Septal rhinopharyngeal flap: a novel technique for skull base reconstruction after endoscopic endonasal clivectomies.

Endoscopic endonasal reconstruction techniques have improved CSF leak rates that were initially reported after surgery for cranial base and intradural lesions. However, wide surgical defects still pose a problem, especially if located in the clival region. The authors propose and describe a novel reconstruction technique they call a septal rhinopharyngeal flap (SRF) specifically designed to address this issue. The SRF is formed by three components of mucosa: 1) septal, 2) rhinopharyngeal roof, and 3) rhinopharyngeal posterior wall components, which allows for the coverage of the tuberculum/sellar region, midclivus, and lower clivus, respectively. A step-by-step procedure is described and its results analyzed in cases in which it has been used. The SRF was performed in 8 patients, which included diagnoses of 4 chordomas, 2 petroclival meningiomas, 1 invasive pituitary adenoma, and 1 chondrosarcoma. The size of the flap was considered optimal in all patients (100%). Postoperative MRI revealed contrast enhancement covering the entire surface of the flap. No CSF leaks were encountered after at least 1 postoperative year. The SRF is a novel vascularized reconstruction technique specifically indicated for wide endosanasal clivectomies focused on the middle clivus with caudal extension into the lower clivus and craniocervical junction, as well as rostral extensions into the tubercular or planum sphenoidale. This new reconstruction technique could be added to the skull base reconstruction armamentarium as a safe and optimal option.

Spontaneous CSF rhinorrhea as a presenting symptom of a clival chordoma / Fístula espontánea de LCR como síntoma de presentación de un cordoma de clivus

CSF rhinorrhea is a rare presenting symptom of a clival chordoma as these tumors do not tend to show dural invasion as a histological feature. Here we discuss the case of a 61 years old female with spontaneous rhinorrhea secondary to a clival chordoma, and the value of some specific MRI sequences in accurate identification of a tumor with such atypical presentation, enabling the surgeon to plan accordingly (AU)

Los cordomas de clivus en muy raras ocasiones se presentan como fístulas espontáneas del líquido cefalorraquídeo, ya que estos tumores no suelen invadir el plano dural. Se presenta el caso de una paciente de 61 años con rinolicuorrea espontánea secundaria a un cordoma de clivus, haciendo énfasis en secuencias específicas de resonancia magnética para la correcta identificación de un tumor con una manifestación inicial tan atípica, permitiendo una planificación quirúrgica adecuada (AU)

Spontaneous CSF rhinorrhea as a presenting symptom of a clival chordoma.

CSF rhinorrhea is a rare presenting symptom of a clival chordoma as these tumors do not tend to show dural invasion as a histological feature. Here we discuss the case of a 61 years old female with spontaneous rhinorrhea secondary to a clival chordoma, and the value of some specific MRI sequences in accurate identification of a tumor with such atypical presentation, enabling the surgeon to plan accordingly.

Gangliogliomas in the pediatric population.

BACKGROUND: Gangliogliomas are neoplastic lesions composed by a mixed population of neoplastic glial and dysplastic neural cells. They represent around 5% of all CNS tumors in the pediatric population. These usually are well-differentiated, slow-growing tumors, meaning that complete resection could cure most of these patients. Although most lesions remain stable over time after incomplete resection, some patients develop progression of the residual lesions: the optimal approach to treat these tumors is still to be defined. METHODS: This is a retrospective study in which we obtained data from medical records of pediatric patients who had a histological diagnosis of ganglioglioma following surgical treatment at a single center between 2001 and 2020. RESULTS: We included 17 pediatric subjects with gangliogliomas. The median age at diagnosis was 6.7 years, and the median follow-up duration was 60 months. The most common clinical presentation was epileptic seizures (41.1%). Hydrocephalus was present in 29.4% of cases. 52.9% of tumors involved exclusively the cerebral hemispheres, with the temporal lobe being the most affected location. Gross total tumor resection (GTR) was accomplished in 47% of all cases and in 75% of hemispheric tumors. Of patients, 33% in whom GTR could not be achieved showed progression of the residual tumor. BRAF V600E mutation was present in 44.4% of cases. CONCLUSION: Gangliogliomas are typically grade I tumors that occasionally affect children. They classically localize in the cerebral hemisphere but may involve deep structures like the basal ganglia, brain stem, and cerebellum, which seems to be particularly frequent in the pediatric population, implying further challenge to achieve adequate oncological control with surgery as the only treatment modality. Although most cases in which GTR could not be performed remained stable over the follow-up, significant progression of the tumor remains was observed in some patients. BRAF inhibitors should be considered as a feasible treatment option in this setting.