Résumé
An unusual retinal manifestation of arteriovenous communications is reported in a case showing a combination of Sturge-Weber and Kippel-Trenaunay syndromes. The arteriovenous communications are thought to be similar to those seen in the limbs in Parkes-Weber syndrome. This retinal finding indicates that the three syndromes are intricately related to each other and may be an incomplete manifestation of a single pathophysiologic entity which may be named neurocutaneous angiomatosis.
Sujets)
Adolescent , Malformations artérioveineuses , Angiographie fluorescéinique , Fond de l'oeil , Humains , Syndrome de Klippel-Trénaunay , Mâle , Artère centrale de la rétine/malformations , Veine centrale de la rétine/malformations , Syndrome de Sturge-WeberRésumé
Surgery in idiopathic congenital nystagmus to correct an abnormal head posture is based on the shifting of neutral point. Torsional Kestenbaum has been done in cases of congenital nystagmus with torticollis, without definite localisation of null point and sustained improvement of head posture subsequent to surgery has been attributed to presumed shift of the null point. We present a 6 year-old boy with congenital horizontal nystagmus with marked head tilt towards the left shoulder. Electronystagmography showed dampening of nystagmus on left tilt. Recession/advancement of all four oblique muscles was done to shift the null point and nystagmus dampened in the primary position eliminating the head tilt. This report emphasises the significance of electronystagmography in critical decision of horizontal/torsional Kestenbaum and documentation of innervational changes following surgery and subsequent followup.
Sujets)
Enfant , Électrophysiologie , Humains , Mâle , Nystagmus pathologique/complications , Torticolis/complicationsRésumé
An unusual case of cyclic pupillary movements in an otherwise complete oculomotor nerve palsy in a five year-old girl is reported. This is considered to be due to destruction of somatic and visceral nuclei of the oculomotor nerve following injury to its fascicular part. Pupillary automatism has been explained on the basis of the presence of aberrant autonomic cells in the ciliary ganglion which are discharging in a regular rhythm independent of higher control.