Long term outcomes of transcutaneous non-image guided bleomycin sclerotherapy in orbital/adnexal lymphatic malformations: a protocol-based management in 69 eyes.

OBJECTIVES: To study the efficacy and the long-term outcomes of treating micro and macrocystic orbital and/or adnexal lymphatic malformations (OA-LM) with protocol-based bleomycin sclerotherapy. METHODS: A retrospective interventional study of 69 eyes having OA-LM treated with non-image guided transcutaneous or transconjunctival bleomycin sclerotherapy (1IU/ml aqueous solution) between December 2014 and December 2018. Based on clinical regression, the outcomes were classified as excellent, good, fair and poor. RESULTS: The mean age at presentation was 20 ± 16 years (median 16; range 1 month to 70 years). The orbital-palpebral variant was the most common presentation, seen in 29 patients (42%). Microcystic morphology was seen in 34(49%), macrocystic in 22 (32%) and mixed cyst in 13 (19%) patients. Mean units of bleomycin injected per patient were 9 ± 8 IU (median 5.5 IU, range 1-38 IU). Mean number of treatment sessions required were 2 ± 1 (median 2, range 1-6). The response was excellent in 43 (62%), good in 12 (17%), fair in 9 (13%) and poor in 5 (7%) patients. These responses were comparable across the morphological subgroups (p = 0.24, chi-square test). Adverse reactions noted were inflammation in 11 eyes (16%) and peri-ocular pigmentation in 15 (22%). There was a sustained tumour regression over a mean follow-up duration of 3.5 years (median 3; range 1.5-5 years). CONCLUSIONS: Seventy-nine percent of eyes with OA-LM showed a good outcome with transcutaneous and/or transconjunctival non-image guided bleomycin sclerotherapy with no serious adverse events. The results were promising over long-term follow-up.

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

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Solitary orbital myofibroma in a child: A rare case report with literature review.

Myofibroma is a rare benign mesenchymal tumor of uncertain histogenesis. A six-year-old boy presented with a unilateral lower eyelid mass of six weeks' duration. MRI revealed a circumscribed mass in the inferolateral orbit with bony erosion. A systemic examination was unremarkable. Excision with histopathology revealed a partially infiltrative spindle cell tumor with bland nuclear morphology expressing smooth muscle actin and muscle-specific actin, compatible with myofibroma. Solitary myofibroma is a rare childhood orbital tumor and may clinico-radiologically closely mimic a malignancy. Histopathology and immunohistochemistry can help reach a definitive diagnosis. Systemic evaluation and close follow up are crucial in such cases.