Tracheal Varicose Veins Associated with Klippel–Trenaunay Syndrome

2021 ◽  
Author(s):  
Keika Mukaihara ◽  
Kohei Godai ◽  
Takahiro Moriyama
Author(s):  
Ayesha Shamim Siddiqui ◽  
Ibtesam Zafar ◽  
Ayesha Isani Majeed ◽  
Ramish Riaz

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.


Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri

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.


Ultrasound ◽  
2019 ◽  
Vol 28 (2) ◽  
pp. 91-102
Author(s):  
Olga Ivanitskaya ◽  
Elena Andreeva ◽  
Natalia Odegova

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.


2005 ◽  
Vol 20 (2) ◽  
pp. 63-81 ◽  
Author(s):  
B McDonagh ◽  
S Sorenson ◽  
A Cohen ◽  
T Eaton ◽  
D E Huntley ◽  
...  

Objective: To study the clinical profile of Klippel–Trenaunay syndrome (KTS) and the management of venous malformations (VMs) with ultrasound-guided foam sclerotherapy using the compass technique. Methods: In this open prospective series, 11 consecutive patients with KTS seeking therapy at a phlebology group practice were included. Seven (64%) of them were post-surgical recurrences. Results: Pain was the commonest presenting complaint (100%), accompanied with VMs/ varicose veins in all except one (91%). Chronic venous insufficiency (CVI) was seen in seven (64%). Soft tissue and bone deformity was present in all (100%). Limb lengthening was frequent and detected in eight (73%), thickening in six (54%); unusually, one patient (9%) presented with severe thinning and shortening of the leg. Venous malformation was the primary vascular problem in nine patients (82%), with capillary malformation (CM)/port wine stain in seven (64%) and isolated lymphatic malformation (LM) in two (18%). The lateral embryonic/Klippel–Trenaunay (KT) vein was detected in 10 patients (91%). Deep vein deformity was not detected in any of them. All the deformities were of the lower limb (100%), while two (18%) had deformities of the thorax and one (9%) had gastrointestinal (GI) malformation. None of the patients gave family history of KTS, while five (45%) had history of varicosities in the family. Eight patients (73%) completed the therapeutic plan with a mean follow-up of 5±3.9 years. An excellent to good type result could be documented in six of the eight cases (75%). These patients had an event-free, good quality of life. Follow-up duplex studies revealed the evolving refluxing tracts requiring sequential therapies. Conclusions: The results in this series demonstrate the utility of foam sclerotherapy using the compass technique in the management of KTS. Chronicity of the VMs in KTS necessitates strict clinical monitoring and sequential therapies.


Open Medicine ◽  
2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Valter Martino ◽  
Alessia Ferrarese ◽  
Borello Alessandro ◽  
Silvia Marola ◽  
Alessandra Surace ◽  
...  

Abstract Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder. KTS can be diagnosed on the basis of any 2 of 3 features: cutaneous capillary malformations, soft tissue or bony hypertrophy and varicose veins. We present an unusual case of KTS complicated by an infection of venous ulcers of the lower limb by larvae. The treatment of infection was a complete debridement; however baseline treatment of KTS is still in evaluation.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Franck Katembo Sikakulya ◽  
Walufu Ivan Egesa ◽  
Sonye Magugu Kiyaka ◽  
Philip Anyama

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.


2013 ◽  
Vol 33 (2) ◽  
pp. 147-149
Author(s):  
Shatanik Sarkar ◽  
Chaitali Patra ◽  
Chandrasekhar Dey ◽  
Malay Kumar Dasgupta ◽  
Tapan Kumar Kundu

Sturge Weber syndrome (SWS) is a mesodermal phakomatosis characterized by meningo-facial angiomas with cerebral calcification. Klippel Trenaunay syndrome (KTS) is another very rare type of phakomatosis with cutaneous angiomas, varicose veins and enlargement of soft tissue or bones. Overlap between SWS & KTS is very rarely encountered. We report a three and half year old girl with overlapping features of both SWS and KTS. DOI: http://dx.doi.org/10.3126/jnps.v33i2.7704 J Nepal Paediatr Soc. 2013; 33(2):147-149


2015 ◽  
Vol 31 (3) ◽  
pp. 209-215 ◽  
Author(s):  
Rafael D Malgor ◽  
Peter Gloviczki ◽  
Jennifer Fahrni ◽  
Manju Kalra ◽  
Audra A Duncan ◽  
...  

Background Klippel–Trenaunay syndrome (KTS) is a mixed mesenchymal malformation characterised by varicose veins, venous and capillary malformations, and hypertrophy of soft tissue and bone. The purpose of this study was to evaluate the surgical outcomes in KTS patients to provide standards for comparison with endovenous therapy. Methods The clinical data of consecutive patient with KTS who underwent open venous surgical treatment between January 1987 and December 2008 were reviewed. Demographics, clinical presentation, operative data, and clinical outcomes were recorded. Follow-up information was obtained from the medical records, mailed questionnaires and phone calls. Descriptive statistics, the Kaplan–Meier method and Log-rank statistics were used where appropriate. Results Twenty-seven females and 22 males, (mean age 26.5 years, range 7.7–55.8) were included in this study. All had varicose veins, 36 (73%) had limb hypertrophy, and 33 (67%) had capillary malformations, with two of three clinical features present in all. The most frequent symptom was pain (N = 43, 88%). Forty-nine patients underwent operations on 53 limbs. Stripping of the GSV, small and accessory saphenous and lateral embryonic veins was performed in 17 (32%), 10 (19%), 9 (17%), and 15 (28%) limbs, respectively. Two patients developed deep vein thrombosis, one had pulmonary embolism (PE), and one patient had peroneal nerve palsy. Freedom from disabling pain at 1, 3 and 5 years was 95%, 77% and 59%, respectively, and freedom from secondary procedures was 78% at 3 years, and 74% at 5 years. At the last follow-up visit, the venous clinical severity score had decreased from 9.48 ± 3.27 to 6.07 ± 3.20 (P < 0.001). Conclusions In selected symptomatic patients with KT syndrome, open surgical treatment is safe and durable. Three-fourths of the patients remain free of disabling pain at five years, but secondary procedures are required in one-fourth of the patients. These data can serve as standards for comparison of endovenous therapy for KT syndrome.


Sign in / Sign up

Export Citation Format

Share Document