Lam, Stacey C.; Yuen, Hunter K.L.
Purpose of review 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.
Recent findings 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.
Summary The development of new agents allows multimodal management either as monotherapy or combined therapy to achieve better outcomes in this difficult to manage disease.
Adam M. Hanif, Justin A. Saunders, C. Matthew Hawkins, Ted H. Wojno & Hee Joon Kim
Purpose: 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.
Hamzah Mustak FCOphth(SA) Shoaib Ugradar MD Robert Goldberg MD Daniel Rootman MD
No abstract available
Hirabayashi, Kristin E.; Kalin-Hajdu, Evan; Bever, Greg J.; Vagefi, M. Reza; de Alba Campomanes, Alejandra G.; Cooke, Daniel L.; Dowd, Christopher F.; Kersten, Robert C.
The authors describe, for the first time to their knowledge, a case of a congenital macrocystic lymphatic malformation of the orbit with associated venous stasis retinopathy that acutely normalized after drainage and sclerotherapy of the lesion. Prenatal ultrasound revealed prominence of the left orbital soft tissue, and at birth, the patient was noted to have unilateral proptosis, tortuous retinal vessels, and intraretinal hemorrhages in all 4 quadrants in the left eye. MRI demonstrated a primarily intraconal, multiloculated, T2-hyperintense mass consistent with a lymphatic malformation. Ultrasound-guided cyst aspiration and sclerotherapy was performed, with subsequent improvement of the proptosis and resolution of the vessel tortuosity and intraretinal hemorrhages. Although venous stasis retinopathy is usually related to central retinal vein occlusion or carotid artery occlusive disease, any entity that increases orbital venous resistance can generate retinal venous dilation and intraretinal hemorrhages, including an orbital lymphatic malformation.
Lally, Sara E.
Purpose of review:The purpose of this article is to review the recent published literature in 2015 on lymphatic malformation and provide updated information on the disease. Specifically, this article will describe recent advances in identifying and managing lymphatic malformations.
Recent findings:All articles discussed in this article emphasize the difficulty in treating lymphatic malformations. Most patients have long complicated histories with multiple recurrences. Studies are trying to identify which lesions will be successful treated by certain therapeutic modality. Treatment includes nonsurgical and surgical therapies.
Summary:Lymphatic malformations are difficult lesions to treat because they do not respect tissue planes. The use of sclerosing agents shows great promise for treating macrocysts. More knowledge is needed in the development of the disease process. Hopefully, antilymphangiogenic agent can provide targeted treatment.