Microsurgical Resection of a Giant Posterior Fossa Aneurysmal Malformation in a 21-Month-Old.

Pediatric aneurysms commonly occur in the vertebrobasilar circulation with complex morphologies.1 "Aneurysmal malformations," or fistulous vessel dilations without a nidus, have also been described.2 Vessel friability and sensitivity to blood loss can complicate surgery. A 21-month-old male with motor and speech delay was found to have a giant posterior fossa aneurysmal malformation. He was lethargic, with minimal speech, and moved all extremities with mild hypotonia. Imaging demonstrated a 6.9 × 5.1 × 4.6 cm aneurysm arising from a fenestrated right V4 segment. This communicated via a single connection with the deep venous system, draining through the superior vermian cistern veins, posterior mesencephalic vein, basal vein of Galen, and inferior sagittal sinus, consistent with an arteriovenous fistula with secondary aneurysmal dilatation. Endovascular sacrifice was not feasible, in addition to concern for swelling after embolization. Three-dimensional modeling confirmed close proximity of the single inflow and outflow tracts. A suboccipital and left far lateral craniotomy for clip trapping and excision of the aneurysmal arteriovenous malformation was performed in a lateral position to completely decompress the brainstem (Video 1). Angiography before closure and postoperative vascular imaging demonstrated complete aneurysmal resection and fistula disconnection, with patency of normal vasculature. The postoperative course was notable for transient swallowing difficulties likely from lower cranial nerve irritation and refractory hydrocephalus requiring a shunt. The patient was meeting all developmental milestones at 2-year follow-up. This case highlights the complex vascular pathology often seen in pediatric patients, as well as the importance of presurgical planning and careful microsurgical technique in achieving a successful outcome.

Arteriovenous malformation surgery in children: the Rady Children's Hospital experience (2002-2019).

PURPOSE: Compared to adult AVMs, there is a paucity of data on the microsurgical treatment of pediatric AVMs. We report our institutional experience with pediatric AVMs treated by microsurgical resection with or without endovascular embolization and radiation therapy. METHODS: We retrospectively reviewed all patients ≤ 18 years of age with cerebral AVMs that underwent microsurgical resection at Rady Children's Hospital 2002-2019. RESULTS: Eighty-nine patients met inclusion criteria. The mean age was 10.3 ± 5.0 years, and 56% of patients were male. In total, 72 (81%) patients presented with rupture. Patients with unruptured AVMs presented with headache (n = 5, 29.4%), seizure (n = 9, 52.9%), or incidental finding (n = 3, 17.7%). The mean presenting mRS was 2.8 ± 1.8. AVM location was lobar in 78%, cerebellar/brainstem in 15%, and deep supratentorial in 8%. Spetzler-Martin grade was I in 28%, II in 45%, III in 20%, IV in 6%, and V in 1%. Preoperative embolization was utilized in 38% of patients and more frequently in unruptured than ruptured AVMs (62% vs. 32%, p = 0.022). Radiographic obliteration was achieved in 76/89 (85.4%) patients. Complications occurred in 7 (8%) patients. Annualized rates of delayed rebleeding and recurrence were 1.2% and 0.9%, respectively. The mean follow-up was 2.8 ± 3.1 years. A good neurological outcome (mRS score ≤ 2) was obtained in 80.9% of patients at last follow-up and was improved relative to presentation for 75% of patients. CONCLUSIONS: Our case series demonstrates high rates of radiographic obliteration and relatively low incidence of neurologic complications of treatment or AVM recurrence.

Survival in unresectable sinonasal undifferentiated carcinoma treated with concurrent intra-arterial cisplatin and radiation.

We report the successful use of RADPLAT to treat a patient with an unresectable T4N0 sinonasal undifferentiated carcinoma. This patient received 4 cycles of weekly intra-arterial cisplatin together with thiosulfate infusion with concurrent radiation therapy. Radiation therapy was given in 28 daily fractions to 54 Gy using intensity-modulated radiation therapy followed by a hypofractionated stereotactic boost of 3 fractions to 13 Gy to a total dose of 67 Gy in 31 fractions to the nasal sinus and bilateral neck. Intra-arterial cisplatin was administered using a bilateral approach due to the midline site of this tumor. Within days of the first intra-arterial cisplatin, there was an obvious decrease in tumor size. She has been followed with magnetic resonance imaging and positron emission tomography, and remains disease-free 47 mo post-treatment. Centers with expertise in intra-arterial chemotherapy could consider the RADPLAT approach for patients with unresectable sinonasal undifferentiated carcinoma.