Staphyloma

Key Points at a Glance

Essentials of This Disease

• A staphyloma is a localized outward bulging of the eye wall (sclera and choroid), with posterior staphyloma being the most common type.
• Approximately 55% of pathological myopia (axial length >26 mm) is complicated by posterior staphyloma. ²⁾
• In tilted disc syndrome (TDS), inferior staphyloma (Curtin type V) is observed in about 2.6% of cases. ⁴⁾
• Major complications include macular retinoschisis, retinal detachment, and choroidal neovascularization (CNV), which can lead to legal blindness in some cases.
• There is no established treatment, and current management is individualized according to complications.
• SS-OCT and wide-angle OCT are the main tools for understanding the condition and follow-up.

1. What is a staphyloma?

A staphyloma is a condition in which the eye wall (sclera and choroid) becomes locally thinned and bulges outward. It is caused by intraocular pressure and degeneration of the outer membrane. The name derives from the bulging part resembling a grape.

Depending on the location, staphylomas are classified as anterior, ciliary, equatorial, or posterior. The most clinically important is posterior staphyloma, which frequently occurs in pathological myopia.

The prevalence of posterior staphyloma in patients with pathologic myopia is reported to be approximately 55%, ²⁾ which significantly affects long-term visual prognosis. A study by Khan (2025) on unilateral pathologic myopia showed that the incidence of legal blindness (best-corrected visual acuity ≤0.1) reaches 19%. ²⁾

Terminology clarification

“Pathologic myopia” refers to myopia accompanied by abnormal elongation of the axial length (generally >26 mm). It is characterized not merely by high refractive error but by complications such as ocular deformation and chorioretinal atrophy.

Q Does everyone with myopia develop staphyloma?

A

High myopia that progresses to “pathological myopia” with excessive elongation of the ocular axis carries a high risk of complications. Posterior staphyloma is reported in about 55% of pathological myopia cases²⁾, but it does not occur in all myopic eyes.

2. Main Symptoms and Clinical Findings

Subjective Symptoms

• Decreased visual acuity: The most common subjective symptom. It occurs due to deformation, atrophy, or complications of the macula.
• Metamorphopsia (distorted vision): Becomes prominent when combined with macular schisis or epiretinal membrane.
• Visual field defect: Peripapillary staphyloma may present characteristic visual field changes.
• TVL (transient visual loss): In contractile peripapillary staphyloma (CPPS), transient visual loss has been reported upon standing, etc. ³⁾

Clinical Findings

Posterior staphyloma presents various findings on slit-lamp microscopy, fundus examination, and OCT.

• Marked elongation of axial length: In a 2025 study by Khan, the mean axial length of pathologic myopic eyes reached 34 mm (compared to 24 mm in non-myopic eyes). ²⁾
• Peripapillary findings: In a 2021 report by Hokazono on contractile peripapillary staphyloma (CPPS), a depression of the posterior pole and a clearly demarcated bulge around the optic disc were observed. ³⁾
• Macular findings: SD-OCT and SS-OCT can identify macular schisis, retinoschisis, and epiretinal membrane. In cases with CFAP410 mutations, OCT showed marked thinning of the retina and choroid. ¹⁾
• MRI findings: Localized deformation and bulging of the posterior pole of the eyeball are visualized, useful for evaluating high myopic posterior staphyloma. ²⁾

Q Which test is best for evaluating posterior staphyloma?

A

OCT (especially SS-OCT and wide-angle OCT) is excellent for evaluating the morphology of the macula and peripapillary area ^{2, 3)}. MRI is useful for assessing the degree of overall eyeball deformation. ²⁾

3. Causes and Risk Factors

The main cause of posterior staphyloma is thinning and degeneration of the sclera. As the axial length of the eye elongates, remodeling of scleral collagen fibers is impaired, leading to localized weakening of the wall.

• Pathologic myopia: The greatest risk factor. Chronic mechanical stress due to axial elongation promotes scleral degeneration. ²⁾
• Tilted disc syndrome (TDS): A congenital condition involving tilting and deformation of the optic disc, complicated by inferior staphyloma (Curtin type V) in approximately 2.6% of cases. ⁴⁾ TDS frequently coexists with myopia and often presents with inferior visual field defects.
• Genetic predisposition: Mutations in the CFAP410 gene have attracted attention as a genetic background for staphyloma. Chiu (2022) reported a case with a CFAP410 mutation followed for 16 years, confirming progression of posterior staphyloma and thinning of the sclera and choroid. ¹⁾

Prevention and Daily Care

For those with high myopia, regular eye examinations to check for posterior staphyloma are recommended. Repeated heavy lifting or straining movements can cause intraocular pressure fluctuations, so consult your doctor before engaging in strenuous exercise. If you notice changes in symptoms (vision changes or distortion), seek medical attention promptly.

4. Diagnosis and Examination Methods

Diagnosis of staphyloma requires morphological confirmation using indirect ophthalmoscopy and fundus photography, as well as tomographic imaging.

Curtin Classification

The Curtin 10-type classification (1977) is widely used as an anatomical classification of staphyloma.

Type	Location
I	Posterior pole
II	Macula
III	Subpapillary
IV	Nasal side of the optic disc
V	Inferior
VI	Peripapillary + Macular
VII	Peripapillary + inferior
VIII	Peripapillary + macular + inferior
IX	Nasal side
X	Mixed type

Type V (inferior staphyloma) is common in tilted disc syndrome, with a reported prevalence of approximately 2.6%. ⁴⁾

Main Examination Methods

• Indirect ophthalmoscopy: Observation of fundus morphology. Check for color changes in the posterior pole and peripapillary changes.
• SS-OCT (swept source OCT): Excellent for visualizing deep tissues, enabling detailed morphological evaluation of peripapillary staphyloma. Hokazono (2021) clarified blood flow changes in CPPS using SS-OCT and OCTA. ³⁾
• Wide-angle OCT: Useful for assessing the extent and boundaries of posterior staphyloma. ²⁾
• MRI: Used to evaluate three-dimensional deformation of the eye and to understand the overall picture in severe cases. ²⁾
• OCTA (OCT angiography): Used to evaluate CNV and peripapillary blood flow changes. ³⁾

5. Standard Treatment

Currently, there is no established treatment that directly suppresses the progression of staphyloma. Treatment focuses on managing complications.

Main complications and management

The complications arising from posterior staphyloma and their treatments are described below.

Complication	Main treatment
Choroidal neovascularization (CNV)	Intravitreal anti-VEGF injection
Macular schisis	Observation or vitrectomy
Retinal detachment	Pars plana vitrectomy (PPV)
Myopic traction maculopathy	Vitrectomy ± internal limiting membrane peeling

• Choroidal neovascularization (CNV): Intravitreal injection of anti-VEGF drugs (e.g., ranibizumab, aflibercept) is the first-line treatment.
• Foveoschisis: Asymptomatic cases are observed. For cases with decreased vision or progression of schisis, vitrectomy (PPV) is considered.
• Retinal detachment: Vitrectomy is the standard surgical procedure.
• Posterior scleral reinforcement (PSR): Surgery aimed at suppressing axial elongation. Its efficacy remains debated, and ²⁾ it is not considered a standard treatment in Japan.

Points to note in treatment

There is no established treatment for posterior staphyloma itself. Regular OCT examinations are important for early detection of complications (CNV, macular retinoschisis). Even when anti-VEGF therapy is performed, repeated injections are often required.

Q Is posterior scleral reinforcement (PSR) a standard treatment?

A

PSR is a surgery expected to suppress axial elongation, but evidence regarding its efficacy is limited, and it is currently under debate. ²⁾ It cannot be considered a widely established standard treatment in Japan.

6. Pathophysiology and Detailed Mechanism of Onset

The formation of posterior staphyloma involves a complex interplay of structural changes in the sclera, vascular impairment, and local mechanical stress.

Degeneration of Scleral Collagen

With excessive elongation of the ocular axis, the arrangement of scleral collagen fibers becomes disordered, and the diameter and density of the fibers change. This leads to localized thinning and weakening of the sclera. ²⁾ Over the long term, the sclera at the posterior pole cannot withstand the internal pressure and bulges outward.

Mechanism of Contractile Peripapillary Staphyloma (CPPS)

Hokazono (2021) reported that the essence of CPPS is caused by the autonomous contraction and expansion movements of the eyeball. ³⁾ It has been suggested that this periodic dynamics leads to peripapillary venous stasis, which may result in transient vision loss (TVL). ³⁾

Relationship with Tilted Disc Syndrome (TDS)

Cohen (2006) reported the relationship between inferior staphyloma and chorioretinal folds associated with TDS. ⁴⁾ In TDS, the optic nerve penetrates the ocular wall obliquely, causing chronic mechanical stress on the sclera inferior to the optic disc. This is thought to lead to the selective development of inferior staphyloma.

Involvement of CFAP410 Mutation

In a 16-year follow-up case reported by Chiu (2022), CFAP410 mutation was suggested to be involved in the formation and progression of staphyloma. ¹⁾ CFAP410 encodes a cilia-associated protein and is presumed to play a role in the development and maintenance of ocular tissues.

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7. Latest Research and Future Perspectives (Reports at Research Stage)

For patients: Please read this

The following content is currently at the research stage or in clinical trials and is not standard treatment available at general hospitals. It is reference information for professionals regarding future medical developments.

Advances in Imaging Technology

The widespread use of SS-OCT and wide-angle OCT has enabled detailed evaluation of the shape, extent, and boundaries of posterior staphyloma. ²⁾ This is expected to improve the accuracy of staging and the quality of long-term follow-up.

Khan (2025) conducted a study on unilateral pathologic myopia, performing a systematic evaluation of posterior staphyloma using wide-angle OCT, and recorded detailed pathology in cases with axial length reaching 34 mm. ²⁾

Blood flow assessment by OCTA (OCT angiography)

Hokazono (2021) used OCTA to visualize peripapillary blood flow dynamics in CPPS (contractile peripapillary staphyloma). ³⁾ In the future, monitoring blood flow changes may become a predictor of visual prognosis.

Genetic research on CFAP410 mutations

Following a case report ¹⁾ showing an association between CFAP410 mutations and staphyloma/retinal degeneration, cilia-related genes are attracting attention as genetic predisposing factors for staphyloma. Elucidating the pathology through large-scale genomic analysis is a future challenge.

Q Will a curative treatment for staphyloma be developed in the future?

A

Currently, there is no established curative treatment. Therapies aimed at regenerating and strengthening scleral collagen, as well as gene therapy, are under research but have not yet reached clinical application. Early diagnosis through advances in imaging technology and early intervention for complications are the main strategies for now.

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8. References

1. Chiu N, Lee W, Liu PK, et al. A homozygous in-frame duplication within the LRRCT consensus sequence of CFAP410 causes cone-rod dystrophy, macular staphyloma and short stature. Ophthalmic Genet. 2022;43(3):378-384. doi:10.1080/13816810.2021.2010773.
2. Khan S, et al. Unilateral pathological myopia with posterior staphyloma: clinical features, complications and visual outcomes. Eye (Lond). 2025.
3. Hokazono K, Carstens LD, Monteiro MLR. Contractile peripapillary staphyloma: OCTA documentation of increased peripapillary vessel density during transient visual loss episodes. Am J Ophthalmol Case Rep. 2021;21:101010. doi:10.1016/j.ajoc.2021.101010.
4. Cohen SY, Quentel G. Chorioretinal folds as a consequence of inferior staphyloma associated with tilted disc syndrome. Graefes Arch Clin Exp Ophthalmol. 2006;244(11):1536-1538. doi:10.1007/s00417-006-0302-x.