Successful en bloc resection with acceptable cosmesis of a right recurrent giant scalp-orbital AVM causing severe disfigurement: a case report

Sushil Krishna Shilpakar,Dipendra Kumar Shresha,Anjan Singh Karki,Gopal Sedain

doi:10.3126/jssn.v24i1.41003

Sushil Krishna Shilpakar, Dipendra Kumar Shresha + Show 2 more

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https://doi.org/10.3126/jssn.v24i1.41003

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Abstract

Scalp arteriovenous malformation (AVM) accounts for only 8.1% of all AVM cases. These lesions are made up of an abnormal fistulous tangle of tortuous dysmorphic network of vessels “vascular nidus” directly connecting between the feeding arteries and draining veins, without capillary connection, located within the incision subcutaneous layer. With time, these congenital lesions may evolve and enlarge and clinically manifest with variable features. The only effective method of preventing evolution of these malformations is to exclude the lesion completely from the circulation. Involvement of the orbit and face may cause severe facial disfigurement, proptosis, visual obstruction and even facial palsy. These lesions may be complicated by ulceration, infection and profuse bleeding. We present a rare case of 40-year-old female with a recurrent congenital scalp-orbital AVM causing proptosis, visual obstruction on the right eye with severe facial disfigurement. The lesion was successfully excised with acceptable cosmesis and removal of the visual obstruction. A brief literature review, imaging findings and the surgical techniques have been presented.

Highlights

Scalp arteriovenous malformation (AVM) presents as a small pulsatile mass; but with time, if untreated, it will progressively enlarge and spread to the entire scalp, giving a grotesque appearance and involves the craniofacial structures and skull bone and even extends intracranially, leading to seizures and hemorrhages
Scalp arteriovenous malformation (AVM) is a rare congenital entity, accounting for only 8.1% of all AVM cases.[1]
A 40-year-old housewife, from Western Nepal, presented to our Neurosurgical Out-patient Clinic with a large pulsatile grotesque mass over the right fronto-orbital region. She was very depressed; and she had visited numerous hospitals to have this lesion removed so that she could see with her ULJKWH\HDQGJHWULGRIWKHQRWLFHDEOHIDFLDOGLV¿JXUHPHQW She was born with a swelling in the right frontotemporal region, which over time increased in size progressively

Summary

Introduction

Scalp arteriovenous malformation (AVM) is a rare congenital entity, accounting for only 8.1% of all AVM cases.[1]. Scalp AVM presents as a small pulsatile mass; but with time, if untreated, it will progressively enlarge and spread to the entire scalp, giving a grotesque appearance and involves the craniofacial structures and skull bone and even extends intracranially, leading to seizures and hemorrhages. Management of these complex lesions may require multidisciplinary approach with preoperative embolization DQG VXUJLFDO H[FLVLRQ$V LW LV RIWHQ GL൶FXOW WR FXUH VFDOS AVM with embolization alone, surgical resection is usually required. She underwent surgical excision of right orbital region (Figure 1).

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