The discriminative role of PROX-1 immunohistochemistry between venous malformation and lymphatic malformation of the deep type with no visible diagnostic surface skin lesion.

BACKGROUND: Venous malformations (VMs) are distinguished from lymphatic malformations (LMs) when specific diagnostic skin lesions are present. In the deep type, this is difficult by clinico-radiologic evaluation alone. We aimed to investigate the usefulness of lymphatic vessel endothelial cell (LEC) markers for the differential diagnosis of the deep VMs and LMs. METHODS: A retrospective study was conducted based on the medical records of patients with VMs and LMs who underwent biopsy with both D2-40 and PROX-1 immunohistochemistry. We compared the initial clinico-radiological diagnosis with the final pathological diagnosis and identified which ones showed a difference. RESULTS: From 261 patients who had VMs and LMs, 111 remained after the exclusion of those who showed definite surface diagnostic features. After pathological diagnosis with the expressions of D2-40 and PROX-1, 38 of 111 (34.2%) patients' final diagnoses were changed. Among these 38 cases, diagnosis was not changed by D2-40 positivity alone, but changed by PROX-1 positivity alone (52.6%) or by both (47.4%). The diagnostic changes were more frequent in the deep category (43.7%) than in the superficial category. CONCLUSIONS: Identifying the expression of D2-40, and especially PROX-1, in the differential diagnosis of VMs and LMs may provide important treatment guidelines and understanding their natural course.

Efficacy and safety of transarterial bleomycin sclerotherapy of early-stage facial arteriovenous malformation: Single-center multidisciplinary team experience.

BACKGROUND: To evaluate the efficacy and safety of transarterial bleomycin sclerotherapy of early-stage facial arteriovenous malformation (AVM). METHODS: A retrospective review was performed of patients who underwent bleomycin sclerotherapy for early-stage AVM (Schobinger stage I or II) in a single-referral vascular anomalies center. Bleomycin was slowly infused transarterially with flow control techniques to prolong the effects of bleomycin. Procedure details, AVM characteristics, and previous treatments were reviewed. Initial therapeutic outcomes were determined by 5 categories using both radiological and clinical findings in a 6-month follow-up. Further follow-up outcomes were reviewed to evaluate the long-term efficacy and safety of the treatment. Procedure-related complications were also analyzed. RESULTS: Nineteen patients (mean age 22.4 ± 14.0 years, 14 females) with 31 sessions of sclerotherapies were enrolled. All AVMs were Cho-Do classification type III (type IIIa [n = 13], type IIIb [n = 2], and type IIIa+b [n = 4]). Patients received a mean of 1.6 (range, 1-4) sessions of treatment. The mean cumulative bleomycin dose was 23,600 IU ± 14,500 (range, 8000 - 60,000 IU). The results showed that 14 patients (74%) were responsive to transarterial bleomycin sclerotherapy, including complete response (n = 3), marked improvement (n = 1), and partial improvement (n = 10). The remaining 5 (26%) showed no response. During a mean follow-up of 32.6 months, 5 (26%) showed slight progression compared with 6-month outcomes and 14 (74%) were stable. There were only 2 minor complications [hyperpigmentation (n = 1) and cellulitis (n = 1)]. CONCLUSIONS: Transarterial bleomycin sclerotherapy using flow control techniques can be a safe and feasible alternative treatment option for facial early-stage AVM.

Hypoxia Promotes Angiogenic Effect in Extracranial Arteriovenous Malformation Endothelial Cells.

Arteriovenous malformation (AVM) is characterized by high-flow blood vessels connecting arteries and veins without capillaries. This disease shows increased angiogenesis and a pathophysiological hypoxic environment in proximal tissues. Here, we analyzed the effects of hypoxia on angiogenesis in the endothelial cells (ECs) of AVM and normal tissues. ECs from human normal and AVM tissues were evaluated using immunocytochemistry with CD31. In vitro tube formation under hypoxia was tested in both ECs using Matrigel. The relative expression of angiogenesis-related genes was measured using real-time PCR. Under normoxia, CD31 was significantly higher in AVM ECs (79.23 ± 0.65%) than in normal ECs (74.15 ± 0.70%). Similar results were observed under hypoxia in AVM ECs (63.85 ± 1.84%) and normal ECs (60.52 ± 0.51%). In the tube formation test under normoxic and hypoxic conditions, the junction count and total vessel length were significantly greater in AVM ECs than normal ECs. Under both normoxia and hypoxia, the angiogenesis-related gene FSTL1 showed a significantly higher expression in AVM ECs than in normal ECs. Under hypoxia, CSPG4 expression was significantly lower in AVM ECs than in normal ECs. Accordingly, the angiogenic effect was increased in AVM ECs compared with that in normal ECs. These results provide a basic knowledge for an AVM treatment strategy.

Safety and Efficacy of Bleomycin Slow Infusion Sclerotherapy Using a Syringe Pump for Microcystic and Mixed Lymphatic Malformations.

PURPOSE: To evaluate the safety and efficacy of bleomycin infusion sclerotherapy using a syringe pump in microcystic and mixed (microcystic components with the presence of a cyst over 1 cm) lymphatic malformations (LMs). MATERIALS AND METHODS: Patients who received bleomycin sclerotherapy with a syringe pump for microcystic or mixed LMs were reviewed. Cystic components of LMs were accessed under sonographic guidance, followed by injection of an opacified bleomycin solution using a syringe pump (infusion rate, 10-20 mL/h) under fluoroscopic guidance. Imaging outcomes were graded as complete (> 90% size reduction), partial (25-90%), or no response (< 25%). Clinical outcomes and procedure-related complications were also reviewed. RESULTS: Forty-nine patients with 81 sclerotherapies were analyzed. The mean age was 17 years (range 0.1-65 y). Thirty-one (63%) patients had microcystic LMs, and 18 (37%) had mixed. A mean of 1.7 sessions (range 1-4) of sclerotherapy was performed using a mean cumulative dose of bleomycin of 10.8 U (range 1.5-39 U). The mean infusion time was 39 min (range 14-130 min). Regarding imaging outcomes, there was a complete response in 29% (n = 14), a partial response in 57% (n = 28), and no response in 14% (n = 7). Regarding clinical outcomes, there was a complete response in 39% (n = 19), a partial response in 51% (n = 25), and no response in 10% (n = 5). According to the CIRSE classification, no major complications were identified. CONCLUSIONS: Bleomycin slow infusion sclerotherapy provides gradual filling of sclerosant to target microcystic components. This technique is safe and feasible for the management of microcystic or mixed LMs. LEVEL OF EVIDENCE: Level 4, Case series.

A Case of Hepatic Immunoglobulin G4-Related Disease Presenting as an Inflammatory Pseudotumor and Sclerosing Cholangitis.

An inflammatory pseudotumor is a benign disease characterized by tumor-like lesions consisting of inflammatory cells including plasma cells and fibrous tissue. Recently, some inflammatory pseudotumor cases proved to be a form of Immunoglobulin G4-related disease (IgG4-RD). This novel clinical entity, recognized as a fibroinflammatory condition, is characterized by lymphoplasmacytic infiltration with a predominance of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We report a case of IgG4-RD in the form of an inflammatory pseudotumor in the liver with combined sclerosing cholangitis. We recommend that for diagnosing IgG4-RD accurately, it is important to obtain adequate tissue samples and follow-up the lesion in clinical practice.

What makes acute cholecystitis recur after removing the percutaneous cholecystostomy tube?

ABSTRACT: Percutaneous transhepatic gallbladder drainage (PTGBD) is an alternative treatment option for acute cholecystitis. However, the disease may recur after PTGBD catheter removal. This study aimed to evaluate the role of endoscopic sphincterotomy and other risk factors in reducing the recurrence of cholecystitis.We retrospectively analyzed data from 1088 patients who underwent PTGBD for cholecystitis at Kyungpook National University Hospital, Republic of Korea, between January 2011 and April 2018.A total of 115 patients were enrolled in the study. The recurrence rate of cholecystitis was 17.4% (n = 20) during a median follow-up period of 1159 (range, 369-2774) days. Endoscopic biliary sphincterotomy did not significantly affect the recurrence rate of cholecystitis (P = .561). In multivariable analysis, cystic duct stones (P = .013) and PTGBD catheter migration before the prescheduled removal time (P = .002) were identified as independent risk factors for cholecystitis recurrence after PTGBD.To reduce post-PTGBD recurrence in cholecystitis, caution must be exercised to avoid inadvertent dislodging of the PTGBD catheter. In cases of cholecystitis with cystic duct stones, cholecystectomy should be considered only after careful assessment of postoperative risks. Instead, transluminal endoscopic gallbladder drainage could represent a promising option for the prevention of recurrent cholecystitis.

Wall shear stress on vascular smooth muscle cells exerts angiogenic effects on extracranial arteriovenous malformations.

BACKGROUND: In addition to vascular endothelial cells, vascular smooth muscle cells (VSMCs) are subject to continuous shear stress because of blood circulation. The angiogenic properties of VSMCs in extracranial arteriovenous malformations (AVMs) may exceed those of normal blood vessels if the body responds more sensitively to mechanical stimuli. This study was performed to investigate the hypothesis that rapid angiogenesis may be achieved by mechanical shear stress. METHODS: VSMCs were obtained from six patients who had AVMs and six normal controls. The target genes were set to angiopoietin-2 (AGP2), aquaporin-1 (AQP1), and transforming growth factor-beta receptor 1 (TGFBR1). Reverse-transcriptase polymerase chain reaction (RT-PCR) and real-time PCR were implemented to identify the expression levels for target genes. Immunofluorescence was also conducted. RESULTS: Under the shear stress condition, mean relative quantity values of AGP2, AQP1, and TGFBR1 in AVM tissues were 1.927±0.528, 1.291±0.031, and 2.284±1.461 when compared with neutral conditions. The expression levels of all three genes in AVMs were higher than those in normal tissue except for AQP1 under shear stress conditions. Immunofluorescence also revealed increased staining of shear stress-induced genes in the normal tissue and in AVM tissue. CONCLUSIONS: Shear stress made the VSMCs of AVMs more sensitive. Although the pathogenesis of AVMs remains unclear, our study showed that biomechanical stimulation imposed by shear stress may aggravate angiogenesis in AVMs.

Laboratory and radiological discrimination between tuberculous and malignant pleural effusions with high adenosine deaminase levels.

BACKGROUND/AIMS: Pleural fluid adenosine deaminase (ADA) levels are useful in discriminating tuberculous pleural effusions (TPEs) from malignant pleural effusions (MPEs). However, some patients with MPE exhibit high-ADA levels, which may mimic TPEs. There is limited data regarding the differential diagnosis between high-ADA MPE and high-ADA TPE. This study aimed to identify the predictors for distinguishing high-ADA MPEs from high-ADA TPEs. METHODS: Patients with TPE and MPE with pleural fluid ADA levels ≥ 40 IU/L were included in this study. Clinical, laboratory, and radiological data were compared between the two groups. Independent predictors and their diagnostic performance for high-ADA MPEs were evaluated using multivariate logistic regression analysis and receiver operating characteristic curve. RESULTS: A total of 200 patients (high-ADA MPE, n = 30, and high-ADA TPE, n = 170) were retrospectively included. In the multivariate analysis, pleural fluid ADA, pleural fluid carcinoembryonic antigen (CEA), and pleural nodularity were independent discriminators between high-ADA MPE and high-ADA TPE groups. Using pleural ADA level of 40 to 56 IU/L (3 points), pleural CEA level ≥ 6 ng/mL (6 points), and presence of pleural nodularity (3 points) for predicting high-ADA MPEs, a sum score ≥ 6 points yielded a sensitivity of 90%, specificity of 96%, positive predictive value of 82%, negative predictive value of 98%, and area under the receiver operating characteristic curve of 0.965. CONCLUSION: A scoring system using three parameters may be helpful in guiding the differential diagnosis between high-ADA MPEs and high-ADA TPEs.

The effects of surgical treatment and sclerotherapy for intramuscular venous malformations: a comparative clinical study.

BACKGROUND: Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision. METHODS: Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement. RESULTS: Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group. CONCLUSIONS: Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.

Clinical assessment and management of auricular arteriovenous malformation: retrospective study.

BACKGROUND: Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. METHODS: Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. RESULTS: Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. CONCLUSION: Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

Case report of juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome: first report in Korea with a novel mutation in the SMAD4 gene.

Juvenile polyposis/hereditary hemorrhagic telangiectasia (JPS/HHT) syndrome is a rare, autosomal dominant disorder caused by mutations in the SMAD4 gene, presenting with features of both juvenile polyposis syndrome (JPS) and HHT. Reports and studies of JPS/HHT syndrome are mostly from Western countries, while there are scarce reports from East Asian countries. We report a case of a Korean boy who had been previously diagnosed with JPS at 7 years and had first visited to our center at 15 years of age. Genetic studies of the patient and parents revealed a novel variant in the SMAD4 gene, SMAD4 c.1146_1163del; p.His382_Val387del (NM_005359.5), which had developed de novo. Numerous pedunculated and sessile polyps were observed throughout the gastrointestinal (GI) tract. Mucocutaneous telangiectases were observed on the lips, tongue, and jejunum, and arteriovenous malformations (AVMs) were observed in both lungs. This is the first case report of JPS/HHT syndrome in Korea, with a novel deletion variant in the SMAD4 gene. Patients with JPS should undergo genetic evaluation of associated genes including SMAD4, and those with genetically confirmed SMAD4 variants should undergo further evaluation for coexisting asymptomatic AVMs in order to prevent life-threatening complications of thrombotic emboli and pulmonary hemorrhage.

Oscillatory shear stress promotes angiogenic effects in arteriovenous malformations endothelial cells.

BACKGROUND: Vascular endothelial cells (ECs) are subject to continuous shear stress due to blood circulation. Mechanical stress due to high shear flow can also cause arteriovenous malformation (AVM) when ECs respond hyper-sensitively to shear flow. This study was conducted to test the hypothesis that angiogenesis could be promoted in response to mechanical stress via regulation of pro-angiogenic factors in AVM cells. METHODS: ECs were extracted from the tissue samples from six AVM patients and six normal patients. Shear stress at 7 dynes/cm2 were applied for 24 h. Before and after application of shear stress to each group, RT-PCR was performed to access the expression levels of angiopoietin2(AGP2), aquaporin1(AQP1) and TGFßR1. Immunofluorescences was also performed to evaluate the level of protein expressions. RESULTS: In both normal and AVM tissues, AGP2 and TGFßR1 under the shear stress showed increased expression in the ECs compared to the non-sheared samples. When AVMs and normal arterial vasculature were compared, the expression levels of both AGP2 and TGFßR1 in AVMs were higher when compared to normal arterial vasculature with or without shear stress. Immunofluorescence-based protein analysis also confirmed shear-induced AGP2 and TGFßR1 in both samples of normal and AVM patients. CONCLUSIONS: AVMs exhibited higher sensitivity to shear stress by producing higher expressions of some marked genes and proteins that regulate the endothelial functions upon exposure to shear stress. While the physiological mechanism for AVMs remain elusive, our study shows the plausibility of physical stress imposed by the shearing flow can cause the occurrence of AVMs.

Unusual Presentation of Anal Pain and Tenesmus from Rectal Arteriovenous Malformation Successfully Treated with Ethanol Sclerotherapy.

Gastrointestinal arteriovenous malformation (AVM) is reported as one of the possible causes of intestinal bleeding, and its occurrence in the rectum is rare. We report the case of a rectal AVM patient who experienced uncommon symptoms of anal pain and tenesmus and was treated successfully with percutaneous transarterial ethanol sclerotherapy. The patient underwent routine colonoscopy with biopsy at the time of visit; however, an accurate diagnosis was difficult. Subsequent contrast-enhanced computed tomography (CT) and angiography revealed a rectal AVM emerging from the distal inferior mesenteric artery with engorged superior rectal veins. The feeding artery was catheterized, and concurrent transarterial sclerotherapy with 80% ethanol was performed. There was no major complication related to the procedure. Disappearance of AVM nidus and improvement of associated venous congestion were shown by follow-up CT. There was no recurrence of symptoms after 10 months of clinical observation. Transarterial ethanol sclerotherapy is safe and effective in treating rectal AVM and can be considered as one of the nonsurgical treatment options.

Percutaneous portal vein embolization for intractable hepatic biloma after radiofrequency ablation.

Intrahepatic biloma is a rare complication of radiofrequency ablation (RFA). When present, it can only be conservatively managed. Here, we report the case of a patient with hepatocellular carcinoma complicated by an intractable biloma after RFA. The biloma could not be managed via percutaneous catheter drainage and antibiotic administration for over 3 months. The intractable biloma was finally cured using percutaneous transhepatic portal vein embolization. The present case demonstrates a useful treatment option for intractable biloma after RFA.

Combined treatment of surgery and sclerotherapy for sinus pericranii.

Sinus pericranii is a rare vascular anomaly characterized by abnormal venous communication between the inner and outer regions of the cranial cavity. Here, we report a case of sinus pericranii and venous malformations in the right periorbital region of a 2-year-old girl. Radiologic findings showed venous malformations in the right parietal region communicating with the superior sagittal sinus in the intracranial region. There were notable improvements following surgical resection for the abnormal venous lesions and several sclerotherapies. Presence of a bluish and pulsating mass on the scalp, which showed bruit on auscultation, may indicate sinus pericranii, which should be included in the differential diagnosis.

Idiopathic Pleural Effusions: Characteristics and Discrimination From Cytology-Negative Malignant Pleural Effusions.

BACKGROUND: The etiology of pleural effusions often remained unknown notwithstanding surgical pleural biopsy and further clinical observation. A better understanding of clinical characteristics of patients with idiopathic pleural effusion (IPE) may improve the ability to differentiate between IPEs and cytology-negative malignant pleural effusions (MPEs) and facilitate the identification of patients requiring invasive investigation. However, little is known about the clinical factors that can help distinguish patients with IPE from those with cytology-negative MPE. MATERIALS AND METHODS: Patients who were diagnosed with IPE or cytology-negative MPE between 2010 and 2017 were enrolled in this retrospective study. Clinical, laboratory and radiologic characteristics were compared between patients with IPE and cytology-negative MPE. Diagnostic performances of predictors for IPE were assessed using receiver operating characteristic curves. RESULTS: Of 146 patients undergoing pleural biopsy owing to cytology-negative pleural effusion of uncertain cause, MPE was confirmed in 54 patients. IPE was ultimately diagnosed in 22 patients. Multivariate analysis demonstrated that a minimal amount of pleural effusion (odds ratio [OR] = 12.41, P = 0.039), presence of pleural nodularity (OR = 0.01, P < 0.001) and pleural fluid carcinoembryonic antigen levels less than 14 ng/mL (OR = 87.59, P = 0.002) were independent factors for distinguishing IPEs from cytology-negative MPEs. A combination of the absence of pleural nodularity with pleural fluid carcinoembryonic antigen levels less than 14 ng/mL yielded an area under the curve of 0.94 (sensitivity = 91% and specificity = 96%). CONCLUSIONS: Using these readily available parameters to identify IPE in patients with cytology-negative exudative effusion of unknown cause can help guide decision-making when choosing to perform an invasive pleural biopsy or to take a conservative approach.

Characteristics and survival impact of polymorphonuclear leucocyte-predominant malignant pleural effusions secondary to lung cancer.

INTRODUCTION: In comparison with mononuclear leucocyte (MNL)-predominant malignant pleural effusions (MPEs), polymorphonuclear leucocyte (PMNL)-predominant MPEs have rarely been investigated and may be associated with a poorer prognosis. OBJECTIVES: To investigate the characteristics and survival impact of PMNL-predominant MPEs secondary to lung cancer. METHODS: This retrospective study included patients with MPE secondary to lung cancer, which were classified into the PMNL- and MNL-predominant groups according to cellular predominance in the pleural fluid. Clinical, hematological, radiological, and pleural fluid data were compared between the groups, and the survival impact of PMNL predominance in MPE was evaluated. RESULTS: Of the 193 MPEs included, 37 (19%) were characterised by PMNL predominance. Compared to the MNL-predominant group, the PMNL-predominant group showed significantly poorer patient performances (P = .001), higher white blood cell counts (P = .009), higher neutrophil counts, higher blood neutrophil-to-lymphocyte ratio (P = .046), higher serum C-reactive protein (P = .003), lower serum albumin (P < .001), lower pleural fluid pH (P = .002) and higher pleural fluid lactate dehydrogenase (P = .029) levels. In contrast, most clinical and radiological findings, including the duration of symptoms, showed no significant intergroup differences. A shift towards MNL predominance was observed in only 38% of the PMNL-predominant patients who underwent repeat thoracentesis. Overall survival of the PMNL-predominant group was significantly shorter than the MNL-predominant group (P = .003). CONCLUSIONS: PMNL predominance in MPEs secondary to lung cancer may be observed in variable phases with respect to the duration of symptoms and the time of thoracentesis. Overall, PMNL-predominant MPEs were associated with more advanced stages and poorer survival outcomes, compared to MNL-predominant MPEs.

Palliative Measures with Ethanol Gallbladder Ablation and Endobiliary Radiofrequency Ablation Followed by Endoscopic Biliary Stent Placement in an Advanced Case of Common Bile Duct Cancer: A Case Report.

Endobiliary radiofrequency ablation (RFA) is a procedure performed widely to induce locoregional tumor control by the transfer of thermal energy to the lesion and subsequent tumor necrosis. A 72-year-old male with a prior history of acute calculous cholangitis and perforated cholecystitis was admitted to the Kyungpook National University Hospital complaining of fever and nausea. He had an indwelling percutaneous transhepatic gallbladder drainage (PTGBD) catheter from the previous episode of perforated cholecystitis. An abdominal CT scan showed marked dilation of both the intrahepatic and extrahepatic bile ducts. Common bile duct cancer was confirmed histologically after an endobiliary biopsy. A surgical resection was considered to be the initial treatment option. During open surgery, multiple metastatic nodules were present in the small bowel mesentery and anterior abdominal wall. Resection of the tumor was not feasible, so endobiliary RFA was performed prior to biliary stenting. Cholecystectomy was required for the removal of the PTGBD catheter, but the surgical procedure could not be performed due to a cystic ductal invasion of the tumor. Instead, chemical ablation of the gallbladder (GB) with pure ethanol was performed to breakdown the GB mucosa. Palliative treatment for a biliary obstruction was achieved successfully using these procedures. In addition, a PTGBD catheter was removed successfully without significant side effects. As a result, an improvement in the patient's quality of life was accomplished.

Surgical approach for venous malformation in the head and neck.

BACKGROUND: Treatment for venous malformations of the head and neck includes sclerotherapy, surgical resection, or a combination of both. Surgical resection can remove or reduce the volume of vascular lesions; however, surgery can cause postoperative scarring and potential surgical complications. This study sought to determine the effectiveness of surgery for the treatment of venous malformations of the head and neck. METHODS: A retrospective review of the medical records of patients who received surgeries for venous malformations of the head and neck from January 2011 to July 2019 was performed. Using clinical photographs, preoperative and postoperative Doppler ultrasonography, outpatient clinic records, and operation records, the postoperative result and complications were evaluated for each case. RESULTS: Among patients who visited our vascular anomalies clinic, 43 patients (ratio of male to female= 24:19) received surgeries for venous malformations of the head and neck. Twenty-nine patients had undergone surgery only, five patients received sclerotherapy after surgery, and nine patients received surgery after preoperative sclerotherapy. In postoperative evaluations, the result was excellent in 24 patients, good in 18 patients, and poor in one patient. Four patients experienced a recurrence of lesions with lagophthalmos, drooping of the corner of the mouth, partial wound necrosis, and scar widening found in one patient each. CONCLUSION: Because the head and neck region is the most exposed area in the body, more active implementation of surgical treatments with or without sclerotherapy is essential to reduce the functional and cosmetic impairments associated with venous malformations.

Thoracic aortic aneurysms exerting high extrinsic pressure on the airway.

BACKGROUND: Thoracic aortic aneurysms, although mostly asymptomatic, are life threatening owing to the risk of rupture. Moreover, the extrinsic pressure of a ruptured aneurysm may encroach the mediastinum. CASE PRESENTATION: A 74-year-old woman diagnosed with ruptured descending thoracic aortic aneurysm compressing the lower trachea and both main bronchi underwent thoracic endovascular aortic repair; however, the extrinsic pressure on the airway persisted. Following failing of endobronchial silicon stents insertion, extracorporeal membrane oxygenation support was required, and endobronchial metallic stents were inserted. The patients' hypoventilation resolved, and the patient was withdrawn from the ventilator. CONCLUSIONS: Technological improvement in endovascular or endobronchial procedures has provided more options for managing complex cases. However, we must be aware of how high the extrinsic pressure might be before management and take steps to minimize complications.