1/38. 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.- - - - - - - - - - ranking = 1keywords = microphthalmos (Clic here for more details about this article) |
2/38. optic nerve coloboma with retinal degeneration associated with cystic microphthalmia of the other eye.In a seventy-five-year old man an optic nerve coloboma with generalised retinal degeneration associated with a cystic microphthalmia of the other eye is described. The MR imaging revealed the existence of a left microphthalmic eye with a lower lid cyst. From the other eye an optic nerve coloboma with a cystic ectasia of the coloboma area freely open to the vitreous cavity was apparent. The ERG recorded from this eye was extinguished.- - - - - - - - - - ranking = 0.030426743431169keywords = microphthalmia (Clic here for more details about this article) |
3/38. microphthalmos and optic disc coloboma associated with a retrobulbar cyst.The combination of microphthalmos, optic nerve coloboma, and retrobulbar cyst is very rare. We present such a case, demonstrated by ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI).- - - - - - - - - - ranking = 0.33333333333333keywords = microphthalmos (Clic here for more details about this article) |
4/38. Bilateral microphthalmos with colobomatous orbital cyst and de-novo balanced translocation t(3;5).A term Caucasian male infant, born to a healthy non-related couple, was noted at birth to have bilateral edema and bluish discoloration of the lower eyelids. On physical examination, the eye globes were not visualized and hypertelorism was noted. Radiological imaging revealed large bilateral orbital cysts, microphthalmos, and severe optic nerve hypoplasia. Histological study of the excised orbital masses showed cysts lined by primitive, immature retinal tissue which contained neuroglial elements and scattered dysplastic rosettes. Chromosome analysis revealed an apparent balanced reciprocal translocation between the long arm of chromosome 3 and 5, i.e. 46, XY, t (3; 5) (q27; q11.2).Chromosome studies in parents were normal. To our knowledge, the association of this balanced translocation and microphthalmos with cyst has not been previously described in the English literature.- - - - - - - - - - ranking = 2keywords = microphthalmos (Clic here for more details about this article) |
5/38. Colobomatous microphthalmia and orbital neuroglial cyst: case report.We present the case of a boy with a congenital right orbital cyst with bilateral colobomatous microphthalmia. neuroimaging studies excluded communication between the cyst and the eye and between the cyst and the central nervous system. Analysis of cyst fluid obtained by aspiration detected beta 2-transferrin by high resolution immunofixation (IFE). The cyst recurred two months following aspiration. It was then completely excised and histopathologic studies demonstrated a cyst containing neuroglial tissue. No recurrence was observed for 12 months following excision.- - - - - - - - - - ranking = 0.030426743431169keywords = microphthalmia (Clic here for more details about this article) |
6/38. Bilateral ocular malformations in a newborn with normal karyotype: histologic findings.microphthalmos with cyst is a rare condition characterized by a small globe and an inferior uveoretinal coloboma. There is also a defect in the posterior aspect of the eye through which a cyst lined by neuroectodermically derived tissue protrudes into the orbit. A case of isolated bilateral colobomatous and cystic microphthalmos is reported in an otherwise healthy child, showing no evidence of chromosomal abnormalities. Microscopic findings in the enucleated eye consisted of iris and retinal dysgenesis, ectopia lentis, persistent anterior tunica vasculosa lentis and pupillary membrane, intrachoroidal smooth muscle, and optic nerve hypoplasia. In the orbital cyst, a thick membrane reminiscent of the retinal inner limiting membrane lay between the fibroadipose and vascularised outer wall and the inner neuroectodermal lining.- - - - - - - - - - ranking = 0.33333333333333keywords = microphthalmos (Clic here for more details about this article) |
7/38. Orbital aspiration as treatment of microphthalmos with orbital cyst: a case report.A 6-month-old girl came to the hospital with swelling of the right lower eyelid, exophthalmos, chemosis and upward deviation of the eyeball--all of which had been present since birth. iris, optic disc, and chorioretinal coloboma were also apparent. magnetic resonance imaging revealed a small globe with a large cystic lesion in the orbit of the right eye. Pre- and post-operative photographs and magnetic resonance imaging indicated a safe, simple single orbital aspiration as an alternative treatment for mild microphthalmos with an orbital cyst.- - - - - - - - - - ranking = 1.6666666666667keywords = microphthalmos (Clic here for more details about this article) |
8/38. Bilateral microphthalmia with cyst, facial clefts, and limb anomalies: a new syndrome with features of waardenburg syndrome, cerebro-oculo-nasal syndrome, and craniotelencephalic dysplasia.We report a patient with bilateral microphthalmia with cyst, limb anomalies, and multiple facial malformations. This patient has clinical features similar to Waardenburg ophthalmo-acromelic syndrome, cerebro-oculo-nasal syndrome, and craniotelencephalic dysplasia. Although all of these syndromes are characterized by microphthalmia, the presently reported patient does not have the complete pattern of any of these syndromes, It is possible that he has a previously undescribed syndrome, most closely related to the cerebro-oculo-nasal syndrome with malformations outside the craniofacial region. More case reports are needed to further delineate this possibly new syndrome.- - - - - - - - - - ranking = 0.036512092117403keywords = microphthalmia (Clic here for more details about this article) |
9/38. microphthalmos with cyst--case presentation.The author has experienced a case of microphthalmos with large orbital cyst in a 4 months old female, that was found at the time of birth. To facilitate fitting a cosmetic prosthesis, the microphthalmos with cyst was removed surgically. On serial section I could find an area of discontinuation of the sclera that was suspected to be the defective closure of the embryonic cleft. Some aberrant retinal tissue was found in the wall of the cyst, and markedly disorganized ocular tissue forming a tumor-like mass filled the microphthalmic eyeball. In view of these histopathologic findings I could draw the conclusion that developmental failure of the embryonic eyeball and consequential proliferation of the embryonic neuroepithelial cells occurred at an early developmental stage causing the formation of microphthalmos with cyst.- - - - - - - - - - ranking = 1keywords = microphthalmos (Clic here for more details about this article) |
10/38. microphthalmos with bilateral colobomatous orbital cyst accompanied by polycystic kidney disease and vacuolization of myeloid progenitor cells.A case of microphthalmos with bilateral colobomatous orbital cyst accompanied by polycystic kidney disease and vacuolization of myeloid progenitor cells is presented. association of these three entities has not been described previously in the literature.- - - - - - - - - - ranking = 0.33333333333333keywords = microphthalmos (Clic here for more details about this article) |
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