Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy (ETV).
BACKGROUND
In a small subset of patients, a Chiari malformation can present with signs of raised intracranial pressure due to obstruction of cerebrospinal fluid flow or with the raised intracranial pressure as the primary pathological driving force resulting in tonsillar herniation.


METHODS
The authors report a unique case in a 14-year-old boy with a Chiari malformation type 1-syringomyelia complex with slit-like ventricles. We have successfully managed the acute presentation of raised intracranial pressure, mimicking idiopathic intracranial hypertension, utilizing a frameless stereotactic image-navigated endoscopic third ventriculostomy alone. We present the preoperative and postoperative radiological and ophthalmological findings and discuss the possible mechanisms related to the pathophysiology and treatment in this case. The patient's symptoms resolved immediately postoperatively with resolution of papilledema. At 18-month follow-up, the patient remains entirely asymptomatic and the need for craniovertebral decompression appears to have been obviated.


CONCLUSIONS
We advocate that in the Chiari malformation type 1-syringomyelia complex with normal or small ventricles, patients presenting with isolated signs and symptoms of raised intracranial pressure alone can be safely and effectively managed with an electromagnetic-guided stereotactic endoscopic third ventriculostomy as a primary treatment option.
Kandasamy
Jothy
J
Department of Neurosurgery, Royal Liverpool Children's NHS Trust, Eaton Rd, Liverpool, L12 2AP, UK. jothy.kandasamy@thewaltoncentre.nhs.uk
Kneen
Rachel
R
Gladstone
Melissa
M
Newman
William
W
Mohamed
Tawil
T
Mallucci
Conor
C
D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000175:diagnosis; D006801:Humans; D019586:Intracranial Hypertension / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D044583:Neuroendoscopy / Q000379:methods*; D020542:Third Ventricle / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome; D014696:Ventriculostomy / Q000379:methods*