STEPTRACKERSPECIAL SENSES + ONCOLOGY | PATHOLOGY + MICROBIOLOGY + GENETICS | HIGH-YIELD REVIEW

Orbit & Ocular Tumors

Infections behind and in front of the septum, the swollen orbit of thyroid disease, and the tumors of the eye in children and adults.

FOCUS ON
Orbital cellulitis (behind the septum): pain with eye movement, ophthalmoplegia, proptosis, ↓ vision — often from ethmoid sinusitis; IV antibiotics, CT. Preseptal: lid swelling only. Graves orbitopathy: proptosis, lid retraction. Retinoblastoma: leukocoria, RB1. Uveal melanoma: most common primary intraocular tumor in adults, liver metastases.
DISORDER
CAUSE · MECHANISM
FEATURES
CLUE · TREATMENT
Preseptal (periorbital) cellulitis
Infection anterior to the orbital septum; skin trauma, insect bite, sinusitis
Red swollen eyelids; normal eye movements and vision, no proptosis
Oral antibiotics
Orbital cellulitis
Spread from ethmoid sinusitis through thin lamina papyracea; S aureus, strep, H influenzae
Fever, pain with eye movement, ophthalmoplegia, proptosis, ↓ vision, chemosis
CT orbits · IV antibiotics · abscess drainage
Cavernous sinus thrombosis
Spread of facial/orbital infection via valveless ophthalmic veins
Bilateral eye signs, CN III, IV, V1, V2, VI palsies, headache, fever
"Danger triangle" of the face
Rhino-orbital mucormycosis
Mucor/Rhizopus in DKA, neutropenia; angioinvasive
Black necrotic eschar of nose/palate, proptosis, ophthalmoplegia
Emergency debridement + amphotericin B
Graves orbitopathy
TSH-receptor antibodies + T cells activate orbital fibroblasts → GAGs, fat, muscle swelling
Proptosis, lid retraction, stare, diplopia, exposure keratopathy; compressive optic neuropathy
Worse with smoking and radioiodine · steroids, teprotumumab
Endophthalmitis
Infection inside the globe after surgery, trauma or bacteremia/candidemia
Severe pain, ↓ vision, hypopyon, vitreous haze
Intravitreal antibiotics
Retinoblastoma
most common intraocular tumor in children
RB1 loss (chr 13); heritable (germline, bilateral, early) or sporadic (unilateral)
Leukocoria (white pupillary reflex), strabismus; small round blue cells, Flexner-Wintersteiner rosettes
Germline → osteosarcoma later
Uveal melanoma
most common primary intraocular tumor in adults
Choroid melanocytes; GNAQ/GNA11, BAP1
Often asymptomatic; blurred vision, detachment, pigmented mass on exam
Hematogenous spread to liver
Metastases to the eye
most common intraocular malignancy overall
Choroid seeded by blood: breast, lung
Yellow choroidal masses, often bilateral
Known cancer + new visual symptoms
Orbital tumors
Children: rhabdomyosarcoma, optic glioma (NF1), dermoid · adults: lymphoma (MALT), cavernous hemangioma
Painless progressive proptosis
Rapid proptosis in a child → rhabdomyosarcoma
Eyelid malignancy
UV exposure; meibomian glands
BCC (most common, lower lid) · sebaceous carcinoma (recurrent "chalazion") · SCC
Biopsy recurrent lid lesions

Mechanism — sinus to orbit

Ethmoid sinusitis
→
Thin lamina papyracea+ valveless veins
→
Orbital cellulitis / subperiosteal abscess
→
Cavernous sinus thrombosisMeningitis, brain abscess

The orbital septum is the barrier: in front of it, only the lids swell; behind it, muscles, nerve and globe are involved.

Preseptal vs orbital cellulitis

FEATURE
PRESEPTAL
ORBITAL
Lid swelling
Yes
Yes
Pain with eye movement
No
Yes
Ophthalmoplegia
No
Yes
Proptosis
No
Yes
Vision
Normal
May be ↓, RAPD
Management
Oral antibiotics
Admit, IV antibiotics, CT

How it's tested

COMMON CONFUSIONLeukocoria in an infant can be retinoblastoma, congenital cataract or retinopathy of prematurity — retinoblastoma is the one that can kill.
THE TRAPA child with bilateral retinoblastoma has the germline RB1 mutation; siblings need screening, and the child faces sarcomas later in life.
YOUR APPROACHSwelling in front of or behind the septum → eye movement and vision → source (sinus, skin, blood) → host (DKA, neutropenia) → or mass: age, location, gene, spread.

If you see… think…

Child with sinusitis, proptosis and painful eye movements → orbital cellulitis
Swollen red lids with normal eye movements after a bug bite → preseptal cellulitis
Bilateral ophthalmoplegia after a facial boil → cavernous sinus thrombosis
DKA with black nasal eschar and proptosis → mucormycosis
Proptosis and lid retraction in hyperthyroidism → Graves orbitopathy
White pupil in a flash photo of a toddler → retinoblastoma
Bilateral retinoblastoma, later osteosarcoma → germline RB1
Adult with choroidal pigmented mass, later liver lesions → uveal melanoma
Rapidly growing orbital mass in a 6-year-old → rhabdomyosarcoma
"Chalazion" that keeps recurring in an older adult → sebaceous carcinoma