[New developments in 2005 for the management of visual deficit in the infant]. / Quoi de neuf en 2005 dans la prise en charge de l'enfant: "déficit visuel".

The main progress in ophthalmopediatrics is the knowledge of the importance and efficacy of visual refraction defects screening. This screening has to be the earliest possible, allowing a glass equipment preventing complications: amblyopia, strabismus. This screening allows the same time screening of organic anomalies and early treatment.

[Maxillary and sphenoid sinusitis complicated by acute inflammatory optic neuritis in a 12-year-old patient]. / Sinusite maxillaire et sphénoïdale compliquée de neuropathie optique inflammatoire aiguë chez un patient de 12 ans.

Acute inflammatory optic neuritis (retrobulbar optic neuritis) is an uncommon but described complication of acute sinusitis. It is due to a close anatomic relationship between the sinus and the optic nerve. Inflammatory mechanism is dominant with a possible bilateral neuritis, especially among children. In the reported case, as usual in childhood, visual acuity loss has been particularly severe, and recovery slow (it takes usually 6 weeks) but almost complete. Primary treatment is medical, with antibiotics and corticosteroids that must be prescribed for a prolonged time.

[Eyelid hemangiomas in infants: contribution of MRI]. / Les hémangiomes palpébraux du nourisson: apport de l'IRM.

OBJECTIVES: To describe the MR imaging features and patterns of local extension of hemangiomas of the eyelid in correlation with the clinical presentation. PATIENTS AND METHODS: Retrospective study including 21 MRI (GE, 1.5T, T1 +/- Gadolinium, T2 +/- fat saturation, 3 planes) examinations performed for eyelid hemangiomas with occlusion>50% and/or ocular deviation. All examinations were reviewed by two observers using a standardized list of criteria. RESULTS: All hemangiomas had a heterogeneous signal described as "salt and pepper" on T2W sequences. The extension was extra-orbital in 8 cases, intra-orbital in 13 cases, extra-conal in 9 cases, intra-conal in 4 cases. The "fat sat T2" sequence provided the best anatomical details. There was a strong correlation between ocular deviation at clinical examination and intra-orbital extension, but no correlation between the extent of eyelid involvement and orbital location of the hemangioma. Dysplastic cerebellum anomalies related to the PHACES syndrome were present in 3 patients. CONCLUSION: MRI of the brain and orbits provides information that appears essential for optimal management of infants with hemangioma of the eyelid.

[Isolated intracranial hypertension due to Lyme's disease]. / Hypertension intracrânienne isolée par maladie de Lyme.

Lyme's borreliosis is characterized by the variety of its revealing symptoms, which may explain an often delayed diagnosis. We report on a case of a child affected by Lyme's disease, confirmed by serology, who presented a particular form consisting in an isolated intracranial hypertension. This rare form must be known and diagnosed early in order to avoid serious complications such as optic nerve atrophia in the absence of an appropriate treatment.

[Diagnosis of paroxysmal episodes of eye rising in a child]. / Diagnostic des épisodes paroxystiques d'élévation des yeux chez l'enfant.

This report presents a case of a 7.5-year-old boy who was given a complete check-up with normal results for paroxysmal elevation of the eye with onset during seizures. The clinical aspect showed a tonic upgaze that has never been described in such an old patient. We describe this rare anomaly and review the literature. The main differential diagnosis was the presence of a tic.

[Optic pathway gliomas in neurofibromatosis type I. Longitudinal study of 30 cases in two multidisciplinary practices]. / Les gliomes des voies optiques dans la neurofibromatose de type 1. Etude longitudinale de 30 cas suivis dans deux consultations multidisciplinaires.

UNLABELLED: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol. PATIENTS AND METHODS: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination. RESULTS: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis. CONCLUSION: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.

[Alternating hemiplegia of childhoood and oculomotor anomalies]. / Hémiplégies alternantes de l'enfant et leurs anomalies oculo-motrices.

BACKGROUND: Alternating hemiplegia of childhood is a syndrome which begins in the first year of life. It is characterized by repeated attacks of uni-or bilateral hemiplegia or hemiparesia. In most cases paroxysmal manifestations are observed: movements or dystonia++ attacks, episodic nystagmus, abnormal eye movements and disturbance of the neurovegetative system, predominantly in the first year of life. ANALYSIS: In half of the cases, neurological anomalies begin during the neonatal period with a non characteristic aspect. Typical attacks take place after one year of life, sometimes associated with partial epilepsy. In a quarter of cases, the oculomotor anomalies have been known since early life. The diagnosis is made prior to one year on the basis of associated oculomotor anomalies and other symptoms without EEG arguments for epilepsy. Paroxysmal nystagmus is always found. One eye is affect in most cases, generally with horizontal and seldom with vertical movements of large variable pendular amplitude. One eye with nystagmus and the other with mydriasis is sometimes reported. Most attacks last from 30 sec to 3 min. Paroxysmal strabismus described in half of the cases seems to be generally unilateral internuclear transitory ophthalmoplegia. Finally, ocular deviations on the hemiparetic side are described. They are generally unique or sometimes associated with head deviation. Spontaneous blinking is reduced. CONCLUSION: Alternating hemiplegia of childhood is a non-epileptic sporadic, paroxysmal manifestation of unknown pathogenesis. Prognosis is poor. The presence of oculomotor signs suggests the diagnosis.

[Importance of multidisciplinary consultations for children with neurofibromatosis]. / Intérêt d'une consultation multidisciplinaire de neurofibromatose de l'enfant.

Over a one year period, the multidisciplinary consultation of the Saint-Vincent-de-Paul Hospital for neurofibromatosis examined 26 children. Twenty-two children underwent an ophthalmological examination (21 NF1, 1 NF2). Lisch nodule was the most frequent symptom (12 cases) and corneal nerves were visible in 3 cases. We had only one case of retinal and choroidal hamartoma. Glioma was a frequent symptom (5 cases). Other signs were uncommon. The case of NF2 showed a 3rd nerve palsy and lens droplets. Our evaluation confirms the frequency of Lisch nodules as the most frequent symptom of NF1. The visibility of corneal nerves and hamartoma are suggestive signs. The glioma number fits well with its statistical frequency. One year evaluation of our NF clinic allows us to confirm the relative frequency of the different signs associated with neurofibromatosis. It emphasizes the importance of teaching its diagnosis and follow-up to ophthalmologists.

[Biopercular polymicrogyria associated with congenital ophthalmoplegia caused by nuclear lesion of the common oculomotor nerve]. / Polymicrogyrie bioperculaire associée à une ophtalmoplégie congénitale par atteinte du noyau du nerf moteur oculaire commun.

Developmental pseudobulbar palsies seem to be different from the adult form described by Foix, Chavany and Marie. They usually include a major speech delay and severe epileptic seizures. In one clinicopathological case, neuroradiological imaging showed a macrogyric aspect of both rolandic operculi and unilateral destruction of pes pedunculari. Microscopic examination showed a four-layered polymicrogyria involving the first temporal gyrus and in the brainstem a selective destruction of the left oculomotor nucleus. Thus, the macrogyric aspect could be related to post migratory disorder occurring late in the cortical development. The brain stem lesion, destroying unilaterally the third cranial nerve nucleus gives a good example of the complex somatotopia of this oculomotor nucleus.

[Uveo-meningitis revealing a West African trypanosomiasis in a 12-year-old girl]. / Uvéo-méningite révélatrice d'une trypanosomiase "ouest-africaine" chez une enfant de 12 ans.

The case of a 12 year-old European girl who lived in Senegal for 9 months and who presented with Western-African trypanosomiasis is reported. The diagnosis was made because of the occurrence of uveomeningitis following progressive alteration of her general condition. A favorable outcome was observed after two intravenous courses of Eflornithine (difluoromethylornithine, DFMO).

[Corneal toxicity of cytarabine. Apropos of a case]. / Toxicité cornéenne de la cytarabine. A propos d'un cas.

A 4 1/2 year old girl with acute lymphoblastic leukemia developed corneal toxicity while receiving courses of chemotherapy once a month (standard doses of vincristine, cyclophosphamide or teniposide, always associated with cytarabine and asparaginase). Symptoms began after 18 courses of treatment and consisted of ocular pain, foreign body sensation, blurred vision, bilateral conjunctival hyperemia. The symptoms appeared during the course of chemotherapy and decreased before the following course. Symptomatic treatment appeared to be effective. Ocular toxicity of antineoplastic agents and particularly cytarabine is discussed.

[Ankyloblepharon filiforme adnatum and associated syndromes]. / L'ankyloblépharon filiforme congénital et les syndromes associés.

The authors present 2 cases of ankyloblepharon filiforme adnatum (AFA). In the first case, the ocular anomaly was unaccompagnied by other malformations; in the second patient, there was an associated popliteal pterygium syndrome. The authors review histologic, embryologic features and etiopathogenic theories of AFA and also fully discuss the various syndromes which may be associated with this ocular anomaly. Rosenman's classification is presented.

[Unusual ocular anomaly in 9 p trisomy: retinal depigmentation]. / Anomalie oculaire inhabituelle de la trisomie 9 p: dépigmentation rétinienne.

A new case of 9 p trisomy is reported in a 7-month-girl having typical morphological abnormalities and a mental retardation. During the examination, unusual ocular abnormalities were observed: pigmented linear dots of the mid peripheral retinal. Strabismus and hypertelorism were present. However, the other ocular features, blepharophimosis, correctopia, ptosis, microphthalmia, iris coloboma, unusual position of the eyelashes were not observed.

[Oculocerebral anomalies in Walker's lissencephaly]. / Les anomalies oculo-cérébrales au cours de la lissencéphalie de Walker.

Two new cases of Walker's lissencephaly are reported. In this disease first described by Walker in 1942 important cerebral malformations and various ocular anomalies are associated. The main cerebral malformations consist of hydrocephalus and agyria, and the ocular anomalies concern the anterior segment as well as the retina which is frequently dysplastic. The originality of our cases is due to the retina which was not dysplastic but showed particular modifications which are discussed. Moreover, in the second case the cerebral cortex was rather microgyric. The aspect of the ocular lesions and of some of the cerebro-meningeal findings bring the authors to discuss the autosomal recessive inheritance proposed by some neuropathologists; these aspects can suggest a possible foetopathy.

[Optic neuritis in children. Clinical aspects and evolution in 14 patients (author's transl)]. / Névrites optiques de l'enfant. Aspects cliniques et évolutifs à propos de 14 observations.

Fourteen children presenting with optic neuritis are reported. Bilateral ocular lesions and papilledema were present in 80% of cases. Biological investigations did not show any local production of IgG or anti-measles antibodies, contrary to what is observed in adults. CAT scan most always rules out a tumoral compression that could be responsible for a sudden decrease in visual acuity. Prognostic factors helpful in predicting evolution towards multiple sclerosis are discussed.