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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.

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Alberto Campione, Gianluca Agresta, Davide Locatelli, and Fabio Pozzi

Epidural varicosis is a rare though well-known cause of cauda equina syndrome (CES). Although inferior vena cava (IVC) obstruction is the most common finding in such cases, portal vein hypertension can lead to epidural venous plexus engorgement by means of lumbar portocaval shunt activation.

A 40-year-old woman presented with right-sided sciatica, which progressed to right foot drop and a 3-day history of vesical tenesmus and fecal retention. She was initially diagnosed with L4–5 lumbar disc protrusion. However, contrast-enhanced lumbar MRI scan showed the presence of epidural varices in the L3–S1 tract. Given the absence of vascular anomalies amenable to resection, etiological conservative treatment was addressed. Therefore, a complete diagnostic workup was performed and revealed deep vein thrombosis (DVT), pulmonary embolism, and portal vein thrombosis. Oral anticoagulant therapy was initiated and prompt resolution of CES was observed. To the authors’ knowledge, this is the first report of CES secondary to epidural varicosis in the setting of acute portal vein thrombosis and extrahepatic portal vein obstruction (EHPVO). In cases of epidural varicosis, conservative etiological treatment is the most appropriate choice as CES may be the epiphenomenon of underlying systemic pathophysiological processes.