Orbital Vascular Lesions Research Articles

INFERIOR RECTUS FIBROSIS AND RESIDUAL DIPLOPIA FOLLOWING SCLEROTHERAPY FOR ORBITAL VENOUS MALFORMATION

Orbital vascular lesions are challenging to treat and observation is often preferred if symptoms are mild. Historically, surgical excision of the lesions used to be the preferred treatment, but intraoperative bleeding and incomplete removal were common. Therefore, sclerotherapy has become the treatment of choice by virtue of being minimally invasive and extremely effective. Sclerotherapy involves multiple intralesional injections of sclerosing agents like bleomycin and sodium tetradecyl sulfate. In this communication, we describe an unusual complication associated with sclerotherapy to treat an orbital venous malformation in a young woman diagnosed with a type-2 low flow venous malformation (orbital varices) in her left orbit. There was complete resolution of the lesion. However, following the procedure, she complained of diplopia accompanied by left hypotropia. The condition persisted six months after the procedure. Forced duction test confirmed a tight inferior rectus and she refused any further intervention. This case report discusses the possible mechanisms of this rare and unusual complication associated with sclerotherapy while treating orbital vascular lesions.

ORBITAL VENOUS LYMPHATIC MALFORMATION : REVIEWING THE CLINICORADIOLOGICAL ASPECT OF A LESS KNOWN ENTITY.

Hemangiomas and venous lymphatic malformations are the two most common orbital vascular lesions seen in pediatric patients. Orbital venouslymphatic malformations (OVLMs) (previously referred to as ′lymphangiomas′) are relatively less common, benign cystic type I vascular malformations. OVLMs may remain clinically unapparent or might manifest in late childhood with slowly progressive proptosis, periorbital swelling and displacement of globe. These malformations usually enlarge gradually. Sudden proptosis may be seen with spontaneous intraorbital hemorrhage or venous thrombosis. Acute presentation includes severe pain, compressive optic neuropathy or loss of vision, when intervention is indicated. Imaging plays critical role in diagnosis. The imaging mainstay is based on USG with high resolution probes and MRI with contrast.

A clinicopathological reappraisal of orbital vascular malformations and distinctive GJA4 mutation in cavernous venous malformations

Vascular anomalies are common orbital lesions, while variations in previous nomenclature might hamper robust characterization of their clinicopathological and genetic features. We reviewed and reclassified 92 orbital vascular lesions by the modified International Society for the Study of Vascular Anomalies (ISSVA) classification with reappraising clinicopathological parameters of 4 main types of vascular malformations, including orbital venous malformation 1 (OVM1, cavernous venous malformation), OVM2 (varix), OVM3 (infiltrating venous malformation), and arteriovenous malformation (AVM). GJA4, BRAF, and KRAS mutations were assessed by Sanger sequencing. There were 90 cases of vascular malformations, consisting of 60 OVM1 (67%), 13 AVM (14%), 8 OVM2 (9%), 8 OVM3 (9%), and 1 lymphatic-venous malformation (1%). The prevailing OVM1, histologically characterized by well-delineated borders and a uniform cavernous growth pattern, predominantly occurred in intraconal space (57%, P=.019) with an older median age (49 years) and female predilection (73%). OVM2, OVM3, and AVM exhibited differences in the distributions of patients' ages and lesion locations. Sizes of lesions were significantly correlated with periorbital and intraconal/extraconal locations (P<.001). OVM1 had the lowest rate of residual and recurrent diseases (3%). GJA4 mutations were identified in 75% (44/59) of OVM1 but not in OVM2/3 and AVM. No BRAF or KRAS mutations were detected. In conclusion, the modified ISSVA scheme enables meaningful classification of orbital vascular malformations by highlighting the molecular correlation between the distinct clinicopathological features and specific GJA4 mutation in OVM1, which implies OVM1 as a unique variant of venous malformation genetically akin to cutaneous and hepatic counterparts.

A Female Patient with Intraorbital Varix: A Case Report

: The present study reported the case of a 23-year-old female patient with right-sided enophthalmos. After several episodes of spontaneous periorbital ecchymosis, she developed positional proptosis. The ophthalmologic examination was normal, except a 3-mm enophthalmos measured using the Naugle exophthalmometer, which improved significantly by the Valsalva maneuvers. The diagnostic imaging tests in orbital disease consisting of orbital computed tomography and orbital magnetic resonance imaging showed a retro-orbital vascular lesion. The most likely diagnosis was orbital varix, which is a rare orbital vascular lesion. It was decided to manage the patient conservatively, in the absence of complications such as proptosis with corneal exposure, optic nerve compression, or unacceptable appearance.

Molecularly targeted agents in oculoplastic surgery.

Significant advances have been made in oncology and rheumatology with the introduction of molecularly targeted agents (MTAs). MTAs consist of monoclonal antibodies and small molecule inhibitors. The purpose of this manuscript is to review the recent applications of MTAs to orbital, lacrimal, and eyelid disease. The use of monoclonal antibodies has been described in the treatment of orbital vascular lesions, lymphoma, and squamous cell carcinoma. Inflammatory conditions treated with monoclonal antibodies include thyroid eye disease, IgG4 disease, and granulomatosis with polyangiitis. Immunotherapy with checkpoint inhibitors has also found applications to orbital disease. Use of small molecule inhibitors has been described in the treatment of basal cell carcinoma, squamous cell carcinoma, and Erdheim-Chester disease. There are many orbital, lacrimal, and eyelid side effects of MTAs with which the oculoplastic surgeon should be familiar, including hypertrichosis, edema, and orbital and eyelid inflammation. MTAs represent the future of treatment of oncologic and inflammatory conditions. Application of these agents to orbital, lacrimal, and eyelid disease will continue to expand. Elucidating the molecular mechanisms of oculoplastic disorders will facilitate additional potential pathways that could be targeted for therapy.

Expression of Vascular Endothelial Growth Factor Receptors in Benign Vascular Lesions of the Orbit: A Case Series

Vascular lesions of the orbit, although not malignant, can cause morbidity because of their location near critical structures in the orbit. For the same reason, they can be challenging to remove surgically. Anti-vascular endothelial growth factor (VEGF) drugs are increasingly being used to treat diseases with prominent angiogenesis. Our study aimed to determine to what extent VEGF receptors and their subtypes are expressed on selected vascular lesions of the orbit. Retrospective case series of all orbital vascular lesions removed by one of the authors (JAG) at the Mayo Clinic. A total of 52 patients who underwent removal of vascular orbital lesions. The pathology specimens from the patients were retrieved, their pathologic diagnosis was confirmed, demographic and clinical information were gathered, and sections from vascular tumors were stained with vascular endothelial growth factor receptor (VEGFR), vascular endothelial growth factor receptor type 1 (VEGFR1), vascular endothelial growth factor receptor type 2 (VEGFR2), and vascular endothelial growth factor receptor type 3 (VEGFR3). The existence and pattern of staining with VEGF and its subtypes on these lesions. There were 28 specimens of venous malformations, 4 capillary hemangiomas, 7 lymphatic malformations, and 6 lymphaticovenous malformations. All samples stained with VEGF, 55% stained with VEGFR1, 98% stained with VEGFR2, and 96% stained with VEGFR3. Most (94%) of the VEGFR2 staining was diffuse. Most orbital vascular lesions express VEGF receptors, which may suggest a future target for nonsurgical treatment.

Dynamic Arterial and Valsalva-Augmented Venous Phase Multidetector CT for Orbital Vascular Lesions

To describe the value and purpose of a new method of dynamic arterial and Valsalva-augmented venous phase CT angiographic assessment of vascular orbital lesions. Description of a dynamic arterial and Valsalva-augmented venous phase multidetector CT protocol currently being used at the institution of the authors for investigation of orbital vascular lesions. Research ethics approval was not required by the institution because this is a 1-case report, with a pictorial review of imaging findings in various representative orbital vascular lesions. One case of a distensible venous malformation depicts the clinical application of the imaging protocol. Key features of the malformation were demonstrated by arterial and venous phases of imaging. This technique is of value in diagnosis and management of vascular malformations of the orbit.

Immunohistochemical Investigations of Orbital Infantile Hemangiomas and Adult Encapsulated Cavernous Venous Lesions (Malformation Versus Hemangioma)

Immunohistochemical studies have begun to advance knowledge regarding the pathogenesis of vascular anomalies in many anatomical regions. However, the immunohistochemical features of most orbital tumors have been overlooked. Therefore, a comparative immunohistochemical study of a series of the 2 most common orbital vascular lesions- infantile hemangioma (IH) and encapsulated cavernous venous lesion (ECVL), the latter also termed cavernous hemangioma or venous malformation-was undertaken. Twenty surgically excised orbital tumors diagnosed clinically and histopathologically as IHs (10 cases) or "cavernous hemangioma" (10 cases) were evaluated pathologically and immunohistochemically using hematoxylin and eosin, Alcian blue, Masson trichrome, GLUT-1, CD31, CD34, D2-40, smooth muscle actin (SMA), desmin, and Ki-67 probes. All cases reacted strongly with the traditional blood vessel endothelial markers CD31 and CD34 and were negative for D2-40, a selective marker for lymphatic endothelium. All IH were positive for GLUT-1, and all ECVL were negative for GLUT-1. In IH, SMA (but not desmin) stained a monolayer of pericytes and in ECVL multilaminar smooth muscle vascular mural cells and intravascular (interstitial) stromal cells. Nuclear Ki-67 immunostaining was strongly positive in IH (average of 16.3%) and close to zero in ECVL. Immunophenotypic results for ECVL and IH demonstrated no overlapping staining patterns. Infantile hemangioma had the classical architecture of capillaries. Because of the constant presence of mural smooth muscle, it was concluded that ECVL is an accurate and descriptive term. However, desmin negativity in ECVL indicates myofibroblastic differentiation rather than full-fledged smooth muscle differentiation. Infantile hemangioma may display ectatic channels as the lesion ages but does not acquire multilaminar smooth muscle walls. Its pericytes lack cytoplasmic filaments and desmin reactivity but are SMA-positive because of the presence of poorly polymerized actin in the cytosol. In IH, Ki-67 positivity was observed in the endothelial cells of the solid and more ectatic regions. In contrast, the virtual absence of Ki-67 positivity in ECVL lends further support for the interpretation that it is more closely related to a malformation than a benign neoplasm.

Orbital vascular lesions: a review of clinical and imaging findings

Orbital vascular lesions include vascular malformations and vascular tumors. Overlapping clinical findings and similar imaging features between both types of lesions can lead to misdiagnosis and incorrect treatment choices. Classifying vascular malformations according to the nature of the vascular element(s) involved (artery, vein or lymphatic) facilitates correct lesion recognition, developing a correct differential diagnosis and characterization of findings. Imaging is critical in mapping the extent of these lesions and their response to therapy, both medical and surgical.

Orbital Fibrous Histiocytoma Mimicking Cavernous Hemangioma on Dynamic Contrast-Enhanced MRA Imaging

Orbital lesions include a broad spectrum of tumors, vascular abnormalities, and inflammatory conditions. High-resolution imaging has become an invaluable tool toward formulating an accurate diagnosis, and facilitates proper counseling regarding appropriate interventions. Imaging may guide whether partial excision to minimize damage to orbital structures, or en bloc removal to prevent potential recurrence, as seen in mesenchymal tumors, is indicated., Recently, dynamic contrast-enhanced magnetic resonance angiography (MRA) has demonstrated use in helping differentiate orbital vascular lesions. This imaging modality uses rapid MRI acquisition to provide noninvasive, dynamic flow information with high spatial resolution. However, even with modern imaging, reaching a diagnosis prior to histopathological analysis can be challenging. We present a case of orbital fibrous histiocytoma that appeared nearly identical to cavernous hemangioma on dynamic contrast-enhanced MRA.

Confirmation of and Differentiation Among Primary Vascular Lesions Using Ultrasonography

To demonstrate the utility of orbital ultrasound in the evaluation and management of suspected orbital vascular lesions. Clinical case series of 15 patients with suspected orbital vascular lesions seen in the Neuro-ophthalmology clinic of the Wilmer Eye Institute. The techniques of standardized echography (combined use of a contact B-scan and standardized A-scan) were performed by an experienced echographer, and data obtained were compared with other available imaging modalities to determine appropriate management. Patients were monitored for improvement, stability, or worsening of symptoms and signs. Based on echographic data obtained, 6 patients underwent surgery, 9 were observed over a follow-up period of 0-14 (mean 3.3) years. All patients demonstrated improvement or remained stable, and no complications developed from misdiagnosis during subsequent follow-up. Orbital ultrasound provides reliable imaging parameters and can be used as the primary imaging modality when evaluating suspected orbital vascular lesions.

Clinical and Imaging Findings in Multifocal Orbital Vascular Lesions: A Case Series

Introduction: A variety vascular disorders can affect the orbit, often manifesting as solitary space-occupying lesions. This study presents a case series of 3 patients with multi-focal orbital vascular conditions emphasizing on the clinical and imaging findings which could assist in the diagnosis and treatment planning.Methods: Retrospective consecutive case series of 3 patients with multifocal orbital vascular conditions treated at the Department of Ophthalmology of the University Hospital of Heraklion, in Crete, Greece.Results: Presenting symptoms included diplopia with compensatory head tilt (1 case) and eyelid protrusion enhanced by Valsalva maneuver (2 cases). MRI and CT studies showed multiple contrast-enhancing vascular lesions, associated with calcifications (1 case). Two lesions were surgically removed, proving to be orbital varices.Conclusions: Orbital vascular lesions may present multi-focally and displace adjacent structures. MRI and CT imaging may reveal their multi-focal presence as well as associated features, such as phleboliths.

Growth of a Presumed Orbital Venous Anomaly During Pregnancy With Spontaneous Resolution Postpartum

A 27-year-old pregnant woman in her second trimester presented with a 3-month history of gradual proptosis, decreased vision, and choroidal folds in her right eye. MRI revealed an intraconal mass with inhomogeneous enhancement consistent with a vascular lesion. The patient was followed clinically and the lesion remained stable for the remainder of her pregnancy and delivery by Cesarean section. Three months postpartum, the proptosis, choroidal folds, and decreased vision had resolved. Repeat scanning revealed complete resolution of the lesion. Pregnant patients with orbital vascular lesions need to be followed carefully during their pregnancy and after delivery. These lesions can worsen during pregnancy and may resolve spontaneously in the postpartum period.

Emphasis on pathology and hemodynamics based classification of orbital vascular lesions

Orbital vascular lesions, mainly including hemangioma and vascular malformation, are the most common disorders among orbital diseases. Vascular lesions of the orbit have clinically complicated presentations and are very hard to deal with. Selection of therapeutic modality on orbital vascular lesions requires an appropriate classification of them. An improved classification of these lesions has been proposed by the Orbital Society based on the pathological types and hemodynamic features and has been extensively used in developed countries. However, orbital vascular lesions are still being classified based on morphology in China. This classification system does not represent the pathological difference between hemangioma and vascular malformation of the orbit, nor the hemodynamic characteristics among different vascular malformations of the orbit. Morphology based classification has hampered scientific dialogue and confused clinicians, with occasionally serious consequences for patients. Thus, the use of a pathology and hemodynamics based classification of orbital vascular lesions should be highly advocated. We can expect that the use of appropriate classification can improve the management of these lesions in our country.

Noninvasive Dynamic Magnetic Resonance Angiography With Time-Resolved Imaging of Contrast KineticS (TRICKS) in the Evaluation of Orbital Vascular Lesions

To evaluate the clinical utility of time-resolved contrast-enhanced magnetic resonance angiography (MRA) in the evaluation of vascular orbital tumors. Retrospective medical record review of patients with vascular orbital lesions imaged with Time-Resolved Imaging of Contrast KineticS (TRICKS; GE Healthcare [Chalfont St Giles, England]) MRA, a noninvasive dynamic imaging modality. Five patients with orbital vascular tumors were evaluated using TRICKS MRA. These included 1 patient with a cavernous hemangioma, 2 patients with orbital varices, 1 patient with an orbitocutaneous arteriovenous malformation, and 1 patient who had a solitary fibrous tumor with features of a hemangiopericytoma. In 2 patients, diagnoses were altered as a result of TRICKS MRA. In addition, a young patient with a large orbitocutaneous arteriovenous malformation involving the ophthalmic artery was followed perioperatively and noninvasively using TRICKS MRA, which produced exquisite images and added substantial value in the care of these patients. Dynamic MRA in the form of TRICKS is a newly available imaging modality with great potential for improving the evaluation and management of patients with complex orbital tumors.

Orbital vascular pathology: clinical and imagiological differential diagnosis

Orbital vascular lesions represent an important group of orbital pathology, because of their high prevalence, particularly in the pediatric population. They are also the most controversial group of lesions, due to the polemics in regard to their nature. Based on cases of their experience, the authors analyze the orbital vascular pathology, especially its clinical and imagiological characteristics, important to the diagnosis.

ORBITAL VASCULAR LESIONS

Orbital vascular lesions include a spectrum of disorders that have in common one or more of the cellular components of blood vessels: endothelial cells, pericytes, and smooth muscle cells. Beyond that, there are major differences in their frequency, clinical presentation, morbidity, and management. In addition to the local mass effect and possible systemic implications that characterize any orbital tumor, the hemodynamic relationships of orbital vascular lesions influence their clinical behavior and dictate their appropriate management. Whereas every lesion has an intrinsic blood supply to meet its own metabolic needs, some are more closely integrated into the systemic vasculature and convey considerably more blood than needed for their own sustenance. We prefer to divide vascular lesions between two major categories: developmental malformations and neoplastic proliferations (Table 1). The cellular complement and basic structure of developmental malformations are present at birth, and growth occurs by expansion. The neoplasms, on the other hand, enlarge by proliferation of their component vascular units or predominant cell of origin.

ORBITAL VASCULAR LESIONS: Role of Imaging

Vascular lesions represent an important subgroup of orbital abnormalities; however, there is much controversy regarding their pathophysiology and nomenclature. Attempts at resolution of some of the issues have resulted in changes of names of some of the lesions (e.g., lymphangioma to venolymphatic malformation). Imaging plays an important role in delineation and characterization of these lesions. A radiologist informed about the pathophysiology and attentive to the internal architecture of the lesions shown on images, particularly MR images, often can predict the histologic diagnosis. Imaging information helps clarify some of the controversies surrounding these lesions.

Vascular tumors of the orbit.

Eighty-five vascular lesions of the orbit examined and treated between 1963-1993 were reviewed retrospectively to reveal the types of vascular tumors, age and sex distribution, clinical characteristics, treatment options and prognosis. Capillary hemangioma was the most frequent orbital vascular tumor accounting for 37 of 85 cases making up 43.5% of the entire orbital masses. Cavernous hemangioma accounted for 35 cases (41.2%), varices for 3 cases (3.5%), anteriovenous malformations for 3 cases (3.5%), angiosarcomas for 3 cases (3.5%), lymphangiomas for 3 cases (3.5%) and hemangiopericytoma for one case (1.2%). Of the 85 vascular tumor cases 43 were encountered in children aged 18 years or less. Capillary hemangioma was the most frequent pediatric orbital vascular lesion, making up 86% of the vascular tumors in this age group. Capillary hemangiomas have been treated with observation and intralesional corticosteroid injections. Cavernous hemangiomas, vascular malformations, lymphangiomas and two cases of angiosarcomas received orbitotomy. One case each of angiosarcoma and hemangiopericytoma underwent exenteration because of the advanced stage of the lesion. There was one tumor-related death who was a 9-year-old boy diagnosed with angiosarcoma. Vascular tumors with the exception of angiosarcomas and hemangiopericytoma behaved as benign lesions.

Vascular tumors of the orbit

The author presents 536 cases of vascular tumors of the orbit. The most common orbital vascular lesions are cavernous hemangioma, capillary hemangioma, and lymphangioma. The hemangiopericytoma are less frequent than the three other types. The clinical and paraclinical characteristics of the most frequent tumors are analyzed and the various possibilities of treatment are discussed.