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Early presentation of an extremity arteriovenous malformation
The British Association of Plastic Surgeons (2004) 57, 785–788
CASE REPORT
Early presentation of an extremity arteriovenous malformation Jill B. Webb*, Mary O’Brien, Philip R. John, Hiroshi Nishikawa Department of Plastic Surgery, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B46NH, UK Received 30 November 2003; accepted 17 June 2004
KEYWORDS Arteriovenous malformation; Embolisation; Sclerotherapy
Abstract We report a very rare case of a high flow arteriovenous malformation (AVM) of the upper limb that caused high output cardiac failure at birth. There was early transfer of the baby to the care of a multidisciplinary team. After radiological intervention, the arm distal to the malformation became ischaemic and an urgent amputation through the upper-humerus followed. Methods of treatment are discussed, together with a review of results in the literature. Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved.
AVMs are rare congenital vascular malformations, which can become symptomatic with pain or bleeding, most commonly, in adolescence or early adulthood.1 They are difficult to manage, and frequently require repeated treatments using combinations of surgical and interventional radiological techniques. When arteriovenous shunting is sufficient to precipitate cardiac failure, urgent medical treatment is required together with early treatment of the malformation by a multidisciplinary team with expertise in managing vascular anomalies.
Case report The male baby was the first born of non-consanguineous parents. He had an uncomplicated vaginal delivery at term and developed respiratory distress * Corresponding author. E-mail address: [email protected] (J.B. Webb).
within 12 h of birth secondary to high output cardiac failure. A high flow vascular malformation extending over the left anterior chest wall and a separate one involving a substantial part of his left arm was present (Fig. 1). Initially the cardiac failure was controlled with diuretics and fluid restriction. He was then transferred to a multidisciplinary centre at two days of age. An echocardiogram demonstrated that the right atrium and ventricle were dilated, with severe tricuspid regurgitation. There was a large atrial septal defect but no patent ductus arteriosus or ventricular defect. The left subclavian artery was very dilated. The innominate vein and superior vena cava were also dilated and both had arterial pulsations on Doppler. At 3 days of age, catheter angiography and embolisation were performed under general anaesthetic. This demonstrated that the left subclavian and axillary arteries were equal in size to the aorta (Fig. 2A). The vascular malformation over the anterior chest showed only as a late blush. There
S0007-1226/$ - see front matter Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2004.06.029
786
Figure 1 arm.
J.B. Webb et al.
Pre procedure vascular malformation of left
was an extensive AVM in the soft tissues around the left elbow and proximal forearm arising from the brachial artery. The elbow joint was also affected (Fig. 2B). The forearm vessels were abnormal with a single serpiginous artery, and a persistent median artery, feeding the palmar arch (Fig. 2C). Embolisation was attempted; microcoils were placed into the larger arterial feeders (Fig. 2D). The procedure continued with percutaneous sclerotherapy using absolute alcohol (3.5 ml) mixed with contrast. Tourniquets were inflated above and below the AVM to prevent escape of embolisation material. Initial injection of contrast confirmed that there was minimal arterial flow distally and no proximal venous return. The tourniquets were released 10 min after the alcohol injection. About 5 min later the child briefly desaturated, which was thought to be due to alcohol in the pulmonary circulation. The hand also became very discoloured, with poor capillary return. In view of the respiratory problems he was transferred to the intensive care unit for continued ventilation and monitoring. The following day (four days of age), the cardiac failure had resolved, but the arm became increasingly swollen, dusky and cold. Thirty hours post embolisation it became evident that the ischaemic limb was unsalvageable and he underwent amputation through the upper-humerus (Fig. 3). The skin was left open for secondary closure the following day. The postoperative recovery, including wound healing, was unremarkable. The amputation completely resolved the high output cardiac failure and by 10 days of age all fluid restrictions were lifted, the diuretics reduced and later stopped. Eighteen months post embolisation the infant remains well and requires no cardiac medication. The cutaneous vascular malformation over the anterior chest wall remains asymptomatic and continues to be reviewed.
Figure 2 (A–D) Radiological images: (A) aortogram demonstrating enlarged subclavian and axillary arteries, (B) the complex arteriovenous malformation around the elbow, (C) showing the single vestigial vessel supplying the hand, (D) following catheter treatment, showing the microcoils in situ and the tourniquets inflated with a little distal filling but no proximal run off.
Early presentation of an extremity arteriovenous malformation
Figure 3
The arm 18 h post radiological intervention.
Discussion AVMs are amongst the rarer congenital vascular anomalies. It is important to recognise which type of anomaly the patient has because many, in particular most haemangiomas, should be treated conservatively. The literature is confusing due to the plethora of names these malformations are given. However, a simple and memorable classification was described in 1982 and has been accepted by the International Society for the Study of Vascular Anomalies in 1996.2 Consistent use of this classification enhances communication and aids management decisions. It is rare for an AVM to present in the neonatal period. In a series of 138 AVMs, only one presented at birth.1 High output cardiac failure in neonates would be manifested as respiratory distress. If the systemic blood pressure falls below the pulmonary pressure, neonates could develop a right to left shunt through the foramen ovale and ductus arteriosus. In some cases this has lead to early death,3 thus emphasising the importance of urgent referral to an appropriately experienced team to care for these patients. Management of AVMs includes conservative management, surgery and interventional radiology using catheter embolisation or direct percutaneous sclerotherapy. Combinations of these techniques are frequently used, in particular radiological intervention followed by surgery a few days later. As there are reports of spontaneous closure of AVMs, it may be appropriate in selected cases, even symptomatic patients, to treat the AVM expectantly.4 Distal ischaemia can be a feature of extremity AVMs, and any intervention may decrease the distal circulation even further. If intervention is considered, it must be carefully planned, taking into account that future treatment will probably be required. Proximal occlusion of feeding vessels by surgical
787
ligation or embolotherapy is considered poor practice, as it not only precludes further transcatheter imaging and intervention, but also encourages the development of the malformation’s collateral circulation. To have any chance of cure, these lesions require aggressive treatment using multiple treatment modalities. Imaging has a pivotal role in the management of these malformations. However, since the risk of femoral artery thrombosis is 5% in children weighing less than 10 kg, angiography should be performed as part of a sequence of intervention (e.g. catheter embolisation). Radiological intervention must be performed in a facility that also has vascular or plastic surgeons in case of the need for surgical intervention, as was required in this case. Ideally, surgery should be carried out within 72 h of embolisation. However, successful outcomes are reported even when delayed by 10 days. Surgery is often difficult, sometimes dangerous and frequently fails.1 While total excision is the ideal, AVMs do not respect tissue planes, and even radical excision in a method similar to sarcoma excision, has a high recurrence rate. The results of managing large AVMs can be disappointing. In this patient the cardiac failure, which was life threatening, was successfully treated, but only at the cost of losing most of the upper limb. The literature suggests that most symptomatic AVMs require repeated series of treatments usually with multiple modalities of radiological intervention and intermittent surgery. There is also some evidence that upper limb and shoulder malformations may be particularly difficult to treat. Dickey, Pollak et al. describe four such patients, all adults.5 The amputation rate varies in reports, however, if an AVM of the extremity requires surgery, 10–30% will require an amputation, with upper limbs having a worse outcome than lower limb malformations.1 Symptomatic AVMs rarely present in the neonatal period. When extensive, they can present with high output cardiac failure. Urgent care by a multidisciplinary team is necessary for medical stabilisation. Ideal treatment should be embolisation or, sclerotherapy followed by early surgery. Even with aggressive treatment, recurrence is frequent. AVM’s of the extremity are associated with a high risk of amputation.
References 1. Young AE. Arteriovenous malformations. In: Mulliken JB, Young AE, editors. Vascular birthmarks: hemangiomas and malformations. Philadelphia: Saunders; 1988, p. 228–45.
788 2. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (New issues). In: James WD, Cockerell CL, Dzobow LM et al, editors. Advances in dermatology. St Louis: Mosby; 1997, p. 375–423. 3. Pernot C. Malignant brachial arteriovenous fistula in a newborn child: a case report. Angiology 1979;30:640–4.
J.B. Webb et al. 4. Levine OR, Antillon J, Marquis JR, Curtis G. Arteriovenous malformations in young infants. J Med Soc New Jersey 1979; 76:118–21. 5. Dickey KW, Pollak JS, Meier III GH, Denny DF, White RI. Management of large high-flow arteriovenous malformations of the shoulder and upper extremity with transcatheter embolotherapy. JVIR 1995;6:765–73.
CASE REPORT
Early presentation of an extremity arteriovenous malformation Jill B. Webb*, Mary O’Brien, Philip R. John, Hiroshi Nishikawa Department of Plastic Surgery, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B46NH, UK Received 30 November 2003; accepted 17 June 2004
KEYWORDS Arteriovenous malformation; Embolisation; Sclerotherapy
Abstract We report a very rare case of a high flow arteriovenous malformation (AVM) of the upper limb that caused high output cardiac failure at birth. There was early transfer of the baby to the care of a multidisciplinary team. After radiological intervention, the arm distal to the malformation became ischaemic and an urgent amputation through the upper-humerus followed. Methods of treatment are discussed, together with a review of results in the literature. Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved.
AVMs are rare congenital vascular malformations, which can become symptomatic with pain or bleeding, most commonly, in adolescence or early adulthood.1 They are difficult to manage, and frequently require repeated treatments using combinations of surgical and interventional radiological techniques. When arteriovenous shunting is sufficient to precipitate cardiac failure, urgent medical treatment is required together with early treatment of the malformation by a multidisciplinary team with expertise in managing vascular anomalies.
Case report The male baby was the first born of non-consanguineous parents. He had an uncomplicated vaginal delivery at term and developed respiratory distress * Corresponding author. E-mail address: [email protected] (J.B. Webb).
within 12 h of birth secondary to high output cardiac failure. A high flow vascular malformation extending over the left anterior chest wall and a separate one involving a substantial part of his left arm was present (Fig. 1). Initially the cardiac failure was controlled with diuretics and fluid restriction. He was then transferred to a multidisciplinary centre at two days of age. An echocardiogram demonstrated that the right atrium and ventricle were dilated, with severe tricuspid regurgitation. There was a large atrial septal defect but no patent ductus arteriosus or ventricular defect. The left subclavian artery was very dilated. The innominate vein and superior vena cava were also dilated and both had arterial pulsations on Doppler. At 3 days of age, catheter angiography and embolisation were performed under general anaesthetic. This demonstrated that the left subclavian and axillary arteries were equal in size to the aorta (Fig. 2A). The vascular malformation over the anterior chest showed only as a late blush. There
S0007-1226/$ - see front matter Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2004.06.029
786
Figure 1 arm.
J.B. Webb et al.
Pre procedure vascular malformation of left
was an extensive AVM in the soft tissues around the left elbow and proximal forearm arising from the brachial artery. The elbow joint was also affected (Fig. 2B). The forearm vessels were abnormal with a single serpiginous artery, and a persistent median artery, feeding the palmar arch (Fig. 2C). Embolisation was attempted; microcoils were placed into the larger arterial feeders (Fig. 2D). The procedure continued with percutaneous sclerotherapy using absolute alcohol (3.5 ml) mixed with contrast. Tourniquets were inflated above and below the AVM to prevent escape of embolisation material. Initial injection of contrast confirmed that there was minimal arterial flow distally and no proximal venous return. The tourniquets were released 10 min after the alcohol injection. About 5 min later the child briefly desaturated, which was thought to be due to alcohol in the pulmonary circulation. The hand also became very discoloured, with poor capillary return. In view of the respiratory problems he was transferred to the intensive care unit for continued ventilation and monitoring. The following day (four days of age), the cardiac failure had resolved, but the arm became increasingly swollen, dusky and cold. Thirty hours post embolisation it became evident that the ischaemic limb was unsalvageable and he underwent amputation through the upper-humerus (Fig. 3). The skin was left open for secondary closure the following day. The postoperative recovery, including wound healing, was unremarkable. The amputation completely resolved the high output cardiac failure and by 10 days of age all fluid restrictions were lifted, the diuretics reduced and later stopped. Eighteen months post embolisation the infant remains well and requires no cardiac medication. The cutaneous vascular malformation over the anterior chest wall remains asymptomatic and continues to be reviewed.
Figure 2 (A–D) Radiological images: (A) aortogram demonstrating enlarged subclavian and axillary arteries, (B) the complex arteriovenous malformation around the elbow, (C) showing the single vestigial vessel supplying the hand, (D) following catheter treatment, showing the microcoils in situ and the tourniquets inflated with a little distal filling but no proximal run off.
Early presentation of an extremity arteriovenous malformation
Figure 3
The arm 18 h post radiological intervention.
Discussion AVMs are amongst the rarer congenital vascular anomalies. It is important to recognise which type of anomaly the patient has because many, in particular most haemangiomas, should be treated conservatively. The literature is confusing due to the plethora of names these malformations are given. However, a simple and memorable classification was described in 1982 and has been accepted by the International Society for the Study of Vascular Anomalies in 1996.2 Consistent use of this classification enhances communication and aids management decisions. It is rare for an AVM to present in the neonatal period. In a series of 138 AVMs, only one presented at birth.1 High output cardiac failure in neonates would be manifested as respiratory distress. If the systemic blood pressure falls below the pulmonary pressure, neonates could develop a right to left shunt through the foramen ovale and ductus arteriosus. In some cases this has lead to early death,3 thus emphasising the importance of urgent referral to an appropriately experienced team to care for these patients. Management of AVMs includes conservative management, surgery and interventional radiology using catheter embolisation or direct percutaneous sclerotherapy. Combinations of these techniques are frequently used, in particular radiological intervention followed by surgery a few days later. As there are reports of spontaneous closure of AVMs, it may be appropriate in selected cases, even symptomatic patients, to treat the AVM expectantly.4 Distal ischaemia can be a feature of extremity AVMs, and any intervention may decrease the distal circulation even further. If intervention is considered, it must be carefully planned, taking into account that future treatment will probably be required. Proximal occlusion of feeding vessels by surgical
787
ligation or embolotherapy is considered poor practice, as it not only precludes further transcatheter imaging and intervention, but also encourages the development of the malformation’s collateral circulation. To have any chance of cure, these lesions require aggressive treatment using multiple treatment modalities. Imaging has a pivotal role in the management of these malformations. However, since the risk of femoral artery thrombosis is 5% in children weighing less than 10 kg, angiography should be performed as part of a sequence of intervention (e.g. catheter embolisation). Radiological intervention must be performed in a facility that also has vascular or plastic surgeons in case of the need for surgical intervention, as was required in this case. Ideally, surgery should be carried out within 72 h of embolisation. However, successful outcomes are reported even when delayed by 10 days. Surgery is often difficult, sometimes dangerous and frequently fails.1 While total excision is the ideal, AVMs do not respect tissue planes, and even radical excision in a method similar to sarcoma excision, has a high recurrence rate. The results of managing large AVMs can be disappointing. In this patient the cardiac failure, which was life threatening, was successfully treated, but only at the cost of losing most of the upper limb. The literature suggests that most symptomatic AVMs require repeated series of treatments usually with multiple modalities of radiological intervention and intermittent surgery. There is also some evidence that upper limb and shoulder malformations may be particularly difficult to treat. Dickey, Pollak et al. describe four such patients, all adults.5 The amputation rate varies in reports, however, if an AVM of the extremity requires surgery, 10–30% will require an amputation, with upper limbs having a worse outcome than lower limb malformations.1 Symptomatic AVMs rarely present in the neonatal period. When extensive, they can present with high output cardiac failure. Urgent care by a multidisciplinary team is necessary for medical stabilisation. Ideal treatment should be embolisation or, sclerotherapy followed by early surgery. Even with aggressive treatment, recurrence is frequent. AVM’s of the extremity are associated with a high risk of amputation.
References 1. Young AE. Arteriovenous malformations. In: Mulliken JB, Young AE, editors. Vascular birthmarks: hemangiomas and malformations. Philadelphia: Saunders; 1988, p. 228–45.
788 2. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (New issues). In: James WD, Cockerell CL, Dzobow LM et al, editors. Advances in dermatology. St Louis: Mosby; 1997, p. 375–423. 3. Pernot C. Malignant brachial arteriovenous fistula in a newborn child: a case report. Angiology 1979;30:640–4.
J.B. Webb et al. 4. Levine OR, Antillon J, Marquis JR, Curtis G. Arteriovenous malformations in young infants. J Med Soc New Jersey 1979; 76:118–21. 5. Dickey KW, Pollak JS, Meier III GH, Denny DF, White RI. Management of large high-flow arteriovenous malformations of the shoulder and upper extremity with transcatheter embolotherapy. JVIR 1995;6:765–73.