|Year : 2013 | Volume
| Issue : 2 | Page : 112-115
Surgery for macular holes associated with unusual concomitant pathologies
Dhanashree Ratra, Vishal Raval
Department of Vitreoretinal Services, Bhagawan Mahavir Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India
|Date of Web Publication||19-Aug-2013|
Apollo Hospitals, 154/11, Opp IIM, Bannerghatta Road, Bangalore - 560 076, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Macular hole (MH) is uncommonly seen in association with retinitis pigmentosa (RP), optic disc pit or choroidal coloboma. The visual disability in eyes with RP, optic disc pit or choroidal coloboma can range from mild to severe. However, the macula may not be involved in the primary pathology and hence, the central vision may be preserved in these eyes. If a MH develops in such eyes the central vision is lost, making the patient severely vision disabled. A perceived poor prognosis in such patients may lead to no intervention. However, an attempt should be made to repair and restore the macula in these situations in order to conserve the vision and limit disability. These eyes can however pose unique surgical challenges. We report our experiences of operating on cases of MH associated with unusual concomitant pathologies such as RP, choroidal coloboma, and optic pit. The outcomes and the surgical challenges differed greatly from the conventional idiopathic MH surgery.
Keywords: Choroidal coloboma, macular hole, optic disc pit, retinitis pigmentosa, vitrectomy
|How to cite this article:|
Ratra D, Raval V. Surgery for macular holes associated with unusual concomitant pathologies. Oman J Ophthalmol 2013;6:112-5
| Introduction|| |
It is quite uncommon to have a macular hole (MH) in the presence of concomitant disease conditions such as retinitis pigmentosa (RP), choroidal coloboma, and optic disc pit. RP causes gross restriction of the peripheral visual field with nyctalopia. A choroidal coloboma and an optic disc pit each can cause a large visual field defect. However, these patients may still maintain good central vision if the macula is not involved in the disease condition. However, the presence of a MH greatly affects the vision in such eyes, rendering the patients severely disabled. Various theories have been put forward, but the exact cause of MH development in these patients is still uncertain. In some patients of RP, associated cystoid macular edema (CME) secondary to chronic ischemia resulting from poorly perfused foveal capillaries may be the predecessor to MH formation.  Likewise, a schisis of the inner and outer retinal layers due to chronic leakage from an optic disc pit can result in an outer lamellar or full thickness MH.  Other factors responsible for MH formation are traction by condensed vitreous and glial proliferation along the internal limiting membrane (ILM). In some cases of choroidal coloboma the macula may not be involved in the colobomatous defect. In such cases; however, a MH is quite uncommon. Retinal breaks are more commonly seen in the colobomatous area or in the peripheral retina.  An attempt should be made to repair and restore the macula in these cases in order to conserve the vision and limit disability. However, surgical repair of the MH may be challenging in the presence of such concomitant pathologies. A PubMed based literature search revealed only single case reports of MH surgery with coloboma  and optic pit  each. Reports of MH surgery associated with RP are also very few. , We report the results of MH surgery in eyes with RP (three cases), optic disc pit (one case) and choroidal coloboma (one case).
| Case Reports|| |
A 55-years-old woman, known to have RP and CME, sought an opinion concerning recent decrease in vision of the right eye. Best corrected visual acuity (BCVA) in the right eye was 20/800. Fundus examination demonstrated multiple bony spicule-like pigmentation in the mid-peripheral region. A full thickness macular hole (FTMH) 350 microns in diameter with surrounding cuff of subretinal fluid and yellowish deposits at the base, was noted in the right eye [Figure 1]a. The left eye showed retinal pigment alterations in the macular area [Figure 1]b. Electroretinogram in both eyes showed reduced scotopic and photopic responses [Figure 1]c. Optical coherence tomography (OCT) showed a FTMH with cystic spaces in the right eye [Figure 1]d and inner lamellar defect with epiretinal membrane in the left eye [Figure 1]e The patient underwent 23 G pars plana vitrectomy. After posterior vitreous detachment induction, the ILM was stained with trypan blue dye (0.06%) for a contact time of 2 min. ILM peeling was carried out from arcade to arcade. Gas tamponade with C3F8 (14%) was used. The patient was advised prone positioning for 14 h/day for 2 weeks. At 2 months follow-up, visual acuity improved to 20/200. Post-operative OCT showed closure of MH with regaining of foveal contour [Figure 1]f. However, thinning of inner segment/outer segment junction of the photoreceptors, and retinal pigment epithelial alterations were noted.
|Figure 1: (a) Fundus photograph showing a full thickness macular hole (FTMH) with a cuff of subretinal fluid in the right eye. (b) Fundus photograph showing retinal pigment alterations in the macular area of the left eye. There is mild disc pallor, arteriolar attenuation and tapetoretinal reflex in both the eyes. (c) Electroretinogram showing reduced scotopic and photopic responses in both the eyes. (d) Optical coherence tomography (OCT) showing FTMH with cystic spaces in right eye. (e) OCT showing inner lamellar defect with epiretinal membrane in left eye. (f) OCT showing closure of macular hole in right eye with thinning of the inner segment/outer segment region|
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A 54-years-old man was diagnosed with RP 30 years back. He presented with recent onset of decreased vision in the left eye. BCVA was 20/80 in the left eye. Fundus examination revealed typical RP features. A FTMH was noted in the left eye. Same technique as in case one was followed for the surgical repair. At 2 months, follow-up ophthalmoscopy showed non-closure of MH; BCVA was 20/120 in the left eye.
A 32-year-old man with high myopia had a history of RP in both the eyes since 6 years of age. He presented with diminution of vision in the right eye of 1 year duration. BCVA in the right eye was 20/120. Typical RP features were associated with a FTMH in the right eye and retinal pigment epithelial (RPE) atrophy at central macula in the left eye. Same technique as in case one was followed for the surgical repair. At 2 months follow-up, ophthalmoscopy showed type 2 closures of the MH with flat edges. BCVA noted was 20/200 in the right eye. One year later he underwent phacoemulsification with intraocular lens (IOL) implantation and yttrium aluminium garnet laser (YAG) capsulotomy 4 years later. Last follow-up at 5½ years from the surgery showed type 2 closure of MH; BCVA was 20/2000 in the right eye.
A 22-year-old man presented with blurring of vision in the right eye for 1 month. BCVA was 20/800 in the right eye. He had no perception of light in the left eye. Dilated right eye fundus examination demonstrated inferior choroidal coloboma involving the disc with a FTMH at the edge of the coloboma in the uninvolved macula [Figure 2]a. The MH was horizontally oval in shape, 625 microns in size with surrounding cuff of subretinal fluid. OCT showed large FTMH with shallow retinal detachment nasal to the hole [Figure 2]a. A 20 G vitrectomy with lensectomy was performed. Posterior vitreous detachment was induced from the superior part of the attached retina. Vitreous was an adherent at the edge of coloboma. ILM was stained using trypan blue (0.06%) for contact time of 2 min. ILM peeling was carried out using ILM peeling forceps. Two to three rows of endolaser were applied along the edge of choroidal coloboma as a prophylactic measure considering that the patient has only one functioning eye. Encircling scleral band was secured followed by silicon oil injection. At 2 months and 4 months follow-up, OCT showed flat edges of MH with decreased diameter (type 2 closure) [Figure 2]b and c. At 7 months silicon oil removal was carried out. OCT showed type 1 closure of MH with a thin neuronal tissue bridging across the fovea [Figure 2]d. With low-vision aid BCVA was 20/160.
|Figure 2: (a) Fundus photograph showing inferior choroidal coloboma involving the disc with a full thickness macular hole (FTMH) at the edge of the coloboma in the uninvolved macula while Optical coherence tomography (OCT) shows large FTMH with shallow retinal detachment nasal to the hole. (b and c) At 2 months and 4 months follow-up OCT showed flat edges of macular hole (MH) with decreased diameter (type 2 closure). (d) OCT showing type 1 closure of MH with a thin neuronal tissue bridging across the fovea after 7 months|
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A 33-year-old man presented with gradual, progressive diminution of vision in the right eye for 1 year. BCVA was 20/60 in the right eye. The left eye showed clinical anophthalmos. Fundus examination of the right eye showed optic disc pit in the temporal part of the optic nerve head. A large FTMH with surrounding neurosensory detachment was noted [Figure 3]a. FFA confirmed optic disc pit with no evidence of leakage from any other point [Figure 3]b. OCT showed FTMH with schisis like separation of outer and inner retinal layers with large subretinal fluid collection [Figure 3]c and d. The apex diameter of the hole was 526 microns. The patient underwent three-port pars plana vitrectomy. After inducing posterior vitreous detachment, the ILM was stained using 0.25 mg/ml Brilliant Blue G for 2 min and then peeled. C3F8 (14%) was used as a tamponade. At 3 months follow-up, fundus examination showed non-closure of MH. Spectral domain optical coherence tomography (SD-OCT) showed flattening of the edges of the hole as well as the neurosensory elevation [Figure 3]e. However, the isolated pockets of intraretinal fluid persisted. At last follow-up BCVA was 20/80.
|Figure 3: (a) Fundus photograph showing optic disc pit in the temporal part and a large full thickness macular hole (FTMH) with surrounding neurosensory detachment in the right eye. (b) Fluorescein angiogram in the late phase confirmed optic disc pit with no evidence of leakage. (c) Optical coherence tomography (OCT) showing FTMH with schisis like separation of outer and inner retinal layers with large subretinal fluid collection. (d) OCT of the optic disc showing connection between the pit and the subretinal space. (e) OCT showing flattening of the edges of the hole as well as the neurosensory elevation|
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| Discussion|| |
The current treatment of MH is aimed at relieving tangential vitreoretinal forces and re-approximation of the elevated edges with intraocular tamponade. With the advances in vitreoretinal techniques such as ILM peeling and gas tamponade, the success rate of idiopathic MH surgery has improved remarkably.  MH associated with unusual concomitant pathologies pose greater surgical challenge as compared to idiopathic MH surgery. In these patients the peripheral visual acuity is already compromised due to underlying disease and associated MH can lead to loss of remaining central visual acuity. In most of such patients MH surgery is not attempted due to perceived poor anatomical and functional results. With better understanding of the etiopathogenesis of MH formation and advances in vitreoretinal surgery, it is worth attempting surgery in these patients.
The development of a MH in patients with RP has been attributed to traction due to condensed vitreous strands and incomplete vitreous detachment.  Furthermore in some cases a recalcitrant, chronic CME that is often associated with RP may also be responsible for MH formation.  In our study, among the three patients with RP, two revealed incomplete posterior vitreous detachment and one patient had long standing CME. The surgical results were satisfactory in the patient with CME. The MH closed and the vision improved despite the presence of preoperative longstanding CME. The other two patients with RP and MH; however, failed to show good results. In case three, despite closure of the MH, the vision deteriorated due to progressive atrophic changes in the macula. This patient also had high myopia. Associated high myopia has been thought to be responsible for surgical failure.  Saperstein  and Jin et al.,  have reported good surgical outcome in patients of RP with MH.
Choroidal coloboma causes distortion of the posterior pole of the retina and many times it may be difficult to identify the fovea. In our patient (case no. four), a hole was seen in the area temporal to the disc, along the edge of the coloboma, which we identified as the macula based on visual fixation and vascular pattern of the retina. A large oval hole was seen with a small area of subclinical retinal detachment nasally. The inner neurosensory layers of the retina continue as the intercalary membrane (ICM) over the coloboma. The rest of the layers are absent over the coloboma. Gopal et al.,  have described a transitional zone between the retina and the ICM at the edge of the coloboma. They have also described traction by the ICM leading to a connection between the sub ICM and subretinal space causing a subclinical retinal detachment at the edge of coloboma. Both these factors may have been responsible for a large MH at the edge of coloboma in our patient. The patient showed good result with initial type 2 closure, which later progressed to type 1 closure with good visual improvement. Ahmad and Sheard  have similarly reported good surgical result in a case of MH with choroidal coloboma.
MH associated with optic disc pit poses a difficult challenge as there is schisis like separation of inner and outer retinal layers with subretinal fluid collection.  Post-operatively there was complete resolution of subretinal fluid and flattening of the edges without closure of the MH. Persisting communication with the optic disc pit led to persistence of the intra-retinal fluid spaces. Bechmann et al.,  reported a case of optic disc pit with MH which required three attempts at closure. They recommend long term tamponade with silicone oil in such cases. The role of argon laser photocoagulation to obliterate the communication between the pit and the serous macular detachment has also been reported. 
| Conclusion|| |
Despite variable results it is worth attempting surgery for MH in the presence of other ocular co-morbidities such as RP, coloboma or optic pit. Successful restoration of remaining central visual acuity not only will improve their vision, but also the quality of life.
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[Figure 1], [Figure 2], [Figure 3]