1990, The British Journal of Radiology, 63, 581-582
Case of the month Just another case of varicose veins? By R. C. Travis, FRCR, *D. M. Rowan, FACD and J . Miller Departments of Radiology and 'Dermatology, Middlemore Hospital, Otahuhu, Auckland, New Zealand (Received May 1989 and in revised form August 1989)
A 49-year-old Maori woman was referred to the Surgical Outpatients Department because of increasing discomfort and disability in the left leg. She had varicose vein surgery as a child, and the veins later recurred. Views from her left lower limb venogram and bilateral aorto-femoral angiogram are shown. •Address correspondence to Dr Travis.
Figure 1. Antero-posterior views from the left leg venogram. Vol. 63, No. 751
Figure 2. Views from the lower limb arteriogram.
581
Case of the month
Figure 3. Clinical photograph of the left lower limb demonstrating soft-tissue hypertrophy and the cutaneous haemangioma.
The venogram demonstrates multiple venous abnormalities. Varicose veins are seen and there is a venous aneurysm at the level of the left knee. Thrombus is present in the superficial veins. The deep veins appear patent. The angiogram shows cortical thickening of the tibia and soft-tissue hypertrophy, together with multiple small vascular malformations in the left leg. No arteriovenous shunt was seen. On clinical examination, there was a classical port wine stain (Fig. 3) and lengthening and soft-tissue hypertrophy of the left leg. Discussion
The patient has Klippel-Trenaunay Syndrome (KTS). This was first described in 1900 by two French physicians, Klippel and Trenaunay as the "naevus variqueux osteo-hypertrophique" syndrome. The clinical manifestations are usually monomelic, and consist of venous varicosities, a port wine haemangioma and bone and soft-tissue hypertrophy of the affected limb (Taybi, 1983). Parkes-Weber described a similar syndrome in
582
1907 seen in association with a arteriovenous fistula. It is generally accepted that in KTS there is no clinically apparent or angiographic arteriovenous fistula (Lindenauer, 1971). Cases that have a significant arteriovenous fistula tend to be classified as Klippel-TrenaunayWeber Syndrome. A mesodermal defect which involves angiogenesis in the primitive limb bud has been postulated as a cause for KTS (Baskerville et al, 1985a). It is thought that late regression of the vascular reticular network causes persisting arteriovenous connections and venous abnormalities. There are no known hereditary factors, and no sex predeliction. The syndrome presents at birth with the port wine nevus. The varicose veins become more prominent when the child begins to stand erect. Limb hypertrophy may be gross at birth or may become more noticeable at puberty with the growth spurt. Multiple venous abnormalities are usually present. There may be hypoplasia or aplasia of the deep venous system. A valveless lateral venous channel can be seen in the leg which drains into the pelvic veins, and accompanies the sciatic nerve. Varicosities can be seen in the peroneal and supra-pubic areas, and may involve bladder, colon and uterus. The pelvic varicosities can contain phleboliths. Patients may present with haematuria, rectal blood loss or menorrhagia (Azouz, 1983). Superficial thrombophlebitis is not uncommon. Some authors report a high incidence of pulmonary embolus (Baskerville et al, 1985b). The manifestations tend to progress until the end of the third decade. Surgery may be indicated for troublesome superficial varicosities. However, generally it should be avoided and only attempted after careful venography. Incisions through the cutaneous haemangioma tend to heal poorly. Epiphysiodesis can be used to correct the leg length discrepancy. Most patients can be managed successfully with elastic stockings. Symptomatic haemodynamically significant arteriovenous malformations may be amenable to embolotherapy. References Azouz, E. M., 1983. Hematuria, rectal bleeding and pelvic phleboliths in children with the Klippel-Trenaunay Syndrome. Pediatric Radiology, 13, 82-88. BASKERVILLE, P. A., ACKROYD, J. S. & BROWSE, N. L., 1985a.
The etiology of the Klippel-Trenaunay Syndrome. Annals of Surgery, 202, 624-627. BASKERVILLE, P.
A.,
ACKROYD, J.
S.,
LEA THOMAS, M.
&
BROWSE, N. L., 1985b. The Klippel-Trenaunay Syndrome: clinical, radiological and hemodynamic features and management. British Journal of Surgery, 72, 232-236. LINDENAUER, S. M., 1971. Congenital arteriovenous fistula and the Klippel-Trenaunay Syndrome. Annals of Surgery, 174, 248-263. PARKES-WEBER, F., 1907. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. British Journal of Dermatology, 19, 231-235. TAYBI, H., 1983. Radiology of Syndromes and Metabolic Disorders (Year Book Medical Publishers, Chicago), p. 210.
The British Journal of Radiology, July 1990
Case of the month Just another case of varicose veins? By R. C. Travis, FRCR, *D. M. Rowan, FACD and J . Miller Departments of Radiology and 'Dermatology, Middlemore Hospital, Otahuhu, Auckland, New Zealand (Received May 1989 and in revised form August 1989)
A 49-year-old Maori woman was referred to the Surgical Outpatients Department because of increasing discomfort and disability in the left leg. She had varicose vein surgery as a child, and the veins later recurred. Views from her left lower limb venogram and bilateral aorto-femoral angiogram are shown. •Address correspondence to Dr Travis.
Figure 1. Antero-posterior views from the left leg venogram. Vol. 63, No. 751
Figure 2. Views from the lower limb arteriogram.
581
Case of the month
Figure 3. Clinical photograph of the left lower limb demonstrating soft-tissue hypertrophy and the cutaneous haemangioma.
The venogram demonstrates multiple venous abnormalities. Varicose veins are seen and there is a venous aneurysm at the level of the left knee. Thrombus is present in the superficial veins. The deep veins appear patent. The angiogram shows cortical thickening of the tibia and soft-tissue hypertrophy, together with multiple small vascular malformations in the left leg. No arteriovenous shunt was seen. On clinical examination, there was a classical port wine stain (Fig. 3) and lengthening and soft-tissue hypertrophy of the left leg. Discussion
The patient has Klippel-Trenaunay Syndrome (KTS). This was first described in 1900 by two French physicians, Klippel and Trenaunay as the "naevus variqueux osteo-hypertrophique" syndrome. The clinical manifestations are usually monomelic, and consist of venous varicosities, a port wine haemangioma and bone and soft-tissue hypertrophy of the affected limb (Taybi, 1983). Parkes-Weber described a similar syndrome in
582
1907 seen in association with a arteriovenous fistula. It is generally accepted that in KTS there is no clinically apparent or angiographic arteriovenous fistula (Lindenauer, 1971). Cases that have a significant arteriovenous fistula tend to be classified as Klippel-TrenaunayWeber Syndrome. A mesodermal defect which involves angiogenesis in the primitive limb bud has been postulated as a cause for KTS (Baskerville et al, 1985a). It is thought that late regression of the vascular reticular network causes persisting arteriovenous connections and venous abnormalities. There are no known hereditary factors, and no sex predeliction. The syndrome presents at birth with the port wine nevus. The varicose veins become more prominent when the child begins to stand erect. Limb hypertrophy may be gross at birth or may become more noticeable at puberty with the growth spurt. Multiple venous abnormalities are usually present. There may be hypoplasia or aplasia of the deep venous system. A valveless lateral venous channel can be seen in the leg which drains into the pelvic veins, and accompanies the sciatic nerve. Varicosities can be seen in the peroneal and supra-pubic areas, and may involve bladder, colon and uterus. The pelvic varicosities can contain phleboliths. Patients may present with haematuria, rectal blood loss or menorrhagia (Azouz, 1983). Superficial thrombophlebitis is not uncommon. Some authors report a high incidence of pulmonary embolus (Baskerville et al, 1985b). The manifestations tend to progress until the end of the third decade. Surgery may be indicated for troublesome superficial varicosities. However, generally it should be avoided and only attempted after careful venography. Incisions through the cutaneous haemangioma tend to heal poorly. Epiphysiodesis can be used to correct the leg length discrepancy. Most patients can be managed successfully with elastic stockings. Symptomatic haemodynamically significant arteriovenous malformations may be amenable to embolotherapy. References Azouz, E. M., 1983. Hematuria, rectal bleeding and pelvic phleboliths in children with the Klippel-Trenaunay Syndrome. Pediatric Radiology, 13, 82-88. BASKERVILLE, P. A., ACKROYD, J. S. & BROWSE, N. L., 1985a.
The etiology of the Klippel-Trenaunay Syndrome. Annals of Surgery, 202, 624-627. BASKERVILLE, P.
A.,
ACKROYD, J.
S.,
LEA THOMAS, M.
&
BROWSE, N. L., 1985b. The Klippel-Trenaunay Syndrome: clinical, radiological and hemodynamic features and management. British Journal of Surgery, 72, 232-236. LINDENAUER, S. M., 1971. Congenital arteriovenous fistula and the Klippel-Trenaunay Syndrome. Annals of Surgery, 174, 248-263. PARKES-WEBER, F., 1907. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. British Journal of Dermatology, 19, 231-235. TAYBI, H., 1983. Radiology of Syndromes and Metabolic Disorders (Year Book Medical Publishers, Chicago), p. 210.
The British Journal of Radiology, July 1990
