|Year : 2015 | Volume
| Issue : 2 | Page : 95-98
Intra-conal cavernous haemangioma orbit
Umesh U Harakuni, Shivanand B Patil, Umakant S Dandavatimath, Sohail N Kothare
Department of Ophthalmology, J.N.M.C., Dr. Prabhakar Kore Hospital, KLE University, Belgaum, Karnataka, India
|Date of Web Publication||14-May-2015|
Sohail N Kothare
Room No. 47, Charaka Hostel, J.N.M.C. Campus, Belgaum - 590 010, Karnataka
Source of Support: None, Conflict of Interest: None
Orbital cavernous haemangiomas are most frequent intra-conal, intra-orbital primary tumor in adults. They represent 4% of all orbit tumors and 9-13% of all intracranial cavernous haemangiomas. Forty-two-year-old female came with complaints of protrusion of right eye (RE) since 3 months associated with diminution of vision, headache, and eye ache. Absolute proptometry was 28 mm OD and 20 mm OS. 2 mm of the inferior scleral show was seen in RE. Axial computed tomography (CT) showed unilaterally, moderately enhancing, homogeneous, smooth-contoured mass. The patient underwent right lateral orbitotomy with mass excision under general anesthesia. The histopathological evaluation proved it to be a cavernous haemangioma. We report a case of intra-orbital, intra-conal cavernous hemangioma occurring in a 42-year-old female presenting with proptosis. The tumor was diagnosed on CT and was removed using lateral orbitotomy approach. Patient had significant improvement in symptoms with the mild deterioration of vision.
Keywords: Haemangioma, intra-conal, orbit
|How to cite this article:|
Harakuni UU, Patil SB, Dandavatimath US, Kothare SN. Intra-conal cavernous haemangioma orbit. J Sci Soc 2015;42:95-8
| Introduction|| |
Orbital cavernous haemangiomas are the most frequent intra-conal, intra-orbital primary tumor in adults. They represent 4% of all orbital tumors and 9-13% of all intra-cranial cavernous haemangiomas.
Most of these tumors are unilateral and can increase intra-orbital volume with a resultant mass effect. Visual acuity or field compromise, diplopia, and extraocular muscle or pupillary dysfunction can result from compression of intra-orbital contents. Because they often occur in the muscle cone, they usually produce slowly progressive axial proptosis.
| Case report|| |
A 42-year-old female, came to the ophthalmology department with complaints of protrusion of right eye (RE) since 3 months that were insidious in onset, progressive in nature and painless [Figure 1]. She gave a history of spontaneous remissions and recurrences at intervals of 15 days. She also complained of diminution of vision in RE since 2 months that were gradual, painless and progressive. Headache and eye ache were present since 2 months. Headache was throbbing, intermittent in nature, more during morning hours and associated with protrusion of eyes. No history diplopia, painful eye movements, intolerance to heat/cold, palpitations, previous eye infections, ear nose throat infections.
On ocular examination, the best corrected visual acuity was 6/60 RE and 6/9 left eye (LE). Pupils were 3 mm in size, sluggishly reactive to light. Examination of ocular motility revealed the full range of movements in all directions of gaze. Colour vision was normal. Absolute proptometry from the lateral orbital rim was 28 mm RE and 20 mm LE. External examination revealed 2 mm of the inferior scleral show on the right. Prominent insertions of horizontal rectus muscles were noted in the RE. No orbital mass was palpable. Lid lag was present. Slit-lamp biomicroscopy, applanation tonometry, automated visual fields, and fundus examination were normal [Figure 2].
1-5 mm axial computed tomography (CT) views of both orbits, with coronal reconstructions, were obtained with and without contrast. Unilateral, moderately enhancing, homogeneous, smooth-contoured mass was seen [Figure 3]. The right-sided lesion was ovoid, displaced the globe anteriorly, displaced the optic nerve nasally measuring around 18.6 mm × 23.4 mm × 12.5 mm [Figure 4].
|Figure 4: Saggital view of computed tomography showing mass displacing the globe anteriorly|
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For the above, patient underwent right lateral orbitotomy with mass excision under general anesthesia. Mass was identified medial to lateral rectus and gentle dissection done with it, taking care not to handle optic nerve [Figure 5] [Figure 6] [Figure 7] [Figure 8]. It was removed in two pieces and was sent for histopathological evaluation [Figure 9]. Histologically this proved to be a cavernous haemangioma. Ocular movements and proptosis returned to normal postoperatively.
|Figure 5: Skin incision extending from tragus through vertex to 5 cm above the glabella was taken|
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|Figure 6: Skin reflected, fascia, periosteum and temporalis muscle dissected|
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|Figure 7: Zygomatic bone was sawed above, at the zygomaticofrontal and below, at the zygomaticomaxillary|
sutures. Lateral orbital rim was removed
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|Figure 8: Mass identifi ed and gentle dissection done around it, taking care not to handle optic nerve. The mass was removed in two pieces|
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| Discussion|| |
Cavernous haemangiomas are the most common benign primary tumor of the orbit in adults.  They are seen more frequently in the female, with a peak incidence in fifth decade of life.  Di Tommaso postulated that the expression of progesterone receptors in epithelial cells of orbital cavernous haemangiomas could explain the high proportion of female patients.  The incidental diagnosis of an orbital cavernous haemangioma does not necessarily mandate its removal. Surgical treatment is indicated only in symptomatic patients. 
Painless exophthalmos with a slow onset is the most frequent clinical sign and often remains initially unnoticed by the patient. Delayed noticing of proptosis is probably related to its slow onset and progression.  Motility disorders and compression of the optic nerve are often limited to large and apically located lesions. Acute clinical onset and sudden deterioration of symptomatology are exceedingly rare because, commonly, orbital cavernous haemangiomas have no tendency to cause hemorrhages.  While restricted extraocular motility and proptosis caused by mass effect usually disappear after removal of the tumor, decreased visual acuity and visual field defects are a result of compression of optic nerve or its vascular supply, and, therefore, may persist after a successful intervention.  Imaging studies conducted by CT and magnetic resonance imaging demonstrate a well-described, round to oval mass with mild enhancement with contrast agents.
Management ranges from the periodic observation for small, asymptomatic lesions to surgical excision for larger symptomatic tumors. The choice of surgical approach is determined by the size and location of the lesion. There are three major types of surgical approaches to orbital tumors. The anterior approach is the choice when the lesions are located anterior in the orbital space or basal to the optic nerve. The transcranial route is used for all tumors with intracranial extension, for tumors located in the orbital apex and/or invading the optic canal, and for tumors located in the deep medial orbital compartment. The lateral approach is used for tumors located in the superior, lateral, or inferior compartment of the orbit. The lateral orbitotomy, extended to superior orbital fissure and anterior temporal dura, can provide access to the deep lateral portion of the muscle cone. Several microsurgical or endoscopic approaches have been reported in the literature to approach lesions located in infero-medial aspects of orbit. 
The most serious complication of orbital cavernous haemangiomas surgery is blindness as a result of damage to the optic nerve. It can be related to a direct damage, traction of the nerve or lesion of its vascular supply. The optic nerve decompression, in particular for tumors located in the orbital apex and tight relationship with the optic nerve, is useful to reduce the intraoperative traction of the nerve during tumor dissection. After tumor removal, the superior and lateral orbital walls with the orbital rim are fixed in place using titanium microplates and screws. This reconstruction prevents enophthalmos and orbital pulsation and assures an optimal cosmetic result. 
| Conclusion|| |
In our case, we report a case of intra-orbital, intra-conal cavernous hemangioma occurring in a 42-year-old female presenting with proptosis. The tumor was diagnosed on CT and was removed using lateral orbitotomy approach. Patient had significant improvement in symptoms with a mild deterioration of vision.
Visual outcome in the surgical treatment of orbital cavernous haemangiomas is affected by the choice of the proper surgical approach according to the location of the lesion and by the tumor dissection technique.
Visual function and cosmetic result are the main parameters to evaluate the clinical and functional outcome and play an important role in the determination of patients' quality of life after surgery.
| Acknowledgements|| |
We express our sincere thanks to Department of Ophthalmology for guidance and encouragement pertaining to the case and its outcome.
We also would like to express our heartfelt thanks to Department of Radiology for helping in the diagnosis of the case.
We are thankful to Dr. Lingaraj, Professor, Department of Oral Maxillofacial Surgery, for assisting us in surgical removal of the lesion.
| References|| |
Alfred PR, Char DH. Cavernous hemangiomas of the orbit. Orbit 1996;15:59-66.
Henderson JW. Orbital Tumors. 2 nd
ed. New York: Thieme Stratton; 1980. p. 115-76.
Di Tommaso L, Scarpellini F, Salvi F, Ragazzini T, Foschini MP. Progesterone receptor expression in orbital cavernous hemangiomas. Virchows Arch 2000;436:284-8.
Boari N, Gagliardi F, Castellazzi P, Mortini P. Surgical treatment of orbital cavernomas: Clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien) 2011;153:491-8.
Acciarri N, Giulioni M, Padovani R, Gaist G, Pozzati E, Acciarri R. Orbital cavernous angiomas: Surgical experience on a series of 13 cases. J Neurosurg Sci 1995;39:203-9.
Brusati R, Goisis M, Biglioli F, M Guareschi, Nucci P, Gianni AB, et al.
Surgical approaches to cavernous haemangiomas of the orbit. Br J Oral Maxillofac Surg 2007;45:457-62.
Schick U, Dott U, Hassler W. Surgical treatment of orbital cavernomas. Surg Neurol 2003;60:234-44.
Maroon JC, Kennerdell JS. Surgical approaches to the orbit. Indications and techniques. J Neurosurg 1984;60:1226-35.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]