Rate of Spontaneous Resolution of Congenital Nasolacrimal Duct Obstruction Prior to Scheduled Probing in Children Older Than 1 Year.

The authors conducted a 10-year retrospective review of all patients within one physician's practice to determine the frequency of spontaneous resolution of congenital nasolacrimal duct obstruction in children older than 1 year during the interval between scheduling and date of probing. This review found 4.4% of eyes resolved within an average interval period of 39 days. [J Pediatr Ophthalmol Strabismus. 2019;56:e31-e33.].

Anirdia-like phenotype caused by 6p25 dosage aberrations.

Axenfeld-Rieger spectrum (ARS) includes the anterior segment abnormalities posterior embryotoxon, irido-corneal adhesions, corectopia, and other abnormalities of pupil size and shape. Glaucoma occurs in approximately 50% of affected children. It is often caused by mutations of FOXC1 or PITX2. Timing of expression and dosage of these transcription factors appear to be very critical in the development of the anterior segment. We report on one child with a deletion and another with a duplication involving 6p25, causing an anirdia-like phenotype. Classic anirdia is a pan-ophthalmic disorder caused by heterozygous mutations involving the paired homeobox gene PAX6 at 11p13. It is often associated with optic nerve hypoplasia, foveal hypoplasia, corneal pannus, nystagmus, and cataract. Microdeletion of 11p13 may be associated with life threatening Wilms tumor. Distinguishing these two syndromes has critical implications for prognosis and treatment. We demonstrate how chromosomal microarray can be instrumental in differentiating these phenotypes.

The differential pupillary response to 2.5% phenylephrine in patients taking tamsulosin.

PURPOSE: To determine if a pharmacological test could be developed to determine iris dilator dysfunction in patients taking tamsulosin. METHODS: Patients taking tamsulosin and controls were recruited from the Urology and Ophthalmology clinics at the Montefiore Medical Center. The patient's right eye (OD) was dilated with phenylephrine hydrochloride 2.5% and tropicamide 1%. The patient's left eye (OS) was dilated with tropicamide 1% alone. Forty minutes after dilation, pupillary diameter was measured in both eyes. RESULTS: Thirty-eight tamsulosin subjects and 43 controls met the inclusion criteria for the study. The tamsulosin-treated patients dilated less with phenylephrine than controls (0.61±0.4 vs. 1.10±0.45 mm, respectively P<0.001). Receiver operating characteristic curves comparing maximal pupillary dilation versus differential pupillary dilation in tamsulosin patients relative to controls shows a greater area under the curve for differential dilation (0.8 vs. 0.6, respectively). A correlation between smooth muscle dysfunction and length of time on tamsulosin was observed. Patients using tamsulosin for <1 month had an average OD-OS difference of 0.85±0.5 mm. Patients who were on tamsulosin for >1 month had an average OD-OS difference of 0.52±0.32 mm (P<0.01, Mann-Whitney). CONCLUSION: Patients treated with tamsulosin demonstrated a significantly decreased iris dilatory response to the selective adrenergic effects of phenylephrine compared to controls. Additionally, it appears that longer duration of exposure to tamsulosin increases the likelihood of dilator dysfunction.

Bilateral posterior tenectomy of the superior oblique muscle for the treatment of A-pattern strabismus.

PURPOSE: To evaluate the efficacy of bilateral posterior superior oblique tenectomy for the treatment of A-pattern strabismus due to superior oblique overaction regardless of the magnitude of the pattern. METHODS: The medical records of patients with A-pattern esotropia or exotropia in the presence of superior oblique overaction who underwent combined horizontal muscle surgery along with bilateral superior oblique posterior 7/8 tenectomy from 2003 to 2013 were retrospectively reviewed. Patients with at least 3 months' follow-up were included. RESULTS: A total of 73 patients were included. Of these, 46 had esotropia; 27, exotropia. The preoperative A-pattern deviation for the study population was 19.6(Δ) ± 11.4(Δ) (range, 10-60), with a final postoperative patten collapse of 18.2 ± 3.6. Superior oblique overaction was 2.3 ± 0.7 preoperatively and 0.3 ± 0.7 postoperatively. Overall, 87.7% of patients had a successful collapse of their pattern to <10(Δ) following the initial bilateral superior oblique posterior tenectomy, with an additional 4.1% following a second procedure. Of patients with a pattern deviation of <25(Δ), 87.9% had successful collapse of the pattern following 1 surgery, and 86.7% of patients who had a pattern of ≥25(Δ) had successful collapse. Postoperatively, 7 patients demonstrated mild inferior oblique overaction. No surgical complications were noted. CONCLUSIONS: A uniform dose of bilateral posterior 7/8 tenectomy surgery successfully collapses A-pattern deviations of all magnitudes.

Anterior lentiplane associated with posterior lenticonus and microcornea.

We report a 12-year-old boy with a rare condition consisting of familial bilateral anterior lentiplane (a flat anterior lens capsule) posterior lenticonus, and microcornea.

Physician use of white coats in pediatric ophthalmology.

PURPOSE: Recent literature reports that patients and parents of pediatric patients prefer their physician to wear a white coat and to address them informally. This study aims to characterize current practice patterns of pediatric ophthalmologists regarding their use of white coats and salutations during outpatient pediatric encounters. METHODS: An eight-question survey was e-mailed to members of the American Academy of Pediatric Ophthalmology and Strabismus in 2012. The questions focused on clinical setting, use of white coats in out-patient encounters, and preferred language used to address the patient's parents. Surveys not completed in full were excluded from data analysis. RESULTS: Of approximately 1,266 members who received the survey, 606 completed the survey. Five hundred ninety-nine surveys were included in the data analysis. Sixty-three percent of attending physicians and 80% of fellows reported they did not routinely wear white coats while examining outpatient children. Forty-six percent of attending physicians and 48% of fellows addressed the patient's parents as "mom" or "dad". There was no significant association between wearing a white coat and type of practice setting, practice characteristics, or location in a children's hospital for attending physicians or fellows. CONCLUSIONS: Contrary to preferences expressed by patients and their parents, a majority of pediatric ophthalmologists do not routinely wear white coats during pediatric outpatient examinations. Practice patterns appear to be in line with previously reported parental greeting preferences.

Funduscopic and angiographic appearance in the neuronal ceroid lipofuscinoses.

PURPOSE: To characterize the retinal features of neuronal ceroid lipofuscinoses (NCLs) and to determine if retinal abnormalities are detectable in carriers of these autosomal recessively inherited diseases. METHODS: Carriers of the NCLs and their affected children underwent ophthalmic examination including color fundus photography in all patients and fluorescein angiography in selected patients. Twenty-nine patients with NCL were examined and photographed: 3 with infantile form, 2 with late-infantile form, and 24 with juvenile form. Fourteen patients underwent fluorescein angiography. RESULTS: Infantile and late-infantile retinal findings include fine retinal pigment epithelium pigment atrophy with no bone spicule changes and disk pallor. Juvenile retinal findings include macular retinal pigment epithelium atrophy and pigment stippling (>50%), epiretinal membrane (33%), bull's eye maculopathy (25%), and peripheral bone spicules (46%) and variable disk pallor. Fluorescein angiography of juvenile patients demonstrated diffuse retinal pigment epithelium atrophy with stippled hyperfluorescence (93%). Heterozygous NCL carriers had no identifying retinal abnormalities. CONCLUSION: Significant variability exists in the retinal appearance of the NCLs, but, in general, ophthalmoscopy and fluorescein angiography distinguish these patients from other more common blinding disorders of childhood such as retinitis pigmentosa and Stargardt disease. Examining retinas of parents of affected children does not aid in the diagnosis of NCL.

The impact of morphine after a spinal cord injury.

Nociceptive stimulation, at an intensity that elicits pain-related behavior, attenuates recovery of locomotor and bladder functions, and increases tissue loss after a contusion injury. These data imply that nociceptive input (e.g., from tissue damage) can enhance the loss of function after injury, and that potential clinical treatments, such as pretreatment with an analgesic, may protect the damaged system from further secondary injury. The current study examined this hypothesis and showed that a potential treatment (morphine) did not have a protective effect. In fact, morphine appeared to exacerbate the effects of nociceptive stimulation. Experiment 1 showed that after spinal cord injury 20mg/kg of systemic morphine was necessary to induce strong antinociception and block behavioral reactivity to shock treatment, a dose that was much higher than that needed for sham controls. In Experiment 2, contused rats were given one of three doses of morphine (Vehicle, 10, 20mg/kg) prior to exposure to uncontrollable electrical stimulation or restraint alone. Despite decreasing nociceptive reactivity, morphine did not attenuate the long-term consequences of shock. Rats treated with morphine and shock had higher mortality rates, and displayed allodynic responses to innocuous sensory stimuli three weeks later. Independent of shock, morphine per se undermined recovery of sensory function. Rats treated with morphine alone also had significantly larger lesions than those treated with saline. These results suggest that nociceptive stimulation affects recovery despite a blockade of pain-elicited behavior. The results are clinically important because they suggest that opiate treatment may adversely affect the recovery of function after injury.

Instrumental learning within the rat spinal cord: localization of the essential neural circuit.

Following spinal transection of the upper thoracic spinal cord, male Sprague-Dawley rats given legshock whenever a hindlimb is extended learn to maintain the leg in a flexed position. The region of the cord that mediates this instrumental learning was isolated using neuroanatomical tracing, localized infusion of lidocaine, and surgical transections. DiI and Fluoro-Gold microinjection at the site of shock application labeled motor neuron bodies of lamina IX in the lower lumbar region. Local application of the Na-super++ channel blocker lidocaine disrupted learning when it was applied over a region extending from the lower lumbar (L3) to upper sacral (S2) cord. The drug had no effect rostral or caudal to this region. Surgical transections as low as L4 had no effect on learning. Learning also survived a dual transection at L4 and S3, but not L4 and S2. The results suggest that the essential neural circuit lies between L4 and S3.