Клиническая картина и исход кровоизлияния на диске зрительного нерва и перипапиллярного субретинального кровоизлияния у трех пациентов

    Introduction

    Intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage (IHAPSH) syndrome, first identified in 1975, is a rare, benign condition predominantly observed in myopic eyes with tilted disks. This syndrome is characterized by acute onset of non-specific visual impairment, floaters, and scotoma [1]. IHAPSH is characterized by a tilted optic disc, mixed types of bleeding, good visual acuity, benign prognosis, and rare recurrence [2]. Most cases regress spontaneously without the need for treatment. However, IHAPSH may be responsible for outer retinopathy like sequela in the long term [3, 4]. However, complicated cases can present diagnostic challenges, leading to unnecessary and incorrect treatments.

    The diagnosis is primarily clinical, based on suspicion, and involves a comprehensive assessment of clinical symptoms and detailed multimodal imaging to confirm the characteristic features associated with this rare clinical syndrome [2]. It must be differentiated from other pathologies that can cause hemorrhage at the optic disk head, like optic disc drusen or peripapillary choroidal neovascularization (CNV) [5, 6]. Accurate diagnosis is essential to prevent unnecessary and inappropriate treatments in this self-resolving benign condition.

    This case series underscores the significance of recognizing IHAPSH in young myopic individuals, highlighting the role of imaging techniques in understanding the pathogenesis and clinical features of this rare condition.

    Patients and methods

    This case series is a single-center, retrospective, observational study that included three patients diagnosed with IHAPSH over the past seven years (table). All patients were monitored at our clinic, and either they or their legal guardians provided informed consent. Patient records were retrieved from our archives, and relevant imaging data were extracted. Cycloplegic refractions were measured via autorefractor (Nidek ARK-510a, Tokyo, Japan). Fundus images and fundus fluorescein angiographies (FFA) were obtained using a Visucam 500 (Carl Zeiss Meditec, Jena, Germany). Optical coherence tomography (OCT) images were captured using a spectral domain OCT system (Heidelberg Spectralis, Heidelberg Engineering, Heidelberg, Germany). Ultrasonographic scans were performed with a Cinescan S (Quantel Medical, Cedex, France), and visual field tests were conducted using the SITA-standard 30-2 protocol on a Humphrey Visual Field Analyzer III (HVF Analyzer III, Carl Zeiss Meditec, Dublin, CA, USA).

    Case presentations

    Case 1

    A 19-year-old female patient presented with subacute visual loss in her left eye. Cycloplegic refraction revealed −1.5 −0.5 × 180 in the right and −1.75 −0.75 × 170 in the left eye, with best corrected visual acuity (BCVA) of 1.0 in the right eye and 0.6 in the left eye. Biomicroscopic examination was unremarkable. Dilated fundus examination, along with OCT imaging, revealed peripapillary subretinal hemorrhage and preretinal hemorrhage (Fig. 1 a, b). Visual field testing revealed enlargement of the blind spot and temporal arcuate scotoma in the affected left eye. Ultrasound imaging indicated mild swelling at the optic disc. Cranial and orbital magnetic resonance imaging (MRI) showed no pathology, and FFA detected intrapapillary hemorrhage with no additional findings. An 8-month follow-up revealed complete resolution of the hemorrhages and a return to a BCVA of 1.0 in both eyes.

    Case 2

    A 12-year-old female with no significant medical history presented with acute visual loss in the right eye. Refraction was −3.0 −1.5 × 180 in the right and −2.25 −1.5 × 170 in the left eye. BCVA was 0.9 in both eyes. Biomicroscopic examination showed no pathological findings. Fundoscopic examination revealed peripapillary subretinal and preretinal hemorrhages in the right eye (Fig. a, b). Visual field testing did not detect any increased scotoma. Ultrasound and OCT imaging highlighted optic disc swelling. FFA detected intrapapillary hemorrhage with no additional findings. The hemorrhages resolved spontaneously during follow-up.

    Case 3

    A 46-year-old female with congenital ptosis, high myopia, severe amblyopia and esotropia presented with acute visual field loss in her right eye. Visual acuity was counting fingers at 1 meter in the right eye and 0.2 in the left eye. Biomicroscopic examination was normal, and dilated fundus examination along with OCT revealed a tilted disc, extensive peripapillary hemorrhage, and intravitreal hemorrhage in the right eye (Fig. a, b). Differential diagnosis included CNV and macroaneurysm. A reliable visual field test could not be performed due to severe amblyopia in the right eye. FFA did not reveal any CNV or leaking areas. Ultrasound was ruled out buried drusen. Cranial and orbital MRI findings were reported as normal. The symptoms and hemorrhages were resolved spontaneously.

    Discussion

    IHAPSH is a clinical finding rather than the disease itself. It is very uncommon and has been reported rarely [1]. The common ocular findings in this disease include sudden onset in macular regions, increased risk in eyes with myopic tilted disks, frequent involvement of the superior and nasal sides of the optic disk, the coexistence of multiple types of hemorrhages, a benign course, and rare recurrences [7– 9]. Potential pathogenic mechanisms have been proposed to include vitreopapillary traction, bleeding from fragile prelaminar vessels in swollen optic disks, hemodynamic effects of the Valsalva maneuver, and complications from optic disk edema [1].

    Hemorrhages observed in our study typically resolved as expected; intrapapillary blood was absorbed within weeks, and subretinal hemorrhage regressed over months, aligning with findings reported in the literature. None of the patients in our three cases developed complete posterior vitreous detachment (PVD), consistent with previous reports [1, 2, 4, 10].

    Despite significant and striking changes in the optic disk, the syndrome was considered benign and did not necessitate extensive further investigation. We performed ancillary tests, such as magnetic resonance imaging, to rule out other neuro-ophthalmological diseases, although such tests are not always mandatory. The wide spectrum of symptoms associated with vitreopapillary traction complicates the prediction of clinical outcomes for clinicians. In cases with longstanding subretinal hemorrhage, there may be pigment epithelial toxicity, which could manifest as outer retinal findings in OCT [4]; however, none of our cases developed chronic sequelae.

    Differential diagnoses for IHAPSH include optic nerve head drusen, optic disk vasculitis, optic neuritis, ischemic optic neuropathy, and peripapillary subretinal neovascularization [3, 4, 7]. In cases with a suspicion of CNV, FFA can be invaluable in ruling out this condition, as demonstrated in our cases.

    In conclusion, this case series enriches the existing literature on IHAPSH syndrome by detailing the benign prognosis and potential for spontaneous resolution while also highlighting the critical need for differential diagnosis. Future studies should aim to elucidate further the pathophysiological mechanisms underlying IHAPSH and to explore potential genetic or environmental factors contributing to its incidence. This could lead to more targeted surveillance strategies and therapeutic approaches in at-risk populations.

    Информация об авторах

    Мехмет Омер Киристиоглу, преподаватель кафедры офтальмологии, Медицинский факультет Университета Бурса Улудаг, Бурса, Турция, momerkiristioglu@gmail.com

    Гамзе Учан Гюндюз, доцент кафедры офтальмологии Медицинского факультета Университета Бурсы Улудаг, Бурса, Турция, gamzeucan@gmail.com

    Сонгул Сейис, резидент кафедры офтальмологии, Медицинский факультет Университета Бурса Улудаг, Бурса, Турция, drsongulseyis@ gmail.com

    Озгур Йалчынбайыр, профессор кафедры офтальмологии Медицинского факультета Университета Бурса Улудаг, Бурса, Турция, yalcinbayir@yahoo.com

    Онерн Гелишкен, профессор, Частная больница Джимер Бурсы, Бурса, Турция, gelisken@uludag.edu.tr

    Information about the authors

    Mehmet Omer Kiristioglu, Instructor, Department of Ophthalmology, Bursa Uludag University School of Medicine, Bursa, Turkey, momerkiristioglu@gmail.com

    Gamze Ucan Gunduz, Associate Professor, Department of Ophthalmology, Bursa Uludag University School of Medicine, Bursa, Turkey, gamzeucan@gmail.com

    Songul Seyis, Resident, Department of Ophthalmology, Bursa Uludag University School of Medicine, Bursa, Turkey, drsongulseyis@gmail.com

    Ozgur Yalcinbayir, Professor, Department of Ophthalmology, Bursa Uludag University School of Medicine, Bursa, Turkey, yalcinbayir@yahoo.com

    Oner Gelisken, Professor, Bursa Private Jimer Hospital, Bursa, Turkey, gelisken@uludag.edu.tr

    Вклад авторов:

    Мехмет Омер Киристиоглу – концептуализация, обработка данных, написание оригинального проекта, исследование и методология.

    Гамзе Учан Гюндюз – концептуализация, обработка данных, написание оригинального проекта, исследование и методология.

    Сонгул Сейис – обработка данных, выполнение исследования.

    Озгур Йалчынбайыр – написание рецензии и редактирование.

    Онерн Гелишкен – редактирование.

    Аuthor’s сontribution:

    Mehmet Omer Kiristioglu – conceptualization, data curation, writing original draft, investigation and methodology.

    Gamze Ucan Gunduz – conceptualization, data curation, writing review and editing, investigation and methodology.

    Songul Seyis – data curation, investigation.

    Ozgur Yalcinbayir – writing review and editing, supervision.

    Oner Gelisken – editing

    Финансирование: авторы не получали конкретный грант на это исследование от какого-либо финансирующего агентства в государственном, коммерческом и некоммерческом секторах.

    Financial transparency: Аuthors have no financial interest in the submitted materials or methods.

    Конфликт интересов: отсутствует.

    Conflict of interest: None.

    Поступила: 16.04.2024

    Переработана: 28.06.2024

    Принята к печати: 5.08.2024

    Originally received: 16.04.2024

    Final revision: 28.06.2024

    Accepted: 5.08.2024