Orbital varices: Epidemiology, clinical presentation, and treatment outcomes - A scoping review.

Background: Orbital varices are vein dilations in the orbit presenting various symptoms. This scoping review synthesizes existing evidence on their epidemiology, clinical features, and treatment efficacy. Methods: Literature was reviewed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. PubMed and Scopus were searched until April 31, 2024, for articles on clinically diagnosed ocular varices detailing diagnostic methods, treatments, and outcomes. Exclusions were reviews, animal studies, and incomplete case reports. Data on study characteristics, diagnosis, management, and outcomes were extracted and assessed for quality and bias. Results: Eight studies met the inclusion criteria, with sample sizes ranging from 4 to 30 and ages from 1 to 87 years. Diagnostic tools included magnetic resonance imaging and computed tomography, while treatments ranged from conservative methods to invasive procedures and radiosurgery. Notably, higher symptom resolution rates were associated with observational strategies and minimally invasive surgeries. However, Gamma Knife radiosurgery, although promising, posed risks of vision impairment in some cases. Conclusion: The management of orbital varices has evolved significantly with newer, less invasive techniques improving outcomes and reducing recovery times. Despite advancements, challenges such as disease recurrence and the need for personalized treatment regimens persist, underscoring the ongoing need for research to refine and standardize treatment approaches.

Different Vortex Vein Anomalies Observed in a Single Case: Macular Vortex Vein in One Eye and Varix of Vortex Vein Ampulla in the Other Eye.

We present a 56-year-old female with a macular vortex vein in her right eye and a varix of vortex vein ampulla in the inferior nasal fundus of her left eye. The choroidal lesions were evaluated by multimodal imaging including fundoscopy with contact lens, ultra-widefield fundus photography, swept-souse optical coherence tomography (SS-OCT), enface image of widefield optical coherence tomography (widefield enface-OCT), and ultra-widefield fundus angiography. Widefield enface-OCT revealed submacular large choroidal vessels in the right eye. Ultra-widefield indocyanine green fluorescence angiography (UWICGA) of the right eye showed the dye of those submacular choroidal vessels drained from the ampullae beneath the macula. Fundoscopy revealed an elevated lesion with crescent shadows in the inferior nasal fundus of the left eye. Dynamic fundoscopy with compression of the left eye resulted in a diminishing of the elevation and release of the compression resulted in an enlargement of the elevation. Ultra-widefield fundus photography of the left eye in the right inferior gaze revealed an elevated lesion with a crescent shadow in the inferior nasal fundus, while it is not prominent in the primary gaze. The B-scan of SS-OCT revealed a hyporeflective lesion in the choroid beneath the elevated lesion of the left eye. The elevated lesion was consistent with the vortex vein ampulla on UWICGA. This is the first case where two different choroidal vascular anomalies, macular vortex vein and varix of vortex ampulla, coexist in a single patient. Multimodal imaging is useful to visualize and diagnose choroidal vascular anomalies.

Vascular loops mimicking herniated lumbar discs: fair warning.

The authors report their experience with twenty-one consecutive patients who presented with symptoms and imaging characteristics of a herniated lumbar disc; of whom, at the time of surgery had a vascular loop instead. The procedure was performed on 14 women and seven men with a mean age of 39 years. Clinical complaints included lumbar aching with one limb overt radiculopathy in all patients; with additional sphincter dysfunction in two cases. Symptoms had developed within a mean period of three months. In all patients, the disc was exposed through an L5-S1 (n = 10); L4-L5 (n = 5) and L3-L4 (n = 6) open minimal laminotomy. In 16 patients, rather than a herniated disc they had a lumbar epidural varix, while an arterio-venous fistula was found in the remaining five cases. In all cases, the vascular disorder was resected and its subjacent disc was left intact. One patient had a postoperative blood transfusion. While the radiculopathy dysfunction improved in all patients, four patients reported lasting lumbar pain following surgery. The postoperative imaging confirmed the resolution of the vascular anomaly and an intact disc. The mean length of the follow-up period was 47 months. Either epidural varix or arterio-venous fistula in the lumbar area may mimic a herniated disc on imaging studies. With the usual technique they can be operated safely. Resection of the anomaly can be sufficient for alleviating radiculopathy symptoms.

Embolization with or without portal vein stenting for bleeding ectopic jejunal varices in hepatopetal portal collaterals due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery.

PURPOSE: To evaluate the efficacy and safety of embolization with or without portal vein stenting for bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery. MATERIALS AND METHODS: This study included consecutive patients who underwent embolization for bleeding ectopic jejunal varices in the hepatopetal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery between September 2012 and December 2020. The safety, technical and clinical success rates (no re-bleeding within 1 month) and re-bleeding-free survival after the first therapy and overall survival were assessed. RESULTS: Fourteen sessions in 11 patients were included. Four patients (7 sessions) underwent variceal embolization only, and the remaining seven patients (7 sessions) underwent portal vein stenting and variceal embolization. Technical success was achieved in all 14 sessions (100%). Clinical success was achieved in 13 of 14 sessions (92.9%). No treatment-related serious complications including liver failure were observed. One-year and 2-year re-bleeding-free survival rate after the first endovascular therapy in all 11 patients was 90.9 and 60.6%, respectively. Two patients who experienced re-bleeding had repeat embolization treatment. There was no significant difference in re-bleeding-free survival after endovascular therapy between the combination with stenting and embolization group and the embolization-only group (p = 0.13). CONCLUSION: Embolization with or without portal vein stenting of bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery can be considered a safe, effective, and repeatable therapy for long-term hemostasis of uncontrollable bleeding.

N-Butyl-cyanoacrylate-assisted retrograde transvenous obliteration (NARTO) for gastric varices.

We describe the usefulness of n-butyl-cyanoacrylate (nBCA)-assisted retrograde transvenous obliteration (NARTO) for gastric varices in 3 consecutive patients. In all patients, balloon catheters were inserted into the gastrorenal shunt via the left renal vein. After injecting sclerosant into the gastric varix under balloon occlusion, nBCA was injected to the proximal side of the shunt, to completely embolize the shunt. NARTO is a simple technique to achieve stagnation of the injected sclerosant in gastric varices and to occlude a gastrorenal shunt. This procedure is also cost-effective, and may improve procedure time compared with original or modified balloon-occluded retrograde transvenous obliteration.

A Case of Idiopathic Portal Hypertension Diagnosed by Noninvasive Fibrosis Evaluation Using Elastography.

Idiopathic portal hypertension (IPH) is often misdiagnosed as liver cirrhosis. Because it is difficult to distinguish between the two using diagnostic imaging, invasive tests, such as pathology and hepatic vein pressure gradient measurement, are necessary to make a diagnosis. Several studies have shown that the measurement of liver and spleen stiffnesses using elastography is useful in the diagnosis of IPH; however, there are few concrete reports on this subject. Herein, we report the case of a 58-year-old woman with IPH in which elastography was helpful for the diagnosis.

Spontaneous echo contrast and decreased umbilical vein blood flow may predict thrombus formation in fetal intra-abdominal umbilical vein varix.

PURPOSE: Fetal intra-abdominal umbilical vein varix (FIUVV) can cause thrombosis, fetal growth restriction (FGR), and intrauterine fetal death (IUFD). However, its management and evaluation to avoid fetal risks have not been elucidated. The aim of this study was to develop a novel method to evaluate fetal risks, including FGR and fetal dysfunction via frequent ultrasound examinations. METHODS: A 28-year-old pregnant woman was diagnosed with FIUVV via ultrasound at 26 weeks of gestation and admitted to our hospital. Ultrasound examinations were performed two to three times weekly to evaluate size and shape of the FIUVV and umbilical vein blood flow at the inflow and outflow sites of the FIUVV. RESULTS: The outflow site of the FIUVV was constricted and collapsed, and the blood flow velocity at the inflow site of the FIUVV was decreased. At 32 weeks of gestation, spontaneous echo contrast (SEC), which indicates increased echogenicity, appeared. At 35 weeks of gestation, the patient noticed decreased fetal movement, and CTG showed non-reassuring fetal status. SEC in the FIUVV was remarkable. Fetal movement could not be confirmed at ultrasound. Cesarean section was performed and a 1,854-g healthy infant was delivered with an umbilical cord arterial pH of 7.266. CONCLUSION: The echographic changes, such as decreased umbilical vein blood flow and SEC, in FIUVV observed in this case could indicate thrombus formation, which can lead to fetal dysfunction. Frequent ultrasound examinations can help determine the timing of delivery and improve the neonatal prognosis.

Role of Portosystemic Shunt and Portal Vein Stent in Managing Portal Hypertension Due to Hematological Diseases.

INTRODUCTION: Patients with hematological diseases experience complications related to portal hypertension, including life-threatening complications such as variceal bleeding. METHODS: We analyzed the prognosis of patients with hematological diseases and portal hypertension treated with transjugular intrahepatic portosystemic shunts (TIPS) or portal vein stents. We retrospectively assessed patients with hematological diseases and portal hypertension who had variceal bleeding. We evaluated the characteristics and prognosis of the enrolled patients. A total of 11 patients with hematological diseases who underwent TIPS, or portal vein stenting, were evaluated. RESULTS: The median follow-up period was 420 days. Of the 11 patients, eight showed resolution of portal hypertension and its complications following TIPS, or stent insertion. One patient experienced rebleeding due to incomplete resolution of portal hypertension, and two other patients also experienced rebleeding because they underwent TIPS closure or revision due to repetitive hepatic encephalopathy. CONCLUSION: Portosystemic shunt and stent installation are effective treatment options for portal hypertension due to hematological diseases.

Uterine vascular abnormalities linked to pregnancy complications: color and power Doppler-assisted transvaginal ultrasound evaluation.

Aim: Abnormal uterine vascular pattern can be observed during transvaginal ultrasound examination used for investigating post-abortion bleeding and secondary postpartum hemorrhage. The purpose of this series of cases was to evaluate almost all the rare causes of uterine vascular abnormalities linked to pregnancy complications, and determine how to arrive at the diagnosis to optimize patient management, which is crucial for preventing life-threatening massive vaginal bleeding. Material and methods: Retrospective observational case series study including 20 women with postpartum or post-abortion vaginal bleeding who were found to have an abnormal uterine vascular pattern during a transvaginal color duplex assessment. Results: The study yielded the following findings: 10 cases of enhanced myometrial vascularity, two cases of pseudoaneurysm in the uterine artery, one case of myometrial venous varix, one case of large uterine venous pseudoaneurysm, one case of uterine arteriovenous malformation, one case of retained placental polyp, one case of invasive vesicular mole, and three cases of subinvolution of the placental implantation site. Conclusions: Transvaginal color duplex ultrasound plays a crucial role in detecting uterine vascular abnormalities as a cause of post-abortion or secondary postpartum hemorrhage and can help differentiate the pathologies responsible for the abnormal vascular pattern, which is highly recommended to optimize patient management.

Downhill Varices and Apical Lung Cancer Without Superior Vena Cava Syndrome.

A 75-year-old woman with hypertension, dyslipidemia, thalassemia trait, osteoarthritis of the knees, and hyperthyroidism presented with bloating, abdominal discomfort, and change in stool caliber underwent an esophagogastroduodenoscopy. The esophagogastroduodenoscopy revealed varices at the middle of esophagus, which were diagnosed to be downhill esophageal varices (DEVs). DEVs occur mainly from the superior vena cava (SVC) obstruction; however, in the absence of obstruction, angiogenesis and thrombosis could be the etiology. In our patient, computed tomography showed a pulmonary nodule located at periphery of the right upper lung without SVC contact nor obstruction. The nodule was later proven to be an adenocarcinoma. Thus, our patient showed the possibility that adenocarcinoma of the lung might associated with the DEV through a mechanism other than SVC obstruction. Recognition and differentiation of DEV from other benign venous blebs of the esophagus are important, and once detected, further investigation with computed tomography of the chest is recommended.

Balloon-occlusion Retrograde Transvenous Obliteration Using Gadoteridol As an Alternative Contrast Agent in a Patient with Iodine Allergy.

A 70-year-old woman with liver cirrhosis presented with gastric varices and recurrent hepatic encephalopathy. Magnetic resonance imaging (MRI) showed a splenorenal shunt, and balloon-occluded retrograde transvenous obliteration (B-RTO) was indicated but could not be performed due to iodine allergy. We then performed B-RTO using gadoteridol, an MRI contrast medium, instead of iodine contrast and successfully occluded the shunt vessel. After the procedure, hepatic encephalopathy did not recur, and the size of the gastric varices was reduced. This experience may aid in the management of iodine-allergic patients requiring interventional radiological treatment.

Plug-Assisted Retrograde Transvenous Obliteration for the Treatment of Duodenal Variceal Bleeding - A Case Report and Literature Review.

Duodenal varices are uncommon complications of portal hypertension. Although duodenal variceal bleeding is infrequent, it is a life-threatening condition with a high mortality rate. Non-surgical methods for duodenal variceal bleeding include endoscopic band ligation, endoscopic sclerotherapy, transjugular intrahepatic portosystemic shunt, and retrograde transvenous obliteration. On the other hand, an optimal treatment strategy for this rare condition has not been established. A 38-year-old male with a history of alcoholic liver cirrhosis presented with hematemesis. An emergency esophagogastroduodenoscopy (EGD) revealed large, multiple varices in the second portion of the duodenum, and plug-assisted retrograde transvenous obliteration (PARTO) was performed accordingly. Gastrointestinal bleeding was resolved after the procedure. Follow-up EGD conducted after 11 weeks revealed complete obliteration of the previously observed duodenal varices. PARTO may be considered a viable option for treating duodenal variceal bleeding.

[Risk factors and construction of a nomogram model for cirrhotic portal vein thrombosis combined with esophagogastric variceal bleeding].

Objective: To investigate the risk factors and construct a nomogram model for predicting the occurrence of cirrhotic portal vein thrombosis in patients combined with esophagogastric variceal bleeding (EVB). Methods: Clinical data on 416 cirrhotic PVT cases was collected from the First Hospital of Lanzhou University between January 2016 and January 2022. A total of 385 cases were included after excluding 31 cases for retrospective analysis. They were divided into an esophagogastric variceal bleeding group and a non-esophagogastric variceal bleeding group based on the clinical diagnosis. The esophagogastric variceal group was then further divided into an EVB group and a non-bleeding group. All patients underwent gastroscopy, serology, and imaging examinations. The risk factors of PVT combined with EVB were identified by univariate analysis using SPSS 26. The prediction model of cirrhotic PVT in patients combined with EVB was constructed by R 4.0.4. The prediction efficiency and clinical benefits of the model were evaluated by the C-index, area under the receiver operating characteristic curve, calibration plots, and decision curve. The measurement data were examined by a t-test or Mann-Whitney U test. The counting data were tested using the χ(2) test or the Fisher exact probability method. Results: There were statistically significant differences in the etiology, Child-Pugh grade,erythrocyte count, hematocrit, globulin, and serum lipids between the esophageal and non-esophageal varices groups (P < 0.05). There were statistically significant differences in etiology, erythrocyte count, hemoglobin, hematocrit, neutrophil percentage, total protein, globulin, albumin/globulin, urea, high-density lipoprotein cholesterol, calcium, and neutrophil lymphocyte ratio (NLR) between the EVB and non-bleeding groups (P < 0.05). Multivariate logistic regression analysis showed that etiology (OR = 3.287, 95% CI: 1.497 ~ 7.214), hematocrit (OR = 0.897, 95% CI: 0.853 ~ 0.943), and high-density lipoprotein cholesterol (OR = 0.229, 95% CI: 0.071 ~ 0.737) were independent risk factors for cirrhotic PVT patients combined with EVB. The constructed normogram model predicted the probability of bleeding in patients. The nomogram model had shown good consistency and differentiation (AUC = 0.820, 95% CI: 0.707 ~ 0.843), as verified by 10-fold cross-validation (C-index = 0.799) and the Hosmer-Lemeshow goodness of fit test (P = 0.915). The calibration plot and the decision curve suggested that the prediction model had good stability and clinical practicability. Conclusion: The risk factors for EVB occurrence include etiology, erythrocyte, hemoglobin, hematocrit, percentage of neutrophils, total protein, globulin, albumin/globulin, urea, high-density lipoprotein cholesterol, calcium, and NLR in patients with cirrhotic liver. The constructed prediction model has good predictive value, and it can provide a reference for medical personnel to screen patients with high bleeding risk for targeted treatment.

Vallecular Varix: An Uncommon Cause for a Common Clinical Scenario.

Vallecular varix can be a rare, potentially life-threatening source for Upper Gastrointestinal Bleeding. It is often a diagnostic dilemma and can present as persistent Hematemesis, Haemoptysis, or Malena. We describe a case of Vallecular varix, highlighting the presentation, diagnosis, management, along with the post-operative follow-up.

Intermittent Orbital Pain due to Hemodynamic Collapse of an Orbital Varix: A Case Report.

Orbital varices typically present with symptoms related to dilation or thrombosis. We describe a rare presentation of an orbital varix with pain caused by hemodynamic collapse of the varix. A woman in the third decade presented with position-dependent orbital pain and enophthalmos. She was found to have an intraorbital varix and a separate pterygoid varix. The patient underwent endovascular treatment of the pterygoid varix using coils and sclerosing agents which altered the venous outflow from the orbital varix. The patient had immediate resolution of symptoms after the procedure. Our findings suggest that extraorbital venous outflow abnormalities may be the cause of symptoms in selected cases of orbital varices. By understanding the venous structures on cerebral angiography and treating the extraorbital component, orbital intervention may be avoided, reducing the risk of complications.

A model for predicting poor survival in patients with cirrhosis undergoing portosystemic shunt embolization.

BACKGROUND AND AIM: Portosystemic shunt embolization (PSSE) is a promising treatment for hepatic encephalopathy (HEP) and gastric varix (GV) in cirrhotic patients with a spontaneous portosystemic shunt. However, PSSE may worsen portal hypertension causing hepatorenal syndrome, liver failure, and mortality. This study aimed to develop and validate a prognostic model that helps identify patients with a risk of poor short-term survival after PSSE. METHODS: We included 188 patients who underwent PSSE for recurrent HEP or GV at a tertiary center in Korea. To develop a prediction model for 6-month survival after PSSE, Cox proportional-hazard model was used. The developed model was validated in a separate cohort of 184 patients from two other tertiary centers. RESULTS: In multivariable analysis, the 1-year overall survival after PSSE was significantly associated with baseline levels of serum albumin, total bilirubin, and international normalized ratio (INR). We therefore developed the albumin-bilirubin-INR (ABI) score by assigning 1 point each for albumin < 3.0 g/dL, total bilirubin ≥ 1.5 mg/dL, and INR ≥ 1.5. Time-dependent areas under the curve of the ABI score for predicting 3-month and 6-month survival were 0.85 and 0.85 in the development cohort and 0.83 and 0.78 in the validation cohort, indicating good discrimination performance. The ABI score showed a better discrimination and calibration performance than the model for end-stage liver disease and the Child-Pugh scores, especially in high-risk patients. CONCLUSIONS: The ABI score is a simple prognostic model that helps decide whether to proceed with PSSE for the prevention of HEP or GV bleeding in patients with spontaneous portosystemic shunt.

Thrombosed orbital varix of the inferior ophthalmic vein: A rare cause of acute unilateral proptosis.

Background: Orbital varices are rare, accounting for only 0-1.3% of orbital masses. They can be found incidentally or cause mild to serious sequelae, including hemorrhage and optic nerve compression. Case Description: We report a case of a 74-year-old male with progressively painful unilateral proptosis. Imaging revealed the presence of an orbital mass compatible with a thrombosed orbital varix of the inferior ophthalmic vein in the left inferior intraconal space. The patient was medically managed. On a follow-up outpatient clinic visit, he demonstrated remarkable clinical recovery and denied experiencing any symptoms. Follow-up computed tomography scan showed a stable mass with decreased proptosis in the left orbit consistent with the previously diagnosed orbital varix. One-year follow-up orbital magnetic resonance imaging without contrast showed slight increase in the intraconal mass. Conclusion: An orbital varix may present with mild to severe symptoms and management, depending on case severity, ranges from medical treatment to escalated surgical innervation. Our case is one of few progressive unilateral proptosis caused by a thrombosed varix of the inferior ophthalmic vein described in the literature. We encourage further investigation into the causes and epidemiology of orbital varices.

Acute unilateral orbital varix thrombosis in preexisting bilateral orbital varices: illustrative case.

OBJECTIVE: Orbital varix is a rare distensible orbital venous malformation. Most patients present with unilateral intermittent periorbital pain and positional proptosis that is exacerbated by the Valsalva maneuver. Complications include hemorrhage and thrombosis, leading to sudden painful proptosis and visual disturbance. OBSERVATIONS: A 42-year-old female with a history of bilateral intermittent painless proptosis that was accentuated by a postural head-down position presented with acute painful proptosis in her right eye. Ophthalmic examination revealed right eye proptosis with a bluish mass at the right upper eyelid, and another bluish mass at the left lower eyelid that was prominent during the Valsalva maneuver. Computed tomography scans of the orbits indicated right orbital varix thrombosis and left orbital varix. Surgical excision of the right thrombosed varix was performed along with intralesional bleomycin injection in the left orbital varix. Histopathological examination confirmed the right thrombosed varix diagnosis. The patient had good clinical improvement at the 6-month follow-up. LESSONS: Orbital varices have a spectrum of clinical manifestations, from asymptomatic to severe visual loss. Most cases are successfully treated conservatively; however, in complicated cases, interventions with a multidisciplinary team approach such as sclerotherapy, embolization, and surgical excision should be considered.

Emergency postpartum hysterectomy as a consequence of cervical varix during pregnancy; a case report and literature review.

INTRODUCTION AND IMPORTANCE: Vaginal bleeding might accrue during pregnancy and it has different causes due to the pregnancy trimester and the diagnosis and management would be crucial to prevent maternal-fetal life-threatening situations. In uncommon cases, varicose veins can emerge in the neck of the uterus, leading to a severe maternal hemorrhage. CASE PRESENTATION: We presented a pregnant woman with vaginal bleeding and spotting during pregnancy with the diagnosis of cervical varix at 22 weeks of gestation. Close monitoring and proper patient education led to a term delivery at 37 weeks of gestation. Otherwise, an emergency postpartum hysterectomy after a cesarean section was performed due to uncontrolled bleeding from cervical varix. CLINICAL DISCUSSION: Although rare, cervical varix should be included in the differential diagnosis in a pregnant patient who appears with extensive vaginal bleeding to reduce maternal and/or neonatal morbidity or fatality. The approved diagnosis for that is not clear. CONCLUSION: This case report showed that Doppler and transvaginal sonography could be suitable diagnostic tools. The best management for cervical varix needs further research.

Orbital Venous Malformation Excision after Transcaruncular Embolization with Onyx.

Orbital venous malformations are low-flow lesions resulting from vascular dysgenesis during development. Patients may present with vision loss, proptosis accentuated by Valsalva, and/or painful spontaneous thrombosis. The preferred treatment for symptomatic lesions is embolization combined with excision. A 34-year-old male presented to our institution from an outside emergency department with a diagnosis of presumed idiopathic orbital inflammation. For the prior month, he had been experiencing left orbital pressure, subjective eye bulging, and both diplopia and blurry vision when in peripheral gaze or when bending over. Despite initial improvement with steroids, his symptoms recurred with tapering. Visual acuity was reduced to 20/25, but pupils and motility remained normal. Biopsy demonstrated a vascular lesion characterized by fibroadipose tissue with histologically unremarkable blood vessels, and cerebral arteriography showed no high-flow components. A diagnosis of orbital venous malformation was made. He then underwent intraoperative angiography and Onyx embolization followed by excision via a transcaruncular approach. Two prior reports have described the use of Onyx in venolymphatic malformations. This report highlights a detailed approach to defining flow characteristics pre- and intraoperatively and expands upon our understanding of the use of Onyx for such cases.