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Surgical Management of Slow-flow Venous Malformation of External Jugular Vein
SHORT REPORT
Surgical Management of Slow-flow Venous Malformation of External Jugular Vein A. Bhatnagar
*
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Introduction: Cervicofacial venous malformation (VMs) arising from the external jugular vein (EJV) are very rare. These distinct clinical entities may be easily and optimally treated with surgical excision without any resultant morbidity. Report: Three adult patients underwent excision of the malformation after unsuccessful sclerotherapy. Lesions were excised after ligating multiple large feeders between the EJV and the malformation. Discussion: Sclerotherapy failed due to small contact time of the sclerosant with the endothelium. Surgical excision of these lesions is simple and straightforward, and should be considered as the first line of treatment whenever this clinical entity is recognised. Ó 2012 European Society for Vascular Surgery. Published by Elsevier Ltd. Open access under CC BY-NC-ND license. Article history: Received 21 July 2012, Accepted 6 November 2012, Available online XXX Keywords: Venous malformation, External jugular vein, Excision INTRODUCTION Venous malformations may present as isolated neck masses in adults. Cervicofacial venous malformations (VMs) arising from the external jugular vein (EJV) are very rare with less than 10 cases described in the literature. Treatment is sought on account of the swelling for cosmetic reasons. Sclerotherapy has now become the mainstay of treatment for cervicofacial malformations. The clinical importance of recognising EJV VMs as distinct clinical entities lies in the fact that they may be easily and optimally treated with surgical excision without any resultant morbidities. Singlestage surgical excision was done in three cases with preservation of the EJV after unsuccessful sclerotherapy.
findings were compatible with the diagnosis of a slow-flow VM intimately connected with the EJV, which demonstrated normal flow. Intra-operatively, VM arising from the EJV was
REPORT Three patients, one 25-year-old male and two females aged 25 and 40 years, presented with a left neck mass that had progressively increased in size for few years. Cosmetic deformity was the main complaint in all cases. Two patients had undergone three sessions of sclerotherapy with no improvement. On physical examination, soft, non-tender masses were observed in the posterior triangle (Fig. 1). The lesions were free from the underlying muscle and soft tissue in all the patients, involved the overlying skin in two cases and became prominent on straining and Valsalva. No pulsation or thrill was demonstrated. Rest of the neck was normal. Sonography and magnetic resonance imaging (MRI) DOI of Original Article: http://dx.doi.org/10.1016/j.ejvs.2012.11.008 * A. Bhatnagar, Sanjay Gandhi Post Graduate Institute of Medical Sciences, NH24B, Lucknow 226014, Uttar Pradesh, India. Tel.: þ91 5222418648. E-mail address: [email protected] (A. Bhatnagar). 1078-5884 Ó 2012 European Society for Vascular Surgery. Published by Elsevier Ltd. Open access under CC BY-NC-ND license. http://dx.doi.org/10.1016/j.ejvsextra.2012.11.002
Figure 1. Venous malformation at root of neck with infra clavicular extent.
e10
EJVES Extra
Figure 2. Very large communication between VM and EJV.
identified. Multiple feeding vessels were identified in the first two cases while the third patient showed a large communicating vein (Fig. 2). Feeding vessels were ligated, and lesion completely excised. All patients underwent subcuticular skin closure after ensuring meticulous haemostasis and advised routine anti-scar measures. Macroscopically, the excised lesion was a well-circumscribed darkbrown nodule. Microscopically, the nodule was composed of ectatic vascular spaces with a thin wall and lined by a layer of endothelium. Smooth muscle was found in the wall of some vessels. Histological analysis was compatible with VM. DISCUSSION VMs have an incidence of one to two in 10,000 births, prevalence of 1%, are present at birth and grow proportionately with age causing various clinical presentations.1,2 Slow-flow VM closely associated with the EJV is rare.3 Less than 10 cases have been described.4 They are connected to the EJV by single or multiple veins, mostly of large calibre. Histologic abnormalities of the smooth muscleepericyte component within vascular channel walls of VMs are hypothesised as a potential cause of many VMs.2 Jackson and co-workers identified the need for further augmentation of Mulliken’s classification to answer the (therapeutic) questions of ‘what to do’ and ‘when to do it’.2 Overzealous and incomplete treatment can, on many occasions, lead to worsening of features. Post-treatment morbidity can be more distressing for the patients as compared to their pre-treatment status. Treatment is
Volume 25 Issue 2 February/2013
indicated for lesions causing pain and for functional and aesthetic impairment. Pain is due to venous engorgement and local stretching of tissues.2 Sclerotherapy has become the mainstay of treatment for cervicofacial VM. The most important variables predicting successful sclerotherapy are sclerosant concentration and dwell time, especially when there are multiple wide channels of communication between the VM and its draining vein as in our cases. Sclerotherapy failed in our patients due to small contact time of the sclerosant with the endothelium as most of the drug was washed off. The major complications of sclerotherapy include skin necrosis, residual lesions, skin pigmentation, peripheral nerve palsies and hypotensive crisis, as well as fatalities such as cardiac arrest and pulmonary emboli. Two of our cases had significant skin involvement. The patient had undergone three attempts at sclerotherapy at some other treatment centre. Keeping the risk of skin necrosis, unsuccessful sclerotherapy and proximity of the EJV in mind, surgical excision was planned. Surgery for this lesion was relatively easy as no vital structures were present in the vicinity of the lesion. In VM arising from EJV, the vein is generally normal and can be spared during the excision. Its direct and numerous communications with the EJV render sclerosing it a ‘more than usually’ hazardous option for treatment. Considering that surgical excision of these lesions is rather simple and straightforward, the author would recommend it to be considered as the first line of treatment whenever this clinical entity is recognised. Surgery ensures least down time, morbidity and recurrence rate.5 Clinicians dealing with vascular problems should be aware of this lesion where early surgical intervention provides optimal results. CONFLICT OF INTEREST/FUNDING None. REFERENCES 1 Lee IH, Kim KH, Jeon P, Hong SB, Kim H-J, Kim ST, et al. Ethanol sclerotherapy for the management of craniofacial venous malformations: the interim results. Korean J Radiol 2009;10(3):269e76. 2 Legiehn GM, Manraj KS. Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin N Am 2008;46:545e97. 3 Jose Â, Luis O, Rodrõaguez G, Fernandez J, Gregorio M. Jugular venous malformation in an 8-year-old boy: treatment with endovascular sclerotherapy. Eur J Pediatr 2001;160:392e4. 4 Ahuja AT, Yuen Hok-Yuen, Wong Ka-Tak, King Ann D, Abdullah Victor, To Edward, et al. External jugular vein vascular malformation: sonographic and MR imaging appearances. Am J Neuroradiol 2004;25:338e42. 5 Kang GCW, Song C. Forty-one cervicofacial vascular anomalies and their surgical treatment e retrospection and review. Ann Acad Med Singap 2008;37:165e79.
Surgical Management of Slow-flow Venous Malformation of External Jugular Vein A. Bhatnagar
*
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Introduction: Cervicofacial venous malformation (VMs) arising from the external jugular vein (EJV) are very rare. These distinct clinical entities may be easily and optimally treated with surgical excision without any resultant morbidity. Report: Three adult patients underwent excision of the malformation after unsuccessful sclerotherapy. Lesions were excised after ligating multiple large feeders between the EJV and the malformation. Discussion: Sclerotherapy failed due to small contact time of the sclerosant with the endothelium. Surgical excision of these lesions is simple and straightforward, and should be considered as the first line of treatment whenever this clinical entity is recognised. Ó 2012 European Society for Vascular Surgery. Published by Elsevier Ltd. Open access under CC BY-NC-ND license. Article history: Received 21 July 2012, Accepted 6 November 2012, Available online XXX Keywords: Venous malformation, External jugular vein, Excision INTRODUCTION Venous malformations may present as isolated neck masses in adults. Cervicofacial venous malformations (VMs) arising from the external jugular vein (EJV) are very rare with less than 10 cases described in the literature. Treatment is sought on account of the swelling for cosmetic reasons. Sclerotherapy has now become the mainstay of treatment for cervicofacial malformations. The clinical importance of recognising EJV VMs as distinct clinical entities lies in the fact that they may be easily and optimally treated with surgical excision without any resultant morbidities. Singlestage surgical excision was done in three cases with preservation of the EJV after unsuccessful sclerotherapy.
findings were compatible with the diagnosis of a slow-flow VM intimately connected with the EJV, which demonstrated normal flow. Intra-operatively, VM arising from the EJV was
REPORT Three patients, one 25-year-old male and two females aged 25 and 40 years, presented with a left neck mass that had progressively increased in size for few years. Cosmetic deformity was the main complaint in all cases. Two patients had undergone three sessions of sclerotherapy with no improvement. On physical examination, soft, non-tender masses were observed in the posterior triangle (Fig. 1). The lesions were free from the underlying muscle and soft tissue in all the patients, involved the overlying skin in two cases and became prominent on straining and Valsalva. No pulsation or thrill was demonstrated. Rest of the neck was normal. Sonography and magnetic resonance imaging (MRI) DOI of Original Article: http://dx.doi.org/10.1016/j.ejvs.2012.11.008 * A. Bhatnagar, Sanjay Gandhi Post Graduate Institute of Medical Sciences, NH24B, Lucknow 226014, Uttar Pradesh, India. Tel.: þ91 5222418648. E-mail address: [email protected] (A. Bhatnagar). 1078-5884 Ó 2012 European Society for Vascular Surgery. Published by Elsevier Ltd. Open access under CC BY-NC-ND license. http://dx.doi.org/10.1016/j.ejvsextra.2012.11.002
Figure 1. Venous malformation at root of neck with infra clavicular extent.
e10
EJVES Extra
Figure 2. Very large communication between VM and EJV.
identified. Multiple feeding vessels were identified in the first two cases while the third patient showed a large communicating vein (Fig. 2). Feeding vessels were ligated, and lesion completely excised. All patients underwent subcuticular skin closure after ensuring meticulous haemostasis and advised routine anti-scar measures. Macroscopically, the excised lesion was a well-circumscribed darkbrown nodule. Microscopically, the nodule was composed of ectatic vascular spaces with a thin wall and lined by a layer of endothelium. Smooth muscle was found in the wall of some vessels. Histological analysis was compatible with VM. DISCUSSION VMs have an incidence of one to two in 10,000 births, prevalence of 1%, are present at birth and grow proportionately with age causing various clinical presentations.1,2 Slow-flow VM closely associated with the EJV is rare.3 Less than 10 cases have been described.4 They are connected to the EJV by single or multiple veins, mostly of large calibre. Histologic abnormalities of the smooth muscleepericyte component within vascular channel walls of VMs are hypothesised as a potential cause of many VMs.2 Jackson and co-workers identified the need for further augmentation of Mulliken’s classification to answer the (therapeutic) questions of ‘what to do’ and ‘when to do it’.2 Overzealous and incomplete treatment can, on many occasions, lead to worsening of features. Post-treatment morbidity can be more distressing for the patients as compared to their pre-treatment status. Treatment is
Volume 25 Issue 2 February/2013
indicated for lesions causing pain and for functional and aesthetic impairment. Pain is due to venous engorgement and local stretching of tissues.2 Sclerotherapy has become the mainstay of treatment for cervicofacial VM. The most important variables predicting successful sclerotherapy are sclerosant concentration and dwell time, especially when there are multiple wide channels of communication between the VM and its draining vein as in our cases. Sclerotherapy failed in our patients due to small contact time of the sclerosant with the endothelium as most of the drug was washed off. The major complications of sclerotherapy include skin necrosis, residual lesions, skin pigmentation, peripheral nerve palsies and hypotensive crisis, as well as fatalities such as cardiac arrest and pulmonary emboli. Two of our cases had significant skin involvement. The patient had undergone three attempts at sclerotherapy at some other treatment centre. Keeping the risk of skin necrosis, unsuccessful sclerotherapy and proximity of the EJV in mind, surgical excision was planned. Surgery for this lesion was relatively easy as no vital structures were present in the vicinity of the lesion. In VM arising from EJV, the vein is generally normal and can be spared during the excision. Its direct and numerous communications with the EJV render sclerosing it a ‘more than usually’ hazardous option for treatment. Considering that surgical excision of these lesions is rather simple and straightforward, the author would recommend it to be considered as the first line of treatment whenever this clinical entity is recognised. Surgery ensures least down time, morbidity and recurrence rate.5 Clinicians dealing with vascular problems should be aware of this lesion where early surgical intervention provides optimal results. CONFLICT OF INTEREST/FUNDING None. REFERENCES 1 Lee IH, Kim KH, Jeon P, Hong SB, Kim H-J, Kim ST, et al. Ethanol sclerotherapy for the management of craniofacial venous malformations: the interim results. Korean J Radiol 2009;10(3):269e76. 2 Legiehn GM, Manraj KS. Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin N Am 2008;46:545e97. 3 Jose Â, Luis O, Rodrõaguez G, Fernandez J, Gregorio M. Jugular venous malformation in an 8-year-old boy: treatment with endovascular sclerotherapy. Eur J Pediatr 2001;160:392e4. 4 Ahuja AT, Yuen Hok-Yuen, Wong Ka-Tak, King Ann D, Abdullah Victor, To Edward, et al. External jugular vein vascular malformation: sonographic and MR imaging appearances. Am J Neuroradiol 2004;25:338e42. 5 Kang GCW, Song C. Forty-one cervicofacial vascular anomalies and their surgical treatment e retrospection and review. Ann Acad Med Singap 2008;37:165e79.