PURPOSE: Cornelia de Lange syndrome is a rare disease showing characteristic facial appearance, developmental delay, growth retardation, low birth weight, skeletal formation anomaly, hirsutism and various ophthalmologic problems. METHODS: We experienced a case of an 18-year-old female with Cornelia de Lange syndrome showing superficial keratitis with entropion, ptosis, high myopia, lacrimal cutaneous fistula and characteristic facial appearance. She was born with low birth weight, operated for cleft palate and diagnosed with ventricular septal defect. In addition, she showed psychological lag and developmental impairment. RESULTS: We performed entropion correction surgery, administered medical therapy for superficial keratitis and prescribed glasses for her myopia. CONCLUSIONS: This is the first case report on the successful correction of entropion with Cornelia de Lange syndrome in Korea.
Adolescent ; *Asian Continental Ancestry Group ; Blepharoptosis/*complications ; De Lange Syndrome/*complications/*ethnology ; Entropion/*complications/surgery ; Eyeglasses ; Female ; Humans ; Keratitis/*complications/drug therapy ; Myopia/*complications/therapy

We report a case of CMV corneal endotheliitis that was treated with intravitreal ganciclovir injection. A 56-year-old man who has suffered from uveitis was referred to our clinic due to corneal endothelial abnormality. Slit lamp examination showed a localized sectoral corneal edema and linear keratic precipitates along the boundary of edema. In spite of treatment with oral steroid and acyclovir, the disease progressed and two new coin-like lesions were developed. After topical ganciclovir and intavitreal injection of ganciclovir, the corneal lesions disappeared.
Antiviral Agents/administration & dosage ; Cytomegalovirus Infections/*complications/*drug therapy ; Endothelium, Corneal/*virology ; Ganciclovir/*administration & dosage ; Humans ; Intravitreal Injections ; Keratitis/*drug therapy/*virology ; Male ; Middle Aged

We report a case of CMV corneal endotheliitis that was treated with intravitreal ganciclovir injection. A 56-year-old man who has suffered from uveitis was referred to our clinic due to corneal endothelial abnormality. Slit lamp examination showed a localized sectoral corneal edema and linear keratic precipitates along the boundary of edema. In spite of treatment with oral steroid and acyclovir, the disease progressed and two new coin-like lesions were developed. After topical ganciclovir and intavitreal injection of ganciclovir, the corneal lesions disappeared.
Antiviral Agents/administration & dosage ; Cytomegalovirus Infections/*complications/*drug therapy ; Endothelium, Corneal/*virology ; Ganciclovir/*administration & dosage ; Humans ; Intravitreal Injections ; Keratitis/*drug therapy/*virology ; Male ; Middle Aged

We report on a case of necrotizing keratoscleritis in primary Sjogren's syndrome. A 66-year-old female patient who was complaining of ocular pain, tearing and decreased vision in her right eye for the previous two days was admitted to our hospital. Visual acuity in the right eye was hand movement, and initial examination showed a 3.0 x 1.8 mm uveal mass bulging through a corneoscleral melting site in the nasal region of the right eye. Positive anti-nuclear antibody was identified at a titer of 1:320 with a speckled pattern, and both Sjogren's syndrome A and Sjogren's syndrome B antibody tests were positive, with titers >200 U/mL. A technetium 99m pertechnetate salivary scan revealed chronic sialoadenitis in the submandibular glands. We diagnosed the lesion as necrotizing keratoscleritis due to primary Sjogren's syndrome. A corneoscleral patch graft was performed, followed by immunosuppression including oral cyclosporin and topical prednisolone. During a follow-up period of 12 months, the corneoscleral graft was well maintained with no recurrence.
Aged ; Diagnosis, Differential ; Disease Progression ; Female ; Follow-Up Studies ; Glucocorticoids/administration & dosage ; Humans ; Keratitis/drug therapy/*etiology/pathology ; Necrosis ; Ophthalmic Solutions ; Scleritis/drug therapy/*etiology/pathology ; Sjogren's Syndrome/*complications/drug therapy/pathology ; Visual Acuity

This research focuses on four cases of patients having undergone eximer laser photorefractive surgery who were diagnosed with adenoviral keratoconjunctivitis during the postoperative period and who later developed epidemic keratoconjunctivitis (EKC)-like keratitis. Two of the patients had undergone laser-assisted subepithelial keratectomy (LASEK), one had undergone laser in situ keratomileusis and one had photorefractive keratectomy. After the surgery adenoviral keratoconjunctivitis and recurrent late-developing EKC-like keratitis were observed in the patients. Recurrent late-developing EKC-like keratitis occurred in one of the patients, who had received LASEK as many as three times. The others had only one or two episodes.The corneal infiltrates of keratitis mainly occurred in the central cornea. Successful resolution of recurrent late-developing EKC-like keratitis was achieved through the use of topical steroids without sequelae and the final best-corrected visual acuity was as good as the base line. These keratitis infiltrates have been presumed to represent an immune response to the suspected adenoviral antigens deposited in corneal stroma during the primary adenoviral infection. Previous reports argued that patients with a history of adenoviral ketatoconjunctivitis were succeptible to adenoviral keratoconjunctivitis becoming reactivated; however, in our research, our patients had their first adenoviral infections after the eximer laser photorefractive surgery and reactivation was confirmed. We recommend that attention be paid to adenoviral infection after laser refractive operations, because these patients seem to have more frequent recurrences.
Adenovirus Infections, Human/diagnosis/drug therapy/*etiology ; Adult ; Conjunctivitis, Viral/diagnosis/drug therapy/*etiology ; *Corneal Surgery, Laser ; Eye Infections, Viral/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Keratectomy, Subepithelial, Laser-Assisted ; Keratitis/diagnosis/drug therapy/*etiology ; Keratomileusis, Laser In Situ ; Lasers, Excimer/therapeutic use ; Photorefractive Keratectomy ; *Postoperative Complications ; Recurrence ; Young Adult