MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

Abstract

Encephalocraniocutaneous lipomatosis is a very rare neurocutaneous disorder. A 30 year old lady came to OPD presenting withsmall periocular papules, epibulbarchoriostomasalong with a lipomatous swelling in the frontotemporal area and a patchy alopecia on the scalp1. On imaging of the brain it showed dilatation of the ventricle along with cerebral atrophy of the right side. It was finally diagnosed as a case of encephalocraniocutanouslipomatosis based on the Moog’s criteria.

Keywords: encephalocraniocutaneous lipomatosis, neurocutaneous disorder

INTRODUCTION

Encephalocraniocutaneous lipomatosis is a very rare neurocutaneous disorder. It was first reported by Haberland and Perou in the year 1970 and hence it is also called as the” Haberland syndrome” and “Fishman’s syndrome”. It is very sporadic in nature with ocular manifestations like choriostoma and subcutaneous papules, dermatological features like subcutaneous lipomas and auricular tags and neurological abnormalities like intracranial lipomas.Thesymptom of ECCL very closely resemblesthat of Goldenhar syndrome and Oculocutaneous syndrome and hence its diagnosis can be challenging.

Case report

A 30 year lady came to the eye OPD with complaints of a small growth seen on her right eye. It has been theresince 2 years, asymptomatic and not increasing in size. On further assessment the visual acuity was found to be 6/6 in both the eyes. Examination of the anterior segment showed a diffuse yellow growth both on the nasal and temporal side of the bulbar conjunctiva more in favour of a lipodermoid. There were small papular lesions seen in the periocular area, near the canthus and along the upper lid margin. Fundus examination was done and it revealed a slightlyenlarged disc with a peripapillarystippling. The left eye appeared to be normal.

A diffuse swelling was noticed in the rightfrontotemporal region. It was subcutaneous in nature, measuring 8*6cm ,soft in consistency with well defined margins. The skin over the swelling appeared normal. It resembled a typical lipomatous subcutaneous swelling.

A skin tag was noticed behind the rightear. More importantly the lady developed a tongue shaped patch of alopecia extending from the forehead near the right frontotemporal region.

A C T scan of the brain was done and it showed dilatation of the right lateral ventricle along with increased CSF fluid.

Acomplete cardiac and neurological evaluation was done and it was normal. The lady had a normal birth history, with normal developmental milestones. No history of seizures in the past

Figure 1 : small papular lesions in the periocular area along with diffuse yellow  growth on the nasal and bulbar conjunctiva .

Figure 2: diffuse swelling in the right fronto temporal region along witha tongue shaped patch of alopecia.

MODIFIED MOOG’S CRITERIA FOR DIAGNOSING ENCEPHALOCRANIOCUTANEOUS LIPOMATOSIS.

SYSTEMMAJOR CRITERIAMINOR CRITERIA
EyeChoriostoma with or withour associated abnormalities1.corneal or anterior chamber anomalies 2. ocular or eyelid coloboma 3. calcification of the globe.
SkinProven nevus psiloliparius(NP)2.Probable NP and ≥one of the minor criteria2-5≥ two of the minor criteria 2- 51.possible NP 2. patchy non scarring alopecia 3.subcutaneous lipomas 4.Focal skin aplasia/hypoplasia 5.skin tags on eyelids
Central nervous systemIntracranial lipomasIntraspinallipomasTwo of the minor criteria1.abnormal intracranial vessels 2.arachnoid cyst /abnormality of meninges 3.completet /partial atrophy of hemisphere 4.a porencephalic cyst 5.dilated ventricles 6.calcification
Others1 .Jaw tumours 2.Coarctation of aorta 3.Multiple bone cysts 

APPLICATION OF DIAGNOSTIC CRITERIA

Definite caseInvolvement of three systems with major criteria >2Involvement of three systems,proven NP or possible NP and >1 minor skin criteria 2-5Involvement of two systems with major criteria >2,one of which is proven NP or possible NP along with  >1 minor skin criteria 2-5  
Probable caseInvolvement of two systems, major criteria in bothTwo systems involved, proven or possible NP.

Based on Moog’s modified criteria4 our patient had one major eye criteria(epibulbarchoriostoma), one major skin criteria(three minor criteria- alopecia, lipomatous swelling in the fronto temporal  region, auricular tags)and one major CNS  criteria( two minor criteria- dilatation of the lateral ventricle, cerebral hemi atrophy).

The lady was followed up again after one year and then after 3 years .The vision still remained 6/6 in both eyes. The anterior and posterior segment findings were thesame as the initial presentation. Eye pressure was measured and as foundto be 15 mm Hg and 12 mm Hg in theright and left eye respectively. Dermatological findings were similar. Thepatientdid not have any systemic abnormalities andwas leading a normal life.

DISCUSSION

ECCL is a very rareneurocutaneous  disorder with varied ocular , dermatological and neurological abnormalities. Though the exact aetiology still remains unknown,studies have suggested that a mosaic rat sarcoma gene (RAS)opathy could be considered3. A group of developmental disorders caused by mutation in the RAS subfamily- mitogen activated protein kinase pathway was responsible. It has also been reported that mutation in thegene encoding for the fibroblast growth factor receptor 1is also a possibility.

It has ocular, dermatological and neurological manifestations of which ocular abnormalities are most common. It includes epibulbarchoriostomas and small periocular skin tags2. Apart from these other findings includeeye lid coloboma, high myopia, pallor of the optic nerve, hypertelorism, epicanthus inversus, , cicatricialectropion of the  eye lid, proptosis due to fatty infiltration of the orbit.

Skin manifestations include subcutaneous lipomas, an area of non scarring alopecia,lipomas in the neck,lumbar,axillaryregion. Periocular papules have also been noted. CNS abnormalities include intracranial lipomas, intraspinallipomas, intracranial cysts, hydrocephalous, atrophy of the cerebral hemispheres.

There have been reports where ECCL have been diagnosed as Goldenhar syndrome until the neurological manifestations were to be seen. If this condition is diagnosed early it will help us to rule out co exiting systemic abnormalities and also start genetic counselling at the earliest5.

In our case the patient was diagnosed to have ECCL based on Moogs’s criteria.  Shewas able to lead  a normal life and do her day to day life activities. Hence it is important for physicians to consider and keep in mind a case of ECCL ,which is very rare though , in patients having mainly alopecia and epibulbardermoids.

BIBLIOGRAPHY

  1. Bieser S ,Reis M et al. Pilocytic astrocytoma in a 3 month old patient with ECCL-  a case study. Am J med. 2015 ;(167 A) : 878-881
  2. Moog U .Encephalocraniocutaneous lipomatosis. J med Genet. 2009;( 46 ):721-729
  3. Boppudi S, HoveHB, Percin EF. Specific mosaic KRAS mutation in ECCL. Clin Genet. 2016;( 90):334-342
  4. Parazzini C ,Tiriulzi F. ECCL – a complete neuroradiologic evaluation .Am J Neuroradiol.1999;(20 ):173-176
  5. Almer Z, Vishnevskia-Dai. Encephalocraniocutaneous lipomatosis. Indian J ophthalmol.2003;( 22 ):389-390

MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

ABSTRACT

To analyse the efficacy of topical nepafenac 0.1% eye drops in the treatment of diabetic macular edema(DME). Method: a prospective interventional study  in 14 eyes having centre involving DME were taken into consideration. They were administered topical nepafenac eye drops 0.1% thrice daily for 6 months. Their vision and foveal thickness were recorded systematically at the start of the treatment and then at 2nd ,4th  and 6 th month follow up.Results: The mean baseline and final LogMAR visual acuity were 0.33 and 0.15 respectively and the mean baseline and final foveal thickness were 450 µm and 290 µm respectively. Conclusion: A statistically significant improvement was noticed in the visual acuity and foveal thickness after the administration of 0.1% of topical nepafenac eye drops.

KEYWORDS: centre involving diabetic macular edema, foveal thickness

INTRODUCTION

Diabetic macular edema is one of the common causes of decreased vision in patients having diabetic retinopathy1. Focal laser was the initial modality of treatment for clinically significant macula edema. Early treatment diabetic retinopathy study (ETDRS) showed that focal laser reduced the risk of visual loss by 50%.But however focal laser treatment is associated with various side effects like scotoma, reduced colour vision, vision loss due to the formation of choroidal neovascular membrane, subretinal fibrosis formation, migration of hard exudates into the foveal centre, enlargement of the laser scars towards the foveal centre.

Hence various other treatment modalities were looked into and evaluated which included intravitreal injections of steroids, agents that target vascular endothelial growth factors(VEGF).Intravitreal injection of triamcinolone for DME was effective for a short period and again was associated with adverse effects like development of cataract, increase in the intraocular pressure as was seen in nearly 50% of the patients. Anti VGEF agents were effective in DME2. But it required repeated injections which carried an increased risk of endophthalmitis. And hence a safer alternative modality of treatment became necessary.

It is known that inflammation plays a key role in the pathogenesis of diabetic retinopathy. Cyclo- oxygenase -2(COX-2) is up regulated in the retina in diabetic individuals which in turn leads to increased production of prostaglandins E2(PGE2) which is an important mediator of inflammation. An elevated PGE2 leads to increased VEGF in diabetic retinopathy and also leads to retinal endothelial cell degeneration.

Nepafenac is a non steroidal anti-inflammatory agent. It is a pro-drug and gets converted to Amfenac by intraocular hydrolases, which inhibits both COX1 and 2.

 In a study conducted on a rat model it was shown that topical nepafenac inhibits diabetes induced retinal micro vascular abnormalities .Topical nepafenac reaches its bioactive concentration in the posterior segment.

METHOD

A prospective interventional case study was done on patients who came to the ophthalmology OPD and was confirmed to have a centre involving DME. A written consent was taken from the patients prior to the procedure. The patients were administered topical nepafenac eye drops 0.1% three times a day.

INCLUSION CRITERIA

1. Diabetic retinopathy patients with centre involving DME, as demonstrated by optical coherence tomography (OCT) .

2. Patients who are not willing for anti VGEF or triamcinolone intravitreal injections.

3 . Patients who were willing for a follow up of 6 months.

EXCLUSION CRITERIA

  1. Any patient who was subjected to intravitreal steroid or anti VGEF injection within 6 months period.
  2. Patients who had undergone focal/grid laser treatment in the previous 6 months.

3.  Vision loss not solely attributed to diabetic retinopathy.

The patients’ vision was assessed with the help of Snellen’ s chart and the anterior segment was evaluated by a slit lamp bio microscope and the posterior segment was assessed by a slit lamp bio microscope with 90D lens and indirect ophthalmoscope. OCT was done for all the selected patients and the central macular thickness was evaluated.

The patients were followed up on the 2nd,4th and 6 th month and during each follow up their visual acuity and CMT were recorded. Snellen’s chart was converted into LogMAR for analysis. Wilcoxon signed rank test for visual acuity analysis and paired T test for CMT were used. The software used was STATA 13.

RESULTS.

In our study 14 eyes were taken into consideration. Figure 1 shows the sex distribution of the patients that had 10 male and 4 female patients having an average age distribution of 56 years as seen in figure 2. The mean duration of diabetes among the patients were 7.4 years(figure 3).Nine eyes were phakic and five were pseudophakic who had undergone cataract surgery atleast 1 year ago. Eleven eyes had moderate non proliferative diabetic retinopathy, one eye had severe non proliferative diabetic retinopathy and two eyes had stable proliferative diabetic retinopathy post panretinal photocoagulation(figure 4). Eight eyes had previously received focal laser treatment and two eyes had received intravitreal triamcinolone injection 8 months before the study.

The baseline mean LogMAR visual acuity was 0.34 and was 0.25 at 2 nd month, 0.22 at 4 th month and 0.18 at 6 th month follow up. There was a statistically significant improvement between the baseline and the final visual outcome. (p= 0.025)(Table 1)

The mean baseline CMT was 465.78 µ and it decreased to 353.18µ at the 2 nd month follow up and to 303.07 µ at the 4 th month and to 290 µ at the 6 th month. There was statistically significant improvement in the CMT from the baseline to the final follow up visit(p=0.0014)(Table 1 )

Figure 1 shows the sex distribution of patients.

Figure 2 shows the age distribution of patients.

Figure 3 shows the mean duration of diabetes

Figure 4 shows the diabetic status of the patients

Table 1 showing a comparative study between the initial and final visual acuity(VA) and central macular thickness(CMT)

Baseline VABaseline CMTFinal VAFinal CMT
6/123806/6303
6/64936/6240
6/66356/6321
6/64206/6236
6/64836/6412
6/122896/6297
6/183156/18276
6/125536/12593
6/123406/9312
6/64046/9236
6/244566/12187
6/604786/18254
6/365676/12189
6/365786/12197

DISCUSSIONS

It is seen that the in diabetic retinopathy there is an increase in the production of prostaglandins. Inhibition of PG production helps to prevent diabetic retinal micro vascular abnormalities3. In a study done by Baudoin et al,he said that aspirin which is an anti-inflammatory agent tends to inhibit the mean yearly increase in the number of microaneurysms but it was contrary to the ETDRS study which revealed that aspirin did not have any beneficial effect on diabetic retinopathy. Another study showed that Celecoxib which is a COX- 2 inhibitor only helps to decrease the fluorescein leakage and has no drastic effect on the visual outcome in DME4.

Nepafenac inhibits both COX1  and COX 25 and has an overall effect on the prostaglandin production, which plays a key role in the pathogenesis of diabetic retinopathy. Since there is most of the time a drop in the vision in patients with DME and the necessity to undergo repeated anti VGEF injections for the treatment of DME, topical nepafenac was considered as a therapeutic measurement. In our study the use of topical nepafenac showed a positive result in the visual outcome. The study showed that 7 eyes had an improvement in the vision, 6 eyes maintained the same visison and 1 eye had a drop despite having a reduction in the CMT. 11 eyes had a decrease in the CMT and 3 eyes had an increase in the CMT during the final follow up. It was noted that none of the patients had any side effects due to topical nepafenac.

LIMITATION

Our study had a limitation as the sample size was small and the systemic control status of the patients was not adequate. Considering the beneficial effect of topical nepafenac in the treatment of centre DME, a larger study group could also be taken and assessed.

CONCLUSION.

Topical nepafenac is a safe and a cost effective option in the treatment of DME. It can be used as a therapeutic optic in cases of DME with good vision and in patients who have multiple systemic co-morbidities such as uncontrolled hypertension, diabetes, recent myocardial infarction, stroke. It is also helpful in conditions where the usage of antiVGEF needs to be contraindicated or used with extra caution.

BIBLIOGRAPHY

1. Bhagat N,Grigorian RA. Diabetic macular edema: pathogenesis and treatment. Surv ophthalmol. 2009 ;(54): 1-32

2. Salam A, Da Costa J. Antivascular endothelial growth factor agents for diabetic maculopthy. Br J Ophthalmol. 2010;( 94):821-826

3. Sennlaub F,Valamnsesh F.Cyclooxygenase- 2 in human and ischemic proliferative retinopathy. Ophthalmology .2003;(10): 198-204

4. DuY ,Sarthy T. Interaction between NO and Cyclooxygenase pathways in retinal cells. Am J Physiol.2004;(67 ):23-39

5.Chew KT,Kim J.Preliminary assessment of Celecoxib in diabetic macular edema. 2010;( 30): 456-457

MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

ABSTRACT

Peribulbar anaesthesia is the most commonly used anaesthetic technique in most of the intraocular surgeries especially in glaucoma and vitreoretinal surgeries. This is a case study of  a 70 year old man who underwent a combined glaucoma and cataract surgery under Peribulbar anaesthesia and developed loss of consciousness and untoward complications of brainstem anaesthesia. Though the complications are very rare with Peribulbar anaesthesia, one need to be extremely careful and should be equipped with the necessary facilities to tackle the life threatening implications’ if any occurs in the operation theatre.

KEYWORDS: Peribulbar anaesthesia, ocular surgeries

INTRODUCTION

Surgeries in ophthalmology are performed under a wide range of anaesthesia techniques. In 1884 it was Sir Carl Koller who used cocaine as a topical anaesthetic agent for the ocular surgery. Later Knapp introduced retro bulbar anaesthesia. However in the late 1980s Sir Davis and Mandel discovered the Peribulbar anaesthesia technique. Since then it has been the most popular and successful mode of anaesthesia for a wide variety of ocular surgeries1. Peribulbar anaesthesia has several advantage when compared to retro bulbar  as it is less painful, less rise in the intraocular pressure, avoidance of a facial block  and most importantly there is minimum sight and life threatening complications like retro bulbar haemorrhage, damage to optic nerve which can cause loss of vision, oculocardiac reflex and brain stem anaesthesia3.In a prospective study done by Davis Mandel in 1994, he reported that though Peribulbar anaesthesia is safe it can still have life threatening complications. The incidence of brain stem anaesthesia was found to be 0.02% after Peribulbar block.

CASE STUDY

A 70 year old male patient came to the eye OPD with complaints of reduced vision. He was not a diabetic and hypertensive and his systemic examination and blood parameters were well within the normal limits.  After a thorough evaluation of the eye, the patient was advised to undergo a phacotrabeculectomy  surgery of the right eye. It was recommended to perform the surgery under the Peribulbar block.

  A 24 gauze  disposable needle was taken for the block. The anaesthetic agent was a mixture of 1 % of Lignocaine and 0.5% Bupivacaine in equal proportion in 50 ml hyaluronidase. The area which had to be injected was cleaned with Povidone iodine. The patient was placed on the OT table in a primary gaze position. The needle was introduced through the skin of the lower lid sulcus in the inferotemporal region. The needle was then inserted in a vertically downward direction along the orbital wall 2.5cm deep. Aspiration was done and the volume of 8 ml of the anaesthetic agent was injected.

It was noted that after close to 5 minutes, the patient’s speech started becoming incoherent and nystagmus was noted in both the eyes. The BP rose to 190/120 mmHg and tachycardia was seen (150 beats/min). The patient was immediately put on an oxygen mask. Since no spontaneous respiration was seen, the anaesthetist intubated the patient and put him on a mechanical ventilator. Simultaneously intravenous injection of Emolol (80mg) was given. In few minutes, the patients BP started to normalise but later it further started dipping down. Injection atropine (1mg) was given to control the hypotension.  After 15 minutes the patient had an episode of a grandmal seizure. Intravenous Midazolam 2mg was given to control the seizure. The patient was monitored extensively and slowly the parameters started to stabilise. After an hour the patient was extubated and was kept under observation. The patient was discharged the following day. The surgery was performed 2 weeks later but this time it was uneventful and the outcome was a great success.

DISCUSSION

When compared to retro bulbar anaesthesia, Peribulbar anaesthesia is a safer technique with fewer complications. Care has to be taken to maintain the ocular positioning of the patients while giving the block. The technique which was followed in our patient was the standard technique according to the literatures.

The doctor was at the head end of the patient while giving the block and the patient was asked to maintain the primary gaze position. However in some cases despite requesting the patients to maintain the primary gaze2, some of them tend to look towards the doctor. As a result the eyeball rolls up and thereby exposing the optic nerve. This makes the nerve more susceptible to the approaching needle. However if the doctor approaches the patient from the side position and patients attempts to look towards the doctor, then in that case there would be rolling down of the eyeball and minimal damage of the optic nerve.

 There are multiple ways and mechanisms as to how local anaesthetic toxicity can occur. In conditions were large volumes of the anaesthetic agent is given , it can lead to light headedness, auditory and visual disturbances, twitching of the facial muscles, seizures followed by respiratory depression and then respiratory arrest. But in our case the volume of the drug taken was very minimal to cause a systemic toxicity. Moreover in our case the symptoms of respiratory failure came ahead of central nervous system excitement or seizures.

In scenarios where  the anaesthetic agent4 is directly  injected into the meningeal coverings of the optic nerve and gains access to the subarachnoid space it can lead to respiratory depression prior to CNS manifestations but then the initial cardiovascular events cannot be explained. But however accidental intra-arterial injection can lead to a rise in the level of the anaesthetic agent in the brain via retrograde flow through the internal carotid artery. And this can cause the cardiovascular manifestations like rise in pulse and BP. This is very temporary as observed in our case. But once the drug starts redistributing out of the brain, the symptoms tends to wane off.

Hence in our case the initial rise in BP and pulse could indicate an inadvertent intra arterial injection of the LA while the respiratory arrest and seizure could be due to injection of LA into the meningeal coverings. In cases of brain stem anaesthesia5 the signs and symptoms will start within 5 to 10 minutes after the block and complete recovery is gained within an hour. In our case study the patient had respiratory complications occurred in less than 5 minutes with altered consciousness. However the patient regained consciousness and was stabilised within an hour.

CONCLUSION

Complications with Peribulbar anaesthesia are quite rare. But it shows that one needs to be handy and keep the operation theatre well equipped along with trained personnel to handle the complications and emergencies.

BIBLIOGRAPHY

  1. Davis DB,Mandel. Posterior Peribulbar anaesthesia: an alternative to retro bulbar anaesthesia. Indian J ophthalmol .1989;(37):59-61
  2. Eke T,Thompson JR. Complications for local anaesthesia for cataract surgery. Br J Ophthalmol. 2007;( 91): 470-475
  3. Hessemer V. Peribulbar anaesthesia vs retrobulbar anaesthesia. J Catact Refract Surg. 1994 ;(20): 327-337
  4. Berde CB, Strichatz. Local anaesthetics. Indian J OPhthalmol.2015;(24): 1047-1052
  5. Gomez RS,Andrade LO. Brainstm anaesthesia. Can J Anaesth.1997;( 44): 732-745.

MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

ABSTRACT

Aim: To study the association between the incidence, risk factors and treatment outcomes in retinopathy of prematurity.

Methodology: A longitudinal observational study was done among 70 babies who fulfilled the criteria for ROP screening at a tertiary care center.

Results: Out of the 70 babies that were screened at our hospital, 25 babies developed some stage of ROP with 10 babies developing Type 1 ROP. It was noted that the mean birth weight, mean gestational age and the duration of oxygen therapy was significantly associated with the development of ROP (P= 0.045,P<0.001 and P<0.001 respectively). Of the 25 babies with ROP, zone 3 was involved in 14 babies and zone 2 was involved in 11 babies.7 babies had stage 1,10 had stage 2 and 8 babies had stage 3. Of the 25 babies with ROP , 12 babies required treatment with laser photocoagulation and all the babies showed regression of ROP following treatment.

Conclusion: Birth weight less than 1.70 kg, gestational age <32 weeks and oxygen therapy were associated with the development of ROP and ROP regressed with laser treatment,

Keywords: laser photocoagulation, retinopathy of prematurity, laser.

INTRODUCTION:

In a developing country like India, the incidence of ROP is between 38% to 52.3 % among low birth weight babies.  And it is estimated that out of 27 million annual births in India, nearly 2 million babies are less than 2000 grams in weight and are more prone to develop retinopathy of prematurity (ROP). However with the recent advances in the neonatal care, the survival rates have increased significantly.1

ROP is one of the most common causes of blindness in preterm babies. Initially it was considered that oxygen supplementation is an important risk factor for the development of ROP. However ROP can develop without oxygen supplementation and babies who have received oxygen need not necessarily develop ROP2.  This suggested that there are other risk factors that can lead to ROP such as an early gestational age, low birth weight, apnea, hyperoxia , intraventricular haemorrhage, blood transfusions and maternal bleeding. 

Following a severe ROP it can lead to an impaired vision, large refractive error mainly myopia, strabismus and in some cases it can even lead to potential blindness3. Treatment for ROP mainly includes cryotherapy, laser photocoagulation and intravitreal injection of anti VGEF.Though  each one has its own merits and demerits, they have been found to be effective in the regression of ROP4.

AIM: to assess and study the incidence, risk factors and treatment outcomes in ROP.

OBJECTIVES:

1. To assess the incidence of ROP

2. To find out the various factors that influence ROP.

3. To assess the outcome after the treatment of ROP.

METHODOLOGY– A  prospective longitudinal study was done on preterm babies  in the neonatal intensive care unit of our hospital. The study was approved by the ethical committee. Informed consent was taken prior to the study.

Inclusion criteria

Babies with a birth weight less than 1700 g, gestational age <34 weeks at birth, exposure to oxygen, multiple gestations, respiratory distress syndrome , sepsis, intraventricular hemorrhage ,maternal complications like gestational diabetes mellitus, gestational hypertension, ante partum hemorrhage was identified and taken into consideration.

A detailed obstetric history and postnatal course was noted. For fundus evaluation, the pupils were dilated using half strength tropicamide 0.8 % with phenylephrine 5 % eye drops by diluting commercially available drops (AUROMIDE PLUS) with tear substitutes.  The fundus examination was done using indirect ophthalmoscope and with+28 D lens by a vitreoretinal specialist.

High risk babies and babies with features of ROP were monitored at weekly intervals. Those babies who required treatment were subjected to laser photocoagulation with an 810 nm diode laser. The follow up of those babies who underwent treatment were done at weekly interval until the ROP regressed or the retina matured.

The statistical analysis was done using the SPSS version 2.0 .The continuous variables were expressed in terms of percentage, mean, standard deviation and analyzed with the chi square test. Pearson’s correlation was used to compare the risk factors and its effects on the treatment outcome.

RESULTS:

Of the 70 preterm babies that were screened,25(35.7%) babies  develop ROP and 45(64.2%)babies did not develop ROP. The mean birth of the babies with ROP was 1.64±0.53 kg and in babies without ROP was 1.93±0.47 kg The mean gestational age in babies with ROP was  30.87±2.53 weeks and in babies without ROP it was 34.21± 1.79 weeks. The mean duration of oxygen therapy in babies with ROP was 12 days and in babies without ROP was 3.45 days.

It was noted that there was a significant relationship between the occurrence of ROP and birth weight(0.047), GA (<0.001), duration of oxygen therapy(<0.001), post conceptional age(0.002) and respiratory distress syndrome. However in this study it was seen that the relationship between ROP and the maternal risk factors such as antepartum haemorrhage, GDM,GHTN and multiple pregnancies was not significant. Out of the 70 babies screened,25(35.7%)developed ROP 4 babies(16%) had stage zone 2 stage 1,2 (8%) had zone 2 stage 2 and 5(24%) had zone 2 stage 3 .3 babies(12%) had zone 3 stage 1,8(32%) had zone 3 stage 3 and 3(12%) babies had zone 3 stage 3 .12 babies  had plus disease. There was no case of stage 4 or stage 5 of ROP.

Table 1 – relationship between retinopathy of prematurity and the risk factors.

parametersWith ROPWithout ROPP
Birth weight1.64 ±0.53 kg1.93±0.47kg0.047
GA30.87±2.53 weeks34.21±1.79 weeks<0.001
Oxygen therapy12 days3.45 days<0.001
Post conceptional age35.33± 3.54 weeks39.27±5.66 weeks0.002

Table 2 – Other risk factors associated with retinopathy of prematurity.

 With ROP (n=25)Without ROP (n=45)
Intraventricular hemorrhage1(4%)0
Gestational diabetes mellitus2(8%)1(2%)
Gestational hypertension2(8%)7(15.5%)
Ante partum hemorrhage2(8%)2(4.4%)
Twin pregnancy03(6.6%)
No maternal risk factors16(64%)27(60%)
Respiratory distress syndrome.15(62.5%)11(24.4%)

  Table 3 -Stages of retinopathy of prematurity

ROP STAGEN=25
Zone 2 stage 14(16%)
Zone 2 stage 22(8%)
Zone 2 stage 35(24%)
Zone 3 stage13(12%)
Zone 3 stage 28(32%)
Zone 3 sage 33(!2%)

Table 4 – Treatment of retinopathy of prematurity with laser photocoagulation.

With ROP(n=25)Treatment (n=12) With Plus diseaseNo treatment(n=13)
Zone 2 stage 130
Zone 2 stage 220
Zone 2 stage 360
Zone 3 stage111
Zone 3 stage 208
Zone 3 sage 304

 Out of the 25 babies with ROP , 12 babies required intervention with laser photocoagulation. 3  babies in zone 2 stage 1, 2 babies in zone 2 stage 2 and 6 babies in zone 2 stage 3 while 1 baby in zone 3 stage 3. All these 12 babies had plus disease and they showed improvement on follow up. The other 13 babies regressed spontaneously without any intervention.

DISCUSSION:

In our study it was found out that among the various risk factors that are responsible for ROP, low birth weight was the most common followed by duration of oxygen therapy and low GA. Our study was in correlation with various other studies done previously. Although multiple gestations, GDM , GHTN and interventicular hemorrhage were considered as risk factors for the development of ROP, it was not significant. In a study conducted by Ameen et al, it showed that multiple gestation as an independent risk factor for the development of ROP5. In our study it was seen that zone 3 stage 2(32%) was the most common followed by zone 2 stage 3 (24%).This could be attributed to the meticulous screening protocols and our cohort mainly had older babies. In our study it was noticed that zone 2 was the most commonly involved area. Stage 4 or 5 of ROP was not seen as all the cases were screened regularly in a timely fashion.

In our study, out of the 25 babies with ROP,12 required intervention with laser photocoagulation. It was seen that laser photocoagulated eyes showed a regression of the disease and the results were excellent. Other studies have also shown that laser therapy has a better outcome7.  A study done by Erick Dan compared the efficacy of laser with anti vascular growth factor as it has been used in severe forms of ROP6. It was concluded that Bevacizumab gives good results in stage 3 + ROP in zone 1 but not in zone 2. However in our study we have not compared the treatment outcomes with Bevacizumab as there were no babies with zone 1 ROP8.

Sine ROP is essentially asymptomatic is the early stages, it is very crucial to do a timely evaluation of the retina in infants that are at a risk of developing ROP to prevent any unseen complications9.

Limitation of our study was that a small sample size was taken and a single center study

CONCLUSION

The incidence of ROP among preterm babies was 37%. The prominent risk factors for the development of ROP were low birth weight, prematurity, duration of oxygen therapy and respiratory distress syndrome. Among the treatment choice, laser photocoagulation was found to be effective.

No financial support and sponsorship.

BIBLIOGRAPHY

1. Sen p, Rao G. Retinopathy of prematurity. Sci J Med.2018;vol(23):112-345

2.Jalala S,Anand R. Screening strategy in ROP. Indian J Ophthalmol. 2008;vol(2):76-90

3.Terry T.Eye changes in premature babies. Trans am ophthalmol. 2012,vol(14):556-667

4.Zubun D’sa, Bansal H.Risk factors for retinopathy of prematurity. Med Arch 2015; vol(20):77-90

5.Owen T,Lawrence J. Current concepts of oxygen management in ROP. J OphthalmicVis Res .2017;vol(20):45-78

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MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

ABSTRACT

Aim: To evaluate and analyze the outcome of preoperative subconjunctival Mitomycin C to conjunctival auto grafting using fibrin glue with respect to the recurrence rate and complications.

Methodology:  A prospective randomized hospital based study was conducted. 60 eyes were taken into consideration with primary progressive pterygium and were randomly divided into 2 groups of 30 each. In group A subconjunctival injection of Mitomycin C (MMC) 0.1 ml of 0.1 mg/ml was given 1 month before bare sclera technique and in group B pterygium excision with conjunctival auto graft using fibrin glue was performed. They were regularly followed up for 18 months.

Results: The mean age of the patients in this study was a 39.6±12.3 year with females outnumbering the males. The mean follow up period of group A was 10.6 ±5.44 months while in group B it was 11.2 ±4.49 months. The average surgery time was more in group B than compared to Group A. Both the techniques were not associated with any vision threatening complications.

Conclusion: Administration of subconjunctival injection of MMC 1 month before the bare sclera technique is considered to be safer, economical, less time consuming, technically less demanding and as effective as conjunctival autograft with fibrin glue.

Keywords: pterygium, bare sclera, MMC, auto graft.

INTRODUCTION

Pterygium is an elastotic subconjunctival degenerative fibro- vascular tissue proliferation. It is more commonly seen among those who are exposed to dry hot climatic conditions and ultra violet radiation exposure. Surgical intervention is indicated only if the individual has severe irritation, recurrent inflammation, obstruction to the visual axis, induced astigmatism, restriction in the motility and cosmetic blemish1.

Due to its high recurrence rates (30-70%), adjuvant modalities are required such as radiation, antimetabolites, conjunctival grafts and limbal grafts to reduce its recurrence. Among them it is observed that the best available option to prevent recurrence after pterygium excision is conjunctival autograft, where in after the excision of the pterygium a graft is fixed with the help of sutures or fibrin glue and autologous blood2.

Usage of Mitomycin as an adjunctive was first described in the year 1963 in Japan by Kunitomo and Mori. Since then various modifications have been made to use them either preoperatively or intra operatively. However the long term usage of Mitomycin C eye drops can lead to various complications such as secondary glaucoma, corneal edema,scleral necrosis, sudden onset of mature cataract thereby limiting its usage to a single intraoperative  application3. In a study done by Donnenfeld et al he reported a success rate of 94% with subconjunctival injection of MMC 1 month before the pterygium excision with bare sclera technique. It was observed that it allows the exact titration of MMC delivery to the activated fibroblasts and minimizes the epithelial toxicity.

The purpose of this study was to analyze if a simple and easy technique like subconjunctival MMC before pterygium excision by bare sclera technique can give  results comparable to conjunctival autograft with regards to its recurrence rates. To observe if subconjunctival injection of MMC is associated with complications such as ocular toxicity that is seen with intra operative application of MMC.

Materials and methods:

A prospective randomized hospital based study was conducted for a period of 12 months. In this study 60 eyes were taken who had primary progressive pterygium. In this study 12 patients had bilateral pterygium of which 5 were wiling to get both their eyes operated and hence one eye was allocated in group A and the other in group B. In the remaining 7 patients only one eye was included for the study. Approval to conduct the study was obtained from the ethical committee and a prior consent was taken from all the patients.

 A detailed preoperative eye examination was done for all the patients. Following the procedure the patients were regularly followed up from 6 to 18 months.

Inclusion criteria

All patients less than 50 years of age having primary progressive pterygium and willing to undergo the procedure were taken into consideration.

Exclusion criteria

Patients having atrophic pterygium, recurrent pterygium, dry eye syndrome, collagen vascular diseases, co existent conjunctival diseases like previous alkali  burns, Mooren’s ulcer which can predispose to pseudo pterygium, history of uveitis, scleritis, glaucoma and those who could not come for a regular follow up were excluded from the study

The patients were divided into 2 groups, Group A and Group B

In group A subconjunctival injection of MMC 0.1 ml of 0.1 mg/ml was given 1 month before the bare sclera technique. And in group B pterygium excision with conjunctival auto grating using fibrin glue was done. In order to reduce the cost in Group B, a group of 5 to 6 patients were made and each group was operated with fibrin glue on the same day.

Under aseptic precautions 0.5% proparacaine was instilled and with a30G needle on a tuberculin syringe0.1ml of 0.1 mg/ml of MMC  was injected into the body of the pterygium approximately 1.5 mm away from the limbus under an operating microscope. A sterile cotton bud was placed on the injection site for a few seconds to prevent the regress of MMC. After the injection, the conjunctival sac was washed with normal saline to wash out the excess MMC. The patient was advised to administer Ofloxacin 0.3% eye drops one drop 4 times a day for the next 4 days. The patient was reviewed on day 1 , after 1 week and after 1 month .  During the follow up period a complete ophthalmic examination was done including fluorescein staining. One month after the MMC injection, the patients were subjected to removal of the pterygium by the bare sclera technique. The eye was prepared  and draped in a sterile manner. A Peribulbar block was given using 5 cc of 2% xylocaine and 1:200000 adrenaline. The eye speculum was applied. The neck of the pterygium was grasped with the help of Saint Marin’s forceps and the head was dissected from the cornea with the help of conjunctival scissors following which the sclera portion of the pterygium was excised. A thorough removal of the subconjunctival fibrous tissue was done. The scleral bed and the cornea was polished with the help of a 15 surgical blade.  Antibiotic steroid combination ointment (polymyxin B sulfate 10000 units+ chloramphenicol 10 mg + dexamethasone sodium phosphate 1 mg/g ointment ) was instilled  and a sterile eye pad was applied. The time taken for the entire procedure was noted. 

In group B, the initial steps till the polishing of the corneal and scleral bed are the same, as in group A. The vertical and horizontal extents of the bare sclera was measured with Castroviejo’ s measuring caliper. And a free conjunctiva limbal graft of the same size was taken from the superotemporal conjunctiva. In cases where the exposure of the donor site was poor, a superior rectus bridle suture was applied  to fix the eye in the down gaze to get a better exposure of the donor site. The dissection was started from the forniceal end and brought towards the limbus. During this process extreme care had to be taken to avoid button holing of the graft and involvement of the tenon capsule. Once the graft reached the limbus , it was flipped on to the cornea and the teno’n s attachment at the cornea was meticulously dissected, the graft was then cut at the limbus  and once again flipped over the cornea  so that its tenon’ s surface faces the cornea. With the help of a duploject, fibrin glue was applied over the bare sclera and the conjunctival autograft was immediately flipped over the conjuctival defect. Proper care was taken to not disturb the orientation and the sides of the graft were opposed to the edges of the receipt conjunctiva. After a period of 3 minutes which was given for drying ,the lid speculum was removed, antibiotic steroid eye drops (oflocacin +dexamethasone) was instilled and a sterile eye pad was applied. The duration of the procedure was noted. The eye patch was removed after 6 hours followed by instillation of the antibiotic steroid eye drops 4 times a day for 15 days. Follow up of  both the groups were done at 1 week, 1 month, 3 months, 6 months,12 months and 18 moths post operatively.

Statistical analysis was done using SPSS version 2.0.The age of the patients , surgical time, follow up time of patients in group A and B were compared. Simple Z test was used to compare the recurrence and complications between group A and group B and p value < 0.05 was considered statistically significant.

RESULTS:

8 (13.33%) patients were in the age group of 21-30 years, 25 (41.6%) were in the age group 31-40 years and 27(45%) patients were in the age group 41 to 50 years. The average age of the patients in this study was 39.6±12.3 years. Out of the 60 patients in the study group, 22 (36.66 %) were males and 38 (63.33%) patients were females. Among the patients included for the study 26 (43.33%) patients had an indoor occupation while the remaining 43 (56.66%) of the patients had an outdoor occupation. The average surgical time taken for bare sclera with preoperative subconjunctival MMC was 16.77±1.73 minutes   while in group B where conjunctival autografting with fibrin glue was done, the average time taken was 23.36± 1.53 minutes.

Table 1 : Shows the recurrence rates of the two techniques used in this study

GroupSurgical TechniqueNumber of eyes operatedNumber of eyes with recurrence
ABare sclera with preoperative MMC301(3.4%)
BConjunctival autograft with fibrin glue302(6.7%)

Table 2: To compare the complications in group A and group B

ComplicationsGroup AGroup BP value
Conjunctival congestion30(100%)30(100%) 
Subconjunctival hemorrhage4(13.33%)1(3.3%)>0.05
Conjunctival granuloma2(6.7%)1(3.3%)>0.05
Graft edema 2(6.7%)>0.05
Graft hemorrhage 2(6.7%)>0.05
Graft loss 1(3.3%)>0.05

DISCUSSION:

Though pterygium excision is a very simple procedure but the only drawback and main concern is its recurrence rate. It is assumed that the surgical trauma and post operative inflammation activate the subconjunctival fibroblast which causes proliferation of the fibroblasts and deposition of extracellular matrix which causes the recurrence of the pterygium4.

However conjunctival autograft helps to reduce the recurrence rate by the presence of limbal stem cells which helps to restore the limbal barrier.But the drawback of conjunctival autografting is that it can adversely affect the outcome of future glaucoma filtration surgeries if ever required and it is of limited use with a large double headed pterygium and scarred conjunctiva as enough donor conjunctiva tissue might not be available.

The usage of fibrin bioadhesive in conjunctival limbal autograft surgery simplifies the surgical technique, shortens the duration and has lesser postoperative complications5. Though cost is a concern the average cost decreases with increasing number of patients scheduled for the surgery on the same day. But for this we need to get an adequate number of patients on the same day which might increase the waiting period for the patients which is not a practical and feasible solution.

Recurrence of pterygium is common among younger patients6. In our study the youngest patient was 23 year old and the oldest was 48 years old. The average age of the study was 39.6 ±12.3 In a study done by Gazzard et al, the prevalence rates of pterygium in subjects over the age of 51 years were 6 times than that  between 21-30 years of age. A study conducted by the Kim et al showed that 39.3 % were males and 66.5% were female which was similar to our study where the females outnumbered the males. In a study done in Meiktila it was found that the rates of pterygium and pingucuela in rural residents were more than five times as high as in urban residents and it was primarly as a result of ocular sun exposure.

The average surgical time taken for bare sclera with preoperative subconjunctival MMC was 16.77±1.73minutes. The average surgical time for conjunctival autografting with fibrin glue was 23.36±1.53 minutes. It was observed that the time taken for conjunctival autografting using fibrin glue was significantly more than that of bare sclera with preoperative MMC. . In our study the recurrence rate was 3.4% in group A at the end of mean follow up of 10.6±5.44 months.  And in group B it was 6.7 % at the end of the mean follow up of 11.2±4.49 months. Recurrence rates after conjunctival autografting with fibrin glue in various studies ranges from 4 to 12 %.

All patients had conjunctival congestion postoperatively due to surgical trauma which subsided with topical antibiotics- steroid eye drops,  4 patients in Group A had sub conjunctival hemorrhage after injection of MMC which cleared in 5 days. No patient had any sign of conjunctival or corneal staining after injection of MMC. 2 patients had conjunctival granuloma at 1 week and at 1 month post operatively after the bare sclera technique. No patient had vision threatening complications s during the study period like glaucoma, corneal edema, corneal perforation, scleral melting  and cataract which are usually associated with intraoperative application of MMC. In group B it was observed that after conjunctival autografting  using fibrin glue 2 patients had graft edema and 1 patient had hemorrhage under the graft after the first post operative day which cleared up with topical steroid drops. One patient had donor site granuloma on the 1 st week post operatively which resolved with topical steroids and lubricating eye drops.

CONCLUSION:

It was found out that the recurrence rate with subconjunctival MMC before bare sclera technique was 3.4 %while the recurrence rate with conjunctival autograft using fibrin glue was6.7%. Subconjunctival MMC 1 month before the bare sclera technique is simple, safe, economical, less time consuming, technically easy and as effective as various other techniques7. It is useful in patients with recurrent pterygium, large double headed pterygium, patients with glaucoma who require filtration surgery or combined cataract and pterygium operation.

It can be concluded that subconjunctival MMC 1 month before the bare sclera excision has a potential to replace other methods of management. However a longer follow up period along with a larger sample size are needed to further establish the safety and efficacy of preoperative MMC in pterygium surgery.

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