The authors report the case of a 13-year-old girl with Kikuchi-Fujimoto disease who presented with features of raised intracranial pressure due to subdural effusions. A biopsy revealed dural involvement in the disease process. The authors review the medical literature of this rare disease and highlight the paucity of documented cases with CNS involvement.
Marcelo Volpon Santos, Pasquale Gallo, Fozia Roked, Ina Nicklaus-Wollenteit and Desiderio Rodrigues
John A. Emelifeonwu, Drahoslav Sokol, Pasquale Gallo, Jothy Kandasamy and Chandrasekaran Kaliaperumal
The authors report a case of a child with hypothalamic-origin pilocytic astrocytoma and hydrocephalus, which was refractory to treatment with a ventriculoperitoneal shunt due to high CSF protein content. With parental education, the child's hydrocephalus was managed long-term in the community with a long-tunnelled external ventricular drain, which was maintained by his parents. To the authors' knowledge this is the first report of this management option as a long-term measure. No harm has come to the patient. The authors propose long-term, long-tunnelled external ventricular drain as a viable treatment option for such patients.
Gianluca Agresta, Drahoslav Sokol, Chandrasekaran Kaliaperumal, Jothy Kandasamy and Pasquale Gallo
Neurenteric cysts (NCs) are rare congenital lesions with epithelial mucin-secreting walls. They can occur anywhere along the neural axis, and an intrinsic midbrain cyst is extraordinary. Surgical management may pose a challenge due to the location of the lesion and adhesion of the cyst wall to the surrounding brainstem. The authors describe the first case of pediatric NC that was treated successfully with intracystic interferon-α (IFN-α).
A 16-month-old baby girl presented with a 2-week history of progressive croup, vomiting, and swallowing difficulty. MRI revealed a 1.8-cm cystic intrinsic lesion in the pontomedullary region. She initially underwent posterior fossa craniotomy and drainage of the cyst under intraoperative neurophysiology monitoring. Three weeks following the procedure, her symptoms recurred, and follow-up MRI demonstrated cystic recurrence. She underwent repeat aspiration of the cyst and biopsy of the cyst wall, and INF-α-2b was injected into the cystic cavity. Her symptoms improved and completely resolved after 5 months. A 9-month follow-up brain MRI study showed complete resolution of the NC. Intracystic IFN-α injection after cystic content aspiration may be a safe treatment option for the management of intrinsic brainstem NCs. Long-term clinical and radiological follow-up is recommended.