Non-traumatic enophthalmos: a review

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 defined 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 first 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 fibrosis – orbital metastases – pseudoenophthalmos – 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

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 )

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have been added to the literature. In this review, we discuss the clinical features, pathophysiology and causes of non-traumatic enophthalmos. Definition

Enophthalmos is best defined 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 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 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 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 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 Hertel’s exophthalmometry. A single patient with oscillopsia secondary to enophthalmos has also been reported (Zambarakji & 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 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

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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 opacification of the left maxillary sinus with thinning and bowing of inferior orbital floor 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 opacification (metastatic disease with fibrosis) 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 floor 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 [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 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 significant, 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 fibrosis 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 fibrosis 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 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 has been postulated that the drooping globe pulls down on Whitnall’s ligament leading to upper lid retraction (Anderson & Dixon 1979). Hypoglobus 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).

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

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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, congenital fibrosis of extraocular muscles. *Isolated case reports, not discussed further in the text.

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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 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 diagnostic and shows partial or total opacification of the maxillary sinus with reduction in sinus volume, downward bowing of the orbital floor and concomitant increase in orbital volume (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 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 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 hypothesized that the bony changes in neurofibromatosis 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 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).

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(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 right posterior orbit with absence of the greater wing of sphenoid (arrow) and part of the medial orbital wall (arrowhead).

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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 neurofibroma 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 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 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 Lockwood’s 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 five subsets of localized scleroderma classified 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 superficial 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 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 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 lipofilling and submalar silicone implants can produce good cosmesis and 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 classically reveal dwarfism 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).

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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-flow, 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 flow 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.

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Traction Metastases

Metastatic tumours represent 2.5– 3.7% of all orbital tumours and usually present with an infiltrative or mass effect (Goldberg et al. 1990). Enophthalmos is seen in approximately 7–24% 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, 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-traumatic enophthalmos, especially when restrictive features are present. Biopsy usually confirms 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 & 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 ophthalmoplegia (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 specific 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 (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

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(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 fibrosis of the extraocular muscles with ptosis and enophthalmos was made. (B) CT scan of the same patient showing thickened, fibrotic 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 fibrosis 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-inflammatory restriction Wegener’s granulomatosis

Orbital involvement is the most common ophthalmic manifestation of Wegener’s 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-inflammatory 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 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. 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 specific bony orbital 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 the ptosis. 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 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 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 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 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 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 patient’s appearance may provide a clue to the diagnosis, as in Cockayne’s 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 neurofibromatosis) 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 inflammatory 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

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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, fibrotic 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

Conflicts 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 briefly. 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 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 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 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).

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Conclusion

The 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]