Eyelid ectropion in Down's syndrome related to allergic conjunctivitis: case report

This is a case report of a patient with Down's syndrome with an important mechanical ectropion and elongation of the lower lid related to chronic allergic conjunctivitis, besides cataract and keratoconus. The tarsal strip technique and resection of redundant and thick conjunctiva was the surgical approach chosen for this case. Functional and cosmetic outcomes were considered satisfactory.     

A case of congenital oblique retraction syndrome with upshoot in adduction

We treated a 27-year-old man who suffered from a congenital eye movement abnormality. The patient could not adduct the left eye, and showed exotropia, hypertropia and excyclotropia of the left eye. The range of abduction was also limited. The left eye showed marked upshoot on right gaze and eye globe retraction on right down gaze under conditions of right-eye viewing. The superior rectus muscle of the left eye was recessed and transposed nasally. The insertion of the superior oblique muscle was found to be defective. The upshoot and face turn improved after surgery. The globe retraction on right down gaze was no longer evident. We believe that the superior rectus muscle was innervated by an anomalous branch of the oculomotor nerve, which was originally destined to innervate the medial rectus, and that simultaneous contraction of the superior and inferior rectus muscles caused the globe retraction on right down gaze. This case also suggests the possibility that some part of the upshoot in adduction in Duane's syndrome is due to the co-contraction of the superior and medial rectus muscles due to this innervation anomaly.     

A vision screening of a Down's syndrome population

A vision screening of 55 (24 male, 31 female) Down's syndrome children was done by SUNY vision training residents. In the past, similar screenings have been done with older children. The children in this study ranged in age from 2 years, 2 months, to 8 years, 5 months. The median age was about 3.5 years. This study will enable vision care practitioners to be more aware of visual conditions in this population which have a greater incidence than the normal population.     

Diabetic retinopathy in Down's syndrome

AIM—To determine the prevalence of diabetic retinopathy in patients with Down's syndrome and diabetes mellitus.
METHOD—Nine patients with Down's syndrome and diabetes mellitus were assessed. Factors recorded included type and duration of diabetes, level of diabetic control, blood pressure, urinalysis, and results of ophthalmological examination.
RESULTS—The duration of diabetes ranged from 8 to 41 years (mean 17.6 years). All had satisfactory glycaemic control and blood pressure measurements on the low side of normal (mean 106.6/70 mm Hg). One patient had early background diabetic retinopathy. The remainder had no evidence of diabetic retinopathy.
CONCLUSION—The low prevalence of diabetic retinopathy in these Down's syndrome patients, despite the long duration, is an interesting finding. It suggests some inherent protective factor against the development of diabetic retinopathy in this patient subgroup.

Keywords: Down's syndrome; retinopathy; diabetes; hypertension     

Penetrating keratoplasty in Down's syndrome

BACKGROUND: Purpose of this retrospective study was to analyze outcome and complications after penetrating keratoplasty (PK) in patients with Down's syndrome. PATIENTS AND METHODS: The medical records of all patients with Down's syndrome and PK from 1985 were reviewed retrospectively. Age, sex, systemic diseases, communication, indication for surgery and surgical technique, age at first PK, complications, visual acuity and best corrected visual acuity and findings at last follow-up were noted. Age matched mentally non-retarded patients with keratoconus and PK served as a control group. RESULTS: All eyes of 9 patients had keratoconus, 16 were operated. Indication for surgery was spontaneous perforation in 3 and refractive in the remaining 13 eyes. Five grafts were clear at last follow-up. In 7 eyes, at least 1 re-PK had to be performed. Seven eyes developed graft rejection and 4 eyes needed conjunctival grafting because of non-healing epithelial defects. Ten eyes developed cataracts, 3 secondary glaucoma. In the control group 22 out of 25 operated corneas were clear at last follow-up. CONCLUSIONS: Prognosis of PK in patients with Down's syndrome is worse than in other patients with keratoconus. Poor results are not only explained due to the lack of cooperation of patients, but also because of: 1) high rate of emergency procedures (3/16), 2) presence of lid anomalies and 3) very advanced keratoconus, necessitating bigger sized grafts, leading to frequent immunological reactions, suture loosening and secondary glaucoma. Indication for PK in Down's syndrome patients with keratoconus and floppy eyelids should be very restrictive. Postoperative complications and the high rejection rate might be decreased by performing deep lamellar keratoplasty as this would not be an intraocular procedure.     

Impression cytology in Down's syndrome

AIM—To evaluate both the number and the average distribution of goblet cells, which are responsible for the production of the mucin layer of the tear film, in the bulbar conjunctiva of patients with Down's syndrome. Previous research had used the ferning test to indicate an alteration in Down's syndrome, but had not determined which film layer was involved.
METHODS—The presence of goblet cells in the bulbar conjunctiva of 30 subjects (15 with Down's syndrome, and 15 normal control subjects) was evaluated using impression cytology.
RESULTS—A marked reduction of goblet cells was found in the Down's syndrome group (81.4 per mm²) when compared with the control group, where (209.8 per mm²) was found.
CONCLUSION—The deficit observed appears to be the cause of the tear film alterations observed in Down's syndrome. In turn, this may often lead to the formation of dry spots, and to consequent frequent infections of the anterior segment of the eye. While it is further hypothesised that the alteration of the conjunctival epithelium in Down's syndrome may be due to an altered metabolism of some element or elements, such as vitamin A, further research will be necessary to corroborate this.

     

Oedipism and Down's syndrome: case report and review of the literature

Patients with Down's syndrome usually have mild and pleasant temperaments, rarely exhibiting temper tantrums or behavioral problems. Blindness occurs in 5% of these patients. When present, it is usually due to keratoconus and cataracts and their associated complications. In this paper the authors report a case of self-induced ocular blindness that apparently resulted from the patient's severe mental retardation. To their knowledge, this has not been described in Down's syndrome.     

Ferning test in Down's syndrome.

The tear film was evaluated by the ferning test. We tested tear film in right and left eye on 28 subjects (total 56 eyes), of whom 14 were affected by Down's syndrome, age range 18 to 35 years. The results obtained show that there exists an alteration of the tear ferning in subjects affected by Down's syndrome. We did not find any difference between the right or left eye in each individual. This anomaly could be responsible for frequent infectious pathologies found in the anterior eye segment in these subjects.     

Disc vascularity in Down's syndrome.

Ocular fundus photographs of 100 eyes were obtained from an institutionalized population of patients with Down's Syndrome (66 males, 34 females). The vascularity of the optic nerve head was assessed in each photograph by counting the number of arterioles, venules, and fine vessels crossing the disc margin. The results of this analysis were compared to similar data obtained from 100 ocular fundus photographs of normal patients and show that there are more large vessels crossing the disc margin in those with Down's Syndrome.     

Optic disk elevation in Down's syndrome

Of five children who had Down's syndrome with optic nerve head elevation, without associated intracranial lesions, three underwent enhanced computed tomography for which no abnormalities were found. Partial, complete, or intermittent resolution of the optic disk elevation occurred in three children. In none of the children were retinal vessel dilation, splinter hemorrhages, optic nerve drusen, subsequent optic atrophy, or apparent visual loss noted. All of the children were hyperopic, but only one child had a hyperopia of greater than 3.50 diopters.