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.

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 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 Internal and External Radiofrequency Assisted Lipo-Coagulation (RFAL) in the Control of Soft Tissue Contraction during Liposuction: Part 2 “Outside In” RFAL Thermal Tissue Tightening

2021 ◽  
Author(s):  
Robert Stephen Mulholland
Keyword(s):  
Soft Tissue ◽  
Invasive Procedure ◽  
Thermal Stimulation ◽  
Thermal Coagulation ◽  
Soft Tissue Envelope ◽  
Non Invasive ◽  
Tissue Contraction ◽  
Contraction Effect ◽  
Positively Charged ◽  
Coated Electrodes

The new Morpheus8 is a novel external RFAL device that uses the proven soft tissue contraction of BodyTite in an external, non-invasive procedure. This external RF applicator, which is also powered by BodyTite, inserts up to 40 positively charged, coated electrodes 8 mm into the subcutaneous, soft tissue envelope. A monopolar ablative lesion is generated from the tip of the electrode, stimulating contraction of the FSN and adipose coagulation. The RF then flows up to the distant negative, return electrodes on the surface of the skin, providing a non-ablative thermal stimulation to the papillary dermis. The “burst” feature of the Morpheus8, delivers simultaneous multiple levels of internal coagulation in a single one second pulse, amplifying the adipose ablation and contraction effect. Studies, show, that the combination of BodyTite internal thermal coagulation and external Morpheus8 at the time of liposuction can result in 60–70% area skin contraction, greatly improving the soft tissue contours and Body shaping outcomes following lipo-contouring procedures.

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.

scholarly journals Overgrowth syndrome in neonates: a rare case series with a review of the literature

2019 ◽  
Vol 12 (1) ◽  
pp. e225640
Author(s):  
Aakash Pandita ◽  
Astha Panghal ◽  
Girish Gupta ◽  
Kirti M Naranje
Keyword(s):  
Lower Limb ◽  
Rare Case ◽  
Case Series ◽  
Port Wine Stain ◽  
Port Wine ◽  
Review Of The Literature ◽  
Term Neonates ◽  
Overgrowth Syndrome ◽  
Klippel Trenaunay Syndrome

We present here two-term neonates presenting with right lower limb hypertrophy, a port-wine stain, acral abnormalities and clubfeet. These neonates had overlapping features of Klippel Trenaunay syndrome and congenital lipomatous overgrowth, vascular malformation, epidermal nevi and scoliosis/skeletal abnormalities. Such overgrowth syndrome has not been previously described in the literature. Both the neonates are doing well and are under regular follow-up.

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.

Glaucoma in children with facial port wine stain

2018 ◽  
Vol 30 (1) ◽  
pp. 168-174 ◽  
Author(s):  
Nader Hussein Lotfy Bayoumi ◽  
Eman Nabil Elsayed
Keyword(s):  
Success Rate ◽  
Chart Review ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Retrospective Chart Review ◽  
Port Wine Stain ◽  
Port Wine ◽  
Intravitreal Gas ◽  
Paediatric Ophthalmology

Purpose: To report on the clinical presentation and surgical treatment (procedure and outcome(s)) of glaucoma in children with facial port wine stain. Materials and methods: This is a retrospective chart review of children with facial port wine stain referred to Alexandria University paediatric ophthalmology practice from 2005 to 2016. The charts of 22 children (44 eyes) with facial port wine stain were reviewed. The data extracted included demographics, results of ophthalmic examination findings and treatment(s). The main outcome measures were the number of eyes stratified as glaucoma, glaucoma suspects and no glaucoma at the initial and final presentations. Results: The average age of presentation was 18.2 (±33.9) months. After a follow-up of over 16.1 (±24.8) months, there were 34%, 30% and 36% of the study eyes diagnosed as glaucoma, glaucoma suspects and no glaucoma, respectively with mean ± standard deviation of intraocular pressure of 20.6 ± 5.1, 13.6 ± 5.4 and 7.5 ± 1.7 mmHg. The majority (91%) of eyes presenting with glaucoma had clear corneas. In total, 11 eyes were operated upon for glaucoma. The recorded success rate was 91%. Two eyes developed a postoperative exudative choroidal detachment, of which one resolved spontaneously and the other was successfully managed by intravitreal gas injection. Conclusion: Glaucoma is a significant ocular hazard in children with facial port wine stain that may not be evident on the initial presentation. The presentation is usually with a clear cornea and surgical intervention is associated with a high success rate and a low rate of complications.

scholarly journals Gingival Tissue Alterations in 2 Patients with Port Wine Stain

2014 ◽  
Vol 18 (2) ◽  
pp. 109-112
Author(s):  
Ayse Kocak-Buyukdere ◽  
Esra Guzeldemir
Keyword(s):  
Soft Tissue ◽  
Gingival Tissue ◽  
Port Wine Stain ◽  
Port Wine ◽  
Wisdom Tooth ◽  
Upper Jaw ◽  
Vascular Birthmarks ◽  
The Right ◽  
Tissue Alterations ◽  
Nevus Flammeus

SUMMARYNevus flammeus, which is also known as port-wine stain (PWS), is one of the vascular birthmarks. PWS occurs in 0.3% of the newborns in both genders. It is a capillary vascular malformation, characterized by a pink or red stain and may involve skin, soft tissue and/or bone. There are a very limited number of reports regarding intraoral involvement of PWS. We report 2 female patients with PWS from date of birth. The first patient was an 11-year-old female who applied to our clinics for the treatment of her non-aesthetic and deviated intraoral view and discoloration of her gingiva, and the second patient was a 56-year-old female who applied for the extraction of her wisdom tooth. Extraoral examination in both patients revealed a diffuse PWS on the right side of their face over the cheek, extending from the midline. While the first patient had reddish skin, gingiva on right site her both jaws and lips, the second patient had only her upper jaw and lip. Because of the first patient’ age, the treatment postponed to her 20’, and the second patient did not accept any treatment. PWS is a rare and non-fatal condition; however, the unique appearance of these patients can lead to psychological problems especially in early ages.

scholarly journals Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain

2017 ◽  
Vol 58 (11) ◽  
pp. 1234
Author(s):  
Mi Jin Kim ◽  
Won June Lee ◽  
Ki Ho Park ◽  
Tae-Woo Kim ◽  
Eun Ji Lee ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Port Wine Stain ◽  
Port Wine
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