Amyloidosis of the orbit and adnexae.

PURPOSE: To present a series of patients with orbital and adnexal amyloidosis and illustrate the diversity of disease and the challenges of managing such cases. METHODS: Descriptive case series of ten patients with biopsy proven amyloidosis involving the orbit, conjunctiva and eyelids. The presentation, clinical findings and management are discussed for each case. RESULTS: All patients had some form of eyelid abnormality or malposition. Presenting complaints included ptosis, epiphora and ocular discomfort. Other clinical findings included conjunctival lesions and proptosis. The majority of patients had localised amyloidosis and one patient had systemic disease. Conservative management included lubrication and the use of bandage contact lenses. Surgical management included debulking, ptosis or other lid surgery. CONCLUSION: Amyloidosis can present to an Occuloplastic clinic in a wide variety of ways. Definitive diagnosis is based on the histopathological findings. Management is often challenging. Multi-disciplinary team involvement is critical in view of its systemic associations.

Spontaneous perforation of the cornea in mild keratoconus.

PURPOSE: Spontaneous corneal perforation during acute hydrops in keratoconus is a very rare complication and has only been described in patients with advanced keratoconus. We describe a case of spontaneous corneal perforation in mild keratoconus in a patient with no identifiable risk factors. METHODS: We report retrospectively on a 34-year-old woman with mild keratoconus (Krumeich stage II) who presented with the spontaneous perforation of the cornea. A literature review was undertaken. RESULTS: The handful of previously reported cases included patients with advanced keratoconus. Our patient had none of the risk factors identified in previously reported cases (eye rubbing, topical steroid use, and raised intraocular pressures). Corneal gluing was performed to restore globe integrity. She retained a visual acuity of 6/9 even through the acute episode. With the resolution of the acute episode, her corneal astigmatism improved and she achieved a final best-corrected visual acuity of 6/6 with spectacles. CONCLUSIONS: To our knowledge, this is the first reported case of spontaneous perforation of the cornea in mild keratoconus. We demonstrate that in this event, a good visual prognosis is possible from gluing alone.

A modified technique for descemet membrane stripping automated endothelial keratoplasty to minimize endothelial cell loss.

In an attempt to enhance postoperative survival of donor endothelium, the conventional technique for Descemet membrane stripping automated endothelial keratoplasty (DSAEK) was modified using the prototype of a glide specially designed to facilitate graft delivery and minimize surgical trauma. Instead of using the so-called taco technique, the Busin glide is loaded with the donor lamella, and a microincision forceps is inserted into a temporal side entry and passed across the anterior chamber, exiting through a nasal clear cornea tunnel to grab the graft and drag it into the eye. In 10 patients who underwent DSAEK, mean (SD) postoperative endothelial cell loss was 20.0% (2.6%) at 6 months, 23.5% (2.8%) at 12 months, and 26.4% (2.7%) at 18 to 24 months. Reduced trauma to the graft using our modified technique limits endothelial cell loss after DSAEK to the level recorded after conventional penetrating keratoplasty (PK).

Late detachment of donor graft after Descemet stripping automated endothelial keratoplasty.

We describe the case of an 85-year-old man with pseudophakic bullous keratopathy. Descemet stripping automated endothelial keratoplasty (DSAEK) was performed, and 6 weeks postoperatively, subtle separation of the 2 corneal layers was noted. To our knowledge, this case of post-DSAEK graft detachment involves the longest interval between surgery and successful reattachment. It provides new insight into the survival of detached grafts following DSAEK, as well as a possible treatment for this complication.

Peripheral ulcerative keratitis due to a 'long lost' hard contact lens.

Peripheral ulcerative keratitis (PUK) is a disorder consisting of a crescent-shaped destructive inflammation of the perilimbal corneal stroma. PUK can occur in a variety of ocular and systemic conditions including infections, lid abnormalities, dermatological disorders and connective tissue disorders. We present a case of PUK associated with a hard contact lens (CL) retained in the superior fornix for over 16 years. After removal of the embedded CL, a superior forniceal conjunctival pedicle graft was performed to prevent corneal perforation. The patient was managed postoperatively with a combination of topical steroids and antibiotics. The use of systemic immunosuppressive therapy was not necessary. Micro-trauma and micro-keratitis may have occurred as a result of the mechanical effect of the CL but if this was the sole mechanism, one would expect presentation at a much earlier date. We discuss the pathogenetic mechanisms which may have contributed to the development of this ulceration. This report highlights the importance of lid eversion when examining patients with anterior segment pathology.

A simple correction for congenital tarsal kink associated with distichiasis.

Congenital tarsal kink is a rare condition. The folded edge of the upper tarsus, or the inturned lashes, may traumatize the cornea causing ulceration. We describe a case of unilateral upper eyelid horizontal tarsal kink associated with distichiasis and congenital corneal ulceration. Several different surgical treatment options have been previously alluded to in the literature including lamellar tarsoplasty, full-thickness eyelid fracture, and rotation and excision of the kink with tarsal reapposition. Repair by means of eyelid everting sutures via an anterior lamellar approach is presented as a novel technique to correct this congenital anomaly.