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Ruptured external jugular varix
Ruptured external jugular varix Bogdan A. Kindzelski, BS,a Huiting Chen, MD,a Yogendra Kanthi, MD,b Thomas Wakefield, MD,a and Dawn M. Coleman, MD,a Ann Arbor, Mich Venous dilations of the jugular veins are uncommon and external jugular varix even rarer. We present the case of a 57-year-old woman with ruptured external jugular varix and the surgical repair of such. Surgical
intervention is warranted in these rare venous malformations to prevent known complications of thromboembolism and rupture. (J Vasc Surg: Venous and Lym Dis 2016;-:1-3.)
A varix is a venous anomaly characterized by dilatation and tortuosity. Isolated external jugular varices are extremely rare, with only a few cases reported in the literature.1,2 Conversely, congenital phlebectasia, defined as outward dilatation of the vein without tortuosity, has been described in >100 cases involving pediatric neck veins.3-6 We present a case of external jugular varix with subsequent rupture requiring urgent surgical intervention. Consent was obtained from the patient for publication of this case report.
Repeat duplex imaging at this time showed a nonvascularized mass in the right neck measuring 2.86 cm in maximum transverse diameter and 4.7 cm in maximum longitudinal diameter. Subsequent computed tomography venogram imaging confirmed superficial venous rupture with resulting anterior cervical hematoma (Fig 2). Urgent repair was recommended. A left cervical oblique incision within a skin crease was created sharply across the mass and subplatysmal flaps created. The hematoma and associated ruptured varix was encountered with a punctate area visible anteriorly extruding subacute clot (Fig 3). The external jugular vein distal to the hematoma was identified and ligated. The ruptured varix was circumferentially dissected sharply and enucleated with evacuation of the thrombus, which was sent for culture and pathologic analysis. There remained inflow branches off the proximal external jugular vein; all branches were ligated and the variceal remnants were resected and sent for pathologic analysis. The wound was closed in a standard fashion. The patient tolerated the procedure well and was discharged home on the same day without event. Histopathological analysis showed vein with thrombus and no evidence of neoplastic changes. Culture was negative for pathogens.
CASE A 57-year-old woman with a history of asymptomatic right carotid artery stenosis and hypothyroidism presented electively with a superficial, painless right neck mass of 9 months duration. She described no concerning neurologic symptoms, dysphagia, or odynophagia. Physical examination revealed a soft, compressible superficial mass measuring approximately 3 cm in maximum diameter overlying the anterior right neck. The mass became visibly more prominent with Valsalva maneuver and speech. Diagnostic evaluation using cervical duplex and axial imaging revealed a superficial, tortuous, and compressible cystic mass suggestive of a right external jugular varix without evidence of thrombus. Elective surgical excision was recommended for the risk of thromboembolism in addition to cosmetic concerns voiced by the patient. The patient re-presented in the interim with enlargement of the mass associated with new pain (Fig 1). She continued to deny focal neurologic symptoms, dysphagia, difficulty breathing, or stridor. The mass was no longer soft and compressible in clinical examination, but rather firm and more consistent with hematoma.
From the Division of Vascular Surgerya and Division of Cardiovascular Medicine,b University of Michigan. Author conflict of interest: none. Correspondence: Dawn M. Coleman, MD, University of Michigan Cardiovascular Center, 1500 E Medical Center Dr, Ann Arbor, MI 48109-5867 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 2213-333X Copyright Ó 2016 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvsv.2016.03.003
DISCUSSION Venous dilations in general are common, with an estimated >25 million Americans affected by varicose veins of the lower extremities.7 Similar venous malformations in the neck veins are rare. Jugular vein phlebectasia is a congenital fusiform or spindle dilation of the jugular vein that generally presents in children around the age of 10 years as a soft compressible mass that becomes more prominent with increased intrathoracic pressure.4,8 Although a unilateral internal jugular vein is most commonly affected in these pediatric cases, bilateral cases have been described along with cases affecting the anterior and external jugular veins. Surgical indications remain controversial, however, intervention is often recommended for risks of thromboembolism, theoretic risk of rupture, and cosmesis.4 Unfortunately, long-term follow-up for these children is limited and it remains unclear if pediatric phlebectasia is a risk factor for jugular venous malformation or varix in adulthood. In adults, jugular venous ‘phlebectasia’ is restricted to individual case reports.9 ‘Varices’ of the jugular veins, which entail dilation and tortuosity, are even more 1
2 Kindzelski et al
JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERS --- 2016
Fig 2. Computed tomography venogram at the time of interim presentation showing interval enlargement of a 4.3- 3.2- 3.7-cm lesion centered just lateral to the right sternocleidomastoid muscle, intimately associated with the right external jugular vein.
Fig 1. Patient photograph at interim presentation. The enlarged right cervical mass resulting from rupture of an external jugular varix and associated hematoma is shown.
sporadic. A review of the literature showed only two previous cases of external jugular ‘varix’ detected using head and neck ultrasound imaging and an additional case of external jugular venous ‘aneurysm.’1,2,10 Inconsistent terminology across the literature along with the rarity of external jugular venous malformations limits our understanding of the true natural history. This patient specifically noted symptoms for only 9 months duration with enlargement over time and gave no history of cervical mass or venous dilation in childhood or young adulthood when questioned. The authors cannot presume a diagnosis of congenital jugular phlebectasia in this specific case and combined tortuosity accompanying dilation of this superficial external jugular vein justifies the classification of this malformation as a varix. Mean external jugular venous diameter has previously been identified as 9.7 mm (range, 0-16 mm) in a patient population of 50 adults.11 Aneurysmal dilation of the face and neck veins (including the external jugular vein) might best be defined as $20 mm.10 Cervical venous duplex scanning with provocative Valsalva maneuver should be considered the diagnostic gold standard for cases of suspected cervical venous malformation with contrastenhanced axial imaging (ie, computed tomography venogram or magnetic resonance venogram imaging) reserved
for select cases and surgical planning. Because of the rarity of the condition, it is difficult to establish a set of guidelines for intervention because the natural history remains unclear. Elective excision of an external jugular varix or venous aneurysm (>2 cm) at the time of diagnosis should be considered for cosmesis and risks of rupture and thromboembolism.12,13 Resection seems clearly indicated for patients with symptoms or those showing enlargement with surveillance imaging. Histopathologic analysis is necessary to exclude malignancy because external jugular varix has been associated with hemangioendothelioma.1
Fig 3. Intraoperative photograph showing the thrombosed external jugular varix with associated hematoma.
JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERS Volume -, Number -
CONCLUSIONS External jugular varix is a rare venous malformation. To our knowledge, this report represents the first instance of rupture and hematoma complicating an external jugular venous malformation. In the case of suspected external jugular varix, surgical resection is indicated to prevent or treat thromboembolism and rupture. Simple surgical excision can be performed safely with negligible morbidity. REFERENCES 1. Lau H, Cheng SW. External jugular varix with an intravascular hemangioendothelioma. Surgery 1998;123:359-61. 2. Ajuluchuku EU, Da-Rocha Afodu JT, Bode C, Adebanjo AK, Elesha SO. Varix of the external jugular vein. Thorac Cardiovasc Surg 1986;34:135-6. 3. Paleri V, Gopalakrishnan S. Jugular phlebectasia: theory of pathogenesis and review of literature. Int J Pediatr Otorhinolaryngol 2001;57: 155-9. 4. Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children. Am J Surg 2006;192:286-90.
Kindzelski et al 3
5. Hu X, Li J, Hu T, Jiang X. Congenital jugular vein phlebectasia. Am J Otolaryngol 2005;26:172-4. 6. Hung T, Campbell AI. Surgical repair of left internal jugular phlebectasia. J Vasc Surg 2008;47:1337-8. 7. White JV, Ryjewski C. Chronic venous insufficiency. Perspect Vasc Surg Endovasc Ther 2005;17:319-27. 8. Lubianca-Neto JF, Mauri M, Prati C. Internal jugular phlebectasia in children. Am J Otolaryngol 1999;20:415-8. 9. Siani A, Flaishman I, Schioppa A, Zaccaria A, Baldassarre E. Jugular venous phlebectasia: uncommon in children, anecdotal in adults. Am J Surg 2008;195:419-20. 10. Calligaro KD, Ahmad S, Dandora R, Dougherty MJ, Savarese RP, Doerr KJ, et al. Venous aneurysms: surgical indications and review of the literature. Surgery 1995;117:1-6. 11. Stickle BR, McFarlane H. Prediction of a small internal jugular vein by external jugular vein diameter. Anaesthesia 1997;52:220-2. 12. Matsunaga K, Kishi K. Phlebectasia of the external jugular vein with thrombosis: report of a case. Surg Today 2014;44:1180-3. 13. Ioannou CV, Kostas T, Tsetis D, Georgakarakos E, Gionis M, Katsamouris AN. External jugular vein aneurysm: a source of thrombotic complications. Int Angiol 2010;29:284-5. Submitted Feb 4, 2016; accepted Mar 12, 2016.
intervention is warranted in these rare venous malformations to prevent known complications of thromboembolism and rupture. (J Vasc Surg: Venous and Lym Dis 2016;-:1-3.)
A varix is a venous anomaly characterized by dilatation and tortuosity. Isolated external jugular varices are extremely rare, with only a few cases reported in the literature.1,2 Conversely, congenital phlebectasia, defined as outward dilatation of the vein without tortuosity, has been described in >100 cases involving pediatric neck veins.3-6 We present a case of external jugular varix with subsequent rupture requiring urgent surgical intervention. Consent was obtained from the patient for publication of this case report.
Repeat duplex imaging at this time showed a nonvascularized mass in the right neck measuring 2.86 cm in maximum transverse diameter and 4.7 cm in maximum longitudinal diameter. Subsequent computed tomography venogram imaging confirmed superficial venous rupture with resulting anterior cervical hematoma (Fig 2). Urgent repair was recommended. A left cervical oblique incision within a skin crease was created sharply across the mass and subplatysmal flaps created. The hematoma and associated ruptured varix was encountered with a punctate area visible anteriorly extruding subacute clot (Fig 3). The external jugular vein distal to the hematoma was identified and ligated. The ruptured varix was circumferentially dissected sharply and enucleated with evacuation of the thrombus, which was sent for culture and pathologic analysis. There remained inflow branches off the proximal external jugular vein; all branches were ligated and the variceal remnants were resected and sent for pathologic analysis. The wound was closed in a standard fashion. The patient tolerated the procedure well and was discharged home on the same day without event. Histopathological analysis showed vein with thrombus and no evidence of neoplastic changes. Culture was negative for pathogens.
CASE A 57-year-old woman with a history of asymptomatic right carotid artery stenosis and hypothyroidism presented electively with a superficial, painless right neck mass of 9 months duration. She described no concerning neurologic symptoms, dysphagia, or odynophagia. Physical examination revealed a soft, compressible superficial mass measuring approximately 3 cm in maximum diameter overlying the anterior right neck. The mass became visibly more prominent with Valsalva maneuver and speech. Diagnostic evaluation using cervical duplex and axial imaging revealed a superficial, tortuous, and compressible cystic mass suggestive of a right external jugular varix without evidence of thrombus. Elective surgical excision was recommended for the risk of thromboembolism in addition to cosmetic concerns voiced by the patient. The patient re-presented in the interim with enlargement of the mass associated with new pain (Fig 1). She continued to deny focal neurologic symptoms, dysphagia, difficulty breathing, or stridor. The mass was no longer soft and compressible in clinical examination, but rather firm and more consistent with hematoma.
From the Division of Vascular Surgerya and Division of Cardiovascular Medicine,b University of Michigan. Author conflict of interest: none. Correspondence: Dawn M. Coleman, MD, University of Michigan Cardiovascular Center, 1500 E Medical Center Dr, Ann Arbor, MI 48109-5867 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 2213-333X Copyright Ó 2016 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvsv.2016.03.003
DISCUSSION Venous dilations in general are common, with an estimated >25 million Americans affected by varicose veins of the lower extremities.7 Similar venous malformations in the neck veins are rare. Jugular vein phlebectasia is a congenital fusiform or spindle dilation of the jugular vein that generally presents in children around the age of 10 years as a soft compressible mass that becomes more prominent with increased intrathoracic pressure.4,8 Although a unilateral internal jugular vein is most commonly affected in these pediatric cases, bilateral cases have been described along with cases affecting the anterior and external jugular veins. Surgical indications remain controversial, however, intervention is often recommended for risks of thromboembolism, theoretic risk of rupture, and cosmesis.4 Unfortunately, long-term follow-up for these children is limited and it remains unclear if pediatric phlebectasia is a risk factor for jugular venous malformation or varix in adulthood. In adults, jugular venous ‘phlebectasia’ is restricted to individual case reports.9 ‘Varices’ of the jugular veins, which entail dilation and tortuosity, are even more 1
2 Kindzelski et al
JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERS --- 2016
Fig 2. Computed tomography venogram at the time of interim presentation showing interval enlargement of a 4.3- 3.2- 3.7-cm lesion centered just lateral to the right sternocleidomastoid muscle, intimately associated with the right external jugular vein.
Fig 1. Patient photograph at interim presentation. The enlarged right cervical mass resulting from rupture of an external jugular varix and associated hematoma is shown.
sporadic. A review of the literature showed only two previous cases of external jugular ‘varix’ detected using head and neck ultrasound imaging and an additional case of external jugular venous ‘aneurysm.’1,2,10 Inconsistent terminology across the literature along with the rarity of external jugular venous malformations limits our understanding of the true natural history. This patient specifically noted symptoms for only 9 months duration with enlargement over time and gave no history of cervical mass or venous dilation in childhood or young adulthood when questioned. The authors cannot presume a diagnosis of congenital jugular phlebectasia in this specific case and combined tortuosity accompanying dilation of this superficial external jugular vein justifies the classification of this malformation as a varix. Mean external jugular venous diameter has previously been identified as 9.7 mm (range, 0-16 mm) in a patient population of 50 adults.11 Aneurysmal dilation of the face and neck veins (including the external jugular vein) might best be defined as $20 mm.10 Cervical venous duplex scanning with provocative Valsalva maneuver should be considered the diagnostic gold standard for cases of suspected cervical venous malformation with contrastenhanced axial imaging (ie, computed tomography venogram or magnetic resonance venogram imaging) reserved
for select cases and surgical planning. Because of the rarity of the condition, it is difficult to establish a set of guidelines for intervention because the natural history remains unclear. Elective excision of an external jugular varix or venous aneurysm (>2 cm) at the time of diagnosis should be considered for cosmesis and risks of rupture and thromboembolism.12,13 Resection seems clearly indicated for patients with symptoms or those showing enlargement with surveillance imaging. Histopathologic analysis is necessary to exclude malignancy because external jugular varix has been associated with hemangioendothelioma.1
Fig 3. Intraoperative photograph showing the thrombosed external jugular varix with associated hematoma.
JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERS Volume -, Number -
CONCLUSIONS External jugular varix is a rare venous malformation. To our knowledge, this report represents the first instance of rupture and hematoma complicating an external jugular venous malformation. In the case of suspected external jugular varix, surgical resection is indicated to prevent or treat thromboembolism and rupture. Simple surgical excision can be performed safely with negligible morbidity. REFERENCES 1. Lau H, Cheng SW. External jugular varix with an intravascular hemangioendothelioma. Surgery 1998;123:359-61. 2. Ajuluchuku EU, Da-Rocha Afodu JT, Bode C, Adebanjo AK, Elesha SO. Varix of the external jugular vein. Thorac Cardiovasc Surg 1986;34:135-6. 3. Paleri V, Gopalakrishnan S. Jugular phlebectasia: theory of pathogenesis and review of literature. Int J Pediatr Otorhinolaryngol 2001;57: 155-9. 4. Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children. Am J Surg 2006;192:286-90.
Kindzelski et al 3
5. Hu X, Li J, Hu T, Jiang X. Congenital jugular vein phlebectasia. Am J Otolaryngol 2005;26:172-4. 6. Hung T, Campbell AI. Surgical repair of left internal jugular phlebectasia. J Vasc Surg 2008;47:1337-8. 7. White JV, Ryjewski C. Chronic venous insufficiency. Perspect Vasc Surg Endovasc Ther 2005;17:319-27. 8. Lubianca-Neto JF, Mauri M, Prati C. Internal jugular phlebectasia in children. Am J Otolaryngol 1999;20:415-8. 9. Siani A, Flaishman I, Schioppa A, Zaccaria A, Baldassarre E. Jugular venous phlebectasia: uncommon in children, anecdotal in adults. Am J Surg 2008;195:419-20. 10. Calligaro KD, Ahmad S, Dandora R, Dougherty MJ, Savarese RP, Doerr KJ, et al. Venous aneurysms: surgical indications and review of the literature. Surgery 1995;117:1-6. 11. Stickle BR, McFarlane H. Prediction of a small internal jugular vein by external jugular vein diameter. Anaesthesia 1997;52:220-2. 12. Matsunaga K, Kishi K. Phlebectasia of the external jugular vein with thrombosis: report of a case. Surg Today 2014;44:1180-3. 13. Ioannou CV, Kostas T, Tsetis D, Georgakarakos E, Gionis M, Katsamouris AN. External jugular vein aneurysm: a source of thrombotic complications. Int Angiol 2010;29:284-5. Submitted Feb 4, 2016; accepted Mar 12, 2016.