Duane's retraction syndrome: a retrospective review from Kathmandu, Nepal

Purpose: The aim was to study the clinical characteristics of Duane's retraction syndrome (DRS) in Nepalese patients. Method: Medical records from 52 cases of DRS from May 2003 to April 2010 were retrospectively reviewed for age, gender, laterality and clinical characteristics. Forty‐one case records (78.8 per cent) that had complete clinical findings were considered for further evaluation. Examination included visual acuity by Snellen chart, refraction, associated horizontal and vertical strabismus in primary gaze, upshoot and downshoot on attempted adduction, binocular vision assessed with the Worth four‐dot test on adopted gaze and stereopsis examined with the Titmus stereo test. Results: DRS type I was the most common type observed in 73.2 per cent of cases, followed by DRS type II (14.6 per cent) and DRS type III (12.2 per cent). It was more common in female patients (58.5 per cent) than male patients (χ²= 4.6, df = 1, p = 0.03). DRS was more common in the left eye (68.3 per cent) than the right eye and unilaterally present in 95.1 per cent of subjects. In primary gaze, orthotropia (41.5 per cent) was more common than exotropia (34.1 per cent) and esotropia (24.4 per cent) and vertical strabismus was present in 24.4 per cent of subjects. Upshoot and downshoot on attempted adduction was seen in 14.6 and 9.8 per cent, respectively. Binocular single vision was present in 68.3 per cent of subjects by Worth four‐dot test at near. Stereopsis of 3,000 seconds of arc was present in 9.8 per cent, 100 to 200 seconds of arc in 14.6 per cent and 40 to 60 seconds of arc in 43.9 per cent with the Titmus stereo test. Conclusion: DRS is more common in female patients and the left eye. DRS type I is the most common type.     

Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature

Hyperimmunoglobulinemia E (Job''s) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job''s syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job''s syndrome.     

Paralytic Strabismus in Children: Etiologic Incidence and Management of the Third,Fourth, and Sixth Nerve Palsies

The results of a retrospective study of the etiologic incidence of all cases of third, fourth, and sixth nerve palsies in patients at Wills Eye Hospital and St Christopher's Hospital are reported. A total of 121 patients ranging in age from birth to sixteen were studied. Thirty-two cases of third nerve palsy, eighteen cases of fourth nerve palsy, and sixty-two cases of sixth nerve palsy were identified in this series. Nine additional cases illustrated combinations of multiple nerve involvement. With few exceptions, acquired third nerve palsy in children is an ominous sign whereas the congenital form is generally indicative of developmental anomaly or birth trauma. Acquired sixth nerve palsy in a child is always a matter of concern since it may represent the first sign of a brain-stem glioma. Active force studies may be carried out periodically to assess the speed of recovery and indicate when it is time to initiate a more aggressive role in the surgical management. The surgical results in children with paralytic strabismus that have been carefully evaluated offer a favorable prognosis.     

Carotid-cavernous and orbital arteriovenous fistulas: ocular features,diagnostic and hemodynamic considerations in relation to visual impairment and morbidity

The author investigated 101 cases with direct dural carotid-cavernous and orbital arteriovenous fistulas (CCF). The characteristic clinical findings, such as specific epibulbar arterialized loops, are described and the differential diagnosis of the striking diagnostic triad (exophthalmos, the above-mentioned loops and glaucoma) is discussed, together with the exclusion criteria for other causes of red eyes, episcleral measurements and blood flow. The results of various diagnostic procedures, such as ultrasonography, Doppler hematotachography and color Doppler of the orbit and carotid systems, magnetic resonance imaging and angiography, and of conservative treatment and embolization processes are dealt with successively.The classification of different types of carotid-cavernous fistulas is presented, together with the clinical signs in relation to morbidity and mortality during or after conservative or intervention therapies.The importance of patient follow-up, in the clinic as well as with Doppler methods, is emphasized in order to differentiate a progressive or diminished clinical condition caused by spontaneous thrombosis in the healing process or more arteriovenous flow. A ‘decision tree’ for use in daily practice is provided. In this study, of the 101 cases in which the localization was diagnosed by angiography, 42 were direct (30 traumatic, 12 spontaneous), 31 were dural (3 traumatic, 28 spontaneous) and 10 were orbital CCFs. In 18 other cases, usually dural or orbital shunts, angiography was not performed. For the management of 42 direct fistulas, conservative treatment was used in 12 cases (7 with success; 58%) and balloon embolization was performed in 18 cases (17 with success; 94.5%); the other cases were treated by direct or indirect surgery. Of the 48 (spontaneous and traumatic) dural fistulas, 39 were treated conservatively (32 recovered or were much improved: 82%, of the total cases, 67%). All seven cases in which embolization was performed were cured and/or much improved. In two cases, one fistula was conservatively treated while one was embolized at another location, both with success. Of the 10 orbital arteriovenous shunts showing signs of dural fistulas, the features disappeared in 8 cases, although after a much longer follow-up period than for the typical dural carotid-cavernous sinus fistulas; in one patient, direct surgery was performed successfully and in one patient the original, non-progressive, orbital features could still be observed.     

Surgical correction of Hallermann-Streiff syndrome: a case report of esotropia, entropion, and blepharoptosis

We report a case of surgical treatment for Hallermann-Streiff syndrome in a patient with ocular manifestations of esotropia, entropion, and blepharoptosis. A 54-year-old man visited Yeouido St. Mary's Hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years. He had a bird-like face, pinched nose, hypotrichosis of the scalp, mandibular hypoplasia with forward displacement of the temporomandibular joints, a small mouth, and proportional short stature. His ophthalmic features included sparse eyelashes and eyebrows, microphthalmia, nystagmus, lower lid entropion in the right eye, and upper lid entropion with blepharoptosis in both eyes. There was esodeviation of the eyeball of more than 100 prism diopters at near and distance, and there were limitations in ocular movement on lateral gaze. The capsulopalpebral fascia was repaired to treat the right lower lid entropion, but an additional Quickert suture was required to prevent recurrence. Blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis. Three months after lid surgery, the right medial rectus muscle was recessed 7.5 mm, the left medial rectus was recessed 7.25 mm, and the left lateral rectus muscle was resected 8.0 mm.     

Progressive essential iris atrophy,Chandler's syndrome,and the iris nevus (Cogan-Reese) syndrome: A spectrum of disease

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome are considered to be variations of a single disease process, which is characterized by abnormalities of the cornea, anterior chamber angle, and iris. In each variation, the typical patient is a white woman with unilateral disease, negative family history, and an onset of symptoms in early to middle adulthood. Since the membrane theory of Campbell suggests that the disease is a fundamental abnormality of the corneal endothelium, rather than the iris, the term “iridocorneal endothelial syndrome”, as proposed by Yanoff, may be an appropriate inclusive term for the spectrum of disease, although further study of the pathogenesis is needed. For each variation of the disease, corneal edema and secondary glaucoma are both treated primarily by medical or surgical reduction of the intraocular pressure, although penetrating keratoplasty is occasionally required for cases with advanced corneal edema.     

Gastrointestinal microbiome and primary Sjögren's syndrome: a review of the literature and conclusions

The recognition of the profound impact of the human gastrointestinal microbiome (GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjögren''s syndrome (pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with pSS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and pSS, with the aim of contributing to future research and to the development of new clinical applications.     

Idiopathic Serous Detachment of the Choroid,Ciliary Body,and Retina (Uveal Effusion Syndrome)

Nine patients with bilateral idiopathic serous detachment of the peripheral choroid, ciliary body, and retina (uveal effusion syndrome)are presented. The following features are emphasized: (1) dilationof episcleral vessels, (2) vitreous cells, (3) characteristic leopardspot pigment epithelial changes, and (4) ultrasonic and angiographic findings. One patient presented with recurrent serous macular detachment and was misdiagnosed as idiopathic central serous choroidopathy. Although the pathogenesis of this syndrome is uncertain, the available clinical and histopathologic findings suggest that obstruction of the venous outflow of the uveal tract occurring predominantly in nanophthalmic or normal size eyes with abnormally thick sclera and vortex vein anomalies may be the primary cause of the uveal and retinal detachment.     

Species differences in the responses of the eye to irritation and trauma: a hypothesis of divergence in ocular defense mechanisms, and the choice of experimental animals for eye research

Information published during the past century, especially the last decade, has identified pronounced species differences, not only in the morphological organization of ocular structures, but also in the functional responses of the eyes of different mammals to experimental and surgical procedures, as well as to drugs and autacoids. For the most part, these differences have been regarded as peculiarities or weakness rather than as fundamental evolutionary adaptations optimally suited to the environment and behavior of each species. This paper proposes a working hypothesis of evolutionary divergence in ocular defense mechanisms, based on some of the known morphological and functional differences among mammals, and discusses the implications of these differences with regard to the choice of appropriate animals for use as models in different areas of ophthalmic research.     

A new approach to the study of detail perception in Autism Spectrum Disorder (ASD): investigating visual feedforward, horizontal and feedback processing

Enhanced detail processing is a characteristic of ASD. However, previous studies could not yet provide a neural explanation of this trait. Since the balance between visual feedforward and feedback processing is probably essential for the character of visual perception, we conjectured that this balance is disturbed in ASD. Using a new texture discrimination task, where surface segregation was varied independently from orientation boundaries, we showed that subjects with ASD scored lower than controls, probably caused by enhanced feedback. Interestingly, performance improved in the ASD group when repeating the task two additional times, indicating a compensation for the imbalance between feedforward and feedback processing.     

Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP): report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment

Ichthyosis follicular, alopecia, and photophobia (IFAP) syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina) and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.     

The different contributions of prostaglandins (E1, E2, F2alpha,D2) to the tone and neurogenic response of bovine iris sphincter muscle

The function and sites of action of prostaglandins (PGs) vary in different animal species and tissues. In this study the influence of PGs (E₁, E₂, F_2alpha, D₂) on muscle tone and nerve-mediated contraction was investigated in isolated bovine iris sphincter muscles.None of these PGs exogenously applied influenced the neuromuscular transmission. By contrast, after treatment with indomethacin, all PGs tested contracted the muscle much more than in the absence of indomethacin and under these conditions the PGs potentiated responses to cholinergic nerve stimulation. Their ED₅₀ were (2.2 ± 0.2) × ^–7 M for PGE₁, (6.7 ± 0.3) × ^–8M for PGE₂, and (7.3 ± 0.4) × ^–8 M for PGF_2alpha. PGE₁ acted both on nerves and the muscle cell. PGE₂ had its influence mostly via nerves. Whereas PGF_2alpha was less potent in the absence of indomethacin, PGF_2alpha had much more potent action primarily on nerves and partly on muscles after treatment with indomethacin. High concentrations of PGD₂ had both pre- and post-junctional action with accompanying weak contraction of the muscle. Thus the degrees of pre- and post-junctional involvement were different from one another.There is a possibility that the application of these PGs alone masked the role of such endogenous agents. In order to understand and clarify the site and action of PGs, pretreatment with indomethacin may be useful in the iris muscle. In conclusion, PGs modulate cholinergic activity in the bovine iris sphincter muscles, as well as regulate the muscle tone.     

The contribution of on-bipolar cells to the electroretinogram of rabbits and monkeys: A study using 2-amino-4-phosphonobutyrate (APB)

We have obtained electroretinograms (ERGs) from rabbits and macaque monkeys after vitreal administration of 2-amino-4-phosphonobutyrate (APB), which selectively blocks light-responsiveness in retinal on-bipolar cells. Microelectrode recordings from central visual structures provided an independent measure of the drug effects. In rabbits, APB blocked the b-wave, a sustained corneal-positive potential, and a transient corneal-negative off-response. In monkeys, APB abolished the b-wave but had little or no effect on the d-wave. The d-wave could be eliminated, however, by the use of broadband blue stimuli. The results are discussed in light of recent hypotheses about the cellular origins of the ERG.