Journal of neurosurgery. Case lessons最新文献

Background: Gorham-Stout disease (GSD) is an extremely rare osteolytic disorder characterized by resorption of skeletal bone due to aggressive, nonneoplastic lymphatic proliferation.

Observations: The authors report the 14th case of GSD of the craniovertebral junction (CVJ), which resulted in cranial settling, basilar impression, and an acquired Chiari malformation type I (CM-I) in a pediatric patient. A 4-year-old girl initially presented with recurrent symptoms of pharyngitis, bacteremia, and neck pain. She continued experiencing waxing and waning symptoms of fever and neck pain that gradually worsened. At 12 years old, imaging revealed progressively worsening osteolytic changes with bone marrow edema involving the CVJ and subaxial cervical spine with progressive cranial settling, basilar impression, and 14 mm of tonsillar herniation, consistent with an acquired CM-I. She underwent a frontal bone biopsy, which confirmed the diagnosis of GSD. Treatment with sirolimus and bisphosphonates stabilized the osteolysis.

Lessons: GSD represents a rare group of osteolytic bone disorders with a highly variable clinical course. A high index of suspicion is warranted, as diagnosis can be challenging. Early referral to a specialized center is key, as treatment is directed toward symptomatic management and a multidisciplinary team approach. GSD is another syndrome associated with CVJ bony changes and tonsillar herniation. https://thejns.org/doi/10.3171/CASE24298.

Background: Malignant transformation of epidermoid cysts (ECs) into squamous cell carcinoma (SCC) is extremely rare, with associated leptomeningeal carcinomatosis being much less likely. Malignant transformation of ECs has a poor prognosis with a median overall survival of 10 months following transformation.

Observations: A 59-year-old male with a history of a subtotally resected posterior fossa EC presented with worsening symptoms 14 years following the initial subtotal resection. Imaging showed obstructive hydrocephalus and extensive leptomeningeal carcinomatosis. Laboratory workup confirmed malignant transformation to SCC.

Lessons: Nonmalignant recurrence of benign ECs is relatively common, often due to the complexity of complete resection and adherence to adjacent neurovascular structures. On the other hand, malignant degeneration of an EC is extremely rare. Any rapid change in symptoms, failure to recover following surgery, and new areas of irregular thickening and enhancement on imaging should prompt further evaluation for this process. https://thejns.org/doi/10.3171/CASE24738.

Background: Direct carotid-cavernous fistulas (CCFs) are relatively rare but dangerous complications of penetrating traumatic brain injury or maxillofacial trauma. A variety of clinical signs have been described, including ophthalmological and neurological ones. In some cases, severely altered cerebral blood flow can present as massive life-threatening bleeding through the nose, subarachnoid hemorrhage, and/or intraparenchymal hemorrhage. Although intuitively it makes sense that the elevation of venous pressure can obstruct the absorption of cerebrospinal fluid after the formation of a CCF, reports of CCFs presenting with acute acquired hydrocephalus do not exist.

Observations: This case illustrates the development of hydrocephalus in a patient with a direct CCF.

Lessons: In the absence of a direct path of access to the cavernous sinus due to occlusion of the carotid artery and difficult venous access, a transcirculation route was used to obliterate the CCF and resolve the hydrocephalus in this patient. https://thejns.org/doi/10.3171/CASE24618.

Background: Optic nerve schwannomas are an extremely rare pathology in neurosurgery. Their origin is rather debatable given the structure of the optic nerve, which does not typically have Schwann cells therein. However, a number of clinical cases of optic nerve tumors classified as schwannomas have been described in the literature. At present, there is no fundamental understanding of the etiology and pathogenesis of these tumors or treatment due to their rare incidence.

Observations: The authors describe the clinical case of a 40-year-old female patient with blurred vision in the left eye for 6 months who was operated on for an optic nerve tumor via a minimally invasive supraorbital approach. Complete resection of the tumor was achieved. Histopathological examination revealed a schwannoma. The patient had no postoperative complaints or neurological deficits. The authors also performed a detailed review of the literature for cases with optic nerve schwannomas. Only 18 patient outcomes have been published so far. There are significant differences in the structure, localization, size, and surgical treatment of optic nerve schwannomas.

Lessons: Optic nerve schwannomas are extremely rare lesions. Hence, there is a need to accumulate knowledge in order to study the etiology, pathogenesis, and treatment of these tumors. The minimally invasive supraorbital approach can be successfully used in the surgical treatment of optic nerve schwannomas located in the optic canal. https://thejns.org/doi/10.3171/CASE24638.

Background: Pedicle screw insertion in posterior spinal surgery can cause vascular injuries, including rare intercostal artery pseudoaneurysms, which are typically discovered incidentally during reimaging. Onyx embolization is an effective treatment for small artery pseudoaneurysms.

Observations: A 36-year-old man who had initially presented with back pain that remained unresponsive to nonsteroidal anti-inflammatory drugs was diagnosed with a T7-8 sarcomatous lesion confirmed by magnetic resonance imaging and biopsy. He underwent a posterior resection and T5-10 stabilization with pedicle screws. After surgery, significant hemorrhage led to his transfer for digital subtraction angiography, which revealed a prominent vascular tumor blush and a pseudoaneurysm of the right T5 intercostal artery due to screw placement. The tumor and pseudoaneurysm were effectively embolized using superselective catheterization and Onyx.

Lessons: This report highlights the dangers of intercostal artery damage from posterior spinal screws, emphasizing the need for meticulous postoperative imaging. Onyx embolization is an effective treatment for such complications. https://thejns.org/doi/10.3171/CASE24291.

Background: Septic arthritis of the lumbar facet joint (SALFJ) is a rare condition that can lead to serious complications. The authors present an uncommon case in which SALFJ resulted in bacterial meningitis (BM) with hydrocephalus and pyogenic ventriculitis, causing a disturbance of consciousness. Reports describing perforation of the dura mater by an epidural abscess are rare, and the present case offers valuable insights into the management of complex and severe complications arising from SALFJ.

Observations: A 54-year-old woman presented with impaired consciousness and paraplegia. The patient was eventually diagnosed with SALFJ at the left L3-4 facet joint complicated by BM, hydrocephalus, pyogenic ventriculitis, and acute transverse myelitis. Surgery confirmed a dural perforation, enabling direct invasion of the abscess into the central nervous system (CNS). Surgical interventions, including debridement, dural reconstruction, and local antibiotic perfusion, along with systemic antibiotics, significantly improved the patient's condition.

Lessons: This case highlights the potential for an epidural abscess of the SALFJ to directly perforate the dura mater, causing severe CNS complications. This emphasizes the importance of prompt diagnosis and appropriate surgical intervention in managing such complex cases. Timely lumbar surgery, combined with targeted antibiotic therapy, can be life-saving in this rare but critical scenario. https://thejns.org/doi/10.3171/CASE24621.

Background: Radiation-induced sarcoma (RIS) is an exceptionally rare occurrence following radiation therapy, and manifestation usually occurs after a several-year latency period. Herein, the authors report the development of a radiation-induced osteosarcoma of the frontoparietal calvaria following treatment for an oligodendroglioma in an 84-year-old woman.

Observations: The patient had been diagnosed with a grade III anaplastic oligodendroglioma when she was 78 years old. She had undergone resection and subsequent chemoradiation therapy for treatment of the tumor. In the following years, she experienced progressive neurological decline, which was complicated by multiple ischemic strokes and seizure episodes. When she was approximately 84 years of age, a small mass was noted overlying the previous craniotomy site during examination. Several months later, approximately 5 years after chemoradiation therapy, she presented to the emergency department with a suspected stroke. Examination revealed that the mass had rapidly grown and was pressing into the intracranial space. She underwent craniotomy, and specimens were taken. Pathological analysis demonstrated a high-grade osteosarcoma.

Lessons: Despite the lesion's rarity, suspicion for RIS should be raised with the rapid growth of a mass at the site of previous radiotherapy. Furthermore, it is especially important to carefully monitor vulnerable patients who may not be able to recognize or report the development of a growing mass. https://thejns.org/doi/10.3171/CASE24535.

Background: Medically refractory hypertonia (MRH) within the pediatric population causes severe disability and is difficult to treat. Neurosurgery for mixed MRH includes intrathecal baclofen (ITB) and lumbosacral ventral-dorsal rhizotomy (VDR). Surgical efficacy limitations can be mitigated by combining the two into a multimodal strategy. The authors examined outcomes following a multimodal neurosurgical strategy combining intraspinal ITB catheter revision to the cervical level and lumbosacral VDR.

Observations: Two patients with severe MRH resistant to ITB delivered through a thoracic catheter tip were identified: 1) a 16-year-old boy with quadriplegic mixed hypertonia and 2) a 17-year-old girl with secondary dystonia. The patient in case 1 experienced improvement in his Barry-Albright Dystonia Scale (BADS) score from 29 to 17 and lower-extremity modified Ashworth Scale score from 4 to 0 at 18 months postoperatively; the patient in case 2 experienced a decrease in her BADS score from 30 to 13 at 6 months postoperatively. Significant improvement in caregiving provisions, including patient positioning and transfers, was reported.

Lessons: The authors highlight favorable outcomes using multimodal surgery in pediatric patients. Multimodal therapy is surgically feasible and better addresses MRH, particularly in patients in whom ITB monotherapy and polypharmacy have failed. Future studies with larger patient volumes are necessary to optimize indications and make more definitive outcome conclusions. https://thejns.org/doi/10.3171/CASE24599.

Background: Carotid webs are rare nonatherosclerotic disorders in the carotid artery and are increasingly recognized as factors of ischemic stroke in the young population. Asymptomatic webs can be treated with antithrombotic therapy, whereas symptomatic cases frequently require surgical interventions, including carotid endarterectomy (CEA). However, guidelines for the optimal timing of these treatments remain unestablished, especially compared to atherosclerotic stenotic lesions, due to the rarity of carotid webs.

Observations: A 50-year-old female patient had a carotid web-induced left hemispheric ischemic stroke. Mechanical thrombectomy successfully restored blood flow, but a thrombus reformed in the carotid web within 1 month following the procedure. This caused early CEA, during which the carotid web and thrombus were removed. This case highlights rapid thrombus reformation, indicating that some carotid webs can be more unstable than previously thought.

Lessons: This case emphasizes the importance of timely surgical intervention in symptomatic carotid webs to prevent recurrent strokes. Additionally, antithrombotic therapy can manage asymptomatic webs, but surgical treatment may be required in unstable cases. Thus, further studies are warranted to establish standardized treatment guidelines for carotid webs. https://thejns.org/doi/10.3171/CASE24682.

Background: Intravascular injection of liquid adhesive hemostats is a rare but serious complication that can result in cerebral thromboembolism.

Observations: A 64-year-old female underwent orbitozygomatic craniotomy for posterior communicating artery aneurysm clipping with the routine use of a flowable hemostatic agent during extradural dissection. After placement of the aneurysm clip, flow was confirmed through the parent vessel and nearby branches. On postoperative day 1, the patient was found to have superficial middle cerebral vein thrombosis with retrograde embolization and subsequent hemorrhagic infarction of the temporal lobe. Eight prior cases of intracranial infarction or hemorrhage secondary to thrombosis with intravascular liquid adhesive were identified. With a craniotomy approach, the transverse and sigmoid sinuses were the most common sites of thrombosis, and the vertebral and basilar arteries had the highest incidence of thrombosis after cervical fusion.

Lessons: Intravascular injection of liquid adhesive agents can result in cerebral venous thrombosis, typically of a dural venous sinus. This may present following retrograde embolization, which has not previously been reported. The choice of hemostat, surgical approach, nearby vasculature, and anatomical variations should be carefully considered in the operative management of neurosurgical patients. https://thejns.org/doi/10.3171/CASE24607.