Enhancing Diagnostic Support for Chiari Malformation and Syringomyelia: A Comparative Study of Contextualized ChatGPT Models.

OBJECTIVES: The rapidly increasing adoption of large language models in medicine has drawn attention to potential applications within the field of neurosurgery. This study evaluates the effects of various contextualization methods on ChatGPT's ability to provide expert-consensus aligned recommendations on the diagnosis and management of Chiari Malformation and Syringomyelia. METHODS: Native GPT4 and GPT4 models contextualized using various strategies were asked questions revised from the 2022 Chiari and Syringomyelia Consortium International Consensus Document. ChatGPT-provided responses were then compared to consensus statements using reviewer assessments of 1) responding to the prompt, 2) agreement of ChatGPT response with consensus statements, 3) recommendation to consult with a medical professional, and 4) presence of supplementary information. Flesch-Kincaid, SMOG, word count, and Gunning-Fog readability scores were calculated for each model using the quanteda package in R. RESULTS: Relative to GPT4, all contextualized GPTs demonstrated increased agreement with consensus statements. PDF+Prompting and Prompting models provided the most elevated agreement scores of 19 of 24 and 23 of 24, respectively, versus 9 of 24 for GPT4 (p=.021, p=.001). A trend toward improved readability was observed when comparing contextualized models at large to ChatGPT4, with significant decreases in average word count (180.7 vs 382.3, p<.001) and Flesch-Kincaid Reading Ease score (11.7 vs 17.2, p=.033). CONCLUSIONS: The enhanced performance observed in response to ChatGPT4 contextualization suggests broader applications of large language models in neurosurgery than what the current literature indicates. This study provides proof of concept for the use of contextualized GPT models in neurosurgical contexts and showcases the easy accessibility of improved model performance.

A Quantitative Assessment of ChatGPT as a Neurosurgical Triaging Tool.

BACKGROUND AND OBJECTIVES: ChatGPT is a natural language processing chatbot with increasing applicability to the medical workflow. Although ChatGPT has been shown to be capable of passing the American Board of Neurological Surgery board examination, there has never been an evaluation of the chatbot in triaging and diagnosing novel neurosurgical scenarios without defined answer choices. In this study, we assess ChatGPT's capability to determine the emergent nature of neurosurgical scenarios and make diagnoses based on information one would find in a neurosurgical consult. METHODS: Thirty clinical scenarios were given to 3 attendings, 4 residents, 2 physician assistants, and 2 subinterns. Participants were asked to determine if the scenario constituted an urgent neurosurgical consultation and what the most likely diagnosis was. Attending responses provided a consensus to use as the answer key. Generative pretraining transformer (GPT) 3.5 and GPT 4 were given the same questions, and their responses were compared with the other participants. RESULTS: GPT 4 was 100% accurate in both diagnosis and triage of the scenarios. GPT 3.5 had an accuracy of 92.59%, slightly below that of a PGY1 (96.3%), an 88.24% sensitivity, 100% specificity, 100% positive predictive value, and 83.3% negative predicative value in triaging each situation. When making a diagnosis, GPT 3.5 had an accuracy of 92.59%, which was higher than the subinterns and similar to resident responders. CONCLUSION: GPT 4 is able to diagnose and triage neurosurgical scenarios at the level of a senior neurosurgical resident. There has been a clear improvement between GPT 3.5 and 4. It is likely that the recent updates in internet access and directing the functionality of ChatGPT will further improve its utility in neurosurgical triage.

Solitary Pediatric Osteochondroma of the Spine With Cord Compression.

Osteochondromas typically arise in the appendicular skeleton, with axial lesions occurring less commonly. Osteochondroma of the spine resulting in cord compression and symptomatic myelopathy is relatively rare. Most cases are reported in adolescents and adults. Consequently, there is a scarcity of literature regarding its occurrence in the pediatric population. We report the case of a cervical osteochondroma of C4-6 with cord compression in a nine-year-old girl. Surgical excision with laminectomy and laminotomy successfully resolved all neurologic deficits. A literature review revealed 27 cases of pediatric osteochondromas with cord compression, suggesting that these lesions are not as rare in the pediatric population as previously thought.

Comparison of surgical outcomes in patients with Chiari Type I malformation receiving posterior fossa decompression with and without duraplasty.

PURPOSE: The goals of this study were to compare clinical outcomes in patients with Chiari Malformation Type I (CMI) receiving posterior fossa decompression with (PFDD) or without duraplasty (PFD). METHODS: We conducted a retrospective analysis of 178 consecutive cases of 157 patients undergoing PFDD or PFD for CMI at Cohen Children's Medical Center between 2007 and 2017. Clinical improvement was defined as a clear reduction of preoperative symptoms after surgery as reported by patients and parents. An improvement of syrinx was derived from radiologic comparison of pre- and postoperative MR imaging. Chi-square analysis was performed to analyze the association between duraplasty and clinical parameters (alpha = 0.05). RESULTS: The primary presenting complaint was headache (142/178; 80%), followed by neck, back, and upper extremity pain, and numbness or tingling (49/178; 28%). Seventy patients (78/178; 44%) underwent PFD, and 100 patients (100/178; 56%) underwent PFDD. Overall, 143 patients (143/178; 80%) experienced subjective improvement with no statistically significant difference between the two surgical techniques (p = 0.705). The number of patients receiving PFDD with syrinx improvement or stabilization (55/59; 93%) was statistically larger than those that received PFD (8/13; 62%) (p = 0.008). PFDD was associated with greater complications than PFD alone. There were 35 cases of reoperation overall (35/178; 19%), and there was no statistically significant difference in reoperation rate between PFD and PFDD (p = 0.255). CONCLUSIONS: There appears to be a role for PFDD in patients with severe syringomyelia, but overall, PFD alone may be safely offered as the initial surgical intervention for symptomatic CMI patients.

Pedicled nasoseptal flap reconstruction for craniopharyngiomas in pediatric patients.

PURPOSE: Though the use of the pedicled nasoseptal flap (NSF), a reconstructive technique used after endoscopic endonasal approaches (EEA) for resection of craniopharyngiomas, has been shown to reduce the occurrence of post-operative cerebrospinal fluid (CSF) leaks in adults, less is known about its use in pediatric populations, specifically in children under the age of 7. The goal of this retrospective cohort study is to determine the viability of the pedicled NSF for pediatric patients. METHODS: Retrospective review of 12 pediatric patients (ages 2-16) undergoing 13 NSF reconstructions after resection of craniopharyngiomas. Radioanatomic analysis of computed tomography (CT) scans was utilized to classify the pneumatization of the sphenoid sinus depending on the thickness of the sphenoid bone margin. Intercarotid distances were measured from magnetic resonance imaging (MRI) scans to assess the feasibility of this reconstruction technique in pediatric patients. RESULTS: At the time of surgery, all patients were noted to have adequate NSF length and width. No post-operative high-flow CSF leaks were found within the group. Lack of pneumatization of the sphenoid sinus and narrow intercarotid distances in the youngest of patients did not lead to negative clinical outcomes. CONCLUSIONS: Based on our results and experience, the pedicled nasoseptal flap is a viable reconstructive option after EEA in the pediatric population, including even the youngest of patients. In these patients, a narrowed window between the intercarotid arteries and the lack of pneumatization of the sphenoid sinus present a challenge that can be overcome by using stereotactic navigation and advanced endoscopic techniques.

Robotic Surgical Assistant (ROSA™) Rehearsal: Using 3-Dimensional Printing Technology to Facilitate the Introduction of Stereotactic Robotic Neurosurgical Equipment.

BACKGROUND: The use of frameless stereotactic robotic technology has rapidly expanded since the Food and Drug Administration's approval of the Robotic Surgical Assistant (ROSA™) in 2012. Although the safety and accuracy of the ROSA platform has been well-established, the introduction of complex robotic technology into an existing surgical practice poses technical and logistical challenges particular to a given institution. OBJECTIVES: To better facilitate the integration of new surgical equipment into the armamentarium of a thriving pediatric neurosurgery practice by describing the use of a three-dimensional (3D)-printed patient model with in situ 3D-printed tumor for presurgical positioning and trajectory optimization in the stereotactic biopsy of a pontine lesion in a pediatric patient. METHODS: A 3D model was created with an added silicone mock tumor at the anatomical position of the lesion. In a preoperative rehearsal session, the patient model was pinned and registered using the ROSA platform, and a mock biopsy was performed targeting the in Situ silicone tumor. RESULTS: Utilization of the 3D-printed model enabled workflow optimization and increased staff familiarity with the logistics of the robotic technology. Biopsy trajectory successfully reached intralesional tissue on the 3D-printed model. The rehearsal maneuvers decreased operative and intubation time for the patient and improved operative staff familiarity with the robotic setup. CONCLUSION: Use of a 3D-printed patient model enhanced presurgical positioning and trajectory planning in the biopsy of a difficult to reach pontine lesion in a pediatric patient. The ROSA rehearsal decreased operative time and increased staff familiarity with a new complex surgical equipment.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients.

PURPOSE: The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. METHODS: Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. RESULTS: Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10% of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. CONCLUSIONS: Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.

Hydrocephalus associated with childhood nonaccidental head trauma.

OBJECTIVE The incidence of posttraumatic ventriculomegaly (PTV) and shunt-dependent hydrocephalus after nonaccidental head trauma (NAHT) is unknown. In the present study, the authors assessed the timing of PTV development, the relationship between PTV and decompressive craniectomy (DC), and whether PTV necessitated placement of a permanent shunt. Also, NAHT/PTV cases were categorized into a temporal profile of delay in admission and evaluated for association with outcomes at discharge. METHODS The authors retrospectively reviewed the cases of patients diagnosed with NAHT throughout a 10-year period. Cases in which sequential CT scans had been obtained (n = 28) were evaluated for Evans' index to determine the earliest time ventricular dilation was observed. Discharge outcomes were assessed using the King's Outcome Scale for Childhood Head Injury score. RESULTS Thirty-nine percent (11 of 28) of the patients developed PTV. A low admission Glasgow Coma Scale (GCS) score predicted early PTV presentation (within < 3 days) versus a high GCS score (> 1 week). A majority of PTV/NAHT patients presented with a subdural hematoma (both convexity and interhemispheric) and ischemic stroke, but subarachnoid hemorrhage was significantly associated with PTV/NAHT (p = 0.011). Of 6 patients undergoing a DC for intractable intracranial pressure, 4 (67%) developed PTV (p = 0.0366). These patients tended to present with lower GCS scores and develop ventriculomegaly early. Only 2 patients developed hydrocephalus requiring shunt placement. CONCLUSIONS PTV presents early after NAHT, particularly after a DC has been performed. However, the authors found that only a few PTV/NAHT patients developed shunt-dependent hydrocephalus.

HbSC Disease and Spontaneous Epidural Hematoma with Kernohan's Notch Phenomena.

Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease. A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. During his hospital stay he developed rapid deterioration of his mental status and computed topography revealed a spontaneous massive epidural hematoma with mass effect and midline shift with Kernohan's notch phenomena for which urgent craniotomy and evacuation was done. We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration. The etiopathogenesis and the rare presentation are discussed in detail in this case report.

Delay in Arrival to Care in Perpetrator-Identified Nonaccidental Head Trauma: Observations and Outcomes.

BACKGROUND: Children who sustained nonaccidental head trauma (NAHT) are at severe risk for mortality within the first 24 hours after presentation. OBJECTIVE: Extent of delay in seeking medical attention may be related to patient outcome. METHODS: A 10-year, single-institution, retrospective review of 48 cases treated at a large tertiary Children's Hospital reported to the New York State Central Registrar by the child protection team was conducted. The perpetrator was identified in 28 cases on the basis of confession or conviction. The medical and legal records allowed for identification of time of injury and the interval between injury and arrival to the hospital; this information was categorized as follows: <6 hours (without delay); 6-12 hours (moderate delay); and >12 hours (severe delay). The King's Outcome Scale for Childhood Head Injury (KOSCHI) score was recorded for each case. RESULTS: All children were 3 years of age or younger (2.1-34 months) and predominantly male (68%; 19/28). On arrival, 61% of patients (17/28) presented with moderate or severe delay. A low arrival Glasgow Coma Scale (GCS) score (P < 0.0001) and extracranial injuries (P < 0.0061) correlated with worse clinical patient outcomes. Patients with an arrival GCS score <7 predominantly arrived without delay or with moderate delay. Patients presenting without delay or with severe delay were more likely to have a higher KOSCHI outcome score on discharge (P < 0.0426). Four of the 6 patients who died presented after moderate delay. CONCLUSION: Patients presenting to medical care 6-12 hours after NAHT (moderate delay) appeared to have worse outcomes than those presenting earlier or later.

Choroid plexus papilloma and Pierpont syndrome.

The authors describe the case of a child who presented with hydrocephalus and phenotypic features characteristic of a multiple congenital anomalies/mental retardation syndrome. Dysmorphic facies, medial plantar lipomatosis, and developmental delay were observed in this case and are identical to documented findings of Pierpont syndrome diagnosed in 3 boys. This is the fourth case reported to date and is the first documented case of an oncological process- an intraventricular atypical choroid plexus papilloma tumor-found in association with Pierpont syndrome. Syndromes associated with choroid plexus papilloma are reviewed.

Iatrogenic spinal epidermoid cyst after lumbar puncture using needles with stylet.

Iatrogenic spinal epidermoid tumors are extremely rare and may be caused by implanted skin fragments in the spinal canal, most commonly due to lumbar punctures using hollow needles without a stylet. The authors report for the first time an iatrogenic spinal epidermoid tumor that developed 26 months after several lumbar punctures, performed using disposable spinal needles with stylet, in an 8-year-old boy with persistent lower back pain. The authors hypothesize that an ill-fitting stylet compounded by multiple therapeutic lumbar punctures and young age as possible risk factors for the development of the tumor in the present case. A total excision of the tumor with laminectomy yielded good symptomatic recovery.

Role of Rac1-regulated signaling in medulloblastoma invasion. Laboratory investigation.

OBJECT: Medulloblastomas are the most common malignant brain tumors in children. These tumors are highly invasive, and patients harboring these lesions are frequently diagnosed with distant spread. In this study, the authors investigated the role of Rac1, a member of the Rho family of small guanosine triphosphatases, in medulloblastoma invasion. METHODS: Three established medulloblastoma cell lines were used: DAOY, UW-228, and ONS-76. Specific depletion of Rac1 protein was accomplished by transient transfection of small interfering RNA. Cell invasion through extracellular matrix (Matrigel) was quantified using a transwell migration assay. Mitogen activated protein kinase activation was determined using phospho-MAP kinase-specific antibodies, and inhibition of MAP kinase pathways was achieved by specific small molecule inhibitors. Localization of Rac1 and its expression levels were determined by immunohistochemical analysis using a Rac1-specific antibody, and Rac1 activation was qualitatively assessed by Rac1 plasma membrane association. RESULTS: Small interfering RNA-mediated depletion of Rac1 strongly inhibited medulloblastoma cell invasion. Although depletion of Rac1 inhibited the proliferation of UW-228 cells, and of ONS-76 cells to a lesser extent, it stimulated the proliferation of DAOY cells. Depletion of Rac1 also inhibited the activation of the ERK and JNK MAP kinase pathways, and inhibition of either pathway diminished invasion and proliferation. Immunohistochemical analysis demonstrated that the Rac1 protein was overexpressed in all medulloblastoma tumors examined, and indicated that Rac1 was hyperactive in 6 of 25 tumors. CONCLUSIONS: The authors' data show that Rac1 is necessary for the invasive behavior of medulloblastoma cells in vitro, and plays a variable role in medulloblastoma cell proliferation. In addition, these results indicate that Rac1 stimulates medulloblastoma invasion by activating the ERK and JNK pathways. The authors suggest that Rac1 and signaling elements controlled by this guanosine triphosphatase may serve as novel targets for therapeutic intervention in malignant medulloblastomas.

Resting tremor secondary to a pineal cyst: case report and review of the literature.

BACKGROUND: Patients with pineal lesions typically present with the classical signs of increased intracranial pressure (headache, nausea, vomiting) and/or Parinaud's syndrome. Rare symptomatology of secondary parkinsonism attributed to pineal lesions has been previously reported in the literature. We describe an unusual case of a pineal cyst in a patient with the presenting sign of a resting tremor. CASE DESCRIPTION: We report an 18-year-old Caucasian female who presented with a 1-month history of a new-onset progressive, unilateral low-frequency right-hand resting tremor with associated headache, nausea, vomiting, and excessive diarrhea. Magnetic resonance imaging demonstrated an atypical appearance with enhancement of a mildly prominent pineal gland, possibly representing a pineal cyst. The patient did not exhibit radiographic signs of hydrocephalus. Based upon the radiographic appearance, one could not exclude with absolute certainty the presence of a malignancy. The patient ultimately underwent a bilateral suboccipital craniotomy with gross total resection of the lesion. Postoperatively, the patient exhibited immediate resolution of her preoperative resting tremor and continues to be symptom free at 1 year. CONCLUSIONS: A new-onset, resting tremor and/or other secondary parkinsonism symptoms should raise clinical suspicions of pineal lesions. Treatment can be guided based on tissue type and the presence or absence of hydrocephalus. We observed that complete surgical resection of the lesion provided the best treatment option for the total resolution of symptoms attributed to the disturbance of the microvasculature surrounding the nigro-striatal-pallidal system.

Jugular foramen fibromatosis in a 3-month-old male.

A 3-month-old male with a chief complaint of episodic choking with feeds and a hoarse cry is presented. Left eye ptosis and asymmetric soft palate elevation were detected on physical examination. Fiberoptic examination showed a left vocal fold paresis and pooling of secretions in the pyriform sinuses. MRI demonstrated an ill-defined lesion at the left jugular foramen extending into the left carotid sheath. A fine needle biopsy revealed spindle shaped cells consistent with fibromatosis. The histopathology of fibromatosis and the differential diagnosis of jugular foramen masses in children will be described. To our knowledge, this represents the earliest reported case of fibromatosis in the jugular foramen.

Circumferential cervical spine surgery in an 18-month-old female with traumatic disruption of the odontoid and C3 vertebrae. Case report and review of techniques. Case report and review of techniques.

STUDY DESIGN: A case study of an 18-month-old female with craniovertebral instability and spinal cord compression requiring circumferential stabilization. A review of surgical techniques in upper cervical spine and craniovertebral stabilization for young children is provided. OBJECTIVES: To describe an interesting surgical approach in a young pediatric patient requiring circumferential stability at the craniovertebral junction. BACKGROUND DATA: Craniovertebral instability is problematic in the young pediatric population due to the inability to secure hardware for stabilization. We present an interesting case of spinal cord compression with craniovertebral instability in an 18-month-old female requiring circumferential cervical spine and craniovertebral stabilization. METHODS: The patient presented with acute onset quadriparesis after a fall. Radiographs demonstrated C2-C3 disruption with canal compromise. Magnetic resonance imaging revealed signal changes of the spinal cord at C2-C3. Neurological examination revealed normal muscle volume with strength 1/5 in the upper extremities and 0/5 in the lower extremities. Respirations were normal with normal diaphragmatic function. Cranial nerves were intact. RESULTS: Halo-traction attempted at 0.453 kg induced occipital-atlantal dislocation. The patient underwent anterior corpectomy of C3 and the base of C2 with autologous rib grafts placed from C2 to C4 and macropore as an anterior plating system. Posteriorly the patient had occiput-C3 fusion with a titanium rod and autologous rib grafts bilaterally. Postoperatively the patient regained normal neurological function with circumferential fusion after 4 months in a halo vest. CONCLUSIONS: This case demonstrates the ability to achieve circumferential stabilization in the young pediatric patient. Injuries at the odontoid synchondrosis can be difficult to treat and are only complicated by having to achieve a posterior fusion at the craniovertebral junction. We present a successful case of circumferential fusion and offer a surgical technique to achieve spinal cord decompression and fusion of the upper cervical spine and craniovertebral junction in the young pediatric population.