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Acta Ophthalmologica 2008

Review Article

Non-traumatic enophthalmos:
a review
Paul 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 dened as a relative, posterior displacement of a normal-sized globe in relation to the bony orbital margin. Non-traumatic enophthalmos has a wide variety of clinical presentations and may be the rst
manifestation of a number of local or systemic conditions. It may present with
cosmetic problems such as deep superior sulcus, pseudoptosis or eyelid retraction; 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 enophthalmos and include a brief description of the more important causes. An approach
to 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 brosis orbital metastases pseudoenophthalmos silent sinus syndrome

Acta Ophthalmol. 2008: 86: 356364


2008 The Authors
Journal compilation 2008 Acta Ophthalmol

doi: 10.1111/j.1755-3768.2007.01152.x

Introduction
Enophthalmos is a relative posterior
displacement of a normal-sized globe
in relation to the bony orbital margin. It may be unilateral or bilateral
but comes to the attention of the clinician 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 frequently the cause. In recent years,
several causes of this condition )
such as the silent sinus syndrome )

356

have been added to the literature. In


this review, we discuss the clinical
features, pathophysiology and causes
of non-traumatic enophthalmos.
Denition

Enophthalmos is best dened as a relative posterior displacement of a normal-sized eye in relation to the bony
orbital margin. Some authors have
used an absolute reading on exophthalmometry to indicate enophthalmos, such as less than 1012 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 exophthalmometry between the two eyes


(Wagener 1933; Kempster et al. 2005).
Koo et al. (2006), however, found that
enophthalmos becomes clinically obvious only when there is a difference of
34 mm or more between the two
eyes. It needs to be emphasized here
that the denition of enophthalmos is
not entirely objective and factors
other than ocular position alone )
such as orbital structure ) inuence
the perception of enophthalmos.
Clinical features

Enophthalmos is not always evident


clinically and may present with variable 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 present with both cosmetic and functional
issues. Cosmetic concerns include an
altered appearance because of a deepseated globe, asymmetric position of
the eyes, deep superior sulcus, pseudoptosis or eyelid retraction (Rubin &
Rumelt 1999; Yip et al. 2005). Functional 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 movements caused by a cicatrizing process
(Lagreze et al. 1997). In cases of severe
enophthalmos, the globe is drawn
away from the eyelids, and this leads to

Acta Ophthalmologica 2008

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 very
rare condition that is caused by the
transmission of intracranial pulsation
to the eye via a congenital or iatrogenic bony defect. The pulsation is
usually obvious clinically, but is especially noticeable when performing
applanation tonometry or Hertels
exophthalmometry. A single patient
with oscillopsia secondary to enophthalmos has also been reported (Zambarakji & Rose 2001).
Measurement

Hertels 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 signicantly when using this
instrument. In such cases, instruments
that rest on the superior and inferior
rims, such as the Naugles orbitometer, may be used (Naugle &
Couvillion 1992). In all cases of enophthalmos, vertical and horizontal
displacement of the globe should also
be measured; this is performed most
simply using a transparent scale to
compare the relative pupillary or limbal 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 cornea)clinoid distance has been measured 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

(A)

(A)

(B)

(B)

Fig. 1. (A) Clinical photograph of a 38-yearold female with silent sinus syndrome leading
to enophthalmos of the left eye. (B) The CT
scan shows opacication of the left maxillary
sinus with thinning and bowing of inferior
orbital oor resulting in expansion of left
orbital volume.

Fig. 2. (A) Clinical photograph of a 59-yearold female with enophthalmos of the left eye
secondary to metastatic scirrhous carcinoma
of the breast. (B) The CT scan demonstrates
irregular opacication (metastatic disease
with brosis) in the left posterior orbit
(arrow) with resulting retraction of the globe.

compared to the volume of its constituents, 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 oor secondary 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
[Pagets 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 agerelated process (senile enophthalmos)
or a manifestation of lipodystrophy
but can also be a result of associated
orbital pathology such as orbital varices. It is interesting that weight loss,
even when signicant, does not result
in orbital fat decrease and enophthalmos. In this context, a guinea-pig
study has suggested that intraorbital
adipocytes may compensate for a systemic 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
brosis of the extraocular muscles or
connective tissues; this is usually

associated with ocular motility problems. This mechanism is responsible


for the enophthalmos seen in some
orbital metastases and also for that
associated with brosis of the extraocular muscles.
The clinical features of enophthalmos are a direct result of the mechanism causing the enophthalmos. In
most cases, the retrusion of the globe
is associated with a deep superior sulcus and narrowed palpebral ssure
(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 has
been postulated that the drooping
globe pulls down on Whitnalls ligament leading to upper lid retraction
(Anderson & Dixon 1979). Hypoglobus may be a result of orbital volume
decit or orbital oor changes, and in
these cases pressure by the globe on
the inferior rectus may also lead
to lower lid retraction (Rubin &
Rumelt 1999).

Aetiology
The various non-traumatic causes of
enophthalmos are listed in Table 1.

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Acta Ophthalmologica 2008

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
Neurobromatosis
Orbital varix
Congenital
Other
Iatrogenic (Salem & Qahtani 2001;
Rose & Lund 2003; Wu et al. 2004)
Orbital varix
Bone disease
Pagets 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)*
Lebers congenital amaurosis
(Babel et al. 1989)*
Radiation
Post-radiotherapy
Unknown
Cockaynes 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
Duanes retraction syndrome
CFEOM
Iatrogenic (Gittinger et al. 1986;
Khan 2005)
Post-inammatory
Wegeners 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
Horners syndrome (Daroff 2005)
GWS, greater wing of sphenoid; LCA,
leber congenital amaurosis; CFEOM, congenital brosis of extraocular muscles.
*Isolated case reports, not discussed further
in the text.

358

The causes are listed according to the


proposed mechanism of causation,
where known. A brief discussion of
the more important causes follows.

Structural abnormality
Maxillary sinusitis and silent sinus
syndrome

Chronic maxillary sinusitis with


obstruction of the sinus ostium can, in
some cases, lead to centripetal collapse of the sinus walls (atelectasis)
with subsequent enophthalmos (Montgomery 1964). Often, no prior history
of sinusitis is present, and patients
present with spontaneous enophthalmos and hypoglobus (silent sinus syndrome) (Soparkar et al. 1994). This
condition is always unilateral and is
seen equally in males and females in
their third to fth 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 diagnostic and shows partial or total
opacication of the maxillary sinus
with reduction in sinus volume, downward bowing of the orbital oor and
concomitant increase in orbital volume (Fig. 1B). The orbital oor 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 pressure with subsequent sinus collapse
(simulating maxillary hypoplasia) (Buono 2004). It should be mentioned
here that many of the previously
reported cases of enophthalmos associated with maxillary sinus mucocele
probably share a similar pathophysiology (Cline & Rootman 1984).
Treatment is usually surgical and
involves restoration of normal sinus
ventilation with endoscopic external
antrostomy and repair of the orbital
oor (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
neurobromatosis I. It is hypothesized that the bony changes in neurobromatosis may be a result of the
interaction between the developing
orbit and the orbital neurobromas
(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 rarely
noted in patients with absence of the
GWS. Enophthalmos is a result of
expansion of the orbital cavity and
the pulsation is caused by the transmission of intracranial pulsations to
the eye. Alternatively, and more frequently, the temporal lobe can herniate through this defect to cause
pulsatile exophthalmos (Dabezies &
Walsh 1961; Savino et al. 1977; Jackson et al. 1993).

(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
ssure, deep superior sulcus and pseudoptosis
of the right eye (B) CT scan of the same
patient shows a lobulated mass in the right
posterior orbit with absence of the greater
wing of sphenoid (arrow) and part of the
medial orbital wall (arrowhead).

Acta Ophthalmologica 2008

Computerized tomography (CT)


will show partial or complete absence
of the greater wing of the sphenoid
bone with enlargement of the superior
orbital ssure and may also reveal the
presence of associated pathology such
as optic nerve glioma, plexiform neurobroma or orbital varices (Binet
et al. 1969; Mortada 1977; Jacquemin
et al. 2003).
A number of operations may result
in enophthalmos, including sinus surgery, base of skull reconstructions and
excessive orbital decompression surgery; a careful history should be correlated 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 disorder in which initial bone destruction


is followed by a disorganized reparative process causing distortion (Paget
1877). Nearly a third of cases involve
the skull (Griz et al. 2006). Enophthalmos is a rare manifestation of
Pagets 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 characteristic widening of the bone, thickening of its cortex, osteolytic areas and
osteosclerosis (Griz et al. 2006). The
progression of PDB can be followed
with a number of blood tests, including alkaline phosphatase levels (Griz
et al. 2006).

Fat atrophy
Senile changes

Facial lipoatrophy occurs with aging,


starting at the age of 20 and becoming
noticeable at the age of 30 in most people (Ascher et al. 2006). This atrophy
may form part of the process responsible 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 Lockwoods suspensory ligament
occurs with age, and this causes the

globe to sink thus pushing the orbital


fat pads forward. Relocating the herniated fat pad may correct the enophthalmos (Camirand et al. 1996).
Regardless of the pathogenesis, this is
a common cause of bilateral enophthalmos and comparison with old
photographs is useful in diagnosis.
Localized scleroderma and Parry)
Romberg syndrome

Scleroderma is a chronic autoimmune


disease that can be systemic or localized to the skin. There are ve subsets
of localized scleroderma classied by
the type and extent of cutaneous
involvement: plaque; generalized; bullous; 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 atrophy and enophthalmos. Parry)Romberg 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 generally has minimal involvement of the
supercial cutaneous layers (Aleem
et 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. Traditionally, scleroderma has cutaneous atrophy, 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; Jablonska & Blaszczyk 2005; Laxer &
Zulian 2006; Sommer et al. 2006).
Diagnosis of both PRS and scleroderma 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 enophthalmos 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 erythematosus (Ishiguro et al. 2002), dermatomyositis and Sjogrens 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 enophthalmos (Merchante et al. 2004;
Ascher et al. 2006). The medical management 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
lipolling and submalar silicone
implants can produce good cosmesis
and psychological benet in patients
with facial lipodystrophy (Koshy &
Evans 1998; Jones 2005; Mori et al.
2006).
Cockaynes dystrophy

This rare autosomal recessive disorder


was rst 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 classically reveal dwarsm and microcephaly as well as a variable number of
other problems such as retinal atrophy, cataracts and corneal opacity
(McElvanney et al. 1996; Ozdirim
et al. 1996). Enophthalmos is a wellrecognized feature of this syndrome
and is believed to be caused by orbital
lipodystrophy, but cranial bony orbital
malformation may play a role (Pasquier et al. 2006; Sonmez et al. 2006).

359

Acta Ophthalmologica 2008

Orbital varices

Orbital varices usually present in the


third decade of life with exophthalmos, but occasionally present with
enophthalmos (Haritoglou & Hintschich 2003). They are vascular
hamartomas consisting of a plexus of
low-pressure, low-ow, thin-walled
and distensible vessels (Islam et al.
2004). When the variceal vessels distend, 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 vessels are not distended (Haritoglou &
Hintschich 2003) (Fig. 3A).
Computed tomography demonstrates the varix, associated orbital fat
atrophy and (in some cases) bony
defects in the orbital wall (Islam et al.
2004) (Fig. 3B). Colour ow sonography may be a useful tool in diagnosing this condition (Lieb et al. 1990;
Wildenhain et al. 1991). Management
of orbital varices is usually conservative, but trial of radiological embolization then elective surgical removal
may be performed in selected cases
(Cline & Rootman 1984; Takechi
et al. 1994).
Post-irradiation

Orbital radiation in children, usually


for retinoblastoma or rhabdomyosarcoma, is a well-known cause of enophthalmos 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. Clinical signs that point to this diagnosis
in an infant include pendular nystagmus 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 secondary to constant rubbing of the eye
(Babel et al. 1989). Pseudoenophthalmos may also be present secondary to
a short axial length and hyperopia in
some cases.

360

Traction
Metastases

Metastatic tumours represent 2.5


3.7% of all orbital tumours and usually present with an inltrative or
mass effect (Goldberg et al. 1990).
Enophthalmos is seen in approximately
724% of cases, depending on the series (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 frequent 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 gradual 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 & Rootman 1984; Lagreze et al. 1997).
Tumour-induced desmoplasia, brosis
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-traumatic enophthalmos, especially when
restrictive features are present. Biopsy
usually conrms the diagnosis and is
recommended even in patients with
known history of systemic cancer,
prior to planning management. Prognosis is usually poor and management
is essentially palliative but may
improve survival (Goldberg et al.
1990).

Restrictive muscle
syndromes
Duane 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 suggestion that DRS may progress with age
and that enophthalmos may be more
visible in adults with DRS type I
(Noonan & OConnor 1995).
Congenital brosis of the extraocular
muscles

Congenital brosis of the extraocular


muscles (CFEOM) is a rare group of
disorders characterized by congenital,
non-progressive external ophthalmoplegia (Shivaram et al. 2001). These
disorders are most often bilateral and
familial and have been subclassied
into three subtypes on the basis of
phenotype, which correlates with specic chromosomal defects (CFEOM1
12cen; CFEOM2, 11q13; CFEOM3,
16q24.2q24.3) (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 brosis with enophthalmos,
ptosis and restricted motility (Laughlin 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 49year-old female with enophthalmos of her
left eye that was present since birth. Clinical examination revealed almost total
restriction of extraocular movements and
accompanying ptosis in the left eye. A diagnosis of congenital brosis of the extraocular muscles with ptosis and enophthalmos
was made. (B) CT scan of the same patient
showing thickened, brotic extraocular muscles on the left side with retraction of the
globe into the orbit.

Acta Ophthalmologica 2008

condition represents a unique disorder


because it possesses a typical phenotype in the absence of characteristic
genetic defects.
Imaging usually shows thickening
of the extraocular muscles and an
orbital mass (intra- or extraconal) is
visualized frequently (Hertle et al.
1992) (Fig. 4B). Tumours adjacent to
the involved orbit have also been
noted (destruction of associated orbital wall may be present), but the relationship with brosis is unclear
(Effron et al. 1985; Vijayalakshmi
et al. 2006). The cause of this condition is unknown, although prenatal
orbital trauma has been postulated as
a possible causative factor (Effron
et al. 1985). Treatment includes occlusion for amblyopia and strabismus
surgery to release the muscle restrictions in an attempt to align the eye in
the primary position (Shivaram et al.
2001). Restoring a normal appearance, 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 recurrent pterygium that was associated
with enophthalmos and positive
forced ductions. Similarly, Gittinger
et al. (1986) reported a case of enophthalmos that occurred following traumatic enophthalmos repair.

Post-inammatory
restriction
Wegeners granulomatosis

Orbital involvement is the most common ophthalmic manifestation of


Wegeners granulomatosis (WG), but
enophthalmos is rarely noted (TalarWilliams 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 enophthalmos may develop during treatment of active orbital disease. This is
a post-inammatory socket contracture that may lead to chronic orbital
pain, restrictive ophthalmopathy and
ischaemic optic neuropathy (TalarWilliams et al. 2005). None of these
are responsive to systemic immunosuppression; thus the enophthalmos
seen in WG may be associated with
signicant visual and ocular morbidity
(Talar-Williams et al. 2005).
Other causes of post-inammatory
enophthalmos are very rare, with only
isolated cases having been reported;
these conditions are summarized in
Table 1.
Pseudoenophthalmos

Before 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. Meticulous clinical examination, exophthalmometry and ocular axial length
measurement can usually provide the
correct diagnosis.
The most common form of pseudoenophthalmos is probably facial
asymmetry (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 specic bony orbital abnormalities (Cline & Rootman
1984). Using standardized techniques
of measuring enophthalmos, such as
Hertels exophthalmometer, may give
inaccurate readings in these patients.
Horners syndrome can also cause
pseudoenophthalmos secondary to the
ptosis. While traditional teaching
holds that enophthalmos is a feature
of Horners syndrome, it has been
shown that sympathetic paralysis leads
to enophthalmos in other animals but
not in humans (Daroff 2005).

Approach to a patient
with enophthalmos
A 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 enophthalmos may indicate a brosing


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 an
important step and, as discussed earlier, this can be performed in a number of ways. As part of this step, the
clinician should rule out pseudoenophthalmos by measuring the axial
length of the globe. Vertical position
should also be assessed, perhaps with
the Naugles 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 previously.
Testing ocular motility is important
because it is rarely affected by structural changes (except trauma) or fat
atrophy, but is frequently affected by
tractional processes (Cline & Rootman 1984).
A complete systemic examination
should also be performed. Sometimes
the patients appearance may provide
a clue to the diagnosis, as in Cockaynes syndrome. In other cases, a
search for primary carcinomas (e.g.
breast), peripheral stigmata of systemic illness and periorbital signs of
disease (e.g. lid neurobromatosis)
will be pertinent in making an accurate 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 useful tests may be tumour markers,
cANCA (raised in WG) (Woo et al.
2001) and other inammatory markers 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 enophthalmos. These provide a detailed

361

Acta Ophthalmologica 2008

view of the orbital anatomy and delineate 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 fat
atrophy, brotic changes and the nature and extent of any orbital lesions.
Tissue diagnosis is useful in a number of settings and is used most frequently in diagnosing orbital lesions
such as metastases. Furthermore,
biopsy can evaluate lipodystrophies or
subcutaneous atrophy, as in scleroderma and PRS (Nasr et al. 1997;
Burroughs et al. 2003).

Non-traumatic enophthalmos is an
uncommon condition with a variety of
presentations and requires a careful
examination for accurate diagnosis.
With an understanding of the many
possible 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 enophthalmos, but ) more importantly
) may also reveal a potentially lifethreatening systemic illness, which can
then be addressed.

Management

Conicts of interest

A review of the management of nontraumatic enophthalmos is beyond the


scope of discussion of this article, but
a few points will be discussed briey.
As touched on earlier, many cases
are treated non-surgically. Deciding
whether to operate (or when) is perhaps the most important part of management. If surgical treatment is
possible, then surgery should be considered when there is either a functional decit or a signicant 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 displacement of the globe.
Enophthalmos secondary to traction may require a more complex
approach including removing the
cause of the traction (e.g. releasing
tight muscles), restoration of extraocular motility and consideration of
adjuvant chemotherapy or radiotherapy in cases of metastatic disease
(Char et al. 1997; Koshy & Evans
1998).
It has also been emphasized in a
number of papers that clinically evident enophthalmos has a signicant
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).

362

Conclusion

The authors are aware of no conicts


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: eye@health.sa.gov.au