This thesis represents an attempt to further our knowledge of the ophthalmic complications of spina bifida and hydrocephalus by means of literature review and a long term clinical study, and to determine whether regular ophthalmic supervision can assist in the general management of affected children.The ophthalmic complications of spina bifida have often been reported in the literature and thought to merit regular supervision of affected children, yet few centres currently offer this service.In this study 322 children attending one regional centre were examined repeatedly over a six year period by one ophthalmologist. Ophthalmic complications were found to be very common. They frequently provided evidence of raised intracranial pressure due to shunt dysfunction even when other objective evidence was lacking.Every spina bifida and hydrocephalus clinic should have an ophthalmologist in its medical team. Preservation of visual function and early diagnosis of raised intracranial pressure in these children should result from this arrangement. Preface "The object of this study was to determine if regular, long term examination of children with spina bifida or hydrocephalus by an ophthalmologist working in the spina bifida clinic would assist in their general management, particularly in the diagnosis of shunt obstruction as it is often difficult to determine this by other means. SummaryThe majority of children with spina bifida and hydrocephalus require cerebrospinal fluid shunts at some time in their lives. These shunts commonly become obstructed. Shunt obstruction is followed by the development of an acute or chronic rise in intracranial pressure which may be difficult to -diagnose early, as the symptoms and signs are often non specific. The main objects of this study were two-fold. Firstly, to determine whether papilloedema, as assessed by a trained ophthalmologist, is a reliable diagnostic sign of raised intracranial pressure and shunt malfunction in hydrocephalic children. Secondly, to determine the incidence of other ocular complications in children with hydrocephalus and whether recognition and treatment of these disorders sigoificantly benefit their development, education and employment prospects.A background review of the literature outlines the pathology and treatment of spina bifida, hydrocephalus and associated lesions, and evaluates previous observations of the ophthalmic complications of these conditions. In this study 322 children who were suffering from either spina bifida or hydrocephalus were examined over a six year period by one ophthalmologist working in the clinic alongside paediatric staff. Regular examinations were made of visual acuity and field, ocular motility and fundi, starting at the time of birth or diagnosis and repeated whenever possible, at each clinic visit or hospital admission, A particular effort v-as to s:;; nchildren about to have exploratory surgery for suspected shunt dysfunction or raised intracranial pressure to determine the frequency of positive eye findings in such cases.T...

322 children who were suffering from either spina bifida or hydrocephalus were examined over a six year period. Regular examinations were made of visual acuity and field, ocular motility and fundus starting at the time of birth or diagnosis and repeated, whenever possible, at each clinic visit or hospital admission. A particular effort was made to see children about to have exploratory surgery for suspected shunt dysfunction or raised intra-cranial pressure to determine the frequency of positive eye findings in such cases. Ophthalmic complications were found to be very common in the children studied. 136 (42%) had a manifest squint, 93 (29%) a lateral rectus palsy or musculo-paretic nystagmus, 44 (14%) had papilloedema and 55 (17%) had optic atrophy. Only 86 (27%) definitely had normal visual function. 70% of proven episodes of shunt dysfunction had positive ophthalmological evidence of raised intracranial pressure. The presence of an ophthalmologist in the spina bifida and hydrocephalus clinic was increasingly valued as the study progressed. Every spina bifida and hydrocephalus clinic should have an ophthalmologist in its medical team. He should undertake regular examinations of visual acuity and field, ocular motility and fundi, starting at the time of birth or diagnosis, and tailor ophthalmic care to the special needs of these children. This should ensure that these children achieve and maintain the best possible standard of vision and that the earliest ophthalmic signs of raised intracranial pressure can be detected in patients where the diagnosis is in doubt.

Thirty-four patients with connective tissue diseases used dexamethasone drops 01% in one eye for 6 weeks. There was a higher incidence of positive steroid response than would be expected in a normal population. Most of the male patients were responders. Care should be exercised when prescribing local steroids for these patients. Males should be offered regular screening for glaucoma. Eye involvement is a prominent feature of connective tissue diseases (CTD). Keratoconjunctivitis sicca (KCS) is the commonest complication, occurring in 11% of patients with rheumatoid arthritis (RA)I and sporadically in systemic lupus erythematous (SLE), progressive systemic sclerosis (PSS), polymyositis, and psoriatic arthritis. Scleritis appears in 067% of RA patients and in other cases of CID (e.g., ankylosing spondylitis and Behget's disease) from time to time. Uveitis is of particular importance in juvenile rheumatoid arthritis, ankylosing spondylitis, and Reiter's syndrome. Conjunctivitis is one of the diagnostic criteria for Reiter's syndrome. Proptosis, diplopia due to extraocular myopathy, corneal disturbances, optic neuritis, and retinal vascular changes may occur is SLE and lid shortening and iris atrophy in PSS. Discolouration and oedema of the lids and extraocular muscle changes occur in polymyositis and dermatomyositis and retinal vasculitis in up to 20% of patients with polyarteritis nodosa. The large host of ocular phenomena that are reported make it unlikely that the trabecular meshwork is exempt from autoimmune inflammatory processes, though glaucoma is not a recognised complication of CTD. The intertrabecular spaces of the 'pore' area of the trabecular meshwork are lined by a layer of mucopolysaccharide.2 If this layer is removed by hyaluronidase, resistance to aqueous outflow falls. Topical steroids increase outflow resistance. This may be due to increased production of mucopolysaccharide

The HLA-Cw*0701 allele and KIR haplotype AA are associated with AMD. This genotype combination suggests that natural killer cells have a role in the pathogenesis of AMD. Replication studies are needed to confirm these novel HLA-KIR associations with AMD.