ABSTRACT
Congenital loss of innervation to the extra-ocular muscles (EOMs) can have a profound effect on the target muscle. This has been well recognised in Duane's retraction syndrome. However, it has been less emphasised in other congenital oculo-motor disorders. Such congenital ocular motor defects have been expanded to include DRS, congenital fibrosis of EOMs, monocular elevation defect, Möbius syndrome, as well as several other non-ocular muscles supplied by cranial nerves such as facial muscles. Such loss of innervation to motor muscles can be unified as a defined clinical entity, which can be labelled as congenital innervation dysgenesis syndrome or CID for short. CID may also affect other muscles supplied by nerves other than the cranial nerves and may be sensory as well as motor.
Subject(s)
Duane Retraction Syndrome , Mobius Syndrome , Oculomotor Muscles/innervation , Duane Retraction Syndrome/diagnosis , Duane Retraction Syndrome/genetics , Duane Retraction Syndrome/physiopathology , Female , Humans , Male , Mobius Syndrome/diagnosis , Mobius Syndrome/genetics , Mobius Syndrome/physiopathology , Oculomotor Muscles/abnormalities , PedigreeABSTRACT
A total of 101 patients (total group) with primary large angle esotropia who had primary bimedial rectus recession (BMR) of 6.5 mm or more were reviewed in a retrospective study. The average pre-operative size of the deviation was 63.5 prism diopters (PD) for near and 58.2 PD for distance. Of these, 26 patients (group I) attended for one post-operative visit only. The remaining 75 patients (group II) were followed for an average period of 17.7 months. Among group II patients there was an average shift in the post-operative deviation towards divergence consisting of-6.4 PD for near and-5.5 PD for distance. The alignment (deviation of 10 PD or below) rate in the first postoperative visit was 31.7%; undercorrection was 60.4% and overcorrection was 7.9%. At the end of the period of follow-up the above rates were 40%, 42.7%, and 17.3% respectively without further surgery. This increase in the overcorrection rate was solely derived from the initially aligned patients. Amblyopia and/or poor fixation were found to be of significance in the production of overcorrection. There was no direct relationship between the age of surgery, duration of follow-up or the degree of the BMR and the rate of correction. Among group II patients, post-operative ocular movements analysis revealed limitation of adduction of-0.5 to-0.75 (out of scale 0 to-4.00) in 22.7% (17/75) and-1.00 to-1.50 in 6.7% (5/75). None had more than-1.5 limitation of adduction. Large BMR is an acceptable surgical modality for the management of large angle esotropia. It has approximately 50% alignment rate. Slight undercorrection is recommended especially in the presence of significant amblyopia and/or poor fixation. No significant limitation of adduction was encountered.
ABSTRACT
Fifty-two children with infantile esotropia had surgery for their esotropia between two and seven years of age. Another 49 had similar surgery before the age of two years. Of these 101 operated children, the esotropia in 70 patients was aligned to within 10 prism diopters. The patients with successful alignment were reviewed to study the effect of early versus late surgical intervention of their deviation, i.e. before and after two years of age. The motor and sensory states of the selected patients were analysed before and after surgical correction. The choice and number of surgical procedures employed are discussed. It appears that patients two years and older when strabismus was surgically corrected have a good chance of alignment, but less chance of attaining binocularity. In many cases there was a V-pat-tern esotropia, requiring surgery on the overacting inferior oblique muscles. In comparison, the younger group with earlier surgery appeared to show a better chance of attaining binocularity and fewer patients had significant V-pattern requiring surgery on the inferior oblique muscles.
Subject(s)
Amblyopia/therapy , Sensory Deprivation , Strabismus/therapy , Child , Child, Preschool , Humans , Infant , Time FactorsABSTRACT
Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency plays an important biochemical role in the metabolism of the lens. Controversies exist in the literature on the possible association between G-6-PD deficiency and the development of cataracts. The authors present ten patients, aged between infancy and 40 years of age, who were admitted for bilateral congenital or presenile cataracts. These patients had no ocular or systemic disease which might have caused their cataract. The only systemic finding they had was G-6-PD deficiency. Two other patients among the families described suffered from bilateral congenital or presenile cataracts with no G-6-PD deficiency. This deficiency state does not appear to play a role in the production of their cataracts.
Subject(s)
Cataract/enzymology , Glucosephosphate Dehydrogenase Deficiency/diagnosis , Adolescent , Adult , Cataract/congenital , Child , Child, Preschool , Erythrocytes/enzymology , Female , Glucosephosphate Dehydrogenase Deficiency/classification , Humans , Infant , Lens, Crystalline/enzymology , Male , Pedigree , PhenotypeABSTRACT
Optokinetic nystagmus (OKN) testing is a useful tool for visual assessment. It has been used for assessing visual function in infants, hysterical patients, and malingerers. It is also of value in the diagnosis of disorders of the central nervous system. At present the devices used for the clinical testing of OKN are the OKN drum and OKN tape. A method is presented for OKN testing using an IBM-personal computer or compatible. This method is compared with the other two standard methods.
Subject(s)
Microcomputers , Nystagmus, Pathologic/diagnosis , Diagnosis, Computer-Assisted , Humans , Nystagmus, Physiologic/physiology , SoftwareABSTRACT
Three brothers had bilateral retinoblastoma: one other sibling, a girl, had normal eyes. The parents were second cousins once removed. The father's right divergent eye, 'amblyopic' from childhood, showed a large atrophic patch in the macular area, probably a spontaneously resolved retinoblastoma.
Subject(s)
Eye Neoplasms/genetics , Retinoblastoma/genetics , Adult , Child , Consanguinity , Eye Neoplasms/pathology , Female , Humans , Male , Pedigree , Remission, Spontaneous , Retinoblastoma/pathology , Sex FactorsABSTRACT
Six hundred forty-three patients had retinal detachment surgery during 81/2 years; 17.6% were under the age of 30 years. Analysis of this group of patients showed that males were more commonly affected than females (68%). Trauma was the most frequent cause, reported in 40% of cases. Dialysis was the commonest retinal break, occurring in 51% of patients, and 73% of these occurred in the inferior temporal quadrant. In patients with a history of trauma, the type of the retinal break produced and operative success was related to the severity of ocular damage, with more successful outcomes in nontraumatic cases and fewer of these in cases of ocular perforation.
Subject(s)
Craniocerebral Trauma/complications , Retinal Detachment/etiology , Adolescent , Adult , Contusions/complications , Eye Injuries/complications , Facial Injuries/complications , Female , Humans , Male , Retinal Perforations/etiology , Retrospective Studies , Time Factors , Wounds, Penetrating/complicationsABSTRACT
Of 2649 patients with strabismic amblyopia who attended clinics between 1941 and 1978, 1904 patients were reviewed. Of these, 115 patients had transfer of fixation after occlusion. These were analysed according to the type of transfer of fixation and relation to occlusion and age. The period of maximum sensitivity to short periods of occlusion extended to 18 months, declining to about 30 months of age. Sensitivity to occlusion reaches low levels about the age of 5 to 51/2 years, though it is still present to some degree, especially in patients with relatively good visual acuity of 6/36 or better. The upper limit of the sensitive period was found to be about the end of the seventh year of age.
