Functional outcome and quality of life following resection of the proximal humerus performed for musculoskeletal tumors and reconstruction done by four different methods.

INTRODUCTION: The proximal humerus is a frequent site for both primary and secondary bone tumors. Several options are currently available to reconstruct the resected humerus, but there is no consensus regarding optimal reconstruction. The aim of this retrospective study was to compare the functional outcome, complications and patient compliance following four different types of reconstructive techniques. MATERIAL AND METHODS: The authors performed 90 proximal humerus resections due to primary and secondary bone tumors over the past 21 years. Four different procedures were performed for reconstruction following the resection: fibula autograft transplantation, osteoarticular allograft implantation, modular tumor endoprosthesis (hemiarthroplasty) and reconstruction of the defect with a reverse shoulder prosthesis-allograft composite. A retrospective analysis of the complications and patient's physical status was performed. Functional outcome and life quality was evaluated by using the MSTS and SF-36 scores. RESULTS: The best range of motion was observed following arthroplasty with a reverse shoulder prosthesis-homograft composite followed by a fibula autograft reconstruction. Revision surgery was required due to major complications most frequently in the osteoarticular allograft group, followed by the reverse shoulder prosthesis-allograft composite group, the autologous fibula transplantation group; the tumor endoprosthesis hemiarthroplasty group had superior results regarding revision surgery (40, 25, 24 and 14% respectively). MSTS was 84% on average for the reverse shoulder prosthesis-allograft composite group, 70% for the autologous fibula group, 67% for the anatomical hemiarthroplasty group and 64% for the osteoartricular allograft group. Using the SF-36 questionnaire for assessment no significant differences were found between the four groups regarding quality of life. DISCUSSION: Based on the results of our study the best functional performance (range of motion and patient compliance) was achieved in the a reverse prosthesis-allograft combination group-in cases where the axillary nerve could be spared. The use of an osteoarticular allograft resulted in unsatisfying functional results and high complication rates, therefore we do not recommend it as a reconstructive method following resection of the proximal humerus due to either primary or metastatic bone tumors. Young patients who have good life expectancy but a small humerus or intramedullar cavity reconstruction by implantation of a fibula autograft is a good option. For patients with a poor prognosis (i.g. bone metastases) or in cases where the axillary nerve must be sacrificed, hemiarthroplasty using a tumor endoprosthesis was found to have acceptable results with a low complication rate. According to the MSTS and SF-36 functional scoring systems patients compliance was nearly identical following all four types of reconstruction techniques; the underlying cause may be the complexity of the shoulder girdle. However, we recommend the implantation of a reverse shoulder prosthesis-allograft whenever indication is appropriate, as it has been demonstrated to provide excellent functional outcomes, especially in young adults.

Nerve stretch injury induced pain pattern and changes in sensory ganglia in a clinically relevant model of limb-lengthening in rabbits.

We used a model of tibial lengthening in rabbits to study the postoperative pain pattern during limb-lengthening and morphological changes in the dorsal root ganglia (DRG), including alteration of substance P (SP) expression. Four groups of animals (naive; OG: osteotomized only group; SDG/FDG: slow/fast distraction groups, with 1 mm/3 mm lengthening a day, respectively) were used. Signs of increasing postoperative pain were detected until the 10(th) postoperative day in OG/SDG/FDG, then they decreased in OG but remained higher in SDG/FDG until the distraction finished, suggesting that the pain response is based mainly on surgical trauma until the 10(th) day, while the lengthening extended its duration and increased its intensity. The only morphological change observed in the DRGs was the presence of large vacuoles in some large neurons of OG/SDG/FDG. Cell size analysis of the S1 DRGs showed no cell loss in any of the three groups; a significant increase in the number of SP-positive large DRG cells in the OG; and a significant decrease in the number of SP-immunoreactive small DRG neurons in the SDG/FDG. Faster and larger distraction resulted in more severe signs of pain sensation, and further reduced the number of SP-positive small cells, compared to slow distraction.

The dangers of intraosseous fibrosing agent injection in the treatment of bone cysts. The origin of major complications shown in a rabbit model.

Direct intraosseous injection of fibrosing agent is widely used in the treatment of aneurysmal bone cysts. The purpose of this study was to evaluate the consequences of fibrosing agent penetrating the medulla of bones. This may be the case when, by mistake, the fibrosing agent is administered into the medulla or when the wall of the cyst ruptures and fibrosing agent is able to drift into the medulla. Twelve rabbits were injected transcutaneously with a fibrosing agent directly into the proximal metaphysis of the tibia. Prior to injection 0.5 ml of liquid-like, bloody, intraosseal tissue was aspirated, then 0.5 ml of fibrosing agent was administered. Fibrosing agent was introduced slowly (20 s) to avoid overpressure. Nine rabbits (75%) died within minutes after the introduction of fibrosing agent. A full body roentgenogram was taken of each rabbit and the animals that died underwent autopsy to find the exact cause of death. Roentgenograms of the chest showed massive multiple pulmonary emboli confirmed in all lethal cases by the autopsy. This animal model was created to draw attention to the dangers of any leakage of the fibrosing agent into the medulla of bones.

Relative ability of young and mature muscles to respond to limb lengthening.

The response of the muscle is critical in determining the functional outcome of limb lengthening. We hypothesised that muscle response would vary with age and therefore studied the response of the muscles during tibial lengthening in ten young and ten mature rabbits. A bromodeoxyuridine technique was used to identify the dividing cells. The young rabbits demonstrated a significantly greater proliferative response to the distraction stimulus than the mature ones. This was particularly pronounced at the myotendinous junction, but was also evident within the muscle belly. Younger muscle adapted better to lengthening, suggesting that in patients in whom a large degree of muscle lengthening is required it may be beneficial to carry out this procedure when they are young, in order to achieve the optimal functional result.

Response of the tendon during limb lengthening.

Little is known about the increase in length of tendons in postnatal life or of their response to limb lengthening procedures. A study was carried out in ten young and nine adult rabbits in which the tibia was lengthened by 20% at two rates 0.8 mm/day and 1.6 mm/day. The tendon of the flexor digitorum longus (FDL) muscle showed a significant increase in length in response to lengthening of the tibia. The young rabbits exhibited a significantly higher increase in length in the FDL tendon compared with the adults. There was no difference in the amount of lengthening of the FDL tendon at the different rates. Of the increase in length which occurred, 77% was in the proximal half of the tendon. This investigation demonstrated that tendons have the ability to lengthen during limb distraction. This occurred to a greater extent in the young who showed a higher proliferative response, suggesting that there may be less need for formal tendon lengthening in young children.

Response of the physis to leg lengthening.

UNLABELLED: The physeal response to limb lengthening by callotasis was investigated in an animal model by measuring 1) the growth rate using an oxytetracycline labelling technique, 2) histomorphological changes, and 3) proliferative cellular activity using bromodeoxyuridine. An osteotomy without lengthening stimulated growth at the physis. At 20% lengthening, regardless of the rate of distraction, no significant changes were detected. However, the growth rate was drastically reduced after 30% or more of lengthening and in 50% of cases there was evidence of premature closure of the distal growth plate. CLINICAL RELEVANCE: Caution should be exercised in carrying out high percentages of lengthening of children's bones.

Effect of Val34Leu polymorphism on the activation of the coagulation factor XIII-A.

Coagulation factor XIII (FXIII) is a protransglutaminase involved in the last step of the coagulation cascade by stabilising the fibrin clot. Recently, a common variation (FXIII Val34Leu) has been associated with a decreased risk of myocardial infarction and deep venous thrombosis. Val34Leu is critically located near the thrombin activation site of FXIII-A. In this study we investigated its effects on the activation of FXIII. Both recombinant and platelet-derived FXIII Val34Leu variants were shown to be more susceptible to thrombin cleavage than the wild type FXIII. The rate of enzymatic activation of FXIII Val34Leu was found increased, however, the specific activity of fully activated wild type FXIII and the Val34Leu mutant did not differ. During the course of thrombin-induced activation of FXIII fibrin gamma-chain dimerisation and alpha-chain polymerisation developed more rapidly with the Val34Leu mutant. The increased rate of fibrin stabilisation brought about by the Val34Leu FXIII seems to be paradoxically associated with a protective effect against pathological thrombosis.

Val34Leu polymorphism of plasma factor XIII: biochemistry and epidemiology in familial thrombophilia.

Val34Leu polymorphism of the A subunit of coagulation factor XIII (FXIII-A) is located in the activation peptide (AP) just 3 amino acids away from the thrombin cleavage site. This mutation has been associated with a protective effect against occlusive arterial diseases and venous thrombosis; however, its biochemical consequences have not been explored. In the current study it was demonstrated that the intracellular stability and the plasma concentration of FXIII of different Val34Leu genotypes are identical, which suggests that there is no difference in the rate of synthesis and externalization of wild-type and mutant FXIII-A. In contrast, the release of AP by thrombin from the Leu34 allele proceeded significantly faster than from its wild-type Val34 counterpart. By molecular modeling larger interaction energy was calculated between the Leu34 variant and the respective domains of thrombin than between the Val34 variant and thrombin. In agreement with these findings, the activation of mutant plasma FXIII by thrombin was faster and required less thrombin than that of the wild-type variant. Full thrombin activation of purified plasma FXIII of different genotypes, however, resulted in identical specific transglutaminase activities. Similarly, the mean specific FXIII activity in the plasma was the same in the groups with wild-type, heterozygous, and homozygous variants. Faster activation of the Leu34 allele hardly could be associated with its presumed protective effect against venous thrombosis. No such protective effect was observed in a large group of patients with familial thrombophilia.

Injury to the growth plate after Pemberton osteotomy.

The Pemberton osteotomy involves cutting directly into the iliopubic and ilioischial limbs of the triradiate cartilage of the acetabulum. Complete closure of the triradiate cartilage after this osteotomy has been described in case reports. The present experimental study was performed to determine whether physeal osseous bars formed after Pemberton osteotomy. Eight Pemberton osteotomies were performed in six piglets. The animals were killed, and the acetabula were studied with use of radiography, computed tomography, and histological analysis for evidence of physeal injury. Plain anteroposterior radiographs of the pelvis did not clearly demonstrate the formation of osseous bars. However, Bucholz radiographs, made with the acetabulum placed directly on the cassette, showed osseous bars in three of the four specimens that were studied in this manner. Histological sections of the eight specimens of triradiate cartilage demonstrated five osseous bars in the iliopubic limb and four in the ilioischial limb. In two specimens, there was disruption of the cartilage without osseous bridging. Only two of the eight specimens had normal histological findings in both the iliopubic and the ilioischial limb of the triradiate cartilage.

Wound infection after spinal fusion in children with cerebral palsy.

One hundred and seventy-two children with cerebral palsy were operated on for neuromuscular scoliosis by spinal fusion with unit rod instrumentation between January 1988 and June 1996. There were 15 (8.7%) postoperative wound infections (seven deep, eight superficial) in 15 patients (five males, 10 females) who had a mean age of 13.9 years. The mean follow-up after diagnosis of infection was 3.3 years (range, 1-7.2). Twelve of the 15 infected cases, including all seven deep infections, occurred in the distal portion of the incision. In 14 patients, the wound infections were diagnosed within the first 2 months of the original spinal fusion. All the superficial wound infections were treated successfully by local wound care and intravenous antibiotics. The removal of hardware was necessary in the one late deep wound infection that occurred 2 years after the spinal fusion. The remaining six deep infections were treated by irrigation and debridement with the wound left open, allowing it to heal by secondary intention. One patient's wound was closed over suction-irrigation drains; however, due to a recurrent abscess, the wound was reopened and allowed to granulate. All the wound infections occurred in severely neurologically involved spastic quadriplegics who were nonambulatory and severely mentally retarded and had seizure disorders.

Legg-Calvé-Perthes disease in girls. A comparison of the results with those seen in boys.

We reviewed the records and roentgenograms of all patients with Legg-Calvé-Perthes disease who had been seen at our institution between 1940 and 1996. One hundred and five girls (122 hips) and 470 boys (531 hips) were identified. Thus, 18 per cent of the 575 patients in the present series were girls. Seventeen (16 per cent) of the girls and sixty-one (13 per cent) of the boys had bilateral involvement. Although more girls than boys had severe involvement of the femoral head and the lateral pillar, we could not detect a significant difference between the two groups with respect to the distribution of the involvement of the hips according to the system of Catterall or the lateral pillar classification (p > 0.05, beta = 0.99). Serial roentgenograms that showed all four stages of the disease according to the system of Waldenström were available for fifty-two hips in girls and 184 hips in boys. A review of these roentgenograms revealed that the average ages of the girls at the stages of necrosis, fragmentation, reossification, and remodeling were 6.8, 7.3, 7.9, and 9.5 years, respectively, whereas the average ages of the boys were 6.8, 7.3, 7.9, and 9.9 years, respectively. Girls, however, had closure of the affected proximal femoral physis at an average age of 12.9 years, whereas boys had closure at an average age of 15.8 years. Therefore, girls had a shorter potential period for remodeling of the femoral head (average, 3.4 years) compared with boys (average, 5.9 years). Sixty-four girls (seventy-eight hips) and 363 boys (416 hips) had reached skeletal maturity by the time of the latest follow-up and were evaluated according to the system of Stulberg et al.; we could not detect a significant difference between boys and girls with respect to the distribution of the hips according to this system (p > 0.05, beta = 0.99). Although the numbers were too small for statistical analysis, our findings suggest that boys and girls who have the same Catterall or lateral pillar classification at the time of the initial evaluation can be expected to have similar outcomes according to the classification system of Stulberg et al.

Femoral remodelling after subtrochanteric osteotomy for developmental dysplasia of the hip.

Children who present late with hip dislocation may require femoral osteotomy after reduction, to correct valgus and anteversion deformity of the femoral neck. After these procedures proximal femoral growth is unpredictable. We have studied proximal femoral growth in 40 children who had been treated by femoral osteotomy. Preoperatively, the mean femoral neck-shaft angle was 5 degrees greater on the affected side than on the contralateral side. Postoperatively, it was 28 degrees less. There was progressive recorrection; after five years the angle was not significantly different from that on the contralateral side. In our series 70% of the capital epiphyses became abnormally shaped, taking the appearance of a 'jockey's cap'. All the growth plates became angulated but this corrected with time. Correction of the neck-shaft angle probably results from the more normal mechanical environment provided by reduction. The abnormal radiographic appearance of the epiphysis and growth plate is probably due to the rotation produced by the osteotomy.

Medial-approach open reduction of hip dislocation in amyoplasia-type arthrogryposis.

In 95 children with amyoplasia-type arthrogryposis multiplex congenita, 40 hip dislocations in 26 patients were found. In 16 of these 26 patients, bilateral (nine patients) and unilateral (seven patients) dislocations were reduced by a medial-approach open reduction. The mean age at the time of surgery was 8.9 months. Acetabular development was satisfactory. Complications included one early redislocation, two hips with stiffness, and four of 25 hips with avascular necrosis (types 1 and 2). Overall 80% (five of seven unilateral, 15 of 18 bilateral hips) were rated good and 12% fair, and 8% (one of seven unilateral, one of 18 bilateral) were poor. Stiffness or asymmetry was not observed in the nine bilateral cases. This study suggests that dislocations in infants with amyoplasia may be successfully reduced by medial-approach open reduction. Bilateral reduction and concurrent correction of other lower limb contractures may be accomplished during the same surgical session.

Osteoarticular manifestations of congenital insensitivity to pain with anhydrosis.

We report the case history of a boy who suffered from congenital insensitivity to pain with anhydrosis. We discuss the orthopaedic disorders occurring in 21 cases reported in the literature.

Idiopathic carpotarsal osteolysis with Bartter's syndrome. A case report and review of the literature.

Idiopathic carpotarsal osteolysis is 1 of the rare types of disappearing bone diseases characterized by painful limitation of movement and deformity of the wrist and tarsal joints, and associated with frequent episodes of joint inflammation and a radiographic appearance of osteolysis. Reported here is an 8-year-old girl with idiopathic carpotarsal osteolysis and Bartter's syndrome with significantly decreased re-absorption of chloride at distal renal tubules, low level of serum potassium, a metabolic alkalosis, and high levels of aldosterone and renin. Bartter's syndrome associated with idiopathic carpotarsal osteolysis has not been published previously. The typical plantar nodule in this patient confirmed the presence of fibromatosis with aggressive histologic signs in light and electron microscopic examination.

[Experience with methylprednisolone-acetate therapy of juvenile bone cysts]. / A juvenilis csontcysta methylprednisolon-acetáttal történö kezelésével szerzett tapasztalataink.

Authors injected until 1989 in 92 patients with juvenile bone cyst Methylprednisolone-acetate locally after punction of the cyst. They report on the results and give a short literary review.

[A new case of idiopathic carpo-tarsal osteolysis with Bartter's syndrome (case report and short review of the literature)]. / Az idiopathiás carpo-tarsalis osteolysis (ICTO) egy újabb esete Bartter-szindrómával (esetismertetés és az irodalom rövid áttekintése).

The author gives in connection with the description of the case of an idiopathic carpo-tarsal osteolysis of a six year old female patient a short literary review of this rare disease. The nephropathy previously frequently observed in the cases described appeared in the form of a typical Bartter's syndrome, this connection was not yet observed before.

Surgical treatment of the hip in cerebral palsy.

Hips are affected generally in CP but in favourable cases we can prevent dislocation by adductor tenotomy performed in time (530 cases). Posterior transposition of the origins of the adductor muscles is performed if the patient walks with inwood rotated inferior extremities (105 cases). In the treatment of real subluxation and valgus deformity the open tenotomy of adductor muscles and femur osteotomy with derotation and varisation were used (180 cases). We accomplish open reduction in frank dislocation (35 cases). In such cases it is often necessary to shorten the femur. Also normal acetabulum is found in elder children.

[Management of hallux varus]. / A hallux varus és kezelése.

Authors describe the congenital and acquired forms of the rarely occurring hallux varus on the basis of the material of 14 patients, treated in 1951-87 on the Orthopaedic Department of the Semmelweis University Budapest. In 11 cases the deformity was congenital and in the majority bilateral, multiple, together with other foot deformities.