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Jugular venous phlebectasia: uncommon in children, anecdotical in adults
Letters to the Editor / The American Journal of Surgery 195 (2008) 419 – 420
419
Jugular venous phlebectasia: uncommon in children, anecdotical in adults To the Editor: We read with great interest the article recently published by Jianhong et al, which described 51 cases of jugular vein phlebectasia in childhood. As reported by the investigators, phlebectasias are uncommon causes of neck swelling and, when in children in particular, seem to be related to weakness of the congenital elastic layers and muscle cells [1]. Several theories– such as trauma, local inflammation, and increased pressure in the vena cava system– have been proposed for phlebectasias in adults [2,3]. The etiology, natural history, diagnosis, and treatment of phlebectasias have not yet been elucidated. Here we report the case of a 33-year-old woman who was admitted to the Department of Vascular Surgery at our hospital for asymptomatic swelling on the left side of the neck. On previous examination, lymphadenopathy was suspected, and Doppler ultrasound scanning showed true fusiform dilatation of the left external jugular vein. Her clinical history was negative for episodes of trauma, local or systemic inflammation, other significant diseases, and interventions. On clinical examination, the mass was located in the middle part of external jugular vein behind the sternocleidomastoid muscle. The mass measured approximately 2 ⫻ 3 cm and was soft, with absence of abnormal pulsations. No other superficial veins in the neck, thorax, abdomen, or upper and lower limbs were apparently involved in the aneurysmal degeneration. The remainder of the clinical examination, blood test results, and hormone assessment were all normal. Doppler ultrasound scanning was performed and showed a fusiform focal dilatation, measuring 2 ⫻ 2.8 cm and with partial thrombosis. No thoracic outlet syndrome, jugular vein lesions, or malformations were observed. The intervention was performed with the patient under local anesthesia. The dilatated vein was easily exposed using a 3-cm longitudinal presternocleido incision. After proximal and distal ligation of the external jugular vein was performed, the aneurysmal portion of the vein was extracted (Fig. 1). On microscopy, it was evident that it was a true dilatation of the jugular vein, with wall thickness and focal absence of smooth-muscle cells. The patient’s postoperative course was uneventful, and she was discharged on postoperative day 1. At 1-year follow-up, the patient appeared healthy, and no venous aneurysms were found. As reported by Jianhong et al, the majority of neck venous phlebectasias involve the internal jugular vein, followed by the external and anterior veins. Idiopathic external jugular vein (IEJV) phlebectasias in the adult patient are rare, and only a few cases have been reported in the literature [4,5]. Primitive forms of phlebectasias, which are typical in pediatric patients, do not present a clear etiology, and histologic examination usually shows congested venous structures, with thinning of the elastic layer. The acquired forms of phlebectasias, which are seen more frequently among adult patients, may be caused by trauma, thoracic
Fig. 1. Intraoperative photograph showing external jugular vein phlebectasia after dissection and distal as well as proximal ligation of the external jugular vein through a presternocleido muscle incision.
outlet syndrome, tumors, local or systemic inflammatory status, hormonal therapy, or increased pressure in the vena cava system. In our patient, a complete hormonal profile and blood tests were performed, and no evidence of alteration was noted. The IEJV phlebectasias occur more frequently in male patients and occur more often on the right side of the neck. Differential diagnosis to rule out other neck masses– such as cystic hygroma, lymphadenopathy, thyroid swelling, and thyroglossal cyst–may be difficult. On clinical examination, the presence of a soft, but not throbbing, mass with enlargement during the Valsalva maneuver generally suggests an IEJV aneurysm. Doppler ultrasound scanning is the examination of choice to confirm the diagnosis. Computed axial tomography and magnetic resonance imaging are helpful in clarifying the diagnosis in cases of suspected intrathoracic pathology or malformative jugular vein duplication causing venous phlebectasia [6,7]. These lesions, such as that in our patient, are generally asymptomatic. Cases of thromboembolism, phlebitis, rupture, or other lifethreatening complications have not been reported in these patients, and surgical treatment is usually indicated for cosmetic purposes or in symptomatic patients [7–9]. Andrea Siani, M.D. Isaac Flaishman, M.D. Annalisa Schioppa, M.D. Alvaro Zaccaria, M.D. Department of Vascular Surgery San Pietro Hospital–Fatebenefratelli Rome, Italy Emanuele Baldassarre, M.D. Department of Urology Regional Hospital Aosta, Italy doi:10.1016/j.amjsurg.2007.01.041
420
Letters to the Editor / The American Journal of Surgery 195 (2008) 419 – 420
References [1] Porcellini M, Selvetella L, Bernardo B, et al. Aneurysms of external jugular vein. G Chir 1996;17:238 – 41. [2] Verbeeck N, Hammer F, Goffette P, et al. Saccular aneurysm of external jugular vein, an unusual cause of neck swelling. J Belge Radiol 1997;80:63– 4. [3] Al-Shaikhi A, Kay S, Laberge JM. External jugular venous aneurysm: an unusual cause of a neck mass in young child. J Pediatr Surg 2003;38:1557–9. [4] Ekim H, Ozen S. Primary venous aneurysm of the external jugular vein. Eur J Med 2002;7:25–5.
[5] Regina G, Rizzo S, Impedova G. Aneurysm of external jugular vein: case report and review of literature. A case report. Angiology 1990; 108:92–5. [6] Karapolat S, Erkut B, Unlu Y. Multiple aneurysms of the left external jugular vein. Turk J Med Sci 2005;35:43–5. [7] Zannella FE, Brusis T, Modder U. Use of imaging procedures in the diagnosis of jugular vein thrombosis. Laryngol Rhinol Otol 1986;65:322–6. [8] Calligaro KD, Ahmad S, Dandora R, et al. Venous aneurysms: surgical indications and review of the literature. Surgery 1995;117:1– 6. [9] Andrev A, Petkov D, Kavrakov T, et al. Jugular venous aneurysms: when and how to operate. Int Angiol 1998;17:272–5.
419
Jugular venous phlebectasia: uncommon in children, anecdotical in adults To the Editor: We read with great interest the article recently published by Jianhong et al, which described 51 cases of jugular vein phlebectasia in childhood. As reported by the investigators, phlebectasias are uncommon causes of neck swelling and, when in children in particular, seem to be related to weakness of the congenital elastic layers and muscle cells [1]. Several theories– such as trauma, local inflammation, and increased pressure in the vena cava system– have been proposed for phlebectasias in adults [2,3]. The etiology, natural history, diagnosis, and treatment of phlebectasias have not yet been elucidated. Here we report the case of a 33-year-old woman who was admitted to the Department of Vascular Surgery at our hospital for asymptomatic swelling on the left side of the neck. On previous examination, lymphadenopathy was suspected, and Doppler ultrasound scanning showed true fusiform dilatation of the left external jugular vein. Her clinical history was negative for episodes of trauma, local or systemic inflammation, other significant diseases, and interventions. On clinical examination, the mass was located in the middle part of external jugular vein behind the sternocleidomastoid muscle. The mass measured approximately 2 ⫻ 3 cm and was soft, with absence of abnormal pulsations. No other superficial veins in the neck, thorax, abdomen, or upper and lower limbs were apparently involved in the aneurysmal degeneration. The remainder of the clinical examination, blood test results, and hormone assessment were all normal. Doppler ultrasound scanning was performed and showed a fusiform focal dilatation, measuring 2 ⫻ 2.8 cm and with partial thrombosis. No thoracic outlet syndrome, jugular vein lesions, or malformations were observed. The intervention was performed with the patient under local anesthesia. The dilatated vein was easily exposed using a 3-cm longitudinal presternocleido incision. After proximal and distal ligation of the external jugular vein was performed, the aneurysmal portion of the vein was extracted (Fig. 1). On microscopy, it was evident that it was a true dilatation of the jugular vein, with wall thickness and focal absence of smooth-muscle cells. The patient’s postoperative course was uneventful, and she was discharged on postoperative day 1. At 1-year follow-up, the patient appeared healthy, and no venous aneurysms were found. As reported by Jianhong et al, the majority of neck venous phlebectasias involve the internal jugular vein, followed by the external and anterior veins. Idiopathic external jugular vein (IEJV) phlebectasias in the adult patient are rare, and only a few cases have been reported in the literature [4,5]. Primitive forms of phlebectasias, which are typical in pediatric patients, do not present a clear etiology, and histologic examination usually shows congested venous structures, with thinning of the elastic layer. The acquired forms of phlebectasias, which are seen more frequently among adult patients, may be caused by trauma, thoracic
Fig. 1. Intraoperative photograph showing external jugular vein phlebectasia after dissection and distal as well as proximal ligation of the external jugular vein through a presternocleido muscle incision.
outlet syndrome, tumors, local or systemic inflammatory status, hormonal therapy, or increased pressure in the vena cava system. In our patient, a complete hormonal profile and blood tests were performed, and no evidence of alteration was noted. The IEJV phlebectasias occur more frequently in male patients and occur more often on the right side of the neck. Differential diagnosis to rule out other neck masses– such as cystic hygroma, lymphadenopathy, thyroid swelling, and thyroglossal cyst–may be difficult. On clinical examination, the presence of a soft, but not throbbing, mass with enlargement during the Valsalva maneuver generally suggests an IEJV aneurysm. Doppler ultrasound scanning is the examination of choice to confirm the diagnosis. Computed axial tomography and magnetic resonance imaging are helpful in clarifying the diagnosis in cases of suspected intrathoracic pathology or malformative jugular vein duplication causing venous phlebectasia [6,7]. These lesions, such as that in our patient, are generally asymptomatic. Cases of thromboembolism, phlebitis, rupture, or other lifethreatening complications have not been reported in these patients, and surgical treatment is usually indicated for cosmetic purposes or in symptomatic patients [7–9]. Andrea Siani, M.D. Isaac Flaishman, M.D. Annalisa Schioppa, M.D. Alvaro Zaccaria, M.D. Department of Vascular Surgery San Pietro Hospital–Fatebenefratelli Rome, Italy Emanuele Baldassarre, M.D. Department of Urology Regional Hospital Aosta, Italy doi:10.1016/j.amjsurg.2007.01.041
420
Letters to the Editor / The American Journal of Surgery 195 (2008) 419 – 420
References [1] Porcellini M, Selvetella L, Bernardo B, et al. Aneurysms of external jugular vein. G Chir 1996;17:238 – 41. [2] Verbeeck N, Hammer F, Goffette P, et al. Saccular aneurysm of external jugular vein, an unusual cause of neck swelling. J Belge Radiol 1997;80:63– 4. [3] Al-Shaikhi A, Kay S, Laberge JM. External jugular venous aneurysm: an unusual cause of a neck mass in young child. J Pediatr Surg 2003;38:1557–9. [4] Ekim H, Ozen S. Primary venous aneurysm of the external jugular vein. Eur J Med 2002;7:25–5.
[5] Regina G, Rizzo S, Impedova G. Aneurysm of external jugular vein: case report and review of literature. A case report. Angiology 1990; 108:92–5. [6] Karapolat S, Erkut B, Unlu Y. Multiple aneurysms of the left external jugular vein. Turk J Med Sci 2005;35:43–5. [7] Zannella FE, Brusis T, Modder U. Use of imaging procedures in the diagnosis of jugular vein thrombosis. Laryngol Rhinol Otol 1986;65:322–6. [8] Calligaro KD, Ahmad S, Dandora R, et al. Venous aneurysms: surgical indications and review of the literature. Surgery 1995;117:1– 6. [9] Andrev A, Petkov D, Kavrakov T, et al. Jugular venous aneurysms: when and how to operate. Int Angiol 1998;17:272–5.