Terminology of Peters' anomaly variants: Summary of histopathological findings in 6 corneas and detailed clinicopathological correlation in 2 cases

Abstract

Peters' anomaly accounts for the highest type of Anterior Segment Dysgenesis (ASD). The main features of Peters' anomaly are: congenital corneal opacity centrally, defect in the posterior stroma and absence of Descemet's membrane and the endothelium. However, this condition has wide clinical and histopathological variations in appearance, associations and severity. In this case series, we summarize 6 corneas in 5 Saudi cases of Peters' anomaly (and describe 2 in detail) with unique histopathological findings that are additional to the typical known ones, shedding some light on the nomenclature of these variants according to the reported cases in the English-written literature. This will widen the spectrum of findings known to ophthalmic pathologists and ophthalmologists about this anomaly. This is also of importance in the assessment of the congenital glaucoma cases commonly seen in Saudi Arabia that often happens in association with ASD.

Keywords: Anterior Segment Dysgenesis; Corneal opacity; Glaucoma; Myxoma; Peters’ anomaly; Posterior keratoconus.

Fig. 1

(A) An example of Peters’ anomaly with anterior bullous changes, absent Bowman’s layer and alteration of the normal stromal lamellar architecture. (Original magnification ×200 Periodic Acid Schiff). (B) Lower power of the same cornea. Note the thickened corneal stroma with edema and intact peripheral portion of Descemet’s membrane (Original magnification ×100 Periodic Acid Schiff). (C) The classic absence of Descemet’s membrane centrally with adherent iris tissue in the area of defective Descemet’s membrane (Original magnification ×200. Hematoxylin & Eosin). (D) Another cornea in Peters’ anomaly with cataract and lenticular adhesion (Type 2) showing thick corneal stroma, total absence of Descemet’s membrane, and adherent iris tissue as well as a residual lens capsule following cataract surgery (Original magnification ×100 Hematoxylin & Eosin).

Fig. 2

(A)The histopathological appearance of the posterior concavity in one of the cases of posterior keratoconus. (Original magnification ×100. Hematoxylin & Eosin). (B) Higher power of the same cornea showing thick interrupted Bowman’s layer and absent Descemet’s membrane centrally. (Original magnification ×200, Periodic Acid Schiff). (C) One of the cases of Peters’ variant with absent Descemet’s membrane centrally and thick multilaminated Descemet’s membrane (Black arrow) at the periphery. (Original magnification ×100, Periodic Acid Schiff).

Fig. 3

(A) The clinical appearance of the bilateral corneal opacity in Case 5. (B) The elevated central part of the right eyelid skin because of the underlying dome-shaped corneal mass. (C) The histopathology of the corneal opacity in the right eye showing neovascularization and adherent iris tissue. (Original magnification ×50. Hematoxylin & Eosin). (D) Higher magnification of the anterior part of the cornea showing keratinized thick epithelium. (Original magnification ×100. Hematoxylin & Eosin). (E) The histopathological appearance of the dome-shaped area of the cornea showing sub-epithelial myxoma composed of stellate and spindle cells. (Original magnification ×200. Hematoxylin & Eosin).

Fig. 4

(A) Clinical appearance of the right cornea with unilateral central opacity in case 6. (B) The clinical appearance of the clear left eye cornea in the same patient. (C) The histopathological appearance of the right cornea showing central stromal scarring and infiltration by chronic inflammatory cells in addition to the absence of Descemet’s membrane. (Original magnification ×100. Hematoxylin & Eosin). (D) Higher magnification of the inflammatory cells, which are positive with CD68 stain. (Original magnification ×400).