Pseudonystagmus in trochlear nerve palsy.

We present the case report of a patient who presented with intermittent diplopia and left hypertropia associated with an apparent left superior oblique palsy. After dissociation with cover testing, he showed conjugate rhythmic vertical eye movements present in all gazes, thought to represent rapid rhythmically alternating fixation. The vertical rhythmic movement resolved after strabismus surgery.

Instrument-based screening for amblyopia risk factors in a primary care setting in children aged 18 to 30 months.

Amblyopia is the leading cause of vision impairment in children, and its early detection can avoid irreversible consequences for a child's visual ability. In this prospective study, to detect amblyopia risk factors, we examined patients aged 18 to 30 months in primary care settings. Patients were referred to an ophthalmologist for confirmation. The main aims were to detect amblyogenic risk factors and to assess the usefulness of a photoscreener in such settings. Out of 453 patients, 42 (9.3%) presented visual alterations according to the photoscreener, with astigmatism being the most common. The instrument had good sensitivity (89%) and specificity (91%), with a positive predictive value of 76% and a negative predictive value of 96%. Overall, 38% of the patients required follow-up, and 47% needed glasses. The automated screening device allowed these children to be diagnosed at an early stage.Conclusions: The use of a photoscreener to screen 2-year-old children in primary care settings was helpful and accurate. What is Known: • Early detection of amblyopic risk factors is important to avoid vision defects. However, it is very difficult to measure visual acuity using visual charts in children younger than 4 years old. What is New: • Instrument-based screening in children aged 18 to 30 months allows excellent detection of early amblyopia risk factors in primary care settings.

Growth Hormone Treatment and Papilledema: A Prospective Pilot Study

Objective: To investigate the incidence of pseudotumor cerebri syndrome (PTCS) in children treated with growth hormone (GH) in a paediatric hospital and to identify risk factors for this complication. Methods: Prospective pilot study of paediatric patients treated with recombinant human GH, prescribed by the Paediatric Endocrinology Department, between February 2013 and September 2017. In all these patients, a fundus examination was performed before starting treatment and 3-4 months later. Results: Two hundred and eighty-nine patients were included, of whom 244 (84.4%) had GH deficiency, 36 (12.5%) had short stature associated with small for gestational age, six (2.1%) had a mutation in the SHOX gene and three (1.0%) had Prader-Willi syndrome. Five (1.7%) developed papilledema, all were asymptomatic and had GH deficiency due to craniopharyngioma (n=1), polymalformative syndrome associated with hypothalamic-pituitary axis anomalies (n=2), a non-specified genetic disease with hippocampal inversion (n=1) and one with normal magnetic resonance imaging who had developed a primary PTCS years before. Conclusion: GH treatment is a cause of PTCS. In our series, at risk patients had GH deficiency and hypothalamic-pituitary anatomic anomalies or genetic or chromosomal diseases. Fundus examination should be systematically screened in all patients in this at-risk group, irrespective of the presence or not of symptoms.

Frontalis Muscle Flap Versus Maximal Anterior Levator Resection as First Option for Patients With Severe Congenital Ptosis.

PURPOSE: To compare 2 surgical techniques (frontalis flap versus maximal anterior levator resection) as first surgical options for the treatment of congenital ptosis with poor levator function in patients younger than 2 years of age with a follow up of 10 years. METHODS: A retrospective study of 58 patients (71 eyelids) with severe ptosis and poor levator function who underwent frontalis muscle flap (FMF = 47) or maximal anterior levator resection (ALR = 24) for correction of their ptosis. Eyelid measurements were taken at baseline, 1, 5, and 10 years after surgery. The presence of complications, need for reoperations, and palpebral contour were evaluated. RESULTS: Most patients in both groups required only one surgical procedure with a stable average margin-reflex distance 1 over the 10-year follow-up period in both groups, with no statistically significant difference between the 2 techniques in achieving an adequate palpebral height after one single procedure. Eleven eyelids treated with FMF (23%) and 12 treated with ALR (50%) needed a reoperation, with a statistically significant difference between the 2 techniques. Five ALR patients (21%) and 6 FMF patients (13%) had alterations of eyelid contour. Pop-eyelid and eyelash ptosis were observed in 8% of patients operated with FMF. CONCLUSION: Good functional and aesthetic results were obtained with both surgical techniques. FMF required fewer reoperations compared with maximal ALR, offering a better long-term result without residual ptosis.

Intracranial Hypertension in Cystinosis Is a Challenge: Experience in a Children's Hospital.

BACKGROUND: Cystinosis is a rare systemic lysosomal disease affecting mainly the kidney and eye. Ocular involvement in cystinosis is universal being the presence of cystine crystals in the cornea a diagnostic criterion and one of the earliest manifestations of the disease. Neuro-ophthalmologic manifestations are considered a rare and late complication in these patients. The aim of this article is to report the unexpectedly high incidence of intracranial hypertension in children with cystinosis at our centre. METHODS: This study included eight children (0-16 years of age) with cystinosis seen at the paediatric ophthalmology department, Hospital Universitari Vall d'Hebron (Barcelona, Spain), a tertiary hospital, over the last 5 years. RESULTS: Three girls and five boys, mean age: 9.6 years (range: 5-14 years), were studied. During follow-up, 4 out of 8 developed papilledema and confirmed high cerebrospinal fluid (CSF) pressure. The only symptomatic child presented an Arnold-Chiari anomaly with enlarged ventricles, whereas the other three, all asymptomatic, were diagnosed by scheduled fundoscopy and had normal neuroimaging studies. All four patients had at least one known risk factor for developing intracranial hypertension: initiation of growth hormone therapy, tapering of corticosteroids, acute renal failure and Arnold-Chiari malformation. Two of them required a ventriculoperitoneal shunt. CONCLUSIONS: Our results show that intracranial hypertension can occur more frequently than expected in patients with cystinosis. Furthermore, visual prognosis depends on early diagnosis and prompt treatment. A multidisciplinary approach is necessary, and we recommend fundoscopic examinations in all paediatric patients with cystinosis whether or not they present symptoms.

Early frontalis flap surgery as first option to correct congenital ptosis with poor levator function.

UNLABELLED: In congenital blepharoptosis the upper eyelid cannot be lifted normally because of congenital impairment in the levator function. The descended eyelid margin partially or completely obstructs of the visual axis with the consequent risk of amblyopia. Frontalis suspension is the surgery of choice for ptosis with poor levator function creating a linkage between the frontalis muscle and the tarsus; the frontalis muscle is used to elevate the eyelid. Direct transplantation of frontalis muscle to the upper eyelid has been widely described. We report our experience using frontalis flap in congenital ptosis with poor levator function in children. METHODS: Retrospective study of 30 eyes with severe congenital ptosis and poor levator function treated by means of direct frontalis flap. Mean age 2 years. Eyelid measurements were taken at baseline, 1, 3, 12 months postoperatively and last visit. Mean ptosis degree was 5 mm (3-8 mm) and levator function 2 mm (1-5 mm). The presence of complications, flap function and palpebral contour were evaluated. Mean follow up time was 27 months. At last visit, ptosis degree ranged from 0 to 3 mm. DISCUSSION: Direct advancement of the frontalis muscle to treat severe eyelid ptosis is effective and stable in the long term avoiding the use of a linking structure, therefore the risk of foreign-body reaction, absorption, granuloma and late exposure, as well as the need for a second visible incision in the forehead. Patients learn how to control the lid height by means of the frontalis muscle achieving more symmetry.

Diffuse lamellar keratitis and corneal edema associated with viral keratoconjunctivitis 2 years after laser in situ keratomileusis.

A 47-year-old woman with a history of laser in situ keratomileusis (LASIK) 2 years previously for myopia and astigmatism, presented with bilateral loss of vision due to diffuse lamellar keratitis (DLK) with corneal edema in the context of a pseudomembranous viral keratoconjunctivitis. After intense and early treatment with topical corticosteroids, the corneal edema and DLK resolved and corneal transparency was achieved with complete restoration of visual acuity. This case shows that DLK may occur associated with a viral pseudomembranous keratoconjunctivitis in patients who have had LASIK. Diffuse lamellar keratitis may present up to 2 years after lamellar surgery, which would indicate that the plane created by the microkeratome at the interface may remain unhealed for at least this period of time. Early diagnosis and treatment with topical corticosteroids can achieve complete resolution without visual loss.

Conjunctival healing after amniotic membrane graft over ischemic sclera.

PURPOSE: To present a case of chemical injury associated with calcification and severe conjunctival and scleral ischemia, in which tissue regeneration was achieved using an amniotic membrane graft. METHODS: A 65-year-old man presented to our department 8 weeks after suffering a chemical injury of his left eye with sulfuric acid. There was extensive calcification of the cornea and conjunctiva, associated with severe ischemia of the adjacent sclera. After resection of the calcified tissue and nonviable tissue, amniotic membrane was grafted to cover the extensive zone of scleral ischemia. RESULTS: In the weeks following grafting, slow epithelialization and revascularization was observed over the amniotic membrane, stemming from the surrounding healthy conjunctiva. CONCLUSION: Although the presence of ischemia at the base of the graft has been considered a contraindication for amniotic membrane transplantation, this case demonstrates that, provided that the surrounding tissue is not affected, a graft may be useful. In such cases an amniotic membrane graft could be attempted before other alternatives, such as conjuntival or oral mucosal autografts.

Surgery for subfoveal choroidal neovascularization in toxoplasmic retinochoroiditis.

PURPOSE: To report a case of subfoveal choroidal neovascularization in a patient with toxoplasmic retinochoroiditis who underwent surgical excision. DESIGN: Interventional case report. METHODS: A 36-year-old woman with toxoplasmic retinochoroiditis presented with sudden dimness of vision and metamorphopsia in the left eye. The patient was examined with ophthalmoscopy and fluorescein angiography. RESULTS: Fundus examination and fluorescein angiography of the left eye revealed a subfoveal choroidal neovascularization. Pars plana vitrectomy with submacular surgery was performed, with a postoperative improvement of visual acuity and resolution of the distortion. CONCLUSIONS: This case report describes a case of subfoveal choroidal neovascularization associated with toxoplasmic retinochoroiditis that responded remarkably well to vitrectomy surgery.

Histologic findings after amniotic membrane graft in the human cornea.

OBJECTIVE: To describe the histopathologic findings in the human cornea several months after a stromal amniotic membrane graft. To show the clinicopathologic correlation after the graft in two cases with different follow-up times. DESIGN: Two interventional case reports with clinicopathologic correlation. PARTICIPANTS: Two patients with neurotrophic corneal ulcer unresponsive to medical treatment (one with stromal vascularization and the other without stromal vascularization). INTERVENTION: Amniotic membrane graft was performed in both patients to treat the neurotrophic ulcer. Three and 7 months after amniotic membrane grafting, a penetrating keratoplasty was needed, and the removed corneas were analyzed. MAIN OUTCOME MEASURES: Clinical and histopathologic examinations, including routine histopathologic and immunohistochemical studies. RESULTS: Complete epithelialization was observed on histologic examination over the basement membrane of the amniotic membrane graft. The amniotic membrane was slowly reabsorbed in the cornea without stromal vascularization with no inflammatory reaction produced. In the cornea that had stromal vascularization the amniotic membrane was rapidly reabsorbed because of the presence of abundant inflammatory cells. Once reabsorbed, the amniotic membrane was replaced by new fibrotic stroma, that was different from that found in the rest of the cornea but that helped to maintain corneal thickness. CONCLUSIONS: The amniotic membrane graft allows for correct epithelialization in cases of neurotrophic corneal ulcer. Once the amniotic membrane is reabsorbed, it is replaced by a new fibrotic stroma, which can reduce corneal transparency. In corneas that have no stromal vascularization, the graft may remain in the stroma for many months, compromising corneal transparency during this period.

Amniotic membrane implantation as a therapeutic contact lens for the treatment of epithelial disorders.

PURPOSE: To evaluate the efficacy and safety of amniotic membrane implantation as a therapeutic contact lens in the treatment of different epithelial defects without stromal ulceration. METHODS: We used amniotic membrane implantation as a therapeutic contact lens in 20 consecutive patients with epithelial defects. Group 1 included 10 patients with persistent epithelial defects that did not respond to medical treatment. Group 2 included 10 patients with surgically induced epithelial defects. RESULTS: No intra- or postoperative complications were observed. The amniotic membrane implant remained in place for a mean of 12.5 days (range, 3-34). In 11 of the 20 patients, the amniotic membrane implant became detached within the first 8 days. When the corneal implant was postoperatively covered with a soft contact lens, this time increased. In group 1, complete epithelialization was achieved in three of the four cases in which the amniotic membrane remained in place for 2 or more weeks. There were no cases of complete epithelialization in which the implant remained in place for less than 1 week. In group 2, epithelialization was achieved in all cases, regardless of the time that the implant remained in place. CONCLUSION: Amniotic membrane implanted as a therapeutic contact lens can be an effective and safe option for the treatment of different epithelial defects. In patients with persistent epithelial defects, the number of cases with complete postoperative epithelialization was higher when the amniotic membrane remained in place longer. The early detachment of the amniotic membrane implant remains a major problem, even with the use of multiple fixation sutures.