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Management of a Challenging Arteriovenous Malformation of the Scalp and Orbit in a Patient With Polycystic Kidney Disease
Management of a Challenging Arteriovenous Malformation of the Scalp and Orbit in a Patient With Polycystic Kidney Disease Nupur Bit, T. Vidyasagaran, J. Amalorpavanathan, T.M. Balakrishnan, and N. Sritharan, Chennai, Tamil Nadu, India
Arteriovenous malformations are notorious for their propensity to bleed, sometimes with fatal consequences. We describe an unusual case of a patient with polycystic kidney disease and with a large arteriovenous malformation involving the orbit and scalp who presented with multiple episodes of profuse bleeding from the upper eyelid and loss of vision in the corresponding eye.
CASE REPORT A 50-year-old Asian man presented with complaints of a large painless mass involving the left eye and side of the head for 10 years. It was associated with multiple episodes of profuse bleeding from the upper eyelid and decreased vision for a period of 1 month. He had no known diabetes or hypertension. Physical examination revealed pallor, high blood pressure (180/100 mm Hg), and bilateral palpable kidneys. He had a large 10 8 cm pulsatile mass involving the left orbit and frontotemporal region of the scalp. There was an audible bruit over the mass, dilated veins, pulsatile proptosis, corneal scarring, and conjunctival congestion. There was an ulcer on the upper eyelid from which there was profuse pulsatile bleeding, controlled by compression bandages and oral antihypertensive medication (Fig. 1A). No genetic tests or other imaging was done to rule out the presence of other arteriovenous malformations (AVMs). Magnetic resonance angiogram revealed that the AVM involved the extraocular muscles of the left eye and had feeders from the left supraorbital artery and superficial temporal artery (Fig. 1B). There was no intracranial Madras Medical College, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Correspondence to: Nupur Bit, MBBS, MS, Postgraduate resident MCh Vascular Surgery, Department of Vascular Surgery, Madras Medical College, Rajiv Gandhi Government General Hospital, Park Town, Chennai, Tamil Nadu 600003, India; E-mail: nupur.bit@ gmail.com Ann Vasc Surg 2012; 26: 1129.e9e1129.e11 http://dx.doi.org/10.1016/j.avsg.2012.04.010 Ó Annals of Vascular Surgery Inc. Published online: September 13, 2012
extension. As there was no residual vision in the left eye and the right eye was at risk for sympathetic ophthalmitis, it was decided to perform left enucleation and excision of the AVM. Meticulous planning preceded the surgery. The team comprised the vascular surgery team, an ophthalmologist, the plastic surgery team, an anesthesiologist, and a neurosurgery backup. The patient underwent dialysis twice preoperatively to optimize his renal status. Intraoperatively, first left common, internal and external carotid artery control samples were taken through a cervical incision along the anterior border of the left sternocleidomastoid muscle (Fig. 2A). The AVM including the eyeball was excised in toto, with particular attention paid to the ligation of the superficial temporal and supraorbital feeders. After excision, there was a large defect involving the left orbit and infraorbital region which was devoid of periosteum (Fig. 2C). The defect was reconstructed using a free transverse abdominis myocutaneous flap. The deep internal epigastric artery and two venae comitantes from the flap were anastomosed to the superficial temporal artery, superficial temporal vein, and infraorbital vein, respectively. The veins had become arterialized owing to the presence of the AVM and had served as good recipient vessels. Although the carotid artery control samples had been taken pre-emptively in anticipation of bleeding, temporary occlusion was not required during the procedure; hence, the patient was not treated with heparin. The approximate duration of the surgery was 6 hours, and the amount of intraoperative blood loss was 800 mL. Owing to the prolonged duration of the surgery and intraoperative hemodynamic instability, the patient
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Fig. 1. (A) Preoperative photograph showing left orbital swelling. (B) Magnetic resonance imaging of the orbit, coronal section showing multiple serpiginous dilated
vessels within the left orbital cavity, and the adjacent soft tissue causing downward and outward displacement of the globe.
Fig. 2. (A) Intraoperative photograph showing internal carotid artery (ICA) and external carotid artery (ECA) control. (B) Postoperative photograph of the patient
showing viable transverse abdominis myocutaneous flap. (C) Intraoperative photograph showing enucleated orbital cavity with a large defect.
required inotropes and ventilator support for 1 day, after which he made a good recovery. The patient had a viable flap cover 6 months after the procedure and was on regular renal replacement therapy (Fig. 2B).
DISCUSSION AVMs have been a therapeutic challenge to surgeons from time immemorial. Multiple classifications
Vol. 26, No. 8, November 2012
have been proposed to categorize them. The most widely accepted is the Hamburg classification, which describes it as a subtype of congenital vascular malformations. Mulliken and Glowacki further described AVMs as truncular or extratruncular, based on their embryologic stage of development.1 AVMs need to be treated because of their propensity for bleeding, ischemic steal leading to skin ulceration, and their risk for hemodynamic compromise. Over the years, the policy has changed from radically aggressive surgical approach to a combination of endovascular and surgical excision. Transcatheter embolization of the nidus with N-butylcyanoacrylate glue, polyvinvyl alcohol particles, or microcatheter coils have all been described, with success.2,3 Meticulous planning is essential because if the nidus is not treated, it inevitably leads to revascularization through newly recruited collaterals, and these recurrences are notoriously difficult to treat and are associated with loss of access to the malformation for future embolization attempts. Although polycystic kidney disease has been known for its association with intracranial aneurysms, there is no known association in the literature with extracranial AVM.4 To the best of our knowledge, this is the first case report of a patient with polycystic kidney disease and an extracranial AVM. In our patient, bleeding from the AVM was exacerbated by uncontrolled hypertension secondary to the polycystic kidney disease and undetected chronic renal failure. Treatment of the high blood pressure decreased the bleeding. As the lesion was large and unsightly and he had no vision in that eye, surgical resection of the lesion with reconstruction by free flap was undertaken for him. Precautions to control the bleeding included pre-emptive control of the
Case reports 1129.e11
external and internal carotid arteries (as the predominant feeders to the lesion were from supraorbital and superficial temporal arteries), careful dissection and ligation of the supraorbital artery and superficial temporal artery, and strict maintenance of blood pressure during the procedure. In a situation where the nidus of the AVM is multiplanar and multifocal, as in this patient, with involvement of the left orbit and frontotemporal region, we planned a meticulous surgical approachd wide aggressive excision of the lesion with enucleation of the orbit and removal of the periosteum on the deeper aspect and control of the carotid vessels in the neck. Preoperative embolization leading to deafferentiation and skeletonization of the nidus of the AVM was not feasible.
CONCLUSION Even surgically challenging large AVMs with multiplanar and multifocal involvement can be successfully excised with meticulous planning. REFERENCES 1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982;69: 412e22. 2. Duncan IC, Fourie PA. Circumferential flow reduction during percutaneous embolotherapy of extracranial vascular malformations: the ‘‘cookie-cutter’’ technique. Am J Neuroradiol 2003;24:1453e5. 3. Ryu CW, Whang SM, Suh DC, et al. Percutaneous direct puncture glue embolization of high-flow craniofacial arteriovenous lesions: a new circular ring compression device with a beveled edge. Am J Neuroradiol 2007;28:528e30. 4. Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int 1994;45:1140e6.
Arteriovenous malformations are notorious for their propensity to bleed, sometimes with fatal consequences. We describe an unusual case of a patient with polycystic kidney disease and with a large arteriovenous malformation involving the orbit and scalp who presented with multiple episodes of profuse bleeding from the upper eyelid and loss of vision in the corresponding eye.
CASE REPORT A 50-year-old Asian man presented with complaints of a large painless mass involving the left eye and side of the head for 10 years. It was associated with multiple episodes of profuse bleeding from the upper eyelid and decreased vision for a period of 1 month. He had no known diabetes or hypertension. Physical examination revealed pallor, high blood pressure (180/100 mm Hg), and bilateral palpable kidneys. He had a large 10 8 cm pulsatile mass involving the left orbit and frontotemporal region of the scalp. There was an audible bruit over the mass, dilated veins, pulsatile proptosis, corneal scarring, and conjunctival congestion. There was an ulcer on the upper eyelid from which there was profuse pulsatile bleeding, controlled by compression bandages and oral antihypertensive medication (Fig. 1A). No genetic tests or other imaging was done to rule out the presence of other arteriovenous malformations (AVMs). Magnetic resonance angiogram revealed that the AVM involved the extraocular muscles of the left eye and had feeders from the left supraorbital artery and superficial temporal artery (Fig. 1B). There was no intracranial Madras Medical College, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Correspondence to: Nupur Bit, MBBS, MS, Postgraduate resident MCh Vascular Surgery, Department of Vascular Surgery, Madras Medical College, Rajiv Gandhi Government General Hospital, Park Town, Chennai, Tamil Nadu 600003, India; E-mail: nupur.bit@ gmail.com Ann Vasc Surg 2012; 26: 1129.e9e1129.e11 http://dx.doi.org/10.1016/j.avsg.2012.04.010 Ó Annals of Vascular Surgery Inc. Published online: September 13, 2012
extension. As there was no residual vision in the left eye and the right eye was at risk for sympathetic ophthalmitis, it was decided to perform left enucleation and excision of the AVM. Meticulous planning preceded the surgery. The team comprised the vascular surgery team, an ophthalmologist, the plastic surgery team, an anesthesiologist, and a neurosurgery backup. The patient underwent dialysis twice preoperatively to optimize his renal status. Intraoperatively, first left common, internal and external carotid artery control samples were taken through a cervical incision along the anterior border of the left sternocleidomastoid muscle (Fig. 2A). The AVM including the eyeball was excised in toto, with particular attention paid to the ligation of the superficial temporal and supraorbital feeders. After excision, there was a large defect involving the left orbit and infraorbital region which was devoid of periosteum (Fig. 2C). The defect was reconstructed using a free transverse abdominis myocutaneous flap. The deep internal epigastric artery and two venae comitantes from the flap were anastomosed to the superficial temporal artery, superficial temporal vein, and infraorbital vein, respectively. The veins had become arterialized owing to the presence of the AVM and had served as good recipient vessels. Although the carotid artery control samples had been taken pre-emptively in anticipation of bleeding, temporary occlusion was not required during the procedure; hence, the patient was not treated with heparin. The approximate duration of the surgery was 6 hours, and the amount of intraoperative blood loss was 800 mL. Owing to the prolonged duration of the surgery and intraoperative hemodynamic instability, the patient
1129.e9
1129.e10 Case reports
Annals of Vascular Surgery
Fig. 1. (A) Preoperative photograph showing left orbital swelling. (B) Magnetic resonance imaging of the orbit, coronal section showing multiple serpiginous dilated
vessels within the left orbital cavity, and the adjacent soft tissue causing downward and outward displacement of the globe.
Fig. 2. (A) Intraoperative photograph showing internal carotid artery (ICA) and external carotid artery (ECA) control. (B) Postoperative photograph of the patient
showing viable transverse abdominis myocutaneous flap. (C) Intraoperative photograph showing enucleated orbital cavity with a large defect.
required inotropes and ventilator support for 1 day, after which he made a good recovery. The patient had a viable flap cover 6 months after the procedure and was on regular renal replacement therapy (Fig. 2B).
DISCUSSION AVMs have been a therapeutic challenge to surgeons from time immemorial. Multiple classifications
Vol. 26, No. 8, November 2012
have been proposed to categorize them. The most widely accepted is the Hamburg classification, which describes it as a subtype of congenital vascular malformations. Mulliken and Glowacki further described AVMs as truncular or extratruncular, based on their embryologic stage of development.1 AVMs need to be treated because of their propensity for bleeding, ischemic steal leading to skin ulceration, and their risk for hemodynamic compromise. Over the years, the policy has changed from radically aggressive surgical approach to a combination of endovascular and surgical excision. Transcatheter embolization of the nidus with N-butylcyanoacrylate glue, polyvinvyl alcohol particles, or microcatheter coils have all been described, with success.2,3 Meticulous planning is essential because if the nidus is not treated, it inevitably leads to revascularization through newly recruited collaterals, and these recurrences are notoriously difficult to treat and are associated with loss of access to the malformation for future embolization attempts. Although polycystic kidney disease has been known for its association with intracranial aneurysms, there is no known association in the literature with extracranial AVM.4 To the best of our knowledge, this is the first case report of a patient with polycystic kidney disease and an extracranial AVM. In our patient, bleeding from the AVM was exacerbated by uncontrolled hypertension secondary to the polycystic kidney disease and undetected chronic renal failure. Treatment of the high blood pressure decreased the bleeding. As the lesion was large and unsightly and he had no vision in that eye, surgical resection of the lesion with reconstruction by free flap was undertaken for him. Precautions to control the bleeding included pre-emptive control of the
Case reports 1129.e11
external and internal carotid arteries (as the predominant feeders to the lesion were from supraorbital and superficial temporal arteries), careful dissection and ligation of the supraorbital artery and superficial temporal artery, and strict maintenance of blood pressure during the procedure. In a situation where the nidus of the AVM is multiplanar and multifocal, as in this patient, with involvement of the left orbit and frontotemporal region, we planned a meticulous surgical approachd wide aggressive excision of the lesion with enucleation of the orbit and removal of the periosteum on the deeper aspect and control of the carotid vessels in the neck. Preoperative embolization leading to deafferentiation and skeletonization of the nidus of the AVM was not feasible.
CONCLUSION Even surgically challenging large AVMs with multiplanar and multifocal involvement can be successfully excised with meticulous planning. REFERENCES 1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982;69: 412e22. 2. Duncan IC, Fourie PA. Circumferential flow reduction during percutaneous embolotherapy of extracranial vascular malformations: the ‘‘cookie-cutter’’ technique. Am J Neuroradiol 2003;24:1453e5. 3. Ryu CW, Whang SM, Suh DC, et al. Percutaneous direct puncture glue embolization of high-flow craniofacial arteriovenous lesions: a new circular ring compression device with a beveled edge. Am J Neuroradiol 2007;28:528e30. 4. Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int 1994;45:1140e6.