Case 6. Distensible venous malformationof the left inferomedial orbit is shown before (A) and after (B) Valsalvamaneuver. C, Computed tomographic scan of orbits and coronal section demonstratingoval enhancing lesion in the left inferomedial orbit. D, Venous phase of thecerebral angiogram, lateral projection, demonstrating the venous malformation(double arrowheads). E, Percutaneous puncture of the malformation under fluoroscopyand sclerotherapy, the alcohol-contrast mixture is injected into the lesionas noted in this lateral projection (double arrowheads). F, Complete resolutionof the lesion 1-month postoperatively.
Case 11. A, Exposure of the lesionintraoperatively. B, Shrinkage of the lesion after carbon dioxide laser ablation.
Case 17. A, Computed tomographicview of the orbits, axial section, 6 years after subtotal surgical excision,showing an enhancing mass in the superior aspect of the right orbit (arrow)and additional abnormal enhancement in the right temporal area intracranially(double arrowheads). B, Orbital venogram in Waters projection, 19 years postoperatively,showing the intraorbital venous malformation (arrow). There is also refluxin multiple dilated irregular cutaneous and subcutaneous facial veins on thesame side (double arrowheads). C, At the same session, Guglielmi detachablecoil (GDC) embolization of the intraorbital venous malformation after intraoperativeexposure of an orbital vein, access to the malformation through the exposedvein with a microcatheter (white arrow), and deposition of GDCs (black arrow).D, Platinum GDCs within the right orbit with no opacification of the intraorbitalvenous malformation (open arrow) 2 years after the embolization. A smallersubcutaneous venous malformation located over the bridge of the nose (blackarrow) and another venous malformation over the right maxilla (double arrows)is also noted.
Case 22. Computed tomographicview of orbits, coronal section, showing a hyperdense lesion in the superiorportion of the left orbit (black arrow) and associated enlargement of theorbit secondary to bony remodeling in the orbital roof (white arrow).
Case 14. Dilated irregular veinlined by single layer of endothelium (thin arrow). A thrombus was noted centrally(thick arrows) with recanalization (asterisk) (hematoxylin-eosin, originalmagnification ×10).
Case 2. Fragment of the wall ofa vein (thin arrow) showing proliferation of vascular endothelial cells thatform papillary projections (thick arrow) in the lumen (hematoxylin-eosin,original magnification ×26).
Arat YO, Mawad ME, Boniuk M. Orbital Venous MalformationsCurrent Multidisciplinary Treatment Approach. Arch Ophthalmol. 2004;122(8):1151-1158. doi:10.1001/archopht.122.8.1151
To evaluate the clinical, radiological, and histopathological features,treatment, and outcome of a series of orbital venous flow malformations (OVMs)with the aim of delineating a more systematic approach for treatment.
A 38-year retrospective review of 22 patients with OVMs followed upat 1 institute.
Eighteen of 22 patients (13 women and 9 men) showed clinical or radiologicalevidence of distensibility. The mean age at the initial manifestation was28.3 and 50.7 years in patients with distensible and nondistensible OVMs,respectively. Eight patients (36.3%) had deep orbital lesions, 6 (27.3%) hadsuperficial orbital lesions, and 8 (36.3%) had combined orbital lesions. All3 patients with deep nondistensible OVMs had a sudden onset of proptosis andpain or diplopia secondary to thrombosis or hemorrhage. Seventeen patientsrequired treatment. All 4 nondistensible lesions were treated by surgicalexcision. A variety of techniques were used to treat distensible OVMs includingcarbon dioxide laser ablation, percutaneous alcohol sclerotherapy, or embolizationwith Guglielmi detachable coils after surgical exposure and surgical excision.Of the 14 patients with follow-up, 8 had complete resolution of the signsand symptoms and 6 patients showed marked improvement following surgery. Meanfollow-up was 57.8 months.
If intervention is indicated, less invasive methods such as carbon dioxidelaser ablation and percutaneous alcohol sclerotherapy for superficial andcombined orbital lesions and endovascular treatment by Guglielmi detachablecoil embolization for deep orbital lesions should be considered before proceedingwith more invasive procedures.
Understanding of orbital vascular malformation (OVM) has been difficultowing to the confusion in correctly classifying these lesions and in providingtreatment on a rational basis.1 Wright andcolleagues2,3 suggested that segregationof OVMs as lymphangiomas and orbital varices is artificial and incorrect andtheir origin, clinical manifestations, and treatment must be considered asa whole. Others resist the unification of lymphangiomas and orbital varicesunder the heading of orbital venous anomalies because separation of the conditionsalong hemodynamic relationships is consistent with their clinical, pathophysiological,and diagnostic features and it also influences therapeutic decisions and surgicalapproaches.4 In 1999, a consensus statementon terminology of the OVMs was published by the members of the Orbital Society.1 According to the new terminology, lymphangiomas wereclassified as no flow malformations and primary varices, including the mixedlesions with venous no flow components, were classified as orbital venousflow malformations.1
This article concentrates on the OVMs as classified by the Orbital Society.This is the category, which includes both distensible OVMs (with the evidenceof connection to the venous system either shown clinically or radiologicallyby distensibility with increased venous pressure) and nondistensible OVMs.1
Distensible OVMs are a part of a spectrum of vascular malformationsinvolving periorbital skin, conjunctiva, and/or extraorbital sites such asthe central nervous system, face, and nasal sinuses, or a combination of thesesites. The coexistence of these vascular malformations at different sitesis believed to represent aberrations of vasculogenesis occurring at the samestage of embryonic development.5 Our objectivein this study was to describe the clinical, radiological, and histopathologicalfeatures, treatment, and outcome of a series of OVMs with the aim of delineatinga more systematic approach for treatment.
This study is a retrospective review of 22 consecutive patients havinga diagnosis of OVMs who were followed up at our clinic from 1964 to 2002.All except 3 patients were treated and followed up by the same surgeon (M.B.)These 3 patients required endovascular treatment that was done as combinedcases with the interventional neuroradiologist (M.E.M.). Our classificationof OVMs is listed in Table 1,which was derived from the consensus statement on terminology published bythe Orbital Society.1 No flow malformations(lymphangiomas) and arterial flow malformations were excluded from this study.Distensibility was considered present when there was clinical or radiologicalevidence of transient expansion with increased venous pressure associatedwith dependent positioning, the Valsalva maneuver, and coughing or by demonstrationof distention with increased venous pressure by ultrasonography or computedtomography (CT).1 Diagnosis in all 4 nondistensibleOVMs was confirmed histopathologically. Venogram was not performed in thesenondistensible lesions to demonstrate the flow because of a low clinical suspicionof a venous malformation. Even if a venogram was performed, it might not demonstrateany significant flow in these nondistensible OVMs. Orbital venous flow malformationswere classified as superficial if they involved the periorbital skin, conjunctiva,or eyelid without extension posterior to the equator of the globe. Deep orbitallesions were those located posterior to the equator of the globe without extraorbitalinvolvement. Lesions with both superficial and deep orbital components weredescribed as combined lesions.5
Seventeen patients required intervention. The patients were treatedby surgical excision, carbon dioxide (CO2) laser ablation, embolizationwith platinum Guglielmi detachable coils (GDCs) (Target Therapeutics, Fremont,Calif), and percutaneous alcohol sclerotherapy depending on the distensibilityand location of the lesion.
Carbon dioxide laser ablation was performed transcutaneouslyin superficial lesions involving the skin and after surgical exposure in deeperlesions as a primary treatment without excision. A CO2 laser (modelXL-40; Coherent Inc, Palo Alto, Calif) was used with a power setting of 2W in the continuous mode.
Guglielmi detachable coil embolization was performed after intraoperativeexposure of an orbital vein. An intraoperative venogram was obtained priorto the procedure. Following the access to the malformation through the exposedvein with a microcatheter, platinum GDCs were deposited.
Percutaneous sclerotherapy was performed after the puncture of the OVMunder fluoroscopy and 3 mL of a combination 0.75:0.25 mixture of absolutealcohol and ethiodized oil (Ethiodol; Savage Laboratories, Melville, NY) wasinjected into the lesion without any occlusion using a 20-gauge angiocatheter.
Histopathological characteristics were examined in 11 patients. Mainoutcome measures were self-assessment of the patients regarding the improvementin symptoms or cosmesis as well as the objective improvement in visual acuity,extraocular muscle motility, and Hertel measurements. The patients were examined1 day, 1 week, and 1 month postoperatively by the same physician (M.B.) andthen followed up every 6 months. Six patients were lost to follow-up.
The cases of 18 of 22 patients (13 women and 9 men) reviewed in thisstudy showed clinical or radiological evidence of distensibility. The remaining4 patients had nondistensible OVMs. The age at onset of the initial signsand symptoms ranged from 5 months to 79 years, with a mean age of 32.4 years.The mean age at the initial manifestation of patients with distensible andnondistensible OVMs was 28.3 and 50.7 years, respectively. Eight patients(36.3%) had deep orbital lesions, 6 patients (27.3%) had superficial orbitallesions, and 8 patients (36.3%) had combined orbital lesions. All 3 patientswith deep nondistensible orbital lesions were initially seen with a suddenonset of proptosis and pain or diplopia secondary to thrombosis or hemorrhage.Of the 18 patients with distensible lesions, no patients were initially seenwith acute symptoms. Five of the 18 patients with distensible OVMs had deeplesions and 4 of them were initially seen with proptosis or pain increasingwith the head in a dependent position. One patient with a deep distensiblelesion was initially seen with diplopia and enophthalmos. Of the remaining13 patients with superficial and combined distensible OVMs, 7 were initiallyseen with swelling at the location of the lesion.
Location of the lesion within the orbit was variable, but superior involvementwas the most common (59%). Three patients had associated extraorbital venousmalformations, involving the same side of the brain, face, and body as theorbital lesion.
Computed tomography (CT) was performed in 12 patients and typicallyshowed tubular or lobulated homogeneous-nonhomogeneous–enhancing masslesions. Four patients underwent magnetic resonance imaging. Magnetic resonanceimaging showed enhancing mass lesions with variable signal intensities dependingon the flow, presence of hemorrhage, or thrombosis within the lesion. Diagnosticorbital venogram was performed in 11 patients to confirm the diagnosis andto determine the routes of inflow and outflow as well as the extent of thelesion. Venogram was not performed if the lesion was small and superficial,if the OVM was not suspected clinically before the surgery, or if the patientwas unable to afford the additional expense. Transfemoral cerebral angiographywas performed in 7 patients with clinical signs or symptoms suggestive ofan arterial flow lesion (ie, pulsation, bruit, and others) to rule out arteriovenousmalformations.
Indications for intervention were cosmesis (7 patients), pain (6 patients),diplopia (2 patients), and optic nerve compression (2 patients). Five patientswho did not receive any surgical intervention were observed. Seventeen patientsrequired intervention. Four patients with nondistensible lesions were treatedby surgical excision. Of the 13 patients with distensible lesions, 3 had superficiallesions that were treated by surgical excision (1 patient), CO2 laserablation (1 patient), or percutaneous alcohol sclerotherapy (1 patient) (Figure 1). Of the 7 patients with combineddistensible lesions; 3 were treated by CO2 laser ablation (Figure 2) and 2 by surgical excision. Twopatients with combined distensible lesions (patients 16 and 17) had associatedextraorbital venous malformations and they required multiple interventionsincluding embolization of the venous malformations with platinum GDCs aftersurgical exposure (Figure 3) forthe deep component and CO2 laser ablation or percutaneous alcoholsclerotherapy for the superficial portion. Three patients with deep distensiblelesions had lateral orbitotomy and were treated with CO2laser ablation(1 patient), subtotal excision (1 patient), or ligation and cauterization(1 patient) of the vascular malformation.
Histopathological characteristics were examined in 11 patients. Twopatients had associated intravascular papillary endothelial hyperplasia arisingin a thrombosed vein.
Clinical and diagnostic features, treatment, and outcome of the patientsare summarized in Table 2. Fourteenof the 17 patients treated had follow-up. Mean follow-up was 57.8 months (range,2 months to 22 years). Eight patients had complete resolution of the signsand symptoms; the remaining 6 patients had marked improvement following surgery.Four of 11 treated patients who had distensible OVMs had long-term follow-up(>2 years). Postoperative ptosis developed in 2 patients. Transient skin blisteringwas noted at the site of injection in 1 patient after percutaneous alcoholsclerotherapy and resolved spontaneously in 1 month. No patient lost visionduring follow-up. Best-corrected visual acuity was improved in 1 patient.Of the 5 patients who received no intervention and were only observed, 3 patientswere lost to follow-up. The condition of the remaining 2 patients is stable,at a mean follow-up of 25.5 years.
The term "venous flow malformations" is applied to weakened segmentsof the orbital venous system of variable complexity including distensiblelesions that have direct and rich communication with the venous circulationand nondistensible anomalies that have minimal communication with the venoussystem. The nondistensible vascular malformations seem to have a definitecommunication with the systemic venous circulation but to a much lesser extentthen the distensible variety.5 To our knowledge,no large series of patients treated for OVMs, other than a series of 15 patientsdescribed by Lacey et al5 has been published.We report the clinical, radiological, and histopathological features, treatment,and outcome of 22 OVMs with the aim of describing a systematic approach fortreatment.
Distensible and nondistensible OVMs showed different clinical featuresbesides their different clinical and radiographic findings owing to distensibility.In our study mean age at manifestation was 28.3 years in patients with distensibleOVMs. Nondistensible lesions were seen in an older-age group, with an averageage of 50.7 years. Spontaneous orbital hemorrhage and thrombosis can be notedin all types of vascular malformations, but it is uncommon in distensibleOVMs compared with the nondistensible OVMs, which have a stagnant blood flow.5 We noted thrombosis or orbital hemorrhage in 17.6%in the distensible and 100% in the nondistensible lesions. All except 1 patientwith deep distensible OVMs were seen with proptosis or pain increasing withthe head in a dependent position. One patient with a deep distensible lesionwas seen with diplopia and was noted to have enophthalmos in the sitting orstanding position. Distension of the lesion with the head in a dependent positioncaused an enlargement of the orbit probably secondary to remodeling of theorbital bones as demonstrated by the CT scan (Figure 4). Most of the patients with superficial and combined lesionsinitially had swelling at the location of the lesion.
Histopathological examination was performed in 11 patients and thisrevealed dilated irregular veins lined by a single layer of endothelium withor without the presence of thrombus (Figure5). Histopathological features of distensible and nondistensiblelesions did not differ, although there was a higher incidence of thrombosisin the nondistensible ones. Histopathological examination of 2 nondistensiblelesions with thrombosis showed associated intravascular benign endothelialhyperplasia, which is an unusual condition characterized by a benign proliferationof vascular endothelial cells that form papillary projections in the lumenof the blood vessel (Figure 6).It is believed to be a reactive response that develops secondary to a thrombusin vascular malformations or hemangiomas.6
Orbital CT with contrast enhancement is a reliable method of demonstratingOVMs.7 In our series orbital CT with contrastenhancement was the most frequently used radiological study to demonstratethe venous malformations. Orbital venography can be used to demonstrate theircommunication with the native venous circulation and routes of inflow andoutflow as well as the extent of the lesions.5
The treatment of OVMs is largely conservative although extreme orbitalpressure or intractable pain, profound orbital hemorrhage leading to visualdeterioration, and cosmetic disfigurement can be indications for intervention.8 In our study all patients with nondistensible lesionsand 72.2% of the patients with distensible lesions required intervention.The high percentage of cases requiring intervention may reflect the tertiaryreferral nature of our center.
To date there has been no definite standardization of treatment of OVMs.Thrombosed OVMs can be successfully treated by surgery if intervention isindicated.9 In our series 3 patients with deepthrombosed nondistensible OVMs required surgical treatment for optic nervecompression or severe pain and were treated by evacuation of clotted bloodand subtotal excision of the lesion with good results. Recently, Gigantelliet al10 described an endoscopic transethmoidaldecompression of a thrombosed OVM and evacuation of thrombus as a less invasiveapproach.
Superficial and combined lesions can be treated by CO2 laserablation either transcutaneously or after surgical exposure of the lesionwithout surgical excision. It is particularly useful if the lesion is extensiveand diffuse. Carbon dioxide laser ablation has been used as an adjunct tosurgical excision in the past, but we believe that it can also be successfullyused as a primary treatment in selected superficial and combined OVMs as aless invasive approach. If the lesion is small and circumscribed, it can beexcised surgically with the help of the CO2laser. The CO2 laser can also be helpful for treatment of deep OVMs. One patient withan intraconal distensible OVM underwent lateral orbitotomy and CO2 laserablation of the lesion with a successful result.
Although percutaneous alcohol sclerotherapy of venous malformationsin various anatomical sites were found to be effective and safe, experiencewith OVM has been limited and this might be explained by the concern aboutthe risk of optic nerve injury secondary to leakage of alcohol within theorbit.5 We describe a 10-month-old patientwith a superficial distensible OVM treated by percutaneous alcohol sclerotherapy.Following treatment, this patient had self-limiting skin blistering over thelesion and the OVM completely resolved in 3 months. It is our opinion thatpercutaneous alcohol sclerotherapy can be applied to superficial OVMs withcaution as a less invasive treatment alternative. Best results have been reportedwith small well-defined OVMs instead of large infiltrating lesions in otherparts of the body.11
Deep distensible OVMs have been traditionally treated by surgical excision.Surgical excision can be facilitated by the use of clips5,12 orby the recently described technique of embolization with cyanoacrylate glueafter surgical exposure5 to prevent bleedingduring excision. Embolization with cyanoacrylate glue is not free of complicationsincluding vision loss. The lesion with the material must be removed surgicallysince it causes a foreign body reaction if it is left in the orbit.5 Hard consistency of the glue can also lead to difficultyin surgical dissection of the embolized lesion. Nonadhesive liquid agentssuch as ethylene vinyl alcohol copolymer (Onyx; Microtherapeutics Inc, Irvine,Calif) have been recently shown to be useful in the treatment of arteriovenousmalformations.13 These can be potentially usefulfor future treatment of OVMs.
Endovascular treatment by platinum GDC embolization is a less invasivealternative to surgical excision for deep distensible OVMs. Preoperative orintraoperative venography can identify the patients for whom embolizationis appropriate. Takechi et al14 reported acase of an orbital varix treated with percutaneous transfemoral venous catheterizationand embolization with platinum GDCs. This approach can be technically difficultowing to developmental variations. Embolization of OVMs after surgical exposurehas the advantage of avoiding difficult catheterization and limiting traumaticdissection.15 Weill et al15 reporteda case of an orbital varix treated by embolization with platinum GDCs aftersurgical exposure with good result. In our series 2 patients with complexOVMs were treated by embolization with platinum GDCs after surgical exposure.One of these patients (case 17) who had a history of subtotal excision ofthe lesion during childhood underwent 2 endovascular treatments with embolization.The patient's symptoms improved markedly after the second embolization. Thefollow-up venogram showed complete occlusion of the OVM 2 years postoperatively(Figure 3D).
The other patient (case 16) had endovascular treatment for the deeporbital component of the lesion and also had multiple ablations with CO2 laser for the superficial portion, with considerable improvement ofthe signs and symptoms. Embolization is more useful for saccular or segmentalvenous dilations than for tangled plexus of venous channels.15 Embolizationwith platinum GDCs is not a preferred choice of treatment for the superficiallesions since it shows its effect by inducing thrombosis in the lesion withoutan actual decrease in the size of the lesion, and this may not be cosmeticallyacceptable.
Our preferred treatment choices can be summarized by the following criteria:
For distensible or nondistensible OVM without intractablepain, orbital hemorrhage leading to visual deterioration, or cosmetic disfigurement:observation
For distensible OVM:
Percutaneous alcohol sclerotherapy
surgical exposure with excision
b. Superficial diffuse: CO2 laser ablation; transcutaneousor after surgical exposure
i. Embolization with platinum GDCs
ii. Surgical exposure followed by CO2 laser ablation
iii. Surgical excision facilitated by the use of clips or prior embolizationwith cyanoacrylate tissue glue
For nondistensible OVM: Lesions associated withthrombosis and/or hemorrhage:
a. Without intractable pain, threatened optic nerve compression, orsevere proptosis: Observation if the diagnosis is made clinically
b. With intractable pain, optic nerve compromise, severe proptosis,or repeated/nonresolving episodes of thrombosis: Surgical exposure and evacuationof clot with subtotal excision
If intervention is indicated, the aim should be to consider the leastinvasive method as a first-line treatment since these lesions are benign andsymptomatic treatment is satisfactory in most patients. Over the years, thesemalformations have been treated by surgical excision facilitated by differentmethods. We believe the patients who require intervention should be giventhe choice of a less invasive method such as CO2 laser ablationand percutaneous alcohol sclerotherapy for superficial and combined lesionsand endovascular treatment by platinum GDC embolization for deep lesions.In cases of acute thrombosis or hemorrhage associated with severe pain, proptosis,or visual loss, surgery may be required to remove the thrombosed lesion orhematoma. The complex venous lesions fall into a clinical spectrum of vascularmalformations affecting the orbit, face, and brain; they are difficult totreat and treatment must be customized for each patient individually.
Correspondence: Milton Boniuk, MD, Baylor College of Medicine, ScurlockTower, 6560 Fannin, Suite 902, Houston, TX 77030 (firstname.lastname@example.org).
Submitted for publication May 28, 2003; final revision received January2, 2004; accepted February 12, 2004.
This study was presented in part at the 20th Meeting of the EuropeanSociety of Ophthalmic Plastic and Reconstructive Surgery; September 21, 2002;Muenster, Germany; and as a poster at the 2002 Joint Meeting of the AmericanAcademy of Ophthalmology and Pan-American Association of Ophthalmology; October20-23, 2002; Orlando, Fla.