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Resolution Ofan External Layer Macular Hole Associated with An BritishJournal ofOphthalmology 1993; 77: 457-459 457 Resolution of an external layer macular hole associated with an optic nerve pit after laser Br J Ophthalmol: first published as 10.1136/bjo.77.7.457 on 1 July 1993. Downloaded from photocoagulation Philip M Falcone, Peter L Lou Optic nerve pits are estimated to occur in 1 in 11000 patients.'2 Maculopathy associated with optic nerve pits occurs in 30%-63%23 and is characteristically described as a serous retinal detachment. Brown et al3 reviewed 75 patients with optic nerve pits and found that 24 of the 38 patients with macular detachments had cystic changes in the macula. Lamellar macular holes were present in the outer layer of the retina in 10 ofthe 24 eyes. Lincoff et al noted an associated retinoschisis in patients with optic pits. A macular hole was observed in the outer retinal layer in 14 out ofthe 15 eyes examined and was associated with severely diminished visual acuity. We now present a case of an optic nerve pit Figure 2 Earlyframefluorescein angiography discloses with retinoschisis and a outer layer detachment hypofluorescence ofthe optic nerve pit with an RPE with an external layer macular hole that was transmission defect in thefovea. treated promptly with krypton red barrier photocoagulation. The marked visual improve- had a 0-2 cup/disc ratio with a optic pit at the ment seen in our patient corresponded to the temporal border. A schisis-like elevation of the resolution of the external layer macular hole retina was observed extending temporally from observed clinically and on fluorescein the optic pit to the macula area. A localised angiography. serous retina detachment of the macula was present but did not appear to communicate with the disc. At the fovea, a 700 im external layer Case report hole was present. The external layer hole had http://bjo.bmj.com/ In February 1989, a 45-year-old woman was scalloped edges which turned inward towards referred after complaining of blurred vision in the overlying intact retinal layers (Fig 1). her left eye over a 1 week period. She had no Fluorescein angiography revealed early hypo- significant medical or ocular history. She fluorescence of the temporal margin of the disc reported to have enjoyed excellent vision in both with late leakage, consistent with the diagnosis of eyes throughout her life. The examination an optic nerve pit. A retinal pigment epithelium showed best corrected visual acuities to be 20/20 (RPE) window defect was present in the macula on September 29, 2021 by guest. Protected copyright. in the right eye and counting fingers at 6 feet in corresponding to the external layer observed the left eye. Amsler grid testing. revealed a clinically (Figs 2 and 3). Testing with laser central scotoma in the left eye. External and slit- scotometery4 revealed an absolute scotoma using lamp examination were unremarkable. The right 50 Fm spot size at the highest intensity. fundus appeared normal. The left optic nerve Given the poor visual acuity and macular Department of Ophthalmology, Massachusetts Eye and Ear Infirmary/Harvard Medical School, Boston, MA, USA P M Falcone P L Lou Correspondence to: Philip M Falcone, MD, West Reading Ophthalmic Associates, 206 South Sixth Avenue, West Reading, PA 19610, USA. Figure I Optic nerve pit with peripapillary retinoschisis. A Figure 3 Lateframefluorescein angiography reveals Accepted for publication serous detachment ofthe macula is present with a 700 tsm leakagefrom the optic nerve pit. Note the intact inner retinal 8 March 1993 external layer hole. layer overlying the lamellar macular hole. 458 Falcone, Lou Br J Ophthalmol: first published as 10.1136/bjo.77.7.457 on 1 July 1993. Downloaded from Figure 4 After treatment, the retina has reattached with Figure S Mid-venous phase ofthefluorescein angiogram. peripapillary RPE change. Mildpigment mottling is seen in There is resolution ofthe RPE window defect. the otherwise normal appearing macula. change, treatment was initiated with krypton red Cox8 reviewed various therapeutic modalities photocoagulation. Using 200 tm spot size, two in the treatment of macular detachments with rows oflaser were placed concentric to the disc in optic nerve pits. Only two patients were described a barrier fashion previously described56 from 12 with symptomatology of 4 months or less in o'clock clockwise to 6 o'clock extending into duration. Both patients regained better than 20/ attached retina both above and below the disc. 50 vision after prompt intervention combining Thepatientreturned forfollow up examination pars plana vitrectomy with gas tamponade and in March 1989. The vision in her left eye laser photocoagulation. improved to 20/160. Laser scars were evident in In our patient treatment with laser photo- the peripapillary retina but there was otherwise coagulation alone resulted in reattachment of the negligible change in the appearance of the retina with resolution of both the macular macula. Additional photocoagulation was detachment and presumed external layer macular applied in a row temporal to the area previously hole. The markedly improved visual acuity may treated. be due to either ofthese events independently or In April 1989, 6 weeks after her initial treat- in association. However, the absence of the ment, the patient reported improved vision. On previously noted central scotoma on both laser examination, visual acuity in the left eye was scotometry and Amsler grid testing confirms measured at 20/60. The schisis-like change that the macular hole did in fact resolve. http://bjo.bmj.com/ surrounding the disc had resolved along with the Histopathologically, a macular hole represents external layer detachment and external layer an area devoid of photoreceptors.9 Since closure hole. The central fovea exhibited pigment ofthehole may cause reabsorption ofsurrounding mottling (Fig 4). Fluorescein angiography in the subretinal fluid and oedema, this may result in macular region appeared normal (Fig 5). return ofgood vision assuming the neighbouring The patient has been followed for 2 years and photoreceptors are functioning.10 the retina has remained attached. At the last Current treatment recommendations for on September 29, 2021 by guest. Protected copyright. follow up examination, the vision in the left eye treating an optic nerve pit-related sensory retinal was 20/40. The patient reported a small supero- detachment suggest observation for 3 months temporal scotoma on Amsler grid testing and did unless there is macular pathology (such as foveal not note any scotoma upon testing with laser cyst or window defect).6 Our patient was treated scotometry. after 1 week of pronounced visual loss. The recovery of good visual acuity with evidence of resolution ofthe external layer macular hole after Comment prompt laser photocoagulation supports the Macular holes complicating optic pit detach- premise that early reattachment ofthe retina may ments were first described by Reis' in 1908. play a role in preventing photoreceptor Sugar7 reported nine cases of macular pathology degeneration." associated with optic nerve pits. One patient Resolution of the RPE transmission defect described had what was initially believed to be a seen on fluorescein angiogram suggests that the macular hole, but the return ofgood visual acuity damage to the RPE was temporary or regenera- and the cystic retinal appearance led to the tion of the pigment epithelial layer occurred. author's conclusion that this was a 'false hole'. Absence of RPE presumably does not preclude Lincoff and colleagues,4 using stereoscopic good visual acuity. 12 techniques, discovered that 13 of 15 eyes they We must consider that our patient may have examined with optic nerve pits and macular had spontaneous reattachment of the macula and detachments had a schisis-like retinal elevation our intervention had little to do with the eventual that communicated with the optic pit and 14 out outcome. Although this event cannot be ruled ofthe 15 eyes had external layer lamellar macular out, the poor natural course in the majority of holes. The internal limiting membrane remained optic pit detachments with the marked intact in every patient and none of the holes anatomical and clinical improvement in our reached full thickness. patient within 6 weeks ofinitial laser surgery lead Resolution ofan external layermacular hok associatedwith an optic nervepitafterlaserphotocoagulation 459 us to believe that there was indeed a treatment 5 Brockhurst RJ. Optic pits and posterior retinal detachment. Trans Am Ophthalmol Soc 1975; 73: 264-88. effect. 6 Schatz H, McDonald HR. Treatment of sensory retinal Since reattachment ofthe retina and resolution detachment associated with optic nerve pit or coloboma. Ophthalmology 1988; 95: 178-86. ofthe macular hole was achieved solely with laser 7 Sugar HS. Congenital pits in the optic disc and their equiva- Br J Ophthalmol: first published as 10.1136/bjo.77.7.457 on 1 July 1993. Downloaded from photocoagulation, we believe this should be the lents (congenital coloboma and colobomalike excavations) associated with submacular fluid. Am J Ophthalmol 1967; initial procedure in the management of these 63:298-307. patients. 8 Cox MS, Witherspoon CD, Morris RE, Flynn HW. Evolving techniques in the treatment of macular detachment caused by optic nerve pits. Ophthalmology 1988; 9S: 889-96. 9 Frangieh GT, Green WR, Engle HM. A histopathologic study 1 Reis W. Eine wenig bekannte typische missbildung am ofmacula cysts and holes. Retina 1981; 1: 311-36. sehnerveneintritt: umnschiebene grubenbildung auf papilla 10 Fine SL. Vitreous surgery for macula hole in perspective. Is n optici. ZeitschrAugenheilkd 1908; 19: 505-28. there an indication? Arch Ophthalmol 1991; 109: 635-6. 2 Kranenburg EW. Crater-like holes in the optic disc and central 11 Kelly NE, Wendell RT. Vitreous surgery for idiopathic serous retinopathy. Arch Ophthalmol 1960; 64: 912-28. macular holes. Results of a pilot study. Arch Ophthalmol 3 Brown GC, Shields JA, Goldberg RE. Congenital pits of the 1991; 109:654-9.
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