Journal of AAPOS

Improving safety for babies during the outpatient retinopathy of prematurity examination

Graham E. Quinn, Jacquelyn Evans — 2009-08-24

In their report “Apnea and bradycardia in two premature infants during routine outpatient ROP screening,” in this month's issue of the Journal of AAPOS, Wood and Kaufman report 2 serious and unexpected episodes of apnea and bradycardia that resulted in emergent hospital admissions for the babies. These near tragedies certainly warrant close examination and have led Drs. Wood and Kaufman to develop a new protocol for monitoring the babies and improving the safety for these very important examinations. They now strongly recommend that the families return to the hospital from which the babies were discharged. The babies are placed in a room adjacent to the neonatal intensive care unit (NICU) and have drops instilled by a NICU nurse, who remains with the baby during the examination. Drs. Wood and Kaufman have entered this new arrangement under contract with the hospital, which has assumed not only responsibility for safety during the retinopathy of prematurity (ROP) examinations but also responsibility for ensuring follow-up. The authors also suggest an alternative of providing advanced life support equipment and expertise to care for such infants in an outpatient setting.

When and how to strengthen the superior oblique muscle

Richard A. Saunders — 2009-10-01

Purpose: To review the history of procedures used to strengthen the action of the superior oblique (SO) muscle and methods of quantifying surgical dosage and to determine the relationship between congenital onset and tendon laxity measured at the time of surgery.Methods: We reviewed medical records over a 10-year period of 30 patients who had undergone SO tendon tuck for SO muscle palsy using intraoperative assessment of forced ductions to determine surgical dosage. We also designed and tested a modified Bishop tucker, which can simultaneously measure tendon shortening and the force required. This allowed development of length tension curves between 0 and 200 g for individual SO tendons in patients with and without evidence of muscle palsy.Results: In distinction to most other procedures on the extraocular muscles, intraoperative forced ductions are used to determine appropriate surgical dosage. Patients undergoing SO tendon tuck using a uniform and repeatable forced duction method receive a greater amount of tuck (mean, 3 mm) when there is known congenital onset. Patients with congenital SO muscle palsy received a mean tuck of 10.8 mm (range, 8-16 mm), whereas patients with equivocal or known adult-onset SO palsy received a mean of 7.8 mm (range, 4-12 mm; p = 0.002).Conclusions: Patients with congenital SO muscle palsy have increased tendon laxity when measured directly during SO tendon tuck. The excursion of presumed normal SO tendons through the trochlea is variable and may be less than previously thought. Modifying the Bishop tucker to provide length and tension data provides information that may be useful in determining surgical dosage.

Image analysis for retinopathy of prematurity diagnosis

2009-10-01

Purpose: To review findings from the authors' published studies involving telemedicine and image analysis for retinopathy of prematurity (ROP) diagnosis.Methods: Twenty-two ROP experts interpreted a set of 34 wide-angle retinal images for presence of plus disease. For each image, a reference standard diagnosis was defined from expert consensus. A computer-based system was used to measure individual and linear combinations of image parameters for arteries and veins: integrated curvature (IC), diameter, and tortuosity index (TI). Sensitivity, specificity, and receiver operating characteristic areas under the curve (AUC) for plus disease diagnosis were determined for each expert. Sensitivity and specificity curves were calculated for the computer-based system by varying the diagnostic cutoffs for arterial IC and venous diameter. Individual vessels from the original 34 images were identified with particular diagnostic cutoffs, and combined into composite wide-angle images using graphics editing software.Results: For plus disease diagnosis, expert sensitivity ranged from 0.308-1.000, specificity from 0.571-1.000, and AUC from 0.784 to 1.000. Among computer system parameters, one linear combination had AUC 0.967, which was greater than that of 18 of 22 (81.8%) experts. Composite computer-generated images were produced using the arterial IC and venous diameter values associated with 75% under-diagnosis of plus disease (ie, 25% sensitivity cutoff), 50% under-diagnosis of plus disease (ie, 50% sensitivity cutoff), and 25% under-diagnosis of plus disease (ie, 75% sensitivity cutoff).Conclusions: Computer-based image analysis has the potential to diagnose severe ROP with comparable or better accuracy than experts, and could provide added value to telemedicine systems. Future quantitative definitions of plus disease might improve diagnostic objectivity.

Retinopathy of prematurity in extremely low birth weight infants in Malaysia

May May Choo, Frank Joseph Martin, Lim Chin Theam, Chan U-Teng — 2009-10-01

Purpose: To identify differences in incidence, risk factors, and outcomes of retinopathy of prematurity (ROP) between 2 birth weight categories within a cohort of extremely low birth weight (ELBW) infants in Malaysia.Methods: This was a prospective study of infants in the Special Care Nursery at the University of Malaya Medical Centre between 2003 and 2005. Outcome measures were presence or absence of ROP, most severe stage of ROP observed, and whether laser treatment was performed. Risk factors for treatment were analyzed in this cohort of treated patients, who were further divided into groups of birth weight <751 g and birth weight 751–1,000 g.Results: The study protocol identified 70 qualifying ELBW infants. Of these, 41 (58.6%) developed ROP and 23 (32.9%) required laser treatment. Mean birth weight was 806.8 g (SD ± 142.5); mean gestation was 27.4 weeks (SD ± 2.2). Infants with birth weight <750 g were twice as likely to require treatment (OR = 2, p = 0.038). The risk factors for laser treatment by bivariate analysis were gestation <28 weeks (OR = 1.8, p = 0.001), duration of ventilation >1 week (OR = 1.5, p = 0.012), and intraventricular hemorrhage (OR = 2.5, p = 0.010). Zone 1 ROP was observed only in infants <751 g.Conclusions: The incidence of ROP in ELBW infants in Malaysia is comparable to that seen in the ETROP and CRYO-ROP studies. Within this group, birth weight <750 g doubled the likelihood that treatment would be required.

Effect of granulocyte colony-stimulating factor on the incidence of threshold retinopathy of prematurity

Rahul Bhola, Todd Purkiss, Stephen Hunter, Dan Stewart, Paul J. Rychwalski — 2009-10-01

Purpose: To study the effect of granulocyte colony-stimulating factor (GCSF) on the incidence of threshold retinopathy of prematurity (ROP).Methods: A retrospective chart review of all neonates who received GCSF at our neonatal intensive care unit over a period ranging from January 2003 to September 2007 was performed. Of the 213 patients identified, 50 patients with birth weight <1,500 g and gestational age <32 weeks were included in this study. The incidence of threshold ROP in this subset was compared to a control group (n = 161) obtained from Vermont Oxford Network ROP database over the same time period. The two data sets were cross-referenced to exclude any patient receiving GCSF from the control data.Results: The average birth weight was 913 g in GCSF patients and 847 g in controls. The average dose of GCSF was 10.4 μg (range, 1.2–21.6 μg). The average age at administration of GCSF was 12 days after birth (range, 0-34 days). Out of 50 patients in the GCSF group, 5 (10%) required laser treatment, whereas in the contol group, 30 (18.6 %) required laser treatment; this difference was not statistically significant.Conclusions: There was a suggestion of a decreased incidence of threshold ROP, leading to reduced need for laser treatment, in patients who received GCSF, but the observed differences were not statistically significant. The authors anticipate that these findings will lead to further study of the upstream regulators of neovascularization.

Outcomes of cataract surgery following radiation treatment for retinoblastoma

John F. Payne, Amy K. Hutchinson, G. Baker Hubbard, Scott R. Lambert — 2009-10-01

Purpose: To report the outcomes after cataract extraction in eyes previously treated for retinoblastoma.Methods: We reviewed the medical records of consecutive patients treated with cataract extraction at our institution for radiation-induced cataract following retinoblastoma treatment between 1992 and 2007.Results: Twelve eyes of 11 patients were included. The median age at diagnosis of retinoblastoma was 7.4 months. The median quiescent interval before cataract surgery was 34.6 months (range, 17-240 months). All patients underwent limbal-based extracapsular cataract extraction with scleral-tunnel wound construction. Anterior vitrectomy and posterior capsulotomy were performed in 7 eyes; an intraocular lens was placed in 10 eyes. A laser capsulotomy was subsequently performed in 5 eyes. No postoperative complications occurred. One patient underwent a pars plana vitrectomy for vitreous hemorrhage and proliferative retinopathy 5 years after cataract extraction. The median follow-up after surgery was 6.0 years (range, 1.1-12.3 years). Final visual acuity was between 20/20 and 20/60 in 6 eyes, between 20/70 and 20/200 in 2 eyes, and worse than 20/200 in 4 eyes. No intraocular tumor recurrences or metastases occurred. Two patients developed secondary rhabdomyosarcomas; 1 patient succumbed to the disease.Conclusions: Limbal-based cataract extraction in our series was not associated with tumor recurrence or metastasis. The optimal quiescent interval is not known; however, no tumor recurrences occurred in our series when a minimum 17-month quiescent period was achieved. Visual improvement was noted in every patient, although several patients only experienced modest gains.

The value of serial personal photographs in timing the onset of unilateral cataracts in children

Gagan K. Sawhney, Amy K. Hutchinson, Scott R. Lambert — 2009-10-01

Purpose: To determine the value of serial personal photographs in timing the onset of unilateral cataracts in children over 6 months of age.Methods: Personal photographs from children with unilateral cataracts who underwent cataract extraction and intraocular lens implantation when ≥6 months of age were reviewed. Photographs were evaluated for changes in the red reflex, which might indicate the presence of a cataract.Results: Twelve children underwent cataract surgery at a mean age of 37 months. They were followed for a mean of 32 months. Ten children were diagnosed as having an acquired cataract by photographic review documenting a previously normal red reflex. The visual acuity in the affected eye of 4 of these children improved to ≥20/60. Cataracts were visible on photographs prior to clinical diagnosis in 6 patients, from 0.5 to 22 months prior to clinical diagnosis. Visual outcomes did not relate closely to the photographically documented duration of the cataract prior to treatment. Photographs were not helpful in timing the onset of cataract in 2 children due to the poor quality of the images.Conclusions: Serial personal photographs are sometimes helpful in determining whether cataracts are acquired. However, the usefulness of personal photographs alone in predicting the visual outcome after cataract surgery was limited in this small, retrospective study.

Which Leber congenital amaurosis patients are eligible for gene therapy trials?

Arlene V. Drack, Rebecca Johnston, Edwin M. Stone — 2009-10-01

Background: In 2007, clinical trials began for gene-replacement therapy for RPE65-associated Leber congenital amaurosis. To enroll, subjects must have both disease-causing RPE65 alleles identified. Determining which patients have true disease-causing mutations requires a multistep approach.Methods: This study is a retrospective case series using the estimate of pathogenic probability (EPP) algorithm and genotyping of family members to establish phase.Results: Five probands and their families were studied. Patient 1 had genetic testing elsewhere and was reported to have 2 disease-causing AIPL1 mutations. The family received incorrect prenatal counseling based on this result. We found both variations to be benign ethnic polymorphisms (EPP = 0). Case 2 had possible disease-causing mutations in RPE65, RPGRIP1, and CRB1; however, screening of family members revealed that only CRB1 variations were disease causing and the RPE65 change was a polymorphism found in 11% of African Americans. Case 3 had a diagnosis of CRB1-associated Leber congenital amaurosis, but this mutation had an EPP = 0; a true homozygous disease-causing mutation was later found in RDH12. Patient 4 had 3 mutations found in RPE65, but only 2 were disease causing. Patient 5 had a homozygous mutation in RPE65. Only Patients 4 and 5 would be eligible for clinical trials of RPE65 gene replacement.Conclusions: To be eligible for participation in current RPE65 gene therapy trials, patients' DNA must contain 2 correctly segregating alleles with an EPP = 2 or 3. Interpretation of DNA variants is complex; genetic misdiagnosis may lead to ineffective treatment in some patients and lack of treatment in others.

Accuracy of the Welch Allyn SureSight for measurement of magnitude of astigmatism in 3- to 7-year-old children

2009-10-01

Purpose: To evaluate the accuracy of the Welch Allyn SureSight in noncycloplegic measurements of astigmatism as compared to cycloplegic Retinomax K+ autorefractor measurements of astigmatism in children from a Native American population with a high prevalence of high astigmatism.Methods: Data are reported for 825 3- to 7-year-old children with no ocular abnormalities. Each child had a Retinomax K+ cycloplegic measurement of right eye astigmatism with a confidence rating ≥8 and 3 attempts to obtain a SureSight measurement on the right eye.Results: SureSight measurement success rates did not differ significantly across age or measurement confidence rating (<6 vs ≥6). Ninety-six percent of children had at least 1 measurement (any confidence), and 89% had at least 1 measurement with confidence at the manufacturer's recommended value (≥6). Overall, the SureSight tended to overestimate astigmatism. If the SureSight measurement had any dioptric value (0.00 D to 3.00 D), astigmatism of 2.00 D or less was likely to be present. If the SureSight showed astigmatism beyond the instrument's dioptric range (>3.00 D), Retinomax K+ measurements indicated that >2.00 D of astigmatism was present in 136 of 157 (86.6%). In cooperative children for whom the SureSight would not give a reading, 32 of 34 (94%) had >3.00 D of astigmatism.Conclusions: The SureSight does not provide an accurate, quantitative measure of amount of astigmatism. However, it does allow accurate categorization of amount of astigmatism as ≤2.00 D, >2.00 D, or >3.00 D, and it has high measurement success rate in young children.

Results of extraocular muscle surgery for superior oblique myokymia

Swati Agarwal, Burton J. Kushner — 2009-08-31

Purpose: To report results of extraocular muscle surgery for superior oblique myokymia when medical treatment fails.Methods: A retrospective review of 14 consecutive patients undergoing superior oblique tenectomy and inferior oblique myectomy between 1976 and 2008.Results: The mean age of onset of symptoms was 35.4 ± 12.6 years (range, 16-59.5), with a mean duration of oscillopsia of 5 ± 4 years (range, 1.5-17) prior to surgery. Medical treatment was unsuccessful in all 14. Preoperatively, 2 had a small hypertropia that was consistent with an ipsilateral fourth (trochlear) nerve palsy; 12 had no manifest tropia. Postoperatively, all had complete elimination of oscillopsia, and 12 of 14 were free of diplopia in the primary position at 6 meters and 1/3 meter. The only 2 with diplopia in the primary position after surgery were the 2 with a manifest hypertropia preoperatively. Of the remaining 12 patients, 5 had a hypertropia of the affected eye limited to downgaze after surgery (mean of 6.2Δ ± 1.6Δ). Of the 5, 3 needed contralateral inferior rectus surgery, and 1 required prism for downgaze. The mean follow-up was 4.1 ± 2.4 years (range, 0.5-10). At the final visit, none had oscillopsia or uncontrolled diplopia, but 3 (21%) needed prisms.Conclusions: Superior oblique tenectomy and inferior oblique myectomy effectively eliminate oscillopsia associated with superior oblique myokymia but result in diplopia in downgaze in approximately 36% of patients, which may cause symptoms in patients who require a bifocal for near work.

The psychosocial aspects of strabismus: correlation between the AS-20 and DAS59 quality-of-life questionnaires

Jonathan Mark Durnian, Manon Elena Owen, Ian Bruce Marsh — 2009-10-01

Purpose: To compare two quality-of-life (QoL) scales and subscales: the Derriford Appearance Scale 59 (DAS59) and the Adult Strabismus-20 (AS-20) scale in a series of strabismic and nonstrabismic patients and to illustrate the differences in results between strabismic and nonstrabismic patients.Methods: The DAS59 is a self-report QoL questionnaire generating an assessment of distress caused by problems of appearance. The AS-20 is a newly developed strabismus specific QoL scale. A prospective, noninterventional study of a series of consecutive adult patients undergoing strabismus surgery and a control group was performed. Patients were given both questionnaires to complete before surgery. The underlying condition, previous number of surgeries, and deviation measurements were recorded.Results: Thirty-four patients and 30 controls correctly completed both questionnaires. In the strabismus group, the mean AS-20 score was 50.5. The mean DAS59 score was 122.5 (out of 269). There is strong correlation between the scales (r = −0.813; 95% CI, −0.90 to −0.66; p < 0.0001). In the control group, the AS-20 score was 98.8 and the DAS59, 84.4, with both showing significant differences to the strabismic group (t = 12.9 [p < 0.0001] and t = −4.642 [p < 0.0001], respectively).Conclusions: The AS-20 shows strong correlation to the widely used DAS59. Strabismus patients show significantly more psychosocial distress than controls. The AS-20 is specific to strabismus patients and the result is not influenced by any other systemic factors that can impinge on the more general DAS59 scale.

Predictive factors of surgical outcome in oculomotor nerve palsy

Laura Cabrejas, Francisco J. Hurtado-Ceña, Jaime Tejedor — 2009-10-01

Purpose: To investigate the outcomes and predictive factors of surgical treatment of oculomotor nerve palsy.Methods: Records of patients requiring eye muscle surgery for oculomotor nerve palsy in our institution were retrospectively reviewed. Age, sex, etiology, deviation, completeness of involvement, time between onset and surgery, botulinum toxin treatment, and number of surgical procedures were recorded as potential predictive factors. Muscle function, presence of diplopia, and torticollis were also recorded. The main outcome measure was motor function. Secondary outcome measures were presence of diplopia, torticollis, and limitation of muscle function.Results: Surgery was required in 22 patients, of whom motor success was obtained in 14 (63.6%). Frequency of diplopia and torticollis were significantly reduced by surgery. After multivariate regression analysis, longer time between onset and surgery (p = 0.03) and larger initial deviation (p = 0.05) were significantly associated with poorer postsurgical results in terms of motor function.Conclusions: Longer time from onset to surgery and larger eye deviation are negative prognostic factors of postsurgical motor success for oculomotor nerve palsy.

Traumatic superior oblique tendon rupture

Ajay Yeliathaya Harish, Sandra C. Ganesh, Kalpana Narendran — 2009-10-01

Traumatic rupture of an extraocular muscle is rare, and most commonly affects the horizontal and vertical rectus muscles. We report a case of a 45-year-old man injured by a metal hook, with rupture of the superior oblique tendon. The resulting deficit was less severe than expected, raising the question of whether a few superior oblique fibers remained intact.

A child with bilateral orbital cellulitis one day after strabismus surgery

Ahmed Basheikh, Rosanne Superstein — 2009-10-01

Orbital cellulitis is a rare complication of strabismus surgery. We report a case of a 4-year-old boy with bilateral orbital cellulitis after uneventful surgery to correct esotropia. Concomitant sinusitis was the likely source of infection. To our knowledge, this is the first case of bilateral orbital cellulitis following strabismus surgery.

Acute traumatic orbital cerebrospinal fluid cystocele mimicking orbital abscess

Suhair Twaij, Palpandian Viswanathan, A.B. Page — 2009-08-31

Posttraumatic cranio-orbital cerebrospinal fluid (CSF) fistula is rare, occurring when there is a communication between orbit and subarachnoid space. This communication can be caused by orbital tumors with intracranial extension or can be a result of orbital surgery. Low suspicion for traumatic orbital CSF fistula can lead to delay in diagnosis, which increases the risk of meningitis. We report a cranio-orbital CSF cystocele and fistula that mimicked orbital cellulitis in a 3-year-old boy following an orbitocranial penetrating injury.

Septo-optic dysplasia with bilateral congenital corneal anesthesia

Clement C. Chow, Rashmi Kapur, Michael G. Wood, Pete Setabutr, Elmer Y. Tu — 2009-10-01

Septo-optic dysplasia, or de Morsier syndrome, is characterized by optic nerve hypoplasia with an absent septum pellucidum and/or pituitary abnormalities. Congenital corneal anesthesia is a rare disorder that has been associated with many neurological disorders. Here we present a patient with both conditions who was successfully treated with permanent lateral tarsorrhaphy and aggressive lubrication. To our knowledge, congenital corneal anesthesia has not been reported in association with septo-optic dysplasia. The purpose of this report is to make pediatric ophthalmologists aware of a potential association since the diagnosis of congenital corneal anesthesia is often difficult and delayed.

Misleading clinical stereoacuity levels in pediatric maculopathy

Joan Parkinson, Francois Tremblay — 2009-08-31

Because visual acuity and stereoacuity are assumed to be almost directly related, pediatric ophthalmologists and orthoptists may be inclined to compare them in cases of suspected functional visual acuity loss; good stereoacuity in the presence of reduced visual acuity frequently suggests a functional component. We report 3 cases involving pediatric maculopathy in which stereoacuity was relatively unaffected by reduced visual acuity. Along with the initial normal fundus appearance, which is frequently observed in pediatric maculopathy, good stereoacuity suggested functional visual acuity loss and delayed proper diagnosis in the first 2 cases. In the third case, visual-evoked potentials, electroretinography, and fluorescein angiography documented organic components; the eventual addition of a multifocal electroretinogram afforded a prompt diagnosis of maculopathy.

Spontaneous resolution of retinal detachment in morning glory disk anomaly

2009-08-31

Morning glory disk anomaly is generally a unilateral, sporadic congenital abnormality of the optic disk. These eyes are at risk of developing retinal detachment requiring surgical repair. The etiology of retinal detachment is incompletely understood in this condition and some reports have attributed it to a retinal break. We report an unusual case of morning glory disk anomaly with total nonrhegmatogenous exudative retinal detachment that displayed remarkable subsequent spontaneous resolution.

Apnea and bradycardia in two premature infants during routine outpatient retinopathy of prematurity screening

Michael G. Wood, Lawrence M. Kaufman — 2009-06-01

Two unrelated premature infants at risk for retinopathy of prematurity (ROP) were discharged home from a neonatal intensive care unit. Appropriately timed ROP examinations were performed in an outpatient setting in a free-standing pediatric ophthalmologist's private office remote from any hospital. The infants developed severe apnea and bradycardia at the end of the ROP examinations. As a result, a new protocol for outpatient ROP screening was developed.

Semiautomated digital image analysis of posterior pole vessels in retinopathy of prematurity

2009-10-01

Plus disease is a major indicator for treatment in retinopathy of prematurity (ROP), and computer-assisted image analysis of vessel caliber and tortuosity in the posterior pole may indicate disease progression and severity. We sought to determine whether semiautomated digital analysis of posterior pole vessels using narrow field images with varying severity of ROP correlated with vessel width and tortuosity.

Image analysis of posterior pole vessels identifies type 1 retinopathy of prematurity

Deepika N. Shah, Karen A. Karp, Gui-shuang Ying, Monte D. Mills, Graham E. Quinn — 2009-10-01

Identification of type 1 retinopathy of prematurity (ROP) relies heavily on the presence of characteristics of plus disease, especially tortuosity. However, a relatively infrequent subset of eyes with type 1 ROP, eyes with zone 1, stage 3 ROP without plus disease, is included in treatment indications. We examined if posterior pole vessel width is associated with type 1 ROP in a subset of eyes with zone 1, stage 3 ROP without plus disease and whether vessel width differentiates type 1 from non–type 1 ROP.

Longitudinal assessment of plus disease in retinopathy of prematurity using color Doppler imaging

2009-10-01

Retinal vascular changes and the development of plus disease are the hallmarks of retinopathy of prematurity (ROP). The purpose of this study was to evaluate whether or not serial examinations of retrobulbar blood flow characteristics, as measured by color Doppler imaging (CDI) performed repeatedly over a period of several weeks, would be useful for predicting those infants at risk for developing plus disease and to determine whether this technique may be used as an objective tool for confirming the presence of plus disease. Of the 73 infants followed in this study, 14 (19%) developed plus disease confirmed by a panel of experts. When comparing the group of infants developing plus disease with those infants who did not develop plus disease, we did not find any significant differences in the retrobulbar blood flow characteristics of either the central retinal or ophthalmic arteries. Color Doppler imaging did not appear to be a clinically useful tool in the longitudinal management of ROP, nor did it appear to be useful as an objective determinant of plus disease in these premature infants.

Ocular manifestations of the Johanson-Blizzard syndrome

Jason C. Cheung, Hugh Thomson, J. Raymond Buncic, Elise Héon, Alex V. Levin — 2009-08-31

Johanson-Blizzard syndrome is a rare autosomal-recessive congenital disorder characterized by hypoplastic nasal alae, midline scalp defects, deafness, microcephaly, hypothyroidism, absent permanent teeth, malabsorption, and failure to thrive. The literature was reviewed to define the reported spectrum of ocular manifestations, which are not well documented. We found that nasolacrimal system malformations are a common feature of Johanson-Blizzard, whereas intraocular malformations are rare. This report describes the ophthalmologic findings and management of 2 affected children.

Juvenile xanthogranuloma masquerading as melanoma

2009-10-01

Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. We present an atypical case of bilateral JXG involving the entire uveal tract that presented with an aggressive phenotype. The patient was unresponsive to topical and systemic corticosteroids, cyclosporine, and maximal doses of radiation therapy. The disease was ultimately controlled with the alkylating agent chlorambucil.

Successful treatment of ligneous conjunctivitis with topical cyclosporine and heparin

Nadia Azad, Saemah Zafar, Ayesha Khan — 2009-10-01

Ligneous conjunctivitis is a rare disease characterized by wood-like pseudomembranes developing on the ocular and extraocular mucosae. We present a case of ligneous conjunctivitis and cataracts in a 10-year-old boy with a positive family history of the disease and consanguinity. His condition was managed with topical cyclosporine, heparin, and steroids.

A novel method for management of conjunctival inclusion cysts following strabismus surgery using isopropyl alcohol with paired injection technique

Mihir Kothari — 2009-10-01

Conjunctival inclusion cysts following strabismus surgery are typically treated by surgical excision in a hospital setting. Cysts may be aspirated in the office setting with the aid of a slit lamp, but this approach is associated with recurrence. Thermal cautery and intracyst injection of doxycycline may also be performed in the office. We present 2 cases of conjunctival inclusion cysts treated with isopropyl alcohol injection in an office setting. There was no recurrence at 9 months follow-up.

Resolution of recurrent conjunctival papilloma after topical and intralesional interferon α2b with partial excision in a child

Mihir Kothari, Kruti Mody, Debapriya Chatterjee — 2009-10-01

The human papilloma virus (HPV) is a double-stranded DNA virus that infects the epithelium of skin and mucosa. Simple treatment of conjunctival papilloma is associated with recurrences, and while most medical therapy has met with limited success, interferon alpha 2b (IFN α2b) may be effective. In this report, we describe a 7-year-old child with severe, recurrent conjunctival papilloma who had complete regression of the papilloma after topical and intralesional IFN α2b with partial excision of the lesion.

Extraocular muscle hypertrophy in myotonia congenita: Mutation identified in the SCN4A gene (V445M)

Bradley Wakeman, Ian M. MacDonald, Ieke Ginjaar, Jack Tarleton, Deepti Babu — 2009-10-01

To the Editor: Nondystrophic myotonia (NDM) is a group of skeletal muscle disorders characterized by persistent muscle contraction after voluntary contraction. These disorders are caused by mutations in the genes encoding the sodium (SCN4A) and chloride (CLCN1) ion channels involved in the action potential of the T-tubules of skeletal muscle.

Congenital Osteoma Cutis of the Lateral Canthus

Arif O. Khan — 2009-10-01

To the Editor: In their report of apparently isolated congenital osseous choristoma of the lateral canthus in 2 children, Gupta and colleagues do not cite our previous description of this entity that was published in the Journal of AAPOS. In our case, the lesion had a unique U-shaped configuration, which led us to believe that it originated from pluripotential cells that inappropriately migrated around the lateral canthus during embryogenesis. It would be interesting to know what the gross configurations were of the 2 excised lesions described by Gupta and colleagues.

Reply

Shelly R. Gupta, Martin S. Cogen — 2009-10-01

To the Editor: The isolated congenital osseous choristoma of the lateral canthus reported by Khan and colleagues appears to remain unique in its U-shaped configuration. In our cases, both lesions appeared nodular on clinical examination and also had attachments to the bulbar conjunctiva. On gross pathology, the lesion in the first case was described as “polypoid”; in the second case, as “ovoid.” We greatly appreciate the comment by Khan and colleagues.

The critical period for surgical treatment of dense congenital bilateral cataracts

Niranjan K. Pehere, Garudadri Chandrasekhar, Ramesh Kekunnaya — 2009-10-01

To the Editor: We congratulate Birch and colleagues for their important contribution to the study of surgical treatment of bilateral dense congenital cataracts. We would like to make the following observations.

Reply

Eileen Birch, Christina Cheng, David Stager, David Weakley, David Stager — 2009-10-01

To the Editor: We thank the correspondents for their interest in our article. Drs. Pehere, Chandrasekhar, and Kekunnaya raise 4 issues. First, they state that there is evidence to suggest that cataract surgery in the first month of life is associated with a high risk of glaucoma. This association, which has been found in a number of retrospective studies, was presented in our introduction. However, association of risk and age at surgery does not imply causation. It may also be the case that those children who are surgical candidates during the first month of life have more involved eyes (dense cataracts, small eyes, associated structural abnormalities) and thus are more likely to develop glaucoma regardless of age at surgery. In addition, even among the children who developed glaucoma in our cohort, no patient who had surgery by 4 weeks of age had a long-term visual acuity outcome poorer than 20/60 and the mean visual acuity outcome was 20/40; that is, the glaucoma was treatable and did not compromise visual acuity outcome.

Treatment of severe amblyopia with weekend atropine: Results from 2 randomized clinical trials

Philip Lempert — 2009-10-01

To the Editor: The authors of this report assert that “weekend atropine can improve visual acuity” in children with severe amblyopia. An earlier PEDIG study documented a consistent change from poor to better acuity with increasing age for both the amblyopic and the fellow eyes prior to treatment (Table 3). In particular, 65% of children less than 4 years old had visual acuities of 20/80 or less in the amblyopic eye prior to initiation of treatment, while in the 6- to 7-year-old group only 37% had visual acuities of 20/80 or less.

Reply

2009-10-01

To the Editor: Dr. Lempert selected cross-sectional pretreatment visual acuity data from our earlier study of moderate amblyopia to suggest that there is spontaneous improvement in visual acuity of amblyopic eyes occurring over a four-year span between “early and late childhood.” In fact, there was only a 0.7 line difference in mean amblyopic eye acuity before treatment between the younger and the older children which may represent a small age effect. These cross-sectional acuity data from children four years apart in age are not comparable to our data from a longitudinal study of four months of treatment.

Books Received

2009-10-01

Barry SR. Fixing my Gaze: A Scientist's Journey into Seeing in Three Dimensions. New York: Basic Books; 2009. 249 p. Hardcover. ISBN: 978-0-465-00913-8.

Errata

2009-10-01

The Journal regrets and apologizes for the following error in the June 2009 issue. Traboulsi EI. Morning glory disk anomaly—More than meets the eye. J AAPOS 2009;13:333-34.

Editorial Board

2009-10-01

David G. Hunter, MD, PhD Boston, MA

2009-10-01

Journal of AAPOS

Improving safety for babies during the outpatient retinopathy of prematurity examination

When and how to strengthen the superior oblique muscle

Image analysis for retinopathy of prematurity diagnosis

Retinopathy of prematurity in extremely low birth weight infants in Malaysia

Effect of granulocyte colony-stimulating factor on the incidence of threshold retinopathy of prematurity

Outcomes of cataract surgery following radiation treatment for retinoblastoma

The value of serial personal photographs in timing the onset of unilateral cataracts in children

Which Leber congenital amaurosis patients are eligible for gene therapy trials?

Accuracy of the Welch Allyn SureSight for measurement of magnitude of astigmatism in 3- to 7-year-old children

Results of extraocular muscle surgery for superior oblique myokymia

The psychosocial aspects of strabismus: correlation between the AS-20 and DAS59 quality-of-life questionnaires

Predictive factors of surgical outcome in oculomotor nerve palsy

Traumatic superior oblique tendon rupture

A child with bilateral orbital cellulitis one day after strabismus surgery

Acute traumatic orbital cerebrospinal fluid cystocele mimicking orbital abscess

Septo-optic dysplasia with bilateral congenital corneal anesthesia

Misleading clinical stereoacuity levels in pediatric maculopathy

Spontaneous resolution of retinal detachment in morning glory disk anomaly

Apnea and bradycardia in two premature infants during routine outpatient retinopathy of prematurity screening

Semiautomated digital image analysis of posterior pole vessels in retinopathy of prematurity

Image analysis of posterior pole vessels identifies type 1 retinopathy of prematurity

Longitudinal assessment of plus disease in retinopathy of prematurity using color Doppler imaging

Ocular manifestations of the Johanson-Blizzard syndrome

Juvenile xanthogranuloma masquerading as melanoma

Successful treatment of ligneous conjunctivitis with topical cyclosporine and heparin

A novel method for management of conjunctival inclusion cysts following strabismus surgery using isopropyl alcohol with paired injection technique

Resolution of recurrent conjunctival papilloma after topical and intralesional interferon α2b with partial excision in a child

Extraocular muscle hypertrophy in myotonia congenita: Mutation identified in the SCN4A gene (V445M)

Congenital Osteoma Cutis of the Lateral Canthus

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The critical period for surgical treatment of dense congenital bilateral cataracts

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Treatment of severe amblyopia with weekend atropine: Results from 2 randomized clinical trials

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Books Received

Errata

Editorial Board

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