Orbital Lymphatic Malformations Research Articles

Transnasal Endoscopic Sclerotherapy for Orbital Apex Lymphatic Malformation.

Orbital lymphatic malformations are benign, slowly progressive vascular malformations. Management of these malformations is challenging due to their infiltrative and diffuse nature. The authors present a case with orbital apex lymphatic malformation treated with transnasal endoscopic sclerotherapy.

Intralesional Bleomycin for Orbital Lymphatic Malformations: Comparison of Clinical Versus Radiologic Regression by Volumetric Analysis.

The aim of the study was to investigate the clinical resolution versus radiologic regression of orbital lymphatic malformations (LMs) following treatment with intralesional bleomycin sulfate sclerotherapy. A retrospective interventional study of 24 eyes with orbital LMs treated with nonimage-guided bleomycin sclerotherapy. The clinical and radiologic outcomes were classified as excellent, good, fair, and poor. Regression was assessed clinically and by radiologic volumetrics. Mean age at presentation was 17 ± 18 years (median 11, range 5 months to 70 years). Lesion morphology was microcystic in 11 (46%), macrocystic in 8 (34%), and mixed in 5 (21%) eyes. Mean units of bleomycin injected per session were 4 ± 2 IU (median 5 IU, range 1-6 IU). Mean number of treatment sessions required was 2 ± 1 (median 2, range 1-6). Cumulative units of bleomycin injected were 11 ± 9 (median 9, range 1-38 IU). The clinical response was excellent in 19 (79%), good in 4 (17%), and fair in 1 (4%). The mean preoperative and postoperative lesion volumes were 7 ± 4 cm3 and 0.8 ± 1.2 cm3, respectively (p < 0.0001, 95% CI, -7.89 to -4.51). Radiologic resolution of LM was excellent in 6 (25%), good in 8 (33%), fair in 7 (29%), and poor in 3 (13%) eyes. Spearman's rank correlation coefficient for correlation between clinical and radiologic grading was 0.51 (p = 0.01, 95% CI, 0.13-0.75%). There was a sustained tumor resolution without recurrence over a mean follow-up duration was 2 years (median 18 months; range 12-60 months). Bleomycin sclerotherapy for orbital LMs gives an excellent to good clinical response in 93%. However, a parallel radiologic regression is seen only in 58%. The endpoint to assess response should be clinical. Treatment till complete radiologic resolution may not be necessary.

Low-dose Bleomycin Injections for Orbital Lymphatic and Lymphatic-Venous Malformations: A Multicentric Case Series Study.

Orbital lymphatic malformations (LM) are associated with ocular morbidity and facial disfigurement. Surgery is challenging and may not be effective. We describe the outcome of bleomycin injections for venous LM and lymphatic-venous malformation (LVM) malformations of the orbit in 5 tertiary referral centers between January 2010 and December 2018. Multicenter retrospective case series, 5 oculoplastic referral centers: Sheba and Rabin Medical Centers, Israel; Mulago Hospital, Uganda; Sri Sankaradeva Nethralaya, India; and Clinique Ophtalmologique de Tunis, Tunisia. All patients diagnosed with orbital LM/LVM were assigned to successive (range 1-6) intralesional 5 international units bleomycin injections. They all underwent complete ophthalmic and orbital evaluations, orbital imaging, and ancillary testing as needed. Clinical photographs were assessed pre- and posttreatment along with objective assessments of clinical improvement. Additional injections were provided in cases of incomplete response. A total of 21 patients (17 women, mean ± standard deviation age 18 ± 13 years, range 2-48 years) underwent bleomycin injections. The mean injection dose was 12 ± 10 international units in 1-3 injections. There was a dramatic improvement in lesion size, appearance, proptosis, and ocular motility in 20/21 patients (95%) after a mean follow-up of 18 months. Visual acuity slightly improved after treatment (20/50-20/30; P = 0.076). No side effects were noted after bleomycin injections. Bleomycin injections for LM/LVM of the orbit are effective; local or systemic side effects were not seen in this series. To the best of our knowledge, this is the largest reported series of this treatment.

Acute approach to orbital lymphatic malformations

Pediatric lymphatic malformations (LMs) represent a rare, benign subtype of vascular malformations that can commonly occur in the head or neck region, specifically in or around the orbit. Orbital LMs typically require a multidisciplinary team of specialists and diagnostic imaging. Historically, treatment options for these periorbital LMs include imaging-guided drainage with sclerotherapy in addition to surgery, at times in conjunction with medical management. There exists debate in terms of an approach for management timing of these lesions across various treatment centers, and interventional radiologists should be informed regarding treatment timing considerations in evaluating patients for potential intervention. The aim of this case series is to review the current standards regarding therapeutic interventions for LMs in the literature and discuss an organized approach to guide the optimal treatment timing for the management of these lesions. In this case series, two pediatric patients – one 2 years old and one 12 years old – presented with a unilateral periorbital LM and received treatment at an academic tertiary care children’s hospital. The first patient was diagnosed with macrocystic periorbital LM after presenting with eye pain and eyelid swelling. After initial ultrasound-guided drainage to relieve eye pressure, the patient rebled into the LM and underwent sclerotherapy intervention. The second case involved a periorbital LM with intralesional hemorrhage presenting as a violaceous eyelid mass diagnosed after bumping into a swing. The patient was treated medically with interval growth of the LM and underwent subsequent sclerotherapy. Both patients responded well to ultrasound-guided sclerotherapy. The management timing and decision to intervene were based on consults from multidisciplinary services, age, risk factors, and clinical stability on presentation. We present two cases of pediatric periorbital LM and review an organized institutional approach for treatment timing based on paradigms in the literature.

Elective sclerotherapy of an orbital lymphatic malformation: A case report and review of the literature

Orbital lymphatic malformations (LMs) are a unique subtype of vascular anomaly that can swell and lead to blindness if left untreated in the acute setting. Detrimental acute and chronic effects of the malformation on vision suggest that treatment should be considered pre-emptively rather than in the acute setting. We describe the effectiveness and safety of treating these LMs electively using sclerotherapy, a minimally invasive treatment method.

The Use of Sirolimus for Treatment of Orbital Lymphatic Malformations: A Systematic Review.

Orbital lymphatic malformations are rare congenital choristomas associated with pain, proptosis, exposure keratopathy, and vision loss. Current treatments of surgery, drainage, and sclerotherapy may have adverse effects including risk of damage to surrounding structures, swelling, and malformation persistence or recrudescence. Sirolimus, which inhibits mammalian target of rapamycin, a regulator of cell growth and vascular endothelial growth factor expression, has successfully treated systemic vascular malformations. However, its efficacy and safety have not yet been well established for orbital lymphatic malformations. Systematic review and analysis of relevant published literature were performed. PubMed, Embase, and World of Science searches were conducted for studies involving sirolimus treatment of orbital lymphatic malformations through July 2019. Nine case series and reports with 10 total patients who received sirolimus for treatment of orbital lymphatic malformations were included. The age at sirolimus initiation ranged from 1 week to 23 years. The malformation was lymphatic in 6 patients, lymphaticovenous in 3 patients, and lymphatic-arteriovenous in 1 patient. Six patients underwent ineffective prior therapy including sclerotherapy, surgery, or medical therapy. Initial sirolimus dosage ranged from 0.05 mg/kg twice a day to 1 mg twice a day, and duration ranged from 6 months to 53 months. Seven patients had partial response, and 3 patients, all of whom had a microcystic malformation component, experienced complete response. Adverse effects included mild reversible leukopenia, hypertriglyceridemia, hypercholesterolemia, and transaminitis with adverse effects denied or not specified for 6 patients. Sirolimus may be a safe and effective treatment for orbital lymphatic malformations, especially microcystic malformations.

Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what's new?

Currently, there is no ideal management for orbital lymphatic malformations. Significant advances have been made since the discovery of new agents in the treatment. The purpose of this manuscript is to review the recent evidence on new sclerotherapy agents and systemic medications. Traditional sclerosants are OK-432, sodium tetradecyl sulphate and ethanol. More recent developments are the use of doxycycline, bleomycin, and pingyangmycin. Sirolimus as a systemic medication has revolutionized the medical management of lymphatic malformations. Other oral drugs such as propranolol and sildenafil are controversial. Future treatment involves targeting lymphangiogenic pathways including inhibition of vascular endothelial growth factors and the phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit. The development of new agents allows multimodal management either as monotherapy or combined therapy to achieve better outcomes in this difficult to manage disease.

S1-19 ORBITAL LYMPHATIC MALFORMATIONS IN CHILDREN. TRANSCRANIAL APPROACH RESULTS IN SEVERE CASES. OUR EXPERIENCE.

Introduction: Orbital lymphatic malformations are unusual benign vascular lesions, representing 1–3% of space-occupying orbital lesions and 5% of the vascular lesions fo the orbit. They are found mainly in children, over 3 years of age. Although their origin is not wellknown, thay can be associated to intracranial vascular lesions and enlargement of the orbital bone. Their clinical course include hemorragic episodes which cause proptosis, decrease of the visual aquity and oculomotor disturbances. Those located around the optic nerve, spcecially in the retroorbital intracapsular area, could be cause a severe optic neuropathy and amaurosis. The treatment depend on the lesion extension, clinical symptoms and the cystic characteristics of the lesion, many different apporaches have been proposed. As high difficulties to remove the whole lymphatic lesion exist, recurrences are encountered in a high rtate, up to 55%. We present our experience in the treamtent of the most extended lesions affecting the retroorbital area, around the optic nerve. Methods: We have review 8 cases with this pathology. The surgery was indicated wthn optic nerve function became affected. MRI was performed as diagnostic tool, presenting all the patients ocular protrusion, and hemorragic cytic lesions ocuping the optic nerve area, around 270–360°. We perform a frontal craniotomy incluying the orbital roof in one piece, with a variable extension ot the lateral wall. The periorbital fascial was open and a wide resection was perfomed with microsurgical technicque. The orbital fascia was closed with a periostial graft. Close follow up was necessary to control visual aquity and oculomotor function. Results: Eight patients have been treated using this approach, all of them recovering at least 30% vision and oculomotor function. The ocular position improves widely, reaching eyelid closure and symmetric ocular position. Recurrences have been very low, with mild symptoms. Conclusion: Althougm many different techiques have been proposed in the treatment ol lymphatic orbital malformation, cases affecting 210360° around optic nerve requiere complex treatments. The transcranial approach in the tretment of very wide orbital lymphatic malformations allows recovering or preserving the visulal aquity, improves the proptosis and secondarily the aesthetic appearance and improve the oculomotor funcions. Main complications are related with the transient oculomotor deficits, CSD leakage (only in 1 case, managed convervatively) and partial recurrence of the lesion.

Image-Guided Percutaneous Bleomycin and Bevacizumab Sclerotherapy of Orbital Lymphatic Malformations in Children.

To evaluate the effectiveness and safety of image-guided percutaneous sclerotherapy using bleomycin for macrocystic and bevacizumab (Avastin™) for microcystic orbital lymphatic malformations in children. Between October 2015 and July 2018, we prospectively evaluated 10 pediatric patients who presented clinically and radiologically with lymphatic malformations and were treated with percutaneous sclerotherapy. Patients with venous malformations were excluded. Eight females and two males with ages ranging from 3 to 17years (mean: 8.8, SD: 4.9) were included. Guided with ultrasound and fluoroscopy, macrocysts were treated with bleomycin instillation. For microcystic components in three patients, bevacizumab was injected intralesional. All patients underwent ultrasound and non-contrast MRI to evaluate response to treatment after 6weeks. The malformations were macrocystic in seven patients and complex (macro/microcystic) in three. Twenty sclerotherapy sessions were performed, (range: 1-3 sessions, mean: 2, SD: 0.8). Clinically, there was a significant reduction in the proptosis after treatment (P = 0.007) and dystopia (P = 0.018). The local radiological response showed a reduction in the maximum lesions diameters and volumes after treatment (P = 0.005 and 0.005, respectively). Two of the three patients treated with bevacizumab showed a reduction in the lesions volumes by 90.4% and 63.4%, respectively, whereas one patient did not show volume reduction. Transient periorbital edema and ecchymosis occurred following the procedure with no major complications encountered. Follow-up ranged from 9-33months, mean: 20.3, SD: 7.4. Bleomycin sclerotherapy is a safe and effective treatment for orbital macrocystic lymphatic malformations. Further use of bevacizumab for microcystic lesions in a larger series is required to outline its efficacy and safety.

Use of percutaneous bleomycin sclerotherapy for orbital lymphatic malformations

ABSTRACTPurpose: Bleomycin sclerotherapy has been shown to be a viable treatment for lymphatic malformations. However, its use for these lesions confined to the orbit is becoming increasingly documented in the literature. In this study, we summarize the clinical manifestations and outcomes observed following percutaneous bleomycin sclerotherapy for orbital lymphatic malformation.Methods: A 5-year retrospective chart review of patients with clinical, radiographic, and/or biopsy-confirmed diagnoses of orbital lymphatic malformation that received bleomycin sclerotherapy was conducted at the Emory Hospital and Clinics. Data examined included patient demographics, patient history and symptoms, clinical findings, radiographic findings, route of bleomycin delivery, and outcome.Results: Of the 10 patients who met inclusion criteria, the median age of treatment was 7 years. The most common presenting symptoms included vision change and proptosis. Nine of 10 patients demonstrated macrocysts (>1 cm) on imaging. Seven of 10 patients had histories of prior interventions including resections, cyst drainage, and debulking. Because 2 of these 10 patients were lost to follow-up, 8 patients remained for post-procedural evaluation. Four of these eight showed improvement of visual acuity after post-bleomycin sclerotherapy. In seven of eight patients, extraocular motility either improved or remained stable. Pretreatment and posttreatment exophthalmometer measurements obtained in four patients revealed an average improvement in proptosis of 65% from their average pretreatment measurements.Conclusions: Our findings suggest that percutaneous bleomycin sclerotherapy is a viable option for treatment of orbital lymphatic malformations, with potentially greater benefit to those with macrocystic features.

Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience

BackgroundPercutaneous sclerotherapy is an alternative to surgery for the treatment of orbital lymphatic malformations (LMs). We present a large series of patients undergoing sclerotherapy for macrocystic LMs with detailed visual...

Lymphatic Malformations of the Orbit

ObjectivesLymphatic malformations of the orbit are rare lesions that constitute approximately 1% to 8% of all orbital masses. They are difficult to treat since they do not remain within anatomic boundaries and tend to penetrate into normal orbital structures. The aim was to analyze clinical courses and therapy options in patients with lymphatic malformations of the orbit.MethodsThirteen patients with orbital lymphatic malformations confirmed by magnetic resonance imaging between 1998 and 2009 were enrolled in this study. Patients' charts were retrospectively reviewed to analyze clinical courses and treatment options.ResultsFour patients suffered from isolated intraorbital lymphatic malformations without conjunctival involvement, in three of them the masses were completely resected, in one patient close controls were performed. Three patients had isolated intraorbital lymphatic malformations with conjunctival involvement. Surgical volume reduction of the exterior parts of the lymphatic malformation were performed without any complications and satisfying outcome in these cases. Six patients suffered from intra- and periorbital lymphatic malformations. In 3 patients a watch-and-wait strategy was initiated. In the other 3 patients a surgical therapy was performed, one patient additionally received sclerotherapy with OK-432; however, these 3 patients suffered from residual lymphatic malformations.ConclusionThe presented cases underline the inconsistencies in the malformations behavior and underscore the inability to make specific recommendations regarding treatment. The treatment decision should be based on the size and location of the lymphatic malformation. The untreated patient must be watched for signs of visual detoriation, which may signal the need for therapeutic intervention.

Percutaneous Drainage and Ablation as First Line Therapy for Macrocystic and Microcystic Orbital Lymphatic Malformations

To review the management of orbital lymphangiomas and to propose a new treatment for both macrocystic and microcystic lymphatic malformations of the orbit. A retrospective case series of all patients from the authors' practice from 2001 to the present who met the histopathologic and/or diagnostic imaging criteria for orbital lymphatic malformation was reviewed. Lymphatic malformation was diagnosed if there was a multilobulated pattern on CT or a cystic internal structure on ultrasonography. In patients that were treated, macrocysts (>1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline injections were used for microcysts. The goal of treatment was complete cyst ablation documented by ultrasonography or MRI. Twenty patients met the inclusion criteria. They were separated in 3 groups based on the anatomical location of the lymphatic malformation: deep, superficial, or combined. Deep orbital lymphatic malformation presented in 14 patients (70%), superficial presented in 4 patients (20%), and both deep and superficial presented in 2 patients (10%). Thirteen of the 20 patients underwent percutaneous sclerotherapy. Of those treated, 7 patients (53.8%) had lymphatic malformations (LM), while 6 patients (46.2%) had venous-lymphatic malformations (VLMs). The average number of treatments required to achieve complete cyst ablation in patients with LM was 1.7. The average number of treatments required for patients with VLM was 3.0; however, some of these patients continue to have the venous component of their lesions treated. Clinically, all treated patients maintained or improved an average of one Snellen line (-0.16 decimal Snellen equivalent) from their preoperative visual acuity to their last recorded follow-up visit. There was a mean reduction in proptosis of 2.4 mm (p - 0.003, confidence interval [CI] 0.838 to 3.962), which was statistically significant. There were no recurrences (0%) in patients who completed treatment with cyst ablation (n - 8) at an average follow-up period of 43 months (range 6-96, standard deviation 30). There were no data available as to the recurrence status of one patient. Four patients were still undergoing treatment for a venous component at the time of this review. Percutaneous sclerotherapy provides a safe and effective treatment for both macrocystic and microcystic orbital lymphatic malformations as a primary treatment or for recurrence after surgical intervention.

Orbital lymphatic malformation showing the symptoms of orbital complications of acute rhinosinusitis in children: A report of 2 cases

Orbital lymphatic malformations are benign cystic malformations of the lymphatic system. The present report shows two cases with symptoms of orbital complications of acute rhinosinusitis with proptosis, compressive optic neuropathy, loss of vision and cellulites in children. Magnetic resonance imaging (MRI) revealed a well-demarcated intraorbital mass with heterogeneous signal conformable with lymphatic malformation in both cases. A tumor extirpation was performed via lateral orbitotomy in both cases. Postoperatively the symptoms and especially the loss of vision improved completely. Histological analysis of the surgical specimens verified lymphatic malformations of the orbit. Orbital lymphatic malformations can mimic the symptoms of orbital complications of acute rhinosinusitis. The existence of lymphatic malformation should be considered in every orbital complication of rhinosinusitis in children.

Direct Puncture and Sclerotherapy with Sotradecol®

We evaluated sclerotherapy in the treatment of orbital lymphatic malformations. Six consecutive patients with unilateral orbital cystic masses and recurrent episodes of orbital swelling were included in this retrospective study. All have been treated with percutaneous puncture and injection of Sotradecol (sodium tetredecyl sulphate) under radiographic guidance, on one or more occasions. Reduction of orbital mass volume was documented clinically and radiologically within a few weeks in all cases. There was total regression of proptosis in three instances. There were no immediate complications. One subject suffered a presumably coincidental orbital hemorrhage two weeks after treatment. Follow-up times ranged between six months and four years. Sotradecol sclerotherapy appears to be a useful adjunct to the therapeutic arsenal for orbitallymphatic malformations.