|Year : 2015 | Volume
| Issue : 2 | Page : 288-291
A rare case of arteriovenous malformation of the upper eyelid
Avinash Mishra1, Ruchi Kabra2, Somesh Aggarwal2, Vinod Kumar Baranwal3
1 Classified Specialist (Ophthalmology), Military Hospital Ahemdabad Cant; Hanuman Camp, Shahibag, Ahemdabad, India
2 M & J Western Regional Institute of Ophthalmology, Ahemdabad, India
3 Vitero-retinal Surgeon, M & J Western Regional Institute of Ophthalmology, Ahemdabad, Gujarat, India
|Date of Web Publication||16-Dec-2015|
Classified Specialist (Ophthalmology), Military Hospital, Ahemdabad Cant, Hanuman Camp, Shahibag, Ahemdabad, Gujarat
Source of Support: None, Conflict of Interest: None
Arteriovenous malformations (AVM's) are serious abnormalities that very rarely affect the eye. They are due to anomalous communications between the arterial and venous systems without any interposing capillaries. Therapeutic management of orbital AVM is not standardized, and it's most essential to strike a balance between spontaneous and iatrogenic risk of any visual loss. Management has to be very patient-specific and includes observation, embolization, surgical excision or a combined preoperative embolization followed by excision. Surgical intervention can also sometimes lead to life-threatening complications like excessive haemorrhage or cardiovascular instability and /or vision threatening complications like retinal vein occlusions. Here we describe an eyelid AVM in a young lady, with a relatively good vision in the affected eye .She was accurately diagnosed and finally after much deliberation involving multiple specialities was offered a combined embolization followed by complete surgical resection, however she declined any active intervention and is now on a regular follow-up
Keywords: Arteriovenous malformation, conservative management regular follow-up, upper eyelid
|How to cite this article:|
Mishra A, Kabra R, Aggarwal S, Baranwal VK. A rare case of arteriovenous malformation of the upper eyelid. Arch Med Health Sci 2015;3:288-91
| Introduction|| |
Arteriovenous malformations (AVMs) are serious abnormalities that rarely affect the eye.  Their treatment is usually by embolization and resection; however, in this, there always is an underlying risk to the patient's life and vision.  Here, we describe an eyelid AVM in a young lady, who was accurately diagnosed and finally after many deliberations involving multiple specialties was offered surgical management; however, she declined and was finally discharged without any active intervention and is now on a regular follow-up.
| Case Report|| |
A 25-year-old lady presented with complaints of a gradually progressive swelling on her right upper eyelid for the past 10 years. It was initially small and had enlarged slowly over the years. Earlier, elsewhere, she had been diagnosed as a case of chalazion and advised surgery (incision and curettage), however, she had refused. Subsequently when the swelling became noticeably bigger the patient finally reported to this center. During this intervening period, the patient took several courses of homeopathic/aryuvedic medicines, but without any improvement. Ocular examination revealed a 10 mm × 8 mm sized protuberant swelling on the medial part of the right upper lid, causing a mechanical ptosis extending up to the medial pupillary margin. Prominent veins were evident over its surface [Figure 1]a and b]. It was reducible on external pressure and on palpation a thrill could be appreciated. Valsalva manoeuvre did not cause an increase in its size. The left eyelid was normal. Her best-corrected visual acuity was 6/12 in the right and 6/6 in the left. The refractive error detected was-1.5 D cyl 70° in the right eye; however, there was no improvement in her vision with this correction, most probably due to amblyopia. The intraocular pressure was a normal 14 mm of hg both eyes, as measured with a noncontact tonometer. Dilated fundoscopy was normal. Magnetic resonance imaging brain and orbits revealed a rounded multilobulated lesion with multiple tortuous vascular channels [Figure 2]a and b]. A tentative diagnosis of a vascular lesion - Either an AVM or a venous varix was made. Since the expertise of managing this case was not available, she was referred to a higher tertiary center. There a multidisciplinary approach was adopted with the patient being evaluated by the neurosurgeon, intervention radiologist, reconstructive surgeon and an ophthalmologist. A digital subtraction angiography (DSA) of the external carotid artery (ECA) and internal carotid artery revealed an ill-defined AVM at the medial aspect of the right orbit with vascularity from branches of the right ECA, facial, superficial temporal, middle meningeal and ophthalmic arteries [Figure 3]a and b]. After weighing all the pros and cons, she was offered surgical management; however, at this point, the patient backed out and refused any kind of surgical/radiological intervention. She is presently being followed up on an outpatient basis, with instructions to report urgently in case of any complications like ulceration, bleeding, cardiac decompensation or visual disturbances occur.
|Figure 2: a (sagittal view) and b (axial view): Magnetic resonance imaging brain and orbits revealed a well-defi ned rounded multilobulated lesion in the upper eyelid and medial aspect of the right eye|
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| Discussion|| |
A wide variety of processes can produce space-occupying lesions in and around the orbit including neoplasms, vascular lesions, inflammatory diseases, congenital lesions, and infection. Vascular malformations (VM's) are congenital lesions involving arterial, venous, capillary, and lymphatic systems and considered as one of the most difficult diagnostic and therapeutic enigmas encountered in medicine. Clinical manifestations are extremely diverse and because of their rarity, diagnosis and management of these lesions by clinicians are limited. In our case too initially the patient was misdiagnosed as a case of chalazion and advised surgery, which if done, might have had catastrophic results. 
The most accepted classification of congenital VM's was described by Mulliken and Glowacki in 1982,  and later modified by the International Society for the Study of Vascular Anomalies. Wherein they are classified into simple forms which includes capillary, venous, lymphatic, AVM's and the complex-combined forms. Hemodynamically, they are further classified into three types [Table 1].  Type 1 (no flow) includes lymphangiomas or combined venous lymphatic malformations and have little connection to the vascular system. Type 2 (venous flow) lesions have a direct and rich communication with the venous system. The type 3 (arterial flow) lesions, are characterized by direct, antegrade, high flow through the lesion toward the venous side and includes the AVM's and the AV fistulas.  Our patient can thus be classified as a type 3 lesion.
|Table 1: Classification of the vascular malformations on the basis of their hemodynamics|
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AVM's are anomalous communications between arterial and venous systems without interposed capillaries. They are the result of an error of vascular development between the 4 th and 6 th weeks of gestation.  Usually noted at birth they grow progressively.  Enlargement is due to changes in pressure and flow, ectasia, shunting and collateral proliferation, rather than cellular proliferation. , Their exact incidence is unclear due to the asymptomatic course in many cases; however, the available data suggest around 140-500 cases/100,000, that is, 0.14-0.5% of overall population.  The anatomical structure of AVM's is known as nidus (from the Latin nidus and nidi, meaning nest). It is formed by one or several supplying arterioles together with drainage veins usually dilated and tangled.
They can occur in any soft tissue, bones, solid organs, or the central nervous system including the brain, spinal cord, and dura. Approximately 50% are located in the craniofacial region with those in the head and neck region being far less common than the intracranial AVMs. ,
Magnetic resonance imaging is considered as the gold standard for workup of a malformation  and we too evaluated our patient using it. Subsequently the patient was evaluated by a multidisplinary team , and selective angiography was carried out to characterize the nidus, flow pattern and the micro or macro AVM's, preceding any interventional therapy. 
Therapeutic management of orbital AVM is not standardized, and it's most essential to strike a balance between spontaneous and iatrogenic risk of visual loss. Management has to be very patient-specific and focus on eradication of the nidus rather than the feeders. It includes observation, embolization, surgical excision or a combined preoperative embolization followed by excision.  Due to the high flow rates and hyper vascularity of these lesions, surgery may also sometimes lead to life-threatening complications like excessive hemorrhage and/or cardiovascular instability.  Furthermore, improper treatment can exacerbate the lesions by stimulating growth and further complicating treatment. , Surgery performed alone that is, without preceding embolization may not have been ideal in our case, as has been proved by a large scale study which showed that surgical intervention could not obtain complete functional and aesthetic restoration in many orbital vascular anomalies (OVA's) specially those involving the upper lid as well as those (OVA's) whose dimensions were greater than one third of the orbit dimension.  Nowadays endovascular procedures are becoming less invasive but more effective and rapidly emerging as popular treatment options for cerebrovascular diseases and neoplasms of the head and neck.  Hence the procedure planned for this patient was a highly selective embolization (super-selective), using micro catheters being introduced into the distal feeding arteries very close to the nidus and followed by complete surgical resection within 48 h, as has been recommended by another large scale study. 
| Conclusion|| |
Orbital AVM's are rare lesions having an extremely diverse clinical presentation. In addition, due to their arterial nature, extensive bleeding typically occurs if any unsuspecting surgeon attempts its removal with simple excision or blunt dissection techniques, often with dire consequences. Once accurately diagnosed their treatment too is challenging and multifaceted, often requiring a combination of therapeutic modalities best managed by a team that regularly sees such cases.
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[Figure 1], [Figure 2], [Figure 3]