Selective vascular embolization is currently a standard accepted procedure for the treatment of various conditions of the head and neck, including aneurysms, intractable epistaxis, and vascular tumors. Juvenile nasopharyngeal angioma (JNA) is a common benign tumor with a tendency to bleed profusely intraoperatively. Embolization of the external carotid artery branches before surgical resection in JNA has been shown to reduce intraoperative bleeding and decrease the chances of recurrence. Potential complications of preoperative embolization include stroke and blindness from occlusion of branches of ophthalmic artery. This report highlights the rare occurrence of hemi-central retinal artery occlusion (hemi-CRAO) following embolization in a case of recurrent JNA.
A 14-year-old boy, a known case of recurrent JNA, presented with a history of repeated episodes of nasal bleeding. He had undergone embolization and endoscopic tumor resection twice (May 2016 and February 2017) in another hospital. Both surgeries were uneventful. He was posted for repeat surgery in our hospital in October 2017 in view of recurrent epistaxis following two previous procedures. Pre-surgical embolization of the branches of right external carotid artery was done with polyvinyl alcohol particles to reduce the tumor vascularity. The patient complained blurring of vision in right eye after embolization procedure was completed. On immediate ophthalmic examination, pupils were found briskly reacting and fundus examination of both eyes was within normal limits. Six hours later, the patient noticed sudden-onset vision loss in his right eye. On examination, there was a relative afferent pupillary defect and visual acuity (VA) was noted to be 4/60 in the right eye. Fundus examination of the right eye showed whitening (edema) of the superior hemiretina, both nasal and temporal to the disc, at the posterior pole and along the superior arcade (Figure 1a). No embolus was identified in any of the retinal vessels. Left eye examination was within normal limits. There was no evidence of any neurological deficits. Based on the clinical examination, a diagnosis of hemi-CRAO was made. He was given immediate globe massage along with oral acetazolamide (Diamox Sequels; Teva Pharmaceuticals, Petah Tikva, Israel) and topical antiglaucoma medications to reduce intraocular pressure (IOP). Paracentesis could not be performed, as the child was not cooperative. No improvement in VA was noted. The next day, the patient's vision further deteriorated to 1/60. Visual field charting showed presence of inferior hemifield defect (Figure 1b). The head and neck angiograms were retrospectively analyzed, but no abnormal communications were noticed between the internal and external carotid artery systems. Parents of the child received explanation regarding this post-embolization complication, and on their consent, endoscopic resection of the tumor was carried out under general anesthesia. No surgical site complications were noted in the postoperative period, and the patient was discharged on fifth postoperative day. Thereafter, gradual improvement in vision was noticed by the patient, and on tenth day follow-up visit, vision was noted to be 6/6. Decrease in retinal whitening was noted at 4 weeks and 7 weeks postoperatively (Figures 1c and 1e), with improvement in field defect (Figures 1d and 1f).
(a) Fundus photo showing opacification of the right eye superior hemi-retina 3 days post-procedure. (b) Humphrey Visual Field (30-2) showing presence of inferior hemi-field defect in the right eye. (c, e) Fundus photos showing decrease in retinal whitening at 4 weeks and 7 weeks, respectively. (d, f) Visual fields showing improvement in hemi-field defect at 4 weeks and 7 weeks, respectively.
JNA is a highly vascular tumor with tendency to bleed profusely during surgical resection. Preoperative embolization of branches of external carotid artery is a commonly employed procedure to minimize intraoperative bleeding;1 however, embolization is not always safe and may have thromboembolic complications like cerebral infarcts and vision loss due to untoward migration of the embolizing material into other circulations, which are generally secondary to dangerous collaterals from the internal maxillary artery to the intracranial/intraorbital contents.2
Embolic occlusion of the central retinal artery leading to vision loss has been noted to occur during procedures like cardiac catheterization; radiologic procedures, including hysterosalpingography; arteriography; and interventional procedures.3 Occurrence of CRAO after embolization for JNA has been reported by various authors.2,4 Our patient developed hemi-CRAO after embolization for a recurrent nasopharyngeal angiofibroma. The occurrence of hemi-CRAO is rare, and reported causes include hypercoagulable state (hyperhomocysteinemia, hyperlipidemia, anticardiolipin antibody, polycythemia, thrombocytosis, protein S deficiency, and renal disorder), cardiac valvular defects, and isolated systemic hypertension.5 To the best of our knowledge, there are no reports of hemi-CRAO after any embolization procedure.
Presence of abnormal collaterals between the external and internal carotid systems has been presumed to be the cause of abnormal migration of the embolus towards ophthalmic artery causing CRAO. In our case, detailed retrospective analysis of the head and neck angiograms did not reveal any abnormal collateral between the two systems. Önerci et al. presented a patient with JNA who developed CRAO following preoperative embolization.6 They could not demonstrate any responsible communicating artery and therefore assumed the existence of a branch of the internal maxillary artery supplying the intraorbital contents and the retina in their case. Perhaps in our case also, similar small collaterals (shunts) might have become patent after embolization leading to migration of the embolus.
This report highlights a rare complication, hemi-CRAO following preoperative embolization of recurrent JNA. It also focuses on the necessity of careful detection of any abnormal collateral vessels prior to embolization. Despite a lack of visible communication between external and internal carotid circulation such a complication can occur. It is also important to inform patients regarding the rare possibility of vision loss from embolization procedures of head and neck.
- Deschler DG, Kaplan MJ, Boles R. Treatment of large juvenile nasopharyngeal angiofibroma treatment of large juvenile nasopharyngeal angiofibroma. Otolaryngol Neck Surg. 1992;106(3):278–282. doi:10.1177/019459989210600315 [CrossRef]
- Casasco A, Houdart E, Biondi A, et al. Major complications of percutaneous embolization of skull-base tumors. AJNR Am J Neuroradiol. 1999;20(1):179–181.
- Sanborn G. Arterial obstructive disease of the eye. In: Tasman W, Jaeger EA, eds. Duane's Clinical Ophthalmology [CD-ROM]. Philadelphia, PA: Lippincott Williams & Wilkins Publishers; 2006; chap 14.
- Lloyd G, Howard D, Phelps P, Cheesman A. Juvenile angiofibroma: The lessons of 20 years of modern imaging. J Laryngol Otol. 1999;113(2):127–134. doi:10.1017/S0022215100143373 [CrossRef]
- Ratra D, Dhupper M. Retinal arterial occlusions in the young: Systemic associations in Indian population. Indian J Ophthalmol. 2012;60(2):95–100. doi:10.4103/0301-4738.94049 [CrossRef]
- Onerci M, Gumus K, Cil B, Eldem B. A rare complication of embolization in juvenile nasopharyngeal angiofibroma. Int J Pediatr Otorhinolaryngol. 2005;69:423–428. doi:10.1016/j.ijporl.2004.10.015 [CrossRef]