An Analysis of Thirty Cases of Orbital Lymphangioma: Pathophysiologic Considerations and Management Recommendations

doi:10.1016/S0161-6420(90)32370-9

Ophthalmology

Volume 97, Issue 12, December 1990, Pages 1583-1592

https://doi.org/10.1016/S0161-6420(90)32370-9 Get rights and content

Abstract

Thirty cases of orbital lymphangioma were reviewed. Clinical, imaging, and microscopic findings were integrated to develop a pathophysiologic construct and management guidelines. The basic lesion might be considered an abortive vascular system which arborizes among normal structures. Intrinsic hemorrhage expands portions of the small-caliber network into large blood cysts, prompting clinical recognition. While major hemorrhage led to early surgery in 12 cases, long pretreatment intervals could be analyzed in 17 others. Twelve patients had second hemorrhages of varied magnitude, from 2 weeks to 15 years after initial recognition; five patients did not in an average of 6.8 years. Some blood cysts contracted spontaneously. Among 23 operated cases, 12 patients had major new bleeds from 4 days to 12 years after initial surgery; 11 patients did not in an average of 4.2 years. Poor final visual acuity was associated with multiple surgeries. The authors advocate conservatism in surgical case selection and in operative dissection.

References (17)

IS Jones
Lymphangiomas of the ocular adnexa: an analysis of sixty-two cases
Am J Ophthalmol
(1961)
WJ Iliff et al.
Orbital lymphangiomas
Ophthalmology
(1979)
J Rootman et al.
Orbital-adnexal lymphangiomas: a spectrum of hemodynamically isolated vascular hamartomas
Ophthalmology
(1986)
ME Wilson et al.
Conservative management of childhood orbital lymphangioma
Ophthalmology
(1989)
JS Kennerdell et al.
Surgical management of orbital lymphangioma with the carbon dioxide laser
Am J Ophthalmol
(1986)
JW Henderson
IS Jones et al.
Management of orbital neurofibromatosis and lymphangiomas
DH Nicholson et al.

There are more references available in the full text version of this article.

Cited by (111)

Clinical and radiographic features of hydrolyzed MIRAgel scleral buckles: A comparative analysis
2020, Clinical Imaging
The MIRAgel (hydrogel) scleral buckle, introduced in the 1980s, was a novel material to repair retinal detachments. It was later discontinued due to the frequency of long-term complications related to buckle hydrolysis and expansion. These complications included pain, limited extraocular motility, and more serious complications such as infection or scleral perforation, which ultimately necessitated surgical extraction as late as 20–30 years after placement. Prompt and proper diagnosis and treatment is often delayed as these buckle-associated complications frequently mimic other orbital pathologies such as tumors or infections. The hydrolyzed MIRAgel buckle exhibits distinct radiographic features that are helpful in arriving at the correct diagnosis, particularly in cases of ambiguous clinical presentation or history. Here, we expand on the previously described radiographic features of hydrolyzed MIRAgel and compare them to features of common, mimicking orbital pathology.
Orbital cystic lymphangioma in a child: Case report
2019, Journal Francais d'Ophtalmologie
A prospective study of the role of intralesional bleomycin in orbital lymphangioma
2017, Journal of AAPOS
To evaluate the efficacy of intralesional bleomycin injection in the management of lymphangiomas of the orbit.
This prospective noncomparative interventional case study included 13 patients with orbital lymphangiomas. Reconstituted bleomycin 1–5 ml (0.5 IU/kg body weight; maximum, 15 IU/ml) was injected with 2% lignocaine in the lesion as seen on imaging or, in deeper lesions, under ultrasound guidance. Repeat injections were administered when required after every 4 weeks. The decision to retreat was based on clinical and radiological evidence of response.
Patients were treated with 1-6 injections of bleomycin 0.5 IU/kg body weight. Dramatic response was achieved in all cases. During the mean follow-up period of 19.69 months (range, 7-26 months) none of the patients experienced recurrence or significant complication.
In this patient cohort lymphangiomas of the orbit showed favorable and promising results with intralesional injections of bleomycin. This treatment should be considered as a first-line therapy for lymphangiomas of the orbit.
Lid and orbital infantile periocular hemangiomas (capillary hemangiomas) and other vascular diseases
2016, Taylor and Hoyt's Pediatric Ophthalmology and Strabismus, Fifth Edition
Orbital lesions with low signal intensity on T2-weighted imaging
2016, Clinical Radiology
The purpose of the present review is to discuss and display orbital lesions that demonstrate hypo-intense signal on T2-weighted images (T2WI). The physical basis for hypo-intense signal on T2WI produced by various substances is discussed. Orbital lesions that are hypo-intense on T2WI are subsequently reviewed, including a discussion on their composition as well as relevant clinical and imaging clues that may aid in their diagnosis.
Orbital lymphaticovenous malformations: Current and future treatments
2015, Survey of Ophthalmology
Orbital lymphaticovenous malformations consist of abnormal vascular channels lined by endothelial cells with a spectrum from venous to lymphatic characteristics. They may be venous-dominant or lymphatic-dominant. These lesions continue to present management challenges. Total excision or obliteration is not always achievable, recrudescence is common, and interventions carry a risk of damaging normal structures. Patients likely benefit most from a multidisciplinary approach, including both surgical and nonsurgical (e.g., sclerosants and liquid polymers) therapeutic modalities. Targeted biologic therapy would be ideal; nevertheless, this goal is complicated by the heterogeneous venous–lymphatic and stromal characteristics of these lesions. Ideally, antiangiogenic agents targeting both lymphatic and blood vascular endothelial cells will be developed to treat these lesions and reduce their regrowth. Further studies are warranted to enhance our understanding of these orbital lesions with regard to their angiogenic (proliferative) activities and profiles of marker expression, with a goal to produce effective medical therapies.

View all citing articles on Scopus

: Presented in part at the 19th Annual Scientific Symposium of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Las Vegas, October 1988.

: Supported in part by an unrestricted department grant from Research to Prevent Blindness, Inc, New York, New York, and NEI core grant EY01931.

View full text

An Analysis of Thirty Cases of Orbital Lymphangioma: Pathophysiologic Considerations and Management Recommendations

Abstract

Am J Ophthalmol

Ophthalmology

Ophthalmology

Ophthalmology

Am J Ophthalmol

Management of orbital neurofibromatosis and lymphangiomas