Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 63
Filter
Add more filters

Publication year range
1.
Pediatr Radiol ; 53(11): 2305-2308, 2023 10.
Article in English | MEDLINE | ID: mdl-37612542

ABSTRACT

Portal vein arterialization is a rarely used, temporary surgical salvage solution to prevent biliary and hepatic ischemia and necrosis in acute liver de-arterialization. However, it can induce portal hypertension, causing increased morbidity and mortality. We report the case of a 5-year-old girl with portal hypertension and right ventricle volume overload following the creation of an iliacoportal shunt graft for portal vein arterialization due to vessel-adhering neuroblastoma. Partial shunt graft closure was accomplished by placing a stent graft in an hourglass configuration via the right femoral artery using two slender-sheaths in a line with the second more distal than the first. Subsequently, the patient's symptoms of right ventricle volume overload and portal hypertension decreased. In conclusion, endovascular reduction of elevated portal blood flow after portal vein arterialization is feasible, even in pediatric patients.


Subject(s)
Hypertension, Portal , Liver Transplantation , Female , Humans , Child , Child, Preschool , Portal Vein/diagnostic imaging , Portal Vein/surgery , Hypertension, Portal/diagnostic imaging , Hypertension, Portal/surgery , Prostheses and Implants
2.
Vasa ; 52(4): 264-274, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37082826

ABSTRACT

Background: The marginal vein (MV) is a persisting embryonic vein located at the lateral aspect of the lower limb. The Weber-classification, which was developed on the basis of phlebography in the 1990s, is the only existing classification system for this rare disease. Aim of this study was the structured characterization of the lateral marginal vein (MV) using magnetic resonance imaging (MRI) and evaluation of the applicability of the Weber-classification. Patients and methods: Institutional Review Board approval was obtained for this retrospective, single-center study. All patients who underwent contrast-enhanced MRI (using a prospectively determined protocol) of the untreated MV were included. MV anatomy and associated findings were characterized in a structured way taking into account the criteria of the Weber-classification for MV: inflow, outflow and extension. If three criteria of the Weber-classification were fulfilled the MV was categorized as "classifiable according to Weber". The MV was categorized as "partially classifiable according to Weber", if two criteria were met and as "not classifiable according to Weber" if less than two criteria were applicable. Results: 56 imaging studies of 58 MV (7 thoracoabdominal, 51 lower extremities) were reviewed. 18/51 MV of the lower extremities were "classifiable" according to the Weber-classification. 33/51 lower extremity MV were not definitely categorized according to the Weber-classification: 19/51 MV were "partially classifiable" and 14/51 MV were "not classifiable". 30/51 MV presented with hypoplastic, 1/51 with aplastic deep venous system. 34/51 lower extremity and 6/7 thoracoabdominal MV were associated with an additional vascular malformation (VM). Conclusions: MRI is suitable for detailed anatomic characterization of the MV and reveals additional therapy relevant findings like associated VM. The Weber-classification was not applicable in most cases, reflecting its limits and the heterogeneity of this rare disease. Structured reports rather than an obsolete classification system should be preferred for MRI of the MV.


Subject(s)
Vascular Diseases , Vascular Malformations , Humans , Retrospective Studies , Rare Diseases , Magnetic Resonance Imaging , Phlebography , Saphenous Vein , Vascular Malformations/therapy
3.
Int J Cancer ; 148(9): 2345-2351, 2021 05 01.
Article in English | MEDLINE | ID: mdl-33231291

ABSTRACT

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor in children, which can be accompanied by life-threatening thrombocytopenia, referred to as Kasabach-Merritt phenomenon (KMP). The mTOR inhibitor sirolimus is emerging as targeted therapy in KHE. As the sirolimus effect on KHE occurs only after several weeks, we aimed to evaluate whether additional transarterial embolization is of benefit for children with KHE and KMP. Seventeen patients with KHE and KMP acquired from 11 hospitals in Germany were retrospectively divided into two cohorts. Children being treated with adjunct transarterial embolization and systemic sirolimus, and those being treated with sirolimus without additional embolization. Bleeding grade as defined by WHO was determined for all patients. Response of the primary tumor at 6 and 12 months assessed by magnetic resonance imaging (MRI), time to response of KMP defined as thrombocyte increase >150 × 103 /µL, as well as rebound rates of both after cessation of sirolimus were compared. N = 8 patients had undergone additive embolization to systemic sirolimus therapy, sirolimus in this group was started after a mean of 6.5 ± 3 days following embolization. N = 9 patients were identified who had received sirolimus without additional embolization. Adjunct embolization induced a more rapid resolution of KMP within a median of 7 days vs 3 months; however, tumor response as well as rebound rates were similar between both groups. Additive embolization may be of value for a more rapid rescue of consumptive coagulopathy in children with KHE and KMP compared to systemic sirolimus only.


Subject(s)
Embolization, Therapeutic/methods , Hemangioendothelioma/drug therapy , Kasabach-Merritt Syndrome/drug therapy , Sarcoma, Kaposi/drug therapy , Sirolimus/therapeutic use , Female , Humans , Male , Retrospective Studies , Sirolimus/pharmacology
4.
Gastrointest Endosc ; 93(2): 496-502, 2021 02.
Article in English | MEDLINE | ID: mdl-32553568

ABSTRACT

BACKGROUND AND AIMS: Recommendations for the treatment of lower GI bleeding do not include bleeding from venous malformations (VMs). The aim of this study was to delineate the usefulness of a novel hybrid intervention (fluoroscopy-guided endoscopic sclerotherapy) for the treatment of symptomatic VMs in the rectosigmoidal colon with bleeding. METHODS: The magnetic resonance images of 421 patients with VM, referred to multicenter vascular anomaly centers from 2009 to 2017, were analyzed retrospectively. Treatment was performed for all patients who experienced bleeding from rectosigmoidal VMs using fluoroscopy-guided endoscopic sclerotherapy with polidocanol foam as a novel approach. RESULTS: A total of 27 patients displayed VM in the rectosigmoidal area. Eleven of these presented with acute or previous bleeding and received treatment. Active bleeding was observed in 8 patients (72.7%), whereas 3 patients (27.3%) had signs of previous bleeding. Six of the 11 patients had anemia (54.5%). There were no adverse events within 24 hours of the intervention. In a 2-year follow-up period, only 1 patient (9.1%) presented with recurrent bleeding after 13 months and was successfully treated again with fluoroscopy-guided endoscopic sclerotherapy. CONCLUSIONS: Fluoroscopy-guided endoscopic sclerotherapy was shown to be a safe and effective treatment of symptomatic VMs of the rectosigmoidal area. Thus, fluoroscopy-guided endoscopic sclerotherapy should be considered for patients with bleeding from VMs of the rectosigmoid after a comprehensive workup and interdisciplinary case discussion.


Subject(s)
Sclerotherapy , Vascular Malformations , Colon , Fluoroscopy , Humans , Retrospective Studies , Sclerosing Solutions/therapeutic use , Treatment Outcome , Vascular Malformations/complications , Vascular Malformations/therapy , Veins
5.
J Pediatr ; 226: 157-166, 2020 11.
Article in English | MEDLINE | ID: mdl-32622671

ABSTRACT

OBJECTIVE: To describe the clinical, radiologic, and histopathologic features of "congenital disseminated pyogenic granuloma" involving various organs with high morbidity related to cerebral hemorrhagic involvement. STUDY DESIGN: We searched the database of the Vascular Anomalies Center at Boston Children's Hospital from 1999 to 2019 for patients diagnosed as having multiple vascular lesions, visceral vascular tumors, congenital hemangiomatosis, multiple pyogenic granulomas, or multiple vascular lesions without a definite diagnosis. A retrospective review of the medical records, photographs, histopathologic, and imaging studies was performed. Only patients with imaging studies and histopathologic diagnosis of pyogenic granuloma were included. RESULTS: Eight children (5 male, 3 female) had congenital multifocal cutaneous vascular tumors. Lesions also were found in the brain (n = 7), liver (n = 4), spleen (n = 3), muscles (n = 4), bone (n = 3), retroperitoneum (n = 3), and intestine/mesentery (n = 2). Less commonly affected were the spinal cord, lungs, kidneys, pancreas, and adrenal gland (n = 1 each). The mean follow-up period was 21.8 months. The cerebral and visceral lesions were hemorrhagic with severe neurologic sequelae. The histopathologic diagnosis was pyogenic granuloma with prominent areas of hemorrhage and necrosis. The endothelial cells had enlarged nuclei, pale cytoplasm and were immunopositive for CD31 and negative for D2-40 and glucose transporter 1. CONCLUSIONS: Congenital disseminated pyogenic granuloma is a distinct multisystemic aggressive disorder that primarily affects the skin, brain, visceral organs, and musculoskeletal system. Differentiation of this entity from other multiple cutaneous vascular lesions is critical because of possible cerebral hemorrhagic involvement.


Subject(s)
Granuloma, Pyogenic/congenital , Granuloma, Pyogenic/diagnosis , Skin Diseases/congenital , Skin Diseases/diagnostic imaging , Diagnosis, Differential , Female , Humans , Infant, Newborn , Magnetic Resonance Imaging , Male , Retrospective Studies
6.
Laryngorhinootologie ; 99(10): 682-693, 2020 10.
Article in German | MEDLINE | ID: mdl-32987413

ABSTRACT

Hereditary hemorrhagic telangiectasia (HHT; Osler-Weber-Rendu syndrome; Morbus Osler) represents a syndrome affecting capillary vessels, leading to arteriovenous shunting. With an average worldwide prevalence of 1:5.000-8.000 HHT is considered an orphan disease. Arteriovenous shunts involve predominantly the nasal mucosa, the intestine, lung, liver and central nervous system. Epistaxis is the primary and most bothersome complaint of patients with HHT. A multistage therapeutic concept includes nasal ointment, laser therapy under local anesthesia and surgery under general anesthesia, as well as drug therapies. In addition, screening to determine affection of internal organs is carried out. Lesions that require therapy should be treated in an interdisciplinary setting. Treatment of lesions of the skin, oral and gastrointestinal mucosa and liver is carried out in regard to patients' symptoms, whereas vascular malformations of the lung and brain might need treatment without being symptomatic, due to possible life-threatening complications.


Subject(s)
Telangiectasia, Hereditary Hemorrhagic , Epistaxis/diagnosis , Epistaxis/etiology , Epistaxis/therapy , Humans , Nasal Mucosa , Prevalence , Rare Diseases , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/therapy
7.
AJR Am J Roentgenol ; 211(6): 1390-1396, 2018 12.
Article in English | MEDLINE | ID: mdl-30300008

ABSTRACT

OBJECTIVE: The objective of this study was to identify differences between venous malformations (VMs) with arteriovenous (AV) microshunts and VMs without AV microshunts. MATERIALS AND METHODS: In this retrospective study, 83 patients with untreated VMs of the extremities underwent a prospectively defined MRI protocol performed at 3 T. MR images were reviewed to determine localization and extent of each VM and tissue involvement. The VM volume and the lesion volume relative to body surface were measured. The number of phleboliths within the lesions was determined. Using time-resolved MR angiography, the hemodynamic subtype (VM with AV microshunts versus VM without AV microshunts) was classified, and the interval between the onset of arterial enhancement and lesion enhancement (tonset) was measured. Clinical data were reviewed to determine patient demographic characteristics, the onset of symptoms, the frequency of thrombophlebitis, and the pain score. RESULTS: Of the 83 patients, 56 (67%) presented with AV microshunts. The tonset (p ≤ 0.001), absolute and relative lesion volume (p = 0.001), and number of phleboliths (p = 0.038) differed significantly between these two subgroups. With use of a multivariable regression model, the strongest indicator for VM with AV microshunts was the presence of more than two phleboliths (odds ratio, 2.80; p = 0.007). No differences were noted regarding patient demographic characteristics and history or lesion morphologic features or anatomy. CONCLUSION: Occurrence of AV microshunts in untreated VM is a frequent phenomenon. This hemodynamic subtype is significantly associated with phleboliths and large lesions. The subtypes did not differ in terms of history and clinical course.


Subject(s)
Arteriovenous Malformations/complications , Arteriovenous Malformations/diagnostic imaging , Thrombophlebitis/complications , Thrombophlebitis/diagnostic imaging , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Extremities/blood supply , Extremities/diagnostic imaging , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective Studies , Young Adult
8.
Zentralbl Chir ; 143(5): 516-525, 2018 Oct.
Article in German | MEDLINE | ID: mdl-30357795

ABSTRACT

BACKGROUND: Visceral artery aneurysms (VAA) are rare and often incidental findings. Indications for treatment are symptomatic patients, pseudoaneurysms and a true aneurysm of diameter of ≥ 2.5 cm for mesenteric arteries and ≥ 3 cm for renal artery aneurysms. Pregnancy and liver transplantation play an important role in aneurysm rupture. Technical success after open or endovascular procedure and maintenance of organ perfusion are crucial. The aim of this review is to evaluate our data and experience of more than 20 years and to develop a strategy to deal with visceral artery aneurysm in elective and emergency cases. PATIENTS: Between 1995 and 2018, 179 patients (84 males, 95 females, median age 62 [18 - 87] years) were diagnosed with VAA at the Regensburg University Hospital. The site of aneurysm was the splenic artery in 113 (63%) patients (pts), hepatic and renal arteries in 22 and 21 pts (12% each), and gastropancreaticoduodenal artery in 14 (8%) and superior/inferior mesenteric artery in 9 (5%) cases. Surveillance without intervention occurred in 110 (62%) pts, and 34 (19%) pts underwent open and 35 (19%) endovascular repair. In all patients, preoperative imaging was performed, preferably by computed tomography angiography (CTA). RESULTS: A total of 69 patients underwent open or endovascular repair. 51 (74%) pts were treated electively, 18 (26%) pts presented urgently with acute bleeding. 16 emergency pts received endovascular treatment, and in 2 pts open surgery was performed. After emergency treatment, two pts exhibited segmental liver malperfusion without consequences. In one case, segmental bowel resection was necessary. 32/51 (63%) patients were treated electively by open surgery, 19/51 (37%) by endovascular procedures. There were no liver or bowel infarctions. Four splenectomies and one unilateral nephrectomy were necessary in patients with splenic or renal artery aneurysms. Moreover, three partial renal infarctions were noticed postoperatively (overall 8/21 [38%]). After endovascular repair of splenic or renal artery aneurysms, two cases of splenic and three cases of renal segmental infarction were observed. Splenectomy had to be performed twice (overall 7/14 [50%]). Organ perfusion was monitored by CTA, and preferentially by contrast enhanced ultrasound. CONCLUSION: The endovascular approach is the preferred option in an emergency to control bleeding in pseudoaneurysms. Patients for elective splenic or renal artery aneurysm repair have to be evaluated very carefully to achieve technical success with occlusion of the aneurysm and excellent organ perfusion. Imaging techniques such as ultrasound, especially CEUS, are strongly recommended in postoperative follow-up. Partial or complete splenic infarction leads to vaccination.


Subject(s)
Blood Vessel Prosthesis Implantation , Endovascular Procedures , Adolescent , Adult , Aged , Aged, 80 and over , Aneurysm , Female , Humans , Male , Middle Aged , Retrospective Studies , Treatment Outcome , Viscera , Young Adult
9.
Eur Radiol ; 27(6): 2482-2488, 2017 Jun.
Article in English | MEDLINE | ID: mdl-27699470

ABSTRACT

OBJECTIVES: To assess the treatment-induced changes of health-related quality-of-life (HRQoL) in patients with venous malformations (VM) who underwent ethanol gel sclerotherapy. METHODS: The prospective study in children and adults was approved by the local ethics committee. 31 patients (mean age 23.42 years, range 6.6 - 46.5; 26 female, 5 male) with VM were included. Patients' self-assessed HRQoL was measured before and after treatment using psychometrically validated questionnaires for adults and children. Differences were analysed with a paired t test. RESULTS: 58 sclerotherapy sessions were performed. The Physical Component Summary (PCS) at baseline was 43.69 and increased significantly (p = 0.01122) to 48.95 after treatment. The bodily pain (BP) scale increased significantly from 37.94 to 48.56 (p = 0.00002), the general health (GH) scale increased significantly from 46.69 to 52.17 (p = 0.00609). Baseline Physical Summary Score (PHS) in children increased significantly after treatment (p < 0.00001) from 25.25 to 45.89. The baseline Psychosocial Summary Score (PSS) in children was 51.08 and increased significantly (p = 0.00031) after treatment to 58.84. CONCLUSION: Paediatric and adult patients with VM suffer from bodily pain with overall reduced physical functioning. After sclerotherapy, these restrictions are successfully returning to normal levels with a positive effect on mental and psychosocial domains. KEY POINTS: • Sclerotherapy in venous malformation patients has an unknown effect on health-related quality-of-life • Prospective study showed improvements in bodily pain and general health in adults • Children and adults improved from treatment with ethanol gel • Sclerotherapy is an appropriate therapy.


Subject(s)
Ethanol/administration & dosage , Sclerosing Solutions/administration & dosage , Sclerotherapy/methods , Vascular Malformations/therapy , Activities of Daily Living , Adolescent , Adult , Child , Female , Gels , Humans , Male , Middle Aged , Musculoskeletal Pain/prevention & control , Prospective Studies , Quality of Life , Surveys and Questionnaires , Treatment Outcome , Young Adult
10.
Eur Radiol ; 27(1): 296-305, 2017 Jan.
Article in English | MEDLINE | ID: mdl-26905867

ABSTRACT

OBJECTIVES: Quantitative evaluation of hemodynamic characteristics of arteriovenous and venous malformations using time-resolved magnetic resonance angiography (MRA) at 3.0 Tesla. METHODS: Time-resolved MRA with interleaved stochastic trajectories (TWIST) at 3.0 Tesla was studied in 83 consecutive patients with venous malformations (VM) and arteriovenous malformations (AVM). Enhancement characteristics were calculated as percentage increase of signal intensity above baseline over time. Maximum percentage signal intensity increase (signalmax), time intervals between onset of arterial enhancement and lesion enhancement (tonset), and time intervals between beginning of lesion enhancement and maximum percentage of lesion enhancement (tmax) were analyzed. RESULTS: All AVMs showed a high-flow hemodynamic pattern. Two significantly different (p < 0.001) types of venous malformations emerged: VMs with arteriovenous fistulas (AVF) (median signalmax 737 %, IQR [interquartile range] = 511 - 1182 %; median tonset 5 s, IQR = 5 - 10 s; median tmax 35 s, IQR = 26 - 40 s) and without AVFs (median signalmax 284 %, IQR = 177-432 %; median tonset 23 s, IQR = 15 - 30 s; median tmax 60 s, IQR = 55 - 75 s). CONCLUSIONS: Quantitative evaluation of time-resolved MRA at 3.0 Tesla provides hemodynamic characterization of vascular malformations. VMs can be subclassified into two hemodynamic subgroups due to presence or absence of AVFs. KEY POINTS: • Time-resolved MRA at 3.0 Tesla provides quantitative hemodynamic characterization of vascular malformations. • Malformations significantly differ in time courses of enhancement and signal intensity increase. • AVMs show a distinctive high-flow hemodynamic pattern. • Two significantly different types of VMs emerged: VMs with and without AVFs.


Subject(s)
Hemodynamics/physiology , Magnetic Resonance Angiography/methods , Vascular Malformations/diagnostic imaging , Vascular Malformations/physiopathology , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Retrospective Studies , Young Adult
11.
AJR Am J Roentgenol ; 209(6): 1390-1395, 2017 Dec.
Article in English | MEDLINE | ID: mdl-28929808

ABSTRACT

OBJECTIVE: In the treatment of venous malformations, ethanol may be administered in a gelified form to increase local effects and reduce systemic ones. The purpose of this prospective study was to evaluate the efficacy and safety of a commercially available viscous ethanol gel in the treatment of venous malformations. SUBJECTS AND METHODS: Thirty-one patients (mean age, 23.4 years; age range, 6.6-46.5 years) with venous malformations were prospectively scheduled for two ethanol-gel sclerotherapy sessions. Venous malformations were located at the lower extremity (n = 18), the upper extremity (n = 9), and the face (n = 4). Questionnaires to assess pain, clinical examinations, professional photographs, and contrast-enhanced MRI of the venous malformations were performed before and after therapy to measure therapy-induced changes. Two experienced radiologists blinded to the examination date and clinical status compared photographs and MR images before and after treatment. RESULTS: A mean of 4.2 mL of ethanol gel were administered per session. The technical success rate was 100%. Clinical success, defined as improvement or resolution of symptoms, was noted in 81% of patients. Mean pain score decreased, and the difference was statistically significant (3.9 vs 3.1, p = 0.005). In 54 treatment sessions where follow-up was available, four minor complications occurred. Comparison of photographs and MR images before and after treatment showed improvement in 35% and 93% of patients, respectively. CONCLUSION: Ethanol gel is an effective and safe sclerosing agent in the treatment of venous malformations.


Subject(s)
Ethanol/therapeutic use , Gels/therapeutic use , Sclerosing Solutions/therapeutic use , Sclerotherapy/methods , Vascular Malformations/therapy , Adolescent , Adult , Child , Contrast Media , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Pain Measurement , Prospective Studies , Treatment Outcome , Vascular Malformations/diagnostic imaging
12.
Radiology ; 274(3): 906-16, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25380455

ABSTRACT

PURPOSE: To determine computed tomographic (CT) features of early type II endoleaks associated with aneurysm sac enlargement after endovascular aortic aneurysm repair (EVAR) of abdominal aortic aneurysm. MATERIALS AND METHODS: Institutional review board approval was not required for this retrospective study. The authors reviewed imaging and clinical data from 56 patients (seven women, 49 men; mean age ± standard deviation, 71 years ± 7.9; age range, 52-85 years) with early type II endoleak who had undergone EVAR between December 2002 and December 2011 and who had been followed up with imaging and clinical evaluation for at least 6 months. The number and diameter of all feeding and/or draining arteries were measured, and endoleaks were classified according to their sources into simple inferior mesenteric artery (IMA), simple lumbar artery (LA), complex LA, and complex IMA-LA type II endoleaks. Volume and attenuation of the nidus were measured. Aneurysm enlargement was defined as an increase in the aneurysm volume of more than 5% during follow-up. Simple and multivariate logistic regression analyses were performed to identify independent clinical and imaging variables associated with aneurysm enlargement. RESULTS: Twenty-three of the 56 patients (41%) showed aneurysm sac enlargement during follow-up (mean follow-up, 3.0 years ± 2.0). With the multivariate model, the variables that showed the strongest indicators for aneurysm sac enlargement were complex IMA-LA type II endoleak (odds ratio [OR] = 10.29, P = .004) and the diameter of the largest feeding and/or draining artery (OR = 4.55, P = .013). Patients without complex IMA-LA type II endoleak in whom the largest feeding and/or draining artery was larger than 3.8 mm and patients with a complex IMA-LA type II endoleak in whom the largest feeding and/or draining artery was larger than 2.2 mm were at high risk for aneurysm sac enlargement. CONCLUSION: The strongest indicators for aneurysm sac enlargement are complex IMA-LA type II endoleak and the diameter of the largest feeding and/or draining artery.


Subject(s)
Aortic Aneurysm, Abdominal/pathology , Aortic Aneurysm, Abdominal/surgery , Endoleak/diagnostic imaging , Endoleak/etiology , Endovascular Procedures/adverse effects , Tomography, X-Ray Computed , Aged , Aged, 80 and over , Endoleak/classification , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Prognosis , Retrospective Studies
13.
J Pediatr ; 166(4): 1048-54.e1-5, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25681199

ABSTRACT

OBJECTIVES: To test the hypothesis that somatic phosphatidylinositol-4,5-bisphospate 3-kinase, catalytic subunit alpha (PIK3CA) mutations would be found in patients with more common disorders including isolated lymphatic malformation (LM) and Klippel-Trenaunay syndrome (KTS). STUDY DESIGN: We used next generation sequencing, droplet digital polymerase chain reaction, and single molecule molecular inversion probes to search for somatic PIK3CA mutations in affected tissue from patients seen at Boston Children's Hospital who had an isolated LM (n = 17), KTS (n = 21), fibro-adipose vascular anomaly (n = 8), or congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (n = 33), the disorder for which we first identified somatic PIK3CA mutations. We also screened 5 of the more common PIK3CA mutations in a second cohort of patients with LM (n = 31) from Seattle Children's Hospital. RESULTS: Most individuals from Boston Children's Hospital who had isolated LM (16/17) or LM as part of a syndrome, such as KTS (19/21), fibro-adipose vascular anomaly (5/8), and congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (31/33) were somatic mosaic for PIK3CA mutations, with 5 specific PIK3CA mutations accounting for ∼ 80% of cases. Seventy-four percent of patients with LM from Seattle Children's Hospital also were somatic mosaic for 1 of 5 specific PIK3CA mutations. Many affected tissue specimens from both cohorts contained fewer than 10% mutant cells. CONCLUSIONS: Somatic PIK3CA mutations are the most common cause of isolated LMs and disorders in which LM is a component feature. Five PIK3CA mutations account for most cases. The search for causal mutations requires sampling of affected tissues and techniques that are capable of detecting low-level somatic mosaicism because the abundance of mutant cells in a malformed tissue can be low.


Subject(s)
Abnormalities, Multiple , DNA/genetics , Klippel-Trenaunay-Weber Syndrome/genetics , Lymphatic Abnormalities/genetics , Mutation , Phosphatidylinositol 3-Kinases/genetics , Vascular Malformations/genetics , Child , Child, Preschool , Class I Phosphatidylinositol 3-Kinases , Female , High-Throughput Nucleotide Sequencing , Humans , Infant , Klippel-Trenaunay-Weber Syndrome/diagnosis , Klippel-Trenaunay-Weber Syndrome/metabolism , Lymphatic Abnormalities/diagnosis , Lymphatic Abnormalities/metabolism , Male , Phosphatidylinositol 3-Kinases/metabolism , Polymerase Chain Reaction , Vascular Malformations/diagnosis , Vascular Malformations/metabolism
15.
Radiol Case Rep ; 19(11): 5313-5317, 2024 Nov.
Article in English | MEDLINE | ID: mdl-39280744

ABSTRACT

A 68-year-old male with liver cirrhosis presented with dizziness and dyspnea two days after endoscopic Histoacryl occlusion of gastric varicses. Imaging revealed a large endovascular embolization of Histoacryl glue, spanning from porto-caval collaterals via the inferior vena cava to the right atrium, partially occluding right atrial inflow. This case report describes the successful removal of this large net-like mass of Histoacryl glue using thrombectomy devices from the inferior vena cava and the right atrium. Postprocedure imaging showed near-complete clearance with residual fragments in the superior mesenteric vein and small emboli in the pulmonary arteries. The patient was discharged in stable condition. Histoacryl glue can cause severe complications if embolized. This case highlights the potential of advanced thrombectomy devices for managing embolic complications from endovascular treatments.

16.
Acta Radiol Open ; 13(6): 20584601241253780, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38766646

ABSTRACT

The management of metastatic hepatocellular carcinoma (HCC) is complex, particularly when complicated by pulmonary embolism. In these cases, atezolizumab-bevacizumab therapy is contraindicated due to an elevated risk of thromboembolic events. Differentiating pulmonary tumor embolism from thromboembolic disease is diagnostically challenging. This report outlines the benefit of transcatheter aspiration to obtain pathological evidence of pulmonary artery tumor embolus in an HCC patient. The intervention enabled a significant shift in the management strategy, leading to an escalation of systemic HCC therapy. This case underscores the importance of precise diagnostic techniques such as transcatheter aspiration in guiding treatment decisions, particularly in cases where pulmonary embolism may signify an underlying malignancy-driven process.

17.
Diagnostics (Basel) ; 14(15)2024 Aug 02.
Article in English | MEDLINE | ID: mdl-39125552

ABSTRACT

This study uses magnetic resonance imaging (MRI) to investigate the potential of the hepatospecific contrast agent gadolinium ethoxybenzyl-diethylenetriaminepentaacetic acid (Gd-EOB-DTPA) in distinguishing G1- from G2/G3-differentiated hepatocellular carcinoma (HCC). Our approach involved analyzing the dynamic behavior of the contrast agent in different phases of imaging by signal intensity (SI) and lesion contrast (C), to surrounding liver parenchyma, and comparing it across distinct groups of patients differentiated based on the histopathological grading of their HCC lesions and the presence of liver cirrhosis. Our results highlighted a significant contrast between well- and poorly-differentiated lesions regarding the lesion contrast in the arterial and late arterial phases. Furthermore, the hepatobiliary phase showed limited diagnostic value in cirrhotic liver parenchyma due to altered pharmacokinetics. Ultimately, our findings underscore the potential of Gd-EOB-DTPA-enhanced MRI as a tool for improving preoperative diagnosis and treatment selection for HCC while emphasizing the need for continued research to overcome the diagnostic complexities posed by the disease.

18.
Cardiovasc Intervent Radiol ; 47(5): 621-631, 2024 May.
Article in English | MEDLINE | ID: mdl-38639781

ABSTRACT

PURPOSE: CT-guided percutaneous core biopsy of the lung is usually performed under local anesthesia, but can also be conducted under additional systemic opioid medication. The purpose of this retrospective study was to assess the effect of intravenous piritramide application on the pneumothorax rate and to identify risk factors for post-biopsy pneumothorax. MATERIALS AND METHODS: One hundred and seventy-one core biopsies of the lung were included in this retrospective single center study. The incidence of pneumothorax and chest tube placement was evaluated. Patient-, procedure- and target-related variables were analyzed by univariate and multivariable logistic regression analysis. RESULTS: The overall incidence of pneumothorax was 39.2% (67/171). The pneumothorax rate was 31.5% (29/92) in patients who received intravenous piritramide and 48.1% (38/79) in patients who did not receive piritramide. In multivariable logistic regression analysis periinterventional piritramide application proved to be the only independent factor to reduce the risk of pneumothorax (odds ratio 0.46, 95%-confidence interval 0.24, 0.88; p = 0.018). Two or more pleura passages (odds ratio 3.38, 95%-confidence interval: 1.15, 9.87; p = 0.026) and prone position of the patient (odds ratio 2.27, 95%-confidence interval: 1.04, 4.94; p = 0.039) were independent risk factors for a higher pneumothorax rate. CONCLUSION: Procedural opioid medication with piritramide proved to be a previously undisclosed factor decreasing the risk of pneumothorax associated with CT-guided percutaneous core biopsy of the lung. LEVEL OF EVIDENCE 4: small study cohort.


Subject(s)
Analgesics, Opioid , Image-Guided Biopsy , Lung , Pirinitramide , Pneumothorax , Radiography, Interventional , Tomography, X-Ray Computed , Humans , Pneumothorax/prevention & control , Pneumothorax/etiology , Female , Male , Retrospective Studies , Tomography, X-Ray Computed/methods , Middle Aged , Analgesics, Opioid/administration & dosage , Radiography, Interventional/methods , Aged , Image-Guided Biopsy/adverse effects , Image-Guided Biopsy/methods , Risk Factors , Lung/diagnostic imaging , Lung/pathology , Pirinitramide/administration & dosage , Pirinitramide/therapeutic use , Adult , Incidence
19.
Acad Radiol ; 2024 Sep 30.
Article in English | MEDLINE | ID: mdl-39353826

ABSTRACT

PURPOSE: To quantitatively and qualitatively evaluate and compare the performance of leading large language models (LLMs), including proprietary models (GPT-4, GPT-3.5 Turbo, Claude-3-Opus, and Gemini Ultra) and open-source models (Mistral-7b and Mistral-8×7b), in simplifying 109 interventional radiology reports. METHODS: Qualitative performance was assessed using a five-point Likert scale for accuracy, completeness, clarity, clinical relevance, naturalness, and error rates, including trust-breaking and post-therapy misconduct errors. Quantitative readability was assessed using Flesch Reading Ease (FRE), Flesch-Kincaid Grade Level (FKGL), SMOG Index, and Dale-Chall Readability Score (DCRS). Paired t-tests and Bonferroni-corrected p-values were used for statistical analysis. RESULTS: Qualitative evaluation showed no significant differences between GPT-4 and Claude-3-Opus for any metrics evaluated (all Bonferroni-corrected p-values: p = 1), while they outperformed other assessed models across five qualitative metrics (p < 0.001). GPT-4 had the fewest content and trust-breaking errors, with Claude-3-Opus second. However, all models exhibited some level of trust-breaking and post-therapy misconduct errors, with GPT-4-Turbo and GPT-3.5-Turbo with few-shot prompting showing the lowest error rates, and Mistral-7B and Mistral-8×7B showing the highest. Quantitatively, GPT-4 surpassed Claude-3-Opus in all readability metrics (all p < 0.001), with a median FRE score of 69.01 (IQR: 64.88-73.14) versus 59.74 (IQR: 55.47-64.01) for Claude-3-Opus. GPT-4 also outperformed GPT-3.5-Turbo and Gemini Ultra (both p < 0.001). Inter-rater reliability was strong (κ = 0.77-0.84). CONCLUSIONS: GPT-4 and Claude-3-Opus demonstrated superior performance in generating simplified IR reports, but the presence of errors across all models, including trust-breaking errors, highlights the need for further refinement and validation before clinical implementation. CLINICAL RELEVANCE/APPLICATIONS: With the increasing complexity of interventional radiology (IR) procedures and the growing availability of electronic health records, simplifying IR reports is critical to improving patient understanding and clinical decision-making. This study provides insights into the performance of various LLMs in rewriting IR reports, which can help in selecting the most suitable model for clinical patient-centered applications.

20.
Clin Hemorheol Microcirc ; 87(2): 221-235, 2024.
Article in English | MEDLINE | ID: mdl-38306026

ABSTRACT

BACKGROUND: Differentiation of high-flow from low-flow vascular malformations (VMs) is crucial for therapeutic management of this orphan disease. OBJECTIVE: A convolutional neural network (CNN) was evaluated for differentiation of peripheral vascular malformations (VMs) on T2-weighted short tau inversion recovery (STIR) MRI. METHODS: 527 MRIs (386 low-flow and 141 high-flow VMs) were randomly divided into training, validation and test set for this single-center study. 1) Results of the CNN's diagnostic performance were compared with that of two expert and four junior radiologists. 2) The influence of CNN's prediction on the radiologists' performance and diagnostic certainty was evaluated. 3) Junior radiologists' performance after self-training was compared with that of the CNN. RESULTS: Compared with the expert radiologists the CNN achieved similar accuracy (92% vs. 97%, p = 0.11), sensitivity (80% vs. 93%, p = 0.16) and specificity (97% vs. 100%, p = 0.50). In comparison to the junior radiologists, the CNN had a higher specificity and accuracy (97% vs. 80%, p < 0.001; 92% vs. 77%, p < 0.001). CNN assistance had no significant influence on their diagnostic performance and certainty. After self-training, the junior radiologists' specificity and accuracy improved and were comparable to that of the CNN. CONCLUSIONS: Diagnostic performance of the CNN for differentiating high-flow from low-flow VM was comparable to that of expert radiologists. CNN did not significantly improve the simulated daily practice of junior radiologists, self-training was more effective.


Subject(s)
Deep Learning , Magnetic Resonance Imaging , Vascular Malformations , Humans , Vascular Malformations/diagnostic imaging , Magnetic Resonance Imaging/methods , Female , Male , Adult , Middle Aged , Adolescent , Child , Aged , Child, Preschool
SELECTION OF CITATIONS
SEARCH DETAIL