Cerebrospinal fluid fistula in a patient with chronic constipation related to an autonomic dysfunction and revealed by bacterial meningitis - A case report

Background: Cerebrospinal fluid (CSF) fistula represents a rare neurosurgical entity that can be defined as a communication between the subarachnoid space and nasal fossa or less commonly the ear cavity. It can be spontaneous without an evident etiology or secondary following a skull base surgery or trauma. The early diagnosis of spontaneous forms remains a challenge as clinical signs (e.g., unilateral rhinorrhea) can be absent or neglected by patients and can result in meningitis.

Case description: Here, we report the case of a 31-year-old man with chronic constipation complicated by chronic intracranial hypertension, and resulting in rhinorrhea with bacterial meningitis. The etiological assessment of chronic constipation retained an autonomic dysfunction with sympathetic hyperactivity (e.g., pure autonomic failure) as an underlying cause. Beta-2 transferrin testing associated with cerebral magnetic resonance imaging and computed tomography scan confirmed the diagnosis and localization of the fistula at the cribriform plate. The patient underwent an endoscopic endonasal approach with a repair of fistula. He presented with recurrent rhinorrhea 17 months later which required a surgical revision along with CSF diversion with a ventriculoperitoneal shunt.

Conclusion: Although rare, autonomic dysfunction can result in chronic constipation in young patients, with intermittent or permanent intracranial hypertension, leading to CSF leaks. The early identification and treatment of the underlying etiology may prevent severe complications and improve the management and outcome of CSF fistula patients.

Keywords: Cerebrospinal fluid rhinorrhea; Constipation; Meningitis; Pure autonomic failure.