Divergence Excess Intermittent Exotropia: Long-Term Effect of Augmented Bilateral Lateral Rectus Recession.

PURPOSE: To investigate the long-term (10 years) effects of augmented bilateral lateral rectus (aBLR) recession in patients affected by divergence excess intermittent exotropia (deIXT). METHODS: Data of 58 patients affected by deIXT who underwent aBLR muscle recession were retrospectively analyzed. All patients were treated with 8.0 to 9.5 mm (mean 8.6 ± 0.5 mm) recession of BLR. RESULTS: Our result showed a significant decreasing of both distance and near residual deviation during follow-up. The percentage of patients with successful surgical outcome (defined as a residual deviation ranging from 10pd intermittent exotropia to 5pd esotropia) increased during follow-up (from 51.7% to 81% at last follow-up). Stereopsis increased in 39.7% of patients at last follow-up. Moreover, there was a significant correlation between age at surgery and short/mid-term residual deviation. CONCLUSIONS: Our long-term results suggest that aBLR recession surgery is a very effective approach in patients affected by deIXT.

Cytoplasmic myosin-exposed apoptotic cells appear with caspase-3 activation and enhance CLL cell viability.

The degree of chronic lymphocytic leukemia (CLL) B-cell antigen receptor (BCR) binding to myosin-exposed apoptotic cells (MEACs) correlates with worse patient outcomes, suggesting a link to disease activity. Therefore, we studied MEAC formation and the effects of MEAC binding on CLL cells. In cell line studies, both intrinsic (spontaneous or camptothecin-induced) and extrinsic (FasL- or anti-Fas-induced) apoptosis created a high percent of MEACs over time in a process associated with caspase-3 activation, leading to cytoplasmic myosin cleavage and trafficking to cell membranes. The involvement of common apoptosis pathways suggests that most cells can produce MEACs and indeed CLL cells themselves form MEACs. Consistent with the idea that MEAC formation may be a signal to remove dying cells, we found that natural IgM antibodies bind to MEACs. Functionally, co-culture of MEACs with CLL cells, regardless of immunoglobulin heavy-chain variable region gene mutation status, improved leukemic cell viability. Based on inhibitor studies, this improved viability involved BCR signaling molecules. These results support the hypothesis that stimulation of CLL cells with antigen, such as those on MEACs, promotes CLL cell viability, which in turn could lead to progression to worse disease.

Evaluation of retinal nerve fiber layer and ganglion cell complex thickness after ocular blunt trauma.

PURPOSE: To evaluate circumpapillary retinal nerve fiber layer (cpRNFL) and ganglion cell complex (GCC) after ocular blunt trauma. METHODS: Best-corrected visual acuity (BCVA), cpRNFL and GCC were evaluated by RTVue-100 OCT in all consecutive patients with previous monocular blunt trauma seen between January 2012 and December 2012. RESULTS: Twenty-two patients (11 females, 11 males, mean age 43.9 ± 14.2 years) were included in the study. Patients were seen after a mean of 8.42 ± 13.3 (range, 0.08-55.3) years from ocular blunt trauma. BCVA was normal in 11 cases and was less than 0.4 LogMAR in all cases. In 9/22 patients (40.9%), cpRNFL and GCC were reduced, whereas in one case an isolated reduction of GCC with normal cpRNFL was present. In patients with reduction of cpRNFL and GCC, mean BCVA was 0.17 ± 0.17 LogMAR. In 6/9 patients (66.6%) with cpRNFL and GCC reduction, BCVA was ≤ 0.1 LogMAR. CONCLUSION: cpRNFL and GCC reduction may be present after ocular blunt trauma and may be associated with preserved visual acuity.

Functional changes after treatment of optic pathway paediatric low-grade gliomas.

OBJECTIVE: To evaluate the functional changes after treatment of paediatric optic pathway gliomas (OPGs). METHODS: All patients with monofocal OPG seen from January 2004 to January 2011 were included. Best corrected visual acuity (BCVA, LogMAR), contrast sensitivity (Hiding-Heidi low-contrast 'face' test (HH) and Pelli-Robson (PR) contrast sensitivity test), and the Color Test (Ishihara plate) were obtained. RESULTS: Twenty-one patients (10 boys and 11 girls with a mean age of 5.5 ± 4.4 years at diagnosis) were included in the study. Neurofibromatosis was present in four cases. Eighteen patients (85.7%) were treated with initial surgery and three patients (14.3%) with initial chemotherapy. BCVA was 0.67 ± 0.8 LogMAR at baseline and 0.62 ± 0.9 LogMAR at last visit (P=0.41). The Color test was not significantly changed at last visit (P=0.62). Contrast sensitivity with the HH test was 9.1 ± 11.1% at baseline and 3.8 ± 6.4% at last visit (P=0.03). Contrast sensitivity with PR chart was 1.33 ± 0.9 log at baseline and 1.05 ± 0.7 log at last visit (P=0.005). A reduction in contrast sensitivity at both tests was significantly greater in patients who relapsed than in patients who did not relapse (P=0.001). CONCLUSION: After the treatment of paediatric optic pathway low-grade gliomas, a reduction in contrast sensitivity during follow-up was observed and may be correlated with tumour relapses.

Erectile dysfunction after prostate three-dimensional conformal radiation therapy. Correlation with the dose to the penile bulb.

PURPOSE: Erectile dysfunction is associated with all the common treatment options for prostate cancer. The aim of this research was to evaluate the relationship between erectile function and radiation dose to the penile bulb (PB) and other proximal penile structures in men receiving conformal radiotherapy (CRT) without hormonal therapy (HT) for prostate cancer, whose sexual function was known before treatment. PATIENTS AND METHODS: The study included 19 patients treated with 3D-CRT for localized prostate cancer at our department, who were self-reported to be potent before treatment, had not received HT, and had complete follow-up data available. Our evaluation was based on the International Index of Erectile Function (IIEF-5). Dose-volume histograms (DVHs) were used to evaluate the dose to the PB. Statistical analysis was performed with an unconditional logistic regression model. RESULTS: All patients reported change in potency after radiation. Eight patients (42%) remained potent but showed a decrease of 1 or 2 levels of potency, as defined by the IIEF-5 questionnaire (reduced potency group), while 11 patients (58%) reported a change of higher levels and revealed a severe erectile dysfunction after 2 years (impotence group). Multivariate analysis of morphological and dosimetric variables yielded significance for the mean dose (p = 0.05 with an odds ratio of 1.14 and 95% CI 1-1.30). Patients receiving a mean dose of less than 50 Gy to the PB appear to have a much greater likelihood of maintaining potency. CONCLUSION: Our data suggest a possible existence of a dose-volume correlation between the dose applied to the PB and radiation-induced impotence.

Prenatal screening for congenital toxoplasmosis in Campania: preliminary report on activities and results.

By 1997, an open cohort of 1,652 live newborn of 1,637 mothers with gestational toxoplasmosis had been recruited in the Campania region to monitor the burden of congenital toxoplasmosis (CT). Of the 1,556 mother-child pairs that completed the follow up, 92 definite cases were detected, yielding a 5.9% (4.8-7.1 95% CI) transmission rate. The onset was patent for 43% of patients and sensorineural complications were shown for a further 15% of subclinical onset patients later than two years of age. The overall prevalence of toxoplasmosis during gestation was 2.46 of 1,000 deliveries, while the prevalence of definite CT was 1.38 of 10,000 live newborns. However, there is still room for intervention, as only 23% of the maternal diagnoses were proven through seroconversion, 63 of the late-gestation seroconverters remained untreated, and six probable CT diagnoses were made following referrals due to patent sequelae and born during the study period. There was a positive secular trend on the rates of infant referral and definite CT diagnosis, according to the live birth rate (Chi2 for trend < 0.001). Extension of this surveillance system across the country could help to define a future strategy for prevention.

Prenatal screening for congenital toxoplasmosis in Campania: preliminary report on activities and results

By 1997, an open cohort of 1,652 live newborn of 1,637 mothers with gestational toxoplasmosis had been recruited in the Campania region to monitor the burden of congenital toxoplasmosis (CT). Of the 1,556 mother-child pairs that completed the follow up, 92 definite cases were detected, yielding a 5.9 percent (4.8-7.1 95 percent CI) transmission rate. The onset was patent for 43 percent of patients and sensorineural complications were shown for a further 15 percent of subclinical onset patients later than two years of age. The overall prevalence of toxoplasmosis during gestation was 2.46 of 1,000 deliveries, while the prevalence of definite CT was 1.38 of 10,000 live newborns. However, there is still room for intervention, as only 23 percent of the maternal diagnoses were proven through seroconversion, 63 of the late-gestation seroconverters remained untreated, and six probable CT diagnoses were made following referrals due to patent sequelae and born during the study period. There was a positive secular trend on the rates of infant referral and definite CT diagnosis, according to the live birth rate (Ç2 for trend < 0.001). Extension of this surveillance system across the country could help to define a future strategy for prevention.

Simultaneous surgery in bilateral congenital cataract.

PURPOSE: To assess the efficiency of simultaneous surgery in bilateral congenital cataract not only in patients with a high anesthesiologic risk profile. METHODS: A retrospective study was carried out on 40 bilateral congenital cataract patients (80 eyes) who presented to the outpatient department of pediatric ophthalmology of the University Federico II of Naples in the period from 1990 to 2005. All patients had undergone cataract extraction from both eyes in a single surgical session. Visual rehabilitation was achieved in all patients by corneal lenses; successively 25 patients had a secondary intraocular lens implant in the posterior chamber after a period of 2.5-3 years. RESULTS: The mean age at cataract surgery was 7 months (1-17 months). Visual acuity (VA) was assessed in 52 eyes: 8 eyes (15%) presented VA or=20/40. Ocular motility disorders were seen in 28 patients (70%). Eight patients had postoperative complications: the formation of secondary membranes in five patients, and secondary glaucoma in three patients. There were no cases of endophthalmitis. CONCLUSIONS: Simultaneous surgery in bilateral congenital cataract may be taken into consideration not only for patients with a high anesthesiologic risk profile.

Congenital cataract extraction with primary aphakia and secondary intraocular lens implantation in the posterior chamber.

PURPOSE: To evaluate visual outcomes, eye movement abnormalities, and postoperative complications after secondary posterior chamber intraocular lens (IOL) implantation in aphakic children who had initial LAC correction. METHODS: A retrospective study was carried out on 53 patients (94 eyes). Following aphakic surgery and LAC correction the patients received secondary IOL implantation in the posterior chamber. Special attention was paid to factors that may have influenced their visual outcomes, such as eye movement abnormalities, cataract unilaterality, and cataract density. RESULTS: No association was found between age at surgery and the onset of strabismus (p=0.611) or with visual acuity (p=0.086). However, unilaterality and total cataract density were found to have a negative association with poor vision (p<0.001). Strabismus and nystagmus were found to have a statistically significant negative association with visual acuity (p=0.002). Posterior capsule opacification occurred in six eyes of five patients following cataract extraction; IOL dislocation occurred in four eyes after IOL intraocular implantation, and secondary glaucoma occurred in one eye. CONCLUSIONS: IOL implantation before 12 months of age may be useful in unilateral cataract; in bilateral cataract, simultaneous surgical aphakia, LAC correction, and then IOL implantation at 2.5-3 years of age, together with anti-amblyopic therapy when strabismus or asymmetric cataract density occur, are efficient methods to obtain visual recovery. Extraocular muscle surgery may be required to correct strabismus.

Photorefractive keratectomy for myopic anisometropia: a retrospective study on 18 children.

PURPOSE: To evaluate the safety and efficacy of photorefractive keratectomy (PRK) performed under topical anesthesia in children with myopic anisometropia. METHODS: Medium to high unilateral myopia was corrected in 18 patients by PRK. At the time of surgery patients were between 7 and 17 years of age (mean 10 years). All the surgical procedures were performed under topical anesthesia. Pre- and postoperative data regarding visual acuity, eye alignment, and binocular vision were analyzed. RESULTS: The mean correction (SE) obtained with excimer laser was -8.21 D (range: -2.25 to -14.50, SD 3.90). The mean preoperative best spectacle-corrected visual acuity (BSCVA) was 20/70, and the postoperative mean BSCVA was 20/50. A significant difference was observed between the arithmetic mean of the preoperative and postoperative BSCVA (p=0.001). Two of 18 patients improved stereopsis; furthermore, surgery variation strabismus was registered in 33.3% of the patients, following PRK. Patients were followed up for a mean of 39 months. CONCLUSIONS: The treatment of medium to high unilateral myopia with PRK under local anesthesia was found effective and safe in pediatric and adolescent patients. This procedure may improve ocular alignment and stereopsis. Further studies are needed to increase the patient experience and extend follow-up time in order to assess the long-term stability of the RESULTS.

Strabismus in developmental cataract.

PURPOSE: To evaluate the presence of strabismus in patients with developmental cataract rendered pseudophakic and how this influences their visual acuity. METHODS: A retrospective study was carried out on 113 patients with developmental cataract who came under the authors' observation at the outpatient department of the Pediatric Ophthalmology Unit of the University of Federico II of Naples from 1990 to 2005. All patients were followed up for a long period (mean 62 months, range 36-144 months). Age at diagnosis, sex, laterality, age at cataract extraction, morphology, and cataract density were all considered as possible factors associated with strabismus. Visual acuity and ocular motility before and after cataract extraction surgery were especially noted. Statistical evaluation was performed using t-test, Chi-square test, and Fisher exact test. RESULTS: Out of the 113 patients a total of 181 eyes were affected: 68 patients (60%) presented bilateral cataract, 45 patients (40%) monolateral cataract. Strabismus was present in 39 patients (34%) before cataract surgery. Age at cataract diagnosis, age at surgery, sex, and cataract morphology were not found to be statistically associated with strabismus. However, laterality was found to be statistically associated with the onset of strabismus. Cataract density was found to be statistically associated with poor vision. Patients with strabismus presented a non statistically significant lower visual acuity. CONCLUSIONS: Strabismus has a greater incidence in developmental cataract compared to the general population, and can influence visual acuity, especially in monolateral and total cataracts. Intraocular lens implants produced satisfactory visual rehabilitation.

Ocular manifestations in a case of childhood cicatricial pemphigoid.

PURPOSE: Cicatricial pemphigoid is a rare chronic autoimmune disease, characterized by progressive alterations on the skin and mucous membranes. Ocular lesions consists of chronic conjunctivitis, progressive subepithelial fibrosis on the conjunctival fornix together with symblepharon formation, obstruction of the Meibomian ducts, sicca syndrome, and occasionally, entropion associated with consequent trichiasis and corneal ulcers. METHODS: A 9-year-old patient with cicatricial pemphigoid with severe eye involvement came to our observation. A complete anamnesis, ophthalmologic examination, and systemic evaluation, including serum antibody levels evaluation and biopsies of mouth, vulva, parotid, and intestine mucosa, were performed. RESULTS: Ocular examination showed blepharospasm, conjunctival hyperemia, symblepharon, total cicatricial corneal leucoma, severe dryness, trichiasis, and eyelid edema. The results of medical and surgical treatment are reported. CONCLUSIONS: Given the serious clinical picture, possibly due to a late diagnosis, it was not possible to avoid relapse and appearance of new cicatricial adherences for which we considered wait and see the most appropriate approach, protecting the anterior segment of both eyes, sacrificing their function. Further follow-up was not possible as the patient died. An early diagnosis would have had a significant influence on the clinical course and on the response to therapy.

Incidence of ocular pathologies in Italian children with Down syndrome.

PURPOSE: This study identifies the incidence of primary ocular pathologies in a population of Italian children with Down syndrome. METHODS: A total of 157 Italian children with Down syndrome, age between 1 month and 18 years, were screened between February 2005 and October 2006. The ophthalmologic evaluation included a global inspection of orbit and bulbus oculi, evaluation of ocular motility and visual acuity, slit lamp biomicroscopy, cycloplegic skiascopy, tonometry, and indirect ophthalmoscopy. RESULTS: The overall incidence of ocular abnormalities was epicanthal fold (132 patients, 84%), hyperopia (93 patients, 59%), astigmatism (44 patients, 28%), myopia (14 patients, 9%), strabismus (56 patients, 36%, 45 cases of esotropia and 11 cases of exotropia), congenital nasolacrimal duct obstruction (35 patients, 22%), cataract (18 patients, 11%), nystagmus (9 patients, 6%), blepharitis and conjunctivitis (6 patients, 4%), and retinal anomalies (10 patients, 6%). Unlike previous reports in patients with Down syndrome, no congenital glaucoma, keratoconus, or Brushfield spots were observed. CONCLUSIONS: Compared to other studies in patients with Down syndrome, we observed a higher incidence of hyperopia and a lower incidence of myopia. The incidence of nystagmus, blepharitis,and conjunctivitis was less than that reported in other studies, while strabismus, especially exotropia (20%), had a high incidence in our cohort. We also frequently observed obstruction of the nasolacrimal duct (22%), but no keratoconus or glaucoma.

Treatment of blepharospasm with botulinum neurotoxin type A: long-term results.

PURPOSE: To describe the long-term efficacy and side effects of treatment of blepharospasm with botulinum neurotoxin type A (Botox). METHODS: A total of 178 patients with blepharospasm were treated by injections of botulinum toxin in the Eye Clinic of the University of Naples from 1980 to 2001. The severity of spasm for each patient was graded on a four-point scale. Duration of improvement was assessed and reported in months. RESULTS: Of 178 cases, 10 were lost to follow-up; of the remaining patients, 93% reported improvement after treatments. The mean duration of improvement was 3.6 months. Twelve patients (76%) who underwent more than 14 treatments maintained stable relief. Three patients (1.7%) had a total remission of spasms. Side-effects were local; none of the 168 patients experienced any systemic or toxic reaction. CONCLUSIONS: Botulinum toxin therapy for blepharospasm can provide long-lasting relief and reduction of spasms in the majority of patients. This therapy has the advantages of being safe, simple, and repeatable.

Spontaneous resolution of congenital bilateral Brown's syndrome.

We report a case of bilateral Brown's syndrome in a 4-year-old girl. Forced duction testing confirmed the clinical diagnosis. After 7 months we observed a spontaneous resolution in the right eye. The problem in her left eye did not show any significant change over 36 months of follow-up. Various spontaneous resolutions in bilateral cases are reviewed.

Laryngeal long-term morbidity after supraglottic laryngectomy and postoperative radiation therapy.

PURPOSE: This study was performed to investigate factors associated with laryngeal morbidity when postoperative radiation therapy (RT) is added to supraglottic laryngectomy. MATERIALS AND METHODS: From 1980 to 1994, 56 patients affected with T1 to 4 N0 to 2c supraglottic squamous cell carcinoma selected for standard (59%) or extended (41%) supraglottic laryngectomy at 2 different institutions were retrospectively analyzed. Most of the patients (91%) also underwent neck dissection. Approximately 80% of the patients had stage T4 primary lesions or N2 neck disease. Postoperative RT was added for presumed microscopic disease at the primary site (13 patients), regional nodes (23 patients), or both (20 patients). Median delivered doses to the larynx and to the neck were 50 Gy (range, 40 to 64 Gy) and 46 Gy (range, 40 to 64 Gy), respectively. Median follow-up for living patients is 11 years (range, 2.8 to 16.9 years). Laryngeal complication was defined as the appearance of grade 2 or higher toxicity according to the European Organization for Research and Treatment of Cancer (EORTC) and the Radiation Therapy Oncology Group (RTOG) scoring systems. RESULTS: Two- and 5-year actuarial locoregional control rates were 85+/-5% and 83+/-5%, respectively. Thirty patients (54%) developed laryngeal complications. However, just one patient experienced grade 4 laryngeal oedema requiring permanent tracheostomy. Estimated actuarial survival without laryngeal complications were 50+/-7%, 43+/-7%, and 39+/-7% at 2, 5, and 10 years, respectively. At univariate analysis, treated volumes (P = .03) and total dose to the larynx (P = .03) were significantly associated with local toxicity. A trend was observed also for the maximum dose to the neck (P = .06) and dose per fraction (P = .09). A multivariate Cox proportional hazards model showed total dose to the larynx to be the only independent predictor of toxicity (P = .03). The hazard ratio of laryngeal toxicity was 2.2 (95% confidence interval: 1.1/4.6), for a total dose to the larynx greater than 50 Gy. CONCLUSION: After supraglottic laryngectomy, postoperative RT to the neck does not affect local morbidity, but careful RT treatment planning is necessary to avoid delivering a total dose to the larynx greater than 50 Gy.

Presence of bilateral limbal dermoids and choroidal osteomas in a family with inherited limbal dermoids.

We report a case of bilateral limbal dermoids and bilateral choroidal osteomas in a 14-year-old girl with no extraocular anomalies. Histopathological examination of a limbal lesion confirmed the clinical diagnosis of dermoid. Computerized tomography and ultrasonography were compatible with a diagnosis of choroidal osteoma. Limbal dermoids were present in the patient's mother, in a brother with Down syndrome, and in an aunt with no choroidal osteoma. The present pedigree is compatible with autosomal dominant inheritance of bilateral limbal dermoids. The same gene may be involved in the pathogenesis of ocular choristomas in same patients.

Metabolism of Dichloromethane by the Strict Anaerobe Dehalobacterium formicoaceticum.

The metabolism of dichloromethane by Dehalobacterium formicoaceticum in cell suspensions and crude cell extracts was investigated. The organism is a strictly anaerobic gram-positive bacterium that utilizes exclusively dichloromethane as a growth substrate and ferments this compound to formate and acetate in a molar ratio of 2:1. When [C]dichloromethane was degraded by cell suspensions, formate, the methyl group of acetate, and minor amounts of methanol were labeled, but there was no nuclear magnetic resonance signal corresponding to the carboxyl group of acetate. This finding and previously established carbon and electron balances suggested that dichloromethane was converted to methylene tetrahydrofolate, of which two-thirds was oxidized to formate while one-third gave rise to acetate by incorporation of CO(2) from the medium in the acetyl coenzyme A synthase reaction. When crude desalted extracts were incubated in the presence of dichloromethane, tetrahydrofolate, ATP, methyl viologen, and molecular hydrogen, dichloromethane and tetrahydrofolate were consumed, with the concomitant formation of stoichiometric amounts of methylene tetrahydrofolate. The in vitro transfer of the methylene group of dichloromethane onto tetrahydrofolate required substoichiometric amounts of ATP. The reaction was inhibited in a light-reversible fashion by 20 muM propyl iodide, thus suggesting involvement of a Co(I) corrinoid in the anoxic dehalogenation of dichloromethane. D. formicoaceticum exhibited normal growth with 0.8 mM sodium in the medium, and crude extracts contained ATPase activity that was partially inhibited by N,N'-dicyclohexylcarbodiimide and azide. During growth with dichloromethane, the organism thus may conserve energy not only by substrate-level phosphorylation but also by a chemiosmotic mechanism involving a sodium-independent F(0)F(1)-type ATP synthase.

A further observation of corneal dystrophy and perceptive deafness in two siblings.

We studied two siblings with the rare association of corneal dystrophy and perceptive deafness (Harboyan syndrome). To our knowledge, this is the third description of this hereditary disorder. The results of the clinical, genetic, audiometric, and ocular examination of the two siblings and the type of inheritance, which agree with the previous description of the syndrome, are reported. Various hereditary syndromes associated with corneal dystrophy are reviewed.

Anaerobic Desulfonation of 4-Tolylsulfonate and 2-(4-Sulfophenyl) Butyrate by a Clostridium sp.

Alkyl- and arylsulfonates were tested as sole added sources of sulfur for the growth of enrichment cultures under strictly anaerobic denitrifying or fermentative conditions. Cultures that utilized taurine, ethylsulfonate, the dyestuffs orange II and acid red I, tolylsulfonate, 2-(4-sulfophenyl)butyrate (SPB), a dialkyltetralinesulfonate, and 1-(4-sulfophenyl)octane were readily obtained. We chose to work with the simple aromatic compounds and isolated a fermentative bacterium, strain EV4, which utilized SPB as the sole added source of sulfur in glucose-mineral medium. The organism was identified as a Clostridium sp. related to Clostridium beijerinckii. Clostridium sp. strain EV4 utilized seven of seven tested arylsulfonates quantitatively. The growth yield was about 3 kg of protein per mol of sulfur, whether sulfonate or sulfate was utilized. A major product specific to each sulfonate could be observed. Although no product was identified, the existence of anaerobic desulfonation has been established.