Biomarkers in retinopathy of prematurity: a systematic review and meta-analysis.

Aim: Retinopathy of prematurity is a significant global cause of childhood blindness. This study aims to identify serum biomarkers that are associated with the development of ROP. Methods: A systematic review and meta-analysis was conducted using PRISMA guidelines. Three databases were searched (Pubmed, Scopus and Web of Science) from 2003 to March 2023. Only studies investigating serum biomarker levels in preterm infants (<37 weeks gestation) were included. Results: Meta-analysis suggests that low serum IGF-1 levels have a strong association with the development of ROP [SMD (95% CI) of -.46 [-.63, -.30], p < .001]. Meta-analysis suggests that higher serum glucose levels were associated with the development of ROP [SMD (95% CI) of 1.25 [.94, 1.55], p < .001]. Meta-analysis suggests that thrombocytopenia is associated with the development of ROP [SMD (95% CI) of -.62 [-.86, -.37], p < .001]. Conclusion: Low levels of serum IGF-1, high levels of serum glucose and thrombocytopenia all appear to have the strongest association with the development of ROP out of the 63 biomarkers investigated in this review. These associations highlight their potential use as diagnostic biomarkers in ROP, though further research is needed to establish the exact relationship between these biomarkers and disease pathogenesis.

Local anaesthetic eye drops for prevention of pain in preterm infants undergoing screening for retinopathy of prematurity.

BACKGROUND: Screening examinations for retinopathy of prematurity (ROP) are performed routinely in the neonatal intensive care unit and are a recognised cause of pain in the newborn. OBJECTIVES: To determine the effect of instillation of topical anaesthetic eye drops compared with placebo or no treatment on pain in infants undergoing ROP screening. SEARCH STRATEGY: We used the standard search strategy of the Cochrane Neonatal Review Group. This included a search of the Cochrane Neonatal Group register and the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 10, 2010). We identified relevant studies by searching the following: (1) computerised bibliographic databases: MEDLINE (1966 to October 2010), EMBASE (1988 to October 2010) and Web of Science (1975 to March 2010; (2) the Oxford Database of Perinatal Trials. We searched electronically abstracts from PAS from 2000 to 2010 and handsearched abstracts from ESPR from 2000 to 2009. SELECTION CRITERIA: All randomised, or quasi-randomised controlled trials, or randomised cross-over trials. DATA COLLECTION AND ANALYSIS: We used the standard methods of the Cochrane Neonatal Review Group. MAIN RESULTS: We identified two studies for inclusion. Both studies were randomised cross-over trials performed in single centres. Both studies used the Premature Infant Pain Profile (PIPP) score as a measure of pain response. Different methods of evaluating PIPP scores are presented including the absolute PIPP score, a PIPP score > 10 or > 12 and an increase in PIPP ≥ 4 from the baseline value. There is a nonsignificant reduction in pain scores at one minute and a nonsignificant increase at five minutes post insertion of the speculum. PIPP score > 12 at one minute resulted in a statistically significant reduction in the number of patients who experienced pain (typical risk ratio (RR) 0.56, 95% CI 0.36 to 0.89; typical risk difference (RD) -0.23, 95% CI -0.39 to -0.86; number needed to treat to benefit (NNTB) 4). When pain was defined as an increase in PIPP > 4 there was a statistically significant reduction in the absolute number of patients who experienced pain at one minute (typical RR 0.70, 95% CI 0.52 to 0.94; typical RD -0.19, 95% CI -0.34 to -0.04; NNTB 5.3). AUTHORS' CONCLUSIONS: The administration of topical proparacaine 30 seconds prior to the ophthalmological evaluation was associated with a reduction in pain scores especially at the time of speculum insertion. However, despite treatment, screening remains a painful procedure and the role of nonpharmacological and pharmacological intervention including different local anaesthetic agents should be ascertained in future randomised trials.

Paediatric cataract in the uveitis setting.

BACKGROUND/AIMS: Cataract formation is common in uveitis and is visually more threatening in the paediatric cohort due to the risk of amblyopia. In addition, paediatric uveitis can often be difficult to manage. We report our experience with IOL placement in cataract surgery in the setting of paediatric uveitis. METHODS: This non-comparative, retrospective interventional case series examined our cases of paediatric cataract occurring in patients with uveitis from 2003 to 2016. Parameters examined included visual acuity (VA), underlying diagnosis, immunosuppression status, intra-operative complications and requirement for further surgery. RESULTS: In total, 10 eyes of seven patients were identified. The mean age at diagnosis of uveitis was 7.7 years (range 5.2-14 years) with onset of cataract at a mean of 29.3 months later (range 0-66 months). Three cases were bilateral and four cases were unilateral. Final visual outcomes were excellent with 80% showing improvement in VA achieving greater than 6/9.5 (p < 0.05). These patients had significant co-morbidities with concurrent glaucoma, band keratopathy and cystoid macular oedema. Uveitis was quiet for a minimum of 6 months in all cases prior to surgery with augmentation of immunosuppression pre-operatively as well as intra-operative local or intra-venous steroids. Tight post-operative care was necessary as 80% developed further flare-up of uveitis requiring increased immunosuppression and surgical interventions to manage their uveitis. CONCLUSION: Paediatric uveitis patients who develop cataract can have good visual outcomes with IOL insertion at the time of surgery when there is aggressive control of uveitis in the pre, peri and post-operative period.

Migration to aphakia and contact lens treatment is the trend in the management of unilateral congenital cataract in Britain and Ireland.

BACKGROUND: The Infant Aphakia Treatment Study (IATS) compared the treatment of unilateral cataract in infants aged 1-6 months with primary intraocular lens (IOL) implantation vs aphakia with contact lens (CL) correction. AIMS: This study aims to assess the current trends in the treatment of unilateral congenital cataract in infants less than 6 months at surgery in the UK and Ireland. METHODS: An anonymous survey was emailed to the 200 members of the BIPOSA mailing list with 14 questions to assess treatment choice (primary intraocular lens (IOL) vs aphakia with contact lens (CL)), reasons for this choice, and assessment of local CL services. RESULTS: There were 56 respondents, 39 of whom completed the entire survey. Aphakia with CL was the treatment choice for 74.4% of respondents. A quarter (25.6%) of respondents said they were performing primary IOL implantation prior to the publication of the Infant Aphakia Treatment Study (IATS), but now choose aphakia with CL. Amongst the 20.5% (n = 8) of respondents who chose primary IOL implantation, 5 attributed their choice to "inadequate CL service". The majority (84.6%) of respondents rated their infant CL service as either "good" or "very good". CONCLUSION: Aphakia with CL rehabilitation was the most common approach to the treatment of unilateral congenital cataract in infants less than 6 months in this study. The results of the IATS appear to have influenced a change in practice from primary IOL implantation to aphakia and CL visual rehabilitation in approximately one quarter of those surveyed.

Characterization of optical coherence topography findings in Kenny-Caffey syndrome.

An unusual congenital syndrome was first reported in 1966 by Kenny and Linarelli, who described two patients with dwarfism, cortical thickening of the long bones, transient hypocalcemia, and normal intelligence,(1) the radiological features in the condition being reported by Caffey.(2) The constellation of dwarfism, medullary stenosis, transient hypocalcemia, and ophthalmologic abnormalities has been classically recognized as Kenny-Caffey syndrome with additional manifestations ranging from hypoplastic nails, persistent neutropenia, abnormal T-cell function, and neonatal liver disease.(3) Ocular findings range from uncomplicated nanophthalmos with hypermetropia to extreme pseudopapilloedema, vascular tortuosity, and macular crowding. Other reported ophthalmic findings include bilateral band keratopathy,(4) bilateral optic atrophy,(5) and myelinated nerve fibers.(6) We report two cases of Kenny-Caffey syndrome with an ellipsoid macular fold orientated horizontally involving the fovea and document this unusual feature with optical coherence topography (OCT).

Rhombencephalosynapsis presenting antenatally with ventriculomegaly/hydrocephalus in a likely case of Gomez-López-Hernández syndrome.

Rhombencephalosynapsis is a rare cerebellar malformation that can be associated with anomalies of the cerebral hemispheres and variable degrees of neurodevelopmental delay. A syndromic association, comprising rhombencephalosynapsis, developmental delay, scalp alopecia and trigeminal anaesthesia (Gomez-López-Hernández syndrome) has been described in seven individuals. We report the case of a 2-year-old boy with rhombencephalosynapsis, and review the evidence for a possible diagnosis of Gomez-López-Hernández syndrome. We also discuss other malformations reported in combination with rhombencephalosynapsis, and consider the possibility that a genetic aetiology for syndromic and nonsyndromic forms of rhombencephalosynapsis may be established with more detailed clinical and genetic studies.

Ocular complications of autoimmune polyendocrinopathy syndrome type 1.

PURPOSE: To report the ocular complications in a series of patients with autoimmune polyendocrinopathy syndrome, type 1 (APS1). METHODS: A retrospective study of 17 patients with APS1 syndrome treated at the department of ophthalmology, Our Lady's Hospital for Sick Children in Crumlin, Dublin, Ireland. All patients had clinical manifestations of the disease in keeping with the diagnostic criteria of APS1. Each patient had a comprehensive ophthalmic history taken and examination, including ocular symptoms, best-corrected visual acuity, slit-lamp biomicroscopy, tear film evaluation, and dilated ophthalmoscopic examination. RESULTS: Six of 17 patients (35%) had corneal changes. Two patients (12%) had severe keratoconjunctivitis requiring hospitalization and intensive topical steroids and lubricants. The inflammation resulted in visual acuity reduction in one patient secondary to central corneal scarring. Other ocular findings included reduced tear production, as tested with Schirmers tear strips (63%), lens opacities (18%), hypotrichosis (12%), hypertrichosis (5.9%), anisometropic amblyopia (5.9%), and myopia (5.9%). CONCLUSIONS: The most common and clinically important ocular manifestation of APS1 was keratoconjunctivitis associated with dry eye. This can result in progressive corneal scarring and vision loss.

Involution of retinopathy of prematurity after laser treatment: factors associated with development of retinal detachment.

PURPOSE: To identify specific features during the process of involution of retinopathy of prematurity after treatment at threshold that are associated with development of a retinal detachment. DESIGN: Retrospective case series. METHODS: The evolution of retinal detachments over time was analyzed retrospectively in 262 treated eyes of 138 infants. Specific features hypothesized to be associated with development of a retinal detachment were analyzed, including vitreous organization defined as clinically important, active stage 3 disease and active plus disease more than 21 days after treatment, and vitreous hemorrhage defined as clinically important. RESULTS: A retinal detachment developed in 36 (13.7%) of 262 eyes. Vitreous organization meeting our clinically important definition was associated with a 31-fold (confidence interval [CI] 5.37-183.63; P < .0001) and 13-fold (CI 2.97-58.59; P < .0001) increase in the odds for retinal detachment for right and left eyes, respectively. Vitreous hemorrhage defined as clinically important was associated with a 38-fold (CI 2.69-551.19; P = .007) and 15-fold (CI 1.65-144.12; P = .02) increase in the odds for retinal detachment for right and left eyes, respectively. The timing of retinal detachment relative to vitreous hemorrhage was not determined. Prolonged activity of Stage 3 disease or plus disease more than 21 days after treatment was not associated with development of a retinal detachment. CONCLUSIONS: Clinically important vitreous organization and vitreous hemorrhage were predictive for development of a retinal detachment. Evaluation of preemptive reintervention strategies for eyes at highest risk for developing a retinal detachment may be reasonable.

Involution of threshold retinopathy of prematurity after diode laser photocoagulation.

OBJECTIVE: To characterize the process of involution of threshold retinopathy of prematurity after transpupillary diode laser photocoagulation. DESIGN: Retrospective case series. PARTICIPANTS: Neonates with threshold retinopathy who underwent diode laser photocoagulation of the peripheral avascular retina. METHODS: A retrospective chart review was done of the weekly examination records of infants treated for threshold disease. Features that were studied included the presence of residual stage 3 neovascularization, plus disease, and development of retinal detachment (RD). MAIN OUTCOME MEASURES: Timing of full involution and/or development of an RD. RESULTS: Of 262 eyes of 138 infants treated, full involution without RD was seen in 8%, 43%, 64%, 73%, and 86% of eyes at postoperative weeks 1, 2, 3, 4, and 9+/-3, respectively. Retinal detachments were diagnosed cumulatively in 0%, 1.5%, 4.2%, 6.5%, and 14% of eyes at weeks 1, 2, 3, 4, and 9+/-3, respectively. CONCLUSIONS: Full involution of laser-treated threshold retinopathy of prematurity required more than 2 weeks in more than half of treated eyes. Most RDs were not detected until > or =3 weeks after treatment.

Corneal thickness in children.

OBJECTIVE: To determine normal central and paracentral corneal thickness measurements in the pediatric population and to determine if these measurements are consistent across different pediatric age groups and different racial groups. DESIGN: Prospective observational case series. METHODS: Pachymetry measurements were performed on 198 eyes of 108 children. The measurements were taken centrally as well as at four paracentral sites 3 mm from the corneal center at the 3, 6, 9, and 12 o'clock positions. The two-tailed t test was used for comparison of the continuous means for values of corneal thickness. Analysis of variance (ANOVA) was performed to determine differences among age and ethnic groups RESULTS: The mean central corneal thickness (CCT) was 549 +/- 46 microm. Paracentral corneal thickness mean values, as measured 3 mm from the corneal center, were as follows: superior, 575 +/- 52 microm; nasal, 568 +/- 50 microm; inferior, 568 +/- 51 microm; and temporal, 574 +/- 47 microm. The mean CCT values were significantly thinner than at each of the mean paracentral points (P < .05 for each comparison, paired t test). Paracentral corneal thickness measurements demonstrated no significant differences between locations (P > .05, variance analysis). The mean CCT +/- SD for each age group was as follows: 6 to 23 months, 538 +/- 40 microm; 2 to 4 years, 546 +/- 41 microm; 5 to 9 years, 566 +/- 48 microm; and 10 to 18 years, 554 +/- 35 microm (ANOVA P = .012). ANOVA performed on central pachymetry values demonstrated no significant differences among racial subgroups. CONCLUSIONS: Pediatric central and paracentral corneal thicknesses increase slowly over time and reach adult thicknesses at 5 to 9 years of age.

Surgical management of the lens and retrolenticular fibrotic membranes associated with persistent fetal vasculature.

PURPOSE: To evaluate a surgical technique that allows safe, effective, near-total removal of retrolenticular fibrotic membranes in eyes with persistent fetal vasculature syndrome (PFVS). SETTING: Pediatric ophthalmologist's academic practice. METHODS: This retrospective review comprised 5 children (6 eyes) who had excision of a retrolenticular fibrovascular membrane during cataract surgery over a 6-month period. Long, spoke-like radial incisions of the membrane were made to the ciliary processes, resulting in wedge-shaped segmentation of the membrane. A vitrector was used to excise each wedge and perform an anterior vitrectomy. RESULTS: The mean age at time of surgery was 8 months (range 1 to 24 months) and the mean follow-up, 9 months (range 2 to 13 months). The membrane was successfully removed in all eyes. One eye of a patient who did not comply with the postoperative medical regimen or follow-up developed pupillary block glaucoma requiring pupilloplasty and trabeculectomy. CONCLUSION: This anterior surgical technique to remove tenacious retrolenticular membranes associated with PFVS was effective, allowing near-total removal of the offending fibrovascular membrane.

Epithelial healing and ocular discomfort after photorefractive keratectomy in children.

OBJECTIVE: To document the rate of healing of the corneal epithelial defect created by photorefractive keratectomy (PRK) and the degree of discomfort experienced by children treated with PRK. SETTING: Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA. METHODS: Ten patients between 3 and 10 years of age were treated with PRK for severe anisometropia. All had anisometropic amblyopia refractory to conventional therapy with glasses or contact lenses and occlusion therapy. The size of the corneal epithelial defect was documented daily until the defect healed completely. Postoperative discomfort was documented daily using a pain-assessment scale until the epithelial defect healed completely. RESULTS: Two children were treated with PRK for hyperopic anisometropia; the rest were treated for myopic anisometropia. Cumulatively, the corneal epithelium had healed completely by day 3 in 6 patients (60%), by day 4 in 9 patients (90%), and by day 5 in all patients. The mean healing time was 3.5 days. Patients experienced mild discomfort on the day of surgery and on the first postoperative day. They had minimal pain on day 2. After day 2, no patient reported pain or other discomfort. CONCLUSIONS: The corneal epithelial defect created by PRK healed promptly and was associated with minimal postoperative discomfort in children treated with the protocol described.

Successful implementation of a protocol for photorefractive keratectomy in children requiring anesthesia.

PURPOSE: To describe a protocol for treating children with photorefractive keratectomy (PRK) under general anesthesia and to review intraoperative and postoperative complications. SETTING: Institutional academic practice. METHODS: Nine patients between 3 years and 9 years of age were treated with PRK under general anesthesia for anisometropia with unilateral high myopia or high hyperopia and amblyopia of the affected eye. Induction of anesthesia and the surgical procedure were carried out in separate rooms. The laser beam was centered on the entrance pupil, and eye position was monitored throughout the procedure. Specific precautions were taken before and during the procedure to prevent unwanted effects of inhalational anesthetic agents on laser performance. RESULTS: All children did well, with no anesthesia-related or treatment-related complications. CONCLUSIONS: Our protocol for PRK under general anesthesia was effective and efficient in children who were unable to cooperate for the procedure using local anesthesia. It can be adapted for laser in situ keratomileusis and other refractive surgical procedures in children and uncooperative adults.

Molecular analysis of the pediatric ocular surface for fungi.

PURPOSE: To analyze the conjunctival flora of individuals 21 years of age or less for fungi using polymerase chain reaction (PCR) methodology. METHODS: Before povidone-iodine antisepsis, eye-swab specimens were collected from adolescent corneal donors preceding corneal excision and from children during preparation for strabismus surgery. Nucleic acid was extracted from the specimens and analyzed by PCR using primers designed for the detection of broad-spectrum fungal DNA and of Candida albicans-specific DNA. RESULTS: Twelve (38%) of 32 eye donor surfaces and 7 (23%) of 30 patient samples were positive for fungal DNA (P = 0.1). C. albicans DNA was detected in 6 (19%) of the decedents' eyes but from none of the surgical patients (P = 0.04). CONCLUSIONS: Fungi were present on the normal ocular surface of children and adolescents. C. albicans was more likely to be found postmortem than pre-surgically.

Up-slanting palpebral fissures and oblique astigmatism associated with A-pattern strabismus and overdepression in adduction in spina bifida.

PURPOSE: To describe the spectrum of adnexal and ophthalmologic features in spina bifida. METHODS: A retrospective review of the medical records of 73 patients was conducted. Data concerning ocular motility, palpebral fissure orientation, presence and axis of astigmatism, visual acuity, amblyopia, and stereopsis were analyzed. RESULTS: Forty-three (59%) of the 73 patients had strabismus: 28 (65%) had esotropia, 12 (28%) had exotropia, and 3 (7%) had orthotropia in primary gaze and a significant A-pattern. Of the strabismic patients, 20 (47%) had an A-pattern, of which 13 (65%) demonstrated overdepression in adduction. Twenty-seven (84%) of the 32 patients with documented palpebral fissure orientation had exaggerated up-slanting palpebral fissures. Forty-nine (77%) of 64 patients exhibited astigmatism greater than 0.75 D in at least 1 eye, with a mean power of 1.6 D. The axis of cylinder was oblique in 76% of these patients. The astigmatic axis was consistently oriented perpendicular to the eyelid fissure orientation in the group of patients with up-slanting palpebral fissures, with the mean axis of cylinder being incyclorotated (OD axis, 77; OS axis, 108). Up-slanting palpebral fissures were associated with a 15-fold increased chance that the axis of cylinder would be incyclorotated (P =.07, chi-square test). An orbital computed tomographic (CT) scan of a spina bifida patient with A-pattern esotropia, overdepression in adduction, and up-slanting palpebral fissures demonstrated significant incyclorotated extraocular muscle pulley heterotopy. CONCLUSIONS: Exaggerated up-slanting palpebral fissures are a prominent feature in spina bifida. Up-slanting palpebral fissures in spina bifida patients are associated with incyclorotated oblique astigmatism, A-pattern strabismus, and overdepression in adduction. These associations might be related to an anomaly of orbital skeletal or extraocular muscle pulley development. Further prospective study is encouraged.

Accuracy of clinical estimation of abnormal head postures.

PURPOSE: Correct diagnosis and analysis of an abnormal head posture (AHP) is critical in the clinical assessment of patients with strabismus and nystagmus. This study was performed to evaluate the accuracy and precision of clinicians estimating the degree of face turns and head tilts. DESIGN: Experimental simulation study. PARTICIPANTS: Two normal volunteers and 4 pediatric and 1 general ophthalmologists. METHODS: Using a cervical range of motion (CROM) device, single axis head position (chin up, chin down, right and left face turn, and head tilts) were modeled, and clinical estimates of the amount of deviation were estimated by the four ophthalmologists. Each of the 4 examiners estimated 108 single axis AHPs in a randomized and masked pattern. MAIN OUTCOME MEASURES: Accuracy of clinical estimation of AHP, intra- and inter- observer variability, and variability in terms of head position. RESULTS: The examiners tended to overestimate the AHPs by an average of 5 degrees (P less than 0.001). Two "right" positions (right head tilt and face turn) were the worst. The average error was 10 +/-8 degrees for the right tilt and turn positions, while the error for all other positions, excluding these two, averaged 0 +/-10 degrees (P = 0.0001). CONCLUSIONS: Clinical estimation of an AHP by ophthalmologists is in part remarkably accurate, even if imprecise, but some positions, notably right head tilt and right face turn in this study, were significantly overestimated. Objective methods of measuring AHPs such as the CROM may be preferable for these RHT and RFT AHPs and when maximum precision is needed for tracking changing AHPs or deviations.

Quantification of incidental needle and suture contamination during strabismus surgery.

PURPOSE: To quantify the level of bacterial contamination of needles and sutures immediately after use in strabismus surgery. METHODS: Strabismus surgery was performed on 56 eyes from 31 pediatric strabismus surgical cases. Preoperative site preparation included instillation of 5% povidone-iodine in the conjunctival fornices in all cases. A total of 124 needles and 127 sutures were cultured immediately after final scleral passage. RESULTS: Seventeen of the 31 cases (54.8%) produced at least one positive specimen. Nineteen per cent of the needles and 25.2% of the sutures were culture positive. The bulk of positive specimens (96.7% of needles, 91.3% of sutures) produced 3 or less colony forming units, corresponding to 7 or less total viable organisms per needle or sutures in accordance with the dilution scheme. Coagulase-negative staphylococci overwhelmingly predominated. CONCLUSIONS: Needles and sutures used in strabismus surgery can become contaminated during surgery despite preoperative povidone-iodine preparation. The number of viable bacterial contaminants is usually below the level known to consistently produce experimental endophthalmitis. However, a few needles and sutures carried a high contaminant load, suggesting the possibility that contaminated needles and sutures could potentially cause postoperative intraocular infection.

Major review: the clinical spectrum of pediatric myasthenia gravis: blepharoptosis, ophthalmoplegia and strabismus. A report of 14 cases.

BACKGROUND: Myasthenia gravis is infrequently encountered in pediatric ophthalmology practice. The purpose of this study is to evaluate the clinical spectrum of this condition in children and to identify factors that may aid the clinician in its diagnosis and management. SUBJECTS AND METHODS: A retrospective chart review was performed on all pediatric patients presenting with myasthenia gravis to the Departments of Pediatric Ophthalmology and Neuro- ophthalmology at the Texas Children's Hospital from 1989-1999. Information regarding mode of presentation, myasthenic classification, ocular and systemic involvement, diagnostic investigations, therapy and outcome was collected and evaluated. RESULTS: Fourteen patients were included in the study whose ages ranged from 1-17 years at presentation. One patient had congenital myasthenia gravis and 13 had juvenile myasthenia gravis. Thirteen of 14 (93%) patients presented with ocular findings; two of whom had associated systemic disease at presentation. Six of 14 (43%) patients had systemic involvement during the course of their illness, of whom three (21%) had respiratory compromise requiring assisted ventilation. Thirteen of 14 (93%) patients received pyridostigmine as first line treatment. Ten of 14 (71%) patients had a favorable response. A favorable response was defined as improvement in the extraocular motility to within 10 prism diopters of orthotropia with resolution of the blepharoptosis. Three of 14 patients (21%) received a combination of pyridostigmine and steroids, all of whom had a favorable response. Seven of 14 patients (50%) underwent thymectomy; all had a favorable response. Two of 14 patients (14%) required both blepharoptosis and strabismus surgery. CONCLUSION: Pediatric myasthenia gravis may present initially to the ophthalmologist and should be considered in any pediatric patients with blepharoptosis and an ocular motility disturbance. Prompt diagnosis may be associated with significant reduction in morbidity. A favorable response to medical and surgery therapy was noted in most of our patients.

Spontaneous intracranial hypotension with unique strabismus due to third and fourth cranial neuropathies.

PURPOSE: To report an atypical case of Spontaneous Intracranial Hypotension (SIH) with bilateral trochlear nerve palsies and an oculomotor nerve palsy. DESIGN & METHOD: Case report and literature review. RESULTS (CASE REPORT): A 42 year old man was treated for SIH. His neurological symptoms resolved following neurosurgical treatment with the exception of diplopia due to bilateral trochlear nerve palsies and a pupil-sparing oculomotor nerve palsy. The cranial nerve palsies are believed to be secondary to brainstem ischemia and compression occurring during the acute phase of events. They did not spontaneously improve, but were treated successfully with eye muscle surgery. CONCLUSION: SIH is a rare disease that has been associated with a variety of symptoms and signs including cranial neuropathies. A diagnosis of SIH should be considered in a patient presenting with headache, diplopia secondary to cranial neuropathy and typical radiologic features. This is the first reported case in which bilateral trochlear nerve paresis has been reported in association with this condition.