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Shortly after birth, a massive enlargement of the right eye was observed in an otherwise healthy male child. The cornea of the affected eye was vascularized and completely cloudy without a sharp border between cornea and sclera. The diagnosis of a congenital glaucoma was made but an operation was not undertaken because of the difficult anatomical situation and the lost function. When the child was almost 3 years old enucleation was performed to prevent complications due to corneal exposure, and to improve the cosmetic aspect. The morphological investigations of the enucleated eye disclosed findings typical of what is called in the literature "congenital anterior staphyloma" or "congenital corneal staphyloma", namely a massively staphylomatous cornea with superficial neovascularization, destruction of Bowman's layer, and absence of Descemet's layer as well as corneal endothelium. Angle structures were completely absent, and the corneal back-side was lined by a pigment epithelial layer and focally by an additional inner layer of non-pigmented epithelium. There was no anterior chamber. The lens was markedly diminished in size (microphakia) and partly embedded in the corneal stroma. Pars muscularis and pars ciliaris of the ciliary body were separated. Elongated, thin ciliary processes were extended towards the small lens while the pars muscularis was fully covered by the retina. This rare, complex malformation syndrome which can be easily distinguished from primary congenital glaucoma should not be reduced conceptually to the corneal staphyloma because this staphyloma constitutes only a part of the whole. Taking the leading morphological aberrations into consideration we would rather propose the new term " corneal staphyloma- anterior chamber agenesia- microphakia syndrome (CSAMS). We hypothesize that CSAMS may be due to a pathological fusion of the early anterior optic cup. As the posterior eye segment is often normal in CSAMS, a staphyloma excision along with a sclero-keratoplasty might be an alternative therapeutic option to avoid enucleation and restore ambulatory vision.


J M Rohrbach, D Süsskind, P Szurman, K Siepmann. Corneal staphyloma-anterior chamber agenesia-microphakia syndrome]. Klinische Monatsblätter für Augenheilkunde. 2006 Feb;223(2):168-75

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PMID: 16485232

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