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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Ophthalmology

Treatments, Surgery, and Finding the Right Specialist

At a Glance

Most people with Posterior Polymorphous Corneal Dystrophy (PPCD) only need regular monitoring and glasses. If the cornea swells, modern partial-thickness transplants like DMEK or DSAEK can restore clear vision. Co-management with a glaucoma specialist may also be needed to protect eye health.

Managing Posterior Polymorphous Corneal Dystrophy (PPCD) is a long-term journey that focuses on two goals: protecting clear vision today and ensuring the health of the eye for the future. For many, this simply means regular monitoring, but for those whose corneas begin to fail, modern surgical options offer excellent outcomes.

Non-Surgical Care: The First Line of Defense

For most patients, especially children, the priority is managing how light enters the eye. Because PPCD can cause the cornea to be steep or irregularly shaped, the first steps often include:

  • Corrective Lenses: Glasses or specialized rigid gas permeable contact lenses can correct the high astigmatism and nearsightedness often seen in PPCD [1][2].
  • Amblyopia Treatment: In children, if one eye is more affected than the other, “patching” the stronger eye may be necessary to ensure the brain doesn’t ignore the vision from the PPCD-affected eye [3].
  • Glaucoma Eye Drops: If eye pressure begins to rise, the first-line defense before considering any surgery is typically standard, non-surgical treatment like daily eye drops to safely lower the pressure.
  • Long-Term Monitoring: Regular exams to check Endothelial Cell Density (ECD) and eye pressure are the standard of care to catch any changes early [4][5].

When the ‘Pumps’ Fail: Modern Surgical Options

If the endothelial cells (the “pumps”) can no longer keep the cornea clear, the cornea becomes swollen (decompensation). When this happens, surgery is indicated [6].

In the past, a full-thickness transplant (Penetrating Keratoplasty) was required. Today, specialists prefer Endothelial Keratoplasty (EK)—partial-thickness transplants that only replace the diseased inner layer of the cornea [7][8].

  1. DMEK (Descemet Membrane Endothelial Keratoplasty): This is often the preferred technique. It involves transplanting a very thin layer of cells. It typically offers the fastest recovery and the best visual quality [6][9].
  2. DSAEK (Descemet Stripping Automated Endothelial Keratoplasty): This involves a slightly thicker graft. While DMEK is widely used, surgeons sometimes opt for DSAEK in PPCD because the abnormal ‘skin-like’ cells can alter the Descemet membrane, making the tissue behave unpredictably during a DMEK procedure [7][10].

What to Expect During Recovery: Recovery from a DMEK or DSAEK typically involves lying flat on your back for several days right after surgery to help an air or gas bubble hold the new tissue in place. Vision usually begins to improve over the following weeks [6]. These partial-thickness surgeries are preferred because they have a lower risk of rejection and preserve the structural integrity of the eye better than full-thickness transplants [11][12].

The Challenge of Secondary Glaucoma Surgeries

If PPCD leads to high eye pressure that cannot be controlled by eye drops alone, management can become more complex. The “skin-like” cells characteristic of PPCD can grow over the eye’s drainage system [13].

  • MIGS (Minimally Invasive Glaucoma Surgery): While these newer, gentler surgeries are an option, they require extreme caution in PPCD patients. The abnormal cell growth can make it difficult for surgeons to see the drainage angle clearly during the procedure [14].
  • Care Coordination: It is often recommended that patients with both PPCD and high pressure be co-managed by a Corneal Specialist and a Glaucoma Specialist [15].

Building Your Care Team

PPCD is a rare condition, and finding a doctor with specific experience is crucial. When vetting a specialist, consider asking:

  • “How many patients with endothelial dystrophies do you treat?” Experience with conditions like Fuchs Dystrophy often translates well to PPCD management.
  • “Do you perform DMEK and DSAEK?” A surgeon should be proficient in modern partial-thickness transplant techniques [16].
  • “Are you part of a multi-specialty practice?” Because PPCD can affect eye pressure, having easy access to a glaucoma specialist within the same care team is ideal [17].

Early referral to a specialist at a major academic or tertiary eye center is often the best way to ensure access to the latest imaging and surgical techniques [18][19].

Common questions in this guide

What is the first-line treatment for PPCD?
For most patients, the first step in managing PPCD is using corrective glasses or specialized contact lenses to improve vision. Regular eye exams are also required to monitor the health of the cornea and check for any increases in eye pressure.
Will I need a full corneal transplant for PPCD?
In the past, full-thickness transplants were common, but today specialists prefer partial-thickness transplants like DMEK or DSAEK. These modern surgeries only replace the diseased inner layer of the cornea, offering faster recovery and lower rejection risks.
Why are DMEK and DSAEK preferred over traditional corneal transplants?
Partial-thickness transplants like DMEK and DSAEK have a lower risk of tissue rejection compared to traditional full-thickness transplants. They also preserve the natural structure of the eye and generally result in faster, clearer visual recovery.
Can PPCD cause glaucoma?
Yes, the abnormal cells associated with PPCD can sometimes grow over the eye's natural drainage system, leading to high eye pressure and secondary glaucoma. This is typically managed first with eye drops, but may require specialized glaucoma surgery if drops are insufficient.
What kind of doctor should treat my PPCD?
PPCD should be managed by a fellowship-trained corneal specialist, ideally at a major academic eye center. If you also develop high eye pressure, your care team should include a glaucoma specialist to help protect your vision.
How is amblyopia treated in children with PPCD?
If PPCD affects one eye more than the other in a child, doctors may use patching therapy over the stronger eye. This forces the brain to use the weaker eye, helping to prevent permanent vision loss from amblyopia, commonly known as a lazy eye.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How many DMEK or DSAEK procedures do you perform annually for patients with corneal dystrophies?
  2. 2.If my child (or I) eventually needs a transplant, do you prefer DMEK or DSAEK, and why?
  3. 3.Is there evidence that abnormal cells are blocking the drainage angle of the eye (gonioscopy)?
  4. 4.Do you have access to In Vivo Confocal Microscopy (IVCM) or specular microscopy in your clinic?
  5. 5.Do you work closely with a glaucoma specialist who has experience with rare corneal conditions?
  6. 6.What are the long-term success rates you see in pediatric patients who receive endothelial transplants?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (19)
  1. 1

    c.-61G>A in OVOL2 is a Pathogenic 5' Untranslated Region Variant Causing Posterior Polymorphous Corneal Dystrophy 1.

    Janeschitz-Kriegl L, Kamdar D, Quinodoz M, et al.

    Cornea 2022; (41(1)):89-94 doi:10.1097/ICO.0000000000002843.

    PMID: 34469340
  2. 2

    Contact lens fitting in a patient with Alport syndrome and posterior polymorphous corneal dystrophy: a case report.

    Rosa JM, Andrade Sobrinho MV, Lipener C

    Arquivos brasileiros de oftalmologia 2016; (79(1)):42-3.

    PMID: 26840166
  3. 3

    Unilateral Posterior Polymorphous Corneal Dystrophy Presented as Anisometropic Astigmatism: 3 Case Reports.

    Jeon HS, Hyon JY

    Case reports in ophthalmology 2017; (8(1)):250-258 doi:10.1159/000472704.

    PMID: 28559836
  4. 4

    Clinical Features in Children with Posterior Polymorphous Corneal Dystrophy.

    Ahn YJ, Choi SI, Yum HR, et al.

    Optometry and vision science : official publication of the American Academy of Optometry 2017; (94(4)):476-481 doi:10.1097/OPX.0000000000001039.

    PMID: 28009792
  5. 5

    Retinal pathology in the PPCD1 mouse.

    Shen AL, Moran SM, Glover EA, et al.

    PloS one 2017; (12(10)):e0185094 doi:10.1371/journal.pone.0185094.

    PMID: 28981549
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    Descemet membrane endothelial keratoplasty in iridocorneal endothelial syndrome and posterior polymorphous corneal dystrophy.

    Sorkin N, Einan-Lifshitz A, Boutin T, et al.

    Canadian journal of ophthalmology. Journal canadien d'ophtalmologie 2019; (54(2)):190-195 doi:10.1016/j.jcjo.2018.05.012.

    PMID: 30975342
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    Endothelial keratoplasty for posterior polymorphous corneal dystrophy in a 4-month-old infant.

    Hermina Strungaru M, Ali A, Rootman D, Mireskandari K

    American journal of ophthalmology case reports 2017; (7()):23-26 doi:10.1016/j.ajoc.2017.05.001.

    PMID: 29260073
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    Bilateral posterior lamellar corneal transplant surgery in an infant of 17 weeks old: Surgical challenges and the added value of intraoperative optical coherence tomography.

    Muijzer MB, Kroes HY, van Hasselt PM, Wisse RPL

    Clinical case reports 2022; (10(4)):e05637 doi:10.1002/ccr3.5637.

    PMID: 35387289
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    Review: Optimizing DMEK Technique, 2018-2020.

    Ramirez DA, Schmidt GA, Worthington KS, Sales CS

    International ophthalmology clinics 2021; (61(1)):45-55 doi:10.1097/IIO.0000000000000340.

    PMID: 33337793
  10. 10

    A Review of Outcomes of Descemet Membrane Endothelial Keratoplasty and Descemet Stripping Automated Endothelial Keratoplasty Interventions in Patients with Pre-Existing Glaucoma.

    Jafari K, Ashena Z, Niestrata M

    Journal of clinical medicine 2025; (14(10)) doi:10.3390/jcm14103534.

    PMID: 40429529
  11. 11

    Mediators of Visual Acuity in Descemet Membrane Endothelial Keratoplasty and Ultrathin Descemet Stripping Automated Endothelial Keratoplasty.

    Lin CC, Chamberlain WD, Kakigi C, et al.

    Cornea 2024; (43(7)):853-859 doi:10.1097/ICO.0000000000003434.

    PMID: 37986182
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    Ultrathin Descemet stripping automated endothelial keratoplasty.

    Durrani AF, Faith SC, Jhanji V

    Current opinion in ophthalmology 2019; (30(4)):264-270 doi:10.1097/ICU.0000000000000575.

    PMID: 31033735
  13. 13

    [Imaging features of posterior polymorphous corneal dystrophy observed by in vivo confocal microscopy].

    Gu SF, Peng RM, Xiao GG, Hong J

    [Zhonghua yan ke za zhi] Chinese journal of ophthalmology 2022; (58(2)):103-111 doi:10.3760/cma.j.cn112142-20210228-00099.

    PMID: 35144350
  14. 14

    Poor intraoperative visibility and postoperative astigmatism associated with trabecular micro-bypass stent for corneal dystrophy: A case report.

    Nakagawa S, Ishii K

    Medicine 2024; (103(31)):e39211 doi:10.1097/MD.0000000000039211.

    PMID: 39093770
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    Graft versus host disease: what should the oculoplastic surgeon know?

    Tung CI

    Current opinion in ophthalmology 2017; (28(5)):499-504 doi:10.1097/ICU.0000000000000400.

    PMID: 28598869
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    Outcome of Descemet membrane endothelial keratoplasty for graft failure after Descemet stripping automated endothelial keratoplasty.

    Agha B, Shajari M, Slavik-Lencova A, et al.

    Clinical ophthalmology (Auckland, N.Z.) 2019; (13()):553-559 doi:10.2147/OPTH.S194185.

    PMID: 30988597
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    Establishing a Virtual Corneal Clinic: A Real-Time Teleophthalmology Approach.

    Siregar SR, Ardiani LS, Chua A, et al.

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    PMID: 36729599
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    Prevalence of limbal stem cell deficiency at an academic referral center over a two-year period.

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    The rising incidence of Acanthamoeba keratitis: A 7-year nationwide survey and clinical assessment of risk factors and functional outcomes.

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This page is for informational purposes only and does not replace professional medical advice. Always consult an ophthalmologist or corneal specialist about your specific PPCD treatment options.

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