巨大静脈奇形（頚部口腔咽頭びまん性病変）

BACKGROUND: Coronary microvascular dysfunction (CMD) was frequently encountered in patients with angina in the absence of epicardial coronary stenosis. CMD was associated with adverse outcomes while the etiology was unclear. OBJECTIVE: In this study, we aimed to investigate the association between lipidomic abnormalities, biological aging and CMD. METHODS: We consecutively enrolled 43 patients with angina exhibiting no epicardial coronary stenosis on angiography. CMD was defined by a coronary angiography-derived index of microvascular resistance (caIMR) >25. Targeted lipidomic profiling quantified 781 lipids. PhenoAge was calculated from clinical parameters. Using machine learning, lipidomic data and clinical characteristics were screened to identify candidate biomarkers. RESULTS: There were 25 patients having CMD with the mean caIMR of 34.0 (Interquartile range [IQR], 31.0-36.5), in contrast to 23.0 (IQR, 19.0-24.5) in the control group. Patients with CMD had substantially older PhenoAge (61 vs 52, p = 0.002). Forty-eight lipid species were dysregulated in patients with CMD, which predominantly belonged to triacylglycerol (TAG). PhenoAge, triglyceride glucose index, hypertension, TAG47:2-FA14:0, TAG48:4-FA18:1 and phosphatidylcholine (18:1/20:3) were independent risk factors of CMD. Incorporating dysregulated lipids significantly improved CMD prediction compared to traditional clinical factors alone (area under the curve, 0.94 vs 0.86, p < 0.05). CONCLUSION: PhenoAge acceleration and dysregulated lipidome were associated with CMD. Larger-scale studies and external validation are needed in the future.

The Kawashima procedure is a variant of the bidirectional Glenn used in patients with interrupted inferior vena cava (IVC) and azygos continuation, though optimal timing and the role of fenestration remain debated. We report a 5-month-old boy with functional single ventricle physiology, transposition of the great arteries, pulmonary valve atresia with ventricular septal defect, interrupted IVC with azygos continuation, and bilateral superior vena cavae, who presented with cyanosis and failure to thrive. Imaging confirmed complex venous anomalies with hepatic venous drainage directly into the right atrium. The patient underwent a Kawashima procedure without fenestration, with stable postoperative hemodynamics, improved oxygen saturation, and an uncomplicated recovery. This case demonstrates that early Kawashima without fenestration is feasible in carefully selected infants, although exclusion of hepatic venous flow may predispose to pulmonary arteriovenous malformations, necessitating eventual Fontan completion. Favorable outcomes can be achieved in resource-limited settings with appropriate patient selection and multidisciplinary care.

Cavernous hemangiomas were originally taught as "no touch lesions" as they were benign, hypervascular, venous malformations. They are commonly found in the liver. Patients typically present asymptomatically, and these hemangiomas can be found incidentally on imaging as solitary or multiple lesions. A 42-year-old male patient arrived at our institution after a motor vehicle collision, and a giant hemangioma was found incidentally on imaging with a classic feature of peripheral nodular enhancement on arterial phase computed tomography (CT) imaging. However, the patient continued to report right upper quadrant pain. Given the patient's age and size of the lesion, percutaneous sclerotherapy using bleomycin and lipiodol was performed. At the two-week follow-up, the patient denied fever, nausea, vomiting, or pain. At six months, the patient was asymptomatic, and follow-up CT angiography (CTA) demonstrated an approximately 50% reduction in lesion size. Therefore, percutaneous sclerotherapy is an effective, low-risk, and technically straightforward treatment alternative to the traditional intraarterial approach, resulting in significant lesion size reduction and meaningful symptomatic relief with a favorable safety profile.

Arteriovenous shunting involving the middle meningeal artery (MMA) has been reported after cranial trauma, neurosurgical procedures, and endovascular interventions. However, its angiographic frequency and characteristics after chronic subdural hematoma (cSDH) surgery remain poorly defined. This study aimed to assess the frequency and angiographic features of MMA arteriovenous shunting in patients undergoing MMA embolization after surgical evacuation of cSDH. We retrospectively analyzed consecutive patients who underwent selective and superselective MMA angiography during MMA embolization after surgical evacuation of cSDH between January 2020 and March 2023. Angiograms were reviewed for angiographic evidence of arteriovenous shunting, shunt morphology, flow characteristics, venous drainage, relationship to embolization, and angiographic closure after embolization. Surgical technique was classified as burr-hole craniotomy or formal craniotomy. Thirty patients met the inclusion criteria. Angiographic evidence of MMA arteriovenous shunting was identified in 11 patients (36.7%). Shunting was significantly more frequent after craniotomy than after burr-hole craniotomy (90.9% vs. 5.3%, Fisher's exact test, p < 0.001). Most shunts were detectable before microcatheter advancement, while superselective angiography improved visualization of shunt morphology and venous drainage. All shunts demonstrated low-flow angiographic characteristics without cortical venous reflux. Post-embolization control angiography demonstrated disappearance of the angiographic shunt in all affected patients. Low-flow arteriovenous shunting involving the MMA is a frequent angiographic finding in patients undergoing MMA embolization after cSDH surgery, particularly following craniotomy. These findings provide systematic angiographic characterization of a previously underrecognized postoperative vascular phenomenon. The clinical relevance of such shunting remains uncertain and warrants prospective investigation.

Acute cholangitis is a potentially life-threatening infection of the biliary tract that usually results from biliary obstruction, most commonly secondary to choledocholithiasis. Although prompt diagnosis and treatment often lead to favorable outcomes, uncommon complications such as pylephlebitis (septic thrombosis of the portal vein) may significantly increase morbidity and mortality. We report the case of a 47-year-old woman with no significant past medical history who presented with right upper quadrant abdominal pain, fever (38.2°C), asthenia, and nausea. Physical examination revealed diffuse abdominal tenderness and mild jaundice. Abdominal point-of-care ultrasound (POCUS) made by the emergency physician on call showed multiple gallbladder calculi with sludge but without visible biliary dilatation. Laboratory evaluation demonstrated an inflammatory syndrome with C-reactive protein of 72 mg/L, normal leukocyte count, total bilirubin of 87 IU/L, and direct bilirubin of 56 IU/L, and cholestatic liver enzyme abnormalities, including alkaline phosphatase (ALP) of 190 IU/L and gamma-glutamyl transferase (GGT) of 108 IU/L. The diagnosis of acute angiocholitis was thus suspected. Abdominal imaging (computed tomography (CT) with contrast) showed intrahepatic and extrahepatic biliary dilatation caused by a distal common bile duct stone, multiple gallbladder calculi, and segment II left portal vein thrombosis consistent with pylephlebitis. A diagnosis of acute cholangitis secondary to choledocholithiasis complicated by pylephlebitis was established. The patient was treated with intravenous (IV) antibiotics, fluid resuscitation, and supportive care, with planned biliary decompression and definitive surgical management. This case highlights a rare but serious vascular complication of biliary stone disease. Clinicians should maintain a high index of suspicion for portal venous involvement in patients with cholangitis and persistent systemic symptoms. Early imaging, timely antimicrobial therapy, and coordinated multidisciplinary management are essential to reduce the risk of severe complications and improve outcomes.