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Home , Blepharochalasis, Meibomian gland

666 British3rournal ofOphthalmology 1994; 78: 666-670

ORIGINAL ARTICLES - Clinical science Br J Ophthalmol: first published as 10.1136/bjo.78.9.666 on 1 September 1994. Downloaded from

The lax syndrome

Willem A van den Bosch, Hans G Lemij

Abstract original description, the affection of all patients The floppy eyelid syndrome (FES) was first was none the less diagnosed as FES. Its cause, described in middle aged, obese men. In later however, remained obscure. descriptions, age and sex were not specifically In this paper, four patients are described who mentioned. Associations of FES with various had several signs and symptoms similar to those other syndromes have been described. The described in FES. All of them had an upper authors question whether all these cases repre- eyelid laxity with varied, albeit clearcut, causes. sent the same, single, syndrome. They suggest We shall question whether the term FES applies, that a clinical picture similar to FES may occur or whether a more general term should be in lax upper of any cause. Four such adopted. cases are reported here. The authors therefore coin the more general term 'lax eyelid syn- drome'. They suggest using the term 'floppy Case reports eyelid syndrome' uniquely for patients with the classic signs and symptoms. CASE 1 (BrJ Ophthalmol 1994; 78: 666-670) An 86-year-old woman (height 160 cm, weight 51 kg) had presented elsewhere with a history of chronic discharge and irritation ofthe left eye for Culbertson and Ostler first described the floppy more'than a year. The symptoms showed no eyelid syndrome (FES) in 1981.1 The syndrome diurnal variation. She did not report sleeping was characterised by a 'floppy' - that is, easily with the affected side on the pillow, nor had she evertible, upper eyelid, and a papillary con- ever noticed upper eyelid eversion during sleep. junctivitis of the upper palpebral . On examination, her left eye had shown purulent Patients often presented with a long history of discharge, a papillary , and a dif- irritation and discharge. The syndrome only fuse punctate . She had contracted a occurred in middle aged, obese men. Unnoticed moderate of the left lower eyelid. No http://bjo.bmj.com/ eversion of the upper eyelid during sleep was corneal vascularisation had been present. A thought to cause the papillary conjunctivitis. At culture from the conjunctiva had grown first, effective treatment consisted of shielding Staphylococcus aureus. She had been treated with the affected eye at night.' Later, surgical correc- several topical . Because of a high tion of upper and, in some cases, lower eyelid intraocular pressure, timolol eyedrops also had laxity by means of full thickness eyelid shorten- been prescribed. Despite several weeks of con- ing proved to be very effective.2" Interestingly, tinued treatment, her symptoms had not on September 27, 2021 by guest. Protected copyright. later papers described similar signs and symp- improved. She was then referred to our toms in young or non-obese patients, or in oculoplastic service, where we corrected her association with various disorders such as kera- lower eyelid ectropion by excising a 7 mm full toconus, sleep apnoea syndrome, Meibomian thickness block from the lateral part ofher eyelid dysfunction, and the blepharochalasis syn- margin. Thereafter, her symptoms diminished drome. '" Despite these departures from the markedly. After 2 years, however, the discharge

Eye Hospital Rotterdam, Rotterdam, The Netherlands W A van den Bosch H G Lemij Correspondence to: Willem van den Bosch, MD, Eye Hospital Rotterdam, Schiedamsevest 180, 3011 BH Rotterdam, The Netherlands. Fig IA Fig IB Accepted for publication Figure I (A) Case 1 atfirst presentation. The affected left eye is closed. (B) The upper eyelid is pulled laterally. Conjunctival 11 April 1994 changes and severe discharge are visible. The nasal is not visible, owing to laxity ofthe medial canthal tendon. The lax eyelid syndrome 667 Br J Ophthalmol: first published as 10.1136/bjo.78.9.666 on 1 September 1994. Downloaded from

Fzg 2A PFig 2B Figure 2 (A) Case 1, I month after excision ofafull thickness block from the left upper eyelid and shortening the posterior lamella ofthe left lower eyelid under a skin muscleflap. (B) When the upper eyelid is lifted, no conjunctival injection is visible.

and keratitis of the left eye recurred. This time, been treated with eyelid massage, povidone both the medial canthal tendon and the lateral iodine eyedrops, and eyedrops containing tri- canthal ligament were very lax. Surgical reinser- methoprim and polymyxin B for several months, tion ofboth was performed. This resulted clinic- without any improvement. When we examined ally in a better apposition of both upper and him, the lashes ofthe right upper eyelid showed a lower eyelid against the . Within a few . However, they did not touch the . weeks after the operation her signs and symp- In addition, his right upper and lower eyelid toms improved dramatically. Two years later, at proved to be very lax (Fig 3). age 90, she presented for the third time with There was no excessive laxity of the medial severe discharge and a diffuse punctate keratitis canthal tendon or ofthe lateral canthal ligament. (Fig 1). The eyelid laxity appeared to be due to the On examination, she showed a recurrence of combined effects of involutional changes of the the laxity ofhermedial canthal tendon and lateral tarsal plate and to an age-related canthal ligament. We treated her upper eyelid caused by atrophy of orbital fat. On eversion of laxity by removing an 11 mm full thickness block the upper eyelid, the tarsal plate had a normal from the temporal part of the lid. To treat her consistency. A moderate papillary conjunctivitis lower eyelid laxity, we excised a block of pos- ofthe tarsal conjunctiva was present. He also had terior lamella in the lateral canthal angle under a an ulcerative . There was a slight skin muscle flap. In the following weeks, the injection ofthe bulbar conjunctiva, together with discomfort, keratitis, and discharge disappeared. a diffuse punctate keratitis. The upper eyelid A slight left upper eyelid ptosis remained. She laxity was corrected by excision of a 15 mm full has been free of the symptoms for 6 months thickness block ofthe temporal part ofthe lid and http://bjo.bmj.com/ (Fig 2). the lower eyelid laxity was treated by excision ofa of the excised upper eyelid block 5 mm full thickness block in the lateral canthal showed severe, active, and chronic conjunctivitis angle. Two weeks after surgery, the patient was with bacterial colonies on the conjunctival sur- free ofthe symptoms. The punctate keratitis and face. The tarsal plate showed moderate to severe the blepharitis disappeared fully within a month.

lipomatous atrophy. In the Meibomian , Interestingly, after correction of the horizontal on September 27, 2021 by guest. Protected copyright. the mite Demodex brevis was present. laxity, the lash ptosis also disappeared. He has been free of symptoms now for 18 months (Fig 4). CASE 2 Histological sections of the excised upper An 84-year-old man (height 170 cm, weight eyelid block showed a moderate subconjunctival, 56 kg) was referred to our oculoplastic service chronic, inflammatory infiltration. In the tarsal because of a punctate epithelial keratitis of his plate a lipogranulomatous inflammation was right eye, attributed to an upper eyelid entro- present. Demodex brevis was demonstrated in the pion. He reported that he had been having discharge from the eye, and a gritty feeling, for more than 6 months. Symptoms were worse in the evening. He did not report eyelid eversion during the night. Elsewhere, his symptoms had been ascribed to a coexisting blepharitis. He had

Figure 3 (A) Case 2 before surgery. (B) When the eyelid is pulled laterally, the severe laxity ofthe right upper eyelid is visible. Fig3A Fig 3B 668 van den Bosch, Lemrij

Figure 4 Case 2, 2 months after excision ofafull Histological slides of the excised upper eyelid block showed a chronic thickness block from right subconjunctival follicu- Br J Ophthalmol: first published as 10.1136/bjo.78.9.666 on 1 September 1994. Downloaded from upper and lower eyelid. lar inflammation. In the tarsal plate, a severe lipomatous atrophy ofthe Meibomian glands was noted. There was a dense, chronic inflammation around the accessory lacrimal tissue.

CASE 4 A 66-year-old, frail woman (height 156 cm, Meibomian glands. The dermis showed severe weight 39 kg) had undergone a extrac- elastic degeneration. tion of the left eye elsewhere 18 months before she was referred to our hospital. A few days after the cataract extraction, she had developed a CASE 3 . About the same time, a ptosis of An 83-year-old, moderately obese woman the left upper eyelid had come about. Treatment (height 165 cm, weight 82 kg) was referred to our with topical antibiotics had cured the corneal oculoplastic service because of punctate epithe- ulcer, although stromal loss of the lower part of lial keratitis ofthe right eye, thought to be due to the cornea had remained. Since the cataract a facial palsy. The facial palsy had been present extraction, she had been suffering almost perma- since an operation of the mastoid bone at the age nently from a purulent conjunctivitis, for which of 38. She had never undergone any surgery to she had been treated unsuccessfully with various improve eyelid apposition or function. For topical antibiotics. Finally, she was referred for several weeks she had suffered from strong evaluation of the chronic conjunctivitis. She irritation, discharge, and tearing from her right complained of chronic discharge and irritation eye. Her complaints did not vary during the day. without diurnal variation. She had not experi- She had not experienced any nocturnal eyelid enced eyelid eversion during sleep. When we eversion. Treatment with lubricant eyedrops had examined her, her left upper eyelid showed a not been satisfactory. When we examined her, ptosis with a vertical eyelid fissure of 3 5 mm. the right lower eyelid showed an ectropion with The levator function was 12 mm and the upper keratinisation 6f the eyelid margin. Her right lid skin crease position was high, suggesting that upper eyelid was slightly ptotic. On forceful the ptosis was due to a disinsertion or thinning of squeezing, her upper eyelid fully covered the the levator aponeurosis (Fig 7). cornea, although the eyelids did not close com- Both upper and lower eyelids were very lax. pletely, because of the lower lid ectropion. The The temporal sclera was almost fully covered by conjunctiva of the affected eye was injected, and the eyelids, probably because of laxity of the the cornea showed punctate epithelial keratitis of lateral canthal ligament. The lower eyelid the lower and central part, as in exposure showed a medial ectropion. On eversion of the keratitis due to facial palsy. We repositioned her upper eyelid, the consistency of the tarsal plate lower eyelid through a lateral tarsal strip pro- was normal. The tarsal conjunctiva showed a http://bjo.bmj.com/ cedure. Postoperatively, a slight ectropion of the slight papillary conjunctivitis. A blepharitis eyelid margin persisted, but eyelid closure was coexisted on her upper and lower eyelids. Inspec- adequate. However, her signs and symptoms did tion of the cornea revealed a diffuse punctate not improve (Fig 5). On further investigation, epithelial keratitis, in addition to the stromal loss the upper eyelid appeared to be moderately lax, and vascular ingrowth of the lower part. The and the tarsal plate of the upper lid showed a bulbar conjunctiva was moderately injected. papillary conjunctivitis. Her blepharitis was treated successfully with on September 27, 2021 by guest. Protected copyright. The upper eyelid laxity was then treated by eyelid scrubbing and application of 1% fusidic excision of a 7 mm full thickness block. Unlike acid eye gel on the eyelids. Nevertheless, the typical cases, the wound touched the cornea keratitis remained during the following 3 postoperatively. Nevertheless, the symptoms months. We then corrected the laxity of upper disappeared within 2 weeks. The ptosis also and lower eyelid by reinserting the lateral canthal disappeared. Although the lower lid ectropion ligament to the orbital rim. At the same time we has recurred slightly, she has been free of symp- treated her ptosis through a monitored anterior toms for 8 months (Fig 6). levator reinsertion. On postoperative examina-

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Figure S (A) Case 3, 3 weeks after correction of right lower lid ectropion, before correction ofupper lid laxity. (B) Moderate laxity ofthe right upper eyelid is visible when the eyelid is pulled laterally. Fig 5A 14ig SB The lax eyelid syndrome 669

Figure 6 Case 3, 4 months related to recurrence of eyelid laxity. In the after excision ofafull thickness block ofthe right second case, involutionai changes of the tarsal Br J Ophthalmol: first published as 10.1136/bjo.78.9.666 on 1 September 1994. Downloaded from upper eyelid. Although the plate and atrophy oforbital fat accounted for the lower lid ectropion has upper eyelid laxity. In the third case, both the recurred slightly, no upper eyelid laxity and lower eyelid ectropion conjunctival injection is visible. could be attributed to the combined effects of involution and a paretic orbicularis muscle. Whiile lower eyelid repositioning failed to cure this patient, upper eyelid shortening did. This suggests a causative role ofupper eyelid laxity. In the last case, both the ptosis and the disinsertion of the lateral canthal ligament were probably caused by either traction on the eyelids during cataract extraction, or by excessive squeezing as a result of the induced by a corneal tion, the reinsertion of the lateral canthal liga- ulcer. ment had caused a much tighter apposition of Of interest is the alleged possible role of tear both upper and lower eyelid against the globe. film disorders in the pathogenesis of FES. One Although some overcorrection of the upper eye- paper reported on seven patients with both FES lid ptosis existed, the patient was pleased with and chronic conjunctivitis. Three of these the result. After the operation, the punctate patients showed evidence of both quantitative keratitis and conjunctival redness disappeared and qualitative tear film disorders.5 In another within 2 weeks. She has been free of symptoms series, all three patients with FES had an inade- without the use ofany eyedrops for 6 months. quate tear film.9 Another paper reported on the histology ofthe tarsal plate ofa patient with FES. Cystic degeneration and squamous metaplasia of Discussion the Meibomian glands were found, probably To date, FES has not been clearly defined in the causing qualitative tear film abnormalities.'0 literature. Culbertson and Ostler described it as In each of our four cases, a coexisting tear film 'an idiopathic disease occurring in middle-aged, disorder may have been present. Microscopy of obese men, characterised by an easily everted, the excised upper eyelid blocks revealed that the floppy upper eyelid and upper palpebral papil- Meibomian glands of patients 1 and 2 were lary conjunctivitis'.' However, later definitions infested with Demodex brevis. The tarsal plate of did not include the original criteria of age, sex, patients with the symptoms of FES is frequently and the altered consistency of the tarsal plate.5`0 infested by Demodex brevis.'2 This mite may Whatever its exact definition, the cause of the cause a tear film disorder through destruction of syndrome has remained unclear. Nevertheless, the Meibomian glands.'01' On microscopy, the associations between symptoms of FES and upper eyelid tarsal plate ofcase 3 showed a strong various other clinical syndromes have been lipomatous atrophy of the Meibomian glands. http://bjo.bmj.com/ described, such as the sleep apnoea syndrome, On clinical examination, cases 2 and 4 showed a , Meibomian gland dysfunction, blepharitis which might have been associated chronic conjunctivitis, and the blepharochalasis with Meibomian gland loss.'3 In conclusion, all syndrome.5"-lo Again, all these reports provide four patients probably had tear film disorders. different descriptions of FES. One could there- However, they were cured by improving the fore question whether all these cases of FES apposition force between the eyelids and the represent the same clinical entity. Therefore, any globe. This was achieved by upper and, if on September 27, 2021 by guest. Protected copyright. speculations on the possible causes of FES necessary, lower eyelid shortening in the first should be viewed with caution. three cases, and by reinserting the lateral canthal Contrary to most of the literature on FES, ligament in the last case. Similar results have there was a clearcut cause for the eyelid laxity in been reported in the literature.510 Since eyelid each ofour four presented cases. In our first case, shortening cannot be expected to improve tear the eyelid laxity was caused by involution of the film quality, tear film disorders can, at most, only medial canthal tendon and the lateral canthal be regarded as contributing factors in the patho- ligament. Recurrence of the symptoms was genesis ofFES.

Figure 7 (A) Case 4 at presentation. (B) Two months afterptosis correction and reinsertion ofthe lateral canthal ligament. Fig 7A P'i8 7B 670 van denBosch, Lemij

Taken together, we would argue that eyelid and ocular discharge related to upper eyelid laxity of any cause is the most important causa- laxity the term 'lax eyelid syndrome' (LES) tive factor of a clinical picture resembling FES. should be introduced. We hope that this distinc- Br J Ophthalmol: first published as 10.1136/bjo.78.9.666 on 1 September 1994. Downloaded from This picture consists ofa papillary conjunctivitis, tion will lead to an earlier recognition, and punctate epithelial keratitis, and ocular dis- subsequently, more adequate and timely treat- charge. We would speculate that a drop in the ment ofpatients suffering from lax eyelids ofany apposition force between the eyelids and eyeball cause. below a critical level probably might cause these signs and symptoms. Furthermore, any coexist- ing tear film abnormalities might luxate or aggra- 1 Culbertson WW, Ostler HB. The floppy eyelid syndrome. AmJ Ophthalmol 1981; 92: 568-75. vate the clinical picture. Such tear film 2 Dutton JJ. Surgical management of the floppy eyelid syn- abnormalities could not singularly account for drome. AmJ Ophthalmol 1985; 99: 557-60. 3 Moore MB, Harrington M, McCulley JP. Floppy eyelid the entire clinical picture, because that would not syndrome. Management including surgery. Ophthalmology explain the success ofupper eyelid shortening. In 1986; 93: 184-8. 4 Goldberg RA, Coden DJ, Hornblass A, Mitchell JP. Floppy our experience, many elderly patients have a eyelid syndrome associated with marked lower eyelid ectro- similar eyelid laxity, without any symptoms. pion. AmJ Ophthalmol 1989; 108: 610-1. 5 Schwartz LK, Gelender H, Forster R. Chronic conjunctivitis Therefore, other not yet identified factors may associated with 'floppy eyelids'. Arch Ophthalmol 1983; 101: play a role in the aetiology ofthis clinical picture. 1884-8. 6 Eiferman RA, Gossman MD, O'Neill K, Douglas CH. Floppy We contend that the middle aged, obese men eyelid syndrome in a child. Am J7 Ophthalmol 1990; 109: with a rubbery tarsal plate originally described 356-7. 7 Woog JJ. Obstructive sleep apnea and the floppy eyelid by Culbertson and Ostler comprise a typical and syndrome. AmJ Ophthalmol 1990; 110: 314-5. easily identifiable subgroup within the group of 8 Donnenfeld ED, Perry HD, Gibralter RP, Ingraham HJ, Udell IJ. Keratoconus associated with floppy eyelid syn- patients with signs and symptoms related to drome. Ophthalmology 1991; 98: 1674-8. upper eyelid laxity. Unfortunately, such symp- 9 Goldberg R, SeiffS, McFarland J, Simons K, Shorr N. Floppy eyelid syndrome and . Am J Ophthalmol toms resulting from upper eyelid laxity are often 1986; 102: 376-81. thought to be limited to this subgroup. As a 10 Gonnering RS. Meibomian gland dysfunction in floppy eyelid syndrome. Ophthalmol Plast Reconstr Surg 1987; 3: 99-103. result, many patients will presumably not be 11 English FP, Nutting WB. Demodicosis of ophthalmic con- diagnosed and treated correctly. Therefore, we cern. AmJ Ophthalmol 1981; 91: 362-72. 12 van Nouhuys HM, van den Bosch WA, Mooy CM, Lemij HG. suggest reserving the term 'floppy eyelid syn- Demodex brevis infection found in floppy eyelid syndrome. drome' (FES) to the originally described sub- (in press). 13 Mathers WD, Shields WJ, Sachdev MS, Petroll WM, Jester group. For patients who have a chronic papillary JV. Meibomian gland dysfunction in chronic blepharitis. conjunctivitis, punctate epithelial keratitis, Cornea 1991; 10: 277-85. http://bjo.bmj.com/ on September 27, 2021 by guest. Protected copyright.