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-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
BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder.A detailed prenatal ultrasound examination plays an important role in the diagnosis of KTS and the subsequent counseling and follow-up of the pat...BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder.A detailed prenatal ultrasound examination plays an important role in the diagnosis of KTS and the subsequent counseling and follow-up of the patient.CASE SUMMARY A 25-year-old woman attended our department for a regular examination.The whole of the right lower extremity and right buttock were observed to be markedly thicker compared to the left one at 18 wk of gestation.However,the lengths of the right femur,tibia and fibula were in the normal range.No marked edema and fluid/cystic spaces were detected in the lower limbs.There were no other organ abnormalities.The vasculature in the right limb was visibly dilated,with much higher intensive blood flow signals.No congenital embryonic veins were visible in both limbs.The right lower limb exhibited much more hypertrophy compared to the left limb two weeks later.Amniocentesis and genetic tests showed normal results with 46 XX.Despite the normal karyotype,the family opted to terminate the pregnancy.The post-mortem examination confirmed asymmetric hypertrophy of the right limb in the fetus and revealed a large area of marked dark-purple superficial capillary malformations occupying the skin of the right lower extremity.The enlargement of veins and soft tissue hypertrophy were also seen on postnatal X-ray and Magnetic Resonance Imaging.Autopsy revealed severe congestion in the right lower limb.A final diagnosis of KTS was made.CONCLUSION KTS may be diagnosed prenatally based on the typical features observed during ultrasound examination.展开更多
BACKGROUND Klippel-Trenaunay syndrome(KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation(al...BACKGROUND Klippel-Trenaunay syndrome(KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation(also known as port-wine stain),varicose veins and malformations, and bony and/or soft tissue hypertrophy with or without lymphatic malformation, which are known as the “classic clinical triad”. Herein, a rare case of KTS characterized by crossed-bilateral limb hypertrophy accompanied by intermittent hematochezia and hematuria is reported.CASE SUMMARY We described a 37-year-old female with KTS. She was admitted to our hospital owing to the gradual enlargement of the left lower extremity along with intermittent hematochezia and hematuria. The patient was diagnosed to have hemorrhoid bleeding by other hospitals and treated with conventional hemostatic drugs, but continued to have intermittent gastrointestinal bleeding and hematuria. Therefore, she visited our hospital to seek further treatment. During hospitalization, relevant imaging and laboratory examinations and colonoscopy were performed. In combination with the patient’s history and relevant examinations, we considered that the patient had a complex form of KTS. We recommended a combined diagnosis and treatment from the vascular, interventional,anorectal, and other departments, although she declined any further treatment for financial reasons.CONCLUSION The clinical manifestations of KTS are extensive and diverse and chiefly include the typical triad. However, Vascular malformations of KTS can also involve several parts and systems such as digestive and urogenital systems. Therefore, the atypical manifestations and rare complications necessitate the clinician’s attention and are not to be ignored.展开更多
BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder characterized by a combination of capillary malformations,soft-tissue or bone hypertrophy,and varicose veins or venous malformations.The syndrome...BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder characterized by a combination of capillary malformations,soft-tissue or bone hypertrophy,and varicose veins or venous malformations.The syndrome predisposes patients to hypercoagulable states,including venous thromboembolism and pulmonary embolism(PE).CASE SUMMARY A 12-year-old girl with KTS was scheduled excision of verrucous hyperkeratosis in the left foot and posterior aspect of the left leg and left thigh and excision of a cutaneous hemangioma in the right buttock.After induction,the surgeon elevated the patient’s leg for sterilization,whereupon she experienced a massive PE and refractory cardiac arrest.Extracorporeal membrane oxygenation(ECMO)was performed after prolonged resuscitation,and she had a return of spontaneous circulation.After this episode,the patient was discharged without any neurologic complications.CONCLUSION The mechanism of PE,a lethal disease,involves a preexisting deep vein thrombosis that is mechanically dislodged by compression or changing positions and travels to the pulmonary artery.Therefore,patients predisposed to PE should be prescribed prophylactic anticoagulants.If the patient has unstable vital signs,resuscitation should be started immediately,and extracorporeal cardiopulmonary resuscitation should be considered in settings with existing ECMO protocols,expertise,and equipment.Awareness of PE in patients with KTS while leg raising for sterilization is critical.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 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-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BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder.A detailed prenatal ultrasound examination plays an important role in the diagnosis of KTS and the subsequent counseling and follow-up of the patient.CASE SUMMARY A 25-year-old woman attended our department for a regular examination.The whole of the right lower extremity and right buttock were observed to be markedly thicker compared to the left one at 18 wk of gestation.However,the lengths of the right femur,tibia and fibula were in the normal range.No marked edema and fluid/cystic spaces were detected in the lower limbs.There were no other organ abnormalities.The vasculature in the right limb was visibly dilated,with much higher intensive blood flow signals.No congenital embryonic veins were visible in both limbs.The right lower limb exhibited much more hypertrophy compared to the left limb two weeks later.Amniocentesis and genetic tests showed normal results with 46 XX.Despite the normal karyotype,the family opted to terminate the pregnancy.The post-mortem examination confirmed asymmetric hypertrophy of the right limb in the fetus and revealed a large area of marked dark-purple superficial capillary malformations occupying the skin of the right lower extremity.The enlargement of veins and soft tissue hypertrophy were also seen on postnatal X-ray and Magnetic Resonance Imaging.Autopsy revealed severe congestion in the right lower limb.A final diagnosis of KTS was made.CONCLUSION KTS may be diagnosed prenatally based on the typical features observed during ultrasound examination.
基金Supported by the Basic Research Projects of Science and Technology Department of Guizhou Province,No. Qian Ke He-zk[2022]-646Master Start-up Foundation of Affiliated Hospital of Zunyi Medical College,No. 2016-45Collaborative Innovation Center of Chinese Ministry of Education,No. 2020-39。
文摘BACKGROUND Klippel-Trenaunay syndrome(KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation(also known as port-wine stain),varicose veins and malformations, and bony and/or soft tissue hypertrophy with or without lymphatic malformation, which are known as the “classic clinical triad”. Herein, a rare case of KTS characterized by crossed-bilateral limb hypertrophy accompanied by intermittent hematochezia and hematuria is reported.CASE SUMMARY We described a 37-year-old female with KTS. She was admitted to our hospital owing to the gradual enlargement of the left lower extremity along with intermittent hematochezia and hematuria. The patient was diagnosed to have hemorrhoid bleeding by other hospitals and treated with conventional hemostatic drugs, but continued to have intermittent gastrointestinal bleeding and hematuria. Therefore, she visited our hospital to seek further treatment. During hospitalization, relevant imaging and laboratory examinations and colonoscopy were performed. In combination with the patient’s history and relevant examinations, we considered that the patient had a complex form of KTS. We recommended a combined diagnosis and treatment from the vascular, interventional,anorectal, and other departments, although she declined any further treatment for financial reasons.CONCLUSION The clinical manifestations of KTS are extensive and diverse and chiefly include the typical triad. However, Vascular malformations of KTS can also involve several parts and systems such as digestive and urogenital systems. Therefore, the atypical manifestations and rare complications necessitate the clinician’s attention and are not to be ignored.
文摘BACKGROUND Klippel-Trenaunay syndrome(KTS)is a rare congenital disorder characterized by a combination of capillary malformations,soft-tissue or bone hypertrophy,and varicose veins or venous malformations.The syndrome predisposes patients to hypercoagulable states,including venous thromboembolism and pulmonary embolism(PE).CASE SUMMARY A 12-year-old girl with KTS was scheduled excision of verrucous hyperkeratosis in the left foot and posterior aspect of the left leg and left thigh and excision of a cutaneous hemangioma in the right buttock.After induction,the surgeon elevated the patient’s leg for sterilization,whereupon she experienced a massive PE and refractory cardiac arrest.Extracorporeal membrane oxygenation(ECMO)was performed after prolonged resuscitation,and she had a return of spontaneous circulation.After this episode,the patient was discharged without any neurologic complications.CONCLUSION The mechanism of PE,a lethal disease,involves a preexisting deep vein thrombosis that is mechanically dislodged by compression or changing positions and travels to the pulmonary artery.Therefore,patients predisposed to PE should be prescribed prophylactic anticoagulants.If the patient has unstable vital signs,resuscitation should be started immediately,and extracorporeal cardiopulmonary resuscitation should be considered in settings with existing ECMO protocols,expertise,and equipment.Awareness of PE in patients with KTS while leg raising for sterilization is critical.
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.
