[Classification of vascular tumours and vascular malformations. Contribution of the ISSVA 2014/2018 classification]. / Classification des tumeurs et malformations vasculaires. Apport de la classification ISSVA 2014/2018.

The study of vascular anomalies, "angiomas", vascular tumours and vascular malformations is made difficult by the great variety and confusion of the names used in the literature for these diseases, some of which are rare. The great merit of the classification proposed by the International Society for the Study of Vascular Anomalies (ISSVA), adopted in 2014 and modified in 2018, is to propose a unambiguous nomenclature and to try to group these lesions in a logical way, contrasting with the lists of the usual "classifications". This classification is based on the distinction between proliferative lesions (tumours and reactive lesions) and those which are due to a congenital anomaly of vascular morphogenesis (vascular malformations). It incorporates recent data on the molecular causes of these diseases. The major groups of lesions recognised in this classification will be presented and some lesions of interest briefly discussed. This classification aims to be usable by all medical specialties and applicable to all tissues and organs, even if efforts are still needed to integrate organ-specific names in order to unify the nomenclature and eliminate confusion. Even if it does not solve all the problems in this complex field, the unification of the nomenclature is a major contribution of this classification and pathologists are strongly encouraged to refer to it in daily practice.

Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients.

PURPOSE: To analyse quantitatively and qualitatively asymptomatic hepatic and pancreatic involvement in hereditary haemorrhagic telangiectasia (HHT) using 64-section helical CT. MATERIALS AND METHODS: The 64-section helical CT examinations of 19 patients with HHT (8 men, 11 women; mean age, 58.6 years) were quantitatively and qualitatively analysed and compared to those of 19 control subjects who were matched for age and sex. Comparisons were made using univariate analysis. RESULTS: Dilated and tortuous intrahepatic arterial branches was the most discriminating independent variable (P < 0.0001) and had the highest specificity (100%; 19/19; 95%CI: 82%-100%) and accuracy (97%; 37/38; 95%CI: 86%-100%) for the diagnosis of HHT. Heterogeneous enhancement of hepatic parenchyma, intrahepatic telangiectases, hepatic artery to hepatic vein shunting, hepatic artery enlargement (i.e. diameter > 6.5 mm) and portal vein enlargement (i.e. diameter > 13 mm) were other variables that strongly correlated with the presence of HHT. Intrapancreatic telangiectases and arteriovenous malformations were found in 42% and 16% of patients with HHT, respectively. CONCLUSION: Liver and pancreatic involvement in asymptomatic HHT patients is associated with myriad suggestive findings on 64-section helical CT. It can be anticipated that familiarity with these findings would result in more confident diagnosis of HHT.

Injection of bevacizumab and cyanoacrylate glue for hereditary hemorrhagic telangiectasia.

OBJECTIVES/HYPOTHESIS: The objective of this study was to report for the first time on the results of submucosal injections of bevacizumab used in conjunction with cyanoacrylate glue sclerotherapy in hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: Retrospective analytic chart review. METHODS: We performed a chart review that included all patients with HHT treated with intranasal bevacizumab and cyanoacrylate glue for refractory epistaxis at Lariboisiere University Hospital from 2013 with a minimum follow-up of 6 months. We injected 100 mg (25 mg/mL) of bevacizumab diluted in 2 mL of serum at the base of the telangiectasias, and sclerotherapy with an injection of cyanoacrylate glue was used adjunctively. Treatment efficacy was based on changes in Epistaxis Severity Scores (ESS) and the Bergler-Sadick Scale. Quality of life and patient satisfaction were evaluated using the Cantril Self-Anchoring Ladder (CL) and Likert scale, respectively. RESULTS: Thirty-one patients were included, with a mean follow-up of 26.6 months. The average ESS score significantly decreased from 7.82 to 3.89 (P < .05). The Bergler-Sadick score significantly improved (P < .05) following the treatment, including the frequency (from 2.74 to 1.64) and the quantity (from 2.54 to 1.51) scales. Quality of life was significantly improved (P < .05) using the CL score (from 4.16 to 7.22). The Likert satisfaction scale related to the treatment efficacy was high, with an average of 7.03 out of 10. No complications were noted. CONCLUSIONS: Submucosal injections of bevacizumab in conjunction with cyanoacrylate glue sclerotherapy significantly reduced epistaxis and improved the quality of life in HHT. Prospective comparative studies are needed to further evaluate the significance of this treatment modality. LEVEL OF EVIDENCE: 3b Laryngoscope, 129:2210-2215, 2019.

Diode endovascular laser treatment in venous malformations of the upper aerodigestive tract.

PURPOSE: Venous malformations of the upper aerodigestive tract can cause pain, dysphagia, obstructive sleep apnea, and rarely bleeding. We studied 980-nm diode endovenous laser therapy. MATERIAL AND METHODS: This is a 2007-2014 retrospective study in our vascular anomalies center. Data on patients' clinical history, polysomnography, magnetic resonance imaging, and treatment were collected. Patients were contacted for Epworth Sleepiness Scale and Eating Assessment Tool (EAT-10) scores to evaluate sleepiness and dysphagia before and after laser therapy. RESULTS: We included 32 patients (mean age 41 years) presenting with obstructive sleep apnea (n = 18) and dysphagia (n = 13). With a mean follow-up of 39 months, average Epworth Sleepiness Scale score fell from 17.3 to 10.4 (p = 0.015), EAT-10 score from 8.2 to 3.5 (p = 0.002) and apnea-hypopnea index from 47.5 to 24.7 (p = 0.01). Of the sleep apnea patients, 89% required continuous positive airway pressure before and 50% afterward (p = 0.016). CONCLUSIONS: Diode endovascular laser treatment seems to be a safe and effective treatment option in venous malformations of the upper airways.