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Retinitis Pigmentosa Associated with Ectopia Lentis

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Retinitis Pigmentosa Associated with Ectopia Lentis CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES SECTION EDITOR: W. RICHARD GREEN, MD tion of the oil progressing through the In January 2001, glaucoma sur- Silicone Oil Egressing tube and histopathologic analysis of gery was needed to control elevated Through an Inferiorly the orbital tissue surrounding the ex- intraocular pressure (IOP). The eye Implanted Ahmed Valve truded silicone oil. was aphakic and had total traumatic aniridia. An Ahmed valve was im- Silicone oil use as an adjunct to com- Report of a Case. A 69-year-old white planted inferonasally in an attempt plicated vitreoretinal surgery is be- man lost his left eye to trauma at age to avoid the silicone oil bubble coming more frequent. Refractory 12 years. In September 2000, blunt (Figure 1 and Figure 2). The pa- glaucoma in these patients is com- trauma resulted in a lacerated eye- tient’s IOP responded well initially mon. Isolated reports have men- brow, scleral rupture, uveal prolapse, but rose subsequently to 30 mm Hg. tioned the possibility of silicone oil extrusion of his crystalline lens, reti- A bubble of silicone oil was wrap- migrating and/or obstructing the nal detachment, and suprachoroidal ping the tip of the tube (Figure 3). tube in the anterior chamber of Mol- hemorrhage in his right eye. A limited Silicone oil could be seen migrating teno implants (IOP, Costa Mesa, anterior chamber washout was per- through the Ahmed tube (Figure 4 Calif).1,2 This report describes a case formed at the time of the primary re- and Figure 5) and the bleb over the of intraocular silicone oil egressing pair. Ten days later, he underwent implant progressively enlarged and through an Ahmed implant (New pars plana vitrectomy, silicone oil in- appeared encapsulated during the World Medical, Rancho Cucamonga, jection, and a scleral buckle. A pars next few months. A glistening mate- Calif), impairing the functioning of plana vitrectomy revision with endo- rial was noted in cystic spaces over- the tube and requiring replacement laser,membranestripping,andsilicone lying the Ahmed implant under the of the implant plus oil removal. We oilreinjectionwereperformed1month conjunctiva. An inferior ectropion present photographic documenta- laterforarecurrentretinaldetachment. that progressed gradually was also Figure 1. Slitlamp photograph showing the Ahmed tube inferonasally short Figure 2. Retroillumination photograph showing a patent Ahmed tube. after implantation. Notice total traumatic aniridia and superotemporal paralimbal scleral wound with interrupted sutures. Figure 3. Slitlamp photograph showing “candle wax” appearance of the Figure 4. Retroillumination photograph showing a level of silicone oil silicone oil wrapped around the tip of the Ahmed tube. (arrow) inside the Ahmed tube. (REPRINTED) ARCH OPHTHALMOL / VOL 120, JUNE 2002 WWW.ARCHOPHTHALMOL.COM 831 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Figure 5. Retroillumination photograph taken on a different day shows a Figure 6. Histopathologic examination of orbital tissue excised during different level of silicone oil (arrow) inside the Ahmed tube. removal of valve shows empty vacuoles consistent with silicone oil and larger deposit of oil surrounded by epithelioid histiocytes and foreign body giant cells (hematoxylin-eosin, original magnification ϫ200). noted. The volume of the silicone the migration of the silicone oil It seems that a “unicameral” eye bubble in the vitreous cavity de- through the Ahmed implant. The in- with silicone oil, particularly with sig- creased from an estimated 85% fill to flammatory reaction observed in the nificant iris defects, is a poor candi- an estimated 50% fill. Ectropion re- periocular tissues, apparently caused date for successful IOP control with pair was necessary in June 2001. by the silicone oil, has been docu- a seton in a 1-stage procedure. In our Owing to persistently elevated mented before.1,2 This contrasts with case, the inferior location of the im- IOP measurements, transcorneal re- no observed clinical reaction in in- plant did not prevent silicone oil moval of the silicone oil combined traocular tissues, although histo- movement out of the eye with sec- with replacement of the Ahmed im- pathologically foreign-body granu- ondary impairment of IOP control. plant was performed. Multiple sili- lomas have been documented in Silicone oil removal needs to be con- cone oil–filled conjunctival cysts intraocular tissues. sidered prior to implantation of a se- were found surrounding the Ahmed The silicone oil did impair the ton in such cases. If silicone oil re- plate. A tissue sample was taken in- drainage of aqueous through the im- moval is not an option, diode laser ferotemporally from a thick cap- plant as evidenced by the elevated cyclophotocoagulation is another al- sule surrounding the Ahmed im- IOPs. Encapsulation of the bleb might ternative for IOP control. plant. Histopathologic analysis of the also have contributed to the obstruc- tissue surrounding the plate dem- tion of the implant. The photographs Jose Morales, MD onstrated fibroconnective tissue with (Figures 3 and 4), showing oil at dif- Michel Shami, MD numerous small vacuoles. Surround- ferent levels of the tube, demonstrate Geert Craenen, MD ing this tissue were numerous for- the progression of the silicone oil Thom F. Wentlandt, CRA eign-body giant cells and histio- through the tube of the Ahmed im- Lubbock, Tex cytes (Figure 6). plant. We believe that aphakia with total aniridia resulted in an anatomic Corresponding author: Jose Morales, Comment. To our knowledge, this situation (a truly unicameral eye) that MD, Texas Tech University Health Sci- is one of the first documented cases favored the anterior migration of the ences Center, Department of Ophthal- of silicone oil exiting the eye through silicone oil when the patient inadvert- mologyandVisualSciences,3601Fourth an Ahmed implant. Review of the lit- ently assumed a supine position. This St, STOP 7217, Lubbock, TX 79430- erature yielded 2 previous reports, was probably favored by the well- 7217(e-mail:[email protected]). both involving Molteno implants in known physical attraction of the sili- aphakic patients1,2 and 1 recent re- conetubetowardthesiliconeoil.Once 1. Hyung SM. Min JP. Subconjunctival silicone oil 3 drainage through the Molteno implant. KorJOph- port involving an Ahmed implant. an oil bubble made it to the entrance thalmol. 1998;12:73-75. Minckler4 describes adhesion of the ofthetube,thecombinedeffectofcap- 2. Senn P, Buchi ER, Daicher B, Schipper I. Bubbles silicone oil to the anterior chamber illary action with elevated IOP may in the bleb: troubles in the bleb? molteno im- plant and intraocular tamponade with silicone portion of the drainage tube, resem- have facilitated the migration of the oil in an aphakic patient. Ophthalmic Surg. 1991; bling candle wax, without lumen ob- oil to the subconjunctival space. The 26:379-382. 3. Nazemi PP, Chong LP, Varma R, Burnstine MA. struction. He recommends placing patient’s IOP has been under control Migration of intraocular silicone oil into the sub- the tube in an inferior location to since replacement of the Ahmed im- conjunctival space and orbit through an Ahmed minimize the chance of oil-tube ob- plant and removal of the silicone oil. glaucoma valve. Am J Ophthalmol. 2001;132: 929-931. struction. In our case, the inferior lo- His last corrected visual acuity was 20/ 4. Minckler D. Silicone oil glaucoma: cases in con- cation of the tube did not prevent 50 OD. troversy. J Glaucoma. 2001;10:51-54. (REPRINTED) ARCH OPHTHALMOL / VOL 120, JUNE 2002 WWW.ARCHOPHTHALMOL.COM 832 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 olol administered daily. The pa- utes, and then washed in Tris- Lens Dislocation tient’s grandfather, cousin, and buffered saline. The sections were in Marfan Syndrome: mother also had Marfan syndrome. incubated with a 1:5 dilution of pre- Potential Role of Matrix Her mother developed bilateral lens immune serum from the secondary Metalloproteinases in subluxation and glaucoma. A sys- host species. Tissue sections were in- temic examination revealed arachno- cubated with 1:100 goat primary poly- Fibrillin Degradation dactyly and a high arched palate. clonal antifibrillin antibody (Santa Ophthalmologic examination re- Cruz Biotechnology, Santa Cruz, Marfan syndrome is an autosomal vealed a best-corrected visual acuity Calif) and 1:100 mouse primary dominant disorder with pleiotro- of 20/220 OD and 20/120 OS. The monoclonal anti-MMP–1, anti- pic manifestations that involve the right eye had an intraocular pres- MMP–2, anti-MMP–3, anti-MMP–9 ocular, cardiovascular, and skeletal sure of 19 mm Hg; and the left eye, (ICN Pharmaceuticals, Costa Mesa, systems. Marfan syndrome re- 18 mm Hg. Goldmann visual fields Calif), 1:100 TIMP-1, TIMP-2 (ICN), mains primarily a clinical diagno- were normal. Slitlamp examination and 1:100 TIMP-3 (Calbiochem, San sis with a frequency of 2 to 3 indi- confirmed bilateral superonasal lens Diego, Calif) overnight at 4°C. The viduals per 10000. Patients with this subluxation that was worse in the sections were then washed in 0.05M disorder may have a variety of ocu- right eye than in the left (Figure). Tris-buffered saline (pH, 7.6) before lar complaints, most commonly, The fundus was normal, with cup- the addition of a biotinylated rabbit subluxation of the lens, which oc- disc ratios of 0.2 for each eye. The antigoat secondary antibody (for curs in more than 60% of patients.1 ocular examination was otherwise fibrillin) and biotinylated goat anti- Several studies have identified the normal. Because of the advanced zo- mouse secondary antibody (for MMPs FBN1 fibrillin gene located on chro- nular dialysis, bilateral intracapsular and TIMPs). The antibodies di- mosome 15 as defective in this syn- lens extractions were performed with rected against human antigens dis- 2 ␣ drome. a cryoprobe and without -chymo- play no cross-reactivity and are all IG1 Matrix metalloproteinases trypsin.
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