George R. Beauchamp

Filtering Surgery in Children: Barriers to Success

The use of trabeculectomy in advanced pediatric glaucoma is reviewed. Intraocular pressure was controlled in only 50% of the patients, and the best visual results was 20/200. Complications of vitreous loss, scleral collapse, ectasia, retinal detachment, and endophthalmitis were encountered. No evidence was found to support the claim that in the seriously compromised buphthalmic eyes, trabeculectomy is safer than other filtering operations since it filters under a scleral flap.

Extent of resection in temporal lobectomy for epilepsy. II. Memory changes and neurologic complications

Summary: We present correlations of extent of temporal lobectomy for intractable epilepsy with postoperative memory changes (20 cases) and abnormalities of visual field and neurologic examination (45 cases). Postoperative magnetic resonance imaging (MRI) in the coronal plane was used to quantify anteroposterior extent of resection of various quadrants of the temporal lobe, using a 20‐compartment model of that structure. The Wechsler Memory Scale‐Revised (WMS‐R) was administered pre‐operatively and postoperatively. Postoperative decrease in percentage of retention of verbal material correlated with extent of medial resection of left temporal lobe, whereas decrease in percentage of retention of visual material correlated with extent of medial resection of right temporal lobe. These correlations approached but did not reach statistical significance. Extent of resection correlated significantly with the presence of visual field defect on perimetry testing but not with severity, denseness, or congruity of the defect. There was no correlation between postoperative dysphasia and extent of resection in any quadrant. Assessment of extent of resection after temporal lobectomy allows a rational interpretation of postoperative neurologic deficits in light of functional anatomy of the temporal lobe.

Ocular Findings Associated with Neurofibromatosis Type II

BACKGROUND Neurofibromatosis has been recently acknowledged as consisting of a number of different diseases. Neurofibromatosis (NF) type I and NF type II are the most clearly defined. Type II is characterized by bilateral acoustic neuromas and is rare (its incidence is 1/50,000). The previously reported ocular associations of NF type II are posterior subcapsular cataracts, Lisch nodules, and combined hamartomata of the retinal pigment epithelium and retina. In this study, the authors attempt to define further the ocular manifestations of NF type II. METHODS The authors prospectively examined 9 patients who met the diagnostic criteria for NF type II (age, 18 to 38 years; mean, 25 years). RESULTS Seven of nine patients had epiretinal membranes in the posterior pole. None of these epiretinal membranes were visually significant. In addition, five patients had central posterior cortical cataracts and five had peripheral wedge-shaped cortical cataracts. CONCLUSION The presence of epiretinal membranes in young patients may represent another clinical finding associated with NF type II. Epiretinal membranes, central posterior cataracts, peripheral cortical cataracts, or combined hamartoma of the retinal epithelium and retina in young patients should alert the ophthalmologist to include NF type II in the differential diagnosis in patients with stigmata of NF type II.

Role of the Neural Crest in Anterior Segment Development and Disease

Certain eye and associated systemic developmental anomalies are apparently related by virture of a common neural crest origin. The development of the anterior segment is extremely complex and is dependent upon the presence or absence of certain local factors (including extracellular matrices and glycoproteins), inductors, receptors, and specific time sequencing. Understanding anterior segment anomalies and their systemic associations requires an understanding of neural crest proliferation and migration patterns; and they may be unified under the designation of neurocristopathies. Goldenhars syndrome, not previously considered a neurocristopathy, may be considered one on the basis of the relationship between clinical findings and neural crest embryology.

Pediatric keratoplasty: problems in management.

A summary of experience in pediatric keratoplasty is given by adding ten recent cases to a previous report. A review of keratoplasty in infants and children from the literature generally recommends the procedure as safe and effective. Recent reports, however, cast some doubt on the effectiveness of the procedure for certain conditions. An update of experience is given as a focal point for a discussion of problems in clinical management of children who undergo keratoplasty. These problems are arbitrarily separated by pre-, intra-, and postoperative time frames.

Screening for amblyopia in preverbal children with photoscreening photographs: II. Sensitivity and specificity of the MTI photoscreener.

OBJECTIVE To determine the sensitivity and specificity of vision screening using the Medical Technology and Innovations (MTI), Inc., PhotoScreener. DESIGN Cross-sectional study. PARTICIPANTS AND TESTING: Three hundred ninety-two children less than 4 years of age received a complete ophthalmologic examination and were photographed using the MTI PhotoScreener. One hundred three children had normal examinations, and the remaining 284 children had conditions of interest for pediatric screening: ptosis, media opacity, refractive error, or strabismus. Five children were excluded. MAIN OUTCOME MEASURES The grading of the photographs by the manufacturers representative was compared with the results of the ophthalmologic examinations. Sensitivity and specificity of vision screening were determined. RESULTS The analysis of all informative photographs resulted in a sensitivity of 65% and a specificity of 87%. The sensitivity of detection for children with some forms of strabismus was high, up to 95% for esotropia of 10Delta or more. Sensitivities for the detection of ptosis, media opacity, and refractive error were poor in patients where strabismus was not also present. CONCLUSIONS The MTI PhotoScreener may play a role in preverbal vision screening; identification of two of three children with amblyopiogenic factors before age 4 would be an exciting advance in public health. However, improvement in the ability to identify children with media opacity and refractive error is necessary. Improvements may be possible with modifications of the examination failure and photograph grading criteria.

Terrien's Marginal Corneal Degeneration

Terriens disease is an uncommon (Terrien himself saw only three cases in thirty years of practice) corneal condition characterized by vascularization, opacification, lipid deposition, and thinning. With progression, large degrees of astigmatism occur and perforation is a threat. One-third of cases exhibit an inflammatory component. While typically described as a disease of middle to late age, these four cases indicate the full spectrum of clinical disease may be seen by an ophthalmologist who cares for children.

Epikeratophakia in children with traumatic cataracts.

Epikeratophakia provides a permanent optical correction for aphakia in children with congenital or traumatic cataracts; suturing the epikeratophakia graft onto the cornea eliminates the problems of contact lens or spectacle non-compliance in these young and generally uncooperative patients and provides tectonic support to scarred and irregular corneas. Eighteen children under the age of six years underwent epikeratophakia for the correction of aphakia after the removal of trauma-induced cataracts. Graft success rate was 88%; the average change in keratometry in the patients with successful grafts was 14.82 +/- 2.0 diopters. In the 13 patients eligible for visual acuity tabulation, preoperative acuities ranged from light perception to 20/200, and postoperative acuities ranged from hand motions to 20/30. Ten (77%) had acuities of 20/80 or better. Poor results in three patients with less than 20/200 acuities were likely the results of non-compliance with amblyopia therapy. Present work indicates that in cases of traumatic cataract, the epikeratophakia procedure facilitates amblyopia therapy and decreases the astigmatism in scarred and irregular corneas.

Brown's Syndrome in Twins

Monozygotic twin girls concordant for Browns syndrome with reversed asymmetry are presented. This appears to be the first report of concordance for the presence of Browns syndrome in twins. Embryological factors and a possible explanation for the etiology of this condition in the form as presented are discussed.

Photophobia, Epiphora, and Torticollis: A Masquerade Syndrome

Three young children presented with photophobia, epiphora, and torticollis as the initial manifestation of a posterior fossa tumor. In each case there was a delay in treatment due to the presumptive diagnosis of a local ocular inflammatory condition. We recommend that children with unexplained photophobia, epiphora, and torticollis undergo an imaging technique to evaluate the posterior fossa.