ARTERIAL EMBOLIZATION OF ORBITAL HEMANGIOMAS* BY Robert E. Kennedy, MD Angiography is the most accurate method for evaluating patients with an orbital arteriovenous malformation or vascular lesion. Theoretically, transcatheter arterial embolization is an ideal method of therapy to selectively obliterate the feeder vessels in the management of vascular lesions.1'2'3 Its use as a valuable adjunct to surgery, or possibly as an alternate definitive mode of treatment, warrants consideration in the field of ophthalmology. Embolization with various materials has been used quite extensively in other fields of medicine. The unique response to therapeutic transcatheter selective embolization as a valuable adjunct in the management of a patient with multiple orbital hemangiomas thus warrants discussion even though only a single case is presented. The potential for its more extensive use should be kept in mind. CASE REPORT

A 4-year-old girl was the smallest of a set of dizygotic twins with a birth weight of five pounds one ounce. At birth she was noted to have a large hemangioma on the left buttock which subsequently bled and required hospitalization. A small superficial hemangioma was present in the small of the back. A lesion in the right medial canthal region (Figure 1) was associated with lacrimal obstruction. She was seen by an ophthalmologist at four months of age. The lesion of the right medial canthal region caused exotropia and temporal displacement of the globe in addition to encroaching over the cornea. At nine months of age roentgenograms of the skull and orbit were interpreted as normal. Various methods of treatment were considered. Radiation therapy, injection of sclerosing agents, and carbon dioxide cryotherapy were not felt to be advisable. A course of systemic steroids produced no improvement. *From the Department of Ophthalmology of the University of Rochester School of Medicine and Dentistry, Rochester, New York. TR. AM. OPHTH. Soc., vol LXXVI, 1978

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FIGURE 1

Child at 4 months with right orbital hemangioma encroaching over comea. Dilated lid vessels.

The vascular lesion continued to enlarge, producing proptosis of the right eye with lateral displacement of the globe and encroaching over the nasal half of the cornea. Roentgenograms at 14 months showed enlargement of the right orbit (Figure 2). With this evidence of increased growth of the lesion, it was felt advisable to have further evaluation by angiography. Right internal and external angiograms demonstrated three areas of hemangioma of the right orbit with extension into the superior portion of the right antrum (Figure 3). These lesions derived blood supply from the right ophthalmic as well as from branches of the right external carotid artery (Figure 4). There appeared to be no intracranial extension of the vascular malformation. Because of the multiplicity of the lesions with many feeder vessels, it was felt that there was a significant risk in operating upon the patient at age 15 months. With an expanding lesion and probably an amblyopic eye, it was felt advisable to reduce the blood supply hoping to prevent further progression. Accordingly, the surgical and neuroradiological services performed the technique of selective transcatheter arterial embolization through the right internal maxillary and external carotid artery. Fifty or

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FIGURE 2

Roentgenogram of child at 14 months showing enlarged right orbit.

more 0.5 mm to 1 mm diameter silicone pellets were injected blocking the feeder vessels from this source (Figures 5). Interspersed with injection of the pellets, several carotid angiograms monitored the response which could be seen to successfully embolize the external carotid. The external carotid was then ligated at its origin from the common carotid. Five days later the neurosurgical service performed a right frontal craniotomy with clipping of the right ophthalmic artery. There was a noticeable reduction in the proptosis, size, and vascularity of the lesion. There was also less lid edema and a better lid fold (Figure 6). At age three and one-half years there was extensive resection of the hemangioma from the inner canthus and anterior orbital region performed by the ophthalmology service without incident. At age four and one-half years the child has a most satisfactory cosmetic result with straight eyes but vision limited to hand motions in the right eye. The left eye has normal vision (Figure 7). DISCUSSION

It behooves the ophthalmologist to realize the availability of this relatively new modality of treatment as a possible adjunct to

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FIGURE 3

A: Selective extemal carotid lateral subtraction view showing feeder vessels (arrow above) and large hemangioma masses (lower two arrows). B: External carotid anteroposterior subtraction view showing feeder vessels above (smaller arrows) and large hemangioma masses (lower arrows) also extending into antrum. C: Intemal carotid lateral subtraction view showing ophthalmic artery feeder vessel (small arrows) and large hemangioma mass (large arrow). D: Internal carotid anteroposterior subtraction view showing hemangioma masses.

surgery in handling various vascular lesions of the orbit. In some patients selective obliteration of the arterial supply may result in total amelioration of symptoms by embolization alone. Therapeutic embolization is a true interdisciplinary approach. Careful angiographic study is necessary before the efforts of an experienced surgical-neuroradiological team carries out the embolization procedure. Monitoring is necessary during the procedure to note the changing appearance of the lesion. The neurosurgeon, plastic surgeon, or ophthalmologist may be involved with additional surgery. Material used for embolization should be physiologically inert and would best be nonallergenic and nonabsorbable. Various materials have been used such as muscle fragments, fat fragments, au-

FIGURE 4

A: External carotid angiogram showing internal and external maxillary artery feeder vessels. B: Internal carotid angiogram showing ophthalmic artery feeder vessel (small arrows) to hemangioma masses (large arrows).

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FIGURE 5

A: Lateral external carotid angiogram at time of embolization with feeder vessels (small arrows) to hemangiomas (large arrows). B: Minutes later with arrows showing embolized vessels. c: Minutes later with arrows showing still more embolized vessels.

FIGURE 6

A: Patient with smaller lesion following embolization, carotid ligation, ophthalmic artery closure. B: Same, close up.

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FIGURE 7

A: Patient one year after ophthalmologic resection of orbital hemangioma. B: Same, close up.

togenous clots, silastic spheres, metallic pellets, gelfoam, liquid plastics, and other material. A radiopaque material would have the advantage for ease of monitoring during the embolization technique and for follow-up roentgenographic examination. It should be realized that arterial embolization is not without problems, such as thrombus formation at the site of introduction of the catheter and an inadvertent embolization of blood vessels supplying normal tissue. The over all morbidity, however, is low when properly performed. Some hemangiomas may be self limiting and even resolve or be amenable to orbital surgery. Others, however, may be more extensive and progressive, leading to cosmetic and functional deformity. Such lesions, if controllable at a young age, may spare the problems encountered in later life and avoid extensive and often mutilating dissection for their removal or control. Our patient has had general surgery with adjunct arterial embolization, neurosurgery, and ophthalmic surgery at a young age but appears to have a fine future. It was felt justified to close the ophthalmic artery as a main feeder to the hemangiomas because of the evidence of the progression of the lesion. It was anticipated that this patient, with a high degree of compound myopic astigmatism and anisometropia as is commonly seen in this type of orbital problem,4 together with the exotropia, was probably predisposed to an amblyopic right eye. Any loss of vision in the right eye was felt justified as compared to the potential problems of progression of such a lesion.

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FIGURE 8

A: A 17-year-old man with extensive right orbital hemangioma, which has grown and subsequently required extensive surgery. B: Close up of same, right eye. C: Roentgenogram enlarged right orbit, same patient.

A: A 45-year-old woman, left orbital hemangioma operated neurosurgically and ophthalmologically at age 10. Cosmetic deformity, amblyopic, repeat superficial bleeding episodes. B: Close up same, left eye.

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FIGURE 10

A: A 15-year-old girl with pulsating lesion below right eye, exophthalmos, enlarged orbit. B: Same, side view. C: Right lateral carotid angiography same patient. D: Right anteroposterior carotid angiography.

Less fortunate patients with orbital vascular lesions or arteriovenous malformations may show progression leaving them with cosmetic or functional deformity. This can even be life threatening with intracranial involvement. Figure 8 illustrates a patient with an orbital lesion with cosmetic deformity and an enlarged orbit which has required subsequent extensive surgery and tarsorrhaphy. Figure 9 demonstrates a patient with a cosmetic deformity, having had neurosurgical and ophthalmological procedures who still has external hemorrhage episodes. Figure 10 shows a patient with a pulsating lesion below the right globe, exophthalmos, and an enlarged orbit, with extensive intracranial arteriovenous communications demonstrated by angiography. The judgement ofthe ophthalmologist is taxed to decide whether such orbital lesions may be self limiting and not require extensive surgery. These last three patients illustrate the problems of control by any method. To avoid the extensive and often mutilating surgery

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for their removal, arterial embolization may offer an attractive alternative or adjunct method of management in selected patients. SUMMARY

A patient with multiple orbital hemangiomas benefitting from the adjunct treatment of transcatheter selective arterial embolization has been presented. The interdisciplinary team effort for this relatively new modality of treatment is called to the attention of the ophthalmologist for consideration in management with difficult or inoperable orbital vascular lesions or arteriovenous malformations with or without intracranial manifestations. ACKNOWLEDGEMENTS

The interdisciplinary approach demands thanks to my colleagues in the Department of Ophthalmology, Pediatrics, Surgery, Neurosurgery, Neuroradiology, and Ear, Nose and Throat at the University of Rochester, School of Medicine and Dentistry. REFERENCES

1. Djindjian R, Cophignon J, Theron J, et al: Embolization by superselective arteriography from the femoral route in neuroradiology. Review of 60 cases. Neuroradiology 6:20, 1973. 2. Djindjian R: Superselective internal carotid arteriography and embolization. Neuroradiology 9:145, 1975. 3. Hilal SK, Michelsen JW: Therapeutic percutaneous embolization for extra-axial vascular lesions of the head, neck, and spine. J Neurosurg 43:275, 1975. 4. Robb RM: Refractive errors associated with hemangiomas of the eyelids and orbit in infancy. Am J Ophthalmol 83:52, 1977. DISCUSSION

DR IRA SNOW JONES. Vascular malformations of the lid and orbit may be hemangiomas, lymphangiomas, venous angiomas, or arteriovenous fistulas. Most of these lesions suitable for embolization will be in the last group, but it is common for a shunt component to be present in any of the others. Delineation of the vascular mechanics is dependent upon internal and external carotid arteriography with subtraction and with magnified CT scanning. The internal maxillary and the ophthalmic arteries are the most common feeding vessels and the drainage may be posteriorly to the cavernous sinus or anteriorly to the face.

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Suitable regression can usually be obtained by arterial embolization exclusive of the ophthalmic artery. Sometimes, thermal thrombosis of the cavernous sinus, in those draining that way, will suffice. Embolization may be accomplished by catheter introduction of silicone beads alone, by beads plus a low-viscosity silicone polymer, or by the adhesive alone. The procedure is not without risk, and the indications for it are progression, threat to sight, or severe cosmetic defect. Embolization is a well-known and long-used procedure, but the refinement offered by the combination of the radiologist, the neurosurgeon, and the recently developed tools constitute a giant step forward. I enjoyed reading and hearing this paper. I congratulate Doctor Kennedy on bringing this to our attention. Thank you. DR JOHN S. CRAWFORD. Mr. President, I have been interested in hemangioma of the orbit for some time and we were reviewing these cases at the time that Doctor Richard Robb published his paper on the effects that result from lid hemangioma pressing on the globe (astigmatism and anisometropia amblyopia). We never really considered embolization until I came upon this one case. [Slide] This is the largest hemangioma that I think I've ever seen involving the lids and orbit. The father of this child was a teacher and he took a job in a private country school where they had large grounds and this child has actually never gone away from the school until we saw him. His mother wouldn't take him to the corner grocery store because people would stare at him. This is another view of it [Slide]. This is after embolization [Slide]. They used Gelfoam emboli and put about 60 of them in his maxillary, facial, and superficial temporal. You can see that it is considerably smaller. We were concerned about his vision which had become quite poor. I was just wondering if the author has any suggestions about further treatment? DR FREDERICK T. FRAUNFELDER. In any disease like this you're hunting for multiple modalities because no one modality works. We are very interested in cryotherapy and probably have treated almost 2,000 periocular and ocular lesions with cryosurgery. The kind of orbital hemangiomas we treat are the ones in which the eye has already been enucleated; but even so, the patient has the inability to close the lids and also has problems with bleeding. We treat these using a method developed by Doctor Andy Gage of Buffalo, New York. We take a 5 cc plastic syringe, cut off the hub, put this tightly over the orbital hemangioma and seal the edges to the conjunctiva with silicone grease. We then pour 1-2 cc of liquid nitrogen in the barrel and repeat as needed. You then obtain a very deep freeze. We have had some dramatic results in decreasing the mass and causing extensive scarring so as to prevent recurrent bleeding. If the eye is present, this would, of course, destroy the eye. If we had a periocular hemangioma that is superfi-

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cial, we might be able to control recurrent bleeding with liquid nitrogen spray. We would not get a significant reduction in the mass of the hemangioma. DR JOHN W. HENDERSON (ANN ARBOR). I would like to congratulate Doctor Kennedy on an excellent paper. I think it should be brought to our attention that this selective embolization is being done in other conditions which we as ophthalmologists should be aware of. Selective embolization is being done for a type of condition that is called arteriovenous dural shunts. These are cases that resemble in many ways an early carotidcavernous fistula and which can be successfully treated in this way. The first publication on this was by Newton & Hoyt from San Francisco in 1970 in Neuro-radiology, and I can recommend it to you. I happened to have a patient whom we sent there, with arteriovenous dural shunts. Embolization was done and the patient is now entirely normal. She was in her early 70's. If you have a patient with findings that resemble an early carotid-cavenous fistula, this is a treatment that can be offered without invasive surgery.

DR ROBERT E. KENNEDY. I would like to thank Doctor Ira Jones for his very kind remarks. As usual he is very gracious and I appreciate his kindness. Unfortunately, I have no suggestions regarding further treatment of Dr. Crawford's patient. In talking with Doctor Ira Jones earlier during this meeting he described some of their work. After treating this one particular little girl I have decided that I shall conclude my series with the one patient and refer any future similar patients to Doctor Jones. We have had very little experience with the progressive action mentioned by Doctor Fraunfelder since my experience has been limited to patients with the eye still in place and trying to preserve the globe. Doctor Henderson's remarks certainly show that the method has further application and that it should be kept in mind. I appreciate the opportunity to have presented this paper at this meeting, particularly on the occasion of Doctor Samuel McPherson being President of the Society. He was my mentor and chief resident in Baltimore 30 years ago.

Arterial embolization of orbital hemangiomas.

ARTERIAL EMBOLIZATION OF ORBITAL HEMANGIOMAS* BY Robert E. Kennedy, MD Angiography is the most accurate method for evaluating patients with an orbital...
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