Primary
Lesser
Saphenous
By Fuad Ramadan
Vein Aneurysm and George Johnson,
in a Child
Jr
Chapel Hill, North Carolina l True primary venous aneurysms are rare. This is the first report of such an aneurysm involving the lesser saphenous vein. Though most often a medical curiosity, such lesions can result in serious morbidity and even mortality. Because of their rarity they are often misdiagnosed. Salient features pertaining to classification, diagnosis, and treatment are reviewed. Copyright o 1991 by W.B. Saunders Company INDEX WORDS:
Aneurysm, saphenous vein.
NOUS ANEURYSMS are usually observed in adults, most often associated with trauma, arteriovenous fistulae, or altered venous hemodynamics.‘,2True primary venous aneurysms are exceedingly rare. In children they occur most frequently in the mediastinal and cervical regions. To our knowledge, only one report of a lesser saphenous vein aneurysm has been published.3 It involved a middle-aged man and was traumatic in origin. This is a report on a primary aneurysm invoIving the lesser saphenous vein of a 9-year-old girl.
v”
extension into the gluteus maximus muscle but no extension into the popliteal area. Angiodynography showed dilation of the distal portion of the lesser saphenous vein near its junction with the popliteal vein. The lesion was approached through a posterior popliteal incision with the patient in the prone position. There were multiple abnormally tortuous and dilated veins in the subcutaneous tissues; an extension of the hemangioma described previously. They had no communication with the aneurysm or any of the deeper venous structures. The aneurysm was subfascial in location and measured 5 x 6 cm. It arose from the most distal portion of the lesser saphenous vein and communicated with the popliteal vein via two branches (Fig 2). The aneurysm was carefully dissected until all its relations were clearly demonstrated. The aneurysm was then removed by dividing the lesser saphenous vein caudally and the two branches that communicated with the popliteal vein. Histological examination showed some segments of the aneurysm wall to contain normal components of smooth muscle and intact endothelium. However, in numerous other areas there was marked thinning of the wall with a pronounced decrease in the amount of smooth muscle and the presence of significant intimal proliferation. Some areas showed even complete absence of smooth muscle cells. The patient had an uneventful recovery and was discharged on the third postoperative morning.
CASE REPORT DISCUSSION A 9-year-old white girl was referred because of intermittent painful swelling in the left popliteal area. The mass became noticeable when the patient was 4 years old and has become progressively more symptomatic. It was most prominent with exercise and produced aching pain with running and swimming. The patient also had a cutaneous hemangioma of the left buttock and posterior thigh dating since birth. In fact, the initial impression was that this and the popliteal mass were part of the same lesion. On physical examination, the only abnormal findings involved the left lower extremity. There was a 4 x 5 cm nonpulsatile, nontender, soft mass in the left popliteal area that was easily compressible. A cutaneous hemangioma was present over the left buttock and the posterior aspect of the left thigh. Arteriograms of the left pelvis, thigh, and knee regions were obtained and showed no arterial abnormality. The previously mentioned superficial hemangioma was not observed. Antegrade venography showed the deep veins of the calf to be normal. The popliteal vein was flattened by a venous aneurysm situated just posterior and superficial to it (Fig 1). The aneurysm appeared to arise from the lesser saphenous vein and communicated with the popliteal vein. Magnetic resonance imaging scan demonstrated a cutaneous hemangioma of the left buttock with
From the Division of Vascular Surgery, Department of Surgery University of North Carolina School of Medicine, Chapel Hill, NC. Address reprint requests to Fuad Ramadan, MD, Division of Vascular Surgery, University of North Carolina School of Medicine, 210 Burnett-Worna& Campus Box 7210, Chapel Hill, NC 275997210. Copyright o 1991 by W.B. Saunders Company 0022-3468191/2606-0023$03.00/O
738
Venous aneurysms are extremely rare and are not even mentioned in most surgery and pathology texts. The first venous aneurysm recognized clinically was reported by Sir William Osler in The Lancet in 1913.4 It was associated with an arteriovenous fistula. A primary venous aneurysm was first reported by Harris in 1928 in a S-month-old infant.’ However, it was not until the report by Abbott in 1950 and the review by Abbott and Leigh in 1964: that venous aneurysms were established as a clinical entity. In the latter publication Abbott and Leigh proposed a classification of venous aneurysms based on etiology. According to this classification, a true primary venous aneurysm (thought to be congenital) is one that has no associated history of trauma or local inflammation and no abnormal increase in venous blood flow or pressure. Most venous aneurysms fall into the acquired category secondary to trauma or arteriovenous fistulae. Isolated aneurysm formation within a vein in the absence of hemodynamic change is rare, especially in the extremities. We have found only two reports of primary greater saphenous vein aneurysms,‘,7 only one of which was in a child.’ The only aneurysm of the lesser saphenous vein reported to date was associated with trauma and was in an adult JournalofPediatric Surgery, Vol 26, No 6 (June), 1991: pp 738-740
739
VENOUS ANEURYSMS
However, in some cases the mass may be persistent and hard due to thrombosis. Duplex scanning, with or without color-coded flow, as well as nuclear scintigraphyi315have been successful in making the diagnosis. However, definitive diagnosis can be established by
b
Popliteal v.
Fig 1.
Antegrade venogram showing the venous aneurysm located
posteriorly to the popliteal vein.
man.3 The present case is the first primary lesser saphenous vein aneurysm that we know of. Although rare, venous aneurysms have been found in the following veins: facial, jugular (internal, external, and anterior), subclavian, innominate, superior vena cava, azygous, portal, splenic, renal, femoral, popliteal, greater and lesser saphenous, brachial, and ulnar. Regardless of their etiology, they should be considered in the differential diagnosis of subcutaneous masses. Several cases of popliteal vein aneurysms have been reported, some of which were complicated by thrombosis and pulmonary embolism.8~1’ Several cases of femoral vein aneurysms simulating inguinal hernias have been reported.12-‘4 Diagnosis can be made on clinical grounds, when a soft mass is encountered that changes in size with the Valsalva maneuver or with the position of the limb.
Fig 2. (A) Operative photograph and (B) corresponding diagram showing the aneurysm and its relations to the lesser saphenous and popliteal veins and to the tibia1 nerve.
740
RAMADAN
venography or by observing the venous phase of an arteriogram.2 In the extremities and the neck, successful treatment can be achieved by simple excision with ligation of the tributaries.* Venous aneurysms can be an integral part of congenital arteriovenous malformations, manifested by the presence of port-wine stains or hemangiomata. It is interesting that the present patient had a hemangioma of the ipsilateral thigh and buttock. This finding initially led us to believe that the popliteal
AND JOHNSON
mass was an integral component of the hemangioma. Neither careful dissection at operation, nor any of the imaging studies detailed previously could demonstrate any connection between the two lesions. ACKNOWLEDGMENT We would like to thank Dr Robert Reddick from the Department of Pathology for providing the photomicrographs; and Gwynne Moore from the Department of Surgery for help with the illustrations.
REFERENCES 1. Yasumoto M, Shibuya H, Goto Y, et al: Primary saphenous venous aneurysms presenting in a child. Clin Nucl Med 12:29-30, 1987 2. Yao JST, Van Bellen B, Flinn WR, et al: Aneurysms of the venous system, in Bergan JJ, Yao JST (eds): Aneurysms: Diagnosis and Treatment. Philadelphia, PA, Grune & Stratton, 1982, pp 515-530 3. Schatz IJ, Fine G: Venous aneurysms. N Engl J Med 266:1310-1312,1962 4. Osler W: An arteriovenous aneurysm of the axillary vessels of 30years duration. Lancet 2:1248,1913 5. Harris RI: Congenital venous cyst of the mediastinum. Ann Surg 88:953,1928 6. Abbott OA, Leigh TF: Aneurysmal dilatations of the superior vena caval system. Ann Surg 159:858-871,1964 7. Lippert KM, Frederick LD Jr: A sacculated aneurysm of the saphenous vein. Ann Surg 134:924-926,195l 8. Greenwood LH, Yrizarry JM, Hallett JW Jr: Peripheral venous aneurysms with recurrent pulmonary embolism: Report of
a case and review of the literature. Cardiovasc Intervent Radio1 543-45, 1982 9. Chahlaoui J, Julien M, Nadeau P, et al: Popliteal venous aneurysm: A source of pulmonary embolism. AJR 136:415-416, 1981 10. Dahl JR, Freed TA, Burke MF: Popliteal vein aneurysm with recurrent pulmonary thromboemboli. JAMA 236:2.531-2532, 1976 11. Marin J, Gosselin J, Kbayat A, et al: A propos d’un cas d’anevrisme de la veine poplitee avec embolie pulmonaire a repetition. Phlebologie 31:433-438,1978 12. Sproul G: Venous aneurysm: A rare cause of reducible inguinal mass in infancy. Surgery 58:1027-1030, 1965 13. Kuzmina IM, Kuzmin KP: Femoral venous aneurysms simulating hernias. Vestn Khir 86:136-137,196l 14. Maltov AI, Golovan VE: Venous aneurysm simulating a strangulated femoral hernia. Klin Khir 8:75,1972 15. Subramanian KS, Freeman ML, Gergans G, et al: Nuclear scintigraphy as a diagnostic tool in venous aneurysms. J Otolaryngo1 13:409-411,1984
Lesser
Saphenous
By Fuad Ramadan
Vein Aneurysm and George Johnson,
in a Child
Jr
Chapel Hill, North Carolina l True primary venous aneurysms are rare. This is the first report of such an aneurysm involving the lesser saphenous vein. Though most often a medical curiosity, such lesions can result in serious morbidity and even mortality. Because of their rarity they are often misdiagnosed. Salient features pertaining to classification, diagnosis, and treatment are reviewed. Copyright o 1991 by W.B. Saunders Company INDEX WORDS:
Aneurysm, saphenous vein.
NOUS ANEURYSMS are usually observed in adults, most often associated with trauma, arteriovenous fistulae, or altered venous hemodynamics.‘,2True primary venous aneurysms are exceedingly rare. In children they occur most frequently in the mediastinal and cervical regions. To our knowledge, only one report of a lesser saphenous vein aneurysm has been published.3 It involved a middle-aged man and was traumatic in origin. This is a report on a primary aneurysm invoIving the lesser saphenous vein of a 9-year-old girl.
v”
extension into the gluteus maximus muscle but no extension into the popliteal area. Angiodynography showed dilation of the distal portion of the lesser saphenous vein near its junction with the popliteal vein. The lesion was approached through a posterior popliteal incision with the patient in the prone position. There were multiple abnormally tortuous and dilated veins in the subcutaneous tissues; an extension of the hemangioma described previously. They had no communication with the aneurysm or any of the deeper venous structures. The aneurysm was subfascial in location and measured 5 x 6 cm. It arose from the most distal portion of the lesser saphenous vein and communicated with the popliteal vein via two branches (Fig 2). The aneurysm was carefully dissected until all its relations were clearly demonstrated. The aneurysm was then removed by dividing the lesser saphenous vein caudally and the two branches that communicated with the popliteal vein. Histological examination showed some segments of the aneurysm wall to contain normal components of smooth muscle and intact endothelium. However, in numerous other areas there was marked thinning of the wall with a pronounced decrease in the amount of smooth muscle and the presence of significant intimal proliferation. Some areas showed even complete absence of smooth muscle cells. The patient had an uneventful recovery and was discharged on the third postoperative morning.
CASE REPORT DISCUSSION A 9-year-old white girl was referred because of intermittent painful swelling in the left popliteal area. The mass became noticeable when the patient was 4 years old and has become progressively more symptomatic. It was most prominent with exercise and produced aching pain with running and swimming. The patient also had a cutaneous hemangioma of the left buttock and posterior thigh dating since birth. In fact, the initial impression was that this and the popliteal mass were part of the same lesion. On physical examination, the only abnormal findings involved the left lower extremity. There was a 4 x 5 cm nonpulsatile, nontender, soft mass in the left popliteal area that was easily compressible. A cutaneous hemangioma was present over the left buttock and the posterior aspect of the left thigh. Arteriograms of the left pelvis, thigh, and knee regions were obtained and showed no arterial abnormality. The previously mentioned superficial hemangioma was not observed. Antegrade venography showed the deep veins of the calf to be normal. The popliteal vein was flattened by a venous aneurysm situated just posterior and superficial to it (Fig 1). The aneurysm appeared to arise from the lesser saphenous vein and communicated with the popliteal vein. Magnetic resonance imaging scan demonstrated a cutaneous hemangioma of the left buttock with
From the Division of Vascular Surgery, Department of Surgery University of North Carolina School of Medicine, Chapel Hill, NC. Address reprint requests to Fuad Ramadan, MD, Division of Vascular Surgery, University of North Carolina School of Medicine, 210 Burnett-Worna& Campus Box 7210, Chapel Hill, NC 275997210. Copyright o 1991 by W.B. Saunders Company 0022-3468191/2606-0023$03.00/O
738
Venous aneurysms are extremely rare and are not even mentioned in most surgery and pathology texts. The first venous aneurysm recognized clinically was reported by Sir William Osler in The Lancet in 1913.4 It was associated with an arteriovenous fistula. A primary venous aneurysm was first reported by Harris in 1928 in a S-month-old infant.’ However, it was not until the report by Abbott in 1950 and the review by Abbott and Leigh in 1964: that venous aneurysms were established as a clinical entity. In the latter publication Abbott and Leigh proposed a classification of venous aneurysms based on etiology. According to this classification, a true primary venous aneurysm (thought to be congenital) is one that has no associated history of trauma or local inflammation and no abnormal increase in venous blood flow or pressure. Most venous aneurysms fall into the acquired category secondary to trauma or arteriovenous fistulae. Isolated aneurysm formation within a vein in the absence of hemodynamic change is rare, especially in the extremities. We have found only two reports of primary greater saphenous vein aneurysms,‘,7 only one of which was in a child.’ The only aneurysm of the lesser saphenous vein reported to date was associated with trauma and was in an adult JournalofPediatric Surgery, Vol 26, No 6 (June), 1991: pp 738-740
739
VENOUS ANEURYSMS
However, in some cases the mass may be persistent and hard due to thrombosis. Duplex scanning, with or without color-coded flow, as well as nuclear scintigraphyi315have been successful in making the diagnosis. However, definitive diagnosis can be established by
b
Popliteal v.
Fig 1.
Antegrade venogram showing the venous aneurysm located
posteriorly to the popliteal vein.
man.3 The present case is the first primary lesser saphenous vein aneurysm that we know of. Although rare, venous aneurysms have been found in the following veins: facial, jugular (internal, external, and anterior), subclavian, innominate, superior vena cava, azygous, portal, splenic, renal, femoral, popliteal, greater and lesser saphenous, brachial, and ulnar. Regardless of their etiology, they should be considered in the differential diagnosis of subcutaneous masses. Several cases of popliteal vein aneurysms have been reported, some of which were complicated by thrombosis and pulmonary embolism.8~1’ Several cases of femoral vein aneurysms simulating inguinal hernias have been reported.12-‘4 Diagnosis can be made on clinical grounds, when a soft mass is encountered that changes in size with the Valsalva maneuver or with the position of the limb.
Fig 2. (A) Operative photograph and (B) corresponding diagram showing the aneurysm and its relations to the lesser saphenous and popliteal veins and to the tibia1 nerve.
740
RAMADAN
venography or by observing the venous phase of an arteriogram.2 In the extremities and the neck, successful treatment can be achieved by simple excision with ligation of the tributaries.* Venous aneurysms can be an integral part of congenital arteriovenous malformations, manifested by the presence of port-wine stains or hemangiomata. It is interesting that the present patient had a hemangioma of the ipsilateral thigh and buttock. This finding initially led us to believe that the popliteal
AND JOHNSON
mass was an integral component of the hemangioma. Neither careful dissection at operation, nor any of the imaging studies detailed previously could demonstrate any connection between the two lesions. ACKNOWLEDGMENT We would like to thank Dr Robert Reddick from the Department of Pathology for providing the photomicrographs; and Gwynne Moore from the Department of Surgery for help with the illustrations.
REFERENCES 1. Yasumoto M, Shibuya H, Goto Y, et al: Primary saphenous venous aneurysms presenting in a child. Clin Nucl Med 12:29-30, 1987 2. Yao JST, Van Bellen B, Flinn WR, et al: Aneurysms of the venous system, in Bergan JJ, Yao JST (eds): Aneurysms: Diagnosis and Treatment. Philadelphia, PA, Grune & Stratton, 1982, pp 515-530 3. Schatz IJ, Fine G: Venous aneurysms. N Engl J Med 266:1310-1312,1962 4. Osler W: An arteriovenous aneurysm of the axillary vessels of 30years duration. Lancet 2:1248,1913 5. Harris RI: Congenital venous cyst of the mediastinum. Ann Surg 88:953,1928 6. Abbott OA, Leigh TF: Aneurysmal dilatations of the superior vena caval system. Ann Surg 159:858-871,1964 7. Lippert KM, Frederick LD Jr: A sacculated aneurysm of the saphenous vein. Ann Surg 134:924-926,195l 8. Greenwood LH, Yrizarry JM, Hallett JW Jr: Peripheral venous aneurysms with recurrent pulmonary embolism: Report of
a case and review of the literature. Cardiovasc Intervent Radio1 543-45, 1982 9. Chahlaoui J, Julien M, Nadeau P, et al: Popliteal venous aneurysm: A source of pulmonary embolism. AJR 136:415-416, 1981 10. Dahl JR, Freed TA, Burke MF: Popliteal vein aneurysm with recurrent pulmonary thromboemboli. JAMA 236:2.531-2532, 1976 11. Marin J, Gosselin J, Kbayat A, et al: A propos d’un cas d’anevrisme de la veine poplitee avec embolie pulmonaire a repetition. Phlebologie 31:433-438,1978 12. Sproul G: Venous aneurysm: A rare cause of reducible inguinal mass in infancy. Surgery 58:1027-1030, 1965 13. Kuzmina IM, Kuzmin KP: Femoral venous aneurysms simulating hernias. Vestn Khir 86:136-137,196l 14. Maltov AI, Golovan VE: Venous aneurysm simulating a strangulated femoral hernia. Klin Khir 8:75,1972 15. Subramanian KS, Freeman ML, Gergans G, et al: Nuclear scintigraphy as a diagnostic tool in venous aneurysms. J Otolaryngo1 13:409-411,1984
