Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report

Purpose: We present a case of macular hole retinal detachment (MHRD) in a patient with Straatsma syndrome, a rare condition characterized by extensive unilateral myelinated retinal nerve fibers (MRNF), high myopia, and amblyopia. This study aimed to highlight the clinical presentations, diagnostic c...

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Main Authors: Ayumi Usui-Ouchi, Nobuyuki Ebihara, Shintaro Nakao
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:American Journal of Ophthalmology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2451993624001786
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author Ayumi Usui-Ouchi
Nobuyuki Ebihara
Shintaro Nakao
author_facet Ayumi Usui-Ouchi
Nobuyuki Ebihara
Shintaro Nakao
author_sort Ayumi Usui-Ouchi
collection DOAJ
description Purpose: We present a case of macular hole retinal detachment (MHRD) in a patient with Straatsma syndrome, a rare condition characterized by extensive unilateral myelinated retinal nerve fibers (MRNF), high myopia, and amblyopia. This study aimed to highlight the clinical presentations, diagnostic challenges, and success of surgical interventions. Observation: A 32-year-old Asian woman with a history of high myopia and poorly corrected vision in her right eye since childhood presented with a sudden loss of vision in the right eye. Examination revealed extensive MRNF and retinal detachment with a macular hole. A standard three-port pars plana vitrectomy was performed, and tight vitreous retinal adhesions were observed. PFCL-assisted inverted internal limiting membrane (ILM) flap technique was performed. Silicone oil was used owing to its tight vitreous retinal adhesion. Postoperatively, the macular hole was closed, the retina was reattached, and partial disappearance of the MRNF was observed. Conclusion and importance: This case report describes a successful surgical intervention for MHRD associated with Straatsma syndrome. The PFCL-assisted inverted ILM flap technique is effective for managing complicated cases of MHRD. The partial disappearance of MRNF after vitrectomy suggests potential nerve fiber layer damage, possibly due to retinal detachment or the use of silicone oil. This case highlights the unique features of MHRD, a rare disease associated with Straatsma syndrome.
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publishDate 2024-12-01
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series American Journal of Ophthalmology Case Reports
spelling doaj-art-c6f69aabdd5b41a1a59edc007b6c9c1c2024-12-18T08:50:39ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362024-12-0136102168Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case reportAyumi Usui-Ouchi0Nobuyuki Ebihara1Shintaro Nakao2Department of Ophthalmology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan; Corresponding author. Department of Ophthalmology, Juntendo University Urayasu Hospital, 2-1-1, Tomioka Urayasu-shi, Chiba, Japan.Department of Ophthalmology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, JapanDepartment of Ophthalmology, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, JapanPurpose: We present a case of macular hole retinal detachment (MHRD) in a patient with Straatsma syndrome, a rare condition characterized by extensive unilateral myelinated retinal nerve fibers (MRNF), high myopia, and amblyopia. This study aimed to highlight the clinical presentations, diagnostic challenges, and success of surgical interventions. Observation: A 32-year-old Asian woman with a history of high myopia and poorly corrected vision in her right eye since childhood presented with a sudden loss of vision in the right eye. Examination revealed extensive MRNF and retinal detachment with a macular hole. A standard three-port pars plana vitrectomy was performed, and tight vitreous retinal adhesions were observed. PFCL-assisted inverted internal limiting membrane (ILM) flap technique was performed. Silicone oil was used owing to its tight vitreous retinal adhesion. Postoperatively, the macular hole was closed, the retina was reattached, and partial disappearance of the MRNF was observed. Conclusion and importance: This case report describes a successful surgical intervention for MHRD associated with Straatsma syndrome. The PFCL-assisted inverted ILM flap technique is effective for managing complicated cases of MHRD. The partial disappearance of MRNF after vitrectomy suggests potential nerve fiber layer damage, possibly due to retinal detachment or the use of silicone oil. This case highlights the unique features of MHRD, a rare disease associated with Straatsma syndrome.http://www.sciencedirect.com/science/article/pii/S2451993624001786Straatsma syndromeMyelinated retinal nerve fiberMacular hole retinal detachmentHigh myopiaVitrectomyRetinal detachment
spellingShingle Ayumi Usui-Ouchi
Nobuyuki Ebihara
Shintaro Nakao
Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
American Journal of Ophthalmology Case Reports
Straatsma syndrome
Myelinated retinal nerve fiber
Macular hole retinal detachment
High myopia
Vitrectomy
Retinal detachment
title Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
title_full Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
title_fullStr Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
title_full_unstemmed Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
title_short Macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with Straatsma syndrome: A case report
title_sort macular hole retinal detachment in extensive myelinated retinal nerve fiber and high myopia with straatsma syndrome a case report
topic Straatsma syndrome
Myelinated retinal nerve fiber
Macular hole retinal detachment
High myopia
Vitrectomy
Retinal detachment
url http://www.sciencedirect.com/science/article/pii/S2451993624001786
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