OPHTHO—DANIEL TODD, MD ORBITAL ANATOMY SKIN: THE THINNEST DERMIS IN THE BODY BLEPHAROCHALASIS—A RARE DISORDER OF THE UPPER LIDS—-USUALLY IN YOUNG FEMALES—RECURRENT UNI OR BILAT EDEMA CAUSING DERMAL ATROPHY AND LOSS OF ELASTICITY PALPEBRAL DERMATOCHASASIS—AGE RELATED SKIN CHANGES—CAN OFTEN MANIFEST AS PSUEDOPTOSIS STEATOBLEPHARON—PSEUDOHERNIATION OF FAT BEHIND THE SEPTUM— -REALLY THOUGHT TO BE DUE TO WEAKENING OF THE GLOBE SUPPORT SYSTEM (PRIMARILY LOCKWOOD’S SUSPENSORY LIGAMENT) ALLOWING IT TO DESCEND AND CAUSING ENOPHALMOS AND AN INFERIOR VERTICAL DYSTOPIA REDUCING THE SPACE BETWEEN IT AND THE FLOOR OF THE ORBIT—CAUSING FORWARD PROJECTION OF THE EXTRACONICAL FAT—CREATING PSEUDOHERNIATED FAT PADS—–THIS IS THE NEW THINKING BEHIND ORBITAL FAT REPOSITIONING (VIA REPOSITIONING OR MOBILIZATION) FESTOONS—-THE ORBICULARIS DRAPING/FOLDED OVER ITSELF SOOF—–SUBORBICULARIS OCULI FAT MALAR BAGS—-AREAS OF FULLNESS OVER THE LATERAL INF ORBITAL RIM BEFORE CONSIDERING—-TEST VISUAL ACUITY, CORNEAL SENSATION, HEIGHT OF EYELID CREASE, LEVATOR FUNCTION, PERIMETRY (FORMAL VISUAL FIELDS), LID LAXITY “SNAP TEST”, AMOUNT OF PROPTOSIS “BIG EYES=BIG PROBLEMS” ASIAN EYELID——–CLASSICALLY STATED THAT THE LEVATOR APONEUROSIS FUSES WITH THE ORBITAL SEPTUM BELOW THE SUP BORDER OF THE TARSUS —–ALSO OFTEN HAVE AN EPICANTHAL FOLD AS WELL AS LACKING AN UPPER EYELID CREASE—-THE FIBERS OF THE CONJOINED TENDON (LEVATOR APONEUROSIS AND ORBITAL SEPTUM) AND TARSAL PLATE DO NOT TEATHER THE ORBICULARIS AND DERMIS MUSCLE: ORBICULARIS OCULI HAS AN ORBITAL (MORE ADHERENT TO OVERLYING SKIN) AND PALPEBRAL PORTION—-THE PALPEBRAL PORTION (RESPONSIBLE FOR BLINKING) IS FURTHER DIVIDED INTO PRETARSAL AND PRESEPTAL REGIONS CORRUGATORS—PEIRCED BY THE SUPRATROCHLEAR AND SUPRAORBITAL BUNDLES—THE ONLY MUSCLE WITH BONEY ATTACHMENTS MUST RELEASE THE ARCUS MARGINALIS (WHERE THE FRONTAL PERIOSTEUM MEETS THE PERIORBITA) TO GET IDEAL LIFT BROW ELEVATORS—ONLY ONE=FRONTALIS (OCCIPITO-GALEAL-FRONTALIS COMPLEX)—NOT PRESENT LATERALLY BROW DEPRESSORS—CORRUGATOR, PROCERUS, AND THE STRONGEST=ORBICULARIS OCULI CANNOT OVERCORRECT LATERALLY—-HAVE UNOPPOSED DEPRESSOR=ORBICULARIS FORM HORIZONTAL RHYTIDS—-FRONTALIS AND PROCERUS FORM VERTICAL RHYTIDS—-CORRUGATORS, ORBICULARIS, AND DEPRESSOR SUPERCILII, OSTEOLOGY: 7 BONES—MAXILLA, ZYGOMA, FRONTAL, LACRIMAL (CONTAINS LACRIMAL FOSSA), ETHMOID (LAMINA PAPYRECEA), PALATINE, AND LESSER AND GREATER WINGS OF SPHENOID 1/10 HAVE A SUPRAORBITAL FORAMEN 9/10 HAVE A SUPRAORBITAL NOTCH DEFINE LACRIMAL CREST ANT. ETHMOID IS 24 MM POST, 12MM TO POST. ETHMOID, 6MM TO OPTIC NERVE ORBIT DIVIDED INTO INTRA AND EXTRA-CONAL PORTIONS BY TENON’S CAPSULE (A STRONG FASCIAL SHEATH WHICH ENVELOPES THE RECTUS EOM’S AND GLOBE)—-CONTAINS THE EOM’S, OPTIC NERVE, V1 AND V2, BV’S AND THE CILIARY GANGLION THE EXTRACONAL ORBIT CONTAINS ORBITAL FAT, BV’S, THE LACRIMAL GLAND AND SAC, THE LEVATOR PALPEBRAE SUPERIORIS MUSCLE, AND THE CHECK LIGAMENTS—–ALL OF THIS IS STILL ENVELOPED BY THE PERIORBITA (PERIOSTEUM) AND ANTERIORLY BY THE SEPTUM (THE JUNCTION OF THESE 2 IS THE ARCUS MARGINALIS—MUCH MORE TIGHTLY ADHERENT TO THE BONE THAN THE REMAINING PERIORBITAL) EYELID STRUCTURES ARE OFTEN REFERED TO AS PRE (ANT.) AND POSTERIOR LAMELLAR IN RELATION TO THE ORBITAL SEPTUM THE TARSAL PLATES ARE ATTACHED LATERALLY TO THE PERIORBITA —SUPERIORLY BY WHITNALL’S LIGAMENT AND INFERIORLY BY LOCKWOODS LIGAMENT–HAS A LATERAL TUBERCLE ON THE BONE AS WELL FAT COMPARTMENTS-—THEORY HAS BEEN CHALLENGED–PROBABLY DISCOUNTED — BUT IT IS USEFUL CLINICALLY TO KEEP THE SURGEON AWARE OF THE IMPORTANT STRUCTURES—ESPECIALLY THE INF OBLIQUE AND LEVATOR SUPERIORIS ONLY 2 FATPAD SUP—MEDIAL (TROCHLEAR FASCIA) AND CENTRAL THE LAT UPPER COMPARTMENT CONTAINS THE LACRIMAL GLAND—CAN PEXY THIS UP IF NESCESSARY THE LACRIMAL GLAND IS BILOBED AND STRADDLES THE LATERAL EDGE OF THE LEVATOR APONEUROSIS (SUP = ORBITAL LOBE AND DEEP = PALPEBRAL LOBE)—(MUCH LIKE THE SUBMANDIBULAR GLAND STRADDLING THE POST MYLOHYOID) THE SOF CONTAINS: THE TROCHLEAR NERVE/CN4 (SUP OBLIQUE), THE OPTH BR OF THE TRIGEMINAL/V1 (THE FRONTAL AND LACRIMAL BR), AND THE OPHTHALMIC VEIN ALSO WITHIN THE SOF AND ANULUS TENDINEUS OF ZINN—CN 3 SUP DIVISION OF OCULOMOTOR (SUP RECTUS AND LEVATOR) AND INF DIV OCULOMOTOR (MEDIAL AND INF RECTUS AND INF OBLIQUE), CN 6/ABDUCENS (LATERAL RECTUS), AND THE NASOCILIARY BRANCH OF V1 (“HUTCHINSON’S SIGN”) SOF SYNDROME—-CN 3 AND 4 OUT EARLY—-DUE TO COMPRESSION (OPHTHALMOPLEGIA AND LID PARESIS)—6 OUT EARLY DUE TO INFXN OR HIGH ICP (LAT GAZE PAULSEY)–VI OUT—–SUPRAORBITAL HYPESTHESIA—NO XN TO LIGHT(PARASYMP ON CN 3) (PHOTOPHOBIA–NOT ABLE TO CONSTRICT PUPIL) LATER LOSS OF VISION DUE TO VENOUS CONGESTION (COMPRESSION OF THE OPTIC VEIN) THE OPTIC CANAL (ALSO CONTAINED WITHIN THE ANULUS) CARRIES THE OPTIC NERVE(CN2) AND THE OPTH ARTERY (1ST BR OFF THE INT CAROTID) THE INF. ORBITAL FISSURE (IOF) CONTAINS THE INF OPHTH VEIN AND THE MAXILLARY OR INF ORBITAL NERVE/V2 ALL OF THESE STRUCTURES ENTER THE CRANIUM AROUND THE SPHENOID AND THEN ENTER THE CAVERNOUS SINUS AROUND THE PITUITARY
LYMPHATIC DRAINAGE IS VARIABLE AND USUALLY TO THE IPSILATERAL PAROTID, FACIAL, AND SUBMANDIBULAR NODES Hx—VISUAL CHANGES, PAIN, DIPLOPIA¼. ASTHENOPIA = EYE STRAIN (A RARE CAUSE OF HA) PE: –GROSS HORDELEDUM (STY)=EYELID INFXN—INTERNAL=MEIBOMIAN TARSAL /SEBACEOUS GLANDS OR EXTERNAL = SEBACEOUS GLANDS OF ZEISS OR SWEAT GLANDS OF MOLL CHALAZION=CHRONIC (PAINLESS/GRANULOMATOUS) INFXN OF MEIBOMIAN GLAND (CHRONIC INT STY) PTERYGIUM=FLEXHY MEMBRANOUS EXCRESCENCE OF CONJUNCTIVA OVER CORNEA (NASAL > TEMPORAL) CORNEAL ARCUS =A WHITE RING NEAR THE LIMBUS – ASSOC WITH CADz CHEMOSIS =CONJUNCTIVAL EDEMA BAND KERATOPATHY =HYPER CA++/CORNEAL DEPOSITS XANTHELASMA =YELLOWISH BILAT PALPEBRAL PLAQUES KAYSER FLEISHER RINGS =CU++ DEPOSITS IN DESCEMET’S MEMBRANE (WILSON’S Dz) LISCH NODULES (IRIS HAMARTOMAS) AND OPTIC GLIOMAS—-NF1 BRUSHFIELD SPOTS = SPECKLED IRIDES—DOWN SYNDROME LEUKOCORIA = WHITE PUPIL (CATARACT OR RETINOBLASTOMA)–CONG. CATARACT = CONG RUBELLA, GALACTOSEMIA, LOWE’S DISLOCATED LENS OR LENTICONIA = MARFANS/HOMOCYSTINURIA DACRYOCYSTADENITIS =LACRIMAL GLAND OR DRIANAGE SYSTEM INFXN DRUSEN =HYALIN DEPOSITS IN BRUCH’S MEMBRANE (AGING OR ACTINOMYCOSIS) CORNEAL LEUKOMA =DENSE WHITE CORNEAL SCARRING ENDOPHTHALMITIS =INFXN WITHIN THE GLOBE HYPOPYON =PUS IN THE ANT CHAMBER HYPOPHEMA =BLOOD IN THE ANT CHAMBER—OFTEN WITH A DEPENDENT MENISCUS—-GET AN OPTHO CONSULT GLOBE DYSTOPIA—EXOPHTHALMOS, ENOPHTHALMOS, VERTICAL OR HORIZONTAL DYSTOPIA (HERTEL EXOPHALMOMETER) OCULAR BALLOTTEMENT, VISUAL ACUITY, VISUAL FIELDS (PERIMETRY VS CONFRONTATIONAL TESTING), PERRL-A (PERRL WITH NL VISUAL ACUITY WILL ALWAYS ACCOMIDATE—–DO NOT NEED PERRL-A) ANISOCORIA—PUPILS NOT EQUAL (NL IN 5-15%) PTOSIS—CONG (NO EYELID FOLD) VS SENILE(INVOLUTIONAL)–HIGH EYELID FOLD ARGYLL ROBERTSON (PROSTITUTES) PUPIL—ACCOMIDATES BUT DOESN’T REACT (NEUROSYPHILIS OR DM) MARCUS GUNN —AFFERENT PUPILLARY DEFECT (SWINGING PENDULUM FLASHLIGHT TEST)–THE CONSENSUAL RESPONSE OF THE INVOLVED PUPIL IS GREATER THAN ITS RESPONSE TO DIRECT LIGHT PUPILLARY AFFERENTS (V1)–-CORNEAL BLINK REFLEX NYSTAGMUS PARINAUD’S PHENOM =HIGH ICP OR LESION COMPRESSING THE MESENCEPHALIC TECTUM (PRETECTAL REGION OF THE SUP COLLICULUS)—SUPRA NUCLEAR GAZE PAULSEY—UNABLE TO LOOK UP INTERNUCLEAR OPHTHALMOPLEGIA—-MLF LESION—CHILD—PONTINE GLIOMA, ADULT—MS, ELDERLY—VBI TROCHLEAR NERVE PAULSEY—FALL DOWN THE STAIRS—PT CANNOT LOOK DOWN AND IN—-4TH CN = LONGEST INTRACRANIAL COARSE—COMES OFF THE POST BRAINSTEM 3 & 4 OFTEN AFFECTED EARLIEST BY COMPRESSION ABDUCENS NERVE PAULSEY—LAT GAZE DIPLOPIA–THE EYE PULLS OR CROSSES IN—CN 6 OFTEN SPARRED FROM EARLY COMPRESSION AS IT SITS LAT TO THE CAROTID–BUT IT IS FIRST TO BE AFFECTED BY INFXN, THROMBOSIS, ECT…RIDES ON THE BONE IN DORELLOS CANAL UNDER THE PETROCLINOID/PETROSPHENOID LIGAMENT OF GRUBER FIXED TO THE SKULL BASE—SUSCEPTIBLE TO T-BONE Fx—ANY INCREASE IN ICP CAN CAUSE COMPRESSION OF ITS ORIGIN VIA THE AICA OR LABYRINTHIAN A.—OF LITTLE LOCALIZING VALUE— –MOBIUS SYNDROME MG-–OFTEN PRESENT WITH DIPLOPIA OF PTOSIS TROPIA=DEVIATION OF THE EYES (WHERE BINOCULAR VISION IS NOT POSSIBLE) HETEROTROPIA = STRABISMUS HETEROPHORIA = LATENT HETEROTROPIA (STRABISSMUS WHEN BINOCULAR VISION IS NOT POSSIBLE) ESO (IN), EXO (OUT), HYPER (UPWARD), AND HYPOTROPIA (DOWNWARD DEVIATION) GET PSUEDO ESOTROPIA WITH TRAUMATIC TELECANTHUS OR INFANTILE EPICANTHUS (EPICANTHAL FOLDS) ABNL PIGMENT -OI, WAARDENBURG’S, PIEBALDNESS ABNL PIGMENT -OI, WAARDENBURG’S, PIEBALDNESS INTERSTITIAL KERATITIS—SYPHILIS, COGANS DISLOCATED LENS—MARFANS, HOMOCYSTINURIA, SULFITE OXIDASE DEFEICIENCY OPHTHALMOSCOPY OPTIC DISK OPTIC ATROPHY (PALLOR)—-MS, ISCHEMIA PAPILLEDEMA—-HIGH ICP = BIH (PSEUDOTUMOR CEREBRI–PULSATILE TINNITUS), OTOTIC HYDROCEPHALUS—CUP/DISK MARGINS OBLITERATED ENHANCED CUP TO DISK RATIO—-GLAUCOMATOUS CUPPING–ROCK HARD GLOBE WITH PAIN AND HALOS AROUND LIGHTS JUVENILE GLAUCOMA—STURGE WEBER, NF, RUBELLA, LOWE’S RETINA CVDz=HOLLENHORST PLAQUES—AMAUROSIS FUGAX
HYPERLIPIDEMIA=HARD RETINAL EXUDATES (LIPOID DEPOSITS) HTN=SEGMENTAL NARROWING, A/V NICKING, DOT-BLOT HEMORRAGES (FLAME SHAPED) SBE/LEUKEMIA–ROTH SPOTS (TARGET LESIONS) DM–COTTON-WOOL SPOTS, MICROANEURYSMS, NEOVASCULARIZATION(TELANGIECTASIA), LIPIDEMIA RETINALIS (PALE FUNDUS AND CREAM COLORED BV’S, DOT-BLOT HEMORRAGES AIDS–COTTONWOOL SPOTS, CMV RETINITIS, KAPOSIS SARCOMA RP–THE PRIMARY FORM OF RETINAL DEGENERATION– OFTEN PRESENT WITH NYCLOPSIA—USHERS, HALLGRENS, REFSUMS, LAWRENCE-MOON-BIEDLE RETINAL DETACHMENT–ELEVATION AND HYPOPIGMENTATION DRUSEN—DEGENERATIVE HYALIN DEPOSITS—ALSO SEEN IN ACTINOMYCOSIS CHOROIDAL MELANOMAS—RAISED VS FLAT BENIGN CHOROIDAL NEVUS CENTRAL VEIN OCCLUSION—-SUDDEN PAINLESS VISUAL LOSS–UNILAT—BLOOD SPLASHED FUNDUS (BLOOD AND THUNDER) CENTRAL ARTERY OCCLUSION—CHERRY RED SPOT AND MILKY PALE RETINA CHERRY RED SPOT—NEIMAN PICK, TAY SACHS, CENTRAL RETINAL ARTERY OCCLUSIOQN EPIRETINAL MEMBRANES AND RETINAL HAMARTOMAS—-NF2 CT WITH CONTRAST—-GOLDD STANDARD CAVEATES: ORBITAL PAIN WITH COLORED HALOS AND RAINBOW LIKE FRINGES AROUND LIGHTS = ACUTE GLUACOMA (EMERGENCY) STOCK SOLUTIONS OF FLOURESCIENE ARE OFTEN COLONIZED WITH PSEUDOMONAS IF PAPILLEDEMA IS EVERUNILAT—THINK FOSTER KENNEDY SYNDROME OF AN OLFACTORY GROOVE MENENGIOMA NEVER GIVE TOPICAL STEROIDS OR ANESTHETICS CHRONICALLY!—MAY END UP WITH CATARACTS OR GLAUCOMA OR—CORNEAL ABRASION DUE TO LOSS OF PROTECTIVE REFLEXES TRANSCARUNCULAR APPROACH—-EXCELLENT FOR SAFE THOUROUGH ETHMOIDECTOMIES AND ORBITAL DECOMPRESSION—NO INCISIONS ON THE FACE—-RETRACT THE GLOBE MEDIALLY WITH A MALLEABLE EXPOSING THE MEDIAL ORBITAL WALL POST TO THE LACRIMAL FOSSA—INCISE TO THE BONE WITH A 15 BLADE—RAISE THE PERIORBITA WITH A FREER—VIEW THE FREER FROM INSIDE THE NOSE ENDOSCOPICALLY PROBABLY NO NEED TO CLOSE THE INCISION POST SEPTAL RETRO-ORBITAL HEMATOMA—-REALLY 2 VARIETIES–FAST (ARTERIAL) AND SLOW (VENOUS) NEED TO THINK ABOUT THESE WITH BOTH BLEPHS AND SINUS SURGERY—-LOOTS OF ORBITAL BALLOTMENT AND PUPIL EXAMINATION—-DO NOT PARALYZE PTS IF POSSIBLE DIAGNOSIS—-CALL OPHTHO—MEASURE IOP—REMOVE TARSORAPHY—IF AWAKE TEST VISION—ELEVATE HOB—REMOVE PACKING OR SUTURES—-DECADRON–?ACETAZOLAMIDE/MANNITOL—ORBITAL MASSAGE—INF CANTHOTOMY AND CANTHOLYSIS—-EXTERNAL ETHMOIDECTOMY –INTRANASAL DECOMPRESSION AND HEMOSTASIS–STAY CALM! ORBITAL MASSES PRIMARY CAUSE OF PROPTOSIS IN CHILDREN = INFXN (ORBITAL CELLULITIS (PRESEPTAL) FROM ETHMOIDITIS PRIMARY CAUSE OF PROPTOSIS IN ADULTS = GRAVES PRIMARY ORBITAL TUMOR IN CHILDREN = HEMANGIOMA PRIMARY ORBITAL TUMOR IN ADULTS = PLEOMORPHIC ADENOMA PRIMARY ORBITAL CA IN CHILDREN = RHABDOMYOSARCOMA PRIMARY ORBITAL CA IN ADULTS = ADENOID CYSTIC CA INFLAMMATIONS ORBITAL INFXNS—-CELLULITIS—-MOST COMMON UNDER AGE 5–CAN BE AN EXTENSION OF ETHMOID SINUSITIS OR SUB PERIOSTEAL ABSCESS—CAN LEAD TO ORBITAL ABSCESS ORBITAL COMPLICATIONS—(CHANDLER STAGING)——MOST COMMON USUALLY BETA HEMOLYTIC STREP > H inf (PREVELENT IN PEDS) > PNEUMOCOCCUS > DIPLOCOCCUS STAGE I—-PRESEPTAL (PERIORBITAL) CELLULITIS—ANT TO THE SEPTUM—-NO FLUCTUANCE—–USUALLY ONLY ONE EYE (92%)—BOTH MAY BE INVOLVED—NO PROBLEMS WITH VISUAL ACUITY OR EOM’S STAGE II—-SUBPERIOSTEAL ABSCESS—-PRESEPTAL CELLULITYS PRESENT AS WELL AS AN ABSCESS—USUALLY FROM THE ETHMOIDS DISPLACING THE PERIORBITA—-CHEMOSIS, ASYMETRIC PROPTOSIS, EOM RESTRICTION, AND POSSIBLY DECREASED VISION STAGE III—-ORBITAL CELLULITIS—–INFLAMMATION WITHIN THE RETROBULBAR CONTENTS OF THE ORBIT–BUT COMPLETELY ENCLOSED BY THE PERIORBITA—SPIKING FEVERS, MARKED PRESEPTAL CELLULITIS, CHEMOSIS, PROPTOSIS(AXIAL), OPHTHALMPLEGIA, AND DECREASED VISION STAGE IV—-ORBITAL ABSCESS—–WITHIN THE CONE OF THE PERIORBITA—INCLUDES RETROBULBAR ABSCESS—SEVERE PROPTOSIS AND VISUAL LOSS STAGE V—-CAVERNOUS SINUS THROMBOSIS—-(COAG + S. a IS MOST COMMON)-WITH ORBITAL CELLULITIS—OFTEN BILATERAL OR WILL BECOME BILATERAL—-CN 3, 4, AND 6 PALSEYS—EPISCLERAL VENOUS DILATION AND PROPENSITY FOR EPISTAXIS AS HIGH VENOUS PRESSURES EXIST—MENINGITIS OR BLINDNESS MAY OCCUR COMPLICATIONS OF ANY OF THESE ENTITIES—OPHTHALMOPLEGIA—ORBITAL APEX SYNDROME—HIGH IOPS—CENTRAL RETINAL ARTERY OR VEIN OCCLUSION—OPTIC NEURITIS—ENDOPHTHALMITIS—MENINGITIS OR EVEN DEATH CROSS OF DEATH IS WHEN THE TEMP FALLS AND THE PULSE QUICKENS—OMINOUS
Dx–CLINICALLY DOCUMENT VISUAL ACUITY AND IOPS AND EOMI GET THIN CUT CT SCAN (CONTRASTED) OF THE SINUSES Rx:CONSULT OPTHO— I&D (PREFERRABLY ENDOSCOPICALLY)—FOR A PERIORBITAL ABSCESS MOST PRACTIONERS WILL DO AN EXT ETHMOIDECTOMY VIA LYNCH—FOR AN ABSCESS WITHIN THE PERIORBITA GET OPHTHO INVOLVED MOISTURE CHAMBER, LACRILUBE, SEDATIVES, ANALGESICS, TOPICAL ABX EYE DROPS, AFRIN, SYSTEMIC DECONGESTANTS, IV AVX (UNASYN AND CLEOCIN), WARM COMPRESSES PHYCOMYCOSIS / RHINOCEREBRAL PHYCOMYCOSIS = FACIAL FUNGAL INFXN (SAPROBES) LIFE THREATENING—PRIMARILY IMMUNOCOMPROMISED HOST, UNCONTROLLED DM RHIZOPUS, ABSIDIA, RHINOSPORIDIOSIS, MUCORMYCOSIS PAY SPECIAL ATTN TO THE APPEARANCE OF THE MIDDLE TURBINATE RHINOSPORIDIUM SEEBERI (RHINOSPORIDIOSIS) A PHYCOMECETES M/O SRI LANKA AND SOUTHERN INDIA NAO, EPISTAXIS, SNEEZING, RHINORRHEA “STRAWBERRY”, INDOLENT, PAINLESS, WARTY, FRIABLE, POLYPOID, MUCOSAL MASS—FAIRLY VASCULAR Rx = EXCISION AND AMPHO B MUCORMYCOSIS IMMUNOCOMPROMISED HOST(AIDS), NEUTROPENIA, AND OR DKA(ACIDOSIS AND HYPOGLYCEMIA), DEFEROXIMINE LOW GRADE FEVER, DULL SEVERE SINUS PAIN, EPISTAXIS, FACIAL HYPESTHESIA——DIPLOPIA, OBTUNDATION, FEVER, BLACK TURBINATES, PROPTOSIS, FACIAL SWELLING, BLINDNESS—-COMA—-DEATH Rx = EARLY DIAGNOSIS—SUSPICION, BIOPSY—BRANCHING(90 DEGREES) AT RIGHT ANGLES, THICK WALLED NON-SEPTATE HYPHAE “M”—DEBRIDE–RADICAL MAXILLECTOMY, ORBITAL EXENTERATION, AMPHO B LIPOSOMAL IRRIGATIONS, SYSTEMIC AMPHO B AND HBO!—-CONSIDER GRANULOCYTE STIMULATING FACTORS STAIN + WITH HMB-45 (MELANIN STAIN) AMPHO B TEST DOSE 0.3MG/KG DISSOLVED IN 500 CC D5W IN OVER 1-3 HOURS MEASURE BUN, CR ADVANCE TO 0.5-0.6MG/KG IN 500 CC D5W IN OVER 1-3 HOURS Q D LIPOSOMAL? MAY USE CONCURRENT ALPHA-2-IFN DYSTHYROID OPHTHALMOPATHY—THE MOST COMMON CAUSE OF BILAT (70%) OR UNILAT (30%) EXOPHTHALMOS IN ADULTS LID LAG–AND A GENERAL RESTRICTIVE OPHTHALMOPTHY (SYMMETRIC EOM SWELLING SPARING THE TENDINOUS ATTACHMENTS) ORBITAL PAIN IS NOT COMMON! THERE IS A RELATION WITH THE DEGREE OF HYPERTHYROIDISM AND THE SEVERITY OF THE ORBITOPATHY RT—GOOD IF EARLY IN A PROLIFERATIVE Dz 1/5 PTS GET ORBITOPATHY PRIOR TO THE HYPERTHYROIDISM PROPTOSIS SELDOM RESOLVES WALSH-OGURA—-DECOMPRESSED MEDIAL AND INFERIORLY—–KENNEDY USES THE ENDOSCOPES(OTHERS ALSO DO LAT—BALANCED DECOMPRESSION) ORBITAL PSEUDOTUMOR–90% UNILAT–SUDDEN PAINFUL PROPTOSIS A LYMPHOCYTIC INFILTRATE WITH POLYMORPHIC CELLULAR RESPONSE AND FIBROVASCULAR TISSUE LINKED WITH RIEDELS STRUMA IN SOME CASES SEEM TO HAVE VARYING GRADES OF SEVERITY AND CAN RANGE FROM SPONT RESOLUTION, TO INFLAMMATORY TO TUMEFACTIVE A NONSPECIFIC INFLAMMATION OF THE ORBIT—EOM MUSCLE ENLARGEMENT—INF RECTUS IS MOST COMMON LACRIMAL GLAND ENLARGEMENT—-STREAKY INTRACONAL FAT—SCLERAL ENHANCEMENT ?EVOLVE INTO A LYMPHOMA?A LOW GRADE LYMPHOMA?A GRANULOMATOUS Dz? PSEUDOTUMOR—-LOW GRADE ANGIOCENTRIC T-CELL LYMPHOMA? PERIVASCULAR CUFFING—A HIST PATH BUZZ WORD Rx: STEROIDS—?RT—–TOLOSA HUNT SYNDROME USUALLY NOT PROPTOTIC UVEAL SARCOIDOSIS CAN MIMIC PSUEDOTUMOR PRIMARY ORBITAL TUMORS LYMPHOID TUMORS REACTIVE LYMPHOID TUMORS TO LYMPHOMAS OR LEUKEMIAS PAIN ANDVISUAL CHANGES ARE RARE “FLESHY SALMON PATCH” APPEARANCE OF SUBCONJUNCTIVAL LESIONS THE TUMOR MOLDS TO THE ORBITAL BONES AND EXTENDS ALONG FASCIAL PLANES Dx WITH INCISIONAL Bx Rx: LIKE ANY OTHER LYMPHOMA—CHEMO/RT DERMOID CYST—REALLY A BENIGN FORM OF MATURE TERATOMA DYSONTOGENIC=DEFECTIVE EMBRYOGENESIS TERM USED LOOSELY FOR OVARIAN NEOPLASMS
IS SEQUESTERED ECTO AND MESODERM—LINED WITH ADNEXAL CELLS LOCATED IN THE S.Q.—BENEATH THE DERMIS—CAN BE ATTACHED TO THE UNDERSURFACE OF THE DERMIS SKIN MOVES OVER THEM ON PE PRIMARILY IN THE MIDLINE(TISSUE FUSION PLANES) OR IN THE LATERAL ORBIT OR THE SUBMENTAL REGION—–DO NOT CONFUSE WITH TGDC OR RANNULA—DOES NOT MOVE WITH TONGUE EXTRUSION OR DEGLUTTITION 30% IN THE H & N MAY COMMUNICATE WITH DURAL ECTODERM IN THE NOSE Rx: EXCISION—TAKE THE CAPSULE 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 RAPID ONSET OF BLUE RUBBERY MASS IN INFANCY 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 ACUTE HEMORRAGE INTO ONE OF THE CYSTIC SPACES–”CHOCOLATE CYST” 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 OPTIC NERVE TUMORS OPTIC NERVE GLIOMA-–(PILOCYTIC ASTROCYTOMA)—-LOW GRADE TUMOR OF CHILDHOOD FUSIFORM SHAPE—FROM THE OLIGODENDROGLIAL SUPPORTING CELLS WHICH COVER THE NERVE SLOWLY PROGRESSIVE PROPTOSIS AND VISUAL DEMISE
25% OF PTS HAVE NF1, 15% OF ALL PTS WITH NF1 HAVE OPTIC NERVE GLIOMAS—ALSO LOOK FOR LISCH NODULES = IRIS HAMARTOMAS OPTIC SHEATH MENINGIOMA USUALLY OF THE ARACHNOID CELLS (ARACHNOID CELL CLUSTERS “CAP CELLS” WITHIN THE DURA) USUALLY ADJACENT BONEY HYPEROSTOSIS TUBULAR SHAPE—”RAIL ROAD TRACK” APPEARANCE—CALCIFICATIONS = 20% OF CNS NEOPLASMS ONLY 2% ECTOPIC 2/100,000, FEMALE 2:1, INCREASED INCIDENCE WITH AGE AND RT EXPOSURE PRIMARILY MET TO LUNGS INCREASED ASSOC. WITH NF CLASSIC PATH: PSAMMOMA BODIES (LIKE PAPILLARY THYROID CA)=CALCIFICATIONS FOUR HISTO PATTERNS: SYNCYTIAL(POSYGONAL), TRANSITIOAL(PSAMMOMATOUS), FIBROUS, ANGIOBLASTIC + S-100, VIMENTIN, EMA(EPITHELIAL MEMBRANE ANTIGEN) Rx—-SURGERY, RU-486 (PROGESTERONE INHIBITOR) MAY HELP FOSTER-KENNEDY SYNDROME(SPHENOID MENINGIOMA OF THE OLFACTORY OR OPTID GROOVE) IPSI OPTIC ATROPHY–MARCUS GUN PUPIL CONRALATERAL PAPILLEDEMA (ICP FROM MASS EFFECT) CAN PRESENT WITH ATYPICAL BEHAVIOR AND PERSONALITY CHANGES AND ANOSMIA/PAROSMIA PERIPHERAL NERVE TUMORS PLEXIFORM NEUROFIBROMA IS THE MOST COMMON PERIPHERAL NERVE TUMOR IN THE ORBIT—VIRTUALLY PATHOGNEUMONIC OF NF USUALLY PRESENTS AS AN “S” SHAPED MASS IN THE UPPER EYELID OF A CHILD MESENCHYMAL TUMORS RHABDOMYOSARCOMA—THE MOST COMMON SOFT TISSUE MALIGNANCY IN PEDIATRICS (40% IN THE H&N) PRIMARY LOCATION IS THE ORBIT—FROM STRIATED MUSCLE THE MOST COMMON ORBITAL CANCER IN CHILDREN (LESS THAN 15 YEARS OF LIFE) 70% IN THE 1ST DECADE OF LIFE MOST OF THE EMBRYONAL AND ALVEOLAR CELL TYPES MOST HAVE A FULMINANT ONSET IN THE SUP ORBIT RARELY IN THE T-BONE—PRESENT WITH A HEMORRAGIC MASS IN THE EAC OFTEN WITH CN DEFICITS AND REGIONAL METS Dx: PROMPT INCISIONAL Bx Rx: COMBINATION THERAPY IS BEST—-SURG, CHEMO, RT TREAT WITH ALL POSSIBLE MODALITIES–DEBULK SURGICALLY, RT, CHEMO–PARAMENINGEAL=BAD Px HISTOLOGIC SUBTYPES—75% EMBRYONAL, BOYTRYOID, ALVEOLAR, PLEOMORPHIC PROGNOSIS=GRADE DESMIN AND VIMENTIN + FIBROUS HISTIOCYTOMA—-PRIMARY MESENCHYMAL TUMOR IN THE ADULT ORBIT MFH (MALIGNANT FIBROUS HISTIOCYTOMA)—–A HIGH GRADE AGGRESSIVE SARCOMA LACRIMAL GLAND TUMORS PLEOMORPHIC ADENOMA—THE MOST COMMON TUMOR PAINLESS GROWTH AND SMOOTH ADJACENT BONE EROSION—-LIKE THE PAROTID –POSSESS A PSUEDOCAPSULE–OFTEN NEED TO DO A ORBITOTOMY–EN BLOC EXCISION ADENOID CYSTIC CA-–PRIMARY CANCER—SUBACUTE ONSET OF PAIN—NEUROTROPIC, IRREG MARGINS, BONEY INFILTRATION, AND CALCIFICATION ON CT SCAN CARCINOMA EX PLEOMORPHIC ADENOMA—-POOR PX IF BILAT INVOLVEMENT—PROBABLY LYMPHOMA, SARCOID, SJOGRENS, ECT¼ INTRAOCULAR CA RETINOBLASTOMA—THE MOST COMMON INTRAOCULAR CANCER OF CHILDHOOD PRESENTS DURING THE FIRST 2 YEARS OF LIFE—LEUKOCORIA AND STRABISMUS 10% INHERETED—AUTO D (30% BILAT)–”2 HIT HYPOTHESIS” 90% SPORATIC 1/15,000 LIVE BIRTHS OFTEN PRESENT WITH AMBLYOPIA, STRABISMUS NEED NEURO W/U AS THEY CAN MET VIA THE OPTIC NERVE TO THE BRAIN THREE HISTO PATTERNS UNDIFFERENTIATED DIFFERENTIATED (MIXTRE OF PSEUDOROSSETTE FORMATION (HOMER-WRIGHT ROSETTES AND FLEXNER-WINTERSTEINER ROSETTES) ABUNDANT FLEURETTES UVEAL MELANOMA—MOST COMMON INTRAOCULAR CANCER OF ADULTHOOD HIGH PROPENSITY FOR DISTANT METS RAISED VS A BENIGN CHOROIDAL NEVUS (FLAT)
NPC, OLFACTORY NEUROBLASTOMA, CUTANEOUS MALIGNANCIES, AND SINONASAL CA