[Ondine's Curse and rare oculomotor abnormalities: a case report].
Ondine's Curse or congenital central hypoventilation syndrome (CCHS) is a neurocristopathy (failure of migration or differentiation of neural crest-derived precursor cells) and is characterized by hypoventilation or apnea, which is most pronounced during sleep, with no other abnormalities of the neuro-respiratory system. Because of respiratory distress soon after birth, patients must be intubated and ventilated for a long time. This disorder may be associated with other symptoms of neurocristopathy (Hirschsprung disease, neuroblastoma, neuroganglioma) and other abnormalities of the autonomic nervous system (vasomotor dysfunctions or ophthalmic abnormalities: abnormal pupils, insufficient convergence, strabismus, or ptosis). We report the original case of a CCHS patient who presented with alternative ptosis of both the right and left eyes and esotropia. The ocular findings should lead to earlier diagnosis and speedier adequate treatment.
Michel
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G
Clinique Ophtalmologique, Hôpital Civil, Hôpitaux Universitaires de Strasbourg. gmichel@télé2.fr
Villega
F
F
Desprez
P
P
Dollfus
H
H
Speeg-Schatz
C
C
D006801:Humans; D007231:Infant, Newborn; D008297:Male; D015840:Oculomotor Nerve Diseases / Q000150:complications*; D020919:Sleep Disorders, Intrinsic / Q000150:complications* / Q000151:congenital*