Missed feeder vessel induced recurrent and refractory arteriovenous vascular malformation in the upper eye lid

Abstract

A 25-year-old male presented with recurrent swelling in the left upper eyelid despite multiple surgeries in the past 6 years. He was initially misdiagnosed as capillary hemangioma elsewhere, for which multiple surgical excision procedures were done. In 2020, he presented to us for a second opinion regarding the same painless swelling which recurred at the same site. Clinical examination and Doppler ultrasound shifted the diagnosis in favor of arteriovenous malformation (AVM). Computed tomography of the brain and cerebral angiogram (three-dimensional reconstructed) images confirmed the AVM in the left upper eyelid with an arterial feeder from the left ophthalmic artery branch. The patient had multiple recurrences due to the inadvertent misdiagnosis and missed management of the feeder vessel from the ophthalmic artery. We highlight this case, to bring to light the deceptive diagnosis and management of recurrent and refractory AVM due to a feeder vessel from the ophthalmic artery.

A 25-year-old male presented with chief complaints of painless recurrent swelling in the left upper eyelid for the past 6 years despite multiple surgical excisions. When the history of presenting illness was elicited, he revealed that he was apparently normal till 2014, when he developed an insidious swelling in the left upper lid, which was misdiagnosed as capillary hemangioma elsewhere, for which surgical excision was performed. Similarly, 3 years later in 2017, a similar episode occurred at the same site for which surgical excision was performed, at the same center once again, in view of the misdiagnosed recurrent capillary hemangioma, only for the swelling to recur again in 2020, when he presented to us for a second opinion.

On examination, the best-corrected visual acuity in both eyes was 20/20. The swelling was multi-lobular, soft and exhibited occasional color changes to a darkish hue with severe mechanical ptosis, demonstrating postural variability [Figure 1]. The lesion was compressible with spontaneous refilling [Figure 2]. Provisionally, a re-diagnosis of arteriovenous malformation (AVM) was made after performing a Doppler ultrasound, which revealed a hypo-echoic lesion measuring 6 × 5 mm. Computed tomography of the brain (axial section) and cerebral angiogram (three-dimensional reconstructed) images revealed a mildly enhanced lesion, confirming the diagnosis of AVM with an arterial feeder from the left ophthalmic artery branch [Figures 3 and 4].

Figure 1: (a) A multilobular soft swelling over the left upper lid causing severe mechanical ptosis. (b) Reduction in the size of the swelling was noted on lying down

Figure 2: (a-c) Demonstration of the compressibility of the lesion

Figure 3: Axial section of computed tomography of the brain revealing mildly enhanced lesion in the left upper eyelid

Figure 4: (a-c) Cerebral angiogram (three-dimensional reconstructed) images revealing feeder vessel (yellow arrows) arising from the left ophthalmic artery branch (red arrows)

The current management options for vascular malformations are based on their hemodynamic characteristics, vascular anatomy, and location of these lesions.^[1] The management includes observation, embolization, surgical excision or a combined preoperative embolization followed by excision. Preoperative embolization closes off surgically inaccessible arterial feeders, thus reducing intraoperative blood loss, aiding in complete surgical resection, and preventing recurrence.^[2–4] This patient was advised endovascular embolization with surgical excision due to its refractory nature.

CONCLUSION

Appropriate/adequate hemodynamic work up is recommended. Proper diagnosis, management and looking out for a feeder vessel in these type of lesions are called for.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.