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 Table of Contents  
DIAGNOSTIC AND THERAPEUTIC CHALLENGES
Year : 2016  |  Volume : 28  |  Issue : 2  |  Page : 133-136

Optic disc maculopathy


Vitreo-Retina Clinic, Little Flower Hospital and Research Centre, Kochi, Kerala, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Awaneesh Upadhyay
Vitreo-Retina Clinic, Little Flower Hospital and Research Centre, Kochi - 356 872, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_14_17

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How to cite this article:
Upadhyay A, Nataraj A. Optic disc maculopathy. Kerala J Ophthalmol 2016;28:133-6

How to cite this URL:
Upadhyay A, Nataraj A. Optic disc maculopathy. Kerala J Ophthalmol [serial online] 2016 [cited 2019 Nov 12];28:133-6. Available from: http://www.kjophthal.com/text.asp?2016/28/2/133/202467




  Case Top


A 15-year-old girl presented with complaints of wavy vision with blurring in the left eye of 2 weeks duration. Past medical and personal history was insignificant. General examination was within normal limits. Ocular examination showed the following:



Anterior segment examination with slit lamp biomicroscopy was within normal limits in both the eyes.

Indirect ophthalmoscopy showed right eye disc and macula normal, CDR 0.3, periphery normal; left eye fundus photo and optical coherence tomography (OCT) findings are shown in [Figure 1],[Figure 2],[Figure 3],[Figure 4],[Figure 5].
Figure 1: Fundus photo right eye

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Figure 2: Fundus photo left eye

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Figure 3: OCT right eye

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Figure 4: OCT left eye

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Figure 5: OCT -disc left eye

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  What Is the Diagnosis and What Are the Treatment Options? Top


Dr. Parag Shah

Senior retina consultant, Aravind eye hospitals, Coimbatore, Tamil Nadu

Diagnosis – Congenital optic nerve head pit

Treatment options

  1. Observation.
  2. Diode laser in papillomacular bundle.
  3. Pars plana vitrectomy with ILM peeling with gas tamponade.
  4. Pars plana vitrectomy and plugging of Optic Nerve Head (ONH) pit with scleral autograft or autologous ILM.


Seeing the size of the macular edema, I would prefer vitrectomy with scleral autograft as it is effective and technically easier than ILM peeling, which may easily cause iatrogenic macular hole in less experienced surgeons

Dr. Arindam Chakravarti

Senior Consultant, Center for Sight, New Delhi

Optic disc pit (ODP) is a rare congenital anomaly of the optic disc, which can be complicated by a maculopathy associated with progressive visual loss. Optic disc pits are usually unilateral and sporadic in occurrence, and the development of maculopathy is unpredictable with no known triggers. Optic disc pit maculopathy is characterized by intraretinal and subretinal fluid at the macula, causing visual deterioration. The source of this fluid is uncertain; several theories have suggested it may be vitreous fluid, cerebrospinal fluid, leakage from blood vessels at the base of the pit, or leakage from the choroid. The mechanism of pathogenesis has not been fully explained, however, vitreous liquefaction and traction and pressure gradients within the eye have been thought to play a role. There are no clear guidelines regarding the management of patients with optic disc pit maculopathy; numerous techniques have been described, including laser photocoagulation, intravitreal gas injection, macular buckling, and pars plana vitrectomy with many different modifications.

Although spontaneous resolution is possible in as many as 25% of the cases and visual improvement is possible, observation will generally lead to significant visual loss. Initially, it has been recommended to wait for spontaneous resolution for up to 3 months before considering surgery, but according to current practices, observation is deemed unjustified. This is especially true in the presence of progression of fluid accumulation or visual loss.

Laser photocoagulation at the temporal disc margin has been proposed as a treatment for optic disc pit maculopathy with the reasoning that the laser scars will create a chorioretinal adhesion, which will act as a barrier between the ODP and the subretinal space. The time for improvement was variable and often long and the location of the laser treatment could also cause significant visual field defects.

Intravitreal gas injection has been proposed as a treatment option for optic disc pit maculopathy with the reasoning that pneumatic displacement will cause reattachment of the macula and improve vision.

An alternative approach proposed for the treatment of optic disc pit maculopathy is macular buckling surgery. This procedure includes an implant that is fixed to the posterior aspect of the globe along the 6–12 o'clock meridian, creating a buckling effect under the macula. This technique has been reported to achieve complete resolution of fluid in approximately 85% of the cases, as well as significant improvements in vision and visual fields.

The predominant approach for the treatment of optic disc pit maculopathy is pars plana vitrectomy (PPV). Several anecdotal reports from the late 1980s and early 1990s described successful anatomical and visual restoration in patients with optic disc pit maculopathy who underwent PPV with or without endolaser to the temporal disc margin and gas tamponade. It has been proposed that induction of complete PVD during surgery is essential for relief of traction required to achieve macular reattachment.

Another controversial aspect is the necessity to peel the internal limiting membrane (ILM) in patients with optic disc pit maculopathy. It has been suggested to be an important component of the surgical treatment of optic disc pit maculopathy, and a few cases of PPV with PVD induction, ILM peeling, and tamponade with gas or air have been reported to achieve successful resolution.

Subretinal drainage, peeling of glial tissue, and sealing of the optic disc pit are controversial.

Limited vitrectomy with intraretinal fenestration is a recently described technique that has shown promising results.

In the above case, although the duration of symptoms is only 2 weeks, the poor presenting best corrected visual acuity and large amount of SRF makes spontaneous resolution unlikely. After a maximum waiting period of 2–3 weeks more, persistence of fluid on OCT and non-improvement of vision would be an indication for surgical intervention.

Dr. Sheshadri Mahajan

Senior Consultant Retina, Hyderabad

This is a case of left eye optic disc pit maculopathy. Although rare and unilateral, it needs intervention as it leads to gradual progressive vision loss and has a poor prognosis.

In such symptomatic cases for patients having best corrected visual acuity (BCVA) >6/12, I prefer laser photocoagulation temporal to disc with C3F8 injection and prone positioning as a first line of management.

Patients having BCVA <6/12, pars plana vitrectomy (PPV) with posterior vitreous detachment (PVD) induction, ILM peeling with laser photocoagulation and autologous scleral graft with C3F8 tamponade works best with successful anatomical outcome and stabilization of progressive vision loss.


  Conclusion Top


The above patient was diagnosed as case of optic disc pit maculopathy (ODP-M) which describe macular changes that occur in the context of an optic disc pit including intraretinal and subretinal fluid accumulation, and retinal pigment changes.[1]

Although spontaneous resolution with improvement in vision has been reported, majority of cases have a poor prognosis, with a natural history of gradual worsening and a final VA of 6/60 or worse.[2]

The source of this fluid is still unclear, and several competing theories have suggested it may be vitreous fluid, cerebrospinal fluid, leakage from blood vessels at the base of the pit or leakage from the choroid.[3],[4] The mechanism of pathogenesis of ODP-M has not been fully elucidated, however, vitreous liquefaction and traction and pressure gradients within the eye have been implicated to be involved.[5],[6]

These cases are challenging to manage. This makes patient counseling, expectation setting, and decision making regarding the timing and choice of surgical intervention very difficult.

There are no clear guidelines on the management of patients with ODP-M, and numerous techniques have been described, including laser photocoagulation, intravitreal gas injection, macular buckling, and pars plana vitrectomy with many different modifications.

The most commonly used procedure for the treatment of ODP-M is Pars plana vitrectomy with or without gas tamponade.[7]

Additional elements of surgery, such as peripapillary laser, ILM peeling, subretinal drainage, peeling of glial tissue and sealing of the ODP are controversial. Both success and failure of surgical management have been reported. All surgical techniques have been reported to achieve complete resolution of fluid and significant visual improvement, but these were documented after at least 3 months and generally only after 6–12 months from the surgery.[8],[9],[10]

This particular case underwent pars plana vitrectomy, ILM peeling, sealing of pit with autologous scleral patch graft, and gas tamponade. There was near complete resolution of subretinal fluid at 1 month with stabilization of vision.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic disc pit: A review. Graefes Arch Clin Exp Ophthalmol 2011;249:1113-22.  Back to cited text no. 1
    
2.
Sobol WM, Blodi CF, Folk JC, Weingeist TA. Long-term visual outcome in patients with optic nerve pit and serous retinal detachment of the macula. Ophthalmology 1990;97:1539-42.  Back to cited text no. 2
    
3.
Ohno-Matsui K, Hirakata A, Inoue M, Akiba M, Ishibashi T. Evaluation of congenital optic disc pits and optic disc colobomas by swept-source optical coherence tomography. Invest Ophthalmol Vis Sci 2013;54:7769-78.  Back to cited text no. 3
    
4.
Kuhn F, Kover F, Szabo I, Mester V. Intracranial migration of silicone oil from an eye with optic pit. Graefes Arch Clin Exp Ophthalmol 2006;244:1360-2.  Back to cited text no. 4
    
5.
Brown GC, Shields JA, Goldberg RE. Congenital pits of the optic nerve head. II. Clinical studies in humans. Ophthalmology 1980;87:51-65.  Back to cited text no. 5
    
6.
Gregory-Roberts EM, Mateo C, Corcóstegui B, Schiff WM, Chang LK, Quiroz-Mercado H, et al. Optic disk pit morphology and retinal detachment: Optical coherence tomography with intraoperative correlation. Retina 2013;33:363-70  Back to cited text no. 6
    
7.
Todokoro D, Kishi S. Reattachment of retina and retinoschisis in pitmacular syndrome by surgically-induced vitreous detachment and gas tamponade. Ophthalmic Surg Lasers 2000;31:233-5.  Back to cited text no. 7
    
8.
Avci R, Yilmaz S, Inan UU, Kaderli B, Kurt M, Yalcinbayir O, et al. Long-term outcomes of pars plana vitrectomy without internal limiting membrane peeling for optic disc pit maculopathy. Eye 2013;27:1359-67.  Back to cited text no. 8
    
9.
Mohammed OA, Pai A. Inverted autologous internal limiting membrane for management of optic disc pit with macular detachment. Middle East Afr J Ophthalmol 2013;20:357-9.  Back to cited text no. 9
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10.
Ooto S, Mittra RA, Ridley ME, Spaide RF. Vitrectomy with inner retinal fenestration for optic disc pit maculopathy. Ophthalmology 2014;121:1727-33.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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