[Osteochondritis Dissecans of the Talus - A Critical Review]. / Osteochondrosis dissecans tali ­ eine kritische Übersicht.

The etiology remains unclear. Mechanical factors seem to play an important role. Most histologies report clefts between the cartilage and bone and necrotic areas. Several classifications have been published based on observations from imaging techniques or on intraoperative findings. Clinical symptoms are unspecific. Imaging techniques are of great importance: X-rays of the ankle joint enable a quick diagnostic overview. MRT is the imaging-technique of choice for diagnosing OCDT-lesions. Regarding treatment, a tremendous number of retrospective publications exist. Only a few are classified as level I or II papers. Patients with open physes and low-grade lesions have good results with conservative therapy. Adults do not profit from a longer conservative procedure. Where surgery is necessary, the procedure depends on the stage of the lesion and on the state of the cartilage. With intact cartilage, retrograde procedures or transchondral drilling are to be favoured. Where the cartilage is damaged, several techniques can be used: while techniques such as drilling and microfracturing, or matrix-associated microfracturing produces repair cartilage, other techniques reconstruct the defect with osteochondral grafts or cell-based procedures such as chondrocyte transplantation. Regarding the outcome, it is difficult to make a precise, scientifically-based recommendation which has been confirmed by more than one prospective study. Only limited recommendations are possible: In juveniles with intact cartilage, initially conservative treatment is indicated. If there is no improvement, conservative invasive techniques such as drilling may help. In adults conservative therapy should be limited to a few weeks. Regarding the surgical treatment options, there is a tendency towards better results with procedures which reconstruct the bone and the cartilage and there is also a trend towards better long-term results when co-morbidities are treated. Severe grades of osteoarthrosis are rare.

Radiologically inserted gastrostomy: differences of maintenance of balloon- vs. loop-retained devices.

PURPOSE: To compare outcome and associated complications of ballon- vs. loop-retained devices for radiologically inserted gastrostomy (RIG). METHODS: From 2007 to 2011 233 patients (age 63.7 ± 10.6 years) were referred for a RIG because of pharyngeal stricture Intervention was performed with four different devices: balloon-retained - Freka® GastroTube, Fresenius Kabi (n = 121); MIC® Gastrostomy Feeding Tube, Kimberly-Clark (n = 34); Russell® Gastrostomy Tray, Cook Medical Inc. (n = 17); and loop-retained - Tilma® Gastrostomy Set, Cook Medical Inc. (n = 50). Follow-up was performed with regard to RIG-related complications, cause of removal and fatalities. Revision-free survival times after RIG were evaluated using Kaplan-Meier analysis and group differences by log-rank tests. For analysis of demographic and methodical variables multivariate Cox regression models were used. RESULTS: With a primary technical success rate of 95.3% (222/233) a total of 92 instances of revisions were necessary in 66 patients (66/233, 28.3%) during follow-up (mean 182.8 ± 86.6 days). The most common complication was tube dislodgement (14.3%). There were no significant differences between the distinct devices (p = 0.098), but analyzing the data in subgroups of balloon-compared to loop-retained gastrostomy tubes we observed a significantly higher probability of minor complications for the latter (p = 0.023). CONCLUSION: As it is significantly less prone to minor complications we recommend the use of balloon-retained gastrostomy tubes to improve the practicability and maintenance of RIG.

Comprehensive analysis of dural ectasia in 150 patients with a causative FBN1 mutation.

The purpose of this study was to perform a comprehensive study of dural ectasia (DE) related to FBN1 mutations. We performed a database analysis of two German metropolitan regions of 150 patients (68 men, 82 women; mean age 35 ± 16 years). All patients had a FBN1 mutation and underwent dural magnetic resonance imaging. Age was <16 years in 20, 16-25 in 27, 26-35 in 67, and >35 in 36 patients. Prevalence of dural ectasia was 89% with criteria of Oosterhof and Habermann, 83% with Fattori, 78% with Lundby, and 59% with Ahn. DE was less frequent in patients <16 years with Ahn and Fattori. DE related to skeletal manifestations with all criteria, to aortic Z-scores and mitral valve prolapse with criteria of Habermann and Lundby, and to age with criteria of Fattori. The Fattori-grade of DE increased with age, aortic Z-scores, and skeletal score points. There was no consistent relationship of DE with any type of FBN1 mutation. DE is frequent in patients with FBN1 mutations irrespective of age and its severity increases during life. Criteria of Oosterhof and Habermann yielded most consistent diagnostic results. DE relates to skeletal involvement, aortic Z-scores, and mitral valve prolapse.

Dural ectasia in Loeys-Dietz syndrome: comprehensive study of 30 patients with a TGFBR1 or TGFBR2 mutation.

The purpose of this study was to assess the frequency, severity, and clinical associations of dural ectasia (DE) in Loeys-Dietz syndrome (LDS). Database analysis of three German metropolitan regions identified 30 patients with LDS and TGFBR1 mutation in 6 and a TGFBR2 mutation in 24 individuals (17 men; mean age: 31 ± 19 years), as well as 60 age and sex-matched control patients with Marfan syndrome carrying a FBN1 mutation. DE was present in 22 patients with LDS (73%), and it related to skeletal score points (p = 0.008), non-skeletal score points (p < 0.001), and to the presence of ≥7 systemic score points (p = 0.010). Similarly, the severity of DE was related to body height (p = 0.010) and non-skeletal score points (p = 0.004). Frequency (p = 0.131) and severity of DE (p = 0.567) was similar in LDS and Marfan syndrome. DE is a manifestation of LDS that occurs with similar frequency and severity as in Marfan syndrome. Severity of DE may serve as a marker of the overall connective tissue disease severity. LDS may be considered in patients with DE.

Shiga toxin-producing E. coli O104:H4 outbreak 2011 in Germany: radiological features of enterohemorrhagic colitis.

PURPOSE: In 2011 a nationwide outbreak of Shiga toxin-producing E. coli (STEC) O104:H4 infection occurred in Germany with severe hemorrhagic colitis and hemolytic-uremic syndrome (HUS). We defined abdominal radiologic findings in these patients and correlated them with clinical parameters. MATERIALS AND METHODS: 23 patients (7 men; age: 48 ± 19 years) with O104:H4 colitis and/or HUS received abdominal CT (n = 12) or radiographs (n = 11). Colonic distension, air-fluid levels, and free intraabdominal air were assessed. Colonic wall thickening, contrast enhancement, pericolic stranding, and ascites were evaluated on CT. Laboratory parameters and clinical presentation were reviewed. Chi-square test, Student's t-test, McNemar's test and Spearman correlation were performed. RESULTS: Colonic lumen distension was seen in 16/23 patients (69.6 %). The ascending colon (11/23 patients; 47.8 %) and transverse colon (12/23 patients; 52.2 %) were dilated significantly more often (p = 0.006 and p = 0.003, respectively) than the descending colon (1/23; 4.3 %). All 12 patients undergoing CT scanning had abnormally thickened colonic wall segments, 3 (25 %) had pancolic involvement and 9 (75 %) had segmental involvement. The descending colon was predominantly affected (11/12 patients; 91.7 %) and thickened significantly more often than other colonic segments (p < 0.001). CONCLUSION: The segmental type of STEC O104:H4 colitis mainly affects the descending colon with upstream distension of the transverse/ascending colon and differs from other types of colitis.

Is high-resolution ultrasonography suitable for the detection of temporomandibular joint involvement in children with juvenile idiopathic arthritis?

OBJECTIVES: The purpose of this study was to determine the potential of high-resolution ultrasonography for the detection of temporomandibular joint (TMJ) changes in children with juvenile idiopathic arthritis (JIA). METHODS: We investigated prospectively 20 children (17 female and 3 male; mean age 11.06 years, standard deviation 3.43 years) with TMJ disorders caused by JIA, over a period of 16 months. Using a 12 MHz array transducer, four images in each TMJ (160 images) were acquired. Each image was analysed with regard to five different aspects (condylar erosion, thickness of the condylar disc, synovial thickness, joint effusion and enlargement of the intra-articular space). RESULTS: Diagnosis of JIA was ensured for every child and involvement of the TMJ was proven by MRI. Overall 287 changes (35.9%) were detected by using high-resolution ultrasonography. On 124 images (77.5%) condylar erosions were diagnosed; on 55 images (34.4%) synovial thickness was abnormal; on 48 images (30%) we could see higher thickness of the condylar disc; on 40 images (25%) irregularities of the bony surface were detected; and on 20 images (12.5%) we found joint effusion. CONCLUSION: High-resolution ultrasonography could be a sufficient diagnostic method, especially for the detection of condylar involvement in children with JIA, even if not all parts of the TMJ are visible for ultrasonography. High-resolution ultrasonography is a valuable tool in particular situations: (i) when MRI examination is not available; (ii) when children fear MRI examination; (iii) in more advanced stages of JIA; and (iv) for monitoring the progression of TMJ involvement and response of therapy.

Comparison of diagnostic accuracy of Magnetic Resonance Imaging and Multidetector Computed Tomography in the detection of pelvic fractures.

OBJECTIVE: To compare diagnostic accuracy and interobserver reliability of Magnetic Resonance Imaging (MRI) and Multidetector Computed Tomography (MDCT) in the detection of acute pelvic fractures. MATERIALS AND METHODS: In 38 consecutive patients (mean age 74.7 years) with a positive finding of anterior pelvis fracture in the conventional X-rays, pelvic MRI and MDCT were performed for further evaluation of pelvic ring fractures. Two radiologists independently read all data sets. Sensitivity and specificity were calculated based on mean scores for each method. Sensitivities of CT and MRI were compared using a paired proportion test (McNemar). Diagnostic validity of both methods was assessed by the interobserver variability using kappa statistics. Combined clinical data and findings from all imaging studies served as the reference standard. RESULTS: 122 fractures were identified in the reference standard (37 sacral, 58 pubic, 22 acetabular, 1 ischial, 4 ilial). On average, MRI detected 96.3% whereas CT detected 77% of all fractures. With regard to sensitivity, MRI proved to be significantly better compared to MDCT (observer 1, p=0.0009; observer 2, p=0.0003 by observer 2). In particular, MRI performed better in the depiction of sacral fractures, reaching a sensitivity of 98.6% compared to 66.1% at CT. The interobserver variability was determined to be very good (k=0.955 for MRI and 0.902 for MDCT). CONCLUSION: MRI reaches a significantly higher sensitivity than CT in the detection of acute pelvic fractures, particularly of the sacrum. Especially in elderly patients with suspicion of a sacral fracture and negative results at MDCT, MRI may be considered as the next step in diagnostic workup.

Analysis of phenotype and genotype information for the diagnosis of Marfan syndrome.

Marfan syndrome is considered a clinical diagnosis. Three diagnostic classifications comprising first, Marfan genotype with a causative FBN1 gene mutation; second, Marfan phenotype with clinical criteria of the original Ghent nosology (Ghent-1); and third, phenotype with clinical criteria of its current revision (Ghent-2) in 300 consecutive persons referred for confirmation or exclusion of Marfan syndrome (150 men, 150 women aged 35 ± 13 years) were used. Sequencing of TGBR1/2 genes was performed in 128 persons without FBN1 mutation. Marfan genotype was present in 140, Ghent-1 phenotype in 139, and Ghent-2 phenotype in 124 of 300 study patients. Marfan syndrome was confirmed in 94 and excluded in 129 persons consistently by all classifications, but classifications were discordant in 77 persons. With combined genotype and phenotype information confirmation of Marfan syndrome was finally achieved in 126 persons by Ghent-1 and in 125 persons by Ghent-2 among 140 persons with Marfan genotype, and exclusion was accomplished in 139 persons by Ghent-1 and in 141 persons by Ghent-2 among 160 persons without Marfan genotype. In total, genotype information changed final diagnoses in 22 persons with Ghent-1, and in 32 persons with Ghent-2. It is concluded that genotype information is essential for diagnosis or exclusion of Marfan syndrome.

Follow-up of abdominal aortic aneurysm after endovascular aortic repair: comparison of volumetric and diametric measurement.

PURPOSE: To determine the correlation of maximal diameter measurements with volumetric evaluation of size after endovascular aortic repair (EVAR) of abdominal aortic aneurysms (AAA) using computed tomography angiography (CTA) and to survey its applicability for clinical follow-up. MATERIALS AND METHODS: 73 consecutive patients (2 females, 71 males; age 38-84 years; mean age, 69.1 ± 8 years) with AAA were treated with percutaneous EVAR in a single institution. For follow-up, CTA was performed periodically after EVAR. Images were evaluated for maximal diameter in consensus by two experienced radiologists. Using OsirixTM, volumetric measurements were done by one radiologist, including the entire infrarenal abdominal aorta. RESULTS: In 73 patients 220 CTA examinations were performed after EVAR with a mean follow-up of 17.3 months (range, 1.8-42.7 months). The mean postinterventional volume of aneurysm was 165.63 ml ± 93.29 ml (range, 47.94-565.67 ml). The mean maximal postinterventional diameter was 5.91 ± 1.52 cm (range, 3.72-13.82 cm). At large over the entire observation period a slight, non-significant decrease of 1.6% (2.58ml ± 69.05 ml, range 82.82-201.92 ml) in volumes and a 9.3% (mean 0.55 cm ± 1.22 cm, range 2.85-1.93cm) in diameters were observed. For all examinations a high correlation of volume and diameter was calculated (r = 0.813-0.905; α<0.01). CONCLUSION: For follow-up of abdominal EVAR using CTA there is a high correlation between volumetric and diametric measurements of aneurysm. Based on a daily clinical routine setting, measurements of maximal diameters in cross sectional imaging of AAA after EVAR seems to be sufficient to exclude post interventional enlargement.

Interventional management of intractable sympathetically mediated pain by computed tomography-guided catheter implantation for block and neuroablation of the thoracic sympathetic chain: technical approach and review of 322 procedures.

We retrospectively evaluated the safety and efficacy of computed tomography-guided placement of percutaneous catheters in close proximity to the thoracic sympathetic chain by rating pain intensity and systematically reviewing charts and computed tomography scans. Interventions were performed 322 times in 293 patients of mean (SD) age 59.4 (17.0) years, and male to female ratio 105:188, with postherpetic neuralgia (n = 103, 35.1%), various neuralgias (n = 88, 30.0%), complex regional pain syndrome (n = 69, 23.6%), facial pain (n = 17, 5.8%), ischaemic limb pain (n = 7, 2.4%), phantom limb pain (n = 4, 1.4%), pain following cerebrovascular accident (n = 2, 0.7%), syringomyelia (n = 2, 0.7%) and palmar hyperhidrosis (n = 1, 0.3%). The interventions were associated with a total of 23 adverse events (7.1% of all procedures): catheter dislocation (n = 9, 2.8%); increase in pain intensity (n = 8, 2.5%); pneumothorax (n = 3, 0.9%); local infection (n = 2, 0.6%); and puncture of the spinal cord (n = 1, 0.3%). Continuous infusion of 10 ml.h(-1) ropivacaine 0.2% through the catheters decreased median (IQR [range]) pain scores from 8 (6-9 [2-10]) to 2 (1-3 [0-10]) (p < 0.0001). Chemical neuroablation was necessary in 137 patients (46.8%). We conclude that this procedure leads to a significant reduction of pain intensity in otherwise obstinate burning or stabbing pain and is associated with few hazards.

[Fluoroscopic-guided primary single-step percutaneous gastrostomy: initial results using the Freka® GastroTube]. / Primäre einzeitige durchleuchtungsgesteuerte perkutane Gastrostomie (PG): Erste Ergebnisse mit dem Freka® GastroTube.

PURPOSE: To determine the practicability and outcome of fluoroscopic-guided primary one-step treatment of percutaneous gastrostomy (PG) with the system Freka® Gastro Tube (Fresenius Kabi, Germany). MATERIALS AND METHODS: In 39 patients (mean age 62.7 ± 12.0 years), primary PG was performed based on clinical indication from August 2009 to April 2010. The intervention was performed by an experienced radiologist under aseptic conditions by direct puncture with Freka® Gastro Tube under fluoroscopic guidance. The clinical data and outcome as well as any complications originated from the electronic archive of the University Medical Center Hamburg-Eppendorf. RESULTS: The intervention was technically successful in all 39 patients. Within the mean follow-up time of 155.3 ± 73.6 days, 29 patients (74.4 %) did not experience complications. 10 patients (25.6 %) had to be revised. Complications manifested after a mean of 135.6 ± 61.2 days and mainly corresponded to accidental dislocation (50 %). One patient had to be surgically revised under suspicion of a malpositioned tube and suspected intestinal perforation. Clinically relevant wound infections were not detected. The total costs per patient were 553.17 € for our single-step treatment (OPS 5 - 431.x) vs. 963.69 € (OPS 5 - 431.x and OPS 8 - 123.0) for the recommended two-step treatment. CONCLUSION: Fluoroscopic-guided primary single-step treatment with Freka® Gastro Tube system is feasible and not associated with an increased complication rate when compared to published literature applying a two-step treatment approach. Material costs as well as human and time resources could be significantly reduced using the single-step treatment.

Dural ectasia in individuals with Marfan-like features but exclusion of mutations in the genes FBN1, TGFBR1 and TGFBR2.

Mutations in the genes FBN1, TGFBR1, and TGFBR2 can result in heritable connective tissue disorders comprising the Marfan syndrome and the Loeys-Dietz syndrome. Dural ectasia is a characteristic manifestation of both syndromes. However, dural ectasia has not yet been investigated in connective tissue disorders that are unrelated to mutations in the FBN1, TGFBR1 or TGFBR2 genes. Here, we assessed dural ectasia in 33 individuals both with typical manifestations of heritable connective tissue disease and in whom mutations in all three genes had been excluded. We identified 19 individuals with dural ectasia (58%), who exhibited major skeletal manifestations of the Marfan syndrome more frequently than the remaining 14 persons without dural ectasia (p = 0.06). Moreover, only persons with dural ectasia fulfilled clinical criteria of the Marfan syndrome (p = 0.01). Conversely, aortic aneurysm (12 patients; p = 0.8), aortic dissection (five patients; p = 0.1), spontaneous dissection of the carotid arteries (five patients; p = 1), and mitral valve prolapse (13 patients; p = 0.4) were similarly frequent irrespective of dural ectasia. We conclude that dural ectasia is a marker for connective tissue disease which coincides with skeletal rather than with cardiovascular manifestations, and which may involve currently uncharacterized pathogenetic mechanisms and syndromes.

The use of CT derived solid modelling of the pelvis in planning cancer resections.

CLINICAL PROBLEM: Resection of malignant tumors of the pelvis is demanding. To avoid disabling hemipelvectomies, years ago internal hemipelvectomy combined with partial pelvic replacements had become a surgical procedure. To achieve adequate reconstructions custom-made replacements were recommended. In early stages of the surgical procedure using megaprostheses, individual pelvic models were manufactured. AIM OF THE STUDY: Since little is known about the accuracy of such models we analysed the charts of 24 patients (25 models) for whom an individual model of the osseous pelvis had been manufactured. RESULTS: Two patients refused surgery. In 23 patients partial resection of the bony pelvis was performed followed by a partial pelvic replacement (13x), hip transposition procedure (5x), ilio-sacral resection (4x), or revision surgery. In all patients who received a partial pelvic replacement, the fit of the replacement was optimal. No major unplanned resection was necessary. The same was observed in patients who received a hip transposition procedure or an ilio-sacral resection. Oncologically, in most of the patients we achieved wide resection margins (14x). In 5 patients the margins were marginal (4x) or intralesional (1x). In two cases the aim was a palliative resection because of a metastatic disease (1x) or benign entity (1x). CONCLUSION: Pelvic models are helpful tools to planning the manufacture of partial pelvic replacements and ensuring optimal osseous resection of the involved bone. Further attempts have to be made to evaluate the aim of navigational techniques regarding the accuracy of the osseous and soft-tissue resection.

Sjögren's syndrome of the parotid gland: value of diffusion-weighted echo-planar MRI for diagnosis at an early stage based on MR sialography grading in comparison with healthy volunteers.

PURPOSE: To investigate the value of diffusion-weighted echo-planar imaging (DW-EPI) for quantifying functional changes of the parotid gland in Sjögren's disease and to evaluate whether ADC mapping allows for early diagnosis based on MR sialography grading. MATERIALS AND METHODS: Using a DW-EPI sequence at 1.5 T (b-factors: 0, 500 and 1000 sec/mm (2)), the parotid glands of 52 healthy volunteers and 13 patients with histologically verified affection of Sjögren's disease were examined. All scans were performed prior to and following gustatory stimulation with 5 ml of lemon juice. ADC maps were evaluated by placing an inordinate region-of-interest (ROI) enclosing the entire parotid gland. Sjögren's disease was graded based on MR sialography findings using a 4-point grading-scale. Statistics included student t-test and kappa-analysis. RESULTS: In healthy volunteers mean ADCs of 1.14 x 10 (-3 )mm (2) /sec before and 1.2 x 10 (-3) mm (2) /sec after stimulation were observed. Higher ADCs were determined for early-stage Sjögren's disease, averaging 1.22 x 10 (-3) mm (2) /sec before and 1.29 x 10 (-3) mm (2) /sec after stimulation. Advanced disease revealed significantly lower ADCs (0.97 x 10 (-3) mm (2) /sec (p = 0.002) before and 1.01 x 10 (-3) mm (2) /sec (p < 0.001) after stimulation). CONCLUSION: DW-EPI seems to display functional changes of the parotid gland affected by Sjögren's disease. Combined with MR sialography, it might be a useful tool for discriminating healthy from affected glands and seems to allow differentiation between the early and advanced disease.

Diffusion-weighted echo-planar MR imaging of primary parotid gland tumors: is a prediction of different histologic subtypes possible?

BACKGROUND AND PURPOSE: Our aim was to determine the value of echo-planar diffusion-weighted MR imaging (epiDWI) in differentiating various types of primary parotid gland tumors. MATERIALS AND METHODS: One hundred forty-nine consecutive patients with suspected tumors of the parotid gland were examined with an epiDWI sequence by using a 1.5T unit. Image analysis was performed by 2 radiologists independently, and the intraclass correlation coefficient was computed. Histologic diagnosis was obtained in every patient. For comparison of apparent diffusion coefficients (ADCs), a paired 2-tailed Student t test with a Bonferroni correction was used. RESULTS: In 136 patients, a primary parotid gland tumor was confirmed by histology. Among the observers, a high correlation was calculated (0.98). ADC values of pleomorphic adenomas were significantly higher than those of all other entities, except for myoepithelial adenomas (P = .054). ADC values of Warthin tumors were different from those of myoepithelial adenomas, lipomas, and salivary duct carcinomas (P < .001, 0.013, and .037, respectively). Mucoepidermoid carcinomas, acinic cell carcinomas, and basal cell adenocarcinomas were not differentiable from Warthin tumors (P = .094, .396, and .604, respectively). CONCLUSION: epiDWI has the potential to differentiate pleomorphic adenoma and myoepithelial adenomas from all other examined entities. Due to an overlap not only within the group of benign and malignant lesions but also between groups, diagnoses should not be addressed on the basis of ADC values solely. Therefore, further studies combining DWI, morphologic criteria, and probably other MR imaging techniques seem warranted.

[Pigmented villo-nodular synovitis and teno-synovial giant-cell tumors]]. / Pigmentierte villonoduläre Synovialitis und tenosynovialer Riesenzelltumor.

Pigmented villo-nodular synovitis (PVS) and teno-synovial giant-cell tumors (TSGCT) are rare diseases of joints, tendon-sheaths and other synovial structures. Regarding PVS the knee joint is the most often involved joint and TSGCT are mostly located in the tendon-sheaths of fingers. Both diseases mostly occur in adults but are also observed in children. Symptoms of both diseases are non-specific. Optimal diagnosis includes an X-ray of the involved structure and a MRI. For PVS optimal therapy is a complete synovectomy, whereas, in TSGCT tumors resection of the tumor itself within marginal margins is sufficient. Because of the high rate of recurrence (up to 50 %) in cases suffering from PVS adjuvant therapeutical strategies such as postoperative radiation or radio-synoviorthesis are recommended. Although there are no significant analyses confirming the effect of an adjuvant therapy on the rate of recurrence scientifically at least there is a tendency that these strategies may reduce the rate of recurrence. Malignant dedifferentiation is rarely seen. The prognosis of the mostly benign disease depends on the extent of the disease, the involved structure, additional bony involvement and still existing degenerative changes. In TSGCT no severe sequelae are known. In contrast, PVS in the hip regularly is followed by a secondary osteoarthrosis, in other joints degenerative changes are depending on the still existing changes at the date of diagnosis. Data on the long-term prognosis are rare and only analysed retrospectively.