[Long-term results of Imhäuser osteotomy for chronic slipped femoral head epiphysiolysis]. / Langzeitergebnisse der Imhäuser-Osteotomie bei Epiphyseolysis capitis femoris lenta.

BACKGROUND: The treatment of slipped capital femoral epiphysis (SCFE) has produced in its complexity a great deal of discussion. One well-established method of operative treatment is the corrective intertrochanteric osteotomy according to Imhäuser. This study presents the clinical and radiological long-term results and the biomechanical impacts of this invasive intervention. PATIENTS AND METHODS: A total of 28 patients with chronic SCFE with slip angles between 30° and 60° were treated by Imhäuser osteotomy at an average age of 13.7 years (SD± 2.2 years, range 9-19 years) and were reexamined after an average period of 24 years (SD± 6.7 years, range 12-32 years). RESULTS: The results of the biomechanical analyses indicated an increase of force affecting the articulating joint postoperative compared to preoperative, even though the pressure on the joint decreased. This can be interpreted as a result of the enlargement of the articulating joint surface. Of the patients 17 achieved an excellent Harris hip score, 8 a good and 3 a satisfying result in the clinical assessment. There were significant differences in the degree of arthrosis between the side with and the side without osteotomy. Out of 27 cases 10 showed a decrease in the degree of arthrosis of the side with osteotomy whereas 3 cases showed an increase. In 14 cases no difference was measured. CONCLUSIONS: Despite the valgisation the Imhäuser osteotomy relieves the hip joint thus probably counteracting degenerative alterations even though being unable to fully prevent this progress. The good biomechanical, clinical and radiological results support indications for Imhäuser osteotomy for slip angles between 30° and 60°.

[Botulinum toxin a treatment in spastic forms of cerebral palsy: a retrospective clinical study]. / Anwendung von Botulinumtoxin A in der Behandlung der spastischen infantilen Zerebralparese: Retrospektive klinische Beobachtungsstudie.

BACKGROUND: Botulinum toxin therapy now has a firm place in the treatment of spastic forms of cerebral palsy in children. This paper considers the subjective and practical results and the degrees of satisfaction from the point of view of the patients and their families. It also documents the data of infiltrations carried out under anaesthesia. PATIENTS AND METHODS: 57 patients with an average age of 11 (± 6.7; 2-30) and with infantile spastic cerebral palsy underwent altogether 118 botulinum toxin A infiltrations. The patients were divided into two groups: those with spastic hemiparesis or diparesis, and those with tetraparesis, and then compared with each other. The results of the treatment were evaluated from the point of view of the patients and their families with the help of a specially developed questionnaire. RESULTS: The study shows that, broadly-speaking, patients less affected with spastic hemiparesis or diparesis felt they profited more from the treatment than patients more severely affected with tetraparesis. The statistics also show that the first group's expectations were significantly more often fulfilled and that they more frequently perceived greater success after each infiltration than the group with tetraparesis. Most patients and their families from both groups were satisfied with the treatment. CONCLUSION: Patients and their families feel that the use of botulinum toxin in the management of spastic cerebral palsy in children is an effective and accepted form of treatment. The aims of the therapy should be defined accurately and realistically before starting treatment, especially for those severely affected with tetraparesis, and discussed in detail with both the patient and his/her family.

[Current strategies of conservative and operative treatment of the most frequent muscular disorders]. / Aktuelle Strategien der konservativen und operativen Therapie der häufigsten Muskelerkrankungen.

Though up to now no causal treatment for the majority of neuromuscular disorders is available, their disease progress and above all the quality of life of these patients can be decisively improved by established medical procedures. The main symptom is variably rapid, progressive muscle weakness, which leads to muscular imbalance and differently manifested impairment of motor functions. This results in the essential orthopaedic problems in these patients, e.g. contractures and deformities of the lower and upper extremities, foot deformities and sitting instability due to progressive scoliosis. Since the affected muscles have no physiological adaptability, they cannot be trained like healthy muscles. The orthopaedic treatment includes conservative methods, e.g. physiotherapy, orthotic devices and aids as well as surgical spine stabilisation and correction of the contractures of the lower extremities. Very important in the care of patients with neuromuscular disorders are the early prophylaxis and treatment of respiratory insufficiency by regular respiratory therapy to learn breathing and coughing techniques and self-exercises as well as the timely initiation of assisted ventilation.

Onset and duration of mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy.

BACKGROUND: To determine the response to mivacurium, we prospectively studied onset time and complete spontaneous recovery from mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy (DMD). METHODS: Twelve boys with DMD, age 5-14 yr, seven of them wheelchair-bound, ASA II-III, and 12 age- and sex-matched controls (ASA I) were enrolled in the study. Anaesthesia was induced with fentanyl 2-3 microg kg(-1) and propofol 3-4 mg kg(-1) titrated to effect, and maintained by continuous i.v. infusion of propofol 8-12 mg kg(-1) and remifentanil as required. The lungs were ventilated with oxygen in air. Neuromuscular transmission was assessed by acceleromyography using train-of-four (TOF) stimulation every 15 s. After baseline readings, a single dose of mivacurium 0.2 mg kg(-1) was given. The following variables were recorded: (i) lag time; (ii) onset time; (iii) peak effect; (iv) recovery of first twitch from the TOF response to 10, 25 and 90% (T(10), T(25), T(90)) relative to baseline; (v) recovery index (time between 25 and 75% recovery of first twitch); and (vi) recovery time (time between 25% recovery of first twitch and recovery of TOF ratio to 90%). For comparison between the groups the Mann-Whitney U-test was applied. RESULTS: There were no differences between the groups in lag time, onset time and peak effect. However, all recorded recovery indices were significantly (P<0.05) prolonged in the DMD group. The median (range) for time points T(10), T(25) and T(90) in the DMD and control group was 12.0 (8-16) vs 8.4 (5-15) min, 14.1 (9-20) vs 10.5 (7-17) min and 26.9 (15-40) vs 15.9 (12-23) min, respectively. The recovery index and recovery time were similarly prolonged in the DMD group. CONCLUSIONS: These results support the assumption that mivacurium-induced neuromuscular block is prolonged in patients with DMD.

Value of myofibrillar protein catabolic rate in Duchenne muscular dystrophy. A study after lower limb surgery.

Previous studies have shown a decreased progression of the course in Duchenne muscular dystrophy (DMD) patients treated by lower limb surgery in early childhood. The use of 3-methylhistidine (3-MH) excretion and 3-MH/creatinine excretion ratio as an appropriate indicator for the myofibrillar protein catabolic rate (MPCR) in muscle disorders is discussed controversially. To explore this issue we studied the renal excretion of (3-MH) over a period up to 24 months (on average 20.8 +/- 1.4) after operation in 15 consecutive DMD patients with an average age at operation of 8.75 (+/- 2.43 years) to evaluate the myofibrillar protein catabolic rate. No significant change of the MPCR could be found in our population over the follow-up period. However, the formula for the calculation of the MPCR contains quantities which are not precisely known in DMD or assumed to be constant over the progressive course of DMD. Summarizing MPCR cannot be recommended for the assessment of therapeutic efficacy in DMD.

Pain therapy following joint replacement.A randomized study of patient-controlled analgesia versus conventional pain therapy.

A prospective randomized trial in 42 patients undergoing elective total hip or knee arthroplasty under general anaesthesia was carried out to evaluate the efficacy of patient-controlled analgesia (PCA) versus demanded conventional pain therapy (CPT) for controlling postoperative pain. Four patients had to be excluded from the study (2 postoperative confusion, 1 elevated piritramid dosage caused by chronic pain therapy, 1 stressed by PCA pump handling). PCA group (n = 19) received piritramid via PCA pump, CPT group (n = 19) received tramadol (oral or intramuscularly) or piritramid intravenously. PCA or CPT was started in the intensive care unit. Pain was measured with a standard 100 mm visual analogue scale (VAS) for 60 h postoperatively. Over this period of time, no significant differences were found in the pain score of both groups, nor did the incidence of side-effects differ significantly. The PCA group required on average twice as much piritramid-equivalent than the CPT group (P < 0.001). Patient satisfaction was good in both groups, but significantly better in the PCA group (P < 0.01), although the measured postoperative individual pain scores were above the preoperatively determined individual subjective pain threshold in the majority of both groups. From these results we draw the conclusion that even if the patients feel satisfied by the pain therapy administered, the majority are objectively treated below their individual subjective pain threshold.

Correlation of muscle fiber type measurements with clinical and molecular genetic data in Duchenne muscular dystrophy.

Clinical improvement following surgery in patients with Duchenne muscular dystrophy (DMD) may be influenced by the severity of muscle fiber damage. This study correlates morphometric alterations of muscle fiber types, severity of fat tissue proliferation and fibrosis with Western blots, multiplex polymerase chain reaction (PCR), and postoperative state in DMD. The main results of this study show that the mean diameter of type 2 fibers is usually markedly larger than that of type I fibers in DMD although the number of type 2 fibers is severely reduced. The mean percentage of the remaining type 1 fibers was in the range of 57-82%. The morphometric and histopathological results were in agreement with the clinically estimated postoperative state, especially in the patients who presented a severe state or suggestive clinical improvement. It is concluded that combination of both quantitative and qualitative evaluation of muscle biopsies is suitable for better evaluation of the postoperative state in patients with DMD, whereas severity of exon deletions correlated poorly with morphometry and postoperative clinical state.

Lower limb surgery in Duchenne muscular dystrophy.

Two hundred and thirteen of 428 patients with Duchenne muscular dystrophy (DMD) of a prospective and open study were operated on bilaterally with hip and knee release, aponeurectomy of the iliotibial band and Achilles tendon lengthening. In 87 patients the operation was carried out during early restrictions of the lower limb joint mobility at an average age of 6.56 years (4.02-8.26, SD 1.42). The follow-up was on average 5.4 years (0.25-9.01, SD 2.7). This group was compared to a control group (natural history) consisting of 100 non-operated DMD patients. A significant (P < 0.001) release of the contractures could be obtained. Loss of walking ability occurred in the control group at an average of 9.29 years (5.85-13.63, SD 1.98) and in the operated group at an average of 10.55 years (8.17-14.39, SD 1.76). This shows that early lower limb surgery leads to a prolongation of independent ambulation of 1.25 years on average. In contrast to the patients of the control group all treated patients between ages 6 and 8 years could walk independently. The positive influence of early lower limb surgery could also be shown by the development of Hammersmith motor ability score, CIDD (Council of Investigation of Duchenne Dystrophy) grading and Vignos scale. Nevertheless, in consideration of the well-known course of DMD not only the prolongation of ambulation but also the achieved prolongation of assisted standing ability with no or mild contractures are aims of lower limb surgery. Since no improvement of muscle strength could be observed after lower limb surgery, further studies have to investigate if additionally administered steroids can prolong ambulation after early lower limb surgery.

Platelet function deficiency in Duchenne muscular dystrophy.

Platelet function and plasmatic coagulation of 51 patients with Duchenne muscular dystrophy (DMD) were studied in order to find an explanation for the known substantial blood loss in spinal surgery of these very patients. A normal plasmatic coagulation and a slight but not significant increase of bleeding time was found. However, a significant deficiency of platelet adhesion and ristocetin induced aggregation (P < 0.001) as well as a marked reduction of expression of glycoprotein i.v. (synonyms: GPIV, GPIIIb, CD36) to 50% were detected. We conclude from our study, that this finding of deficiency of platelet function in DMD patients is of no importance in everyday life and minor operations, e.g. lower limb surgery. In major spinal surgery, however, the platelet function deficiency occurs. A decompensation of platelet adhesion as well as aggregation capacity can be assumed due to the unavoidable intraoperative dilution effect by the inevitable volume replacement.

Spontaneous osteonecrosis of the femoral condyle: causal treatment by early core decompression.

In 16 patients with an average age of 64.6 +/- 9.8 years and sudden onset of severe knee pain, the initial stage of Ahlbäck disease (spontaneous osteonecrosis of a femoral condyle) was verified by magnetic resonance imaging (MRI) and subsequent histology. The first radiological sign of osteonecrosis (flattening of the affected femoral condyle) was present in only one case. All patients were treated surgically by extra-articular drilling into the affected femoral condyle to achieve core decompression. The knee pain disappeared immediately after surgery in all patients. Successful healing was confirmed by normalization of the bone marrow signal on MRI (on average, 35.8 months follow-up). Core decompression by extra-articular drilling into the femoral condyle can be recommended as an effective treatment in initial osteonecrosis of the knee (still radiologically invisible). However, if radiologically a flattening of the affected femoral condyle becomes apparent, progression of this disease cannot be avoided.

[Characteristics in the treatment of scoliosis in muscular diseases]. / Besonderheiten in der Behandlung von Skoliosen bei Muskelsystemerkrankungen.

INTRODUCTION: Patients suffering from the most frequent muscle disorders Duchenne muscular dystrophy (DMD) and spinal muscular atrophies (SMA), who ceased walking respectively are confined from the outset to the wheel-chair, are developing commonly a progressive scoliosis (collapsing spine) due to an increasing muscle weakness. Basing on the pelvic obliquity these scolioses are leading first of all to problems in sitting as well as difficulties in trunk and head control. Along with the increasing weakness of respiratory muscles these phenomena entail a restrictive respiratory insufficiency. CONSERVATIVE TREATMENT: An effective conservative treatment is not available for these scolioses. The use of a corset, however, can only be taken into consideration as a compromise, either for very young patients or those who refused an operation respectively who have reached an inoperable stage. The exclusive use of so-called "anatomic sitting supports" in the wheel-chair in order to treat or prevent a progressive scoliosis in DMD or SMA is absolutely to be rejected. They should only be applied for very young patients with SMA type II as a transitional solution until a corset or better an surgical stabilisation of the spine will be effected, or as a palliative measure in late stages. SURGICAL TREATMENT: Only the early as possible performed surgical stabilisation of the spine using adequate instrumentation (Luque, CD or modifications), enabling an early mobilization without corset or cast, is the most effective treatment of these scoliosis. Patients with DMD or SMA type III should be stabilized after loss of walking ability and definitive confinement to wheel-chair, if the curve is more than 20 degrees-30 degrees Cobb and progressive and forced vital capacity (FVC) is > 35%. The instrumentation should be applied between D3 or D4 and sacrum. The bony fusion mass should include the lumbar and lumbosacral region. The unfused instrumentation with the telescope-rod after Naumann is a good solution for patients with SMA type II and progressive curves already in the early childhood from ca. 5 years of age. First of all surgical spinal stabilisation improves the sitting comfort. Over and above this the improved cosmetic appearance should not be underestimated for the psychological condition of these patients. Additionally it is proved, that surgical stabilisation of the spine prolongs the life expectancy of patients with DMD. Furthermore stabilization of lung function can be achieved for both DMD and SMA patients in comparison to the natural history of these diseases. Altogether a decisive improvement of quality of life can be reached for all these patients.

Pathogenetic relevance of the pregnancy hormone relaxin to inborn hip instability.

The etiology of inborn hip dysplasia is unknown. In general, a multifactorial genesis is assumed. The influence of hormones on the development of the fetal hip joint and its stability is discussed as well as mechanical influences. This study was carried out with the intention to examine the correlation between the concentration of the pregnancy hormone relaxin and the stability of the hip joint in newborns. Both hips of 90 newborn children were examined clinically and sonographically. In 25 hips (13.9%), pathological sonograms according to the classification of Graf were found. The relaxin concentration was measured in cord blood using a heterologous radioimmunoassay. Statistical evaluation revealed an insignificant decrease of relaxin concentration with increasing sonographic hip instability. The results indicate that hip instability frequently occurs with decreasing relaxin concentration. These facts contradict the earlier assumption that hip instability coincides with increased relaxin concentrations in newborns. We assume that there is a worse preparation of the pelvis and the birth canal during pregnancy due to the lower relaxin concentration and thus that there could be a higher pressure on the fetus in the perinatal phase. A decreased relaxin concentration seems to have no direct effect on the hip joint tissue, but indirectly there is consequent rigidity of the tissue in mother and child, which can further promote the development of hip instability.

Scoliosis in Duchenne muscular dystrophy: aspects of orthotic treatment.

The x-linked Duchenne muscular dystrophy (DMD) is the most frequent generalized muscle disorder arising from a lack of the sarcolemmic protein "dystrophin". Patients with DMD develop in the majority a progressive scoliosis when they cease walking and/or standing at the age of 10 years and become confined to a wheelchair. Increasing muscle weakness leads to a progression of the curvature, the pelvic tilt and problems in sitting. Together with the simultaneous progressive weakness of the respiratory muscles a restrictive pulmonary insufficiency will occur. Surgical stabilization of the spine (> 20 degrees Cobb, forced vital capacity > 35%) by an adequate multisegmental instrumentation enabling early mobilization is now the treatment of choice. However, orthotic treatment may offer an acceptable compromise in exceptional cases, if the patient rejects surgical intervention or is in the late (inoperable) stages of the disease. Such a treatment is superior to a primary sitting support provision with insufficient possibilities of correction. The authors' experiences with 48 scoliosis orthoses made for 28 patients with DMD are reported. A "double plaster" cast has emerged as the best method to optimize adaption, especially in severe curvatures and the time taken for manufacturing the orthosis. A great deal of experience, patience and the consideration of the patients' individual demands are inevitable for a successful orthotic treatment.

N-aryl-3,3,3-trifluoro-2-hydroxy-2-methylpropanamides: KATP potassium channel openers. Modifications on the western region.

A subset of antiandrogen compounds, the N-aryl-3,3,3-trifluoro-2-hydroxy-2-methylpropanamides 1, were found to activate ATP sensitive potassium channels (KATP) and represent a new class of potassium channel openers (PCOs). A structure-activity relationship was carried out on the western region of this series with the goal of obtaining an activator of the ATP sensitive potassium channel suitable for use in the treatment of urge urinary incontinence. In particular three large 4-(N-aryl) substituents, the (N-phenyl-N-methylamino)sulfonyl, benzoyl, and 4-pyridylsulfonyl moieties, yielded non-antiandrogen, KATP potassium channel openers (39, 41, and 64, respectively) that are bladder selective in an in vivo rat model that simultaneously measures bladder contractions, heart rate, and blood pressure. Substitutions of the aryl rings of 41 and 64 gave several derivatives that also display selectivity in the in vivo rat model; however, none appear to offer a substantial advantage over 41 and 64. The PCO activity of 41 and 64 resides in the (S)-(-) enantiomers. ZD6169, 41(S), has been selected into development for the treatment of urge urinary incontinence.

Anatomo-experimental study for lace fixation of winged scapula in muscular dystrophy.

Winging of the scapula is one of the major features of the rare facio-scapulo-humeral muscular dystrophy. Several methods of retention and fixation of the scapulae have been published, but most have technical disadvantages or complications. A modified method of operative fixation of the scapula to the chest using three polyester laces is described with the results of cadaveric studies on the stability of this system. In order to determine the optimal region for the scapula fixation using polyester laces we performed pull-out tests on twenty cadaver scapulae. Four points of insertion in the inferior part of the scapula were tested. The lateral margin showed the best results with regard to the tensile strength and the morphology of the resulting fractures. The elongation of the laces was measured as well. Compared to scapulothoracic arthrodesis interscapulo-scapulocostal scapulopexy leads to greater preserved mobility between the scapula and the chest wall and conserves vital capacity.

An experimental study of tensile strength of six different suture techniques.

Tests were carried out on the strength of six different suture techniques used to join polyester lace ends, for example, in interscapulo-scapulocostal scapulopexy. The following sutures were used: four 5-cm rows of a continuous suture, four 2.5-cm rows of a continuous suture, two 5-cm rows of a continuous suture, 4 or 8 interrupted sutures, and 4 U-sutures. The laces were tested in tension, and load and elongation recorded. The strongest techniques involved a suture of four 5-cm rows of continuous thread (average maximum load at point of failure 1484 N). This was higher than the maximum load at failure of the point of attachment to the scapula.

[The MR tomography of avascular necrosis of bone: the primary findings and the follow-up observations after core decompression]. / MR-Tomographie avaskulärer Knochennekrosen: Primärbefunde und Verlaufsbeobachtungen nach Markraumdekompression.

PURPOSE: To assess the signal changes of avascular bone necrosis after core decompression. PATIENTS AND METHODS: 17 patients with avascular epiphyseal necrosis were examined by MRI using T1-weighted spin echo sequences before and after gadopentetate dimeglumine application, T2-weighted spin echo sequences and in some patients with fat-saturated 2D gradient echo sequences up to 22 months after core decompression. RESULTS: All patients but one recovered from symptoms after core decompression. Although the signal morphology of the necrotic area remained unchanged in the majority of the cases, a decrease of the joint effusion was observed as well as an ongoing signal increase after gadopentetate dimeglumine application. The last examinations displayed similar signal characteristics as on the preoperative scans; however, a reduction of the necrotic zone became evident. CONCLUSIONS: The decrease of joint effusion indicates successful core decompression, while a persistent signal increase after gadopentetate dimeglumine application reflects the viability of the necrotic area as well as an ongoing healing process.

Ipsilateral peroneus brevis tendon grafting in a complicated case of traumatic rupture of tibialis anterior tendon.

Ruptures of tibialis anterior tendon can be caused by open, closed, direct, or indirect trauma, as well as spontaneously. Sixty-three cases of tibialis anterior tendon ruptures have been reported in the international literature. The treatment of choice is the surgical end-to-end or side-to-side anastomosis after previous Z-lengthening. The case of a 28-year-old world-class female triathlete who sustained an open laceration of the tibialis anterior tendon from the bicycle chain guard is reported. The primarily applied tendon suture became infected and a wound revision with wide resection of the tendon stumps was necessary. This lead to an extensive defect of the tendon combined with a deep-seated keloidal scar reaction of the skin. The surgical closure was performed using free ipsilateral peroneus brevis tendon grafting. Four months after the operation the patient was completely rehabilitated. Eight months later she became the second European triathlon champion.

[Differential therapy of traumatically-induced persistent posterior shoulder dislocation. Review of the literature]. / Differentialtherapie der traumatisch induzierten persistierenden hinteren Schulterluxation. Eine Literaturstudie.

Three-hundred and twenty-nine cases of posterior dislocation of the shoulder documented in 300 articles published in the international literature are reviewed. They included 130 cases in which the duration of the dislocation was longer than 6 weeks and the dislocation could be classified as persistent primary dislocation. This group is the second largest group following that with acute primary dislocation. The mechanism of injury may be direct or indirect force: trauma, convulsions or electrocution are usually responsible for this type of dislocation, which often persists for longer than 6 weeks. Anatomically, 97.5% of dislocations are classified as subacromial. Posterior dislocation of the shoulder is commonly misdiagnosed on plain antero-posterior radiographs, and in over 50% of cases the diagnosis was missed on first examination. The typical signs of primary traumatic posterior dislocation of the shoulder are described. Management of persistent traumatic posterior dislocation of the shoulder depends on the size of the anterior Hill-Sachs lesion, the precipitating mechanism and the duration of dislocation. The results of 109 surgically and 24 conservatively treated dislocations of this type that have been published in the international literature are reviewed. Closed reduction is indicated in carefully selected cases with an anterior Hill-Sachs lesion under 15% of the size of humeral head (measured in the axillary view) that has been dislocation for less than 2 months. In most due to convulsions there was a distinct anterior Hill-Sachs lesion, which led to recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)