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Jugular Venous Ectasia in Children
Pediatric Radiology
A Report of 3 Cases and Review of the Literature 1
David H. Gordon, M.D., Judith S. Rose, M.D., Peter Kottmeier, M.D., and David C. Levin, M.D. Three cases of jugular venous ectasia in children were diagnosed preoperatively by selective venography. These rare lesions appear to be congenital dilatations of the veins and should not properly be called aneurysms, as their histological structure is normal. A typical history and complex of physical findings are associated with this entity. It is important to include it in the differential diagnosis of neck masses in children. INDEX TERM:
Veins, jugular
Radiology 118:147-149, January 1976
• tion of the specimen revealed a histologically normal venous structure with a normal amount of elastic tissue.
F
USIFORM dilatation or ectasia of the neck veins . is a rare entity in the pediatric population. Since neck lesions are not uncommon in children, these masses may pose a problem in differential diagnosis. The first case of neck vein "aneurysm" known to us was reported in 1928 by Harris (4), who described a venous anomaly involving the right internal jugular, left innominate, and inferior thyroid veins. Since then there have been sporadic reports of venous ectasia in the neck, most of them in adults. We wish to present 3 pediatric cases of fusiform dilatation of the neck veins and review the preoperative diagnostic studies as well as the management of this entity.
CASE III (M.B.): A 10-year-old boy presented with a right-sided neck mass which increased in size with the Valsalva maneuver. The mass had been present since early childhood but had recently increased in size. A venous hum was audible. Percutaneous right femoral venous catherization and injection of contrast material (Renografin 60) into the right internal jugular vein revealed an aneurysmal dilatation in the area of the right supraclavicular fossa (Fig. 3, A), At surgery, an ectatic though structurally normal vein was removed (Fig. 3, B). DISCUSSION
CASE REPORTS CASE I (M.R.): A 6-year-old boy was first noted to have a soft nontender mass in the right side of the neck at age 2. On admission, the mass measured 2 X 1 em and increased in size during crying, straining during bowel movements, or the Valsalva maneuver (Fig. 1, A). His mother had noted a gradual increase in the size of the mass over the previous four years. A palpable thrill and continuous flow murmur were found in the right supraclavicular area. Plain radiographs of the neck and larynx demonstrated no abnormalities. Following percutaneous right femoral venous catheterization, internal jugular venography demonstrated ectasia of the right internal jugular vein (Fig. 1, B) and the lesion was excised. Pathological sections of the excised tissue revealed normal venous structures and elastic tissue. CASE II (W.A.): A 2-year-old girl was admitted for evaluation of a right supraclavicular mass. The mass had a doughy consistency and enlarged when the patient was crying or laughing; it had first been noted at the age of 7 months and had slowly increased in size since then. No thrills or murmurs were detected. Direct injection of contrast material (Hypaque) into the mass revealed a dilated right external jugular vein (Fig. 2). At surgery, a large fusiform dilatation of the external jugular vein was found and excised. Pathological examina-
In a large series of phlebectasias in adults, the superior vena cava, portal, greater and lesser saphenous, splenic, femoropopliteal, and facial veins were among those most frequently involved (7). Venous ectasias or aneurysms in the neck are rare entities, especially in children; since the first case report in 1928, there have been scattered reports of venous ectasia in the neck but only one such report in the radiological literature (5). In adults, they have been identified more often on the left side. It has been suggested that such dilatations are secondary to mechanical compression either of the left innominate vein' by a high tortuous aorta in hypertension (1) or of venous structures between the sternum and the left innominate artery in patients with pectus excavatum (2). However, these explanations are not applicable in children with no history of hypertension or evidence of pectus excavatum. All ~ of our patients were in good health, with normal central venous pressure; none had congenital heart disease, infection, cervical ribs, trauma, or other factors which might predispose to the development of venous ectasia. Therefore it seems most likely that these findings represent congenital ve-
1 From the Departments of Radiology (D.H.G., J.S.R., D.C.L.)and Pediatric Surgery (P.K.), State Universityof New York-Downstate Medical sjh Center, Brooklyn, N. Y. Accepted for publicationin June 1 9 7 5 . '
147
148
DAVID H. GORDON AND OTHERS
January 1976
Fig. 1. CASE I. A. Soft 2 X 1-cm mass in the neck (arrows) posterior to the sternomastoid muscle. B. Internal jugular venogram via the right femoral route demonstrates fusiform dilatation of the right internal jugular vein.
Fig. 2. CASE II. A dilated external jugular veln is demonstrated by direct injection of contrast material into the neck mass. Fig. 3. CASE III. A. Right internal jugular venogram from below reveals aneurysmal dilatation of the right internal jugular vein. B. Surgical exposure of the aneurysmally dilated right internal jugular vein.
nous malformations and probably should not be termed "aneurysms" in view of their normal histological structure.
Neck lesions in childhood are not uncommon, and accurate diagnosis of the mass is important. The dltterentlal diagnosis of cyst-like neck masses includes bronchi-
Vol. 118
149
JUGULAR VENOUS ECTASIA IN CHILDREN
al cysts, enterogenous cysts, cavE!rnous hemangiomas, largyngeal diverticula, Iymphocele, cervical adenitis, cystic tumors such as hygromas, and persistent jugular lymph sacs (3, 8). The diagnosis of venous ectasia is frequently made by the discovery of a soft mass which enlarges during the Valsalva maneuver and the presence of a venous hum which can be obliterated by compression above the mass. Since the therapy of these masses is questionable, a diagnostic work-up should include a venous study rather than arteriography, which carries increased risks in children. We prefer doing a percutaneous study via the femoral vein to avoid direct puncture of the ectatic segment and permit evaluation of other veins in the area for similar lesions. Histological examination of the tissue removed from our patients failed to clarify the etiology of the venous ectasia. However, Pataro et st. (6) found absence of the elastic layer and connective tissue proliferation in 4 adults with external jugular phlebectasia. In our patients, surgical treatment was performed basically for cosmetic purposes, since there have been no reports of rupture of venous ectasias of the neck. The children in our series have all remained well after surgery.
Pediatric Radiology
REFERENCES 1. Cortes FM: The kinked innominate vein: case proved by angiocardiography. Dis Chest 42: 114-116, Jul 1962 2. Geary FJ, Altman AR, Borrelli FJ, et al: Pectus excavatum as a cause of compressed innominate vein syndrome. NY State J Med 66: 1346-1349, 1 Jun 1966 3. Gilbert MG, Greenberg LA, Brown WT, et al: Fusiform venous aneurysm of the neck in children: a report of four cases. J Pediatr Surg 7:106-111, Apr 1972 4. Harris RI: Congenital venous cyst of the mediastinum. Ann Surg 88:953-956, Nov 1928 5. Okay NH, Bryk D, Kroop IG, et al: Phlebectasia of the jugular and great mediastinal veins. Radiology 95:629-630, Jun 1970 6. Pataro VF, Crosbie JC, Conde RM: Jugular phlebectasias. J Cardiovasc Surg 2:3-8, Jan 1961 7. Schatz IJ, Fine G: Venous aneurysms. N Engl J Med 266: 1310-1312,21 Jun 1962 8. Steinberg I, Watson RC: Lymphangiographic and angiographic diagnosis of persistent jugular lymph sac. Report of a case. N Engl J Med 275:1471-1474,29 Dec 1966
Department of Radiology State University of New York Downstate Medical Center Brooklyn, N. Y. 11203
•
Jugular Venous Ectasia in Children
Pediatric Radiology
A Report of 3 Cases and Review of the Literature 1
David H. Gordon, M.D., Judith S. Rose, M.D., Peter Kottmeier, M.D., and David C. Levin, M.D. Three cases of jugular venous ectasia in children were diagnosed preoperatively by selective venography. These rare lesions appear to be congenital dilatations of the veins and should not properly be called aneurysms, as their histological structure is normal. A typical history and complex of physical findings are associated with this entity. It is important to include it in the differential diagnosis of neck masses in children. INDEX TERM:
Veins, jugular
Radiology 118:147-149, January 1976
• tion of the specimen revealed a histologically normal venous structure with a normal amount of elastic tissue.
F
USIFORM dilatation or ectasia of the neck veins . is a rare entity in the pediatric population. Since neck lesions are not uncommon in children, these masses may pose a problem in differential diagnosis. The first case of neck vein "aneurysm" known to us was reported in 1928 by Harris (4), who described a venous anomaly involving the right internal jugular, left innominate, and inferior thyroid veins. Since then there have been sporadic reports of venous ectasia in the neck, most of them in adults. We wish to present 3 pediatric cases of fusiform dilatation of the neck veins and review the preoperative diagnostic studies as well as the management of this entity.
CASE III (M.B.): A 10-year-old boy presented with a right-sided neck mass which increased in size with the Valsalva maneuver. The mass had been present since early childhood but had recently increased in size. A venous hum was audible. Percutaneous right femoral venous catherization and injection of contrast material (Renografin 60) into the right internal jugular vein revealed an aneurysmal dilatation in the area of the right supraclavicular fossa (Fig. 3, A), At surgery, an ectatic though structurally normal vein was removed (Fig. 3, B). DISCUSSION
CASE REPORTS CASE I (M.R.): A 6-year-old boy was first noted to have a soft nontender mass in the right side of the neck at age 2. On admission, the mass measured 2 X 1 em and increased in size during crying, straining during bowel movements, or the Valsalva maneuver (Fig. 1, A). His mother had noted a gradual increase in the size of the mass over the previous four years. A palpable thrill and continuous flow murmur were found in the right supraclavicular area. Plain radiographs of the neck and larynx demonstrated no abnormalities. Following percutaneous right femoral venous catheterization, internal jugular venography demonstrated ectasia of the right internal jugular vein (Fig. 1, B) and the lesion was excised. Pathological sections of the excised tissue revealed normal venous structures and elastic tissue. CASE II (W.A.): A 2-year-old girl was admitted for evaluation of a right supraclavicular mass. The mass had a doughy consistency and enlarged when the patient was crying or laughing; it had first been noted at the age of 7 months and had slowly increased in size since then. No thrills or murmurs were detected. Direct injection of contrast material (Hypaque) into the mass revealed a dilated right external jugular vein (Fig. 2). At surgery, a large fusiform dilatation of the external jugular vein was found and excised. Pathological examina-
In a large series of phlebectasias in adults, the superior vena cava, portal, greater and lesser saphenous, splenic, femoropopliteal, and facial veins were among those most frequently involved (7). Venous ectasias or aneurysms in the neck are rare entities, especially in children; since the first case report in 1928, there have been scattered reports of venous ectasia in the neck but only one such report in the radiological literature (5). In adults, they have been identified more often on the left side. It has been suggested that such dilatations are secondary to mechanical compression either of the left innominate vein' by a high tortuous aorta in hypertension (1) or of venous structures between the sternum and the left innominate artery in patients with pectus excavatum (2). However, these explanations are not applicable in children with no history of hypertension or evidence of pectus excavatum. All ~ of our patients were in good health, with normal central venous pressure; none had congenital heart disease, infection, cervical ribs, trauma, or other factors which might predispose to the development of venous ectasia. Therefore it seems most likely that these findings represent congenital ve-
1 From the Departments of Radiology (D.H.G., J.S.R., D.C.L.)and Pediatric Surgery (P.K.), State Universityof New York-Downstate Medical sjh Center, Brooklyn, N. Y. Accepted for publicationin June 1 9 7 5 . '
147
148
DAVID H. GORDON AND OTHERS
January 1976
Fig. 1. CASE I. A. Soft 2 X 1-cm mass in the neck (arrows) posterior to the sternomastoid muscle. B. Internal jugular venogram via the right femoral route demonstrates fusiform dilatation of the right internal jugular vein.
Fig. 2. CASE II. A dilated external jugular veln is demonstrated by direct injection of contrast material into the neck mass. Fig. 3. CASE III. A. Right internal jugular venogram from below reveals aneurysmal dilatation of the right internal jugular vein. B. Surgical exposure of the aneurysmally dilated right internal jugular vein.
nous malformations and probably should not be termed "aneurysms" in view of their normal histological structure.
Neck lesions in childhood are not uncommon, and accurate diagnosis of the mass is important. The dltterentlal diagnosis of cyst-like neck masses includes bronchi-
Vol. 118
149
JUGULAR VENOUS ECTASIA IN CHILDREN
al cysts, enterogenous cysts, cavE!rnous hemangiomas, largyngeal diverticula, Iymphocele, cervical adenitis, cystic tumors such as hygromas, and persistent jugular lymph sacs (3, 8). The diagnosis of venous ectasia is frequently made by the discovery of a soft mass which enlarges during the Valsalva maneuver and the presence of a venous hum which can be obliterated by compression above the mass. Since the therapy of these masses is questionable, a diagnostic work-up should include a venous study rather than arteriography, which carries increased risks in children. We prefer doing a percutaneous study via the femoral vein to avoid direct puncture of the ectatic segment and permit evaluation of other veins in the area for similar lesions. Histological examination of the tissue removed from our patients failed to clarify the etiology of the venous ectasia. However, Pataro et st. (6) found absence of the elastic layer and connective tissue proliferation in 4 adults with external jugular phlebectasia. In our patients, surgical treatment was performed basically for cosmetic purposes, since there have been no reports of rupture of venous ectasias of the neck. The children in our series have all remained well after surgery.
Pediatric Radiology
REFERENCES 1. Cortes FM: The kinked innominate vein: case proved by angiocardiography. Dis Chest 42: 114-116, Jul 1962 2. Geary FJ, Altman AR, Borrelli FJ, et al: Pectus excavatum as a cause of compressed innominate vein syndrome. NY State J Med 66: 1346-1349, 1 Jun 1966 3. Gilbert MG, Greenberg LA, Brown WT, et al: Fusiform venous aneurysm of the neck in children: a report of four cases. J Pediatr Surg 7:106-111, Apr 1972 4. Harris RI: Congenital venous cyst of the mediastinum. Ann Surg 88:953-956, Nov 1928 5. Okay NH, Bryk D, Kroop IG, et al: Phlebectasia of the jugular and great mediastinal veins. Radiology 95:629-630, Jun 1970 6. Pataro VF, Crosbie JC, Conde RM: Jugular phlebectasias. J Cardiovasc Surg 2:3-8, Jan 1961 7. Schatz IJ, Fine G: Venous aneurysms. N Engl J Med 266: 1310-1312,21 Jun 1962 8. Steinberg I, Watson RC: Lymphangiographic and angiographic diagnosis of persistent jugular lymph sac. Report of a case. N Engl J Med 275:1471-1474,29 Dec 1966
Department of Radiology State University of New York Downstate Medical Center Brooklyn, N. Y. 11203
