Various Classifications and Grading Systems in Oculoplasty

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Dr Joyeeta Das MBBS,DNB, Disha Eye Hospital , Kolkata

Articles Oculoplasty Oculoplasty Must Read

Published Online: April 1st, 2021 | Read Time: 23 minutes, 36 seconds

Introduction:

Grading & Classification of diseases is an ever-evolving concept. This article is a collection of various clinical grading and Classification systems in the practice of various Oculoplasty and Orbital diseases. These grading systems are useful for Trainee doctors, PG students, PG residents, and optometrists which will help them in preparations of various examinations and the initial phase of their clinical practice for systematic management of various oculoplasty cases.

Commonly used classifications in oculoplasty are:

Classification of Entropion
Classification Ectropion
Classification Ptosis
Classification of Contracted Socket
Classification of Eyelid Tumor
Classification of Facial Nerve Palsy
Classification of TRO
Classification of Orbital Cellulitis

Entropion :

Entropion is a common eyelid malposition in which the margin turns inward against the globe. Two other conditions that are close to it is trichiasis and distichiasis which must be well differentiated from trichiasis (misdirection of eyelashes) and distichiasis (anomalous eyelashes row). If untreated, this condition can cause symptoms like ocular discomfort, corneal abrasion, microbial keratitis, corneal vascularization, and visual loss

Entropion may affect both upper and lower lid but lower lid entropion is much common than upper lid entropion which is mostly due to cicatrix.

A. Classification of upper lid entropion

Etiological classification:

A. Congenital UL entropion	B. Acquired UL entropion
Rare condition d/t vertical kink develops in the tarsal plate in utero resulting in direct opposition of lid margin to the globe	Relative vertical shortage of posterior lamellar tissue [ tarsus and or palpebral conjunctiva or UL retractor
	Causes : Infection [65% of total] trachoma chronic blepharoconjunctivitis HZO Trauma [19%] Chemical injury iatrogenic mechanical anophthalmic socket Immunologic Erythema multiforme ocular cicatricial pemphigoid ocular vernal cicatricial conjunctivitis Dysthyriod
Treatment: urgent surgical excision of tarsal kink and lid eversion	Treatment: Surgical according to grade and pathology

Kemp and Collin Classification and management scheme of Upper Lid Entropion

Degree of UL entropion	Clinical signs	Surgical management
Minimum	Apparent migration of meibomian gland orifice conjunctivalization of the lid margin lash globe contact onUPGAZE	Anterior lamellar reposition +/- lid split at grey line
Moderate	Features of Minimum plus Lash globe contact on PRIMARY GAZE thickening of the tarsal plate Lid retraction	Anterior lamellar reposition + lid split at grey line + tarsal wedge resection Lamellar division
Severe	Lid retraction causing incomplete closure Gross lid distortion metaplastic lashes presence of keratin plaques	Lamellar advance Rotation of terminal tarsus [TENZEL] Rotation of terminal conjunctiva and posterior lamellar graft [ BMM graft or post auricular cartilage or hard palate graft

B. Lower lid entropion

Congenital L/L entropion

Acquired L/L entropion

Types of congenital inversion of eyelids

Epiblepharon
Entropion

Involutional
Cicatricial
Spastic

Congenital entropion	Congenital epiblepharon
Lid margin inversion present Cilia directed towards the globe	No lid margin inversion Only cilia vertically oriented
Worsen with time	Improves spontaneously
Treatment - Surgery -Hotz procedure	conservative rarely- Hotz procedure

Acquired Entropion:

Types	Mechanism	Management outline
Involutional	Horizontal lid laxity [HLL] LL retractor disinsertion overriding of preseptal orbicularis oculi on pretarsal orbi oculi.	Temporary - Transfixation suture [Quickert-Rothburn suture] Permanent treatment If no horizontal laxity - Weis procedure [full thickness transverse lid split +everting suture If Lid laxity present Quickert procedure [ Transverse lid split+everting sut+horizontal lild shortening] Jones Procedure : Plication of L/L retractor + horiz lid shortening Wheelers orbicularis transposition [less common procedure]
Cicatricial	Contracture of scar tissue in posterior lamella Vertical shortening post lamella	If trichiasis present : Grey line split + tretractor repositioning If no trichiasis, only LL retraction below inferior limbus <1.5 mm - tarsal fracture+everting suture >1.5 mm- posterior lamellar graft by post.auricular cartilage, hard palate or BMM
Spastic	Irritation of ocular surface - -Reflex contraction of Orbicularis oculi- In turning of of L/L	Conservative Botulinum toxin

Ectropion :

Upper lid Ectropion:

Congenital UL ectropion

Acquired UL ectropion

Rare
total B/L eversion of U/L in newborn
Causes :
- Venous obstruction
- Chlamydia

Cicatricial : due to anterior lamellar shortening by trauma, burn,acid injury etc

Floppy eyelid syndrome :
1. obese men
2. sleep apnoea
3. spontaneous eversion of UL during sleep - loss of lid globe contact

Lower Lid Ectropion :

Congenital Lower lid ectropion	Acquired Lower lid ectropion
Blepharophimosis syndrome	Involutional
Down syndrome	Paralysis
Secondary to Anophthalmos microphthalmos buphthalmos Euryblepharon icthyosis cyst	Cicatricial
	Mechanical

Ptosis :

Ptosis or blepharoptosis is defined as a drooping or abnormally low position of the upper eyelid with respect to the globe. It can be classified as :

Congenital

Acquired ptosis

Primary - levator maldevelopement

Aponeurotic

Senile ptosis
post-surgery
post-trauma

Secondary

Neurogenic
- 3rd Nv palsy
- Horner's syndrome
- Marcus-Gunn JAw winking syndrome
- Mysthenia gravis
myogenic
- Blepharophimosis
- congenital fibrosis
- CPEO
Aponeurotic- post birth trauma
mechanical - lid mass

Neurogenic

3rd Nv palsy
Horner's syndrome
Marcuss-Gunn JAw winking syndrome
Mysthenia gravis

Myogenic

Chronic progressive external ophthalmoplegia [CPEO]
Muscular dystrophy

Mechanical

Lid mass
scarring

Contracted socket :

Baylis et al.¹ classification of contracted sockets into two groups

Moist conjunctival surfaces and
Dry surfaces

Molgat²and Guyuron³classification of contracted socket:

Primary volume deficits
Primary mucosal deficits
Both volume and mucosal deficits

Bonavolonta^2,3 classified contracted sockets into 6 categories:

contraction of the conjunctival fornix
contraction of conjunctival fornix and lid
contraction of conjunctival fornix and orbital fat
absence of conjunctival fornix
severe contraction of orbital fat
any of the above plus developmental bone abnormalities

Gopal krishna classification

The soft tissue sockets were divided into five grades for the sake of convenience in management of contracted sockets.

Grade-0: Socket is lined with the healthy conjunctiva and has deep and well-formed fornices.
Grade-I: Socket is characterized by the shallow lower fornix or shelving of the lower fornix. Here the lower fornix is converted into a downwards sloping shelf which pushes the lower lid down and out, preventing retention of a artificial eye
Grade-II: Socket is characterized by the loss of the upper and lower fornices,
Grade- III: Socket is characterized by the loss of the upper, lower, medial and lateral fornices
Grade-IV: Socket is characterized by the loss of all the fornices, and reduction of palpebral aperture in horizontal and vertical dimensions
Grade-V: In some cases, there is recurrence of contraction of the socket after repeated trial of reconstruction

1) Baylis HI, Schorr N, Tanenbaum M, McCord CD. The anophthalmic socket: evaluation and management of surgical problems. In: McCord CD, Tanenbaum M, editors. Oculoplastic Surgery. New York: Raven Press; 1987. pp. 609–37

2) Molgat YM, Hurwitz JJ, Web MC. Buccal mucous membrane-fat graft in the management of the contracted socket. Ophthal Plast Reconstr Surg. 1993;9:267–72

3) Bonavolontà G. Temporalis muscle transfer in the treatment of the severely contracted socket. Adv Ophthalmic Plast Reconstr Surg 1992;9:121-9

4) Krishna G. Contracted sockets -I (Aetiology and types). Indian J Ophthalmol 1980;28:117-20

Classification of Eyelid Tumor :

Clinical classification:

Pigmented eyelid mass :
1. Basal cell CA
2. Nevus
3. Malignant melanoma
4. Nevus of Ota
Non-pigmented eyelid mass :

A. Epithelial

Papilloma- sessile, pedunculated
Keratoacanthoma- central crater with a keratin plug
Actinic keratosis- dry rough scales
Seborrhic keratosis-greasy, brown, friable
Basal cell CA - shiny
Squamous cell CA- crusting, erosion, fissure

B. Subepithelial

Solid:
1. Sebaceous cell CA
2. Meibomian cyst
Cystic
1. Cyst of Moll
2. Cyst of Zeiss
3. Sebaceous cyst

Benign eyelid tumor

Malignant eyelid tumor

Pigmented Mass

Nevus
Nevus of Ota

Primary

Basal Cell CA
Squamous cell CA
sebaceous cell CA
Malignant melanoma
Kaposi sarcoma

Non-pigmented benign mass

Epithelial

Papilloma
keratoacanthoma-
actinic keratosis
seborrhic keratosis

Subepithelial

meibomian cyst
cyst of Mall
cyst of Zeiss
sebaceous cyst

Secondary

Lymphoma
Maxillary CA
others

Classification and grading of Facial nerve palsy :

House-Brackmann facial paralysis scale

Grade	Descriptions	Characteristics
1	Normal	Normal facial function
2	Mild dysfunction	Gross: slight weakness noticed in close observation At rest : normal symmetry and tone Motion : Forehead - Moderate to good function Eye - complete closure with minimum effort Mouth - slight asymmetry
3	Moderate dysfunction	Gross: obvious asymmetry but not disfiguring, no synkinesis At rest : normal symmetry and tone Motion : Forehead - slight to moderate function Eye - complete closure with effort Mouth - slight weak with maximum effort
4	Moderate to severe dysfunction	Gross: obvious asymmetry and /or disfiguring At rest : normal symmetry and tone Motion : Forehead - none Eye - incomplete closure Mouth - asymmetry with maximum effort
5	Severe dysfunction	Gross: only barely perceptible motion At rest: Asymmetry Motion : Forehead - none Eye - incomplete closure Mouth - slight movement
6	Total paralysis	No movements

The CADS grading scale :

The CADS grading scale is specifically designed for periorbital involvement with a combination of objective and subjective parameters. There are 4 main aspects of the scale: Cornea, static Asymmetry, Dynamic function, and Synkinesis. Cornea takes into account the amount of corneal staining, presence of corneal sensation, Schirmer’s test, and patient’s Bell’s phenomenon

Cornea	Score	Static Asymmetry	Score	Dynamic asymmetry	score	Synkinesis	score
No staining	0	No brow ptosis No ectropion No upper or lower eyelid retraction	0	No blink lagophthalmos	0	absent	0
PEE < 5	1	Mild brow ptosis Medial ectropion Upper eyelid at limbus Mild lower eyelid retraction; ≤2 mm inferior scleral show LMBD >5 mm shorter than contralateral side	1	Lagophthalmos on blink <5 mm Brow elevation reduced but present	1	Mild eye closure when smiling/ speaking eating Gustatory epiphora: not bothersome	1
PEE≤ 1/2 cornea	2	Severe brow ptosis Significant ectropion Superior scleral show >2 mm inferior scleral show LMBD ≤ 20 mm	2	Lagophthalmos on blink ≥5 mm Lagophthalmos on gentle closure ≤5 mm Brow elevation: none or twitch	2	Signifcant eye closure when smiling/speaking/ eating Gustatory epiphora: bothersome	2
PEE ≥1/2 cornea or epithelial defect	3	-		Lagophthalmos on gentle closure >5 mm Lagophthalmos on forced closure >2 mm	3	-
If any of the below present add Absent corneal sensation Absent/reduced Bell’s phenomenon Schirmer’s ≤ 5 mm* The affected eye is the only eye	a	-
*Schirmer’s test with anesthetic and if the lower eyelid is apposed to the globe. .		PEE, punctate epithelial erosions		LMBD, lid–margin brow distance

Reference: Tan P, Siah WF, Wong J, Malhotra R. Validation of CADS Grading Scale: An Ophthalmic Specific Grading Instrument for Facial Nerve Palsy. Ophthalmic Plast Reconstr Surg. 2017 Nov/Dec;33(6):419-425. PMID: 27811632.

Thyroid Orbitopathy [TRO] classifications and grading systems :

Thyroid eye disease or thyroid-related orbitopathy (TRO) or Graves orbitopathy is the most common cause of bilateral proptosis. It is a poorly understood ailment of autoimmune pathology.

The ocular presentation can vary from mild disease to severe irreversible sight-threatening complications and the varied presentations posses challenge to treating ophthalmologist according to various stages of the disease.

The ocular disease has an active and inactive phase. Since 1960 various classification and scoring systems have been proposed for better evaluation of the clinical course of the disease and its management.

The clinical examination, activity, and severity aid the ophthalmologist to decide the stage of the disease and come up with the treatment strategy for each patient

Several classification systems have been proposed to assess the clinical manifestations of TED. The evolution of various classification systems has been summarized in the table below

Classification systems	Proposed by	Year
NO SPECS classification	Werner	1969
Modified NO SPECS	Werner	1977
CAS	Maurits et al	1989
Modified CAS	Maurits et al	1997
EUGOGO classification	EUGOGO	1999
VISA classification	Rootman and Dolman	2006

CAS: clinical activity scale

EUGOGO: the European Group of Graves’ Orbitopathy

VISA: Vision, Inflammation, Strabismus, Appearance

Werner in 1969 devised a mnemonic NO SPECS to document disease severity which was modified in 1977 and is broadly used since then^.This classification, however, grades clinical severity but does not distinguish the active inflammatory phase from the inactive phase, and indication for treatment was decided only according to the severity of the disease.

Modified NO SPECS classification

	Class	Grade	Signs and symptoms
N	0		No symptoms or signs
O	1		Only signs (upper lid retraction, without lid lag or proptosis)
S	2		Soft tissue involvement with symptoms (excess lacrimation, sandy sensation, retrobulbar discomfort, and photophobia, but not diplopia); objective signs as follows:
		0	absent
		a	minimal (edema of conjunctivae and lids, conjunctival injection, and fullness of lids, often with orbital fat extrusion, palpable lacrimal glands, or swollen extraocular muscles beneath lower lids)
		b	Moderate (above plus chemosis, lagophthalmos lid fullness)
		c	marked
P	3		Proptosis associated with classes 2 to 6 only (specify if inequality of 3 mm or more between eyes, or if the progression of 3 mm or more under observation)
		0	absent (20 mm or less)
		a	minimal (21-23 mm)
		b	moderate (24-27 mm
		c	marked (28 mm or more)
E	4		Extraocular muscle involvement (usually with diplopia)
		0	absent
		a	minimal (limitation of motion, evident at extremes of gaze in one or more directions)
		b	moderate (evident restriction of motion without fixation of position)
		c	marked (fixation of the position of a globe or globes)
C	5		Corneal involvement (primarily due to lagophthalmos)
		0	absent
		a	minimal (stippling of the cornea)
		b	moderate (ulceration)
		c	marked (clouding, necrosis, perforation)
S	6		Sight loss (due to optic nerve involvement)
		0	absent
		a	minimal (disc pallor or choking, or visual field defect, vision 20/20 to 20/60)
		b	moderate (disc pallor or choking, or visual field defect, vision 20/70 to 20/200)
		c	marked (blindness, i.e., failure to perceive light; vision less than 20/200)

In 1989, Mourits et al. described the Clinical Activity Score (CAS). This score discriminates between the active and quiescent stage of the disease as it is based on the classical signs of inflammation (pain, redness, swelling, and impaired function). This was further modified in 1997. Modified CAS is used to evaluate disease activity, and a score out of 10 is given. A score of 3 or less is considered as inactive and 4 or more is considered as active eye disease at first examination. A score of 4 or more on follow-up examination indicates active disease. This scoring criterion has the disadvantage of being subjective in nature with a large interobserver variation. The advantage lies in its easy applicability in everyday clinical practice.

Clinical Activity Score amended by European group on Graves’ Orbitopathy

	For initial CAS score items 1-7
1	Spontaneous orbital pain
2	Gaze evoked orbital pain
3	Eyelid swelling that is considered to be due to active GO
4	Eyelid erythema
5	Conjunctival redness considered due to active GO
6	chemosis
7	Inflammation of caruncle or plica

	Follow‑up after 1-3 months score items including 8-10
8	Increase of >2 mm proptosis
9	A decrease in a uniocular ocular excursion in any one direction of >8 degrees
10	Decrease of acuity equivalent to 1 Snellen line

One point is given for the presence of each of the parameters assessed.

The sum of all points define clinical activity:

Active ophthalmopathy [TR] if at first examination score is >3/7 or in successive examination >4/10

The other grading systems used are VISA classification and the European Group on Graves' Orbitopathy (EUGOGO) classification.

Both VISA and EUGOGO systems are assessed with practical implications for guiding the management of patients, which was missing in earlier classifications by Werner. These two classification systems are not interchangeable. VISA is widely used in the United States and Canada and EUGOGO in Europe.

EUGOGO Classification :

Stage	Features
Mild thyroid eye disease	Minor impact on activities of daily living Insufficient justification for immunosuppression or surgical treatment One or more of the following Minor lid retraction (<2 mm) Mild soft-tissue involvement Proptosis<3 mm above normal for race and gender No or transient diplopia Corneal exposure responsive to lubricants
Moderate‑to-severe thyroid eye disease	Impact on activities of daily living Justifies treatment (immunosuppression and/or surgical treatment) Two or more of the following Lid retraction 2 mm or more Moderate to severe soft-tissue involvement Proptosis≥3 mm above normal for race and gender Diplopia (inconstant or constant)
Sight threatening thyroid eye disease	Compressive optic neuropathy Corneal ulceration

The VISA system was developed by Dolman and Rootman in 2006 and was adopted with modifications by the International TED Society (ITEDS). Each section records subjective inputs and measurable objective inputs. This helps direct appropriate management for patients with TED in a logical sequence depending on the aspect of the disease affecting them.

VISA grading of Thyroid Orbitopathy:

Category

Subjective input

Objective examination

Vision

Visual blurring

Color desaturation

Visual acuity, refraction

Color vision

Pupil reaction

Fundus and optic nerve examination

Standard perimetry

Neuroimaging

Visual evoked potential

Inflammation

Retrobulbar ache

Eyelid or conjunctival swelling

Eyelid or conjunctival redness

Inflammatory index (worst eye/eyelid)

Retrobulbar ache

At rest (0-1)
With gaze (0-1)
Chemosis (0-2)*

Eyelid edema (0-2)**

Conjunctival injection (0-1)

Eyelid injection (0-1)

Inflammatory index total: 10

Strabismus

Diplopia

No diplopia (0)

With gaze (1)

Intermittent (2)

Constant (3)

Extraocular movements

Restriction of Ductions

>45 deg (0)
30-45 deg (1)
15-30 deg (2)
<15 deg (3)

Head posture

Prism cover test

Field of binocular vision

Appearance

Eyelid retraction

Proptosis

Tearing

Foreign body

sensation

Eyelid retraction

Scleral show

Lid lag and von Graefe’s sign

Lagophthalmos

Presence of redundant skin and fat

prolapse

Exophthalmometry

Slit-lamp examination ‑.cornea

VISA – Vision, Inflammation, Strabismus, and Appearance

Disease grading: Global severity grade (maximum of 20 points):

Vision (optic neuropathy) y/n (1 point),
Inflammation (0‑10),
Strabismus ‑.diplopia (0‑3); restriction (0-3),
Appearance/exposure: mild/moderate/ severe (0-3).

*Chemosis

.0: Absent;
1: Conjunctiva behind the grey line;
2: Conjunctiva anterior to the grey line),

**Eyelid edema

0: Absent;
1: Present without redundant tissue;
2: Present and causing bulging in palpebral skin including lower lid festoon.

VISA inflammatory index (activity score):

<4/10‑.treat conservatively,

>5/10 or evidence of disease progression - treat aggressively,

Grading and stages of Orbital cellulitis :

Chandler class	Stage	Clinical features
I	Inflammatory edema/ preseptal cellulitis	Eyelid edema and erythema Normal extraocular movements Normal visual acuity
II	Orbital cellulitis	Diffuse edema of orbital contents without abscess formation
III	Subperiosteal abscess	Collection of purulent exudate beneath the periosteum displacement of globe
IV	Orbital abscess	purulent pus collection in orbit [ CT scan ] proptosis chemosis ophthalmoplegia diminished vision
V	Cavernous sinus thrombosis	Bilateral eye findings Prostrations meningism

Articles Oculoplasty Oculoplasty Must Read

Dr Joyeeta Das MBBS,DNB,

Disha Eye Hospital , Kolkata

Dr. Joyeeta Das is a specialist Orbit and Oculoplastic surgeon having extensive clinical and surgical expertise in the field. Dr. Das joined Disha Eye hospital in 2016 as a consultant Orbit and oculoplastic surgeon. She has attended NRS Medical College, University of Calcutta as an undergraduate and received her MBBS degree. Subsequently, she did her postgraduate ophthalmology degree training in Rotary Narayana Nethralaya, Kolkata and awarded Diplomate of National Board degree in ophthalmology. Following extensive training and Long Term Fellowship in Orbit And oculoplasty from Disha Eye hospital, Kolkata she has joined Sadguru Netra Chikitsalaya, Chitrakoot, MP as consultant and Head of the department, Orbit, and Oculoplasty in 2012 and Sankara Eye hospitals since 2014 to 2016. She was actively involved in teaching postgraduate and fellowship students. To her credit, she has presented numerous papers, lectures in various national and international conferences. She has received Best lecture Award in Confluence-an International conference on-orbit and oculoplasty in 2013 held in Hyderabad and Award Proff. Dr. Ranabir Mukherjee award for best paper in Kolkata academy of Opthalmology Annual Meet 2019. She has published multiple article in various international journal of ophthalmology including BMJ case reports, Orbit and Ophthalmic plastics and reconstructive surgery and Indian journal of ophthalmology(IJO) etc. She is a reviewer of BMJ case reports journal . Her area of interest are complex eyelid reconstructive surgery , facial nerve palsy , facial dystonia and orbital disease.