Endoscopic Endonasal Treatment of a Sinonasal Vascular Neoplasm in the Postnatal Period: Case Report and Review of Literature

Objectives: Congenital vascular lesions commonly present in the head and neck, and most are managed conservatively. Location and rapid growth, however, may necessitate surgical intervention. Endoscopic endonasal surgery (EES) in the pediatric population has emerged as a viable option in treating sinonasal and skull base lesions. Utilizing these techniques in newborns carries unique challenges. The objective of this report is to describe the successful use of direct intralesional embolization followed by endoscopic endonasal resection of a venous malformation in a postnatal patient. Methods: We reviewed the case reported and reviewed the pertinent literature. Results: A 6-week-old infant was found to have a large right-sided sinonasal lesion confirmed as a venous malformation. Rapid growth, impending orbital compromise, and potential long-term craniofacial abnormalities demanded the need for urgent surgical intervention. Risk of bleeding was mitigated with direct intralesional embolization. Immediately afterward, the patient underwent endoscopic endonasal resection of the lesion. EES in the very young presents multiple challenges both anatomically and behaviorally. A multidisciplinary approach lead to a successful outcome. Conclusion: We report a case of a 6-week-old infant, the youngest reported patient to the authors’ knowledge, who successfully underwent direct intralesional embolization followed by endoscopic endonasal resection of a sinonasal vascular malformation. This report highlights the challenges of this technique in the very young and demonstrates it as a viable treatment strategy for sinonasal vascular anomalies in this population.

Endoscopic endonasal resection of sinonasal teratocarcinosarcoma with intracranial breakthrough: illustrative case

BACKGROUND Teratocarcinosarcoma traversing the anterior skull base is rarely reported in literature. The heterogenous and invasive features of the tumor pose challenges for surgical planning. With technological advancements, the endoscopic endonasal approach (EEA) has been emerging as a workhorse of anterior skull base lesions. To date, no case has been reported of EEA totally removing teratocarcinosarcomas with intracranial extensions. OBSERVATIONS The authors provided an illustrative case of a 50-year-old otherwise healthy man who presented with left-sided epistaxis for a year. Imaging studies revealed a 31 × 60-mm communicating lesion of the anterior skull base. Gross total resection via EEA was achieved, and multilayered skull base reconstruction was performed. LESSONS The endoscopic approach may be safe and effective for resection of extensive teratocarcinosarcoma of the anterior skull base. To minimize the risk of postoperative cerebrospinal fluid leaks, multilayered skull base reconstruction and placement of lumbar drainage are vitally important.

Endoscopic Transorbital Approach to Mesial Temporal Lobe for Intra-Axial Lesions: Cadaveric Study and Case Series (SevEN-008)

BACKGROUND Endoscopic transorbital approach (ETOA) has been proposed as a minimally invasive technique for the treatment of skull base lesions located around mesial temporal lobe (MTL), mostly extra-axial pathology. OBJECTIVE To explore the feasibility of ETOA in accessing intraparenchymal MTL with cadaveric specimens and describe our initial clinical experience of ETOA for intra-axial lesions in MTL. METHODS Anatomic dissections were performed in 4 adult cadaveric heads using a 0° endoscope. First, a stepwise anatomical investigation of ETOA to intraparenchymal MTL was explored. Then, ETOA was applied clinically for 7 patients with intra-axial lesions in MTL, predominantly high-grade gliomas (HGGs) and low-grade gliomas (LGGs). RESULTS The extradural stage of ETOA entailed a superior eyelid incision followed by orbital retraction, drilling of orbital roof, greater and lesser wing of sphenoid bone, and cutting of the meningo-orbital band. For the intradural stage, the brain tissue medial to the occipito-temporal gyrus was aspirated until the temporal horn was opened. The structures of MTL could be aspirated selectively in a subpial manner without injury to the neurovascular structures of the ambient and sylvian cisterns, and the lateral neocortex. After cadaveric validation, ETOA was successfully performed for 4 patients with HGGs and 3 patients with LGGs. Gross total resection was achieved in 6 patients (85.7%) without significant surgical morbidities including visual field deficits. CONCLUSION ETOA provides a logical line of access for intra-axial lesions in MTL. The safe and natural surgical trajectory of ETOA can spare brain retraction, neurovascular injury, and disruption of the lateral neocortex.

Comparison of Outcomes following Primary and Repeat Resection of Craniopharyngioma

Introduction The management of recurrent craniopharyngioma is complex with limited data to guide decision-making. Some reports suggest reoperation should be avoided due to an increased complication profile, while others have demonstrated that safe reoperation can be performed. For other types of skull base lesions, maximal safe resection followed by adjuvant therapy has replaced radical gross total resection due to the favorable morbidity profiles. Methods Seventy-one patients underwent resection over a 9-year period for craniopharyngioma and were retrospectively reviewed. Patients were separated into primary resection and reoperation cohorts and stratified by surgical approach (endonasal vs. cranial) and survival analyses were performed based on cohort and surgical approach. Results Fifty patients underwent primary resection, while 21 underwent reoperation for recurrence. Fifty endonasal transsphenoidal surgeries and 21 craniotomies were performed. Surgical approaches were similarly distributed across cohorts. Subtotal resection was achieved in 83% of all cases. There were no differences in extent of resection, visual outcomes, subsequent neuroendocrine function, and complications across cohorts and surgical approaches. The median time to recurrence was 87 months overall, and there were no differences by cohort and approach. The 5-year survival rate was 81.1% after reoperation versus 93.2% after primary resection. Conclusion Compared with primary resection, reoperation for craniopharyngioma recurrence is associated with similar functional and survival outcomes in light of individualized surgical approaches. Maximal safe resection followed by adjuvant radiotherapy for residual tumor likely preserves vision and endocrine function without sacrificing overall patient survival.

Safety and Feasibility Analysis of a Prospective Trial on Stereotactic Body Radiotherapy for Solitary Bone Plasmacytoma

<b><i>Background:</i></b> There have been reports on the use of hypofractionated stereotactic body radiotherapy (SBRT) for bone plasmacytomas, but no prospective data are available. We present the initial analysis of an ongoing prospective protocol on SBRT addressing the feasibility and safety of this treatment for solitary bone plasmacytomas. <b><i>Patients and Methods:</i></b> A prospective cohort of SBRT for solitary bone plasmacytoma was developed. Patients could receive different doses depending on the index bone, from single fraction for skull base lesions, 24 Gy in 3 fractions for spine lesions, and 30 Gy in 5 fractions for other bones. Overall survival, bone events, local control, and progression to multiple myeloma (MM) were measured and compared to our retrospective cohort of patients treated with conformal standard-dose radiotherapy. Quality of life was assessed via the EORTC QLQ-C30 questionnaire, and toxicities were assessed by the CTCAE v5.0 criteria. After 1 year or the inclusion of 5–10 patients, a feasibility and safety analysis was programmed. <b><i>Results:</i></b> Between April 2018 and April 2019, 5 patients were included. All were male, with a median age of 53.1 years. The median follow-up was 21.8 months. No patient had local progression, bone event, or died. Two patients had progressions to MM. The mean survival free of progression to MM was 18.6 months, compared to 19 months in the retrospective cohort; median values were not reached. There were no grade 3 toxicities. <b><i>Conclusion:</i></b> SBRT for plasmacytoma is safe and feasible. More robust data are awaited.

Pediatric multicompartmental trigeminal schwannoma: illustrative case

BACKGROUND Trigeminal schwannoma (TS) is an uncommon and histologically benign intracranial lesion that can involve any segment of the fifth cranial nerve. Given its often impressive size at diagnosis and frequent involvement of critical neurovascular structures of the skull base, it represents a challenging entity to treat. Pediatric TS is particularly rare and presents unique challenges. Similarly, tumors with extension into multiple compartments (e.g., middle cranial fossa, posterior cranial fossa, extracranial spaces) are notoriously difficult to treat surgically. Combined or staged surgical approaches are typically required to address them, with radiosurgical treatment as an adjunct. OBSERVATIONS The authors presented the unusual case of a 9-year-old boy with a large, recurrent multicompartmental TS involving Meckel’s cave, the cerebellopontine angle, and the infratemporal fossa. Near-total resection was achieved using a frontotemporal-orbitozygomatic craniotomy with a combined interdural and extradural approach. LESSONS The case report adds to the current literature on multicompartmental TSs in children and their management. The authors also provided a simplified classification of TS that can be generalized to other skull base tumors. Given a lack of precedent, the authors intended to add to the discussion regarding surgical management of these rare and challenging skull base lesions.

Invisible compression, anterior fossa tumor causing trigeminal neuralgia

Background: Trigeminal neuralgia secondary to posterior and middle fossae tumors, whether ipsilateral or contralateral, has been well described. However, this disabling disease has never been reported in the context of anterior fossa neoplasms. Case Description: A 75-year-old female with right hemifacial pain was diagnosed with an anterior clinoid meningioma. Despite neuroimaging did not show any apparent anatomical or neurovascular conflict, a detailed MRI analysis revealed a V3 hyperintensity. Not only symptoms completely resolved after surgical resection but also this radiological sign disappeared. Nowadays, the patient remains asymptomatic and V3 hyperintensity has not reappeared during her follow-up. Conclusion: A surgical definitive treatment can be offered to patients suffering from trigeminal neuralgia secondary to lesions adjacent to Gasserian ganglion or trigeminal branches. In this respect, posterior and middle fossae tumors are well-reported etiologies. Nevertheless, in the absence of evident compression, other neoplasms located in the vicinity of these critical structures and considered as radiological findings may be involved in trigeminal pain. Microvascular and pressure gradient changes could be an underlying cause of these symptoms in anterior skull base lesions. Here, we report the case of a patient with uncontrollable hemifacial pain resolved after anterior clinoid meningioma removal.

Extended Frontobasal Approach for Skull Base Lesions

Background Lesions involving the skull base can be approached by a variety of surgical corridors and extended frontobasal approach is one of them. It provides quite a wide exposure to lesions in the midline of anterior skull base, paranasal sinuses, and sphenoclival region. Objective To share our experience, and list the merits and demerits, of this approach for anterior skull base lesions. Methods A total of six cases were operated using extended frontobasal approach. Four of them were skull base tumors with extensive involvement of paranansal sinuses and extension into sellar, parasellar, and clival region. Fronto-orbital and sphenoethmoidal osteotomy provided adequate surgical access, thereby facilitating their excision. Two cases of frontonaso-orbital encephalocele with large bone defect at anterior skull base were also operated upon. Skull base repair was performed using autologous bone graft, pericranium, and fibrin glue. Results Gross total excision was achieved in four cases of skull base tumors with good cosmesis as transfacial access was obviated. Excision, repair, and reconstruction of two patients with frontonaso-orbital encephalocele were also done with acceptable cosmesis. Conclusion The extended frontobasal approach is an excellent alternative for extensive anterior skull base tumors (up to posterior skull base), and also for the repair of large malformative lesions of the anterior skull base.