A Fix for a Scalp Varix! A Rare Case of Cirsoid Aneurysm in a Child.

Cirsoid aneurysm (CA) is a rare arteriovenous fistula of the scalp. There exists scant literature on the incidence and approach to CA in children. We describe a case of CA in a 7-year-old boy which was diagnosed by angiography and managed with angiographic embolization followed by surgical excision. Copyright:

INTRODUCTION

Cirsoid aneurysm (CA) is a rare arteriovenous fistula (AVF) of the scalp, with few cases described in the pediatric population. Cirsoid derives its roots from the Greek word “kirsos” meaning varix, owing to its serpentine appearance. They present as pulsatile subcutaneous lesions which progressively enlarge following a spontaneous or traumatic onset. Diagnosed by angiography, they can be managed by various modalities of interventions either alone or in combination such as embolization and surgery. Here, we describe our experience of combination of endovascular embolization and surgical excision and repair for the management of CA in a child along with a literature review.

CASE REPORT

A 7-year-old boy presented with a swelling over the right parietal aspect of the scalp for the past 4 years [Figure 1]. The parents initially noticed a reddish, lemon-sized swelling over the right side of the head at 3 years of age which progressively increased in size. The child did not have a history of headache, discharge, bleed, tinnitus, swellings elsewhere, or head trauma.

Figure 1

Postoperative (left) and preoperative (right)

Examination revealed a single 6 cm × 5 cm nontender, compressible, pulsatile swelling over the right parietal bone located in the subcutaneous plane. It was soft in consistency and was not mobile. The overlying skin was thinned out. The swelling decreased in size on application of pressure over the superficial temporal artery. A bruit was appreciated over the lesion on auscultation.

Contrast-enhanced magnetic resonance imaging (MRI) of the head revealed a right temporal AVF. Digital subtraction angiography confirmed right temporoparietal CA with feeding vessels from the right frontal and parietal branches of the superficial temporal artery and right and left occipital arteries with no intracranial connection [Figure 2].

Figure 2

Glue embolization of the feeding vessels. Right external carotid artery. Image show pre-embolization arterial supply – branches of external carotid artery and venous drainage – extracranial veins

The CA was subjected to transarterial embolization with glue under computed tomography angiographic guidance [Figure 2].

Following embolization, the swelling acquired variegated consistency and the skin over the inferior aspect was indurated.

Surgical excision and repair of the scalp defect were done 2 weeks after the embolization. The approach included a circumferential excision of aneurysmal scalp in toto. Skin defect was closed with curvilinear Z-plasty technique which reduced the defect size to 1.5 cm2. The remnant defect was allowed to heal by the second intention. Compressive head bandage was applied postoperatively to keep pressure over the scalp.

Postoperative recovery was uneventful, and there is no evidence of recurrence at 2 years of follow-up with a cosmetic scar and absence of residual lesion [Figure 1].

DISCUSSION

CA is a rare AVF of the scalp with about 200 cases described worldwide and scanty literature in the pediatric age group. About six cases have been described in the pediatric population so far.[1] The term CA was applied to vascular malformations of the scalp in 1833 by Brecht.

Etiologically, they are classified into spontaneous (congenital), traumatic, and iatrogenic. The possible hypothesis, for their origin, includes vascular hamartoma, persistent embryonic arteriovenous communication with associated capillary agenesis, or spontaneous fistulization at arteriovenous crossings.[2]

Patients present with a pulsatile swelling on the scalp or headache, occasionally tinnitus, and complications such as bleeding and ulceration. Most have a spontaneous onset while some lesions are preceded by trauma. CAs are more common in males and occur most frequently over the right frontal followed by temporal regions, with superficial temporal artery being the most common feeding vessel.[1]

Angiography is essential for diagnosis and planning for therapy of CA by delineating lesion, its extent, and feeding vessels. MRI helps to rule out intracranial communication. Catheter angiography also adds therapeutic benefit at the time of diagnosis. Magnetic resonance angiographic studies are comparable to delineate anatomy with advantage of being noninvasive.[2]

Therapeutic options available for cirsoid AVF include embolization, surgery, or both. Embolization can ensure a decrease in the size of lesion and thereby ameliorate associated complications, but it cannot offer complete cosmetic relief and has a recurrence rate of about 8.3%. Surgery, on the other hand, still remains the mainstay of treatment, leading to complete obliteration of lesion and its complications. Various reconstructive procedures ensure good cosmetic outcomes and prevent scalp buttonholes. Surgical excision ensures low recurrence rates (6.3%). An important aspect of surgery is vascular control, which is achieved either preoperatively or intraoperatively by endovascular embolization, percutaneous embolization, proximal arterial occlusions, and percutaneous ligation of feeding vessels executed as a single or staged procedure. Multimodality approaches have shown near-zero recurrence rates.[2]

Endovascular interventions include placement of glues, thrombogenic coils, radiopaque gel foam either in the fistula or feeding arteries through transarterial or transvenous routes. Other options include intralesional injection of sclerosing agents such as sodium tetradecyl sulfate, absolute alcohol, and thrombogenic coils. This technique often results in reduction in size and vascularity of the lesions but may be complicated by remnant mass being esthetically unpleasant, painful, overlying hair loss, multiple aneurysm formation, migration of embolic material, and higher recurrence rate.[3]

Successful surgical treatment dates back to 1930 when Searby excised a CA by two-staged surgery. Basic steps of surgery include incising the scalp in short segments with meticulous hemostasis and raising scalp flaps (including pericranium). Feeding vessels are ligated followed by excision of AVF.[4] Vascular control to shrink the lesion and reduce blood loss can be achieved with preoperative endovascular embolization or with operative pressure bandage application, percutaneous ligation,[5] or injection of sclerosants into feeding vessels,[1] or temporary occlusion of external carotid or superficial temporal arteries. Reconstruction depends on the preservation of vascularity of the scalp tissue after excision of CA and its feeding vessels. Several flaps have been described to approximate the scalp defect with good cosmetic outcomes. Management of CA requires preparedness for dealing with transfusion requiring blood loss, well-equipped armamentarium to achieve vascular control, and innovation techniques to reconstruct the defect in the scalp.

CAs are rare vascular fistulae which require thorough understanding of involved anatomy and multimodality therapy to achieve good cosmetic and functional outcomes. Here, we describe a technique of excision of CA along with its overlying skin en masse postintra-arterial embolization. Reconstruction was achieved by curvilinear Z-plasty to achieve near-complete closure which subsequently healed secondarily to give an excellent cosmetic outcome.

CONCLUSION

CAs are rare in children. The keys to the successful management of this entity include adequate anatomical delineation, preoperative embolization, and preparedness for intraoperative events such as bleeding. A robust knowledge of various flap procedures is a must for proper skin coverage. A thorough workup, multimodality approach for de-vascularizing the lesion, and apt intraoperative decisions ensured a successful outcome in our case.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.