Background. Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of arteriovenous or capillary vascular malformations, atypical varicosities, and bony or soft tissue hypertrophy usually affecting one extremity. 

Material and Methods. We reviewed the clinical characteristics and outcome of treatment IN 252 patients (116 male and 136 female) with KTS evaluated at the Mayo Clinic between January 1956 and January 1995. 

Results. Arteriovenous or capillary vascular malformations (port-wine stains) were found in 246 patients (98 percent), varicosities in 182 (72 percent), and limb hypertrophy in 170 (67 percent). All three features of KTS occurred in 159 patients (63 percent), and 93 (37 percent) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72 percent). Abnormalities of the deep veins included aneurysmal dilation, hypoplasia, aplasia, and absent or incompetent valves. Operations done for 145 patients included epiphyseodesis, stripping of varicose veins, excision of vascular malformations, amputations, and debulking procedures. 

Conclusions. Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2 cm at skeletal maturity. This can be treated with epiphyseodesis in the growing child. Symptomatic varicosities or localized superficial venous malformations can be removed in selected patients with good results provided there is a functioning deep vein system. 

Key Words. Hemangioma, hypertrophy, port-wine stain, birthmark, varicose veins 

In 1900, the French physicians Klippel and Trenaunay described a syndrome "noevus variqueux osteohypertrophiques" characterized by three features: (1) a cutaneous vascular nevus of the affected extremity (capillary malformation), (2) soft tissue and bony hypertrophy of the extremity, and (3) varicose veins.1 The syndrome currently is defined as the combination of (1) capillary malformations (usually port wine stains) which frequently are located laterally, need not extend over the entire affected limb, and may be found at sites other than the hypertrophied limb, (2) soft tissue or bony hypertrophy, or both and (3) varicose veins, often with persistent lateral embryologic veins.2,3,4,5 However, the diagnosis can be made with any two of the three features. In addition, there frequently are associated deep venous anomalies (hypoplasia, aplasia, venous incompetence) as well lymphatic anomalies. As emphasized by Young, the vascular malformation in KTS is a combined capillary/venous/lymphatic malformation without evidence of any significant arteriovenous shunting.6 

In recent years, newer diagnostic techniques and treatment options have added to our knowledge of this syndrome and its management.2,5,7,8,9,10,11,12 In this article we review the records of 252 patients with Klippel-Trenaunay syndrome (KTS) evaluated at the Mayo Clinic. 

All patients with KTS evaluated at the Mayo Clinic between January 1956 and January 1995 were included in the study. Serial scanograms (radiographs of the long bones), when indicated, were obtained to assess discrepancies in limb length. Noninvasive examinations included arterial and venous Doppler studies, impedance plethysmography, duplex scanning, and magnetic resonance imaging . Detailed evaluation of the gastrointestinal, genitourinary, and other organ systems was done if symptoms indicated involvement of these organs. Venograms were obtained when surgical treatment of varicosities or venous malformations was considered. 

Study Population  

The study group included 116 male (46 percent) and 136 female (54 percent) patients. The age at diagnosis ranged from birth to 83.4 years, and the median age was 11.9 years. The patient or family first noted evidence of the syndrome between birth and 9.1 years. Two hundred thirty patients (91 percent) had evidence of the syndrome at birth. 

Most of the patients were white. One was black, two were Hispanic, and two were of Middle Eastern origin. The mean follow-up period was 3.58 years, and the range was between 3 days and 32.8 years 

Clinical Features  

One hundred fifty-nine patients (63 percent) had all three features of KTS, and 93 (37 percent) had two of the three features. None of the patients had clinical evidence of an arteriovenous fistula. There was no difference between male and female patients in the occurrence of capillary malformations, limb hypertrophy, and varicosities. The characteristic findings in the different age groups during initial evaluation at the Mayo Clinic are shown in Figure 1. It is evident that varicosities become more apparent with increasing age. Typical features are illustrated in Figures 2 through 5. 

None of the patients had a family history of KTS, but "hemangiomas" were reported in family members of eight patients. These consisted of capillary malformation in the families of six patients (with spontaneous resolution in three of the family members), telangiectasia in the mother of one patient, and a large cavernous arteriovenous malformation in the father of another patient. True hemangiomas of the lower limb developed in the mothers of two patients in the first week after delivery of the affected offspring. 

Concomitant congenital anomalies were found in 74 patients (29 percent). The most common were developmental dysplasia of the hip (10 patients) and syndactyly (10 patients). Other anomalies that occurred in these patients are listed in Table 1. One patient had coexisting Sturge- Weber syndrome, and another patient had neurofibromatosis. Two patients had atresia of the major veins of the head and neck. In one, a 9-year-old boy with Sturge-Weber syndrome, the right internal jugular vein and superior vena cava were absent. In the other, a 6-year-old boy, the internal jugular vein and sigmoid sinus on the affected side were absent, and he had macrocephaly and an abnormally shaped skull with thin, dysplastic diploic spaces. 

The sites of involvement of the disease are presented in Table 2. Only the lower extremity was involved in 177 patients (70 percent), and 28 (11 percent) had only upper extremity involvement. Two patients had the crossed dissociated form. Eighteen patients (7 percent) had a shorter affected limb, and in six of them, the limb also was of smaller girth. 

Additional features or symptoms are listed in Tables 3 and 4. Of particular importance, 11 (4%) patients had deep vein thrombosis and 9 (4%) patients had pulmonary embolism, which was fatal in 1 patient. The factors temporally associated with pulmonary embolism were pelvic surgery, oral contraceptive therapy, pregnancy, sclerotherapy, and invasive studies. 

Investigations 

Scanograms were obtained in 105 patients (42 percent). Limb length discrepancy varied widely, with a range between 0.1 and 9.6 cm (mean, 1.75 cm). Rate of progression of the length discrepancy was neither uniform nor predictable. Many discrepancies remained static for years. Other discrepancies increased by as much as 0.6 cm per month. 

Noninvasive arterial and venous studies were done in 24 (10 percent) and 48 (19 percent) patients, respectively. Results of the arterial studies were normal in all the patients, with no evidence of arteriovenous fistulae. Of the 48 patients who had noninvasive venous studies, 4 had evidence of deep vein obstruction and 28 had evidence of deep vein incompetence. 

Preoperative venography was done in 45 patients (18 percent). The venograms of 30 patients were available for review, and the results are described in Tables 5 and 6. Results of magnetic resonance imaging studies performed at the Mayo Clinic in 54 patients were reviewed. There was evidence of hypertrophy of the limb in 37 patients, with subcutaneous fatty hypertrophy in 34 and "enlargement" of the muscles in 15. Eight patients had evidence of smaller muscle mass in the affected extremity than in the normal limb. Varicosities/venous malformations were observed in 49 patients, 37 of whom had infiltration of the muscles by the venous malformation. Two patients had venous malformation involving the bone. Of the 47 patients who had imaging of the pelvic structures, 34 had evidence of pelvic involvement with varicosities/venous malformations. Twenty-nine patients had a distinct lateral leg vein, 22 had a persistent sciatic vein, and 14 had suprapubic varicosities. 

Management 

Fifty-four patients (21 percent) had no treatment. The rest had nonoperative or surgical treatment or a combination of both. 

Nonoperative Therapy. One hundred sixty-six patients (66 percent) required some form of medical treatment. Fifty- six used elastic support of the limb or heel lift alone. Thirty-eight were treated with antibiotics for recurrent cellulitis. Eleven patients, including those who had rectal bleeding or hematuria, had iron replacement for anemia. In some of these patients, recurrent bleeding was severe enough to require multiple blood transfusions, and four patients ultimately had resection of portions of the rectosigmoid colon.11 Five patients received steroid treatment. Two patients were treated elsewhere with radiation to induce regression of venous malformation, and results were poor. Five patients with significant edema of the lower limbs were treated with diuretics. Other drugs used were anticoagulants for patients with deep vein thrombosis or pulmonary embolism and aspirin for patients with recurrent superficial vein thrombophlebitis. 

Surgical Therapy. One hundred forty-five patients had some form of surgical treatment. The types of operations and the results are described in Figure 6. In general, operations on varicose veins initially reduced symptoms, but symptoms tended to recur later. Procedures not included in Figure 6 were laser treatment of cutaneous capillary malformations in 20 patients, amputation of digits or the involved limb in 19, evacuation of hematomas in 7, removal of cystic hygromas or angiolipomas in 7, excision of fibromas in 2, and deep venous reconstruction in 2. 

Some patients did poorly after surgical procedures. In one patient who had a debulking procedure of the arm at another institution, massive lymphedema and a nonhealing wound developed postoperatively. One patient had epiphysiodesis done elsewhere prematurely, resulting in overgrowth of the normal leg. Another patient had increased symptoms after stripping of varicose veins at another institution. This patient had an atretic superficial femoral vein on the operated side, and his symptoms worsened after the superficial varicosities were removed. Use of the contralateral saphenous vein to reconstruct his deep veins resulted in improvement2. 

Mortality 

There were three deaths (1 percent). One patient had extensive involvement of the trunk with venous malformations. Severe edema, enlarging abdominal and thoracic venous malformations, and progressive cachexia developed, and she died at the age of 11. Another patient died of pulmonary embolism at age 21. The third patient died at age 10 with necrotizing pneumonia, glomerulonephritis, and an unspecified immunodeficient state. 

Etiology 

The cause of KTS remains obscure. Grouping this syndrome with inherited phakomatosis13 is no longer viewed as appropriate. Bliznak and Staple14 suggested intrauterine injury to the sympathetic ganglia or intermediolateral tract resulting in dilation of microscopic arteriovenous anastomoses as the underlying cause. Servelle et al.,3 on the basis of animal experiments, proposed that deep vein abnormalities resulting in obstruction to normal venous flow lead to venous hypertension, development of varices, and hypertrophy of the limb. Baskerville et al.15 concluded that KTS is caused by a mesodermal defect leading to maintenance of microscopic arteriovenous communications. McGrory and Amadio,8 on the basis of abnormal epidermis in one patient, proposed that this syndrome may result from a mixed mesodermal and ectodermal dysplasia. We believe that KTS is a sporadic mesenchymal abnormality in the development of the limb and that the soft tissue and bony abnormalities are not hemodynamic consequences of the venous abnormalities. 

Genetics 

KTS reportedly has occurred in more than one family member,4,16 but we have not observed this. It has been suggested that the syndrome could result from a lethal gene that survives by mosaicism.17 We have observed interesting genetic associations in two patients. One patient had a coexisting prolonged QT syndrome, now localized to defects of chromosomes 3,7 and 11.18 The other patient had a balanced translocation of chromosomes 8 and 14 (8;14)(q22.3 q 13). Whether these associations are coincidental or provide a clue to the chromosomal location of a genetic defect as the underlying cause of this syndrome is unknown. 

Signs and Symptoms 

The capillary malformation (port-wine stain) is the most common cutaneous manifestation of KTS.19,20,21,22 Histologically, these are vascular malformations with ectatic capillaries in the upper dermis. Because they are nonproliferative, they never regress, although the appearance and the intensity of the color may change with age. Some, however, may be complicated by skin breakdown, bleeding, and infection. Nodular lesions, which are believed to be ectatic venous or lymphatic channels may develop in some patients.8 We have observed these on parts of the body that are otherwise effected and uneffected with KTS. 

Varicose veins may not always be evident at birth. They become more obvious, as demonstrated in this study, as the child becomes ambulatory. Aneurysmal dilation of deep and subcutaneous veins in patients with KTS is frequent and may result from either congenital weakness of the walls or a progressive high pressure in the venous system during ambulation. The occurrence of lateral varicosities in the absence of saphenous vein abnormalities suggests that intrinsic weakness of the walls of the persistent, frequently avalvular, embryonic veins rather than a high-pressure state may be the underlying cause. 

Abnormalities can occur in the superficial and the deep venous systems and in the perforating veins of the limbs.2,3,22,23,24 Abnormalities of the superficial veins range from ectasia of small veins and varicosities to persisting embryologic veins and large venous malformations.23 The persisting embryologic veins include the lateral vein of the thigh and the sciatic vein.3 These embryologic veins frequently are large, tortuous, and dysplastic and may not contain valves. They frequently produce symptoms of fatigue and heaviness of the legs. Significant venous reflux is encountered, particularly in patients with long embryonic trunks and in those with abnormal perforating veins.25 Deep vein anomalies include aneurysmal dilation, duplication, hypoplasia and aplasia, and external compression by fibrous bands or anomalous vessels.2,3,15,19,23,24,26 The popliteal and superficial femoral veins are most commonly affected, although all the veins of the limbs, including the lower inferior vena cava, can be affected.3 The degree of venous hypertension in patients with outflow obstruction depends on the existent collaterals. The four major types of collaterals described in patients with aplasia of the deep thigh veins are the lateral veins (which can have connections with the deep veins of the lower limb at different levels),2,3 the sciatic vein (draining into the internal iliac veins), the retroadductor vein (draining into the deep femoral vein), and the greater saphenous vein (draining into the common femoral vein).3,27 The receiving veins usually are dilated. Varicose veins around the rectum and bladder are believed to be due to high flow in the internal iliac vein.28 Venous malformations in the rectal and bladder walls develop in some of these patients. Large suprapubic veins may be a sign of atresia of the iliac vein. In our series, 9 out of the 46 patients with pelvic involvement (20 percent) had no evidence of significant varicosities in the affected lower limb. 

Hypertrophy is the most variable of the three cardinal features of KTS. In our experience, all the patients who had evidence of the disease at birth had noticeable hypertrophy of the extremity. There is no predictable rate at which the hypertrophy progresses, but major changes after birth are unusual. Edema of the extremities may result from coexisting lymphedema secondary to lymphatic abnormalities.3 Recurrent cellulitis may be associated with lymphedema. 

Recently, Samuel and Spitz29 reported their experience with 47 patients with KTS. Varicosities developed in 79 percent of their patients, and we observed these in 72 percent of our patients. Thrombophlebitis occurred in 45 percent of their patients and in 19 percent of ours. In their series, no patient had evidence of macrofistulous arteriovenous communications but, interestingly, 13 percent had high-output congestive heart failure. We have not observed congestive heart failure in a patient with KTS. Congestive heart failure should suggest the diagnosis of Parkes-Weber syndrome with significant arteriovenous shunting6 rather than KTS. 

Evaluation 

In children with limb length discrepancies, serial scanograms, orthoroentgenograms, computed tomography, or other standardized radiographs for measurement of leg length are necessary for proper timing of leg length equalization procedures. Such radiographs rarely are necessary before 3 years of age. The leg length discrepancy does not progress after physeal closure at skeletal maturity. 

Careful evaluation by duplex scanning, contrast venography to assure normal anatomy of the deep vein system is very important before removal of superficial dilated venous channels. Ascending and descending venography is useful to determine the anatomy of the deep veins, including valves, embryologic veins and their connections to the deep vein system, and the degree of venous incompetence. 

Treatment 

Nonoperative. Nonoperative management includes external compression with graduated compression stockings and garments. Indications are chronic venous insufficiency, lymphedema, recurrent cellulitis, and recurrent bleeding from capillary or venous malformations of the extremity. Compression therapy can be very helpful to protect the limb, even from minimal trauma that can cause bleeding of the large superficial malformations. Compression therapy helps diminish symptoms of venous insufficiency and lymphedema. It has no effect, however, on the ultimate size of the limb. Patients with lymphedema may benefit from regular use of intermittent pneumatic compression pumps. Compressive devices usually are not recommended in very young children because they do not tolerate these garments and rapidly outgrow them. 

Patients with recurrent attacks of cellulitis may benefit from prophylactic antibiotic therapy. Other forms of medical treatment include anticoagulants after deep vein thrombosis or pulmonary embolus. Patients with recurrent superficial thrombophlebitis frequently require daily administration of aspirin or ibuprofen. However, this may promote bleeding problems. 

Oral contraceptives should be avoided by patients with significant venous abnormalities because of the risk of deep venous thrombosis and pulmonary embolus. Patients should be warned of exacerbation of symptoms during pregnancy, either increased lower extremity swelling and varicosities or increased symptoms of venous insufficiency, as in several of our patients. There may be periuterine varicosities, which can complicate pregnancy, delivery, and caesarian section.21 

Surgical. In general, operation should not be done to improve cosmesis at the expense of function. An obvious indication for operation is a leg length discrepancy of more than 2 cm. In such cases, epiphysiodesis often is recommended to stop the growth of the abnormally long extremity or digit. The timing of epiphysiodesis is crucial so that the affected and the unaffected extremities attain a similar length at skeletal maturity. Minor leg length discrepancies (less than 2 cm) can be managed with a lift in the contralateral shoe, if symptoms warrant.27 Digits with severe deformity, poor skin coverage, or chronic infection may need to be amputated. Ray resection of a foot with or without debulking of the foot to improve gait characteristics is sometimes indicated. Rarely, amputation of a limb may be necessary to improve function. 

Removal of varicose veins to relieve discomfort or, rarely, to improve appearance should be performed for selected patients only. It is critical, however, to confirm that the patient has a patent deep vein system before superficial veins are removed.2,7,15 Recurrence of at least some varicosities is frequent. 

Laser therapy is useful in some patients for decreasing the intensity of the color of capillary malformations, but it must be kept in mind that multiple treatments may be required and that the procedure is not always successful. 

Excision of venous or lymphatic malformations is technically challenging and in some cases can lead to severe blood loss intraoperatively. The potential benefits of excision, such as reduction in bulk and correction of severe deformity of cartilaginous or bony structures, should be weighed carefully against the potential problems of pain, recurrent infections, and bleeding. It should be recognized that complete excision of extensive malformations with debulking procedures seldom is possible. 

Debulking procedures have limited usefulness and should be considered only when the excess bulk severely limits function. Debulking procedures can damage venous and lymphatic structures and lead to increased edema of the affected part. Other problems associated with debulking procedures are scar formation, recurrence, chronic wound infection, and chronic weeping lymphedema. 

Recommendations 

Management in most patients with KTS should be nonoperative. Indication for epiphysiodesis is a leg length discrepancy exceeding 2 cm in the growing child. Amputation of a grossly hypertrophied, poorly functioning digit may be necessary but a more proximal foot, hand, or limb amputation is rarely required. Symptomatic varicosities or localized venous malformations can be removed in selected patients with good results provided that there is a functioning deep vein system. 

Poor wound healing 

Skin atrophy 

Orthostatic hypotension 

Varicocele 
Urethral or vaginal discomfort secondary to vascular malformations and tissue hypertrophy 

Anemia secondary to gastrointestinal or genitourinary bleeding 

Hemimegalencephaly 

Chronic diarrhea 

Pulmonary atelectasis secondary to mass effect 

Spontaneous rupture of the thoracic duct 

Osteomyelitis 

Truncal soft tissue masses 

Absent pectoralis major muscle 

Absent breast development 

Vascular malformations of bones predisposing to fractures 

Visceral vascular malformations involving 

Liver 

Kidney 

Bladder 

Rectum and lower gastrointestinal tract 

Retroperitoneum 

Pericardium 

Spine 

Lung 

Seizures 

Developmental delay 

Intravascular coagulopathy 

* includes ascending and descending venography 

Fig. 1. Relative number of patients with the principal features of KTS stratified by age. 

Fig. 2. The most common distribution of the features of KTS. 

Fig. 3. KTS involving both lower and right upper extremities. Note abnormal feet and toes. 

Fig. 4. KTS involving the right arm and trunk. Arrow indicates a subcutaneous mass. 

Fig. 5. Young adult with KTS of the leg. Note the varicosities and the verrucous lesions. 

Fig. 6. Outcome of surgical procedures. 

1. Klippel M, Trenaunay P. Du Noevus variquex osteohypertrophiques. Archives of General Medicine (Paris) 1900;3:641-672. 

2. Gloviczki P, Stanson A, Stickler G, Johnson C, Toomey B, Meland N, Rooke T, Cherry K. Klippel Trenaunay Syndrome: The risks and benefits of vascular interventions. Surgery. 1991. 110;469-479. 

3. Servelle M. Klippel and Trenaunay’s Syndrome. Annals of Surgery 1985; 201:365-373 

4. Lindanauer SM. Klippel and Trenaunay’s Syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Vasa 1974; 3:231-241 

5. Stickler G. Klippel Trenaunay Syndrome. In Neurocutaneous diseases, A Practical Approach. Gomez M, Ed., Butterworths, Boston, 1987. 

6. Young, A., Ackroyd, J., Baskerville P., Combined Vascular Malformations, in Vascular Birthmarks: Hemangiomas and Malformations, Mulliken, J., ed, W. B. Saunders Co., Philadelphia, pp. 246-274, 1988. 

7. Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB. Surgical Implication of Klippel Trenaunay Syndrome. Annals of Surgery 1983; 197: 353-362. 

8. McGrory, Amadio PC. Klippel Trenaunay Syndrome: Orthopedic Considerations. Orthopedic Review 1993; 22(1): 41-50. 

9. Parkes WF. Hemangiectatic Hypertrophy of limbs: phlebarterectasias and the so called congenital varicose veins. Br J Child Dis 1918; 15: 13-17 

10. McGrory BJ, Amadio PC, Dobyns JH, et al. Anomalies of the fingers and toes associated with Klippel Trenaunay Syndrome. Journal of Bone and Joint surgery (Am) 1991;73:1537-1546. 

11. Telander RL, Kaufman BH, Gloviczki P, Stickler GB, Hollier LH. Prognosis and Management of Lesions of the Trunk in Children with Klippel-Trenaunay Syndrome. Journal of Pediatric Surgery 1984; 19(4): 417-422. 

12. Cherry,K, Gloviczki, P, Stanson, A, Persistent Sciatic Vein: The diagnosis and treatment of a rare condition, Journal of Vascular Surgery, in press. 

13. Louis-Bar D, Legros J. Les hypertrophies partielles avec angioma (syndrome de Klippel Trenaunay) et leurs rapports avec les phacomatoses. Confinia Neurol 1947; 7:245-263. 

14. Bliznak J, Staple TW. Radiology of angiodysplasia of the limb. Radiology. 1974; 110:35-44. 

15. Baskerville P, Ackroyd J, Browse N. The Etiology of the Klippel-Trenaunay syndrome. Annals of Surgery 1985; 202:624-627. 

16. Aelvoet GE, Jorens PG, Roelen LM. Genetic Aspects of Klippel-Trenaunay syndrome. British Journal of Dermatology 1992; 126: 603-607. 

17. Happle R. Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. Journal of the American Academy of Dermatology 1987; 16: 899-906. 

18. Weitkamp LR, Moss AJ, Lewis RA, Hall WJ, MacCluer JW, Schwartz PJ, Locati EH, Tzivoni D, Vincent GM, Robinson JL, et al. Analysis of HLA and disease susceptibility: chromosome 6 genes and sex influence long-QT phenotype. American Journal of Human Genetics. 1994; 55(6): 1230-1241. 

19. Mulliken JB, Glowacki J. Hemangiomas and Vascular Malformations in Infants and Children: A Classification based on Endothelial Characteristics. Plastic and Reconstructive Surgery 1982; 69(3):412-420. 

20. Martorell F, Monserrat J: Aplasia of the iliac vein and Klippel Trenaunay syndrome. Angiologia 1962; 14: 2-4 

21. Palatsi R. A case of the Klippel-Trenaunay-Park-Weber syndrome. Acta Dermatovener (Stockholm) 1975; 55:233-236. 

22. Baskerville PA, Ackroyd JS, Thomas ML, Browse NL. The Klippel Trenaunay Syndrome: clinical, radiological, and hemodynamic features and management. British Journal of Surgery 1985; 72: 232-236. 

23. Gorenstein A, Katz S, Schiller M. Congenital Angiodysplasia of the Superficial Venous System of the Lower Extremities in Children. Annals of Surgery 1988; 207: 213-218. 

24. Gorenstein A, Shifrin E, Gordon RL, Schumel K, Schiller M. Congenital Aplasia of the deep veins of lower extremities in children: The role of ascending functional phlebography. Surgery 1986; 99(4):414-419 

25. Gorenstein A, Gordon RL, Shifrin E, Schiller M. Phlebography of the deep venous system of the leg in children. Radiology 1982; 144: 519-524. 

26. Paes EHJ, Vollmar JF. Aneurysma transformation in congenital venous dysplasias in the lower extremities. International Angiology 1990; 9: 90-96. 

27. Stewart G, Farmer G. Sturge-Weber and Klippel Trenaunay Syndromes with absence of inferior vena cava. Arch Disease Childhood (England) 1990; 65:546-547. 

28. Servelle M, Bastin R, Loygue J, et al. Hematuria and rectal bleeding in the child with Klippel and Trenaunay syndrome. Annals of Surgery 1976;183:418-428. 

39. Samuel, M., Spitz, L., Klippel-Trenaunay Syndrome: Clinical Features, Complications and Management in Children, British Journal of Surgery, 1995, 82:757-761. 

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