[Post-operative rehabilitation of functional amblyopia in primary infantile glaucoma: 11 year-follow-up]. / Rééducation de la part fonctionnelle de l'amblyopie dans le glaucome primitif infantile opéré, recul de 11ans.

PURPOSE: To evaluate the efficacy of functional amblyopia treatment in children having undergone surgery for primary infantile glaucoma. PATIENTS AND METHOD: Parameters studied included: age at surgery, current age, visual acuity (VA) (initial and most recent acuity), refraction, alignment, adherence to amblyopia treatment, condition of the cornea (Haab striae, stromal scarring, pachymetry, cell count), condition of the optic disc (cupping, RNFL OCT) and intraocular pressure. Pearson's p-value was fixed at 5%. RESULTS: Retrospective study of 29 eyes; average age at surgery was four months, mean follow-up was 11.2 years. Initial VA averaged 4.5 over 10, most recent VA 7 over 10, i.e. a gain of 2.3 over 10. Refraction revealed 37.9% myopia, 10.3% emmetropia, 48.2% hyperopia and an average astigmatism of 2.14 D. A negative correlation was found between astigmatism and most recent VA (rho=-0.7; P<0.001). A positive correlation was found between the quality of the occlusion therapy and the recovery of VA with a gain of 3.2 over 10 (P=0.001). A strong positive correlation was found between the cylinder power and the number of Haab striae [rho 0.702, P<0.0001]; final VA is best when the striae show no organized scar formation (P=0.04). CONCLUSION: This study underlines the necessity of prolonged treatment of functional amblyopia in primary infantile glaucoma for the best possible visual rehabilitation.

[MRI in Duane retraction syndrome: Preliminary results]. / Apport de l'IRM dans le syndrome de Stilling-Duane : résultats préliminaires.

INTRODUCTION: Duane retraction syndrome (DRS) is a congenital ocular motility disorder with innervational dysgenesis. MRI improves our understanding of this disease by providing in vivo access to nerves and oculomotor muscles. The goal of this prospective study (2000-2008) was to analyze DRS clinically and neuroradiologically. PATIENTS AND METHODS: Twenty-four patients (27 eyes) received a complete ophthalmologic evaluation and a brain-orbital MRI. The average age was 6.1 years. MRI was performed with 3D T2 CISS-weighted images through the brainstem to visualize the cisternal segments of the cranial nerves and the orbit (lateral and medial recti muscles). MRI anomalies were classified according to type I, II, and III and depending on their condition in the posterior fossa (absence, hypoplasia) and in the orbit (muscle anomalies). RESULTS: Of 27 eyes, 70% were type I, 19% type II, and 11% type III. MRI showed abducens nerve abnormalities in 93% of the cases (78% absence) and muscle abnormalities in 57.5% of the cases. A detailed description showed 100% abducens nerve abnormalities and 58% abnormal lateral rectus muscle in type I, 60% abducens nerve abnormalities and 60% abnormal lateral rectus muscle in type II, and 100% abducens nerve abnormalities and 66% abnormal lateral rectus in type III. DISCUSSION-CONCLUSION: This study presents two major findings: detection of abducens nerve abnormalities in most cases of DRS whatever the type, associated with muscle abnormalities, and the confirmation that this absence may exist in type II (2/5). Thus MRI proved to be a valuable tool for investigating these patients, improving the comprehension of the physiopathogenics of this disease.

[Prospective study on the repercussions of oculomotor surgery on children's corneal endothelium]. / Etude prospective sur la répercussion de la chirurgie oculomotrice sur l'endothélium cornéen de l'enfant.

PURPOSE: To compare corneal endothelial cells following strabismus surgery in children. METHODS: Thirty-four children, 18 boys and 16 girls (mean age, 8.21 years) who had undergone strabismus surgery were included in a prospective study from September 2007 to November 2008. Following non-contact specular microscopy (type Topcon SP 2000P), endothelial cell density (ECD), endothelial pleomorphism (hexagonality (H)) and area cells (polymegethism) were measured in a 3 x 6-cm area. Four groups were established according to the type of surgery: group 1 (n=8), isolated posterior fixation on the medial rectus; group 2 (n=18), posterior fixation with recession of the medial rectus; group 3 (n=19), recession of the lateral rectus muscle; group 4 (n=5), resection of the superior oblique muscle. RESULTS: The mean preoperative values for ECD, hexagonality (H) and cell areas were 3413.8 cells/mm(2), 68.4 % and 11.62 % for 100 microm(2), 39.56 % for 200 microm(2), 36.66 % for 300 microm(2), 8.78 % for 400 microm(2), 1.59 % for 500 microm(2) and 1.79 % for 600 microm(2), with homogenous distribution. The mean postoperative values were 3391.9 cells/mm(2), 63.95 % (-6.16 %, statistically significant). Postoperatively, we found a nonsignificant increasing percentage for 200 and 300microm(2) and decreasing for 400 and 500 microm(2). Group 1 showed mean pre- and postoperative ECD at 3501.7 + or - 399.7 and 3499.7 + or - 243.28 cells/mm(2), respectively; H was + 0.43 %; group 2 showed 3498.3 + or - 182.8 and 3422.8 + or - 274.2 cells/mm(2), respectively; -13.66 % for H (p=0.039); group 3 had 3238 + or - 385.5 and 3366 + or - 334.4 cells/mm(2), respectively; -3.29 % for H; and group 4 had 3264 + or - 325.36 and 3328 + or - 124.42 cells/mm(2), respectively; -6.2 % for H. A decrease in H was found in group 2 (p<0.0001). DISCUSSION-CONCLUSION: Strabismus surgery cannot result in a significant decrease in ECD but reveals pleomorphism changes (significant) for the most aggressive procedure.

[MRI in congenital nystagmus]. / Apport de l'IRM dans les nystagmus congénitaux.

INTRODUCTION: Congenital nystagmus (CN) that is present by the age of 3 months is the most common form of nystagmus in childhood. We present a prospective study (2001-2008) in which we report imaging findings in 48 children with CN. MATERIALS AND METHODS: Twenty-six boys and 22 girls with CN underwent a complete ophthalmologic assessment and a cerebral MRI (mean age of examination under general anesthesia: 11 months). Three CN groups were formed: neurologic (n=27), sensory visual disturbance (n=14), and isolated (n=7). Cerebral MRI was interpreted by the same pediatric neuroradiologist (NG). Of the children studied, 98 % were born at term. The MRI abnormalities were classified as morphologic abnormalities (malformative or nonmalformative) and as signal abnormalities. The location of brain abnormalities was within the posterior fossa, (brain stem, cerebellum, dental nuclei, cisterna magna) and the cerebral hemisphere (white matter, perivascular spaces, midline commissures, basal ganglia). RESULTS: Pendular nystagmus was prevalent in sensory and neurologic nystagmus. On fundus examination, optic disc abnormalities were present in 70 % (19) of neurologic CN and associated with white matter abnormalities of the optic radiations in 40 % of cases. On MRI, malformative morphologic abnormalities were present in 27 cases, nonmalformative abnormalities were found in 67, and signal abnormalities in 68. Within the brain stem, signal abnormalities were found as a cockade appearance of the posterior pons in the reticular regions (neurologic n=14, sensory n=6, isolated n=3). Other bright (most frequent) signal abnormalities were found within the dentate nuclei of the posterior fossa (neurologic n=10, sensory n=3, isolated n=3) and the cerebral white matter (neurologic n=17, sensory n=7, isolated n=5) of which 24 (neurologic n=15, sensory n=5, isolated n=4) involved the optic radiations. Most of these abnormalities were related and were seen most frequently in neurologic nystagmus. The most frequent association was signal abnormalities of the white matter, ventricular dilatation, and dilatation of the perivascular spaces (60.4 %) (neurologic n=13, sensory n=6). CONCLUSION: This study showed the fundamental contribution of the cerebral MRI in CN. Cerebral abnormalities were found at the pathways for ocular motility, particularly at the saccadic pathways.

[Primary exotropia: importance of cerebral MRI]. / Intérêt de l'IRM cérébrale dans les exotropies précoces.

INTRODUCTION: Primary exotropia is a divergent strabismus that appears from the first day of life to the second year. It can be isolated or associated with a pathological context requiring cerebral imaging to determine diagnosis and prognosis. The objective of this study was to report the sensorimotor state and the result of MRI in infantile exotropia. PATIENTS AND METHOD: Forty-seven children with primary exotropia had a complete ophthalmologic assessment (visual acuity, binocular vision, refraction with cycloplegia, eye fundus) and cerebral MRI (average age, 16 months). Deviation was classified into four categories<4 degrees, > or =4<10 degrees, > or =10<20 degrees, > or =20 degrees. MRI results were classified into seven categories: white matter abnormalities (gliosis, delay of maturation, periventricular leukomalacia, aspecific hyperintense signal, and necroses); Virchow-Robin enlargement space and enlarged subarachnoid space; gray matter abnormalities (necroses, cerebral atrophy, occipital cortex, basal ganglia); ventriculomegaly; thin corpus callosum; cerebellar injury; and tumor. RESULTS: There was a statistically significant increase in the rate of pathological MRI as the angle deviation increased: 76.6% of patients had a pathological ophthalmologic exam (amblyopia, ptosis, head posture, Duane's syndrome, cataract, albinism, or pigmentary retinopathy). We found 38% nystagmus and 38% optic nerve hypoplasia. There was a real pathological context in 61.7% of the exotropia cases: 27.7% prematurity, 31.9% fetal distress, 21.3% facial dysmorphy (plagiocephaly), 12.8% psychomotor delay, and 14.9% epilepsy. Only three children had isolated exotropia. Thirty-four cerebral MRI (72.3%) were not normal. In pathological MRI, there was 61.8% white matter injury, 41.2% gray matter injury, and 17.6% thin corpus callosum. CONCLUSION: This study demonstrates the fundamental contribution of cerebral MRI in infantile exotropia. The greater the deviation, the more abnormal the MRI results are. The pathological context and ophthalmological abnormalities are important in infantile exotropia.