Dr Edoardo Cervi

Dr Maurizio Ronconi

Clinica Chirurgica - Spedali Civili di Brescia

Recurrence of Klippel-Trenaunay syndrome symptoms after surgery a single case

treated with foam sclerotherapy

HISTORYIn 1900 the French physicians Maurice Klippel and Paul Trenaunay described 2 patients with a port-wine stain and varicosoties of an extemity associated with soft issue and bone hypertrophy and coined the term ldquonaevus vasculosus osteohypertrophicusrdquo

In 1907 the eminent London physician F Parkers Weber described a patient with 3 aforerementioned symptoms as well as an arteriovenous malformation of the affected extremity He termed the process hemangiectatic hypertrophy

Thus 1918 is became know Klippel-Trenaunay-Weber syndrome

Today conflicting opinion exist in the literature as whether to separately designate the original triad as Klippel Trenaunay and the triad with arteriovenous malformation as Parkes Weber syndrome

Definition

congenital mixed vascular malformation

bull with anomalous vascular elements veins capillaries

lymphatics

bull with abnormal bone growth

bull also called ldquoangio-osteo-hypertrophic syndromerdquo

(AOH)1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

Epidemiology

Rare (prevalence 1-9 1 000 000)

About 1000 cases

No difference about sex

No difference about race

1 wwworphanet

Etiology

Unknown

Some familiar cases with autosomal dominant

inheritance

Intrauterine damage to the sympathetic ganglia

1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-589

5 Klippel-Trenaunay syndrome diagnostic criteria and hypotesis on etiology C E Oduber C M van der Horst R C Hennekam Ann Plast Surg 2008 60(2) 217-223

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

HISTORYIn 1900 the French physicians Maurice Klippel and Paul Trenaunay described 2 patients with a port-wine stain and varicosoties of an extemity associated with soft issue and bone hypertrophy and coined the term ldquonaevus vasculosus osteohypertrophicusrdquo

In 1907 the eminent London physician F Parkers Weber described a patient with 3 aforerementioned symptoms as well as an arteriovenous malformation of the affected extremity He termed the process hemangiectatic hypertrophy

Thus 1918 is became know Klippel-Trenaunay-Weber syndrome

Today conflicting opinion exist in the literature as whether to separately designate the original triad as Klippel Trenaunay and the triad with arteriovenous malformation as Parkes Weber syndrome

Definition

congenital mixed vascular malformation

bull with anomalous vascular elements veins capillaries

lymphatics

bull with abnormal bone growth

bull also called ldquoangio-osteo-hypertrophic syndromerdquo

(AOH)1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

Epidemiology

Rare (prevalence 1-9 1 000 000)

About 1000 cases

No difference about sex

No difference about race

1 wwworphanet

Etiology

Unknown

Some familiar cases with autosomal dominant

inheritance

Intrauterine damage to the sympathetic ganglia

1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-589

5 Klippel-Trenaunay syndrome diagnostic criteria and hypotesis on etiology C E Oduber C M van der Horst R C Hennekam Ann Plast Surg 2008 60(2) 217-223

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Definition

congenital mixed vascular malformation

bull with anomalous vascular elements veins capillaries

lymphatics

bull with abnormal bone growth

bull also called ldquoangio-osteo-hypertrophic syndromerdquo

(AOH)1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

Epidemiology

Rare (prevalence 1-9 1 000 000)

About 1000 cases

No difference about sex

No difference about race

1 wwworphanet

Etiology

Unknown

Some familiar cases with autosomal dominant

inheritance

Intrauterine damage to the sympathetic ganglia

1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-589

5 Klippel-Trenaunay syndrome diagnostic criteria and hypotesis on etiology C E Oduber C M van der Horst R C Hennekam Ann Plast Surg 2008 60(2) 217-223

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Epidemiology

Rare (prevalence 1-9 1 000 000)

About 1000 cases

No difference about sex

No difference about race

1 wwworphanet

Etiology

Unknown

Some familiar cases with autosomal dominant

inheritance

Intrauterine damage to the sympathetic ganglia

1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-589

5 Klippel-Trenaunay syndrome diagnostic criteria and hypotesis on etiology C E Oduber C M van der Horst R C Hennekam Ann Plast Surg 2008 60(2) 217-223

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Etiology

Unknown

Some familiar cases with autosomal dominant

inheritance

Intrauterine damage to the sympathetic ganglia

1 wwworphanet

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983

4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-589

5 Klippel-Trenaunay syndrome diagnostic criteria and hypotesis on etiology C E Oduber C M van der Horst R C Hennekam Ann Plast Surg 2008 60(2) 217-223

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Etiology

Hypotesis

Heterozygous inactivating RASA1 mutations (locus CMC1

chromosome 5q)

Mosaic gene abnormality

Chromosome translocation (511)

Mutation E113K

Mutated angiogenic factor VSG5

Altered level of angiopoietin-2 antagonist of VEGF

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

It belongs to arteriovenous malformations

ISSVA ndash International Society for the Study of Vascular Anomalies- classification (Rome 1996) 3

Vascular malformations

simple complexCapillary (C) A-V fistula

Venous (V) A-V malformations

Lymphatics (L) CVLM

Arterious (A) CVM

LVM

CAVM

CLAMV

KTS is the most frequent type of complex malformation seen at Vascular Malformation Classification

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Clinical features Classical triad of

1- capillary malformations (port wine stain)

2- soft tissue and bone hypertrophy or occasionally

hypotrophy (1 or more limbs)

3- atypical mostly lateral varicosity

but wide spectrum of presentation

(also incomplete forms)

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Clinical features

Main symptoms

hemangiomas lymphangiomas varicosity

different length of the limbs (up to 10 cm)

haemorrhage ( rectal bledding intracerebral or

intraspinal hematuria )

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Clinical features

2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-2983 4 Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome a literature search and report of two cases M Rohany A Shabani O Arafat et al AJNR 2007 28 584-5896 Klippel-Trenaunay syndrome incidence and treatment of genitourinary sequelae D A Husmann S R Rathburn D J Driscoll J Urol 2007 177(4) 1244-12497 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-668 Urogenital involvement in the Klippel-Trenaunay-Weber syndrome Treatment options and results F C Vicentini F T Denes C M Gomes International Braz J Urol 2006 32(6) 697-7049 The Klippel-Trenaunay syndrom associated with multiple visceral arteries aneurysms S Pourhassan D Grotemeyer V Klar et al Vasa 2007 36(2) 124-129

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Diagnosis

Differential diagnosis

Parkes-Weber syndrome (high-flow high-shunt

arteriovenous malformations)

vein dysplasia

lymphedema

bone tumours

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Diagnosis

Prenatal diagnosis US

cutaneoussubcutaneous cystic or multicystic lesions

asymmetric limb hypertrophy

limb edema

cardiomegaly

hydrops

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Diagnosis Clinical examination of the limbs

X- rays of the long bones

Duplex scanning

MRI

CT

Venography1 wwworphanet2 Klippel-Trenaunay syndrome current management P Gloviczi D J Driscoll Phlebology 2007 22 291-29837 Lower gastrointestinal bleeding hematuria and splenic hemangiomas in Klkippel-Trenaunay syndrome a case report and literature review O Kocaman A Alponat C Aygun et al Turk J Gastroenterol 2009 20(1) 62-6610 Prenatal diagnosis and hemodynamic evaluation of Klippel-Trenaunay-Weber syndrome D Paladini A Lamberti A Teodoro et al Ultrasound Obstet Gynecol 1998 12 215-21711 Klippel-Trenaunay syndrome M Samimi G Lorette Presse Med 2010

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Clinical features

Venous malformations due to persistence of embryonic

veins

lateral marginal vein or vein of Servelle

sciatic vein

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Management

bull Compression (is the hallmark of conservative management)

bull Surgical treatment

bull Subfascial endoscopic perforator surgery (SEPS)

bull Selective endovenous thermal ablation

bull Sclerotherapy with foam

bull Deep vein reconstructions (rare cases)

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Treatment of Venous MalformationsWith Sclerosant in Microfoam FormJuan Cabrera MD Juan Cabrera Jr MPharm Ma Antonia Garcıa-Olmedo DPharm Pedro Redondo MD PhDVascular Surgery Clinic Granada SpainArch Dermatol 2003 Nov139(11)1494-6

Efficacy and safety of microfoam sclerotherapy in a patient with Klippel-Trenaunay syndrome and a patent foramen ovaleRedondo P Bastarrika G Sierra A Martiacutenez-Cuesta A Cabrera JUnit of Vascular Malformations Department of Dermatology University Clinic of Navarra 31008 Pamplona Spain predondounavesArch Dermatol 2009 Oct145(10)1147-51

Venous angiomata treatment with sclerosant foamPascarella L Bergan JJ Yamada C Mekenas LDepartment of Bioengineering University of California San Diego La Jolla CA USAAnn Vasc Surg 2005 Jul19(4)457-64

Case report

bull Inferior limb varix at birth

bull 2 years old arthrocentesis right knee

bull 4 years old arthrocentesis left knee

bull 7 years old impair of function and left inferior limb pain

ndash angiography (1982) small angiodysplasia new formation nourrished by a

muscular tract of deep femoral artery at its middle third second new

formation in the soft tissue of the knee nourrished by genicolar lateral

inferior a and by genicolar lateral superior a

bull The patient is underwent a multiple surgical excisions of

angiodysplasias

Case report

bull A 20 years old the patient presented with large varicosities

swelling and pain

bull Treated between June 2006 and June 2008 with ultrasound-

guided foam sclerotherapy (USFS)

bull Sclerosant agent Polidocanol 3

bull Foam produced by Tessarirsquos tecnique

bull Number of sessions 20

bull Dose utilized for each session 4 - 8cc

Case report

No major complications were encountered

The patient reported improvement in signs and symptoms

The patient was very satisfied with the cosmetic results

Conclusion 1

1 KTS is a mixed malformation with a vascular component

2 The management is complex and involves

bull predictionprediction of the disease (genetic evaluation)

bull evaluationevaluation with diagnostic studies

bull preventionprevention of vascular and orthopaedic compliacations

bull treatmenttreatment of clinical manifestations

3 Sclerosant foam is a satisfactory tool to use in treating venous

angiomata including the Klippel-Trenaunay syndrome

4 Use of foam sclerotherapy in this experience has proven the tecnique

to be effective pain free and durable in the short term

Conclusion 2

Case report

bull A 20 years old the patient presented with large varicosities

swelling and pain

bull Treated between June 2006 and June 2008 with ultrasound-

guided foam sclerotherapy (USFS)

bull Sclerosant agent Polidocanol 3

bull Foam produced by Tessarirsquos tecnique

bull Number of sessions 20

bull Dose utilized for each session 4 - 8cc

Case report

No major complications were encountered

The patient reported improvement in signs and symptoms

The patient was very satisfied with the cosmetic results

Conclusion 1

1 KTS is a mixed malformation with a vascular component

2 The management is complex and involves

bull predictionprediction of the disease (genetic evaluation)

bull evaluationevaluation with diagnostic studies

bull preventionprevention of vascular and orthopaedic compliacations

bull treatmenttreatment of clinical manifestations

3 Sclerosant foam is a satisfactory tool to use in treating venous

angiomata including the Klippel-Trenaunay syndrome

4 Use of foam sclerotherapy in this experience has proven the tecnique

to be effective pain free and durable in the short term

Conclusion 2

Case report

No major complications were encountered

The patient reported improvement in signs and symptoms

The patient was very satisfied with the cosmetic results

Conclusion 1

1 KTS is a mixed malformation with a vascular component

2 The management is complex and involves

bull predictionprediction of the disease (genetic evaluation)

bull evaluationevaluation with diagnostic studies

bull preventionprevention of vascular and orthopaedic compliacations

bull treatmenttreatment of clinical manifestations

3 Sclerosant foam is a satisfactory tool to use in treating venous

angiomata including the Klippel-Trenaunay syndrome

4 Use of foam sclerotherapy in this experience has proven the tecnique

to be effective pain free and durable in the short term

Conclusion 2

Conclusion 1

1 KTS is a mixed malformation with a vascular component

2 The management is complex and involves

bull predictionprediction of the disease (genetic evaluation)

bull evaluationevaluation with diagnostic studies

bull preventionprevention of vascular and orthopaedic compliacations

bull treatmenttreatment of clinical manifestations

3 Sclerosant foam is a satisfactory tool to use in treating venous

angiomata including the Klippel-Trenaunay syndrome

4 Use of foam sclerotherapy in this experience has proven the tecnique

to be effective pain free and durable in the short term

Conclusion 2

3 Sclerosant foam is a satisfactory tool to use in treating venous

angiomata including the Klippel-Trenaunay syndrome

4 Use of foam sclerotherapy in this experience has proven the tecnique

to be effective pain free and durable in the short term

Conclusion 2