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Congenital jugular vein phlebectasia
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 26 (2005) 172 – 174 www.elsevier.com/locate/amjoto
Congenital jugular vein phlebectasia Xianliang Hua, Jianhong Lia,T, Tingze Hub, Xuewu Jianga a
Department of Pediatric Surgery, Medical College of Shantou University, Shantou City, Guangdong Province, China Department of Pediatric Surgery, West-China Medical Center of Sichuan University, Chengdu, Sichuan Province, China
b
Revised 19 January 2005
Abstract
Background/purpose: The purpose of the present study was to elucidate the clinical presentation, diagnosis, treatment, and postoperative complications of jugular vein phlebectasia (JVP), and to recommend diagnostic methods and treatment choices. Materials and methods: Thirty-six cases of JVP were reviewed (right vein in 27, left in 6, and bilateral in 3). The internal jugular veins were the most commonly involved. The main complaint was the soft and compressible mass in the neck. Ultrasound or color Doppler flow imaging (CDFI) was performed on all the children. Surgical intervention was performed in 32 cases, and the other 4 cases were conservatively followed up for 4 to 6 years. Results: Ultrasound or CDFI demonstrated local dilatation of the unilateral or bilateral veins in all the patients. Surgical intervention included ligation of the involved jugular vein in 31 cases and draping with medical Dacron cloth in 1 case. All of the operated children recovered uneventfully, except 3 for cases who underwent ligation of the right internal jugular vein. Conclusions: Valsalva maneuver was most important for establishing the diagnosis. Ultrasound or CDFI was the diagnostic procedure of choice to confirm the diagnosis of JVP. Surgical ligation or excision of unilateral jugular vein was recommended for cosmetic and psychological purposes. However, special attention must be paid to prevent postoperative complications in case of ligation of right internal jugular vein. Or else, treatment should be conservative (follow-up). D 2005 Elsevier Inc. All rights reserved.
1. Introduction
2. Materials and methods
Jugular vein phlebectasia (JVP) is a congenital fusiform dilatation of the jugular vein that appears as a soft, compressible mass in the neck during straining or triggered by the Valsalva maneuver. It has been described in almost all cervical veins and is usually asymptomatic. Because of its rarity, this entity is frequently ignored or misdiagnosed [1]. Until 2001, about 70 cases had been reported in the English medical literature. In the recent 20 years, 36 cases had been admitted into our hospital, and in this report the experiences of management were discussed combined with the related literature.
Among 36 cases (24 male, 12 female), the ages ranged from 1.5 to 15 years with an average of 8.4 years. The dilatation involved bilateral jugular veins in 3 cases, right vein in 27, and left in 6. The internal jugular veins were involved in 29 cases (3 bilateral, 22 right, and 4 left), and the external jugular veins were involved in 7 cases (5 right, 2 left). There was no history of trauma or inflammation to the neck or the upper thorax. The soft and compressible mass in the neck was usually the main complaint. Occasionally, some children complained of slight discomfort or pain during deglutition. On physical examination, the soft mass was well demarcated without bruit and pulsation. Valsalva maneuver was performed in all the patients. Ultrasound or color Doppler flow imaging (CDFI) was performed on all the children and demonstrated local dilatation of the involved unilateral or bilateral veins. Surgical intervention was performed in
T Corresponding author. Department of Pediatric Surgery, Medical College of Shantou University, Shantou City, Guangdong Province, 515031, China. Tel.: +86 754 8528370. E-mail address: [email protected] (J. Li). 0196-0709/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.02.009
X. Hu et al. / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 26 (2005) 172–174
32 cases: the involved vein was ligated or excised in 31, and 1 patient underwent draping of the dilated vein with medical Dacron cloth. And the other 4 cases were conservatively followed up for 4 to 6 years. 3. Results Valsalva maneuver showed positive results in all 36 patients and was most important for establishing the diagnosis. Ultrasound or CDFI demonstrated local fusiform dilatation of the involved unilateral or bilateral veins in all the patients. During the Valsalva maneuver the diameter of the dilatation increased at least one time in comparison with that at rest, which undoubtedly confirmed the diagnosis. Among 32 cases treated by operations, 1 case with bilateral internal JVP underwent draping of the dilated left vein with medical Dacron cloth because marked discomforts appeared when either side of the internal jugular vein was temporarily occluded. For this patient, the proximal part of the dilatation was first compressed until the dilated vein restored their normal diameter, then the vein was wrapped longitudinally with Dacron cloth. For the other 31 cases, the involved vein was ligated or excised (the right internal jugular vein was ligated in the 2 cases with bilateral lesions) and sent for pathological examination. Postoperative complications occurred in 3 children. The right internal jugular vein was ligated after being temporarily occluded for more than 10 minutes in 2 children with bilateral lesions, but ipsilateral neck and craniofacial swelling appeared soon after the operation. These 2 children were intensively cared for, and the swelling disappeared spontaneously in 3 days. However, in another child with right internal JVP, severe intracranial hypertension symptoms such as headache and vomiting appeared soon after ligation, although cerebral flow temporary occlusion test showed that the right internal jugular vein could be ligated. Magnetic resonance imaging showed thrombosis of the right internal jugular vein and lacular infarction of the right pontine. This child was given mannitol and flurosemide, and in 1 week the headache and vomiting disappeared. Three months later, magnetic resonance imaging showed the thrombosis and infarction did not exist and he recovered completely. 3.1. Pathological examination Generally, there were no pathological characteristics in the specimens. In some cases, the vein wall was structurally normal; but in other cases, the wall became thinner; the elastic fibers and muscle fibers decreased or disappeared. 4. Discussion Phlebectasia of the jugular veins is a venous anomaly that usually presents in children as a soft cystic swelling in the neck during straining. The term phlebectasia indicates abnormal outward dilatation of the vein without tortuosity
173
and differs from the term varix, which implies dilatation plus tortuosity [2]. Because there have been only sporadic reports of JVP, the exact etiology of this lesion still remains unclear. The possible causes of JVP are as follows: gross anatomic abnormality of the vein, mechanical compression or trauma of the vein, congenital structural defects in the vein wall, and idiopathy [3]. In our opinion, it is thought that congenital structural defects in the vein wall play an important role in the etiology, which has been partly proved by the results of pathological examination, so JVP is considered as a congenital venous anomaly. In our cases, 24 children (67%) were admitted before the age of 7 years, which also suggests a possible congenital origin. Even in the 4 adult women operated on in our hospital, JVP was first noticed in their teens and they were followed up for more than 20 years. Although phlebectasia might affect anterior, external, and internal jugular veins, in the majority of the patients the internal jugular vein was most frequently involved, which was evidenced in 29 cases (80.6%) of the patients in our group, and in all of 3 cases with bilateral lesions the internal jugular vein was affected, too. Jugular phlebectasia occurred with more frequency on the right side, as evidenced by our data. Clinically, this condition presented with enlargement with a Valsalva maneuver and decreased at rest, which was the most important characteristic. Although there were many causes of neck masses in the children, only 3 of them, namely, superior mediastinal tumor or cyst, laryngocele, and JVP, enlarged on Valsalva maneuver. The most common cause of a mass in the neck that appeared only during straining was a laryngocele [4,5]. On the other hand, the rare jugular phlebectasia might present in a similar manner, and the absence of superior mediastinal widening and air in the mass on plain roentgenograms eliminated superior mediastinal tumor and larygocele, respectively. The absence of a bruit or pulsation excluded any mass of arterial origin such as aneurysm. The smooth surface of the dilated vein was differentiated easily from hemangioma. Although accurate diagnosis relied on carefully directed history, physical examination, radiological study, and careful process of differential diagnosis, a number of diagnostic techniques had been recommended. Once the diagnosis of JVP was suspected clinically, it could be readily confirmed by ultrasound or CDFI, which were the most useful and noninvasive methods for the investigation, as confirmed in both our study and other reports [6,7]. It was also important to perform these examinations bilaterally and comparatively, both during testing and straining based on our experience. There was no controversy about the indication of operation in the patients with symptomatic unilateral lesion. The key problem was focused on the asymptomatic cases: operation or follow-up? Based on our experience, if the patient could not endure the probable emotional torture and frequently requested surgical intervention, and if there was a tendency for the lesion to increase in size over time, the
174
X. Hu et al. / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 26 (2005) 172–174
indication of surgical intervention was enough. We recommended surgical ligation or excision in asymptomatic cases for cosmetic and psychological purposes. Especially when complication arose in asymptomatic cases, the management should be aggressive, but this complication added more difficulty and risks for both the patients and surgeons. Furthermore, once the untreated jugular phlebectasia was ignored or misdiagnosed, disastrous complication might occur, which has been frequently reported in the literature [8]. Of course, if the patients would not accept surgical intervention the treatment should be conservative. Still, there was another thing to be noticed, namely, ligation of the right internal jugular vein should be performed cautiously, no matter in right lesion alone or in bilateral ones. Generally, there are extensive communicated branches between the intracranial and extracranial veins. Ligation of the extracranial vein is relatively safe, and ligation of one side of the internal jugular vein should not cause regurgitation problems. However, in our patients postoperative complications in 3 cases occurred only in the children undergoing ligation of the right internal jugular vein, even undergoing 10 minutes of temporary occlusion. According to the anatomy of cerebral blood flow, about 70% of cerebral blood flow is regurgitated through the right internal jugular vein. Once congenital defect of anastomosis between intracranial and extracranial veins existed or the anastomoses were incompletely potent, ligation of the right internal jugular vein might be risky. Therefore, we suggested preoperative internal jugular occlusion training be performed for 1 month
or even longer, so as to facilitate the establishment of anastomosis between the intracranial and extracranial veins. Hemodynamic examination might be helpful to learn the blood varicosity state, too. For patients with bilateral JVP or high blood viscosity and unilateral JVP who are unsuitable for ligation, draping of the dilated vein with medical Dacron cloth might be taken as an alternative procedure, and medication for high blood viscosity was also needed.
References [1] Lubianca-Neto JF, Mauri M, Prati C. Internal jugular phlebectasia in children. Am J Otolaryngol 1999;20(6):415 - 8. [2] Paleri V, Gopalakrishnan S. Jugular phlebectasia: theory of pathogenesis and review of literature. Int J Pediatr Otorhinolaryngol 2001;57(2): 155 - 9. [3] Sander S, Elicevik M, Unal M, et al. Jugular phlebectasia in children: is it rare or ignored? J Pediatr Surg 1999;34(12):1829 - 32. [4] Kwok KL, Lam HS, Ng DK. Unilateral right-sided internal jugular phlebectasia in asthmatic children. J Paediatr Child Health 2000;36(5): 517 - 9. [5] Uzun C, Taskinalp O, Koten M, et al. Phlebectasia of left anterior jugular vein. J Laryngol Otol 1999;113(9):858 - 60. [6] Jeon C-W, Choo M-J, Bae I-H, et al. Diagnostic criteria of internal jugular phlebectasia in Korean children. Yonsei Med J 2002;43(3): 329 - 34. [7] Shimizu M, Takagi Y, Yoshio H, et al. Usefulness of ultrasonography and Doppler color flow imaging in the of diagnosis internal jugular phlebectasia. Heart Vessels 1992;7(2):95 - 8. [8] Burstin PP, Hooper RE. Massive primary hemorrhage during tonsillectomy from a large venous varicosity. Otolaryngol Head Neck Surg 1997;117(3 Pt 1):287 - 90.
Congenital jugular vein phlebectasia Xianliang Hua, Jianhong Lia,T, Tingze Hub, Xuewu Jianga a
Department of Pediatric Surgery, Medical College of Shantou University, Shantou City, Guangdong Province, China Department of Pediatric Surgery, West-China Medical Center of Sichuan University, Chengdu, Sichuan Province, China
b
Revised 19 January 2005
Abstract
Background/purpose: The purpose of the present study was to elucidate the clinical presentation, diagnosis, treatment, and postoperative complications of jugular vein phlebectasia (JVP), and to recommend diagnostic methods and treatment choices. Materials and methods: Thirty-six cases of JVP were reviewed (right vein in 27, left in 6, and bilateral in 3). The internal jugular veins were the most commonly involved. The main complaint was the soft and compressible mass in the neck. Ultrasound or color Doppler flow imaging (CDFI) was performed on all the children. Surgical intervention was performed in 32 cases, and the other 4 cases were conservatively followed up for 4 to 6 years. Results: Ultrasound or CDFI demonstrated local dilatation of the unilateral or bilateral veins in all the patients. Surgical intervention included ligation of the involved jugular vein in 31 cases and draping with medical Dacron cloth in 1 case. All of the operated children recovered uneventfully, except 3 for cases who underwent ligation of the right internal jugular vein. Conclusions: Valsalva maneuver was most important for establishing the diagnosis. Ultrasound or CDFI was the diagnostic procedure of choice to confirm the diagnosis of JVP. Surgical ligation or excision of unilateral jugular vein was recommended for cosmetic and psychological purposes. However, special attention must be paid to prevent postoperative complications in case of ligation of right internal jugular vein. Or else, treatment should be conservative (follow-up). D 2005 Elsevier Inc. All rights reserved.
1. Introduction
2. Materials and methods
Jugular vein phlebectasia (JVP) is a congenital fusiform dilatation of the jugular vein that appears as a soft, compressible mass in the neck during straining or triggered by the Valsalva maneuver. It has been described in almost all cervical veins and is usually asymptomatic. Because of its rarity, this entity is frequently ignored or misdiagnosed [1]. Until 2001, about 70 cases had been reported in the English medical literature. In the recent 20 years, 36 cases had been admitted into our hospital, and in this report the experiences of management were discussed combined with the related literature.
Among 36 cases (24 male, 12 female), the ages ranged from 1.5 to 15 years with an average of 8.4 years. The dilatation involved bilateral jugular veins in 3 cases, right vein in 27, and left in 6. The internal jugular veins were involved in 29 cases (3 bilateral, 22 right, and 4 left), and the external jugular veins were involved in 7 cases (5 right, 2 left). There was no history of trauma or inflammation to the neck or the upper thorax. The soft and compressible mass in the neck was usually the main complaint. Occasionally, some children complained of slight discomfort or pain during deglutition. On physical examination, the soft mass was well demarcated without bruit and pulsation. Valsalva maneuver was performed in all the patients. Ultrasound or color Doppler flow imaging (CDFI) was performed on all the children and demonstrated local dilatation of the involved unilateral or bilateral veins. Surgical intervention was performed in
T Corresponding author. Department of Pediatric Surgery, Medical College of Shantou University, Shantou City, Guangdong Province, 515031, China. Tel.: +86 754 8528370. E-mail address: [email protected] (J. Li). 0196-0709/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.02.009
X. Hu et al. / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 26 (2005) 172–174
32 cases: the involved vein was ligated or excised in 31, and 1 patient underwent draping of the dilated vein with medical Dacron cloth. And the other 4 cases were conservatively followed up for 4 to 6 years. 3. Results Valsalva maneuver showed positive results in all 36 patients and was most important for establishing the diagnosis. Ultrasound or CDFI demonstrated local fusiform dilatation of the involved unilateral or bilateral veins in all the patients. During the Valsalva maneuver the diameter of the dilatation increased at least one time in comparison with that at rest, which undoubtedly confirmed the diagnosis. Among 32 cases treated by operations, 1 case with bilateral internal JVP underwent draping of the dilated left vein with medical Dacron cloth because marked discomforts appeared when either side of the internal jugular vein was temporarily occluded. For this patient, the proximal part of the dilatation was first compressed until the dilated vein restored their normal diameter, then the vein was wrapped longitudinally with Dacron cloth. For the other 31 cases, the involved vein was ligated or excised (the right internal jugular vein was ligated in the 2 cases with bilateral lesions) and sent for pathological examination. Postoperative complications occurred in 3 children. The right internal jugular vein was ligated after being temporarily occluded for more than 10 minutes in 2 children with bilateral lesions, but ipsilateral neck and craniofacial swelling appeared soon after the operation. These 2 children were intensively cared for, and the swelling disappeared spontaneously in 3 days. However, in another child with right internal JVP, severe intracranial hypertension symptoms such as headache and vomiting appeared soon after ligation, although cerebral flow temporary occlusion test showed that the right internal jugular vein could be ligated. Magnetic resonance imaging showed thrombosis of the right internal jugular vein and lacular infarction of the right pontine. This child was given mannitol and flurosemide, and in 1 week the headache and vomiting disappeared. Three months later, magnetic resonance imaging showed the thrombosis and infarction did not exist and he recovered completely. 3.1. Pathological examination Generally, there were no pathological characteristics in the specimens. In some cases, the vein wall was structurally normal; but in other cases, the wall became thinner; the elastic fibers and muscle fibers decreased or disappeared. 4. Discussion Phlebectasia of the jugular veins is a venous anomaly that usually presents in children as a soft cystic swelling in the neck during straining. The term phlebectasia indicates abnormal outward dilatation of the vein without tortuosity
173
and differs from the term varix, which implies dilatation plus tortuosity [2]. Because there have been only sporadic reports of JVP, the exact etiology of this lesion still remains unclear. The possible causes of JVP are as follows: gross anatomic abnormality of the vein, mechanical compression or trauma of the vein, congenital structural defects in the vein wall, and idiopathy [3]. In our opinion, it is thought that congenital structural defects in the vein wall play an important role in the etiology, which has been partly proved by the results of pathological examination, so JVP is considered as a congenital venous anomaly. In our cases, 24 children (67%) were admitted before the age of 7 years, which also suggests a possible congenital origin. Even in the 4 adult women operated on in our hospital, JVP was first noticed in their teens and they were followed up for more than 20 years. Although phlebectasia might affect anterior, external, and internal jugular veins, in the majority of the patients the internal jugular vein was most frequently involved, which was evidenced in 29 cases (80.6%) of the patients in our group, and in all of 3 cases with bilateral lesions the internal jugular vein was affected, too. Jugular phlebectasia occurred with more frequency on the right side, as evidenced by our data. Clinically, this condition presented with enlargement with a Valsalva maneuver and decreased at rest, which was the most important characteristic. Although there were many causes of neck masses in the children, only 3 of them, namely, superior mediastinal tumor or cyst, laryngocele, and JVP, enlarged on Valsalva maneuver. The most common cause of a mass in the neck that appeared only during straining was a laryngocele [4,5]. On the other hand, the rare jugular phlebectasia might present in a similar manner, and the absence of superior mediastinal widening and air in the mass on plain roentgenograms eliminated superior mediastinal tumor and larygocele, respectively. The absence of a bruit or pulsation excluded any mass of arterial origin such as aneurysm. The smooth surface of the dilated vein was differentiated easily from hemangioma. Although accurate diagnosis relied on carefully directed history, physical examination, radiological study, and careful process of differential diagnosis, a number of diagnostic techniques had been recommended. Once the diagnosis of JVP was suspected clinically, it could be readily confirmed by ultrasound or CDFI, which were the most useful and noninvasive methods for the investigation, as confirmed in both our study and other reports [6,7]. It was also important to perform these examinations bilaterally and comparatively, both during testing and straining based on our experience. There was no controversy about the indication of operation in the patients with symptomatic unilateral lesion. The key problem was focused on the asymptomatic cases: operation or follow-up? Based on our experience, if the patient could not endure the probable emotional torture and frequently requested surgical intervention, and if there was a tendency for the lesion to increase in size over time, the
174
X. Hu et al. / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 26 (2005) 172–174
indication of surgical intervention was enough. We recommended surgical ligation or excision in asymptomatic cases for cosmetic and psychological purposes. Especially when complication arose in asymptomatic cases, the management should be aggressive, but this complication added more difficulty and risks for both the patients and surgeons. Furthermore, once the untreated jugular phlebectasia was ignored or misdiagnosed, disastrous complication might occur, which has been frequently reported in the literature [8]. Of course, if the patients would not accept surgical intervention the treatment should be conservative. Still, there was another thing to be noticed, namely, ligation of the right internal jugular vein should be performed cautiously, no matter in right lesion alone or in bilateral ones. Generally, there are extensive communicated branches between the intracranial and extracranial veins. Ligation of the extracranial vein is relatively safe, and ligation of one side of the internal jugular vein should not cause regurgitation problems. However, in our patients postoperative complications in 3 cases occurred only in the children undergoing ligation of the right internal jugular vein, even undergoing 10 minutes of temporary occlusion. According to the anatomy of cerebral blood flow, about 70% of cerebral blood flow is regurgitated through the right internal jugular vein. Once congenital defect of anastomosis between intracranial and extracranial veins existed or the anastomoses were incompletely potent, ligation of the right internal jugular vein might be risky. Therefore, we suggested preoperative internal jugular occlusion training be performed for 1 month
or even longer, so as to facilitate the establishment of anastomosis between the intracranial and extracranial veins. Hemodynamic examination might be helpful to learn the blood varicosity state, too. For patients with bilateral JVP or high blood viscosity and unilateral JVP who are unsuitable for ligation, draping of the dilated vein with medical Dacron cloth might be taken as an alternative procedure, and medication for high blood viscosity was also needed.
References [1] Lubianca-Neto JF, Mauri M, Prati C. Internal jugular phlebectasia in children. Am J Otolaryngol 1999;20(6):415 - 8. [2] Paleri V, Gopalakrishnan S. Jugular phlebectasia: theory of pathogenesis and review of literature. Int J Pediatr Otorhinolaryngol 2001;57(2): 155 - 9. [3] Sander S, Elicevik M, Unal M, et al. Jugular phlebectasia in children: is it rare or ignored? J Pediatr Surg 1999;34(12):1829 - 32. [4] Kwok KL, Lam HS, Ng DK. Unilateral right-sided internal jugular phlebectasia in asthmatic children. J Paediatr Child Health 2000;36(5): 517 - 9. [5] Uzun C, Taskinalp O, Koten M, et al. Phlebectasia of left anterior jugular vein. J Laryngol Otol 1999;113(9):858 - 60. [6] Jeon C-W, Choo M-J, Bae I-H, et al. Diagnostic criteria of internal jugular phlebectasia in Korean children. Yonsei Med J 2002;43(3): 329 - 34. [7] Shimizu M, Takagi Y, Yoshio H, et al. Usefulness of ultrasonography and Doppler color flow imaging in the of diagnosis internal jugular phlebectasia. Heart Vessels 1992;7(2):95 - 8. [8] Burstin PP, Hooper RE. Massive primary hemorrhage during tonsillectomy from a large venous varicosity. Otolaryngol Head Neck Surg 1997;117(3 Pt 1):287 - 90.