Insertion of a totally implantable venous access port in a patient with isolated persistent left superior vena cava: A case report / 中国胸心血管外科临床杂志

@#Persistent left superior vena cava (PLSVC) with absence of right superior vena cava (SVC), also known as isolated PLSVC, is a relatively rare type of congenital body venous malformation. Isolated PLSVC is asymptomatic, however, it will bring clinical difficulties to the implantation of the totally implantable venous access port (TIVAP). We reported a 41 years, male patient with esophageal cancer, who needed neoadjuvant chemoimmunotherapy. Through doppler ultrasonography, computed tomography (CT) and vascular 3D-reconstruction, we found him to be a patient with PLSVC with absence of right SVC before the insertion of TIVAP. Hence, we chose the left approach in which the needle was inserted into the sternocleidomastoid clavicular head lateral notch in left supraclavicular fossa as the puncture point. The depth of the catheter tip from the root of the neck to the puncture point was 21.5 cm and the catheter tip was located at the junction of the PLSVC and the right atrium, at the dilated coronary sinus. The procedure was successful and the patient received expected neoadjuvant chemotherapy combined with immunotherapy after operation, and anticoagulant therapy was performed to prevent thrombosis in coronary sinus and superior vena cava. There was no major catheter-related complication during the period of TIVAP.

Venous malformation of the conjunctiva and orbit

A 28-year-old male presented with a large conjunctival lobulated, nonpulsatile, red vascular lesion involving a large part of the temporal quadrant of the left globe. There was no proptosis or globe displacement, but the left eye abduction was limited. T2-weighted magnetic resonance imaging scan of the brain and orbit revealed an extensive contrast-enhancing lobulated lesion in the left half of the face involving the upper lip, cheek, oral cavity, extraconal space of left orbit, and nasal cavity. He underwent surgical excision of the conjunctival lesion with amniotic membrane reconstruction.

Value of conventional ultrasonography combined with cervical compression in the diagnosis of orbital venous malformation / 中华超声影像学杂志

Objective:To explore the value of conventional ultrasonography combined with cervical compression in the diagnosis of orbital venous malformation (OVM).Methods:A total of 43 patients with suspected OVM were admitted in sequentially from January 2019 to July 2022 in the Third Medical Center of PLA General Hospital. All patients were examined by ultrasonography combined with cervical compression and demonstrated by operation or digital subtraction angiography (DSA). The conventional ultrasound features of OVM were summarized, and the value of conventional ultrasonography combined with cervical compression in the diagnosis of OVM was discussed.Results:The features of the conventional ultrasound combined with cervical compression for diagnosis of OVM were as follows: the interior of lesion was mainly tubular structure, and the compression test was positive. After cervical compression, the lesion enlarged and the inner diameter of the internal tubular structure widened. Doppler flow imaging showed that the interior of lesion was mainly venous blood flow. Compared with the results of postoperative pathology or DSA, the sensitivity, specificity, accuracy and positive predictive value of ultrasonography combined with cervical compression were 0.952, 1.000, 95.3% and 100%, respectively. The results of Fisher exact diagnosis showed that there was no significant difference between ultrasonography and operation or DSA of OVM( P>0.05). Conclusions:Conventional ultrasound combined with cervical compression can be used as an effective method for the diagnosis of OVM.

Effects of sirolimus on cell proliferation and apoptosis of vascular endothelial cells of venous malformation induced by TIE2-L914F mutation and its mechanism in vitro / 安徽医科大学学报

Objective@#The purpose of this study was to investigate the effects of sirolimus ( SIR) on proliferation and apoptosis of vascular endothelial cells of venous malformation ( VM) caused by mutations in TIE2-L914F and its potential molecular mechanism．@*Methods @#The expression of TEK receptor tyrosine kinase ( TIE2 ) in human umbilical vein endothelial cell (HUVEC) was interfered to construct vascular endothelial cells of VM model (TIE2-L914F group) ．Subsequently part of vascular endothelial cells of VM was exposed to 1 000 ng / ml SIR for 48 h (TIE2-L914F + SIR group) ，and the proliferation and apoptosis of vascular endothelial cells of VM were detected by MTT and flow cytometry．The mRNA and protein expressions of CXCL1 and CXCR2 were detected by qRT-PCR and Western blot. @*Results @#Compared with the cells in TIE2-L914F group，the proliferation activity of the cells in TIE2-L914F + SIR group was inhibited，and the apoptosis rate increased (P ＜0. 05) ．The expression of CXCL1 increased in TIE2-L914F cells but decreased after SIR treatment (P＜0. 05) ．Compared with TIE2-L914F + pcDNA3．1 group，the cell proliferation activity increased but apoptosis rate decreased in TIE2-L914F + pcDNA-CXCL1 group．Compared with TIE2-L914F + pcDNA-CXCL1 group，cell proliferation activity was inhibited but apoptosis rate increased in TIE2-L914F + pcDNA-CXCL1 + SIR Group (P＜0. 05) ．In addition，compared with TIE2-L914F group，CXCR2 expression decreased in TIE2-L914F + SIR group(P＜0. 05)．@*Conclusion @#SIR inhibits VM cell proliferation，induces cell apoptosis of vascular endothelial cells of VM，and inhibits the expression of CXCL1 / CXCR2.

A Case of Laryngeal Venous Malformation Successfully Treated with Keishibukuryogan and Daiokanzoto / 日本東洋医学雑誌

Laryngeal venous malformation has a risk of airway obstruction due to its enlargement or bleeding. It requires treatment such as sclerotherapy, laser treatment or surgery. However, adult-type laryngeal venous malformation occurs frequently in the upper part of the glottis, and causes almost no symptoms except when it occurs in the vocal cords. So some patients do not wish to undergo surgery. This time, we describe a case of an asymptomatic laryngeal venous malformation patient who did not wish to receive laser treatment, and was significantly improved by Kampo medicine. The patient was a 69-year-old man. He consulted us because of an accidental laryngeal mass on upper gastrointestinal endoscopy during a medical examination, but he had no particular pharyngeal symptoms. A venous malformation was found in the upper part of the right glottis, and we recommended him laser treatment. But he did not wish it because he had no symptoms. We considered the condition of static blood (oketsu), and administered keishibukuryogan for 7 weeks, but there was no change. Since he had constipation, we administered daiokanzoto together with keishibukuryogan, and his bowel movement improved. Thirteen weeks after the beginning of the combined use, the venous malformation also remarkably reduced.

Primary intra-ventricular hemorrhage in 33-year-old man: a case report

The aim of the study wasto provide insight into cases of primary intra-ventricular hemorrhagecaused by arterial venous malformations. A 33-year-old man visited the emergency room and admitted that he had a chief complaint of headache for the last 7 days. Neurological clinical examination only found nuchal rigidity. The CT scan of the head showed intra-ventricular hemorrhage and the CTA results showed an AVM with a left occipital nidus with arterialization of the posterior cerebral artery. One day before the DSA was performed, the patient had a seizure and based on a repeat CT scan, the bleeding spread to the parenchyma. The patient refused decompression and decided to go home after his condition stabilized and his complaints improved with anti-edema treatment. A repeat DSA action was planned but the patient had financial problems. Primary intra-ventricular hemorrhage occurs when the nidus arterial venous malformation is adjacent to the ventricle although this is only 7% of all cases of primary intra-ventricular hemorrhage. In the case of primary intra-ventricular hemorrhage, complications need to bewary of when hydrocephalus appears. In addition, digital subtraction angiography should be performed prior to endovascular surgery as the main modality of therapy. Arterial venous malformation can be one of the causes. Digital angiography and Endovascularmeasures should be performed in this patient to prevent further complications. However, the refusal of the patient and family until the time of this case report was made is a separate obstacle in the diagnostic and therapeutic process.

MRI findings and pathological analysis of intramuscular fibro-adipose vascular anomaly / 中华放射学杂志

Objective:To investigate the MRI and pathological features of intramuscular fibro-adipose vascular anomaly (FAVA).Methods:The clinical and imaging data of 44 patients with intramuscular FAVA confirmed by pathology from December 2012 to March 2021 in Henan Province People′s Hospital were retrospectively analyzed. Twenty-five females and 19 males were included, with the age of (15±6), from 5 to 29 years old. The clinical and MRI features including the type, location, boundary, signal intensity, enhancement mode and degree, and the vascular flow voids in the lesion were summarized and compared with pathological results.Results:The thigh and calf muscles were involved in 1 patient simultaneously, and 1 site was involved in 43 patients, including 20 calf muscles, 15 thigh muscles, 5 forearm muscles, 1 upper arm muscle, 1 gluteal muscle, and 1 shoulder muscle. The gastrocnemius muscle of lower leg was most commonly involved (13/44), followed by soleus muscle (10/44) and quadriceps femoris muscle (9/44). All the lesions were solid on MRI, including 24 cases of focal mass type, 15 cases of diffuse infiltration type and 5 cases of local infiltration type. The long axis of all the lesions were consistent with the long axis of the muscles. All lesions showed inhomogeneously moderate hyperintensity on T 1WI and T 2WI, and significantly hyperintensity on fat suppression T 2WI. All lesions showed tortuous and dilated abnormal vessels, of which 18 cases showed vascular flow voids. Thrombosis was found in 10 cases. On contrast-enhanced imaging, the lesions showed moderate to obvious inhomogeneous enhancement. Pathologically, the diseased skeletal muscle was infiltrated by fibrous tissue, fat components, irregular abnormal veins and vessels, which led to inhomogeneous MRI signals. Among the 7 patients who underwent human PIK3CA gene mutation detection, and 6 were mutant. Conclusions:Intramuscular FAVA has certain characteristics in clinic, MRI imaging and histopathology, and its MRI signal characteristics can reflect its complex pathological components.

Imaging characteristics analysis of high resolution CT and MRI of facial nerve venous malformation / 中华放射学杂志

Objective:To analyze imaging features on high resolution CT (HRCT) and MRI of facial nerve venous malformation (FNVM).Methods:A total of 23 patients with pathologically confirmed FNVM were retrospectively included from January 2016 to December 2020 in the Shanghai Ninth People′s Hospital, Shanghai Jiao Tong University School of Medicine. All patients underwent preoperative temporal bone HRCT, non-contrast MRI, diffusion weighted imaging (DWI) and dynamic contrast enhanced MRI (DCE-MRI). The location, morphology, and signal intensity of the lesions, as well as the enhancement pattern on DCE-MRI were reviewed. The time-intensity curve (TIC) was analyzed and the apparent diffusion coefficient (ADC) was measured.Results:The locations of FNVM lesions were geniculate ganglion ( n=19), mastoid segment ( n=1) and internal auditory canal ( n=3). Among 19 cases in geniculate ganglion, HRCT showed honeycomb-like bone change ( n=13) or expansive bone destruction with sharp border ( n=6). The case located in mastoid segment also presented as honeycomb-like changes. The other 3 cases in internal auditory canal presented with patchy hyperdensity bone change ( n=2) or resorptive bone destruction ( n=1). All 23 lesions demonstrated as irregular nodules with obviously heterogeneous enhancement on MRI. The lesions showed isointensity ( n=14), hypointensity ( n=8) or hyperintensity ( n=1) on T 1WI, and hyperintensity ( n=15) or isointensity ( n=8) on fat-suppression T 2WI. The ADC values of the lesions were (1.18±0.20)×10 -3 mm 2/s. A characteristic "point-to-plane" progressive enhancement pattern displayed on DCE-MRI, with type Ⅰ TIC (persistent pattern) presented. Conclusion:The imaging characteristics of FNVM are honeycomb-like bone changes on HRCT and"point-to-plane"progressive enhancement pattern on DCE-MRI.

Progress in evidence-based research on the clinical treatment of infantile hemangioma and vascular malformations / 口腔疾病防治

@#Hemangiomas and vascular malformations are common clinical diseases. According to their clinical and imaging characterizations, the International Society for the Study of Vascular Anomalies (ISSVA) has systematically classified infantile hemangioma and vascular malformations, and the classification has been widely recognized and applied. To date, most vascular malformations involve the following important signaling pathways: PI3K/Akt/mTOR and RAS/MAPK/ERK. This discovery has major impacts on the diagnosis and treatment of vascular malformations including the following: the understanding of the biology of vascular malformations has been increased; the understanding of vascular malformations based on genotype has been refined; and the development of targeted drugs for the treatment of vascular malformations has been promoted. Despite facing many challenges, with the development of gene sequencing, molecular biology and imaging technology, the relevance of vascular malformation classification and the accuracy of diagnosis are improving, and this is accompanied by innovations in surgical treatment and sclerotherapy, interventional embolization, and continuous progress in targeted therapy. At present, investigations on vascular malformations are mostly retrospective clinical studies or low-level clinical trials. The purpose of this paper is to review the literature on the treatment of infantile hemangioma, lymphatic malformation, venous malformation and arteriovenous malformation and to review the research progress in evidence-based treatment of infantile hemangioma and vascular malformation.

Giant complicated glosso-cervical arterio venous malformation managed with external carotid artery ligation and serial injection sclerotherapy: a case report and review of literature

Giant arterio venous malformation (AVM) of the tongue and floor of mouth are rare. They become life threatening when ignored by the patients. Treatment protocols are not well established. This report describes the treatment of a 35 years old female who presented to us with complains of swelling of tongue, floor of mouth and left sub mandibular region since 15 years causing dysphagia, and oral bleed since one day. Magnetic resonance Angiography revealed a giant AVM at the above site with dilated left external carotid artery (ECA) and left lingual artery. Patient underwent ECA ligation under general anaesthesia and serial injection sclerotherapy at the local site. After 3 months and 6 cycles of sclerotherapy, the patient had a satisfactory outcome. A detailed report with review of literature is presented.

The analysis of equinus deformity caused by intramuscular venous malformation onset posterior muscles of leg and the treatment of surgery / 中华整形外科杂志

Objective@#To analyze the causes of equinus deformity caused by intramuscular venous malformation onset posterior muscles of leg, and discuss the corresponding treatment methods.@*Methods@#A retrospective study was conducted on 69 cases of intramuscular venous malformations with equinus deformity from January 2012 to December 2017. Based on patient's main complain, physical examination and imaging data, the causes were divided into two categories: pain disorder and contracture disorder. Classification was on the basis of definite diagnosis of MRI. When the main complaint of medical history and physical examination indicated pain relief or passivity of the affected limb, and when the back extension of ankle joint was greater than 75 degrees, it was a pain disorder; when the medical history and physical examination indicated pain relief or passivity of the affected limb, the back extension of ankle joint was less than 15 degrees, it was a contracture disorder. Therapeutic methods included drug conservative treatment and surgical treatment. For the patients with pain disorder, the first choice was drug conservative treatment, and for the patients with contracture disorder, the first choice was surgery. Operative methods include simple venous malformation resection, venous malformation resection and Z-type Achilles tendon anastomosis lengthening. After operation patients received systematic functional rehabilitation exercise and calculated the satisfaction rate.@*Results@#13 cases of painful disorders were firstly treated by conservative medicine, but 4 cases were treated by operation after series of conservative treatments, satisfaction rate was 69.2%(9/13). 56 contracture cases were treated by operation, including 11 cases of simple venous malformation resection, 45 cases resection and Z-type anastomosis lengthening of Achilles tendon. All the patients were followed up for 6 months to 2 years after operation. 53 patients recovered to normal walking after operation, and 3 patients had mild limp, satisfaction rate was 94.6%(53/56). Patient satisfaction was 100%.@*Conclusions@#The equinus deformity caused by intramuscular venous malformation onset posterior muscles of leg affect the quality of life. Muscle/tendon contracture was the main cause. Correct surgical treatment combined with early rehabilitation exercise post operation can restore normal walking posture.

Venous malformation of lower limb with hypertrophy of limb, early terminalization of hair and hyperhidrosis

Vascular anomalies are the commonest developmental disorders. Venous malformations (VM) result due todysmorphogenesis in the development of veins. Most commonly these disorders are localized to skin and subcutaneoustissue. Deeper venous malformation may affect the underlying muscle, bone and joints. Early terminalization of hair andincreased sweating in VM plaque has been rarely reported. The present study deals with the case of a 15-year-old boywho had VM involving skin, subcutaneous tissue and bones with terminal hair and increased sweating over the plaque.Radiological examination showed bone hypertrophy with slow flow channels and phleboliths due to superficial and deepVM. He was treated with ethanol sclerotherapy.

Efficacy and security of lauromacrogol and pingyangmycin in treatment of hemangioma and venous malformation: A meta-analysis / 解放军医学杂志

Objective: To systematically evaluate the clinical efficacy and safety of pingyangmycin and lauromacrogol in treatment of hemangioma or venous malformation. Methods: All the databases of PubMed, Cochrane Library, Embase, Web of Science, Wanfang, CBM, VIP and CNKI were searched from their inception to November 30, 2018 to seek the randomly controlled trials (RCTs) involving the efficacy and adverse reaction of lauromacrogol and pingyangmycin in treatment of hemangioma and venous malformation. According to the inclusion and exclusion criteria, two reviewers were respectively responsible for screening researches, extracting data and assessing the risk of bias of included studies. Subsequently, meta-analysis was performed with RevMan 5.3 software. Results: A total of 12 studies containing 1619 individuals with hemangioma or venous malformation were incorporated. Meta analysis showed that the cured rates of hemangioma and venous malformation were superior when treated with lauromacrogol than with pingyangmycin, the difference was statistically significant (OR=1.98, 95%CI 1.58-2.49, P<0.001). While no significant difference existed in the efficiency (OR=1.17, 95%CI 0.40-3.41, P=0.77) and inefficiency (OR=0.44, 95%CI 0.12-1.66, P=0.23) when treating hemangioma and venous malformation with lauromacrogol or pingyangmycin. The incidence of complication was distinctly lower in lauromacrogol group than in pingyangmycin group with statistical significance (OR=0.27, 95%CI 0.17-0.44, P<0.001). Conclusion: In the treatment of hemangioma and venous malformation, lauromacrogol is obviously superior to pingyangmycin in the therapeutic effect and safety, but there is no significant difference in effectively reducing the focus.

Progress in the genetics research of infantile hemangioma and vascular malformations / 口腔疾病防治

@#The etiology and pathogenesis of hemangiomas and vascular malformations are still unclear and face many challenges in terms of treatment. This article focuses on the etiology and genetic mechanism of common vascular tumors (such as infantile hemangiomas, congenital hemangioma and pyogenic granuloma) and vascular abnormalities (such as sporadic venous malformations, blue rubber bleb nevus syndrome, hereditary cutaneomucosal venous malformations, glomuvenous malformations, verrucous venous malformations, lymphatic malformations, and arteriovenous malformations). Some gene mutations have been identified and established. Several mutations in key proteins in the signaling pathways of endothelial cells (ECs) have been shown to play a major role in the pathogenesis of vascular abnormalities. Mutations in PIK3CA and G-protein coupled receptors were most frequently identified. The detection of genetic or somatic gene mutations is important for elucidating the underlying molecular mechanisms and developing effective therapeutic approaches.

Classification and treatment of orbital venous malformations: an updated review / 医学前沿

Orbital venous malformation (OVM) is a congenital vascular disease. As a common type of vascular malformation in the orbit, OVM may result in vision deterioration and cosmetic defect. Classification of orbital vascular malformations, especially OVMs, is carried out on the basis of different categories, such as angiogenesis, hemodynamics, and locations. Management of OVM is complicated and challenging. Treatment approaches include sclerotherapy, laser therapy, embolization, surgical resection, and radiotherapy. A satisfactory outcome can be achieved only by selecting the appropriate treatment according to lesion characteristics and following the sequential multi-method treatment strategy. This article summarizes the current classification and treatment advances in OVM.

Surgical treatment for intramuscular venous malformation combined with nerve and tendon encapsulated / 中华整形外科杂志

Objective@#This study aims (1) to evaluate the surgical outcome of intramuscular venous malformations, and associated nerve and tendon encapsulated; (2) to summarize the surgical procedure, with the purpose of providing individualized surgical treatment.@*Methods@#This is a retrospective study of 114 patients of intramuscular venous malformations with associated nerve and tendon encapsulated. All the patients were admitted in the Henan Provincial People′s Hospital from January 2012 to December 2014. The surgeries were produced, according to the patient′s condition. The surgical outcome was evaluated after 1-3 years follow up, by the overall evaluation of radiological imaging, symptoms and functions of limbs. The evaluation criteria: (1) Invalid: clinical symptoms or radiological imaging showed no improvement or even worsen; (2) Improvement: clinical symptoms or radiological imaging was significantly improved; (3) Cure: clinical symptoms were gone or the radiological imaging was normal.@*Results@#All the 114 patients were followed up for 1-3 years, with the average of 1.8 years. Three cases were evaluated as invalid, 26 cases were improved, and 85 cases were cured. The effective rate was 97%, and the cure rate was 75% on 1 year after surgery.@*Conclusions@#It is difficult to treat intramuscular venous malformations as it often involves nerve and tendon. Universal treatment is few. Therefore, an individualized surgical plan is recommendable.

Anhydrous ethanol combined with lauromacrogol in the treatment of a case of diffuse embellish venous malformation / 中华整形外科杂志

This article reported the diagnosis and treatment process of a patient with diffuse infiltrating venous malformation on right lower extremity, perineum and buttock. This is a 14 years old female patient, presented with painless dark blue mass on right perineum, buttock, thigh and leg, after birth. The right perineum lost its normal appearance and the right lower extremity was thicker than contralateral. Imaging examination suggested venous malformations of perineum, buttock and right lower extremity. Anhydrous ethanol combined with lauromacrogol was used to embolize the venous malformations by several times. The patient was satisfied with the treatment outcome, and there was recurrence within a year.

CT features of jaw venous malformations and differential diagnosis from osteosarcoma of the j aw / 实用放射学杂志

Objective To analyze the CT imaging features of jaw venous malformation (VM),and to evaluate the effect and diagnostic significance of CT in differentiating VM from osteosarcoma of the j aw.Methods 22 cases with pathological diagnosis of j aw VM and 13 cases of jaw osteosarcoma were reviewed on their CT images’morphologic characters and CT values.SPSS statistics 25.0 software package was used for statistical analysis.Results 50% of jaw VM cases showed fanGshaped bone trabecula structure,and 31.8% showed lumbricoid vessels.No invasive periosteal reaction was found in jaw VM cases.53.5% of jaw osteosarcoma cases showed invasive periosteal reactions.Neither fanGshaped bone trabecula structure nor lumbricoid vessels was found in jaw osteosarcoma cases.Significantly statistical differences existed in these morphologic characters between these two groups (P＜0.01).Minimum enhanced CT value and maximum nonGenhanced CT value were statistically different between the two groups (P＜0.05 ,U＞ 0).The AUC of minimum enhanced CT value,maximum plain CT value,and the combined predict parameter of these two values were 0.735,0.864,and 0.956,respectively.Conclusion FanGshaped bone trabecula structure and lumbricoid vessels are key features in diagnosing jaw VM on CT.Jaw VM has a lower maximum nonGenhanced CT value and a higher minimum enhanced CT value than jaw osteosarcoma has.The combined predict parameter of these two values has good diagnostic efficacy.

Clinical significance of galectin-3 expression in malformed hepatic venous tissue

Background & objectives: Hepatic venous malformation gradually develops over time and exhibits the malignant biological behaviours of being locally invasive, causing morphological and functional damage to local tissue, and may even cause systemic coagulopathy. Studies show that galectin-3(Gal-3) expression is closely associated with local invasion of malignant tumours. In this study an attempt was made to assess the clinical significance of Gal-3 in local invasion during hepatic venous malformation in patients. Methods: Gal-3 protein and its mRNA expression were examined using immunohistochemistry and in situ hybridization in a total of 126 patients with hepatic venous malformation. For control tissue, 20 cases of normal tissue distal to surgical margins were also examined. In addition, the association between Gal-3 expression and pathological parameters was analyzed in hepatic venous malformation patients. Results: Gal-3 mRNA positivity was observed in 65.08 per cent (82/126) of hepatic venous malformation tissue samples, which was higher than the rate of 20 per cent (4/20) (P <0.05) seen in control tissues. Gal-3 protein positivity was observed in 58.73 per cent (74/126) of hepatic venous malformation tissue samples, which was higher than the rate of 15 per cent (3/20) (P <0.05) seen in the normal tissue. Gal-3 expression was not significantly associated with age or gender. However, there was a significant association between Gal-3 positivity and lesion size, local invasion depth, and involvement with the hepatic vein and the portal system. Interpretation & conclusions: Local tissue invasion and destruction by hepatic venous malformation may be related to the upregulation of Gal-3. Gal-3 expression and the development of venous malformation may be related and needs to be studied further.

Telangiectasia hemorrágica hereditaria: a propósito de un caso clínico / Hereditary hemorrhagic telangiectasia: on the subject of a clinical case

La epistaxis, es un síntoma frecuente en la consulta de otorrinolaringología. Dentro de las causas posibles encontramos la telangiectasia hemorrágica hereditaria (síndrome de Rendu Osler Weber), que corresponde a un desorden autosómico dominante caracterizado por sangrados nasales y gastrointestinales asociados a malformaciones arteriovenosas sistémicas. Su manifestación más frecuente es la epistaxis, presentándose más frecuentemente en personas mayores de 40 años, sin predilección por género. Se presenta el caso de un paciente de sexo masculino de 46 años quien consulta por epistaxis a repetición y severa. Durante la hospitalización se efectúa el tratamiento convencional de la epistaxis, diagnóstico retroactivo del síndrome de Rendu Osler Weber y manejo multidisciplinario de la patología. Se realiza revisión de la literatura y discusión del manejo del paciente que cursa con esta enfermedad.

Epistaxis is a common symptom in the otorhinolaryngology consultation. Among the possible causes are hereditary hemorrhagic telangiectasia (Rendu Osler Weber syndrome), which corresponds to an autosomal dominant disorder characterized by nasal and gastrointestinal bleeding associated with systemic arteriovenous malformations. Its most frequent manifestation is epistaxis, presenting more frequently in people over 40 years of age, without gender preference. We present the case of a male patient of 46 years old who consults for recurrence and severe epistaxis. During hospitalization, conventional treatment of epistaxis, retroactive diagnosis of Rendu Osler Weber syndrome and multidisciplinary management of pathology are performed. We review the literature and discuss the management of patients with this disease.