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2.1
Scleral Staphyloma
Ring Staphyloma

Presentation

The common sites affected are nose and nasopharynx, although it may also be present on the eye, ear and even genitalia in both sexes. [bmcophthalmol.biomedcentral.com]

@inproceedings{Amrita2021StructuralAF, title={Structural and Functional Outcome of Scleral Patch Graft for Ciliary Staphyloma in Rhinosporidiosis}, author={Verma Amrita and Pandey Nidhi and Kaushik Kalpana}, year={2021} } A twenty year old female presented [semanticscholar.org]

Diagnosis Symptoms[5][6] Staphylomas often present in highly myopic patients. Presenting symptoms of staphyomas include significantly worsening of vision in previously myopic patients. [eyewiki.aao.org]

Case Presentation On July 15, 2005, a 58-year-old man presented with sudden onset of severe pain and blurred vision in his right eye, without significant irritation, of one day’s duration. [hindawi.com]

Eyes

  • Scleral Disease

    Anterior scleral staphyloma associated with neurofibromatosis. Ann Ophthalmol. 1991;23:356-8. 2. Pavlin CJ, Easterbrook M, Hurwitz JJ, Harasiewicz K, Eng P, Foster S. Ultrasound biomicroscopy in the assessment of anterior scleral disease. [oftalmoloji.org]

    Keywords: Optic disk drusen; Scleral diseases; Retina; Ultrasonography; Congenital peripapillary staphyloma; Retinography; Autofluorescence Introduction O ptical disc drusen are acellular deposits located both intracellularly and extracellularly, first [scielo.br]

    Destructive corneal and scleral disease associated with rheumatoid arthritis. Medical and surgical management. Cornea. 1995;14:408–17. 9. Waheed NK, Miserocchi E, Foster CS. Ocular concerns in juvenile rheumatoid arthritis. [aafp.org]

  • Iridodonesis

    The left eye had iridodonesis with 8 clock hours of nasal subluxation of a clear crystalline lens [Fig. 3]. Fundus was noted to be normal on indirect ophthalmoscopy. Intralenticular lens aspiration was planned for the patient's left eye. [journals.lww.com]

Neurologic

  • Dystonia

    Cerebellar hypoplasia-intellectual disability-congenital microcephaly-dystonia-anemia-growth retardation syndrome MedGen UID: 1780242 •Concept ID: C5543287 • Disease or Syndrome CIMDAG syndrome is a multisystemic disorder characterized by severely impaired [ncbi.nlm.nih.gov]

Workup

Workup, Diagnosis and Treatment Figure 3. (A) Fluorescein angiography and (B) indocyanine green angiography at 1 minute, 9 seconds after injection showing a slight macular window defect with associated hypercyanescence. [reviewofophthalmology.com]

[…] infection Streptococcal infection Staphylococcal infection Aspergillosis Leprosy Others Physical agents (irradiation, thermal burns) Chemical agents (alkali or acid burns) Mechanical causes (trauma, surgery) Lymphoma Rosacea Unknown Table 7-8: Laboratory workup [oculist.net]

X-Ray

  • Nephrolithiasis

    Uric acid nephrolithiasis has been reported. Ophthalmologic abnormalities are typically described as optic nerve coloboma or dysplasia. Iris colobomas have not been reported in any individual with PAX2–related disorder. [ncbi.nlm.nih.gov]

Treatment

One method of treatment is to monitor progression of the staphyloma. Many do not require treatment and remain stable over time. If there is observed thinning of the sclera, one treatment option is posterior scleral reinforcement (PSR). [eyewiki.aao.org]

Treatment was conducted by conjunctival resection and tectonic corneoscleral graft (13x13mm) over the staphyloma. Within 1 year of follow-up the patient presented a partial staphyloma reduction, 9x9x2.5 mm, and the patch detached from the lesion. [ncbi.nlm.nih.gov]

Giacinto C...Tognetto D 30984418 2019 3 Partial regression of large anterior scleral staphyloma secondary to rhinosporidiosis after corneoscleral graft - a case report. 61 Costa EF...Silva GEB 29486755 2018 4 Complications Assoicated with MIRAgel for Treatment [malacards.org]

Even staphylomas with a slight degree of outpouching can now be detected.[3] No specific treatment exists for posterior staphylomas. [medscape.com]

Prognosis

Prognosis[2] Prognosis is good for patients with staphyloma, as progression is generally slow. Many do not need treatment and remain unchanged for years. [eyewiki.aao.org]

AF images are helpful in these patients to confirm the presence of the MH, which has a poorer prognosis. [mivision.com.au]

PMID: 4453405 Prognosis Mizuno H, Suzuki H, Mimura M, Fukumoto M, Sato T, Kida T, Ikeda T J Med Case Rep 2022 Jan 29;16(1):36. doi: 10.1186/s13256-022-03252-7. [ncbi.nlm.nih.gov]

Sclerokeratoplasty can restore the globe structurally but has poor visual prognosis. From the Department of Ophthalmology, University of Florida, Gainesville, FL. [journals.lww.com]

The visual prognosis is also poor. Clinical features I. Presentation is with gradual onset of pain and localized redness. [archive.org]

Etiology

Etiology:- Weak outer coat of the eye due to ocular diseases ( as in perforated corneal ulcer or high axial myopia). Increased intraocular pressure ( in cases of absolute glaucoma). [thegeniusprof.com]

Suggested reading • Etiology, clinical features and management of Episcleritis • Etiology, classification, clinical features and management of Scleritis • Types & etiology of staphyloma. • Treatment of anterior scleritis. [slideshare.net]

Secondary etiologies such as trauma or infection can disrupt the structure of the sclera, placing the injured region at risk for subsequent scleral thinning, to eventual development of staphyloma. [eyewiki.aao.org]

While the etiology of posterior staphylomas has remained unclear, local choroidal factors and a locally decreased biomechanical resistance of the sclera against a posteriorly expanding Bruch's membrane have been one of the assumed pathogenic parameters [ncbi.nlm.nih.gov]

Epidemiology

Epidemiology[3] Up to 9% of Asian and 2% of White population in the United States have pathologic myopia, which increases their likelihood of having concurrent posterior staphyloma. [eyewiki.aao.org]

Generally, myopia is the result of elongated axial length in the region of 25.5 or 26.5mm. (1,2) Degenerative myopia is also known as other terminology including: High Myopia Degenerative Myopia Pernicious Myopia Malignant Myopia EPIDEMIOLOGY The epidemiology [optometrystudents.com]

Epidemiology and disease burden of pathologic myopia and myopic choroidal neovascularization: an evidence-based systematic review. Am J Ophthalmol. 2014; 157:9-25 2. Hsiang HW, Ohno-Matsui K, Shimada N et al. [mivision.com.au]

Especially, conventional 50° fundus photos are widely used in many epidemiological studies. [retinalphysician.com]

In most early epidemiologic studies, the refractive error [−5.0 diopters (D), −6.0 D, −8.0 D], the axial length (>26.0 mm, >26.5 mm), or a combination of both have been used to define “PM”. [aes.amegroups.com]

Pathophysiology

Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology. [books.google.it]

However, the pathophysiology might be similar to that of retinal detachment in combination with another excavated optic disc anomaly. [jamanetwork.com]

Pathophysiology[2] The etiology of the formation of staphylomas are unclear, however there is postulation that local choroidal factors and a decreased resistance of the sclera leading to a protruding Bruch’s membrane can result in staphylomas. [eyewiki.aao.org]

Prevention

/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis of Alcohol 0% Unclassified 100% Some eye [medicbind.com]

Later procedures focused on modifying the axial length of the eye, by preventing elongation and staphyloma progression by placing grafts over the posterior part of the eye. [fr.bab.la]

Buckling adult eyes can stabilize axial length and minimize future macular degeneration, but it cannot prevent the continuation of degenerative processes, which are already at work. [lowvision.preventblindness.org]

Thus, in the future, therapies targeting Bruch’s membrane may also become a promising approach to prevent staphyloma development. [retinalphysician.com]

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