Case. Variations in lower limb venous anatomy are common. 1 INVESTIGATION AND TREATMENT OPTIONS IN ACQUIRED DEEP VENOUS HYPOPLASIA - A CASE REPORT

Transcription

1 pp14-19 Case P R E S E N T A T I O N INVESTIGATION AND TREATMENT OPTIONS IN ACQUIRED DEEP VENOUS HYPOPLASIA - A CASE REPORT DR JACQUELINE CHIRGWIN MB BS (Hons) Phlebologist, Newcastle Vein Clinic, Newcastle, NSW, Australia Introduction Variations in lower limb venous anatomy are common. 1 However, whilst variations in the superficial venous anatomy are usually recognised, the possibility of such variations in the deep venous anatomy can be overlooked. This case study highlights the need for adequate investigation of both the superficial and deep venous systems. Regarding treatment, there is no consensus as to the single best approach to the treatment of varicose veins. 2 This case study also highlights the need for the phlebologist to consider all available treatment options before deciding on a course of action. Case Presentation The patient is a twenty-year-old male who presented with a history of varicosities involving the left calf for five years. (Figure 1) These were associated with telangiectasia over the medial malleolus. The patient had experienced bleeding from the telangiectasia on a weekly basis for the three months prior to presentation. His poor skin condition in this area had been contributed to by repeated minor trauma from hockey and rollerblading. He had suffered from nonvaricose eczema over the lateral aspect of the left foot and in other areas of the body, but all had reduced in severity over the past 18 months. His occupation is as a chef. On examination, there were varicosities and telangiectasia involving the left medial malleolus, together with hemosiderin deposits. (Figure 2) There was no evidence of ABSTRACT Case presentation of acquired deep venous hypoplasia in a 19 year-old male following osteomyelitis, fracture of the femur and subsequent femoral osteotomy and osteosynthesis aged between 2 and 4 years. Variations in lower limb venous anatomy are common 1 and this case study highlights the need for adequate investigation of both the superficial and deep venous systems. It also demonstrates the need for careful history taking and investigation of every patient. The practical application of the modified Perthes test is demonstrated. This test can be applied simply and effectively in an office setting and provides important information regarding the function of the deep venous system in the presence of gross abnormalities of that system. In the past, it has generally been accepted that the removal of varicose veins in patients with absent or hypoplastic deep veins was contraindicated. In this paper a range of treatment options is considered, including both sclerotherapy and surgery. Key Words: Common femoral vein, ambulatory phlebectomy, ambulatory venous pressure, bleeding telangectasia, chronic venous hypertension, acquired deep venous hypoplasia, deep venous system function, duplex ultrasound scanning, femoral osteotomy, modified Perthes test, osteomyelitis, pathological fractures femur, perforator, phlebography, photopletysmography, sclerotherapy, subfascial collaterals, surgery, thrombophilia screen. lipodermatosclerosis and in particular, there was no swelling of the left lower limb. There were two vertical scars, extending over two-thirds of the anteromedial aspect of the thigh (Figure 3) and over a similar distance on the lateral aspect. (Figure 4) The patient believed these were related to an operation on his femur at the age of four during which a plate had been inserted. He was unclear on any further details relating to this procedure at the initial consultation. There was no past history of varicose vein surgery, sclerotherapy, deep venous thrombosis, superficial thrombophlebitis or ulceration. He denied any family history of thrombosis. Address Correspondence to: Dr Jacqueline Chirgwin P.O. Box 429 Newcastle, 2300 NSW AUSTRALIA. Telephone: : Facsimile: jacqui_tom@hunterlink.net.au 14 V OLUME 8(1):DECEMBER 2004 AUSTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY

2 Acquired deep venous hypoplasia - a case report Figure 1: Left medial calf varicosities. Figure 3: Anteromedial thigh scar. Figure 4: Lateral thigh scar. Figure 2: Haemosiderin deposits and recently healed bleeding site. Figure 5: Anterior duplex venous map. Figure 6: Posterior duplex venous map. A USTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY V OLUME 8(1):DECEMBER

3 J Chirgwin Figure 7: Duplex ultrasound showing small bifid femoral vein in mid-thigh. Figure 8: Duplex ultrasound showing 2mm diameter femoral vein in mid-thigh. Investigations Colour Duplex Ultrasound Left Leg The initial investigation was a Colour Duplex Ultrasound of the left lower limb. (Figures 5 & 6) This revealed a grossly abnormal deep venous system in addition to the superficial varicosities. The deep venous findings can be summarised as follows: The external iliac vein was patent but displayed low venous flow. An absent common femoral vein. The superficial femoral vein appeared bifid and very small in calibre throughout the thigh, but displayed normal flow. (Figures 7 & 8) The popliteal, posterior tibial and peroneal veins were normal. There was a small competent communication from the proximal great saphenous vein to the proximal superficial femoral vein. Interestingly, it was noted by the ultrasonographer that the deep vein walls did not appear to be thickened, and the venous channels did not appear to be tortuous. Figure 9: Modified Perthe s test using blood pressure cuff below knee. Superficially, there was an incompetent vein from within the posterior thigh muscle that communicated with the Giacomini vein, which displayed bi-directional flow in the posterior thigh. The Giacomini vein communicated with the proximal great saphenous vein, which then displayed reflux for a short distance in the thigh. The great saphenous vein then gave rise to an incompetent vein travelling down the medial aspect of the leg, with branches over the anteromedial thigh, proximal antero-medial calf and posterior calf. There was a 5mm incompetent perforator in the region of the medial knee. The ultrasonographer did not note any increased flow through the great saphenous system. 16 V OLUME 8(1):DECEMBER 2004 AUSTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY

4 Acquired deep venous hypoplasia - a case report In light of these abnormal findings, a second Duplex scan was performed to allow the ultrasound technician more time to assess in particular, the major source of the deep venous outflow in the limb. In addition to the above findings, the second scan revealed a competent continuation of the great saphenous vein above the groin and onto the supra-pubic area. The perforator at the medial knee displayed flow out of the deep venous system. However, as a result of the bony landmarks in this area, the actual communication with the deep veins could not be localized. The popliteal vein communicated with a competent vein coursing proximal through the posterior thigh muscle and this could be followed as far as the buttock. Subsequently, a vascular physician performed a third duplex scan. Dr Mark Malouf of Sydney, to whom the patient was referred, arranged this scan. He considered the deep venous outflow was still unclear despite the first two ultrasounds. The findings of this scan confirmed those of the previous two ultrasound examinations and again noted a large vein running from the left great saphenous vein, proximally across the symphysis pubis, to link with the saphenofemoral junction on the other side. Pathology A thrombophilia screen was performed with the results as follows: FBC - Normal Protein S and C Normal Prothrombin Gene Mutation Not detected Anti-thrombin III High (low levels associated with thrombosis) Lupus Inhibitor No evidence Cardiolipin Antibodies Normal (The last two tests act as screens for Anti-Phospholipid Syndrome) Homocysteine Normal Factor V Leiden Not detected Past Medical Records In view of the patient s age at the time of the femoral plating, with his permission medical records were obtained from the Royal Newcastle Hospital, his former GP and his parents. It transpired that at the age of 2 1/2 he had suffered from acute haematogenous osteomyelitis of the left distal femur whilst living in Wales. The records surrounding this episode were not available. However, soon after his arrival in Australia, he suffered a pathological fracture of the distal femur and biopsies of the femur were taken. He ultimately experienced a mal-union of the femoral fracture that was corrected with a femoral osteotomy and osteosynthesis one year later. There was no documentation of a deep venous thrombosis at any stage of his treatment. Modified Perthes Test Perthe s test traditionally involves using a rubber strip tourniquet to establish whether the subfascial collaterals are functioning well. A modification of this test has been used in a study in the detection of the development of subfascial collaterals in post-thrombotic deep-vein occlusion cases. 3 It has also been used in a recently published study by Bihari et al 4 to establish whether patients with deep vein aplasia or hypoplasia were suitable for treatment of their superficial varicosities. A tensiometer or blood pressure cuff is placed on the limb just below or just above the knee. The cuff is inflated to 110 mm Hg, and the patients are asked to walk quickly for 5 minutes. The test is considered positive when the limb becomes livid and the patient complains of heavy pain within 1 to 2 minutes. In negative cases, when collateral channels in the subfascial space are sufficient in number and diameter to drain the venous blood from the leg, the patient s leg is unaffected. Despite three duplex venous scans, the functional outflow of the deep venous system was still unclear. The patient was therefore recalled and this modified Perthe s test was performed. (Figure 9) The cuff was placed both above and below the knee in an attempt to isolate the function of the 5mm perforator found at the knee on duplex scanning. Initially, the patient experienced some venous engorgement and pain in the left foot and calf when the cuff was positioned below the knee. Repeating the test on a further two occasions produced negative results. That is, the patient noticed no adverse effects from the cuff. At no stage did positioning the cuff above the knee result in any engorgement or pain. Proposed Treatment This patient is young, with long-standing varicosities, early venous hypertensive changes and has suffered from numerous episodes of bleeding. His occupation as a chef involves long hours of standing. It was therefore felt an active rather than a passive approach to his varicosities was warranted. The patient was ultimately referred to Dr Mark Malouf, Sydney, for consideration for ambulatory phlebectomy. Following further discussion, it was decided that the patient would benefit from avulsion of the incompetent perforator at the knee and varicosity over the medial calf. It is planned this procedure will be performed in hospital on a short stay basis. A USTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY V OLUME 8(1):DECEMBER

5 J Chirgwin In addition to the active treatment of the lower limb varicosities, with the patient s permission a conference was held with the patient, his parents and girlfriend. In view of his age and the nursing background of his mother, it was agreed that the likelihood of any inadvertent complications of future treatment to his venous system could be reduced if more of his family were informed of his condition. Discussion In the assessment and treatment of any patient in the field of medicine, the underlying mantra is always to do no harm. This stresses the importance of the treating practitioner fully assessing the presenting complaint and making an informed decision regarding not only the efficacy of any proposed treatment, but its subsequent impact on the patient. The patient presented here has gross abnormalities of his deep venous system, which were most likely acquired as a result of the surgical interventions to his left femur in his early years. It is unclear whether the patient suffered from a single or multiple episodes of deep venous thrombosis or whether the patient suffered direct damage to the deep veins either as a result of the pathological fracture or subsequent operative intervention. There was certainly no documentation in the medical records of a thrombotic episode at the time, nor was there any familial history of thrombosis or abnormalities on the patient s thrombophilia screen. In the past, studies have suggested the removal of varicose veins in patients with absent or hypoplastic deep veins was contraindicated. 5-8 However, the agenesia or hypoplasia of a shorter or longer segment of deep veins is not a contraindication to radical varicectomy in every patient. 4 According to Comerota, 9 obstruction should be viewed in a linear sense (as a spectrum) rather than all or none. Studies have shown that in some cases of post thrombotic deep venous occlusion, radical varicectomy did not result in any intraoperative or postoperative circulatory disturbance. 3, 10 The difficulty in cases of deep venous aplasia, hypoplasia or obstruction, has been to distinguish between those patients whose overall venous function will be compromised by treatment of any superficial varicosities, and those who will obtain some benefit. In addition to the traditional distinctions of deep and superficial veins, it is believed there is a system of venous channels called subfascial collaterals, which lie in and between the muscles of the lower limb, and which dilate after an occlusion or in the absence of deep veins. As stated by the Phlebologist Robert Linton: While working in this field (in phlebology for 40 years), it has become obvious to me that the great veins of the lower part of the body and the extremities are not absolutely necessary as conduits for the return of blood to the heart, because there are innumerable smaller calibre collaterals that actually suffice and gradually increase in calibre. 11 With time, these collaterals can alone maintain the venous drainage of the limb. 4 Neither phlebography 4 nor venous duplex scan can give useful information about the function of the subfascial collaterals. Raju concluded the anatomy of the venous system could not be the sole basis for therapeutic decisions and that it was the hemodynamic result rather than the anatomic site and extension of obstruction in post-thrombotic limbs that determines the outcome. Ambulatory venous pressure measurements and photopletysmography are useful in venous reflux disease but are not helpful in venous obstruction. 4 Bihari et al 4 have suggested a modification of Perthe s test to assess the function of the subfascial collaterals. This modified test is more readily standardized and is based on sound physiological principles. Their cuff pressure test is calculated to be optimal at 110 mm Hg, as the subfascial veins can develop a pressure greater than 200 to 300 mm Hg 13,14 during walking, but in the muscular compartments, the pressure is even higher. 15 Thus the cuff pressure is high enough to compress the superficial varices but not higher than a walking patient's arterial blood pressure in the lower limb. This test therefore provides the treating practitioner with a simple, non-invasive method of assessing the possible outcome of any proposed treatment to the superficial venous system. It can be performed in an office setting with equipment that is readily available. Having determined the likely outcome of treatment to the superficial venous system, the practitioner will then need to decide on the most appropriate type of treatment. In general, active treatments can be divided into two broad categories, being surgery and sclerotherapy. Surgery can be further divided into ambulatory phlebectomy, and short or long stay hospital based surgery. Ultimately, the goal of any varicose vein surgery is to remove reflux and visible varicose veins with the aim to achieve the most favorable hemodynamic and cosmetic results. 17 There has been a trend toward less invasive procedures to reduce the number of incisions and provide more selective ablation of varicosities. 2 In the situation where the patient is relying on subfascial collaterals for deep venous drainage, it has been stated that operations on the superficial veins can be performed if these pathways are functioning well V OLUME 8(1):DECEMBER 2004 AUSTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY

6 Acquired deep venous hypoplasia - a case report The procedure of ambulatory phlebectomy, as described by Muller, is a remarkable esthetic, effective, and costsparing technique for definitive removal of varicose veins. 18 Sites particularly appropriate for ambulatory phlebectomy include incompetent saphenous veins, their major tributaries, perforating, groin pudendal veins, reticular veins and veins of the ankles and the dorsal venous network of the foot. 19 It effectively removes all varicosities, eliminates the proximal source of reflux, and disconnects potentially outflowing perforators, yet leaves in situ undamaged trunk veins. 20 On reviewing the literature, there is very little in the way of evidence-based medicine to support the safety and efficacy of sclerotherapy in the presence of absent or hypoplastic deep veins. In the case of this patient, the presence of the perforator at the knee was of concern. It measured 5 mm in diameter and its path could not be traced on duplex scanning because of the bony prominences. A venogram was considered but it was felt that while it would provide anatomical data, it would not provide any further information regarding venous function. Additionally, it is an invasive procedure with the associated complications. Sclerotherapy is an extremely safe and effective procedure in the majority of cases. However, in this particular case, any inadvertent sclerosis of the subfascial collaterals or the channel travelling from the popliteal vein into the buttock may have had a severe impact on venous function. For this reason, it was decided the patient should undergo the procedure outlined above. In conclusion, this case demonstrates the practical application of the modified Perthes test, which can be applied simply and effectively in an office setting. This test provides important information regarding the function of the deep venous system in the presence of gross abnormalities of that system. This case also demonstrates the need for careful history taking and investigation of every patient. The patient presented here is young, with obvious varicosities but little on examination to suggest the extent of the deep venous abnormalities. It is the opinion of the author that a comprehensive duplex ultrasound of both the deep and superficial venous systems is the minimum requirement in the assessment of any patient presenting with varicosities, to determine the anatomy and any other features that may impact on treatment. Additionally, practitioners should be prepared to repeat the Duplex Ultrasound if necessary, to allow adequate time for a thorough examination and documentation of venous anomalies. Finally, there is a range of options available for the treatment of varicose veins. As Phlebologists, it is important to maintain a working knowledge of these procedures, and consider which is the most appropriate treatment for each patient. Editor s comment: An acceptable alternative treatment in this case is foam echosclerotherapy. References 1. Quinlan DJ. Alikhan R. Gishen P. Sidhu PS. Variations in lower limb venous anatomy: implications for US diagnosis of deep vein thrombosis. Radiology 2003; 228(2): Brethauer SA. Murray JD. Hatter DG. Reeves TR. Hemp JR. Bergan JJ. Treatment of varicose veins: proximal saphenofemoral ligation comparing adjunctive varicose phlebectomy with sclerotherapy at a military medical center. Vascular Surgery 2001; 35(1): Bihari I. Can varicectomy be performed if deep veins are occluded? J Dermatol Surg Oncol 1990; 16: Bihari I. Tasnadi G. Bihari P. Importance of subfascial collaterals in deep-vein malformations. Dermatologic Surgery 2003; 29(2): Vollmar J, Voss E. Vena marginalis lateralis presistens: die vergessene vene der angiologen. Vasa 1979; 8: Eifert S, Villavicencio L, Kao T-C, et al. Prevalence of deep venous anomalies on congenital vascular malformations of venous predominance. J Vasc Surg 2000; 31: Gorenstein A, Shifrin E, Gordon RL, et al. Congenital aplasia of the deep veins of lower extremities in children: the role of ascending functional phlebography. Surgery 1986; 99: Schobinger RA, Nachbur B, Senn A. The syndrome of Klippel-Trenaunay, a polyvalent angiodysplasia. J Cardiovasc Surg 1987; 28: Comerota AJ. Myths, mystique, and misconceptions of venous disease. J Vasc Surg 2001; 34: Raju S, Easterwood L, Fountain T, et al. Saphenectomy in the presence of chronic venous obstruction. Surgery 1998; 123: Linton RR. John Homan's impact on diseases of the veins of the lower extremity, with special reference to deep thrombophlebitis and the postthrombotic syndrome with ulceration. Surgery 1977; 81: Raju S. New approaches to the diagnosis and treatment of venous obstruction. J Vasc Surg 1986; 4: Browse NL, Burnand KG, Irvine AT, Wilson NM. Diseases of the Veins, 2nd ed. London, Sydney, Auckland: Arnold, Sumner DS. Hemodynamics and pathophysiology of venous disease. In: Rutherford RB, ed. Vascular Surgery. Philadelphia, London, Toronto, Mexico City, Rio de Janeiro, Sydney, Tokyo: W.B. Saunders, 1984: Alimi YS, Barthelemy P, Juhan C. Venous pump of the calf: a study of venous and muscular pressures. J Vasc Surg 1994; 20: Bihari I, Tasnádi G, Bohár L, et al. Varicectomy in deep vein aplasia. Phlebol Suppl 1995; 1: Recek C. [Principles of surgical treatment of varicose veins with regard to new findings on venous hemodynamics]. [Czech] Rozhledy V Chirurgii. 2002; 81(9): Ramelet AA. Complications of ambulatory phlebectomy. [Review] [43 refs] Dermatologic Surgery. 1997; 23(10): Ramelet AA. Phlebectomy. Technique, indications and complications. [Review] [29 refs] International Angiology 2002; 21(2 Suppl 1): Goren G. Yellin AE. Ambulatory stab evulsion phlebectomy for truncal varicose veins. American Journal of Surgery 1991; 162(2): A USTRALIAN & NEW Z EALAND J OURNAL OF P HLEBOLOGY V OLUME 8(1):DECEMBER