[Aphakia correction with rigid contact lenses in congenital cataract]. / Cataractes congénitales : correction de l'aphaquie par lentilles de contact rigides.

PURPOSE: To describe follow-up and evaluate functional results of rigid gas-permeable contact lens wear for the correction of aphakia in infants after surgery for congenital cataract. PATIENTS AND METHODS: This retrospective study was performed on 23 eyes of 17 children (12 boys and five girls), between three days and 36 months of age (median 3.5 months), undergoing cataract extraction without primary intraocular lens implantation and fitted only with Menicon(®) Z material contact lenses. Initial fitting visits included history and family education. Subsequent examinations were performed at eight days, 15 days, 1 month, 6 months and 1 year. The four main parameters studied were fit characteristics, ease of manipulation, ocular integrity and lens integrity. Concurrently, functional results (visual acuity, oculomotor balance) and various prognostic factors (associated pathology, age at time of surgery, cataract density, unilaterality versus bilaterality, postoperative complications) were evaluated. Rigorous systematic amblyopia rehabilitation was performed. RESULTS: At the one-month exam, 60% of parents demonstrated proficiency with manipulating the lens. On one-year follow-up, three cases of failure were attributable to difficulties with lens manipulation. At one year, one infectious complication was detected. The lens material demonstrated good resistance to breakage (9% at one month) and only a mild tendency toward deposits (few or no deposits in 95% of cases at 1 month). With regard to visual outcome, among the children old enough to cooperate, visual acuity was better than 3/10 for nine out of 12 eyes, of which five eyes were 10/10. With regard to alignment, we noted 12 cases (70.6%) of strabismus postoperatively (ten esotropia, two exotropia). The factors predictive of good visual acuity correlated with those described in the literature: partial cataract, bilateral cataract, and absence of postoperative oculomotor disturbance. CONCLUSION: The characteristics of rigid gas-permeable contact lenses provide safety and efficacy in aphakic infant eyes. They represent an attractive alternative to primary intraocular lens implantation in congenital cataract.

[Ocular biometry in children with hypermetropia: utility of the Lenstar LS 900 optical biometer (Haag-Streit(®))]. / Étude de la biométrie oculaire chez l'enfant hypermétrope : apport du biomètre Lenstar LS 900 (Haag-Streit(®)).

PATIENTS AND METHODS: Biometric measurements were recorded in the eyes of 238 children with hypermetropia (3-16 years of age), using the Lenstar 900 biometer (Haag-Streit(®)) with no contact. Four refractive groups were divided by objective refraction and spherical equivalent (group 1, more than 6.00 D; group 2, +4.50 to +99 D; group 3, +3.00 to +4.49 D; group 4, +1.50 to +2.99 D). Many parameters (i.e., axial length of the eye, anterior chamber depth, crystalline lens thickness, and central pachymetry) were analyzed in refractive groups. Two statistical analyzes were carried out: Pearson correlations on the various measurements and nonparametric tests (LSD tests). RESULTS: This statistical study showed the refractive characteristics of this pediatric population and the incidence of refraction on ocular biometry. There was a correlation between crystalline lens thickness, axial length, and anterior chamber depth. Axial length was significantly correlated in a positive way with age (r=0.332, p<0.001) and with anterior chamber depth (r=0.403, p<0.001), and in a negative way with crystalline lens thickness (r=-0.427, p<0.001). The study of refractive group parameters found a significant difference (p<0.01) between groups in axial length level. Anterior chamber depth decreased when the spherical equivalent increased. The study of crystalline lens thickness found a significant difference (p<0.01) between all four groups and pachymetry, as well as between groups 1 and 4 (p<0.05). CONCLUSION: The Lenstar LS 900 biometer (Haag-Streit(®)) provided a complete biometrical assessment of children's eye in a single and easy measurement procedure. In this pediatric population with hypermetropia, axial length of the eye was mainly correlated with spherical equivalent and other biometric parameters. The relation between these various parameters was influenced and modified by age.

[Should minor salivary gland biopsy still be performed?]. / Faut-il encore effectuer des biopsies des glandes salivaires accessoires?

INTRODUCTION: Labial salivary gland biopsy (LSGB) is a very useful technique that is often performed in our specialty. A lot of these LSGB yield normal results and the interest of this technique may be discussed. This study was made to answer two questions: one on the diagnostic interest of LSGB for the suspected pathology, secondly to verify if there was a correlation between the results of LSGB assessment and the patient's final diagnosis. MATERIAL AND METHODS: Ninety-six LSGB were performed in 2004 for Sjögren syndrome, sarcoidosis, amyloidosis and other auto-immune diseases. The histological study assessed the Chisholm-Mason score and screened for amyloidosis and sarcoidosic granuloma. The prescribing unit gave the final diagnosis. RESULTS: The LSGB was very specific (100% of specificity) and there were no false positive results; conversely, its sensitivity was lower, that is 75% for Sjögren syndrome, 67% for amyloidosis, 60% for sarcoidosis and 14% for other auto-immune disease (not defined). DISCUSSION: LSGB can be recommended for Sjögren syndrome; it is also useful for amyloidosis. In sarcoidosis it gives poor results and presents no interest for other auto-immune diseases. The LSGB prescribed for patients with a suspected initial single diagnosis (Sjögren syndrome only, or amyloidosis only, or sarcoidosis only, or an other auto-immune disease only) significantly increases the specificity and the sensitivity of the technique and suggests that specificity and sensitivity are linked to patient selection and not to LSGB itself.

[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.

[Rehabilitation on functional amblyopia in Morning Glory Syndrome]. / Rééducation de la part fonctionnelle de l'amblyopie dans un Morning Glory syndrome.

INTRODUCTION: Morning Glory syndrome, characterized by an enlarged dysplasic optic disc with glial tissue, is one of the congenital anomalies of the optic nerve. This syndrome is rare, prevalent in the girls, and generally unilateral. It can be revealed with nystagmus, strabismus, or amblyopia. OBSERVATION: We report the clinical observation of a 2.5-year-old girl, referred for the diagnosis of Morning Glory syndrome in the left eye with severe amblyopia (1/10 Rossano 1/20) and esotropia. This syndrome has associated central nervous system anomalies with a basal encephalocele. Treated with functional amblyopia therapy, visual acuity was 7/10 Rossano 1/2 after 1 year. DISCUSSION: Rehabilitation on functional amblyopia in organic optic nerve anomalies is essential. A child with a Morning Glory syndrome, detected during the period of sensory maturation, must be treated with occlusion therapy, followed by maintenance treatment. This part of the treatment can prevent deep amblyopia. Moreover, regular ophthalmologic follow-up to detect complications of retinal detachment and multidisciplinary follow-up to detect a cytogenetic disease, CHARGE syndrome, or association with endocrine and central nervous system anomalies are necessary. CONCLUSION: The author recommends occlusion therapy for children with Morning Glory syndrome or other organic asymmetric optic nerve anomalies, during the period of amblyopia reversibility. Most patients' vision improves after treatment. This case is an illustration.

[Abrikossoff tumor of the proximal esophagus misdiagnosed as a thyroid nodule]. / Tumeur d'Abrikossoff de l'oesophage cervical: une cause rare de faux nodule thyroïdien.

The diagnosis of thyroid nodules is straightforward and rarely mistaken. We present a case of a paraesophageal granular cell tumor, discovered incidentally during surgery for what it was diagnosed as a suspicious thyroid nodule by ultrasound and FNA. Complete resection was achieved without disruption of the esophageal mucosa. A terminal branch of the recurrent laryngeal nerve had to be resected en bloc with the tumor. Morphological and immunohistochemical diagnosis was established postoperatively. A review of the literature is presented.