Successful treatment of respiratory failure in Hirayama disease.

Hirayama disease is a self-limiting cervical motor neuron disease, usually affecting the spinal cord at level C7-T1. We share an unusual case of Hirayama disease in a young man affecting roots C4-C6. He presented in coma due to diaphragm weakness and hypercapnic respiratory failure. Diagnosis was achieved via clinical presentation, neurophysiological examination, ultrasonography of the diaphragm and dynamic MR-imaging. Conservative treatment with a cervical collar resulted in remarkable improvement in respiratory and motor function.

Two-sided femoral Campylobacter jejuni osteomyelitis in a patient with acquired hypogammaglobulinemia: a case report.

BACKGROUND: Campylobacter jejuni is a motile, gram-negative rod known for causing self-limiting enterocolitis while rarely causing extraintestinal infections. We report the first case of a patient with Campylobacter jejuni osteomyelitis in both femora. CASE PRESENTATION: A 54-year-old female presented with progressive pain in both upper extremities. Her past medical history mentioned a lymphoplasmacytic lymphoma (LPL) for which she had received dexamethasone, cyclophosphamide and fludarabine and was still receiving maintenance therapy with intravenous rituximab. Two months prior to presentation, she received oral fluoroquinolone for a recurrent enterocolitis with stool cultures positive for Campylobacter jejuni. Palpation of the left quadriceps femoris muscle was remarkably painful during physical examination. Laboratory testing showed elevated C-reactive protein and recent low gamma globulin levels. The presumptive diagnosis at this point was a transformation of LPL to a large B cell lymphoma. In order to determine the preferred site for biopsy, a fluorine-18 fluoro-2-deoxy-D-glucose positron emission tomography combined with computed tomography was done. However, blood cultures taken on admission showed growth of Campylobacter jejuni in both aerobic bottles, with a strain resistant to fluoroquinolones. Diagnosis of Campylobacter jejuni osteomyelitis was confirmed with 16S ribosomal RNA gene polymerase chain reaction performed on femoral bone obtained through biopsy. Treatment with intravenous imipenem/cilastatin followed by intravenous and oral doxycycline proved insufficient. Subsequently, the patient was treated successfully with intravenous meropenem for six weeks and concurrent intravenous immunoglobulin. CONCLUSION: We report the first case of Campylobacter jejuni osteomyelitis in both femora in a patient with acquired hypogammaglobulinemia. Diagnosis was confirmed by blood cultures and positive 16S ribosomal RNA gene polymerase chain reaction for Campylobacter spp. on bone biopsy. Treatment was successful with intravenous meropenem and immunoglobulin. Our report showcases an unusual manifestation in a patient with immunodeficiency and discusses failure of initial antibiotic therapy.

Successful emergent repair of a subacute left ventricular free wall rupture after acute inferoposterolateral myocardial infarction.

BACKGROUND: Myocardial rupture is an important and catastrophic complication of acute myocardial infarction. A dramatic form of this complication is a left ventricular free wall rupture (LVFWR). CASE PRESENTATION: A 70-year-old man with acute inferoposterolateral myocardial infarction and single-vessel coronary artery disease underwent emergency percutaneous coronary intervention (PCI). The circumflex coronary artery was successfully stented with a drug-eluting stent. Fifty days after PCI the patient experienced progressive fatigue and chest pain with haemodynamic instability. Transthoracic echocardiography showed a covered LVFWR of the lateral wall. The patient underwent successful emergent surgical repair of the LVFWR. CONCLUSIONS: In the current era of swift PCI, mechanical complications of acute myocardial infarction, such as LVFWR, are rare. The consequences, however, are haemodynamic deterioration and imminent death. This rare diagnosis should always be considered when new cardiovascular symptoms or haemodynamic instability develop after myocardial infarction, even beyond one month after the initial event. Timely diagnosis and emergency surgery are required for successful treatment of this devastating complication.

Preserved hyperaemic response in (distal) string sign left internal mammary artery grafts.

OBJECTIVE: To correlate supraclavicular ultrasonography at rest and in hyperaemic response with angiographically patent and (distal) 'string sign' left internal mammary artery (LIMA) to left anterior descending (LAD) area grafts. METHODS: Fifty-three patients with LIMA to LAD area grafting were prospectively entered in a follow-up study. Arteriography (native and LIMA) was performed at 1.4+/-0.8 years postoperatively and ultrasonography was performed at rest, in hyperaemic response and 2min after hyperaemic response at 1.8+/-0.8 years postoperatively and was compared to arteriography. Ultrasonographic parameters analysed were systolic and diastolic peak velocity, systolic and diastolic velocity integral, diastolic/systolic peak velocity ratio and diastolic/total velocity integral ratio. RESULTS: One patient was excluded because obesity hampered ultrasonography. Arteriography demonstrated functional grafts in 43 patients (group I), sequential distal 'string sign grafts' in 4 patients (group II) and total 'string sign grafts' in 5 patients (group III). Between the groups all ultrasonographic velocities showed a significant linear relation (p

Anatomical and functional assessment of single left internal mammary artery versus arterial T-grafts 12 years after surgery.

We determined whether ultrasonographic left internal mammary artery (LIMA) findings correspond with 64 multislice computed tomography (MSCT) in patients 12 years after coronary artery bypass grafting. We included 34 patients (63.2+/-9.2 years), 16 with conventional single LIMA (group I) and 18 arterial T-grafts (group II), in a cross-sectional study. Patients underwent transthoracic proximal LIMA ultrasonography at rest and during the Azoulay maneuver, transthoracic echocardiography of the left ventricle and 64-MSCT, 11.5+/-1.4 years postoperatively. MSCT scans showed three string sign LIMA grafts (19%) in group I and three distal string sign LIMA grafts (17%) and 16 occluded T-graft anastomoses (22%) in group II. LIMA diameters and areas are significantly larger in group II in the origin, 3.5+/-0.7 vs. 2.5+/-0.5 mm, P=0.00007 and 0.09+/-0.04 vs. 0.05+/-0.02 cm(2), P=0.00019 and in the third intercostal space, 3.4+/-0.7 vs. 2.5+/-0.5 mm, P=0.00009 and 0.09+/-0.03 vs. 0.05+/-0.02 cm(2), P=0.000047. Most ultrasonographic LIMA findings do not differ between the groups. Thus, proximal LIMA diameters and areas are significantly larger in T-grafts and ultrasonographic variables equalize between the groups at rest and during the Azoulay maneuver 12 years after surgery.

Epicardial coronary artery Doppler: validation in the animal model.

The aim of the study was to validate a newly-designed epicardial coronary artery Doppler probe and test its detection of changes in coronary blood flow velocity. Left anterior descending (LAD) coronary blood flow and flow velocity were evaluated in four pigs with a pericoronary transit time flow (TTF) probe and a newly-designed epicardial Doppler micro-probe. Four consecutive measurements were taken for each of the following conditions: basal, partial stenosis, occlusion, and reperfusion of the LAD. Mean TTF value (ml/min) was 23.2+/-6.6 in basal condition, 16.2+/-5.7 after partial LAD stenosis, 0.1+/-0.3 during LAD occlusion, and 67.4+/-23.3 at reperfusion (P<0.001). Similar patterns were recorded in terms of Doppler velocity (cm/s) with values of 4.0+/-1.9 in basal condition, 3.5+/-2.3 after partial LAD stenosis, 0.5+/-1.4 during LAD occlusion, and 11.1+/-5.5 at reperfusion (P<0.001). No significant differences in both TTF and Doppler velocity were detected between basal condition and partial LAD stenosis (P=ns). Epicardial coronary arterial Doppler represents a valuable tool to detect coronary arterial flow velocity in basal condition. Although changes in flow velocity are easily recorded after coronary occlusion and reperfusion, modifications after partial coronary stenosis are not clearly defined.

Can late supraclavicular echo Doppler reliably predict angiographical string sign of lima to lad area grafts?

OBJECTIVE: To investigate whether supraclavicular ultrasonography of left internal mammary artery (LIMA) to left anterior descending (LAD) area grafts can reliably predict (distal) string sign grafts on arteriography. METHODS: Fifty-five patients (42 M, 61 +/- 7 years) with the LIMA to LAD area grafting were prospectively studied. Control arteriography was performed at 1.4 +/- 0.8 years postoperatively. Angiography demonstrated in 46 patients (group I) functional grafts, in 4 patients (group II) sequential distal string sign grafts and in 5 patients (group III) total string sign grafts. Ultrasonography was performed at 1.8 +/- 0.8 year postoperatively and compared with control angiography. Data were tested by unpaired t- and ANOVA tests. The diagnostic accuracy was assessed by the area under the curve of the Receiver Operator Characteristic. A formula was developed to predict the probability of (distal) string sign phenomena of sequential as well as single LIMA grafts. RESULTS: Between the groups all duplex parameters showed a highly significant linear relation (p < or = 0.004) and all parameters between group I and III are significantly different with high Area Under Curve values. The model for the probability of (distal) string sign grafts fitted best with diastolic and systolic peak velocities as the most discriminative factors for (distal) string sign grafts. CONCLUSIONS: Postoperative supraclavicular duplex as a method to assess the patency of LIMA to LAD area grafts allows discriminating functional grafts from (distal) string sign grafts.

Underestimated abdominal vascular pathology in a patient with Takayasu arteritis.

OBJECTIVE: To describe a 31-year-old female with symptomatic Takayasu disease who was operated for aortic valve replacement. Although she had no preoperative abdominal vascular symptoms, she died on the first postoperative day due to extensive ischemia bowel syndrome. METHODS: Echocardiography and computed tomography revealed progressive dilatation and thickening of the ascending aorta, severe aortic regurgitation and diminished left ventricular function from 1998 onwards. In 2000 she was operated and a prosthesis was placed end-to-end distally of the sinutubular junction with combined reduction plasty of this junction. After the operation, in time echocardiography and computed tomography showed progressive irregularities and dilatation of the thoracoabdominal aorta and progressive aortic regurgitation. A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned. At the time of the reoperation in September 2004 the Takayasu inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase. The aortic valve was uneventfully replaced by a mechanical valve. RESULTS: Medical treatment for Takayasu disease never resulted in the patient in completely normal blood values of white blood cell count, C-reactive protein or erythrocyte sedimentation rate. In August 2004, she was admitted because of severe non-specific thoracic pain and hypertension. During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension. Although the computed tomography revealed severe stenosis of the superior mesenteric artery and the celiac trunk, she was free of abdominal complaints and without further abnormal laboratory findings. One day after the aortic valve replacement a dramatic increase of transaminase and lactate-dehydrogenase with extreme metabolic acidosis appeared. Urgent abdominal surgery was performed and extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia, no surgical interventions were optional. The patient died one day after abdominal exploration. CONCLUSIONS: We conclude that in Takayasu disease scheduled for on pump cardiac surgery, vascular workup should be done, and interventional treatment of asymptomatic but potentially critical lesions should be considered.