Tight orbit syndrome - Wiley Online Library

Acta Ophthalmologica 2010

Tight orbit syndrome: a previously unrecognized cause of open-angle glaucoma Graham A. Lee,1,2,3 Robert Ritch,4,5 Steve Y.-W. Liang,3 Jeffrey M. Liebmann,6 Philip Dubois,7 Matthew Bastian-Jordan,7 Kate Lehmann7 and Prin Rojanapongpun8 1

City Eye Centre, Brisbane, Queensland, Australia University of Queensland, Brisbane, Queensland, Australia 3 Department of Ophthalmology, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia 4 Einhorn Clinical Research Centre, New York Eye and Ear Infirmary, New York City, USA 5 Department of Ophthalmology, New York Medical College, Valhalla, New York, USA 6 Department of Ophthalmology, New York University School of Medicine, New York City, USA 7 Queensland X-Ray, Brisbane, Queensland, Australia 8 Department of Ophthalmology, King Chulalongkorn Memorial Hospital, Bangkok, Thailand 2

ABSTRACT. Purpose: To describe a new syndrome of tight orbit and intractable glaucoma with a poor visual prognosis. Methods: A retrospective observational case series of six patients seen at two centres between 2001 and 2007 assessing intraocular pressure (IOP), best-corrected visual acuity and visual field. Results: Three men and three women, ranging in age at diagnosis from 14 to 53 years, demonstrated similar orbital features and progressive visual field loss despite intensive management with medication and laser and operative surgery. Highest IOPs ranged from 30 to 50 mmHg. Trabeculectomy and ⁄ or glaucoma drainage devices were attempted in five patients but all failed. One patient underwent orbital decompression with achievement of IOP control. Final IOP at last follow-up was variable; only two patients achieved IOP in the normal range, with the rest ranging from 25 to 40 mmHg. All patients had advanced visual field loss. Conclusion: Tight orbit syndrome presents a serious clinical challenge. Despite maximum medical therapy and surgical intervention IOP is difficult to control, resulting in progressive visual field loss. Key words: glaucoma – intraocular pressure – orbit – syndrome

Acta Ophthalmol. 2010: 88: 120–124 ª 2010 The Authors Journal compilation ª 2010 Acta Ophthalmol

doi: 10.1111/j.1755-3768.2009.01755.x

120

Introduction Elevated intraocular pressure (IOP) remains the most important known risk factor for the development of glaucoma. Although Goldmann applanation tonometry remains the gold standard for its measurement, numerous sources of error can affect the measurements made (Whitacre & Stein 1993). External pressure on the globe elevates IOP; among the factors that can lead to IOP elevation in this fashion are extraocular muscle restriction or co-contraction (Coleman & Trokel 1969), lid squeezing by the patient (Gandhi et al. 2001) and lid pressure against the globe by the examiner when measuring IOP. Changes in arterial and venous pressure can also affect IOP readings. Constricting the neck, such as with a tight necktie, may increase both venous pressure and IOP (Teng et al. 2003). A doubling of IOP has been documented with the use of a blood pressure cuff to 40 mmHg around a


patient’s neck (Bain & Maurice 1959). Breath-holding, a common finding in patients during routine applanation tonometry, may be associated with the Valsalva manoeuvre, which may affect IOP variably (Rafuse et al. 1994). Intraocular and extraocular muscle contraction affects IOP measurement. Extreme upgaze produces the greatest IOP elevation of all eccentric eye positions (mean 6.8 mmHg) (Saunders et al. 1981). Orbital congestion, such as occurs in hyperthyroidism, can produce elevated IOP and glaucoma. Glaucoma can occur in infiltrative ophthalmopathy by several mechanisms. The retrobulbar inflammatory process with enlargement of the extraocular muscles can compromise the orbital venous system and raise episcleral venous pressure. Contraction of the extraocular muscles against intraorbital adhesions may cause marked variations in pressure in different positions of gaze (Piltz-Seymour & Stone 1996). The other cause is restriction of the orbital volume caused by tightness of the orbital septum and limitation by the bony orbit relative to the orbital contents. Orbital decompression involves disrupting the floor and ⁄ or the medial and lateral walls of the bony orbit, allowing the fat to protrude from the orbit.

A literature review did not provide any references to what we believe to be a previously undescribed entity, which we have termed the ‘tight orbit syndrome’. We describe six patients with similar characteristics with relentlessly progressive glaucomatous damage who presented significant management challenges.

Materials and Methods We obtained institutional review board approval to review patient records without informed consent. The six patients were examined and managed between 2001 and 2007 at the City Eye Centre and Royal Brisbane Hospital, Brisbane, Australia and the New York Eye and Ear Infirmary, New York City, USA. Clinical follow-up ranged from 6 months to 21 years. A retrospective review of medical records was conducted. These patients had features of tight orbit syndrome – characterized by the eyelids pressing firmly against the globe, limiting globe exposure during tonometry, gonioscopy and surgery – and a high-pressure glaucoma that is extremely difficult to manage. Three patients (Table 1; patients 1, 2, 4) were imaged with a computed tomography (CT) scan in an attempt to quantify relative globe to orbital vol-

umes. CT examination was performed using a Siemens Sensation 16-slice scanner (Seimens AG, Munich, Germany) covering an area from the maxillary to frontal sinuses. The scanning parameters were 100 mAs and 120 kVp in 0.75 mm helical slices reconstructed into 3 mm slices using a bone algorithm. Data were analysed on the Siemens stand-alone work station using the inbuilt volume analysis program. Each axial slice was outlined using the software drawing tool, which generated a pixel count for the anatomical area of interest in each slice. The pixels were converted to voxels and these were summed to generate a total volume for the bony orbit. The anterior margin used was a line connecting the anterior edge of the zygomaticofrontal process to the nasomaxillary suture medially and the posterior boundary was the foramen for the optic nerve. Orbital volume was estimated using methodology based on previously described techniques (Regensburg et al. 2008). This was performed for both orbits by two independent and blinded practitioners in order to examine reproducibility.

Results Three men and three women, ranging in age at diagnosis from 14 to 53 years (Table 1), demonstrated

Table 1. Patient characteristics.

Age Highest Highest Medical Race at Dx IOP (R) IOP (L) treatment

Laser

1M

A

23

40

38

3 drops plus SLT OU acetazolamide

2M

A

53

50

45

3M

W

19

50

50

4F

W

36

32

50

5F

W

49

35

46

2 drops plus Cyclodiode OU acetazolamide 2 drops plus Cyclodiode OU · 3 acetazolamide 3 drops plus ALT OU acetazolamide Cyclodiode OD Cyclodiode OS · 2 3 drops plus Cyclodiode OD · 3 acetazolamide Cyclodiode OS · 3

6 F* W

14

40

30

3 drops

Cyclodiode OD

Surgery

Final Final IOP IOP VA (R) (L) (R)

Trab OU · 2 26 with needlings Canthotomy OU Ahmed valve OU Phaco OU 28 ECCE OU Trab OS Trab OU with needlings Phaco OU Trab OU with needlings ECCE OU Trab OU

VA (L)

MD (R)

MD (L)

6 ⁄ 18

)23.28 )25.50

35

6⁄9

35

6 ⁄ 30

41

30

6 ⁄ 20

6 ⁄ 10

N⁄A

N⁄A

19

13

HM

NPL

N⁄A

N⁄A

25

28

6⁄9

6 ⁄ 21

)14.14 )9.59

40

20

6 ⁄ 7.5 )31.02 )13.42

N⁄A N⁄A

N⁄A

N⁄A

Dx, diagnosis; M, male; F, female; A, Asian; W, White; VA, best-corrected visual acuity; MD, mean deviation; HM, hand movements; NPL, no perception of light; N ⁄ A, not able to measure; SLT, selective laser trabeculoplasty; ALT, argon laser trabeculoplasty; Phaco, phacoemulsification; ECCE, extracapsular cataract extraction; Trab, trabeculectomy; IOP, intraocular pressure; OS, left eye; OD, right eye; OU, both eyes . * Intellectually disabled, Trisomy 13 (Patau’s Syndrome); cup : disc ratio 0.95 (R) 0.95 (L) on examination under anaesthesia.

121


similar orbital features (Figs 1–4) and progressive visual field loss and optic nerve head cupping despite intensive management. The patients’ highest recorded IOPs ranged from 30 to 50 mmHg. The results from the orbital volume calculations were not found to be conclusive – the measured parameters were within the accepted normal sex- and age-matched ranges. Maximally tolerated medical therapy consisted of up to four topical medications and oral acetazolamide. Laser intervention included argon and selective laser trabeculoplasty and transscleral diode cyclophotocoagulation, with some eyes undergoing up to three treatments. Trabeculectomy was attempted in four patients, but all failed. A glaucoma drainage device was attempted in one patient because

(A)

(B)

Fig. 4. (A) Patient 4 in maximum downgaze and examiner lifting upper eyelid. (B) Temporal view with upper lid retraction by the examiner demonstrating extremely narrow palpebral fissure.

of very limited surgical access to the fornices. Orbital decompression was undertaken in one eye of one patient with normalization of the IOP. Final IOP at last follow-up was variable, with only two patients achieving IOP

in the normal range and the rest ranging from 25 to 41 mmHg. Bestcorrected visual acuity was also variable, although all patients had advanced visual field loss.

Selected Case Reports Patient 1

(A)

(B)

Fig. 1. (A) Patient 1’s tight eyelids and narrow palpebral fissures. (B) Temporal views of patient 1.

(A)

(B)

Fig. 2. (A) Patient 2’s tight eyelids and narrow palpebral fissures. (B) Temporal views of patient 2.

(A)

(B)

Fig. 3. (A) Patient 3 in maximum downgaze and examiner lifting upper eyelid, with very restricted exposure of superior conjunctiva. (B) Temporal view during Goldmann applanation tonometry demonstrating difficulty with intraocular pressure measurement.

122

A 32-year-old Chinese man diagnosed as having juvenile primary open-angle glaucoma was referred with IOPs around 35 mmHg on maximally tolerated medical therapy. Selective laser trabeculoplasty was performed in each eye with only transient lowering of the IOP. It was extremely difficult to perform Goldmann applanation tonometry because of the patient’s extremely tight lids and narrow lid fissures (Fig. 1A, B). The lids could not be lifted off the globes by pulling on the eyelashes or by inserting a speculum. In order to obtain applanation tonometry without the patient’s lids squeezing on the tonometer, a tonometer tip for measuring IOP on rhesus monkeys (Kaufman & Davis 1980) was required (supplied courtesy of Paul Kaufman MD). Measurement of IOP with this tip and using a speculum gave an IOP measurement approximately 8 mmHg lower than that obtained without this method. It was felt that the patient’s tight lids might actually be contributing to his elevated IOP, and cathotomies were performed with lowering of the IOP to around 25 mmHg. Despite this procedure, the lids remained tight against the globe and visual field loss continued to progress. Trabeculectomies with adjunctive mitomycin-C were performed in both eyes (OU); these failed, despite multiple attempts at transconjunctival needle revision.


A paediatric Ahmed implant was placed in the inferotemporal quadrants of both eyes with only moderate further lowering of IOP. Orbital floor decompressions were undertaken in his left eye then right eye, with a reduction of IOP to 12 mmHg OU. Patient 2

A 22-year-old White man (Fig. 2A, B) with poorly controlled aphakic glaucoma had had a cerebral haemorrhage at birth with a subsequent left lower nasal homonymous quadrantanopia, bilateral congenital cataract extraction, a five-muscle strabismus operation, two vitrectomies in the right eye (OD) for retinal detachment and redetachment, three diode cyclophotocoagulations OD and a trabeculectomy in the left eye (OS). His presenting best-corrected visual acuities were 6 ⁄ 12 OU, with IOPs of up to 50 mmHg OU and cup : disc ratios of 0.3 OD and 0.5 OS. Morphologically, he had extremely deep-set eyes, tightly applied eyelids and narrowed palpebral apertures. His medication included travoprost qhs, betaxolol bid and acetazolamide 250 mg bid. Although a glaucoma shunt was considered for the left eye, the risk of failure was considered too high to proceed. After two cyclophotocoagulations (40 · 1500 mW · 2000 milliseconds per treatment) OS, IOP was reduced to 25 mmHg. However, 15 months later the IOPs rose to 35 mmHg OD and 40 mmHg OS. Another cyclophotocoagulation OS (30 · 2000 mW · 2000 mseconds) was performed. At last follow-up, bestcorrected visual acuities were 6 ⁄ 24 OD and 6 ⁄ 10 OS. IOPs were 41 mmHg OD and 30 mmHg OS and cup : disc ratios were 0.8 OD and 0.6 OS on dorzolamide ⁄ timolol combination bid, travoprost qhs and acetazolamide 250 mg bid.

with OS. Gonioscopy demonstrated open angles but with intermittent peripheral anterior synechiae from the trabeculoplasty. His deeply set eyes and narrowed palpebral apertures resulted in great difficulty with gonioscopy and applanation. Cup : disc ratio was 0.9 OD and 0.8 OS. Mean deviation on Humphrey field analysis was )24.57 OD and )13.42 OS. Bilateral cataract removal and intraocular lens insertion was undertaken. Despite an initial IOP reduction, 6 months postoperatively IOPs were 42 mmHg OD and 35 mmHg OS. Cyclophotocoagulation was limited by the tightness of his orbits, with poor access of the probe to the superior limbus. Only 20 applications OD at 2000 mW for 2000 milliseconds could be administered to the inferior half of the limbus and 16 applications OS. At final follow-up, IOPs were 28 mmHg OD and 35 mmHg OS on maximum medical treatment. Patient 4

A 36-year-old White woman (Fig. 4A, B) with bilateral juvenile onset openangle glaucoma had a history of bilateral argon laser trabeculoplasties and trabeculectomies. Over the subsequent 20 years, her IOPs fluctuated in the 20– 35 mmHg range, requiring two repeat trabeculectomies and three transconjunctival needling procedures OD and one repeat trabeculectomy with two needling procedures OS. Despite initial IOP lowering, the trabeculectomies failed and bilateral cyclophotocoagulation (25 · 1500 mW · 1500 milliseconds each eye) was undertaken. A second cyclophotocoagulation OS was performed 10 months later. At final follow-up, IOPs were 19 mmHg OD and 13 mmHg OS on brinzolamide bid and latanoprost qhs. Visual acuity was hand movements OD and no perception of light OS.

Patient 3

Discussion

A 61-year-old Vietnamese man (Fig. 3A, B) had IOPs of 25 mmHg OD and 32 mmHg OS on brinzolamide bid, bimatoprost qhs and oral acetazolamide 250 mg qid. He had previously undergone argon laser trabeculoplasty OD. Slit-lamp examination showed slightly shallower anterior chamber depth OD compared

We have described a condition termed tight orbit syndrome, characterized by the eyelids pressing firmly against the globe, limiting globe exposure during tonometry, gonioscopy and surgery, and a high-pressure glaucoma that is extremely difficult to manage. Secondary causes of globe tightness, including thyroid ophthalmopathy because

of increased orbital soft tissue volume, orbital varix and arterio-venous fistula, have been well reported and may have analogous pathophysiology to tight orbit syndrome (Bigger 1975; Madgula & Lukaris 2006). A simulation of external pressure on the globes not from the eyelids but from tight swimming goggles has been described (Ma et al. 2007). Tightness of the eyelids has also been described as altering corneal topography (Collins et al. 2006). Patients with tight orbit syndrome have narrow palpebral apertures, and IOP measurement using the Goldman applanation tonometer is difficult. If applanation tonometry is not possible, then Tonopen, rebound tonometry or pneumotonometry may be required. Patients have bilaterally high IOP and a poor response to medical, laser and surgical treatment. Surgical options such as trabeculectomy and glaucoma drainage devices are challenging because of poor exposure. Limbus-based conjunctival flaps are not recommended, because the conjunctival incision cannot be placed posteriorly enough behind the scleral flap. The blebs tend to be low and fail early. Poor exposure of the superior fornix severely limits the ability to perform needling and 5-fluorouracil administration. A further management option is diode laser cyclophotocoagulation or endocyclophotocoagulation. The exposure of the superior limbus is a major challenge. The G-Probe can be slipped under the upper lid held by an unguarded wire speculum to perform unsighted burns, although this is not always possible because of the extreme eyelid tightness. A corneal traction suture can be used to inferiorly rotate the globe. The success of orbital decompression in one patient’s eye, resulting in reduction of IOP, is encouraging and suggests a potential management strategy analogous to compressive thyroid ophthalmopathy. DaneshMeyer et al. (2001) described 116 eyes that underwent orbital decompression for Graves’ ophthalmopathy with a decrease in IOP of 18.9% in primary position and 27.9% in upgaze (P < 0.001). Patients taking glaucoma medication or who had IOP > 21 mmHg demonstrated a significantly greater reduction in IOP postoperatively. Similar IOP reduction

123


post-orbital decompression has been noted by others (Dev et al. 1998; Crespı´ et al. 2007). In conclusion, tight orbit syndrome results in poor IOP control despite intensive medical and surgical intervention. As a result, there is rapid field deterioration compared with the more slowly progressive usual type of primary open-angle glaucoma. This is a major challenge to both the patient and the treating physician. Unfortunately current management options are limited, but more radical options such as orbital decompression may be of greater utility.

Acknowledgements Supported in part by the Derald H. Ruttenberg Foundation and the New York Glaucoma Research Institute, New York City, USA.

References Bain WES & Maurice DM (1959): Physiological variations in the intraocular pressure. Trans Ophthalmol Soc UK 79: 249–260. Bigger JF (1975): Glaucoma with elevated episcleral venous pressure. South Med J 68: 1444. Coleman DJ & Trokel SL (1969): Directrecorded intraocular pressure variations in

124

a human subject. Arch Ophthalmol 82: 637–640. Collins MJ, Buehren T, Trevor T, Statham M, Hansen J & Cavanagh DA (2006): Factors influencing lid pressure on the cornea. Eye Contact Lens 32: 168–173. Crespı´ J, Rodrı´ guez F & Buil JA (2007): Intraocular pressure after treatment for thyroid-associated ophthalmopathy. Arch Soc Esp Oftalmol 82: 691–696. Danesh-Meyer HV, Savino PJ, Deramo V, Sergott RC & Smith AF (2001): Intraocular pressure changes after treatment for Graves’ orbitopathy. Ophthalmology 108: 145–150. Dev S, Damji KF, DeBacker CM, Cox TA, Dutton JJ & Allingham RR (1998): Decrease in intraocular pressure after orbital decompression for thyroid orbitopathy. Can J Ophthalmol 33: 314–319. Gandhi P, Gurses-Ozden R, Liebmann J & Ritch R (2001): Attempted eyelid closure affects intraocular pressure measurement. Am J Ophthalmol 131: 417–420. Kaufman PL & Davis GE (1980): ‘‘Minified’’ Goldman applanation prism for tonometry in monkeys and humans. Arch Ophthalmol 98: 542. Ma KT, Chung WS, Seo KY, Seong GJ & Kim CY (2007): The effect of swimming goggles on intraocular pressure and blood flow within the optic nerve head. Yonsei Med J 48: 7807–7809. Madgula IM & Lukaris A (2006): Glaucoma secondary to orbital varix. Indian J Ophthalmol 54: 139–140. Piltz-Seymour JR & Stone RA (1996): Glaucoma associated with systemic disease. In:

Ritch R, Shields MB & Krupin T (eds). The glaucomas, 2nd edn. St Louis: Mosby, 1157–1176. Rafuse PE, Mills DW, Hooper PL, Chang TS & Wolf R (1994): Effects of Valsalva’s manoeuvre on intraocular pressure. Can J Ophthalmol 29: 73–76. Regensburg NI, Kok PH, Zonneveld FW, Baldeschi L, Saeed P, Wiersinga WM & Mourits MP (2008): A new and validated CT-based method for the calculation of orbital soft tissue volumes. Invest Ophthalmol Vis Sci 49: 1758–1762. Saunders RA, Helveston EM & Ellis FD (1981): Differential intraocular pressure in strabismus diagnosis. Ophthalmology 87: 59–70. Teng C, Gurses-Ozden R, Liebmann JM, Tello C & Ritch R (2003): The effect of a tight necktie on intraocular pressure. Br J Ophthalmol 87: 946–948. Whitacre MM & Stein R (1993): Sources of error with use of Goldmann-type tonometers. Surv Ophthalmol 38: 1–30.

Received on January 31st, 2009. Accepted on August 6th, 2009. Correspondence: Graham Lee Level 10, 135 Wickham Terrace Brisbane, QLD 4000 Australia Tel: + 61 7 3831 6888 Fax: + 61 7 3831 6883 Email: [email protected]