Unilateral hypertrophic osteoarthropathy in a patient with a vascular graft infection.

We report a patient who presented with high fever; a unilateral, palpable tender swelling along the right shin; and effusions in knee and ankle joints leading to the diagnosis of hypertrophic osteoarthropathy (HOA). The diagnostic workup revealed an infected vascular graft that had been implanted 2 years before because of a ruptured infrarenal aortic aneurysm as the cause of HOA. The patient was treated successfully with antibiotics and surgically replacing the infected graft. Hypertrophic osteoarthritis is a clinical entity characterized by digital clubbing, periostitis, and synovial effusions. Primary and secondary forms have been described. Secondary HOA develops as a consequence of various diseases, mainly intrathoracic malignancies. Vascular graft infection, as reported here, is a rare cause of HOA. This case underlines the typical clinical features of HOA and the importance of a prompt and comprehensive diagnostic workup in cases of HOA. Our aim is to sharpen the awareness of its multiple underlying causes. Unilateral HOA is a rare but strong and important sign of infection of vascular prosthesis.

Whole-body diffusion-weighted MRI in a case of Rosai-Dorfman disease with exclusive multifocal skeletal involvement.

Rosai-Dorfman disease (RDD) is a rare disorder and usually presents with painless bilateral cervical lymphadenopathy. About 43% of RDD patients show extranodal involvement, including bones (8%). As RDD is a systemic disease, which can involve lymph nodes, bones, skin, kidneys, respiratory tract, parotid gland, orbital cavity and the central nervous system, whole-body imaging may be useful for the assessment of extent, distribution and follow-up of disease. Whole-body diffusion-weighted MRI is able to demonstrate lesions and to assess therapy response without the need for radiation or intravenous contrast agent. Here, we report a case of a 15-year-old boy with primary skeletal RDD without lymphadenopathy, who was staged and followed by whole-body diffusion-weighted MRI.

Rescue from hemodialysis by late recanalization of renal artery occlusion.

We report on a patient with terminal renal insufficiency undergoing hemodialysis since four months. Imaging studies showed complete renal artery occlusion of a single kidney with collateral perfusion. Interventional recanalization of the renal artery was successful with a drop of serum creatinine from 1138 to 163 mol/l sparing the patient from further hemodialysis.

Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study.

INTRODUCTION: The acronym SAPHO was introduced in 1987 to unify the various descriptions of a seronegative arthritis associated with skin manifestations and to show synovitis, acne, pustulosis, hyperostosis, and osteitis with and without sterile multifocal osteomyelitis. The etiology of SAPHO syndrome is unknown, but an association with infection by semipathogenic bacteria like Propionibacterium acnes has been suggested. We conducted an interventional study of SAPHO patients receiving antibiotics. METHODS: Thirty-seven patients met the clinical criteria of SAPHO syndrome, 21 of them underwent a needle biopsy of the osteitis lesion, and 14 of them showed positive bacteriological cultures for P. acnes. Thirty patients (14 bacteriological positive and 16 without biopsy) were treated with antibiotics for 16 weeks. The activity of skin disease and osteitis were assessed by a physician using a scoring model (from 0 to 6). In addition, patients completed a Health Assessment Score (HAS, from 0 to 6). The erythrocyte sedimentation rate was determined and a MRI (of the osteitis lesion, radiologic activity score from 0 to 2) was performed in week 1 (W1), week 16 (W16), and week 28 (W28, 12 weeks after antibiotics). RESULTS: Twenty-seven patients continued the medication (azithromycin, n = 25, 500 mg twice a week; clindamycin, n = 1, 300 mg daily; or doxycycline, n = 1, 100 mg daily) for 16 weeks. After W16 the scores for MRI (1.5 to 1.1, P = 0.01), skin activity (3.2 to 1.2, P = 0.01), osteitis activity (4.0 to 2.1, P = 0.02), and HAS (3.3 to 2.1, P = 0.01) decreased significantly. However, this was followed by increasing values for MRI scores (1.2 to 1.4, P = 0.08), skin activity (1.2 to 1.7, P = 0.11), osteitis activity (1.9 to 2.7, P = 0.01), and HAS (2.2 to 3.3, P = 0.02) from W16 to W28. The comparison of the scores in W1 and W28 in these 12 patients showed no significant differences. CONCLUSIONS: For the period of application, the antibiotic therapy seems to have controlled the disease. After antibiotic discontinuation, however, disease relapse was observed. SAPHO syndrome thus groups with other chronic inflammatory arthropathies with a need for permanent therapy.

Unilateral tibia vara in a toddler caused by focal fibrocartilaginous dysplasia.

Focal fibrocartilaginous dysplasia (FFCD) of the tibia is a rare tumor like lesion probably caused by a failure of differentiation in the pes anserinus area. The children usually present with unilateral tibia vara and conspicuous walking features. The radiographic appearance of FFCD is pathognomic. In most cases this benign condition undergoes spontaneous resolution. Curettage or corrective osteotomy is only indicated when the deformity is persistent or progressive. We report the case of a 14 months old toddler diagnosed with FFCD. The characteristic radiographic and MRI features are presented. Further, we present for the first time the sonographic appearance of FFCD.

Stress fracture of the hamate body and fourth metacarpal base following military style push-ups: an unusual trauma mechanism.

We report the case of a 19-year-old male soldier, who sustained stress fractures of the hamate body and fourth metacarpal base due to his daily knuckle push-up routine in the military. We introduce repetitive microtrauma due to daily knuckle push-ups as an unusual, but potential trauma mechanism for metacarpal and carpal stress fractures.

Transhepatic preoperative portal vein embolization using the Amplatzer Vascular Plug: report of four cases.

The Amplatzer Vascular Plug (AVP) is a device originally intended for arterial and venous embolization in peripheral vessels. From December 2004 to March 2007 we implanted a total of 8 AVPs in the portal venous system in our institution for preoperative portal vein embolization in 4 patients (55-71 years) prior to right hemihepatectomy. AVP implantation was successful in all patients. Total occlusion of the embolized portal vein branches was achieved in all patients. There were no major complications associated with the embolization.

Nonossifying fibroma can mimic residual lymphoma in FDG PET: additional value of combined PET/CT.

A 12-year-old girl was diagnosed with Hodgkin's lymphoma and underwent conventional cross-sectional imaging for initial staging. Chemotherapy was given according to standard pediatric protocols. At the end of therapy, an F-18 FDG PET/CT examination was performed to evaluate the therapeutic response. The scan demonstrated focal uptake of FDG in the right distal femur and residual lymphoma was taken into consideration. However, findings in the coregistered CT scan were consistent with nonossfiying fibroma, a common benign skeletal lesion. Combined PET/CT imaging can be helpful to identify benign bone lesions mimicking metastatic or residual disease in F-18 FDG PET as illustrated by this case.

Transarterial chemoembolization using degradable starch microspheres and iodized oil in the treatment of advanced hepatocellular carcinoma: evaluation of tumor response, toxicity, and survival.

BACKGROUND: In a multidisciplinary conference patients with advanced non-resectable hepatocellular carcinoma (HCC) were stratified according to their clinical status and tumor extent to different regional modalities or to best supportive care. The present study evaluated all patients who were stratified to repeated transarterial chemoembolization (TACE) from 1999 until 2003 in terms of tumor response, toxicity, and survival. A moderate embolizing approach was chosen using a combination of degradable starch microspheres (DSM) and iodized oil (Lipiodol) in order to combine anti-tumoral efficiency and low toxicity. METHODS: Fourty-seven patients were followed up prospectively. TACE treatment consisted of cisplatin (50 mg/m(2)), doxorubicin (50 mg/m(2)), 450-900 mg DSM, and 5-30 ml Lipiodol. DSM and Lipiodol were administered according to tumor vascularization. Patient characteristics, toxicity, and complications were outlined. In multivariate regression analyses of pre-treatment variables from a prospective database, predictors for tumor response and survival after TACE were determined. RESULTS: 112 TACE courses were performed (2.4+/-1.5 courses per patient). Mean maximum tumor size was 75 (+/-43) mm, in 68% there was bilobar disease. Best response to TACE treatment was: progressive disease (PD) 9%, stable disease (SD) 55%, partial remission (PR) 36%, and complete remission (CR) 0%. Multivariate regression analyses identified tumor size 30 months, R(2)=36%). Grade 3 toxicity occurred in 7.1% (n=8), and grade 4 toxicity in 3.6% (n=4) of all courses in terms of reversible leukopenia and thrombocytopenia. The incidence of major complications was 5.4% (n=6). All complications were managed conservatively. The mortality within 6 weeks after TACE was 2.1% (one patient). CONCLUSIONS: DSM and Lipiodol were combined successfully in the palliative TACE treatment of advanced HCC resulting in high rates of tumor response and survival at limited toxicity. Favourable tumor response was associated with tumor extent and vascularization. TACE using DSM and Lipiodol can be considered a suitable palliative measure in patients who might not tolerate long acting embolizing agents.

Coccydynia due to a benign notochordal cell tumor.

STUDY DESIGN: Case report. OBJECTIVE: To present a rare case of a notochordal cell tumor. SUMMARY OF BACKGROUND DATA: We report on a 27-year-old female patient with pain at the lower back and muscle cramps in the area of the right hip. Image studies demonstrated a cystic lesion of the coccyx. METHODS: As clinical symptoms became chronic and were resistant to conservative treatment, a resection of the coccyx was performed. RESULTS: Histology revealed an intraosseous benign notochordal cell tumor. This tumor represents a recently described notochordal cell proliferation biologically distinct from chordomas. CONCLUSIONS: Overdiagnosis of these notochordal cell proliferations as chordomas may occur if clinicians and pathologists are unfamiliar with the spectrum of notochordal proliferations.

Oncogenic osteomalacia: exact tumor localization by co-registration of positron emission and computed tomography.

UNLABELLED: In oncogenic osteomalacia, the causative tumor is almost always difficult to find. A novel diagnostic approach is presented that facilitates a precise and rapid localization of the associated lesion by PET-CT co-registration using the radiotracer (68)Ga-DOTANOC. INTRODUCTION: Oncogenic osteomalacia (OOM) is an uncommon disorder characterized by hyperphosphaturia, hypophosphatemia, decreased vitamin D(3) serum levels, and osteomalacia. The paraneoplastic syndrome is exclusively driven by a small somatostatin receptor (sst)-positive tumor that produces phosphatonins, proteins that cause renal phosphate loss. OOM can be cured completely on tumor removal. However, the exact tumor localization is the most challenging step, because the lesion is notoriously difficult to detect by common imaging techniques. MATERIALS AND METHODS: A 60-year-old woman complained of severe pain in her back and chest wall, muscle weakness, and reduced physical activity for >1 year. She suffered a metatarsal fracture and presented with hyperphosphaturia and hypophosphatemia. OOM was suspected, and a meticulous search for the tumor was initiated by conventional imaging techniques, sst-mediated imaging using (111)In-octreotide scintigraphy, and (68)Ga-DOTANOC-based positron emission tomography (PET)-CT co-registration. (68)Ga-DOTANOC is a novel radiopharmaceutical compound in which the somatostatin analog octreotide is modified at position 3, chelated with DOTA, and complexed with (68)Gallium. (68)Ga-DOTANOC has an improved affinity to sst2 and sst5 relative to other radiopeptides. RESULTS: Whereas common imaging techniques such as CT failed to localize the tumor, (111)In-octreotide scintigraphy was able to detect the lesion, but only PET-CT using (68)Ga-DOTANOC revealed the exact tumor localization in the right femoral head. On tumor resection, the well being of the patient improved significantly, and biochemical parameters returned to normal. CONCLUSIONS: (68)Ga-DOTANOC-based PET-CT is a novel and powerful approach to detect sst-positive tumors in a timely manner and to provide highly resolved images facilitating the development of a therapeutic strategy.

Combination of repeated single-session percutaneous ethanol injection and transarterial chemoembolisation compared to repeated single-session percutaneous ethanol injection in patients with non-resectable hepatocellular carcinoma.

AIM: To evaluate the treatment effect of percutaneous ethanol injection (PEI) for patients with advanced, non-resectable HCC compared with combination of transarterial chemoembolisation (TACE) and repeated single-session PEI, repeated single-session PEI alone, repeated TACE alone, or best supportive care. METHODS: All patients who received PEI treatment during the study period were included and stratified to one of the following treatment modalities according to physical status and tumor extent: combination of TACE and repeated single-session PEI, repeated single-session PEI alone, repeated TACE alone, or best supportive care. Prognostic value of clinical parameters including Okuda-classification, presence of portal vein thrombosis, presence of ascites, number of tumors, maximum tumor diameter, and serum cholinesterase (CHE), as well as Child-Pugh stage, alpha-fetoprotein (AFP), fever, incidence of complications were assessed and compared between the groups. Survival was determined using Kaplan-Meier and multivariate regression analyses. RESULTS: The 1- and 3-year survival of all patients was 73% and 47%. In the subgroup analyses, the combination of TACE and PEI (1) was associated with a longer survival (1-, 3-, 5-year survival: 90%, 52%, and 43%) compared to PEI treatment alone (2) (1-, 3-, 5-year survival: 65%, 50%, and 37%). Secondary PEI after initial stratification to TACE (3) yielded comparable results (1-, 3-, 5-year survival: 91%, 40%, and 30%) while PEI after stratification to best supportive care (4) was associated with decreased survival (1-, 3-, 5-year survival: 50%, 23%, 12%). Apart from the chosen treatment modalities, predictors for better survival were tumor number (n < 5), tumor size (< 5 cm), no ascites before PEI, and stable serum cholinesterase after PEI (P < 0.05). The mortality within 2 wk after PEI was 2.8% (n = 3). There were 24 (8.9%) major complications after PEI including segmental liver infarction, focal liver necrosis, and liver abscess. All complications could be managed non-surgically. CONCLUSION: Repeated single-session PEI is effective in patients with advanced HCC at an acceptable and manageable complication rate. Patients stratified to a combination of TACE and PEI can expect longer survival than those stratified to repeated PEI alone. Furthermore, patients with large or multiple tumors in good clinical status may also profit from a combination of TACE and reconsideration for secondary PEI.

Late esophageal perforation complicating anterior cervical plate fixation in ankylosing spondylitis: a case report and review of the literature.

Esophageal perforation in ankylosing spondylitis (AS) is a rare complication in anterior cervical spine surgery and has not been reported before. A 50-year-old patient with AS developed incomplete tetraplegia after minimal trauma. C5 pedicle fracture was diagnosed and treated predominantly by physical therapy until neurological symptoms progressed. Cervical spine MRI showed C6/7 fracture and spinal cord compression. The patient underwent dorsal laminectomy, C5-7 anterior cervical fusion using allograft iliac crest and CASPAR-plate fixation. Delayed esophageal perforation appeared 10 months postoperatively when he came first to our hospital. He complained of dysphagia and developed acute dyspnea. Posterior stabilization with two plates was performed followed by removal of the ventral plate and screws. The esophageal laceration was sutured. The patient was treated with antibiotics and percutaneous endoscopic gastrostomy. Position of fracture and implants were accurate at 18 months postoperatively. The patient had persistent minor neurological deficits (Frankel D) at last follow-up. We conclude that esophageal perforation after anterior spinal fusion is a rare complication. Minor traumas in patients with AS are unstable and can result in significant spinal injury. Dorsoventral stabilization should be performed to avoid further complications.

Improvement of Erdheim-Chester disease in two patients by sequential treatment with vinblastine and mycophenolate mofetil.

Erdheim-Chester disease (ECD) is a rare non-Langerhans' form of histiocytosis with a plethora of different clinical manifestations owing to multiple organ involvement. We report two patients who presented initially with different clinical symptoms. The presenting complaint of the first patient was bone pain, predominantly in the legs, whereas in the other patient the initial symptoms were related to obstruction of both ureters, as in idiopathic retroperitoneal fibrosis. Ultimately, ECD was diagnosed in both patients by the occurrence of both pathognomonic manifestations, the histologic presence of non-Langerhans' histiocytes in bone biopsies, and osteosclerotic lesions of the long bones. Because the extraosseous manifestations progressed and a single application of corticosteroids was ineffective, sequential treatment with vinblastine and mycophenolate mofetil, together with prednisolone, was started. At follow-up respectively 15 and 16 months after the start of treatment a beneficial effect was noted in both patients. These cases illustrate the clinical spectrum of ECD, detail the pathognomonic manifestations of this rare disease, emphasize the need to consider ECD as an uncommon but important differential diagnosis in patients with arthralgias or systemic fibrosis, and give the first evidence for a new treatment option.

Diagnostic management of patients with SAPHO syndrome: use of MR imaging to guide bone biopsy at CT for microbiological and histological work-up.

Propionibacterium acnes (P. acnes) is suspected to be involved in the pathophysiology of SAPHO syndrome, since it has been isolated repeatedly through open surgical bone biopsy. This study demonstrates the role of MRI in identifying inflamed bone areas in patients with SAPHO syndrome and the role of CT-guided bone biopsies in obtaining samples from these areas for microbiological and histopathological investigations, thus obviating open surgery. Fourteen consecutive patients with SAPHO syndrome were investigated by MRI to identify acute inflammatory changes in hyperostotic periarticular bone. The CT-guided biopsies for microbiological investigations were taken from the areas identified. Patients positive for P. acnes were started on long-term antibiotic therapy according to antibiotic susceptibility. On MRI the inflammatory changes appeared as hyperintense areas on fat-saturated T2 fast-spin-echo (FSE) images and showed signal increase on fat-saturated T1 SE images after Gd-DTPA. With MR localization CT-guided bone biopsies yielded P. acnes in 8 patients. No bacteria could be isolated from the remaining 6 patients. Acute inflammatory bone changes in SAPHO syndrome are well localized by MRI. With MR localization, CT-guided bone biopsies offer a minimally invasive alternative to open surgery in the detection of. P. acnes leading to the institution of a specific antibiotic therapy.

Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach.

OBJECTIVES: Current management of extrahepatic portal vein thrombosis (EPVT) comprises endoscopic eradication therapy of esophageal varices and conventional shunt surgery. The authors have used the novel technique of mesenterico-left portal bypass (Rex shunt) in seven children with symptomatic EPVT, and report their results here. METHODS: Median age of the children was 12 years (range, 2-16 years). All children had portal hypertension with hypersplenism and recurrent bleeding from esophageal varices. Furthermore, one patient suffered from a severe hepatopulmonary syndrome. Preoperative evaluation included liver function tests, liver biopsy, hepatic duplex ultrasonography, and radiologic evaluation of the intrahepatic and extrahepatic vascular anatomy. The internal jugular vein was used as vein graft in all patients. RESULTS: Median follow-up period was 15 months (range, 3-28 months). Ultrasound scans revealed sufficient perfusion in all shunts (median, 35 cm/s; range, 28-60 cm/s). The intrahepatic portal perfusion in segment 4 improved from a median of 6 cm/s before surgery to 18 cm/s postoperatively. The platelet count increased within 3 months from a mean of 50,625/microL to 137,750/microL. The clinical signs of hypoxemia in the child with hepatopulmonary syndrome disappeared within 6 months. CONCLUSIONS: In accordance with the limited experience published by others, the authors' data confirmed the mesenterico-portal Rex shunt as the therapy of choice for children with EPVT. Furthermore, this report is the first to show that a hepatopulmonary syndrome can be abolished by mesenterico-portal Rex shunt.