Mark J. Greenwald

Natural history of optic pathway tumors in children with neurofibromatosis type 1: A longitudinal study

To assess the natural history of optic pathway tumors (OPT) in children with neurofibromatosis type 1 (NF-1), from January 1985 through May 1993 we performed a prospective, longitudinal study of OPT in an unselected population of children with NF-1. Of 227 children with NF-1 seen in a specialty clinic, 176 (77%) underwent neuroimaging. Children in whom tumors were identified were followed closely by both repeated neuroimaging and ophthalmologic examinations to detect tumor growth or visual deterioration. Thirty-three children (19%) were found to have OPT at a median age of 4.2 years. The median age of children who had ophthalmologic complaints was significantly lower than that of children who had no such complaints (1.9 vs 5.3 years; p < 0.001). Although eight tumors were discovered because of ophthalmologic complaints or evidence of precocious puberty, 25 children (76%) were free of symptoms at the time of diagnosis. Twenty-one children (64%) had normal ophthalmologic findings at diagnosis; six children, all with chiasmal tumors, had previously unrecognized decreased visual acuity. Only three children (9%) had evidence of either tumor growth or deteriorating vision after diagnosis; the median duration of neuroimaging follow-up was 2.4 years (range, 0.2 to 7.2 years) and of ophthalmologic examinations 3.4 years (range, 0.2 to 8.1 years). All symptomatic OPT were diagnosed before 6 years of age. We conclude that OPT rarely progress during the next few years in children with NF-1 once the OPT have been discovered. The utility of screening neuroimaging for OPT in symptom-free children with NF-1 appears very limited.

Metastatic bacterial endophthalmitis: a contemporary reappraisal

Metastatic bacterial endophthalmitis remains a challenge to the clinician despite the success of antibiotics in reducing its frequency and severity. Controversy currently surrounds the management of this condition because of uncertainty about the value of and indications for vitreous surgery. We review 72 cases of metastatic endophthalmitis from the past decade, including five not previously published. The spectrum of causative bacteria changed significantly during this period, with displacement of meningococcus by Bacillus cereus as the most frequently reported agent and an increasing incidence of infection by organisms of low pathogenicity in immunologically compromised hosts. We propose a new classification scheme for metastatic endophthalmitis based on the location (anterior or posterior segment) and extent (focal or diffuse) of the primary intraocular infection. Focal and anterior cases appear to have a good prognosis, while posterior diffuse disease nearly always leads to blindness. Our analysis of outcomes suggests that systemic antibiotics are more valuable in metastatic than in postoperative or traumatic endophthalmitis and that intraocular antibiotic injection and vitrectomy make only a limited contribution to successful treatment in metastatic infection. We recommend a clinical approach to metastatic endophthalmitis that minimizes exposure of patients to the risks of invasive procedures.

Optic gliomas in children with neurofibromatosis type 1

To determine the frequency and natural history of tumors of the optic nerves and chiasm in patients with neurofibromatosis type 1, we obtained computed tomographic scans of 65 children who had no known visual or ocular abnormalities before their initial evaluation. Optic gliomas were detected in 10 children (15%). The median age of children with gliomas was 4.3 years (mean 5.8 years, range 9 months to 21 years). Three children (30%) had isolated, unilateral tumors, three (30%) had bilateral tumors, and four (40%) had involvement of the optic chiasm and of one or both nerves. Definite abnormalities of vision were found in only two children (20%). Five additional children were referred to the clinic after evaluation of ophthalmologic complaints led to the diagnosis of neurofibromatosis type 1: three had unilateral exophthalmos and two had plexiform neurofibromas of the eyelid with associated glaucoma. Ipsilateral optic gliomas were found in all five children; one child also had a contralateral tumor. Optic gliomas are commonly identified in young children with neurofibromatosis type 1 who have no ocular or visual abnormalities. Optic nerve gliomas may be associated with plexiform neurofibromas of the eyelid and glaucoma.

Optic pathway tumors in children: The effect of neurofibromatosis type 1 on clinical manifestations and natural history

OBJECTIVE To distinguish the clinical manifestations and natural history of optic pathway tumors (OPT) associated with neurofibromatosis type 1 (NF-1 OPT) from that of OPT not associated with NF-1 (non-NF-1 OPT). METHODS Two groups of children with OPT were compared: (1) 17 children with NF-1 OPT who were followed prospectively, and (2) 19 children with non-NF-1 OPT who were identified retrospectively by a review of medical records. RESULTS Precocious puberty was a common initial sign in the children with NF-1 OPT (5/17), and was not found in any patients without NF-1. In contrast, children with non-NF-1 OPT had symptoms attributable to increased intracranial pressure (12/19 and nystagmus (5/19); these symptoms were not found in any patient with NF-1. Decreased visual acuity at the time of diagnosis was common in both groups. There was no significant difference between the children with NF-1 OPT and those with non-NF-1 OPT as to age at diagnosis or sex distribution. Optic nerve involvement was more common in NF-1 (p < 0.001). Both isolated and bilateral optic nerve tumors were found exclusively in children with NF-1, whereas chiasmal (p = 0.016) and optic tract involvement (p = 0.001) were more common in those with non-NF-1 OPT. Radiographic evidence of hydrocephalus was found in none of the children with NF-1 OPT compared with 79% of the non-NF-1 OPT group. Progressive disease was seen in 12% of patients with NF-1 OPT compared with 63% of those with non-NF-1 OPT. CONCLUSIONS Differences exist between NF-1 OPT and non-NF-1 OPT both at the time of diagnosis and during follow-up. Optic pathway tumors caused by NF-1 and non-NF-1 OPT have different biologic properties that distinguish both their initial clinical manifestations and their natural history.

Traumatic Retinoschisis in Battered Babies

Five infants who were victims of physical abuse had extensive bilateral retinal hemorrhages on initial evaluation and subsequently developed signs of permanent retinal damage. None showed external evidence of trauma to the eyes. Vitreous hemorrhage developed after a delay of several days or more in three cases that were followed closely from the time of the traumatic incident. In several eyes, apparent intraretinal blood-filled cavities were seen acutely in the macular region and elsewhere. Late scarring of the macula typically had a cystic or crater-like configuration. Electroretinography showed loss or reduction of the positive B-wave with preservation of the negative A-wave in every case. We propose that splitting of the retina resulting from the direct mechanical effects of violent shaking was responsible for all of these findings.

Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children.

A retrospective analysis of 21 consecutive patients hospitalized with either Stevens-Johnson syndrome or toxic epidermal necrolysis was carried out to assess morbidity and mortality rates and to establish the value of a specific management practice. Fourteen children with Stevens-Johnson syndrome and seven with toxic epidermal necrolysis were cared for at the Childrens Memorial Hospital, Chicago, between 1978 and 1988. All were managed in a well-staffed medical ward or, when necessary, in the pediatric intensive care unit. Supportive measures included reverse barrier isolation, intravenous fluids and nutritional support, meticulous skin care, early detection and treatment of infection, and daily ophthalmologic examination. No patient was treated with systemic steroids. The mortality rate was zero. Eye complications, consisting of dry eyes or mild chronic symblepharon, were the most significant long-term sequelae.

Treatment of Intraocular Retinoblastoma with Carboplatin and Etoposide Chemotherapy

PURPOSE Management of intraocular retinoblastoma was initiated with 2-drug chemotherapy in an effort to improve the rate of vision preservation and ocular salvage and to avoid or delay the use of external beam radiation treatment. METHODS Six patients with intraocular retinoblastoma (five bilateral; one unilateral, 1 month old) received 6 to 7 monthly cycles of intravenously administered carboplatin and etoposide (VP-16) as primary treatment. No eyes were enucleated primarily. Twelve of the 33 discrete tumors in the 11 study eyes received prophylactic supplemental treatment with cryotherapy or laser hyperthermia. Response was documented with frequent eye examinations with the patient under general anesthesia and with repeated fundus photography. RESULTS All eight larger tumors (> 10-mm diameter) underwent dramatic regression after treatment with chemotherapy alone, and six of these tumors ultimately became fully calcific. One larger tumor and two smaller tumors showed post-treatment growth, each within 2 months after completion of chemotherapy. Six larger tumors were observed without growth or further treatment for 7 to 21 months after completion of chemotherapy. Subretinal fluid resorbed completely in four of four eyes with extensive retinal detachment, and vitreous seeding diminished considerably in four of four eyes. In five eyes, intraocular disease recurrence at a distance from any initially observed tumor eventually required treatment with external beam radiation (three eyes) or enucleation (three eyes). Eight of 11 involved eyes were salvaged, including 5 of 8 with larger tumors and 4 of 4 with vitreous seeding; 4 retained eyes received no radiation exposure, including 3 with larger tumors and 1 with vitreous seeding. Good vision was preserved in six eyes, two of which were markedly improved after occlusion therapy for amblyopia. There was no extraocular disease recurrence and no serious harm from treatment during observation ranging from 12 to 40 months after diagnosis. CONCLUSION Chemotherapy with carboplatin and etoposide shows promise as initial treatment for intraocular retinoblastoma. Further study is indicated to define its proper role in the management of this disease.

Orbital roof fractures in the pediatric population.

Twenty-three patients aged 3.3 ± 1.6 years (mean ± SD) presented between January of 1984 and September of 1987 with fronto-orbital trauma resulting in fractures of one (N = 20) or both (N = 3) orbital roofs. All patients had computed tomography (CT) with axial and coronal sections that revealed three fracture patterns of the orbital roof (nondisplaced, superiorly displaced, and infe-riorly displaced fractures). Orbital dystopia was exhibited in 35 percent (N = 8) of the patients. Exophthalmos was noted in 61 percent (N = 14) of the patients. Only 30 percent of the patients (N = 7) sustained associated maxillofacial fractures. Eight percent of fractures exhibited orbital encephaloceles. All patients lacked frontal sinus pneumatization. The majority of children with orbital roof fractures do not exhibit concomitant facial fractures. CT utilizing both axial and coronal sections is valuable in defining the extent and pattern of the fracture as well as in identifying associated neurologic injuries. Large, displaced orbital roof fractures, which occurred in 3 of 13 patients with displaced fractures in our series, should undergo early reduction to avoid late development of encephalocele.

Expanded Binocular Peripheral Visual Fields Following Surgery for Esotropia

We measured the horizontal extent of the binocular peripheral field of vision in ten adult esotropes using Goldmann perimetry before and after strabismus surgery. In every case there was expansion of the field by an amount approximately commensurate with the change in angle of strabismic deviation. This occurred regardless of the presence of amblyopia or recovery of binocular fusion. Subjective improvement in peripheral vision was appreciated by a number of patients. Visual field expansion should be recognized as a significant indication for correction of esotropia.

When may the posterior capsule be preserved in pediatric intraocular lens surgery

PURPOSE To refine indications for primary posterior capsulotomy (PPC) in conjunction with posterior chamber intraocular lens (PCIOL) implantation for cataract in childhood. DESIGN Noncomparative case series. PARTICIPANTS Patients 1 to 13 years old who underwent cataract extraction with intent to preserve the posterior lens capsule and PCIOL implantation between January 1992 and December 1998 at a pediatric hospital. METHODS Medical records were reviewed to determine the frequency and timing of posterior capsule opacification (PCO) after PCIOL surgery with preservation of an intact posterior capsule. Comparison of pseudophakic PCO rates for groups defined by age and several possible risk factors. Assessment of safety and efficacy for PPC with anterior vitrectomy performed through a limbal incision in cases where the posterior capsule could not be preserved. MAIN OUTCOME MEASURES Need for neodymium:yttrium-aluminum-garnet laser capsulotomy or surgical membranectomy to treat PCO. RESULTS PCO occurred in 40% of 30 eyes with intact posterior capsule. Mean follow-up duration was 22 months for eyes that had PCO develop and 24 months for those in which the posterior capsule remained clear. Laser capsulotomy was required for 64% of 14 eyes in the 1- to 6-year-old age range but for only 19% of 16 in the 6- to 13-year-old range (P < 0.05). Mean time from surgery to PCO was 7 months for the younger group and 13 months for the older group. A need for repeated capsulotomy (one eye) or membranectomy with anterior vitrectomy (two eyes) was found only in the younger age group. There was no association of PCO with trauma history, cataract type, residual lens cortex, IOL position, or postoperative fibrin clot. Final vision was possibly compromised as a result of PCO in one eye with amblyopia. None of 24 eyes in which PPC with anterior vitrectomy was performed out of intraoperative necessity before primary PCIOL implantation had secondary opacification develop. No reduction in postoperative vision was attributable to PPC. CONCLUSIONS PPC seems to be advisable for children less than 6 years old when cataract extraction with PCIOL implantation is performed. Preservation of the posterior capsule remains appropriate for older children with pseudophakia.