Posterior polymorphous corneal dystrophy (PPCD) frequently is an asymptomatic condition that may be diagnosed at the time of a routine eye examination. 

• PPCD is a hereditary disease. A family history of corneal disease should be elucidated.
• Asymptomatic patients will have normal or near-normal visual acuity.
• Glare symptoms are frequently reported.
• With careful biomicroscopy, bilateral polymorphic irregularities are evident at the Descemet membrane and the endothelium.
• Although corneal dystrophies are a bilateral entity it is not uncommon to have asymmetric signs.
• The appearance of the lesions is highly variable. Clusters of blister-like vesicles are most commonly encountered.
• Another pathognomonic configuration has parallel linear bands with scalloped edges across or adjacent to the visual axis.
• Larger areas of diffuse opacities are rare but are more likely to diminish visual function. 
• The onset of signs or symptoms should be ascertained. Infants or toddlers within the critical or sensitive period are likely to develop amblyopia.
• Structural irregularities of the Descemet membrane can induce corneal steepening and high astigmatism [20].
• Significant asymmetry in refraction may contribute to the development of anisometropic amblyopia in susceptible children. 
• Rarely corneal edema due to compromised corneal endothelium is evident at birth. 
• Persistent central corneal opacity may contribute to the development of deprivation amblyopia in susceptible children.
• If endothelial findings are present gonioscopy should be performed to evaluate the anatomic angle.
• Peripheral anterior synechia (PAS) can found in isolation or as broad bands. Peripheral corneal edema may overly broad bands of PAS.
• If intraocular pressure is elevated a careful glaucoma workup should be initiated.
• If there are iris or pupillary distortions a glaucoma workup should be initiated.

Careful evaluation of the posterior corneal tissues with slit-lamp biomicroscope allows immediate, in-office visualization of the polymorphous lesions.

• Polymorphous lesions may be visualized at the biomicroscope with moderate to high magnification using direct or, preferentially, retro-illumination techniques.
• Specular microscopy enables high-resolution imaging of the characteristic findings associated with PPCD within Descemet membrane and endothelial layer.
• Endothelial cell count can be diminished. The remaining endothelium demonstrates structural pleomorphic irregularities. 
• Confocal microscopy is of value in definitively identifying features characteristic of PPCD.
• Careful intraocular pressure measurement is necessary to screen for glaucoma.
• Anterior segment optical coherence tomography (OCT) images any abnormalities associated with aberrant endothelium bridging the anatomic angle.
• As an autosomal dominant entity, examination of family members may be helpful in confirming the diagnosis.

Asymptomatic patients with limited findings should be educated regarding the presence and potential significance of posterior polymorphous corneal dystrophy (PPCD). 

• Initiate amblyopia therapy in young children with high or asymmetric refractive error.
• Initiate immediate referral to a pediatric ophthalmologist if non-clearing corneal edema is present.
• Initiate amblyopia therapy in young children that have a history of corneal edema affecting form perception.
• Long-term monitoring of the posterior corneal layers is required.
• If secondary epithelial erosions or bullous keratopathy are present a bandage soft contact lens should be considered.
• If endothelial findings are present, gonioscopy should be performed to evaluate the anatomic angle. 
• If intraocular pressure is elevated, a careful glaucoma workup should be initiated. 
• If findings substantiate a diagnosis of glaucoma, then initiate first-line topical glaucoma therapy.
  • Monotherapy with a prostaglandin analog will control intraocular pressure (IOP) in the majority of cases.
  • Changing class or adding a medication may be necessary to realize IOP control.
  • Laser peripheral iridotomy (LPI) is not recommended if epithelialization of the trabecular meshwork has been identified.
  • If unable to achieve or maintain rigid IOP control, glaucoma filtering surgery may be necessary.
    • Minimally invasive glaucoma surgery (MIGS) offers promise.
• If there are iris or pupillary distortions a glaucoma workup should be initiated.
• Penetrating Keratoplasty (PK) has historically been successful, but has slow and challenging recovery as well as variable visual outcomes.
• Descemet membrane endothelial keratoplasty (DMEK) if the corneal stroma and epithelium are unaffected.
• Descemet stripping automated endothelial keratoplasty (DSAEK) is preferred if the corneal stroma and epithelium are unaffected.
• If iridocorneal adhesions are present corneal grafting procedures are at risk for failure.

The differential diagnoses includes:

• ICE syndrome (Chandler syndrome).
• Congenital hereditary endothelial dystrophy. 
• Pre-Descemet corneal dystrophy.
• Fuchs endothelial dystrophy.
• Primary congenital glaucoma.
• Traumatic rupture of Descemet membrane.

The prognosis for posterior polymorphous dystrophy (PPCD) is dependent on the extent of the disease.

• In most cases, PPCD is a static or very slowly progressive disease.
• The frequency of follow-up required is dependant on the severity of the disease.
• Patients with mild disease that are identified in adulthood have a good prognosis and are not likely to require treatment.
• If significant refractive disparity is present in childhood anisometropic amblyopia may develop.
• Infants with corneal edema require timely intervention to reduce the potential for developing deprivation amblyopia.
• Those with iridocorneal adhesions should be monitored for glaucoma.
• Those with elevated intraocular pressure, visual field defects or glaucomatous optic nerve appearance should be treated for glaucoma.
• Penetrating keratoplasty should be considered if corneal opacities limit visual potential.
• If iridocorneal adhesions are present corneal grafting procedures are at risk for failure.

 Infants and toddlers are at risk for amblyopia if neurodevelopment of the visual system is disrupted.

• Intervention is imperative during the critical period (up to age 3). Strongly recommended during the sensitive period (ages 3 to 9).
• To reduce the risk of complications, patients prescribed bandage soft contact lens (BSCL) therapy must be proficient in the handling and care of the BSCL.
• Patients prescribed BSCL are at risk for infection, pain and vision loss if secondary corneal infection were to occur.
• Those with iridocorneal adhesions should be monitored for glaucoma.
• Those with elevated intraocular pressure, visual field defects or glaucomatous optic nerve appearance should be treated for glaucoma.
• Penetrating keratoplasty should be considered if corneal opacities limit visual potential.
• If iridocorneal adhesions are present corneal grafting procedures are at risk for failure.
• Posterior polymorphous corneal dystrophy can recur in corneal grafts.

• Referral to a pediatric ophthalmologist if non-clearing corneal edema is present in an infant or toddler.
• Referral to strabismologist if strabismus is present in an infant or toddler.
• Referral to a corneal specialist if posterior corneal opacification necessitates corneal transplantation.
• Referral to a corneal specialist if corneal edema or bullous keratopathy necessitates corneal transplantation.
• Referral to a glaucoma specialist if poor intraocular pressure control necessitates glaucoma filtering surgery.
• Referral to a glaucoma specialist if glaucomatous progression necessitates glaucoma filtering surgery.

• Asymptomatic patients with limited findings should be educated regarding the presence and potential significance of posterior polymorphous corneal dystrophy (PPCD). 
• Early vision screening is essential.
  • Amblyopia therapy is required for young children with high or asymmetric refractive error.
• Periodic ophthalmic evaluation is necessary to screen for anterior segment irregularities.
  • Initiate immediate referral to a pediatric ophthalmologist if non-clearing corneal edema is present.
  • Amblyopia therapy is recommended for young children with corneal edema affecting form perception.
  • If endothelial findings are present, gonioscopy should be performed to evaluate the anatomic angle. 
  • If intraocular pressure is elevated, a careful glaucoma workup should be initiated. 
  • If there are iris or pupillary distortions a glaucoma workup should be initiated.
• If findings substantiate a diagnosis of glaucoma, glaucoma therapy is imperative.
  • Options include topical, laser, surgical and minimally invasive glaucoma surgery (MIGS).
• • Laser peripheral iridotomy (LPI) is not recommended if epithelialization of the trabecular meshwork has been identified.
  • If unable to achieve or maintain rigid IOP control, glaucoma filtering surgery may be necessary.
    • Minimally invasive glaucoma surgery (MIGS) offers promise.
• Penetrating Keratoplasty (PK) has historically been successful, but has slow and challenging recovery as well as variable visual outcomes.
• Lamellar corneal transplantation offers faster recovery and improved visual outcomes.
  • Descemet membrane endothelial keratoplasty (DMEK) if the corneal stroma and epithelium are unaffected.
  • Descemet stripping automated endothelial keratoplasty (DSAEK) is preferred if the corneal stroma and epithelium are unaffected.
  • If iridocorneal adhesions are present corneal grafting procedures are at risk for failure.

• Most cases of posterior polymorphous corneal dystrophy (PPCD) do not require any treatment.
• In infancy, this condition is easily confused with buphthalmos or congenital hereditary endothelial dystrophy (CHED).
  • Secondary opinion should be sought if the diagnosis is ambiguous.
• The thickened corneal tissue has greater biomechanical resistance to outside force affecting corneal hysteresis.
  • Intraocular pressure measurements may be overestimated lending to the appearance of ocular hypertension.
• Asymmetric findings involving the cornea, trabecular meshwork and iris must be differentiated from Chandler syndrome.
• Laser procedures for glaucoma may not be effective if the trabecular meshwork is altered by irregular or aberrant endothelial tissues.
• Goniotomy procedures for glaucoma may not be effective if the trabecular meshwork is altered by irregular or aberrant endothelial tissues.

Although onset is at birth or very shortly afterward, many patients with posterior polymorphous corneal dystrophy (PPCD) are visually asymptomatic and the condition is not diagnosed for years to decades later. If lesions at Descemet membrane are consistent with PPCD but there is no evidence of corneal edema, elevated intraocular pressure or irregularities of the angle or iris, then it is appropriate to recommend periodic comprehensive ophthalmic evaluation by a primary eyecare practitioner. 

If glaucoma is diagnosed and topical therapy is prescribed the ophthalmic care provider team must educate regarding the importance of rigid compliance using medications as well as the proper use and storage of topical medications. This can be performed by the physician, nurse, ophthalmic assistant or pharmacy staff.

If intraocular pressure is not adequately controlled or there is evidence of glaucomatous progression, then it is appropriate to seek glaucoma specialist evaluation to determine suitability for altered topical therapy, new laser therapy or glaucoma filtration surgery.

If corneal edema is present and responsive to topical hyperosmotic agents it is appropriate to provide education regarding the proper use of topical medications. This can be performed by the physician, nurse, ophthalmic assistant or pharmacy staff. Similarly, the proper placement, removal, and care for bandage contact lenses should be trained by a qualified physician, nurse or ophthalmic assistant.

If recalcitrant corneal edema obscures vision or leads to painful bullae or corneal erosions, then a corneal specialist should evaluate the suitability for a form of keratoplasty.

Patients must be aware that they too have the right and responsibility to communicate any perceived changes or difficulties to their healthcare team.