Enucleation, Evisceration and Exenteration.docx

7/27/2019 Enucleation, Evisceration and Exenteration.docx

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Enucleation, Evisceration and Exenteration

IntroductionThe surgical procedure to remove an eye should be performed with efforts to minimize cosmetic

deformity. The anophthalmic socket must be able to hold a prosthesis and allow movement of the

prosthesis that matches the movements of the contralateral eye. The eyelids also must bepositioned so their movement matches the contralateral eyelids. To achieve this, a socket with

the following characteristics is required:

A centrally placed, buried, inert implant of adequate size attached to the extraocularmuscles

Deep fornices A lower eyelid that can support a prosthesis A superior eyelid with position and movement symmetric to the normal eyelid Prosthesis and implant volume equal to that of the natural eye An implant peg that couples with the prosthesis

These characteristics apply to both patients undergoing enucleation and patients undergoing

evisceration. Many different implants and techniques can be used to meet these criteria.

Orbital Implants in Anophthalmos

Implants can be integrated or nonintegrated. Nonintegrated implants have no direct attachmentsto the extraocular muscles and are usually inert spheres of silicone or methylmethacrylate, placedin Tenon's capsule within the muscle cone, deep to anterior Tenon's fascia and conjunctiva. The

rectus muscles may be sutured together anterior to the implant.

With an integrated implant, the rectus muscles are attached directly to it. Allen implants havetunnels into which the muscles are attached. With spherical integrated implants made of silicone

or hydroxyapatite, the muscles are sutured to a wrap consisting of sclera, fascia, or an artificialsubstance. Polyethylene implants can be sutured directly and, therefore, do not require a wrap.

They are significantly less expensive than hydroxyapatite implants.

Before 1985, glass, acrylic, or silicone implants were used. Since then, coralline hydroxyapatitespheres have become available. These spheres are popular because they are porous and,

therefore, allow fibrovascular ingrowth. This reduces the incidence of extrusion, migration, and

infection; however, the pores may trap infectious agents during placement. These implants arenot recommended for use in immunocompromised patients or patients with panophthalmitis.
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Slide 1

Implant porosity and fibrovascular ingrowth allow drilling and placement of a peg for direct

coupling to the prosthesis. The peg is fitted into an indentation into the back of the prosthesis somovement of the prosthesis is equal to that of the implant and the other eye.

1The peg also

supports the weight of the prosthesis, preventing lower eyelid sag if the prosthesis is large. Many

patients with porous implants do not require peg insertion; insertion rates range from 11% to76% in published series.

2Porous spheres made from polyethylene, ceramic,

3and bovine

4and

manufactured hydroxyapatite5

have later become available.

Sclera has been the most commonly used wrap for implants. However, the occurrence oftransmissible diseases related to sclera wraps have resulted in a rise in popularity of other

implant wraps. Autogenous tissue, such as fascia lata and posterior auricular muscle complex,

and synthetic materials, such as high porosity expanded polytetrafluoroethylene, have been usedeffectively.

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Implant Size

The socket tissue, implant, and prosthesis must have a total volume equal to the volume of thenatural eye to achieve symmetry(Slide 1). The size of the prosthesis selected is limited by the

weight that can be supported by the lower eyelid. Implants typically are less than 3 cc in volume.

In patients requiring a larger prosthesis, a peg can be used for additional support. The thicknessof the prosthesis is limited by the depth of the anterior chamber. Increased thickness is requiredto allow for an indentation for a peg. The ideal volume of a prosthesis is 2 cc to 2.5 cc.

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The volume of implant used should be equal to the volume of the enucleated eye (pre disease ifphthisical) minus the volume of the prosthesis. If the prosthesis size is 2.5 cc and the volume of

an eye is 7.2 cc with an axial length (AL) of 24 mm, then the implant volume should be 4.7 cc. Ifspherical, the diameter is 21mm (Table 1).

8

Globe volume - prosthesis volume = implant volume

7.2 cc (24 mm AL) - 2.5 cc = 4.7 cc (21 mm diameter)
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A scleral wrap adds approximately 1.5 mm to the diameter of the implant. See Table 1 to

calculate implant size for different sized eyes. An intermediate implant size can be created by

shaving a larger implant.

Natural eyeDiameter

(mm)

Natural

eye

Volume(cc)

Prosthetic

eye Volume

(cc)

Implant

volume

required (cc)

Implant diameter

required (mm)

unwrapped

20.0 4.19 2.5 1.69 15.0

20.5 4.51 2.5 2.01 15.5

21.0 4.85 2.5 2.35 16.5

1.5 5.21 2.5 2.71 17.5

22.0 5.58 2.5 3.08 18.0

22.5 5.97 2.5 3.47 19.0

23.0 6.37 2.5 3.87 19.5

23.5 6.80 2.5 4.30 20.024.0 7.24 2.5 4.74 21.0

24.5 7.70 2.5 5.20 21.5

25.0 8.18 2.5 5.68 22.0

25.5 8.69 2.5 6.19 23.0

Enucleation

Enucleation is the removal of the entire eyeball, which is usually replaced with a spherical orbitalimplant.

Indications for enucleation include:

Intraocular malignancy untreatable by other means Ruptured globe, resulting in a blind eye, with risk of sympathetic ophthalmia A blind eye producing sympathetic ophthalmia A blind, painful eye, usually secondary to end-stage glaucoma, retinal detachment,

chronic inflammation, or trauma

In enucleation surgery, when removing the diseased tissue, a socket must be created that is large

enough to hold an adequate prosthesis and preserves the most conjunctiva possible. The implant

must be of adequate volume with good movement.9,10

TechniqueGeneral anesthesia is preferred during an enucleation procedure, although local anesthesia with

sedation may also be used. A retrobulbar injection of 0.75% bupivicaine and 1:100,000

epinephrine is administered even with general anesthesia to achieve hemostasis and preventpostoperative pain.


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Slide 2

A 360 peritomy, with lateral and medial relaxing incisions, is made. The four rectus muscles are

dissected out, double-end 6-0 polyglactin sutures are attached, and the muscles are divided attheir insertions. The oblique muscles are divided. The optic nerve is divided using a snare, which

is slowly tightened, or by clamping for several minutes then cutting with scissors. These methods

promote hemostasis by crushing the central retinal vessels. Further hemostasis is achieved withbipolar cautery, thrombin, and Gelfoam.

The surgeon should confirm that the predetermined implant will fit with sizing spheres placed inthe muscle cone. If it does not fit, then the largest fit is used.


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Slide 3

The sclera is prepared by turning it inside out and removing any residual choroid. Relaxingincisions are made on either side of the hole where the cornea was located to allow placement of

the implant within. The corneal hole becomes the posterior pole and the optic disc becomes the

anterior pole when placed in the socket. Holes are cut for each of the rectus muscles adjacent tothe optic disc. The holes can be cut using a 2-mm circular punch biopsy blade and making two or

three adjoining cuts. The implant is placed within the sclera and secured by partially closing the

sclera with 5-0 polyester sutures. The rectus muscle holes and the posterior hole where thecornea was removed allow fibrovascular ingrowth.

The implant is placed through posterior Tenon's fascia into the muscle cone. The rectus musclesare attached to the anterior lips of the scleral windows with 6-0 polyglactin sutures(Slide 2).

Posterior Tenon's fascia is pulled forward in quadrants between the rectus muscles and a 6-0

polyglactin purse-string suture used to close this layer in front of the implant(Slide 3). AnteriorTenon's fascia is closed horizontally with interrupted 6-0 polyglactin sutures and conjunctiva

closed horizontally with continuous 6-0 polyglactin sutures(Slide 4).
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Slide 4

Antibiotic ointment is placed in the socket. A conformer that places no tension on the woundwith the lids closed should be placed. A pressure dressing is applied and left on for 48 hours. A

prosthesis is fitted 4 to 8 weeks after enucleation when all swelling has settled.

Slide 5

When the implant is vascularized, usually after 6 to 12 months, the implant can be drilled and a

peg placed. Vascularization can be confirmed with computed tomography (CT) scan with

contrast sensitivity, magnetic resonance imaging (MRI) with gadolinium, or a Technetium-99bone scan. A number of small drill holes made into the implant at the time of surgery will

increase the rate of vascularization. Initially, single-piece plastic pegs were used. These havebeen associated with complications such as loosening, extrusion, coral exposure, pyogenic

granuloma, and infection. Newer titanium, screw in, sleeve and peg systems may decrease thesecomplications.

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The peg is inserted using local anesthetic under sterile conditions. To help correctly place the

peg, a template can be made by the prosthetist with a hole at the anterior pole of the implant. The

conjunctiva is cut and cauterized, then, with the template in position, a hole is drilled. The sleeve


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is screwed in and a flat peg placed in the sleeve, over which the prosthesis is worn. After 4

weeks, the prosthetist replaces the flat peg with a round peg and makes an impression in the back

surface of the prosthesis into which the peg fits. This produces prosthesis movement equal to thatof the implant(Slide 5andSlide 6).

ComplicationsPotential complications of enucleation include the following:

Slide 6

Removal of the wrong eye can be avoided by marking the forehead on the side forenucleation while the patient is awake. In a normal looking eye, dilate the pupil and

confirm the presence of tumor. This reassures the patient.12

Incomplete enucleation is important to avoid in patients with intraocular tumors andinfection to prevent seeding the orbit. With retinoblastoma, as much optic nerve as

possible, perhaps 10 mm, should be removed because this tumor spreads down the nerve.

Traction sutures at the horizontal rectus muscle insertions can be used to pull the globeforward.

Hemorrhage can be minimized by achieving hemostasis and ceasing anticoagulants anadequate time preoperatively.

Swelling and hematoma formation can be minimized by padding the eye postoperativelyfor 48 hours.

Infection of the implant may be treated with systemic antibiotics if it is vascularized. Ifthe implant is not vascularized, then removal is usually necessary with replacement later.
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Systemic antibiotics perioperatively and soaking the implant in antibiotics will help

prevention.

Wound breakdown with implant exposure is the most common complication withhydroxyapatite implants, more so than with silicone implants, with reports of 3% to 20%

versus 1% to 5%.13,14

This may occur early due to tension on the wound or later due to

pressure of a poorly fitting prosthesis. Discharge, pyogenic granuloma formation, andinfection can occur.

Tension on the wound can be avoided by careful hemostasis, posterior placement of theimplant by anterior attachment of the rectus muscles, closure of posterior Tenon's fascia

anterior to the implant, ensuring the conformer does not pull on the wound with the eyeclosed, a pressure bandage for 48 hours, and ensuring the implant is not too large.

Wrapping the hydroxyapatite implant is important because the coarse nature of its surface

can cause breakdown of the conjunctiva and Tenon's capsule over it.15

One study

suggested that autologous wrapping may decrease exposure.16

A poorly fitting prosthesis may cause erosion and should be modified. Implant exposure of less than 3 mm may heal spontaneously. A defect with exposure of a

vascularized implant may also heal spontaneously. A persistent defect should be repairedwith a patch graft of sclera or fascia covered by a flap of conjunctiva. The patch should

be twice the size of the exposed area and placed in a pocket between the conjunctiva and

Tenon's capsule. A bucket handle flap of conjunctiva should be mobilized above and

brought down to cover the patch. The defect above is left to epithelialize.17

Alternatively, free autogenous tissue grafts of hard palate mucosa or thin dermis-fat graftmay be used after burring of the hydroxyapatite and freshening of the conjunctival

edges.18

Implant extrusion rarely occurs with porous implants. It occurs with wound breakdownand epithelial ingrowth around a nonporous implant.

Migration outside the muscle cone may occur with a nonwrapped, nonporous implant.Closing the defect in posterior Tenon's capsule through which the optic nerve passedprior to implantation may prevent this. A migrated implant is removed and replaced by an

integrated implant.

Anophthalmic enophthalmos, or deep superior sulcus syndrome, or post enucleationsocket syndrome occurs when the implant is too small or has migrated inferiorly. There is

enophthalmos and a deep upper sulcus. Ptosis and lower lid sagging may also be present.

If the implant is too small, ptosis and lower lid sagging may be corrected by enlarging theprosthesis. However, the larger prosthesis often causes discomfort and the extra weight

may not be adequately supported by the lower eyelid. Replacement with a larger implant

or an orbital floor implant may be required.

A socket with a short inferior fornix cannot hold an implant. Deepening the fornix isdifficult and requires mucous membrane grafting.

Inadequate movement of the prosthesis may be improved by placing a peg into theimplant, if it is porous and vascularized.

Ptosis should be corrected using an anterior approach so that the conjunctiva is preserved. Conjunctival cyst formation can present as an enlarging mass. A CT scan can help in

diagnosis; then the cyst is excised.

Discharge and irritation caused by the prosthesis is common and giant papillaryconjunctivitis may occur. Treatment involves ensuring that the prosthesis fits properly,


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eliminating protein deposits on the prosthesis and treating with steroid drops, long-term if

necessary.

Retardation of orbital growth may occur with enucleation in childhood. A dermis-fatgraft may grow with the patient and, therefore, may help prevent this retardation.

Peg problems including discharge, pyogenic granuloma, peg falling out, poor transfer ofmovement, clicking, the conjunctiva overgrowing the peg, a peg drilled at an angle or offcenter, hydroxyapatite exposure around the peg hole, excess movement of peg, andimplant infection occurred in 37% of pegged implants in one study.

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Evisceration

Evisceration is the removal of the contents of the eyeball, leaving the sclera (and sometimes the

cornea) intact and preserving the extraocular muscle attachments.

The advantages of evisceration over enucleation are that evisceration is easier to perform,quicker, and less disruptive of the orbital contents than enucleation. Also, it provides better

movement20

and a better cosmetic result than enucleation. Extrusion is less frequent.

Psychologically, evisceration may be better accepted by patients because the whole eye is notremoved. With intraocular infection, the subarachnoid space is not opened so the theoretical risk

of infection spread into the brain is avoided.

Disadvantages of evisceration include the risk of sympathetic ophthalmia21,22

and difficulty in

placing an implant in severe phthisis. It is contraindicated in patients with intraocular tumor andif there is a need for histological examination of the entire globe.

Indications for evisceration include:

Slide 7

A blind, painful eye (if intraocular malignancy can be eliminated)


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Corneal perforation Endophthalmitis (evisceration is preferred in this situation to prevent intracranial spread

via incised dura)

Aims of evisceration are to eliminate the disease process and produce a comfortable socket with

a cosmetically acceptable prosthesis. The requirements for this have been described above in theIntroduction.23

TechniqueMany different techniques both with and without keratectomy have been described.

24-26

Described below is one method, with keratectomy, which is applicable to most situations in

which an evisceration is indicated.

Slide 8

Local anesthesia with intravenous sedation may be used, although general anesthetic is oftenpreferred to decrease patient anxiety.

A 360 peritomy with horizontal relaxing incisions is performed. The cornea is excised andrelaxing incisions made at 45 (i.e., between the rectus muscles)(Slide 7). The ocular contents

are removed carefully ensuring no choroidal tissue is left. Hemostasis is achieved with cautery.The size of the cavity should be checked with sizing spheres. If, as is usual, the cavity is too

small for the required implant, then an expansion sclerostomy may be required. One techniqueinvolves making a posterior scleral incision at 90 to the anterior scleral relaxing incisions(Slide

7). This should extend around the optic nerve and forward to the equator at both ends so the

implant can protrude posteriorly through it. This also helps with fibrovascular ingrowth into aporous implant. The implant is placed into the sceral shell and the sclera is closed anteriorly with

interrupted vertical mattress sutures of 5-0 braided polyester so the edges overlap(Slide 8). The
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anterior Tenon's fascia is closed with interrupted 6-0 polyglactin sutures and the conjunctiva

closed with continuous 6-0 polyglactin sutures(Slide 9).

Implants can consist of glass, silicone, methylmethacrylate, hydroxyapatite, or polyethylene. The

porous implants, hydroxyapatite and polyethylene, allow fibrovascular ingrowth and, therefore,

can be drilled for peg placement later to improve prosthesis motility.

Slide 9

Complications

Potential complications of evisceration include the following:

Hemorrhage within the sclera may cause expulsion of the implant if there is no posterioropening.

Infection of the implant may be treated with systemic antibiotics if it is fullyvascularized. If the implant is not vascularized, then removal and later replacement arerequired. If the implant must be removed, it can be replaced with a prosthesis implanted

behind the posterior sclera, which provides an extra layer of cover.

Wound breakdown with implant exposure may be due to tension on the wound orpressure of a poorly fitting prosthesis. Wound tension can be relieved by removing the

implant and performing a posterior scleral relaxing incision, and replacing the prosthesis

with a smaller implant or burring down of the implant. The defect can also be coveredwith a flap or patch graft.

Implant extrusion rarely occurs with porous implants; it occurs with wound breakdownand epithelial ingrowth around a nonporous implant. In these cases, the implant should be

replaced.

Inadequate movement of the prosthesis may be improved by placing a peg into theimplant, if it is porous and vascularized. This fits into an indentation in the prosthesis to

produce movement equal to that of the implant.

Discharge and irritation caused by the prosthesis is common and giant papillaryconjunctivitis may occur. Treatment involves ensuring a proper fit of the prosthesis,

eliminating protein deposits on the prosthesis, and treating with steroid drops, long-termif necessary.
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Dermis-fat graft

The dermis-fat graft is an alternative to an implant and is useful when there is a contracted socketand inadequate socket volume. It can be used after enucleation or evisceration and has been most

often used to replace an extruded implant.

During the procedure, general anesthesia is used. The donor site of the graft may be the non-weight-bearing lower abdomen or thigh. The dermis-fat graft should be in contact with orbital fat

to enhance graft viability. Thus, Tenon's fascia, sclera, or pseudocapsule left after implantextrusion should be incised or excised to facilitate this.

The graft is harvested by first marking a 25-mm circle. The epidermis and superficial dermis are

excised using a scalpel. An incision is made into subcutaneous tissue to a depth of 15 mm and

the graft is placed into the socket.

The edge of the dermis is sutured to the rectus muscles, in enucleation, or to the anterior scleral

ring, in evisceration. Tenon's capsule and conjunctiva are approximated to the dermiscircumferentially. Excess fat may be removed; however, over correction of 20% to 30% isnecessary to allow for graft atrophy. If there is enough conjunctiva, this can be sutured to cover

some or all of the dermis.

Full epithelialization of the dermis occurs after 4 weeks, so the prosthesis may be fitted after thistime.

Complications

Complications of dermis-fat grafting include the following.

Fat atrophy and volume loss are variable and may require further dermis-fat grafting.

Graft failure usually is associated with a compromised orbital vascular supply afterradiation treatment or chemical injury. It may be more likely to occur in patients withadvanced age, infection, vascular disease, or diabetes, patients who experienced graft

trauma from manipulation, excessive cautery, or inadequate hemostasis, and patients who

smoke.

Minor complications include conjunctival cysts, granulomas, graft ulcers, socketkeratinization and cilia retention.

17

Exenteration


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Slide 10

Exenteration is the removal of the entire contents of the orbit, which is usually performed toremove life-threatening orbital malignancy or infection, such as mucor mycosis. It may also be

done for severely disfiguring or painful conditions such as orbital lymphangioma or sclerosing

pseudotumor. Exenteration may be total, subtotal, or extended. Subtotal exenteration spares theeyelids and can be performed when the disease is behind the globe. Extended exenteration

includes removing surrounding bone. Histopathological diagnosis with permanent sections along

with patient evaluation for metastatic disease should be made before exenteration.27

Slide 11


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Technique

Total exenteration. General anesthesia is required during total exenteration. The procedure isbegun by marking the position of the orbital rim on the skin. The surgeon should infiltrate along

the incision with local anesthetic, and 1:100,000 epinephrine is administered for hemostasis.

Next, an incision is made down to the orbital rim, through the periosteum, to expose bone(Slide10). The periosteum is elevated from the orbital bones. Tissue is divided at the inferior and

superior orbital fissures and the nasolacrimal duct. Care must be taken not to perforate the thin

bone of the medial orbital wall as this will cause sinus-orbit fistularization. The soft tissue orbitalcontents are transsected as far posteriorly as possible and removed(Slide 11). Hemostasis is

achieved with bipolar cautery to avoid damage to adjacent cavernous sinus or middle cranial

fossa contents.

The cavity is then packed with antibiotic- and saline-soaked gauze and allowed to granulate overthe next 8 to 12 weeks. During this period, the gauze should be removed, the cavity cleaned and

repacked every 3 days. Alternatively, and for faster rehabilitation, a split skin graft can be used

to line the cavity. This should be sutured to the surrounding skin and either meshed or puncturedto allow drainage of blood and exudate. It is packed and changed regularly.

Slide 12

When healed, a prosthesis is fitted to an eyeglass frame or to titanium pegs inserted into the

orbital rim. Alternatively, a black patch may be worn.28

Subtotal exenteration. When performing a subtotal exenteration, an incision is made around the

palpable fissure, outside the lashes(Slide 12). The dissection plane is between the orbicularismuscle and orbital septum to the orbital rim(Slide 13). The periosteum is incised and the orbital

contents removed as described above for the total exenteration procedure. A drain tube is placed

in the orbit and the lids sutured together.
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Complications

Complications of subtotal exenteration include the following:

Slide 13

Sensation loss in the first division of the trigeminal nerve occurs due to removal of thatnerve.

A leak of cerebral spinal fluid may occur if there is damage to the roof of the orbit. Thisis life threatening and is treated initially with antibiotics. The leak is then repaired withtemporalis muscle flap through the lateral orbital wall, or a mid forehead skin flap, onto

the defect.

Fistularization to a sinus may occur if there is damage to the medial wall or floor of theorbit. This may cause discharge into the socket or difficulty wearing the prosthesis. It

may be repaired by rotation flap from adjacent orbital wall or with a temporalis orforehead flap.

Skin graft necrosis is treated by debriding then regrafting or allowing granulation andepithelialization.

After identification of the organism, infection is managed with frequent cleaning anddressing of the orbit and topical and systemic antibiotics.

Cluster headaches on the side of the exenteration have been reported. These headachesare treated with oral analgesia.

Tumor recurrence requires further excision with or without adjuvant chemotherapy orradiotherapy.


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DEFINITIONS

Enucleation: surgical removal of the entire globe

Evisceration: surgical removal of the entire contents of the globe leaving a scleral shell Exenteration: removal of the entire orbit including the globe, eyelid, and orbital contents

usually performed for malignant tumors

INTRODUCTIONEnucleation, evisceration, and exenteration surgery all involve the permanent removal of thepatients eye. In this chapter the important aspects of each procedure are emphasized, including:

Indications for surgery

Preoperative patient counseling Surgical techniques

Postoperative management

Complications of surgery

PREOPERATIVE EVALUATION AND DIAGNOSTIC APPROACHIndications for Surgery

Enucleation or evisceration surgery may be indicated for a blind painful eye, endophthalmitis, or

cosmetic improvement of a deformed eye. In cases of intraocular neoplasms or the treatment ofsevere ocular trauma with a ruptured globe, where sympathetic ophthalmia is a concern,

enucleation is appropriate and evisceration is contraindicated. Other indications for enucleation

may include progressive phthisis bulbi and severe microphthalmia.

In the vast majority of situations, the indication for exenteration surgery is to eradicate life-threatening malignancy or life-threatening orbital infection. The extent of the procedure should

be explained to the patient, especially which tissues are to be removed (this includes the eyeball,

orbital soft tissues, and part or all of the eyelid structures). The surgeon should avoid lengthydiscussions regarding the mutilating nature of the procedure but rather should help support the

patient to remain focused on the treatment of this potentially life-threatening problem through

the life-saving nature of the exenteration surgery.

A summary of the indications for surgery is given in Box 97-1 .Preoperative Counseling

Faced with the permanent loss of an eye, a patient requires the physicians reassurance, caring

explanations, and psychological support, both before and after the surgery. The patient (andfamily) should understand that evisceration and enucleation surgery involve the complete,

permanent removal of the diseased or deformed eye. The general nature of the anophthalmic

socket should be explained to the patient, who must be informed that an ocular prosthesis will befitted secondarily approximately 6 weeks following the surgery. The indication for surgery,

whether it is pain, poor visual prognosis, the risk of sympathetic ophthalmia, or the presence of

an intraocular neoplasm, should be clearly explained. The patient should be informed of the

choices between enucleation and evisceration surgery and of the availability of a variety oforbital implants, including common alloplastic implants[1] [2] (e.g., polymethyl methacrylate

sphere), newer implants designed to maximize ultimate ocular prosthesis motility [3] [4] [5] [6]

(e.g., hydroxyapatite implants), or autologous tissue orbital implants [7] [8] [9] [10] (e.g.,

dermis-fat grafts).The patient should understand the risks and benefits of wrapping orbital implants with either

autologous tissues or preserved donor tissue and that donor tissues may carry the risks of

communicable diseases, such as syphilis, hepatitis, and human immunodeficiency virus. Itshould be explained to the patient that if a hydroxyapatite implant is used in primary enucleation


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or evisceration surgery, a delayed second-stage procedure (i.e., second-stage drilling of the

hydroxyapatite implant with placement of the motility peg) may be needed in order to maximize

the ocular prosthesis motility. A thorough explanation allows the patient and family to make awell-informed decision regarding surgery. Although the specific decision for surgery is to be

made by the patient and family, it is reasonable for the surgeon to make a best-judgment

recommendation to help with the myriad of choices available (e.g., enucleation versusevisceration and the variety of types of orbital implants).Following enucleation or evisceration, most patients undergo a grief reaction to varying degrees.

The patient, therefore, requires psychological support from the physician. The exenteration

candidate must also be informed of the nature of the surgery and the more radical amount oftissue to be resected. Although the patient must be given a full and truthful explanation regarding

exenteration surgery, the surgeon should avoid overly gruesome details so as not to deter

inadvertently the patient from receiving necessary treatment, such as for a potentially life-

threatening neoplasm.Removal of the Wrong Eye

Removal of the wrong eye presents one of the greatest disasters that can occur to the ophthalmic

surgeon and patient. Every ophthalmologist and surgeon must be aware of this possibility, nomatter how remote. Preoperatively, the surgeon may mark the forehead or trim the lashes on the

appropriate side. These methods, however, are not foolproof. In the operating room, the surgeon

should thoroughly review the chart, including the operative permit and the examination notes. It

is important, then, that the surgeon him- or herself prepares and drapes the patient. Traquair[11]suggested the use of local anesthesia to prevent removal of the wrong eye, although not even this

method is fail-safe. It must never, never happen that a surgeon hurries into the

Indications for Surgery

ENUCLEATION

Blind painful eye

Intraocular tumor Severe trauma with risk of sympathetic ophthalmia

Phthisis bulbi

Microphthalmia Endophthalmitis/panophthalmitis

Cosmetic deformity

EVISCERATION

As for enucleation, except for intraocular tumors or risk of sympathetic ophthalmia

EXENTERATION Cutaneous tumors with orbital invasion

Lacrimal gland malignancies

Extensive conjunctival malignancies

Other orbital malignancies Mucormycosis

Chronic orbital pain

Orbital deformities


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operating room where the patient is already under general anesthesia and begins the operationwithout an appropriate review of the situation.

Once a sterile operative field is set up, the surgeon must again verify that the correct eye is about

to undergo enucleation. Following severe trauma, the correct eye is often externally deformed. Incases where the external appearance of both eyes is normal, the surgeon must compulsivelyreexamine the fundus to verify the pathology.

The finality of the enucleation procedure cannot be overstressed. No degree of thoroughness is

excessive in order to avoid removal of the wrong eye.ANESTHESIA

Enucleation surgery usually is performed using local anesthesia. For psychological reasons, and

occasionally for medical reasons, general anesthesia may be employed. Under any circumstance,

agents should be used that maximize intraoperative hemostasis, suppress the oculocardiacreflex,[12] and minimize postoperative pain. The authors choice is to instill 10% phenylephrine

eye-drops into the conjunctival cul-de-sac to achieve intense vasoconstriction, and to infiltrate

extensive retrobulbar and peribulbar bupivacaine 0.5% with epinephrine (adrenaline) 1:100,000and hyaluronidase. After adequate time, an excellent anesthetic and vasoconstrictive effect is

achieved.

Most evisceration surgeries are also performed under local anesthesia with intravenous sedation.

A mixture of lidocaine (lignocaine) 2% with epinephrine 1:100,000, bupivacaine 0.5% with1:100,000 epinephrine, and hyaluronidase is injected in retrobulbar fashion into the muscle cone.

The use of intravenous anesthetic sedatives prevents either the local anesthetic injection or the

surgical procedure itself from being unpleasant or producing anxiety. Exenteration surgery isusually performed under general anesthesia, which may be combined with bupivacaine and

epinephrine infiltration to aid hemostasis and provide postoperative analgesia.

SPECIFIC TECHNIQUES

EnucleationThe indications for enucleation surgery and important aspects of preoperative counseling have

already been discussed. Here two surgical techniques are described:

Enucleation with placement of a simple sphere implant Enucleation with placement of a sclera-wrapped hydroxyapatite implant for improved motility

Figure 97-1 Enucleation procedure. Following a 360 conjunctival peritomy, a small pair of

tenotomy scissors is used to dissect bluntly Tenons fascia in all four quadrants.

Before describing the specifics of enucleation surgery, a few aspects in regard to Tenons fascia

must be mentioned. Tenons capsule is the fibroelastic tissue that surrounds the eye andextraocular muscles in the anterior orbit (see Chapter 83 ). Anteriorly, Tenons fascia fuses with

the conjunctiva near the corneal limbus. At its posterior extent, Tenons fascia encircles and

fuses with the dura over the optic nerve. The four recti muscles originate from the annulus of

Zinn and extend anteriorly to the eyeball. Posterior to the equator of the globe, the rectus musclespenetrate through Tenons capsule before inserting into the sclera. That part of Tenons fascia

anterior to the rectus muscles is anterior Tenons, and that part of Tenons fascia posterior to the

site of the rectus muscle penetrations is posterior Tenons. It is critically important to understand


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this anatomical concept in order to achieve the proper, desirable orbital implant placement during

enucleation surgery.

ENUCLEATION WITH SIMPLE SPHERE IMPLANT.A self-retaining lid speculum is placed to expose the entire epibulbar surface. A 360

conjunctival peritomy is performed ( Fig. 97-1 ). Tenons fascia is bluntly dissected away from

the sclera in all four quadrants. Each of the four rectus muscles is sequentially gathered on amuscle hook, secured with double-armed 6-0 Vicryl suture, and detached from the globe. Thesuperior oblique tendon is severed and detached from the globe. The inferior oblique muscle

should be hooked and secured with a 6-0 Vicryl suture, detached, and saved for later attachment

to the inferior border of the lateral rectus muscle. This use of the inferior oblique muscle isperhaps more important as an eventual hammock for the orbital implant than to enhance

meaningfully anophthalmic socket motility.

After the extraocular muscles are detached, the surgeon is ready to sever the optic nerve.

Anterior traction on the globe is useful when cutting the optic nerve and can be achieved with acurved hemostat applied to the medial rectus tendon or with a double-armed 4-0 silk suture sewn

through the medial and lateral tendon insertions. In most cases it is the authors preference to

clamp the optic nerve with a curved hemostat inserted behind the globe in the superonasaldirection ( Fig. 97-2 ). With the hemostat in place, a slender curved Metzenbaum scissors is used

to

Figure 97-2 Each of the four rectus muscles is tagged with a double-armed 6-0 Vicryl suture anddetached from the globe. Some 4-0 silk sutures may be placed through the medial and lateral

recti muscle stumps to provide anterior traction on the globe, as a slender curved hemostat is

used to clamp the optic nerve.

Figure 97-3 The globe has been removed and cautery is applied to the optic nerve stump to

maintain meticulous hemostasis.transect the optic nerve, and the entire eyeball is removed. The surgeon should inspect the entire

globe for intactness and/or unusual findings before submitting the specimen for histopathological

examination. Malleable retractors are placed so as to visualize directly the still clamped cut edgeof the optic nerve, and the central retinal vessels are cauterized to obtain meticulous hemostasis

before removing the clamp ( Fig. 97-3 ). If the optic nerve is not clamped, such as for intraocular

tumors, orbital packing with direct pressure for 510 minutes can be applied to achieve adequatehemostasis. In select enucleations, as with tumors in contact with the optic disc, it may be

necessary to obtain a long segment of optic nerve.[13] [14]

For the average-sized adult orbit a 20?mm polymethyl methacrylate orbital implant is usually

adequate. The implant

Figure 97-4 An orbital implant has been placed behind posterior Tenons fascia. This layer is

then closed with multiple, interrupted 6-0 Vicryl sutures. The four rectus muscle stumps remain

free with the 6-0 Vicryl sutures attached.type and size can, of course, vary, and it may also be wrapped in either autologous fascia or

donor sclera. The orbital implant is inserted behind posterior Tenons fascia, through the central

rent left by cutting the optic nerve. Multiple interrupted 6-0 Vicryl sutures securely closeposterior Tenons fascia that overlies the orbital implant.


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Each of the four rectus muscles is sutured to the adjacent fornix by passing the previously placed

double-armed Vicryl sutures full-thickness through Tenons fascia and conjunctiva[15] (see Fig.

97-4 ). This will provide motility to the ocular prosthesis. Care should be taken to avoidadvancing the superior rectus suture too close to the midline to avoid inadvertent tension or

traction on the superior rectus muscle, which could induce an upper lid ptosis. After anterior

Tenons fascia is closed in the midline with 6-0 Vicryl sutures ( Fig. 97-5 ),[16] the conjunctivaledges are loosely reapproximated with a 6-0 plain gut running suture.At the end of the procedure an additional deep orbital injection with bupivacaine 0.5%,

epinephrine, and hyaluronidase is given. A broad-spectrum ophthalmic antibiotic ointment is

applied to the conjunctiva. A medium-sized clear acrylic lid conformer is placed and a firmpressure bandage applied over the socket.

The pressure bandage remains intact for 34 days postoperatively and, upon removal, the patient

uses topical cool compresses with crushed ice. Pain medication is prescribed as appropriate. This

perioperative and postoperative management regimen allows the large majority of enucleationprocedures to be performed as outpatient procedures, with adequate control of postoperative

pain.

ENUCLEATION WITH HYDROXYAPATITE IMPLANT.The purpose of the hydroxyapatite implant is to allow the potential for maximum motility of the

ocular prosthesis. Coralline hydroxyapatite contains 500?m diameter pores that are similar to the

structure of the haversian systems of cancellous bone. The microstructure of this implant allows

fibrovascular ingrowth of the host tissues in the anophthalmic socket.[3] [4] Once thehydroxyapatite implant is well vascularized, it can be secondarily drilled and fitted with a

motility peg implant. This motility peg is then coupled to the ocular prosthesis to enhance

maximally prosthesis motility.A standard enucleation technique is performed, as already described. The socket may be sized

using sterile trial spheres, but

Figure 97-5 Enucleation surgeryfinal closure. The 6-0 Vicryl rectus sutures are sewn onto their

respective fornices by passing the sutures through Tenons fascia and conjunctiva. The anterior

Tenons is closed with 6-0 Vicryl and the conjunctiva with a running 6-0 plain suture.in most cases an 18?mm or a 20?mm hydroxyapatite implant is appropriate. Keep in mind that

wrapping the implant with sclera or fascia adds approximately 11.5?mm to the overall diameter

of the implant.In most situations, the hydroxyapatite implant is wrapped in donor sclera. The scleral shell

should be cut to the appropriate size and shape to enclose the implant securely. Multiple

interrupted 6-0 Vicryl sutures are suitable for securely closing the sclera. The hexagonal rosettes

of the hydroxyapatite exoskeleton should be aligned in the anterior-posterior direction and anopen scleral window should be present at the posterior apex of the hydroxyapatite implant,

corresponding to the site of the corneal button removal. Rectangular windows, approximately 2

4?mm, are cut through the sclera located within 810?mm from the anteriormost apex of the

implant. To promote further fibrovascular ingrowth into the implant, a handheld 20-gauge needleis used to create drill holes in the hydroxyapatite at the site of each window and at the site of the

posterior round corneal window.[17]

The wrapped hydroxyapatite implant is placed into the anophthalmic orbit and the four rectusmuscles are secured to the anterior lip of the corresponding rectangular scleral window. Anterior


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Tenons fascia is sutured with multiple interrupted 6-0 Vicryl sutures. The conjunctiva can be

closed with a loosely running 6-0 plain suture, which is tied and cut on each end. Some authors

report a higher exposure rate with hydroxyapatite [18] [19] [20] [21] compared with alloplasticsphere implants, [22] thus emphasizing the need for meticulous closure. As is the case with any

enucleation procedure, a polymethyl methacrylate lid conformer is placed in the conjunctival

cul-de-sac with broad-spectrum antibiotic ointment and a pressure bandage applied.The unique properties of a hydroxyapatite implant allow fibrovascular ingrowth and integrationof the implant with the ocular prosthesis. Without placement of the motility peg, no

demonstrable motility difference exists between a sclera-wrapped hydroxyapatite implant and a

similarly wrapped polymethyl methacrylate implant.[23] Thus hydroxyapatite implantation ismost appropriate for patients who express a strong interest in eventual second-stage drilling of

the implant to maximize prosthesis motility. These titanium motility pegs are surgically inserted

after adequate fibrovascular ingrowth into the hydroxyapatite implant has occurred. [24] [25]

[26]

Evisceration

OVERVIEW.Evisceration is the surgical technique that removes the entire intraocular contents of the eye

while leaving the scleral shell and extraocular muscle attachments intact. Evisceration surgery is

a simpler procedure than enucleation surgery and offers better preservation of the orbital

anatomy[27] and natural motility of the anophthalmic socket tissues.In cases of documented or suspected intraocular malignant tumors, evisceration is

contraindicated. Similarly, evisceration may be contraindicated if precise histopathology of the

specimen is needed. Evisceration surgery may be more difficult in eyes with severe phthisis orscleral contracture or that are severely deformed. Finally, the issue of potential sympathetic

ophthalmia should be considered. [28] [29] [30] [31] Evisceration surgery in a previously injured

eye carries a definite small risk of sympathetic ophthalmia in the apposing eye because some

uveal tissue is always left behind in scleral canals.[28]

Figure 97-6 Evisceration procedure. A 360 conjunctival peritomy is made, followed by

complete excision of the corneal button.SURGICAL TECHNIQUE.

Although some surgeons perform evisceration with preservation of the cornea, this author prefers

removal of the cornea. The procedure begins with a 360 conjunctival peritomy ( Fig. 97-6 ).Tenons fascia is bluntly separated from the underlying sclera in all four quadrants. A full-

thickness incision around the corneal limbus is made with a sharp scalpel blade and the entire

corneal button removed. The sclera is grasped with a forceps, and a cyclodialysis spatula is used

to separate the iris root and ciliary body from the sclera. The remainder of the uveal tissue isdissected away from the scleral wall back to the attachment around the optic nerve with an

evisceration spoon ( Fig. 97-7 ). The intraocular contents are lifted from the scleral shell and

submitted for histopathologic examination. All remaining uveal tissue is carefully removed from

the scleral shell with a small curette or the sharp end of a caudal periosteal elevator. Cotton-tipapplicators saturated with 70% ethanol may be used to cleanse the interior of the scleral shell and

denature any remaining uveal pigmented tissue. Cautery is applied if needed to control the

oozing of blood.


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A polymethyl methacrylate or hydroxyapatite spherical implant is placed in the evisceration

scleral shell ( Fig. 97-8 ). When the cornea is removed, it is unusual to place an implant larger

than 1416?mm. The scleral edges are closed with multiple interrupted 6-0 Vicryl sutures, withthe medial and lateral scleral edges cut to reduce any dog ears ( Fig. 97-9 ). The conjunctiva is

gently closed with a running 6-0 plain gut suture. If a larger implant is desired, it is necessary to

perform radial relaxing sclerotomy incisions posteriorly[32] between the rectus muscles ( Fig.97-10 ). If a hydroxyapatite implant is used, such sclerotomy openings are necessary to enhancevascular ingrowth.[33]

Dressing and postoperative care are as for enucleation.

ExenterationOVERVIEW.

Exenteration surgery involves complete removal of the eyeball, the retrobulbar orbital soft

tissues, and most or all of the eyelids. The most common indication for exenteration surgery is

for the treatment of epithelial malignancy with orbital invasion.[34] [35]When exenteration is performed for orbital malignancies, periorbita is usually excised to remove

completely all potentially involved tissues. The bare orbital bone can slowly heal by secondary

Figure 97-7 An evisceration spoon is used to detach the ciliary body and bluntly elevate the

choroid from the scleral wall.

intent, but in most situations the exenterated orbit is covered with a split-thickness skin graft atthe time of the procedure. As there is potential for recurrent tumor, reconstruction with thick,

bulky tissue grafts, which could obscure recurrence, is avoided. In very select situations,

however, a variety of ancillary reconstructive techniques may be of use, such as those involvingipsilateral temporalis muscle flaps, [36] free dermis-fat grafts,[37] latissimus dorsi myocutaneous

free flaps,[38] osseointegrated implant techniques,[39] and other procedures. [40] [41] [42] [43]

SURGICAL TECHNIQUE.

The area of the proposed exenteration incision is marked with adequate wide margins wherenecessary for tumors, yet with preservation of as much normal periocular soft tissue as possible (

Fig. 97-11 ). If necessary, adjacent areas of the medial canthus, temple, or forehead are included

in the excision site. When surgery is necessary for a conjunctival or deep orbital tumor, asubciliary incision around the eyelid margins and wrapping around the inner canthus preserve the

eyelid skin and orbicularis muscle, which can be used for reconstruction.[43]

The skin is incised along the mark and any orbicularis muscle to be spared dissected in asuborbicular plane. The dissection is carried down through periorbita to expose the orbital rim. A

periosteal elevator is used to elevate periosteum over the orbital rim and periorbita from the

orbital walls ( Fig. 97-12 ). Firm attachments to bone are encountered at the lateral orbital

tubercle, the superior oblique trochlea, the medial canthal tendon, the distal lacrimal sac as itenters the bony nasolacrimal canal, the inferior oblique origin near the posterior lacrimal crest,

and the superior and inferior orbital fissure attachments ( Fig. 97-13 ; see Chapter 83 ). Except

for these sites of resistance, the periorbita can be elevated quite easily. Medially, the surgeon

should use particular care when elevating periorbita so as to avoid inadvertent penetration of thelamina papyracea into the ethmoid sinus air cells, which could result in a chronic sino-orbital

fistula.

Superiorly, the superior orbital bone may be quite attenuated in elderly patients and atrophicbony defects may be present. Monopolar cautery to the orbital roof should be avoided, as this


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may cause inadvertent cerebrospinal fluid leakage.[44] It is generally safe to use bipolar cautery

along the orbital roof and deep orbital tissues without the risk of cerebrospinal fluid leakage.

The periorbital lining is mobilized along all orbital walls toward the orbital apex. The dissectionand mobilization of soft tissues must extend posteriorly beyond the extent of tumor invasion. A

thin curved hemostat can be used to clamp the apical

Figure 97-8 A sphere introducer is used to place the orbital implant into the evisceration scleralshelf.

tissues while a slender pair of Metzenbaum scissors are used to excise the exenteration specimen

anterior to the clamp ( Fig. 97-14 ). An enucleation snare may also be used to incise the apicalstump to complete the severing of the exenteration specimen.[45] When necessary, frozen

section pathology analysis of the apical stump tissues should be used to verify that the margins of

resection are free and clear of neoplasm. The orbital bone should be carefully inspected for

subtle bone pitting or other signs of bone erosion or destruction.In patients who have very bulky or massive orbital neoplasms, exenteration may be difficult,

with little space in which to separate periorbita from orbital bone. It may be helpful here first to

enucleate the eyeball to make enough room for access to the deeper apical soft tissues undergood visualization.

In most patients the orbit will be lined with a split-thickness skin graft harvested from the

anterior surface of the thigh. It is

Figure 97-9 The scleral opening is closed with multiple, interrupted 6-0 Vicryl sutures.

Conjunctiva is subsequently closed over the scleral wound using running 6-0 plain gut sutures.

Figure 97-10 A unipolar cautery is used to incise relaxing sclerotomy slits to expand the scleral

shell. This sclerotomy technique to enlarge the scleral shell volume is optional with

polymethyl methacrylate sphere implants. Sclerotomy slits are mandatory when using

hydroxyapatite spheres in order to facilitate vascular ingrowth.

usually preferable to expand the skin graft in a mesher. Multiple interrupted 6-0 Vicryl sutures

secure all residual host skin edges to the meshed skin graft. The graft is tamponaded within theorbit with a Telfa dressing and Xeroform gauze packing under pressure.

If the upper lid and lower eyelid skin and muscle are preserved, it may be possible in elderly

patients with a lot of loose

Figure 97-11 Cross-sectional view of surgical planes of dissection for exenteration surgical

techniques: total exenteration, subtotal exenteration with sparing of myocutaneous eyelid tissue,and enucleation with partial socket ablation.

Figure 97-12 Exenteration procedure. A 360 skin incision is made down to the periosteum of

the orbital rim. A periosteal elevator is used to begin reflecting the superior periorbita downward.eyelid skin simply to suture the skin edges together and then place a pressure dressing to

tamponade the myocutaneous edges against the bare bone.

POSTOPERATIVE MANAGEMENT.


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The orbital pack and pressure dressing should remain in place for approximately 57 days.

Following removal of the dressing, the patient can use gentle

759

hydrogen peroxide rinses to cleanse the socket. Generally, these orbits heal best when left open

to the air, so the patients should wear a patch only when going out in public. The surgeon shouldremain vigilant to the possibility of infection of the skin graft, especially by Pseudomonas,Staphylococcus, or Streptococcus. Systemic antibiotics may be necessary if these infections

arise. In some patients, the exenterated orbit retains chronic, moist, ulcerated areas intermixed

with areas of healthy keratinizing epidermis. The use of a gentle handheld hair dryer can helpcure these slower healing areas.

A combined eyelid-ocular prosthesis can be made by an anaplastologist. Many exenteration

patients prefer simply to wear a black patch.

Figure 97-13 Bony orbit demonstrating the normal sites of increased resistance to dissection

during orbital exenteration.

Figure 97-14 Periorbita has been elevated for 360. Forward traction is applied to the orbital

contents as a hemostat is used to clamp the apical orbital tissues.COMPLICATIONS

Evisceration

Postoperative infection is always of concern when evisceration surgery is performed in thesetting of endophthalmitis or panophthalmitis. The use of broad-spectrum systemic antibiotics

usually minimizes this risk, and the surgeon can generally use a primary orbital implant.

Postoperative extrusion of the orbital implant is a complication of evisceration surgery that may

be related to postoperative scleral shell shrinkage, to poor wound healing of the scleral edges, orto improper selection of the orbital implant size. Postoperative pain is more common when the

cornea is retained.

EnucleationOrbital implant extrusion is also a complication of enucleation surgery. Meticulous attention to

careful Tenons fascia wound closure and the proper selection of implant size are important

principles in avoiding this outcome. Risk of implant extrusion is increased with prior irradiationtreatment of the eye and orbit, severe traumatic injuries to the eye and orbit, and severe eye and

orbital infections. Long-term complications of the anophthalmic socket are numerous, including

generalized volume deficiency of the anophthalmic socket, lower eyelid laxity with poor

prosthesis support, orbital implant migration, upper eyelid ptosis, and chronic conjunctivitis andmucoid discharge.

Exenteration

Exenteration surgery carries the risk of severe blood loss. It is important preoperatively to

discontinue aspirin and all other medicines that could adversely affect blood clotting. Othercomplications unique to exenteration surgery include cerebrospinal fluid leakage via orbital roof

transgression of the dura and chronic sino-orbital fistulas through the region of the lamina

papyracea and ethmoid sinus air cells. During the first few weeks of healing, free skin grafts are


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susceptible to infection. Patients may require treatment with broad-spectrum systemic antibiotics

for coverage of Staphylococcus, Streptococcus, Pseudomonas, and

760

other bacteria. The administration of systemic antibiotics is combined with maintenance of

vigorous topical hygiene of the split-thickness skin graft using hydrogen peroxide rinses. Longterm, the surgeon should always remain vigilant for the possible recurrence of tumor.

Enucleation: surgical removal of the entire globe.

Evisceration: surgical removal of the entire contents of the globe leaving a scleral shell.

Exenteration: removal of the entire orbit including the globe, eyelid, and orbital contents

usually performed for malignant tumors.

Evisceration is the removal of the contents of the globe while leaving the sclera and extraocular muscles

intact. Enucleation is the removal of the eye from the orbit while preserving all other orbital structures.

Exenteration is the most radical of the three procedures and involves removal of the eye, adnexa, and

part of the bony orbit.

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