Congenital microcephaly-linked CDK5RAP2 affects eye development.

Biallelic mutations in the cyclin-dependent kinase 5 regulatory subunit-associated protein 2 gene CDK5RAP2 cause autosomal recessive primary microcephaly type 3 (MCPH3). MCPH is characterized by intellectual disability and microcephaly at birth, classically without further organ involvement. Only recently, congenital cataracts were reported in four patients of one pedigree with MCPH3. Given the lack of a further pedigree with this phenotype, it remained unclear whether this was a true causal relationship. Here we support the link between CDK5RAP2 and eye development by showing that most Cdk5rap2 mutant mice (an/an) exhibit eye malformations ranging from reduced size of one or both eyes (microphthalmia) to total absence of both eyes (anophthalmia). We also detected increased apoptosis in the an/an retinal progenitor cells associated with more mitotic cells. This indicates an important role of Cdk5rap2 in physiologic eye development.

An application of data mining to identify potential risk factors for anophthalmia and microphthalmia.

BACKGROUND: We examined a large number of variables to generate new hypotheses regarding a wider range of risk factors for anophthalmia/microphthalmia using data mining. METHODS: Data were from the National Birth Defects Prevention Study, a multicentre, case-control study from 10 centres in the United States. There were 134 cases of "isolated" and 87 "nonisolated" (with other major birth defects) of anophthalmia/microphthalmia and 11 052 nonmalformed controls with delivery dates October 1997-December 2011. Using random forest, a data mining procedure, we compared the two case types with controls for 201 variables. Variables considered important ranked by random forest were included in a multivariable logistic regression model to estimate odds ratios and 95% confidence intervals. RESULTS: Predictors for isolated cases included paternal race/ethnicity, maternal intake of certain nutrients and foods, and childhood health problems in relatives. Using regression, inverse associations were observed with greater maternal education and with increasing intake of folate and potatoes. Odds were slightly higher with greater paternal education, for increased intake of carbohydrates and beans, and if relatives had a childhood health problem. For nonisolated cases, predictors included paternal race/ethnicity, maternal intake of certain nutrients, and smoking in the home the month before conception. Odds were higher for Hispanic fathers and smoking in the home and NSAID use the month before conception. CONCLUSIONS: Results appear to support previously hypothesised risk factors, socio-economic status, NSAID use, and inadequate folate intake, and potentially provide new areas such as passive smoking pre-pregnancy, and paternal education and ethnicity, to explore for further understanding of anophthalmia/microphthalmia.

Survey of Ocular Prosthetics Rehabilitation in the United Kingdom, Part 1: Anophthalmic Patients' Aetiology, Opinions, and Attitudes.

PURPOSE: Ocular prostheses are constructed to aid cosmetic, functional, and psychological rehabilitation of anophthalmic patients. Part-1 of this study aimed to evaluate anophthalmic patients' opinions, attitudes, and experience about aspects related to their postfit ocular prostheses. METHODS: One hundred sixty questionnaires were delivered to anophthalmic patients inquiring about different information such as age, gender, occupation, eye-loss cause, prosthesis type, prosthesis-wearing frequency, prosthesis-cleaning frequency, and problems encountered. A total of 126 questionnaires were returned (response rate was 78.8%). Data was analyzed using SPSS software (P <0.05). RESULTS: The patients were 74 males and 52 females (57.55 years ±â€Š17.57). Almost 50% of the patients lost their eye due to trauma that was the highest among other causes (P <0.05). High proportion clean their prosthesis daily (37.4%) which was the highest among other cleaning regimes (P <0.05). Almost 30.3% experienced having problems with their prosthetic eye. Patients who clean their prosthetic eye every 6 months have experienced more problems (P <0.05). Majority of patients wear their prosthetic eyes 24 hours (92%) (P <0.05). Half of patients who received a prosthetic eye for the first time experienced problems with it (P <0.05) such as excess discharge (45%), infection (25%), and soreness (20%). However, the problems were independent of prosthesis-type (P >0.05). CONCLUSIONS: Trauma is the most common cause of anophthalmic patients in the North-West of England. Anophthalmic patients are likely to experience problems with their prosthetic eye if they have lost their natural eye due to disease; it is their first prosthesis; or if they clean it once every 6 months.

Integrated versus non-integrated orbital implants for treating anophthalmic sockets.

BACKGROUND: Anophthalmia is the absence of one or both eyes, and it can be congenital (i.e. a birth defect) or acquired later in life. There are two main types of orbital implant: integrated, whereby the implant receives a blood supply from the body that allows for the integration of the prosthesis within the tissue; and non-integrated, where the implant remains separate. Despite the remarkable progress in anophthalmic socket reconstruction and in the development of various types of implants, there are still uncertainties about the real roles of integrated (hydroxyapatite (HA), porous polyethylene (PP), composites) and non-integrated (polymethylmethacrylate (PMMA)/acrylic and silicone) orbital implants in anophthalmic socket treatment. OBJECTIVES: To assess the effects of integrated versus non-integrated orbital implants for treating anophthalmic sockets. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register) (2016, Issue 7), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to August 2016), Embase (January 1980 to August 2016), Latin American and Caribbean Health Sciences Literature Database (LILACS) (1982 to August 2016), the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov), and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 8 August 2016. SELECTION CRITERIA: Randomised controlled trials (RCTs) and quasi-RCTs of integrated and non-integrated orbital implants for treating anophthalmic sockets. DATA COLLECTION AND ANALYSIS: Two authors independently selected relevant trials, assessed methodological quality and extracted data. MAIN RESULTS: We included three studies with a total of 284 participants (250 included in analysis). The studies were conducted in India, Iran and the Netherlands. The three studies were clinically heterogenous, comparing different materials and using different surgical techniques. None of the included studies used a peg (i.e. a fixing pin used to connect the implant to the prosthesis). In general the trials were poorly reported, and we judged them to be at unclear risk of bias.One trial compared HA using traditional enucleation versus alloplastic implantation using evisceration (N = 100). This trial was probably not masked. The second trial compared PP with scleral cap enucleation versus PMMA with either myoconjunctival or traditional enucleation (N = 150). Although participants were not masked, outcome assessors were. The last trial compared HA and acrylic using the enucleation technique (N = 34) but did not report comparative effectiveness data.In the trial comparing HA versus alloplastic implantation, there was no evidence of any difference between the two groups with respect to the proportion of successful procedures at one year (risk ratio (RR) 1.02, 95% confidence interval (CI) 0.95 to 1.09, N = 100, low-certainty evidence). People receiving HA had slightly worse horizontal implant mobility compared to the alloplastic group (mean difference (MD) -3.35 mm, 95% CI -4.08 to -2.62, very low-certainty evidence) and slightly worse vertical implant motility (MD -2.76 mm, 95% CI -3.45 to -2.07, very low-certainty evidence). As different techniques were used - enucleation versus evisceration - it is not clear whether these differences in implant motility can be attributed solely to the type of material. Investigators did not report adverse events.In the trial comparing PP versus PMMA, there was no evidence of any difference between the two groups with respect to the proportion of successful procedures at one year (RR 0.92, 95% CI 0.84 to 1.01, N = 150, low-certainty evidence). There was very low-certainty evidence of a difference in horizontal implant motility depending on whether PP was compared to PMMA with traditional enucleation (MD 1.96 mm, 95% CI 1.01 to 2.91) or PMMA with myoconjunctival enucleation (-0.57 mm, 95% CI -1.63 to 0.49). Similarly, for vertical implant motility, there was very low-certainty evidence of a difference in the comparison of PP to PMMA traditional (MD 3.12 mm 95% CI 2.36 to 3.88) but no evidence of a difference when comparing PP to PMMA myoconjunctival (MD -0.20 mm 95% CI -1.28 to 0.88). Four people in the PP group (total N = 50) experienced adverse events (i.e. exposures) compared to 6/100 in the PMMA groups (RR 17.82, 95% CI 0.98 to 324.67, N = 150, very low-certainty evidence).None of the studies reported socket sphere size, cosmetic effect or quality of life measures. AUTHORS' CONCLUSIONS: Current very low-certainty evidence from three small published randomised controlled trials did not provide sufficient evidence to assess the effect of integrated and non-integrated material orbital implants for treating anophthalmic sockets. This review underlines the need to conduct further well-designed trials in this field.

[Clinical and anatomical features of congenital microphthalmia and anophthalmia in children and conservative methods of rehabilitation]. / Kliniko-anatomicheskie osobennosti vrozhdennogo mikroftal'ma i anoftal'ma u detei i konservativnye metody reabilitatsii.

AIM: To develop a comprehensive classification system of distinctive clinical and anatomical features of congenital microphthalmia and anophthalmia in children and to specify indications, contraindications, and optimal timing of the primary and subsequent prosthetic treatment. MATERIAL AND METHODS: A total of 70 patients with congenital micro- or anophthalmia aged from 1 month to 12 years were examined. Besides the routine ophthalmic examination, all patients underwent eye and orbit ultrasound (axial length measurement and B-scan), computed tomography of the orbits and skull, and immunological tests for infectious diseases (enzyme-linked immunosorbent assays). RESULTS: Basing on the examination RESULTS: we have determined the common types of congenital micro- and anophthalmia in children. We have also developed a stepwise prosthetic treatment aimed at better cosmetic rehabilitation. Indications and contraindications for the use of ocular prostheses in children with congenital micro- and anophthalmia have been identified. CONCLUSION: The proposed method of stepwise prosthetics is the principal option for conservative rehabilitation of children with congenital micro- or anophthalmia.

[The top ten researches of Chinese oculoplastic surgery and orbital disease in recent five years].

Ten researches that may represent the progress in Chinese oculoplastic surgery and orbital disease were selected through voting by specialists from Chinese Society of Ophthalmic Plastics and Orbital Disease. These researches focused on the following fields: the endoscopic navigation system of orbital surgery, orbital bone regeneration and materials, comprehensive sequence treatment of retinoblastoma, the mechanism of thyroid related ophthalmopathy, the intervention strategy and evaluation of orbital development with congenital microphthalmia and anophthalmia, blepharophimosis genotype-phenotype relationship and intervention strategies and clinical study of new materials for frontal muscle suspension. These studies represented the level and influence of Chinese oculoplastics and orbital disease in the international academic community and they were the landmark studies in our areas of expertise.

Anophthalmic syndrome: a review of management.

PURPOSE: To review and summarize current management of anophthalmic syndrome-enophthalmos, superior sulcus syndrome, lower eyelid laxity, and upper eyelid ptosis. METHODS: The authors performed a PubMed search of all articles published in English on the management of anophthalmic socket syndrome. RESULTS: A review of 37 articles demonstrated that anophthalmic syndrome occurs in a significant proportion of this patient population. Primary prevention through careful selection of primary orbital implant is ideal. Residual mild deficits can then be corrected through prosthesis modification. When modification of the prosthesis is no longer sufficient, specifically targeted procedures become necessary. CONCLUSIONS: Ocularists and oculoplastic surgeons should work together closely to treat anophthalmic syndrome. Future studies should establish uniform measurement criteria as the next step in validating the benefit and limitation of each technique.

Postoperative outcomes of anophthalmic socket reconstruction using an autologous buccal mucosa graft.

Socket contracture is one of the most common and difficult problems in anophthalmic patients. This study intended to evaluate postoperative outcomes of anophthalmic socket reconstruction using an autologous buccal mucosa graft in patients with socket contracture. Medical records and photographs of 44 anophthalmic patients who underwent socket reconstruction surgery using an autologous buccal mucosa graft were reviewed retrospectively. The time necessary for the graft surface to be completely vascularized was assessed, and fornix depth was measured before and 6 months after surgery. Postoperative cosmetic and functional outcomes were evaluated, and the factors that influence postoperative outcomes were investigated. The surgery was performed without any significant complications, and the patients only complained of oral discomfort within 1 week. The graft surface was fully vascularized about 1.1 months after surgery. Mean fornix depth after surgery was significantly deeper than that before surgery (9.1 mm, about 68.2% of the vertical size of the implanted graft). Preoperatively, 50.0% of the patient had cosmetic grades 1 and 2; however, 63.6% of the patients achieved grade 4, and 93.2% had higher than grade 3 after surgery. In functional outcomes, 86.4% of the patients presented functional success. Graft recontracture occurred in only 2 patients. Preoperative severe socket contracture was a factor associated with worse cosmetic outcome (P = 0.001). An autologous buccal mucosa can be a safe and effective graft material for the reconstruction of a contracted socket.

The genetics of anophthalmia and microphthalmia.

PURPOSE OF REVIEW: To summarize recent breakthroughs regarding the genes known to play a role in normal ocular development in humans and to elucidate the role mutations in these genes play in anophthalmia and microphthalmia. RECENT FINDINGS: The main themes discussed within this article are the various documented genetic advances in identifying the various causes of anophthalmia and microphthalmia. In addition, the complex interplay of these genes during critical embryonic development will be addressed. SUMMARY: The recent identification of many eye development genes has changed the ability to identify a cause of anophthalmia and microphthalmia in many individuals. Syndrome identification and the availability of genetic testing underscores the desirability of evaluation by a geneticist for all individuals with anophthalmia and microphthalmia in order to provide appropriate management, long-term guidance, and genetic counseling.

[Method of implant insertion in anophthalmic socket].

The method of extraocular muscles fixation is presented, it consist in direct fixation to alloplant according to their physiological attachment to ensure stump movement after enucleation. A total of 54 patients aged 26-82 years were treated. Primary enucleation was performed in 46 patients and 8 patients were previously treated with brachytherapy. Surgery was performed under general anesthesia using microsurgical technique. After alloplant was fixed to the anterior surface of tenon's capsule a running suture was put horizontally and conjunctival suture had vertical orientation. A pressure dressing was applied for 2-3 days. Generally there were no technical difficulties, but in patients after brachytherapy separation of muscles and tenon's capsule was complicated because of scarring in the site of previous radiation.

Conjunctival squamous cell carcinoma in situ in the anophthalmic socket.

A 58-year-old man who had his left eye enucleated at the age of 26 after sustaining ocular trauma presented secondary to an inability to tolerate his prosthesis. Examination revealed upper and lower eyelid cicatricial entropion with areas of symblephara and conjunctival scarring causing forniceal shortening. No areas suspicious for malignancy were noted. Surgical correction was performed, and a segment of the conjunctiva sent for pathology revealed squamous cell carcinoma in situ. Map biopsies of the conjunctiva were subsequently performed, with no further areas of malignancy identified. The patient has been followed with close clinical surveillance, with no evidence of any recurrent suspicious areas within the conjunctiva. This case report confirms that squamous cell carcinoma in situ may occur in an anophthalmic socket and be managed conservatively, reserving the need for orbital exenteration for more invasive disease.

Mid line craniofacial defects and morning glory disc anomaly with clinical anophthalmos-a distinct clinical entity.

Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele. We report a case of a 4-month-old male baby, who was referred to our institute with the complaints of decreased vision. This is the first report from Northeastern part of India as per our knowledge.

[A case of inadequate surgery tactics in the use of an unstandard orbital implant].

By analyzing a clinical case with inadequate surgery tactics in the use of an unstandard orbital implant, the authors discuss possible errors in surgical techniques and in the choice of an implant. Furthermore, by analyzing problems in the use of various implants, including those unstandard ones, they discuss possible causes of complications, consider the optimal time for resurgeries, and propose the ways of solving the problems associated with the occurrence of complications, including treatment policy.

Microphthalmia Syndrome 9: Case Report of a Newborn Baby with Pulmonary Hypoplasia, Diaphragmatic Eventration, Microphthalmia, Cardiac Defect and Severe Primary Pulmonary Hypertension.

BACKGROUND The pulmonary hypoplasia/agenesis, diaphragmatic hernia/eventration, anophthalmia/microphthalmia, and cardiac defect (PDAC) syndrome is a rare medical condition presumably of autosomal recessive way of inheritance with only a few reported cases. Recessive mutations in the STRA6 and both recessive and dominant mutations in RARB gene have been identified as the cause of anophthalmia/microphthalmia and other abnormalities included in the PDAC spectrum. However, those mutations have not been found in all PDAC syndrome cases reviewed from the literature. CASE REPORT We report a case of a full-term living male infant with pulmonary hypoplasia, left diaphragmatic eventration, bilateral microphthalmia, congenital cardiac defects, and severe pulmonary hypertension. CONCLUSIONS The main feature in the reported cases was anophthalmia/microphthalmia. Therefore, screening for the other associated congenital anomalies is highly suggested. Mutations in STRA6 and RARB genes are commonly encountered in this spectrum. However, whole exome sequencing of suspected cases and their parents is recommended to detect possible de novo mutations. Further reports are needed to identify risk factors and prognosis of this rare syndrome.

Microphthalmos-anophthalmos-coloboma (MAC) spectrum in two brothers with Renpenning syndrome due to a truncating mutation in the polyglutamine tract binding protein 1 (PQBP1) gene.

Background: Patients with intellectual disability syndromes frequently have coexisting abnormalities of ocular structures and the visual pathway system. The microphthalmos, anophthalmos, and coloboma (MAC) spectrum represent structural developmental eye defects that occur as part of a syndrome in one-third of cases. Ophthalmic examination may provide important diagnostic clues in identifying these syndromes.Purpose: To provide a detailed and comprehensive description of the microphthalmos, anophthalmos, and coloboma (MAC) spectrum in two brothers with intellectual disability and dysmorphism.Methods: The two brothers underwent a detailed ophthalmic and systemic evaluation. A family pedigree was obtained and exome sequencing was performed in the proband.Results: The two brothers aged 4 and 7 years had intellectual disability, microcephaly, short stature, and characteristic dysmorphic features. Ophthalmic evaluation revealed the presence of the MAC spectrum in both boys. Genetic testing led to the detection of an X-linked hemizygous truncating mutation in the nuclear polyglutamine-binding protein 1 (PQBP1) gene confirming the diagnosis of X-linked recessive Renpenning syndrome.Conclusion: The presence of X-linked intellectual disability and characteristic dysmorphism, in a patient with the MAC spectrum should raise the suspicion of Renpenning syndrome. PQBP1 mutation testing is confirmatory. A comprehensive systemic evaluation is mandatory in all patients with the MAC spectrum and intellectual disability.

[Bioceramic and polymeric bioactive composite implants in orbit zygomatic complex reconstruction: a new prospect for biomaterials]. / O uso de implantes de compósito bioativo de biocerâmica em matriz polimérica na reconstrução do complexo zigomático orbitário: novas perspectivas em biomateriais.

PURPOSE: To evaluate through clinical and tomographic parameters implant behavior in orbital zygomatic reconstruction in six patients. METHODS: The subjects for this preliminary study consisted of six anophthalmic socket patients (3 patients presented residual orbital zygomatic deformities after complex facial fractures and 3 patients presented orbital zygomatic retraction after enucleation and radiotherapy to treat retinoblastoma in infancy). These deformities were surgically corrected with this composite implant. This study was approved and authorized by the Universidade Federal de Minas Gerais Ethical Committee for Research in Human Subjects (ETIC 203/04). Clinical data and tomographic images were utilized to assess the outcome of this study. RESULTS: There were no complications and tomographic findings revealed no implant reactions or migration and a good maintenance of soft tissue projection in the operated areas was achieved. Success of outcome in this preliminary study were encouraging. CONCLUSION: This study will be continued enrolling a larger sample and longer follow-up. Composite biomaterials have presented a good outcome in facial reconstructive surgery. The composite implants in this group have a good biocompatibility and combined with national technology can reduce costs providing more possibilities to many more patients.

Nutrient intake in women before conception and risks of anophthalmia and microphthalmia in their offspring.

BACKGROUND: We previously explored associations between nutrients including folate and other macro and micronutrients and risks of anophthalmia or microphthalmia in the National Birth Defects Prevention Study. In the current study, we expand those previous results with larger sample sizes and conduct analyses with an additional diet quality index using more recent data. METHODS: The National Birth Defects Prevention Study is a population-based, multicenter case-control study of over 30 major birth defects, with estimated due dates from October 1997 to December 2011. Cases were 224 infants diagnosed with anophthalmia or microphthalmia. Controls were 11,109 live-born, nonmalformed infants randomly selected by each study center. Mothers completed a standardized, computer-assisted telephone interview between 6 weeks and 24 months after delivery. Mothers responded to a shortened food frequency questionnaire, assessing their nutrient intake for the year before pregnancy, and questions about periconceptional (2 months before to 2 months after conception) vitamin supplement use. Nutrient intake quartiles were based on the intake among controls. RESULTS: Among vitamin supplement users, odds of anophthalmia/microphthalmia were decreased for women with intake levels in the highest quartile of folate (0.56, 95% confidence interval [CI] 0.32-0.98), magnesium (0.42, 95% CI 0.22-0.82), and vitamin E (0.50, 95% CI 0.29-0.89). Among women not reporting vitamin supplement use, the odds were significantly increased for beta-carotene (2.5, 95% CI 1.10-5.68) and decreased for retinol (0.37, 95% CI 0.19-0.73). CONCLUSIONS: In this expanded analysis, we observed associations for a few nutrients, specifically forms of vitamin A. However, the heterogeneity of results by form and vitamin use necessitates further inquiry.

Rare association of anophthalmia, complex congenital heart disease and pulmonary hypertension: case report. / Rara asociación de anoftalmía, malformación cardíaca compleja e hipertensión pulmonar: reporte de caso.

Clinical congenital anophthalmia is described as the uni- or bilateral absence of the eyeball that might occur in isolation or as part of a syndrome. It has a very low prevalence and its etiology is heterogeneous. Complex congenital cardiac malformations are also rare. The association of congenital anophthalmia and congenital heart disease is rarer still, and the etiology of those associations is not well understood yet. We report the case of a patient who had the very rare association of bilateral anophthalmia, multiple cardiac malformations and severe pulmonary hypertension.

La anoftalmía congénita clínica es la ausencia uni o bilateral del globo ocular, que se presenta de forma aislada o como parte de un síndrome. Tiene muy baja prevalencia y su etiología es heterogénea. La presencia conjunta de malformaciones cardíacas congénitas complejas también es poco frecuente. La asociación de anoftalmía congénita con cardiopatías congénitas es más rara aún, la etiología de tales asociaciones no es bien comprendida todavía. Se reporta el caso de una paciente que presentó la muy infrecuente asociación de anoftalmía bilateral, múltiples malformaciones cardíacas e hipertensión arterial pulmonar grave.

National study of microphthalmia, anophthalmia, and coloboma (MAC) in Scotland: investigation of genetic aetiology.

We report an epidemiological and genetic study attempting complete ascertainment of subjects with microphthalmia, anophthalmia, and coloboma (MAC) born in Scotland during a 16 year period beginning on 1 January 1981. A total of 198 cases were confirmed giving a minimum live birth prevalence of 19 per 100 000. One hundred and twenty-two MAC cases (61.6%) from 115 different families were clinically examined and detailed pregnancy, medical, and family histories obtained. A simple, rational, and apparently robust classification of the eye phenotype was developed based on the presence or absence of a defect in closure of the optic (choroidal) fissure. A total of 85/122 (69.7%) of cases had optic fissure closure defects (OFCD), 12/122 (9.8%) had non-OFCD, and 25/122 (20.5%) had defects that were unclassifiable owing to the severity of the corneal or anterior chamber abnormality. Segregation analysis assuming single and multiple incomplete ascertainment, respectively, returned a sib recurrence risk of 6% and 10% in the whole group and 8.1% and 13.3% in the OFCD subgroup. Significant recurrence risks were found in both unilateral and bilateral disease. In four families, one parent had an OFCD, two of which were new diagnoses in asymptomatic subjects. All recurrences in first degree relatives occurred in the OFCD group with a single first cousin recurrence seen in the non-OFCD group. A total of 84/122 of the MAC cases were screened for mutations in the coding regions of PAX6, CHX10, and SIX3. No pathogenic mutations were identified in the OFCD cases. A single PAX6 homeodomain missense mutation was identified in a subject with partial aniridia that had been initially misclassified as coloboma.

Unusual eye abnormalities associated with congenital cytomegalovirus infection.

Seven children with congenital cytomegalovirus infection demonstrated a higher than expected incidence of "rare" ophthalmological abnormalities, including anophthalmia and Peters' anomaly. These data suggest that appropriate investigation for evidence of cytomegalovirus infection should be instituted in any child with congenital ocular defects.