3.1 Case 1: Gorham-Stout Disease
An 8-year-old male presented with chronic headaches and history of 5
episodes of meningitis caused by Streptococcus viridans orpneumoniae . Past medical history included refractory immune
thrombocytopenia (ITP) which resolved after splenectomy. He was
diagnosed with “lymphangiomatosis” at an outside institution and
initially treated with propranolol and vincristine, and then briefly
with sirolimus. All medications were stopped when he developed
meningitis. After additional episodes of meningitis, he was referred to
our institution. He suffered from daily debilitating headaches initially
thought to be secondary to past meningitis infections. MRI of the brain
and conventional CT of the head were not able to identify the source of
his recurrent meningitis. CT cysternogram demonstrated an active CSF
leak at the central skull base due to osseous erosion of lateral walls
of the sphenoid sinuses (Figure 1A-D) . Comprehensive imaging
showed extensive spinal involvement (Figure 1E) , lytic humeri
lesions and a mediastinal LM. Since blood patch was unable to be
performed due to the location and size of the dura mater defect, patient
was initiated on interferon and zoledronic acid. After two years
meningitis recurrence, interferon was stopped and oral sirolimus was
started.