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Klippel trenaunay

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https://www.readbyqxmd.com/read/28739624/klippel-trenaunay-syndrome-diagnosis-in-a-neonate
#1
Cátia Pereira, Rita Espí Rito Santo, Joana Saldanha
No abstract text is available yet for this article.
July 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28724825/a-rare-case-of-overlapping-sturge-weber-syndrome-and-klippel-trenaunay-syndrome-associated-with-bilateral-refractory-childhood-glaucoma
#2
Manju R Pillai, P P Hasini, Ashish Ahuja, S R Krishnadas
A 6-year-old girl presented with blurred vision and was found to have elevated intraocular pressure (IOP) and glaucomatous optic disc damage in both eyes. She also displayed capillary malformations on the face (port-wine stain), upper back and all four limbs, angiomatosis in the brain and had hypertrophy of the left upper and lower limbs typical of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndromes. She was initially managed with IOP lowering topical medications but required trabeculectomy in the right eye followed by Ahmed valve implantation in both eyes...
July 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28721324/a-39-year-old-pregnant-woman-with-pulmonary-emboli-on-long-term-anticoagulation
#3
Vishisht Mehta, Karishma Bhatia, Amanda M Dave, Zachary S Depew
We present the case of a 39-year-old pregnant woman with Klippel-Trenaunay syndrome (KTS). We demonstrate the risks of multiple, co-existing pro-thrombotic states (pregnancy, KTS), discuss complications of KTS (deep venous thromboembolisms and pulmonary emboli) and highlight general and disease-specific preventive measures against venous thromboembolic events (VTE). KTS is a rare condition and it's co-existence with pregnancy and VTEs is rarer still.
June 15, 2017: Curēus
https://www.readbyqxmd.com/read/28698417/-intravenous-ablation-for-lower-extremity-varices-to-prevent-recurrent-pulmonary-embolism-in-klippel-trenaunay-syndrome-report-of-a-case
#4
Ikutaro Kigawa, Masahiro Aiba, Atsushi Ito
Klippel-Trenaunay syndrome( KTS) is a complex congenital anomaly characterized by lower extremity varix and venous malformations, port-wine stains, and soft tissue and bone hypertrophy. We present a case of a 35-year-old man of KTS with pulmonary embolism (PE). The patient was referred to our hospital complaining of the leg pain and sudden onset of dyspnea. Enhanced computed tomography (CT) showed lower extremity varices, superficial thrombophlebitis and bilateral pulmonary thrombi. No venous malformations except for varicose veins were revealed in the limbs or pelvis...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28671169/hemoptysis-beyond-routine-chest-computed-tomography-and-bronchoscopy
#5
Saurabh Mall, Rahul Kumar Sharma, Deepak Prajapat, Samir K Gupta, Deepak Talwar
Hemoptysis is considered as a medical emergency which requires urgent stabilization with identification and correction of underlying etiology. Diagnosis of the cause of hemoptysis is not always readily identified after bronchoscopy and conventional computed tomography (CT) chest. Arteriovenous malformation (AVM) is a rare but important cause of massive hemoptysis which can be easily picked up by the use of double turn contrast CT chest. We here report a rare congenital AVM anomaly called Klippel-Trenaunay-Parks-Weber syndrome as a cause of massive hemoptysis and utility of double turn CT in diagnosing AVM as a cause of hemoptysis...
July 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28654575/update-on-classification-and-diagnosis-of-vascular-malformations
#6
Catherine C McCuaig
PURPOSE OF REVIEW: This review provides an update of the classification in the classification of vascular anomalies since April 2014 at the International Society for the Study of Vascular Anomalies meeting in Melbourne, Australia. RECENT FINDINGS: The reader will become familiar with how to diagnose the major vascular malformations, including capillary, venous, arteriovenous, and lymphatic and combinations thereof. In addition, vascular malformation syndromes, including those with overgrowth, will be clarified...
August 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28652904/successful-treatment-of-unilateral-klippel-trenaunay-syndrome-with-pulsed-dye-laser-in-a-2-week-old-infant
#7
Hoda Rahimi, Habib Hassannejad, Hamideh Moravvej
Introduction: Klippel-Trenaunay syndrome (KTS) is a rare congenital mesodermal abnormality characterized by varicose veins, cutaneous capillary malformation, as well as bone and soft tissue hypertrophy. Case Report: A 2-week-old female infant presented to our clinic because of vascular nevus and progressive enlargement of her right extremities and trunk since birth. The patient was treated with 595-nm pulsed-dye laser (PDL). Her port-wine stain (PWS) disappeared completely after third PDL session and the soft tissue hypertrophy stopped...
2017: Journal of Lasers in Medical Sciences
https://www.readbyqxmd.com/read/28633736/klippel-trenauney-syndrome-with-axillary-hyperhidrosis
#8
Yeun Jina Lim, David Rosmarin, Giannoula Klement, Shiu-Chung Au
Klippel-Trenaunay syndrome (KTS) is a rare, clinically variable congenital disorder involving capillary malformations, soft tissue or bone hypertrophy, and venous malformations or varicose veins. We report a 28-year-old man who presented with a hypertrophic right arm as well as markedly increased ipsilateral axillary hyperhidrosis and erythematous patches on the back, chest, and arm. This case of KTS is unusual because our patient presented with a markedly increased unilateral axillary hyperhidrosis ipsilateral to the hypertrophic limb...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28624001/diagnosis-and-management-of-the-venous-malformations-of-klippel-tr%C3%A3-naunay-syndrome
#9
REVIEW
S Keisin Wang, Natalie A Drucker, Alok K Gupta, Francis E Marshalleck, Michael C Dalsing
OBJECTIVE: A dearth of information exists in the literature regarding current practice in the management of Klippel-Trénaunay syndrome (KTS), a rare condition. We review and describe the etiology, diagnosis, and treatment of KTS. METHODS: Relevant data were synthesized from a Medline review using a combination of the keyterms "Klippel" and "Trénaunay." The majority of hits described singular case reports and were subsequently excluded. The remaining papers were then reviewed and included on the basis of the quality of evidence and the authors' discretion...
July 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28623992/near-infrared-fluorescence-lymphatic-imaging-of-klippel-tr%C3%A3-naunay-syndrome
#10
John C Rasmussen, Rodrick C Zvavanjanja, Melissa B Aldrich, Matthew R Greives, Eva M Sevick-Muraca
The relationship between lymphatic and venous malformations in Klippel-Trénaunay syndrome is difficult to assess. Herein the authors describe near-infrared fluorescence lymphatic imaging to assess the lymphatics of a subject with a large port-wine stain and right leg edema. Although lymphatic vessels in the medial, affected knee appeared dilated and perhaps tortuous, no definitive abnormal lymphatic pooling or propulsion was observed. The lymphatics in the affected limb were well defined but less numerous than in the contralateral limb, and active, contractile function was observed in all vessels...
July 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28604466/unique-considerations-in-spinal-cord-stimulator-placement-in-pediatrics-a-case-report
#11
Eugene Kim, Giovanni Cucchiaro
Spinal cord stimulation can be a valuable treatment option in the management of neuropathic pain in select pediatric patients. We present a unique case of a 16-year-old girl with Klippel-Trenaunay-Weber syndrome and scoliosis who required the placement of a spinal cord stimulator (SCS) for severe sciatic nerve neuropathic pain after a right above-knee amputation. Several attempts at lead placement were required before successful pain coverage was achieved because of late recognition of significant vertebral body rotation...
June 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28577738/-pik3ca-related-overgrowth-syndrome-pros
#12
Quitterie Venot, Guillaume Canaud
This review presents an overview of a recently characterized spectrum of overgrowth syndrome: phosphoinositide-3 kinase (PI3K)-related overgrowth spectrum (PROS). This spectrum encompasses overgrowth syndromes associated with somatic mosaic activating PIK3CA mutations such as megalencephaly-capillary malformation (MCAP) syndrome, dysplatic megalencephaly (DMEG), congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies (CLOVES) syndrome, hemihyperplasia-multiple lipomatosis (HHML), fibroadipose overgrowth and Klippel-Trenaunay syndrome...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28523345/endovenous-laser-ablation-therapy-in-children-applications-and-outcomes
#13
Premal A Patel, Alex M Barnacle, Sam Stuart, Joao G Amaral, Philip R John
BACKGROUND: Endovenous laser ablation is well recognized as the first-line treatment for superficial venous reflux with varicose veins in adults. It is not widely reported and is not an established practice in pediatric patients. OBJECTIVE: To illustrate a variety of pediatric venous conditions in which endovenous laser ablation can be utilized and to demonstrate its feasibility and safety in children. MATERIALS AND METHODS: We conducted a retrospective review of endovenous laser ablation procedures performed between January 2007 and July 2014 at two large pediatric institutions...
May 18, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28458832/a-rare-presentation-of-klippel-trenaunay-syndrome-with-bilateral-lower-limbs
#14
Akira Baba, Shinji Yamazoe, Yumi Okuyama, Kanichiro Shimizu, Yuko Kobashi, Yosuke Nozawa, Yohei Munetomo, Takuji Mogami
Klippel-Trenaunay syndrome (KTS) is a vascular lymphatic malformation underlying with bony and soft tissue hypertrophy. It is a rare condition presenting in 1 out of 10 000 people. The growth disturbance due to KTS is more commonly unilateral (85%) than bilateral (12.5%), and most rarely crossed-bilateral (2.5%). A man in his 40s presented to our hospital with a complaint of lower limb discomfort. Radiograph, ultrasonography, computed tomography venography, magnetic resonance (and venography) showed various radiological findings characteristic for KTS...
February 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28432715/complications-of-pregnancy-and-labour-in-women-with-klippel-tr%C3%A3-naunay-syndrome-a-nationwide-cross-sectional-study
#15
Ser Horbach, M M Lokhorst, Ceu Oduber, S Middeldorp, Jam van der Post, Cmam van der Horst
OBJECTIVE: To evaluate complications of pregnancy, including thromboembolism, in women with extensive vascular malformations associated with Klippel-Trénaunay syndrome (KTS). DESIGN: Nationwide cross-sectional study. SETTING: Two tertiary expert centres and the Dutch Klippel-Trénaunay patient organisation. SAMPLE: Adult women with KTS. METHODS: Patients with KTS were invited to participate in a comprehensive online survey about their obstetric history...
April 22, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28424013/anterior-spinal-artery-aneurysm-in-aortic-stenosis-of-different-etiology-report-of-three-cases
#16
Vivek Singh, Suprava Naik, Sanjeev K Bhoi, R V Phadke
Isolated aneurysms of spinal arteries are rare. Spinal artery aneurysms are commonly found in association with spinal cord arteriovenous malformation and coarctation of aorta and rarely with aortic arch interruption and Klippel-Trenaunay syndrome. Spinal angiograms are the gold standard for diagnosing these spinal artery aneurysms but with the advances in computed tomography technology these aneurysms can also be very well demonstrated in computed tomography angiograms. We describe three cases of anterior spinal artery aneurysm, those are flow related aneurysms, associated with coarctation of aorta and with Takayasu arteritis...
April 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28328134/somatic-pik3ca-mutations-in-seven-patients-with-pik3ca-related-overgrowth-spectrum
#17
Kit San Yeung, Janice Jing Kun Ip, Chin Pang Chow, Evelyn Yue Ling Kuong, Paul Kwong-Hang Tam, Godfrey Chi-Fung Chan, Brian Hon-Yin Chung
Somatic mutations in PIK3CA cause many overgrowth syndromes that have been recently coined the "PIK3CA-Related Overgrowth Spectrum." Here, we present seven molecularly confirmed patients with PIK3CA-Related Overgrowth Spectrum, including patients with Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal syndrome, Klippel-Trenaunay syndrome, lymphatic malformation and two with atypical phenotypes that cannot be classified into existing disease categories. The literature on PIK3CA-Related Overgrowth Spectrum, suggests that PIK3CA c...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28266961/thoracic-manifestations-of-klippel-trenaunay-syndrome
#18
Mark M Hammer, Wallace T Miller
Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by venous malformations and a predisposition to pulmonary embolism. We reviewed the imaging findings of 7 adult patients with KTS who underwent thoracic CT. While the superficial manifestations of KTS predominantly affect the extremities, patients frequently develop abnormalities of the pulmonary arterial system, particularly chronic thromboembolic pulmonary hypertension. Additionally, some patients are seen to develop pulmonary arteriolar aneurysms; the physiologic significance of this finding is unknown at this time...
May 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28168078/a-rare-case-of-diffuse-hemangiomatosis-of-the-spleen-with-splenic-rupture-following-aortic-valve-replacement
#19
F Capilli, M Weinbeck, M Czerny, M Siepe, T Krauss
In this paper we present a rare case of splenic rupture that occurred after an open aortic valve replacement in a male patient with hemangiomatosis of the spleen and the liver. The patient was treated with an emergency splenectomy. He showed no other sings of associated systemic disorder, such as Klippel-trénaunay syndrome or Proteus syndrome.
2017: Case Reports in Radiology
https://www.readbyqxmd.com/read/27941746/klippel-trenaunay-syndrome-a-case-report
#20
M N Islam, M A Hossain, M S Rahman, T Tazmin, M A Ali, F Sultana, S A Haque
Klippel Trenaunay syndrome refers to a rare congenital anomaly which is characterized by capillary malformation, venous malformation and sometimes lymphatic malformation associated with overgrowth of a limb, with soft tissue hypertrophy and/or bony hypertrophy. The anomaly, if present, is present at birth and usually involves the lower limbs as well as portion of trunk, face, uppper limb or head. Our reporting case is a preterm male neonate having port wine stain, varicose veins and excessive growth of soft tissue of left lower limb clinically consistent with Klippel Trenaunay Syndrome...
October 2016: Mymensingh Medical Journal: MMJ
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