eviscerationandelaboratesontheles- diotherapy ...€¦ · None Painful blind eye Orbital recurrence of retinoblastomab Chemotherapy, orbital exenteration, EBRT 36 No local recurrence

ter selective ophthalmic artery chemotherapy in-fusion for retinoblastoma. Doc Ophthalmol. 2009;119(1):13-22.

3. Reese AB, Hyman G, Tapley N, Forrest AW.The treatment of retinoblastoma by x-ray and tri-ethylene melamine. AMA Arch Ophthalmol. 1958;60(5):897-906.

4. Mohri M. The technique of selective ophthal-mic arterial infusion for conservative treatmentof recurrent retinoblastoma [in Japanese]. KeioJ Med. 1993;70:679-687.

5. Yamane T, Kaneko A, Mohri M. The techniqueof ophthalmic artery infusion therapy for pa-tients with intraocular retinoblastoma. Int J ClinOncol. 2004;9(2):69-73.

Evisceration in UnsuspectedIntraocular Tumors

Enucleation and evisceration are the2 possible surgical management op-tions for a disfigured or a painfulblind eye.1-3 Evisceration is replac-ing enucleation as the favored surgi-cal option because of its potential ad-vantages such as superior cosmesis,better prosthesis motility, and fewerimplant-related complications.1-3

Evisceration, however, is absolutelycontraindicated in suspected intra-ocular malignancy. Before the avail-ability of modern imaging tech-niques, 10% of blind, painful eyeswith opaque media were found tocontain unsuspected malignant tu-mors on histopathologic examina-tion.3,4 Although rare, pathologistscontinue to encounter clinically un-suspected intraocular tumors dur-ing histopathologic examination ofeviscerated eyes. Several anecdotalstudies of unsuspected tumors foundafter evisceration exist.4-13 Althoughthese articles include cases of carci-noma of the nonpigmented ciliaryepithelium, adenocarcinoma of theretinal pigment epithelium, choroi-dal lymphoma, choroidal ganglio-neuroma, spindle cell neoplasm, ana-plastic tumor, and retinoblastoma,uveal melanoma predominates. Eagleet al13 recently described a series of7 additional cases of unsuspecteduveal melanoma diagnosed in evis-cerated specimens and have empha-sized the role of detailed a medicalhistory, clinical evaluation, and ap-propriate imaging before perform-ing evisceration in painful blind eyeswith opaque media. Our series com-prises 6 cases of unsuspected intra-ocular tumors diagnosed following

evisceration and elaborates on the les-sons learned from this experience.

Methods. This is a retrospective, non-randomized, clinicopathological caseseries of patients with previously un-suspected intraocular tumors diag-nosed by histopathology of eviscer-ated blind eyes. The cases werecollected by searching the indexedocular pathology registry from Janu-ary 1998 to December 2007 at a ter-tiary care center in southern India.The medical records were reviewedfor patient age, sex, symptoms, ini-tial clinical findings, initial clinicaldiagnosis, imaging, prior treatment,indication for evisceration, intra-operative findings, histopathology,postevisceration management, and fi-nal outcome for local tumor recur-rence and systemic metastasis.

Results. We identified 6 patients withunsuspected intraocular tumors whohad undergone evisceration (Table).The median patient age was 18 years(range, 8-70 years; mean [SD],27.17[22.46] years), and 5 were male.Preoperative ultrasound B-scan wasavailable for 3 patients; a review of thedocumented images showed no ob-vious intraocular mass in 2 patients.A preoperative computed tomo-graphic scan in one patient did not re-veal an intraocular mass. Indica-tions for evisceration included apainful blind eye in 4 patients, cos-metic concern in 1 patient, and a per-forated hypotonus eye with uveal pro-lapse in 1 patient. No surgeons hadrecorded unusual features of intra-ocular contents observed during evis-ceration. Eviscerated tissue was notsubmitted for histopathology in 2 pa-tients. Histopathology of the eviscer-ated tissue or biopsy from the re-current orbital tumor revealedretinoblastoma in 2 patients, and 1each of uveal melanoma, adenocar-cinoma of the ciliary body, choroi-dal ganglioneuroma, and conjuncti-val squamous cell carcinoma withintraocular invasion. Orbital exen-teration was eventually required totreat 4 of these patients. Two pa-tients with retinoblastoma weretreated with high-dose chemo-therapy, orbital exenteration, and ex-ternal beam radiotherapy. The pa-tient with uveal melanoma had orbitalexenteration and external beam ra-

diotherapy. Adenocarcinoma of theciliary body was managed withenucleation and external beam radio-therapy. The patient with benign cho-roidal ganglioneuroma was ob-served. The patient with intraocularinvasion of conjunctival squamouscell carcinoma had orbital exentera-tion. All of the patients were free oflocal recurrence or systemic metas-tasis at a median follow-up of 28months (range, 4-41 months; mean[SD], 24.50[14.90] months).

Report of Cases. Case 1. A systemi-cally healthy and active 3-year-oldboy presented to the glaucoma clinicin June 2001 with spontaneous pro-gressive enlargement of the left eyeof 2 years’ duration. There was no his-tory of prior trauma or surgery. Find-ings of the examination of right eyewere essentially normal. The childhad no light perception in the left eye.There was ciliary staphyloma, dif-fuse corneal edema, dilated fixedpupil, aphakia, and an intraocularpressure of 34 mm Hg by Perkin ap-planation tonometry under anesthe-sia. The fundus view was unclear. Ul-trasound B-scan showed an increasein the axial length, a clear vitreouscavity, and optic disc cupping(Figure 1A). There was no evi-dence of subluxation of the crystal-line lens or an intraocular mass. Animmersion ultrasound B-scan, how-ever, was not done. The glaucomaspecialist performed semiconductordiode laser transscleral cyclophoto-coagulation to control the intraocu-lar pressure. The child had periodicfollow-up at the glaucoma clinicthereafter. The child reported se-vere pain in the left eye in January2005 and had evisceration by a pe-diatric ophthalmologist. A repeatedultrasound B-scan of the eye was notperformed before evisceration. His-topathology of the eviscerated tis-sue showed a malignant round celltumor with areas of necrosis and cal-cification, based on which a diagno-sis of retinoblastoma was made(Figure 1, B and C), and the child wasreferred to the ocular oncology ser-vice. A computed tomographic scandid not reveal optic nerve invasion ororbital extension (Figure 1D). Therewas no evidence of systemic metas-tasis. Results of bone marrow bi-opsy and cerebrospinal fluid cytol-

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ogy were normal. The child was givenhigh-dose chemotherapy with a com-bination of carboplatin, vincristine,and etoposide for 3 cycles, followedby eyelid-sparing orbital exentera-tion (Figure 1E), 4500-cGy (to con-vert gray to rad, multiply by 100)fractionated external beam radio-therapy to the orbit, and continuedchemotherapy for 12 cycles. Thechild is alive and well, with no localrecurrence or systemic metastasis 41months after completion of treat-ment (Figure 1F).

Case 2. An 8-year-old boy had un-dergone evisceration elsewhere witha history of painful blind right eyefollowing trauma and a clinical di-agnosis of secondary glaucoma.Imaging had not been performed be-fore evisceration. Eviscerated tis-sue had not been submitted for his-topathology by the comprehensiveophthalmologist who had per-

formed the surgery. The child de-veloped a painful, rapidly growingorbital mass 2 months followingevisceration (Figure 2A) and wasreferred to our ocular oncology ser-vice. A computed tomographic scanshowed a large orbital mass withspecks of intralesional calcifica-tion, suggestive of orbital recur-rence of retinoblastoma (Figure 2B).Incisional biopsy of the orbital massconfirmed the diagnosis of retino-blastoma (Figure 2C). There was noevidence of systemic metastasis. Re-sults of bone marrow biopsy and ce-rebrospinal fluid cytology were nor-mal. The child received high-dosechemotherapy with a combinationof carboplatin, etoposide, and vin-cristine for 3 cycles (Figure 2, D andE), followed by an eyelid-sparing or-bital exenteration, 4500-cGy frac-tionated external beam radio-therapy to the orbit, and continued

chemotherapy for 12 cycles. Thechild had no local recurrence or sys-temic metastasis at 36 months fol-lowing completion of treatment.

Case 3. A 41-year-old man pre-sented with a painful, blind right eyeof 3 months’ duration. The right eyehad no light perception. The ante-rior segment showed features of sec-ondary angle-closure glaucoma andcomplicated cataract. Opaque mediaprecluded fundus view. Ultrasound B-scan showed a large ciliochoroidalmass with low internal reflectivity(Figure3A). The mass filled the vit-reous cavity. Enucleation was ad-vised with the clinical suspicion of auvealmelanoma.Thepatientwas sub-sequently lost to follow-up. He de-veloped severe pain 1 month later andconsulted a comprehensive ophthal-mologist elsewhere. The ophthal-mologist, who was unaware of priorimaging and clinical diagnosis, pro-

Table. Clinical Profile of 6 Patients Who Had Evisceration With Unsuspected Intraocular Tumor

Sex/Age, y Imaging

Initial ClinicalDiagnosis

Initial ClinicalManagement

Prior toEvisceration

Indication forEvisceration

FinalHistopathological

DiagnosisFinal

ManagementFollow-up, mo Final Status

M/8 USG: anechoic,no mass

Staphyloma,secondaryglaucoma

TSCPC, medicalmanagementof glaucoma

Painful blind eye Retinoblastoma Chemotherapy,orbitalexenteration,EBRT

41 No local recurrenceor systemicmetastasis

M/8 Not performed Secondaryglaucoma aftertrauma

None Painful blind eye Orbital recurrence ofretinoblastomab

Chemotherapy,orbitalexenteration,EBRT


M/41 USG:a

intraocularmass withlow internalreflectivitysuggestive ofuvealmelanoma

Uveal melanomaa None Painful blind eye Orbital recurrence ofuveal melanomab

Orbitalexenteration


F/70 USG: closedfunnel retinaldetachment,no mass

Retinaldetachmentwith secondaryglaucoma

Trabeculectomy,medicalmanagementof glaucoma

Painful blind eye Primaryadenocarcinomaof the ciliary body

Enucleation,EBRT


M/11 CT: enlargedeyeball, nomass

Neurofibromatosistype 1,developmentalglaucoma

Trabeculectomy,medicalmanagementof glaucoma

Cosmeticconcern

Choroidalganglioneuroma

Observation 33 No local recurrenceor systemicmetastasis

M/25 Not performed Necrotizingscleritis

Excision ofepibulbarmass,medicalmanagementof necrotizingscleritis

Hypotonus eyewith scleralnecrosis andprolapse ofuveal tissue

Squamous cellcarcinoma withintraocularinfiltration

Orbitalexenteration


Abbreviations: CT, computed tomographic scan; EBRT, external beam radiotherapy; TSCPC, transscleral cyclophotocoagulation; USG, ultrasound B-scan.aThe USG and information about the diagnosis of uveal melanoma were not available to the ophthalmologist who performed evisceration.bEviscerated tissue was not submitted for histopathology; diagnosis was confirmed by biopsy of the recurrent orbital tumor.




ceeded with evisceration of the pain-ful blind eye but did not submit evis-cerated tissue for histopathology. Sixmonths later, the patient developedan orbital mass (Figure 3B) and wasreferred to our ocular oncology ser-vice. A computed tomographic scanshowed a large, isodense orbital masssuggestive of orbital recurrence of

uveal melanoma. Systemic evalua-tion did not reveal any metastasis. Thepatient had an eyelid-sparing orbitalexenteration. Histopathology con-firmed the diagnosis of melanoma(Figure 3C). The patient received6500-cGy fractionated orbital exter-nal beam radiotherapy. He was aliveand well at last visit, 10 months after

treatment, with no local recurrenceor systemic metastasis.

Case 4. A systemically healthy 70-year-old with a history of severe painand loss of vision in the right eye of15 years’ duration was seen by us. Shehad earlier undergone cataract sur-gery, followed by trabeculectomyelsewhere about 10 years ago. She had

A B C

D E F

Figure 1. An 8-year-old child with retinoblastoma who had evisceration of a painful blind eye with the clinical diagnosis of staphyloma with secondary glaucoma aftertransscleral cyclophotocoagulation (case 1). A, The posterior segment is anechoic on ultrasound B-scan performed at the initial visit when the child was aged 3 years.B, Histopathology of the eviscerated tissue shows cellular tumor on the surface of the ciliary body (hematoxylin-eosin, original magnification �40). C, Highermagnification shows differentiated and undifferentiated round cells and Flexner-Wintersteiner rosettes (arrow) suggestive of retinoblastoma (hematoxylin-eosinstaining, original magnification �400). D, A postevisceration axial computed tomographic scan shows an intrascleral implant and no evident orbital retinoblastoma.E, An intraoperative photograph shows eyelid-sparing orbital exenteration. F, Appearance of the exenterated socket is shown at the final follow-up.

A B C

D E

Figure 2. An 8-year-old child with orbital recurrence of retinoblastoma following evisceration of a painful blind eye with glaucoma secondary to trauma (case 2).A, A large fleshy right orbital mass is seen. B, An axial computed tomographic scan demonstrates a large soft-tissue mass in the right orbit with specks ofintralesional calcification. C, Histopathology of incisional biopsy of the orbital mass shows a round-cell tumor infiltrating the orbital tissues (hematoxylin-eosinoriginal magnification �40); the inset shows small, round cells with high nuclear cytoplasmic ratio and coarse chromatin clumping, suggestive of retinoblastoma(hematoxylin-eosin, original magnification �400). D, Following 6 cycles of neoadjuvant chemotherapy, the orbital tumor shows significant resolution.E, A computed tomographic scan axial cut shows significant resolution of the orbital tumor following 6 cycles of neoadjuvant chemotherapy.




absent light perception, scarred bleb,and opaque cornea with spheroidaldegeneration, precluding furtherevaluation of the anterior segmentand fundus. Ultrasound B-scanshowed a closed funnel retinal de-tachment and no evidence of an in-traocular mass. Immersion B-scan,however, was not performed. The pa-tient was advised to have eviscera-tion for symptomatic relief by an ocu-loplasty surgeon. Histopathology ofthe eviscerated tissue showed neo-plastic cells in glandular pattern withclear cytoplasm, vesicular nucleus,and prominent nucleoli, suggestiveof adenocarcinoma of the ciliary body(Figure 4). Results of systemicevaluation were unremarkable. Thepatient was further treated withenucleation and 4500-cGy fraction-

ated orbital external beam radio-therapy. She was alive and well 23months following completion oftreatment.

Case 5. An 11-year-old boy had ahistory of an enlarged left eyeball bybirth. He had earlier had a trabecu-lectomy elsewhere with a diagnosis ofdevelopmental glaucoma. He hadlight perception, intraocular pres-sure of 34 mm Hg by applanation to-nometry, plexiform neurofibroma ofthe left upper eyelid (Figure 5A),buphthalmos with scarred bleb, en-larged and edematous cornea, Lischnodules on the iris, ectropion uveae,and total cataract. Systemic evalua-tion revealed cafe au lait spots. Com-puted tomography of the orbitshowed an enlarged left eyeball, andahypoplastic greater wing of the sphe-

noid (Figure 5B). There was no evi-dence of an intraocular mass. Thechild was diagnosed with neurofibro-matosis type 1 with developmentalglaucoma, status post trabeculec-tomy. The child had evisceration ofthe disfigured left eye and debulkingof the eyelid plexiform neurofi-broma by an oculoplastic surgeon forcosmetic concern. Histopathology ofthe eviscerated tissue showed thick-ened choroid with nests of mature po-lygonal ganglion cells admixed withnerve fibers (Figure 5C), focal areasof ganglion cells, and Pacinian bod-ies (Figure 5D), diagnostic of choroi-dal ganglioneuroma. The child wasobserved. He showed no local tu-mor recurrence at the final fol-low-up at 33 months.

Case 6. A 25-year-old immuno-competent man with a history of re-current redness and pain in his righteye of 6 months’ duration was seenby a cornea specialist. A year earlier,he had undergone excision of agrowth on the surface of the eye,twice, at an interval of 1 month, by acomprehensive ophthalmologist. Apathologist had described the ex-cised specimen as having nonspe-cific inflammation. The slides werenot available for a review. His visualacuity was 20/160 OD. He had scleralnecrosis, uveal prolapse, and cells inthe anterior chamber. He was diag-nosed with necrotizing scleritis andtreated with oral prednisolone andmethotrexate, and subsequently withoral azathioprine. However, his vi-sual acuity deteriorated over the next6 months to bare perception of light,scleral necrosis progressed, andthe patient had unbearable pain(Figure 6A). The cornea specialistperformed evisceration along with ex-cision of the entire cornea, contigu-ous necrotic sclera, and overlying un-healthy conjunctiva. UltrasoundB-scan was not performed prior toevisceration. Histopathology showedconjunctival tumor with completeloss of polarity and surface matura-tion and infiltrating the contiguoussclera. The cells were large and po-lygonal, with prominent nuclei withdesmosomal attachments between thecells (Figure 6B). There was dyskera-tosis and squamous pearl formation.Sections from the eviscerated tissueshowed the iris and ciliary body to beinfiltrated by tumor cells (Figure 6C).

A B

C

Figure 3. A 41-year-old man with orbital recurrence of melanoma following evisceration of a painful blindeye with retinal detachment and secondary glaucoma (case 3). A, Ultrasound B-scan with cross vectorshows a large intraocular mass filling the entire vitreous cavity (top panel), with low internal reflectivity(bottom panel), suggestive of uveal melanoma. B, A right orbital mass was noticed 6 months followingevisceration. C, Histopathology of the exenterated orbit confirms orbital recurrence of uveal melanoma(hematoxylin-eosin, original magnification �400).

A B

Figure 4. A 70-year-old woman with adenocarcinoma of the ciliary body discovered followingevisceration of the painful blind right eye after trabeculectomy (case 4). A, Histopathology of theeviscerated tissue shows a cellular tumor of the nonpigmented ciliary epithelium with glandular pattern(hematoxylin-eosin, original magnification �100). B, Histopathology shows epithelial cells in a glandularpattern with a thick basement membrane, clear cytoplasm, large vesicular nucleus, and prominentnucleoli, suggestive of adenocarcinoma of the ciliary body (hematoxylin-eosin, original magnification�400).




A diagnosis of squamous carcinomaof the conjunctiva with intraocular in-vasion was made. The patient wassubsequently referred to our ocularoncology service. Clinical evalua-tion revealed diffuse residual con-junctival tumor. Regional lymphnodes were not involved, and thesystemic evaluation did not reveal me-tastasis. The patient had an eyelid-sparing orbital exenteration. Histo-pathology confirmed the presence of

a residual conjunctival tumor. Fourmonths after completion of treat-ment, the patient was alive and well,with no local recurrence or systemicmetastasis.

Comment. Evisceration has re-placed traditional enucleation as thefavored surgical option for patientswith blind disfigured or painfuleyes.1-3 However, controversy regard-ing the advantages and disadvan-

tages of each procedure continues.14

Potential advantages of eviscerationover enucleation include ease of sur-gery, better implant and prosthesismotility, preservation of orbitalanatomy resulting in fewer compli-cations such as ptosis, socket con-traction, and deep superior sulcus,and scleral barrier precluding im-plant migration and implant extru-sion.15 A survey demonstrated that theoverwhelming majority of ocularists

A B

C D

Figure 5. An 11-year-old boy with neurofibromatosis type 1, with ganglioneuroma of the choroid in the buphthalmic left eye after trabeculectomy, eviscerated forcosmetic concern (case 5). A, A clinical photograph of the face shows plexiform neurofibroma of the left upper eyelid. B, A computed tomographic scan axial cutshows an enlarged left eyeball and a hypoplastic greater wing of sphenoid. Note that there is no evident intraocular mass. C, Histopathology of the evisceratedtissue shows nests of large ganglion cells (arrow) interspersed with spindle-shaped neural cells (hematoxylin-eosin, original magnification �400).D, Histopathology shows Pacinian body diagnostic of ganglioneuroma (arrow) (hematoxylin-eosin, original magnification �400).

A B C

Figure 6. A 41-year-old man with intraocular invasion of conjunctival squamous cell carcinoma of the right eye after excision, eviscerated with the clinicaldiagnosis of necrotizing scleritis with uveal prolapse (case 6). A, A slitlamp photograph showing necrotic sclera, abnormal episcleral feeder vessels, a largeperforation, and prolapse of uveal tissue. B, Histopathology shows well-differentiated squamous cell carcinoma with surface keratinization (hematoxylin-eosin,original magnification �100). C, Histopathology shows the tumor infiltrating the uvea (hematoxylin-eosin, original magnification �400).




believe current evisceration tech-niques result in a superior clinical out-come compared with enucleation.16

A recent comparative analysis, how-ever, indicated that enucleation andevisceration produce functionally andaesthetically similar outcomes.14

Two of the most dreaded com-plications of evisceration are sym-pathetic ophthalmia and eviscera-tion of an eye with an unsuspectedintraocular tumor.1,2,13 Althoughsympathetic ophthalmia is no longera major concern,1,2 evisceration of aneye with an unsuspected intraocu-lar tumor continues to be a poten-tial risk.4-13 The incidence of diag-nostic surprise following eviscerationhas been reported to be 0.62%, 1 caseof unsuspected adenocarcinoma ofthe ciliary body in a series of 161eviscerations over 20 years.17 Eagleet al,13 however, believe that unsus-pected intraocular tumor has beenunderrepresented in the literatureowing to medicolegal concerns.Schefler and Abramson18 furtherquestion if evisceration is appropri-ate at all in blind, painful eyes withno obvious cause and favor enucle-ation specifically in such situa-tions. Our series is a collection of 6eyes with unsuspected intraoculartumor that were eviscerated. Wehave attempted to highlight the les-sons learned from this experience.

Cases 1 and 2 were children withretinoblastoma. Case 1 initially wasseen with glaucoma and staphy-loma. An ultrasound B-scan was per-formed, but retinoblastoma was notdetected, possibly because the tu-mor was anterior and could have beenseen only on an immersion B-scan ora computed tomographic scan. Moreimportantly, when the child re-turned after 5 years with severe pain,the pediatric ophthalmologist whoperformed evisceration did not re-peat the imaging study prior to sur-gery. Case 2 shows the lack of clini-cal acumen enough to performimaging before evisceration in a childwho had a painful blind eye, or evento submit the eviscerated tissue forhistopathology.

Case 3 highlights the importantaspect of paucity of communica-tion in medical practice. This pa-tient had evisceration despite hav-ing a prior diagnosis of uvealmelanoma. Eviscerated tissue was

not submitted for histopathology inthis patient. He seemingly lacked aclear understanding of his clinicalcondition. A detailed medical re-port incorporating the ultrasound B-scan image could have cautioned thesurgeon against performing an evis-ceration. Online standardized elec-tronic medical records in the fu-ture may help make pertinent patientdetails available to the serial care-givers irrespective of logistic andgeographic barriers.

The adenocarcinoma of the cili-ary body in case 4 was not detectedon ultrasound B-scan. An immer-sion B-scan or an ultrasound biomi-croscopy would have been appropri-ate for this anteriorly located tumor.In case 5, a choroidal ganglioneu-roma was not detected on com-puted tomographic scan. It is pos-sible that an ultrasound B-scan wouldhave been able to demonstrate asubtle choroidal mass. Case 6 had aclear history of a lesion on the ocu-lar surface that had been excised. Thehistopathology report, however, wasmisleading, prompting the cornealspecialist to consider a diagnosis ofnecrotizing scleritis. An ultrasoundB-scan may have revealed the intra-ocular tumor in this case.

There are 6 possible steps to pre-vent evisceration surprises and theirconsequences: (1) a thorough clini-cal history and diligent examina-tion, specifically in a blind eye withno evident cause, unusual and ill-explained clinical findings, and mul-tiple prior surgical procedures (thatmodify the clinical picture); (2) ap-propriate imaging and its interpreta-tion; (3) enucleation in lieu of evis-ceration when an intraocular tumorcannot be reliably ruled out by rea-sonable clinical means; (4) careful in-traoperative evaluation for unusualappearance or feel of intraocular con-tents and modification of the sur-gery as appropriate; (5) routine his-topathology of all eviscerated tissues;and (6) appropriate treatment if a ma-lignant intraocular tumor is found.

Older children with retinoblas-toma may manifest with atypical fea-tures simulating developmental glau-coma, staphyloma, uveitis, hypopyon,hyphema, vitreous hemorrhage, en-dophthalmitis, and orbital cellu-lites.9,18-20 A child with unusual clini-cal features such as these should be

thoroughly evaluated. Uveal mela-noma can manifest with pain and in-flammation induced by necrosis andmay simulate endogenous endoph-thalmitis or orbital cellulitis.13,18,21-24

Imaging studies must be interpretedcarefully in patients who have in-flammatory signs that might becaused by necrotic tumors.13 Adeno-carcinoma of the ciliary body is a raretumor and is known to occur in aphthisical eye.25 This, being an ante-riorly located tumor, can be missedunless an immersion B-scan or an ul-trasound biomicroscopy are per-formed. Choroidal ganglioneuromain a child with neurofibromatosis type1 teaches us to be diligent when a pa-tient with a blind eye has an associ-ated systemic cancer predispositionsyndrome.12 Reports of ocular sur-face squamous neoplasia masquer-ading as necrotizing scleritis exist inthe literature.26,27 A careful evalua-tion of the ocular surface for a con-junctival tumor could have helped inthe diagnosis.

The role of appropriate preopera-tive imaging and its adequate analy-sis and interpretation prior to evis-ceration of a blind eye with opaquemedia cannot be overemphasized.Four patients had preoperativeimaging in our series. Of 3 patientswith a preoperative ultrasound B-scan, the tumor was not detectable in2; one was a retinoblastoma in anolder child, and another was an ad-enocarcinoma of the ciliary body.Both these tumors may have beenmissed on routine ultrasoundB-scan because of their anteriorlocation. A computed tomographicscan did not demonstrate thetumor in a patient with ganglio-neuroma. In all, of 6 patients inour series, the surgeon did not per-form or have access to preoperativeimaging in 4, and images in 2 othercases were suboptimal.

WhileultrasoundB-scancontinuesto be an ideal imaging modality to di-agnose intraocular tumors, it isessen-tialthatanultrasonologistexperiencedin intraocular tumor imaging reportsit, or the ophthalmologist either re-viewsthedynamic images inreal timeor has access to the same for remoteinterpretation. Ultrasound examina-tion is incompletewithoutan immer-siontechniqueand/orultrasoundbio-microscopy in a case with suspected




anteriorlylocatedtumor.Whereultra-sound B-scan fails to detect an intra-ocular tumor, or if there is diagnosticdilemma, further evaluation by com-puted tomography or magnetic reso-nance imaging depend on the indexof suspicion. However, preoperativeimaging does not totally exclude thepossibility of inadvertently eviscerat-ing an eye with occult tumor.13 If anintraocular tumorcannotbeexcludedby reasonable clinical means, it maybeprudent toconsiderenucleation inlieu of evisceration.

If an intraocular tumor is indeedmissed on imaging and eviscerationis performed, astute intraoperative ob-servation, intraoperative histopathol-ogy, and appropriate modification tothe surgery (conversion to enucle-ation or orbital exenteration) couldhelp salvage the situation. It is sur-prising that no surgeons reported ordocumented unusual features no-ticed during evisceration of the 6cases. It would be reasonable to as-sume that a tumor would appear andfeel different than a routine eviscera-tion. Use of illumination and magni-fication could help further enhancethe visual clues.

Routine histopathologic examina-tion of eviscerated tissues as a stan-dard of care could help identify tu-mors that are missed on clinicalevaluation, imaging, and intraopera-tive observation. Two cases in our se-ries did not have histopathology of theeviscerated tissues, and the tumor wasdetected only when it recurred in theorbit. Data indicate that only 43% ofsurgically removed eyes and ocularcontents are sent for histopathologicinvestigation.17 Decisions to send arepossibly made on a case-by-case ba-sis and common sense rather than ablanket policy.17 The national spe-cialist ophthalmic pathology serviceof the United Kingdom recognizesthat there is insufficient reporting ca-pacity and advises ophthalmologiststo retain “current pathology speci-men referral practices.”17 The situa-tion may be similar in other coun-tries as well. Logistic issues such asthese may need to be adequatelyaddressed.

Adequate management of re-sidual or recurrent tumor followingevisceration can help minimize therisk of local recurrence and systemicmetastasis. Two patients with retino-

blastoma in our series had chemo-therapy, orbital exenteration, and ex-ternal beam radiotherapy. The patientwith melanoma had orbitalexenteration and external beam ra-diotherapy. The patient with adeno-carcinoma of the ciliary body hadenucleation and external beam radio-therapy, while the patient with be-nign choroidal ganglioneuroma wasobserved, and the patient with squa-mous cell carcinoma had orbital ex-enteration. It is gratifying that no pa-tients had local recurrence or systemicmetastasis at the last follow-up.

We recommend that adequatecaution must be exercised to fol-low standards of care and due dili-gence to rule out an intraoculartumor before performing an evis-ceration in a blind eye. Based on ourexperience, we believe that an as-tute clinical evaluation, appropri-ate imaging and its interpretation,conversion to enucleation if an in-traocular tumor cannot be clini-cally excluded, intraoperative in-spection of intraocular contentsduring evisceration and modifica-tion of the surgery as appropriate,and histopathology of the eviscer-ated tissues can all help minimize therisk and consequences of eviscerat-ing an eye with an occult intraocu-lar tumor. If an eye with an occultintraocular tumor is indeed eviscer-ated, appropriate posteviscerationmanagement can prevent local tu-mor recurrence and systemic me-tastasis.

Correspondence: Dr Honavar, De-partment of Ophthalmic Plastic Sur-gery, Orbit and Ocular Oncology, LVPrasad Eye Institute, Banjara Hills,Hyderabad 500034, India ([email protected]).Financial Disclosure: None re-ported.Funding/Support:Thisstudywassup-ported by Hyderabad Eye ResearchFoundation, Hyderabad, India.

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2. Bilyk JR. Enucleation, evisceration and sym-pathetic ophthalmia. Curr Opin Ophthalmol.2000;11(5):372-386.

3. Migliori ME. Enucleation vs evisceration. CurrOpin Ophthalmol. 2002;13(5):298-302.

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Suryasnata Rath, MD, FRCSSantosh G. Honavar, MDMilind N. Naik, MDRoshmi Gupta, MD, FRCSVijay A. Reddy, MDGeeta K. Vemuganti, MD

Author Affiliations: Department ofOphthalmicPlasticSurgery,Orbit andOcular Oncology, LV Prasad Eye In-stitute, Bhubaneswar (Dr Rath); De-partments of Ophthalmic Plastic Sur-gery, Orbit and Ocular Oncology (DrsHonavar, Naik, and Reddy), and Oph-thalmic Pathology (Dr Vemuganti),LV Prasad Eye Institute, Hyderabad;and the Department of OphthalmicPlastic Surgery, Orbit and Ocular On-cology, LV Prasad Eye Institute,Vishakhapatnam, (Dr Gupta) India.




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Ophthalmic Images

Invasive Orbital Basal Cell Carcinoma

Matthew T. Feng, MD; Lynn Polonski, MD

Author Affiliations: Department of Ophthalmology and Vision Science, University of Arizona (Dr Feng);and Catalina Eye Care (Dr Polonski), Tucson, Arizona.

Figure 1. Clinical appearance of a 78-year-old white man who presented withprogressive left proptosis and vision loss over 2 years. His forehead lesionsdate back 16 years.

A B

Figure 2. Radiologic and histologic appearance. A, Computed tomographic orbits demonstrated a 2.4�3.3-cm left orbital mass enveloping the optic nerveand eroding the orbital roof. B, A light micrograph (hematoxylin-eosin, original magnification �100) shows peripheral palisading within andartifactitious separation from frontal bone marrow, consistent with invasion by basal cell carcinoma. Exenteration with wide margins included craniotomyfor frontal bone invasion.




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