Vascular Engorgement Research Articles

Combined Central Retinal Artery and Vein Occlusion-like Manifestations with Aplastic Anemia: Unveiling Pathophysiological Insights

Purpose: To report the association of a combined central artery and vein occlusion (CCRAVO)-like pattern with aplastic anemia and discuss the pathophysiology associated with ophthalmologic examinations. Methods: We report a female patient with aplastic anemia who presented with retinal hemorrhage, blurred optic disc margins, retinal whitening, peripapillary subretinal fluid, and venous engorgement. The authors described the evolution of ophthalmologic lesions in correlation with improvements in systemic conditions. Results: We discuss and correlate the ophthalmological findings of this patient (optic disc edema, vascular engorgement, subretinal fluid, among others) with the pathophysiology of Aplastic Anemia based on current literature. Conclusion: These findings provide a better understanding of the pathophysiology of this rare disease. In certain cases, ophthalmologic symptoms can be the first presentation, underscoring the importance of reporting ocular findings for the recognition of this disease, which entails significant systemic involvement.

A case of lacrimal gland vascular engorgement in Sturge–Weber syndrome

A case of lacrimal gland vascular engorgement in Sturge–Weber syndrome

Unusual neuroimaging findings in spontaneous intracranial hypotension

The most common neuroimaging manifestations of patients suffering from spontaneous intracranial hypotension (SIH) include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, sagging of the brainstem, and cerebellar hemosiderosis. However, infrequently patients may present with separate neuroradiological findings which could be easily mistaken for other pathology. We describe patients who presented with unique neuroimaging findings who were eventually found to have a spinal CSF leak or venous fistula. Relevant clinical history and neuroradiology findings are presented, and a relevant review of the literature is provided. We present six patients with a proven CSF leak or fistula who presented with dural venous sinus thrombosis, compressive ischemic injury, spinal hemosiderosis, subarachnoid hemorrhage, pial vascular engorgement, calvarial hyperostosis, and spinal dural calcifications. Radiologists should be familiar with atypical neuroimaging manifestations of SIH in order to avoid misdiagnosis and guide the clinical trajectory of the patient towards accurate diagnosis and eventual cure.

Abstract Number ‐ 280: A Unique Case of Pial AVF Presenting as Hemispheric Cerebral Edema

Introduction To describe a unique case of pial arteriovenous fistula presenting as left hemispheric cerebral edema with hemianopsia, sensory change and ataxia. Methods Case report Results Our patient is a 38 year old male with a history of diabetes, hyperlipidemia and congenital deafness with a left cochlear implant who presented to an outside hospital for visual complaints. Non‐contrast CT of the head was negative at that time; he could not obtain an MRI due to his cochlear implant. He developed worsening right hemianopia and aphasia, prompting his transfer to our facility. Upon arrival, repeat imaging was done, including a CTA of the head and neck. This revealed a large region of hypodensity involving the left PCA and distal MCA territories. An MRI was attempted but artifact from his implant obscured visualization of the left hemisphere. Post‐contrast CT was negative for evidence of tumor. CT venogram was negative for veno‐occlusive disease. He complained of worsening headache and demonstrated worsening word‐finding ability. He received100g total of mannitol divided across 3 boluses which improved his symptoms mildly. Over the following days he began to become ataxic and clumsy in the right upper extremity and his headache worsened further and he began to develop focal seizures electrographically localized to the left parietal lobe. Repeat CTA demonstrated expanding hypodensity with marked vascular engorgement in the left hemisphere. He underwent 6‐vessel digital subtraction angiography (DSA) of the head which revealed gross left hemispheric venous congestion with early filling of the left hemispheric venous system by a parasagittal pial ateriovenous fistula believed to be responsible for his hemispheric edema. He was discharged on acetazolamide and acetaminophen for headache management and plans for outpatient follow‐up and potential intervention. Follow‐up DSA revealed improvement in his venous congestion and reduction in the degree of his early venous filling with some symptomatic improvement by medical management. Conclusions Pial arteriovenous fistulae (pAVF) are a rare entity and can present in a variety of ways, with symptomatic intracranial hemorrhage and ischemia being two of the most common. This case illustrates an unusual instance of hemispheric edema initially mistaken for venous infarct caused by a pAVF, and prompts the physician to consider angiography and further exploration when initial workup for cerebral edema and/or venous infarct is initially unrevealing.

An Integrated Radiologic-Pathologic Understanding of COVID-19 Pneumonia.

This article reviews the radiologic and pathologic findings of the epithelial and endothelial injuries in COVID-19 pneumonia to help radiologists understand the fundamental nature of the disease. The radiologic and pathologic manifestations of COVID-19 pneumonia result from epithelial and endothelial injuries based on viral toxicity and immunopathologic effects. The pathologic features of mild and reversible COVID-19 pneumonia involve nonspecific pneumonia or an organizing pneumonia pattern, while the pathologic features of potentially fatal and irreversible COVID-19 pneumonia are characterized by diffuse alveolar damage followed by fibrosis or acute fibrinous organizing pneumonia. These pathologic responses of epithelial injuries observed in COVID-19 pneumonia are not specific to SARS-CoV-2 but rather constitute universal responses to viral pneumonia. Endothelial injury in COVID-19 pneumonia is a prominent feature compared with other types of viral pneumonia and encompasses various vascular abnormalities at different levels, including pulmonary thromboembolism, vascular engorgement, peripheral vascular reduction, a vascular tree-in-bud pattern, and lung perfusion abnormality. Chest CT with different imaging techniques (eg, CT quantification, dual-energy CT perfusion) can fully capture the various manifestations of epithelial and endothelial injuries. CT can thus aid in establishing prognosis and identifying patients at risk for deterioration.

Utility of Cerebral Microvascular Imaging in Infants Undergoing ECMO.

Infants who require extracorporeal membrane oxygenation (ECMO) therapy have an increased risk of neurological complications and mortality. Microvascular imaging (MVI) is an advanced Doppler technique that allows high-resolution visualization of microvasculature in the brain. We describe the feasibility and utility of MVI for the evaluation of cerebral microvascular perfusion in patients undergoing ECMO. We retrospectively analyzed brain MVI scans of neonates undergoing ECMO. Two pediatric radiologists qualitatively assessed MVI scans to determine the presence or absence of tortuosity, symmetry, heterogeneity, engorgement, and hypoperfusion of the basal ganglia-thalamus (BGT) region, as well as the presence or absence of white matter vascular engorgement and increased peri-gyral flow in the cortex. We tested the association between the presence of the aforementioned brain MVI features and clinical outcomes. We included 30 patients, 14 of which were male (46.7%). The time of ECMO duration was 11.8 ± 6.9 days. The most prevalent microvascular finding in BGT was lenticulostriate vessel tortuosity (26/30, 86.7%), and the most common microvascular finding in the cortex was increased peri-gyral flow (10/24, 41.7%). Cortical white matter vascular engorgement was significantly associated with the presence of any poor outcome as defined by death, seizure, and/or cerebrovascular events on magnetic resonance imaging (p = 0.03). MVI is a feasible modality to evaluate cerebral perfusion in infants undergoing ECMO. Additionally, evidence of white matter vascular engorgement after ECMO cannulation could serve as a predictor of poor outcomes in this population.

Efficacy evaluation of intravitreal ranibizumab therapy for three types of retinopathy of prematurity

To evaluate efficacy of intravitreal ranibizumab (IVR) therapy for aggressive posterior retinopathy of prematurity (ROP), threshold ROP disease and type 1 pre-threshold ROP. A retrospective analysis was performed on 40 patients (76 eyes) who had IVR as the primary treatment for ROP from April 2017 to January 2018. According to disease pathogenic features, the 76 eyes were divided into three groups: aggressive posterior ROP (AP-ROP) group (16 eyes), threshold ROP group (28 eyes) and type 1 pre-threshold ROP group (32 eyes). The characteristics of patients and lesions situation before the first intravitreal injection, and posttreatment fundus outcomes determined by wide-angle RetCam fundus imaging were recorded. The birth weight and postmenstrual age of first IVR treatment in AP-ROP, threshold ROP, and type 1 pre-threshold ROP groups were significant difference (1087.50±246.78, 1103.75±168.30, 1257.03±210.82 g, P=0.005; 34.50±1.46, 36.89±2.97, 36.50±2.36wk, P=0.008), while the gestational age was not difference (28.00±2.00, 28.54±1.90, 28.59±1.43wk, P=0.510). The retina hemorrhage ratio (with/without: 14/2, 8/20, 5/27), iris neovascularization or vascular engorgement ratio (with/without: 12/4, 11/17, 6/26), and the zone I (inside/outside: 16/0, 2/26, 5/27) in AP-ROP, threshold ROP, and type 1 pre-threshold ROP group were difference significantly (all P<0.05). The regression rates were 37.5%, 92.86%, and 100%, and the recurrence rates were 62.5%, 7.14%, and 0 in AP-ROP, threshold ROP, and type 1 pre-threshold ROP group, respectively (both P<0.05). The recurrence eyes were cured by secondary IVR or retinal laser photocoagulation. IVR is an effective treatment for all types of ROP. The regression of AP-ROP is significantly lower than type 1 pre-threshold and threshold disease. Birth weight, retinal hemorrhage, iris neovascularization or vascular engorgement and lesions located in zone I may be associated with AP-ROP recurrence and retreatment, which should be noted in follow-up.

Relapsed acute myeloid leukemia presenting as conjunctival myeloid sarcoma: a case report

BackgroundConjunctival myeloid sarcoma (MS) as an isolated presentation of acute myeloid leukemia (AML) relapse is rare. Here, we report a case of unilateral conjunctival MS revealed as a sign of AML relapse.Case presentationA 50-year-old man with a history of AML in remission visited our clinic presenting with a left conjunctival injection persisting for 1 month. Diffuse subconjunctival thickening with conjunctival vascular engorgement was observed. Ultrasound biomicroscopy revealed a hyper-reflective, thickened conjunctiva in his left eye. During the incisional biopsy, the lesion was strongly attached to the underlying sclera; histopathologic examination revealed infiltration of leukemic blasts. The relapse of AML was confirmed by a successive bone marrow biopsy. The ocular lesion disappeared after allogeneic peripheral blood stem cell transplantation (PBSCT) and concomitant salvage radiotherapy on the left eye. The patient has remained in remission for 3 years after allogeneic PBSCT.ConclusionsIncidental conjunctival lesions can indicate AML relapse in patients treated earlier for AML. An ophthalmologist may have a role in the early detection of AML when a patient presents with an atypical conjunctival lesion.

Central serous chorioretinopathy: Treatment.

Central serous chorioretinopathy (CSC) is a pachychoroid spectrum disease characterized by serous detachment of the neurosensory retina with subretinal fluid in young and middle-aged adults. The pathogenesis of CSC is not yet fully understood. However, it is considered a multifactorial disease that is strongly associated with choroidal dysfunction or vascular engorgement. Although there is no consensus on the treatment of CSC, photodynamic therapy has been effectively used to manage serous retinal detachment (SRD) in CSC. Moreover, micropulse diode laser photocoagulation and focal laser treatment have also been used. Recently, oral medications, including mineralocorticoid receptor antagonists, have been proposed for the management of CSC. Multimodal imaging plays a significant role in the diagnosis and treatment of CSC. Optical coherence tomography angiography (OCTA) has the advantage of detecting vascular flow in the retina and choroid layer, allowing for a better understanding of the pathology, severity, prognosis, and chronicity of CSC. In addition, early detection of choroidal neovascularization in CSC is possible using OCTA. This review article aims to provide a comprehensive and updated understanding of CSC, focusing on treatment.

STUDY OF SPLENOMEGALY IN CHILDREN.

Splenomegaly is common clinical finding in pediatric practice. Splenic enlargement occurs when the spleen is increased by cells or tissue components or by vascular engorgement. Various etiologies can cause splenomegaly. The spleen is rarely the primary site of a disease. Splenomegaly is classified according to the length palpable below the costal margin as mild: &lt;3 cm, moderate: 4-7 cm &amp; massive: &gt;7 cm. Severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages. Only in the age group of 1 to 5 years females predominated as compared to males. In present study there is obvious male predominance as male: female ratio is 1.8:1. This difference could be due to more priority to male child to seeking medical care with such chronic illness.Present study also suggested that severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages.

Pulmonary imaging in coronavirus disease 2019 (COVID-19): a series of 140 Latin American children.

BackgroundThe severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which resulted in the worldwide coronavirus disease 2019 (COVID-19) pandemic of 2020, has particularly affected Latin America.ObjectiveThe purpose of the study was to analyze the imaging findings of pulmonary COVID-19 in a large pediatric series.Materials and methodsChildren with SARS-CoV-2 infection confirmed by either quantitative reverse transcription-polymerase chain reaction from nasopharyngeal swabs or presence of circulating immunoglobulin M (IgM) antibodies and who underwent chest radiograph or CT or both were included in this retrospective multicenter study. Three pediatric radiologists independently reviewed radiographs and CTs to identify the presence, localization, distribution and extension of pulmonary lesions.ResultsWe included 140 children (71 female; median age 6.3 years, interquartile range 1.6–12.1 years) in the study. Peribronchial thickening (93%), ground-glass opacities (79%) and vascular engorgement (63%) were the most frequent findings on 131 radiographs. Ground-glass opacities (91%), vascular engorgement (84%) and peribronchial thickening (72%) were the most frequent findings on 32 CTs. Peribronchial thickening (100%), ground-glass opacities (83%) and pulmonary vascular engorgement (79%) were common radiograph findings in asymptomatic children (n=25). Ground-glass opacity and consolidation were significantly higher in children who needed intensive care admission or died (92% and 48%), in contrast with children with a favorable outcome (71% and 24%, respectively; P<0.05).ConclusionAsymptomatic children and those with mild symptoms of COVID-19 showed mainly peribronchial thickening, ground-glass opacities and pulmonary vascular engorgement on radiographs. Ground-glass opacity and consolidation were more common in children who required intensive care admission or died.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00247-021-05055-2.

Relationship between eyelid margin irregularity and meibomian gland dropout

PurposeTo examine the relationship between lid margin abnormalities and meibomian gland loss in infrared meibography. MethodsThis study was a retrospective chart review of 170 patients with dry eye disease. A correlation analysis between eyelid margin abnormalities and meibomian gland dropout in infrared meibography was performed using data from 141 eyes. We graded the following eyelid margin abnormalities: irregular lid margin, vascular engorgement, plugging, anterior placement of the mucocutaneous junction, exposed terminal duct, and presence of tattoos. Multiple regression analyses were performed with meiboscore (meibomian gland dropout grade) as the dependent variable and age, sex, lid margin abnormality grades, and total grading score of lid margin abnormalities as the covariates. In addition, Meibomian glands structure were examined in those with eyelid margin dimpling using meibography in 25 eyes. ResultsIn the multiple regression analysis, an irregular lid margin in the upper eyelid was associated with a higher meiboscore after controlling for age and sex (coefficients B = 1.379, p = 0.025). Other lid margin abnormalities did not significantly affect the meiboscore. In the lower eyelids, irregular lid margin (coefficients B = 0.602, p = 0.009) and total grading score of lid margin abnormality were associated with higher meiboscores (coefficients B = 0.100, p = 0.022). Of the 25 eyes with dimples, 21 (84%) showed focal or complete meibomian gland loss at the site. ConclusionsLid margin abnormalities were found to be associated with meibomian gland dropout.

S2307 COVID-19 in a Patient on Dual Biologic Therapy for Inflammatory Bowel Disease

INTRODUCTION: Preliminary data suggest that dual biologic therapy might be effective and safe in a subgroup of inflammatory bowel disease (IBD) patients. However, the safety and outcomes of dual biologic therapy in IBD with Coronavirus Disease 19 (COVID-19) is unknown. Here, we present a case of a Crohn’s disease (CD) patient on adalimumab (ADA) and ustekinumab (UST) combination therapy who developed COVID-19. CASE DESCRIPTION/METHODS: This is a 24-year-old man with fibrostenotic Crohn’s ileocolitis diagnosed at age 9. He had a secondary loss of response to infliximab despite dose escalation to 10 mg/kg in combination with methotrexate (MTX). He was then transitioned to ADA and dose was escalated to 40 mg weekly with MTX (subsequently self-discontinued MTX). He achieved clinical response but not remission with ADA. Magnetic resonance enterography showed active inflammation in the terminal ileum, distal sigmoid colon, and rectum (Image 1). A colonoscopy showed ulcerated and polypoid mucosa in the terminal ileum and at the ileocecal valve with narrowing and patchy, mild pan-colonic inflammation (Image 2A/B). ADA level was 17 mcg/mL without antibodies. Switching to a different biologic was recommended. The patient was hesitant about discontinuing ADA due to symptomatic improvement. After discussion of risks and benefits, it was decided to bridge with UST and continue ADA with a plan to eventually discontinue ADA if remission was achieved. He received standard intravenous UST loading followed by 90 mg subcutaneously every 8 weeks. Five months later, he tested positive for COVID-19 due to exposure to an infected individual. He was asymptomatic from a respiratory standpoint and his gastrointestinal (GI) symptoms were at baseline. He received a dose of UST 1 day prior to COVID-19 testing and last ADA was 1 week prior to testing. ADA was held and resumed 2 weeks after as he remained without fever, respiratory symptoms or new onset GI symptoms at 2 and 6 weeks after testing positive for COVID-19. DISCUSSION: This case describes a favorable outcome of a CD patient who tested positive for COVID-19 while on ADA and UST. Multiple series have demonstrated that biologic monotherapy is not associated with poor outcomes in COVID-19 infected IBD patients. Instead, corticosteroids, advanced age and co-morbidities are predictors of adverse outcomes. However, more data is needed on the safety of combination biologic therapy in the COVID-19 era.Image 1.: Magnetic resonance enterography (coronal T1 weighted image) showing wall thickening, edema and hyperenhancement involving the terminal ileum over a length of approximately 20 cm with associated vascular engorgement is present.Image 2.: Colonoscopy images showing polypoid stenotic ileoceal valve (A) and the terminal ileum showing ulcerations (B).

Palpebral lobe of the human lacrimal gland: morphometric analysis in normal versus dry eyes

AimTo study the morphological features of the palpebral lobe of the main lacrimal gland in normal and dry eyes.MethodsThis cross-sectional study included 25 healthy subjects and 83 patients with dry...

Characteristics of Chest CT Images in Patients With COVID-19 Pneumonia in London, UK.

Background and objectiveNovel coronavirus 2019 (COVID-19) outbreak was first reported in Wuhan, Hubei Province in China in December 2019; it has then spread quickly and exponentially beyond the Chinese borders and is now regarded as a global pandemic. We aimed to evaluate the chest CT radiological characteristics and lesion distribution patterns in patients of COVID-19 pneumonia in London, UK.MethodsWe performed a retrospective study and reviewed data of patients with clinically suspected COVID-19 who underwent chest CT between February 1 and May 5, 2020. All patients underwent the reverse transcription-polymerase chain reaction (RT-PCR) test. Lung lesion characteristics and distribution patterns were evaluated by two radiologists. Fisher’s exact test was used for statistical analysis, and a p-value of <0.05 was considered statistically significant.ResultsA total of 18 patients (nine men and nine women) were analyzed. All of them had bilateral patchy lesions in the chest CT images. There was no correlation between the severity score and mortality (p=0.790). The distinctive CT features included ground-glass opacity (GGO) and consolidative patchy amorphous lesions, bilateral posterior and peripheral multi-lobar lung involvement, pleural effusions, subpleural fibrotic lines, subpleural sparing, vascular engorgement, occasional crazy paving, occasional mediastinal lymphadenopathy, pleural thickening, lack of cavitation, and absence of reverse halo (atoll) signs.ConclusionCT can facilitate the diagnosis of COVID-19 pneumonia. Our UK cohort showed slight variations compared with previously reported Asian and continental European cases with respect to chest CT images.

Carpal Tunnel Syndrome Associated with Klippel-Trénaunay Syndrome: A Case Report

Background and Importance: Klippel-Trénaunay syndrome is a rare congenital condition that rarely affects peripheral nerves. Median nerve involvement at the carpal tunnel level has only been reported on four occasions of this syndrome in the medical literature. Case Presentation: A 61-year-old Caucasian female patient with Klippel-Trénaunay syndrome presented with a 10-month history of paraesthesia and numbness affecting the median nerve distribution area of her left hand. The clinical and neurophysiological examination confirmed a moderately severe carpal tunnel syndrome that required surgical decompression. Conclusion: Pre-operative findings demonstrated diffuse vascular infiltration and engorgement of the median nerve. Flexor tendons had a normal appearance. The patient had a satisfactory post-operative period with full resolution of her symptoms 4 weeks after the procedure. Although peripheral nerves are rarely affected in patients with Klippel-Trénaunay syndrome, neurological symptoms could indicate nerve involvement and magnetic resonance imaging (MRI) examination should be considered to further assess the extension of the lesion.

Mechanisms, diagnosis and management of eosinophilic asthma

Asthma is a common chronic airway disease affecting about 334 million people worldwide, and up to 10% of asthma patients have severe asthma, which may be uncontrolled despite high doses of the standard treatment modifiers and may require the use of chronic oral corticosteroids. It is the most common chronic disease in children in the developed countries. Asthmamanifests as reversible airflow obstruction, due to airway inflammation, bronchial smooth muscle contraction, increased mucus secretion, vascular engorgement, mucosal oedema, and airway hyper responsiveness, which leads to airflow obstruction and symptoms of asthma. Eosinophilic asthma is a phenotype of asthma that is usually very severe and persistent, with frequent exacerbations. It is usually observed in adult asthmatic patients, although it may occur in children. It is characterized by the presence of high levels of eosinophils, and CD+4 Th2 cells in the lungs and airways, which can be demonstrated by a raised eosinophil count in blood, and induced sputum or bronchial biopsy. It is managed in a similar stepwise treatment for childhood-onset asthma, but some of the patients with eosinophilic asthma do not respond to this standard treatment including inhaled or oral corticosteroids. The logical approach to treat corticosteroid-refractory asthma is to target the eosinophilic interleukins which cause airway inflammation using monoclonal antibodies to block their activity on the eosinophils, and Th2 cells. Currently, the following monoclonal antibodies are used in the treatment of eosinophilic asthma: IgE antibody such as omalizumab, or interleukin receptor 5, or 4, and 13 antagonists, such mepolizumab, reslizumab, and dupilumab. These novel agents have proved to be very useful in relieving the symptoms, and in improving the forced expired volume in one second (FEV1), and in reducing exacerbations. They are also steroid-sparing agents, and improve the quality of lifein this debilitating phenotype of asthma.

1473 A Vanishing Cecal Mass

INTRODUCTION: Systemic mastocytosis (SM) is a rare disease characterized by infiltration of mast cells in extracutaneous organs. Although gastrointestinal (GI) manifestations such as nausea, vomiting, abdominal pain are observed in up to 60-80% of patients, colon lesions are rarely identified. Here we describe an unusual case of SM that manifested as a colon mass on routine screening colonoscopy. CASE DESCRIPTION/METHODS: A 71-year-old, asymptomatic, female with a long history of urticaria pigmentosa was referred to a gastroenterology clinic for evaluation of an eight by twelve centimeter, polypoid mass involving the cecum and ileocecal valve, found on screening colonoscopy. Subsequently, a CT scan noted mucosal hyperenhancement with slight vascular engorgement and colonic wall thickening involving the cecum and proximal ascending colon. Pathology from the initial biopsies revealed dense (&gt;100 per high powered field) infiltration of mast cells that stained positive for CD117 and CD25 cell surface proteins. The initial, large, polypoid, cecal mass was not present on repeat colonoscopy with upper endoscopy. Instead, there was patchy nodular- appearing mucosa in the right colon. Follow-up biopsies of the upper and lower GI tract revealed dense infiltration of mast cells in the duodenum, ileum, ascending colon and transverse colon as well as elevated serum tryptase level (38 ug/L), all findings consistent with GI involvement of SM. Histamine-blocking therapy with ranitidine and cetirizine was commenced. DISCUSSION: Systemic mastocytosis results from a sporadic somatic mutation in the KIT gene that encodes a protein implicated in cell growth, division &amp; survival that results in overproduction of mast cells that accumulate in organs. There are four subtypes of SM that range in severity from slow and indolent to rapidly progressive towards organ failure. It is important to recognize and categorize systemic involvement of mastocytosis to aid in prognosis and treatment. Frequently, GI symptoms are mild but can include more serious effects of mast cell infiltration including peptic ulcer disease, malabsorption and non-cirrhotic portal hypertension. Specifically important to gastroenterologists is the recognition of GI involvement of mastocytosis disorders and understanding of appropriate evaluation, including upper and lower endoscopy with random biopsies to identify mast cell infiltration. Although GI symptoms are frequent and result in endoscopic evaluation, there are only rare reports of colon masses.

2550 Henoch-Schönlein Purpura Mimicking Crohn's Disease in a 65-Year-Old Male

INTRODUCTION: Henoch-Schönlein purpura (HSP) is a small vessel vasculitis typically seen in children, often presenting with abdominal pain, purpuric rash, arthritis, and hematuria. Also known as immunoglobulin A (IgA) vasculitis, it is mediated by the deposition of immune-complexes containing IgA in the skin, gut and kidneys. Although rarely seen in adults, adult cases are associated with a more severe course than in pediatric patients. We present the case of adult-onset HSP with GI involvement. CASE DESCRIPTION/METHODS: A 65-year-old retired male dairy farmer with recent travel to Southwest US presented with a 6-week history of abdominal pain in the setting of a subacute, diffuse, purpuric rash. After admission, computed tomography (CT) imaging revealed ileitis with fat stranding and mesenteric vascular engorgement, concerning for Crohn's disease (Figure 1). Patient was also found to have proteinuria and arthralgia. Stool infectious work-up was unrevealing. Skin biopsy of the rash identified leukocytoclastic vasculitis and IgA deposition. Patient then underwent colonoscopy with ileoscopy, which scattered areas of congested appearing mucosa with deep purplish discoloration surrounded by normal mucosa (Figure 2). Scattered, nonspecific, rare small erosions and focal erythema were also seen throughout colon and rectum (Figure 3). Biopsies indicated mildly active ileitis and focal areas of active colitis with foci of hemorrhage in the right and transverse colon; there was no evidence of chronicity. Though non-specific, histologic findings were consistent with IgA vasculitis, rather than Crohn's. Patient was started on intravenous (IV) steroids and azathioprine. His rash and abdominal pain improved considerably after 48 hours of treatment. DISCUSSION: HSP, although more common in children, can be seen in adults, and should be included on the differential diagnosis when abdominal pain and/or GI bleeding is seen with concurrent skin, renal, &amp; musculoskeletal findings. HSP is associated with significant morbidity and mortality due to the rapid progression of the disease and recognition can be delayed, particularly in adults. This case of adult-onset HSP was quickly diagnosed and treated with appropriate anti-inflammatory therapy. HSP can mimic Crohn's disease, by history and imaging, but the endoscopic findings are very different. The case illustrates some of the endoscopic and radiographic findings of HSP, promoting awareness among adult gastroenterologists.

The retinal foveal avascular zone as a systemic biomarker to evaluate inflammatory bowel disease control

BackgroundInflammatory bowel disease (IBD) is a systemic inflammatory disease and is classified as Crohn’s disease (CD) or ulcerative colitis (UC) depending on the extent of gastrointestinal tract involvement. IBD can be associated with extraintestinal findings, such as fever, weight loss, arthralgia, and mucocutaneous lesions, as well as hepatic, renal and ophthalmological involvement. Clinical parameters and colonoscopy are used to establish the criteria for controlled or non-controlled disease and subsequent definition of treatment. Our objective in the present study was to compare the area of the foveal avascular zone (FAZ) in patients with a diagnosis of IBD during remission and active disease.Methods144 eyes of 72 patients with IBD were evaluated via a complete ophthalmological exam. Fundus photography and optical coherence tomography/angiography (OCT/OCTA) were performed with a Topcon Triton. The macula and posterior pole were evaluated by binocular indirect ophthalmoscopy and fundus biomicroscopy. The area of the FAZ was determined via manual delimitation of superficial retinal vascular layers from OCTA with image6.net software. To establish disease activity, we considered the Mayo Score, fecal calprotectin levels, colonoscopy results and clinical parameters. All retinal parameters were evaluated in a blinded manner. Means were compared between groups using the Mann–Whitney test.ResultsThe participants had a mean age of 42.26 years and included 28 males (38.88%) and 44 females (61.11%). Among the participants, 37 had a diagnosis of CD (51.38%), and 35 had a diagnosis of UC (48.61%). Twenty-five patients (34.72%) had active disease, and 47 (65.27%) were in remission. The area of the FAZ did not differ significantly between the CD and UC groups (p = 0.91 for the right eye and p = 0.76 for the left eye) but did differ significantly between the remission and active disease groups (p = 0.01 for the right eye and p = 0.02 for the left eye).DiscussionOur study is the first to evaluate the area of the FAZ in patients with IBD via swept-source OCTA. The area of the FAZ did not differ significantly in either eye between the CD and UC groups. However, patients classified as having active disease according to clinical parameters and colonoscopy presented a significant decrease in the area of the FAZ compared with patients in remission. The area of the FAZ is an ophthalmological parameter that can be obtained non-invasively and is increased in ischemic diseases such as diabetic retinopathy. The FAZ may decrease due to vascular engorgement or increased systemic inflammation. This parameter can be used to help determine whether a patient is in remission or active IBD, thus potentially reducing the need for invasive exams during disease follow-up.