Here is the expanded and completed version of the notes, filling in what was missing from the previous response:
Evisceration, Enucleation & Exenteration - Complete Ophthalmology Residency Notes
Sources: Kanski's Clinical Ophthalmology 10th Ed.; Wills Eye Manual; Goldman-Cecil Medicine; Robbin's Pathology
1. EVISCERATION
Definition
Removal of all intraocular contents (uveal tract, retina, vitreous, lens) through the sclera, which is left behind together with the intact extraocular muscles.
Surgical Technique (Step-by-Step)
- Perilimbal conjunctival incision 360° - recess conjunctiva and Tenon's capsule a few mm from limbus
- Cornea removed - 360° limbal incision, start with scalpel/Graefe knife, complete with scissors
- Evisceration spoon inserted between uveal tract and sclera - scoop all intraocular contents
- Meticulous removal of all pigmented tissue from the scleral envelope - critical step (reduces sympathetic ophthalmia risk)
- Identify optic nerve head - cut around it to separate from remaining sclera (in some techniques)
- Scleral relieving incisions x2 on opposite sides of limbus (allows accommodation of implant)
- 22 mm ball implant placed within scleral envelope using an introducer
- Scleral closure with 6-0 absorbable sutures; patch of donor sclera if any ball exposed
- Tenon's capsule + conjunctiva closed in two layers with 7-0 absorbable sutures, burying knots
- Conformer placed in socket
Postop note: Chemosis and lid oedema are common
Indications
- Blind, painful eye (this is the preferred procedure for this indication)
- Blind, unsightly eye
- Phthisis is a relative contraindication (scleral shell cannot hold adequate implant)
- Severe trauma with no visual potential - only if no malignancy suspected
- Endophthalmitis (no suspected malignancy)
Advantages over Enucleation
- Better prosthesis motility - extraocular muscles remain naturally attached to sclera
- Less operative time
- Less bleeding
- Less disruption of orbital tissues → less enophthalmos risk
Key Contraindications
| Contraindication | Reason |
|---|
| Suspected intraocular malignancy | Globe disrupted; cannot get adequate histology; risk of tumour seeding |
| Phthisis bulbi | Scleral shell too contracted to hold implant |
Specific Note on Pain Relief
- Evisceration does not always eliminate pain because the posterior ciliary nerves are not cut (unlike enucleation where the optic nerve is transected); though this is debated clinically
2. ENUCLEATION
Definition
Surgical removal of the entire globe, with extraocular muscles detached then re-secured to the orbital implant.
Surgical Technique
- Conjunctival peritomy 360° with Westcott scissors
- Isolate all four rectus muscles - double-armed 6-0 absorbable sutures placed through each before detachment; muscles tagged and cut
- Superior and inferior oblique muscles cut
- Globe delivered forward from the orbit
- Optic nerve transected as far posteriorly as possible with curved enucleation scissors
- For retinoblastoma: minimum 10 mm of optic nerve must be excised (to clear potential retrolaminar spread)
- For uveal melanoma: maximal posterior optic nerve excision; minimal globe manipulation to avoid haematogenous dissemination
- Haemostasis with pressure and gauze
- Orbital implant (18-22 mm sphere) placed into Tenon's space / intraconal space
- Rectus muscles sutured to implant surface - improves prosthesis motility
- Tenon's capsule closed with absorbable sutures
- Conjunctiva closed
- Conformer placed
Indications
- Intraocular malignancy requiring histopathology (uveal melanoma, retinoblastoma group E) - globe must be kept intact
- Severe trauma with no visual potential where sympathetic ophthalmia risk is significant
- Blind, painful eye (if evisceration contraindicated or surgeon preference)
- Neovascular glaucoma refractory to treatment
Specific Indications by Tumour
Uveal Melanoma - Enucleation when:
- Large tumour size
- Optic disc invasion
- Extensive ciliary body or anterior angle involvement
- Irreversible loss of useful vision
- Poor patient motivation to retain eye
- Note: Always confirm correct eye by ophthalmoscopy after draping; keep globe manipulation minimal
Retinoblastoma - Enucleation when (Group E / advanced):
- Neovascular glaucoma
- Anterior chamber infiltration
- Optic nerve invasion
- Tumour occupies >50% vitreous volume
- Chemoreduction failure
- Diffuse retinoblastoma (poor visual prognosis, high recurrence risk)
- Note: Excise ≥10 mm of optic nerve; minimal eye manipulation; adjuvant chemotherapy (CEV) if retrolaminar or massive choroidal spread; external beam radiotherapy if optic nerve cut end involved
3. EXENTERATION
Definition
Removal of the globe plus all orbital soft tissue contents - fat, muscles, nerves, lacrimal gland, and potentially eyelids and orbital bones.
Surgical Technique (General)
- Skin incision around orbital rim (or through eyelids for total exenteration)
- Periosteum elevated from all orbital walls
- Entire orbital contents dissected free en bloc
- Specimen sent for histology
- Haemostasis; socket management
Variants
| Type | Extent | Used When |
|---|
| Subtotal / lid-sparing | Globe + orbital contents, eyelids preserved | Anteriorly sited tumours; eyelid skin used to line socket |
| Total | Globe + all contents + eyelids | Tumour involves eyelids |
| Extended | Above + orbital bone removal | Tumour invades bone or sinuses |
Indications
Malignant (main):
- Orbital malignancy (primary) unresponsive to other treatments
- Tumour invading orbit from: eyelids, conjunctiva, globe, adnexa - when other treatments very unlikely to succeed
- Sebaceous gland carcinoma, squamous cell carcinoma, basal cell carcinoma with orbital invasion
- Lacrimal gland malignancy with orbital extension
Non-malignant (rare):
- Orbital mucormycosis - life-threatening fungal infection, often in diabetics/immunocompromised; requires emergency debridement
Socket Rehabilitation after Exenteration
- Healing by secondary intention - socket granulates over weeks
- Split-skin graft lining
- Prosthesis attachment options:
- Adhesive to surrounding skin
- Mounted on glasses frame
- Osseointegrated magnets on orbital rim bones (best motility and retention)
4. ORBITAL IMPLANTS - Detailed
Why Volume Must Be Replaced
- Enucleation/evisceration removes ~7 ml of orbital volume
- Adult orbit volume ~30 ml
- Without replacement: prosthesis weight stretches lower lid → sags; motility poor; superior sulcus hollowing
Implant Materials
| Category | Material | Properties |
|---|
| Non-integrated (solid) | Silicone, acrylic (polymethylmethacrylate) | Smooth; no ingrowth; lower cost; migration possible |
| Integrated (porous) | Hydroxyapatite (HA), Porous polyethylene (Medpor) | Fibrovascular ingrowth → natural coupling with orbital tissues; better motility; lower extrusion risk when well-vascularised |
Sizes
- Standard implant: 18-22 mm sphere
- Typical: 20 mm for adult enucleation
- Evisceration: 22 mm ball (scleral envelope accommodates slightly larger)
Peg System (Integrated Implants)
- A titanium peg can be drilled into porous implant after fibrovascularisation (typically at 6 months)
- Peg must be covered by intact conjunctiva/socket tissue before placement
- Provides direct coupling between implant movement and prosthesis
- Complication: peg site granuloma, discharge, exposure
Key Surgical Principle
Correct placement technique is more important than implant material choice - implant must be sufficiently deep and covered with well-vascularised tissue to prevent extrusion
Implant Complications
| Complication | Notes |
|---|
| Extrusion | 3-5% with any implant; more common if shallow placement or poor tissue coverage |
| Migration | Less common with porous vs. solid implants |
| Infection | Rare; can lead to implant removal |
| Exposure | Thinning of overlying conjunctiva; repair with patch graft (donor sclera, amniotic membrane) |
5. POST-ENUCLEATION SOCKET SYNDROME (PESS)
Definition
A constellation of signs resulting from orbital volume deficit after enucleation. More common without an implant.
Pathophysiology
- No implant (or inadequate implant) → volume deficit
- Upper lid loses support → ptosis
- Prosthesis tilts posteriorly and rotates upward
- Prosthesis weight/pressure on lower lid → shallow inferior fornix + lower lid lag/sag
- Fat atrophy over time worsens the deformity
Clinical Features (PESS tetrad)
- Enophthalmos (apparent)
- Upper lid ptosis
- Superior sulcus hollowing/deepening
- Sagging/lower lid ectropion
Management
| Finding | Treatment |
|---|
| No implant present | Insert secondary orbital implant |
| Implant present but volume deficient | Silicone block into inferior orbit |
| Significant fat atrophy | Dermis-fat graft |
| Isolated ptosis | Levator advancement / ptosis repair |
| Fornix shallowing | Fornix reconstruction |
6. ANOPHTHALMIC SOCKET - CARE AND COMPLICATIONS
Normal Socket Examination
- Residency tip: Always remove the prosthesis to examine the socket - this is frequently neglected
- Inspect conjunctiva, fornices, implant position, socket discharge
- Work with a trained ocularist for long-term prosthesis management
Discharging Socket
- Mild mucoid discharge: normal; clean with saline
- Purulent discharge: infection; swab and treat
- Persistent discharge: consider implant exposure/extrusion
Prosthesis Fitting Timeline
- Intraoperative: conformer placed
- 6-8 weeks post-op: socket impression; temporary prosthesis
- 3-6 months: custom-fitted, colour-matched permanent prosthesis
- Annual: prosthesis polish and check; replace every 5-7 years
Superior Sulcus Syndrome (Stock Eye Syndrome)
- Occurs when a standard ("stock") prosthesis doesn't match the individual socket
- Prosthesis too small → doesn't fill volume → superior sulcus depression
- Solution: custom prosthesis; fat grafting; orbital volume augmentation
Prosthesis Motility Limitation
- Implant movement transmitted to prosthesis: ~10-15° (conversational range) - adequate for social interaction
- Not at extremes of gaze
- Enhanced by: rectus muscle attachment to implant, porous implant material, pegging
7. SYMPATHETIC OPHTHALMIA (SO) - Full Notes
Background
- Bilateral granulomatous panuveitis triggered by penetrating ocular injury or intraocular surgery
- "Exciting eye" = injured eye; "Sympathizing eye" = fellow eye
- Uveal antigens exposed by injury → T-cell mediated autoimmune attack on both eyes
- Can occur days to decades after injury (peak: 2 weeks to 3 months; 80% within 1 year)
Histology
- Diffuse granulomatous infiltration of the choroid by lymphocytes and epithelioid cells
- Dalen-Fuchs nodules: granulomas between Bruch membrane and RPE - pathognomonic
Prevention with Surgery
- Enucleation OR evisceration within 10-14 days of penetrating injury with no visual potential
- Based on anecdotal evidence; no RCT proof
- Evisceration acceptable only if all uveal tissue meticulously removed
- Once SO has developed in the sympathizing eye, enucleating the exciting eye does not necessarily prevent progression but may reduce severity
Treatment
- High-dose oral prednisolone - mainstay; taper over months based on response
- IV methylprednisolone - for initiation in severe cases
- Topical steroids + cycloplegics - for anterior segment involvement
- Peri/intraocular steroids (slow-release intravitreal implants) - reduce systemic load
- Immunomodulatory therapy (methotrexate, mycophenolate, azathioprine, cyclosporine) - start early in course
8. SPECIAL SCENARIOS & EXAM PEARLS
Trauma Setting
- Primary evisceration/enucleation: extremely severe injuries where scleral repair is impossible and no visual prognosis
- Reduces rehabilitation time; allows rapid return to work
- Secondary evisceration/enucleation: after failed primary repair - blind, damaged, unsightly, uncomfortable eye
- Both are safe with low infection and complication rates
Neovascular Glaucoma (NVG)
- Enucleation or evisceration as last resort if all other treatments fail
- Treat underlying cause to protect fellow eye
Choosing Evisceration vs. Enucleation - Summary Decision Tree
Suspected intraocular malignancy?
YES → ENUCLEATION (histology mandatory)
NO ↓
Phthisis bulbi?
YES → ENUCLEATION (sclera too contracted for evisceration)
NO ↓
Blind painful/unsightly eye, endophthalmitis, trauma with no visual potential?
→ EVISCERATION (preferred: better motility, simpler, shorter)
Anaesthesia Options
- Both procedures can be performed under local (peribulbar/retrobulbar) anaesthesia in selected patients
- General anaesthesia more common for surgical comfort
9. COMPLETE COMPARISON TABLE
| Feature | Evisceration | Enucleation | Exenteration |
|---|
| Structure removed | Intraocular contents | Entire globe | Globe + all orbital contents (±eyelids, ±bone) |
| Sclera | Retained | Removed | Removed |
| Extraocular muscles | Intact on sclera | Detached → re-sutured to implant | Removed with specimen |
| Optic nerve | Not cut | Cut (long stump) | Removed with specimen |
| Posterior ciliary nerves | Intact (pain may persist) | Cut (better pain relief) | N/A |
| Prosthesis motility | Best | Good | Adhesive/glasses/magnets |
| Histology | Disrupted - UNSUITABLE for malignancy | Full globe available | Full specimen |
| Intraocular malignancy | CONTRAINDICATED | Indicated | If orbital extension |
| Phthisis bulbi | CONTRAINDICATED | Acceptable | N/A |
| Sympathetic ophthalmia | Remove all uvea to minimize risk | Eliminates exciting eye | N/A |
| Post-op volume deficit | Less (sclera + muscles intact) | More (need implant) | Most (large cavity) |
| Operative time | Shorter | Longer | Longest |
10. HIGH-YIELD MNEMONICS
PESS tetrad = PUSH (Ptosis - Upper lid, Superior sulcus hollowing, lower lid Hanging)
Evisceration contraindications = PM (Phthisis + Malignancy)
Exenteration indications = MOB (Malignancy, Orbital invasion, [orbital] Mucormycosis - [life-threatening] Bug)
Enucleation for retinoblastoma: cut ≥10 mm optic nerve; adjuvant CEV if retrolaminar spread
Implant extrusion prevention: depth of placement > material choice
References: Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (Chapters 4, 12, 20, 22); Wills Eye Manual; Goldman-Cecil Medicine; Robbins Pathology