Gorham-stout syndrome of the petrous apex causing chronic cerebrospinal fluid leak.

OBJECTIVE

To describe the clinical course, diagnostic features, and treatment of a case of Gorham-Stout syndrome involving the petrous apex and causing chronic cerebrospinal fluid (CSF) leak.

STUDY

DESIGN

Clinical capsule report.

SETTING

Academic pediatric hospital.

PATIENT: A 12-year-old boy presented with a destructive lesion of the right petrous apex and a 1-month history of headache, nausea, and vomiting.

INTERVENTIONS: Computed tomographic and magnetic resonance imaging revealed a nonenhancing lytic lesion of the right petrous apex and mandibular condyle. The lesions were bright on T2-weighted imaging with high signal intensity extending into the surrounding soft tissues. Leptomeningeal enhancement and an opening pressure of 0 cm of water on lumbar puncture suggested chronic CSF hypotension. Initial surgical exploration revealed diffuse infiltration of CSF into the soft tissue lateral to the temporal bone. Subsequent middle ear and mastoid obliteration was performed to definitely repair the CSF leak.

RESULTS

The clinical presentation, physical, laboratory, radiologic, and operative findings are consistent with a diagnosis of Gorham-Stout syndrome. In this case, lymphangiomatosis led to massive osteolysis of the petrous apex with CSF fistula into the surrounding soft tissues and middle ear with chronic intracranial hypotension.

CONCLUSION

This is the second report of chronic CSF leak resulting from lymphangiomatosis of the cranial base (Gorham-Stout syndrome).