Linda R. Dagi

Effect of Age on Response to Amblyopia Treatment in Children

OBJECTIVE To determine whether age at initiation of treatment for amblyopia influences the response among children 3 to less than 13 years of age with unilateral amblyopia who have 20/40 to 20/400 amblyopic eye visual acuity. METHODS A meta-analysis of individual subject data from 4 recently completed randomized amblyopia treatment trials was performed to evaluate the relationship between age and improvement in logMAR amblyopic eye visual acuity. Analyses were adjusted for baseline amblyopic eye visual acuity, spherical equivalent refractive error in the amblyopic eye, type of amblyopia, prior amblyopia treatment, study treatment, and protocol. Age was categorized (3 to <5 years, 5 to <7 years, and 7 to <13 years) because there was a nonlinear relationship between age and improvement in amblyopic eye visual acuity. RESULTS Children from 7 to less than 13 years of age were significantly less responsive to treatment than were younger age groups (children from 3 to <5 years of age or children from 5 to <7 years of age) for moderate and severe amblyopia (P < .04 for all 4 comparisons). There was no difference in treatment response between children 3 to less than 5 years of age and children 5 to less than 7 years of age for moderate amblyopia (P = .67), but there was a suggestion of greater responsiveness in children 3 to less than 5 years of age compared with children 5 to less than 7 years of age for severe amblyopia (P = .09). CONCLUSIONS Amblyopia is more responsive to treatment among children younger than 7 years of age. Although the average treatment response is smaller in children 7 to less than 13 years of age, some children show a marked response to treatment.

Spasm of the Near Reflex Associated with Organic Disease

Spasm of the near reflex is characterized by transient attacks of convergence, accommodation, and miosis. It is usually observed in young individuals and considered functional. We studied seven patients with spasm of the near reflex who had associated neurologic disorders or head trauma. Two patients had posterior fossa abnormalities (cerebellar tumor, Arnold-Chiari malformation), two patients had pituitary tumors, one patient had a vestibulopathy, and two patients had a history of antecedent head trauma.

Superior Rectus Transposition vs Medial Rectus Recession for Treatment of Esotropic Duane Syndrome

IMPORTANCE Superior rectus transposition (SRT) with or without medial rectus recession (MRc) has been introduced as an alternative to MRc alone for treatment of esotropic Duane syndrome; however, the effectiveness of these procedures has not been compared previously. OBJECTIVE To compare the safety and efficacy of MRc and SRT in treatment of Duane syndrome. DESIGN, SETTING, AND PARTICIPANTS Retrospective medical record review of all patients with esotropic Duane syndrome who underwent surgical treatment from January 1, 2006, through December 31, 2012, in a multispecialty, hospital-based pediatric ophthalmology/adult strabismus practice at Boston Childrens Hospital. Patients in the SRT group underwent SRT with or without MRc; those in the non-SRT group underwent unilateral or bilateral MRc. EXPOSURES Surgical treatment of esotropic Duane syndrome. MAIN OUTCOMES AND MEASURES Binocular alignment, ocular ductions, head position, stereopsis, and fundus torsion were recorded before surgery and at the 2-month and final postoperative visits. We also evaluated postoperative drift. RESULTS The medical record review identified 36 patients who underwent 37 procedures, including 19 in the SRT group (13 SRT + MRc and 6 SRT alone) and 18 in the non-SRT group (11 unilateral MRc and 7 bilateral medial rectus resession). Mean MRc was smaller when performed with SRT (3.3 vs 5.3 mm; P = .004). Although the initial deviation was larger in the SRT group, both groups had a similar improvement in esotropia and head turn. Abduction improved by at least 1 unit in 15 of 19 patients in the SRT group (79%) vs 5 of 18 in the non-SRT group (28%). In 24 patients followed up for more than 6 months, mean esotropia decreased from 8.2 to 6.1 prism diopters (Δ) in the SRT group (n = 12) but increased from 7.2 to 10.9Δ in the non-SRT group (n = 12). CONCLUSIONS AND RELEVANCE The combination of SRT and MRc was more effective than MRc or bilateral medial rectus resession at improving abduction while allowing for a smaller recession to align the eyes and eliminate a compensatory head posture. Although any surgery on the vertical rectus muscles should in theory increase the risk for vertical or torsional complications, to date this theory has not been borne out in our patients. Patients treated with SRT appear to have a reduced likelihood of long-term undercorrection. We therefore recommend SRT with adjustable MRc for treatment of Duane syndrome in patients with larger amounts of esotropia.

Thyroid eye disease: Honing your skills to improve outcomes

Thyroid eye disease affects the eyelids, orbital compartment, and extraocular muscles, resulting in a highly variable degree of chemosis and enlargement of the preorbital fat pads, eyelid retraction, proptosis, restrictive strabismus, torticollis, and, rarely, compressive or congestive optic neuropathy. Although most patients with thyroid eye disease are best treated conservatively, those more severely affected may benefit from orbital decompression, strabismus surgery, or eyelid retraction repair after stabilization has occurred. Botulinum A toxin, high-dose intravenous corticosteroids, and radiation treatment are therapeutic options in select cases. Compressive or congestive optic neuropathy and severe corneal exposure warrant consideration of surgical intervention on an urgent basis without waiting for stabilization. Epidemiology and risks and benefits of high-dose steroids and radiation therapy are reviewed along with recommendations to improve conservative as well as surgical management of this disease. Strategies to manage strabismus and optimize outcomes are provided.

Improving ophthalmic outcomes in children with unilateral coronal synostosis by treatment with endoscopic strip craniectomy and helmet therapy rather than fronto-orbital advancement

PURPOSE To compare long-term ophthalmic outcomes in infants treated for unilateral coronal synostosis (UCS) by endoscopic strip craniectomy (ESC) and helmet therapy with those treated by fronto-orbital advancement (FOA). METHODS Consecutive patients with UCS, uncomplicated by other suture synostosis, were identified by a retrospective review of medical records. Assessment of presence of amblyopia, cycloplegic refraction, strabismus, and strabismus surgical intervention at all visits was recorded. RESULTS Between 2004 and 2010, 22 patients were treated by FOA (mean follow-up, 21.5 months) and 21 patients with ESC and helmet therapy (mean follow-up, 23.5 months). The mean aniso-astigmatism was equal; however, the SD was greater for those treated by FOA (P < 0.05). A more severe pattern of strabismus developed in those treated by FOA (P < 0.0001). Those treated by FOA were more likely to have amblyopia (P = 0.0015) and to undergo surgical correction of their strabismus (odds ratio, 6.3:1). CONCLUSIONS Children with UCS treated with ESC and helmeting had less severe overelevation in adduction, amblyopia, extremes of astigmatism, and less need for strabismus surgery than those treated by FOA. Although the reason for these more favorable outcomes remains uncertain, we speculate that the earlier timing of ESC or differences in the anatomical changes resulting from the two procedures may play a role.

Amniotic membrane implantation to reduce extraocular muscle adhesions to a titanium implant

Amniotic membrane grafts are used extensively for ocular surface reconstruction. We describe the case of a 66-year-old man with traumatic, restrictive strabismus. Amniotic membrane was applied to resolve symblepharon between the globe and lids and also to prevent re-formation of adhesions between extraocular muscles and adjacent titanium plates placed during prior surgery.

Using spectral-domain optical coherence tomography to detect optic neuropathy in patients with craniosynostosis

BACKGROUND Detecting and monitoring optic neuropathy in patients with craniosynostosis is a clinical challenge due to limited cooperation, and subjective measures of visual function. The purpose of this study was to appraise the correlation of peripapillary retinal nerve fiber layer (RNFL) thickness measured by spectral-domain ocular coherence tomography (SD-OCT) with indication of optic neuropathy based on fundus examination. METHODS The medical records of all patients with craniosynostosis presenting for ophthalmic evaluation during 2013 were retrospectively reviewed. The following data were abstracted from the record: diagnosis, historical evidence of elevated intracranial pressure, current ophthalmic evaluation and visual field results, and current peripapillary RNFL thickness. RESULTS A total of 54 patients were included (mean age, 10.6 years [range, 2.4-33.8 years]). Thirteen (24%) had evidence of optic neuropathy based on current fundus examination. Of these, 10 (77%) demonstrated either peripapillary RNFL elevation and papilledema or depression with optic atrophy. Sensitivity for detecting optic atrophy was 88%; for papilledema, 60%; and for either form of optic neuropathy, 77%. Specificity was 94%, 90%, and 83%, respectively. Kappa agreement was substantial for optic atrophy (κ = 0.73) and moderate for papilledema (κ = 0.39) and for either form of optic neuropathy (κ = 0.54). Logistic regression indicated that peripapillary RNFL thickness was predictive of optic neuropathy (P < 0.001). Multivariable analysis demonstrated that RNFL thickness measurements were more sensitive at detecting optic neuropathy than visual field testing (likelihood ratio = 10.02; P = 0.002). Sensitivity and specificity of logMAR visual acuity in detecting optic neuropathy were 15% and 95%, respectively. CONCLUSIONS Peripapillary RNFL thickness measured by SD-OCT provides adjunctive evidence for identifying optic neuropathy in patients with craniosynostosis and appears more sensitive at detecting optic atrophy than papilledema.

Associated signs, demographic characteristics, and management of dacryocystocele in 64 infants

PURPOSE To describe the incidence of associated infection, respiratory compromise, apparent intranasal cyst, as well as sex, laterality, and age at presentation in 64 infants with dacryocystocele and to assess characteristics associated with successful interventions. METHODS A retrospective chart review of all patients with dacryocystocele seen at Childrens Hospital Boston between 1996 and 2010 was performed. Inclusion criteria were accuracy of diagnosis, treatment, and follow-up at our institution. Interventions were divided into 3 categories: procedures that did not require general anesthesia; simple procedures requiring general anesthesia, such as nasolacrimal probing with or without stent or balloon dilation; and more complex procedures under general anesthesia, specifically, those aided by intranasal endoscopy. RESULTS Of the 90 identified patients, 64 met inclusion criteria. The majority of patients were female (63%) and had unilateral involvement (77%). More than one-half of all patients were successfully treated without anesthesia; however, patients presenting with infection were more likely to be treated with a simple procedure under general anesthesia. All patients treated endoscopically had intranasal cysts. Age, sex, and infection did not predict the use of intranasal endoscopy. Bilaterality of dacryocystocele was associated with the use of an endoscopic approach. CONCLUSIONS Many infants with dacryocystocoele can be successfully treated without general anesthesia. The incidence of occult intranasal cyst among those treated without endoscopy remains unknown. Patients who were treated under general anesthesia but without the use of nasal endoscopy were more likely to have an infected system, but the clinical significance of this association is not clear.

Adjustable nasal transposition of split lateral rectus muscle for third nerve palsy.

IMPORTANCE Third nerve palsy causes disfiguring, incomitant strabismus with limited options for correction. OBJECTIVE To evaluate the oculomotor outcomes, anatomical changes, and complications associated with adjustable nasal transposition of the split lateral rectus (LR) muscle, a novel technique for managing strabismus associated with third nerve palsy. DESIGN, SETTING, AND PARTICIPANTS Retrospective medical record review appraising outcomes of 6 consecutive patients with third nerve palsy who underwent adjustable nasal transposition of the split LR muscle between 2010 and 2012 with follow-up of 5 to 25 months at a tertiary referral center. INTERVENTION Adjustable nasal transposition of the split LR muscle. MAIN OUTCOMES AND MEASURES The primary outcome was postoperative horizontal and vertical alignment. Secondary outcomes were (1) appraising the utility of adjustable positioning, (2) demonstrating the resultant anatomical changes using magnetic resonance imaging, and (3) identifying associated complications. RESULTS Four of 6 patients successfully underwent the procedure. Of these, 3 patients achieved orthotropia. Median preoperative horizontal deviation was 68 prism diopters of exotropia and median postoperative horizontal deviation was 0 prism diopters (P = .04). Two patients had preoperative vertical misalignment that resolved with surgery. All 4 patients underwent intraoperative adjustment of LR positioning. Imaging demonstrated nasal redirection of each half of the LR muscle around the posterior globe, avoiding contact with the optic nerve; the apex of the split sat posterior to the globe. One patient had transient choroidal effusion and undercorrection. Imaging revealed, in this case, the apex of the split in contact with the globe at an anterolateral location, suggesting an inadequate posterior extent of the split. In 2 patients, the surgical procedure was not completed because of an inability to nasally transpose a previously operated-on LR muscle. CONCLUSIONS AND RELEVANCE Adjustable nasal transposition of the split LR muscle can achieve excellent oculomotor alignment in some cases of third nerve palsy. The adjustable modification allows optimization of horizontal and vertical alignment. Imaging confirms that the split LR muscle tethers the globe, rotating it toward primary position. Case selection is critical because severe LR contracture, extensive scarring from prior strabismus surgery, or inadequate splitting of the LR muscle may reduce the likelihood of success and increase the risk of sight-threatening complications. Considering this uncertainty, more experience is necessary before widespread adoption of this technique should be considered.

Morning Glory Disc Anomaly in Association With Ipsilateral Optic Nerve Glioma

Comment. Improvement in vision rarely occurs in patients with AION due to GCA, presumably because there has been complete occlusion of the posterior ciliary arteries causing optic nerve head infarction. Although some series have reported improvement in up to one-third of patients, there has often not been an improvement in the visual field, suggesting that the apparent recovery could be an artifact of visual acuity testing (eg, learned ability to eccentrically fixate). In series in which visual acuity and visual field changes have been reported, improvement in both has been observed in 4% to 5% of eyes, although the improvement was not substantial in most cases. Factors that predict visual recovery remain unclear, although the chance of improvement might be higher when treatment with corticosteroids is started early. Our patient with biopsy-proven GCA initially had no light perception in one eye, associated with signs of AION. She was immediately treated with prednisone and subsequently experienced dramatic improvement in both visual acuity and visual field over subsequent weeks. The presence of hyperemic rather than pallid optic disc edema and the delayed rather than absent choroidal filling on fluorescein angiography suggest that there was severe inflammatory narrowing, rather than complete occlusion, of the posterior ciliary arteries or development of collaterals. We propose that this unusual extent of vision recovery occurred because there was reversible ischemia rather than infarction of the optic nerve head. We suggest that hyperemic optic disc edema and delayed choroidal filling without posterior ciliary artery occlusion could predict a chance of improvement in patients with AION due to GCA.