R O Y A L A C A D E M Y OF MEDICINE IN IRELAND - SECTION OF O P H T H A L M O L O G Y

Proceedings of meeting held on l l t h June, 1988.

OPTIC NERVE INJURY IN CHILDHOOD

A. Blake, S. Blake, H. O'Donoghue and P. Eustace.

Two boys were sere with seriom optic nerve injury following appanmtly trivial trauma. The first fell from a tree and suffered a small puncture wound in the lower eyelid from a twig. Final vision was counting fingers. The second fell from lab bicycle scraping the side of his face but without slgnificant head injury. Final visual acuity in the second case was 3/60. Magnetic re.sonanee imaging was performed in both cases but did n ~ show any injury to the optic nerve though both boys had relative afferent pupillary defects in the aff~ted eye. The injury in each case was milateral.

Children are particularly susceptible to this type of ocular injury. It is postulated that the injury is principally to the blood supply to the optic nave which is especially fragile. The traumatic force may be rotatory as in the fn'st case where the branch acted as a toning agent after puncturing the eyelid. The mobile globe is turned on a relatively fixed nerve.

A second theory is that the coup-coutrecoup ddect may rapture the vessels at the junction of the fixed nerve with free: the oldie foramen. The cases of these two young males typify the optic nerve injury in childhood.

RECENT EXPERIENCE WITH BOTU- LINUM TOXIN-A IN THE ROYAL

VICTORIA EYE AND EAR HOSPITAL, DUBLIN

IC Coleman, P. Mullaney, P. MoriaRy.

Botufinum toxin-A binds rapidly and avidly ~ the presynaI~ic nerve membrane of the peripheral cholinergic synapses. Here it causes widespread muscular paralysis by reducing the sensitivity to the neurotransmitter re.lease mechanism to ace- tylchdine. In 1973 Scott lust instituted the use of the toxin for human therapy and in November, 1986, the first patient was treated in the Royal Victoria Eye and Ear Hospital. We reviewed our experience with fifteen patients who received toxin (BTX) from November, 1986, to April, 1988, with paRicular interest in indications, ef- fect and duration of treatment and side effects of therapy.

Patients were treated with 0.008 gg neurotoxin (in 9 mis. saline), and site of injection was de- pendent on the indication. In general, all patients received initial subcutaneous injections of 0.2 mis. laterally and medially at the junction of the od~tal and preseptal orbicularis ocul/ in both upper and lower lids. The processems was in- jeered where indicated and mbsequent treatme~lt was modified according to the response.

There were 11 female and 4 male patients. Ages ranged from 28 to 81 years with a mean of 59.5 years. 7 females suffered essential blepha- mqaum including two cases of Meig's Syn- drome with oromandibular dystonia. Both these

cases had histories of thyrotoxicosis. Two males and four females presented with hemifadal Sl~Sm triggered from the outer canthus. Two young males aged 28 and 32 years had shoN histories of hemifacial myokymia.

Relief was complete in seven of the fourteen patients after the first injection. Three required BTX in seven months, two in nine months and two in one year. Side effects included 11 cases of Ptosis, asymmetrical in three cases and usually resolving in one to three months. There were three cases of diplopia, four cases of incomplete closure, two cases of epiphora, two cases of ectropion and two patients suffered reading dif- ficulties due to tear meniscus on the immobile lower lid.

In summary, fifteen patients were successfully treated. Duration of relief does not appear to be related to age duration or severity of blepharo- spasm. In the future side effects may be dimin- ished with concomitant use of an antitoxin.

THE HYDROGEL LENS

P. Kenna and D. Mooney. Research Unit, Royal Victoria Eye and Ear Hospital, Dublin.

Iogel ('I'M) (ALCON) hydrogd lenses were implanted in tm patients (mean age seventy- seven years) between September and December 1987. The results were compared with those of a prospective worldwide study being conducted by Barret et ai (601 patients).

Hydrogels are polymeric materials which swell greater than 20% in contact with water. They have a number of theoretical advantages com- pared with P.M.M.A., i.e. pliability, less endothe- lial cen loss following touch, less uveal irritation and autoclavability.

All our patients had extracapsular cataract ex- traction using the endocapmlar technique. Diffi- culties were encountered in three cases, all in- voiving placement of the upper pole of the lens and resulting in ~ damage and ripping of the anterior capsule.

Best corrected visual acuity after three to six months was 6/12 or better in nine out of ten patients. Nine out of tea eyes were remarkably quiet post-qperatively. One eye suffered a tran- sient sterile hypopyon.

Pre-operative and six mouth post-operative endothelial cell counts were pefformud on nine out of um patients. The average cell loss was 30.54% (0% -~ 37.11%). This was attributed to difficulties in placement of the lens, resulting from the lens length being 12 n u n - greater than that of the crystalline lens, which is I0 mm in diameter.

The Iogel lens, in our hands, was found to offer excellent visual results, with few significant post-

operative complications, apart from an unexpect- edly high endcthelial cell loss. It d f e r s promise for the future, particularly when combined with phacoemulsilication.

ACANTHAMOEBA KERATITIS

E. Bormar, P. Logan and R. Hone. Mater Misericordiae Hospital, Dublin.

A case was presented of Acanthamocba Ker- atitis which responded well to medical treatment.

The patient, a 26 year old corporation worker, developed a painful red eye after a puff of cement dust which was washed out with water. After an initial diagnosis of Herpes Simplex Keratitis the development of a partial ring infiltrate in the cornea aroused suspicion of Acanthamoeba Ker- atitis; this was confirmed by culture and sub- culture of corneal scrapings on special media, Acanthamoeba Polyphaga being identified.

Initial treatment was with topical propamide isethionate (Brolene), neosporin and miconazole nitrate. Later betnesol and atropine were added. Hospitalisation was required because of severe painful anterior segment inflammation, with a complete stromal ring infdtrate and vision re- duced to C.F. Improvement began after 3 weeks and has continued, to give a painless white eye, mild stromal nebula and vision =6/12.

Acanthamoeba is a genus of free living amoeba, almost ubiquitous, and with active trophozite and dormant cystic phases. Of four species, Acantha- mocha Castellani is the commonest isolated ink- eratitis. Non-ocular infestations can cause meningo-encephalitis, sinusitis in AIDS, and ear infections. The f'LrSt case of Acanthamoeba Ker- atitis was reported in 1973, and since then a total of 68 cases has been documented. Common as- sociations are, minor trauma, contact with water, and the wearing of soft contact lenses, partial or complete paracentral stromal ring infiltrate and severe pain. Late cases progress to severe kerati- tis, hypopyon, secondary glauooma, and cataract. Most reported cases have required comeal graft- ing, with frequent recurrences in the graft.

Our patient is on maintenance therapy of Neosporin, Brolene, Miconazole and Betnesol and continues to show a sustained response.

MULTIPLE ENDOCRINE NEOPLASIA AND PROMINENT CORNEAL NERVES

M. Hope-Ross,* F. Breathnach,** A. O'Meara** and J. Walsh.* *Research Foun- dation, Royal Victoria Eye and Ear Hospital,

Dublin; **Our Lady's Hospital for Sick Children, Dublin.

A I0 year old girl was referred for assessment of prominent corneal nerves. A diagnosis of multiple endocrine neoplasia Type IT B had been made previous]y.

In her past medical history, she had received treatment for left talipas equinovams and consti- patio~ as an infant. She was referred to Our Lady's Hospital for sick children, at the age of eight, when biopsy of a cervical lymph node revealed metastatic carcinoma. Examination revealed an abnormal facies, with full,fleshy lips,

24

Vol. 158 No. 1

neuromas of the tongue, scoliosis, high arched palate and arachnodactyly. Investigations re- vealed elevated serum calcitonin and a cold thy- roid nodule. A diagnosis of multiple endocrine neoplasla Type H B, with metastatic meddlary carcinoma of the thyroid was made. A total thy- roidectomy and unilateral block dissection of the neck was performed. She has remained well since. Screening of her family has shown that none at present are affected.

Ocular examination revealed prominent cor- neal nerves. Pupil response to topical mydriatics was poor. The tear f'dm and eyelids w e ~ normal.

Multiple endocrine neoplasia Type H B, or mucosal neuroma syndrome, is characterised by medullary carcinoma of the thyroid, phaeochro- mocytoma, ganglioneuromatosis, connective tis- sue and skdetel abnormalities.

Ocular involvement is manifest by prominent corneal nerves, keratoconjunctivitis sicca, con- junctival neuromas, and pupil abnormalities.

ORBITAL LYMPHANGIOMAS (A REVIEW OF FOUR CASES)

G. N. Kervick, W. C. Logan and D. B. Archer.

Lymphangiomas around the eye are uncom- mon. They are congenital benign slowly progres- sive tumours of the lymph vascular system.

They have been classified into three types de- pending on their location, either superficial, deep or combined. Orbital lymphangiomas frequently haemorrhage. This haemorrhage may occur spontaneously or follow accidental or surgical trauma. Often this haemorrhage is disproportion- ate to the apparent minor degree of injury. The bleeding may be mild as evidenced by the appear- ance of a subeonjunctival haemorrhage, or se- vere, causing a rapid increase in proptosis which may on occasions affect the blood supply to the optic nerve with resultant decrease in vision.

The four patients we reviewed, together illus- trate well the spectrum of disease that may be found in the condition. These include acute pruptosis, spontaneous subeonjunctival haemor- rhage, exophthalmos, often intermittent and oc- casionally varying with upper respiratory tract in- fections, recurrent ecchymoses, muscle imbal- ance, ptosis and enlargement of the affected orbit. The most serious feature was the complete loss of vision in one patient from rapidly progressive pruptosis.

We would suggest that in a young child the onset of acute pruptosis without a preceding history of trauma should suggest the diagnosis of orbital lymphangioma and appropriate measures to surgically decompress the haematoma should be undertaken without undue delay. More per- manent removal of the lesion can be achieved as an elective procedure at a later date through an orbitotomy incision using either carbon dioxide laser or surgical incision

PANCREATIC ISLET AUTOTRANS- PLANTATION PROTECTS AGAINST

DIABETIC RETINOPATHY AT 18 AND 27 MONTHS IN THE DOG

I". M. Walsh t, D. Mooney 2, S. TraversL De- partment of Surgery, Mater Misericordiae Hospi-

Royal Academy of Medicine in Ireland 25

taP, and Research Department, Royal Victoria Eye and Ear Hospital 2, Dublin.

The development of diabetic retinopathy is re- lated to persisting metabolic abnormalities dur- ing conventional insulin therapy (Doman, T., Mann, J. I., Turner, R. Br. Med. J. 1982: 285, 1073). Dispersed islet graft transplantation cor- rects fasting and poa prandial insulin, glucose and intermediary metabolic profiles and may prevent the secondary complications of diabetes. This study examines the effects of islet autotrans- plantation on the retina in successful recipients at 18 months (N=5) and 27 months (N=2).

Six mongrel dogs weighing between 12 and 24 kg underwent total pancreateetomy and a dis- persed islet autograft was prepared and trans- planted as described elsewhere (Alderson, D., Walsh, T. N. and Famdon, J. R. Br. J. Surg. 1984: 71,756-60). Blood glucose was assayed weekly and glycosylated haemoglobin assayed monthly. All animals were followed up for 18-27 months or until graft failure. Fluorescein Angiographic studies of the retina were performed at 18 months (5 dogs) and repeated at 27 months (2 dogs). (Three grafts failed between 18 and 27 months).

angiography was performed under general an- aesthesia with 1% trupicamide and 10% phenyle- phrine to dilate the pupils, using a Zeiss 30 ~ fundus camera fired with spect/x3tech exciter/ barrier filter combination on Kodak Tri X Pan film. Films were compared with age matched controls. Fhiorescein angiograms at 18 months and at 27 months were normal showing no evi- dence of fluorescein leakage or microaneurysm formation. In the three animals with grafts that failed between 18 and 27 mbnths the absence of changes was confirmed histologically. In conclu- sion failure to detect retinal changes at 18 or 27 months by fluorescein angiography suggests that successful islet transplantation may prevent or delay diabetic retinopathy.

about a week. The condition appears to be more common in negroes but the association with Down's syndrome is largely confined to cauca- sians who are often found to have other minor lid abnormalities.

PSEUDO-PARTIAL 11 lrd CRANIAL NERVE PALSY

M. P. HiUery, A. Benedict-Smith, W. Power. L M. T. Collum and P. Eustaee

The direct toxic effects of therapeutic pharma- cological agents on the myoneural junction has been documented for some time. In 1975, Peni- cillamine Hydrechloride was described as induc- ing a myasthenic syndrome in some patients due to a defect in neurotransmission at the pre-synap- tic level, the diagnosis of which can be difficult

A 48 year old lady with severe daeumatoid ar- thritis presented with a five week history of diplopia. This was initially transimt, but at presentation she appeared to have a left partial third cranial nerve palsy with Impll sparing. In- vestigation including an Edrophonitma Chloride test was negative. C.T. scan of the brain showed a small infarct in the right temporal lob~ A presumptive diagnosis or partial third nerve palsy secondary to vasculitis was made.

During tmdergraduate examinations, one of the examiners (P.E.) suggested that the patient was suffering from ocular myasthenic syndrome induced by Penio/Uamine. This was discontinued and the signs resolved over the next few months.

This case serves as an uncommon imposter of cranial nerve palsy and reminds us that care should be taken when prescribing penicillamine since the myoneural junction effect can be poten- tiated by other pharmacological agents; often the Tensilon test is negative; there is a variable re- sponse to anticholinesterase therapy and wound healing is impaired by the drug.

CONGENITAL ECTROPION OF THE EYELIDS ASSOCIATED WITH DOWN'S

SYNDROME

P. W. Sellar. Department of Ophthalmol- ogy, Royal Victoria Hospital, Belfast.

A case of congenital ectropion in the eyelids of an eleven year old boy with Down's syndrome was presented. He had marked eversion of both upper eyelids and less pronounced earopion of the lower eyelids. A triple row of eydashes was present on each upper eyelid. Levator function was absent bilaterally. All four eyelids showed vertical lack of skin and laxity of the lid tissues. The child's late presentation demonstrated the consequences of failure to reve~l the lids at birth. Corneal exposure had resulted in corneal opaci- ties and strabismus. Surgical correction of the ectropion of the upper lids was achieved by Fasanella Servat procedures combined with skin grafting. Wedge resections were carried out on the lower lids.

This case of congenital ectropion of the eyelids is only the second to be reported in Ireland. Almost thirty reports of the condition are now to be found in the literature since it was first repoaed by Adams in 1986. Most cases were successfully treated as infants by maintaining lid reversion for

LONG TERM ADDITIVE EFFECT OF BETAXALOL AND EPINEPHRINE IN

PRIMARY OPEN ANGLE GLAUCOMA

B. Power, J. Fenton, M. Hillery, A. B. Smith and L. M. T. Collum.

Fifteen patients (24 eyes) with ocular hyper- tension or glaucoma were followed up for an average of 13.5 months. All patients were treated with Gutt Betaxalol 0.5% B.D. and Guu Eppy 1% B.D.

At presentation and at each follow up visit visual acuity, intraocular pressures, optic disc cup ratios, and visual fields were assessed.

Of the 24 eyes, 11 achieved complete control on the above regime. Nine were controlled whh the addition of carbonic anhydrase inhibiton or laser treatment and 4 were not controlled.

The mean reduction in intraocular pressure on the Betaxolol/Eppy combination at six months was 8.5 mm Hg and at one year was 8.3 mm Hg.

When subdivided into two groups, those con- trolled on combination treatment alone and those requiring additional treatment, we found that the former's pre treatment intraocular pressures were slightly lower than the latter's - 26.6 nun Hg versus 28.2 mm Hg.

26 Section of Ophthalmology

Two patients complained of local burning after instillation of the Eppy drops but otherwise we encotmtered no side effects from the treatment regime.

We have shown that the Betaxalol/Eppy corn- bination is effective ha lowering intraocular pres- sures and that this effect is maintained up until at least one y e a r .

INCIDENCE AND RISK FACTORS FOR RETINOPATHY OF PREMATURITY IN A

VERY LOW BIRTH WEIGHT POPULA- TION

F. G. McGinnity, A. B. Page, S. S. Johnson.

IIIIII

Dept. of Ophthalmology; H. Halhday, Royal Maternity Hospital; C. Patterson, Dept of Statis-

tics, Royal Victoria Hospital, Belfast.

Over the eight year period from January 1978 until December 1986, 664 neonates of 1500g birth weight and under, were admitted to the Special Care Baby Unit of the Royal Maternity Hospital, Belfast. Of these 538 survived beyond 4 weeks, 455 (85%) of whom formed the basis for a retrospective analysis of the incidence and risk factors for retinopathy of pmmaturity (ROP).

Clinical data, including details of oxygen ad- ministration, was collected on all patients exam- ined. ROP was classified according to the Patz modification of the Reese classification.

Of the total of 455 children, ROP was found in

I t.J.M.S.

January, 1989

20 (4.4%). Although the condition was com- moner in the lowest birth weight infants, increas- hag to 25% in those less than 750g, severe forms of the disease were not restricted to the hghtest babies.

The incidence of various risk factors betweeaq the ROP group and a control group matched for sex, birth weight and gestational age were c ~ - pared. Statistically significant risk factors in- cluded respiratory distress syndrome and bron- chopulmonary dysplasia, patent ductus arterio- sus and hadomethacin therapy, duration of inter- mittent positive pressure ventilation and duration of hypercapnia and acidaemia.

Our study confirms that ROP is a multifacto- rial disease of very sick, very low birth weight babies.

I