Lucio De Maria, Paolo De Sanctis, Megha Tollefson, Samir Mardini, James A. Garrity, Pearse P. Morris, Emily Bendel, Waleed Brinjikji
Percutaneous sclerotherapy is an alternative strategy for the treatment of vascular malformations of the orbital and periorbital regions. The safety and efficacy of sclerotherapy in this setting have not been fully established. We present the results of a systematic review and meta-analysis examining the safety and efficacy of percutaneous sclerotherapy for the treatment of vascular malformations of the orbit. We searched PubMed, MEDLINE, and EMBASE from 2000 to 2018 for studies evaluating the safety and efficacy of percutaneous sclerotherapy for orbital and periorbital vascular malformations. Two independent reviewers selected studies and abstracted data. The primary outcome of this study is the efficacy of sclerotherapy which includes complete response, partial response, or no response to sclerotherapy. Data were analyzed using random-effects meta-analysis. Thirteen studies reporting on 154 patients were included. The rate of complete cure after percutaneous sclerotherapy was 54.9% (95% confidence interval [CI] = 34.2%-75.7%). The rate of emergent decompressive surgery was 3.4% (95% CI = 0.5%-6.2%), and the rate of vision loss was 2.7% (95% CI = 0.1%-5.3%). I2 values were above 50% for most outcomes indicating substantial heterogeneity. Our systematic review and meta-analysis of 13 studies and over 150 patients found that percutaneous sclerotherapy is a safe and effective treatment modality for the treatment of low-flow vascular malformations of the orbit.
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.
Mohammad Javed Ali, Tarjani Vivek Dave, Dilip Kumar Mishra & Milind N. Naik
Purpose: The aim of this study was to perform a histopathological assessment of the efficacy of sclerosing agents in dacryocystosclerotherapy (DCST) and to evaluate its role as an alternative to dacryocystectomy (DCT) in a specific group of patients.
Methods: Thirteen lacrimal drainage systems of 10 patients with primary acquired nasolacrimal duct obstruction (PANDO) were studied. All patients were awaiting a DCT, indications being repeated attacks of dacryocystitis with severe systemic or ocular co-morbidities and/or refusal for a dacryocystorhinostomy. Fluorescent labelled sodium tetradecyl sulphate (SDS) or bleomycin were used as sclerosing agents for a DCST taking specific precautions. All patients underwent a DCT after 4 weeks and histopathological evaluation was performed to assess the changes and extent of DCST.
Results: There were nine females and one male, and all patients were above the age of 60 years. Of the 13 lacrimal systems studied, the mean duration of PANDO was 1.3 years. All the patients had associated systemic and/or ocular co-morbidities. SDS and bleomycin were used in 6 and 7 lacrimal systems, respectively. The mean doses used for SDS and bleomycin were 0.26 and 0.31 cc, respectively. The post-injection phase was uneventful except for one patient who had a moderate inflammation, which resolved with low dose oral steroids. Histopathological features were more marked in the SDS group and included loss of epithelium, luminal closure, gross fibrosis across the lacrimal sac wall, congestion and hyalinization of blood vessels, and multiple areas of haemorrhages in the sac wall.
Conclusions: DCST is a safe and effective alternative to DCT in very carefully selected patients. Histopathology demonstrates SDS to be more effective as compared to bleomycin.
Ying Chen, Catherine J. Choi, Bradford W. Lee
An orbital varix is a distensible venous malformation consisting of vascular channels that directly communicate with the systemic venous system. It can present with variable pain, proptosis, diplopia, spontaneous hemorrhage, or even vision loss, and these symptoms typically worsen with Valsalva maneuver. Treatments proposed include vascular clipping, endovascular glue embolization, endovascular coiling, surgical resection, or combination therapy.1 Recurrence is common, however, and devastating visual complications can occur with procedural interventions such as surgical resection and endovascular procedures.2 Previous reports of using bleomycin sclerotherapy to treat vascular malformations have focused on lymphatic venous malformations, cavernous hemangiomas, and high- and low-flow orbital venous malformations.3, 4 There is only one previous case report of bleomycin sclerotherapy used to treat an orbital varix, and this case utilized angiography-guided catheterization through the cavernous sinus, a microcatheter balloon, and contrast-enhanced sclerotherapy mixed with bleomycin.5 Here the authors describe the first reported case of non-image-guided bleomycin sclerotherapy for treatment of an orbital varix.
Miltiadis Fiorentzis Periklis Katopodis Helen Kalirai Berthold Seitz Arne Viestenz Sarah E. Coupland
To investigate the cytotoxic effect of bleomycin, mitomycin C (MMC) and Fluorouracil (5‐FU) in combination with electroporation (EP) on human conjunctival melanoma (CM) and normal conjunctival cell lines using 2D and 3D cell culture systems in vitro.
Two CM (CRMM1, CRMM2) and one normal conjunctival epithelial cell line (HCjE‐Gi) were treated with various EP conditions and increasing concentrations of 5‐FU, MMC and bleomycin. Cell survival was assessed by MTT viability assay. All cell lines were seeded to create spheroids and were treated with bleomycin on day 3 and day 8 combined with EP. Spheroids were collected, fixed in buffered formalin and subsequently paraffin embedded for histological assessment of the effects of the treatment on cell viability.
CM cell lines were resistant to electroporation alone and showed a reduction in cell number only when treated with 1000 Volts/cm and 8 pulses. HCjE‐Gi cells showed higher sensitivity to electric pulses over 750 Volts/cm. MMC and 5‐FU demonstrated a higher cytotoxicity for the HCjE‐Gi cell line. The CM cell lines were resistant to MMC and 5‐FU. Bleomycin (1 μg/ml) alone had no significant effect on the HCjE‐Gi even when combined with EP conditions ≥750 Volts/cm. In contrast, it significantly (p ‐, paired t‐test) reduced cell viability in the CM cell lines. Spheroids treated with bleomycin and EP showed a reduction in tumour mass and proliferation rates after treatment.
Our in vitro study using 2D and 3D models indicates that the application of EP may effectively enhance chemotherapy with bleomycin in CM. This may offer new viable perspectives for CM treatment.
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.
Michelson, Sarah J.; Ciongoli, Marina R.; Elner, Victor M.; Kahana, Alon
Microphthalmia is defined by a globe axial length greater than or equal to 2 standard deviations below the age-adjusted mean and can occur as part of a broader syndrome. The presence of a colobomatous cyst with microphthalmia signifies failure of the embryonic neuroectodermal fissure to close appropriately during development of the globe, creating a protuberant globular appendage that inhibits normal growth and development of the eye itself. Cystic reaccumulation of fluid is common after aspiration or surgical removal. Here, the authors describe a case of a young boy with a colobomatous cyst who underwent eyelid-sparing orbital exenteration followed by reconstruction with absorbable gelatin sponge (Gelfoam, Pfizer, Inc.) and the chemotherapeutic agent bleomycin to promote scarring, achieving the equivalent of a biointegrated implant and facilitating satisfactory placement of an ocular prosthesis. A 2-year follow-up MRI revealed adequate volume in the posterior orbit.
Maya Hada, Anubhav Upadhyay, Kamlesh Khilnani, Nagendra Shekhawat
Orbital lymphangiomas are isolated, benign vascular malformations of childhood. We report a case of orbital lymphangioma with acute intralesional hemorrhage in a 4-year-old boy that was associated with ipsilateral persistent fetal vasculature and extraorbital vascular malformations. Complete resolution of orbital lesion was achieved with chocolate cyst aspiration and intralesional injection of bleomycin.
Hamzah Mustak FCOphth(SA) Shoaib Ugradar MD Robert Goldberg MD Daniel Rootman MD
No abstract available