were obtained from the medial aspect of the talonavicular joint. Protein was extracted after digestion and delipidation using zip-tip C18. Individual collagenous fractions were detected using a chemiluminescent assay. Amino acid analysis of tissue samples revealed a predominance of collagens, namely collagen types I, III, and VI. The high content of glycine and h-proline suggests a predominance of collagens I and III. A total of 19 extracellular matrix proteins were identified. The major result of the present study was the observation that the extracellular matrix in clubfoot is composed of an additional 16 proteins, including collagens V, VI, and XII, as well as the previously described collagen types I and III and transforming growth factor ß. The characterization of the general protein composition of the extracellular matrix in various regions of clubfoot may help in understanding the pathogenesis of this anomaly and, thus, contribute to the development of more efficacious therapeutic approaches.

Muscle transfers in children and adults improve external rotation in cases of obstetrical brachial plexus paralysis: a comparative study.

PURPOSE: Latissimus dorsi and teres major transfers to the lateral side of the humerus with lengthening of the pectoralis major and subscapularis muscles for residual shoulder deformity were compared in children and skeletally mature patients. METHODS: Fifteen patients (nine children, six skeletally mature patients aged three to 30 years, follow-up one to 22 years) were treated for internal shoulder contracture after birth plexus lesions: C5-C6 (seven patients); C5-7 (five patients); C5-C8-T1 (three patients, respectively). Range of movement, Mallet shoulder function score and radiographs were assessed. RESULTS: Pre-operatively, shoulder function restrictions were comparable in all patients. Postoperatively, external rotation, abduction and Mallet function score improved significantly (p < 0.05) in all patients except one. There were no differences in improvement between children and skeletally mature patients (p = 0.24-1.0). CONCLUSIONS: This technique improves external rotation and abduction of the shoulder for daily living activities in children and young, skeletally mature, patients.

Comparison of the short-term and long-term results of the Ponseti method in the treatment of idiopathic pes equinovarus.

PURPOSE: Congenital club foot is one of the most common birth defects involving the musculoskeletal system. At present two methods are used for the treatment of this deformity: French and Ponseti method. The purpose of this study was to compare the short-term (up to three years) and long-term (three to seven years) results of treatment with the Ponseti method. METHODS: A total of 195 consecutive infants (143 boys and 52 girls) with idiopathic club foot treated with the Ponseti method in the period of 2005-2012 were included in this study; the total number of feet was 303. The severity of the foot deformity was classified according to Diméglio. When relapse occurred up until three years of age, we started with the casting again. If conservative treatment was unsuccessful we proceeded to surgical treatment. RESULTS: Primary correction was attained in all cases. Surgical correction of relapses was performed in 30 % of patients according to the Ponseti method (re-tenotomy of the Achilles tendon and transposition of the tibialis anterior) and in 70 % by alternative techniques. The number of relapses indicated for surgery increased with increasing period of follow-up: whereas in patients where the treatment started already in 2005 relapses occurred in 72 %, in patients included in 2011 the number of recurrences only reached 3 %. CONCLUSIONS: It follows from our results that it is impossible to cure all club feet with casting, tenotomy of the Achilles tendon and transposition of the tibialis anterior only.

Anteromedial wedge reduction osteotomy for the treatment of femoral head deformities.

Aspherical femoral head deformity has poor a prognosis which can lead to early arthritic changes in young adults. The intraartricular anteromedial wedge reduction osteotomy restores sphericity of the femoral head.
We performed seven anteromedial wedge reduction osteotomies. Clinical indications were pain, limp and restriction of movement of the hip joint. The mean age at time of the surgery was 13.3 years. The aetiology of the femroal head deformity was Perthes disease in six patients and in one patient, multiple epiphyseal dysplasia. All seven hips were classified as Stulberg V before surgery.
At a mean follow-up of 17.4 months the Harris hip score increased from a mean of 55.4 preoperatively to a mean of 84.8 postoperatively. The final results were Stulberg IV in one hip, Stulberg III in four hips and Stulberg II in two hips postoperatively. The capital diaphyseal ratio dropped from average of 1.57 preoperatively to 1.21 postoperatively. No signs of osteonecrosis were recorded. One patient suffered a subluxation of the femoral head.
The anteromedial wedge reduction osteotomy has good short-term results in aspherical incongruent hips.

Cruciate ligaments in proximal femoral focal deficiency: arthroscopic assessment.

BACKGROUND: Because there is limited information concerning the cruciate ligaments in proximal femoral focal deficiency, knee arthroscopy was used to identify the changes of cruciate ligaments and their relation to the different types of this deficiency. METHODS: Knee arthroscopy was performed in 21 consecutive patients with deficiency types III, IV, VII-IX using the Pappas classification. A new classification of the knee was created. It contains types I, II, III according to the findings of the anterior cruciate ligament (ACL) (type I: normal, type II: hypoplasia, type III: aplasia) and 3 subtypes A, B, C according to the findings of the posterior cruciate ligament (PCL) (type A: normal, type B: hypoplasia, type C: aplasia), respectively. Instrumented and radiologic drawer testing was provided additionally in 2010. RESULTS: The changes of the cruciate ligaments were found in all but 1 patient. Type I was found in only 2 patients. In 1, both cruciate ligaments were intact (type IA). In the other patient, the ACL was intact, but the PCL was absent (type IC). Hypoplastic ACLs (type II) were found in 4 patients, namely in 3 patients with normal PCLs (type IIA), whereas in 1 patient the PCL was absent (type IIC). In the majority of patients, the ACLs completely failed (type III, 15 patients). Absence of both cruciate ligaments was found in 8 patients (type IIIC). PCLs were intact in 4 patients (IIIA) or were hypoplastic in 3 patients (IIIB), respectively. Instrumental drawer testing was not reliable in patients of our group. Radiologic testing showed a posterior shift of the tibia in the majority of patients on the affected side. Anterior and posterior drawer tests were increased in a majority of patients, but did not directly correlate to the presence/absence of cruciate ligaments. CONCLUSIONS: Variable changes of the cruciate ligaments were found in all but 1 patient with proximal femoral focal deficiency. These changes were not related to the type of Pappas classification. Despite the lower clinical relevance of the changes in majority of patients, imaging of cruciate ligaments is recommend before lengthening of the extremity to avoid dislocation of the knee. LEVEL OF EVIDENCE: I - Testing of previously developed diagnostic criteria in series of consecutive patients.

Computed tomographic angiography in proximal femoral focal deficiency.

BACKGROUND: Because there is limited information concerning the vascular pattern and the role of vessels in patients with proximal femoral focal deficiency, the vascular supply of the lower extremities was studied systematically with use of computed tomographic angiography in order to identify vascular changes, relate any vascular changes to the classification of the deficiency, and establish that there are no major changes in the topographical anatomy of the vessels. METHODS: Standardized computed tomographic techniques were used in twenty-one patients (thirteen boys and eight girls who ranged from one to nineteen years old) with proximal femoral focal deficiency types I through IV and VII, VIII, and IX, according to the Pappas classification. RESULTS: A common anatomical vascular pattern, in which the hypoplastic extremity was supplied through the femoral artery, was detected in nineteen patients. In patients with Pappas type-I through IV disease, the external iliac, femoral, and deep femoral arteries were substantially reduced in length and diameter and the deep femoral artery arose more proximally in comparison with that in the contralateral extremity; however, in the patients with Pappas type-VII, VIII, or IX disease, the diameters of the arteries and the origin of the deep femoral artery were similar to those of the contralateral extremity. In two patients with Pappas type-III disease, atypical anatomy of the vessels was found. The anterior part of the thigh and the pseudarthrosis were supplied through the femoral artery (the external iliac artery) as a terminal branch, while the remainder of the extremity was supplied from the internal iliac artery, which entered the thigh posterior to the hip as the inferior gluteal artery and continued as the artery to the sciatic nerve to the popliteal artery. No substantial anastomoses were found between the femoral and the posterior arteries in these vascular patterns. CONCLUSIONS: Computed tomographic-angiographic reconstruction can be used to depict the spatial configuration of the pseudarthrosis and the vascular pattern of the extremity in patients with proximal femoral focal deficiency. We found distinct vascular changes in the majority of the involved thighs, but there were no typical changes related to the Pappas classification. Because we found atypical vascular patterns in two patients, evaluation of the vessels with use of various diagnostic methods is recommended in patients with severe types of proximal femoral focal deficiency.