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Giant Solitary Trichilemmal Cyst of the Upper Eye Lid: Masquerading Lacrimal Gland Tumor: A Clinico Radio Pathological Case Report-Juniper Publishers

Introduction

Trichilemmal cyst, also known as a pilar cyst, forms from the outer root sheath of hair follicle. They are common benign tumors most often found on the scalp [1]. The involvement of the eyelids is quite rare. It presents as a smooth, firm, mobile, and round nodule without a visible punctum. There is often an autosomal dominant inheritance pattern as the lesion is frequently familial [2]. Till now, there are only few case reports of trichilemmal cyst of the eyelid [3-6].

Case Report

A 58 year old mahemedian male from Bulendsar, UP, India was referred to Sharda Hospital because of a persistent static small swelling upper lid for last 10 years which is progressively increased over last three months. It was horizontally disposed [7-8], approximately 12mm/8mm in horizontal and vertical dimension. According to the patient this tumour first appeared as upper lid nodule. But for the last three months, it was growing rapidly and he was unable to open the eyelid. Patient developed ptosis and difficulties in vision due to large tumour and weight of the mass. There is drooping of upper eye lid since then. He is a non vegeterian and none of his family members had similar problem. An ophthalmic examination revealed a visual acuity of 20/40 in each eye due to presence of cataract which was not improved with pin hole or refraction [9-10]. The right upper lid has covered total cornea and almost 3/4th of palpebral apperature. A closer examination of anterior segment of both eyes were apparently normal expect early cataractous changes. Both the fundii were normal.

Examination of upper lid showed a large swelling arising from the upper lid, involving the 1/3rd of the right upper lid. Surface was smooth, overlying skin stretched. The mass was not with any surface blood vessels, with no colour or temperature changes, no expansible impulse on coughing and on auscultation no bruit was heard. Texture of the tumour mass was firm and solid. Intra Ocular Pressure by Applanation Ton+ometry was 16mm hg in both the eyes. Schirmer value was 18mm and that of BUT 14 in both the eyes. Plain X Ray of right orbit showed a large, well-defined, solid solitary mass at lateral aspect of upper lid just adjacent to lacrimal gland. measuring 3.1x2.1cm, over Routine Blood Investigations were within normal limit A clinical diagnosis of epithelial cyst was made. The patient under wentan excisional biopsy of the right upper lid. A skin incision was made along the lid crease [11-12], the skin was undermined over the mass which was unexpectedly easy in view of its adherence to mass on preoperative examination and the mass was easily separated from the underlying orbicularis. The mass was removed in to and primary skin closure was done. The excised mass was white in color. Post operatively, the lid contour was normal and there was no ptosis. No recurrence or evolution of new lesion was noted on follow-up. The patient is highly satisfied for cosmetic and free of growth. Histopathological examination of the specimen revealed the mass was lined by stratified squamous epithelium that lacks a granular cell layer and was filled with compact «wet» keratin.

(A) Clinical photograph of the right eye showing a growth over lateral aspect of upper lid with ptosis

(B) Photomicrograph from the removed growth showing (haematoxylin and eosin, original magnification x250, inset x325).

Comment

Trichilemmal cysts of the body may run in families and they may or may not be inflamed. They are solitary in 30% of cases and multiple in 70% of cases. These cysts are derived from the outer root sheath of the hair follicle. Their origin is unknown, but it has been suggested that they are produced by budding from the external root sheath as a genetically determined structural aberration. They arise preferentially in areas of high hair follicle concentration, therefore, 90% of cases occur on the scalp. Rarely, these cysts may grow more extensively and form rapidly multiplying trichilemmal tumors, also called proliferating trichilemmal cysts, which are benign but may grow aggressively at the cyst site. Very rarely, trichilemmal cysts can become malignant. They are lined by stratified squamous epithelium that lacks a granular cell layer and are filled with compact “wet”keratin. Areas consistent with proliferation can be found in some cysts. In rare cases, this leads to formation of a tumor, known as a proliferating trichilemmal cyst. The tumor is clinically benign, although it may display nuclear atypia, dyskeratosis, and mitotic figures which often can be mis leaded as the diagnosis of squamous cell carcinoma. Surgical excision is the only mode of management.

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An Interesting Case of Golden Hars Syndrome in 3 Years Male Child-Juniper Publishers

Introduction

Now we have defined d h syndrome as a complex of cong limmal dermoid with cong pre auricular skin tag aor appendage limbal dermoids sometimes are bilateral rarely. Usually they are unilateral involving whole of cornea or may be cofined to conjunctiva only incidence of l dermoids are 1 in 10000 or 500 in 2700 they are graded according to involvement of cornea grade 1. when only corneal epithelium is involved 2 grade 2 des membrane 3 grade 3 whole of ant segment of eye is involved inferno temporal involvement of limbal dermoid is the commonest about 70 percent.

Case Report

6 months back a 3 years male child was seen by me in my office with parents having noticed a small palish. White infero temporal limbal region left eye with cong presence of pre auricular skin appendage ft delivered child after lsc s no other cong anamolies seen vision mydriatic refraction ant segment and fundi were normal child had grade 1 limbal dermoid with the commonest site being infer temporal.

Results and Discussion

Golden hars is not a vision threatening disease most of these pts have normal intelligence and vision only in cases where visual axis is involved the we think of surgery conclusion my case was a simple case of gowhar ahmad grade 1 limbal dermoid so it only need observation in cases where visual axis is involved and vision is threatened then the surgical modalities are

1. Visual

2. Cosmotic

We do

1. Lamellar keratoplaty

2. Amionotic membrane graft

3. Stem cell graft

Their families of golden hars syndrome in Greece 2 in middle east in gulf war children born in different military hospital had golden hars syndrome 3 sometimes optic nerve drusen is associated with g h syndrome.

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 Visual Rehabilitation and Tolerability Using Hybrid Contact Lenses of Patients with Moderate to Severe Keratoconus-Juniper Publishers

Introduction

Keratoconus is non inflamatuary and progressive disease of the younger age group in which corneal thinning occurs and cornea assumes a conical shape associated with abnormal curvature. This changes often results in irregular astigmatism and myopia and leads to mild to marked visual impairment [1]. Corneal topography is a non-invasive technique to detect and monitor the progression of keratoconus. Contact lenses were used to improve visual conditions in keratoconus. Rigid gas permeable (RGP) lenses are most used type of contact lenses but in patient with severe disease RGP lens is insufficient for visual impairment and comfort [2]. Irregular astigmatism which developed after ectasia is not corrected with glasses and soft contact lenses. Mini-scleral, semi- scleral and scleral contact lenses are safe options in the management of irregular corneas. Because of heavy costs of scleral lenses, hybrid lenses with the comfort of the soft lenses and the optical quality of the hard lenses are developed [2]. This article reports the efficiency and accuracy of hybrid contact lens known as Clear Kone (Synerg Eyes Inc., Carlsbad, CA) in patients with moderate to severe keratoconus.

Methods

This study was performed in Medical Park Hospital, Antalya, Turkey. The patients were requested to sign informed consent forms. Patients had been previously diagnosed with keratoconus by corneal topography (Pentacam HR, Oculus, Wetzlar, Germany) and bio microscopic findings of Fleisher ring and Vogt lines. Inclusion criteria were keratoconus patients with severe visual problems and indication of intra corneal ring or transplantation surgery by another eye center. Cross linking was performed for all patients before the study. All patients were not appropriate for rigid gas permeable (RGP) lenses. All eyes were fitted with Clear Kone hybrid keratoconus lenses. The fitting is based on the concept of sagittal depth called as vault in relation to the cornea. Skirt curvature was determined as steep, median or flat according to limbus. Proper fitting was observed with using sodium fluorescein. After fitting, control of lens movement, vision and corneal epitelium were performed, 3 hours later. Corneal topography findings, pachymetry and refraction and vision were determined every 6 months.

Results

In this study, 19 eyes of 11 patients (6 men and 5 women) with a mean age of 26, 4 (16-43) were evaluated. Keratometry findings are between55-75 (Kmax). Before using contact lens, uncorrected and best corrected visual acuity with glasses were 0, 74±0, 3 LogMAR and 0, 58±0, 22 Log MAR respectively. Visual acuity with hybrid contact lenses was 0, 09±0, 05 LogMAR (Table 1). Mean follow-up was 7, 2 months (4-12 months). One patient didn't tolerate because of corneal edema.

Discussion

The first treatment choice for a patient with keratoconus is using a RGP lens [3]. Therefore, most of these patients, visions not corrected by glasses or soft contact lenses, have already tried the RGP lenses. The potential challenges associated with fitting rigid lenses are suboptimal initial comfort on non adapted eyes, the potential for lens decentration and the risk of lens ejection. Soft contact lenses have more comfort but less visual correction especially in advanced disease. Aim of hybrid lens is to combine the preferred properties of rigid and soft contact lenses [4]. Hybrid lenses are combines of a center-zone rigid lens and a peripheral zone soft skirt. Clear Kone lens which is used this study requires the determination of two fitting parameters, vault for rigid component and skirt curvature for soft component [5]. Due to design of the hybrid lens, most of the refraction power is provided by the tear layer, which increases the optical quality and oxygen supply of the cornea. Additionally, a little space between cornea and hard part of the lens prevents mechanical abrasion of the cornea [4] (Figure 1).

In our study, all patients have moderate to severe keratoconus and discomfort while using RGP lenses. Because of this condition, by other eye centers, corneal ring or keratoplasty were advised. Before the surgical treatment, we wanted to try another non- invasive management, hybrid lens fitting. Disadvantage of using hybrid lens, Clear Kone Synerg Eyes, is that this process is time consuming and requires more patience. Because of this, for appropriate final lens and shortening of process, we used the parameters of RGP lenses. This approach improved patients' compliances. Clear Kone lenses with hard central part and tears between lens and cornea improve vision. In our study, all patients had a good visual outcome. Soft peripheral part provides stability and comfort. Except one patient, 10 of 11 patients had a good comfort.

According to findings of this study, using hybrid lens on the keratoconus patients can be good choise for the patients with moderate to severe disease with discomfort of soft or hard lenses before thinking surgical management.

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Why Should SLT be the First-Choice Treatment for Glaucoma? -Juniper Publishers

Introduction

Glaucoma is a chronic, degenerative optic neuropathy which is characterized by changes in the optic disc and visual field loss. Intraocular pressure is considered the major risk factor for the progression of glaucomatous optic neuropathy involving the death of retinal ganglion cells and their axons. Selective Laser Trabeculoplasty (SLT) uses a 532nm frequency-doubled, q-switched neodymium, yttrium-aluminum-garnet laser that delivers a low-energy, large spot, it was designed to selectively target pigmented trabecular meshwork (TM) cells while sparing adjacent cells and tissues from thermal damage.

Discussion

Glaucoma is the second leading cause of blindness worldwide. Glaucoma affects more than 70 million people worldwide with approximately 10% being bilaterally blind, making it the leading cause of irreversible blindness in the world [1]. Meta-analysis of many studies showed that there was no statistically significant difference in IOP-reduction or treatment success between SLT and medical treatment [2].

As a first-line therapy for glaucoma, medications have many disadvantages and drawbacks. Patients have to accept their adverse effects, repeated application of medications and continuous medical costs. The potency of treatments may be undercut when people are non-compliant. The introduction to SLT, which uses a less severe energy to generate IOP-lowering effect without dangerous adverse reactions, has again brought up the question: can laser device treatments defeat eye drops as the main therapy for Glaucoma especially OAG? In the initial released research, SLT was used as the adjunct treatment to medication. Later several researches had recommended that SLT might be the main treatment for POAG or OHT [3]. Melamed et al. [4] found 40 in 45 eyes (89%) which experienced SLT as the main treatment had an IOP loss of 5mmHg or more. Mean IOP- reduction was 7.7±3.5mmHg (30%) at 18 months post-SLT. A potential, multicenter study by McIlraith et al. [5] involved 100 eyes (61 patients) with recently clinically diagnosed POAG or OHT. IOP reduction was 8.3mmHg (31.0%) in the SLT team (74 eyes) in contrast to 7.7mmHg (30.6%) in the latanoprost team (26 eyes) (P=0.208 and P=0.879). The amount of IOP reduction was mathematically considerably less in the latanoprost team than in the SLT team [5].

Selective laser trabeculoplasty (SLT) has been proven secure, well recognized, low-cost and very efficient at reducing intraocular pressure (IOP) as primary treatment in several types of glaucomas. The maintenance of trabecular meshwork (TM) structure and the confirmed effectiveness in decreasing IOP tend to make SLT a very affordable and secure alternative to argon laser trabeculoplasty (ALT). SLT may also be effective for situations of unsuccessful ALT and is a technique that may also be repeatable, compared with ALT. SLT is also a simple procedure for an ophthalmologist to learn and proceed. SLT has been confirmed to be efficient as main therapy and can be an effective adjunct therapy with medications for early stages of glaucoma. Furthermore, SLT can be regarded as a primary treatment choice for patients who cannot accept or who are noncompliant with their glaucoma medications, without disrupting the success of the forthcoming surgery [6].

Conclusion

SLT is easy to perform and well tolerated by patients and provides the benefits of ALT while using much less energy with less obvious harm to the TM. It seems to have comparative effectiveness to eye drops and to be a safe and effective glaucoma treatment without conformity threats or wide spread adverse reactions. Therefore, SLT is now considered the first line therapy for various types of glaucoma and can be an effective adjunct in the early types of glaucoma.

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Rare and Interesting Case of Eye Choriodal Melanoma Presenting as a Congestive Glaucoma in a 55 Years Male Patient-Juniper Publishers

Abstract

55 years male patient presented as a case of a glaucoma in 2011 at ER ophthalmological dept of medical hospital Srinagar Kashmir, he presented with severe pain marked redness of left eye and marked loss of vision on exam he had sever ciliary congestin corneal odema very shallow a chamber and dilated pupil no clear view of fundus and IOP of more than 550mm Hg. He was put on emergency medication of a c glaucoma in form of topical LUPITISS eye drops one drop l eye od 2 britiblu eye drops i drop L eye bd 3 osmotic diuretic 20 percent mannitol 250cc IV 4 500mf of injection diamox I we even with this medication pt dod not respond even after more than 12 hours of this medication he was advised admission which he refused however no a aor B-scan ultrasound was done pt after couple of days sought second ophthalmic consultation this time the con ophthalmologist performed B-scan ultrasound and picked up a solid retinal detachment of l eye to confirm melanoma.ch he was referred for MRI scan of orbits and brain unfortunately for patient and treating ophthalmologist the report of scan was very unsatisfactory the radiologist gave a very unsatisfactory report that pt has pan ophthalmitis l eye however ophthalmologist requested him that this is a case of CD melonoma [1], and it has to be confirmed by MRI in this process p both pt and got confused and patient left the treatment he was unfortunately going from one oph to other as a painful blind eye for two years key words melanoma.ch is the commonest tumor it can be benign or malignant LUPITISS t is prostaglandin analogue and t stands for temilola beta blocker beta blocker 3 britiblu is brimolol 3 mannitol is osmotic duretic 4 diamox is carbonic anhydrase inhibitor od means one dop once a day 5bd is one drop twice a day glaucoma is a v=condition where i o gets raised in eye pathogenesis of IOP aqueous is produced by ciliary process of ciary epethelium im post chamber where [2], the aqueous nourishes lens then through pupil it comes in ant chamber and aqua nourishes cornea and then it is dained though angle of filtration through canal of schlem and finally aqueous comes in aqua veins so any condition which causes obstruction in drainage of aqueous will result in rise of IOP which is called glaucoma classification of glaucoma cong glaucoma occurs during intra uterine life 2 infantile glaucoma if from birth to 3 years 3 juvenile off 3 years to 17 years 2 primary glaucoma open and closed angle 3 sec glaucoma which occurs sec to any intraocular disease 4 ocular hypertension sec to systematic tension dx of cong glaucoma also called buphthalmos big eye ball a large cornea we do under anesthesia in these neonates and measure [3], IOP and measure corneal diameter treatment modalities which r surgical have been

I. Goniotomy

II. Goniopexy

III.Trabeculotomy

IV.Sinostomy

Case Report

In open angle glaucoma there is no pain moderate rise of IOP causes headache there are 3 features

a. Fundus changes

b. Changes in visual fields

Fundus causing glaucomatous cupping and even optic atrophy most the times pts of open angle glaucoma are diagnosed late due to lack of pain and pt may come when pt already [4], had glaucomatous cupping and optic atrophy that's why we are doing glaucoma screening after 40 years of age where we do routine measuring of IOP refraction and fundus examination in this screening we are able t pick up cases of open angle glaucoma treatment is medical and surgical first [5], we put it on medical therapy and if it doesn't not respond to it or it not coming for regular ophthalmic evaluation or pt belongs to fat of place lack of ophthalmic advice one should do surgery the surgical modalities are 1 trabeculectomy advantages of this procedure

1. It is cosmetically better

2. It can be repeated

3. It is a physiologically better operation

4. It communicates the ant c with canal of schlem by splitting the trabecular mesh work

Nowadays, we have better options of lased and operation of laser choice is an argon laser trabeculoplasty closed angle glaucoma comes very early due [6], to severe pain redness and marked loss of vision in most of the these case they respond to anti glaucoma medication after the eye settled down we do laser iridotomy even in normal eye of these pts we do polylectic laser iridotomy then we have ocular hypertension due to systemic hypertension key is control of b p ten we have a miscellaneous group of glaucoma 1 neo vascularisation glaucoma due to c are c occlusion it is said to subside in 3 months last l by least we have absolute glaucoma pt has painful blind eye treatment modalities.

a) Cyclodiathermy

b) Cyclo croprexy

Sometimes due to severe pain we may inject retrobulbar injection of xylocaine or alcohal to revealve pain sometimes one may have to enuck leation if all measures fail introduction ch melanoma is the most common primary malignan intraocular tumor and the second most common type of primary malignant melanoma in the body it most often whites of northern European descent essential update early treatment may prevent metastatic deaths in patients with melanoma.ch in a prospective cohort study of 3072 patients with melanoma. ch researchers we found for some pts early treatment rather than watching and waiting might better prevent metastatic deaths signs and symptoms melanoma.ch remain asymptomatic for prolonged period of time may be found incidentally during ophthalmoscope in general the more the their origin the longer the delay of any symptoms melanoma.ch present with following symptoms:

a. Blured vision

b. Paracentral scotoma

c. Progressive and painless visual loss

d. Floaters

e. Sever ocular pain

f. Weight loss marked fatigue cough or change in bowel or bladder suggests primary non ocular malignancy with melanoma.

ophthalmological examination may reveal following

A. Small melanoma.ch typically takes the form of a nodular dome shaped and well circumscribed mass under the retinal pigment epethelium

B. Melanoma.ch grow they adopt more irregular configurations

C. Diffuse melanoma.ch characterized by growth throughout the choroid with minimal elevation are more difficult to diagnose and often cause significant exudative redetachment

D. Melanoma.ch may have variable coloration some are more pigmented and others are less

E. If tumor is light colored its abnormal vascularisation usually can be seen ohthalmoscopicaly

F. Overlying melanoma. ch

There are usualy tet pig epethelial changes eg drusen dx 1 liver enzyme are indicated in any pt with uveal melanoma is the most common site of melanoma.ch metastases so one has to do

a. Alkaline phosphates

b. Glutamic oxaloacetic trans aminase

c. Lactase dehyrogenase

Gamma glutamyl transpeptidase a scan and B-scan ultrasound MRI scan ct scan f f angoigraphy can help point toits dx 2 small ch melanomas may show floresene angigrahkic changes similar to choriodal nevi 3 large melanomas may show patchy pattern of early hypoflourence and hyper flourescence followed by late intense staining simultaneous flourescence of retinal [7-8], and ch circulation within is fiarly distintive of melanomas.ch management

I. Observation may be acceptable for post uveal tumors not well established in particular tumors of less than

II. To 2.5mm and 10mm in diameter can be observer until growth is documented

III. Plaque brachy therapy is a widely accepted alternative to enucleation medium sised post uveal melanomas less than 10mm

IV. External beam irradiation with protons and helium ions

V. Pp vasectomy

VI. Block excision

VII. Laser photocoagulation

VIII. Orbital exenteration in extreme cases.

Introduction

The above mentioned pt b was seen by me in 2013 his all investigations l f t c bc k f t lipid profile f b s were within normal limits ultrasound abdomen normal no evidence of any metastases i requested for MRI scan of orbits and brain it was very satisfactory showing that optic nerve optic tract Chiasm a basal ganglia pit gland thalmoid brain ventricles and cerebral hemispheres all were normal discussion we have aired mentioned that scleral cavity can be

1. Antto lens so it will cause visual impairment

2. Involve vitrous causind floaters

3. Involve angle of filtration and present and a c glaucoma as was seen in our case.

Discussion

So after all above mentioned investigations under gowhar ahamed I removed whole cornea lens vitrous choriod and retina [9-11], so we were left with intact intra scleral cavity optic nerve and all ocular muscles i stitched ant and pst lips of sclera with 6 zero vicryl kept a drain in intra scleral cavity which was removed after 24 hours later i put a confirmer to give shape to orbit for 15 days after removal of confirmer i fitted a well matching and fitting prosthesis the removed tissues were sent for histopathological examination did not show any evidence of malignancy 3 years [12-15], have passed all repeat tests od blood ultra sonography b abdomen are normal.

Conclusion

So any pt f ac c glaucoma if does not respond to treatment please do a or b scan ultrasound to pick up solid ret det and rule out m melanoma.ch by MRI review of literature 1 vertex varies can present as Uveal melanoma however it is a benign condition and temporary 2 in dept of ophthalmology manchester a pt came with a c glaucoma vision in normal eye unaided was 6,18 have did not respond to treatment MRI showed melanoma.ch so Enucleation was done 2 in Saudi medical lornal and cairio institute of ophthalmology and nophthalmology one pt came with BSEC glaucoma with imp of 56mmhg pt did not respond to treatment MRI was done it revealed melanoma.ch so Enucleation was done.

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Endophthalmitis after Lower Lid Blepharoplasty; A Rare Complication-Juniper Publishers

Case Presentation

A 22-year- old man referred to the emergency ward because of vision loss in his right eye five days after bilateral lower lid blepharoplasty, his right eye showed ciliary injection, cornea showed mild edema and anterior chamber showed 4+cells and faint hypopyon in slit lamp examination, There was severe chemosis, without any site of laceration. However, fundus examination showed severe fibrinous reaction and vitritis. (Figures 1-5 are related to the post-operative status.)

Discussion

Based on the diagnosis of undetermined uveitis the patient underwent diagnostic vitrectomy, interestingly there was a site of laceration in the posterior fundus, leading to peritomy and its repair associated with severe vitreous inflammation and retinal necrosis and vasculitis. The patient underwent pars plana vitrectomy associated with intra vitreal antibiotic injection and silicone oil injection; the culture of the vitreous documented staphylococcus aurous in the vitreous sample. This rare complication has been rarely described in the literature [1,2].

Conclusion

Although rare, doing blepharoplasty a common cosmetic in inexperienced hand could lead to a severe devastating intra ocular complication.

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Pediatric Cavernous Sinus Syndrome as the Initial Presentation of Intracranial Germinoma: A Case Report-Juniper Publishers

Abstract

8-year girl complained of diplopia and her right eye presented ptosis, supraduction, abduction deficit, slight esotropia, and corneal hypoesthesia. Visual acuity was 20/20 in both eyes. Pupillary light reflex showed full, fast constriction and afferent pupillary defect was negative. No optic disc abnormalities were observed and indicated cavernous sinus syndrome. A brain magnetic resonance imaging indicated a tumor lesion from the sellaturcica to the suprasellar region and advancing into the right cavernous sinus, compressing the optic chiasm. Tumor biopsy was performed. Histo pathological examination led to a diagnosis of intracranial germinoma. Intracranial germinoma manifest hypopituitarism and bitemporal hemianopsia but in this case, intracranial germinoma manifesting as cavernous sinus syndrome initially. Cavernous sinus syndrome represents highly in adults and pediatric cases are rare. Malignant lymphoma and Trosa-Hunt syndrome were occasionally reported as diseases causing cavernous sinus syndrome in children. But, to our knowledge, there were a few reports of intracranial germinoma. Although it is very rare, germinoma should be kept in mind as a disease which causes cavernous sinus syndrome in children.

Introduction

8-year-old girl complained of diplopia from April 2006, and started tilting her head to see things. Intermittent ptosis of the right eye appeared in May. Because right eye movement disorder was noted, she initially visited our department on October. The visual acuity was 20/20 in both eyes, the light reaction was complete and rapid, and relative afferent pupillary defect (RAPD) was negative. Ptosis of the right eye was noted. The pupil diameters were 4.6 and 3.7mm on the right and left sides in a bright room, respectively, and 4.6 and 5.7mm in a dark room, respectively, showing laterality. The pupil diameter after a cocaine eye drop test were 4.5 and 5.7mm in a bright room, respectively, being judged as positive for the test. Regarding eye movement, impairment of abduction and supraduction, mild impairment of adduction, and infraduction disorder of the right eye were observed, and corneal sensory reflex of the right eye was reduced. No abnormality was noted in the anterior segment of the eye, optic media, or fundus.Bitempolal hemianopia in visual field was observed (Figure 1). On brain magnetic resonance imaging (MRI) performed (Figure 2), a mass lesion advancing from inside the sellaturcica to the supra sellar region and right cavernous sinus was observed. Contrast enhancement of the tumor continuous from the right cavernous sinus was observed, and it advanced along the oculomotor and abducens nerves, the serum human chorionic gonad otropin-β (hCG-β) level was high suggesting germ cell tumor. Regarding the systemic condition, panhypopituitarism was present, and hormone replacement therapy was initiated. Tumor biopsy was scheduled but it was postponed by her family. The visual acuity of the left eye decreased to 20/50 in February 2007, and impairment of supraduction of the right eye aggravated. On MRI performed in the same period (Figure 3), compression of the chiasma became stronger in the suprasellar region. On the right side, the tumor advanced along the orbital apex and trigeminal nerve. Tumor biopsy by craniotomy was performed on March 6, 2007. Solitary appearance of large cells containing bright and wide cytoplasm was observed on histopathological examination (Figure 4), and cells were positive for placental alkaline phosphatase (PLAP) and c-kit on immunostaining. Based on these findings, the tumor was diagnosed as intracranial germinoma. Radiotherapy and chemotherapy were immediately performed, and the tumor shrank. On follow-up in July, the visual acuity became 20/20 in both eyes, and the tumor has not recurred for 8 years.

Discussion

In the present patient, diplopia and ptosis were the initial symptoms and the features of cavernous sinus syndrome complicated by Horner's syndrome were observed. These clinical findings were consistent with the mass lesion advancing from inside the sellaturcica to the suprasellar region and right cavernous sinus observed on MRI. To our knowledge, only 3 cases [1-3], of intracranial germinoma causing cavernous sinus syndrome as the initial symptoms. In these 3 cases, the tumor was heterogeneously enhanced with gadolinium on MRI and Serum or cerebrospinal fluid hCG-β was positive.One case is children [1]. 11-year-old girl presented of left 6th cranial nerve Palsty without visual field defect. Her head MRI showed a mass mainly located in the left cavernous sinus. First biopsy want clearly histlogycal evidence of tumor cells. Intracranial germinoma was diagnosed by second biopsy five month later. Retrospective analysis of the first specimen revealed a few cells positive for c-kit immune histo chemical stain. Thus c-kit is useful for the differential diagnosis and might have resulted in early diagnosis at the first exploration. Present case test of c-kit, hCG-β and PLAP were positive in serum or cerebro spinal fluid, so we could suspect for intracranial germinoma.

Intracranial germinoma accounts for 15% of pediatric tumor cases and it is higher in Japan than in the other western countries (3-4%) [4]. It arises in the pineal body and suprasellar regions and induces various symptoms. Clinical manifectation are hypopituitarism and bitemporal hemianopsia. In this case, intracranial germinomam anifesting as cavernous sinus syndrome initially. Most cases occuring cavernous sinus syndrome develop in adults and pediatric cases are rare. Malignant lymphoma [5,6] and Trosa-Hunt syndrome [7-9] were occasionally reported as diseases causing cavernous sinus syndrome in children. Although it is very rare, intracranial germinoma should be kept in mind as a disease which causes cavernous sinus syndrome in children.

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 Triamacinolone Acetonide Versus Bevacizumab in Treatment of Neovascular Age Related Macular Degeneration-Juniper Publishers

Abstract

Purpose: To evaluate the effects of intravitreal triamacinolone acetonide (IVTA) and bevacizumab injection on visual acuity, electro physiologic response and foveal thickness of patients with neo vascular age related macular degeneration (CNVs).

Methods: The study included three groups. Group 1 included fifty eyes (50 patients) with progressive occult or predominately occult sub foveal choroidal neovascularization treated with intravitreal injection of trimacinolone acetonide. Group 2 included another fifty eyes (50 patients) with progressive macular degeneration of occult or predominately occult sub foveal choroidal neovascularization treated with intravitreal bevacizumab. The patients is followed up every month by bio microscopy, optical coherence tomography (OCT), photography, fluoresce in angiography, Ganzfeld full field electro retinogram(F-ERG), multifocal electro retinogram (MF-ERG) and determination of best corrected visual acuity (BCVA). Group 3 (control group) included 50eyes (50 patients) with neo vascular age related macular degeneration who did not receive treatment for macular degeneration.

Results: Apparent improvement of morphological and functional characteristics were observed in 40/50 eyes after one month after first injection in group1, and in 45/50 in group 2. The mean±SD visual acuity improved significantly (p=0.003) from (0.12 ±0.19 to 0.35±0.25) in group1 and from 0.13±0.2 to 0.40±0.28 in group 2. Visual acuity was highest 1-2 month after intravitreal injection. Central macular thickness decreased from 325±50^m to 275±40^m at one month after first injection in group 1 and decreased from 320±53^m to 255±41^m in group 2 while in control group, there is statistically insignificant increase of the central thickness. The average amplitude of central macular ring of MFERG was improved from 25.5±5.8nv to 31±8.9nv in group 1 and from 26.6±6.1nv to 33±9.9nv in group 2, while no changes in F-ERG response. Intraocular pressure increased significantly (p=0.009) from 14±2.5mmHg to maximal 23±7.6mmHg in group 1. Intraocular pressure decreased significantly (p=0.006) to 16±2mmHg at the end of follow up while in group 2, there was no increase in intraocular pressure. No other serious drug related adverse events (endophthalmitis, retinal detachment, cataract or proliferative vitreo retinopathy) observed during the course of the study in groups 1, 2. In control group, visual acuity, central foveal thickness and function did not change significantly during follow up period (p=0.6, p=0.4, p=0.1 respectively).

Conclusion: Intravitreal injection of trimacinolone acetonide may transiently stabilize or improve visual acuity in some patients with progressive neo vascular age related macular degeneration. Intravitreal injection of bevacizumab led to a more visual improvement than IVTA in treatment CNVs. MFERG had an important role in describing the effect of treatment on retinal function. Intravitreal injection improved MFERG macular function responses with little insignificant change in F-ERG.

Keywords: Electroretinogram; Optical coherence tomography; Choroid neovascularization; Avastin; Triamacinolone acetate

Introduction

None of the present treatment decreases the loss of vision on the central 35° of the retina in macular degeneration. Neovascular age related macular degeneration is a common reason for irreversible reduction and loss of vision in the world [1] . Triamacinolone acetonide is one of the first drugs used for the management of age related choroidal neo vascularization [2] . Triamacinolone acetonide stabilizes blood retinal barrier, decreases the permeability and inflammation, increases the diffusion and reduces vascular endothelial growth factor [3]. Vascular endothelial growth factor (VEGF) plays important role in AMD pathogenesis [4]. Bevacizumab is humanized antibody to human vascular endothelium growth factor (VEGF) which combines to VEGF and hinders it from attachment to its receptors [5]. Electro retinogram of neo vascular macular degeneration gives information about the treatment safety. MF-ERG represents the photopic retinal response to a rapidly changing stimulus on the central 35° of the retina [6]. Neovascular age related macular degeneration decreases the central peak amplitude which is altered by subretinal fluid [7]. MF-ERG is used to monitor the localized change after treatment. Full field ERG response reflects general retinal electrical response and gives information about treatment toxicity [6,7]. The aim of the study was to evaluate and compare the effects of triamacinolone acetonide and bevacizumab on visual acuity and retinal thickness in neovascular age related macular degeneration patients and to study the effects of triamacinolone acetonide and bevacizumab on the retinal function.

Subjects and Methods

This study was carried out on patients attending the Outpatient's Clinic of Mansoura Ophthalmic Center during the period from February 2012 to December 2015. One hundred and fifty patients (150) with neovascular age related macular degeneration were included in the study.

The patients were divided into three group:

a) Group 1: Included progressive occult subfoveal choroidal neovascularization patients who received intravitreal injection of triamcinolone acetonide.

b) Group 2: Included occult subfoveal choroidal neovascularization patients who received intravitreal injection of bevacizumab.

c) Group 3 (control group): Included neovascular age related macular degeneration patients who refused intravitreal injection.

Exclusion criteria

Included patients with classic type of neovascular age related macular degeneration and any other ophthalmological. All patients were examined on the first day after injection, in first week, then every month for 6 months. A repeated injection was performed if there were activity of choroidal neovascularization (CNV). Intra-retinal and sub-retinal fluid accumulation, new intra-retinal and sub-retinal hemorrhage and CNVs growth were signs of CNVs activity. Re-treatment was done if there were signs of CNV activity or decreasing visual acuity. At baseline of the study and at monthly intervals, all patients underwent a routine ophthalmological examination. Goldman applanation tonometry, direct and indirect ophthalmology, optical coherence tomography (OCT), and electroretinogram (ERG) were done. Fluorescein angiography was done using Topcon Corporation 2000, TRC, 50Ix, Japan. Fluorescein angiography was performed for all patients at beginning and after 3months and 6 months.

Optical coherence tomography (OCT)

OCT was done with Topcon, 3 dimensional OCT-1000 (Topcon Corporation, Tokyo, Japan). Internal fixation was chosen because of better reproducibility. It scanned a cube of 6x6mm length. Central macular thickness of a circular 1-mm radius area around the fovea was calculated.

ERG

Full field ERG and MF-ERG were recorded using Roland Consult, (Germany system). ERG was done according to ISCEV standard [8]. After topical corneal anesthesia (Benoxinate hydrochloride 4%), positive electrode (Dawson, Trick and litzkow (DTL) electrode) was placed just contact with corneal limbus, ground electrode was installed on the forehead and negative electrode was placed near orbital rim temporary. The recording was monocular.

Full field ERG

The test was started and recorded in 5 steps, scotopic rod response, scotopic combined response, oscillatory potential then light adaptation for 10 minute then photopic cone response and flicker response recording.

MF-ERG

Patients were positioned 30cm from the stimulus monitor. Stimulus clarity was adjusted by over-refraction. Each hexagon was temporally modulated between light and dark according to binary m-squence [9,10]. Patients fixated a spot in the center of the stimulus. The results of two 8-minute recordings were averaged to improve the signal to noise ratio.

Intravitreal injection of triamacinolone acetonide (IVTA)

In group 1, 25mg of crystalline triamacinolone acetonide (Volona A, Bristol-Myers-Squibb, Munich, Germany, containing 40mg of triamacinolone acetonide in 1ml) was injected intravitreal. The injection of 25mg of crystalline triamacinolone acetonide was performed using sharp 27-gauge needle in inferio-temporal quadrant 3.5mm from limbus.Then antibiotic ointment was applied.

Intravitreal injection of bevacizumab

All patients in group 2 received Intravitreal injection of 1.25mg/0.05 of bevacizumab. A total of 0.05ml Bevacizumab was injected into vitrous cavity 3.5mm from limbus in inferotemporal quadrant using 30 gauge needles. Postoperative antibiotics were used and a light patch was placed. The eye patch is removed the next day.

Statistical Analysis

Statistical analysis was performed using soft ware (SPSS WIN Version11.5, SPSS Inc, Chicago). Non parametric Wilcoxon test was applied for comparison. Spearman rank test and linear correlation analysis were used in order to evaluate the correlation with changes of MFERG, changes of OCT and best correct visual acuity. Significance was set at p=0.05 (2-tailed) for all statistical tests.

Results

The study included one hundred and fifty (150) patients (one hundred and fifty eyes). The patients were divided into three groups. Group 1 included fifty eyes (50) of fifty patients (50) with progressive occult or predominately occult subfoveal choroidal neovascularization who received one or more than one of intravitreal injection of 25mg of triamcinolone acetonide, Mean age was 60±8.6years ,ranged (55 -75 years old). Group 2 included another fifty eyes (50) of fifty patients with progressive occult or predominately occult sub foveal choroidal neovascularization) who received one or more than one of intravitreal injection of 1.25mg/0.05ml of bevacizumab. The Mean age was 62±6.9 years, ranged (58-74 years). Group 3 (Control group) included fifty eyes (50) of fifty patients (50) (with neovascular age related macular but did not receive intravitreal injection of tri amcinolone acetonide for this disease after explanation the importance of treatment for visual acuity and CNVs. The Mean age was 61 ±7.9 years, ranged (58-74 years). There was no significant difference between groups. In group 1, Ten (10) patients received a second intravitreal injection of 25mg of triamcinolone acetonide. According to flourescein angiography, group 1 were further divided into subgroups with occult or mostly (>50%) occult without hemorrhage (n=40, 80%), subgroup with sub-retinal hemorrhage (n=7, 14%) and subgroups with retinal pigment detachment (n=4, 8%). While in group 2, occult CNV without haemorrhage was presented in 35 eyes (70%), with haemorrhage was present in 10 eyes (20%) and retinal pigment detachment was found in 5 eyes (10%) ( Table1). All three subgroups did not vary significantly (p=0.2) at baseline. In control group, all subjects were having occult CNV without sub retinal hemorrhage.

Visual acuity(VA)

All patients complained of decreased visions which were diagnosed by ophthalmologic examination within three months before IVTA. The mean VA at baseline in group 1, group 2 and control Group (0.12±0.19, 0.13±0.05 and 0.14±0.22) respectively. (Range from finger counting to 0.3 in group 1, from finger count to 0. 32 in group 2 and from finger counting to 0.5 in control group. For the Group 1 and Group 2, mean VA increased significantly (p=0.003) after first injection to maximum 0.35±0.25 during the follow up period (Table 2). The maximum postoperative VA was detected 1-2 months after the injection. The increase in VA was statistically significant in 1st month (p=0.003) and 2nd month (p=0.004) after the injection. The preoperative visual acuity and postoperative visual acuity achieved at the end of the follow up period did not differ significantly (p=0.2) in group 1 while in group 2, there is statistically significant difference between VA at the baseline and VA at the end. In group 1, Visual acuity significantly decreased towards the end of the follow up period, parallel to a disappearance of triamacinolone acetate crystals out of vitreous cavity. In group 1, after 1 month, 40eyes (80%) gained in visual acuity and 4eyes (8%) lost visual acuity. Visual acuity was unchanged for 6 eyes (12%). While, in group 2, 45 eyes (90%) gained in visual acuity. There were no significant correlation between postoperative visual acuity and postoperative change in visual acuity (p=0.6). For three subgroups, there were significant difference in gain in visual acuity (p=0.04). Ten eyes received second injection three months after first injection, visual acuity increased in eight eyes about one month after the re-injection and declined again after about 3 month in group 1 while in group 2, fifteen eyes received second injection after 2 months, and fourteen eyes of fifteen improved after reinjection. 

OCT

For group 1, Central subfield OCT thickness was 325±50μm at baseline. The central subfield OCT thickness decreased to 280±55μm at one week and 275± 40μm at one month (Table 3, Figure 1). In group 2, central subfield OCT thickness was 320±53μm at baseline. The Central subfield OCT thickness decreased to 270± 40μm at one week and 255±41μm at one month

ERG

F-ERG data is presented in (Table 4). No significant worsening of FERG response was observed during follow up period in the three groups. Most of the values were within the limits normal variation. For most subjects retested one month with F-ERG, the amplitude returned to baseline after a slight decrease in scotopic and photopic amplitudes at one week. For all subjects who was received either triamacinolone acetate or bevacizumab had an essentially stable F-ERG.

MFERG

In most cases, there was improvement at one week, one month and two months of IVTA, then return to baseline value at 3 months (Table 5, Figure 2 & 3). In group 2, there was increase in amplitude and decrease in latency reach the maximum after 2 months.

Intraocular complications

In group 1, IOP increased significantly (p=0.005) from 14.5±2.5mmHg at baseline of the study to a mean maximal value of 20±5.6mmHg again decreased significantly to 16.5mm Hg at 6 months after IVTA at p=0.001. IOP measurements at the end were slightly and significantly (P=0.05) higher. During the study, IOP was higher than 22mmHg in 30 eyes (60%). In those patients, IOP normalized by topical anti-glaucomatous drugs. Optic nerve damage was not detected. While in group 2, there was no case with increase in intraocular pressure. With respect to other complication of IVTA, three cases of cataract were detected in group 1, while no case of cataract was observed in group 2. No postoperative infectious endophthalimitis, rhegmatogenous retinal detachment or proliferative vitreo-retinopathy was detected in groups 1 and group 2.

Discussion

While, classic type of subfoveal neovascularization, photodynamic therapy with verteporfin stabilizes or increases visual acuity. Photodynamic therapy for occult subfoveal neovascularization is unsuccessful [11,12]. Steroids have antiinflammatory, antiangiogenic, antifibrotic and antipermeability properties, which contribute to stabilization of the blood- retina barrier [13] Penfold et al. [14,15] Chella et al. [16], injected trimacinolone intraviteal to treat exudative macular degeneration. Additionally, Danis et al. [17], detected a beneficial effect of trimacinolone in the study group compared with control group. Also, Ranson et al. [18], treated recurrent subfoveal neovascularization after laser treatment by IVTA. Chella et al. [16] evaluated the efficacy of intravitreal of trimacinolone for one and half year in exudative age related macular degeneration. They reported that a single intravitreal injection of 4mg of trimacinoloneacetate was helpful in treatment of exudative age related macular degeneration. In this study (in group 1), there was increase in visual acuity, reduction of fluorescein angiography leakage, reduction central macular thickness and increase in amplitude of MFERG with reduction of implicit time in 40 eyes of 50eyes (80%) within 2 months. Ten eyes of 40 eyes (25%) receive another intravitreal injection after 3 months (after beginning of reduction of visual acuity with increase macular thickness, reduction of amplitude of MFERG and increase implicit time). There was improvement of six of ten eyes (60%).

There was correlation between visual acuity and central macular thickness (p=0.008, R=0.5) and visual acuity and MFERG amplitude (P=0.006, R=0.55). Also, there was significant correlation between central macular thickness and MFERG amplitude (P=0.001, R=0.65) in group 1. In this study, we injected high dose of triamcinolone acetate intravitreal in group 1, because the results of previous studies were not clear; Jonas et al found significant increase in visual acuity after intravitreal injection of 25mg of trimacinolone acetate [19,20]. while Gillies et al. [21], reported no effect of 4mg of intravitreal injection of trimacinolone acetate on the development of sever visual loss during one year follow up. The Causes for the difference between studies may be the amount of injected trimacinolone acetate Second cause for difference between studies may be related to the effect of development of cataract on vision. Other cause for discrepancy between this study and investigation of Gillies et al. [21] may be that their study included classic subfoveal neovascularization that had a worse prognosis than occult choroidal neovascularization.

There was significant elevation in intraocular pressure in group 1 compared with other groups. There was 30 eyes (60%) had increased intraocular pressure. All cases were controlled with medical anti-glaucomatous treatment. Various studies have reported increase of IOP ranging from11-30% of subjects following IVTA [17,19,22] None of patients had been shown infectious endophthalmitis, rhgmatogenous retinal detachment , or proliferative vitreo retinopathy in this study .

Jonas et al. [19] found the reduction of visual acuity started 4-5 months after initial increase in visual acuity two months after injection. Similarly, in the present study after initial increase of vision two month after injection, visual performance started to decrease again. This may be result from resolving of trimacinolone acetate crystals Vascular endothelial growth factor (VEGF) plays an important role in the pathogenesis of AMD 22 Intravitreal bevacizumab injection was reported to be effective for treatment exudative AMD. Bevacizumab inhibit VEGF, decrease angiogenesis and decrease vascular permeability. [23-25].

In group 2, there were increase in visual acuity, reduction in retinal thickness and improvement of electrophysiological amplitudes and latencies. The improvement was slightly more significant in group 2 than in group 1 (As seen in Table 1) The cause for this improvement in group 2 more than group 2 is that triamcinolone exerted its antiangiogenic effect by enhancing endostatin expression rather than suppressing VEGF expression [26]. While bevacizumab decrease angiogenesis by decreasing VEGF expression and enhancing endostatin [27]. There was correlation between visual acuity and central macular thickness (p=0.006, R=0.55) and visual acuity and MFERG amplitude (P=0.005, R=0.65). Also, there was significant correlation between central macular thickness and MFERG amplitude (P=0.003, R=0.6) in group 2. Similarly, Rosenfeld et al reported that intravitreal injection of bevacizumab cause marked decrease in retinal thickness without toxicity [28]. Ahmadieh et al. observed improvement of vision and reduction of thickness after bevacizumab [29]. Also, Falkenstein et al showed that primary bevacizumab therapy resulted in significantly visual improvement [30]. The bevacizumab preparation is unpreserved and contains no ingredients that are toxic to the eye [28]. Intravitreal bevacizumab is well tolerated in the majority of patients. In this study, there were no complications in group 2. Only subconjuctival heamorrhage in two cases which resolved within a week. While, Ahmadieh et al. [29]. Observed one case with pigment epithelial detachment without any cases of endophalmitis or sub-conjuctival haemorrhage [29]. Also, Cleary et al. [30]. Found endophthalmitis in 1 of 112 eyes, submacular hemorrhage in 3 of 112 eyes and retinal pigment epithelial tears in 3 of112 eyes. Ronan et al. [31], and Avery said that the presence of pigment epithelial detachment was risk factor for retinal pigment epithelial tear after IVB injection [32]. The limitations of this study were the method of measuring visual acuity. Instead of the charts used for the Early Treatment Diabetic Retinopathy [33], visual acuity was determined using Snellen charts. But, the same method was used to three groups. Other limitation of this study was limited follow up and limited number of the patients, the relatively high dose of trimacinolone acetate injected into eye. In summary, intravitreal injection of trimacinolone acetate and bevacizumab improve visual acuity transiently in patients with neovascular age related macular degeneration. Furthermore, intravitreal injection caused anatomical changes and functional improvement of MFERG. Bevacizumab gave more favorable visual outcome and anatomical and functional improvement than triamcinolone acetate. To stabilize visual acuity, repeated intravitreal injection is recommended with 2-3 months apart with take care of complication especially intraocular pressure in cases of IVTA.

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