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Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 140-141

Macular hole associated retinal detachment with focal choroidal excavation in myelinated nerve fibre syndrome

Department of Ophthalmology, Max Multi Speciality Centre, New Delhi, India

Date of Submission28-Mar-2021
Date of Acceptance25-Jul-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Neha Goel
D-91, Third Floor, Anand Niketan, New Delhi - 110 021
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_702_21

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The Straatsma syndrome (STAS) consists of unilateral myelinated nerve fibers (MNF), high myopia, and amblyopia. This report describes a patient with total cataract and retinal detachment (RD) who underwent phacoemulsification with pars plana vitrectomy. Intraoperatively, a full-thickness macular hole (FTMH) and MNF were found. The internal limiting membrane peeling was carried out with a silicone oil tamponade. Postoperatively, the reduction in the extent of MNF was noted. The spectral-domain optical coherence tomography revealed type 1 closure of the FTMH with a focal choroidal excavation (FCE). To the best of our knowledge, this myriad of findings has not been reported in the STAS or in association with MNF.

Keywords: Focal choroidal excavation, macular hole, myelinated nerve fibers, retinal detachment

How to cite this article:
Goel N. Macular hole associated retinal detachment with focal choroidal excavation in myelinated nerve fibre syndrome. Indian J Ophthalmol Case Rep 2022;2:140-1

How to cite this URL:
Goel N. Macular hole associated retinal detachment with focal choroidal excavation in myelinated nerve fibre syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:140-1. Available from: https://www.ijoreports.in/text.asp?2022/2/1/140/334971

Myelinated nerve fibers (MNF) of the retina appear as opaque white areas with feathery margins seen in 0.5–1% of the population.[1] They are most often congenital, nonprogressive, and asymptomatic, however, associated conditions may affect the visual function.[1],[2] Straatsma is described as a specific syndrome consisting of the triad of MNF, amblyopia, and high myopia with a frequent association of strabismus that occurs in 10% of the patients with MNF.[3] We describe a case with Straatsma syndrome (STAS) which came to light following surgery for cataract and retinal detachment (RD) associated with a full-thickness macular hole (FTMH). Postoperatively, a focal choroidal excavation (FCE) was also found along with a decrease in the extent of MNF.

  Case Report Top

A 35-year-old male presented with a sudden loss of vision in his left eye for a month. He revealed that there was a poorer vision in this eye since early childhood. There was no history of trauma.

The left eye had an exotropia of 30 prism diopters with full ocular motility and a best-corrected visual acuity (BCVA) of perception of light positive and accurate projection of rays. There was a subtle relative afferent pupillary defect (RAPD) with a total cataract. There was no anterior chamber reaction. The intraocular pressure was 16 mmHg on Goldmann applanation tonometry. The fundus was not visible; the ultrasound B scan showed a total RD. The right eye had an uncorrected visual acuity of 6/6 with no abnormality.

After written informed consent, the patient underwent phacoemulsification with 23-gauge pars plana vitrectomy (PPV). Intraoperatively, MNF were noted along with an FTMH [Figure 1]a. Triamcinolone-assisted posterior vitreous detachment (PVD) was induced followed by Brilliant blue G-assisted internal limiting membrane (ILM) peeling, fluid air exchange, and silicone oil injection.
Figure 1: (a) Intraoperative photograph showing total retinal detachment, myelinated nerve fibers (MNF), and a full-thickness macular hole. (b) One month postoperatively, a decrease in the extent of MNF was observed with silicone oil in situ and an attached retina. The FTMH appeared closed. (c) Near-infrared reflectance (NIRR) imaging showed pigmentary alterations at the macula (d) Spectral-domain OCT confirmed type 1 closure of the FTMH with a conforming focal choroidal excavation (FCE)

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The postoperative period was uneventful. At 1 month follow-up, the BCVA improved to 6/60. The retina was attached with a decrease in the extent of MNF nasal to the disk [Figure 1]b. Near-infrared reflectance (NIRR) imaging revealed pigmentary alterations at the macula [Figure 1]c. Spectral-domain optical coherence tomography (SD-OCT) confirmed type 1 closure of the MH with a conforming FCE. There was thickened retinal nerve fiber layer (RNFL) and hyperreflectivity with backscattering in the area of MNF temporal to the disk [Figure 1]d. Three months postoperatively, silicone oil removal with secondary intraocular lens implantation was performed. The axial lengths were 23.85 and 26.79 mm in the right and left eyes, respectively.

  Discussion Top

This case had several peculiar features. This is the first report of FTMH-associated RD in a patient with MNF and STAS. To the best of our knowledge, only one previous report has described FTMH accompanied by MNF in a 74-year-old male, which closed after two surgeries with macular scarring and atrophy.[4] Ellis et al.[5] found that 83% of the patients with MNF had myopia greater than 6 diopters and postulated that myelination about the macula could have stimulated the development of axial myopia. High myopia confirmed on biometry done postoperatively could have been the risk factor for FTMH and RD in this case.

We noted a decrease in the extent of MNF postoperatively [Figure 1]a and [Figure 1]b. MNF usually remain unchanged over time. The loss of MNF has been reported in association with demyelinating optic nerve disease, primary open-angle glaucoma, Behcet disease, retinal arterial occlusions,[1] and with optic atrophy following chronic papilledema.[6] Williams AJ, et al.[7] documented the loss of a superonasal patch of MNF 9 months following PPV, owing to postoperative nerve fiber layer atrophy or ischemia. Similar mechanisms could have contributed to the decrease in MNF in this case.

A postoperative SD-OCT scan of this case confirmed type 1 closure of the FTMH and revealed a subfoveal conforming FCE. It is characterized by one or more focal depressions in the choroid seen on OCT, in the absence of posterior staphyloma. It can be of conforming type, where the outer retinal layers conform to the choroid, or non-conforming type, where SD-OCT shows a hyporeflective space between the neurosensory retina and retinal pigment epithelium.[8] Only a single case of FTMH with an FCE has been reported in the literature, and that had a successful anatomical and visual outcome following surgery.[9]

  Conclusion Top

In conclusion, we present a unique case of STAS with MH-associated RD and FCE. The RD and MH were treated successfully with a moderate visual gain owing to pre-existing amblyopia. The presence of MNF and FCE did not affect the outcome of the surgery. While these associations may be coincidental, this case is valuable as it may provide insights into a common link between these conditions.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ramkumar HL, Verma R, Ferreyra HA, Robbins SL. Myelinated Retinal nerve fiber layer (RNFL): A comprehensive review. Int Ophthalmol Clin 2018;58:147-56.  Back to cited text no. 1
Tarabishy AB, Alexandrou TJ, Traboulsi EI. Syndrome of myelinated retinal nerve fibers, myopia, and amblyopia: A review. Surv Ophthalmol 2007;52:588-96.  Back to cited text no. 2
Straatsma BR, Heckenlively JR, Foos RY, Shahinian JK. Myelinated retinal fibers associated with ipsilateral myopia, amblyopia and strabismus. Am J Ophthlmol 1979;88:506-10.  Back to cited text no. 3
Minami M, Oku H, Ueki M, Maeno T, Satou B, Ikeda T. Vitreous surgery on a patient with macular hole accompanied by myelinated retinal nerve fibers. Jpn J Ophthalmol 2007;51:306-7.  Back to cited text no. 4
Ellis GS Jr, Frey T, Gouterman RZ. Myelinated nerve fibers, axial myopia, and refractory amblyopia: An organic disease. J Pediatr Ophthalmol Strabismus 1987;24:111-9.  Back to cited text no. 5
Shah M, Park HJ, Gohari AR, Bhatti MT. Loss of myelinated retinal nerve fibers from chronic papilledema. J Neuroophthalmol 2008;28:219-21.  Back to cited text no. 6
Williams AJ, Fekrat S. Disappearance of myelinated retinal nerve fibers after pars plana vitrectomy. Am J Ophthalmol 2006;142:521-3.  Back to cited text no. 7
Margolis R, Mukkamala SK, Jampol LM, Spaide RF, Ober MD, Sorenson JA, Gentile RC, Miller JA, Sherman J, Freund KB. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320-5.  Back to cited text no. 8
Fukumoto M, Morishita S, Okuda Y, Suzuki H, Sato T, Kida T, et al. A case of a vitreomacular traction-associated macular microhole in an eye with focal choroidal excavation. Case Rep Ophthalmol 2015;6:71-5.  Back to cited text no. 9


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