ABSTRACT.Purpose: To report the outcome of chronic bilateral keratitis and other ocular manifestations in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Material and Methods: The material consists of 69 patients with APECED, who were examined by the senior author as referrals from the pediatricians. The patients were seen at the Helsinki University Eye Hospital a total of 370 times and the follow-up periods range from 2 to 25 years. Results: 25% of the patients had chronic bilateral keratitis with symptoms of intense photophobia, blepharospasm and lacrimation. On 13 patients the first symptoms appeared before the age of 4 years. Keratitis was the first presenting sign before any evidence of systemic disease in three patients, and in all patients keratitis was among the first three manifestations of the syndrome. The clinical picture from the acute to the chronic cicatricial stage is described. The best corrected visual acuity at the end of the follow-up was 0.6 or better in six patients only. Of the total material 12 patients had lenticular opacities as the manifestation of hypoparathyroidism. Conclusions: Chronic bilateral keratitis is an ocular manifestation of APECED and in its etiology both hypoparathyroidism and candidiasis may be ruled out as single causes. When an ophthalmologist is confronted with a young child with bilateral chronic keratitis with symptoms of intense photophobia, blepharospasm, lacrimation and either with mucocutaneous candidiasis or hypoparathyroidism, the patient should be referred to a pediatric endocrinological consultation for the possibility of underlying APECED.

ABSTRACT.Purpose: To report a case of delayed fungal endophthalmitis by Paecilomyces variotii following uncomplicated cataract surgery. To our knowledge this is the first reported case of postoperative endophthalmitis by this species. Methods: We report the longterm clinical follow-up of an 83-year-old female who underwent uncomplicated sutureless, small-incision cataract surgery. She developed recurring uveitis 4 months after surgery. Vitreous tap and finally complete vitrectomy with removal of the capsular bag including the intraocular lens were performed. Fungi were studied by histopathology and culture. Results: At histopathological examination, the fungi were found to be closely related with the capsular bag. A few mononuclear inflammatory cells were encountered. At culture, Paecilomyces variotii, a common ubiquitous non-pathogenic saprophyte, was identified. Despite systemic, intravitreal and topical antifungal therapy after vitrectomy the uveitis recurred several times, but no fungal organisms were isolated from the repeat intraocular specimen. At 18 months postoperatively the subject's visual acuity was finger counting at 2 metres. At the time of surgery the operating room air-conditioning system was undergoing repairs. Cases of fungal endophthalmitis after contamination from air-conditioning ventilation systems have been reported before, but none of the cases reported have been caused by P. variotii. Conclusion: P. variotii, a non-pathogenic environmental saprophyte, may be disastrous if introduced into the eye. International recommendations on the environmental control of the operating room air-conditioning ventilation system should be strictly followed. No intraoperative surgery should be undertaken while the air-conditioning system is undergoing repairs or service.

Occurrence of exfoliation syndrome was studied in 172 eyes enucleated for haemorrhagic and absolute glaucoma in 1962-82, using histopathological techniques. Exfoliation was found in 33% of haemorrhagic glaucoma cases and in 22% of absolute glaucoma. In patients over 70, exfoliation was found in 53% of the haemorrhagic cases and in 46% of the absolute glaucoma cases. Capsular glaucoma may provoke haemorrhagic glaucoma and may lead to blindness and enucleation of eye with or without vascular catastrophe.

ABSTRACT.Background: Disabling, chronic, persistent keratoconjunctivitis is an essential ocular manifestation in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Purpose: Because of the paucity of previous studies our aim is to describe the main histopathological features of the keratopathy and to report the long-term outcome of corneal grafting. Conclusion:In chronic keratopathy of APECED the anterior corneal layers, the epithelium, the Bowman's membrane and the anterior corneal stroma are affected while the posterior cornea appears normal. As after keratoplasty rejection may be expected, its prevention and management need intensive attention.

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