Enoftalmos Non Traumatik

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IntroductionEnophthalmos is a relative posterior

displacement of a normal-sized globe

in relation to the bony orbital mar-

gin. It may be unilateral or bilateral

but comes to the attention of the cli-

nician more often when unilateral.

Enophthalmos is often secondary to

orbital trauma, but non-traumatic

cases (although uncommon) warrant

a detailed evaluation because an

underlying systemic process is fre-

quently the cause. In recent years,several causes of this condition )

such as the silent sinus syndrome )

have been added to the literature. Inthis review, we discuss the clinical

features, pathophysiology and causes

of non-traumatic enophthalmos.

Definition

Enophthalmos is best defined as a rel-

ative posterior displacement of a nor-

mal-sized eye in relation to the bony

orbital margin. Some authors have

used an absolute reading on exoph-

thalmometry to indicate enophthal-

mos, such as less than 10–12 mm

(Wright 1970) or 14 mm (Yip et al.2005). Unilateral enophthalmos is said

to be present when there is a differ-

ence of more than 2 mm on exoph-

thalmometry between the two eyes

(Wagener 1933; Kempster et al. 2005).

Koo et al. (2006), however, found that

enophthalmos becomes clinically obvi-

ous only when there is a difference of

3–4 mm or more between the two

eyes. It needs to be emphasized here

that the definition of enophthalmos is

not entirely objective and factors

other than ocular position alone )

such as orbital structure ) influence

the perception of enophthalmos.

Clinical features

Enophthalmos is not always evident

clinically and may present with vari-

able signs and symptoms. In fact,

Cline & Rootman (1984) found that

approximately 50% of patients with

enophthalmos were initially referred

for investigation of contralateral

exophthalmos, ptosis or diplopia.

Patients with enophthalmos can pres-

ent with both cosmetic and functional

issues. Cosmetic concerns include analtered appearance because of a deep-

seated globe, asymmetric position of

the eyes, deep superior sulcus, pseu-

doptosis or eyelid retraction (Rubin &

Rumelt 1999; Yip et al. 2005). Func-

tional problems include diplopia, dry

eyes and corneal desiccation leading

to ulceration. Although diplopia is

seen most frequently in traumatic

enophthalmos, it also occurs in

patients with restricted ocular move-

ments caused by a cicatrizing process

(Lagreze et al. 1997). In cases of severeenophthalmos, the globe is drawn

away from the eyelids, and this leads to

Review Article

Non-traumatic enophthalmos:a reviewPaul A. Athanasiov, Venkatesh C. Prabhakaran and Dinesh Selva

Oculoplastic and Orbital Division, Department of Ophthalmology and Visual

Sciences, University of Adelaide and the South Australian Institute of

Ophthalmology, Adelaide, Australia

ABSTRACT.

Enophthalmos can be defined as a relative, posterior displacement of a nor-

mal-sized globe in relation to the bony orbital margin. Non-traumatic enoph-

thalmos has a wide variety of clinical presentations and may be the first

manifestation of a number of local or systemic conditions. It may present with

cosmetic problems such as deep superior sulcus, pseudoptosis or eyelid retrac-

tion; or functional problems such as diplopia or exposure keratopathy. There

are three main pathogenic mechanisms: structural alterations in the bony orbit;

orbital fat atrophy; and retraction. Evaluation of enophthalmos patients

includes orbital imaging and a thorough ophthalmic and systemic examination.

In this review, we discuss the presenting features of non-traumatic enophthal-

mos and include a brief description of the more important causes. An approachto the clinical evaluation of these patients is also discussed together with a

brief overview of the principles of management.

Key words: enophthalmos – orbital fat atrophy – orbital fibrosis – orbital metastases – pseudo-

enophthalmos – silent sinus syndrome

Acta Ophthalmol. 2008: 86: 356–364

ª 2008 The Authors

Journal compilation ª 2008 Acta Ophthalmol

doi: 10.1111/j.1755-3768.2007.01152.x

Acta Ophthalmologica 2008

356



dry eye symptoms and, more seriously,

to corneal drying and ulceration

(Buono 2004). Lagophthalmos may

also be a causative factor for corneal

ulceration in some patients (Yip et al.

2005).

Pulsating enophthalmos is a veryrare condition that is caused by the

transmission of intracranial pulsation

to the eye via a congenital or iatro-

genic bony defect. The pulsation is

usually obvious clinically, but is espe-

cially noticeable when performing

applanation tonometry or Hertel’s

exophthalmometry. A single patient

with oscillopsia secondary to enoph-

thalmos has also been reported (Zam-

barakji & Rose 2001).

Measurement

Hertel’s exophthalmometer is the

instrument most frequently used to

measure the position of the globe in

relation to the orbit. It is important to

note that any asymmetry between the

lateral orbital rims will affect the

readings significantly when using this

instrument. In such cases, instruments

that rest on the superior and inferior

rims, such as the Naugle’s orbito-

meter, may be used (Naugle &

Couvillion 1992). In all cases of en-

ophthalmos, vertical and horizontaldisplacement of the globe should also

be measured; this is performed most

simply using a transparent scale to

compare the relative pupillary or lim-

bal positions between the two eyes.

Radiographic measurements may be

more accurate, but in the absence of

any normative data, their validity is

doubtful. Traditionally, the cor-

nea)clinoid distance has been mea-

sured on lateral radiographs of the

orbit, using a radio-opaque contact

lens to indicate the corneal position(Tengroth 1964; Silva 1968). More

recently, similar measurements have

been performed using computed

tomography and magnetic resonance

imaging (Whitehouse et al. 1994).

Pathophysiology

As proposed by Cline & Rootman,

enophthalmos can be produced by

three main mechanisms: structural

abnormality, fat atrophy and traction

(Cline & Rootman 1984).

Structural abnormality refers to

changes in the bony orbit that result

in an increase in orbital volume

compared to the volume of its constit-

uents, which results in posterior

displacement of the globe. While

trauma is the most common cause of

such structural changes (Cline &

Rootman 1984), other causes include:posterior bowing of the floor second-

ary to chronic sinusitis (silent sinus

syndrome) (Fig. 1B); congenital or

iatrogenic defects in the greater wing

of sphenoid; congenital bony orbital

asymmetry; and bone pathology

[Paget’s disease of the bone (PDB)].

Fat atrophy causes a reduction in

the volume of the contents of the

orbit, thus allowing the globe to sink

backwards within a normal bony

orbit. Fat atrophy can be an age-

related process (senile enophthalmos)

or a manifestation of lipodystrophy

but can also be a result of associated

orbital pathology such as orbital vari-

ces. It is interesting that weight loss,

even when significant, does not result

in orbital fat decrease and enophthal-

mos. In this context, a guinea-pig

study has suggested that intraorbital

adipocytes may compensate for a sys-

temic decrease by enlarging in size so

as to maintain a constant volume

(Mattacks & Pond 1985).

Enophthalmos can also be caused

by posterior traction secondary to

fibrosis of the extraocular muscles or

connective tissues; this is usually

associated with ocular motility prob-

lems. This mechanism is responsible

for the enophthalmos seen in some

orbital metastases and also for that

associated with fibrosis of the extra-

ocular muscles.The clinical features of enophthal-

mos are a direct result of the mecha-

nism causing the enophthalmos. In

most cases, the retrusion of the globe

is associated with a deep superior sul-

cus and narrowed palpebral fissure

(pseudoptosis) (Rubin & Rumelt

1999) (Fig. 2A). In cases with a very

deep superior sulcus, lagophthalmos

may be noted (Yip et al. 2005). Some

cases of superior lid retraction seen in

association with enophthalmos may

be secondary to hypoglobus; it hasbeen postulated that the drooping

globe pulls down on Whitnall’s liga-

ment leading to upper lid retraction

(Anderson & Dixon 1979). Hypoglo-

bus may be a result of orbital volume

deficit or orbital floor changes, and in

these cases pressure by the globe on

the inferior rectus may also lead

to lower lid retraction (Rubin &

Rumelt 1999).

AetiologyThe various non-traumatic causes of

enophthalmos are listed in Table 1.

(A)

(B)

Fig. 1. (A) Clinical photograph of a 38-year-

old female with silent sinus syndrome leading

to enophthalmos of the left eye. (B) The CT

scan shows opacification of the left maxillary

sinus with thinning and bowing of inferior

orbital floor resulting in expansion of left

orbital volume.

(A)

(B)

Fig. 2. (A) Clinical photograph of a 59-year-

old female with enophthalmos of the left eye

secondary to metastatic scirrhous carcinoma

of the breast. (B) The CT scan demonstrates

irregular opacification (metastatic disease

with fibrosis) in the left posterior orbit

(arrow) with resulting retraction of the globe.


357



The causes are listed according to the

proposed mechanism of causation,

where known. A brief discussion of

the more important causes follows.

Structural abnormalityMaxillary sinusitis and silent sinus

syndrome

Chronic maxillary sinusitis with

obstruction of the sinus ostium can, in

some cases, lead to centripetal col-

lapse of the sinus walls (atelectasis)

with subsequent enophthalmos (Mont-

gomery 1964). Often, no prior history

of sinusitis is present, and patients

present with spontaneous enophthal-

mos and hypoglobus (silent sinus syn-

drome) (Soparkar et al. 1994). Thiscondition is always unilateral and is

seen equally in males and females in

their third to fifth decades (Buono

2004). Other features include lid

retraction, deep superior sulcus, lid

lag and lagophthalmos (Fig. 1A).

Rare symptoms include diplopia,

oscillopsia and (rarely) an audible

clicking sound on blinking (caused by

trapping of air in the conjunctival

recess) (Dailey & Cohen 1995).

Imaging of the sinuses (particularly

with computed tomography) is diag-nostic and shows partial or total

opacification of the maxillary sinus

with reduction in sinus volume, down-

ward bowing of the orbital floor and

concomitant increase in orbital vol-

ume (Fig. 1B). The orbital floor is

often thin and may be absent in rare

cases (Buono 2004).

The pathophysiology is not entirely

clear, but the condition is believed to

be a result of negative pressure within

the sinus. Resorption of secretions

within an obstructed sinus leads to the

development of subatmospheric pres-

sure with subsequent sinus collapse

(simulating maxillary hypoplasia) (Bu-

ono 2004). It should be mentioned

here that many of the previously

reported cases of enophthalmos asso-

ciated with maxillary sinus mucocele

probably share a similar pathophysiol-

ogy (Cline & Rootman 1984).

Treatment is usually surgical and

involves restoration of normal sinus

ventilation with endoscopic ⁄ external

antrostomy and repair of the orbital

floor (Thomas et al. 2003). Rarely,

spontaneous resolution may be seen

(Raghavan et al. 2001).

Orbital bony defects

Defects in the bony orbit may be

congenital or iatrogenic (following

surgery). Absence of the greater wing

of the sphenoid (GWS) is the most

common congenital bony defect and

is classically seen in association with

neurofibromatosis I. It is hypothe-

sized that the bony changes in neuro-

fibromatosis may be a result of the

interaction between the developing

orbit and the orbital neurofibromas

(Jacquemin et al. 2003). Absence of

the GWS can also be an isolated

anomaly or associated with orbital

varices (Islam et al. 2004) (Fig. 3B)

or (very rarely) with epidermoid cysts

(Chen & Fairholm 1983; Bitar et al.

1993).

Pulsating enophthalmos is rarelynoted in patients with absence of the

GWS. Enophthalmos is a result of

expansion of the orbital cavity and

the pulsation is caused by the trans-

mission of intracranial pulsations to

the eye. Alternatively, and more fre-

quently, the temporal lobe can herni-

ate through this defect to cause

pulsatile exophthalmos (Dabezies &

Walsh 1961; Savino et al. 1977; Jack-

son et al. 1993).

Table 1. Causes of enophthalmos.

Structural abnormality

Maxillary sinus disease

Chronic maxillary sinusitis

Silent sinus syndrome

Maxillary hypoplasia

(Cline & Rootman 1984)Bony defect

Absence of GWS

Neurofibromatosis

Orbital varix

Congenital

Other

Iatrogenic (Salem & Qahtani 2001;

Rose & Lund 2003; Wu et al. 2004)

Orbital varix

Bone disease

Paget’s disease (Hardy & McNab 2002)

Fat atrophy

Age-related

Senile enophthalmos (Yip et al. 2005)

Periorbital ⁄ systemic diseases

Lipodystrophy

Scleroderma

Parry)Romberg syndrome

Pressure effect

Orbital varix

Blue rubber bleb naevus syndrome

(Sobottka Ventura et al. 2001)*

Schizophrenia (Cline & Rootman 1984)*

Leber’s congenital amaurosis

(Babel et al. 1989)*

Radiation

Post-radiotherapy

Unknown

Cockayne’s dystropyhy

(Pasquier et al. 2006)

Hydrocephalus and V-P shunt(Meyer et al. 1996)*

Retraction

Metastasis

Scirrhous breast carcinoma

Gastric carcinoma

Lung carcinoma

Restrictive myopathy

Duane’s retraction syndrome

CFEOM

Iatrogenic (Gittinger et al. 1986;

Khan 2005)

Post-inflammatory

Wegener’s granulomatosis

Tuberculosis (Shome et al. 2006)*

Atypical mycobacterial infection

(Mauriello 2003)

Mixed

Sarcoidosis (Attia et al. 2006)

Primary orbital leiomyoma

(Wiechens et al. 1999)*

Chromosomal disorders (trisomy 9p,

trisomy 7q) (Smart et al. 1988)

Pseudoenophthalmos

Facial asymmetry

Contralateral proptosis

Microphthalmos

Ptosis

Horner’s syndrome (Daroff 2005)

GWS, greater wing of sphenoid; LCA,

leber congenital amaurosis; CFEOM, con-genital fibrosis of extraocular muscles.

*Isolated case reports, not discussed further

in the text.

(A)

(B)

Fig. 3. This 65-year-old female presented

with enophthalmos of the right eye that was

secondary to an orbital varix. (A) Clinical

photograph demonstrates narrowed palpebral

fissure, deep superior sulcus and pseudoptosis

of the right eye (B) CT scan of the same

patient shows a lobulated mass in the rightposterior orbit with absence of the greater

wing of sphenoid (arrow) and part of the

medial orbital wall (arrowhead).


358



Computerized tomography (CT)

will show partial or complete absence

of the greater wing of the sphenoid

bone with enlargement of the superior

orbital fissure and may also reveal the

presence of associated pathology such

as optic nerve glioma, plexiform neu-rofibroma or orbital varices (Binet

et al. 1969; Mortada 1977; Jacquemin

et al. 2003).

A number of operations may result

in enophthalmos, including sinus sur-

gery, base of skull reconstructions and

excessive orbital decompression sur-

gery; a careful history should be cor-

related with imaging results to make

the diagnosis (Salem & Qahtani 2001;

Georgantopoulou et al. 2003; Rose &

Lund 2003; Wu et al. 2004; Kloek

et al. 2006).

PDB

PDB is a chronic, progressive disor-

der in which initial bone destruction

is followed by a disorganized repara-

tive process causing distortion (Paget

1877). Nearly a third of cases involve

the skull (Griz et al. 2006). Enoph-

thalmos is a rare manifestation of

Paget’s disease and is thought to

result when there is differential

expansion of the orbit compared to

the cranium (Hardy & McNab 2002).

The history consists of insidious

bilateral receding eyes and dry eyes

(caused by exposure keratopathy)

(Hardy & McNab 2002). PDB is

diagnosed radiologically, with charac-

teristic widening of the bone, thicken-

ing of its cortex, osteolytic areas and

osteosclerosis (Griz et al. 2006). The

progression of PDB can be followed

with a number of blood tests, includ-

ing alkaline phosphatase levels (Griz

et al. 2006).

Fat atrophySenile changes

Facial lipoatrophy occurs with aging,

starting at the age of 20 and becoming

noticeable at the age of 30 in most peo-

ple (Ascher et al. 2006). This atrophy

may form part of the process responsi-

ble for senile enophthalmos, along with

redistribution of orbital fat (Yip et al.

2005). According to Camirand et al.

(1997), descent of the lateral canthus

and Lockwood’s suspensory ligament

occurs with age, and this causes the

globe to sink thus pushing the orbital

fat pads forward. Relocating the herni-

ated fat pad may correct the enoph-

thalmos (Camirand et al. 1996).

Regardless of the pathogenesis, this is

a common cause of bilateral enoph-

thalmos and comparison with oldphotographs is useful in diagnosis.

Localized scleroderma and Parry)

Romberg syndrome

Scleroderma is a chronic autoimmune

disease that can be systemic or local-

ized to the skin. There are five subsets

of localized scleroderma classified by

the type and extent of cutaneous

involvement: plaque; generalized; bul-

lous; linear; and deep (Peterson et al.

1995).En coup de sabre (ECDS) is a form

of linear scleroderma that frequently

occurs on the face or scalp, often

resembling a stroke from a sword,

and may result in orbital tissue atro-

phy and enophthalmos. Parry)Rom-

berg syndrome (PRS) is a condition

of hemifacial atrophy (Parry 1825;

Romberg 1846) involving skin and

tissues below the forehead that can

also cause enophthalmos, but gener-

ally has minimal involvement of the

superficial cutaneous layers (Aleemet al. 1999; Rai et al. 2000; Stone

2003). Both conditions are more

common in females and are usually

unilateral.

In both conditions, enophthalmos

occurs as subcutaneous fat and muscle

are replaced with collagen. Tradition-

ally, scleroderma has cutaneous atro-

phy, which can include balding and

loss of eyelashes (Karim et al. 2005).

PRS has also been associated with

other areas of scleroderma and atrophy

of tissues such as the brain or breast

(Lakhani & David 1984; Takahashi

et al. 1996; Aleem et al. 1999).

Given that localized scleroderma

and PRS often occur together and

have similar clinical and pathological

characteristics (Lakhani & David

1984; Burroughs et al. 2003; Karim

et al. 2005), PRS is considered by

many to represent a severe form of

ECDS (Menni et al. 1997; Blaszczyk

& Jablonska 1999; Stone 2003; Jab-

lonska & Blaszczyk 2005; Laxer &

Zulian 2006; Sommer et al. 2006).

Diagnosis of both PRS and sclero-

derma is based on clinical examination,

imaging (showing atrophic tissues) and

biopsy. Antimalarial medication and

methotrexate are the mainstays of

treatment (Laxer & Zulian 2006), but

reconstruction may be useful in some

cases (Dawczynski et al. 2006).

Lipodystrophy

Lipodystrophy can be total, partial or

localized (Garg 2000), but enophthal-

mos is usually seen in association with

the partial form. In acquired partial

lipodystrophy, women are affected

more frequently, and it is usually parts

of the upper body and face that are

involved (Nasr et al. 1997). Lupus ery-

thematosus (Ishiguro et al. 2002), der-

matomyositis and Sjogren’s syndrome

have all been associated with partial

lipodystrophy (Alarcon-Segovia &Ramos-Niembro 1976; Garg 2000).

The most prevalent acquired form

is HIV-associated lipodystrophy,

which can result in severe atrophy of

facial subcutaneous fat and enoph-

thalmos (Merchante et al. 2004;

Ascher et al. 2006). The medical man-

agement of HIV is thought to be the

cause of lipoatrophy, rather than the

disease itself (Garg 2004; Ascher et al.

2006). Reconstructive surgery such as

lipofilling and submalar silicone

implants can produce good cosmesisand psychological benefit in patients

with facial lipodystrophy (Koshy &

Evans 1998; Jones 2005; Mori et al.

2006).

Cockayne’s dystrophy

This rare autosomal recessive disorder

was first described by Cockayne as a

combination of severe postnatal

growth retardation with progressive

neurological dysfunction (particularly

mental disability and deafness)

(Cockayne 1936). The average age of

death is around 12 years old, with a

worse prognosis in those who have

structural eye abnormalities (Nance &

Berry 1992). Examination will classi-

cally reveal dwarfism and microceph-

aly as well as a variable number of

other problems such as retinal atro-

phy, cataracts and corneal opacity

(McElvanney et al. 1996; Ozdirim

et al. 1996). Enophthalmos is a well-

recognized feature of this syndrome

and is believed to be caused by orbital

lipodystrophy, but cranial bony orbitalmalformation may play a role (Pasqu-

ier et al. 2006; Sonmez et al. 2006).


359



Orbital varices

Orbital varices usually present in the

third decade of life with exophthal-

mos, but occasionally present with

enophthalmos (Haritoglou & Hints-

chich 2003). They are vascular

hamartomas consisting of a plexus of

low-pressure, low-flow, thin-walled

and distensible vessels (Islam et al.

2004). When the variceal vessels dis-

tend, the eye is pushed forward. The

varix may eventually cause atrophy of

orbital fat, which allows the globe to

sink back into the orbit when the ves-

sels are not distended (Haritoglou &

Hintschich 2003) (Fig. 3A).

Computed tomography demon-

strates the varix, associated orbital fat

atrophy and (in some cases) bony

defects in the orbital wall (Islam et al.2004) (Fig. 3B). Colour flow sonogra-

phy may be a useful tool in diagnos-

ing this condition (Lieb et al. 1990;

Wildenhain et al. 1991). Management

of orbital varices is usually conserva-

tive, but trial of radiological emboliza-

tion then elective surgical removal

may be performed in selected cases

(Cline & Rootman 1984; Takechi

et al. 1994).

Post-irradiationOrbital radiation in children, usually

for retinoblastoma or rhabdomyosar-

coma, is a well-known cause of en-

ophthalmos that develops later in life

(Jackson et al. 1996). Although orbital

bony changes are often present, it is

the atrophic changes to orbital fat

that are thought to be the cause of

the inward displacement of the globe

(Cline & Rootman 1984; Raney et al.

2000).

Repeated ocular pressure

Leber congenital amaurosis is an

inherited retinal degenerative disorder

that results in blindness at birth. Clin-

ical signs that point to this diagnosis

in an infant include pendular nystag-

mus and a tendency to rub the eyes

(oculo-digital sign of Franceschetti)

(Franceschetti 1947). Enophthalmos

may be present, which is believed to

be related to orbital fat atrophy sec-

ondary to constant rubbing of the eye

(Babel et al. 1989). Pseudoenophthal-

mos may also be present secondary to

a short axial length and hyperopia in

some cases.

TractionMetastases

Metastatic tumours represent 2.5–

3.7% of all orbital tumours and usu-

ally present with an infiltrative or

mass effect (Goldberg et al. 1990).Enophthalmos is seen in approximately

7–24% of cases, depending on the ser-

ies (Goldberg et al. 1990; Gunalp &

Gunduz 1995; Shields et al. 2001; Ben

Simon et al. 2006). While the breast,

lung and prostate are the most fre-

quent sources for orbital metastases

(Shields et al. 2001), enophthalmos is

seen almost exclusively with metastatic

scirrhous breast carcinoma (Cline &

Rootman 1984; Goldberg et al. 1990;

Shields et al. 2001) (Fig. 2A,B), with

only isolated reports of other primaries(lung and stomach) (Cline & Rootman

1984; Goldberg et al. 1990).

Clinical assessment can be almost

diagnostic. The patient may have a

history of proptosis followed by grad-

ual enophthalmos (Ben Simon et al.

2006), or other common symptoms

such as diplopia, pain, visual loss and

mass sensation (Shields et al. 2001).

Examination usually reveals decreased

ocular motility (with positive forced

duction) caused by invasion of the

extraocular muscles (Cline & Root-man 1984; Lagreze et al. 1997).

Tumour-induced desmoplasia, fibrosis

and muscle invasion are responsible

for the enophthalmos; however, fat

atrophy could also be contributory

(Lagreze et al. 1997).

Metastatic disease should be high

on the list of differential diagnoses in

a patient presenting with non-trau-

matic enophthalmos, especially when

restrictive features are present. Biopsy

usually confirms the diagnosis and is

recommended even in patients withknown history of systemic cancer,

prior to planning management. Prog-

nosis is usually poor and management

is essentially palliative but may

improve survival (Goldberg et al.

1990).

Restrictive musclesyndromesDuane retraction syndrome

Duane retraction syndrome (DRS) is

the most common of the retraction

syndromes. Enophthalmos, however,

is uncommon and is seen only in the

more severe cases. There is a sugges-

tion that DRS may progress with age

and that enophthalmos may be more

visible in adults with DRS type I

(Noonan & O’Connor 1995).

Congenital fibrosis of the extraocular

muscles

Congenital fibrosis of the extraocular

muscles (CFEOM) is a rare group of

disorders characterized by congenital,

non-progressive external ophthalmo-

plegia (Shivaram et al. 2001). These

disorders are most often bilateral and

familial and have been subclassified

into three subtypes on the basis of

phenotype, which correlates with spe-

cific chromosomal defects (CFEOM1

12cen; CFEOM2, 11q13; CFEOM3,

16q24.2–q24.3) (Engle et al. 1995;

Wang et al. 1998; Traboulsi 2004;

Aubourg et al. 2005). Very rarely, a

sporadic form of CFEOM may occur

that is characterized by unilateral

muscle fibrosis with enophthalmos,

ptosis and restricted motility (Laugh-

lin 1956; Hertle et al. 1992; Shivaram

et al. 2001) (Fig. 4A). This condition

has been categorized as a type 3

CFEOM, but we believe that this

(A)

(B)

Fig. 4. (A) Clinical photograph of a 49-

year-old female with enophthalmos of her

left eye that was present since birth. Clini-

cal examination revealed almost total

restriction of extraocular movements and

accompanying ptosis in the left eye. A diag-

nosis of congenital fibrosis of the extraocu-

lar muscles with ptosis and enophthalmos

was made. (B) CT scan of the same patientshowing thickened, fibrotic extraocular mus-

cles on the left side with retraction of the

globe into the orbit.


360



condition represents a unique disorder

because it possesses a typical pheno-

type in the absence of characteristic

genetic defects.

Imaging usually shows thickening

of the extraocular muscles and an

orbital mass (intra- or extraconal) isvisualized frequently (Hertle et al.

1992) (Fig. 4B). Tumours adjacent to

the involved orbit have also been

noted (destruction of associated orbi-

tal wall may be present), but the rela-

tionship with fibrosis is unclear

(Effron et al. 1985; Vijayalakshmi

et al. 2006). The cause of this condi-

tion is unknown, although prenatal

orbital trauma has been postulated as

a possible causative factor (Effron

et al. 1985). Treatment includes occlu-

sion for amblyopia and strabismussurgery to release the muscle restric-

tions in an attempt to align the eye in

the primary position (Shivaram et al.

2001). Restoring a normal appear-

ance, however, is usually not possible.

Acquired retraction syndrome

There are very few reported cases of

retraction syndrome that occurs later

in life. Khan (2005) reported a recur-

rent pterygium that was associated

with enophthalmos and positive

forced ductions. Similarly, Gittingeret al. (1986) reported a case of enoph-

thalmos that occurred following trau-

matic enophthalmos repair.

Post-inflammatoryrestrictionWegener’s granulomatosis

Orbital involvement is the most com-

mon ophthalmic manifestation of

Wegener’s granulomatosis (WG), but

enophthalmos is rarely noted (Talar-

Williams et al. 2005). Patients are

usually young and have systemic

symptoms of WG such as pulmonary,

renal or vasculitic involvement, and

usually present with subacute pain,

proptosis, diplopia or visual loss

(Sadiq et al. 2000; Pakrou et al. 2006).

Magnetic resonance imaging (MRI) is

useful in assessing orbital involvement

but biopsy is needed for diagnosis

(Muhle et al. 1997; Perry et al. 1997;

Sadiq et al. 2000). Raised serum

circulating anti-neutrophil cytoplasmic

antibody (cANCA) should increase

suspicion of orbital and systemic

WG: it is present in 52% of cases

(Woo et al. 2001). Fibrosis and en-

ophthalmos may develop during treat-

ment of active orbital disease. This is

a post-inflammatory socket contrac-

ture that may lead to chronic orbital

pain, restrictive ophthalmopathy and

ischaemic optic neuropathy (Talar-Williams et al. 2005). None of these

are responsive to systemic immuno-

suppression; thus the enophthalmos

seen in WG may be associated with

significant visual and ocular morbidity

(Talar-Williams et al. 2005).

Other causes of post-inflammatory

enophthalmos are very rare, with only

isolated cases having been reported;

these conditions are summarized in

Table 1.

PseudoenophthalmosBefore proceeding to investigations

and management, it is very important

to rule out a pseudoenophthalmos.

There are a number of conditions that

frequently present as enophthalmos

such as contralateral exophthalmos,

ptosis and microphthalmos. Meticu-

lous clinical examination, exophthal-

mometry and ocular axial length

measurement can usually provide the

correct diagnosis.

The most common form of pseud-

oenophthalmos is probably facialasymmetry (Cline & Rootman 1984;

Burroughs et al. 2003). Most of these

patients are referred with the diagnosis

of contralateral proptosis, and the

examination usually reveals facial

asymmetry without specific bony orbi-

tal abnormalities (Cline & Rootman

1984). Using standardized techniques

of measuring enophthalmos, such as

Hertel’s exophthalmometer, may give

inaccurate readings in these patients.

Horner’s syndrome can also cause

pseudoenophthalmos secondary to theptosis. While traditional teaching

holds that enophthalmos is a feature

of Horner’s syndrome, it has been

shown that sympathetic paralysis leads

to enophthalmos in other animals but

not in humans (Daroff 2005).

Approach to a patientwith enophthalmosA thorough history in a patient with

enophthalmos includes the time-frame

of onset, current and past ocular

symptoms, past medical history and a

review of systems. For instance, initial

proptosis followed by progressive en-

ophthalmos may indicate a fibrosing

mass such as scirrhous carcinoma.

There are a number of important

features to observe when examining a

patient with enophthalmos. Measuring

the exophthalmos is obviously animportant step and, as discussed ear-

lier, this can be performed in a num-

ber of ways. As part of this step, the

clinician should rule out pseudoen-

ophthalmos by measuring the axial

length of the globe. Vertical position

should also be assessed, perhaps with

the Naugle’s orbitometer. A detailed

ophthalmic examination should be

undertaken, which may reveal clues to

the underlying condition (e.g. Lisch

nodules). Further examination should

look for functional manifestations of enophthalmos, as discussed previ-

ously.

Testing ocular motility is important

because it is rarely affected by struc-

tural changes (except trauma) or fat

atrophy, but is frequently affected by

tractional processes (Cline & Root-

man 1984).

A complete systemic examination

should also be performed. Sometimes

the patient’s appearance may provide

a clue to the diagnosis, as in Cocka-

yne’s syndrome. In other cases, asearch for primary carcinomas (e.g.

breast), peripheral stigmata of sys-

temic illness and periorbital signs of

disease (e.g. lid neurofibromatosis)

will be pertinent in making an accu-

rate diagnosis. Old photographs are a

very useful tool for determining the

rate of progression of enophthalmos

or diagnosing unrecognized congenital

asymmetry.

Investigations

The investigations for a patient with

enophthalmos should be tailored

according to the history and clinical

assessment. Blood tests may play a

role in narrowing the list of causes,

but are rarely diagnostic. Some use-

ful tests may be tumour markers,

cANCA (raised in WG) (Woo et al.

2001) and other inflammatory mark-

ers such as rheumatoid factor and

anti-nuclear antibodies (ANA) that

are associated with some causes of

lipodystrophy (Garg 2004).

CT and MRI are the most useful

radiological investigations for enoph-

thalmos. These provide a detailed


361



view of the orbital anatomy and delin-

eate any structural abnormalities.

Imaging also allows an objective

assessment of the enophthalmos in

comparison to the bony orbit and also

in relation to the contralateral eye.

Soft tissue views can illustrate fatatrophy, fibrotic changes and the nat-

ure and extent of any orbital lesions.

Tissue diagnosis is useful in a num-

ber of settings and is used most fre-

quently in diagnosing orbital lesions

such as metastases. Furthermore,

biopsy can evaluate lipodystrophies or

subcutaneous atrophy, as in sclero-

derma and PRS (Nasr et al. 1997;

Burroughs et al. 2003).

ManagementA review of the management of non-

traumatic enophthalmos is beyond the

scope of discussion of this article, but

a few points will be discussed briefly.

As touched on earlier, many cases

are treated non-surgically. Deciding

whether to operate (or when) is per-

haps the most important part of man-

agement. If surgical treatment is

possible, then surgery should be con-

sidered when there is either a func-

tional deficit or a significant cosmetic

effect (Rubin & Rumelt 1999).The type of repair will depend on

the underlying pathophysiology of the

enophthalmos. Structural changes and

fat atrophy can usually be managed

with orbital implants (Pearl 1992;

Thomas et al. 2003). This can reduce

exposure keratopathy caused by

lagophthalmos, as well as vertical

diplopia secondary to the relative dis-

placement of the globe.

Enophthalmos secondary to trac-

tion may require a more complex

approach including removing the

cause of the traction (e.g. releasing

tight muscles), restoration of extraoc-

ular motility and consideration of

adjuvant chemotherapy or radiother-

apy in cases of metastatic disease

(Char et al. 1997; Koshy & Evans

1998).

It has also been emphasized in a

number of papers that clinically evi-

dent enophthalmos has a significant

psychological effect on patients, and

corrective surgery should therefore be

considered to be reconstructive rather

than simply cosmetic (Cline &

Rootman 1984; Enquist & Arak 1994;

Rubin & Rumelt 1999).

ConclusionNon-traumatic enophthalmos is an

uncommon condition with a variety of

presentations and requires a careful

examination for accurate diagnosis.

With an understanding of the manypossible causes, one can perform an

effective clinical examination in order

to direct further investigations and

make the correct diagnosis. This not

only directs the management of en-

ophthalmos, but ) more importantly

) may also reveal a potentially life-

threatening systemic illness, which can

then be addressed.

Conflicts of interestThe authors are aware of no conflicts

of interest, of any nature, pertaining

to this manuscript.

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Received on February 24th, 2007.

Accepted on November 8th, 2007.

Correspondence:

Dr Paul Athanasiov

Department of Ophthalmology and

Visual Sciences

Royal Adelaide Hospital

North Terrace

Adelaide, SA

Australia 5000

Tel: + 61 88222 2729

Fax: + 61 88222 2741

Email: [email protected]


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