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.

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 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.

Causes of recurrent lower limb varicose veins after surgical interventions in 141 limbs – Five-year retrospective analysis of two centers

Vascular ◽  
2013 ◽  
Vol 22 (4) ◽  
pp. 267-273 ◽  
Author(s):  
Wang Rui Hua ◽  
Meng Qing Yi ◽  
Wu Xue Jun ◽  
Jin Xing ◽  
Liu Zhao Xuan ◽  
...  
Keyword(s):  
Lower Limb ◽  
Varicose Veins ◽  
Color Doppler ◽  
Color Doppler Ultrasound ◽  
Lower Limbs ◽  
Venous Reflux ◽  
Surgical Interventions ◽  
Venous Valves ◽  
Second Surgery ◽  
Clinical Changes

Aim The purpose of this study was to explore the causes of recurrent lower limb varicose veins after surgical interventions. Methods A retrospective five-year survey was conducted on patients who underwent second surgery due to recurrent lower limb varicose veins after surgical interventions. A total of 141 limbs (112 cases), including 72 cases of left lower limbs, 47 of right lower limbs and 22 of both limbs, were involved in the study. All patients underwent lower limb venography (141 limbs were anterograde and 28 cases were retrograde), and then examined with color-Doppler ultrasound. Results The major causes that urged patients to undergo second surgery are clinical changes graded above CEAP IV (93.6%), limb edema without changes on skin (5%), and single varicosity (1.4%). Up to 127 (83%) limbs exhibited perforating venous reflux, 67 (47.5%) limbs had varied degrees of deep venous insufficiency and 68 (48.2%) limbs had through or above-the-knee great saphenous vein trunk residual. Conclusions Preoperative venography before operation is indispensible in confirming the diagnosis and operation strategies. Patients with severe primary deep venous reflux and symptoms up to C3 may need simultaneous repair of the deep venous valves.

scholarly journals A rare association of angiokeratoma circumscriptum with Klippel Trenaunay syndrome

2017 ◽  
Vol 3 (2) ◽  
pp. 296
Author(s):  
Aarti S. Salunke ◽  
Ravindranath B. Chavan ◽  
Vasudha A. Belgaumkar ◽  
Pallavi P. Patil
Keyword(s):  
Lower Limb ◽  
Congenital Abnormalities ◽  
Lymphatic Vessels ◽  
Port Wine ◽  
Equal Distribution ◽  
Rare Association ◽  
Rare Congenital Disorder ◽  
Life Threatening ◽  
Clinical Triad ◽  
Klippel Trenaunay Syndrome

<p class="abstract"><span lang="EN-IN">Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of blood and lymphatic vessels that is characterised by a combined vascular malformation of the capillaries, veins and lymphatics, congenital abnormalities, and associated limb hypertrophy. It may also involve gastrointestinal tract which can lead to life threatening bleeding. This syndrome presents since birth to early infancy or childhood with equal distribution in both genders. An adolescent female presented with right lower limb hypertrophy with port wine stain and overlying hemorrhagic vesiclessince birth. Histopathology of these hemorrhagic vesicles angiokeratoma. On the basis of history and classical clinical triad patient was diagnosed as KTS with angiokeratoma, an association rarely reported in literature.</span></p>

scholarly journals Evaluation of pelvic varicose veins using color Doppler ultrasound: comparison of results obtained with ultrasound of the lower limbs, transvaginal ultrasound, and phlebography

2010 ◽  
Vol 9 (2) ◽  
pp. 15-23 ◽  
Author(s):  
Fanilda Souto Barros ◽  
José Maria Gomez Perez ◽  
Eliana Zandonade ◽  
Sérgio X. Salles-Cunha ◽  
Javier Leal Monedero ◽  
...  
Keyword(s):  
Doppler Ultrasound ◽  
Lower Limb ◽  
Varicose Veins ◽  
Color Doppler ◽  
Transvaginal Ultrasound ◽  
Color Doppler Ultrasound ◽  
Diagnostic Methods ◽  
Lower Limbs ◽  
Female Patients ◽  
Vascular Laboratory

Introduction: Pelvic varicose veins, one of the main causes of chronic pelvic pain and dyspareunia, are an important source of reflux for lower limb varicose veins, especially in recurrent cases. Color Doppler ultrasound of the lower limbs and transvaginal ultrasound are the noninvasive diagnostic methods most commonly used to assess pelvic venous insufficiency, whereas phlebography is still considered as the gold standard. Objectives: To determine the prevalence of lower limb varicose veins originating from the pelvis in a group of female patients and to determine the agreement between results obtained via color Doppler ultrasound of the lower limbs, transvaginal ultrasound, and phlebography. Methods: The sample comprised female patients referred to a vascular laboratory for lower limb screening. Patients diagnosed with deep venous thrombosis were excluded. Data analysis included kappa coefficient of agreement, McNemar's test, sensitivity and specificity values. Results: Of a total of 1,020 patients, 124 (12.2%) had findings compatible with reflux of pelvic origin. Among these patients, 51 (41.2%) were recurrent cases. A total of 249 were submitted to transvaginal ultrasound. There was significant agreement between lower limb ultrasonographic findings and transvaginal findings. Phlebography was performed in 54 patients. The comparison between transvaginal ultrasound and phlebography was associated with a 96.2% sensitivity and 100% specificity. Conclusions: The authors draw attention to the relatively high prevalence of lower limb varicose veins originating from the pelvis, suggesting an important but underdiagnosed cause of recurrent varicose veins.

scholarly journals Childhood Varicose Veins–An Unusual Visceral “May–Thurner” Like Compression

2021 ◽  
Author(s):  
Sreenivasa Narayana Raju ◽  
Rengarajan Rajagopal ◽  
Niraj Nirmal Pandey ◽  
Amarinder Singh ◽  
Sanjeev Kumar
Keyword(s):  
Lower Limb ◽  
Varicose Veins ◽  
Color Doppler ◽  
Left Kidney ◽  
Iliac Vein ◽  
Common Iliac Vein ◽  
Left Common Iliac Vein ◽  
Left Lower Limb ◽  
Limb Edema ◽  
Lateral Decubitus

AbstractWe report the case of 8-year-old girl with left lower limb edema due to superficial venous incompetence and varicosities. Color Doppler demonstrated compression of the left common iliac vein by an ectopic left kidney, which was partly relieved in right lateral decubitus position. CT demonstrated ectopic malrotated pelvic kidney, compressing the left common iliac vein against the L5 vertebra. A “May–Thurner” like syndrome due to visceral compression needs to be suspected in children with unilateral left lower limb varicosities.

Klippel-Trenaunay syndrome in a young patient with the involvement of gastrointestinal and genitourinary tracts: an unusual and rare presentation

2021 ◽  
Vol 14 (3) ◽  
pp. e239420
Author(s):  
Oseen Hajilal Shaikh ◽  
Uday Shamrao Kumbhar ◽  
Ankit Jain ◽  
Sunitha Vellathussery Chakkalakkoombil
Keyword(s):  
Lower Limb ◽  
Varicose Veins ◽  
Venous Malformations ◽  
Rare Presentation ◽  
First Case ◽  
Rare Congenital Disorder ◽  
Left Lower Limb ◽  
Bleeding Per Rectum ◽  
The Right ◽  
Klippel Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterised by capillary and venous malformations and soft-tissue hypertrophy with or without lymphatic malformation. The involvement of the gastrointestinal tract and genitourinary tract is uncommon in KTS. We present a case of a young adult who presented to us with bleeding per rectum and varicosities of the left lower limb. The patient was evaluated and diagnosed to have KTS. The patient was managed conservatively. To best of our knowledge, this is the first case report in literature where KTS was associated with atypical varicose veins of the left lower limb with mild hypertrophy of the lower limb, localised hypertrophy of the right gluteal region with involvement of pelvis, rectum, bladder and seminal vesicle.

Breast Hemangioma: Unique Presentation In a Patient with Klippel-Trenaunay-Syndrome

2021 ◽  
Vol 17 ◽  
Author(s):  
Ayesha Shamim Siddiqui ◽  
Ibtesam Zafar ◽  
Ayesha Isani Majeed ◽  
Ramish Riaz
Keyword(s):  
Varicose Veins ◽  
Accurate Diagnosis ◽  
Vascular Tumors ◽  
Port Wine ◽  
Case Presentation ◽  
Imaging Utilization ◽  
History Of ◽  
Low Incidence ◽  
Klippel Trenaunay Syndrome ◽  
Diagnosis Of Diseases

Background: Klippel–Trénaunay-Syndrome (KTS) is characterized by triad of varicose veins, port wine stain and soft tissue or bony hypertrophy and the diagnosis of KTS can be made if any two of these three features are present. Hemangiomas in various location e.g. skull, brain, epidural and vertebral hemangioma, mediastinal, colonic hemangioma, intraneural/intramuscular hemangiomas are reported with KTS. Case Presentation: Benign vascular tumors may rarely develop malignant transformation as Bugarin-Estrada et al reported breast angiosarcoma in a patient diagnosed as Klippel-Trenaunay-Syndrome. We reported a case of a 40-year-old female with known case of Klipple-Trenaunay-Syndrome with left leg varicosities, cutaneous nevus as well as unfortunate development of deep venous thrombosis and markedly enlarged right breast hemangioma. Due to low incidence or lack of early detection of breast hemangioma, its diagnosis is challenging. Conclusion: The history of patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.

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.

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