Characteristics, fates and complications of long-term silicone oil tamponade after pars plana vitrectomy.

BACKGROUND: Silicone oil tamponade has become a mainstay in treatment of advanced retinal detachment due to multiple etiologies. The aim of this study is to assess the characteristics, fates and complications of long-term silicone oil tamponade after par plana vitrectomy (PPV), and to compare the outcomes of different silicone oil viscosities used in a cohort of consecutive patients. METHODS: This is a retrospective comparative case series of eyes undergoing vitrectomy with silicone oil tamponade for retinal detachment by a single surgeon using different oil viscosities that were followed for one year with the silicone oil in situ. Visual acuity (VA), intraocular pressure (IOP) and complications associated with the follow up period were analyzed and compared. RESULTS: Eighty-five eyes of 85 patients were included in this study. Forty three patients had 1000 centistoke (cs) oil injected and 42 patients had 5000cs oil utilized. Demographic, cause of retinal detachment and preoperative ocular characteristics were similar in both groups. Long term complications in both groups included ocular hypertension (67.4% vs 66.7%), keratopathy due to silicone oil emulsification and migration to the anterior chamber (7.0% vs 11.9%), recurrent retinal detachment (4.7% vs 19%) and epiretinal membrane formation (7% vs 19%). In the 1000cs oil group, there was no significant difference between baseline IOP and any subsequent visit. There was a significant difference between baseline IOP and visits at day 1 (with IOP difference of 2.61 mmHg (±6.5)) (p = 0.028), 1 month (with IOP difference of 3.52 mmHg (±8.1)) (p = 0.026), 4 months (with IOP difference of 6.38 mmHg (±9.3)) (p = 0.005), and one year (with IOP difference of 4.24 mmHg (±11.1)) (p = 0.048), all higher in the post-operative period in the 5000cs oil group. Excluding the first post-operative day, no significant difference was found for VA between baseline visits and subsequent visits for either silicone oil groups. CONCLUSION: In this cohort of patients with long-term silicone oil tamponade after PPV to treat retinal detachment, IOP increased significantly in patients who received 5000cs silicone oil. There was no significant difference between other complication rates in patients receiving either oil viscosities. Long term silicone oil tamponade remains a viable option in certain cases, and a vigilant follow up for complications is necessary to limit any adverse effects and improve visual and surgical outcomes.

Platelet lysate promotes re-epithelialization of persistent epithelial defects: a pilot study.

PURPOSE: To study the use of autologous platelet lysate prepared in a standardized method for the healing of persistent corneal epithelial defects (PED). STUDY DESIGN: Clinical and experimental investigation. METHODS: In this prospective pilot study (ClinicalTrials.gov identifier NCT02979912), ten patients with a PED duration of a minimum 14 days were included. Autologous platelet lysate was prepared in a standardized methodology. Repeated freeze-thaw cycles were used to lyse the platelets. Patients were advised to apply the eye drops four times a day and were evaluated at baseline and on days 7, 14, 21, 28. RESULTS: No adverse events were reported due to the use of undiluted autologous platelet lysate. A total of 70% of patients had complete re-epithelialization within 28 days. Of these, 40% healed within 14 days (effective group) and 30% within 28 days (partially effective group). CONCLUSIONS: Undiluted autologous platelet lysate, prepared according to a standardized methodology, is a safe and effective adjunct therapy for the treatment of PED.

Enucleation and evisceration at a tertiary care hospital in a developing country.

BACKGROUND: To analyze the demographics, indications, and surgical outcomes of anophthalmic surgery (enucleation and evisceration) at Jordan University Hospital during a 5-year period. METHODS: We conducted a retrospective chart review of patients who had undergone evisceration or enucleation between August 2006 and June 2011. The data collected included age at time of surgery, sex, affected eye, surgical indication, implant size, and postoperative complications. RESULTS: Anophthalmic surgery was performed for 68 eyes of 67 patients during the study period (42 (62 %) eviscerations and 26 (38 %) enucleations). Forty-three patients (64 %) were men, and 40 (59 %) eyes were right eyes. Trauma was the leading cause for anophthalmic surgery in 40 % of cases followed by a blind painful eye secondary to glaucoma (19 %) in the enucleation group and endophthalmitis (28.6 %) in the evisceration group. The most common anophthalmic surgery complication was wound dehiscence in 11.5 % of patients in the enucleation and 9.5 % in the evisceration groups. The mean and median sizes of the implants for evisceration were 16.6 and 18.0 mm, respectively; for enucleation, both were 20 mm. CONCLUSIONS: Evisceration was the preferred anophthalmic surgery in our series unless contraindicated. Trauma was the most common predisposing factor for evisceration and enucleation in our tertiary care center followed by blind painful eyes and endophthalmitis. The most common complication was wound dehiscence in both groups.

Prevalence of thrombophilic genetic factors among patients with retinitis pigmentosa.

PURPOSE: To determine the prevalence of thrombophilic factors in patients with retinitis pigmentosa (RP). METHODS: Fifty consecutive patients with RP and 50 controls matched by age and gender were tested for the presence of the following mutations: factor II (GA20210), factor V Leiden (GA1691), methylenetetrahydrofolate reductase (CT677), factor XIIIa (Val→Leu), ß-fibrinogen (GA455), tumor necrosis factor receptor (TNFRII) (M196R), plasminogen activator inhibitor-1 (PAI-1) (4 G/5 G), and plasminogen activator inhibitor-1 (PAI-1) (GA844). RESULTS: The following heterozygous mutations were found in patients/controls: factor V Leiden (12/14), factor XIIIa (20/30), methylenetetrahydrofolate reductase 677 TT (48/52), ß-fibrinogen GA455 (36/36), TNFRII (M196R) (40/42), PAI-1 4 G/5 G (40/48), and PAI-1 GA844 (50/52). The difference between patients with RP and the control group was not statistically significant for the prevalence of any of the studied factors (P > 0.05). CONCLUSION: In this study, thrombophilic mutations were not increased in patients with RP. Thrombophilic mutations do not seem to be risk factors for RP. Routine investigation of hereditary thrombophilia in these patients is not justified.

Long-term efficacy and safety of botulinum toxin A injections to treat blepharospasm and hemifacial spasm.

BACKGROUND: To evaluate efficacy and safety of botulinum toxin A injections after more than 10 consecutive years of treatment for benign essential blepharospasm and hemifacial spasm. DESIGN: Retrospective chart review at university-affiliated hospital. PARTICIPANTS: Study consisted of 64 patients treated with botulinum toxin A injections between October 2005 and May 2006. METHODS: Inclusion criteria included patients treated with at least one annual botulinum toxin-A injection for more than 10 consecutive years. Data collected included diagnoses and patient characteristics, injection dates, doses administered at each visit, response scores, duration of effect, and adverse events. MAIN OUTCOME MEASURES: Included changes in doses, response scores, duration of effects, and adverse events between the first and last botulinum toxin A injections. RESULTS: Thirty-two of 64 patients (mean age at first injection, 57.2 ± 12.4 years; 25 women) met the inclusion criteria. The mean duration of follow up was 14.1 ± 3.1 years (range 10-20 years; mean total visits 44.4 ± 19). A higher mean injection dose per visit was administered during the last year compared with the first year (26.8 ± 10.3 vs. 22.5 ± 7.5 units, respectively) (P = 0.003). The mean durations of effect during the first and last years were 12.4 ± 7.1 and 14.6 ± 7.0 weeks, respectively (P = 0.076). There were no significant differences between genders or between benign essential blepharospasm and hemifacial spasm subgroups. The most common adverse events were ptosis, lagophthalmos and dry eye. CONCLUSIONS: Botulinum toxin A is an effective, safe, long-term treatment for patients with benign essential blepharospasm and hemifacial spasm. Sustained treatment efficacy required higher doses; however, fewer adverse reactions developed.

Bilateral simultaneous pyogenic granuloma after perforated punctal plug insertion.

A 44-year-old woman with recurrent attacks of bilateral adenoviral keratoconjunctivitis infections presented with excessive tearing of 5 months' duration. Slit-lamp examination showed bilateral punctal stenosis. Tearing improved for 2 months after insertion of perforated silicone punctal plugs, after which she had a bilateral foreign-body sensation due to pyogenic granuloma pushing through the lumen of the silicone plugs. The foreign-body sensation improved after removing the plugs and a short course of topical steroid-antibiotic eye drops.

Loss of CHSY1, a secreted FRINGE enzyme, causes syndromic brachydactyly in humans via increased NOTCH signaling.

We delineated a syndromic recessive preaxial brachydactyly with partial duplication of proximal phalanges to 16.8 Mb over 4 chromosomes. High-throughput sequencing of all 177 candidate genes detected a truncating frameshift mutation in the gene CHSY1 encoding a chondroitin synthase with a Fringe domain. CHSY1 was secreted from patients' fibroblasts and was required for synthesis of chondroitin sulfate moieties. Noticeably, its absence triggered massive production of JAG1 and subsequent NOTCH activation, which could only be reversed with a wild-type but not a Fringe catalytically dead CHSY1 construct. In vitro, depletion of CHSY1 by RNAi knockdown resulted in enhanced osteogenesis in fetal osteoblasts and remarkable upregulation of JAG2 in glioblastoma cells. In vivo, chsy1 knockdown in zebrafish embryos partially phenocopied the human disorder; it increased NOTCH output and impaired skeletal, pectoral-fin, and retinal development. We conclude that CHSY1 is a secreted FRINGE enzyme required for adjustment of NOTCH signaling throughout human and fish embryogenesis and particularly during limb patterning.

Intravitreal bevacizumab in the treatment of diabetic ocular neovascularization.

PURPOSE: To evaluate the efficacy of intravitreal bevacizumab for treating diabetic retinal and/or iris neovascularization. METHODS: Consecutive, prospective, interventional case series study of 60 eyes with diabetic retinal and/or iris neovascularization. Patients had a complete ocular examination before receiving 1.25 mg (0.05 mL) of intravitreal bevacizumab. Abnormal new vessels elsewhere in the retina, optic disk, or iris were graded by size and associated hemorrhage or glaucoma. Patients had complete postinjection ophthalmic evaluations with regrading of the abnormal new vessels at 3 months and 6 months. The main outcome measures included clinical partial or total regression of abnormal new vessels, changes in visual acuity, and complications related to the intravitreal injections. RESULTS: Twenty-six patients (47.3%) were men (mean age, 59 years). Abnormal new vessel regression at 6 months occurred in 65% of new vessels of the iris (P = 0.001), 45% of new vessels of the optic disk (P = 0.009), and 43% of new vessels elsewhere (P = 0.008). The visual acuity improved in 20% of eyes, which was not significant (P = 0.235); the visual acuity deteriorated in 23% of eyes (P = 0.163). No systemic or ocular side effects developed except for postinjection hypotony in one eye. CONCLUSION: Intravitreal bevacizumab is a well-tolerated medication that causes regression of abnormal diabetic neovascularization. New vessels of the iris responded more than new vessels of the optic disk and new vessels elsewhere.

Association Between Keratoconus and Shoulder Dislocation: A Cross-Sectional Study.

Introduction Few studies have highlighted the correlation between shoulder dislocation and keratoconus (KC). This study aimed to examine the association between KC and shoulder dislocation using patients with KC and matched controls. Methods This cross-sectional study was conducted at Jordan University Hospital. We included patients diagnosed with KC from Jordan University Hospital's Ophthalmology Department between 2015 and 2018. We also included age- and sex-matched controls recruited randomly from fitness centers and shopping malls. All participants had complete ophthalmic and orthopedic assessments. KC was diagnosed by clinical examination followed by Pentacam (Scheimpflug Images, Oculyzer, WaveLight, Alcon, USA) confirmation. Results A total of 238 patients, with a mean age of 29.53 (±11.20) years, were included in this study. They were 144 (60.5%) men and 94 (39.5%) women. Moreover, 120 (50.4%) had KC while 118 (49.6%) did not have KC. Only 11 (4.6%) patients had previous shoulder dislocation. We did not find a significant difference in the frequency of shoulder dislocation between patients with and without KC (p = 0.512). Conclusion This study provides further evidence on the lack of association between shoulder dislocation and KC, an association that was presumed due to shared collagen characteristics.

Characteristics, management, and outcome of squamous carcinoma of the conjunctiva in a single tertiary cancer center in Jordan.

AIM: To evaluate the features and outcome of management of malignant conjunctival squamous tumors in King Hussein Cancer Center (a referral tertiary cancer center in the Middle East). METHODS: Retrospective case series of 31 eyes for 31 patients with conjunctival squamous neoplasia. Main outcome measures included: age, gender, laterality, tumor location, pathological features, tumor stage, treatment modality, and outcome. RESULTS: Twenty (65%) patients were males and median age was 58y. Twenty-two (71%) eyes had the tumor in the nasal quadrant. Tumor invasion to nearby structures was seen in 19 (61%) eyes, including the cornea, fornix, eyelid, and orbit in 17 (55%), 1 (3%), 2 (6%), and 3 (10%) eyes, respectively. Eye salvage was achieved by surgical excision with cryotherapy followed by topical chemotherapy in 28 (90%) eyes, and orbital exenteration was necessary in 3 (10%) eyes due to orbital tumor invasion. Tumor recurrence was seen in 7 (23%) eyes, and the significant predictive factors for recurrence were tumor extension onto the nearby structures (P=0.04), tumor invasiveness (P=0.038), and tumor TNM stage (P=0.031). No significant change in visual acuity was seen, and disease related mortality was 6% (2 patients, both had orbital invasion by invasive squamous carcinoma). CONCLUSION: Conjunctival squamous carcinoma is more common in males. Advanced American Joint Committee on Cancer (AJCC) T-stage, tumor local invasion, more pathologically aggressive tumors, and surgical treatment alone (without adjuvant therapy) are associated with higher risk for recurrence, and orbital invasion is the most important poor prognostic factor for metastasis and death. Treatment strategies should be affected by tumor characteristics at presentation.

Canalicular pyocele: A new entity and literature review.

Canaliculoceles are rare, and under-recognized lesions of the upper lacrimal drainage system that may present as medial canthal masses or swelling. The current case is that of an 85-year-old female patient presented with a painful right upper lid medial, cystic swelling of 10-day duration involving the punctum and the upper canaliculus. A literature review showed that this is the seventh reported case of a canaliculocele but the first case of acute onset that is associated with inflammatory signs and symptoms and accumulation of purulent material. The term canalicular pyocele is suggested to describe this condition.

Outcomes of Penetrating Keratoplasty With Imported Corneas Compared With Local Corneas.

PURPOSE: The outcomes of penetrating keratoplasty (PK) were compared using local corneas obtained at the same hospital in which they were transplanted with corneas obtained from the United States and grafted at the Jordan University Hospital. METHODS: A retrospective, nonrandomized review included the medical records of 484 patients who underwent PK at the Jordan University Hospital from 2007 to 2009. Patients with at least 18 months of follow-up included 71 patients in the local group and 75 patients who received imported corneas. Data regarding the preoperative vision and diagnosis, tissue characteristics, preservation-to-surgery time of donor tissue on graft survival, and postoperative outcomes were collected. RESULTS: One hundred forty-six patients (mean age, 31.8 yrs ± 18.3 SD; range, 13-80 yrs) were included. Ninety-three (63.7%) patients were men. The most common indications for PK were keratoconus (67.8%) and corneal opacity (11%). Both groups were similar in age, indications, and outcomes. The success rates for PK after 18 months were 86% and 88% in the local and imported cornea groups, respectively; the difference between them did not reach significance (P = 0.196). The only significant (P < 0.001) factor was a longer preservation-to-surgery time in the imported group (8.6 ± 2.9 d) compared with the local group (4.5 ± 2.2 d). CONCLUSIONS: The success rate for PK remains relatively high (average, 87%) with both local and imported corneas. Despite the longer preservation-to-surgery time and older donor age for the imported corneas, no significant difference in graft outcomes was seen between the 2 groups.

Isolated Bilateral Lacrimal Gland Agenesis.

A 5-year-old boy presented to the ophthalmology department complaining of absent tearing while crying. Slit-lamp examination showed decreased tear margin film with normal punctae. Orbit magnetic resonance imaging was done and showed bilateral absent lacrimal glands. This is the third case of isolated bilateral lacrimal gland agenesis in the literature. [J Pediatr Ophthalmol Strabismus. 2016;53:e35-e38.].

Intracranial Hypertension in Children: Etiologies, Clinical Features, and Outcome.

This retrospective study aimed to describe the clinical presentations, possible causes, and outcomes of children with idiopathic intracranial hypertension who presented to the authors' clinic. The mean age at onset of symptoms in the authors' cohort of 19 children was 6 years (range: 7 months to 12 years). Most patients (90%) were under 11 years old and (84.2%) symptomatic. The probable cause was identified in 7/19 (37.0%) patients. The most common cause was vitamin D deficiency (26.3%). Other associated probably coincidental comorbidities included sinusitis (5/19, 26.3%), hypophosphatasia (1/19), Pyle disease (1/19), and measles vaccine (1/19). Apart from 2 patients who required lumboperitoneal shunt, the cerebrospinal fluid pressure returned to normal in all patients within a period of 6 weeks to 1 year (average, 5 months). Of those who followed up with the authors' ophthalmologist, 30.7% developed optic atrophy or pallor; 75% of these patients had previous ocular comorbidities.

Orbital, subconjunctival, and subcutaneous emphysema after an orbital floor fracture.

A 16-year-old boy presented to the emergency department with the complaint of a sudden, painful left eye and proptosis after an episode of sneezing. A few hours earlier, he had sustained a blunt trauma to the left orbit as the result of a fistfight. The initial examination showed subcutaneous and subconjunctival emphysema. Visual acuity in the left eye was 20/30 (0.67), the pupils were reactive with no relative afferent pupillary defect, and there were mild limitations in levoduction and supraduction. A slit-lamp examination showed normal anterior and posterior segments with an intraocular pressure of 26 mmHg. An orbital computed tomography scan showed orbital, subconjunctival, and subcutaneous emphysema associated with a small fracture of the orbital floor. Following conservative management with broad-spectrum oral antibiotics, a topical antiglaucoma drug, and lubricating eye drops, the patient improved dramatically within one week.

Ocular manifestations of Behçet's disease in Jordanian patients.

PURPOSE: To study the prevalence, manifestations and severity of ocular involvement of Behçet's disease in Jordanian patients. METHODS: The study population consisted of 43 patients diagnosed to have Behçet's disease through Rheumatologist's examinations conducted at Jordan University Hospital between January 2002 and July 2009. The sample involved patients who displayed ocular manifestations. This included 18 patients; 12 males and 6 females with a mean age of 35 years (SD = 17.26). Ophthalmological examinations and retrospective analysis of medical files were carried on. RESULTS: Ocular manifestations were seen in 41.9% of patients. The most common manifestation for Behçet's disease was vitritis with a prevalence of 55.6%, followed by anterior uveitis and retinal vasculitis (50% for each). On the other hand, the most frequent complications involved were cataract, cystoid macular edema (CMO), posterior synechiae and glaucoma with a prevalence of (44.4%), (33.3%), (11.1%) and (5.6%), respectively. CONCLUSION: The prevalence and severity of ocular lesions in Behçet's disease is relatively low in Jordanian patients. This result indicates that early diagnoses and intervention might delay or even prevent vision loss for those patients.

Ferrara ring segments implantation for treating keratoconus.

AIM: To investigate the efficacy of Ferrara rings (FR) implantation in the treatment of keratoconus. METHODS: It was a retrospective case series descriptive study. The sample was comprised of 50 patients 79 eyes diagnosed with progressive keratoconus. This included 24 (48%) males and 26 (52%) females between the age of 13 and 44 years. All participants underwent surgical implantation of FR in the period between January 2009 and September 2010 at Jordan University Hospital. Thorough ophthalmologic examinations were applied to measure vital variables for each pathological condition before and after surgery. RESULTS: Findings indicated an overall significant postoperative improvement in both uncorrected visual acuity (UCVA) and best spectacle corrected visual acuity (BSCVA) throughout follow up visits. Moreover, results illustrated a significant decrease in spherical equivalent (SE) and keratometric readings (lower, higher and the average). CONCLUSION: Surgical intervention strategies are being frequently developed to meet the needs of patients with keratoconus. The implantation of Ferrara rings has proven to be a safe and feasible alternative procedure for the treatment of mild-moderate keratoconus especially for patients with contact lenses intolerance. We have found that this procedure has improved visual outcomes in all eyes studied. Nevertheless, further research is needed to investigate long term outcomes.

KeraRing intrastromal corneal ring segments for correction of keratoconus.

PURPOSE: Intrastromal corneal ring segments (ICRSs) are small arc-like implants that are being used increasingly as a minimally invasive treatment for patients with keratoconus. This study assessed the effectiveness of KeraRing implants, a type of ICRS, to treat keratoconus. METHODS: Retrospective case series descriptive study of 43 patients (55 eyes) with keratoconus who underwent KeraRing implantation from February 2008 to June 2009. Patients who had intraoperative or postoperative complications and/or did not complete at least 6 months of follow-up were excluded. All patients underwent complete ophthalmologic examinations that included measurement of the uncorrected visual acuity (UCVA), best spectacle-corrected visual acuity (BSCVA), manifest refraction, and keratometry before and 2 days, 2 weeks, 1 month, and 3 and 6 months after surgery. The ring segments were chosen based on a nomogram from the manufacturer. RESULTS: Six months postoperatively, the mean UCVA increased significantly (P < 0.05) from 0.10 ± 0.11 to 0.32 ± 0.25 and the mean BSCVA increased from 0.36 ± 0.23 to 0.57 ± 0.24 (P < 0.05). The mean spherical refractive error significantly (P < 0.05) improved from -4.85 ± 2.90 diopters (D) to -1.89 ± 2.68 D, and the mean cylindrical refractive error significantly (P < 0.05) improved from -3.65 ± 1.70 D to -2.60 ± 1.62 D. The mean spherical equivalent significantly (P < 0.05) decreased from -6.68 ± 2.93 D to -3.19 ± 2.75 D, and the mean keratometry value decreased from 51.83 ± 4.14 D to 47.27 ± 3.68 D. The improvement in the UCVA and BSCVA continued over the 6-month postoperative period, but significant changes occurred only during the first 3 months. These changes occurred in patients with all grades of keratoconus. CONCLUSIONS: KeraRing implantation provided significant improvement in visual acuity, spherical equivalent, and keratometry results. This ICRS is an effective treatment for managing keratoconus and might delay or even avoid the need for penetrating keratoplasty.