Acta Neurochir (Wien) (2006) 148: 1117–1121 DOI 10.1007/s00701-006-0875-5 Case Report Acute presentation of hydromyelia in a child A. Hamlat 1 , H. Helal 1 , B. Carsin-Nicol 2 , G. Brassier 1 , Y. Guegan 1 , and X. Morandi 1 1 Department of Neurosurgery, CHRU Pontchaillou, Rennes, France 2 Department of Radiology, CHRU Pontchaillou, Rennes, France Received April 27, 2006; accepted July 20, 2006; published online August 31, 2006 # Springer-Verlag 2006 Summary The authors document a rapid development, within 3 weeks, of hydromyelia in a 12 year-old boy. The boy was admitted to a local hospital because of drowsiness and persistent severe neck pain. Neurological examination disclosed a lethargic boy with no neurolog- ical deficit other than Parinaud’s sign. During his transfer to our department, he presented a cardio-respiratory arrest with coma and bilateral mydriasis. External ventricular drain and craniocervical decompression achieved excellent clinical and neuroradiological out- comes. The development of hydromyelia in this case is caused by obstruction to the natural cerebrospinal fluid pathway at the cranio- cervical junction and the cardio-respiratory arrest is provoked by a brain stem compression against the clivus and odontoid process. This report illustrates that hydromyelia may complicate acute obstructive hydrocephalus due to acquired Chiari malformation. Keywords: Hydrocephalus; hydromyelia; syringomyelia; Chiari malformation; arachnoiditis; acute presentation; cardio-respiratory arrest; coma. Introduction Caudal displacement of the cerebellar tonsils, through the foramen magnum, is the main feature of Chiari mal- formation type 1 (CM-1) and it has been associated with hydro-syringomyelia in 30–75% of patients [8, 12]. However tonsils downward with or without either hydro- cephalus or syringomyelia may also be consequent to other diseases [3, 13, 15] predominantly posterior fossa processes [1, 2, 5, 16] and it can be discovered fortu- itously in asymptomatic persons [7]. We documented in the present case a rapid deve- lopment of a hydromyelia (or communicating syringo- myelia) in a 12 year-old boy with hydrocephalus, and complicated by a cardio-respiratory arrest and coma. Case illustration Presentation This 12 year-old-boy was admitted in August 2003 to a local hospital in a lethargic state with papilloedema. History His past medical history was remarkable as he had suffered neonatal meningitis due to Escherichia Coli, and had experienced previous trauma by being dropped from a running tractor; he was seen at a local hospi- tal and discharged because of lack of complaints, normal neurological examination and general examination failed to show any physical abnormality. Four months later he complained of progressive and severe neck pain. Neurological examination was normal but owing to the severity of neck pain he underwent standard cervical spine radiological examina- tions (which were normal) but magnetic resonance imaging (MRI) revealed a moderate hydrocephalus (Fig. 1A) with tonsilar herniation (Fig. 1B). The patient was discharged home, with no complaints and normal neurological examination, a few days later. Three weeks later he complained of fronto-occipital headaches and morning vomiting with visual troubles. Ophthalmological examination showed Parinaud’s sign and unilateral papilloedema. The boy was read- mitted to a local hospital in the afternoon because of drowsiness. Neu- rological examination disclosed a lethargic boy with no neurological anomalies other than Parinaud’s sign. A new MRI was performed and revealed worsening hydrocephalus (Fig. 2A) with a hydromyelia extend- ing from C1 to T3 level (Fig. 2B). During his transfer to our hospital, he experienced a cardio-respiratory arrest and coma with a Glasgow coma scale (GCS) of 4 and bilateral mydriasis. He was intubated, resuscitated, and managed with 200 ml of mannitol at 20%, and 150mg of pentobarbital and transferred to our intensive care unit. Examination On admission neurological examination revealed a comatose patient (although sedated), bilateral unreactive mydriasis and tachycardia at 160 pulses=mn; the brain stem reflexes were present.