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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 692-693

Subfoveal intrachoroidal cavitation secondary to full-thickness macular hole in a case of retinitis pigmentosa

1 Vitreoretina Consultant, Mahatme Eye Hospital and Eye Bank, Nagpur, Maharashtra, India
2 Cornea Fellow, Mahatme Eye Hospital, Nagpur, Maharashtra, India

Date of Submission31-Aug-2020
Date of Acceptance27-Apr-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Pradeep G Tekade
- C/O NR Ekhar, First Floor, Plot No. 109, Rajeev Nagar, Somalwada, Nagpur, Maharashtra - 440025
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2826_20

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We report a rare case of subfoveal intrachoroidal cavitation secondary to full-thickness macular hole in case of retinitis pigmentosa. Intrachoroidal cavitation was typically described in myopic eyes in peripapillary region and North Carolina macular dystrophy previously. Many authors described hypothesis regarding its pathogenesis but conclusive evidence is not available. We are reporting subfoveal intrachoroidal cavitation associated with retinitis pigmentosa and myopia.

Keywords: FTMH, ICC, intrachoroidal cavitation, macular hole, myopia, retinitis pigmentosa, RP

How to cite this article:
Tekade PG, Namdeo N. Subfoveal intrachoroidal cavitation secondary to full-thickness macular hole in a case of retinitis pigmentosa. Indian J Ophthalmol Case Rep 2021;1:692-3

How to cite this URL:
Tekade PG, Namdeo N. Subfoveal intrachoroidal cavitation secondary to full-thickness macular hole in a case of retinitis pigmentosa. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 21];1:692-3. Available from: https://www.ijoreports.in/text.asp?2021/1/4/692/327654

Freund et al.[1] described a clinical and optical coherence tomography (OCT) finding of a peripapillary detachment in high myopia, which others subsequently named intrachoroidal cavitation (ICC). ICC is rare entity predominantly described around peripapillary area in myopic eyes.[1],[2] ICC is seen in 55.8% of highly myopic eyes with the presence of focal Chorioretinal atrophic patch (CRA) or myopic conus and/or presence of intrascleral vessels near the cavitation.[3] ICC is previously described with North Carolina macular dystrophy.[4]

We want to report a rare finding of subfoveal ICC associated with full-thickness macular hole in case of retinitis pigmentosa and myopia.

  Case Report Top

A 50-year-old female presented to us with gradual progressive diminution of vision since 2 years. She had history of night blindness also. Family history was not significant. Her best-corrected vision was 20/40 in right eye and 20/60 in left eye. Both eyes had refractive error of − 5.50 DS. Intraocular pressure was normal in both eyes. Anterior segment of both eyes was within normal limits. On fundus examination, both eyes had pale disc along with generalised arterial attenuation suggestive of retinitis pigmentosa. Macula had retinal pigment epithelium (RPE) alteration in both eyes and full-thickness macular hole in left eye [Figure 1]. OCT examination showed RPE changes in right eye while left had full-thickness macular hole along with irregular border [Figure 2]. On further exploration of OCT, there was breach in RPE [Figure 3] and hypo-reflective space beneath full-thickness macular hole suggestive of ICC. We counselled her regarding retinitis pigmentosa and we did not intervene for macular hole and kept her under regular follow-up.
Figure 1: Left eye fundus photo showing FTMH with RPE atrophy

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Figure 2: Left eye OCT showing FTMH with subfoveal choroidal cavitation

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Figure 3: Left eye showing RPE breach communicating vitreous cavity with choroidal cavity

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  Discussion Top

ICC was first described in the peripapillary area as a peripapillary detachment in pathologic myopia and described it as an elevated, orange-yellow lesion at the inferior border of the myopic conus. On OCT, ICC is characterised by the presence of a hypo-reflective space beneath RPE.[1] These findings were described by another investigator as ICC[2] or choroidal schisis.[5] However, most of literature have reported ICC in peripapillary area.[6],[7],[8]

As per the study done by Venkatesh et al.,[3] 81% of the ICCs were macular in location, while peripapillary ICC was seen in 19% of the cases. Both the macular and peripapillary ICCs were noted around the region of focal CRA. Study showed that ICC was more frequently noted in older patients with PM, on and around the area of patchy CRA with the presence of intrascleral vessels near the cavitation.[3]

Various theories are described regarding pathogenesis of ICC such as congenital defect,[9] vitreous prolapse,[10] scleral stretching, mechanical stretch of the structurally weaker conus area and the surrounding peripapillary tissue resulting in splitting and mechanical damage of the intrachoroidal structures and formation of hypo-reflective cystoid spaces.[8] Ageing leads to poor absorbing ability by the tissues of the fluid originating from the subretinal, suprachoroidal, optic canal and vitreous cavity.[9] Another theory states that posterior staphyloma may stretch and disrupt the tissue at the edge of myopic conus or patchy CRA. This allows the vitreous fluid to gain access into the three potential spaces: intraretinal space causing retinoschisis, subretinal space causing peripapillary or macular detachment and suprachoroidal space causing ICC at the disc or macula.[6] In pathological myopia, due to the thinning of the choroidal vessels, much of the burden of the ocular venous drainage lies with the intrascleral vessels. As a result, there is an increased transluminal pressure within the intrascleral vessels which leads to the transudation of the fluid from the intrascleral vessels through the overlying thin connective tissue into the intrachoroidal space leading to formation of ICC.[11],[12],[15],[16],[17] This should not be confused with focal choroidal excavation in which retinal pigment epithelium gets separated from photoreceptors as RPE in this case is at normal anatomical location without separation from photoreceptors.[13],[14]

Interesting fact about our case is its subfoveal location and association with full-thickness macular hole and retinitis pigmentosa. We hypothesise that in this case ICC was secondary to RPE breach and full thickness macular hole (FTMH).

  Conclusion Top

We report a case of subfoveal ICC secondary to full-thickness macular hole in case of retinitis pigmentosa and myopia.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Freund KB, Ciardella AP, Yannuzzi LA, Pece A, Goldbaum M, Kokame GT, et al. Peripapillary detachment in pathologic myopia. Arch Ophthalmol 2003;121:197-204.  Back to cited text no. 1
Toranzo J, Cohen SY, Erginay A, Gaudric A. Peripapillary intrachoroidal cavitation in myopia. Am J Ophthalmol 2005;140:731-2.  Back to cited text no. 2
Venkatesh R, Jain K, Aseem A, Kumar S, Yadav NK. Intrachoroidal cavitation in myopic eyes. Int Ophthalmol 2020;40:31-41.  Back to cited text no. 3
Schoenberger SD, Agarwal A. Intrachoroidal cavitation in North Carolina macular dystrophy. JAMA Ophthalmol 2013;131:1073-6.  Back to cited text no. 4
Tateno H, Takahashi K, Fukuchi T, Yamazaki Y, Sho K, Matsumura M. Choroidal schisis around the optic nerve in myopic eyes evaluated by optical coherence tomography. Jpn J Clin Ophthalmol 2005;59:327-31.  Back to cited text no. 5
Wei Y-H, Yang C-M, Chen M-S, Shih Y-F, Ho T-C. Peripapillary intrachoroidal cavitation in high myopia: Reappraisal. Eye Lond Engl 2009;23:141-4.  Back to cited text no. 6
Spaide RF, Akiba M, Ohno-Matsui K. Evaluation of peripapillary intrachoroidal cavitation with swept source and enhanced depth imaging optical coherence tomography. Retina Phila Pa 2012;32:1037-44.  Back to cited text no. 7
Shimada N, Ohno-Matsui K, Nishimuta A, Tokoro T, Mochizuki M. Peripapillary changes detected by optical coherence tomography in eyes with high myopia. Ophthalmology 2007;114:2070-6.  Back to cited text no. 8
Holak SA, Holak N, Holak HM. Peripapillary choroidal cavitation. Ophthalmology 2014;121:e6-7.  Back to cited text no. 9
Fellman RL, Grover DS. Myopic peripapillary sinkhole: Prolapse of retinal nerve fiber layer and posterior vitreous into a sclerochoroidal hollow causing peripapillary choroidal thickening and cavitation. Arch Ophthalmol Chic Ill 1960 2012;130:1220-1.  Back to cited text no. 10
Chen Q, He J, Hua Y, Fan Y. Exploration of peripapillary vessel density in highly myopic eyes with peripapillary intrachoroidal cavitation and its relationship with ocular parameters using optical coherence tomography angiography. Clin Exp Ophthalmol 2017;45:884-93.  Back to cited text no. 11
Mazzaferro A, Carnevali A, Zucchiatti I, Querques L, Bandello F, Querques G. Optical coherence tomography angiography features of intrachoroidal peripapillary cavitation. Eur J Ophthalmol 2017;27:e32-4.  Back to cited text no. 12
Margolis R, Mukkamala SK, Jampol LM, Spaide RF, Ober MD, Sorenson JA, et al. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320-5.  Back to cited text no. 13
Hirawat RS, Nagesha CK, Poddar C. Focal choroidal excavation with macular hole in a case of advanced retinitis pigmentosa. Indian J Ophthalmol 2020;68:2262.  Back to cited text no. 14
[PUBMED]  [Full text]  
Farjad H, Besada E, Frauens BJ. Peripapillary schisis with serous detachment in advanced glaucoma. Optom Vis Sci 2010;87:E205-17.  Back to cited text no. 15
Yeh S-I, Chang W-C, Wu C-H, Lan Y-W, Hsieh J-W, Tsai S, et al. Characteristics of peripapillary choroidal cavitation detected by optical coherence tomography. Ophthalmology 2013;120:544-52.  Back to cited text no. 16
Markan A, Handa S, Dogra M, Singh R. “Macular sink hole” with intrachoroidal cavitation in a case of pathological myopia. Indian J Ophthalmol 2020;68:2296-8.  Back to cited text no. 17
[PUBMED]  [Full text]  


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