Congenital bilateral cataracts in newborns exposed to elexacaftor-tezacaftor-ivacaftor in utero and while breast feeding.

Elexacaftor-tezacaftor-ivacaftor (ETI) is known to pass through the placenta and into breast milk in mothers who continue on this therapy while pregnant and breast feeding. Toxicity studies of ivacaftor in rats demonstrated infant cataracts, but cataracts were not reported in human infants exposed to ivacaftor. We describe 3 cases of infants exposed to elexacaftor-tezacaftor-ivacaftor (ETI) in utero and while breast feeding who were found to have bilateral congenital cataracts within six months of birth. None of the infants had significant visual impairment from the cataracts nor any report of elevated liver function testing. These data highlight the need to counsel females who continue ETI throughout pregnancy and while breast feeding to consider cataract screen for their infants.

Clinical Outcomes After Ahmed Glaucoma Valve Implantation for Pediatric Glaucoma After Congenital Cataract Surgery.

PRCIS: Ahmed valve success for glaucoma following congenital cataract surgery lasts at least 5 years in most eyes, and >10 years in some cases. The procedure is a valuable option for these patients. PURPOSE: The aim of the study was to report on the results of Ahmed valve implantation in children with glaucoma following congenital cataract surgery. PATIENTS AND METHODS: Medical records were reviewed for 41 pediatric eyes (27 patients) with glaucoma after congenital cataract surgery with Ahmed glaucoma valve (AGV) implantation between 2007 and 2018. The primary outcome measure was surgical success, defined as intraocular pressure (IOP) ≤22 mm Hg (with or without glaucoma medications) on 2 consecutive follow-up visits, without glaucoma reoperation, and without significant visual complications during the follow-up period. RESULTS: Median age at the time of AGV implantation was 80 months (range: 14 to 146 mo) and the mean follow-up period was 61.1±46.5 months. The cumulative probability of surgical success was 95.1%, 89.8%, 83.1%, and 72.6% at 12, 24, 60, and 84 months, respectively. IOP significantly decreased from 35.8±7.4 mm Hg before valve implantation to 18.7±6.5 mm Hg at the last recorded visit (51.4% decrease; P<0.0001). Most eyes (79%) required medications for pressure control. Complications occurred in 14 eyes (34%), with 12 of these remaining successful. Early hypotony was the most common complication (19.5%). Retinal detachment occurred in 1 eye. CONCLUSIONS: Despite a decrease in surgical success over time, AGV implantation was successfully used for IOP control in the majority of our pediatric eyes with glaucoma after congenital cataract surgery. Most complications were managed effectively and surgical success was maintained.

[CHILDHOOD GLAUCOMA].

INTRODUCTION: "Childhood glaucoma" is a heterogenic group of diseases, characterized by elevated intraocular pressure (IOP) associated with optic-disc damage and other ocular comorbidities. Diagnosis requires two or more of the following: elevated IOP, optic nerve damage, enlarged cornea or Descemet's membrane ruptures, enlarged eye, high myopia and visual field defects. Childhood glaucoma is classified as primary if it occurs as an isolated ocular disease, and secondary, when the disease occurs along with other ocular anomalies or systemic diseases such as Neurofibromatosis and Sturge-Weber, or with acquired conditions such as uveitis complications, ocular trauma, cataract surgery, as well as from systemic and ocular steroid use. The clinical manifestations of childhood glaucoma depend on the age of presentation. In newborns, an enlarged eye with an enlarged cloudy cornea can be found, while infants present with an enlarged eye and signs of tearing, blinking and glare. Older children are usually asymptomatic and the disease is discovered incidentally on eye examination for other ocular problems. Treatment of childhood glaucoma is complicated and demanding. Most types of pediatric glaucoma require surgery in order control IOP, while medical treatment has a supportive role. Different types of glaucoma surgery are indicated for different types of pediatric glaucoma. Regular lifelong monitoring, including IOP control and treatment for the prevention of amblyopia is necessary to obtain and maintain good vision.

Ex-PRESS Miniature Glaucoma Shunt Versus Ahmed Glaucoma Valve in the Surgical Treatment of Glaucoma in Pseudophakic Patients.

PURPOSE: The aim of this study was to compare the 3-year outcome of Ex-PRESS miniature glaucoma shunt versus Ahmed glaucoma valve in pseudophakic patients. PATIENTS AND METHODS: We retrospectively reviewed the records of patients with a history of clear corneal phacoemulsification alone, or failed trabeculectomy following phacoemulsification, who subsequently underwent Ahmed glaucoma valve (AGV) implantation or Ex-PRESS shunt surgery. The main outcome measure, surgical success, was defined as an intraocular pressure between 5 and 21 mm Hg and a 20% intraocular pressure reduction from baseline (with/without glaucoma medications) without glaucoma reoperation. RESULTS: In total, 92 patients (92 eyes) were included (43 AGV, 49 Ex-PRESS). Overall success at 3 years was 92.7% for AGV and 66.1% for Ex-PRESS (P=0.006). AGV had a higher success rate in patients with prior phacoemulsification and failed trabeculectomy (96% AGV vs. 64.1% Ex-PRESS; P=0.023). There was no difference in success rate for patients with only previous phacoemulsification (87.5% AGV vs. 69.4% Ex-PRESS; P=0.205). Glaucoma reoperation rates were 4.6% and 30.6% in the AGV and Ex-PRESS group, respectively (P=0.001). Both procedures had similar complication rates. CONCLUSIONS: AGV compared with Ex-PRESS shunt, had a higher success rate for secondary surgeries but not primary surgeries, in patients with prior clear corneal phacoemulsification. The AGV surgery also had a lower overall glaucoma reoperation rate.

Use of Autologous Scleral Graft in Ahmed Glaucoma Valve Surgery.

PURPOSE: To compare the efficacy of an autoscleral free-flap graft versus an autoscleral rotational flap graft in Ahmed glaucoma valve (AGV) surgery. PATIENTS AND METHODS: Medical records (2005 to 2012) of 51 consecutive patients (51 eyes) who underwent AGV surgery with the use of either an autoscleral free-flap graft or an autoscleral rotational flap graft to cover the external tube at the limbus were retrieved for review. The main outcome measure was the incidence of tube exposure associated with each surgical approach. RESULTS: Twenty-seven consecutive patients (27 eyes) received a free-flap graft and 24 consecutive patients (24 eyes) received a rotational flap graft. The mean follow-up time was 55.6 ± 18.3 months for the former and 24.2± 5 .0 months for the latter (P<0.0001). Two patients in the free-flap group (8.9%) developed tube exposure at 24 and 55 months postoperatively compared with none of the patients in the rotational flap group. Graft thinning without evidence of conjunctival erosion was observed in 15 patients (55%) in the free-flap group and in 7 patients (29.1%) in the rotational flap group. CONCLUSIONS: The use of an autoscleral rotational flap graft is an efficacious technique for primary tube patch grafting in routine AGV surgery, and yielded better results than an autoscleral free-flap graft. Its main advantages over donor graft material are availability and lower cost.

[CANNABIS AND GLAUCOMA: AN ANCIENT LEGEND OR A NOVEL THERAPEUTIC HORIZON?].

Glaucoma causes damage to the optic nerve and compromises the visual field. The main risk factor of the disease is the level of the intra-ocular pressure. Therapeutic options include medical and surgical treatment, aimed to lower the intra-ocular pressure. Consumption of the cannabis plant (Cannabis Satival has been known since ancient times. It can be consumed orally, topically, intra-venous or by inhalation. The main active ingredient of cannabis is THC (Tetra-Hydro-Cannabinol). One of THC's reported effects is the reduction of intra-ocular pressure. Several studies have demonstrated temporary intra-ocular pressure decrease in both healthy subjects and glaucoma patients following topical application or systemic consumption. The effect was a short term one. It was followed by the development of resistance to the drug after prolonged intake and it was also accompanied by topical and systemic side effects. Cannabis may be considered as a therapeutic option in glaucoma. Its limited effect, development of resistance, acquired side effects and the accompanying psycho-active influence limit its advantage and cause its efficacy to be dubious. Therefore, cannabis treatment for glaucoma currently seems impractical and is not recommended by either the Israeli or the American glaucoma societies.

Genotype/phenotype correlation in primary congenital glaucoma patients from different ethnic groups of the Israeli population.

PURPOSE: To investigate the roles of CYP1B1 and MYOC mutations and characterize the phenotype of primary congenital glaucoma in Israeli patients from 3 different ethnic backgrounds. DESIGN: Interventional case series. METHODS: This institutional study included 34 Israeli primary congenital glaucoma patients (26 families) comprising 9 Jews (9 families), 17 non-Bedouin Muslim Arabs (10 families), and 8 Druze (7 families). The patients and their relatives (n = 99) were screened for CYP1B1 and MYOC mutations. RESULTS: Mutations in the CYP1B1 gene were detected in 12 of 26 families (46%) with primary congenital glaucoma (5 Muslim Arab, 5 Druze, and 2 Jewish). The Jewish families had compound heterozygous mutations and digenic mutations (ie, an Ashkenazi family had mutations in the CYP1B1 gene [Arg368His, R48G, A119S, and L432V haplotypes] and an Ashkenazi-Sephardic family had a mutation on the CYP1B1 gene [1908delA, Sephardic] with a second missense mutation on the MYOC gene [R76K, Ashkenazi]). The Muslim Arabs and Druze tended to have a more severe phenotype than that of the Jews. CONCLUSION: The phenotype and spectrum of the CYP1B1 and MYOC mutation roles in the clinical characteristics of primary congenital glaucoma varied according to ethnicity. The rarity of mutations in the CYP1B1 gene among Ashkenazi primary congenital glaucoma patients indicates that a different locus may be involved in the phenotype.

Advances in evaluation and management of pediatric idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Although the demographics of pediatric idiopathic intracranial hypertension have been well described, the best approaches to its management are less well defined. Recent advances in evaluation of optic nerve status make it easier to determine when and if more aggressive intervention is required. Medical and surgical approaches are discussed. RECENT FINDINGS: Modern neuroimaging techniques are used to look for secondary causes of intracranial hypertension such as cerebral venous sinus thrombosis. Automated perimetry is now quicker to perform and is accepted as the gold standard in evaluating optic nerve defects, even in the pediatric population. Other newer techniques for assessing optic nerve injury include optical coherence tomography, laser scanning tomography, and spectral Doppler blood flow analysis of the optic nerve head. Medical management remains the first-line treatment, but increasingly, surgical interventions such as optic nerve sheath fenestration and cerebrospinal fluid shunting are used in the pediatric population. SUMMARY: Pediatric intracranial hypertension patients may be followed with a greater degree of precision than in the past. Management tends to be similar to that used in adults, now including surgical intervention when indicated. The newer technologies allow us to gather data that may help to determine the optimal time for surgical intervention for those patients who require it.

[Selective laser trabeculoplasty treatment for medication-refractory open angle glaucoma].

BACKGROUND: Selective laser trabeculoplasty (SLT) is a new method to reduce intraocular pressure in eyes with primary open angle glaucoma. The laser parameters are set to selectively target pigmented trabecular meshwork (TM) cells without damage to the adjacent non-pigmented tissue. PURPOSE: A clinical retrospective study was conducted to evaluate the 12 months results of SLT in the treatment of medically uncontrolled open angle glaucoma. PATIENTS AND METHODS: During the period March to September 2004, the authors performed a SLT in 50 patients (50 eyes) with open angle glaucoma uncontrolled on maximally tolerated medical therapy. Treatment was carried out with a frequency-doubled, Q-switched Nd:YAG laser 532 nm.(Selectra 7000 Laser Coherent, Inc., Palo Alto,CA). Approximately 85 to 90 non-overlapping laser spots were placed over 180 degrees of the trabecular meshwork at energy levels ranging from 0.6 to 1.4 mJ per pulse. In patients who required additional SLT therapy, the untreated 180 degrees was treated. During the follow-up period, patients were treated with antiglaucoma medications as required. The success rates were defined as decreases in intraocular pressure (IOP) of 3 mmHg or more with no additional medications, laser, or glaucoma surgery. RESULTS: The mean IOP reduction from baseline 6 months after treatment was 21% and 20% after 12 months. The success rates were 66% after 6 months and 55% after 12 months. Four eyes (8%) did not respond to SLT. One hour after SLT, an increase in IOP of more than 5 mm Hg was detected in 5 eyes (10%). Seven patients (14%) required additional SLT. CONCLUSIONS: SLT is efficient in lowering IOP in medication-refractory open angle glaucoma. It should be considered in such patients when surgery is contraindicated or refused.

Ophthalmic complications of dental anesthesia: three new cases.

Two patients had ipsilateral optic neuropathy and one patient had an ipsilateral abduction deficit and a dilated, poorly reactive pupil immediately after anesthesia of upper alveolar teeth. In one patient with optic neuropathy, the optic disc was not swollen, brain and orbit computed tomography (CT) was negative, and vision recovered completely within 2 weeks. In the other patient with optic neuropathy, the optic disc was swollen, brain and orbit CT were negative, and vision did not recover. In the patient with ductional and pupil deficits, recovery was complete within 24 hours. Since 1960, 39 cases of ophthalmic complications have been reported in the English literature. A majority have followed anesthesia of upper alveolar teeth. In all but three cases, the deficits were temporary. Diffusion, inadvertent needle penetration into the orbit, venous injection, or retrograde arterial injection is postulated as the mechanism by which the anesthetic agent reaches the cavernous sinus or orbit to cause the deficits.