An unusual cause of adrenal insufficiency with elevation of 17-hydroxyprogesterone: case report.

BACKGROUND: We present an intriguing case of primary adrenal lymphoma, with associated primary adrenal insufficiency (PAI), in a patient presenting a transitory partial 21-hydroxylase deficiency during the active phase of the adrenal disease. CASE PRESENTATION: An 85-years old woman was referred because of worsening asthenia, lumbar pain, generalized myalgia and arthralgia. During investigations a computed tomography (CT) scan evidenced two large bilateral adrenal masses, highly suspicious for primary adrenal tumor. The hormonal assessment revealed very low levels of morning plasma cortisol and 24-h urinary cortisol, elevated ACTH levels with low plasma concentration of aldosterone, pointing to the diagnosis of PAI. After diagnosis of PAI our patient started glucocorticoid and mineralcorticoid replacement therapy with clinical benefit. In order to further characterize the adrenal lesions, adrenal biopsy, was performed. The histology revealed a high grade non-Hodgkin lymphoma with an immunophenotype consistent with intermediate aspects between diffuse large B-cell and Burkitt lymphoma, with a high proliferation index (KI-67 > 90%). The patient received chemotherapy with epirubicin, vincristine, cyclophosphamide, and rituximab, associated with methylprednisolone that resulted in a complete clinical and radiological remission within one year. After 2 years from the diagnosis and a total of 6 cycles of rituximab, the patient was in good clinical condition and was taking only the replacement therapy for PAI. The patient initially presented also a slight increase of 17-hydroxyprogesterone (17-OHP) for age that normalize after resolution of lymphoproliferative disease. CONCLUSIONS: In the presence of bilateral adrenal disease and/or in the presence of signs and symptoms of PAI clinicians must exclude the presence of PAL. The evidence of elevated ACTH-stimulated 17-OHP levels also in patients with other adrenal masses, together with the detection of elevated basal 17-OHP levels in our patient make it more plausible, in our view, an effect of the lesion on the "healthy" adrenal tissue residue than a direct secretory activity by the adrenal tumor.

Lung distribution of gas and blood volume in critically ill COVID-19 patients: a quantitative dual-energy computed tomography study.

BACKGROUND: Critically ill COVID-19 patients have pathophysiological lung features characterized by perfusion abnormalities. However, to date no study has evaluated whether the changes in the distribution of pulmonary gas and blood volume are associated with the severity of gas-exchange impairment and the type of respiratory support (non-invasive versus invasive) in patients with severe COVID-19 pneumonia. METHODS: This was a single-center, retrospective cohort study conducted in a tertiary care hospital in Northern Italy during the first pandemic wave. Pulmonary gas and blood distribution was assessed using a technique for quantitative analysis of dual-energy computed tomography. Lung aeration loss (reflected by percentage of normally aerated lung tissue) and the extent of gas:blood volume mismatch (percentage of non-aerated, perfused lung tissue-shunt; aerated, non-perfused dead space; and non-aerated/non-perfused regions) were evaluated in critically ill COVID-19 patients with different clinical severity as reflected by the need for non-invasive or invasive respiratory support. RESULTS: Thirty-five patients admitted to the intensive care unit between February 29th and May 30th, 2020 were included. Patients requiring invasive versus non-invasive mechanical ventilation had both a lower percentage of normally aerated lung tissue (median [interquartile range] 33% [24-49%] vs. 63% [44-68%], p < 0.001); and a larger extent of gas:blood volume mismatch (43% [30-49%] vs. 25% [14-28%], p = 0.001), due to higher shunt (23% [15-32%] vs. 5% [2-16%], p = 0.001) and non-aerated/non perfused regions (5% [3-10%] vs. 1% [0-2%], p = 0.001). The PaO2/FiO2 ratio correlated positively with normally aerated tissue (ρ = 0.730, p < 0.001) and negatively with the extent of gas-blood volume mismatch (ρ = - 0.633, p < 0.001). CONCLUSIONS: In critically ill patients with severe COVID-19 pneumonia, the need for invasive mechanical ventilation and oxygenation impairment were associated with loss of aeration and the extent of gas:blood volume mismatch.

Endovascular Aspiration Thrombectomy in the Management of Kidney Ischemia: A Case Report.

Renal artery occlusion is a serious event that can result in significant impairment or loss of renal function, leading to dialysis dependency. The nonspecific signs and symptoms of its presentation frequently result in a delay in diagnosis, thus contributing to delay in treatment. We report the case of a 53-year old woman who suffered renal artery occlusion, in which the renal perfusion was restored after three days of ischemia, by endovascular aspiration thrombectomy.

Varicocele Due to Post-traumatic Arteriovenous Fistula.

Varicocele consists of abnormally dilated and tortuous veins within the pampiniform plexus, usually in the left scrotum. We reported a rare case of right varicocele due to post-traumatic arteriovenous fistula successfully treated by percutaneous embolization.

Priapism and glucose-6-phosphate dehydrogenase deficiency: An underestimated correlation?

Priapism is a rare clinical condition characterized by a persistent erection unrelated to sexual excitement. Often the etiology is idiopathic. Three cases of priapism in glucose-6-phosphate dehydrogenase (G6PD) deficiency patients have been described in literature. We present the case of a 39-year-old man with glucose- 6-phosphate dehydrogenase deficiency, who reached out to our department for the arising of a non-ischemic priapism without arteriolacunar fistula. We suggest that the glucose-6-phosphate dehydrogenase deficiency could be an underestimated risk factor for priapism.

Radiologic Percutaneous Gastrostomy in Nondistended Stomach: A Modified Approach.

INTRODUCTION: Gastrostomy tube placement for patients requiring long-term nutritional support may be performed using different techniques including endoscopic, surgical, and percutaneous radiologically guided methods. Radiologically inserted gastrostomy (RIG), typically performed when percutaneous endoscopic gastrostomy is not possible, requires proper gastric distension that is achieved by insufflating air through a nasogastric tube. We describe a simple technique to prevent air escape from the stomach during gastrostomy tube placement. To the best of our knowledge, this technique has not yet been described in the literature. MATERIALS AND METHODS: Four patients with unsuccessful percutaneous endoscopic gastrostomy were referred for fluoroscopic-guided gastrostomy. One patient had a pyriform sinus tumor and three had an ischemic stroke causing dysphagia. Gastric distention was not achieved in the patients due to air escaping into the bowel during the standard RIG procedure. A modified approach using a balloon catheter inflated in the pylorus to avoid air passing into the duodenum permitted successful RIG. RESULTS: The modified RIG procedure was successfully carried out in all cases without complications. DISCUSSION: Inadequate air distension of the stomach is an unusual event that causes a failure of gastrostomy tube placement and an increased risk of both major and minor complications. The use of a balloon catheter inflated in the first part of the duodenum prevents the air passage into the bowel allowing the correct positioning of the gastrostomy.

Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.

PURPOSE: This study was undertaken to evaluate primary stenting in patients with inferior vena cava torsion after orthotopic liver transplantation performed with modified piggyback technique. MATERIALS AND METHODS: From November 2003 to October 2010, six patients developed clinical, laboratory and imaging findings suggestive of caval stenosis, after a mean period of 21 days from an orthotopic liver transplantation performed with modified piggyback technique. Vena cavography showed stenosis due to torsion of the inferior vena cava at the anastomoses and a significant caval venous pressure gradient. All patients were treated with primary stenting followed by in-stent angioplasty in three cases. RESULTS: In all patients, the stents were successfully positioned at the caval anastomosis and the venous gradient pressure fell from a mean value of 10 to 2 mmHg. Signs and symptoms resolved in all six patients. One patient died 3 months after stent placement due to biliary complications. No evidence of recurrence or complications was noted during the follow-up (mean 49 months). CONCLUSIONS: Primary stenting of inferior vena cava stenosis due to torsion of the anastomoses in patients receiving orthotopic liver transplantation with modified piggyback technique is a safe, effective and durable treatment.

Assessment of extravascular lung water by quantitative ultrasound and CT in isolated bovine lung.

Lung ultrasonography (LUS) and computed tomography (CT) were compared for quantitative assessment of extravascular lung water (EVLW) in 10 isolated bovine lung lobes. LUS and CT were obtained at different inflation pressures before and after instillation with known amounts of hypotonic saline. A video-based quantitative LUS analysis was superior to both single-frame quantitative analysis and visual scoring in the assessment of EVLW. Video-based mean LUS intensity was strongly correlated with EVLW density (r(2)=0.87) but weakly correlated with mean CT attenuation (r(2)=0.49) and physical density (r(2)=0.49). Mean CT attenuation was weakly correlated with EVLW density (r(2)=0.62) but strongly correlated with physical density (r(2)=0.99). When the effect of physical density was removed by partial correlation analysis, EVLW density was significantly correlated with video-based LUS intensity (r(2)=0.75) but not mean CT attenuation (r(2)=0.007). In conclusion, these findings suggest that quantitative LUS by video gray-scale analysis can assess EVLW more reliably than LUS visual scoring or quantitative CT.

Endovascular treatment of totally occluded superior mesenteric artery by retrograde crossing via the Villemin arcade.

Chronic mesenteric ischemia (CMI) is a rare disorder that is commonly caused by progressive atherosclerotic stenosis or occlusion of one or more mesenteric arteries. Endovascular treatment for symptomatic CMI represents a viable option, especially in high-operative risk patients. We report a case of acute symptomatic CMI with chronic totally occlusion of the superior mesenteric artery (SMA) associated with significant stenosis of celiac trunk (CT) and inferior mesenteric artery (IMA) that underwent endovascular treatment of all the three mesenteric arteries: stenting of CT and IMA stenosis, and recanalization of the SMA occlusion by retrograde crossing via the Villemin arcade.

The Amplatzer vascular plug 4: preliminary experience.

The purpose of this communication is to describe our preliminary experience with the Amplatzer Vascular Plug 4 (AVP 4) in peripheral vascular embolization. The AVP 4 was used for peripheral vascular embolization in five patients with renal pseudoaneurysm (n = 2), postsurgical peritoneal bleeding (n = 1), posttraumatic gluteal hemorrhage (n = 1), and intercostal pseudoaneurysm (n = 1). Occlusion time was recorded. Patients were followed up clinically and by imaging for 1 month after the procedure. All treated vessels or vascular abnormalities were successfully occluded within 3 min for low-flow circulation and over 8 min for high-flow circulation. At 1-month follow-up, all patients were symptom-free. All deployed devices remained in the original locations and desirable configurations. In conclusion, the AVP 4 seems to be safe and effective for occluding peripheral vessels and vascular abnormalities. Because of its compatibility with 0.038-in. catheters, it can be deployed through a diagnostic catheter following angiography without exchanging a sheath or guiding catheter. Compared with the previous generation of vascular plugs, the AVP 4 allows for faster procedure times and decreased exposure to radiation.

Hepatic arterial loop with accessory right hepatic artery aneurysm with celiac atresia: endovascular therapy with a stent and detachable coils.

The present report describes an unusual case of an aneurysm of a right hepatic artery (RHA) branching from the superior mesenteric artery; the accessory RHA was looped to the left hepatic artery arising from the celiac axis (CA) and was associated with congenital atresia of the CA. The accessory RHA aneurysm was treated with the placement of a bare stent and detachable coils through the mesh of the stent. Complete and prompt exclusion of the aneurysm was achieved with blood flow preservation in the parent artery at midterm follow-up.

Cavo-portal transposition in rat: a new simple model.

BACKGROUND: Liver transplantation in presence of diffuse portal vein thrombosis is possible by using caval blood as portal inflow, through cavo-portal transposition. However, clinical results are heterogeneous and experimental studies are needed, but similar hemodynamic conditions are difficult to obtain, especially in small animals. Herein we describe a new simple model of cavo-portal transposition in rat. METHODS: Spontaneous porto-systemic shunts are induced by subcutaneous transposition of the spleen. The presence of porto-caval shunts through the spleen permits the interruption of the main portal vein without splanchnic hemodynamic consequences. Cavo-portal transposition is achieved by anastomosing the inferior vena cava and the main portal vein after division of the pancreatic-duodenal vein. RESULTS: Selective angiography revealed total splanchnic blood diversion to the systemic venous circulation through the neoformed collaterals; macroscopical examination showed the absence of any signs of acute portal hypertension with normal liver and gut appearance. CONCLUSION: This model of cavoportal transposition is simple, effective and it simulates the clinical hemodynamic condition since the porto-systemic shunts induced by splenic subcutaneous transposition correspond to the physiological inframesocolic collaterals during chronic portal thrombosis in man.

Transjugular intrahepatic portosystemic shunt, mechanical aspiration thrombectomy, and direct thrombolysis in the treatment of acute portal and superior mesenteric vein thrombosis.

A patient was admitted because of severe abdominal pain, anorexia, and intestinal bleeding. Contrast-enhanced multidetector computed tomography demonstrated acute portal and superior mesenteric vein thrombosis (PSMVT). The patient was treated percutaneously with transjugular intrahepatic portosystemic shunt (TIPS), mechanical aspiration thrombectomy, and direct thrombolysis, and 1 week after the procedure, complete patency of the portal and superior mesenteric veins was demonstrated. TIPS, mechanical aspiration thrombectomy, and direct thrombolysis together are promising endovascular techniques for the treatment of symptomatic acute PSMVT.

Percutaneous transcatheter embolization of a large pulmonary arteriovenous fistula with an Amplatzer vascular plug.

Percutaneous transcatheter embolization has become the treatment of choice for pulmonary arteriovenous fistulas (PAVFs), in most cases replacing surgical intervention. However, while "classic" devices, such as intravascular coils and detachable balloons, have proved to be successful for interventional occlusions of small or medium-sized PAVFs, they are not ideal in larger fistulas because of the risk for embolization to the systemic circulation. We describe the case of a 61-year-old woman with a symptomatic huge solitary pulmonary arteriovenous fistula (4.5 cm in diameter), occupying part of the lung in the lower right lobe with two feeding arteries (10 and 4 mm in diameter, respectively), who underwent successful transcatheter closure with an Amplatzer Vascular Plug, a new device designed for the occlusion of vascular abnormalities.