Spontaneuous CSF Rhinorrhea Secondary to Aqueduct Stenosis in Adult

1. Spontaneuous CSF Rhinorrhea Secondary to Aqueduct Stenosis in Adult Szu-Han Chen, Jung-Shun Lee Section of Neurosurgery, Department of Surgery National Cheng-Kung University Medical College and Hospital

2. Identification data • A 54 year old male kick-boxer • HTN/DM/Hyperlipidemia/goat • AMI/3-V-D CAD s/p recurrent PTCA 3 times since he was 39 years old. • Meningitis (-) • The nose trauma history (+) when he was 14 year-old but without any neurological sequelae • Watery discharge off and on since childhood.

3. Present illness • watery discharge off and on especially when he bowed his head since childhood. • aggravatated and persistent in 9 months ago. • no foul-odor, post nasal dripping, frontal headache, nausea, and vomiting. • our otolaryngologist’s OPD for help. CSF rhinorrhea PE: grossly normal NE: no specific local neurologic sign. Lab. Data: in normal ranges

4. The brain MRI • Aqueduct stenosis • neurologic surgeon → third ventriculostomy

5. Post-OP course • After operation, - the frequency of rhinorrhea ↓ • After half year, - the symptom improved completely - no more CSF leakage was noted in MRI. • BP was stable after surgery and anti-HTN drugs were progressively tapered.

6. Discussion • Spontaneuous CSF rhinorrhea secondary to congenital aqueduct stenosis in Adult • his hypertension was under well medical control after the third ventriculostomy of congenital aqueduct stenosis.

7. Congenital aqueduct stenosis • a common cause of obstructive hydrocephalus in infants. • s/s: raised intracranial pressure • CSF rhinorrhea: 10 (10-30 y/o)

8. Nontraumatic CSF rhinorrhea • The reasons of poor spontaneously healing about CSF fistula secondary to aqueduct stenosis are still unknown. • Chronically high pressure causes erosion and thinning of the cranial base.

9. Hypertension • Hypertension was one sign of hydrocephalus resulted from IICP associated with bradycardia, and altered respiratory rate. (Cushing’s sign) • vascular permeability↑ → cerebral edema → the resistance or outflow of CSF↑ → ventricles↑ → a change of the neuronal activity in the hypothalamic center

10. Management (1) • ventriculostomy • 94% of patients • The minimal trauma and no foreign body implantation were the advantages • failure if CSF circulation obstructions (meningitis, subarachnoid hemorrhage and brain tumor)

11. Management (2) • VP shunt placement • tension pneumocephalus - the negative ICP caused by the siphon effect of the shunt allows air to enter the cranial cavity. → direct repairing of the fistula → changing the shunt system included high pressure shunt valves or antisiphon devices

12. Reference • John R. Little: Tension pneumocephalus after insertion of ventriculoperitoneal shunt for aqueductalstenosis. J. Neurosurg. Vol. 44: 383-385 March, 1976 • K. Ikeda: Tension pneumocephalus complicating ventriculoperitoneal shunt for cerebrospinal fluid rhinorrhea: case report. Jorunal of Neurology, Neurosurgery, and Psychiatry, 1978, 41, 319-322 • Hiroshi Kuba: Pneumocephalus associated with aqueductalstenosis: three-dimensional computed tomographic demonstration of skull-base defects. Child’s NervSyst (2000) 16:1-3 • T. Greitz: High blood pressure and epilepsy in hydrocephalus due to stenosis of the aqueduct of sylvius. ActaNeurochirurgica 24, 201-206 (1971) • Masakazu Washio: Hydrocephalus associated with malignant hypertension and renal failure. Nephron 1990; 56; 222-223 • S. Honeybul: Delayed pneumocephalus following shunting for hydrocephalus. Journal of Clinical Neuroscience 13 (2006) 939-942 • O. B. Eden: Hypertension in acute neurological diseases of childhood. Develop. Med. Child Neurol. 1977, 19, 437-445 • DattatrayaMuzumdar: Spontaneous cerebrospinal fluid rhinorrhea as a presenting symptom of aqueductalstenosis. Neurol Med Chir (Tokyo) 43, 626-629, 2003

13. Reference • Cabezudo JM, Vaquero J, Garcia-de-Sola R, Areitio E, Martinez R: Direct communication between the lateral ventricle and the frontal sinus as the cause of CSF rhinorrhea in aqueductal stenosis. Acta Neurochir (Wien) 57: 95-98, 1981 • Ikeda K, Nakano M, Tani E: Tension pneumocephalus complicating ventriculoperitoneal shunt for cerebrospinal fluid rhinorrhoea: case report. J Neurol Neurosurg Psychiatry 41: 319-322, 1978 • Jooma R, Grant DN: Cerebrospinal fluid rhinorrhea and intraventricular pneumocephalus due to intermittent shunt obstruction. Surg Neurol 20: 231-234, 1983 • Kushner J, Meschan I, Alexander E Jr: Cerebrospinal fluid rhinorrhea, aqueductal stenosis, and the empty sella. Md State Med J 22: 51-54, 1973 • Little JR, MacCarty CS: Tension pneumocephalus after insertion of ventriculoperitoneal shunt for aqueductal stenosis. J Neurosurg 44: 383-385, 1976 • Nishikawa M, Karasawa J, Kamada K, Kikuchi H, Makita Y: [Non-traumatic cerebrospinal fluid rhinorrhea associated with aqueductal obstruction]. No To Shinkei 24: 173-177, 1972 (Jpn) • Rovit RL, Schechter MM, Nelson K: Spontaneous “high-pressure cerebrospinal rhinorrhea” due to lesions obstructing flow of cerebrospinal fluid. J Neurosurg 30: 406-412, 1969 • Schechter MM, Rovit RL, Nelson K: Spontaneous “high pressure CSF rhinorrhoea”: Cerebrospinal fluid leakage caused by long-standing increased intracranial pressure. Br J Radiol 42: 619-622, 1969 • Tokuno T, Ban S, Nakazawa K, Yoshida S, Matsumoto S, Shingu T, Sato S, Yamamoto T: [Non-traumatic cerebrospinal fluid rhinorrhea associated with hydrocephalus: a case report]. No Shinkei Geka 23: 265-269, 1995 (Jpn, with Eng abstract)