1/31. Congenital anophthalmos in benin city, nigeria.Clinical anophthalmos is a very rare condition and this is illustrated in benin City, nigeria where the two cases described are the only cases that have been seen at the University of benin teaching Hospital over a period of twenty years. The two cases were unilateral anophthalmos. The first case is a case of primary anophthalmos while the second case is the consecutive or degenerative type of anophthalmos.- - - - - - - - - - ranking = 1keywords = anophthalmos (Clic here for more details about this article) |
2/31. 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.- - - - - - - - - - ranking = 0.14285714285714keywords = anophthalmos (Clic here for more details about this article) |
3/31. Isolated bilateral anophthalmia in a girl with an apparently balanced de novo translocation: 46,XX,t(3;11)(q27;p11.2).Primary anophthalmos is a heterogeneous condition. In its nonsyndromal form, it is usually considered an autosomal recessive trait. However, other causes such as chromosomal abnormalities and prenatal insults need to be considered. We report on a unique reciprocal translocation 46,XX,t(3;11)(q27;p11.2) in a baby with isolated anophthalmos. Both Chitayat et al. [1996] and Alvarez Arratia et al. [1984] have reported on cases of terminal deletion of the long arm of chromosome 3. In each case the child had multiple anomalies including microphthalmia or anophthalmia. Because our patient appears to have no other anomalies, this break point may indicate that a genetic locus for eye formation exists at chromosome site 3q27. Published 1999 Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.28571428571429keywords = anophthalmos (Clic here for more details about this article) |
4/31. Diencephalic neuronal hamartoma associated with congenital obstructive hydrocephalus, anophthalmia, cleft lip and palate and severe mental retardation: a possible new syndrome.A male infant was born with severe hydrocephalus, bilateral cleft lip/palate, left anophthalmos and right microphthalmos, and an equino-varus foot deformity. Imaging studies showed enlarged lateral ventricles, apparent absence of the corpus callosum and a midline density in the third ventricular region. He had a normal male karyotype. He was severely mentally retarded and died suddenly at 7 years of age. Neuropathological examination of the brain revealed enlarged and polygyric cerebral hemispheres, due to congenital obstructive hydrocephalus, and secondary thinning of the corpus callosum. An unusually large neuronal hamartoma filled the interpeduncular fossa and third ventricle. It was continuous posteriorly with the left thalamus and so was classified as diencephalic rather than as hypothalamic. The right optic nerve merged with the hamartoma, whereas the left nerve was absent. Microscopically the hamartoma consisted of mature grey matter interspersed with narrow bands of white matter. No immature or non-neural elements were identified. This combination of diencephalic neuronal hamartoma, hydrocephalus, ocular and craniofacial abnormalities has not, to our knowledge, previously been described.- - - - - - - - - - ranking = 0.14285714285714keywords = anophthalmos (Clic here for more details about this article) |
5/31. Reconstruction of the lower lid in congenital microphthalmos and anophthalmos.In congenital microphthalmos and anophthalmos, the socket and lids are often underdeveloped. Progressive dilation of the socket often does not increase the horizontal lid aperture or permit the use of a larger prosthesis. The authors present two cases in which a modified Mustarde cheek flap, lined with a tarsal-conjunctival graft, was used to reconstruct and lengthen the lower lid. This procedure results in a larger horizontal lid aperture and permits a larger prosthesis to be contained in the socket.- - - - - - - - - - ranking = 0.71428571428571keywords = anophthalmos (Clic here for more details about this article) |
6/31. 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.- - - - - - - - - - ranking = 0.14285714285714keywords = anophthalmos (Clic here for more details about this article) |
7/31. Bilateral Tessier no. 4 facial cleft with left eye anophthalmos: a case report.Craniofacial clefts are very rare and manifest in a variety of patterns. Tessier classified these clefts in 1973 and numbered them 0 to 14. Tessier No. 4 Facial cleft is a rare variant of craniofacial cleft. Not more than 50 cases are reported in world literature, amongst which only 5 cases are true bilateral in nature. However, combinations of two different variants are not very uncommon. A case of male Indian child aged 4 years with a true bilateral Tessier No. 4 Facial cleft is reported. He also had anophthalmos of the left eye. This is probably the first case in which true bilateral Tessier No. 4 Facial cleft with anophthalmos of one eye is noticed.- - - - - - - - - - ranking = 0.85714285714286keywords = anophthalmos (Clic here for more details about this article) |
8/31. Giant suprasellar aneurysm associated with optic stalk agenesis and unilateral anophthalmos. Case report.The authors report a patient with unilateral anophthalmos who had a large suprasellar aneurysm associated with aplasia of an optic nerve and the chiasmal commissure. The anatomical findings at craniotomy are described.- - - - - - - - - - ranking = 0.71428571428571keywords = anophthalmos (Clic here for more details about this article) |
9/31. genetic heterogeneity of syndromic X-linked recessive microphthalmia-anophthalmia: is Lenz microphthalmia a single disorder?Nonsyndromic congenital microphthalmia or anophthalmia is a heterogeneous malformation with autosomal dominant, autosomal recessive, and X-linked modes of inheritance. Lenz microphthalmia syndrome comprises microphthalmia with mental retardation, malformed ears, skeletal anomalies, and is inherited in an X-linked recessive pattern. Prior studies have shown linkage of both isolated (or nonsyndromic) anophthalmos (ANOP1, [MIM 301590]) and Lenz syndrome [MIM 309800] to Xq27-q28. Nonsyndromic colobomatous microphthalmia [MIM 300345] has been linked to Xp11.4-Xq11.1. We describe a five-generation African-American family with microphthalmia or anophthalmia, mental retardation, and urogenital anomalies, in an X-linked recessive inheritance pattern, consistent with Lenz syndrome. Initial linkage analysis with microsatellite markers excluded the region in Xq27-q28 previously reported as a candidate region for ANOP1 [MIM 301590]. An X-chromosome scan revealed linkage to a 10-cM region between markers DXS228 and DXS992 in Xp11.4-p21.2. Multipoint analysis gave a maximum lod score of 2.46 at marker DXS993. These data show that X-linked recessive syndromic microphthalmia exhibits genetic heterogeneity. In addition, it suggests that Lenz microphthalmia syndrome, previously thought to be a single disorder, may represent an amalgam of two distinct disorders.- - - - - - - - - - ranking = 0.14285714285714keywords = anophthalmos (Clic here for more details about this article) |
10/31. Congenital nasolacrimal duct occlusion with clinical anophthalmos: a possible new association.PURPOSE: To report the association of congenital nasolacrimal duct occlusion and clinical anophthalmos in an eight-year-old girl. methods: A case report. The patient suffered from epiphora and clinical anophthalmos on the right side since birth. This paper presents the clinical presentation, workup, and surgical approach of the case. RESULTS: The nasolacrimal system of the patient was occluded on irrigation. Computed tomography showed a blockage at the level of the sac-duct junction, an enlarged nasolacrimal duct below the obstruction, and a sclera-like ball of tissue surrounded by extraocular muscles in the right orbit. Treatment included a right bicanalicular silicon intubation performed under general anesthesia. CONCLUSION: This case may represent a new association that has not been previously reported.- - - - - - - - - - ranking = 0.85714285714286keywords = anophthalmos (Clic here for more details about this article) |
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