1/99. Recurrent pulmonary embolism associated with klippel-trenaunay-weber syndrome.klippel-trenaunay-weber syndrome (KTWS) is a rare, congenital disorder characterized by the triad of varicose veins, cutaneous hemangiomas, and hypertrophy of soft tissue and bone. We present the case of a woman with KTWS, cor pulmonale, and death due to recurrent pulmonary embolism (PE). The risk of deep venous thrombosis and PE in patients with KTWS is evaluated, and treatment recommendations are made with emphasis on the role of early, aggressive management in the subset of patients with KTWS known to have thromboembolic disease.- - - - - - - - - - ranking = 1keywords = hemangioma, angioma (Clic here for more details about this article) |
2/99. Epidural analgesia in an obstetric patient with Klippel-Trenaunay syndrome.We describe the use of epidural analgesia for vaginal delivery of a parturient with Klippel-Trenaunay syndrome in whom the use of repeated magnetic resonance imaging during her obstetric care allowed us to see deep haemangiomata. This also allowed the safe sitting of an epidural catheter at L1-2 to provide analgesia for labour and delivery. Klippel-Trenaunay syndrome and the anaesthetic implications of the congenital vascular abnormalities and potential coagulopathy are discussed.- - - - - - - - - - ranking = 0.0058611236655813keywords = angioma (Clic here for more details about this article) |
3/99. Prenatal sonographic appearances of klippel-trenaunay-weber syndrome.A case of prenatal Klippel Trenaunay-Weber syndrome is presented, with a description of the sonographically observed disease progression in utero. The appearance of a complex thoracic multicystic mass in association with progressive unilateral lower limb enlargement was strongly suspicious of this disorder. Colour-flow Doppler studies of the cutaneous haemangiomata in utero were not of diagnostic assistance in this case. The prenatal detection of the large cutaneous haemangiomata was of critical importance in minimizing delivery trauma for the mother and fetus.- - - - - - - - - - ranking = 0.011722247331163keywords = angioma (Clic here for more details about this article) |
4/99. Right iliac vein agenesis, varicosities, and widespread hemangiomas: report of a rare case.We present a probable variant of the Klippel-Trenaunay syndrome with the clinical features of capillary hemangiomas, varicosities, and agenesis of the right iliac venous system, but without limb hypertrophy. To our knowledge, this is the 1st such case reported in the medical literature.- - - - - - - - - - ranking = 5keywords = hemangioma, angioma (Clic here for more details about this article) |
5/99. Naevus varicosus osteohypertrophicus. An early diagnostic approach.Naevus varicosus osteohypertrophicus (synonym Klippel-Trenaunay Syndrome KTS) is relatively rare circumscribed, usually quadrant-related gigantism with vascular hyperplasia or malformations based on the embryonic development. The authors observed an 18- and a 30-year-old female with a triad of symptoms: cutaneous nevus flammeus, varicose and dilated veins, and bony and soft tissue hypertrophy of the low limb. The second patient also had two venous ulcers as a dominant clinical feature--a rare manifestation of Klippel-Trenaunay syndrome. A diagnosis of Klippel-Trenaunay syndrome was made by clinical observations, laboratory findings, dermoscan, radiological examination of the bones of the limb, Doppler ultrasonography, photopletismography and venoscan. A bone isotope scan was also done to the first patient. Making an early diagnosis of this sporadic congenital disease with unknown aetiology is important in order to be able to provide early prophylactic and therapeutic measures. Klippel and Trenaunay in 1900 were the first to describe a patient with the simultaneous appearance of osteohypertrophy, hemangiomas and varicose veins involving one extremity [1]. In 1907 Parkes and Weber reported a similar syndrome--they described a patient who had dilated and pulsatile arteries in the affected region including the presence of arterio-venous communications. In 1918 they used the compromise term "haemangiectatic hypertrophy" to embrace all conditions which were associated with congenital vascular malformations including A-V anastomoses associated with bone and soft tissue hypertrophy. Most authors are agreed that Klippel-Trenaunay syndrome and the syndrome of multiple congenital arterio-venous fistulae are two separate features of the Parkes Weber hypertrophy. KTS is manifesting with a triad of symptoms: cutaneous vascular nevus (more frequently nevus flammeus type), superficial venous varicosities and hypertrophy of the affected limb. Usually one quadrant of the body is involved: quite often a leg, an arm, lateral side of the trunk, very rarely the face. More than one quadrant and bilateral involvement are rarely affected. Naevus flammeus appeared at birth. It is extremely variable both in extent and in color--the latter ranging from pale pink to deep purple. Veinous varicosities appear in childhood and adolescence. They are painful and may be complicated by superficial or deep venous thrombosis and rarely, ulceration. hypertrophy of the affected extremities is due to bone and soft tissue hypertrophy. KTS can be associated with other developmental anomalies such as: polydactyly, syndactyly, oligodactyly [2] macrocephaly, blue nevus, epidermal naevus, venous malformations.- - - - - - - - - - ranking = 1keywords = hemangioma, angioma (Clic here for more details about this article) |
6/99. Endoscopic neodymium:yttrium aluminium garnet (Nd:YAG) laser irradiation of a bladder hemangioma associated with Klippel-Weber syndrome.A case of endoscopic neodymium:yttrium aluminium garnet (Nd:YAG) laser coagulation of a bladder hemangioma associated with Klippel-Weber syndrome is presented. The patient presented with extensive nevus and swelling of the left lower limb since birth. She was diagnosed with Klippel-Weber syndrome by angiography at the age of 1 year. Gross hematuria had been observed since she was 1 year old and, in addition, endoscopic examination revealed diffuse bladder hemangiomas. At 8 years of age, gross hematuria became worse and gait disturbance also appeared. She was referred to the Department of urology at Tohoku University School of medicine for endoscopic treatment in June 1998. Under general anesthesia, the bladder was inflated with CO2 gas and the hemangiomas were coagulated by Nd:YAG laser photonic irradiation endoscopically. Gross hematuria was markedly improved immediately following this treatment. Klippel-Weber syndrome is a rather uncommon disease which shows various types of vascular anomaly and hypertrophy of the lower extremities. Three to 6% of the patients have associated bladder hemangiomas. Although Nd:YAG laser irradiation provides results superior to alternative therapy and is the preferred treatment for most patients with bladder hemangioma, in this case, hemangiomas are multiple and present the possibility of re-bleeding, therefore long-term and close follow up is important.- - - - - - - - - - ranking = 10keywords = hemangioma, angioma (Clic here for more details about this article) |
7/99. Klippel-Trenaunay syndrome: frequency of cerebral and cerebellar hemihypertrophy on MRI.We examined 11 patients, clinically and radiographically diagnosed as having the Klippel-Trenaunay syndrome (KTS) by MRI. There were four females and seven males, aged 3-51 years (mean 21 years). Two had clear asymmetry of the cerebral and cerebellar hemispheres. The thickness of the grey matter was normal, without sulcation abnormalities, but the thickness of the white matter was increased; the size of the ipsilateral ventricle was normal. These patients had hypertrophy of the leg and a cutaneous haemangioma on the same side as the brain abnormality. No patient had an intracranial vascular malformation, unilateral megalencephaly, cerebral atrophy or hydrocephalus. The prevalence of cerebral hemihypertrophy in our series of patients with KTS was thus 18%.- - - - - - - - - - ranking = 0.0058611236655813keywords = angioma (Clic here for more details about this article) |
8/99. klippel-trenaunay-weber syndrome presenting as massive lymphangiohemangioma of the thigh: prenatal diagnosis.We report a case of klippel-trenaunay-weber syndrome presenting prenatally as a massive congenital lymphangiohemangioma of the thigh. Routine ultrasonographic examination revealed multiple distorted cystic areas extending from the right flank through the right lower extremity of a 30-week fetus. A diagnosis of cystic lymphangioma of the thigh was suspected prenatally. Neonatal evaluation confirmed the prenatal findings. Neonatal color Doppler imaging revealed blood vessels within the tumor. The differential diagnosis is discussed together with available therapeutic procedures.- - - - - - - - - - ranking = 5.0058611236656keywords = hemangioma, angioma (Clic here for more details about this article) |
9/99. Klippel-Trenaunay syndrome. An unusual presentation of unilateral leg edema.Klippel-Trenaunay syndrome is a rare congenital condition that classically presents as a triad of varicosities, bone or soft-tissue hypertrophy, and cutaneous hemangiomas. The authors describe a case of this disease in an otherwise healthy patient with unilateral leg edema and intermittent bleeding from prominent ankle varicosities. The diagnosis was made on the basis of clinical presentation, diagnostic studies, and a comprehensive literature review. An overview of the pathology, treatment, and prognosis of this disease is provided.- - - - - - - - - - ranking = 1keywords = hemangioma, angioma (Clic here for more details about this article) |
10/99. An unusual association of intracranial aneurysms and oesophageal duplication in a case of Klippel-Trenaunay syndrome.The Klippel-Trenaunay syndrome (KTS) is a congenital disorder resulting from a mesodermal abnormality, characterised by cutaneous capillary haemangiomas, hypertrophy of bone and soft tissues and varicose veins. The presence of intracranial aneurysms has rarely been described, while oesophageal duplication has not been reported previously. We describe a patient with the KTS with both there additional abnormalities, which could be explained by a postulated mosaic gene abnormality.- - - - - - - - - - ranking = 0.0058611236655813keywords = angioma (Clic here for more details about this article) |
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