Dermatology Book


Klippel-Trenaunay-Weber Syndrome

Aka: Klippel-Trenaunay-Weber Syndrome, Parkes-Weber Syndrome
  1. Signs
    1. Port-Wine Stain over an extremity
    2. Extremity soft tissue and bony hypertrophy
  2. Evaluation
    1. Measure length and girth of extremity every 3-6 month
    2. If extremity elongation noted:
      1. Evaluate for Arteriovenous Fistula
      2. Evaluate for venous atresia

Sturge-Weber Syndrome (C0038505)

Definition (NCI) A congenital disorder characterized by the presence of a port-wine nevus birthmark on one or both sides of the face. Additional clinical manifestations may include seizures, leptomenigeal angiomas, glaucoma, progressive hemiparesis and cognitive deficits.
Definition (NCI_NCI-GLOSS) A rare, congenital disorder that affects the brain, skin, and eyes. Abnormal blood vessel growth occurs in the trigeminal nerve in the face and the meninges (covering) of the brain. This abnormal growth causes red or purple skin discoloration (sometimes called a port wine stain), usually on one side of the face, and can also cause seizures, learning disabilities, and glaucoma.
Definition (MSH) A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects.
Concepts Congenital Abnormality (T019)
MSH D013341
ICD10 Q85.8
SnomedCT 19886006, 157030004, 234143003
English Sturge Kalischer Weber Syndrome, Sturge Syndrome, Sturge Weber Syndrome, Sturge's Syndrome, Sturge-Kalischer-Weber Syndrome, Sturge-Weber Syndrome, Syndrome, Sturge, Syndrome, Sturge's, Syndrome, Sturge-Kalischer-Weber, Syndrome, Sturge-Weber, Syndrome, Sturge-Weber-Dimitri, Sturge-Weber-Dimitri Syndrome, Sturge Weber Dimitri Syndrome, Angio ocul-orbit-thalam-enceph, Encephalocutan. angiomatosis, Encephalofacial haemangiomat, Neuroretinoangiomatosis, STURGE-WEBER SYNDROME, PKWS, PARKES WEBER SYNDROME, STURGE DIS, Sturge-Weber disease (diagnosis), encephalotrigeminal syndrome, Meningofacial Angiomatosis-Cerebral Calcification Syndrome, Angiomatosis, Meningo-Oculo-Facial, Meningo Oculo Facial Angiomatosis, Meningo-Oculo-Facial Angiomatosis, Angiomatosis Oculoorbital-Thalamic Syndrome, Sturge-Weber sequence, Sturge-Weber syndrome (disorder), Sturge-Weber syndrome, Sturge-Weber sequence (disorder), Sturge Disease, Encephalofacial Hemangiomatosis Syndrome, Hemangiomatosis Syndrome, Encephalofacial, Syndrome, Encephalofacial Hemangiomatosis, Sturge Weber Krabbe Syndrome, Sturge-Weber-Krabbe Syndrome, Syndrome, Sturge-Weber-Krabbe, Phakomatosis, Sturge Weber, Phakomatosis, Sturge-Weber, Sturge-Weber Phakomatosis, SWS, Sturge-Weber(-Dimitri) syndrome, Sturge-Weber Syndrome [Disease/Finding], parkes weber syndrome, sturge weber disease, sturge syndrome weber, neurocutaneous syndrome, sturge weber syndrome, sturge-weber syndrome, sturges weber syndrome, sturges syndrome weber, syndrome webers, webers syndrome, sturge-weber disease, weber syndrome, Parkes Weber Syndrome, Syndrome, Parkes Weber, Parkes-Weber Syndrome, Syndrome, Parkes-Weber, Milles' syndrome, Schirmer's syndrome, Encephalotrigeminal angiomatosis, Sturge-Kalischer-Weber syndrome, Encephalocutaneous angiomatosis, Encephalofacial haemangiomatosis, Encephalofacial hemangiomatosis, Parkes Weber syndrome, Sturge-Weber disease, Angiomatosis oculo-orbital-thalamo-encephalic syndrome, Sturge-Weber-Dimitri syndrome, Parkes Weber syndrome (disorder), Sturge, encephalotrigeminal; angiomatosis, angiomatosis; encephalotrigeminal, Dimitri-Sturge-Weber, Encephalotrigeminal Syndrome, Sturge-Weber Disease
Italian Angiomatosi encefalocutanea, Sindrome di Schirmer, Sindrome di Milles, Anomalia di Sturge Weber, Sindrome di Sturge-Weber-Dimitri, Sindrome di Sturge-Weber-Krabbe, Angiomatosi meningo-oculofacciale, Sindrome di Sturge, Emangiomatosi encefalo-facciale, SSW, Sindrome di Sturge-Kalischer-Weber, Malattia di Sturge, Sindrome da angiomatosi meningofaciale con calcificazioni cerebrali, Angiomatosi neuroretinica, Emangiomatosi encefalotrigeminale, Facomatosi di Sturge-Weber, Sindrome di Sturge-Weber
Dutch syndroom van Schirmer, Milles-syndroom, angiomatose; encefalotrigeminaal, encefalotrigeminaal; angiomatose, encefalocutane angiomatose, syndroom van Sturge-Weber, Facomatose, Sturge-Weber-, Neuroretinoangiomatosis, Syndroom, Sturge-Weber-
French Syndrome de Mille, Syndrome de Schirmer, Syndrome de Sturge-Weber-Krabbe, Syndrome de Sturge-Weber, SWK (Syndrome de Sturge-Weber-Krabbe), Neuroangiomatose encéphalofaciale, Neuro-angiomatose encéphalo-faciale, Angiomatose encéphalocutanée, Angiomatose congénitale de Sturge-Weber-Krabbe, Maladie de Sturge-Weber-Krabbe, Phacomatose de Sturge-Weber-Krabbe, Angiomatose encéphalotrigéminée, Angiomatose encéphalo-trigéminée
German Schirmer-Syndrom, Milles-Syndrom, Sturge-Weber Krankheit, enzephalokutane Angiomatose, Hamartome, ektoneurodermale, Neuroektodermaldysplasie, kongenitale, Neurokutanes Syndrom, Sturge-Weber-Syndrom, Neuroretinoangiomatose, Phakomatose, Sturge-Weber, Angiomatosis encephalofacialis, Angiomatosis encephalotrigeminalis, Neuroangiomatosis encephalofacialis, Sturge-Weber-Dimitri-Syndrom
Portuguese Síndrome de Schirmer, Síndrome de Milles, Angiomatose encefalocutânea, Facomatose de Sturge-Weber, Neurorretinoangiomatose, Síndrome de Sturge-Weber
Spanish Síndrome de Schimer, Síndrome de Milles, neurorretinoangiomatosis, síndrome de Sturge - Kalischer - Weber, síndrome de Sturge-Weber-Dimitri, síndrome de Sturge-Weber (trastorno), síndrome de Sturge - Weber, sucesión de Sturge - Weber (trastorno), síndrome de Sturge-Weber, enfermedad de Sturge - Weber, hemangiomatosis encefalofacial, síndrome de angiomatosis oculoorbitariatalamoencefálica, sucesión de Sturge - Weber, angiomatosis encefalotrigémina, síndrome de Parkes Weber (trastorno), síndrome de Parkes Weber, Angiomatosis encefalocutánea, Facomatosis de Sturge-Weber, Neurorretinoangiomatosis, Síndrome de Sturge-Weber
Japanese ミレス症候群, スタージ・ウェーバー症候群, 脳皮質血管腫症, シルマー症候群, スタージウェーバーショウコウグン, ノウヒシツケッカンシュショウ, シルマーショウコウグン, ミレスショウコウグン, Sturge-Weber症候群, パルケス・ウェーバー・ディミトリ病, スタージ・ウェーバー・ディミトリー病, スタージ・ウェーバー・ディミトリー・クラッベ症候群, スタージ-ウェーバー症候群, 大脳三叉神経血管腫症, 大脳顔面血管腫症, 眼脳血管腫症, 脳三叉神経血管腫症, 脳三叉神経領域血管腫, 脳三叉神経領域血管腫症, 脳顔面血管腫症
Swedish Sturge-Webers syndrom
Czech Sturgeův-Weberův syndrom, Sturge-Weberův syndrom, Millesův syndrom, Angiomatosis encephalocutanea, Schirmerův syndrom, Sturgeho-Weberův syndrom, Parkesův–Weberův syndrom, encefalofaciální angiomatóza, Sturgeův-Weberův-Krabbeův syndrom, encefalotrigeminální syndrom
Finnish Sturge-Weberin oireyhtymä
Polish Zespół Sturge'a-Webera
Hungarian Sturge-Weber-syndroma, Milles-syndroma, Encephalocutan angiomatosis, Schirmer-syndroma
Norwegian Sturge-Webers syndrom
Derived from the NIH UMLS (Unified Medical Language System)

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