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Orbital cavernous hemangioma of childhood

Maheshwari, Rajat MS; Thool, Alka MD

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Indian Journal of Ophthalmology: Jul–Aug 2007 - Volume 55 - Issue 4 - p 313-315
doi: 10.4103/0301-4738.33052
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Abstract

Most pediatric orbital tumors are benign with capillary hemangioma being the most common benign orbital tumor in children.1 Cavernous hemangiomas are the most common benign tumors in adults.234 Although a rudimentary lesion may be present at birth, cavernous hemangiomas do not usually become symptomatic until the third to fifth decade of life.2 This article reports a case of orbital cavernous hemangioma in a four-year-old girl.

Case Report

A four-year-old girl presented with painless protrusion of the left eye of three to four weeks duration in December 2002. On examination visual acuity was 20/30 in both eyes with picture chart. There was axial proptosis of the left eye [Figs. 1a and b]. Slit-lamp examination was within normal limits in both eyes. There was relative afferent pupillary defect in the left eye. Dilated fundus examination showed indentation on the retina medially. B-scan ultra sonography (USG) showed a low to moderate reflective well-encapsulated mass lesion arising from the medial rectus muscle and occupying the medial orbital space. The lesion measured 19.5 mm x 15.5 mm and was displacing the optic nerve laterally and also indenting the globe [Fig. 2a]. Computed tomography scan showed an extraconal well-defined hyperdense mass of size 27.4 mm x 13.9 mm x 18.4 mm in the superomedial compartment of the left orbit. The lesion showed mild contrast enhancement and was not seen separate from the medial rectus muscle [Fig. 2b].

Figure 1
Figure 1:
a-b. Preoperative photograph showing axial proptosis of the left eye
Figure 2a
Figure 2a:
B-Scan ultrasonogrpahy showing mild to moderate reflectivity from the lesion
Figure 2b
Figure 2b:
Computed tomography scan showing medially located tumor lying close to the medial rectus muscle, pushing the optic nerve laterally

The patient underwent excision of the tumor by medial orbitotomy [Fig. 3a]. Histopathology report confirmed it to be a cavernous hemangioma [Fig. 3b]. The postoperative period was uneventful. The patient again reported in September 2003 with a lesion anteriorly on the left eyelid; B-scan USG showed a well-encapsulated lesion anteriorly in the orbit measuring 13.5 mm. There was no posterior extension of the tumor. This time the lesion was removed by a skin incision over the lower lid crease and the histopathology report confirmed it to be a cavernous hemangioma. The postoperative period was uneventful [Figs. 4a and 4b].

Figure 3a
Figure 3a:
Gross specimen
Figure 3b
Figure 3b:
Histopathology showing large cavernous spaces filled with blood (Heomotoxylin and eosin stain, 40x)
Figure 4
Figure 4:
a-b. Postoperative photograph with absence of proptosis

Discussion

Vascular lesions of the orbit constitute approximately 10 to 15% of orbital tumors and are therefore among the more important causes of non-inflammatory proptosis.56 Cavernous hemangiomas are the most common benign orbital tumors of adults while capillary hemangioma is the most common orbital vascular tumor of childhood.12356 Hemangioma of infancy (capillary hemangioma) is entirely localized in the retrobulbar compartment in less then 10% of pediatric orbital vascular lesions.6

A variety of orbital tumors can occur in children with benign lesions being much more common than malignant ones.17 However, childhood tumors show variable presentations and sometime it is difficult to clinically differentiate benign from malignant lesions. The overall incidence of ophthalmic malignancy is greater during the first five years of life than during any subsequent age interval until the sixth decade of life. The differential diagnosis of orbital lesions in childhood is compounded by the fact that in young patients, both benign and malignant tumors often enlarge very rapidly, making them difficult to distinguish from one another, from infectious and inflammatory disorders and from trauma which occurs with high frequency in children and often without a reliable history. Furthermore, mild to moderate proptosis can be difficult to detect in an uncooperative child.

In our case the absence of trauma, relative short history of proptosis and proximity of lesion to the extraocular muscle in a child was clinically suggestive of malignancy. The main differential diagnoses in a child with proptosis include inflammatory/infectious lesion, structural lesion (dermoid cyst), vascular lesion (capillary hemangioma), lymphoproliferative disease, neurogenic tumor, mesenchymal tumor and metastatic carcinoma.17

Although cavernous hemangioma is a common benign tumor of adults, it should be considered in the differential diagnosis of a child with unilateral painless proptosis.

1. Liesegang TJ, Deutsch TA, Grand MG. Basic and clinical science course Am Acad Ophthalmol. 2002;6:341–50
2. Rodgers R, Grove ASAlbert DM, Jackobiec FA. Vascular lesions of the orbit Principles and practice of ophthalmology: Clinical practice. 2000;4 Philadelphia WB Saunders Company:3144–9
3. Henderson JW Orbital tumors. 19943rd ed New York Raven Press:95–100
4. Reese AB Tumors of the eye. 19763rd ed Hagerstown Harper and Row:264
5. Tucker SMTasman W, Jaeger EA. Vascular lesion of the orbit Duane's Clinical Ophthalmology. 1997;2 Lippincott-Raven:1–10
6. Spencer WH Ophthalmic pathology: An atlas and text. 1996;44th ed WB Saunders Company:2525–45
7. Kodsi SR, Shetlar DJ, Campbell RJ, Garrity JA, Bartley GB. A review of 340 Orbital tumors in Children during a 60 year period Am J Ophthalmol. 1994;117:177–82
Keywords:

Cavernous hemangioma; children orbit.

© 2007 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow