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Isolated aneurysm of the internal jugular vein: A report of three cases
Isolated Aneurysm of the Internal Jugular Vein: A Report of Three Cases By Richard K. Danis St. Louis, M i s s o u r i 9 Isolated venous a n e u r y s m s are rare in children. T h r e e patients p r e s e n t e d w i t h progressively enlarging cervical masses t h a t proved to be fusiform aneurysms of the internal jugular vein. Surgical excision w a s carried out in each instance. Histopathologic e x a m i n a t i o n revealed changes Suggesting elastic tissue dysplasia similar to t h a t previously described in abnormal carotid a r t e r i e s of some children. I N D E X W O R D S : V e n o u s aneurysm.
r E N O U S A N E U R Y S M S are not uncommon in adults and are usually seen in association with previous trauma or diseases involving contiguous veins. Venous aneurysms in children occur most frequently in thc mediastinal and cervical regions.- They may be fusiform or saccular, and a history of trauma or associated disease is not usual. Previous reports have not identified a specific histopathologic abnormality.: Microscopic examination of the lesions in these patients demonstrated several changes including significant elastic tissue dysplasia. Fusiform aneurysms of the internal jugular vein developed in three patients ranging in age from two to nine years. Progressive enlargement of the aneurysm was seen in all patients. Two patients complained of an annoying "rumbling" sensation in the area of the lesion. Operative excision was carried out in each instance. No evidence of distal obstruction, local inflammation or previous trauma was found.
V
After proximal and distal ligation of the jugular vein, the intervening portion of the vein and the aneurysm were excised. Case 2. S.N., 9 yr old female, presented with a large mass in the left neck and complained of a "rumbling" sensation in the area. The mass had progressively enlarged during the previous 6 too. Examination revealed it to be compressible, and signiticant enlargement occurred with the application of a Valsalva maneuver. A venous hum was present. Operative excision of a large aneurysm of the internal jugular vein was carried out in the same manner as in the tirst case. ('ase 3. J.M., a 4 yr old male, had a progressively enlarging, compressible mass of the left neck for 8 mo and a recent onset of a "'rumbling" sensation in the area. The mass reportedly enlarged markedly when the patient coughed. At exploration a large fusiform ancurysm was idcntitied and excised as in the other cases. DISCUSSION The cases arc remarkably similar. Extensive diagnostic studies as recommended by Gilbert ~ were not carried out since the diagnosis was obvious clinically. There were no early or [ate complications. No other aneurysms have appeared in followup ranging 4 7 yr. Surgical excision was elected because the lesion appeared to be progressively enlarging and sacrilicc of the vein could be accomplished wilhout complications. Microscopic examination (11 & E Trichrome VerhocffVan Gieson stains) of the excised tissue showed the architec-
CASE REPORTS
Case I. A.S., a 22-mo old female, presented with a large compressible mass in the left neck. The mass enlarged significantly when a Valsalva maneuver was performed and progressive enlargement had been noted for several months. The lesion was not noted one year earlier when the patient underwent an inguinal hernia repair. At operation an isolated fusiform aneurysm of the internal jugular vein was found.
From the Department o f Surgery, Cardinal Glennon Memorial Children's Hospital and St. Louis University School o f Medicine. Address reprint requests to Richard K. Danis, M.D., 1401 South Grand Blvd., St. Louis, Mo. 63104. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1702m)003501.00/0 130
Fig. 1. Normal internal jugular vein showing intimal elastic fibers regularly arranged and negligible smooth muscle cells9 (Verhoeff-Van Gieson Stain, 78X).
Journal of Pediatric Surgery, Vol. 17; No. 2 (April), 1982
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Fig. 3. Intimal sclerosis and thickening with focal loss and disruption of elastic fibers. Note prominence of hyperplastic smooth muscle cells. (Verhoeff-Van Gieson Stain 196X). Fig. 2. Internal jugular aneurysm showing intimal thickening, focal loss and disordered arrangement of elastic fibers and focally prominent smooth muscle cells. (Verhoeff-Van Gieson Stain, 196X).
ture of the jugular vein adjacent to the aneurysm to be normal (Fig. I), Similar examination of the wall of the aneurysm showed focal intimal thickening, increased amount of connective tissue and increased number of endothelial cells, often arranged perpendicularly to the intimal surface (Fig. 2). In addition the wall showed prominence of smooth
muscle cells and collagen with sparse elastic elements which appear disrupted (Fig. 3). The intimal changes suggest turbulance with resulting mechanical effects. Fibrosis and prominence of muscle cells are probably reactive. The elastic tissue abnormality resembles elastic tissue dysplasia of the internal carotid artery of children described by Ochsner." It is proposed that these aneurysms may well represent the venous counterpart to that apparently congenital, isolated arterial abnormality.
REFERENCES
1. Schatz IJ, Fine G: Venous aneurysms. N Eng J Med 266:1310-1312, 1962 2. Gilbert NG, Greenberg LA, Brown WT, Puranik S: Fusiform venous aneurysm of the neck in children: A report of four cases. Jour Ped Surg 7:106 111, 1972
3, Hell BJ, Felman AH. Talbert JL, et al: ldopathic dilation of the superior vena cava. J Ped Surg 13:193, 1978 4. Ochsner JL, Hughes JP, Leonard JL, et al: Elastic tissue dysplasia of the internal carotid artery, Ann Surg 185:684 690, 1977
r E N O U S A N E U R Y S M S are not uncommon in adults and are usually seen in association with previous trauma or diseases involving contiguous veins. Venous aneurysms in children occur most frequently in thc mediastinal and cervical regions.- They may be fusiform or saccular, and a history of trauma or associated disease is not usual. Previous reports have not identified a specific histopathologic abnormality.: Microscopic examination of the lesions in these patients demonstrated several changes including significant elastic tissue dysplasia. Fusiform aneurysms of the internal jugular vein developed in three patients ranging in age from two to nine years. Progressive enlargement of the aneurysm was seen in all patients. Two patients complained of an annoying "rumbling" sensation in the area of the lesion. Operative excision was carried out in each instance. No evidence of distal obstruction, local inflammation or previous trauma was found.
V
After proximal and distal ligation of the jugular vein, the intervening portion of the vein and the aneurysm were excised. Case 2. S.N., 9 yr old female, presented with a large mass in the left neck and complained of a "rumbling" sensation in the area. The mass had progressively enlarged during the previous 6 too. Examination revealed it to be compressible, and signiticant enlargement occurred with the application of a Valsalva maneuver. A venous hum was present. Operative excision of a large aneurysm of the internal jugular vein was carried out in the same manner as in the tirst case. ('ase 3. J.M., a 4 yr old male, had a progressively enlarging, compressible mass of the left neck for 8 mo and a recent onset of a "'rumbling" sensation in the area. The mass reportedly enlarged markedly when the patient coughed. At exploration a large fusiform ancurysm was idcntitied and excised as in the other cases. DISCUSSION The cases arc remarkably similar. Extensive diagnostic studies as recommended by Gilbert ~ were not carried out since the diagnosis was obvious clinically. There were no early or [ate complications. No other aneurysms have appeared in followup ranging 4 7 yr. Surgical excision was elected because the lesion appeared to be progressively enlarging and sacrilicc of the vein could be accomplished wilhout complications. Microscopic examination (11 & E Trichrome VerhocffVan Gieson stains) of the excised tissue showed the architec-
CASE REPORTS
Case I. A.S., a 22-mo old female, presented with a large compressible mass in the left neck. The mass enlarged significantly when a Valsalva maneuver was performed and progressive enlargement had been noted for several months. The lesion was not noted one year earlier when the patient underwent an inguinal hernia repair. At operation an isolated fusiform aneurysm of the internal jugular vein was found.
From the Department o f Surgery, Cardinal Glennon Memorial Children's Hospital and St. Louis University School o f Medicine. Address reprint requests to Richard K. Danis, M.D., 1401 South Grand Blvd., St. Louis, Mo. 63104. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1702m)003501.00/0 130
Fig. 1. Normal internal jugular vein showing intimal elastic fibers regularly arranged and negligible smooth muscle cells9 (Verhoeff-Van Gieson Stain, 78X).
Journal of Pediatric Surgery, Vol. 17; No. 2 (April), 1982
ANEURYSMS OF INTERNAL JUGULAR VEIN
131
Fig. 3. Intimal sclerosis and thickening with focal loss and disruption of elastic fibers. Note prominence of hyperplastic smooth muscle cells. (Verhoeff-Van Gieson Stain 196X). Fig. 2. Internal jugular aneurysm showing intimal thickening, focal loss and disordered arrangement of elastic fibers and focally prominent smooth muscle cells. (Verhoeff-Van Gieson Stain, 196X).
ture of the jugular vein adjacent to the aneurysm to be normal (Fig. I), Similar examination of the wall of the aneurysm showed focal intimal thickening, increased amount of connective tissue and increased number of endothelial cells, often arranged perpendicularly to the intimal surface (Fig. 2). In addition the wall showed prominence of smooth
muscle cells and collagen with sparse elastic elements which appear disrupted (Fig. 3). The intimal changes suggest turbulance with resulting mechanical effects. Fibrosis and prominence of muscle cells are probably reactive. The elastic tissue abnormality resembles elastic tissue dysplasia of the internal carotid artery of children described by Ochsner." It is proposed that these aneurysms may well represent the venous counterpart to that apparently congenital, isolated arterial abnormality.
REFERENCES
1. Schatz IJ, Fine G: Venous aneurysms. N Eng J Med 266:1310-1312, 1962 2. Gilbert NG, Greenberg LA, Brown WT, Puranik S: Fusiform venous aneurysm of the neck in children: A report of four cases. Jour Ped Surg 7:106 111, 1972
3, Hell BJ, Felman AH. Talbert JL, et al: ldopathic dilation of the superior vena cava. J Ped Surg 13:193, 1978 4. Ochsner JL, Hughes JP, Leonard JL, et al: Elastic tissue dysplasia of the internal carotid artery, Ann Surg 185:684 690, 1977