ABSTRACT
To report five cases of Wolfram syndrome, an autosomal recessive neurodegenerative disease with Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness[DIDMOAD syndrome]. All of the five patients had diabetes mellitus and optic atrophy. Four patients had hearing loss. In spite of persistence of polyuria and polydipsia, diabetes insipidus had been previously detected in only one patient and its presence was discovered after the diagnosis of the syndrome in the other three. Two brothers had open angle glaucoma. Although Wolfram syndrome is rare, it should be considered in all insulin-dependent diabetics in whom polyuria and polydipsia persists despite adequate control of blood sugar. It should also be considered in familial cases of diabetes mellitus type I. Presence of optic atrophy is a helpful sign for diagnosis. Fundoscopy is crucial for early diagnosis. With early management of diabetes insipidus, late urologic complications may be prevented. Quality of life may be improved with visual and hearing aids
ABSTRACT
To introduce the technique and results of vitrectomy surgery with removal of cyclitic membrane by placement of radial cuts over the membrane in traumatized eyes. Vitrectomy in 8 eyes of 8 patients after penetrating ocular trauma revealed that fibrovascular tissue was present over the pars plicata. To remove the membrane, radial cuts were placed over the membrane first and then the pieces were removed by vitrectom. Pre- and postoperative visual acuity [VA], applanation tonometry results, and ophthalmic examination results were retrieved from hospital records of the patients. The patients included 5 males and 3 females. Age range was 2-22 years [median; 11.5 Years]. All eyes had sustained penetrating globe injury involving the sclera 11 to 90 days [median; 23 days] before the operation. Two eyes had undergone vitrectomy once before. Preoperative VA was light perception or hand motion in 7 eyes and 7 eyes were hypotonic before the operation. Extent of the cyclitic membrane was from 150 to 360 degrees [median; 200 degrees]. Patients were followed for 6-1 8 months [median; 12 month]. At the last visit, VA was 20160 or more in 6 eyes and IOP was normal in 7 eyes [8-20 mmHg, median; 11 mmHg]. One eye was lost due to proliferative vitreoretinopathy. There were no other major retinal complications. Treatment of cyclitic membranes has always been difficult. The described technique of surgery had very good results in our patients
ABSTRACT
To evaluate the success rate of vitrectomy in giant retinal tears [GTR] and factors influencing it. In an interventional case series, hospital records of 68 eyes of 63 patients operated for GRT were reviewed. Standard three port vitrectomy plus placement of an encircling band, retinopexy, and internal tamponade was performed for all eyes. Possible influencing factors including age, sex, visual acuity, afferent pupillary defect, grade of PVR, size and location of GRT, macular status, extent of RD, interval from onset of symptoms to operation, co-existing eye diseases, surgical technique, post-operative complications, and results after silicone oil removal were evaluated. Anatomic success was classified as complete success, relative success, and failure. Mean age of the patients was 31.2+]-17.2 and 50 patients [79.3%] were male. Underlying ocular disease existed in 39 eyes, of which high myopia was the most prevalent [33.8%]. The eyes were operated after a mean of 30 days from beginning of symptoms. Silicone oil was used for internal tamponase in 64 eyes and was removed from 36 eyes. Patients were followed from 1 to 54.5 months [median 10 months]. Anatomic success rate was 73.7%. Preoperative stage of PVR was the only factor found to be associated with worse anatomic results. Anatomic success rate was comparable to that of other centers and significantly better than results of a previous from our center. Early operation of eyes with GRT before development of PVR results in better anatomic success