VASIFORM (VASCULAR) LESIONS OF THE H & N
BIOLOGICAL CLASSIFICATION (MULLIKEN AND GLOWACKI, 1981)
(OLD CLASSIFICATION OF CAVERNOUS/CAPILLARY/STRAWBERRY NOT VERY USEFUL–MORE DESCRIPTIVE)
NEOPLASTIC HEMANGIOMAS
NOT USUALLY PRESENT AT BIRTH(30% MAY BE)
MOST COMMON (H &N) TUMOR OF CHILDHOOD—3-8% OF INFANTS
OF THE ENDOTHELIAL CELL–A VASCULAR NEOPLASM–IN THE PAPILLARY DERMIS
A RAPID PROLIFERATIVE PHASE (1ST YEAR OF LIFE)—INCREASED TRITIATED THYMIDINE INCORPORATION–TRULY NEOPLASTIC–MAST CELL COUNTS INCREASE 30 X—MULTILAMINAR BASEMENT MEMBRANE—-INCREASED CELL TURNOVER—80% APPEAR IN THE 1ST YEAR OF LIFE
QUIESCENT PHASE
UNDERGO A SLOW INVOLUTION PHASE AFTER 1ST YEAR OF LIFE
INVOLUTE 50% BY 5, 60% BY 6, 70% BY 7, 80% BY 8, 90% BY AGE 9
OVERALL 80% COMPLETE RESOLUTION
REALLY 2 CLASSES OF INVOLUTERS—-SLOW(80% REQUIRE SOME SURGICAL INTERVENTION) AND RAPID(40% REQUIRE SURGERY)
60% OF ALL HEMANGIOMAS REQUIRE SOME SURGICAL INTERVENTION
FEMALE 3:1 EXCEPT SUBGLOTTIC HEMANGIOMAS– MALE=FEMALE(ABOUT 50% OF PTS WITH A SUBGLOTTIC HEMANGIOMA HAVE A COEXISTING CUTANEOUS LESION
WHITE>BLACK
FHx + 3%
CANNOT EVACUATE BLOOD BY PALPATION–NOT COMPRESSIBLE—FEEL LIKE A FIBROFATTY TUMOR
INTRAMUSCULAR HEMANGIOMAS USUALLY REQUIRE SURGERY(PRIMARILY MASSETER>TRAPEZIUS)
CUTANEOUS LESIONS CAN BE CLASSIFIED BY THEIR DEPTH WITHIN THE PAPILLARY DERMIS
SUPERFICIAL(BRIGHT RED)=CAPILLARY OR STRAWBERRY—-BLANCH WITH PALPATION
MID/COMPOUND=CAPILLARY-CAVERNOUS
DEEP=CAVERNOUS/”BAG OF WORMS”
VERY HIGH FLOW LESIONS CAN PREDISPOSE TO CHF
RAPIDLY PROLIFERATING LESIONS–”FIRE-FIELD” LESIONS CAN ACTUALLY LEAD TO TISSUE NECROSIS
DANGEROUS AROUND THE EYE—ROB THE BLOOD SUPPLY AND LEAD TO A DEPRIVATION AMBLYOPIA OR ASTIGMATISM–GET OPHTHO CONSULT
KASSABACH-MERRIT SYNDROME OF PLT SEQUESTRATION—THROMBOCYTOPENIA—RAPIDLY ENLARGING HEMANGIOMA AND DIC
Dx: MRI/MRA IS BEST—CLEARLY SHOWS THE FLOW AND EXTENT OF THE LESIONS
Rx:OBSERVATION
STEROIDS–BEST IN 1ST YEAR OF LIFE—2-3MG/KG PO QD TO QOD FOR 6-8MONTHS SYSTEMIC
CONSIDER INTRALESIONAL STEROIDS
IFN-ALPHA 2b—EXCELLENT IN KASSELBACH-MERRIT TO AVOID DIC
FLASH LAMP PUMPED PULSED DYE LASER Nd:YAG PHOTOCOAGULATION—UP TO 5 TREATMENTS
PHOTODERM LASER
EMBOLIZE–SURGICAL RESECTION
CHEMO(CYLOPHOSPHAMIDE), RT IF LIFE THREATENING
CRYO
“A LOSER’S GAME”—TEMPLER
LOBULAR CAPILLARY HEMANGIOMA
PRIMARILY ON LIP = PYOGENIC GRANULOMA (OFTEN FROM TRAUMA) — LEADS TO REACTIVE FIBROMA
Rx EXCISION
VASIFORM MALFORMATION/VASCULAR HAMARTOMAS
ALWAYS PRESENT AT BIRTH—A MALFORMATION OF BADLY FORMED VESSELS
NEVER PROLIFERATE–NL ENDOTHELIAL TURNOVER–MAY EXPAND RAPIDLY AS A RESULT OF INFXN, TRAUMA, BP CHANGES, OR HUMORAL MODULATION
NEVER INVOLUTE
UNDERGO COMMENSURATE GROWTH
CAN EVACUATE BLOOD BY PALPATION–COMPRESSIBLE–UNLIKE HEMANGIOMA—NOT SOLID
MALE=FEMALE
SUBCLASSIFIED INTO:
ARTERIAL
VENOUS/VENULAR
AVM—-DIFFICULT TO DIFFERENTIATE —EVEN WITH MRI–ABSENCE OF PRECAPILLARY SPHINCTERS–HYPERTROPHY OF FEEDING ARTERIES—DILATION OF DRAINING VEINS
CAPILLARY
LYMPHATIC/LYMPHANGIOMA—-TRANSILLUMINATE
MIXED/NOS
PWS(PORT WINE STAIN)—-GORBECHEV
CONG. VASCULAR MALFORMATIONS(HAMARTOMAS) OF THE SUPERFICIAL RETICULAR DERMIS
REALLY A VENULAR MALFORMATION
3-5/100,000
COMPRESSIBLE
PRESENT AND PINK AT BIRTH—DARKEN TO DEEP RED/PURPLE WITH COMMENSURATE GROWTH
SHARPLY DEMARCATED
MACULAR–NOT PAPULAR(RAISED)—MAY BECOME PAPULAR/NODULAR–”COBBLESTONED” AS PT REACHES MIDDLE AGE
90% IN THE H & N
MALE = FEMALE
GEOGRAPHIC OR CONFLUENT
NEVUS FLAMMEUS(FLAMMUS/CAPILLARY MALFORMATIONS/NEVUS SIMPLEX/ANGEL KISS)=A MIDLINE LESION—–CLASSICALLY DO BETTER
DO NO CONFUSE WITH NEVUS FLAVUS NEONATORANS—STORK BITE OR STORK’S BEAK MARK= A PHYSIOLOGIC PHENOM ON NUCHAL REGION
SEGMENTAL PWS IN THE DIST OF V2>V1 MAY BE LINKED WITH STURGE-WEBER SYNDROME–ENCEPHALOTRIGEMINAL ANGIOMATOSIS(CHROMOSOME #3) OR OTHER NEURO SYNDROMES(VON HIPPEL LINDAU–CEREBELLAR HEMANGIO BLASTOMA)
CONG VENOUS ANGIOMATA OF THE LEPTOMENINGES—–+ INTRA CRANIAL CALCIFICATION (MR, CONTRALATERAL JACKSONIAN SIEZURES AND PARALYSIS) AND CHOROID–VISUAL DEFICITS AND GLAUCOMA
GET NEURO AND OPHTHO CONSULTS WITH ANY H & N PWS
Dx: MRI
Rx: FPDL(FLASH LAMP-PUMPED DYE LASER)—-MAY RECUR AFTER YEARS
LYMPHANGIOMA/LYMPHATIC HAMARTOMA
OFTEN MIXED WITH VENOUS MALFORMATIONS AS THE TWO ARISE CLOSE EMBRYOLOGICALLY=LYMPHANGIO-HEMANGIOMA
FARBER & LANDING ORIGINALLY CLASSIFIED THEM BY THE SIZE OF THE LYMPHATIC SPACES(1956)
SMALLEST=CAPILLARY=LYMPHANGIOMA SIMPLEX=LYMPHANGIOMA CIRCUMSCRIPTUM=MICROCYSTIC LYMPHANGIOMA—-VERY DIFFICULT TO MANAGE
MEDIUM=CAVERNOUS LYMPHANGIOMA
LARGEST=CYSTIC HYGROMA=CYSTIC LYMPHANGIOMA=LYMPH CYST—-EASIER TO MANAGE
NEW CLASSIFICATION—INCORPORATES OLD AS CYSTIC VS DIFFUSE PLUS DESCRIPTORS SUCH AS MUCOSAL VS CUTANEOUS AND SUPERFICIAL VS DEEP
DIFFERING THEORIES ON LYMPHATIC EMBRYOLOGY–PRIMORDIAL SACS VS MESENCHYMAL CLEFTS
90% PRESENT IN THE POST TRIANGLE—USUALLY IN THE SUPRACLAVICULAR FOSSA—ALMOST ALWAYS INVOLVE THE SPINAL ACCESSORY NERVE
75% DIAGNOSED AT BIRTH
85% DIAGNOSED BEFORE AGE 3
MALE=FEMALE
EXTENT IS ALWAYS GREATER THAN CLINICALLY EVIDENT
OFTEN LARGE—PAINLESS, SOFT, DOUGHY, SPONGY MASS
COMPRESSIBLE
BRILLIANTLY TRANSLUCENT(TRANSILLUMINATES)
INDISCREET MARGINS
PROCLIVITY FOR MUCOSAL SURFACES—-TONGUE
FNA—LYPHOCYTES–NO EPITHELIAL DEBRIS OR CHOLESTEROL LIKE BRANCHIAL APPARATUS CYSTS
Dx: MRI—SHOWS EXTENT
Rx: “A LOSER’S GAME”
IF THEY ARE BORN WITH A SIGNIFICANT ONE—LET THEM GO HOME WITH IT FOR A FEW MONTHS—-PARENTS GET USED TO IT—ANYTHING YOU DO WILL SEEM A TREMENDOUS IMPROVEMENT—-BUT TAKE IT OUT IN ABOUT 3-4 MONTHS—BEFORE IT GETS INFECTED
OK432
AVOID RT AND MOST SCLEROSING AGENTS