LEGEND • SLOW-FLOW CLASSIFICATIONS

CARE4-RARE — SLOW-FLOW — CROSS-REFERENCE MAPPING

A Patient-Synthesized Model  |  Version 1.0  |  2026.06  |  For reference and patient advocacy use only
CLASSIFICATION CATALOG — CODE REFERENCE
The Care4-Rare alpha catalog code identifies each anomaly by its structural anatomy and vessel type — what the malformation is and where it sits in the classification hierarchy. Each entry is cross-referenced to its Hamburg code, which adds the embryological lens. Together the two codes form a complete clinical picture: what it is (Alpha Code) and how it behaves (Hamburg). The S/D-Overlap column distinguishes entries with a one-to-one Hamburg relationship (d-discrete) from those sharing a Hamburg code with related entries (s-shared) — a reflection of Hamburg’s intentionally broader biological groupings.
SVM — SLOW-FLOW · CAPILLARY MALFORMATIONS
Alpha Code Hamburg s/d Clinical behavior note
SVM—SF—CM—NSVE—EX—LIsNevus simplex / Salmon Patch — superficial capillary, fades spontaneously. Extratruncular limited.
SVM—SF—CM—PWVE—EX—LIsPort Wine CM (isolated) — extratruncular, limited. PDL best evidence. Recurrence common.
SVM—SF—CM—PW—ISVE—EX—LIsIsolated PWS incl. Phacomatosis Pigmentovascularis. Same Hamburg behavior as parent.
SVM—SF—CM—PW—SYVE—EX—INsSyndromic PWS — venous pressure driven, deeper infiltration. Extratruncular, infiltrating.
SVM—SF—CM—PW—SY—HYPCO—EX—HIsWith hypertrophy — combined hemolymphatic involvement. High recurrence potential.
SVM—SF—CM—PW—SY—SWSVE—EX—INdSturge-Weber — venous pressure pathophysiology, leptomeningeal involvement. Infiltrating.
SVM—SF—CM—PW—SY—DCMVE—EX—INsDCMO — diffuse capillary-venous pattern. Extratruncular infiltrating.
SVM—SF—CM—RTVE—EX—LIdReticulate / Telangiectatic CM — superficial capillary network. Limited extratruncular.
SVM—SF—CM—RT—ISVE—EX—LIsIsolated reticulate CM.
SVM—SF—CM—RT—SYVE—EX—INsSyndromic reticulate CM — deeper involvement.
SVM—SF—CM—RT—SY—MCCO—EX—HIsM-CM — macrocephaly-CM. Combined hemolymphatic, infiltrating. High recurrence.
SVM—SF—CM—RT—SY—MICO—EX—HLsMIC-CAP — microcephaly-capillary. Combined, limited.
SVM—SF—CM—RT—SY—DCVE—EX—INsDCMO syndromic reticulate variant.
SVM—SF—CM—GPCO—EX—HIsGeographic Pattern CM — broad distribution. Combined hemolymphatic. Infiltrating.
SVM—SF—CM—GP—ISVE—EX—LIsIsolated geographic CM — limited, no systemic involvement.
SVM—SF—CM—GP—SYCO—EX—HIsSyndromic geographic CM — combined infiltrating.
SVM—SF—CM—GP—SY—KTCO—EX—HIdKTS — hemolymphatic, extratruncular, infiltrating. Explains PDL recurrence. Genetic program ongoing.
SVM—SF—CM—GP—SY—CLCO—EX—HIsCLOVES/PROS — PIK3CA driven. Same hemolymphatic infiltrating behavior as KTS.
SVM—SF—CM—LRAV—EX—LIsLow-resistance CM / faster flow — transitional to AV territory. Extratruncular limited.
SVM—SF—CM—LR—ISAV—EX—LIsIsolated low-resistance CM.
SVM—SF—CM—LR—SYAV—EX—INsSyndromic low-resistance CM — AV shunting present. Infiltrating.
SVM—SF—CM—LR—SY—AVAV—EX—INsCM-AVM 1 & 2 — capillary + AV component. High recurrence. Not laser appropriate.
SVM—SF—CM—LR—SY—PWAV—EX—INdParkes-Weber — high-flow AV shunting. Extratruncular infiltrating. Embolization, not laser.
SVM—SF—CM—CCVE—TR—ODdCutis Marmorata Telangiectatic Congenita — truncular, often resolves partially. Less recurrence.
SVM—SF—CM—TSVE—EX—LIsTelangiectasias / Spider Angiomas — superficial extratruncular limited.
SVM—SF—CM—TS—ISVE—EX—LIsIsolated telangiectasias.
SVM—SF—CM—TS—SYAV—EX—LIsSyndromic telangiectasias — AV component emerging.
SVM—SF—CM—TS—SY—AVAV—EX—LIsCM-AVM 1 & 2 telangiectatic variant.
SVM—SF—CM—TS—SY—HJAV—EX—INsHHT 1 & 2 / JPHT — hereditary hemorrhagic, AV infiltrating. Systemic involvement.
* Hamburg capillary malformations are classified within the venous or combined categories — Hamburg predates the ISSVA capillary-specific classification and treats capillary lesions as capillary-venous in nature.
SVM — SLOW-FLOW · LYMPHATIC MALFORMATIONS
Alpha Code Hamburg s/d Clinical behavior note
SVM—SF—LM—ISLY—EX—LIsIsolated LM — extratruncular limited. Most common presentation.
SVM—SF—LM—IS—LDLY—EX—LIsLM Discrete — macrocystic / microcystic / mixed. All extratruncular limited.
SVM—SF—LM—IS—LD—MALY—EX—LIsMacrocystic — best sclerotherapy response. Limited extratruncular.
SVM—SF—LM—IS—LD—MILY—EX—LIsMicrocystic — more resistant to treatment. Still limited extratruncular.
SVM—SF—LM—IS—LD—MXLY—EX—LIsMixed — variable response. Extratruncular limited.
SVM—SF—LM—IS—ANLY—EX—LIsAngiokeratoma — superficial lymphatic-capillary. Limited extratruncular.
SVM—SF—LM—COLY—EX—INsComplex LM — infiltrating, systemic involvement. High recurrence.
SVM—SF—LM—CO—GLLY—EX—INsGLA — Generalized Lymphatic Anomaly. Diffuse extratruncular infiltrating.
SVM—SF—LM—CO—KLLY—EX—INsKLA — Kaposiform Lymphangiomatosis. Aggressive infiltrating. High morbidity.
SVM—SF—LM—CO—GSLY—EX—INsGSD — Gorham-Stout Disease. Bone involvement. Infiltrating.
SVM—SF—LM—CO—GDLY—EX—INsGLD — diffuse complex lymphatic anomaly. Infiltrating.
SVM—SF—LM—CO—CCLY—EX—INsCCLA — central conducting lymphatic anomaly. Truncal, infiltrating.
SVM—SF—LM—CO—CC—ISLY—EX—INCCLA Isolated.
SVM—SF—LM—CO—CC—SYCO—EX—HIsCCLA Syndromic (RASopathy) — combined hemolymphatic, infiltrating.
SVM—SF—LM—LYLY—TR—APsLymphedemas — truncular aplasia/obstruction. Less recurrence post-treatment than extratruncular.
SVM—SF—LM—LY—PRLY—TR—APsPrimary lymphedema — truncular developmental arrest.
SVM—SF—LM—LY—PR—ISLY—TR—APsPrimary lymphedema isolated.
SVM—SF—LM—LY—PR—SYCO—TR—HNsPrimary lymphedema syndromic — combined truncular, no AV shunting.
SVM—SF—LM—LY—SELY—TR—ODsSecondary lymphedema — acquired truncular obstruction/dilation.
SVM — SLOW-FLOW · VENOUS MALFORMATIONS
Alpha Code Hamburg s/d Clinical behavior note
SVM—SF—VM—ISVE—EX—LIsIsolated VM — extratruncular limited. Sclerotherapy first line.
SVM—SF—VM—IS—VDVE—EX—LIsVM Discrete — localized. Limited extratruncular. Good sclerotherapy response.
SVM—SF—VM—IS—PHVE—TR—ODsPhlebectatic VM — truncular dilation of venous trunk. Hemodynamic consequences.
SVM—SF—VM—IS—SPVE—EX—INsSpongiform VM — diffuse infiltrating. High recurrence. Difficult to treat completely.
SVM—SF—VM—IS—VVVE—TR—ODsVVM — venous/vascular malformation. Truncular dilation pattern.
SVM—SF—VM—IS—FAVE—EX—INdFAVA — fibro-adipose vascular anomaly. Deeply infiltrating. Pain predominant. Surgical.
SVM—SF—VM—MUVE—EX—INsMultifocal VM — multiple sites, infiltrating. Systemic monitoring required.
SVM—SF—VM—MU—CMVE—EX—INsVMCM — cutaneomucosal VM, familial. Infiltrating, multiple lesions.
SVM—SF—VM—MU—MSVE—EX—INsMSVM — multisystemic VM. Infiltrating.
SVM—SF—VM—MU—BRVE—EX—INdBRBNS — Blue Rubber Bleb Nevus Syndrome. GI involvement. Infiltrating, systemic.
SVM—SF—VM—MU—GVVE—EX—LIsGVM — glomuvenous malformation. Limited, less aggressive recurrence.
SVM—SF—VM—MU—HCVE—EX—INsHCCVM / CCM — cerebral cavernous malformation. CNS infiltrating. Neurological monitoring.
SVM—SF—VM—MU—VOVE—EX—INsVMOS — VM with osteoporosis/skeletal involvement. Infiltrating.
SVM—SF—VM—SYCO—TR—HNsSyndromic VM — combined truncular, no significant AV shunting.
SVM—SF—VM—SY—PHCO—EX—HIsPHTS (PTEN) — combined hemolymphatic infiltrating. Tumor suppressor involvement.
SVM—SF—VM—SY—CLCO—EX—HIsCLOVES — complex combined. PIK3CA driven. High recurrence. Infiltrating.
SVM—SF—VM—SY—MSCO—TR—HNsMafucci Syndrome — combined truncular. Enchondroma association.
SVM—SF—VM—SY—SPVE—TR—ODdSinus Pericranii — truncular dilation of dural venous sinus. Hemodynamic consequences.
SVM — SLOW-FLOW · COMBINED MALFORMATIONS
Alpha Code Hamburg s/d Clinical behavior note
SVM—SF—CO—ISCO—TR—HNIsolated combined — truncular combined, no AV shunting. Structural.
SVM—SF—CO—IS—CLVCO—EX—HIsCLVM — capillary-lymphatic-venous. Hemolymphatic infiltrating. High recurrence.
SVM—SF—CO—IS—LVMCO—EX—HIsLVM — lymphatic-venous combined. Infiltrating.
SVM—SF—CO—IS—CLMCO—EX—HLsCLM — capillary-lymphatic combined. Limited.
SVM—SF—CO—IS—CVMCO—EX—HLsCVM — capillary-venous combined. Limited.
SVM—SF—CO—IS—HCCCO—TR—HNsHCCVM / VVM — combined truncular. No AV shunting.
SVM—SF—CO—SYCO—EX—HIsSyndromic combined — hemolymphatic infiltrating. Genetic driver present in most.
SVM—SF—CO—SY—PRCO—EX—HIsPROS — PIK3CA spectrum. Hemolymphatic infiltrating. High recurrence. mTOR pathway.
SVM—SF—CO—SY—KTCO—EX—HIdKTS (CLVM with hypertrophy) — hemolymphatic infiltrating. Explains all recurrence patterns. Core audience diagnosis.
SVM—SF—CO—SY—CVCO—EX—HIsCLOVES — complex combined. PIK3CA. Infiltrating. High recurrence.
SVM—SF—CO—SY—CLCO—EX—HLsCLAPO — capillary-lymphatic-AVM with PROS features. Limited combined.
SVM—SF—CO—SY—PSCO—EX—HIsProteus Syndrome — PTEN/AKT1 driven. Hemolymphatic infiltrating. Progressive.
SVM — FAST-FLOW MALFORMATIONS
Alpha Code Hamburg s/d Clinical behavior note
SVM—FF—ISAV—EX—LIsharedIsolated fast-flow — extratruncular limited. Embolization/surgery first line.
SVM—FF—IS—AMAV—EX—INsAVM — nidus present. Extratruncular infiltrating. High recurrence without complete excision.
SVM—FF—IS—INAV—EX—INsIntramuscular fast-flow anomaly — deep infiltrating AV component.
SVM—FF—IS—AFAV—TR—SUsAVF — direct AV fistula, no nidus. Truncular superficial. Better surgical outcomes.
SVM—FF—MUAV—EX—INsMultifocal fast-flow — multiple infiltrating AV lesions. Systemic hemodynamic impact.
SVM—FF—MU—CAAV—EX—INsCM-AVM 1 & 2 — capillary + AV multifocal. RASA1/EPHB4. Infiltrating.
SVM—FF—MU—HJAV—EX—INsHHT 1 & 2 / JPHT — hereditary hemorrhagic telangiectasia. Multifocal AV infiltrating. Pulmonary/hepatic risk.
SVM—FF—MU—PHCO—EX—HIsPHTS — PTEN hamartoma. Combined hemolymphatic infiltrating.
SVM—FF—SYAV—EX—INsSyndromic fast-flow — AV infiltrating, genetic driver.
SVM—FF—SY—PHCO—EX—HIsPHOST (PHTS) — combined hemolymphatic infiltrating.
SVM—FF—SY—PWAV—EX—INdParkes-Weber Syndrome — AV infiltrating, extratruncular. Hemodynamically significant. High cardiac risk.
SVM—FF—SY—SAAV—EX—INsSAMS — AV infiltrating. Spinal involvement.
SVM—FF—SY—CAAV—EX—INsCAMS — cerebral AV malformation syndromic. CNS infiltrating.
SVM — NAMED VESSELS
Alpha Code Hamburg s/d Clinical behavior note
SVM—NV—VCVE—TR—APdVena Cava anomaly — truncular aplasia/stenosis. Major hemodynamic consequences.
SVM—NV—AOAR—TR—APdAortic anomaly — truncular arterial. Hemodynamic. Surgical intervention often required.
SVM—NV—VGAV—TR—DEdVein of Galen malformation — deep truncular AV. High-flow. Cardiac and neurological risk.
SVM—NV—OTVE—TR—ODsOther named vessel anomalies — truncular obstruction/dilation pattern.
© 2026 Care4-Rare Project. All rights reserved. A Patient-Synthesized Model. For personal reference and patient advocacy use only. Diagnosis is the realm of qualified medical professionals. ISSVA alpha catalog codes are original Care4-Rare designations. Hamburg classification cross-references follow Belov (1993, updated 2013) and Lee et al. (2007).