PURPOSE: Protein C deficiency is an autosomal recessive disorder, which predisposes the patient to potentially blinding and widespread lethal thromboembolic complications, especially in the homozygous type. We here report the first Korean case of ophthalmic involvement and its surgical treatment in homozygous protein C deficiency. METHODS: A 3.4kg, full term girl was born by normal delivery but showed bilateral leukocoria on day 2. Laboratory results disclosed a very low protein C activity level (10%) in the patient and moderately decreased levels in the other family members. Ophthalmic examination showed bilateral corneal opacity and shallow anterior chamber. B-scan ultrasonography which showed intravitreal mass lesions without microphthalmos and a funnel-shaped retinal detachment suggested bilateral retinal dysplasia. RESULTS: As the eyes were under progression of secondary glaucoma, bilateral lensectomies were performed at 2 months old and corneal opacity was regressed to some degree. However, at 14 months old, the left eye showed moderate corneal opacity with a band keratopathy. CONCLUSIONS: Although visual outcome was very poor after surgery, we could impede or slow down the progression of secondary glaucoma and save the eyeballs in the infant with homozygous protein C deficiency.
Anterior Chamber/ultrasonography ; Cataract/etiology ; Female ; Glaucoma/*etiology ; *Homozygote ; Humans ; Infant, Newborn ; Lens, Crystalline/surgery ; Protein C Deficiency/*complications/*genetics ; Retinal Diseases/*etiology

PURPOSE: To determine the proper time frame in which to assess retinal attachment status and to evaluate the superior retinal attachment status by performing B-scan ultrasonography in different positions on patients who have undergone pars plana vitrectomy and gas tamoponade. METHODS: In this prospective study, 23 patients (23 eyes) who had undergone pars plana vitrectomy and gas tamponade were investigated between June 2005 and February 2006. SF6 (18%) was injected into the vitreous cavity of 15 eyes, and C3F8 (14%) was injected into the vitreous cavity of 8 eyes. At postoperative day 1, day 3, week 1, week 2, and week 4, B-scan ultrasonography was performed in the supine, sitting, prone, right decubitus, and left decubitus positions. RESULTS: The proper time to evaluate the retinal attachment status was three days post-operatively in 10 eyes (66.7%) with SF6 (18%) injections and two weeks post-operatively in six eyes (75%) that had C3F8 (14%) injections. The superior retinal attachment status can be evaluated from the summation of B-scan ultrasonography results performed in the right decubitus and left ducubitus positions. CONCLUSIONS: The proper time to evaluate the retinal attachment status was related to the degree of the gas absorption when performing B-scan ultrasonography after pars plana vitrectomy and gas tamponade. To evaluate the entire retina, it is useful to perform B-scan ultrasonograhy in the prone, right decubitus and left decubitus positions.
Adult ; Aged ; Aged, 80 and over ; Air ; Female ; Follow-Up Studies ; Humans ; Injections ; Male ; Middle Aged ; Postoperative Period ; Prospective Studies ; Retina/*ultrasonography ; Retinal Diseases/*surgery/ultrasonography ; Time Factors ; Treatment Outcome ; Vitrectomy/*methods ; Vitreous Body