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PubMed2004

[The methodology and accuracy of MRI-based orbital volume calculations].

OBJECTIVE: The aim of the present work was to establish a method for orbital volume calculation based on MR scanning data for the sake of better radiation hygiene. MATERIALS AND METHODS: The orbital volumes of 35 ophthalmologically healthy children were calculated on the basis of MRI scans. After data transfer to a separate workstation, volumetric analysis was carried out by two independent radiologists using semi-automated software. The accuracy of the calculated values was compared with orbital volumes measured by anatomic preparations and given in studies by various authors. RESULTS: Volume calculation was possible in all patients using MRI data. There is an acceptable agreement with the presented anatomic facts and the measured values of Bentheley. In the Wilcoxon test there was not a big difference between the courses of the values (p = 0.507). CONCLUSION: Even though we can obtain a better image of the bizarre structure of the bony orbits with CT, MR-based volumetry of the orbit is a reliable method and is not burdened by radiation exposure. It can thus be an important condition for the planning and the controlling of modern therapeutic concepts in treating anophthalmos and microphthalmos.

Aging↗
PubMed1999

Tarsal switch procedure for the surgical rehabilitation of the eyelid and socket deficiencies of the anophthalmic socket.

PURPOSE: To describe a tarsal transfer procedure, which we have named the "tarsal switch," to correct the eyelid malpositions and camouflage the socket defects of acquired anophthalmos. METHODS: The technique consists of an upper eyelid tarsectomy, with transfer of the autologous tarsoconjunctival graft to the posterior lamella of the lower eyelid. RESULTS: The operation was performed in 21 anophthalmic patients. In 16 patients with eyelid malpositions, excellent results (within 1 mm of the fellow eye) were attained in 100% of the patients with ptosis, and in 88% of patients with lower eyelid retraction. In the remaining 5 patients, orbital volume loss with secondary implant migration, inferior prosthetic displacement and eyelid asymmetry predominated. In these patients the anophthalmic orbital defects and eyelid asymmetry were masked well. Patient satisfaction was high and complications were few during an average follow-up interval of 16 months. CONCLUSION: The tarsal switch procedure is useful in managing the eyelid malpositions and masking the orbital deficiencies of the anophthalmic socket.

Adult↗
PubMed2001

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↗
PubMed1995

Alloplastic materials for orbital surgery.

Alloplastic materials are used as an adjunct to many types of ophthalmic surgery. This article reviews recent advancements in the use of alloplastic materials for orbital surgery, with emphasis on anophthalmic socket and bony orbital reconstruction. Several new porous materials and resorbable implants offer exciting prospects in the management of anophthalmos and orbital fractures.

Anophthalmos↗
PubMed2005

Eye anomalies: seasonal variation and maternal viral infections.

INTRODUCTION: Eye anomalies, including anophthalmos and microphthalmos (absent eye and small eye), are common features in congenital infection syndromes such as rubella. Influenza, Coxsackie virus, and Parvovirus are less well-established teratogens. We report an ecologic analysis of anophthalmia/microphthalmia prevalence in England in relation to temporal variation in these infections using routine infection data. METHODS: The national (England) anophthalmos/microphthalmos register holds details of 406 babies (excluding chromosomal anomalies) born 1988-1994. Weekly infection data were obtained from the U.K. Public Health Laboratory Service and Royal College of General Practitioners Weekly Returns Service. We assigned cases and births an estimated exposure based on the population infection counts in the gestational weeks known to be critical for ocular development. We calculated the rate ratio of anophthalmos/microphthalmos prevalence for infection in the 90th percentile compared with the 10th percentile by Poisson regression. RESULTS: There was a positive association between severe anophthalmos/microphthalmos prevalence and Parvovirus B19 (rate ratio = 1.26; 95% confidence interval = 1.00-1.58) and for influenza (1.41; 1.08-1.84). Coxsackie virus showed a negative association with any anophthalmos/microphthalmos (0.75; 0.58-0.98) and with severe cases (0.66; 0.46-0.95). CONCLUSIONS: This analysis provides suggestive evidence for a teratogenic role of influenza and Parvovirus in anophthalmos/microphthalmos. The role of maternal infections in congenital anomaly etiology is worthy of further investigation.

Anophthalmos↗
PubMed1995

Orbital expansion of the congenitally anophthalmic socket.

BACKGROUND: Congenital anophthalmos is a rare condition in which intervention at an early age can stimulate orbital expansion and maximise facial symmetry. Much is still unknown, however, regarding the degree of soft tissue and bony orbital growth achieved using the orbital expanders presently available. METHODS: A retrospective review of 59 congenitally anophthalmic orbits in 42 patients was carried out. RESULTS: The soft tissue and bony orbital expansion achieved using serial solid shapes is reported, and experience with hydrophilic expanders and inflatable silicone expanders is reviewed. CONCLUSION: Although serially fitted solid shapes in the orbit lead to increased expansion of orbital soft tissue and bone compared with no orbital implant, further orbital tissue enlargement is required. The inflatable silicone expander may allow more rapid and extensive orbital tissue expansion, but design changes are needed to achieve this.

Anophthalmos↗
PubMed2001

Spontaneous reversal of nystagmus in the dark.

AIM: To report five children with horizontal jerk nystagmus in whom eye movement recordings in the dark revealed a spontaneous reversal in the direction of the nystagmus beat. Three patients were blind in one eye and were diagnosed as having a manifest latent nystagmus (MLN), and two patients had strabismus and congenital nystagmus (CN). METHODS: Eye movements were recorded using DC electro-oculography with simultaneous video recording, including infrared recording in total darkness. RESULTS: Four patients had decelerating velocity slow phase jerk nystagmus when recorded under natural lighting conditions; the fifth case had accelerating velocity and linear slow phase jerk nystagmus. Under absolute darkness, nystagmus reversed in direction of beat with a mixture of linear and decelerating velocity slow phase waveforms. One child with unilateral anophthalmos could wilfully reverse the beat direction of his nystagmus by trying to look with his blind eye in the light and dark. CONCLUSIONS: These observations support the theory that LN/MLN beat direction is determined by the "presumed" viewing eye and may be consciously controlled. The spontaneous reversal of beat direction in the dark suggests eye dominance is predetermined. Eye movement recordings identified mixed nystagmus waveforms indicating that CN (accelerating velocity slow phases) and LN/MLN (linear/decelerating velocity slow phases) coexist in these subjects.

Anophthalmos↗
PubMed1994

Anophthalmia with cleft palate and micrognathia: a new syndrome?

A 5 day old male with bilateral anophthalmos, hypospadias, bifid scrotum, micrognathia, and cleft palate with normal chromosomes is described. There have been two case reports with similar clinical manifestations but associated with interstitial deletion of 14q (q22q23). We propose that either our patient represents a new syndrome or has a microdeletion which could not be detected on routine cytogenetics. High quality cytogenetics and FISH may show microdeletions in patients presenting with this clinical picture and normal chromosomes.

Abnormalities, Multiple↗
PubMedDate not supplied

Cryptophthalmos with an orbital cyst and profound mental and motor retardation.

The cryptophthalmos syndrome generally consists of cryptophthalmia, dyscephaly (cleft lip and palate, nasal and ear defects, meningoencephaloceles, etc.), syndactyly, and urogenital malformations. This disorder is rare. Approximately 55 cases have been reported, with only a few described in the American literature. A three-year-old boy with this rare syndrome is described. Major clinical findings include: asymmetric bilateral cryptophthalmos, unilateral orbital cyst of the nasofrontal area, bilateral anophthalmos, right choanal atresia, right microtia, right auditory canal atresia, hypodontia, ankyloglossia, cartilaginous synchondroses of the cervical vertebrae, and bilateral acetabular dysplasia. Striking features of this case, in addition to the above, were profound mental and motor retardation. Syndactyly and renal anomalies were not observed.

Abnormalities, Multiple↗
PubMedDate not supplied

[Basic trends in the organization of ocular prosthetics service].

The key issues related with rendering the ocular prosthetic aid to patients with anophthalmos and with cosmetically inferior, i.e. a smaller-size, blind eye, are in the focus of attention. Around 320,000 persons need ocular prostheses in the territory of the Russian Federation, hence adequate prosthetic measures are taken to cope with the cosmetic, medical and social rehabilitation of patients. The ocular prosthetics service deals with issues related with prostheses manufacturing technologies as well as with other therapeutic and surgical aspects which call for working out a unified policy with its subsequent commissioning into the practice of ophthalmologic institutions.

Adult↗
PubMed2006

[Eye socket reconstruction with free flap].

OBJECTIVE: To discuss the effect of eye reconstruction with free flap. METHODS: Free flap was used in 21 patients of contracted eye socket and anophthalmos. The forearm free flap was utilized in 19 patients,the scapular flap in 2. Free flap with microvascular anastomoses was transferred into contracted eye socket. And hydroxyapatite implant was selectively inserted for intraorbital implantation according to the extent of contracted eye socket postoperation. Eye socket was reconstructed in one stage operation. RESULTS: The flaps were transferred successfully in all patients. All patients were followed up between 4 to 42 months, better cosmetic results were achieved and well-fitted eye prosthesis was weaned. CONCLUSION: Free flap is a useful alternative in treatment of severe contracted eye socket.

Adolescent↗
PubMed1990

The role of tissue expanders in an anophthalmic animal model.

A study of orbital bony expansion using a custom tissue expander was performed in the anophthalmic cat model. Twelve 6-week-old kittens underwent right unilateral enucleations. Six kittens had immediate insertion of a tissue expander into the orbit. The remaining six served as controls. Every 2 weeks 0.5 cc saline was injected into the expander to a maximum of 5 cc. External horizontal and vertical orbital dimensions were obtained by palpation technique weekly. All animals had preoperative and study conclusion head CT scans with three-dimensional reconstructions performed. Dry skull preparations were done at the study conclusion at 24 weeks. Results demonstrated that tissue expanders were successful in maintaining normal orbital growth and size relative to the contralateral control orbit. The animals with enucleation only had an average difference in vertical and horizontal orbital measurements of -27 and -13 percent when compared with the contralateral normal orbit. In contrast, the enucleation and tissue-expansion animals had vertical and horizontal measurements of +4 and +2 percent (p less than 0.05) when compared with the contralateral orbit. Head CT scans with three-dimensional reconstructions demonstrated normal orbital geometry and volume for the animals with tissue expanders, whereas animals with enucleation only had small hypoplastic orbits. In conclusion, orbital tissue expanders offer a promising new technique in the treatment of anophthalmos.

Animals↗
PubMed1989

[Experimental and clinical characteristics of the formation of a locomotor stump after evisceroenucleation and the creation of a mobile prosthesis base].

A new method is proposed for evisceroenucleation with formation of a locomotive stump permitting to remove complications connected with anophthalmos. In experiment, morphologic investigation of changes in the implant (allocartilage) in dynamics from 20 days to 1 year have shown that the implant preserves its form and initial fixation for the whole period of observation. This provides good prosthetic conditions. Ultrasonic scanning is recommended as an effective method to control the implant adaptation and locomotive stump formation.

Animals↗
PubMed1971

Congenital anomalies of the retina.

Review of 50 histopathologic cases and a number of clinical cases of congenital retinal anomalies has permitted classification under the following headings: 1) Coloboma-orbital cyst--"anophthalmos" group due to aberrant closure of the embryonic fissure; 2) Retinal fold-central stalk-detachment group comprising a series that varies from simple retinal folds to total retinal detachment and anomalous stalk formation. Cases of the 13-15 trisomy syndrome constitute a special subgroup in this rubric; 3) Retrolental fibroplasia, due to hyperoxia of premature infants, is manifest by "dragged" disks and gliovascular proliferation with occasional detachment; 4) Persistent hyaloid system is occasionally associated with mild anomalies of the retina; 5) Massive gliosis of the retina is usually a hamartomatous manifestation; 6) Congenital absence of ganglion cells occurs with cerebral maldevelopment and 7) Congenital absence of the photoreceptors is the congenital form of retinitis pigmentosa.

Abnormalities, Multiple↗
PubMedDate not supplied

[Cerebro-oculofacial dysplasia associated with vertebral changes].

A clinical case of craniofacial dysplasia, with multiple and complex congenital anomalies, is presented. The clinical modifications noticed are systematized in four groups: cerebral, ocular, facial and vertebral. The case is a form of cerebrofacial display, associated with anophthalmos, orbitopalpebral chist and vertebral modifications. There are discussed problems of differential diagnosis, pathogeny and therapeutical attitude.

Abnormalities, Multiple↗
PubMed1977

Orbital expansion for anophthalmia and micro-orbitism.

In severe cases of microphthalmos or anophthalmos, the bony orbital cavity is often too small to allow one to create a satisfactory socket for a prosthesis. An omnidirectional expansion of the orbit, by osteotomies done in a step-like fashion, allows the creation of a larger bony cavity and improves the symmetry of the face. The risks of the craniotomy and of the mobilization of the free bone pieces are discussed.

Anophthalmos↗
PubMedDate not supplied

Local research ethics committees' approval in a national population study.

BACKGROUND: Epidemiological research using patient records faces considerable uncertainty regarding requirements for local ethical review, confidentiality of data and patient permission. SETTING AND DESIGN: We report the experiences of the national study of clustering and geographical variation in anophthalmos and microphthalmos, which, from 1994, has been compiling a register of affected children born in England since 1988. The information is obtained from clinicians and local health authority records without any direct contact with patients or their families. RESULTS: Of 110 district health authorities, 47 required ethical approval. Only one committee accepted the approval of any other committee. Procedures and application forms varied widely. Fewer than one-fifth of the forms completed asked about compliance with the Data Protection Act, yet confidentiality safeguards must be the foremost ethical issue faced by the study. Two committees required that the study inform GPs and two committees required that the study obtain parental permission. DISCUSSION: While new mechanisms including regional committees are being established, there is an urgent need for a standard application form to save time and resources for research. Continuing lack of consistency about the need for subject (parental) permission impedes the proper design and costing of research.

Anophthalmos↗
PubMed1995

The development of the fetal eye: in utero ultrasonographic measurements of the vitreous and lens.

Our objective was to establish nomograms for fetal eye measurements from 12 weeks' gestation by using transvaginal and transabdominal high-resolution ultrasound techniques. A prospective cross-sectional study was performed on 450 normal singleton pregnancies between 12 and 37 weeks' gestation. Vitreous and lens circumferences were measured by transvaginal ultrasonography until 17 weeks, and by abdominal ultrasound between 18 and 37 weeks' gestation. Regression analyses were used to create nomograms, and several transformations were done to obtain linearity. Eye measurements of 12 fetuses at risk for ocular disturbances were plotted on the constructed nomograms. Linear relationships were fitted between vitreous (r2 = 0.79) and lens (r2 = 0.88) circumferences and gestational age. In addition, there was a significant correlation between these measurements and the biparietal diameter. Data of the fetuses at risk showed that disturbances in ocular growth were associated mainly with abnormal cerebral development. These normative data may be helpful in the prenatal diagnosis of suspected congenital syndromes that include, among their manifestations, ocular growth disturbances such as microphthalmos and anophthalmos.

Cross-Sectional Studies↗