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Srinivasa V, Jayendiran S, Kayarkar D, Aliyar A, Aravind TI. Bilateral internal jugular vein ectasia: A rare cause of neck swelling. THE NATIONAL MEDICAL JOURNAL OF INDIA 2023; 36:23-25. [PMID: 37615138 DOI: 10.25259/nmji_282_20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 08/25/2023]
Abstract
Internal jugular vein ectasia or phlebectasia is a condition in which there is an isolated fusiform dilatation of the internal jugular vein. The patient usually presents with swelling in the neck, which aggravates in size while coughing or straining. This is a rare condition and is often mis-diagnosed. It can be diagnosed by proper history, clinical examination and imaging. We report a 5-year-old boy who had bilateral internal jugular vein ectasia aggravating in size while straining and coughing. Ultrasonography and computed tomography scan showed dilatation of internal jugular veins on both sides. Since the patient was asymptomatic and had no complications, he was advised regular follow-up. This rare benign condition should be kept in mind as a differential diagnosis of an expansile neck mass.
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Affiliation(s)
- V Srinivasa
- Department of ENT, Vinayaka Mission's Medical College and Hospital, Pondicherry 609609, India
| | - S Jayendiran
- Department of ENT, Vinayaka Mission's Medical College and Hospital, Pondicherry 609609, India
| | - Darshan Kayarkar
- Department of ENT, Vinayaka Mission's Medical College and Hospital, Pondicherry 609609, India
| | - Aleena Aliyar
- Vinayaka Mission's Medical College and Hospital, Pondicherry 609609, India
| | - T I Aravind
- Department of ENT, Vinayaka Mission's Medical College and Hospital, Pondicherry 609609, India
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Stevens KE, Price JE, Marko J, Kaler SG. Neck masses due to internal jugular vein phlebectasia: Frequency in Menkes disease and literature review of 85 pediatric subjects. Am J Med Genet A 2020; 182:1364-1377. [PMID: 32293788 DOI: 10.1002/ajmg.a.61572] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2018] [Revised: 01/31/2020] [Accepted: 02/24/2020] [Indexed: 11/06/2022]
Abstract
Classic Menkes disease is a rare X-linked recessive disorder of copper metabolism caused by pathogenic variants in the copper transporter gene, ATP7A. Untreated affected individuals suffer failure to thrive and neurodevelopmental delays that begin at 6-8 weeks of age and progress inexorably to death, often within 3 years. Subcutaneous injections of Copper Histidinate (US Food and Drug Administration IND #34,166, Orphan product designation #12-3663) are associated with improved survival and neurological outcomes, especially when commenced within a month of birth. We previously identified internal jugular vein phlebectasia (IJP) in four Menkes disease subjects. This feature and other connective tissue abnormalities appear to be consequences of deficient activity of lysyl oxidase, a copper-dependent enzyme. Here, we report results from a prospective study of IJP based on 178 neck ultrasounds in 66 Menkes subjects obtained between November 2007 and March 2018. Nine patients met the criterion for IJP (one or more cross-sectional area measurements exceeding 2.2 cm2 ) and five subjects had clinically apparent neck masses that enlarged over time. Our prospective results suggest that IJP occurs in approximately 14% (9/66) of Menkes disease patients and appears to be clinically benign with no specific medical or surgical actionability. We surveyed the medical literature for prior reports of IJP in pediatric subjects and identified 85 individuals and reviewed the distribution of this abnormality by gender, sidedness, and underlying etiology. Taken together, Menkes disease accounts for 16% (15/94) of all reported IJP individuals. Neck masses from IJP represent underappreciated abnormalities in Menkes disease.
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Affiliation(s)
- Kristen E Stevens
- Section on Translational Neuroscience, Molecular Medicine Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland, USA.,George Washington University School of Nursing, Washington, District of Columbia, USA
| | - Julienne E Price
- Section on Translational Neuroscience, Molecular Medicine Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland, USA
| | - Jamie Marko
- Department of Diagnostic Imaging, NIH Clinical Center, Bethesda, Maryland, USA
| | - Stephen G Kaler
- Section on Translational Neuroscience, Molecular Medicine Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland, USA.,Center for Gene Therapy, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, Ohio, USA
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Figueroa-Sanchez JA, Ferrigno AS, Benvenutti-Regato M, Caro-Osorio E, Martinez HR. Internal jugular phlebectasia: A systematic review. Surg Neurol Int 2019; 10:106. [PMID: 31528444 PMCID: PMC6744733 DOI: 10.25259/sni-217-2019] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2019] [Accepted: 05/01/2019] [Indexed: 01/21/2023] Open
Abstract
Background: Internal jugular phlebectasia (IJP), the abnormal dilatation of internal jugular vein, is generally considered a benign anomaly. However, because IJP is uncommon, little is known about its natural history, and currently, no consensus on the best treatment modality is available. Methods: The purpose of this article is to conduct a systematic review of available literature on recently reported IJP cases to understand the main characteristics of IJP and its most frequent therapeutic approaches. Following the preferred reporting items for systematic reviews and meta-analyses guidelines, literature search for IJP cases was conducted in the COCHRANE, PUBMED, EBSCOHOST, SCOPUS, OVID, and SCIELO databases. Results: A total of 97 original articles were found, with a total of 247 IJP cases reported including both pediatric and adult patients. Conclusions: To the best of our knowledge, this study is the largest systematic review analyzing all the reported cases of IJP. IJP is considered by most authors as a benign abnormality that predominantly affects the right jugular vein. It is most commonly diagnosed in children. At present, conservative treatment is preferred for pediatric but not for adult patients. Multicenter randomized prospective studies are required to further understand this rare anomaly.
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Affiliation(s)
- Jose A Figueroa-Sanchez
- Institute of Neurology and Neurosurgery, Hospital Zambrano Hellion, San Pedro Garza García, N.L., México
| | - Ana S Ferrigno
- Institute of Neurology and Neurosurgery, Hospital Zambrano Hellion, San Pedro Garza García, N.L., México
| | - Mario Benvenutti-Regato
- Institute of Neurology and Neurosurgery, Hospital Zambrano Hellion, San Pedro Garza García, N.L., México
| | - Enrique Caro-Osorio
- Institute of Neurology and Neurosurgery, Hospital Zambrano Hellion, San Pedro Garza García, N.L., México
| | - Hector R Martinez
- Institute of Neurology and Neurosurgery, Hospital Zambrano Hellion, San Pedro Garza García, N.L., México
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Alshehri WM, Alothmani SK, Alshamrani AM, Almadhari RI, Alqahtani AS, Alrasheedi Saud D. Internal jugular vein aneurysm: a case report. J Surg Case Rep 2019; 2019:rjz159. [PMID: 31139340 PMCID: PMC6532136 DOI: 10.1093/jscr/rjz159] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2019] [Revised: 04/26/2019] [Accepted: 05/06/2019] [Indexed: 12/04/2022] Open
Abstract
Venous aneurysms rarely present as a painless mass; they are usually symptomatic and present with pain, swelling, and sometimes, embolism. We present a 41-year-old man who presented with a painless swelling involving the right side of the neck. Examination of the neck revealed a soft, compressible, non-pulsatile and non-tender swelling above the right sternoclavicular joint. Doppler ultrasound showed a well-defined compressible cystic lesion measuring 4.2 × 1.9 cm causing indentation of the right sternocleidomastoid muscle. Further imaging studies confirmed the diagnosis of an aneurysmal dilation in the right internal jugular vein. An excisional biopsy of the aneurysm was performed using a longitudinal incision in the skin at the anterior border of the right sternocleidomastoid muscle and excisional venotomy was performed. Histopathological examination showed a cystic structure containing organizing blood clots surrounded by a thick fibrous wall. A follow-up neck ultrasound performed postoperative showed a patent internal jugular vein.
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Affiliation(s)
| | | | | | | | - Adel Saad Alqahtani
- General Surgery Department, Security Forces Hospital Program, Riyadh 11481, Saudi Arabia
| | - D Alrasheedi Saud
- General Surgery Department, Security Forces Hospital Program, Riyadh 11481, Saudi Arabia
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Delvecchio K, Moghul F, Patel B, Seman S. Surgical resection of rare internal jugular vein aneurysm in neurofibromatosis type 1. World J Clin Cases 2017; 5:419-422. [PMID: 29291199 PMCID: PMC5740185 DOI: 10.12998/wjcc.v5.i12.419] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2017] [Revised: 09/10/2017] [Accepted: 11/22/2017] [Indexed: 02/05/2023] Open
Abstract
Neurofibromatosis type 1 is a congenital condition affecting neurons and connective tissue integrity including vasculature. On extremely rare occasions these patients present with venous aneurysms affecting the internal jugular vein. If they become large enough there presents a risk of rupture, thrombosis, embolization or compression of adjacent structures. In these circumstances, or when the patient becomes symptomatic, surgical exploration is warranted. We present a case of one of the largest aneurysms in the literature and one of only five associated with Neurofibromatosis type 1. A 63-year-old female who initially presented for a Hinchey III diverticulitis requiring laparotomy developed an incidentally discovered left neck swelling prior to discharge. After nonspecific clinical exam findings, imaging identified a thrombosed internal jugular vein aneurysm. Due to the risks associated with the particularly large size of our patient’s aneurysm, our patient underwent surgical exploration with ligation and excision. Although several techniques have been reported, for similar presentations, we recommend this technique.
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Affiliation(s)
- Khortnal Delvecchio
- Department of Surgery, Detroit Medical Center Huron Valley-Sinai Hospital, Commerce, MI 48382, United States
- Department of Surgery, Detroit Medical Center Sinai Grace Hospital, Detroit, MI 48235, United States
| | - Fazaldin Moghul
- Department of Surgery, Detroit Medical Center Huron Valley-Sinai Hospital, Commerce, MI 48382, United States
- Department of Surgery, Detroit Medical Center Sinai Grace Hospital, Detroit, MI 48235, United States
| | - Bipinchandra Patel
- Department of Surgery, Detroit Medical Center Huron Valley-Sinai Hospital, Commerce, MI 48382, United States
- Department of Surgery, Detroit Medical Center Sinai Grace Hospital, Detroit, MI 48235, United States
| | - Susan Seman
- Department of Surgery, Detroit Medical Center Sinai Grace Hospital, Detroit, MI 48235, United States
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Abstract
BACKGROUND External jugular vein aneurysm is a rare condition with few cases described in the literature. Complications include thrombus formation, thrombophlebitis, pulmonary embolism, rupture, and cosmetic complaint. METHODS AND RESULTS The authors present a clinical case and treatment of thrombosed aneurysm of the external jugular vein in a 47-year-old man. CONCLUSIONS Despite its rarity, external jugular vein aneurysm should be remembered in the differential diagnosis of a cervical mass. Surgical excision is indicated in symptomatic aneurysms and for aesthetic reasons. Excision eliminates the risk of complications and allows for histopathologic diagnosis.
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Zamboni P, Cossu A, Carpanese L, Simonetti G, Massarelli G, Liboni A. The So-Called Primary Venous Aneurysms. Phlebology 2016. [DOI: 10.1177/026835559000500110] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
The authors have observed numerous venous aneurysms among patients in Sardinia. In the past 3 years 30 venous aneurysms have been diagnosed and treated. In seven cases the aneurysms were undoubtedly primary; owing to their position in the very low pressure system of the superior vena cava and because trauma, arteriovenous fistulas and cardiac or thoracic malformations were completely excluded. After surgical excision the aneurysms were studied by histological and immunohistochemical methods. Antisera to desmin and to alfa-actin demonstrated significant depletion of smooth muscle cells in the wall of the aneurysm.
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Affiliation(s)
| | - A. Cossu
- Institute of Morbid Anatomy, The University of Sassari, 07100 Sassari, Italy
| | - L. Carpanese
- Institute of Radiology, The University of Sassari, 07100 Sassari, Italy
| | - G. Simonetti
- Institute of Radiology, The University of Sassari, 07100 Sassari, Italy
| | - G. Massarelli
- Institute of Morbid Anatomy, The University of Sassari, 07100 Sassari, Italy
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Vermeer NCA, Elshof JWM, Vriens PWHE. Clinical presentation, diagnosis, and treatment of venous aneurysms. J Vasc Surg Venous Lymphat Disord 2014; 2:349-353.e3. [PMID: 26993399 DOI: 10.1016/j.jvsv.2014.02.007] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2013] [Accepted: 02/25/2014] [Indexed: 11/30/2022]
Abstract
Venous aneurysms have been reported in most major veins and are often asymptomatic but can cause serious complications. Three patients with symptomatic venous aneurysms of the internal jugular vein, portal vein, and popliteal vein are presented, and their treatment and outcomes are discussed. Furthermore, presentation and management of the most frequent venous aneurysms, based on available literature, are evaluated. The literature supports conservative treatment of asymptomatic and nonthrombotic jugular, subclavian, thoracic, and visceral venous aneurysms. Surgery should be considered only for symptomatic venous aneurysms or in case of progressive expansion on follow-up. Prophylactic surgery is recommended for all patients with lower extremity deep venous aneurysms, by means of tangential aneurysmectomy with lateral venorrhaphy, because of the increased risk of pulmonary embolization. Postoperative anticoagulation is recommended for a period of at least 3 months after operative treatment.
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Affiliation(s)
- Nina C A Vermeer
- Department of Vascular Surgery, St. Elisabeth Hospital, Tilburg, The Netherlands.
| | - Jan Willem M Elshof
- Department of Vascular Surgery, St. Elisabeth Hospital, Tilburg, The Netherlands
| | - Patrick W H E Vriens
- Department of Vascular Surgery, St. Elisabeth Hospital, Tilburg, The Netherlands
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Ilica AT, Senturk S, Kertmen M, Bukte Y, Bilici A, Atasoy MM. Facial vein aneurysm associated with sialadenitis. JOURNAL OF CLINICAL ULTRASOUND : JCU 2012; 40:319-321. [PMID: 22253006 DOI: 10.1002/jcu.21881] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/21/2011] [Accepted: 11/30/2011] [Indexed: 05/31/2023]
Abstract
A 20 year-old man presented with pain and swelling of the left submandibular area. Neck sonography revealed enlargement of the submandibular gland, coarsening of its echotexture with a few calculi and a multiloculated cystic lesion. Doppler sonography revealed venous flow within the cystic lesion and aneurysmal dilatation of the adjacent facial vein. CT angiography confirmed the facial vein aneurysm. We hypothesize that inflammation of the gland had weakened the wall of the adjacent facial vein, causing aneurysmal dilatation.
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Affiliation(s)
- Ahmet Turan Ilica
- Gulhane Military Medical School, Department of Radiology, Ankara, Turkey
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Price DJ, Ravindranath T, Kaler SG. Internal jugular phlebectasia in Menkes disease. Int J Pediatr Otorhinolaryngol 2007; 71:1145-8. [PMID: 17482283 PMCID: PMC2917727 DOI: 10.1016/j.ijporl.2007.02.021] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2007] [Revised: 02/25/2007] [Accepted: 02/26/2007] [Indexed: 11/20/2022]
Abstract
Pediatric neck masses should trigger a high index of suspicion for certain genetic disorders of connective tissue. To highlight this, we report on three infants with Menkes disease, an inherited disorder of copper transport, who developed large, unilateral neck masses at between 7 and 17 months of age. All were identified in imaging studies as internal jugular phlebectasia. The masses, which enlarged on crying or exertion, have remained clinically benign in these patients for 20, 17, and 2 months, respectively. While arterial tortuosity and aneurysms have been reported often in Menkes disease, venous phlebectasia has rarely been described. We speculate that low activity of the copper-dependent enzyme, lysyl oxidase, leading to reduced tensile strength in the deep cervical fascia comprising the carotid sheath may predispose to internal jugular phlebectasia in these individuals. Improved survival and neurological outcomes in infants with Menkes disease due to advances in early diagnosis and treatment may be associated with recognition of novel clinical stigmata of this condition such as internal jugular phlebectasia.
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Affiliation(s)
| | - Thyyar Ravindranath
- Division of Critical Care Medicine, Department of Pediatrics, Columbia University Medical Center, New York, NY
| | - Stephen G. Kaler
- Unit on Pediatric Genetics, Laboratory of Clinical Genomics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD
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Jianhong L, Xuewu J, Tingze H. Surgical treatment of jugular vein phlebectasia in children. Am J Surg 2006; 192:286-90. [PMID: 16920419 DOI: 10.1016/j.amjsurg.2006.02.025] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2005] [Revised: 02/13/2006] [Accepted: 02/13/2006] [Indexed: 02/05/2023]
Abstract
BACKGROUND Jugular vein phlebectasia (JVP) is a cervical mass that occurs relatively infrequently and usually presents in children as a soft cystic swelling in the neck during straining. It is liable to be misdiagnosed or managed inappropriately. This report elucidates the clinical presentation, diagnosis, treatment choices, and postoperative complications of JVP, and diagnostic methods and treatment choices are recommended. METHODS Fifty-one cases of JVP were reviewed (right vein in 38 patients, left in 7 patients, and bilateral in 6 patients). The internal jugular vein was involved the most frequently. The main complaint was a soft and compressible mass in the neck, becoming more prominent with the Valsalva maneuver. All of the children except 2 had an ultrasound or color Doppler flow imaging (CDFI) performed in combination with the Valsalva's breathing test. Surgical intervention was performed in 46 patients and the other 5 patients were followed-up conservatively for 2 to 15 years. RESULTS Ultrasound or CDFI showed local dilatation of unilateral or bilateral veins in all patients except 2, and confirmed the diagnosis in combination with the Valsalva's breathing test. Surgical intervention included ligation of the involved jugular vein in 32 patients, and longitudinal constriction suture venoplasty plus encapsulation with medical Dacron cloth or PTFE in 14 patients. All of the children who had surgery recovered uneventfully, except 3 patients undergoing ligation of the right internal jugular vein. CONCLUSIONS The Valsalva maneuver was most important for establishing the diagnosis. Ultrasound or CDFI, or in combination with the Valsalva's breathing test, was the diagnostic procedure of choice to confirm the diagnosis of JVP because of its clarity, safety, and low cost. Surgical intervention was recommended for cosmetic and psychologic purposes. Ligation or excision of the involved jugular vein was very safe, simple, and effective for most patients. However, in cases of lesions of the right and bilateral internal jugular veins, longitudinal constriction suture venoplasty plus encapsulation might be more preferable and safer, and should be recommended. Otherwise, treatment should be conservative (follow-up evaluation).
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Affiliation(s)
- Li Jianhong
- Department of Pediatric Surgery, Second University Hospital of Shantou University Medical College, Dongxia Bei Rd., Shantou City, Guangdong Province, People's Republic of China, 515041.
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Abstract
Jugular phlebectasia is a rare cause of cervical swelling in children. It is a fusiform dilatation of any part of the jugular venous system and can involve the external, internal or anterior jugular veins. Previous reports suggest that the entity is often ignored or misdiagnosed. Unilateral internal jugular phlebactasia presenting as an intermittent neck swelling in a ten-year-old girl is reported. The clinical features are analyzed and the appropriate use of noninvasive imaging modalities is highlighted. The literature is also briefly reviewed.
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Affiliation(s)
- V R Rajendran
- Department of Radiodiagnosis, Medical College, Calicut, Kerala, India.
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Akoudad H, Chaouki S, Cherti M, Benmimounm EG, Arharbi M. [Aneurysm of the internal saphenous vein: report of a case]. Ann Cardiol Angeiol (Paris) 2001; 50:142-5. [PMID: 12555504 DOI: 10.1016/s0003-3928(01)00013-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Venous aneurysms of the lower extremities are rare. They have been reported in both the deep and superficial vein system. Thromboembolism is more common in aneurysms involving the popliteal vein than those involving superficial veins. We report a case of primary aneurysm of the greater saphenous vein presenting as an inguinal hernia. Sonography has confirmed the venous dilatation. we review also the clinical and therapeutic findings of venous aneurysms of the lower extremities.
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Affiliation(s)
- H Akoudad
- Service de cardiologie B, CHU Ibn Sina, Rabat, Maroc.
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Abstract
Jugular phlebectasia is an entity that is being increasingly recognised in recent years. The term phlebectasia indicates dilatation of the vein without tortuosity. It has been described in almost all cervical veins. Internal jugular phlebectasia is seen more often on the right side. This paper reports two new cases, reviews all cases of internal jugular phlebectasia in children published in English literature upto 1996 and recommends diagnostic methods and treatment policy. This article also discusses a theory of pathogenesis for this condition based on the regional anatomical features, principles of vascular physics and pathologic findings.
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Affiliation(s)
- V Paleri
- Department of Ear, Nose and Throat Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
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Affiliation(s)
- X D Fan
- Department of Oral & Maxillofacial Surgery, Ninth People's Hospital, Shanghai Second Medical University, People's Republic of China
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Abstract
BACKGROUND/PURPOSE 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 purpose of this report is to discuss the differential diagnosis, the methods of imaging, the mode of treatment, and to demonstrate some factors that have made us believe that the condition may not be an actual rarity but rather has been ignored. METHODS Eight cases of unilateral internal jugular phlebectasia were treated surgically (ie, excision of the dilated portion of the vein) from 1987 to 1998. The age of the patients ranged from 3 to 14 years. There were 3 girls and 5 boys. The lesions were right sided in 6, and left sided in 2 children. The patients underwent surgery after comparative ultrasonographic confirmation of the diagnosis. Furthermore, the authors prepared a simple questionnaire for evaluating the level of knowledge about this lesion among the related specialists. One hundred ten physicians were asked to describe the jugular phlebectasia and its ideal treatment. RESULTS All of the patients were discharged from the hospital 24 hours after surgery. Follow-up periods ranged from 6 months to 6 years and no complaints were noted at the time of most recent visits. Our questionnaire results showed that 96% of 73 pediatricians, 37% of 22 otorhinolaryngologists, and 40% of 15 pediatric surgeons did not know what the jugular phlebectasia was. CONCLUSIONS Color Doppler sonography alone is sufficient for the diagnosis of jugular phlebectasia. The authors recommend surgical excision in asymptomatic cases for cosmetic and psychological purposes. The rarity of the lesion may be caused by a lack of knowledge among the related physicians and the tendency of reporting only surgical results.
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Affiliation(s)
- S Sander
- SSK Bakirköy Maternity and Children's Hospital, Department of Pediatric Surgery, Istanbul, Turkey
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17
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Isolated venous aneurysm of internal jugular vein—A case report. Indian J Otolaryngol Head Neck Surg 1995. [DOI: 10.1007/bf03047998] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
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Mickelson SA, Spickler E, Roberts K. Management of Internal Jugular Vein Phlebectasia. Otolaryngol Head Neck Surg 1995; 112:473-5. [PMID: 7870454 DOI: 10.1016/s0194-59989570288-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
Affiliation(s)
- S A Mickelson
- Department of Otolaryngology-Head and Neck Surgery, Henry Ford Hospital, Detroit, MI 48202
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Calligaro KD, Ahmad S, Dandora R, Dougherty MJ, Savarese RP, Doerr KJ, McAffee S, DeLaurentis DA. Venous aneurysms: surgical indications and review of the literature. Surgery 1995; 117:1-6. [PMID: 7809821 DOI: 10.1016/s0039-6060(05)80222-3] [Citation(s) in RCA: 184] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
BACKGROUND During the last 20 years we diagnosed five cases of venous aneurysm of the jugular (n = 4) and basilic (n = 1) veins. The purpose of this report was to determine the natural history and indications for surgery of venous aneurysms. METHODS Our five cases were included in an English-language literature review performed through August 1993. RESULTS In our series two aneurysms (one external jugular vein, one basilic vein) were excised for cosmetic reasons. Three internal jugular vein aneurysms were followed up for up to 4 years without complications with serial color duplex ultrasonography. Of 32 patients with abdominal venous aneurysms (18 portal, seven inferior vena cava, four superior mesenteric, two splenic, one internal iliac), 13 (41%) had major complications including five deaths. Of 31 patients with deep venous aneurysms of the extremity (29 popliteal, two common femoral), 22 (71%) had deep vein thrombosis or pulmonary embolism and in 17 recurrent deep vein thrombosis or pulmonary embolism developed when patients were treated with anticoagulation alone. CONCLUSIONS Prophylactic surgery is cautiously recommended for low-risk patients with venous aneurysms of the abdomen and strongly recommended for most patients with lower extremity deep venous aneurysms. Other venous aneurysms should be excised only if they are symptomatic, enlarging, or disfiguring.
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Affiliation(s)
- K D Calligaro
- Section of Vascular Surgery, Pennsylvania Hospital/University of Pennsylvania School of Medicine, Philadelphia
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Natarajan B, Johnstone A, Sheikh S, Palmer O, Madhavan KN. Unilateral anterior jugular phlebectasia. J Laryngol Otol 1994; 108:352-3. [PMID: 8182329 DOI: 10.1017/s0022215100126751] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
We present a rare case of unilateral anterior jugular venous phlebectasia in an 82-year-old female patient presenting as a soft cystic lump in the anterior aspect of the neck increasing in size during straining and valsalva manoeuvre. Although cases of internal and external jugular phlebectasia have been reported, as far as we are aware no case of anterior jugular phlebectasia has been reported in the literature previously.
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Gendeh BS, Dhillon MK, Hamzah M. Bilateral internal jugular vein ectasia: a report of two cases. J Laryngol Otol 1994; 108:256-60. [PMID: 8169515 DOI: 10.1017/s0022215100126465] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
Internal jugular vein ectasia is a venous anomaly commonly presenting as a unilateral neck swelling in children and adults. Literature reports of bilateral presentation are rare. Bilateral Doppler ultrasonography is the diagnostic investigation of choice. The possible pathology, aetiology and management are discussed. Conservative management of bilateral cases is recommended in uncomplicated cases.
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Affiliation(s)
- B S Gendeh
- Department of Otorhinolaryngology, National University of Malaysia (NUM), Kuala Lumpur
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Saponaro SA, Flanders AE, Friedman DP, Mandel S. Superior sagittal sinus thrombosis in the presence of idiopathic bilateral internal jugular vein stenosis. J Neuroimaging 1994; 4:48-50. [PMID: 8136582 DOI: 10.1111/jon19944148] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023] Open
Abstract
Cerebral venous outflow obstruction and anomalies in cerebral venous circulation predispose to dural sinus thrombosis. This case report illustrates the magnetic resonance and angiographic findings in a patient who had superior sagittal sinus thrombosis secondary to idiopathic bilateral internal jugular vein stenosis, a previously unrecognized entity. The findings suggest that bilateral stenosis of the internal jugular veins at their junction with the innominate veins causes obstruction to cerebral venous outflow leading to dural sinus thrombosis.
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Affiliation(s)
- S A Saponaro
- Department of Radiology, Pennsylvania Hospital, Philadelphia
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Abstract
Phlebectasia of the internal jugular vein (IJV) is a rare venous anomaly that usually presents in young children as a soft cystic swelling in the neck and represents a pathologically enlarged vein. We present a case of jugular bulb phlebectasia in a 4-year-old boy. He presented with a blue pulsatile swelling arising from the floor of the left middle ear cleft. The venous phase of digital subtraction vertebral arteriography (DSVA) and computerised axial tomography (CT) with contrast confirmed a diagnosis of phlebectasia of the lateral sinus and proximal part of the left IJV, with a complete bony defect in the floor of the middle ear. The child did not have any neck symptoms or signs. No active treatment was required. To our knowledge, this is the first case of jugular bulb phlebectasia presenting in the middle ear.
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Affiliation(s)
- R M Walsh
- Department of Otorhinolaryngology, Lewisham Hospital, London, UK
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Abstract
Internal jugular phlebectasia is a venous anomaly commonly presenting as a unilateral neck swelling in children. The clinicopathology, aetiology and management are discussed. Bilateral doppler ultrasonography is the diagnostic investigation of choice and should be performed in all suspected cases. Conservative management of the bilateral case is recommended.
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Affiliation(s)
- R M Walsh
- ENT Department, University Hospital, Queen's Medical Centre, Nottingham
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Shimizu M, Takagi Y, Yoshio H, Takeda R, Matsui O. Usefulness of ultrasonography and Doppler color flow imaging in the diagnosis of internal jugular phlebectasia. Heart Vessels 1992; 7:95-8. [PMID: 1400087 DOI: 10.1007/bf01744455] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
A 20-year-old woman presented to our hospital for investigation of a left neck mass. Ultrasonographic examination of the jugular mass demonstrated an echo-free space, the caliber of which markedly increased when the patient shifted from the sitting to the recumbent position or performed a Valsalva maneuver. On color Doppler flow imaging, a slow flow signal flowing in the direction opposite to that of the common carotid artery was found within this space. Ultrasonography and color Doppler flow imaging thus proved to be useful for correctly diagnosing internal jugular phlebectasia.
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Affiliation(s)
- M Shimizu
- Second Department of Internal Medicine, School of Medicine, Kanazawa University, Japan
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Yokomori K, Kubo K, Kanamori Y, Takemura T, Yamamoto T. Internal jugular phlebectasia in two siblings: manometric and histopathologic studies of the pathogenesis. J Pediatr Surg 1990; 25:762-5. [PMID: 2199660 DOI: 10.1016/s0022-3468(05)80014-4] [Citation(s) in RCA: 37] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Two brothers, 4 years and 6 years of age, presented with a swelling in the right side of the neck. Ultrasonography and venography confirmed a diagnosis of phlebectasia of the right internal jugular vein (IJV). In an attempt to elucidate the etiology of this rare lesion, venous pressures in both the dilated right IJV and in the left IJV were taken under general anesthesia with intratracheal intubation at the time of surgery in each patient. No significant difference in pressure elevation with increase of intrathoracic pressure by overinflating the breathing bag was observed between the right and left IJV, suggesting that there would be no mechanical obstructive process generated on exertion, in each case. Microscopic examinations of a dissected portion of the dilated IJV showed paucity of muscle layer of the vein wall in the younger patient, and absence of that in the elder. Therefore, we assume that congenital muscle defect of the right IJV wall, rather than mechanical obstruction in the lower neck or the mediastinum, might cause phlebectasia. To our knowledge, this is the first report of IJV phlebectasia in siblings.
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Affiliation(s)
- K Yokomori
- Department of Pediatric Surgery, Japanese Red Cross Medical Center, Tokyo
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Abstract
Two cases of swollen neck during straining, coughing, or crying caused by internal jugular vein dilatation that were diagnosed by ultrasonography and computed tomography are presented. With a review of the literature the aetiology and diagnostic procedure are discussed.
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Affiliation(s)
- I Kovanlikaya
- Department of Radiology, Dokuz Eylüt University Hospital, Izmir, Turkey
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29
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Abstract
Varicose veins are a common venous anomaly, but rarer varieties also occur including phlebectasia, which is an abnormal dilatation of an isolated vein. Phlebectasia may affect any vein and is usually asymptomatic. When the internal jugular vein is involved, it presents as a cervical swelling that closely mimics the signs of either a pharyngocoele or a laryngocoele and, due to its rarity, is frequently misdiagnosed. A review of the English literature demonstrates that the diagnosis has generally been confirmed either by invasive contrast radiographic techniques, or surgical exploration, though many authors had made a tentative clinical diagnosis prior to surgical confirmation. No treatment is indicated for this benign self-limiting condition, except for the few patients who complain of symptoms and require surgical removal of the affected vein, and therefore, if a diagnosis cannot be made with certainty from the clinical features, investigations should be as non-invasive as possible, employing either a CAT scan or Digital Subtraction Angiography, though the latter is not yet universally available.
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Leung A, Hampson SJ, Singh MP, Carr D. Ultrasonic diagnosis of bilateral congenital internal jugular venous aneurysms. Br J Radiol 1983; 56:588-91. [PMID: 6871612 DOI: 10.1259/0007-1285-56-668-588] [Citation(s) in RCA: 24] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
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