Term
| KTS syndrome is designated how in the new classification system? |
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Definition
| As a "mixed" vascular malformation |
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Term
| Name the three components of KTS Syndrome |
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Definition
1. Capillary malformations (Port wine stain) 2. Soft tissue and bone hypertrophy or occasionally hypotrophy of a limb 3. Atypical, mostly lateral varicosity |
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Term
| KTS is a mixed vascular malformation. What are its mixed components? |
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Definition
| Capillary, venous and lymphatic |
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Term
| What distinquishes KTS syndrome from Parkes Weber Syndrome? |
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Definition
| The absence of significant arteriovenous shunting |
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Term
| Where is the embryologic defect in KTS syndrome |
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Definition
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Term
| Is KTS Syndrome an inherited abnormality? |
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Definition
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Term
Describe the most common venous malformation in KTS Syndrome Describe the particular anatomy of this vein Where does it drain? What is another persistent embryonic vein in KTS Syndrome? |
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Definition
Persistence of embryonic veins with the lateral marginal vein most typical Thick walled, immediately under the skin with absent valves Either into lateral branch of profunda femoris or internal iliac vein Sciatic vein
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Term
| In KTS Syndrome name some major venous malformation of clinical signicance |
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Definition
Absence of the deep venous system Absence of internal jugular veins and IVC |
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Term
| Describe some common soft tissue abnormalities in KTS Syndrome |
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Definition
| Localized mass on back or chest or diffuse mass involving an entire arm or leg |
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Term
| Describe the typical bony abnormality in KTS Syndrome |
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Definition
| Overgrowth of a limb, occasionally a shorter smaller limb |
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Term
| Name some clinical presentations (complications) in KTS Syndrome |
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Definition
Chronic pain syndrome Skin infections thromboembolism and life threatening pelvic or rectal bleeding |
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Term
| What is the mainstay in rx of KTS Syndrome? |
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Definition
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Term
| How are the capillary malformations treated in KTS Syndrome? |
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Definition
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Term
| What is the key principle prior to vascular interventions in KTS Syndrome? |
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Definition
| Determing patency of the deep venous system |
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Term
| Name some therapeutic interventions used in the rx of KTS Syndrome |
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Definition
foam and ethanol sclerotherapy Vein stripping endovenous ablation Preop IVC filter Ambulatory phlebectomy |
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Term
In the new Hamburg classification system, what category(nomenclature) of abnormalities is being standardized? What is its abbreviation? Name one of the most common types and its abbreviation What similiar abnormality is not part of the classification system? |
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Definition
Congenital vascular malformations CVM Venous malformations or VM neonatal or infantile hemangiomas which are a vascular tumor |
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Term
| Define a congenital vascular malformation |
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Definition
| is a malformed vessel that results from developmental arrest during embryogenesis and continues to grow at a rate proportional to the growth rate of the body |
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Term
From what do neonatal hemangiomas arise? How do they differ in growth from CVM'S What term should no longer be used to avoid confusion with CVM's? |
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Definition
They arise from endothelial cells They are vascular tumors with a rapid growth phase followed by involutional phase Cavernous or capillary hemangioma |
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Term
In the new classification system what are the vascular malformation components of KTS and PWS defined as? What is its abbreviation? |
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Definition
The vascular malformation components of KTS and PWS are defined as hemolymphatic malformations or HLM's |
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Term
| The congenital vascular malformation (CVM) component of KTS is a HLM consisting of what malformations and what are their abbreviations? |
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Definition
Venous malformation VM Lymphatic malformation LM Capillary malformation CM |
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Term
| The congenital vascular malformation (CVM) component of Parke Weber Syndrome is a HLM consisting of what malformations and what are their abbreviations? |
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Definition
Venous malformation VM Lymphatic malformation LM Capillary malformation CM Arteriovenous shunting malformation AVM |
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Term
| Congenital vascular malformations are classified into one five types based on which predominant component? |
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Definition
Classification based on predominant vascular component 5 types: Arterial Venous VM Lymphatic Arteriovenous shunting AVM Mixed |
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Term
What is the most common mixed CVM? |
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Definition
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Term
| Besides its predominant vascular component how else are CVM's classified? |
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Definition
One of two forms based on its embryological development stage Truncal Extratruncal |
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Term
Describe the extratruncal form of CVMS its embryological origin, stage of developmental arrest. Why are these lesions clinically important? What is their greatest risk? |
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Definition
Embryonic tissue remnants of mesodermal origin Developmental arrest is in early stage of embryonic life They retain their potential to grow and proliferate when stimulated internally eg pregnancy, hormones They have a significant risk of reoccurence if undertreated |
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Term
What are the two types of extratruncal lesions? |
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Definition
Infiltrating (diffuse) and Limited, localized lesion |
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Term
| What is a potential side effect of a limited, localized extratruncal lesion? |
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Definition
| Mechanical compression to surrounding tissues and organs |
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Term
Describe the truncal form of CVMS its embryological origin, stage of developmental arrest. Why are these lesions clinically important? What is their greatest risk? |
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Definition
| They have lost the embryonic tissue remnants of mesodermal origin Developmental arrest is in later stages of embryonic life They no longer retain their potential to grow and proliferate They do not have a risk of reoccurence They have greater hemodynamic consequences |
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Term
How do truncal CVM's most often present? Give some examples |
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Definition
Present as a persistant fetal remnant (truncal) vessel that does not involute normally Present as a formed vessel with various degrees of developmental defect eg agenesis, aplasia, hyperplasia Sciatic vein, vein web,venous aneurysm |
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Term
| What are the two subclassifications of truncal CVM's |
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Definition
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Term
| What is the most common congenital vascular malformation and how does it present? |
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Definition
| The most common CVM is a venous malformation,VM,which presents as a single lesion |
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Term
Approximately what % of venous malformations VM'S are mixed lesions? |
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Definition
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Term
| What test should always be performed on a patient with a marginal vein? |
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Definition
| Dynamic lymphoscintigraphy |
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Term
In surgical treatment of the marginal vein What is Rx dependent on and explain the options Why is closed stripping not recommended Is laser closure an option? Why? |
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Definition
The status of the deep vein. In hypoplasia of the the deep vein it can be resected because the deep system will dilate. Can not be resected in aplasia. Closed stripping can lead to hematomas and bleeding because of large perforators and AVM's Laser because of the thin overlying skin and resultant uncomfortable scar and long-lasting painful inflammation |
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