Ophthalmological manifestations in 6 patients diagnosed with Williams-Beuren syndrome and literature review.

Medical history of 6 patients diagnosed with Williams-Beuren Syndrome (SWB) in pediatric age was revised. All the patients presented characteristic elf facies and cardiovascular abnormalities. All presented good visual acuity, except one case of unilateral amblyopia. The most frequent refractive error was hyperopia (n = 6; 100%) and astigmatism (n = 5; 83.3%). Ocular motility alterations were found in 2 patients (1 case of exophoria with hyperfunction of right inferior oblique and another of congenital endotropia with bilateral hyperfunction of inferior oblique). On the cognitive function, 66.7% (n = 4) had visoperceptive disorders. Other findings were epicanthus (n = 6; 100%) and congenital obstruction of the nasolacrimal duct with unilateral epiphora (n = 1; 16.7%). SWB is a rare disorder with complex ophthalmological and systemic manifestations. For this reason, ophthalmological follow-up of these children is recommended.

Septo-optic dysplasia: Ophthalmological abnormalities in a series of 5 cases.

Septo-optic dysplasia (SOD) is a rare congenital condition of unknown cause, with a characteristic triad that includes optic nerve hypoplasia, pituitary function abnormalities and midline brain defects, in addition to a broad spectrum of symptoms and associations. A total of five clinical cases are presented, four of which met the complete classic triad. All of them showed a wide variety of ophthalmological, endocrinological and neurological alterations. Within the ophthalmological spectrum of SOD, papillary hypoplasia and ocular motility alterations (nystagmus, strabismus) stand out. Other less frequent ones may also appear, such as pupillary alterations, microphthalmia and coloboma. Given the suspicion of SOD, brain MRI scan should be performed, as well as consultation with the paediatric department in order to complete the study and indicate, if necessary, systemic treatment.

Prospective study of cooperation and satisfaction in glaucoma surgery under sub-Tenon's anesthesia.

OBJECTIVES: To evaluate the analgesic effect as well as patient cooperation and satisfaction with sub-tenon's anesthesia in glaucoma surgery. MATERIAL AND METHODS: Prospective uncontrolled study. Fifty-three patients who underwent glaucoma surgery, with or without cataract extraction, with sub-Tenon's anesthesia have been studied. Satisfaction and cooperation were evaluated on a qualitative scale. The pain level was quantified using a numerical scale from 1 (no pain) to 10 (unsupportable pain). RESULTS: The mean pain score at the different periods was: 2.13 (standard deviation [SD] 1.47) on administering anesthesia, 1.74 (SD 1.27) during the surgery, 1.63 SD (1.23) immediately after the procedure, 1.38 (SD 0.38) 30 min later, and 1.38 (SD 0.63) when leaving the recovery unit. The postoperative satisfaction was "fairly or very satisfied" in 92.6% of the cases for both the surgeons and the patients. Twenty-four patients complained during the surgery, 15 of them at the conjunctival suture; in these cases additional anesthesia was administered, and, in 4 cases, intravenous paracetamol was given. Pain scores were significatively higher during the surgery (P=.033), and immediately after the procedure (P=.027) in trabeculectomy than in deep sclerectomy patients. CONCLUSIONS: The good analgesic level reached led to a high level of patient and surgeon satisfaction, even in longer procedures such as deep sclerectomy combined with cataract extraction, or implantation of drainage devices. Pain scores were significatively higher in trabeculectomy than in deep sclerectomy patients.