Volume 1: Ocular Surface Diseases

In this audio-visual lecture of 40 minutes, Dr Sameera Irfan explains the following:

1. What is Ocular Surface ? How it is affected by chronic conjunctivitis?
2. The Pathogenesis of Atopic and Vernal Conjunctivitis.
3. The Clinical symptoms, signs and GRADING the severity of VKC.
4. A MANAGEMENT PROTOCOL according to disease severity.

In patients with VKC, itchy eyes and frequent rubbing of eyelids is a common complaint. During an acute flare up of the disease, histamine is released from the mast cells that initiates the triple response: periocular itching (rubbing of eyes), vascular dilatation (redness) and oozing of fluid from engorged conjunctival vessels (lid swelling).  The rubbing of eyes further releases histamine that increases the lid swelling and redness. In addition, another harmful affect of vigorously rubbing the eyelids is an acute traumatic dis-insertion of the levator aponeurosis muscle from the tarsal plate, resulting in an acute onset of severe ptosis.

On the other hand, chronic eyelid rubbing results in a gradual stretching of the levator aponeurosis (dehiscence) from the tarsal plate, with a gradual drooping of both eyelids that worsens with time. Both types of ptosis have different clinical presentations; their accurate diagnosis is important as the surgical technique to correct them is different.  Both cases are discussed in detail in this case report.

Case 1: A 14 years old girl presented at the ophthalmic clinic with a sudden drooping of the right upper lid for one week; it occurred  following rubbing that eye vigorously (Fig.1). She gave a history of intermittent episodes of bilateral itchy, irritable eyes, with watering, redness and a thick, scanty whitish discharge since 8 years. She had been using a multitude of eyedrops including anti-histamines and mild steroids on and off. She had recurrent nasal allergies too but otherwise had good general health.

On examination, she had a BCVA of 6/6 (Snellen’s) either eye. There was a marked ptosis of the right upper lid with absent upper lid crease. Levator function (LF) was poor (5 mm) in that lid as demonstrated in Fig.2. The MRD1 was -3.5mm. Extra-ocular movements were full in all directions and a strong Bell’s phenomenon was noted; scleral show or lower lid laxity was absent. No fatiguability or lid twitching were demonstrable on continued up-gaze. There was a mild swelling of the lower lid with redness of palpebral conjunctivae in both eyes. Papillae were present on upper and lower tarsal conjunctivae bilaterally, with mild limbitis (less than 6 clock hours) and a few fine superficial punctate corneal erosions. Stringy mucus was noted in both the lower conjunctival fornices. Intra-ocular pressures were 22 mm Hg in both eyes. The lens and optic discs were healthy.

In view of the chronic recurrent keratoconjunctivitis, a diagnosis of VKC was made. The sudden onset of ptosis in the right lid following rubbing and with poor levator function was assumed to be due to an acute levator dis-insertion from the tarsus. The acute onset of ptosis was confirmed by seeing her old photograph, 2 months prior to the onset of this episode. She was prescribed Olopatadine eyedrops (double acting drugs) twice daily, Tacrolimus skin cream 0.03% to be applied in the lower conjunctival twice daily, lubricant eyedrops and icepacks to control the acute inflammation. After using this treatment for four weeks, ptosis was corrected surgically by levator reinsertion without any resection of the levator aponeurosis. The post-operative follow-up was performed after 1 week, 1 month, and 6 months after surgery.

Surgical Technique

Management of ptosis was planned as levator reinsertion via a skin approach, under local anaesthesia. As the upper lid crease was absent, it was marked at the same level as that in the opposite lid. After making the skin incision at the site of the marked lid-crease, the orbicularis muscle was split and tarsal plate was exposed. The levator aponeurosis was found to be dis-inserted from the tarsal plate with only a frill of tissue present on the surface of the tarsus; the remaining levator muscle was found retracted high up in the upper fornix as shown in Fig. 3. The retracted muscle was brought down with forceps, and reattached to the tarsal plate by 5/0 vicryl sutures. The lid was set at the same height as that in the opposite upper lid intra-operatively. No resection of either the levator aponeurosis or the muscle was performed. The upper lid crease was reformed with interrupted 6/0 vicryl sutures and tiny bites of the levator muscle were taken while suturing the skin. A lower-lid traction suture (Frost) was passed through the grey line with 4/0 silk and secured on the forehead with tape. A pressure dressing was applied for 24 hours to minimise post-operative bleeding and tissue swelling. The result on removing the dressing on the first post-operative day is shown in the Fig 4.

Case 2:

A 17 years old boy presented to the eye clinic complaining of bilateral drooping of upper lids and a reduced vision for the last 10 years. He gave a past history of recurrent episodes of itchy, red eyes since a very young age. The episodes were more during the hot season. He had used various topical medications over the years; the acute episodes had improved but had left him with a reduced vision and droopy lids.

On examination, (Fig.5), there was bilateral, moderate ptosis more in the right eye. The upper lid creases were very high and he was using both eye-brows to lift up the eyelids. The MRD1 was -2.5 mm in the right eye and -1.5 mm in the left eye, after holding the brows and preventing them from lifting the eyelids. The levator function was 8 mm in the right and 10 mm on the left. The ocular movements were full in all directions and the Bell’s phenomenon was strong. There was no fatiguability or lid twitching observed on continued up-gaze for 1 min. The corrected visual acuity was 6/36 in the right eye, due to gross astigmatism producing amblyopia. The visual acuity of the left eye improved to 6/9 with correction. The eyes were white with no discharge; both eyelids were thick due to scarring of upper and lower tarsal conjunctivae, more so of the right side (due to Triamnicolone injection given in that eyelid 7 years back). The right cornea had an old scar in the upper half (due to an old healed ulcer); both astigmatism and corneal scarring resulted in a poor visual acuity in that eye. There was a reduced Tear Film BUT (< 5 sec), with few punctate corneal staining spots and a Schirmer’s test result of 5 mm.  A grade 2 Meibomian gland Dysfunction was present with viscid meibomian secretion and frothing at the lid margins. The IOP (18 mm Hg), lens and optic disc assessment showed no abnormality. His old clinical record mentioned keratoconus being diagnosed four years back but no scan was available. A new orbscan was ordered to assess the present state of corneas.

For the moderately severe dry eyes, he was prescribed Autologous serum 20% eyedrops hourly during the day and a lubricant ointment at bed time. He was advised to apply Tacrolimus skin cream 0.03% inside the lower conjunctival fornix twice daily and tetracycline eye ointment massaged into the lid margins at night, to help restore the tear film. Though the IOP was recorded to be normal, but in view of the corneal thinning, IOP was lowered further by instilling topical beta blocker eyedrops twice daily. The conservative therapy was continued for 6 months, with regular 2 monthly follow-ups. When the Tear Film BUT improved to 15 sec and Schirmer’s reading (with the anesthetic drops) improved to 10 mm, then the ptosis surgery was planned.

Both eyelids were lifted, one after the other, at a month’s interval, with levator resection via a skin approach and the upper lid creases were also formed. The final post-operative result is showed in the Fig.6. Post-operatively, the dry eye therapy was continued for another 6 months. The patient was then referred to the cornea specialist for further management.

Discussion

Ptosis (drooping of the upper lid) can occur at any age, due to a myriad of clinical conditions ¹. A proper history and a good clinical assessment to elucidate the exact cause is mandatory for appropriate management of the ptosis, which can be either surgical or medical ². A relatively sudden onset of ptosis in any age group points toward trauma which can be an accidental injury, due to prolonged contact lens wear ³ or surgical trauma ⁴. According to Fiebel et al,⁵ ptosis occurred in 6% patients following cataract surgery. Similarly, Ocular myasthenia can produce ptosis at any age, as well as a demyelinating process or a third nerve paresis (traumatic, ischemic or an intracranial space occupying lesion).

In case 1, the vigorous rubbing of the eyelid resulted in an acute traumatic dis-insertion of the levator muscle. This caused a severe ptosis with poor LF, and an absent upper lid skin crease; the ptosis remained essentially the same in both up-gaze and the down-gaze. This differentiates an acute levator dis-insertion from the inelastic levator muscle typically seen in a congenital myogenic ptosis ⁶, in which a moderate-severe ptosis is present since birth; the taut muscle neither relaxes nor contracts fully and results in a marked lid-lag in down-gaze. Even though the patient gave a clear history of long standing ocular allergy and rubbing of eyes, a previous photograph of the face demonstrating an absence of ptosis, prior to the traumatic event, was helpful in deciding the recent onset of ptosis.

In levator dis-insertion, a history of trauma is always present. It can be  an acute, blunt injury to the eyelid or stretching of the superior rectus / levator complex by a traction suture applied during cataract surgery; the weight and stretching by the lid speculum contributes to the acute insult.

In Case 2, the chronic rubbing of eyelids over years resulted in a gradual stretching / dehiscence of the levator aponeurosis muscle that caused an asymmetrical ptosis in both upper lids. The same mechanism is responsible for involutional ptosis ^7,8 seen commonly in the elderly as a part of the normal ageing process; as the chronic trauma due to rubbing of eyelids continued, the dehiscence of levator and the ptosis worsened. Since the levator aponeurosis was still attached to the tarsal plate, the LF was good, in comparison to an acute dis-insertion that results in a poor LF. This is demonstrated diagrammatically in Fig.7.

Also in Case 2, long standing rubbing of eyes along with the repeated use of topical steroids led to corneal thinning, gross astigmatism and keratoconus. Luckily he did not develop steroid-induced glaucoma. Topical steroids are known to impair corneal healing and promote thinning.

It is important to recognise the specific clinical entity of Acute levator dis-insertion ⁹, as the recommended surgical procedure for a severe ptosis with poor levator function is a frontalis brow suspension. But in this case, simply reattaching the levator muscle back to the tarsus sufficed; there was no need to resect the muscle or the levator aponeurosis as both were healthy. Any amount of resection would result in an over correction of the lid position. This is in comparison to levator dehiscence that is seen in involutional ptosis as a part of the normal ageing process; the aponeurosis gradually gets stretched by blinking and the levator muscle develops involutional changes with increasing age. Hence in such cases, an advancement of the levator muscle along with resection of the aponeurosis/muscle is required, depending on the extent of involutional change and levator function.

Conclusion

Both these cases demonstrate the complications related to chronic VKC as well as due to an improper therapy. Patients and their care-takers need to be educated regarding the chronic nature of the disease and to avoid rubbing of eyelids as it releases histamine which aggravates the itching of eyes, the kerato-conjunctivitis, and ptosis.

The chronic inflammation of ocular surface,  dry eyes and added chemical toxicity of preservatives as well as topical steroids result in limbal stem cell deficiency that impairs corneal healing, promotes corneal thinning as well as scarring, keratoconus and a permanent visual impairment.

Rubbing the eyelids can result in ptosis due to different pathogenic mechanisms; a proper clinical assessment ensures correct surgical management.

References

1. Royal College of Physicians. Medical Master- class: Neurology, Ophthalmology, and Psychiatry. London, UK: Royal College of Physicians; 2008.
2. Finsterer J. Ptosis: causes, presentation, and management. Aesthetic Plast Surg. 2003;27(3):193-204.
3. Vanden Bosch WH, Lemij HG. Blepharoptosis induced by prolonged hard contact lens wear. Ophthal- mology. 1992;99:1759–1765.
4. Paris GL, Quichet MH. Disinsertion of the aponeurosis of the levator palpebrae after cataract extraction. Am J Ophthalmol. 1976;81:337–340.
5. Fiebel RM, Custer PL, Gordon MO. Post-cataract ptosis—a randomized comparison of peribulbar and retrobulbar anesthesia. Ophthalmology. 1993; 100:660–665.
6. Decock CE, De Baere EE, Bauters W, Shah AD, Delaey C, Forsyth R, et al. Insights into levator muscle dysfunction in a cohort of patients with molecularly confirmed blepharophimosis-ptosis-epicanthus inversus syndrome using high-resolution imaging, anatomic examination, and histopathologic examination. Arch Ophthalmol. 2011 Dec. 129 (12):1564-9. [Medline].
7. Dready JP, Morrell AJ, Sutton GA. Recognizing aponeurotic ptosis. J Neurol Neurosurg Psychiatry. 1989;52:996–998.
8. Fujiwara T, Matsuo K, Kondoh S, Yuzuriha S. Etiology and pathogenesis of aponeurotic blepharoptosis. Ann Plast Surg. 2001;46(1):29-35.
9. Sameera Irfan. Is Benign Essential Blepharospasm a “Benign” and/or an “Essential” Condition? The American Journal of Cosmetic Surgery, 2018, Vol. 35(2) 83–91

In this short video, the author, Dr Priya Desai has demonstrated the indication and the method of supra-tarsal steroid injection, which could be either Triamnicolone 20 mg in 1 ml or Dexamethasone 4mg in 1 ml. It needs to be injected deep into the SUPRA-TARSAL SPACE: the space between the upper surface of the tarsus and the orbicular muscle. A subconjunctival spread of steroids should be avoided as it leads to conjunctival scarring and more chances of a raised IOP.

They are short, straight-forward clinical recommendations, based on experience or observation which the general ophthalmologists can easily integrate into their daily clinical practice. They constitute a vast domain of experience-based medicine, and constitute an important part of the medical education. They are helpful in dealing with clinical problems for which no controlled studies exist.

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