Clinical and oncological outcomes in single-stage versus staged surgery for pediatric craniopharyngiomas: a multicenter retrospective study.

PURPOSE: Craniopharyngiomas (CPGs) are aggressive brain tumors responsible of severe morbidity in children. The best treatment strategies are under debate. Our study evaluates surgical, pituitary, and hypothalamic outcomes of a tailored staged-surgical approach compared to a single-stage radical approach in children with CPGs. METHODS: Multicenter retrospective study enrolling 96 children treated for CPGs in the period 2010-2022. The surgical management was selected after a multidisciplinary evaluation. Primary endpoint includes the inter-group comparison of preservation/improvement of hypothalamic-pituitary function, the extent of resection, and progression-free survival (PFS). Secondary endpoints include overall survival (OS), morbidity, and quality of life (QoL). RESULTS: Gross Total Resection (GTR) was reached in 46.1% of cases in the single-stage surgery group (82 patients, age at surgery 9 ± 4.7 years) and 33.3% after the last operation in the staged surgery group (14 patients age 7.64 ± 4.57 years at first surgery and 9.36 ± 4.7 years at the last surgery). The PFS was significantly higher in patients addressed to staged- compared to single-stage surgery (93.75% vs 70.7% at 5 years, respectively, p = 0.03). The recurrence rate was slightly higher in the single-stage surgery group. No significant differences emerged in the endocrinological, visual, hypothalamic outcome, OS, and QoL comparing the two groups. CONCLUSIONS: In pediatric CPGs' surgical radicality and timing of intervention should be tailored considering both anatomical extension and hypothalamic-pituitary function. In selected patients, a staged approach offers a safer and more effective disease control, preserving psychophysical development.

Delayed recurrence of spontaneous intracranial hypotension syndrome mimicking a Chiari I malformation: Case report with a review of the literature.

INTRODUCTION: Cerebellar tonsils descent seen on brain MRI is, along with other findings, a recognized radiological sign of possible spontaneous intracranial hypotension (SIH). The short-term outcome of SIH is usually favorable with symptoms improvement and reversibility of the low-lying tonsils. Nevertheless, data on the long-term outcome are lacking or inconsistent. CASE REPORT: A 32-year-old woman presented to her general practitioner with a six months history of non-specific headaches. An MRI brain with gadolinium showed a 12mm tonsillar descent with no other remarkable findings. Headaches were initially managed conservatively as migraines. Following the onset of progressive upper back and shoulder pain at rest, nausea, photophobia and fogging in her vision, the patient was referred to our Department with a suspicion of symptomatic Chiari I malformation. After an in-depth anamnesis, it emerged a previous history of SIH, 14 years earlier, successfully treated conservatively in another center. A whole spine MRI confirmed the suspicion of recurrent SIH showing an anterior cervico-thoracic epidural fluid collection. The patient underwent an epidural blood patch with complete resolution of the symptoms and radiological signs. DISCUSSION: To our knowledge, this case is the first report of delayed recurrence of a SIH successfully treated conservatively over 10 years earlier. The etiopathogenesis and management of this rarity with literature review is discussed. CONCLUSION: An isolated cerebellar tonsil descent with no other remarkable findings on brain MRI and a previous history of SIH should always alert the clinician of a possible late recurrence of a CSF leak and avoid unnecessary Chiari I malformation surgical procedures.

Extended Endoscopic Endonasal Approach for Suprasellar Craniopharyngioma.

Objectives We illustrate a suprasellar craniopharyngiomas treated with an extended endoscopic endonasal approach (EEEA). Design Case report of a 43-year-old male affected by cerebral lesion located in suprasellar region involving the third ventricle and compressing the neurovascular structures, causing an anterosuperior dislocation of the chiasma. There is a complete disruption of the pituitary stalk that can explain the clinical finding of partial anterior hypopituitarism and hyperprolactinemia. The lesion is characterized by a solid and cystic component. Considering the absence of lateral extension and the suprasellar location of the lesion, an EEEA is preferred. Setting University Hospital "Ospedale di Circolo," Department of Neurosurgery, Varese, Italy. Participants Neurosurgical and ENT Skull Base Team. Main Outcome Measures A bilateral parasagittal approach is performed using a four-hand technique. The first step of the surgery is the preparation of the Hadad's flap. The approach is extended to the planum sphenoidalis to expose the suprasellar region. The lesion is completely removed employing also an ultrasound aspirator. Skull base reconstruction is performed with three-layer technique: graft of fat tissue, fascia lata, and nasoseptal flap. Results No postoperative complications occurred. In the post-op, the patient presents a panhypopituitarism and an improvement in neurological status. The visual deficit remains stable. Post-op magnetic resonance imaging at 1 year documents the complete absence of pathological contrast enhancement. Conclusions EEEA is a feasible approach in treating craniopharyngioma with suprasellar extension. The advantages include optimal visualization, good resection rate, and absence of brain retraction. The link to the video can be found at: https://youtu.be/IYm-8P1jbBo .