scholarly journals Klippel-Trenaunay Syndrome with Extensive Lymphangiomas

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Sirin Mneimneh ◽  
Ali Tabaja ◽  
Mariam Rajab
Keyword(s):  
Soft Tissue ◽  
Vascular Malformations ◽  
Rare Disorder ◽  
Port Wine ◽  
Port Wine Stains ◽  
Klippel Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by the triad of vascular malformations, venous varicosities, and bone and soft-tissue hypertrophy. We present a case of Klippel-Trenaunay syndrome with limb hypertrophy, port-wine stains, lymphangiomas, and venous varicosities in the limbs.

scholarly journals Prenatal diagnosis of Klippel–Trenaunay syndrome: Series of four cases and review of the literature

Ultrasound ◽  
2019 ◽  
Vol 28 (2) ◽  
pp. 91-102
Author(s):  
Olga Ivanitskaya ◽  
Elena Andreeva ◽  
Natalia Odegova
Keyword(s):  
Quality Of Life ◽  
Prenatal Diagnosis ◽  
Soft Tissue ◽  
Varicose Veins ◽  
Port Wine ◽  
Review Of The Literature ◽  
Unique Case ◽  
Port Wine Stains ◽  
Klippel Trenaunay Syndrome

Klippel–Trenaunay syndrome is a rare disease with a classic triad of port wine stains, varicose veins, and bony and soft tissue hypertrophy of an extremity. The quality of life in these patients is significantly affected, making the prenatal diagnosis of Klippel–Trenaunay syndrome important. We present four prenatally diagnosed cases of this anomaly with a unique case of ectrodactyly of the hand in foetus with Klippel–Trenaunay syndrome. Such a combination has not been previously reported prenatally. A review of the literature for similar cases is also presented.

scholarly journals Klippel–Trénaunay Syndrome – A Very Rare and Interesting Syndrome

2015 ◽  
Vol 9 ◽  
pp. CCRPM.S21645 ◽  
Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri
Keyword(s):  
Soft Tissue ◽  
Vascular Malformation ◽  
Chronic Venous Insufficiency ◽  
Venous Insufficiency ◽  
Varicose Veins ◽  
Lower Limbs ◽  
Port Wine ◽  
Bone Deformity ◽  
Klippel Trenaunay Syndrome

Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.

Klippel–Trénaunay Syndrome and Developmental Coxa Vara in the Same Limb: A Case Report with a Review of the Literature

2021 ◽  
Author(s):  
Balaji Zacharia ◽  
Jittu Alex ◽  
Ashwin Rajmohan
Keyword(s):  
Lower Limb ◽  
Lower Limbs ◽  
Coxa Vara ◽  
Port Wine ◽  
Review Of The Literature ◽  
Left Femur ◽  
Port Wine Stains ◽  
Left Lower Limb ◽  
Klippel Trenaunay Syndrome

AbstractWe present a case of a 14-year-old girl who was first treated when she was 4 years old. She had progressive limping of the left lower limb from the age of 3 years. She was diagnosed to have developmental coxa vara of the left hip and treated by a subtrochanteric valgus osteotomy of the left femur. Later, she developed hypertrophy of the left upper and lower limbs. There were port-wine stains over the left lower limbs with multiple superficial varicosities. Her diagnosis was Klippel–Trénaunay syndrome (KTS). She is asymptomatic at the final follow-up. Both developmental coxa vara and KTS are rare conditions. We present this case to demonstrate the rarest combination of two rare conditions occurring in the same limb.

scholarly journals Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Trénaunay syndrome

2012 ◽  
Vol 140 (11-12) ◽  
pp. 777-781 ◽  
Author(s):  
Marko Spasic ◽  
Dragce Radovanovic ◽  
Dragan Canovic ◽  
Goran Azanjac ◽  
Predrag Djurdjevic ◽  
...  
Keyword(s):  
Congenital Anomaly ◽  
Soft Tissue ◽  
Female Patient ◽  
Port Wine Stain ◽  
Port Wine ◽  
Rare Congenital Anomaly ◽  
Vascular Origin ◽  
Accompanying Symptoms ◽  
Clinical Triad ◽  
Klippel Trenaunay Syndrome

Introduction. Klippel?Tr?naunay syndrome (KTS) is a very rare congenital anomaly of blood vessels, characterized by the following clinical triad: varicose superficial veins, port-wine stain and usually bony and soft tissue hypertophy of extremities, most often located in the lower extremities. It is often accompanied by visceral manifestations, and rarely combined with splenomegaly. Case Outline. A 30-year-old female patient came to the Surgery Clinic because of occasional left hypochondrial pain. After she was diagnosed with KTS combined with splenomegaly, splenectomy was performed. Macroscopic and microscopic spleen examination indicated the presence of tumor of vascular origin, presenting a combination of lymphangioma and hemangioma. Conclusion. Diagnosed KTS demands a thorough clinical examination of the patient because of the potential presence of visceral manifestations. When splenomegaly is present, even though being often benign, splenectomy is usually performed to alleviate accompanying symptoms which occur as a result of organ enlargement and compression, to prevent rupture and consequential bleeding when the vascular spleen tumor is large, and finally to avoid a possibility of malignant transformation.

scholarly journals Klippel Trenaunay Syndrome: A Brief Overview

2021 ◽  
Vol 5 (5) ◽  
pp. 454-461
Author(s):  
Pande Ayu Naya Kasih Permatananda ◽  
I Gusti Agung Made Adnyana Putra
Keyword(s):  
Soft Tissue ◽  
Somatic Mutations ◽  
Clinical Presentation ◽  
Invasive Procedure ◽  
Severe Bleeding ◽  
Port Wine ◽  
Non Invasive ◽  
Weber Syndrome ◽  
Vasculogenesis And Angiogenesis ◽  
Klippel Trenaunay Syndrome

Klippel-Trenaunay syndrome is a rare congenital vascular disease. The pathogenesis of this syndrome is unclear, but it is thought that most cases are the result of somatic mutations that affect genes that play a role in vasculogenesis and angiogenesis. Some patients come with a triad of capillary malformation (hemangioma or port-wine stain), venous varicosities and bony or soft tissue hypertrophy. Clinical presentation of this syndrome can lead to significant morbidities and mortalities due to severe bleeding and emboli. Although the number of cases is low, a doctor must be able to distinguish Klippel-Trenaunay Syndrome from other rare vascular disorders. Parkes Weber syndrome is usually similar to Klippel-Trenaunay syndrome, except in the arterial malformations associated with capillary malformations and soft tissue to skeletal or bone hypertrophy. The diagnosis of Klippel-Trenaunay Syndrome is carried out clinically and is quite difficult to do even with experienced doctors because there is no precise pathognomonic test.  There are several options in relation to the management of Klippel-Trenaunay Syndrome and non-invasive procedure is considered to be the most important of therapy modalities. Early diagnoses, progression monitoring, and proper intervention should be carried out for better prognosis and preventing complication.

scholarly journals Klippel Trenaunay Syndrome: A Brief Overview

2021 ◽  
Vol 5 (2) ◽  
pp. 387-394
Author(s):  
Pande Ayu Naya Kasih Permatananda ◽  
I Gusti Agung Made Adnyana Putra
Keyword(s):  
Soft Tissue ◽  
Vascular Disease ◽  
Clinical Presentation ◽  
Invasive Procedure ◽  
Port Wine Stain ◽  
Port Wine ◽  
Vascular Disorders ◽  
Non Invasive ◽  
Vasculogenesis And Angiogenesis ◽  
Klippel Trenaunay Syndrome

A B S T R A C TKlippel-Trenaunay syndrome is a rare congenital vascular disease. The pathogenesisof this syndrome is unclear, but it is thought that most cases are the result of somaticmutations that affect genes that play a role in vasculogenesis and angiogenesis. Somepatients come with a triad of capillary malformation (hemangioma or port-wine stain),venous varicosities and bony or soft tissue hypertrophy. Clinical presentation of thissyndrome can lead to significant morbidities and mortalities due to severe bleedingand emboli. Although the number of cases is low, a doctor must be able to distinguishKlippel-Trenaunay Syndrome from other rare vascular disorders. Parkes Webersyndrome is usually similar to Klippel-Trenaunay syndrome, except in the arterialmalformations associated with capillary malformations and soft tissue to skeletal orbone hypertrophy. The diagnosis of Klippel-Trenaunay Syndrome is carried outclinically and is quite difficult to do even with experienced doctors because there is noprecise pathognomonic test. There are several options in relation to the managementof Klippel-Trenaunay Syndrome and non-invasive procedure is considered to be themost important of therapy modalities. Early diagnoses, progression monitoring, andproper intervention should be carried out for better prognosis and preventingcomplication.

Treatment of Congenital Telangiectatic Vascular Malformations With the Pulsed-Dye Laser (585 nm)

PEDIATRICS ◽  
1993 ◽  
Vol 92 (4) ◽  
pp. 603-606
Author(s):  
JOSEPH C. MORELLI ◽  
J. CLARK HUFF ◽  
WILLIAM L. WESTON
Keyword(s):  
Natural History ◽  
Vascular Endothelium ◽  
Vascular Malformations ◽  
Benign Tumors ◽  
Port Wine ◽  
Port Wine Stains ◽  
Vascular Abnormalities ◽  
History Of ◽  
Phase Out ◽  
Regressive Phase

The two most common vascular abnormalities of childhood are port-wine stains and hemangiomas. Port-wine stains are congenital capillary malformations, whereas hemangiomas are benign tumors of vascular endothelium. The natural history of each of these lesions is distinct. Port-wine stains begin as discrete pink/red macules and grow proportionately with the child. As they mature they darken, and vascular ectasia and blebbing, as well as soft tissue and bony overgrowth of the affected area, can develop. Hemangiomas undergo a very predictable natural history, progressing through a rapid growth phase out of proportion to the child's growth, followed by a regressive phase and eventual involution in 90% of cases.

Copper vapour laser treatment of port-wine stains and other vascular malformations

1990 ◽  
Vol 43 (3) ◽  
pp. 273-282 ◽  
Author(s):  
John W. Pickering ◽  
E. Peter Walker ◽  
Philip H. Butler ◽  
Chris N. van Halewyn
Keyword(s):  
Laser Treatment ◽  
Vascular Malformations ◽  
Port Wine ◽  
Copper Vapour Laser ◽  
Port Wine Stains

scholarly journals A neonate with Klippel–Trénaunay syndrome: a case report 

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Franck Katembo Sikakulya ◽  
Walufu Ivan Egesa ◽  
Sonye Magugu Kiyaka ◽  
Philip Anyama
Keyword(s):  
Lower Limb ◽  
Varicose Veins ◽  
Color Doppler ◽  
Color Doppler Ultrasound ◽  
Port Wine ◽  
Black African ◽  
Port Wine Stains ◽  
Left Lower Limb ◽  
Clinical Triad ◽  
Klippel Trenaunay Syndrome

Abstract Background Klippel–Trénaunay syndrome is a rare congenital capillary–lymphatic–venous condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft-tissue hypertrophy. It has a very low incidence of about 1:100,000. Case presentation We report the case of 21-day-old neonate Black African female (born in Uganda) with Klippel–Trénaunay syndrome who presented with macrodactyly and ectrodactyly on the left foot, as well as numerous port wine stains on the left thoracoabdominal region and anteroposterior left lower limb. Color Doppler ultrasound examination of the left lower limb and abdomen revealed varicose veins without signs of arteriovenous fistula. Conclusion The report presents the case of a neonate with a rare congenital vascular disorder type Klippel–Trénaunay syndrome.

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