MR findings of the orbit in patients with Vogt–Koyanagi–Harada disease

Purpose

We aimed to evaluate the MR findings of the orbit in patients with Vogt–Koyanagi–Harada disease (VKHD).

Methods

We included 14 patients with clinically diagnosed VKHD, who underwent orbital MR imaging before treatment between May 2011 and August 2017. The mean duration from initial symptom onset to MR imaging was 16 days (range, 2–36 days). Fat-suppressed gadolinium-enhanced T1-weighted images were obtained in six patients. We retrospectively assessed the choroids and Tenon’s capsules for the presence of thickening on unenhanced images and abnormal enhancement on contrast-enhanced images.

Results

Bilateral choroidal thickening was observed in 14 patients (100%) on T1-weighted images and in 12 patients (85.7%) on T2-weighted images. Choroidal thickening showed posterior pole predominance in 11 patients (78.6%) and diffusely distributed in the remaining three patients (21.4%). Bilateral Tenon’s capsule thickening was observed in five patients (35.7%) on T1-weighted images and in 14 patients (100%) on T2-weighted images. On contrast-enhanced images, the choroids and Tenon’s capsules were abnormally enhanced in six patients (100%).

Conclusion

MR imaging sensitively detected abnormalities of the choroids and Tenon’s capsules in patients with VKHD. Bilaterality and predominant posterior pole distribution were characteristic of choroidal VKHD.

     

Osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud’s syndrome

Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We report a patient with Mazabraud’s syndrome (polyostotic fibrous dysplasia and soft tissue myxomas) complicated by the development of osteogenic sarcoma in a bone affected by fibrous dysplasia. This is the third case of osteosarcoma within the small population of reported patients with Mazabraud’s syndrome. There may be an increased incidence of malignant transformation in these individuals’ dysplastic bones above that associated with patients suffering from fibrous dysplasia alone. Received: 31 December 1998 Revision requested: 31 January 1999 Revision received: 13 May 1999 Accepted: 14 May 1999     

Gallium-67 scintigraphic findings in a patient with breast lymphoma complicated with Sjögren syndrome

We report here a patient with mucosa associated lymphoid tissue (MALT)-lymphoma of the breast complicated with Sjögren syndrome. It is speculated that Ga-67 could accumulate not only in lymphoma lesions but also in benign lymphoproliferative locations of Sjögren syndrome. Gallium-67 scintigraphy might be useful for the diagnosis and therapeutic monitoring of MALT-lymphoma complicated with Sjögren syndrome.     

Distal renal tubular acidosis as a cause of osteomalacia in a patient with primary Sjögren's syndrome

BACKGROUND: One half of the patients with primary Sj?gren's syndrome has extraglandular manifestations, including renal involvement. The most frequent renal lesion is tubulo-interstitial nephritis, which manifests clinically as distal tubular acidosis and may result in the development of osteomalacia. CASE REPORT: In a 29-year-old female patient, with bilateral nephrolithiasis, the diagnosis of primary Sj?gren's syndrome, tubulo-interstitial nephritis, distal renal tubular acidosis, and hypokalemia were established. She was treated for hypokalemia. Two years later she developed bone pains and muscle weakness, she wasn't able to walk, her proximal muscles and pelvic bones were painful, with radiological signs of pelvic bones osteopenia and pubic bones fractures. The diagnosis of osteomalacia was established and the treatment started with Schol's solution, vitamin D and calcium. In the following two months, acidosis was corrected, and the patient started walking. CONCLUSION: In our patient with primary Sj?gren's syndrome and interstitial nephritis, osteomalacia was a result of the long time decompensate acidosis, so the correction of acidosis, and the supplementation of vitamin D and calcium were the integral part of the therapy.     

Catheter-directed thrombolysis and mechanical thrombectomy of a thrombosed persistent sciatic vein in a patient with Klippel-Trénaunay syndrome

The authors report a case of a thrombosed persistent sciatic vein in a patient with Klippel-Trénaunay syndrome diagnosed at magnetic resonance (MR) venography and its treatment with endovascular interventional techniques.     

Sudden death from ischaemic heart disease in a female patient with Sjögren syndrome: a case report

Primary Sj?gren syndrome (SjS) has a comparatively good prognosis except when it is complicated by non-Hodgkin's lymphoma. We performed an autopsy on a young female patient with primary SjS who had died suddenly during a meal, and we discuss the relationship between primary SjS and the cause of death. Eosinophilic change of the cardiomyocytes and severe arteriolosclerosis were observed within the myocardium. In the conduction system, lymphocyte infiltration was detected in the bundle of His, in addition to arteriosclerosis in the sinoatrial node and atrioventricular node arteries. The cause of death was diagnosed as ischaemic heart disease induced by arteriolosclerosis: its development can probably be attributed mostly to primary SjS. It should thus be kept in mind that primary SjS can occasionally result in the development of cardiovascular complications, such as ischaemic heart disease, as well as systemic lupus erythematosus or rheumatoid arthritis.     

Coronary involvement in Churg–Strauss syndrome: a case report with CT findings

We report a case of Churg–Strauss syndrome (CSS) associated with coronary artery involvement, as demonstrated on coronary CT angiography (CCTA), without specific cardiac symptoms. A 69-year-old male had an 8-year history of bronchial asthma and chronic sinusitis with hypereosinophilia (35 %), polyneuropathy, and a positive antineutrophil cytoplasmic antibody titer, so he was diagnosed with CSS. The patient had no specific cardiac symptoms, but CCTA showed vasculitis and a saccular aneurysm involving the proximal coronary arteries. The 3-year follow-up CCTA demonstrated an increase in the extent of soft-tissue wall thickening and infiltration involving the coronary arteries. Although vasculitis of the major coronary arteries is not a prominent feature of CSS, our case suggests that the coronary arteries may also be targeted in this syndrome.     

Transjugular intrahepatic portocaval shunt placed through the strut of an inferior vena cava stent in a patient with Budd–Chiari syndrome: a technical modification

Transjugular intrahepatic portocaval shunt (TIPS) is performed in patients with symptomatic Budd–Chiari syndrome (BCS) who do not have repairable hepatic veins. We report the case of a patient who had an inferior vena cava (IVC) stent placed previously as part of the management for BCS, and who subsequently required TIPS. The TIPS tract was created through the strut of the previously placed IVC stent; the TIPS stent was placed after dilatation of the liver parenchyma as well as the strut of the IVC stent. This novel technique of “strutplasty” of a previously placed stent as part of TIPS has not been reported in the literature.The transjugular intrahepatic portocaval shunt (TIPS) procedure, as well as stent technology, is continually being improved [1–5]. Direct intrahepatic portocaval shunt (DIPS) – a modification of the TIPS procedure – is especially useful when there are occluded hepatic veins or an unfavourable angle between the inferior vena cava (IVC) and the hepatic vein resulting from hydrothorax and hypertrophy of the caudate lobe of the liver [1]. Herein, we report a technical modification of the TIPS procedure in Budd–Chiari syndrome (BCS).     

MRI findings of neutrophilic fasciitis in a patient with acute febrile neutrophilic dermatosis (Sweet’s syndrome)

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) is a clinical condition that is histopathologically characterized by infiltration of the dermis with mature neutrophils with or without vessel wall destruction. Frequently, an extracutaneous systemic disease can be seen. We report magnetic resonance imaging (MRI) findings of neutrophilic fasciitis in a 62-year-old man with Sweet’s syndrome and musculoskeletal involvement. The musculoskeletal system is rarely involved in Sweet’s syndrome and, to our knowledge, no previous report of MRI findings of neutrophilic fasciitis with myofascial involvement exists in the literature.     

Pacemaker implantation in a patient with a Behçet's disease associated with superior vena cava obstruction

A 65-year-old man with Behçet's disease developed transient complete A-V block with syncope. An attempt to implant a permanent transvenous endocardial electrode failed owing to obstructions in the subclavian, innominate, and superior vena cava veins demonstrated by angiography. Sutureless epicardial electrodes were successfully implanted through a subxyphoid approach. Obstruction in the great veins is a common feature in patients with Behçet's disease. We suggest that patients with this disorder, who require a permanent pacemaker, should be investigated by angiography prior to implant to rule out obstruction in the upper great veins.     

Intervantional management in a patient with subclavian artery stenosis: case report

The patient is a 79 years old male. He was admitted for recurrent dizziness accompanied tinnitus during the last 2 weeks. These symptoms were aggravated during the last 4 days. He had a history of hypertension. Murmur was detected on the left subclavian artery. Left upper limb had IVth grade muscle force. The pulse of left radial artery was reduced. The systolic blood pressure of left upper limb was 30 mmHg lower than the right counter part. The Doppler sonography of vertebral artery revea…     

The discovery of a Persian family with a form of Birt–Hogg–Dubé syndrome lacking the typical cutaneous stigmata of the syndrome

PurposeThis study was performed in 24 members of a family with spontaneous pneumothorax to test clinical suspicion of Birt–Hogg–Dubé syndrome (BHDS).MethodsComputed tomography scan was performed for confirmation of pneumothorax, while genetic tests were done using real-time quantitative polymerase chain reaction.ResultsGenetic studies showed a deletion of exon 1 in the FLCN gene in the index case as well as nine other individuals, including two with clinical phenotypes of pneumothorax and seven who are symptom-free to date.ConclusionsProper imaging and taking accurate family history could be the keys to test clinical suspicion in some syndromes, including BHDS.     

Management of patellar problems in skeletally mature patients with nail–patella syndrome

Purpose

Nail–patella syndrome (NPS) or hereditary onychoosteodysplasia is a rare autosomal dominant disease, characterized by a tetrad of findings, which include fingernail abnormalities, hypoplasia of the patellae, radial head dislocation and prominent iliac horns. Most of the literature on the treatment of patellar problems in NPS concerns paediatric patients, and there is no standard treatment algorithm for adult patients.

Methods

We reviewed the charts of skeletally mature patients with NPS who presented to our clinic. We reviewed the presenting complaints, the physical examination findings and the radiographic imaging.

Results

We identified seven skeletally mature patients with NPS who presented with patellofemoral complaints. Their symptoms were instability, pain, or a combination of the two. Examination and imaging revealed a wide range of severity but included patellar instability and patellar arthritis. In our series, milder forms of the disease were treated with non-operative measures, but the majority of our patients required surgery including medial patellofemoral ligament reconstruction, tibial tuberosity transposition, patellofemoral and total knee arthroplasty. At midterm follow-up, most patients had good results.

Conclusion

Nail–patella syndrome has a wide range of presentations and severity in skeletally mature patients. Knee surgeons should be familiar with the spectrum of clinical presentation and the range of treatment options available in order to provide optimum treatment for patients with this disorder.

Level of evidence

IV.