[Ocular malformations (microphthalmos, bandelette keratitis, cataract, optic subatrophy and cicatricial chorioretinitis) in a case of congenital toxoplasmosis].
Explore the source record for details and available documents.
Cataract↗
SEARCH · Search PubMed
Results for “MICROPHTHALMOS”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Visual acuity in children with coloboma: clinical features and a new phenotypic classification system.
OBJECTIVE: The aims of this study were to describe the clinical features and biometric findings in the eyes of children with coloboma and to develop a classification of coloboma that correlates with visual function. DESIGN: Retrospective observational case series. PARTICIPANTS: One hundred thirteen children and young adults (48 female, 65 male) aged 0 to 20 years with 196 eyes having coloboma. METHODS: Children with coloboma were recruited from schools for the blind, integrated education programs, schools for the mentally handicapped, community-based rehabilitation services, and hospital clinics in Andhra Pradesh, India, between January 1998 and January 1999. Visual function was assessed, including distance and near visual acuity (VA), and navigational vision. The corneal diameter and axial length of eyes were measured wherever possible. MAIN OUTCOME MEASURES: Anatomic site of coloboma, association with microcornea and/or microphthalmos, VA, presence of navigational vision and reading vision. RESULTS: Of 196 eyes with colobomatous malformations, 11 had microphthalmos with cyst, and 185 eyes had coloboma (associated with microcornea in 155 eyes and with a normal corneal diameter in 30). Microphthalmos was present in 72 of the 185 eyes with coloboma, of which 71 of 72 also had microcornea. The prognosis for vision depended on the phenotype of the better eye. Microphthalmos with cyst had the worst prognosis (VA < 3/60, 100%; reading and navigational vision, 0%). Microcornea with microphthalmos had a worse prognosis than microcornea without microphthalmos (VA < 3/60: 66.7% vs. 23.3%; unable to read N10: 66.7% vs. 34.1 %; no navigational vision: 30.6% vs. 6.73%). Simple coloboma (no microcornea or microphthalmos) had the best prognosis (VA < 3/60: 6.7%; able to read N10: 93.3%; navigational vision: 100%). A corneal diameter <6 mm had a poor visual prognosis, whereas a corneal diameter >10 mm had a good prognosis. CONCLUSIONS: A phenotypic classification of coloboma is proposed, which in this study showed a good correlation with visual acuity, reading, and navigational vision. Microphthalmos with cyst had the worst prognosis, coloboma with microcornea and microphthalmos a poor prognosis, coloboma with only microcornea had an intermediate prognosis, and simple coloboma had the best prognosis.
Adolescent↗
[Familial nanophthalmos].
Microphthalmos is a rare, potentially devastating condition. Catsch found 30 cases of microphthalmos in a population of 26,735 (0.11%); Scouras et al. among 120,000 ophthalmic out-patients found 70 cases of microphthalmos (0.058%); among 3,557 blind adults Lindstedt found 63 cases (1.77%) and Kissel et al. among 210,000 ophthalmic out-patients found 97 cases (0.046%). Congenital microphthalmos may be: colobomatous, complicated, pure. Pure microphthalmos or nanophthalmos is a rare condition in which the eye is reduced in size with a notably high ratio of the lens volume to eye volume, but no other congenital anomalies are present. The sclera is abnormally thick. Nanophthalmos may be sporadic or hereditary: hereditary transmission may be either recessive or dominant. These eyes are anatomically predisposition to angle-closure glaucoma and occasionally associated with uveal effusion. Angle-closure glaucoma probably is the result of the natural increase in the size of the lens with age; in addition, spontaneous choroidal detachment probably may cause elevation and forward rotation of the ciliary body pushing the lens-iris diaphragm forward, with increasing of the relative pupillary block. The uveal effusion probably is the result of choroidal congestion secondary to obstruction of vortex veins by abnormally thickened sclera. Uveal effusion may also occurs spontaneously in patients with nanophthalmos between the ages of 40 to 60 years. Surgical intervention with sudden decompression of the globe, appears to aggravate the degree of uveal effusion. Three cases of familial nanophthalmos associated with angle-closure glaucoma without uveal effusion not microcornea are reported. The occurrence of nanophthalmos in the same family suggests an autosomal recessive inheritance.(ABSTRACT TRUNCATED AT 250 WORDS)
Aged↗
Congenital cystic eye: report of two cases and review of the literature.
A 13-month-old boy and a 2-week-old girl, who were considered to be anophthalmic and who later each developed a cystic lesion in the left orbit with protrusion of the lower eyelid, were studied. The fellow eye in case 1 was subsequently found to be microphthalmic with cyst and was normal in case 2. Histopathologic study of each case revealed a cyst lined externally by dense fibrous connective tissue to which skeletal muscle and adipose tissue were attached. The inner aspect of the cyst was lined by neuroglial tissue, possible immature retinal tissue, and cuboidal epithelium. No fully developed ocular structures or microphthalmos were identified. Fourteen cases of congenital cystic eye, including our cases, have been published in the English-language literature since 1964. We discuss and illustrate the findings in our cases and 10 others in which histopathologic findings were reported. Congenital cystic eye, microphthalmos with cyst, and microphthalmos with cystic teratoma should be suspected in patients with a small or unrecognizable eye and an orbital cystic mass that is detected by palpation or visualization.
Anophthalmos↗
[Developmental eye abnormalities in mouse fetuses induced by retinoic acid].
To clarify the relationship between neural crest cells and ocular anomalies, pregnant mice were treated intraperitoneally with 12.5 mg/kg retinoic acid suspended in corn oil on day 7 of pregnancy (RA group). Control mice were given an equal volume of corn oil (control group). Each group consisted of 5 mother mice, and the offsprings were removed on day 18 of gestation. The fetal mortality was 46.3% in the RA group and 2.2% in the control group. Twenty-two live fetuses of the RA group and 45 of the control group were grossly observed, and the eyes were examined histologically. In the RA group, gross malformations such as microphthalmos (95.5%), cleft lip and palate (36.4%), and central nervous system anomalies (31.8%) were observed, and in the control group, malformations such as microphthalmos (6.7%), central nervous system anomalies (2.2%), and low set ears (2.2%) were seen. Histological examination revealed microphthalmos (47.7%), anophthalmos (38.6%), faulty closure of the embryonic fissure (36.4%), developmental abnormalities of the vitreous (34.1%), aphakia (22.7%), goniodysgenesis (18.2%), and faulty separation of the lens vesicle (15.9%) in the RA group. These anomalies arose from abnormal neural crest cell migration induced by retinoic acid. They were detected in only 3.3, 1.1, 3.3, 8.9, 1.1, 2.2 and 2.2%, respectively of the control group.
Animals↗
Surgical management of the anophthalmic orbit, part 1: congenital.
Congenital microphthalmos and anophthalmos are rare conditions in which orbital growth is deficient. Hypoplasia of the globe affects the bony orbit (micro-orbitism), the conjunctival sac, and eyelids (microblepharism), and it may be associated with abnormalities of the entire hemifacial skeleton (hemifacial microsomia). In the present article, the authors review a series of 19 patients with microphthalmos (nine had right-sided, one had bilateral, and nine had left-sided microphthalmos) who were treated in the Orbitopalpebral Unit at Hospital Foch over a period of 15 years (follow-up, 5 months to 18 years).Orbital expansion was achieved using spherical implants (n = 13), orbital osteotomies (n = 4), and orbital expanders (n = 2). Both expanders were removed within 6 months because of failure (one infection and one rupture). The current preferred method for orbital expansion is to use serial implants in the growing orbit and osteotomies in cases of late referral or insufficient orbital volume in the older child. The target proportions of the reconstructed orbit are not planned to mirror the healthy side exactly. The inferior orbital rim is kept higher to support the orbital implant, and the orbit is kept shallow to avoid a sunken appearance. Cranial bone grafts were used to augment deficient orbital contours; they were assisted by anterior transposition of the temporalis muscle (n = 5) when additional orbital volume was required. Conjunctival sac reconstruction was achieved by the use of serial conformers placed in the conjunctival sac during the neonatal period, followed by grafts of buccal mucosa and full-thickness skin maintained in place with a tarsorrhaphy for 3 to 6 months. Eyelid reconstruction using local flaps and skin grafts proved to be necessary in cases treated by osteotomy expansion, although reconstruction was not required after expansion using serial solid shapes. The results illustrate an evolution in approach and concepts of reconstruction of the microphthalmic orbit and emphasize the need for an integrated craniofacial approach for this complex deformity.
Anophthalmos↗
Congenital retinal disinsertion syndrome.
The congenital retinal disinsertion (CRD) syndrome refers to cases of retinal detachment with disinsertion and may be divided into two groups. Group 1 reported by Hovland and co-workers includes healthy children with bilateral detachments and giant tears nasally, lens coloboma, and center anterior and posterior cortical lens opacities. Group 2 includes healthy children with a unilateral detachment often associated with microphthalmos and catatract. The opposite eye may show a combination of changes including small central anterior and posterior cortical lens opacities, lens colobomas, and paving-stone degeneration. The patients may have an increased risk of developing a detachment in the second eye, and some of these eyes have been treated with cryopexy. Seven patients belonging to group 2 have been described and four of these had unilateral cataract and microphthalmos. Two other patients had some degree of unilateral microphthalmos. Small central anterior or posterior cortical lens opacities were found in five eyes, and lens colobomas were found in two eyes. Six out of seven patients had varying degrees of paving-stone degeneration temporally in the nondetached eye. The CRD syndrome was familial in two patients who were sisters. Pathologic studies were done in the eyes of three patients.
Child↗
[Critical time for developmental eye abnormalities induced by retinoic acid in mouse fetuses].
To identify the critical time for developmental eye abnormalities, pregnant C 57 BL/6 NJcl mice were injected intraperitoneally once with 12.5 mg/kg of retinoic acid on days 7 (day-7 group), 8 (day-8 group), 9 (day-9 group), 10 (day-10 group), and 11 (day-11 group) of pregnancy. Each group consisted of 5 pregnant mice. The fetuses were observed grossly on day 18 of gestation, and the eyes were examined histologically in serial sections. Various gross malformations such as microphthalmos, cleft lip and palate, and central nervous system anomalies were observed in the day-7 group. However, microphthalmos was the only gross malformation found in the day-8 group, and there were no gross malformations in the other 3 groups. The histological examination indicated that the critical time for anophthalmos was day 7 of gestation or earlier because of its appearance in the day-7 group alone. The critical time for microphthalmos, faulty closure of the embryonic fissure, aphakia, and faulty separation of the lens vesicle was day 8 of gestation or earlier considering their occurrence in both day 7 and day 8 groups, and the time for goniodysgenesis was day 9 of gestation or earlier because of its appearance in the day 7, day 8, and day 9 groups.
Animals↗
Glaucoma secondary to pars plana lensectomy for congenital cataract.
BACKGROUND: To identify risk factors related to the onset of secondary glaucoma in eyes that have undergone pars plana lensectomy for congenital cataract. METHODS: We investigated intraocular pressure levels after pars plana lensectomy for congenital cataract in 80 eyes of 44 patients who were followed up for at least 36 months. Mean age at the time of pars plana lensectomy was 11+/-18 months. Mean follow-up period was 116+/-57 months. RESULTS: An elevation in intraocular pressure (>20 mmHg) was found in 21 eyes of 12 patients (26%). In 7 of these 21 eyes surgical treatment was required. The incidence of the coexistence of microcornea (or microphthalmos) with congenital cataract was significantly higher in the glaucoma group than that in nonglaucoma group. The final visual acuity in the nonglaucoma group was significantly better than that in the glaucoma group. CONCLUSION: Our results show the importance of careful follow-up of intraocular pressure after pars plana lensectomy for congenital cataract, especially when microcornea (or microphthalmos) is present.
Aphakia, Postcataract↗
Visual results after surgery for monocular juvenile cataracts of undetermined onset.
Seventeen of 216 operations I performed for juvenile cataract were in patients between 1 and 51/4 years of age who had monocular cataract of unknown origin and unknown time of onset. In 14 of them final visual acuities were 20/50 or better. In an additional eight children in whom the age at onset of the cataract could not be determined with certainty, but in whom microphthalmos suggested that it was congenital, the visual results were uniformly poor. Surgery for a monocular cataract of undetermined onset in a young child without microphthalmos may carry an excellent prognosis.
Adolescent↗
Persistent fetal vasculature and minimal fetal vascular remnants: a frequent cause of unilateral congenital cataracts.
PURPOSE: To determine the significance of persistent fetal vasculature (PFV) and remnants of fetal vessels in the pathogenesis of pediatric unilateral cataracts. STUDY DESIGN: Prospective observational case series. PARTICIPANTS: Thirty-one children with unilateral cataract aged between 2 weeks and 15 years. METHODS: As part of an ongoing prospective clinical trial concerning treatment and etiology of pediatric cataracts, a subgroup of 31 children with unilateral cataracts was defined. The affected eyes received preoperative and intraoperative biomicroscopic examinations to identify characteristic features of PFV and even minimal fetal vascular remnants (MFVRs) at the level of the posterior lens capsule and anterior hyaloid face. In eyes with MFVRs, 3 different severity degrees were assumed, according to different posterior capsule abnormalities: mild, A; moderate, B; and severe, C. All observations were documented on video and analyzed in relation to age (group I, infants between 0 and 1.5 years; group II, preschool children between 1.6 and 5.9 years; group III, schoolchildren between 6 and 16 years). MAIN OUTCOME MEASURES: Frequency and morphology of characteristic features of PFV and MFVRs of the posterior lens capsule/anterior hyaloid face, lens clouding, and microphthalmos. RESULTS: All 31 eyes with unilateral congenital cataracts showed signs of PFV syndrome (100%). Characteristic features of PFV were found in 75% of group I eyes, in 8% of group II eyes, and in 67% of group III eyes. Minimal fetal vascular remnants were found in 25% of group I eyes (severity degree C in all eyes), in 92% of group II eyes (severity degree A in 36.4%, B in 27.2%, and C in 36.4%), and in 33% of group III eyes (severity degree A). Associated microphthalmos was found in all eyes in groups I and III and in 73% of group II, whereas axial lengths were equal in both eyes in 27% of group II children with MFVRs. CONCLUSIONS: Varying degrees of PFV seem to be a frequent cause of unilateral congenital cataracts. Although characteristic features of PFV occurred mainly in infants, eyes of preschool children were usually very mildly affected, showing MFVRs that were detected only by careful observation during surgery. Abnormalities of the central part of the posterior capsule, such as a translucent opacity or a lenticonic area leading to a spontaneous hole during lens aspiration, may be caused by minimal remnants of PFV.
Adolescent↗
Ocular features of the congenital cataracts facial dysmorphism neuropathy syndrome.
OBJECTIVE: To determine the nature and course of ophthalmologic abnormalities in congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome in a genetically verified group of 9 patients. STUDY DESIGN: Observational case series. PARTICIPANTS: Nine affected male individuals of 5 pedigrees aged 1.3 to 16.8 years were examined. Four individuals were recruited during an ongoing prospective study of congenital cataracts; 5 individuals could be assigned to the CCFDN group on the basis of our retrospective data. MAIN OUTCOME MEASURES: Linkage and haplotype analysis, neurologic examinations, bilateral cataracts, axial length, corneal diameter, pupil diameter and pupillary reactions, intraoperative and postoperative complications, lid changes, aphakic correction problems, refractive results, and visual function. RESULTS: All families originated from the eastern part of Serbia, close to the border with Romania. The 8 tested individuals were homozygous for the conserved ancestral CCFDN haplotype in the telomeric region of chromosome 18q. All patients showed a peripheral, demyelinating neuropathy and varying degrees of ataxia. In the older patients, muscular atrophy in distal muscles and facial dysmorphism was evident. Early-onset bilateral congenital cataracts associated with microcornea, microphthalmos, and micropupil could be found in all patients. All children had floppy eyelid syndrome and pseudoptosis. An increased inflammatory reaction to contact lenses and intraocular lenses could be documented in all. All patients had syndrome-associated nystagmus and congenital esotropia. Distant visual acuity could be classified as severe to moderate impairment, whereas near visual acuity was much better (mild to moderate impairment). CONCLUSIONS: Early-onset congenital cataracts associated with microcornea, microphthalmos, and micropupil are essential ocular features of the CCFDN syndrome and are the first recognizable signs during early infancy. Awareness of this syndrome by pediatric ophthalmologists is important, because these typical findings, combined with information on ethnic origin, may lead to very early diagnosis at an age when the nature and severity of nonophthalmologic features are not apparent. Affected individuals may benefit from careful ophthalmologic treatment and follow-up, as well as from early management of the neurologic problems and developmental delay. Affected families will benefit from genetic counseling and predictive testing.
Adolescent↗
Developmental eye abnormalities in mouse fetuses induced by retinoic acid.
To clarify the relationship between neural crest cells and various developmental eye abnormalities, pregnant mice were administered an intraperitoneal injection of 12.5 mg/kg retinoic acid (RA) suspended in corn oil on day 7 of pregnancy (RA group). Control mice received an equal volume of corn oil only (control group). The fetuses were removed by laparotomy on day 18 of gestation. The fetal mortality was 46.3% in the RA group and 2.2% in the control group. The live fetuses in both groups were observed grossly, and the eyes were examined histologically in serial sections. In the RA group, gross malformations were observed, including microphthalmos (95.5%), cleft lip and palate (36.4%), and central nervous system anomalies (31.8%). In the control group, these malformations were seen in only 6.7%, 0%, and 2.2%, respectively. Histologic examinations in the RA group revealed microphthalmos (47.7%), anophthalmos (38.6%), faulty closure of the embryonic fissure (36.4%), developmental abnormalities of the vitreous (34.1%), aphakia (22.7%), goniodysgenesis (18.2%), and faulty separation of the lens vesicle (15.9%). They were detected in only 3.3%, 1.1%, 3.3%, 8.9%, 1.l%, 2.2%, and 2.2%, respectively, of the control group. These developmental eye abnormalities arose from abnormal migration of neural crest cells.
Abnormalities, Drug-Induced↗
Expansion of the human microphthalmic orbit.
OBJECTIVE: To determine the effects of long-term, incremental enlargement of an orbital tissue expander on bone and eyelid growth in microphthalmia. DESIGN: A prospective, noncomparative case series. PARTICIPANTS: Five consecutive patients with microphthalmos treated with orbital expansion were evaluated. INTERVENTION: A tissue expander was placed into the orbits of five children (age, 10 months-6 years) with unilateral microphthalmos and gradually enlarged by saline injections. MAIN OUTCOME MEASURE: The midorbital width of each patient was determined from axial computed tomographic scans before insertion of the device. The length of the normal and abnormal eyelid fissures was measured at surgery. The postexpansion dimensions of both the normal and microphthalmic orbits and the eyelids were remeasured when the expanders were removed. The residual deficits between the normal and the microphthalmic sides were expressed in percentages. RESULTS: Gradual inflation of the expander to a diameter of 22 mm reduced the average preoperative orbital dimension deficit of the group from 14.6% (range, 8%-25%) to 3.8% after surgery (range, 0.5%-6.3%). The average pre-expansion eyelid length deficit for the group was 17.5% (range, 12%-26%) compared to 2.3% (range, 0.0%-5.3%) after expansion. The average expansion period was 56.8 weeks (range, 20-100 weeks). Two outpatient surgical procedures were required in each patient. CONCLUSION: Incremental inflation of a tissue expander placed within the microphthalmic orbit induced sufficient osseous and eyelid growth to ameliorate the major stigmata of this syndrome in all patients treated.
Child↗
Intraocular lens implantation in microphthalmic patients.
Microphthalmos is a developmental disorder of the eye consisting of a smaller than normal eye. This disorder can present as an isolated condition or associated with other systemic alterations. It is not uncommon for patients with microphthalmos to have congenital cataracts along with other ocular and systemic abnormalities. This paper reports the experience with 11 microphthalmic eyes of seven patients who had primary or secondary intraocular lens (IOL) implantation over a six-year period from 1985 to 1991. In all cases the IOL had a 13.5 mm or 14.0 mm overall diameter and a 6.0 mm or 6.5 mm optic. It was difficult to obtain documentation of objective visual improvement in many of these cases because of the associated nystagmus. However, all patients reported subjective improvements. These results suggest that with proper technique and lens selection microphthalmic patients should be considered for IOL implantation with relative safety and success.
Adolescent↗
Bilateral microcornea and unilateral macrophthalmia resulting in incorrect intraocular lens selection.
A 79-year-old man with symmetrical microcornea and a dense unilateral nuclear sclerotic cataract had cataract extraction by phacoemulsification. The SRK/T formula suggested a 10.0 diopter (D) intraocular lens (IOL) for emmetropia (axial length 26.58 mm). The non-cataract eye required a 25.0 D IOL for emmetropia (axial length 21.51 mm). Biometric measurements were rechecked, and an 18.0 D IOL was implanted (axial length 24.02 mm). The 6 week postoperative refraction of -13.0 + 2.0 x 25 necessitated IOL exchange (10.0 D). Six weeks postexchange, the refraction was -3.75 + 2.5 x 30. This illustrates that symmetrical anterior microphthalmos does not always coexist with symmetrical posterior microphthalmos. Awareness of the association of symmetrical microcornea and unilateral colobomatous macrophthalmia may aid appropriate IOL selection in future cases.
Aged↗
Childhood blindness in Uzbekistan.
PURPOSE: To elucidate the aetiology of childhood blindness in the Republic of Uzbekistan and to assess the needs for future provision of ophthalmic services for children. METHODS: Six hundred and seventy-one children in seven schools for the blind and visually impaired throughout Uzbekistan were examined using the WHO/PBL (World Health Organization Prevention Of Blindness) childhood blindness proforma. The locations were chosen to give a representation of the major areas of population within the country. RESULTS: Of the 671 children examined, 506 (75.4%) were blind or severely visually impaired (corrected visual acuity of less than 6/60 (20/200) in the better eye). Cataract-related blindness (35%), retinal dystrophies (24%) and microphthalmos (23%) formed the three largest diagnostic categories. CONCLUSIONS: The commonest avoidable cause of blindness was found to be cataract; the cause of poor vision may be due to unoperated cataract, aphakia, amblyopia or post-operative capsular fibrosis. The high proportion of retinal dystrophies may be related to the common practice of consanguineous marriage. The frequent finding of microphthalmos is discussed and compared with findings from other surveys. Glaucoma accounted for approximately 5% of the avoidable blindness.
Adolescent↗
Congenital unilateral buphthalmos in Walker-Warburg syndrome: a clinicopathological study.
BACKGROUND AND PURPOSE: Walker-Warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. PATIENTS: Two neonates with Walker-Warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in Walker-Warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.
Abnormalities, Multiple↗