1/23. The etiology and treatment of macular detachment associated with optic nerve pits and related anomalies.PURPOSE: Up to two thirds of patients with optic disc pits develop a sight-limiting maculopathy. There is confusion regarding the etiology and nature of the maculopathy in these cases. We present 7 cases of serous macular detachment occurring in association with optic pits or related cavitary anomalies and identify a rhegmatogenous etiology. methods: We reviewed the records of 7 patients with optic nerve anomalies and macular detachment. patients were treated with observation, barricade laser, vitrectomy, and/or gas tamponade. RESULTS: Seven patients were noted to have serous macular detachment associated with an optic nerve pit or other cavitary anomaly. A hole or tear in the diaphanous tissue overlying the optic pit was identified in all cases. None of the patients had a posterior vitreous detachment. Two were treated with photocoagulation only, and 5 underwent pars plana vitrectomy with fluid-gas exchange with or without photocoagulation. Pretreatment visual acuity ranged from 20/30 to 6/200. Posttreatment acuity ranged from 20/25 to 20/100. Five of 7 eyes had final acuities of 20/30 or better, and all treated eyes improved. CONCLUSIONS: A tear in the diaphanous tissue overlying the optic nerve pit is responsible for the development of serous macular detachment and is consistent with findings in similar conditions, such as retinal detachment in association with chorioretinal coloboma. These tears may be quite subtle, and careful biomicroscopic examination is required to appreciate them. The treatment of this condition remains controversial. However, because of the relatively poor prognosis, we believe treatment should include the formation of a barricade to fluid movement as well as sealing and relief of traction from the hole. The value of laser treatment may be increased by the early identification of a defect in the diaphanous membrane prior to the development of macular detachment. Consideration of prophylactic laser might then reduce the need for later, more invasive measures, and improve the prognosis.- - - - - - - - - - ranking = 1keywords = coloboma (Clic here for more details about this article) |
2/23. Management of retinal detachment associated with choroidal coloboma using perfluoroperhydrophenanthrene (Vitreon).Vitreous surgery using an internal tamponading agent and chorioretinal adhesion is commonly used to repair retinal detachment associated with choroidal coloboma. The detachment may, however, recur if fluid continues to leak through a retinal defect in the area of the coloboma, despite internal tamponade. We present a case in which a recurrent retinal detachment associated with coloboma following vitreous surgery with SF6 was successfully repaired in outpatient surgery using perfluoroperhydrophenanthrene (Vitreon) and endolaser photocoagulation.- - - - - - - - - - ranking = 7keywords = coloboma (Clic here for more details about this article) |
3/23. retinal detachment in focal dermal hypoplasia.PURPOSE: focal dermal hypoplasia is a systemic disease that includes well recognized ocular abnormalities. retinal detachment has not previously been reported as a part of this syndrome. AIM: To report a case of focal dermal hypoplasia with an associated retinal detachment. methods: Single case report of a child with a colobomatous retinal detachment and the focal dermal hypoplasia syndrome. CONCLUSIONS: Retinal detachments may occur as part of the focal dermal hypoplasia syndrome. Expanding knowledge of this syndrome may help ophthalmologists diagnose this rare condition.- - - - - - - - - - ranking = 1keywords = coloboma (Clic here for more details about this article) |
4/23. Pathogenic implications of subretinal gas migration through pits and atypical colobomas of the optic nerve.OBJECTIVE: To describe subretinal migration of gas and silicone oil in a series of patients with congenital cavitary optic disc anomalies and to further clarify the pathogenesis of the associated maculopathy. methods: medical records of 4 female patients, aged 8 to 34 years, who developed subretinal gas migration after vitreous surgery for macular detachment associated with cavitary optic disc anomalies were reviewed. A theoretical model was used to calculate the pressure differential required to induce subretinal gas migration through an optic pit. RESULTS: The 4 patients had bilateral atypical optic nerve colobomas or a unilateral large optic pit. A definite defect in the tissue overlying the disc excavation could be seen in one eye, and intraoperative drainage of subretinal fluid through the disc anomaly was possible in all cases. Subretinal migration of gas or silicone oil was seen intraoperatively in one case and first appeared between 1 and 17 days postoperatively in the remaining cases. Theoretical calculations suggest that the pressure differential required for migration of gas through a small defect in the roof of a cavitary disc lesion is within the range of expected fluctuations in cerebrospinal fluid pressure. CONCLUSIONS: These observations provide clinical confirmation of a defect in tissue overlying cavitary optic disc anomalies and imply interconnections between the vitreous cavity, subarachnoid space, and subretinal space. We theorize that intermittent pressure gradients resulting from normal variations in intracranial pressure play a critical role in the pathogenesis of retinopathy associated with cavitary disc anomalies.- - - - - - - - - - ranking = 5keywords = coloboma (Clic here for more details about this article) |
5/23. Spontaneous resolution of optic nerve coloboma-associated retinal detachment.We report a congenital neurosensory retinal detachment associated with an optic nerve coloboma with subsequent spontaneous reattachment. This represents the earliest reported case of such a clinical situation. An observation period is recommended for infants with this clinical course to allow for the opportunity of spontaneous reattachment.- - - - - - - - - - ranking = 5keywords = coloboma (Clic here for more details about this article) |
6/23. Bilateral retinal detachment with large breaks of pars plicata associated with coloboma lentis and ocular hypertension.A 39-year-old man developed bilateral, insidiously progressing detachment of the retina and nonpigmented epithelium of the ciliary body because of a large break in the pars plicata. The anterior segments had multiple malformations including ectopia of the lens associated with coloboma of the lens and hypoplastic ciliary processes. The pars plicata breaks were present in areas corresponding to the anomalies of the lens-zonule-ciliary body complex and caused progressive, shallow rhegmatogenous retinal detachments with anterior chamber cells and elevation of intraocular pressure. Surgeries with scleral buckling procedure combined with vitrectomy and lensectomy brought about reattachment of the retina. This case indicates that subtle anomalies in the lens-zonule-ciliary body complex might be an indication of a break in the nonpigmented epithelium of the ciliary body which could lead to rhegmatogenous retinal detachment. The mode of inheritance of the clinical syndrome remained undefined because of negative family history.- - - - - - - - - - ranking = 5keywords = coloboma (Clic here for more details about this article) |
7/23. Dynamic atypical optic nerve coloboma associated with transient macular detachment.PURPOSE: Macular schisis or detachment is frequently observed in eyes with optic pits or colobomas. Although spontaneous resolution of the maculopathy has been reported, concurrent changes in the optic nerve coloboma have not. We report three cases of atypical optic nerve colobomas in which dynamic optic nerve changes coincide with the development and subsequent resolution of the associated maculopathy. methods: We reviewed the records of three patients with dynamic optic nerve changes associated with maculopathy. All patients were observed for at least 6 months. Fundus photography and fluorescein angiography were used to document the optic nerve and macular changes. RESULTS: Three patients were noted to have macular detachments without apparent optic nerve excavation. With observation, the maculopathy spontaneously resolved in each case. We documented concurrent optic nerve changes whereby atypical optic nerve colobomas became apparent over several months in all cases. In one case, we noted the simultaneous development of maculopathy in association with obscuration of a prior disc anomaly. None of the eyes had a posterior vitreous detachment. We could not identify any associated systemic conditions or reproduce the findings with external stimulation. Initial Snellen acuity ranged from 20/60 to 20/200. Final Snellen acuity ranged from 20/20 to 20/40. CONCLUSIONS: Fluctuating optic nerve changes may occur in the setting of atypical optic nerve coloboma and associated maculopathy. In cases of macular schisis or detachment where an optic nerve coloboma is not readily apparent, and no other causes are identified, consideration of a period of observation prior to therapeutic intervention seems appropriate.- - - - - - - - - - ranking = 10keywords = coloboma (Clic here for more details about this article) |
8/23. retinal detachment with breaks in the pars plicata of the ciliary body.We studied four eyes with retinal detachment accompanied by a break in the nonpigmented epithelium in the pars plicata of the ciliary body in three patients with atopic dermatitis. Each eye had either ectopia lentis or coloboma of the lens. In the region of the lens malformation, ciliary processes were hypoplastic. These rudimentary ciliary processes were observed only in the anterior half of the pars plicata. At their posterior end, the ciliary nonpigmented epithelium had been ruptured and a break by zonular traction was apparent. In the other region of the pars plicata, ciliary processes were normal and no break was detected. The break in the pars plicata was treated by diathermy application and segmental scleral buckling, without subretinal fluid drainage, and with the resulting reattachment of the retina. Hypoplasia of ciliary processes is basically responsible for the development of a break in the pars plicata.- - - - - - - - - - ranking = 1keywords = coloboma (Clic here for more details about this article) |
9/23. Treatment of sensory retinal detachment associated with optic nerve pit or coloboma.Six eyes with optic nerve pit and associated sensory retinal detachment of the macula were treated using a management plan that utilized a combination of techniques including laser photocoagulation, pars plana vitrectomy, and intraocular air/fluid exchange. In each treated eye the retina was eventually flattened. This paper discusses this plan of management and series of treatment modalities.- - - - - - - - - - ranking = 4keywords = coloboma (Clic here for more details about this article) |
10/23. Treatment of combined traction-rhegmatogenous retinal detachment in the morning glory syndrome.A 14-year-old boy had a near total retinal detachment associated with the morning glory optic disc anomaly. A tiny slit-like retinal break with adjacent vitreous traction was found within the coloboma, providing communication between the vitreous cavity and the subretinal space. vitrectomy, fluid-gas exchange, and postoperative confluent krypton laser photocoagulation around the optic disc resulted in a successful reattachment of the retina. The retina has remained attached for 14 months.- - - - - - - - - - ranking = 1keywords = coloboma (Clic here for more details about this article) |
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