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Congenital venous web causing subclavian vein obstruction: A case report
Congenital venous web causing subclavian vein obstruction: A case report Jay B. Fisher, M D , and Marc A. Granson, M D , Easton, Pa. A 7V2-year-old black girl had exercise-induced swelling of the left arm and left anterior chest wall caused by a congenital venous web of the subclavian vein. Operative repair was accomplished by venotomy and excision of the web. A venous digital subtraction angiogram obtained on the sixth postoperative day showed unobstructed flow through the operative area. We believe that an isolated, nonthrombotic functional obstruction of the subclavian vein by a venous web has not been previously reported in the English-language literature. (J VASC SURG 1989;10:460-2.)
Isolated intermittent obstruction of the subclavian vein without thrombosis is due primarily to mechanical compression of the vein by the musculoskeletal and ligamentous structures that occupy the costoclavicular space.1 Patients with this obstruction usually have intermittent swelling, fullness, and exacerbation of their symptoms with exercise or posi-
From the Depamnentsof of Surgery,Boston UniversityMedical Center and Easton Hospital. Reprint requests: Marc A. Granson, MD, Division of Vascular Surgery, Departmentof Surgery,Easton Hospital, Easton, PA 18042. 24/37/14209
tional changes. Rest and elevation of the extremity will usually relieve the symptoms. Nonthrombotic intermittent obstruction o£ a vein by a venous web is even a rarer cause o f such a phenomenon. Two case reports of patients with Budd-Chiari syndrome caused by a web in the inferior vena cava have been publishedY A report of obstruction of the subclavian vein and axillary veins possibly caused by filariasis has also been published. 4 No such case report exists with regard to a congenital venous web of the subclavian vein in a child. We present a case history of a young girl with isolated nonthrombotic obstruction of the subclavian vein caused by a congenital venous web.
Fig. 1. Preoperative venogram shows obstruction of the subclavian vein. 460
CASE REPORT A 7Y~ycar-oldblack girl camc to Easton Hospital with complaints of intermittent swelling of thc left arm and left anterior chest wall. The swelling always startcd after vigorous exercise, usually swimming. Her mother described the swelling as at least twice thc sizc of her normal arm. There was also a history of weakness and numbness of the lcft arm, without cvidcnce of recent trauma. Pcrtincnt past medical history included a fracture of the lcft forearm at the age of 4 years, which resolved completely with conservativc treatmcnt. Other significant past mcdical histon included bronchial asthma. O n physical examination thc patient \vas found to have gcneralixd swelling of the left uppcr extremity with an increase in birth by 4 to 5 cm when compared with the right arm. Brachial and radial pulses were palpable bilaterally, ,md no neurologic dcficit was apprcciatcd. A venograrn of the upper extremity revealcd a highgradc obstruction of the proximal lcft subclavian vein as shown in Fig. 1. Computerized tomography scanning of the neck and chest and conventional ccmical neck films wcrc all unremarkable. The patient undenivnt an esploration of the left subclavian vein via a supraclavicular approach. The exposure was aidcd by disarticulation of the left sternoclavicular joint. Thc entire length of thc left subclavian vein and its confluencc with the intcrnal jugular vein was exposed. After complete cxposure and partial mobilization of the subclavian vein, one could appreciate a change in die caliber of thc vein. A gradual dilation was noted distally, and venotomy in this transition arca revealed the presence of a "vcnous web" that causcd partial obstn~ction of this vein. A thin, filmy diaphragm-like membrane, with a central opcning o n thc web, as shown in Fig. 2, was obsenxd. This weblike structure had a much thickcr tcxturc than that of a venous valve, and there was no cvidencc of a bicuspid nature. The structure was homogeneously circumferential with a small ccntral opcning that
was inciscd during r l process ~ of compieting our vcnotomy. The web was completclp excised, and thc venotomy was closed primarily without restricting the caliber of the vein. The patient's postoperative course was unremarkable. A reduction in the girth of the uppcr extremity by 2 crn was noticed by thc fifth postoperative day. A vcnous digital subtraction angiogranl was performed o n the sixth postoperative day, which revcaled patency of thc left subclavian vein without cvidcnce of postopcrativc stcnosis, as shown in Fig. 3.
DISCUSSION Obstniction of the subclavian win without thrombosis was Ekst reported in 1939 by
Fig. 2. Artists drawing of congenital venous web causing obstrucrion of the subclavian vein.
Fig. 3. Postoperative vcnous digital subtraction angiogratn
Journal of VASCULAR SURGERY
462 Fisherand Granson
McLaughlin and Popma.* All previous clinical presentations were discussed in association with an effort vein thrombosis o f the axillary and subclavian veins, the so-called P a g e t - V o n Schroetter syndrome. A clinical distinction between these two entities is impossible, and only the finding o f a thrombosis will separate these two disease processes. We present a case o f a young girl with a truly functional obstruction. The clinical manifestations o f obstruction became apparent only with exercise. One can assume that with the extremity at rest the normal flow state was easily handled by the constricting web; however, during exercise a state o f venous hypertension was reached locally, producing edema and swelling o f the extremity. Because o f the severity o f the swelling and its incapacitating nature, an operative approach was necessary to correct the problem. Primary excision o f the web was all that was necessary. Postoperative angiographic evaluation revealed no area o f stenosis around the operative site and no
external compressive phenomenon that might result from the local dissection, A case o f a young girl with an isolated nonthrombotic obstruction o f the subclavian vein caused by a congenital venous web is presented. Venotomy and primary excision o f the membrane were necessary to correct the obstruction. N o postoperative sequelae were observed. REFERENCES
1. Adams JT, DeweeseJA, MahoneyEB, Rob CG. Intermittent subclavian vein obstruction without thrombosis. Surgery 1968;43:147-65. 2. Murphy JP, Gregoric I, Cooley DA. Budd-Chiari syndrome resulting from a membranous web of the inferior vena cava: operative repair using profound hypothermia and circulatory arrest. Ann Thorac Surg 1987;43:212-4. 3. ShaffnerF, GadboysHL, SaframAP, Baron MG, AnfsesAH. Budd-Chiari syndrome caused by a web in the inferior vena cava. Am J Med 1967;42:838-42. 4. McLaughlinCW Jr, Popma AM. Intermittent obstruction of subclavian vein. JAMA 1960;113:1939-45.
B O U N D VOLUMES AVAILABLE TO SUBSCRIBERS
Bound volumes of the JOUKNALOF VASCULARSURGERYfor 1989 are available to subscribers only. They may be purchased from the publisher at a cost of $52.00 ($66.00 international) for Vol. 9 (January to June) and Vol. 10 (July to December). Price includes shipping charges. Each bound volume contains a subject and author index, and all advertising is removed. Copies are shipped within 60 days after publication of the last issue in the volume. The binding is durable buckram with the journal name, volume number, and year stamped in gold on the spine. Payment must accompany all orders. Contact Circulation Fulfillment, The C.V. Mosby Company, 11830 Westline Industrial Drive, St. Louis, MO 63146-3318, USA. In the United States call toll free: (800)325-4177, ext. 351. In Missouri call collect: (314)872-8370, ext. 351. Subscriptions must bc in force to qualify. Bound volumes are not available in place of a regular JOURNALsubscription.
Isolated intermittent obstruction of the subclavian vein without thrombosis is due primarily to mechanical compression of the vein by the musculoskeletal and ligamentous structures that occupy the costoclavicular space.1 Patients with this obstruction usually have intermittent swelling, fullness, and exacerbation of their symptoms with exercise or posi-
From the Depamnentsof of Surgery,Boston UniversityMedical Center and Easton Hospital. Reprint requests: Marc A. Granson, MD, Division of Vascular Surgery, Departmentof Surgery,Easton Hospital, Easton, PA 18042. 24/37/14209
tional changes. Rest and elevation of the extremity will usually relieve the symptoms. Nonthrombotic intermittent obstruction o£ a vein by a venous web is even a rarer cause o f such a phenomenon. Two case reports of patients with Budd-Chiari syndrome caused by a web in the inferior vena cava have been publishedY A report of obstruction of the subclavian vein and axillary veins possibly caused by filariasis has also been published. 4 No such case report exists with regard to a congenital venous web of the subclavian vein in a child. We present a case history of a young girl with isolated nonthrombotic obstruction of the subclavian vein caused by a congenital venous web.
Fig. 1. Preoperative venogram shows obstruction of the subclavian vein. 460
CASE REPORT A 7Y~ycar-oldblack girl camc to Easton Hospital with complaints of intermittent swelling of thc left arm and left anterior chest wall. The swelling always startcd after vigorous exercise, usually swimming. Her mother described the swelling as at least twice thc sizc of her normal arm. There was also a history of weakness and numbness of the lcft arm, without cvidcnce of recent trauma. Pcrtincnt past medical history included a fracture of the lcft forearm at the age of 4 years, which resolved completely with conservativc treatmcnt. Other significant past mcdical histon included bronchial asthma. O n physical examination thc patient \vas found to have gcneralixd swelling of the left uppcr extremity with an increase in birth by 4 to 5 cm when compared with the right arm. Brachial and radial pulses were palpable bilaterally, ,md no neurologic dcficit was apprcciatcd. A venograrn of the upper extremity revealcd a highgradc obstruction of the proximal lcft subclavian vein as shown in Fig. 1. Computerized tomography scanning of the neck and chest and conventional ccmical neck films wcrc all unremarkable. The patient undenivnt an esploration of the left subclavian vein via a supraclavicular approach. The exposure was aidcd by disarticulation of the left sternoclavicular joint. Thc entire length of thc left subclavian vein and its confluencc with the intcrnal jugular vein was exposed. After complete cxposure and partial mobilization of the subclavian vein, one could appreciate a change in die caliber of thc vein. A gradual dilation was noted distally, and venotomy in this transition arca revealed the presence of a "vcnous web" that causcd partial obstn~ction of this vein. A thin, filmy diaphragm-like membrane, with a central opcning o n thc web, as shown in Fig. 2, was obsenxd. This weblike structure had a much thickcr tcxturc than that of a venous valve, and there was no cvidencc of a bicuspid nature. The structure was homogeneously circumferential with a small ccntral opcning that
was inciscd during r l process ~ of compieting our vcnotomy. The web was completclp excised, and thc venotomy was closed primarily without restricting the caliber of the vein. The patient's postoperative course was unremarkable. A reduction in the girth of the uppcr extremity by 2 crn was noticed by thc fifth postoperative day. A vcnous digital subtraction angiogranl was performed o n the sixth postoperative day, which revcaled patency of thc left subclavian vein without cvidcnce of postopcrativc stcnosis, as shown in Fig. 3.
DISCUSSION Obstniction of the subclavian win without thrombosis was Ekst reported in 1939 by
Fig. 2. Artists drawing of congenital venous web causing obstrucrion of the subclavian vein.
Fig. 3. Postoperative vcnous digital subtraction angiogratn
Journal of VASCULAR SURGERY
462 Fisherand Granson
McLaughlin and Popma.* All previous clinical presentations were discussed in association with an effort vein thrombosis o f the axillary and subclavian veins, the so-called P a g e t - V o n Schroetter syndrome. A clinical distinction between these two entities is impossible, and only the finding o f a thrombosis will separate these two disease processes. We present a case o f a young girl with a truly functional obstruction. The clinical manifestations o f obstruction became apparent only with exercise. One can assume that with the extremity at rest the normal flow state was easily handled by the constricting web; however, during exercise a state o f venous hypertension was reached locally, producing edema and swelling o f the extremity. Because o f the severity o f the swelling and its incapacitating nature, an operative approach was necessary to correct the problem. Primary excision o f the web was all that was necessary. Postoperative angiographic evaluation revealed no area o f stenosis around the operative site and no
external compressive phenomenon that might result from the local dissection, A case o f a young girl with an isolated nonthrombotic obstruction o f the subclavian vein caused by a congenital venous web is presented. Venotomy and primary excision o f the membrane were necessary to correct the obstruction. N o postoperative sequelae were observed. REFERENCES
1. Adams JT, DeweeseJA, MahoneyEB, Rob CG. Intermittent subclavian vein obstruction without thrombosis. Surgery 1968;43:147-65. 2. Murphy JP, Gregoric I, Cooley DA. Budd-Chiari syndrome resulting from a membranous web of the inferior vena cava: operative repair using profound hypothermia and circulatory arrest. Ann Thorac Surg 1987;43:212-4. 3. ShaffnerF, GadboysHL, SaframAP, Baron MG, AnfsesAH. Budd-Chiari syndrome caused by a web in the inferior vena cava. Am J Med 1967;42:838-42. 4. McLaughlinCW Jr, Popma AM. Intermittent obstruction of subclavian vein. JAMA 1960;113:1939-45.
B O U N D VOLUMES AVAILABLE TO SUBSCRIBERS
Bound volumes of the JOUKNALOF VASCULARSURGERYfor 1989 are available to subscribers only. They may be purchased from the publisher at a cost of $52.00 ($66.00 international) for Vol. 9 (January to June) and Vol. 10 (July to December). Price includes shipping charges. Each bound volume contains a subject and author index, and all advertising is removed. Copies are shipped within 60 days after publication of the last issue in the volume. The binding is durable buckram with the journal name, volume number, and year stamped in gold on the spine. Payment must accompany all orders. Contact Circulation Fulfillment, The C.V. Mosby Company, 11830 Westline Industrial Drive, St. Louis, MO 63146-3318, USA. In the United States call toll free: (800)325-4177, ext. 351. In Missouri call collect: (314)872-8370, ext. 351. Subscriptions must bc in force to qualify. Bound volumes are not available in place of a regular JOURNALsubscription.