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High-density porous polyethylene (Medpor) as a successful anophthalmic socket implant.

BACKGROUND: High-density porous polyethylene (Medpor) has been used successfully as an implant in orbital fracture repair and in the management of both cosmetic and post-traumatic facial deformities. The material is well tolerated, resists infection, is nonantigenic, and promotes tissue ingrowth. Additionally, sutures can be passed through it. These characteristics led to its use as an implant in anophthalmic socket surgery. METHODS: Twenty-one patients with anophthalmia underwent implantation of spherically shaped high-density porous polyethylene implants. The implant was used in six primary enucleations with direct extraocular muscle attachment, ten secondary implant insertions, one repeat secondary implant insertion, and five eviscerations. RESULTS: Postoperative follow-up averaged 19 months. During this period, there were no extrusions, infections, significant inflammatory responses, or implant exposures. One implant was removed 4 months after insertion due to inadequate initial placement secondary to a severe post-traumatic orbital deformity. Successful re-implantation was performed without complication. Postoperatively, all sockets showed good to excellent motility. Results of histologic examination of the removed implant demonstrated minimal inflammatory response and extensive fibrovascular ingrowth involving 60% of the implant substance. CONCLUSIONS: This initial report indicates that high-density porous polyethylene can be used successfully as an implant in anophthalmic socket surgery. Its advantages over other similar implants include a significantly lower material cost and the ability to suture the extraocular muscles directly to it without the need for a covering material such as fascia or sclera. Experimental studies are in progress to determine if this implant can be integrated with an ocular prosthesis to promote improved motility and cosmesis.

Adolescent↗

Some effects of diazepam on pregnancy in the Balb/C mouse.

Female Balb/C mice aged 60 to 72 days were mated with males aged 60 to 150 days and conception was diagnosed when a vaginal plug was noted. Females were randomized into five groups, Group A animals were fed a mixture of diazepam-propylene glycol which contained 0.02 mg. of diazepam in 0.1 ml. solution per day from day 1 to 9 of the gestation. Group B animals were fed the same mixture on days 5 to 12 of the gestation. Groups C and D animals were fed 0.1 ml. of propylene glycol on gestational days 1 to 9 and 5 to 12, respectively. Group E animals served as pure controls and were fed no drug or vehicle. All animals were killed on pregnancy day 15; All pups were counted, weighed, and studied grossly for anomalies. All uteri were scored for absorption sites. Nonpregnant uteri were studied histologically and scored for day of estrogen cycle. Animals fed diazepam on days 5 to 12 of gestation (Group B) had a significantly fewer number of pregnancies than did the other groups (p smaller than 0.001). In addition, pups delivered of diazepam-fed mice were heavier than control animals. No significant differences were noted, however, with respect to litter size, number of resorption sites, or presence of gross anomalies. In view of these data it would seem prudent to continue to avoid the use of diazepam during pregnancy.

Abnormalities, Drug-Induced↗

The importance of developmental timing in cortical specification.

Environmental control of gene expression can occur early or late during development, and this is relevant to understanding species differences in cortical specification. Experiments in the developing visual system of the primate show that the areal limits of striate cortex are specified by the thalamic inputs, so that afferent specification of cortex appears as a general feature of mammalian development. Primates differ from nonprimates in that thalamic afferents control very early stages of corticogenesis when symmetrical cell division is forming the pool of striate neuron precursors. Other cortical features are specified much later in primates than in nonprimates. We speculate that the early specification of certain features and the late specification of others contribute to the sophistication of the cerebral cortex characteristic of primates.

Afferent Pathways↗

[Anesthetic management of a patient with a cryptophthalmos syndactyly syndrome and subglottic stenosis].

We experienced five episodes of anesthesia for a girl with dryptophthalmos syndactyly syndrome and congenital subglottic stenosis from the age of 1.3 year to 4 years. A girl was born at 34 weeks of gestation. The birth weight was 1360 g. The Apgar score was 8 at one minute and there was a hoarseness. She had right cryptophthalmos, syndactyly of hands and left foot, left polydactyly, anomalies of ear and nose, and agenesis of right kidney. The operation was scheduled for syndactyly under general anesthesia when she was 17 days and 5 months. As intubation was unsuccessful in both occasions, the operation was cancelled and subglottic stenosis was pointed out. We decided to postpone the operation until she could cry fully without cyanosis. We evaluated her respiratory ability from the time she became able to cry fully without cyanosis. As a result, we could manage her without any complications such as hypoxia or hypercapnea except mild wheezing.

Abnormalities, Multiple↗

Optic tectum in congenitally monophthalmic fishes and chicks.

Morphometry of optic tectum layers was performed in fishes and birds that were congenitally monophthalmic (or had one very small or malformed eye). The optic chiasm was studied with regard to possible anomalies of the fiber distribution. In two very young fish larvae with only one ocular rudiment, no differences were found between the two tecta. In fishes and chicks with one normal and one eye of reduced size, the volume of superficial tectal layers contralateral to the small eye was significantly more affected (reduction by 40 to 70%) than that of the deep layers (reduction by 10 to 30%). Particularly pronounced differences between the superficial tectal layers of both sides were found in a fish where a thin optic nerve, originating in an unusually small eye, projected to the ipsilateral side of the brain. It is concluded that (1) the development of superficial tectal layers depends more strongly on optic innervation than that of deep layers; (2) obvious differences between tecta with and without optic innervation develop rather late in ontogeny; (3) there seems to be no measurable difference between effects of a poor (less than 25%) innervation of the tectum and a complete lack of this projection.

Anguilla↗

Oculocerebrocutaneous syndrome: a case report, a follow-up, and differential diagnostic considerations.

Oculocerebrocutaneous syndrome consists essentially of orbital cysts and microphthalmia/anophthalmia, focal dermal defects, skin appendages and malformations of the central nervous system. Up to now, about 20 sporadic cases have been reported. Limited information is available on the clinical spectrum and on the natural history. We present the long-term follow-up of a 19 year-old patient who was one of the first two patients described by Delleman and Oorthuys in 1981. Furthermore, we report on a new case presenting with bilateral anophthalmia and orbital cysts, typical skin lesions, a complex brain malformation and cleft lip palate. This infant died due to the severe cerebral malformations at the age of 10 months. Encephalocraniocutaneous lipomatosis, focal dermal hypoplasia and microphthalmia with linear skin defects (MLS) syndrome are discussed as the most important differential diagnostic entities.

Adult↗

[Anophthalmia/micro-ophthalmia in syndromes: epidemiology study of newborns in Spain].

OBJECTIVE: The objective of this study was to perform an epidemiological analysis of the frequency of anophthalmia/microphthalmia (A/M) in syndromes identified in newborn infants in Spain. PATIENTS AND METHODS: Data of the Spanish Collaborative Study of Congenital Malformations during the period of 1976-1994, corresponding to more than 1,200,000 births, was analyzed. Among these, 86 newborn infants with A/M presented some of the recognized syndromes. RESULTS: There is a wide etiological heterogeneity among the syndromes with this ocular defect, with chromosomal syndromes being the most frequent (67.9% of total syndromes with A/M), followed by monogenic syndromes (19.1%), environmental (9.5%) and those of unknown etiology (3.6%). CONCLUSIONS: Some guidelines when a baby is born with A/M are derived from this study. First, given the tendency of the defect to present together with other anomalies, it is advisable to perform a detailed study to rule out or to confirm the existence of other defects. Adequate samples should be taken (even in stillborn infants) for cytogenetic study. Examine carefully the prenatal history, looking for chronic diseases, infectious processes or exposure to teratogens. Depending on the baby's survival, follow-up of the psychomotor development should be made. All of these aspects are always important in malformed babies, but especially in infants with A/M given the tendency of the defect to present in syndromes as the etiologic diagnosis determines the counselling regarding the risk of recurrence, detection of carriers in some cases and possible prenatal diagnosis.

Anophthalmos↗

Human dura mater as a wrapping material for hydroxyapatite implantation in the anophthalmic socket.

Homologous sclera and autologous fascia lata have been accepted as common wrapping materials for hydroxyapatite implantation; however, various problems have been observed, such as the risk of transmittable diseases such as AIDS or hepatitis. This report details the use of cadaver dura mater to wrap hydroxyapatite implants. For 73 eyes of 73 patients with hydroxyapatite implantation, cadaver dura mater was used to wrap the hydroxyapatite after enucleation or secondary implantation. Of the 73 patients, 20 had conjunctival erosion; however, all of the patients but 1 healed spontaneously within an average of 22 days. Only 1 of the 73 patients progressed to implant exposure requiring a dermis graft. The authors suggest that cadaver dura mater is a suitable wrapping material for hydroxyapatite implantation.

Anophthalmos↗

Congenital eye malformations: clinical-epidemiological analysis of 1,124,654 consecutive births in Spain.

We analyzed Spanish Collaborative Study of Congenital Malformations (ECEMC) data on a series of 1,124,654 consecutive births to study congenital eye malformations from an epidemiological standpoint. We studied their frequencies as well as some causal and clinical aspects. Four hundred fourteen infants had eye malformations, for an overall prevalence of 3.68/10,000 newborns. Most frequent were: anophthalmia/microphthalmia (21.34/100,000), congenital cataract (6.31), coloboma (4.89), corneal opacity (3.11), and congenital glaucoma (2.85). In our data, the tendency of eye malformations to be associated with other congenital abnormalities is evident (only 21.01% of cases were isolated). Eye defects are heterogeneous, since we have observed them in clinical patterns with all modes of inheritance or caused by different environmental agents. Chromosomal syndromes represent 60% of total syndromes, followed by syndromes of autosomal-recessive inheritance (15%), environmental syndromes (10%), autosomal-dominant syndromes (5.83%), and other types which have a lower frequency. Regarding defects associated with eye malformations, most frequent are limb anomalies (affecting 59.3% of multiply malformed cases), auricular/facial (47.1%), central nervous system (42.5%), osteomuscular excluding limbs (42.2%), genital defects (30.6%), oral clefts (29.4%), and the rest of the body systems, which are less frequent. Using the method outlined by Prieto and Martínez-Frías [1996: Am J Med Genet 62:61-67], it was demonstrated that the association of coloboma and anophthalmia/microphthalmia was specific, as was the combination of cataract and anophthalmia/microphthalmia, and that of anophthalmia/microphthalmia with holoprosencephaly. From these statistical associations some pathogenetic relationships in human embryos can be inferred, supporting several previously proposed mechanisms.

Anophthalmos↗