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    Eye enucleation

    Background

    A range of lesions and diseases affect the eye that may lead to removal (enucleation). The most common cause is primary malignancy but non-functioning painful eyes may also be removed.

    A separate protocol is provided for biopsies and local excisions of the eye.

    Retinoblastomas usually occur in children under three years of age as chalky-white retinal primary tumours that can be multifocal and bilateral with vitreous fluid and/or subretinal space spread.1

    Malignant melanomas are described as uveal (ocular) or conjunctival (extraocular). Uveal melanomas are likely to be pigmented and lobulated primary choroid or ciliary body tumours but can also arise in the iris. Choroid melanoma may appear as fungating tumours when extending into the subretinal space.1 A range of specimens from biopsy, iridectomy, local resection, enucleation and exenteration can be received.2-4

    Carcinomas of the conjunctiva, eyelid and lacrimal gland as well as sarcomas and lymphomas also may be found in these specimens.

    Extenteration is rare surgery for malignancies such as retinoblastoma and melanoma involving tissues adjacent to the orbit.2

    Record the patient identifying information and any clinical information supplied together with the specimen description as designated on the container. See overview page for more detail on identification principles.

    • No
      • Non-routine fixation (not formalin), describe.
    • Yes
      • Special studies required, describe.
      • Ensure samples are taken prior to fixation.

    See general information for more detail on specimen handling procedures.

    Inspect the specimen and dictate a macroscopic description.


    External Inspection

    Orientate and identify the anatomical features of the specimen.

    Record additional orientation or designation provided by operating clinician:

    • Absent
    • Present
      • Method of designation (e.g. suture, incision)
      • Featured denoted

    Photograph the intact specimen if required.

    Orientate the specimen and identify the relevant landmarks.

    Inspect the globe and identify laterality by observing the posterior surface. The ciliary vessels are located medially and the inferior oblique muscles are located laterally to the optic nerve.1,2,8

    • Long ciliary arteries are visible as a posterior blue line and lie on the horizontal plane 2,8
    • A thick muscular insertion is seen entering the posterotemporal side of the inferior oblique muscle; just below the horizontal plane and temporal long ciliary artery2,8
    • The superior oblique muscle inserts into the superotemporal pole of the globe 2,8
    • The cornea is longer horizontally than vertically2,8
    • The optic nerve is closer to the nasal than temporal limbus2,8

    Complete exenteration specimens can be orientated by the longer eyelashes and fold of the upper eyelid as well as the caruncle and punctae of the medial canthus.3,8

    Photograph the intact specimen if required.

    Describe the following features of the specimen:

    Procedure1-8

    Record as stated by the clinician.

    Record the volume of fluid (ml), describe any tissue fragments present and spin down for processing as a cell block.

    • Enucleation
    • Partial/limited exenteration
    • Complete exenteration

    Complete exenteration includes eyelids, optic nerve, extraocular muscles, orbital fat and periosteum as well as the globe.3

    Specimen laterality

    • Left
    • Right
    • Bilateral, designate and describe each separately

    Anatomical components (more than one may apply) and specimen dimensions (mm)1-8

    Describe and measure the components present, as follows:

    • Globe in three dimensions; antero-posterior x horizontal x vertical maximum dimensions2
    • Optic nerve length and diameter2
    • Cornea in two dimensions; horizontal x vertical2
    • Pupil in two dimensions; horizontal x vertical2

    The normal globe is 22-23mm in each dimension.2,8 Increased axial dimension may be due to myopia or glaucoma. Decreased ocular dimensions can be due to senile atrophy and shrinkage due to chronic pressure loss in the eye.2

    • Total specimen in three dimensions; antero-posterior, horizontal and vertical maximum dimensions
    • Components as for enucleation specimens
    • Other tissues, describe and measure in three dimensions

    Specimen integrity

    • Intact
    • Opened
    • Collapsed
    • Distorted

    Evidence of previous biopsy or surgery (if present)

    Note any scars or evidence of previous surgery1:

    • Crescentic opaque scars/sutures (usually found at the superior limbus)
    • Filtration bleb of glaucoma surgery (usually found at the limbus between 11 and 1 o’clock)
    • Repair of detached retinas (presence of silicone bands or sponges)

    Outer surface1

    Record any of the following seen on the outer surface of the specimen.

    Episclera/sclera8

    • Normal
    • Abnormal
      • Sentinel vessels
      • Staphylomas (thinning and stretching)
      • Trauma wounds
      • Fibrosis
      • Thinning
      • Transcleral spread of intraocular tumour

    Conjunctiva8

    (Conjunctiva usually consists of a small rim of tissue around the cornea)

    • Congested vessels
    • Pigmented and non-pigmented tumours,
    • Sutures
    • Fibrosis
    • Cysts
    • Pterygium and pingueculum

    Cornea8

    • Normal (formalin fixation may result in a mild degree of cloudiness)
    • Abnormal
      • Epithelial separation
      • Ulcer or perforation
      • Stromal pigmentation
      • Note the presence of Arcus lipidis in the stroma immediately internal to the limbus
      • Band keratopathy (superficial white oval area below the horizontal line)
      • Bullous keratopathy
      • Note any stromal vascularisation, particularly at the periphery (pannus)
      • Corneal deposits

    Pupil8

    • Shape

    Iris8

    • Normal
    • Abnormal, describe
    • Colour
    • Ectropian uveae
    • Entropian uveae
    • Rubeosis iridis
    • Atrophy
    • Iridectomy sites, describe/draw location

    Optic nerve8

    • Abnormal
    • Normal
      • Atrophic
      • Demyelination
      • Infarction
      • Other

    Gentle palpation of the globe

    • Normal
    • Abnormal
      • Globally firm/globally soft
      • Focally firm/focally soft

    Transillumination8

    (By light source placed adjacent to the cornea)
    • Normal
    • Abnormal
      • Tumour
      • Areas of thinning

    Retroillumination8

    (By light source placed adjacent to optic nerve on the sclera)

    • Normal
    • Abnormal
      • Iris atrophy
      • Pupillary abnormalities
      • describe

    Tumour

    • Absent
    • Present, if present describe the following.

    Tumour on surface of the eye

    • Absent
    • Present
      • Distance to veins (mm)

    Choroidal invasion3

    • Absent
    • Present

    Extraocular spread3

    • Absent
    • Present
      • <5mm
      • >5mm

    Exenteration8

    • Describe all other relevant external features.

    Dissection

    Various techniques are used for ophthalmic specimens:1-8

    Ensure adequate fixation prior to dissection in at least 60ml of formalin for 24-48 hours.1-8 Intraocular damage will occur if the globe is opened (windows cut) or injected with formalin prior to fixation.8

    Some procedures suggest transferring to 70% ethyl alcohol overnight before dissection. Another suggested technique is to process the specimen whole before undertaking detailed dissection.

    Tumour

    • Section the optic nerve transversely at the resection margin and submit for processing prior to opening the orbit to prevent potential contamination by neoplastic cells.1
    • Sample vortex veins.
    • Usually sectioned in the anteroposterior plane; the plane of the first section is guided by external examination and transillumination. The purpose of this assessment is to ensure that one segment represents the maximum tumour dimension and its relationship with pupil and optic nerve. Section the globe in the antero-posterior plane at 3-4mm intervals according to findings of transillumination.

    Non-tumour

    • For cataracts and glaucoma; vertical sections as these surgical sites are present superiorly
    • For macular pathology; horizontal sections

    Ensure adequate fixation prior to dissection in at least 60ml of formalin for 48 hours.8 One method suggests placing gauze or tissue paper between the eyelids during fixation.8

    After sufficient fixation, paint the external soft tissue surgical margins with ink to highlight the superior and inferior aspects8 and record the colours applied.

    • If bone is included in the specimen, describe, dissect from soft tissue and submit for decalcification before processing.8
    • Take the first section from the optic nerve margin8
    • Then dissect off the inferior calotte
    • Transversely section through the entire specimen (soft extraocular tissues and globe) at 3-4mm intervals in a similar manner to enucleation specimens (but also obtaining sections that demonstrate extent of extraocular spread).
    • Aim to produce at least three thick sections. These will require deeper cassettes (>5mm) for processing.
    • Radially section the medial and lateral slices to demonstrate any tumour close to these margins8

    Internal Inspection

    Describe the internal appearance including the following items:

    Tumour

    • Absent
    • Present
      • Number; if more than one tumour, designate and describe each tumour separately

    Tumour size (mm)

    • Maximum dimension

    Tumour site

    Record the site of the tumour and components involved (more than one may apply).

    Compartments involved4

    • Iris
    • Ciliary body
    • Choroid
    • Extra-scleral invasion (mm)
    • Growth pattern
    • Focal solid
    • Diffuse or ring
    • Bulbar
    • Palpebral
    • Fornix
    • Caruncle
    • Plica seminlunaris
    • Limbus
    • Cornea

    Distance of tumour to margins (mm)

    • Distance of tumour to head of optic nerve
    • Distance of tumour to pars plicata

    Describe the following features of the tumour.

    Vitreous seeding

    • Absent
    • Present

    Tumour growth pattern

    • Endophytic
    • Exophytic

    Anterior chamber8

    • Deep (normal)
    • Shallow

    Aqueous humour8

    • Clear
    • Blood
    • Pus
    • Tumour

    Angle8

    • Closed
    • Open
      • Describe any blood, pigment deposits or abnormal blood

    Lens8

    • Absent
    • Present

    If lens is present, describe:

    • Size
    • Shape
    • Colour
    • Cataract
      • No
      • Yes
    • Opacification
      • No
      • Yes
    • Decentration
      • No
      • Yes
    • Laser capsulotomy site
      • No
      • Yes

    Ciliary body

    • Describe any effusions, evidence of cryotherapy, ciliary body ablation

    Vitreous

    • Consistency
      • Normal (gelatinous) –note that post-mortem specimens will be partly liquid
      • Abnormal, describe
    • Haemorrhage
    • Infection –frank pus
    • Tumour
    • Oil/Silicone –evidence of previous surgery

    Retina

    • Normal
    • Abnormal, describe
      • Detached –note that this may be related to cutting
      • Retinal holes/tears
      • Evidence of previous surgery –e.g. black/white spots
      • Exudates
      • Haemorrhage
      • Pigmentation abnormalities
      • Macular abnormalities –e.g. cysts or yellow exudate

    Optic disc

    • Normal
    • Abnormal, describe
      • Papilloedema
      • Atrophy
      • Cupping
      • Neovascular tissue growth –e.g. diabetic retinal vein occlusion

    Choroid

    • Thickening
      • Due to haemorrhage
      • Exudate
      • Infammation
      • Tumour

    Photograph the dissected specimen.

    Note photographs taken, diagrams recorded and markings used for identification.

    Retrieved from resection specimen

    • Describe site(s)
    • Number retrieved

    Separately submitted

    • For each specimen container, record specimen number and designation
    • Collective size of tissue in three dimensions (mm)
    • Number of grossly identified lymph nodes submitted
    • Maximum diameter of each (mm)

    Processing

    Dissect the specimen further and submit sections for processing according to the illustrations provided.

    Ensure that any artificial lens present in the specimen is removed prior to processing.

    Submit representative sections of:

    • Optic nerve resection margin1-6
    • One section demonstrating tumour, demonstrating relationship with pupil and optic nerve4-6
    • Calotte/cap from superior pole if possible but inferior pole if tumour is present superiorly.1
    • Two further representative sections of ocular tissue. Alternatively, submit both remaining calottes after obtaining tumour sections.4
    • If melanoma is suspected, sample the vortex vessels1,4-8

    In addition to sections described for enucleation specimens, submit representative sections from the nearest orbital soft tissue or cutaneous margins.

    Note that it may not be possible to obtain a vortex vein section due to presence of soft tissue. Note that deeper cassettes will be required for thick sections.

    If received, submit all lymph nodes and identify the site of each.

    Lymph nodes may be received where melanoma or retinoblastoma is suspected to investigate extrascleral metastases as there are no intraocular lymphatics.1

    Record details of each cassette.

    An illustrated block key similar to the one provided may be useful.

    Block allocation keys

    Cassette id
    Site
    No. of pieces
    A
    Optic nerve resection margin
     
    B
    Tumour, demonstrating relationship with pupil and optic nerve
     
    C
    Calotte from superior pole
     
    D
    Ocular tissue, representative sections or remaining calottes
     
    E
    Vortex vessels (for melanoma if applicable)
     
    Cassette id
    Site
    No. of pieces
    A
    Optic nerve resection margin
     
    B
    Tumour, demonstrating relationship with pupil and optic nerve
     
    C
    Calotte from superior pole
     
    D
    Ocular tissue, representative sections or remaining calottes
     
    E
    Vortex vessels (for melanoma if possible, vein  may not be accessible)
     
    F
    Representative sections of nearest orbital soft tissue or cutaneous margins
     

    Acknowledgements

    Assoc Prof Sonja Klebe for her contribution in reviewing and editing this protocol.


    References

    1. Lester SC. Manual of Surgical Pathology, Saunders Elsevier, Philadelphia, 2010.
    2. Mudhar HS, Irion L. Tissue pathways for non-neoplastic ophthalmic pathology specimens. The Royal College of Pathologists; London, 2015.
    3. Mudhar HS, Bründler M-A and Luthert P. Dataset for ocular retinoblastoma histopathology reports, The Royal College of Pathologists, London, 2010.
    4. Menocal NG, Ventura DB and Yanoff M. Routine processing of ophthalmic tissue for light microscopy. Am J Med Techno. 1977; 43(2):156-160.
    5. Torczynski E. Preparation of ocular specimens for histopathologic examination. Ophthalmology. 1981; 88(12):1367-1371.
    6. Ford AL, Mudhar HS, Farr R, Parsons MA. The ophthalmic pathology cut-up—Part 1: The enucleation and exenteration specimen. Current Diagnostic Pathology. 2005;11(4):284-90.

    Jump To

      Eye enucleation 1

      Eye enucleation

      Eye exenteration 1

      Eye exenteration

      Eye exenteration 4

      Eye exenteration dissection

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      26-Mar-2019
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