Die kongenitale Tibiapseudarthrose

Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging conditions in paediatric orthopaedics. The history of CPT treatment is characterised by repeatedly ineffective surgical interventions with the inevitable outcome of severe disability. Most procedures failed to account for the complexity of the problem. The past two decades have witnessed more experience having been gained with the Ilizarov technique. This method provides a comprehensive approach to all aspects of CPT problem, allowing the surgeon to simultaneously address the problems of union as well as those of deformity, length discrepancy joint function, ankle valgus and weight bearing. It appears that the goal to achieve stable fusion before the child begins school is attainable. In the pre-pseudoarthrotic stage, bracing should be preferred to surgery until the patient is in an older age group. This study reports on the results of 19 patients: 15 have finished treatment, and 14 of them with a stable fusion at follow-up. The results were highly encouraging in reaching the ultimate goal of avoiding amputation and achieving a well-functioning limb.     

Testosterone affects language areas of the adult human brain

Although the sex steroid hormone testosterone is integrally involved in the development of language processing, ethical considerations mostly limit investigations to single hormone administrations. To circumvent this issue we assessed the influence of continuous high‐dose hormone application in adult female‐to‐male transsexuals. Subjects underwent magnetic resonance imaging before and after 4 weeks of testosterone treatment, with each scan including structural, diffusion weighted and functional imaging. Voxel‐based morphometry analysis showed decreased gray matter volume with increasing levels of bioavailable testosterone exclusively in Broca's and Wernicke's areas. Particularly, this may link known sex differences in language performance to the influence of testosterone on relevant brain regions. Using probabilistic tractography, we further observed that longitudinal changes in testosterone negatively predicted changes in mean diffusivity of the corresponding structural connection passing through the extreme capsule. Considering a related increase in myelin staining in rodents, this potentially reflects a strengthening of the fiber tract particularly involved in language comprehension. Finally, functional images at resting‐state were evaluated, showing increased functional connectivity between the two brain regions with increasing testosterone levels. These findings suggest testosterone‐dependent neuroplastic adaptations in adulthood within language‐specific brain regions and connections. Importantly, deteriorations in gray matter volume seem to be compensated by enhancement of corresponding structural and functional connectivity. Hum Brain Mapp 37:1738–1748, 2016. © 2016 Wiley Periodicals, Inc .     

Radiographic evaluation of idiopathic clubfeet undergoing Ponseti treatment

BACKGROUND: The Ponseti method for treatment of idiopathic clubfeet involves the use of serial casts, percutaneous Achilles tenotomy in most cases, and bracing with an abduction orthosis to prevent relapse. Although Ponseti recommended evaluation of the infant clubfoot strictly by palpation, many orthopaedic surgeons still rely on radiographs for decision-making during treatment. The aim of this study was to document with radiographs the effect of percutaneous Achilles tenotomy as described by Ponseti. METHODS: We conducted a study of idiopathic clubfeet treated, at two centers, with the Ponseti method, including percutaneous Achilles tenotomy. Cast treatment was started within three weeks after birth, and radiographs were made before and after the tenotomy. Lateral radiographs with the foot in maximal dorsiflexion at the ankle were made for all patients, and anteroposterior radiographs of the foot were made at one center. The lateral tibiocalcaneal angle, the anteroposterior talocalcaneal angle, and the lateral talocalcaneal angle were measured on the radiographs. Foot dorsiflexion at the ankle was evaluated clinically. The results from both centers were evaluated separately and in combination. RESULTS: Lateral dorsiflexion radiographs that showed the foot and ankle were evaluated for eighty-seven clubfeet, and anteroposterior radiographs that showed the foot were evaluated for sixty-five clubfeet. The mean improvement in the lateral tibiocalcaneal angle after the tenotomy was 16.9 degrees . The mean change in the anteroposterior talocalcaneal angle was 2.1 degrees , and the mean change in the lateral talocalcaneal angle change was 1.4 degrees . The mean increase in clinically measured dorsiflexion after the tenotomy (in sixty-five feet) was 15.1 degrees . Only the lateral tibiocalcaneal angle and dorsiflexion as measured clinically changed significantly after the Achilles tenotomy (p < 0.05). When the results at each center were analyzed separately, they were found to be nearly identical. CONCLUSIONS: The increase in the lateral tibiocalcaneal angle after Achilles tenotomy is essentially the same as the increase in ankle dorsiflexion seen on clinical examination. The anteroposterior and lateral talocalcaneal angles are not influenced significantly by the tenotomy. Radiographs confirmed that the additional dorsiflexion obtained from the percutaneous Achilles tenotomy is true dorsiflexion occurring in the ankle and hindfoot and not in the midfoot. LEVEL OF EVIDENCE: Therapeutic Level IV.     

Same-day <Superscript>90</Superscript>Y radioembolization: implementing a new treatment paradigm

Purpose

To assess the feasibility of conducting pretreatment mesenteric angiography, coil embolization, ^99mTc macroaggregated albumin (^99mTc-MAA) scintigraphy, and ⁹⁰Y radioembolization treatment in a single, same-day, combined outpatient encounter.

Methods

This was a retrospective study of 78 patients treated during the period 2008 – 2015 who were managed in a single outpatient encounter under the guidance of the Interventional Radiology Department and The Nuclear Medicine Department. Pretreatment planning was performed by reviewing baseline imaging and estimated perfused liver volume bearing the tumor. The region of interest was estimated using 3-D software; this value was used for dosimetry planning. Maximum lung shunting fractions of 10 % for hepatocellular carcinoma and 5 % for liver metastases were assumed. Subsequently, hepatic angiography and ^99mTc-MAA scintigraphy were performed followed by ⁹⁰Y treatment in one outpatient encounter. Total in-room procedure time was recorded.

Results

All patients underwent same-day angiography, ^99mTc-MAA scintigraphy and ⁹⁰Y radioembolization. Of the 78 patients, 16 received multiple segmental treatments to both lobes, 44 received treatment to the right lobe, and 18 received treatment to the left lobe. The median dose was 106 Gy. The median number of ⁹⁰Y vials needed was two (range one to six). The median in-room time was 160 min (75 – 250 min). The residential status of the patients was as follows, 18 % (14/78) were local residents, 55 % (43/78) traveled from outside the city limits, 18 % (14/78) were from out-of-state, and 9 % (7/78) were resident abroad. Of the 78 patients, 61 (77 %) had hepatocellular carcinoma, and 17 (22 %) had liver metastases. The median lung dose was 3.5 Gy.

Conclusion

This study demonstrated the feasibility of same-day ⁹⁰Y evaluation and treatment while maintaining the principles of safe and effective ⁹⁰Y infusion including tumoricidal dosimetry (lobar, segmentectomy), minimization of nontarget flow, and minimization of lung dose. This paradigm translates into expeditious cancer care and significant cost savings.

     

Skeletal phenotype/genotype in progressive pseudorheumatoid chondrodysplasia

Clinical Rheumatology - Axial and extra-axial deceleration in function and progressive joint pain with subsequent development of antalgic gait associated with swellings, and stiffness of the joints...     

Radiographic and Tomographic Analysis in Patients with Stickler Syndrome Type I

Objective: To further investigate the underlying pathology of axial and appendicular skeletal abnormalities such as painful spine stiffness, gait abnormalities, early onset osteoarthritis and patellar instability in patients with Stickler syndrome type I. Radiographic and tomographic analyses were organized.Methods: From a series of Stickler syndrome patients followed from early life to late childhood. Ten patients (6 boys and four girls of different ethnic origins were consistent with the diagnosis of Stickler syndrome type I ). Phenotypic characterization was the baseline tool applied for all patients and genotypic correlation was performed on four familiesResults: A constellation of axial abnormalities namely; anterolateral ossification of the anterior longitudinal spinal ligament with subsequent fusion of two cervical vertebrae, early onset Forestier disease (progressive spinal hyperostosis with subsequent vertebral fusion on top of bridging osteophytes and “Bamboo-like spine” resembling ankylosing spondylitis) and severe premature spine degeneration were evident. Appendicular abnormalities in connection with generalized epiphyseal dysplasia were the underlying aetiology in patients with Intoeing gait and femoral anteversion, early onset severe osteoarthritis of the weight bearing joint. Remarkable trochleo-patellar dysplasia secondary to severe osteoarthritis causing effectively the development of patellar instability was additional pathology. Mutation of COL2A1 has been confirmed as the causative gene for Stickler syndrome type IConclusion: We concluded that conventional radiographs and the molecular determination of a COL2A1 in patients with (Stickler syndrome type I) are insufficient tools to explain the reasons behind the tremendous magnitude of axial and appendicular skeletal abnormalities. We were able to modify the criteria of the clinical phenotype as designated by Rose et al in accordance with the novel axial and appendicular criteria as emerged from within our current study.     

Comparison of continuously acquired resting state and extracted analogues from active tasks

Functional connectivity analysis of brain networks has become an important tool for investigation of human brain function. Although functional connectivity computations are usually based on resting‐state data, the application to task‐specific fMRI has received growing attention. Three major methods for extraction of resting‐state data from task‐related signal have been proposed (1) usage of unmanipulated task data for functional connectivity; (2) regression against task effects, subsequently using the residuals; and (3) concatenation of baseline blocks located in‐between task blocks. Despite widespread application in current research, consensus on which method best resembles resting‐state seems to be missing. We, therefore, evaluated these techniques in a sample of 26 healthy controls measured at 7 Tesla. In addition to continuous resting‐state, two different task paradigms were assessed (emotion discrimination and right finger‐tapping) and five well‐described networks were analyzed (default mode, thalamus, cuneus, sensorimotor, and auditory). Investigating the similarity to continuous resting‐state (Dice, Intraclass correlation coefficient (ICC), R²) showed that regression against task effects yields functional connectivity networks most alike to resting‐state. However, all methods exhibited significant differences when compared to continuous resting‐state and similarity metrics were lower than test‐retest of two resting‐state scans. Omitting global signal regression did not change these findings. Visually, the networks are highly similar, but through further investigation marked differences can be found. Therefore, our data does not support referring to resting‐state when extracting signals from task designs, although functional connectivity computed from task‐specific data may indeed yield interesting information. Hum Brain Mapp 36:4053–4063, 2015. © 2015 The Authors Human Brain Mapping Published by Wiley Periodicals, Inc.