Journal of neurosurgery. Case lessons最新文献

Background: Eagle syndrome is characterized by an elongated styloid process causing mechanical stress on the internal carotid artery (ICA). The authors present the case of a patient who had cervical ICA dissection with a nonelongated styloid process.

Observations: A 43-year-old man presented with left hemiparesis and hemispatial neglect. The patient underwent endovascular treatment for tandem occlusion of the M1 segment of the right middle cerebral artery and right cervical ICA. After M1 segment thrombectomy, stenting of the right cervical ICA dissection was performed. Notably, the tip of the styloid process matched the caudal end of the dissection cavity, which was located on the lateral side of the ICA, where the styloid process was located. Based on these findings, the authors concluded that mechanical stress from the styloid process caused ICA dissection. After reducing antiplatelet therapy, the styloid process was surgically removed.

Lessons: Mechanical stress from a nonelongated styloid process can lead to ICA dissection-induced occlusion. In patients with cervical ICA dissection, the anatomical relationship between the styloid process and dissection site, as well as the distance between the ICA and styloid process, should be evaluated. https://thejns.org/doi/10.3171/CASE24719.

Background: Kyphotic spinal deformity is a complication of ankylosing spondylitis (AS). In rare cases, particularly in obese patients, the deformity might extend to the cervicothoracic spine, resulting in a severe "chin-on-abdomen" deformity. This condition severely impairs quality of life by affecting gaze, swallowing, and causing chronic pain. While corrective surgery is often performed for lumbar and thoracic kyphosis, cases involving global kyphotic deformities are less common.

Observations: The authors present the case of a 66-year-old obese man with AS and a chin-on-abdomen deformity. Prone positioning for surgery was not feasible due to his body habitus. A three-stage corrective surgery was performed: a C7 extension osteotomy in a semisitting position, an L3 pedicle subtraction osteotomy in the lateral decubitus position, and a T11 vertebral column resection in the prone position. The first stage utilized a seldom-used, earlier osteotomy technique with modern instrumentation and neuromonitoring. Obesity again precluded prone positioning in the second stage, necessitating surgery in the lateral position. Postoperatively, the patient exhibited significant postural improvement, maintained over a 5-year follow-up period.

Lessons: This case underscores the importance of adaptable techniques and positioning strategies in correcting complex spinal deformities in obese patients with AS. Integrating traditional methods with modern technology is crucial for achieving successful outcomes. https://thejns.org/doi/10.3171/CASE24664.

Background: Cases of congenital disorders of glycosylation (CDGs) are rare, and the occurrence of hemorrhagic infarction is also rare. The etiology is unclear.

Observations: A 3-year-old Asian boy with CDG type 1A was hospitalized with pneumonia. Consciousness disturbance and hemiplegia appeared after high fever and were associated with disseminated intravascular coagulation. Magnetic resonance (MR) images showed subcortical hemorrhagic infarction due to anterior superior sagittal sinus occlusion. Follow-up computed tomography revealed the enlargement of a right frontal lobe hematoma with a midline shift. The authors performed emergency craniotomy for hematoma evacuation. The postoperative course was favorable, with improvements in consciousness and hemiplegia. Patients with CDG type 1A have various types of coagulation disorders and sometimes develop several thrombotic and bleeding events; however, there has only been one reported case of CDG with concomitant intracranial hemorrhagic infarction. The authors detected sinus thrombosis on MR images for the first time in a patient with CDG.

Lessons: In patients with CDGs, abnormalities in the coagulation-fibrinolysis system can cause various neurological symptoms, such as intracranial bleeding, cerebral ischemia, and stroke-like episodes. In Asian infants with suspected cerebral venous sinus thrombosis, it is advisable to conduct examinations that include imaging modalities, such as MR venography and/or contrast-enhanced T1-weighted imaging, to confirm complications. https://thejns.org/doi/10.3171/CASE23729.

Background: The diagnosis of intracranial extraosseous Ewing's sarcoma (EES) poses challenges due to the absence of specific clinical and imaging features prior to surgery. It is crucial to differentiate the tumor from other small round cell malignancies postoperatively.

Observations: A 7-year-old patient was admitted to the authors' hospital due to the in situ recurrence of a posterior fossa tumor more than 1 month after the initial surgery for headache. Subsequently, a second surgery was performed at the authors' hospital, and pathological analysis indicated medulloblastoma. Genetic testing indicated Ewing's sarcoma. After 16 cycles of chemotherapy and 8 weeks of focal radiotherapy, the patient continued to exhibit clinical and radiographic remission. Whole-spine magnetic resonance imaging (MRI) revealed intraspinal enhancing lesions, which extended throughout the entire spinal canal. Palliative treatment was administered. The patient has been under observation for 2 months thus far, demonstrating disease stability as a result of the palliative treatment.

Lessons: Primary intracranial EES is an exceptionally rare condition that can be easily misdiagnosed. Genetic testing is necessary to differentiate it from other small round cell tumors. Currently, a combination of surgery and chemoradiotherapy has proven to be an effective treatment approach. Postoperative follow-up should include MRI examination of the entire nervous system to detect any cerebrospinal fluid seeding metastasis and reduce mortality. https://thejns.org/doi/10.3171/CASE24488.

Background: Spinal ependymomas are typically slow-growing tumors with a favorable prognosis. Recently, a new aggressive subtype has emerged with its own distinct histopathological and molecular features characterized by MYCN amplification. However, this subtype of spinal ependymoma is rare, and studies on its imaging characteristics are limited. In this case series, the authors present the imaging findings of three patients with MYCN-amplified spinal ependymoma from their institution.

Observations: Unlike typical spinal ependymomas, the MYCN-amplified spinal ependymomas were intradural extramedullary in location in the thoracic and lumbar spine. Imaging revealed T1 isointensity and T2 hyperintensity with avid enhancement and peritumoral edema. The tumor masses were adherent to the spinal cord with filling of the canal and resultant cord compression. There were focal areas without a clear tissue plane between the mass and the cord, which was concerning for cord infiltration. Thus, complete resection was difficult to achieve, and all patients demonstrated residual tumor in the resection bed. Their clinical course was also characterized by early central nervous system (CNS) dissemination, including one case with intracranial involvement.

Lessons: This case series highlights three instances of MYCN-amplified spinal ependymoma, a rare and aggressive subtype with distinctive imaging features, including an intradural extramedullary location and CNS dissemination at recurrence. https://thejns.org/doi/10.3171/CASE24696.

Background: Posttraumatic retroclival hematomas are rare pathologies among pediatric patients and can result in cranial nerve palsies. The authors sought to survey the literature and characterize the risk factors, treatment considerations, and overall outcomes for pediatric patients experiencing posttraumatic retroclival hematomas.

Observations: A search of the Ovid Embase, Scopus, PubMed, and Web of Science databases from January 1986 to May 2024 was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Additionally, the authors report a novel case of pediatric retroclival hematoma. The systematic review identified 41 eligible articles describing 64 individual cases of posttraumatic clival/retroclival hematoma in pediatric patients. The incidence of abducens nerve palsy in the setting of posttraumatic retroclival hematoma was 40%, and the rate of complete recovery of abducens nerve function was 73%. Analyses revealed that a higher initial Glasgow Coma Scale (GCS) score correlated with general neurological recovery (p < 0.05). However, no significant difference was found in GCS scores between patients with complete and those with incomplete abducens nerve recovery.

Lessons: There is a high rate of spontaneous recovery of abducens nerve function in patients with abducens palsy in the setting of posttraumatic retroclival hematoma. While initial GCS scores can predict general neurological recovery, factors influencing abducens nerve palsy resolution remain unclear. https://thejns.org/doi/10.3171/CASE24474.

Background: Myelomeningocele and sagittal craniosynostosis are 2 neurosurgical pathologies with complications such as increased intracranial pressure (ICP) and hydrocephalus. While the 2 defects commonly occur independently, their simultaneous occurrence is exceptionally rare.

Observations: The authors report the case of a newborn male diagnosed with a simultaneous myelomeningocele and sagittal craniosynostosis. The patient underwent surgical repair of the myelomeningocele 2 days after birth. Wound breakdown and ventriculomegaly were observed 13 days after birth. Surgical wound repair was performed, and a neonatal reservoir was inserted because of concerns for wound healing and increased ICP. The reservoir was tapped daily, and the ventricle size was monitored via biweekly ultrasounds. Due to the increased risk of ICP presented by the sagittal craniosynostosis, an open strip craniectomy was performed at 34 days of life. Following surgery, cranial ultrasound studies demonstrated improving ventricle size and neonatal reservoir tapping was spaced to weekly. The patient was discharged at 68 days of life in stable condition, with no permanent cerebrospinal fluid diversion needed at the 8-month follow-up.

Lessons: Surgical repair of sagittal craniosynostosis in the presence of a myelomeningocele should be considered earlier in life than generally indicated to limit the risk of developing elevated ICP, hydrocephalus, and subsequent complications. https://thejns.org/doi/10.3171/CASE24616.

Background: The authors report the first case of thoracic interdural spinal cysts presenting as radiculopathy attributed to overdrainage-related cervical venous plexus enlargement. This case emphasizes the importance of considering interdural spinal cysts and cerebrospinal fluid overdrainage in the differential diagnosis of radiculopathy.

Observations: A 37-year-old male patient with a history of orthostatic headache presented with bilateral deltoid muscle atrophy consistent with C5 radiculopathy. Postcontrast magnetic resonance imaging (MRI) revealed cervical epidural venous plexus enlargement and nerve root compression. Thoracic MRI showed an interdural cyst extending from C7 to T11. In addition, a small defect in the inner layer of the dura, which connects the subarachnoid space to the cyst at the T10 level, was detected on thin-slice MRI. Surgery was performed to close the dural defect, with endoscopic assistance facilitating definitive treatment. Postoperative MRI confirmed the resolution of the spinal cyst and epidural venous enlargement. Furthermore, the patient's symptoms improved.

Lessons: Evaluating the cervical spinal pathology is the common approach for cervical radiculopathy. However, in the authors' case, the presence of thoracic lesions could have been an underlying cause. Hence, identifying this unique clinical presentation can raise awareness among neurosurgeons and lead to better patient outcomes by addressing the underlying pathology in a timely manner. https://thejns.org/doi/10.3171/CASE24533.

Background: Aneurysmal subarachnoid hemorrhage (aSAH) is often associated with acute high-pressure hydrocephalus. Less commonly, an acute low-pressure hydrocephalus (ALPH) variant can develop and contribute to increased morbidity. ALPH is particularly challenging to diagnose and manage, as patients present with symptoms of increased intracranial pressure (ICP) despite the absence of corroborating evidence from ICP measurements. Misdiagnosis or delayed recognition can result in increased morbidity.

Observations: The authors describe a patient with aSAH who developed neurological deterioration from ALPH. Despite normal ICP readings, the patient displayed symptoms of increased ICP. Significant electroencephalography (EEG) changes preceded the neurological deterioration by many hours. In addition, these EEG changes reversed with cerebrospinal fluid removal and eventual hydrocephalus resolution.

Lessons: ALPH presents with paradoxical ICP dynamics, complicating its diagnosis. Careful monitoring, including EEG, can provide an early indication of neurological deterioration and guide timely intervention. This case underscores the importance of considering ALPH in patients with aSAH who show clinical worsening without corresponding increases in ICP. Tailoring management to address the atypical pressure dynamics is crucial for improving outcomes. https://thejns.org/doi/10.3171/CASE24612.

Background: Exophytic tumors of the calvaria (ETCs) remain a challenging pathology because of their complex management. The authors discuss the case of a woman with a large exophytic mass of the right frontotemporal region and underline their decision-making process on the management of this unique case and possible similar ones.

Observations: Neuroradiological findings showed a calvarial tumor with both epicranial and intracranial extension involving the frontotemporal bone with a mixed component (lytic and sclerotic) and dural infiltration with a pseudonodular pattern. A wide en bloc excision from the skin to the dura mater was performed. The compound 5-aminolevulinic acid (5-ALA) was not very useful in identifying the tumor boundaries. One-step cranioplasty and a skin pedicle flap were used to reconstruct the anatomical defect. Acellular dermal matrix was used to repair the uncovered calvaria. Pathological examination confirmed the diagnosis of pleomorphic dermal sarcoma (PDS).

Lessons: In the evaluation of an ETC, PDS should be considered. Wide en bloc excision, if achievable, should be considered the gold standard. The 5-ALA was not helpful, and a pedicle skin flap could be considered as a less invasive alternative to microsurgical reconstruction. The use of an acellular matrix implant on the contralateral exposed galea increases the rate of reconstruction success. https://thejns.org/doi/10.3171/CASE24457.