Klippel-Trenaunay syndrome (KTS) is not a common congenital vascular abnormality. A trio of capillary malformation, venous varicosities, and bony or soft-tissue hypertrophy define this syndrome. Significant morbiditie...Klippel-Trenaunay syndrome (KTS) is not a common congenital vascular abnormality. A trio of capillary malformation, venous varicosities, and bony or soft-tissue hypertrophy define this syndrome. Significant morbidities associated with this illness include bleeding, deep vein thrombosis, and embolic consequences. Angiokeratoma circumscriptum naeviforme (ACN) is indeed a congenital variant of angiokeratoma that appears as a hyperkeratotic plaque on the lower extremity. Bilateral congenital anorchia (BCA) is the total lack of testicular tissue in a male with a normal phenotype and karyotype. KTS has been linked to ACN. Here we presented an 8-year-old male child who came with a swollen left thigh and the right side of his face with overlying blackish nodules on his left thigh and scrotum. The patient was diagnosed as KTS with angiokeratoma circumscriptum naeviforme and bilateral congenital anorchia based on his history, imaging studies and the typical clinical features of the disease.展开更多
Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins,cutaneous capillary malformation,and hypertrophy of bone and(or)soft tissue.Gastrointestinal vascular malformation...Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins,cutaneous capillary malformation,and hypertrophy of bone and(or)soft tissue.Gastrointestinal vascular malformations in Klippel-Trenaunay syndrome may present with gastrointestinal bleeding.The majority of patients with spleenic hemangiomatosis and/or left inferior vena cava are asymptomatic.We herein report a case admitted to the gastroenterology clinic with life-threatening hematochezia and symptomatic iron deficiency anemia.Due to the asymptomatic mild intermittent hematochezia,splenic hemangiomas and left inferior vena cava,the patient did not seek any help for gastrointestinal bleeding until his admittance to our department for evaluation of massive gastrointestinal bleeding.He was referred to angiography because of his serious pathogenetic condition and inefficiency of medical therapy.The method showed that hemostasis was successfully achieved in the hemorrhage site by embolism of corresponding vessels.Further endoscopy revealed vascular malformations starting from the stomach to the descending colon.On the other hand,computed tomography revealed splenic hemangiomas and left inferior vena cava.To the best of our knowledge,this is the first Klippel-Trenaunay syndrome case presenting with gastrointestinal bleeding,splenic hemangiomas and left inferior vena cava.The literature on the evaluation and management of this case is reviewed.展开更多
Klippel-Trénaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations. Visceral organs may also be involved leading to a variety of manifestations...Klippel-Trénaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations. Visceral organs may also be involved leading to a variety of manifestations. Although seemingly uncommon, genital tract involvement can occur and be a source of significant morbidity. We hereby describe a case of KTS of a 7-year-old female patient presenting with genital bleeding, large venous and capillary malformations and soft tissue hypertrophy of right limb. Patient was treated conservatively and asked to follow-up regularly. KTS should be kept on mind as a cause of vaginal bleeding in preteen girls especially those with suggestive clinical features. Management of this complex condition is challenging because of its progressive nature and wide range of disease severity.展开更多
Klippel-Trenaunay Syndrome (KTS) is a rare triad of congenital vascular malformations involving extensive Port wine stains, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation affecting...Klippel-Trenaunay Syndrome (KTS) is a rare triad of congenital vascular malformations involving extensive Port wine stains, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation affecting limb, pelvicor abdominal organs. Pregnancy is known to exacerbate KTS complications and can put women at increased obstetrical risk due to deep venous thrombosis and other thromboembolic events. We report a case of a pregnant woman with KTS with the personal antecedent of two episodes of pulmonary thromboembolism.展开更多
文摘Klippel-Trenaunay syndrome (KTS) is not a common congenital vascular abnormality. A trio of capillary malformation, venous varicosities, and bony or soft-tissue hypertrophy define this syndrome. Significant morbidities associated with this illness include bleeding, deep vein thrombosis, and embolic consequences. Angiokeratoma circumscriptum naeviforme (ACN) is indeed a congenital variant of angiokeratoma that appears as a hyperkeratotic plaque on the lower extremity. Bilateral congenital anorchia (BCA) is the total lack of testicular tissue in a male with a normal phenotype and karyotype. KTS has been linked to ACN. Here we presented an 8-year-old male child who came with a swollen left thigh and the right side of his face with overlying blackish nodules on his left thigh and scrotum. The patient was diagnosed as KTS with angiokeratoma circumscriptum naeviforme and bilateral congenital anorchia based on his history, imaging studies and the typical clinical features of the disease.
文摘Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins,cutaneous capillary malformation,and hypertrophy of bone and(or)soft tissue.Gastrointestinal vascular malformations in Klippel-Trenaunay syndrome may present with gastrointestinal bleeding.The majority of patients with spleenic hemangiomatosis and/or left inferior vena cava are asymptomatic.We herein report a case admitted to the gastroenterology clinic with life-threatening hematochezia and symptomatic iron deficiency anemia.Due to the asymptomatic mild intermittent hematochezia,splenic hemangiomas and left inferior vena cava,the patient did not seek any help for gastrointestinal bleeding until his admittance to our department for evaluation of massive gastrointestinal bleeding.He was referred to angiography because of his serious pathogenetic condition and inefficiency of medical therapy.The method showed that hemostasis was successfully achieved in the hemorrhage site by embolism of corresponding vessels.Further endoscopy revealed vascular malformations starting from the stomach to the descending colon.On the other hand,computed tomography revealed splenic hemangiomas and left inferior vena cava.To the best of our knowledge,this is the first Klippel-Trenaunay syndrome case presenting with gastrointestinal bleeding,splenic hemangiomas and left inferior vena cava.The literature on the evaluation and management of this case is reviewed.
文摘Klippel-Trénaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations. Visceral organs may also be involved leading to a variety of manifestations. Although seemingly uncommon, genital tract involvement can occur and be a source of significant morbidity. We hereby describe a case of KTS of a 7-year-old female patient presenting with genital bleeding, large venous and capillary malformations and soft tissue hypertrophy of right limb. Patient was treated conservatively and asked to follow-up regularly. KTS should be kept on mind as a cause of vaginal bleeding in preteen girls especially those with suggestive clinical features. Management of this complex condition is challenging because of its progressive nature and wide range of disease severity.
文摘Klippel-Trenaunay Syndrome (KTS) is a rare triad of congenital vascular malformations involving extensive Port wine stains, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation affecting limb, pelvicor abdominal organs. Pregnancy is known to exacerbate KTS complications and can put women at increased obstetrical risk due to deep venous thrombosis and other thromboembolic events. We report a case of a pregnant woman with KTS with the personal antecedent of two episodes of pulmonary thromboembolism.
