Tyrosinemia type II: nine cases of ocular signs and symptoms.

PURPOSE: To report nine cases of tyrosinemia type II, with ocular signs and symptoms. METHODS: Participants included nine patients (18 eyes) who were followed for a mean follow-up period of 6.5 years (range, 2 to 8 years). Intervention included dietary restriction of tyrosine and phenylalanine, which led to resolution of ocular and cutaneous lesions, improved behavior in one patient, and may have prevented developmental delay in others. The main outcome measures were visual acuity and serum tyrosine levels to determine the response to dietary therapy. Intelligence testing and developmental screening were performed when appropriate. RESULTS: All patients presented with ocular signs and symptoms as the primary manifestation of disease and serve to illustrate the typical ocular findings of this syndrome. Three patients presented with cutaneous manifestations, and one patient had mild mental impairment at the time of diagnosis. CONCLUSIONS: Recalcitrant pseudodendritic keratitis may be the presenting sign in tyrosinemia type II. Awareness of the presenting signs and symptoms may accelerate the diagnosis and dietary intervention. Initiation of a tyrosine-restricted and phenylalanine-restricted diet in infancy is most effective in preventing cognitive impairment.

Pituitary adenylate cyclase-activating polypeptide induces hyperthermia in the rat.

The effects of centrally administered pituitary adenylate cyclase-activating polypeptide (PACAP-38) on body temperature were investigated in rats. Intracerebroventricular (i.c.v.) administration of PACAP-38 in doses of 500 and 1000 ng induced a dose-related elevation in colon temperature 2, 3, 4, 5 and 6 h after injection. The i.c.v. pretreatment of the animals with different dilutions of PACAP-38 antiserum prevented the development of hyperthermia in PACAP-38-treated animals, whereas PACAP-38 antiserum alone did not modify the colon temperature. An intramuscular injection of noraminophenazone (a cyclooxygenase inhibitor) abolished the PACAP-38-induced hyperthermia. Our data indicate that PACAP may induce hyperthermia via the central nervous system, and this hyperthermic effect may be mediated via a cyclooxygenase-involved pathway.

Obesity as a factor in penetrating keratoplasty.

PURPOSE: We reviewed the clinical data on 10 obese patients to define the role of obesity as a factor for consideration in planning penetrating keratoplasty. METHODS: We performed a retrospective case series review of penetrating keratoplasty in obese patients. RESULTS: In our series of 10 patients, spontaneous extrusion of lens and vitreous (three patients) and choroidal hemorrhage (one patient) were intraoperative complications. Wound leakage, wound dehiscence (two patients), iridocorneal synechia formation, and graft rejection (one patient) were postoperative complications. Despite these complications, seven of the 10 patients achieved a final postoperative visual acuity of > or =20/30. CONCLUSION: Cornea surgeons must consider obesity as a potential risk factor that may be mitigated partially by careful preoperative patient evaluation, anesthesia planning, and meticulous attention to patient positioning and comfort during surgery.

Staphylococcal endophthalmitis following cataract extraction in a patient with Darier's disease.

PURPOSE: To report staphylococcal endophthalmitis following cataract extraction in a patient with Darier' s disease. METHODS: A 67-year-old man presented with decreased visual acuity OD and hypopyon 3 days status post-cataract extraction with intraocular lens placement. The patient was hospitalized and placed on topical and intravenous antibiotics. A diagnostic vitreous tap, pars plana vitrectomy, and intravitreal antibiotic installation were performed. RESULTS: Vitreal tap cultures indicated Staphylococcus epidermidis. His clinical status improved after vitrectomy and antibiotic therapy. The same bacteria was cultured from the patient's eyelids. CONCLUSIONS: The source of the S. epidermidis was the skin lesions on the patient's face and eyelids. Darier's disease is an exfoliative hyperkeratotic skin disease that affects all areas on the body except the buttocks. Although clean, sterile surgical techniques were followed, the risk of endophthalmitis following intraocular surgery in a patient with Darier's disease may be increased due to his or her dermatologic condition.

Repair of Descemet's membrane detachment with perfluoropropane (C3F8)

PURPOSE: To report the use of perfluoropropane (C3F8) gas in the repair of Descemet's membrane detachments. METHODS: Descemet's membrane detachments after cataract surgery in three eyes and pars plana vitrectomy in a fourth underwent anterior-chamber gas-exchange descemetopexy with an isoexpansile 14% mixture of C3F8 to facilitate reattachment of Descemet's membrane. RESULTS: Descemet's membrane detachment was successfully reattached after anterior-chamber gas exchange with 14% C3F8 in three of the four eyes treated. The fourth eye treated with 14% C3F8 probably failed Descemet's membrane reattachment because of an unrecognized viscoelastic bleb situated anterior to Descemet's membrane. No corneal decompensation or fluctuations in intraocular pressure were believed to be attributable to isoexpansile C3F8 gas exchange. CONCLUSION: Early recognition and repair of Descemet's membrane detachments may prevent complications, such as corneal decompensation, corneal opacities and edema, and an overall decline in visual acuity. Isoexpansile C3F8 is demonstrated as a safe and efficacious alternative for the repair of Descemet's membrane detachment.

Vasoactive intestinal polypeptide induces analgesia and impairs the antinociceptive effect of morphine in mice.

Vasoactive intestinal polypeptide (VIP) has numerous regulatory roles in peripheral, endocrine organs and in the central nervous system. The present study related to the effects of centrally (intracerebroventricularly) administered VIP on pain sensitivity and on opiate tolerance and dependence in intact male CFLP mice. VIP was analgesic when administered alone centrally. Naloxone treatment abolished this analgesic effect. VIP decreased the analgesic effect of a single subcutaneous morphine injection and the development of chronic tolerance to morphine. Morphine withdrawal signs were not significantly affected after VIP pretreatment. These findings indicate that VIP may play a role in pain sensitivity and that an opiate component may participate in this effect.

The results of penetrating keratoplasty for pellucid marginal corneal degeneration.

Over a 14-year period from 1974 to 1988, 12 eyes of 11 patients with pellucid marginal corneal degeneration underwent penetrating keratoplasty. Peripheral corneal thinning required a large eccentric graft in each case. Follow-up ranged from one to eight years (mean, three years). One graft failed because of a persistent epithelial defect with keratolysis of the wound. Although endothelial allograft rejection was common, occurring in seven of 11 (64%) clear grafts, no graft failed because of rejection. Other complications included retinal detachment and a bacterial corneal ulcer. Suture erosion and vascularization of the graft were not problems. Postoperative spectacle correction was dispensed an average of 11 months after surgery. Visual acuity in seven patients without amblyopia, retinal disease, or a previous corneal ulcer at the time of spectacle correction ranged from 20/20 to 20/40 (mean, 20/30). Average final keratometric astigmatism in these patients was 2.46 diopters (range, 0.00 to 5.25 diopters). We believe that penetrating keratoplasty offers an excellent surgical result for patients with pellucid marginal corneal degeneration.

Scanning electron microscopic evaluation of the effects of intracameral injection of cyanoacrylate in the rabbit.

We examined, using scanning electron microscopy, the effects of the injection of an isobutyl cyanoacrylate into the rabbit anterior chamber. Injection of the adhesive produced a rapidly polymerized mass that remained in the anterior chamber throughout the course of the study. Additionally, an active inflammatory response was noted in the anterior chamber, characterized by a progressively enlarging "cocoon" of fibrin and inflammatory cells surrounding the polymerized adhesive, as well as inflammatory cells in the trabecular meshwork. Following cyanoacrylate injection, the corneal endothelial cells were noted to be swollen for the first 14 days of the study.