Vertical strabismus associated with plagiocephaly.
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Adolescent↗
Biomedical subjects
R M Robb
Publications and source records attributed to R M Robb.
Congenital glaucoma unresponsive to conventional therapy: a clinicopathological case presentation.
A clinicopathological case report of a black male with congenital glaucoma is presented. The child died at age three months of the Sudden Infant Death Syndrome. The clinical course was characterized by persistent corneal clouding and elevated intraocular pressure in spite of vigorous medical and surgical therapy. Histopathological findings included corneal enlargement, marked hypoplasia of the canal of Schlemm, poorly developed scleral spur, insertion of the ciliary musculature directly into the trabecular meshwork anterior to the scleral spur, and anterior displacement of the iris root. There was also anomalous dispersion of the sphincter muscle of the iris from the pupillary margin almost to the iris root, and attachment of some ciliary processes to the posterior surface of the iris. A poor clinical response to conventional glaucoma therapy, including trabeculotomy, goniotomy, trabeculectomy and medical therapy, supports the belief that the marked hypoplasia of Schlemm's canal was a major factor contributing to the elevated intraocular pressure. Furthermore, the multiplicity of abnormalities of anterior chamber angle structures suggests that some cases of congenital glaucoma may result from an early and extensive disorder in the formation of anterior segment tissues.
Autopsy↗
Treatment of congenital nasolacrimal system obstruction.
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Ambulatory Surgical Procedures↗
Results of early treatment of unilateral congenital cataracts.
Twelve infants with unilateral congenital cataracts treated at Boston Children's Hospital between 1978 and 1986 have now reached the age of 3.5 years or older. All infants had cataract extractions, aphakic contact lens fitting, and occlusion of the unaffected eye by 6 months of age. Patients with posterior lenticonus or persistent hyperplastic primary vitreous were excluded from this review. Early improvement of visual acuity in the aphakic eye was monitored by preferential looking tests, and occlusion of the sound eye was adjusted accordingly. E card visual acuities are now available on all 12 children. Five patients have 20/70 or better visual acuity, three patients have between 20/100 and 20/400 vision, and four patients have less than 20/400 in the aphakic eye. Two patients whose cataract surgery was done after 4 months of age had the poorest visual results. Difficulty maintaining occlusion therapy and interruptions of contact lens wear limited the development of better vision in some patients. The visual results to date in these 12 patients suggest that early aggressive treatment of unilateral congenital cataracts is worthy of consideration in most instances.
Cataract↗
The variable clinical characteristics and course of early infantile esotropia.
A study of 75 patients with early infantile esotropia treated by one of the authors (RMR) between 1965 and 1980 revealed a broad range of clinical findings and treatment modalities. The study group included nine children born prematurely and 16 with neurologic impairment; the balance had no apparent illness other than strabismus. Only 21 patients had spontaneously alternating fixation; the rest required some form of occlusion therapy. Glasses were prescribed for 75% of the patients sometime during their management, and changes in refractive error were common. One or more operations were performed on 56 patients, but 19 patients required no surgical intervention because of a spontaneous decrease in their deviation. Binocularity was found more frequently in patients straightened surgically before 2 years (50%) than in those straightened after that time (14%), but it was also present in five of the 19 patients who required no surgery. The presence of binocularity during the course of treatment did not insure stability of alignment.
Adolescent↗
Optic disc drusen in children.
Forty children with pseudopapilledema due to optic disc drusen (31 bilateral, nine unilateral) were studied retrospectively to characterize the early natural history of this condition. The average age at the first examination was 10.2 years (range 3.6 to 19.5 years), and the mean follow-up period was 44 months. Discrete hyaline bodies or papillary calcifications were first noted in one or both eyes with pseudopapilledema at a mean age of 12.1 years in 38 of 40 cases. Decreased visual acuity due to drusen occurred in only one eye due to subretinal neovascularization. Visual fields obtained in 35 eyes (21 patients) with pseudopapilledema showed an enlarged blind spot (9), an inferior arcuate/sector/or altitudinal defect (6), or both (3) in 18 eyes. Our study suggests that discrete papillary calcifications or hyaline bodies frequently emerge and visual field deficits are commonly detectable in the second decade of life in patients with pseudopapilledema due to optic disc drusen.
Adolescent↗
Assessment of vision and amblyopia by preferential looking tests after early surgery for unilateral congenital cataracts.
Preferential looking tests obtained over the first 3 years were used to evaluate the development of visual acuity of 21 patients who underwent surgery for unilateral congenital cataracts and fitting of contact lenses before age 6 months. Mean acuity of the aphakic eyes improved rapidly in the first year; however, between the end of the first year and the end of the third year there was no improvement in mean aphakic acuity. Consistent with this, the percentage of patients who were amblyopic by the criterion interocular difference in acuity (IOD) of 0.5 octaves (oct) or greater was 94% at the first test age near the onset of occlusion therapy, decreased to 72% at the end of year 1, and increased to 89% by the end of year 3. Variations in occlusion therapy appear to account for these results: mean hours of occlusion per day were 6.2 in year 1, 4.4 in year 2, and 2.7 hours in year 3. Furthermore, the IOD at age 1 year was negatively correlated with hours of occlusion in the first year and the IOD at age 3 years was negatively correlated with cumulated hours of occlusion over all 3 years. That is, the less the amount of occlusion therapy, the greater the IOD. The patients' ages at time of surgery did not account for any further variance in these results than that accounted for by occlusion therapy. This study demonstrates that PL acuity measurement can aid in monitoring the response to occlusion therapy of infants with deprivation amblyopia due to unilateral congenital cataracts.
Amblyopia↗
Optic disc drusen and primary megalencephaly in children.
Three patients with primary megalencephaly were identified among 40 children seen in our clinic with pseudopapilledema due to optic disc drusen. These patients were genetically unrelated and did not have cutaneous hemangiomas, distinguishing their condition from the Riley-Smith syndrome. Although the nature of the relationship between optic disc drusen and primary megalencephaly is not clear, we feel that the two conditions have more than a chance association. A clinically important consideration is that the concomitant occurrence of pseudopapilledema due to optic disc drusen and a large head due to primary megalencephaly may lead to an erroneous diagnosis of increased intracranial pressure in children.
Adolescent↗
Idiopathic superior oblique palsies in children.
The records of 110 patients with superior oblique palsy seen at the Boston Children's Hospital between 1966 and 1988 were reviewed. Patients with identifiable orbital or neurological disorders affecting the fourth cranial nerve or with a history of head trauma preceding their superior oblique palsy were excluded from analysis. Patients were also excluded because of prior surgery elsewhere or insufficient diagnostic information. The remaining 63 patients were considered to have idiopathic superior oblique palsies. All 63 patients presented with unilateral palsies. All had incomitant hypertropia of the affected eye, greater on gaze to the contralateral side and increased by ipsilateral head tilt. Left eyes were affected in 41 patients. The age at first ophthalmological examination was spread over the first 20 years, with heavier representation in the first 5 years and decreasing numbers in each succeeding 5-year interval. Only one patient was found to have a masked bilateral superior oblique palsy following surgery for an apparent unilateral palsy. A distinction is made between unmasking a bilateral superior oblique palsy and surgical overcorrection of a unilateral palsy. The predominant unilaterality of idiopathic superior oblique palsies is in contrast to the reported frequency of bilaterality in traumatic cases.
Adolescent↗
Ophthalmic manifestations of Setleis forceps marks syndrome: a case report.
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Abnormalities, Multiple↗
Ocular manifestations of deformational frontal plagiocephaly.
Frontal plagiocephaly can be caused by two mechanisms: craniosynostosis and external deformational pressure. Synostotic plagiocephaly is known to be associated with vertical strabismus and contralateral head tilt. Thirteen patients with deformational frontal plagiocephaly were examined to evaluate head position, ocular motility, and alignment. Nine of the patients (70%) were found to have ipsilateral torticollis, but only one patient had strabismus and this was a horizontal deviation not obviously associated with head position. The clinical features of synostotic and deformational plagiocephaly are discussed to distinguish the two conditions, which have a different treatment and outcome.
Child↗
Glaucoma following childhood cataract surgery.
Glaucoma has been recognized as an infrequent but serious complication following surgery for congenital and acquired cataracts in childhood. Little is known concerning the influence of factors on the risk of glaucoma such as age at surgery, type of cataract, associated ocular abnormalities, and type of surgery. We reviewed records of 240 eyes of 155 patients on whom cataract surgery had been performed between January 1965 and July 1990 at Children's Hospital, Boston. After excluding those patients who had been followed up less than 5 years after surgery, and those who had had surgery after the age of 10 years, 125 eyes of 82 patients were included in the study group. We identified 14 eyes of 9 patients that had developed open-angle glaucoma 5.3 to 13.1 years following surgery (average 7.4 years). An additional 4 eyes of 3 patients developed angle closure glaucoma, which was diagnosed at 146, 177, 2911, and 2939 days following surgery. A fifth patient developed acute angle closure 1.7 years following primary cataract surgery and 53 days following secondary discission. All but one of the patients who developed glaucoma had cataract surgery at less than 1 year of age. Age at surgery for the entire study group averaged 1.9 years, and ranged from 25 days to 9.6 years. We conclude that patients having cataract surgery before 1 year of age are at the greatest risk of developing postoperative open-angle glaucoma. Additional increased relative risk was found in eyes with microcornea, congenital rubella syndrome, and poor pupillary dilation with 1% cyclopentolate (Cyclogyl). No significant difference was seen among the various surgical methods of cataract removal.
Aphakia, Postcataract↗
Astigmatic refractive errors associated with limbal dermoids.
A retrospective review of 17 patients with limbal dermoids revealed that 13 (76%) had astigmatism of 1.00 diopter or greater in the involved eye. In all but one patient, the minus cylinder axis of the astigmatism coincided with the location of the dermoid. Thirteen patients had surgery to remove the dermoids at ages ranging from 8 months to 15 years. The preoperative astigmatism persisted postoperatively with little change in its orientation or amount regardless of age at the time of surgery.
Adolescent↗