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指定難病 — No.279

巨大静脈奇形(頚部口腔咽頭びまん性病変)

検索語 Venous Malformation ・ 最終更新 2026-07-21 18:30 ・ 最新に更新

Data Sheet
指定 No.279
Src PubMed · CT.gov · jRCT

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( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

各論文の見出しにある「確からしさ」は、その研究がどれくらい信頼できるかの目安です。「理論段階」はまだ仮説に近く、下にいくほど多くの患者で検証されていて、「メタ解析」がもっとも信頼できます。

観察研究
MK-01 · PMID 42477235

Middle meningeal artery arteriovenous shunting after surgical evacuation of chronic subdural hematoma: a retrospective angiographic cohort study

Abstract / 原文

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.

Journal
Neurosurgical review(2026 Jul)
Authors
5名
Type
Journal Article
PubMedで原文を見る
症例報告
MK-02 · PMID 42472133

Pylephlebitis Following an Acute Angiocholitis: A Case Report

Abstract / 原文

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.

Journal
Cureus(2026 Jun)
Authors
5名
Type
Case Reports, Journal Article
PubMedで原文を見る
症例報告
MK-03 · PMID 42472068

Multiple arteriovenous malformations due to liver failure: An unusual etiology

Abstract / 原文

Arteriovenous malformations (AVMs) are rare vascular anomalies, predominantly congenital and intracranial. We report a unique case of multiple extracranial acquired AVM in a 44-year-old man with ethanol-related decompensated chronic liver disease presenting with severe gingival hemorrhage. Angiography showed focal microarteriovenous shunting with early venous drainage in the absence of a mature, compact nidus involving the facial and lingual arterial systems, which was successfully treated with endovascular embolization. In the absence of earlier vascular pathology, an acquired etiology is suspected. Cirrhosis-associated angiogenic and inflammatory dysregulation may contribute to de novo AVM formation, and reported regression following liver transplantation suggests a potentially reversible, hepatic-driven mechanism.

Journal
Journal of vascular surgery cases and innovative techniques(2026 Oct)
Authors
4名
Type
Case Reports, Journal Article
PubMedで原文を見る
観察研究
MK-04 · PMID 42470022

Analysis of risk factors associated with intimal hyperplasia in arteriovenous fistulas among patients undergoing hemodialysis

Abstract / 原文

Arteriovenous fistula (AVF) intimal hyperplasia (IH) is a major pathological basis of AVF stenosis and dysfunction in patients undergoing maintenance hemodialysis. This single-center retrospective observational cohort study enrolled adult hemodialysis patients with a native AVF at our institution between May 2021 and May 2023. IH was determined primarily by duplex ultrasound (DUS) evidence of pathologic venous intimal thickening and/or hemodynamically significant stenosis attributable to IH, with angiographic confirmation when clinically indicated. Demographic characteristics, hemodialysis vintage, dialysis prescription and adequacy, vascular access profiles, comorbidities, medication exposure, and routine laboratory indices were extracted from electronic medical records, the hemodialysis information platform, and the vascular access imaging database. Comparisons were performed between the IH group (n = 38) and the control group (n = 126), followed by univariate and multivariable logistic regression analyses. Patients with IH were slightly older and had longer hemodialysis vintage and a longer interval from AVF creation to evaluation. The IH group also showed higher rates of diabetes mellitus and recent infection, higher inflammatory burden reflected by C-reactive protein (CRP) and neutrophil-to-lymphocyte ratio (NLR), lower albumin, higher d-dimer, and higher serum phosphate. In multivariable analysis, longer time from AVF creation to evaluation, diabetes mellitus, higher CRP, and higher phosphate independently correlated with IH, whereas higher albumin was protective. These findings suggest that cumulative access exposure, metabolic disease, systemic inflammation, nutritional status, and disordered mineral metabolism jointly contribute to IH risk and may inform targeted surveillance and preventive management in routine hemodialysis care.

Journal
Medicine(2026 Jul)
Authors
2名
Type
Journal Article, Observational Study
PubMedで原文を見る
不明
MK-05 · PMID 42469655

Integrated pregnancy and perinatal management of complex fetal congenital heart disease: a preliminary retrospective study

Abstract / 原文

BACKGROUND: Congenital heart disease (CHD) is the most prevalent congenital abnormality worldwide and is the primary contributor to neonatal mortality. Obstetricians in China continue to face uncertainties about optimal delivery timing, appropriate delivery mode, perinatal interventions, and postnatal surgical outcomes for fetuses with complex CHD. OBJECTIVE: To investigate integrated pregnancy and perinatal management strategies for fetuses diagnosed with complex CHD by prenatal ultrasound in China. METHODS: A retrospective analysis was performed on 123 fetuses with complex CHD diagnosed by prenatal ultrasound at Peking University People's Hospital between 2016 and 2023. RESULTS: The mean gestational age at ultrasound diagnosis was 23.7 ± 3.4 weeks. Among the 123 cases, 11 (8.9%) were total anomalous pulmonary venous connection (TAPVC), 14 (11.4%) pulmonary valve abnormalities (PVA), 7 (5.7%) right ventricular double outlet (DORV), 13 (10.6%) aortic arch abnormalities, 69 (56.1%) complete transposition of the great arteries (D-TGA), and 9 (7.3%) other types. Following prenatal counseling, all pregnancies were continued. Among the 72 fetuses undergoing prenatal genetic testing, 9 (9/72) showed chromosomal variants. Fifty-five pregnant women (44.72%) attempted trial of labor, of whom 46 (37.40%) achieved vaginal delivery, including 6 (4.88%) assisted with forceps. Trial of labor failed in 9 cases, requiring cesarean section. In total, 77 pregnancies were delivered by cesarean section, yielding a cesarean rate of 62.6%. The mean gestational age at delivery was 37.9 ± 1.4 weeks, and the mean neonatal birth weight was 3099.0 ± 479.6 g. The mean umbilical arterial blood gas pH was 7.31 ± 0.05. Postnatally, 86 infants (69.9%) received prostaglandin. Average oxygen saturation increased from 72.8 ± 6.0% before alprostadil administration to 80.5 ± 5.0% afterward, a statistically significant improvement (p = 0.000). A total of 114 neonates underwent surgical intervention; 98.2% achieved favorable outcomes, whereas two infants (1.8%) died postoperatively. Approximately 13.0% were anticipated to require secondary surgery. CONCLUSION: Pregnancies involving fetuses with complex CHD should avoid preterm birth whenever possible. Complex fetal CHD alone is not an indication for cesarean delivery; the delivery mode should instead be determined by standard obstetric considerations, and individualized management may be adopted according to their opinions and preferences. When both maternal and fetal status remain stable, scheduling delivery at or beyond 39 weeks may be considered. Newborns who present with low postnatal oxygen saturation should receive prostaglandin to keep the ductus arteriosus open, followed by urgent transfer to pediatric cardiac surgery for definitive management. Coordinated, multidisciplinary care supports favorable clinical outcomes.

Journal
BMC pregnancy and childbirth(2026 Jul)
Authors
9名
Type
Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

日本で参加できる治験

現在 募集中のもの

日本で現在募集中の治験は見つかりませんでした。下の公式レジストリで条件を変えると見つかる場合があります。
( 03 )REGISTRY / jRCT

治験をもっと探す

日本の公式レジストリで全件を確認

上の一覧は ClinicalTrials.gov の一部です。日本国内の治験の多くは、日本の公式レジストリ jRCT にのみ登録されています。下記から最新の全件をご確認ください。

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