A rare association of angiokeratoma circumscriptum with 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>

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A neonate with Klippel–Trénaunay syndrome: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-03029-4 ◽

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

BMJ Case Reports ◽

10.1136/bcr-2018-225640 ◽

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

International Journal of Angiology ◽

10.1055/s-0041-1729921 ◽

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.

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

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh1212777s ◽

2012 ◽

Vol 140 (11-12) ◽

pp. 777-781 ◽

Cited By ~ 5

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.

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An unusual evolution of a case of Klippel-Trenaunay syndrome

Open Medicine ◽

10.1515/med-2015-0084 ◽

2015 ◽

Vol 10 (1) ◽

Author(s):

Valter Martino ◽

Alessia Ferrarese ◽

Borello Alessandro ◽

Silvia Marola ◽

Alessandra Surace ◽

...

Keyword(s):

Soft Tissue ◽

Lower Limb ◽

Unusual Case ◽

Varicose Veins ◽

Congenital Disorder ◽

Venous Ulcers ◽

Rare Congenital Disorder ◽

Klippel Trenaunay Syndrome ◽

Treatment Of Infection

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.

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Klippel–Trenaunay Syndrome: Current Management

Phlebology The Journal of Venous Disease ◽

10.1177/026835550702200611 ◽

2007 ◽

Vol 22 (6) ◽

pp. 291-298 ◽

Cited By ~ 2

Author(s):

P Glovkzki ◽

D J Driscoll

Keyword(s):

Multidisciplinary Approach ◽

Inferior Vena ◽

Vena Cava ◽

Venous Drainage ◽

Port Wine ◽

Current Management ◽

Arteriovenous Shunting ◽

Endovenous Thermal Ablation ◽

Clinical Triad ◽

Klippel Trenaunay Syndrome

Klippel–Trenaunay syndrome (KTS) is a rare, sporadic, complex malformation characterized by the clinical triad of (1) capillary malformations (port wine stain); (2) soft tissue and bone hypertrophy or, occasionally, hypotrophy of usually one lower limb; and (3) atypical, mostly lateral varicosity. KTS is a mixed vascular malformation, with predominant capillary, venous and lymphatic components, without significant arteriovenous shunting. Management is largely conservative and the extent of diagnostic evaluation is determined by the planned treatment. Compression is the hallmark of conservative management; laser can be used to treat port wine stains. Imaging before vascular interventions must confirm venous anatomy and deep venous drainage. Techniques for ablation of superficial veins and malformations are individualized and may include sclerotherapy with alcohol or foam, endovenous thermal ablation or, as used most frequently in our practice, surgical stripping and phlebectomy. Intraoperative use of tourniquet will decrease bleeding, selective use of an inferior vena cava filter will prevent pulmonary embolism. A multidisciplinary approach to management of KTS is warranted.

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Case Report: Life-Threatening Post-operative Hemorrhage in Klippel–Trenaunay Syndrome Associated With Hypofibrinogenemia

Frontiers in Medicine ◽

10.3389/fmed.2021.669793 ◽

2021 ◽

Vol 8 ◽

Author(s):

Hongna Yang ◽

Binfeng Du ◽

Han Liu ◽

Yong Yao ◽

Chen Li

Keyword(s):

Corpus Luteum ◽

Conservative Therapy ◽

Postoperative Bleeding ◽

Rare Condition ◽

Reproductive Age ◽

Bleeding Complications ◽

Rare Congenital Disorder ◽

First Choice ◽

Life Threatening ◽

Klippel Trenaunay Syndrome

Klippel–Trenaunay Syndrome (KTS) is a rare congenital disorder, characterized by venous and lymphatic malformations of the skin, soft tissue, and bone, causing limb hypertrophy. Although, a ruptured hemorrhagic corpus luteum is a rare condition in women of reproductive age, it can result in lethal outcomes. Here, we have described a patient with KTS and hypofibrinogenemia who went through recurrent lethal postoperative bleeding due to a ruptured hemorrhagic corpus luteum. This case suggested that conservative therapy might be the first choice and effective therapy for the patients with KTS, who suffered from bleeding complications of surgical therapy.

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

BMJ Case Reports ◽

10.1136/bcr-2020-239420 ◽

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.

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Effect of Punarnavadi Mandoor and Shiva Gutika in Acute Deep Vein Thrombosis - A Case Report

Journal of Ayurveda and Integrated Medical Sciences (JAIMS) ◽

10.21760/jaims.v2i4.9371 ◽

2017 ◽

Vol 2 (4) ◽

Author(s):

Wasedar Vishwanath S. ◽

Pusuluri YVSM Krishna ◽

Dani Harshikha

Keyword(s):

Lower Limb ◽

Blood Clot ◽

Acute Case ◽

Vein Thrombosis ◽

Oral Medications ◽

Doppler Study ◽

Brown Discoloration ◽

Left Lower Limb ◽

Life Threatening ◽

Deep Vein

Objectives: To minimise the dose of Anti-platelet drugs and to treat the acute case of DVT through Ayurvedic oral medications. Methods: The present diagnosed case of DVT approached to OPD of KLE BMK Ayurveda Hospital with a complaints of swelling and pain in the calf muscle of the left lower limb associated with reddish brown discoloration in the foot and occasionally nasal and gum bleeding was treated consequently for 5 months with Punarnavadi Mandoor and Shiva Gutika orally. Results: There is significant decrease in the symptoms of DVT and also major changes seen in Venous Colour Doppler study of the left lower limb. Conclusion: Acute DVT is caused by a blood clot in a deep vein and can be life threatening as it may leads to serious complication like pulmonary embolism which can be cured through Ayurvedic oral medications.

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