Case study: Orbital lymphangioma manifests postpartum
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Lymphangiomas, alternatively referred to as lymphatic malformations, are localized congenital benign lesions that affect the vascular and lymphatic systems and are characterized by multiple cysts.
Even though much of the literature considers them as hamartomatous lesions, evidence supports the notion that lymphangiomas should be classified as vascular malformations. The occurrence of orbital lymphangiomas in a tertiary practice was found to be 1% to 4% of all orbital lesions. The clinical manifestations include displacement of the eyeball, ptosis and limitation of eye movements. Moreover, a sudden increase in size can be triggered by intralesional hemorrhage, thrombosis or inflammation, resulting in acute proptosis and compression of the optic nerve. The diffuse nature of a lymphangioma and the associated bleeding make its management a challenging endeavor. As a result, attempts have been made to utilize nonsurgical techniques, including the application of intralesional sclerosing agents, as an alternative or complementary treatment option.
Figure 1b. Fundus image of right eye showing choroidal folds in the posterior pole.
Source: Dhivya Ashok Kumar, MD, FRCS, FICO, FAICO, Keziah Mary Thomas, MBBS, DNB, S. Balasubramanium, MD, and Amar Agarwal, MS, FRCS, FRCOphth
In this report, we present the management of an unusual case of lymphangioma in late pregnancy and the postpartum period.
Case summary
A 27-year-old woman arrived at our hospital with a sudden protrusion of her right eye, accompanied by a loss of vision that had been present for 5 days (Figure 1a). The patient was 3 months postpartum and had a history of peripartum systemic hypertension. There were no records of any other systemic illnesses, recent fevers, traumas or similar episodes in the past.
During the examination, the patient’s best corrected visual acuity was measured as 6/36 in the right eye and 6/6 in the left eye. Hertel exophthalmometry, with a 100 mm base, revealed measurements of 20 mm and 18 mm for the right and left eyes, respectively. Evaluation of the anterior segment of the right eye showed mild temporal chemosis and congestion. The pupil reacted to direct and consensual reflex. No relative afferent pupillary defect was noted. The remainder of the anterior segment examination showed no notable findings. Upon palpation, the retropulsion test yielded positive results in the right eye, but there was no definitive evidence of a palpable mass lesion. Posterior segment evaluation revealed choroidal folds in the posterior pole with a hyperemic optic disc with defined disc margins (Figure 1b). Left eye examination was within normal limits. On assessment, color vision with Ishihara test was within normal limits, and confrontation was also normal. On a follow-up visit, IOP of the right eye was found to be elevated with a Goldmann applanation tonometry value of 30 mm Hg. The patient was subsequently started on anti-glaucoma medications, which controlled the IOP.
The retrobulbar space displayed a well-defined cystic lesion indenting the globe on orbit ultrasonography. Induced hyperopia was noted in the right eye probably due to the indentation and elevation of the macula by the mass lesion (Figure 2). MRI of the right orbit revealed a well-defined cystic lesion with fluid-fluid level appearing hyperintense on T2/T1-weighted imaging with dependent layer showing isointensity to hypointensity intraconally, superotemporal to the optic nerve, measuring 1.5 cm × 2 cm × 1.9 cm and indenting the globe. A few smaller cysts were seen at the inferior part of the above-mentioned cyst. MRI of the brain was within normal limits. Color Doppler of the right orbit showed a well-circumscribed hypoechoic intraconal lesion temporal to the optic disc measuring 1.9 cm × 1.3 cm with low fine internal echoes, indenting the globe and displacing the optic nerve nasally. No flow was appreciated on Doppler study. A diagnosis of lymphangioma of the orbit was established based on clinical findings and investigations.
Ultrasound-guided intralesional bleomycin
The patient was scheduled for right orbital cyst aspiration using negative pressure and administration of intralesional bleomycin under general anesthesia. With sterile precautions in the operating room, under the guidance of ocular ultrasonography, a 23-gauge needle was gently inserted into the superolateral orbital wall, connected to a 5-mL syringe. By applying negative pressure, fluid was aspirated from the cyst. Approximately 3 mL of hemorrhagic fluid was successfully extracted. Subsequently, 1.5 mL of intralesional bleomycin with a concentration of 1.5 IU/mL was injected while maintaining the needle in place, with a syringe change (Figure 3). Using direct visualization on ultrasound with a 23-gauge needle, a dose of bleomycin along with lignocaine in a 4:1 ratio was injected. This treatment approach resulted in significant clinical improvement and only transient side effects.
The extracted fluid was then sent for cytological examination, which revealed altered red blood cells and the absence of malignant cells. Throughout the hours after the injection, pupillary responses were closely monitored. The patient was followed up every 1 week for 1 month and thereafter monthly for 6 months. Improvement in vision was noted with reduction in proptosis. BCVA improved from 6/36 to 6/6 in the right eye with reduction in hyperopic shift by 2 weeks. Follow-up ultrasound showed reduction in size of the lesion (Figure 4). Because there was improvement in vision and cosmesis was good, no further injection of bleomycin was advised. The plan is to keep the patient under regular follow-up and repeat bleomycin injection when indicated.
Lymphangioma and management
The symptoms manifested in our patient 3 months postpartum. To the best of our knowledge, this is the initial documentation of an orbital lymphangioma occurring during the postpartum period. The elevated levels of vascular proliferation factors, including VEGF, during pregnancy play a role in promoting the growth of congenital lymphangiomas, and coexisting minor injury could lead to acute hemorrhage within the lesion.
Bleomycin, described by Umezawa in 1966, is a glycopeptide derived from the soil fungus Streptomyces verticillus. This compound exhibits antitumor, antiviral and antibacterial properties. One of its primary biochemical effects is the cleavage of DNA strands through the release of free radicals upon oxidation of its metal core. Moreover, it has a sclerosing effect on the vascular endothelium, a property that has proven effective in the management of vascular anomalies and for chemical pleurodesis. Furthermore, it induces apoptosis in swiftly proliferating cells and promotes the secretion of tumor necrosis factor. Gooding and colleagues reported the efficacy and safety of bleomycin injection in treating refractory cases of lymphangioma.
Conclusion
Negative pressure aspiration and injection of bleomycin can be successfully used as the first management of vision-threatening intracystic bleeding associated with orbital lymphangiomas. To prevent inadvertent injury to the globe or optic nerve, it is imperative to utilize ultrasound guidance when inserting the needle into the cyst, particularly in cases like ours in which retrobulbar lymphangiomas cause compression of the globe and displacement of the optic nerve. The initiation of treatment in a timely fashion, within a few days of symptom appearance, can lead to the restoration of vision, and the time required to achieve presymptomatic vision is relatively shorter.
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- For more information:
- Amar Agarwal, MS, FRCS, FRCOphth, director of Dr. Agarwal’s Eye Hospital and Eye Research Centre, is the author of several books, including Phaco Nightmares: Conquering Cataract Catastrophes, Bimanual Phaco: Mastering the Phakonit/MICS Technique, Dry Eye: A Practical Guide to Ocular Surface Disorders and Stem Cell Surgery and Presbyopia: A Surgical Textbook. He can be reached at aehl19c@gmail.com; website: www.dragarwal.com.
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