Indian Journal of Ophthalmology - Case Reports

CASE REPORT
Year
: 2021  |  Volume : 1  |  Issue : 4  |  Page : 694--696

Macular buckling and pars plana vitrectomy for retinitis pigmentosa with posterior staphyloma and macular hole


Anshu Arora1, Apoorv Grover2, Akhilesh Aggarwal3,  
1 Consultant, Vitreo Retina Services, Vision First Eye Centre, Noida, Uttar Pradesh, India
2 Director and Consultant, Vitreo Retina and Uveitis Services, Vision Eye Centres, New Delhi, India
3 Consultant, Ophthalmology, Ojas Eye Centre, Bulandshahr, Uttar Pradesh, India

Correspondence Address:
Dr. Anshu Arora
Vision First Eye Centre, D170 A, Sector 50, Noida, Uttar Pradesh
India

Abstract

We describe a case of myopic macular hole (MMH) associated with posterior pole staphyloma (PPS) and foveal detachment (FD) complicated by the presence of Retinitis Pigmentosa (RP). The patient underwent combined macular buckling (MB) and pars plana vitrectomy (PPV). Two month post-operatively, the optical coherence tomography (OCT) showed a closed macular hole with flattened posterior pole. The challenges posed by a FD due to a MMH complicated both by a PPS and RP have been elaborated. A significant anatomical and visual improvement was achieved, providing better central vision in a patient with already compromised peripheral vision.



How to cite this article:
Arora A, Grover A, Aggarwal A. Macular buckling and pars plana vitrectomy for retinitis pigmentosa with posterior staphyloma and macular hole.Indian J Ophthalmol Case Rep 2021;1:694-696


How to cite this URL:
Arora A, Grover A, Aggarwal A. Macular buckling and pars plana vitrectomy for retinitis pigmentosa with posterior staphyloma and macular hole. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Dec 5 ];1:694-696
Available from: https://www.ijoreports.in/text.asp?2021/1/4/694/327719


Full Text



Macular hole (MH) is uncommonly seen in association with retinitis pigmentosa (RP) and presence of coexistent posterior pole staphyloma (PPS) is an additional rarity. With pre-existing loss of peripheral vision due to RP, MH complicates the vision further by loss of central vision and causes further visual disability. A perception of 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 vision and limit disability. This case presents a unique surgical challenge, which was dealt addressing all correctible issues with use of a combination of techniques.

 Case Report



We hereby report a patient with RP presenting with PPS complicating with MH treated with Ab-externo use of macular buckle and ab-interno use of 25-gauge pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling and ILM flap.

A 60-year-old female with no significant systemic complaints presented with blurry vision in her right eye of 2-month duration. She gave a history of poor vision, especially poor night vision since early youth. Her 4-year previous medical records suggested her best corrected visual acuity (BCVA) in right eye was 20/60 and in the left eye was 20/200. OCT (Optical Coherence Tomography) scan done previously [Figure 1] suggested normal foveal contour and vitreoretinal interface in the right eye. Left eye OCT scans however suggested presence of macular retinoschisis explaining her poorer vision.{Figure 1}

On her recent presentation, her right eye BCVA had dropped to 2/60 and that of left eye showed static vision at 20/200. Dilated fundus examination revealed the classic triad of bone spicule pigmentation extending up to arcades, vascular attenuation and disc pallor, suggesting advanced RP. Myopic crescent was noted along temporal margin of optic disc. Right eye macula showed full thickness macular hole (FTMH) with PPS and macular neurosensory detachment. Left eye macula showed dull foveal reflex.

OCT macula [Figure 2] revealed FTMH with increased posterior curvature of the staphyloma in the right eye. The detached retina appeared schitic. Left eye OCT revealed retinoschisis changes. She underwent a macular buckling (Morrin Devin) procedure with pars plana vitrectomy with ILM peeling and inverse ILM flap with perfluoropropane (C3F8) gas tamponade in her right eye. On her last postoperative visit post intraocular gas absorption, she had a BCVA of 20/40 with closed MH. OCT [Figure 3] suggested normal vitreoretinal interface, abolished posterior staphylomatous curvature and a type 1 closed MH with intraretinal hyperreflectivity suggesting approximation over ILM flap. OCT also revealed shadowing due to posteriorly placed scleral buckle with corresponding flattening of posterior pole. Ultrasound B scan [Figure 4]a also showed presence of hypoechoic well defined buckle element behind the globe. Fundus picture [Figure 4]b with red-free image shows closed MH, myopic disc with peripapillary atrophy, and pigmentary retinal changes secondary to RP.{Figure 2}{Figure 3}{Figure 4}

 Discussion



Presence of RP related degenerative changes in a patient with high myopia, PPS, MH, and macular hole associated retinal detachment (MHRD) pose additional challenges with implications for surgery and visual results.

Posterior staphyloma (PPS) is considered an established and important pathological feature for the onset and progression of myopic maculopathy. Excessive axial elongation of the globe and development of PPS can cause mechanical stretching of the ocular structures, accompanied by thinning of the sclera and choroid, resulting in various retinal degenerative changes. PPS is present in 90% of the patients with high myopia.[1]

Among other causative factors, including inner traction due the vitreous cortex, pre-macular structures, ILM, and vascular stiffness, these patients may develop serious complications, such as myopic foveoschisis (MFS) with or without foveal detachment (FD), MH and MHRD.[1]

The mechanism of MH formation in RP cases is yet to be fully understood. As a consequence of chronic blood–retinal barrier breakdown, the retinal inner surface may present with ILM irregularities.[2] Macular cysts tend to be confluent in some cases and, in combination with ILM wrinkling, sometimes may lead to the formation of MH encompassing different stages.[3]

Vitreo-macular interface disorders in RP are known to present concomitantly. Epiretinal membranes (ERMs), vitreo-macular traction (VMT), and MH, both lamellar and full thickness have been reported.[4]

MHRD is the most common indication for performing MB surgery. When done alone, MB relieves the anteroposterior traction only, whereas a combination of vitrectomy and MB allows one to treat tangential and anteroposterior traction of MTM simultaneously.

The largest randomized study of 98 eyes having MHRD with high myopia, which compared PPV (52 eyes) versus “PPV and MB” (46 eyes) conducted by Ma et al.,[5] showed the combined group had faster reattachment and higher hole closure rate during follow-up of 2 years.[5]

Many of the reported MH cases in RP have been operated with variable surgical outcomes.[6] In a study by Hagiwara et al.,[6] PPV was performed on five eyes of RP patients - 2 with MH, with visual improvement noted post operatively in only one patient. The authors concluded that improvement of visual function with vitrectomy may be limited by long-standing retinal dysfunction in RP.

 Conclusion



This rare case highlights the importance of investigating for macular abnormalities in cases of RP, so as to detect treatable pathologies such as FTMH, MFS with or without FD, and also brings out the challenges posed by the association. Treating these macular pathologies by different means to address all outstanding issues may help gain central vision in RP cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2Takezawa M, Tetsuka S, Kakehashi A. Tangential vitreous traction: A possible mechanism of development of cystoid macular edema in retinitis pigmentosa. Clin Ophthalmol 2011;5:245-8.
3Raja M, Goldsmith C, Burton BJ. Spontaneous resolution of full-thickness macular hole in retinitis pigmentosa. BMJ Case Rep 2011;2011:bcr0320113999.
4Fragiotta S, Rossi T, Carnevale C, Cutini A, Tricarico S, Casillo L, et al. Vitreo-macular interface disorders in retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol 2019;257:2137-46.
5Ma J, Li H, Ding X, Tanumiharjo S, Lu L. Effectiveness of combined macular buckle under direct vision and vitrectomy with ILM peeling in refractory macular hole retinal detachment with extreme high axial myopia: A 24-month comparative study. Br J Ophthalmol 2017;101:1386-94.
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