Sinonasal pediatric rhabdomyosarcoma is a rare cancer with a prognosis dependent on stage/grade, histology, and molecular characteristics. Radiation therapy is considered first-line therapy in conjunction with chemotherapy, determined based on the disease risk group. Surgery is generally recommended when margin-free resections are possible. Here we present a unique case report of a patient with recurrent rhabdomyosarcoma and an overview of the salient literature pertaining to the management of this case.
Introduction Synovial cysts of the atlantoaxial joint (AAJ) are rare lesions that may cause progressive spinal cord compression and cervical myelopathy. Surgical management of retro-odontoid pathology is challenging due to complex regional anatomy and proximity to critical neurovascular structures. Traditional approaches, including transoral and posterior transdural techniques, are associated with notable morbidity. The endoscopic endonasal approach (EEA) provides a minimally invasive alternative with direct access to the retro-odontoid space. We report an isolated AAJ synovial cyst treated with an endoscopic endonasal odontoidectomy as the sole anterior approach, highlighting technical refinements that optimized access to the retro-odontoid space. Case Description A 48-year-old woman with a history of C4 corpectomy and C3 to C7 anterior fusion presented with progressive neck pain and cervical myelopathy, secondary to a retro-odontoid cystic lesion causing spinal cord compression. She underwent an endoscopic endonasal odontoidectomy and lesion resection followed by posterior occipitocervical fusion. Surgical exposure was optimized through posterior septectomy and targeted drilling of the odontoid process to access the retro-odontoid space. Excision and adequate decompression of the spinal cord were achieved, and histopathology was consistent with synovial cyst. Conclusion The EEA allowed effective ventral decompression of the AAJ synovial cyst while minimizing morbidity associated with transoral and posterior transdural approaches. Strategic modifications to improve surgical angulation and maneuverability can overcome traditional limitations of the endonasal corridor. This approach represents a viable, minimally invasive option for select retro-odontoid pathologies while reducing trauma to oropharyngeal tissue and avoiding dural violation at the AAJ.
Spinal subdural hematomas (SSDH) are a rare but clinically significant cause of spinal cord compression that constitute a small portion of intradural spinal hematomas. Cervical SSDHs are infrequently documented in existing literature and are uncommon without coagulopathy, direct spinal trauma, spinal instrumentation, or intracranial hemorrhage. Accurate and timely diagnosis is challenging due to radiographic similarities with epidural hematomas and ambiguity in the timing and nature of symptoms. The patient was a 41-year-old male who presented to a Level 1 Trauma Center after being struck by a car while walking. While multiple minor, non-surgical injuries were sustained, initial spinal imaging revealed only mild prevertebral edema. On hospital day three, the patient experienced a significant neurological decline with resultant bilateral upper extremity weakness. Emergent repeat imaging discovered a large hematoma at the level of C3-4 with concomitant severe spinal cord compression and edema. He was taken emergently for anterior decompression; however, the hemorrhage was discovered to be subdural, prompting posterior cervical decompression with eventual resolution of his symptoms. This case highlights the diagnostic challenges of cervical SSDH in the absence of spinal fractures, ligamentous injury, coagulopathy, or recent instrumentation. While magnetic resonance imaging (MRI) may mischaracterize SSDHs for radiographically similar extradural pathology and misdirect surgical planning, intraoperative ultrasound afforded clarification of hematoma location and confirmed adequate decompression. Due to its highly atypical nature, we demonstrate the utility of ultrasound as an operative supplement in unclear hematoma management and the value of considering SSDH in the differential diagnosis of post-traumatic spinal cord compression.
Background Hemangioblastomas are benign, highly vascular World Health Organization (WHO) grade 1 tumors that most commonly arise in the cerebellum. Brainstem involvement is uncommon, and lesions originating from the cervicomedullary junction with extensive caudal cystic extension into the cervical spinal cord are exceptionally rare, posing significant diagnostic and microsurgical challenges. Case Description A 39-year-old man presented with a 2-year history of progressive paresthesia involving both upper limbs, right-sided weakness, gait imbalance, and cerebellar symptoms. Magnetic resonance imaging demonstrated a vividly enhancing mural nodule arising from the dorsal cervicomedullary junction associated with an elongated intramedullary cystic cavity extending caudally to the C3 vertebral level, accompanied by cord edema and syringomyelia. Evaluation for von Hippel-Lindau disease was negative. The patient underwent gross total microsurgical excision through a midline suboccipital craniectomy with C1 laminectomy. Histopathological examination confirmed hemangioblastoma (WHO grade 1). The postoperative course was uneventful, with significant neurological improvement during follow-up. Conclusion Primary cervicomedullary hemangioblastoma with extensive cervical cystic extension is an exceptionally rare lesion. Careful preoperative imaging evaluation, meticulous microsurgical technique, and complete tumor excision can achieve favorable neurological outcomes. Comparison with previously reported cases highlights the rarity of this presentation and supports gross total resection as the treatment of choice whenever safely feasible.
Post-injection sciatic neuropathy is a recognized iatrogenic complication of intragluteal injection. We report a case of a young adult presenting with progressive complete paralysis of the left lower limb following intragluteal injection. Magnetic resonance imaging (MRI) demonstrated focal thickening and T2/short-tau inversion recovery (STIR) hyperintensity of the sciatic nerve in the gluteal region, without neuroma or discontinuity and without associated muscle denervation changes. These findings were consistent with post-injection sciatic neuropathy, likely representing axonotmesis. MR neurography was useful in confirming the site and extent of injury and in excluding structural complications. This case highlights the characteristic imaging appearance of injection-related sciatic nerve injury and emphasizes the importance of correct injection technique.
We report a rare case of delayed peripheral facial paralysis following left anterior temporal lobectomy (ATL) for mesial temporal sclerosis in a 58-year-old woman with a long-standing history of seizures. Preoperative evaluation confirmed left temporal sclerosis as the epileptogenic focus. Surgery with neurophysiologic monitoring was performed, and initial postoperative assessment revealed no facial deficits. However, 4 days later, the patient developed left-sided peripheral facial paralysis (House-Brackmann grade 4), which improved after a 10-day course of oral corticosteroids. This case underscores that delayed facial paralysis, although uncommon, can occur after ATL, potentially due to mechanical or thermal injury near the geniculate ganglion or viral reactivation induced by direct manipulation or surgical stress. Awareness and timely follow-up are essential for early detection and management of such complications.
A review of the literature reveals that fewer than two dozen pediatric cases of nondysraphic intramedullary lipoma have been described, with most lesions spanning limited spinal segments. Extensive or holodorsal involvement has been reported only rarely, including isolated cases involving long cervicothoracic segments or multilevel disease. The present case is notable not only for the patient's age, but also for the exceptional size and extent of the lesion, exceeding that of most previously reported pediatric nondysraphic intramedullary lipomas. This distinction is clinically relevant, as lesion size and longitudinal extent may increase operative complexity and the risk of postoperative neurological deficit and spinal deformity. Because of their rarity, optimal management strategies for nondysraphic intramedullary lipomas remain incompletely defined. Complete resection is often precluded by the absence of a clear dissection plane between the lipoma and normal neural tissue, and subtotal debulking is generally favored to minimize neurological morbidity. Despite these challenges, timely surgical intervention in symptomatic patients can halt neurological deterioration and allow for meaningful functional recovery. We present a case of a nondysraphic intramedullary spinal cord lipoma that, on review of literature, represents one of the largest reported nondysraphic intramedullary lipomas in a pediatric patient. This case adds to the limited body of literature on this rare disease process and underscores important considerations regarding diagnosis, surgical strategy, and long-term management.
Superior orbital fissure syndrome (SOFS) is a rare, vision-threatening condition caused by injury or compression of cranial nerves III, IV, and VI. We report a 16-year-old male who sustained extensive craniofacial trauma, including a displaced left sphenoid wing fracture, after a motorized scooter accident. Initially, he had limited extraocular motility, but by hospital day 5, he developed complete ophthalmoplegia due to progressive nerve compression. Urgent surgical decompression of the superior orbital fissure and reduction of the fracture were performed via a pterional craniotomy on day 7. Postoperatively, the patient experienced full restoration of ocular motility and pupillary function, with no complications. This case highlights the potential for delayed neurological deterioration in sphenoid wing fractures and emphasizes the importance of close serial examination and timely operative intervention once SOFS develops, demonstrating that even delayed intervention can result in complete neurological recovery.
Objective To demonstrate magnetic resonance imaging's (MRI's) utility in investigating auditory brainstem implant (ABI) performance degradation. Design Case study. Setting Academic medical center. Participant This report presents a patient with neurofibromatosis type 2 and an ABI with durable, limited open-set speech who developed nonauditory side effects and a complete lack of auditory perception. ABI paddle placement was assessed with 1.5 Tesla (T) Siemens MRI, and device function was assessed utilizing integrity tests. Main Outcome Measure The etiology of ABI performance degradation utilizing 1.5 T Siemens MRI. Results On initial postoperative 1.5 T Siemens MRI, the ABI paddle approximated the cochlear nucleus. Approximately 1 year later, the patient experienced ABI performance degradation and dizziness. Although ABI device integrity testing was normal, follow-up 1.5 T Siemens MRI revealed lateral ABI paddle migration from the foramen of Luschka toward the flocculus, likely due to observed fat atrophy from the prior craniotomy reconstruction, suggesting a possible etiology for the observed ABI degradation. Conclusion This report presents MRI's ability to successfully delineate postoperative fat atrophy, ABI paddle migration in relation to the brainstem, and residual tumor in a patient with ABI degradation.
Surgical resection of a single brain metastasis improves survival. However, lesions in the parasagittal perirolandic region pose a therapeutic challenge due to high eloquence and the "gravity-buoyancy conflict." Under physiological conditions, cerebrospinal fluid buoyancy reduces the brain's effective weight by approximately 95%. Loss of this support upon opening the dura leads to gravitational sag of the hemisphere. This increased functional weight pulls the bridging veins downward, creating a valve-like effect and a mechanical kink at the rigid dural entry point. A 59-year-old male presented with a 3-cm cystic metastasis in the posterior superior frontal gyrus (SFG). Intraoperatively, gravitational sag of the tumor-expanded gyrus created significant downward traction on the vein of Trolard. This tension induced a potential focal kink at the rigid dural interface, resulting in flow stasis, recognized by distal colorless depletion and dark, pulsatile proximal blood. Consent for the procedure and image publication was obtained, and Queen's University Ethics Board approved this report. Wide splitting of both the superior frontal and precentral sulci allowed for necessary safe access to the tumor and potential decoupling of the tumor-bearing SFG from the hemisphere. To resolve the dural entry kink, a strategic wide arachnoid release was performed along the vein to provide vascular laxity. After early cyst drainage and unloading the pathological weight, the created laxity allowed the vein to return to its physiological location, resolving the stasis. Untethering the vein from its arachnoid anchors allowed the vein to tolerate necessary traction on the vessel wall during tumor resection ( Video 1 ).
Dizziness is a prevalent clinical complaint, and determining the underlying etiology can be challenging. Posterior fossa masses have been observed to cause vertigo, with presentations very similar to Meniere's disease. In this report, we describe a case of a patient who presented with recurrent episodes of Meniere's-like vertigo in the setting of a posterior fossa meningioma, who underwent surgical resection of the tumor with significant improvement postoperatively. We postulate that these symptoms were caused by secondary endolymphatic hydrops and were able to demonstrate improvement in high-frequency vestibulo-ocular reflex with pre- and postoperative video head impulse testing.
Background and Importance Optic nerve gliomas (ONGs) are benign and slow-growing tumors with a variable clinical course that may result in significant morbidity. The variability in tumor burden, challenging anatomy, and associated symptomatology require an individualized and multidisciplinary approach. The relative paucity of cases and variation in surgical anatomy prompt the need for accrual of cases in literature to advance the understanding of surgical techniques to address this rare clinical entity. Clinical Presentation A 19-year-old patient presented with severe and globe-threatening left eye proptosis, negatively affecting quality of life, due to a sporadic intraorbital ONG that had been inexorably growing over the previous 9 years, despite chemotherapy. The patient was taken to the operating room for a single-stage globe-sparing transorbital resection of the intraorbital ONG. The surgical plan consisted of an endoscopic endonasal approach for medial and inferior orbital wall decompression combined with an open lateral orbitotomy, via a lateral canthal incision, for subtotal piecemeal tumor resection to relieve the proptosis, restore alignment of the globe, and yield tissue for molecular analysis. The patient experienced profound improvement in quality of life and opted for radiographic surveillance that has been stable at 5 years. Discussion and Conclusion Surgical approaches to intraorbital tumors have included both transcranial and/or transorbital routes, with the latter more recently encompassing endoscopic endonasal techniques. This report presents the use of a novel combination of transorbital techniques for resection of an intraorbital ONG with severe proptosis, including an endoscopic endonasal approach combined with an open lateral orbitotomy.
The superficial temporal artery (STA) is one of the final branches of the external carotid artery. STA pseudoaneurysms are a rare vascular complication following blunt trauma, and even rarer following craniotomy. A 44-year-old man presented to hospital with altered level of consciousness and left-sided paresis. Neuroimaging showed a right middle cerebral artery bifurcation aneurysm, subarachnoid hemorrhage, and a large right-sided intracerebral hematoma (ICH). An emergent fronto-temporo-parietal craniotomy was performed for hematoma evacuation and clipping of the ruptured aneurysm. On postoperative day 21, the patient developed sudden-onset scalp swelling and a decline in neurological status. Computed tomography imaging showed a ruptured STA pseudoaneurysm, creating a subgaleal hematoma that tracked through the burr hole cover, dural leaflets, and into the brain to create an ICH. An emergent craniotomy for hematoma evacuation and STA resection was done. STA pseudoaneurysms are rare following a craniotomy. Only one previous case has been reported of a pseudoaneurysm secondary to a galeal hook. Neurosurgeons frequently encounter the STA. It is imperative to be mindful of the placement of scalp retractors to avoid STA injury and further postoperative complications.
Background Brain abscess following sinonasal mucormycosis is a rare and life-threatening complication. Since our knowledge is mostly based on case reports, a well-documented way of treatment for such cases is yet to be found. Case Description A retrospective case series study was conducted at a tertiary hospital. Patients whose brain abscesses were treated without opening the dura during surgery were selected for the study. The patients received radical debridement of necrotic tissues as well as the infected bones adjacent to the brain abscess. Then, they were treated medically. Three patients were included in the study. All patients had a history of diabetes and COVID-19 before developing symptoms of sinonasal infection. The average size of the abscesses was less than 2 cm. Conclusion Removing infected tissues and bones surrounding the brain abscess without opening the dura can be a viable treatment option for mucormycosis-induced brain abscesses of less than 2 cm.
Background:Facial nerve damage remains a significant risk during vestibular schwannoma (VS) resection, with reported incidences varying widely (3-46%). Damage risk increases with tumor size. Digital tractography enables nerve reconstruction but typically involves manual procedures, resulting in subjective evaluations that limit reproducibility and validation. We introduce a robust, semi-automatic tractography methodology with reproducible region-of-interest (ROI) generation and present an initial validation using a novel quantitative three-dimensional comparison approach in patients with a large VS. Objective:To assess the accuracy of facial nerve reconstruction employing a semi-automatic ROI selection method in patients with VSs. Materials and Methods:We included six patients with an average tumor size of 28 mm (95% CI: 17-40, 100% left) who underwent translabyrinthine VS surgery. Each VS patient was scanned with the regular neuronavigation magnetic resonance imaging (MRI) protocol and a custom diffusion-MRI protocol before surgery. The facial nerve trajectory was reconstructed with a diffusion tensor imaging-based tractography software package using semi-automatic ROI generation. We validated our reconstructions with the Brainlab neuronavigation system for intraoperative point annotation along the course of the facial nerve. Results:Tracts could be reconstructed in all included patients. The median distance and angle between the points and closest reconstruction were 5.1 mm (IQR: 3.5-7.6) and 38.5 degrees (IQR: 2.7-79.8), respectively. Conclusion:We present a promising methodology for facial nerve reconstruction in patients with VSs. However, further optimization of the methodology is warranted before a proper clinical validation study can be performed.
[This corrects the article DOI: 10.1055/a-2816-7368.].
Background Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a rare neoplasm characterized by a paradoxical clinical course. Despite its high-grade histologic features, HMSC exhibits an unexpectedly indolent clinical behavior, marked by frequent local recurrences but limited metastatic potential. We report the first case of HMSC metastasis to the liver with the earliest known distant progression. Case Report A 53-year-old male who presented with unilateral epistaxis was found to have T4aN0M0 HPV35-positive HMSC of the left maxillary sinus, with erosion into the orbital floor. He underwent induction chemotherapy, endoscopic resection with negative margins, and adjuvant chemoradiation. He did well until surveillance PET-CT at 10 months revealed asymptomatic hepatic metastases confirmed by biopsy. He was treated with transarterial chemoembolization and pembrolizumab before developing locoregional recurrence involving the orbital apex and cavernous sinus 5 months later. Conclusion Several case reports demonstrate HMSC's potential for aggressive behavior, including intracranial extension and rapid recurrence. However, distant metastases (DM) are rare, with only three documented cases of pulmonary metastases and one digital metastasis, occurring years after initial treatment. We present the first reported case of HMSC with metastasis to the liver, marking the earliest known distant recurrence. This case challenges the current paradigm by highlighting HMSC's potential for aggressive systemic progression. Molecularly, this tumor harbored high-risk HPV35, a rarely reported subtype. Despite HMSC's typically indolent course, this case underscores the importance of vigilant surveillance to detect early DM and guide therapeutic management.
Background Primary intraosseous meningiomas (PIM) are rare extradural tumors, representing less than 2% of all meningiomas. They are often slow-growing and may present with nonspecific radiological features, complicating diagnosis.Case Report We report the case of a 48-year-old female with progressive left frontotemporal bulging over 12 months, without neurological deficits. Imaging revealed an osteoblastic lesion in the left sphenoid and temporal bones, causing mild compression of adjacent structures. The patient underwent craniectomy with complete tumor resection and immediate cranioplasty using a preplanned customized prosthesis. Histopathology and immunohistochemistry confirmed a meningothelial meningioma, with tumor cells confined to intertrabecular bone spaces and free surgical margins, consistent with a primary intraosseous origin. Postoperative follow-up demonstrated excellent cosmetic results and no recurrence.Conclusion PIMs pose diagnostic and therapeutic challenges due to their rarity and nonspecific imaging features. Histopathology remains essential for definitive diagnosis. Preoperative 3D prototyping and customized prosthesis planning can optimize surgical reconstruction, reduce deformities, and improve cosmetic and functional outcomes. This case highlights the importance of integrating clinicoradiological evaluation, histopathology, and modern reconstructive techniques to achieve complete resection and minimize recurrence.
Background Intradiploic epidermoid cysts are rare lesions that account for a small fraction of intracranial tumors with occasional malignant transformation. Occipital involvement is particularly uncommon, and lesions may reach giant dimensions with a risk fossa compression.Case Presentation A 60-year-old woman presented with a 12-month history of progressive headache and vertigo. Magnetic resonance imaging (MRI) revealed a large, diffusion-restricting extra-axial mass arising from the occipital bone and extending into the posterior fossa. She underwent a posterior fossa craniotomy with gross-total resection while preserving dural integrity. Histopathology confirmed an epidermoid cyst. The postoperative course was uneventful, and follow-up MRI showed complete resection with no recurrence.Conclusion Giant intradiploic epidermoid cysts of the occipital bone, though histologically benign, can cause extensive bone destruction and significant posterior fossa mass effect. Accurate preoperative imaging and meticulous surgical planning are critical to achieving safe gross-total excision and favorable outcomes, particularly in atypical or extensive presentations.
Background The gyrus rectus (GR) is located at the base of the anterior cranial fossa, above the prechiasmatic optic nerves and optic chiasm. Herniation of the GR has been reported secondary to idiopathic causes or space-occupying lesions of the frontal lobe. However, GR herniation following transsphenoidal pituitary surgery has not been described previously. Case Description A 51-year-old woman with no comorbidities other than diabetes presented with visual disturbance in the right eye. Imaging revealed a non-functioning pituitary macroadenoma compressing the optic chiasm from below. The patient underwent microscopic transsphenoidal resection of the pituitary adenoma, achieving total removal. Early postoperative recovery was uneventful. Six-month follow-up MRI demonstrated inferior herniation of the GR into the sellar cavity, compressing the optic nerves superiorly. Visual complaints partially improved without further progression. Conclusion This case represents the first reported instance of GR herniation following transsphenoidal pituitary surgery. Awareness of this rare complication is essential when evaluating persistent or new-onset visual symptoms postoperatively.
