In vivo confocal microscopic observations of 49 patients with posterior polymorphous corneal dystrophy / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To explore the imaging features of 49 patients with posterior polymorphous corneal dystrophy(PPCD)by in vivo confocal microscopy(IVCM).METHODS: Retrospective case series study. A total of 49 patients(86 eyes), including 32 males and 17 females diagnosed with PPCD between January 2013 and January 2021 were collected. The mean age was 42.5±22.9 years. All patients were scanned by IVCM to analyze the density of corneal endothelial cells and described IVCM characteristics of different types of PPCD.RESULTS: The number of endothelial cells in the lesion area of all patients was lower than that in the peripheral area. Under IVCM, 44 eyes(51%)were categorized into type 1 PPCD(vesicular lesions), characterized by single or multiple, central round or irregular crater-like lesion on paracentral corneal endothelial layer; 16 eyes(19%)were categorized into type 2 PPCD(band lesions), which displayed curved and raised edge with scattered or banded-distributed gutta-like lesion between edges. Type 3 PPCD(diffuse lesion)were in 26 eyes(30%), which showed that endothelial cells were missing in many areas. The blurred images of endothelium in most areas featured with spikes lined in a streak, and the clear images in some areas featured with a band lesions. Two patients were followed up for 4-5a. The IVCM images showed different lesions, including the decrease of central corneal endothelial cell density and the iron deposit in the corneal epithelium, etc.CONCLUSION: IVCM is able to scan the characteristic microstructural alterations at the level of endothelium and Descemet membrane in patients with PPCD, and provide an effective image diagnosis for PPCD.

Iridocorneal Endothelial Syndrome with Features of Posterior Polymorphous Corneal Dystrophy

PURPOSE: To report a case of iridocorneal endothelial syndrome, which overlapped with some of the features of posterior polymorphous corneal dystrophy. CASE SUMMARY: A 61-year-old female presented with tearing pain and blurred vision in her left eye, which was aggravated in the morning. The symptom started approximately 1 year prior to her visit. At the initial visit, the visual acuities were 1.0 in both eyes and the intraocular pressures were normal. On slit-lamp examination, a single pair of horizontal parallel lines was observed at the central corneal endothelial layer in the right eye. In contrast, multiple pairs of oblique parallel lines were observed in the left eye. The lines of the lesions were more prominent and wavier in the left eye than those of the right eye. The overlying cornea was clear, and the corneal thicknesses were in the normal range in both eyes. Using a gonioscopic examination, localized peripheral anterior synechiae were observed only in the left eye. The pupil and iris were normal in both eyes. On specular microscopic examination, the corneal endothelial cell size in the right eye increased and the corneal endothelial density decreased to 668 cells/mm². In the left eye, multiple abnormal endothelial cells with dark-light reversal were observed. In conclusion, the patient was subsequently diagnosed with iridocorneal syndrome, rather than posterior polymorphous corneal dystrophy. CONCLUSIONS: Posterior polymorphous corneal dystrophy and iridocorneal endothelial syndrome may present with many similarities. Therefore, in cases of uncertain diagnosis, an understanding of the clinical features is important for proper diagnosis.

Posterior Polymorphous Corneal Dystrophy (PPCD) Combined with Traumatic Descemet's Membrane Fold

PURPOSE: To present a case of a 72-year-old woman with posterior polymorphous corneal dystrophy (PPCD) combined with traumatic Descemet's membrane fold due to forceps injury in Birth. CASE SUMMARY: A 72-year-old woman without significant ocular history presented complaining of ocular discomfort in her right eye. She had a history of birth that required forceps delivery and poor vision in the right eye since childhood. The rest of perinatal ophthalmic history was unremarkable (no past ophthalmic or family history). The best corrected visual acuity was 20/20 in both eyes. Slit-lamp biomicroscopy showed distinctive, vertical, semitranslucent Descemet's membrane breaks situated on the superior temporal side of the posterior surface of the cornea in her right eye. Also, a horizontal, tram-track line appearance of posterior cornea surface was detected at middle and inferior side of cornea. The fellow eye was normal. The endothelial cell densities were 945 cells/mm2 and 2481 cells/mm2 in right and left eyes, respectively. After 11 years later, (routine follow up exams every 1 year), the endothelial cell densities were 901 cells/mm2 and 2481 cells/mm2 in right and left eyes, respectively, which means there were no significant changes of endothelial densities in both eyes. CONCLUSIONS: We report a case of a patient with posterior polymorphous corneal dystrophy (PPCD) combined with traumatic Descemet's membrane fold due to forceps injury in Birth. The disease does not seem to progress or aggravated in long term follow up and no specific treatment was required.

Confocal Microscopic Findings in Posterior Polymorphous Corneal Dystrophy

PURPOSE: To analyze the features of corneal tissue in patients with posterior polymorphous corneal dystrophy (PPMD) using in vivo confocal microscopy (IVCM). CASE SUMMARY: Three patients with clinically diagnosed PPMD were examined using IVCM. Cross-sectioned corneal images of the corneal epithelium, Bowman's layer, stromal layer, Descemet's membrane, and endothelium were evaluated. IVCM demonstrated a depressed crater-like lesion, hyper-dense streak-like lesion, and surface irregularity of the corneal endothelium. Endothelial hypo-reflective vesicular and band-like lesions were also found. Pleomorphism and polymegathism were present with guttae and hyper-reflective endothelial nuclei. CONCLUSIONS: IVCM is a non-invasive and effective tool to diagnose PPMD.

Descemet membrane endothelial keratoplasty with a stromal rim in the treatment of posterior polymorphous corneal dystrophy.

A 20-year-old patient, diagnosed with posterior polymorphous corneal dystrophy, developed corneal edema for which he underwent Descemet membrane endothelial keratoplasty with a stromal rim (DMEK-S) in the right eye. No intra- or postoperative complications were noted. At the last follow-up 2 years and 9 months after the procedure, the best corrected visual acuity was 1.0 and endothelial cell density declined from 3533 cells/mm2 to 1012 cells/mm2. Despite the endothelial cell loss, DMEK-S appears to be a good alternative to other surgical techniques for the treatment of corneal endotheliopathies, and it may be of benefit to young patients.