[Isolated Unilateral Strabismus Sursoadductorius (Congenital Trochlear Palsy) with and without Hypotrophia of the Superior Oblique Muscle-Orthoptic Differences in Adulthood].

Purpose: The clinical feature of unilateral decompensating strabismus sursoadductorius (dSSA; often called congenital superior oblique palsy, CSOP) is not an etiologically uniform entity. Hypotrophy of the superior oblique muscle (HMOS) is a frequent and immediate cause of dSSA/CSOP. In this study, clinical characteristics of dSSA/CSOP with and without HMOS are compared.

Independent NF1 and PTPN11 mutations in a family with neurofibromatosis-Noonan syndrome.

Neurofibromatosis-Noonan syndrome (NFNS), an entity which combines both features of Noonan syndrome (NS) and neurofibromatosis type 1 (NF1), was etiologically unresolved until recent reports demonstrated NF1 mutations in the majority of patients with NFNS. The phenotypic overlap was explained by the involvement of the Ras pathway in both disorders, and, accordingly, clustering of the NF1 mutations in the GTPase-activating protein (GAP) domain of neurofibromin was observed in individuals with NFNS. We report on an 18-month-old girl with typical findings suggestive of NS in combination with multiple café-au-lait spots and bilateral optic gliomas suggestive of NF1.

Results and complications of hydrophobic acrylic vs PMMA posterior chamber lenses in children under 17 years of age.

Purpose: To compare results and complications of implantation of hydrophobic acrylic foldable intraocular lenses in children with those of polymethylmethacrylate (PMMA) intraocular lenses.

Methods: In a retrospective study, we analyzed results of cataract surgery with posterior chamber lens implantation in 30 eyes of 30 patients aged 1-16 years. In 10 eyes, acrylic (Alcon AcrySof) intraocular lenses, and in 20 eyes, single-piece PMMA posterior chamber lenses were implanted.

[Amblyopia of the fellow eye in assumed unilateral congenital fibrosis syndrome].

Background: Amblyopia of the ptotic eye in spite of head posturing and of the fellow eye in ptosis with motility disorders are well known. We emphasize the importance of exact ophthalmological and orthoptic examinations to avoid amblyopia in both ptosis and fellow eye, even in cases of mild ptosis, especially if they are combined with motility disorders.

Patient: A 5 year-old girl presented to our outpatient clinic because of ptosis of the left eye and hypertropia of the right eye.

[Congenital ptosis: amblyogenic refractive errors, amblyopia, manifest strabismus and stereopsis related to the types of ptosis. Data on 77 patients and review of the literature].

Background: Former reports on amblyogenic refractive errors, amblyopia and binocular vision in congenital ptosis usually comprise all forms of ptosis without any differentiation. This study is an analysis of different kinds of ptosis.

Patients And Methods: 154 eyes (98 ptotic eyes) of 77 patients with congenital ptosis aged > or = 1 year (56 unilateral ptoses: 45 simple, 1 with rectus superior paresis, 7 with Marcus Gunn's syndrome, 2 congenital oculomotor palsies, 1 unilateral fibrosis syndrome; 21 bilateral ptoses: 10 simple, 2 with bilateral double elevator paresis, 7 blepharophimosis syndromes and 2 bilateral fibrosis syndromes) were investigated concerning visual acuity, refractive error (94 % in cycloplegy), strabismus and stereo acuity.