•Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder.•Data on management of multiple cervical neurofibromas are scarce in the literature.•Surgical debulking or resection is the ...main treatment strategy in patients with multiple spinal neurofibromas.•Radiation therapy has a rule in the management of multiple spinal neurofibromas.
Neurofibromatosis type 1 (NF1) is a common disorder in which affected individuals uncommonly develop cervical neurofibromas. The presentation of cervical neurofibroma with myelopathy is clinically challenging. Available data of NF1 patients with cervical cord compression secondary to multiple neurofibromas remain scarce in the literature. To this end, we sought to address this limitation.
Case presentation: We report a case of a 22-year-old man, recently diagnosed with NF1, who presented with progressive cervical myelopathy over the course of 12 months. Imaging revealed multiple cervical neurofibromas with significant spinal cord compression. The patient underwent a C3 to C7 decompressive laminectomy and subtotal resection of the bilateral neurofibromas and instrumented fusion. During the postoperative period, he experienced transient bilateral weakness in C5 and C6 muscle groups that gradually resolved, and his weakness and spasticity significantly improved thereafter.
Systematic review: We performed a systematic review of PubMed and Scopus in English-language literature dated between 1960 and December 2019 for studies that included cervical neurofibromas presenting with myelopathy in patients with NF1.
Fifty-seven articles were identified for full-text examination, of which 19 articles were included in the systematic review; 10 involved studies on surgical treatment, and nine on other treatment modalities. Twelve studies were retrospective, 3 involved prospective cohorts, and 4 were case reports. Most studies included various types of spinal cord tumors with or without neurofibromatosis. Only two studies exclusively involved neurofibromas in NF patients. There was wide variation in surgical and radiation therapy techniques and outcome measures reported.
Surgical decompression is the primary treatment strategy for multiple cervical neurofibromas that cause a progressive neurological deficit. Fusion is recommended to avoid late kyphotic deformity. Data describing the management plan and long-term outcomes in this group of patients remain scarce in the literature, and no standardized treatment strategy is available.
•Diagnosing brown tumors requires high index of clinical suspicion plus markers of HPT and neuroimaging characteristics.•Parathyroidectomy is the first management step in addition to biopsy of the ...bony lesion.•Surgery is reserved for cases with significant neural compression or cases not regressing following parathyroidectomy.
Brown tumors are benign, lytic, expansile bony lesions that represent a delayed manifestation of untreated hyperparathyroidism (HPT). Although they can affect any part of the skeleton, brown tumors of the calvaria or skull base are extremely rare. Accurate diagnosis is challenging, particularly in patients without other signs and symptoms of HPT. We report a rare case of brown tumors in a patient who presented with more than 4 sizable lesions that showed aggressive behavior and contributed to the patient’s near-total blindness. To our knowledge, this is only the fourth case of brown tumors of the skull base and calvaria reported in the literature.
A 28-year-old pregnant woman presented with a 2-month history of gradual right eye proptosis and decreased vision, a 1-day history of acute left eye visual loss, and epistaxis. Brain magnetic resonance imaging revealed multiple lesions, the largest measuring (4 × 5.3 × 5 cm) at the skull base, which occupied the nasopharynx and involved the clivus and sphenoid sinus. Another large lesion was detected at the right sphenoid bone with invasion of the right orbital wall. A nasopharyngeal biopsy showed multiple giant cells, and investigations revealed primary HPT. The patient had parathyroid adenoma resection, followed by right decompressive craniotomy to salvage vision in her right eye. She recovered well with improved right eye movements and vision.
Diagnosis of brown tumors of the skull base requires a high index of clinical suspicion, and it should be included in the differential diagnosis in patients who present with any lytic bony lesion, even in cases of multiple lesions. Management includes accurate diagnosis and prompt treatment of the etiopathogenetic factors associated with HPT and biopsy of the bony lesions to rule out other likely conditions. However, this should not preclude decompressive surgery for neural compression-induced acute deficits.
High-grade gliomas are central nervous system tumors conventionally treated with surgery followed by adjuvant chemoradiotherapy. Secondary cancer due to radiation therapy is a rare yet established ...phenomenon that typically occurs years after radiation therapy.
In this case, we discuss an early presentation of a second cancer adjacent to the radiation field. This case report is of a 52-year-old male who developed a new scalp sarcoma at the site of primary surgery 8 months after radiation therapy. Genetic testing revealed a heterozygous missense variant in the
gene, a variant of uncertain significance. The report highlights that this case does not conform to the expected criteria for postradiation sarcoma in terms of timing.
Secondary cancers may arise earlier than expected, even in phenotypically normal patients, as they may have unmanifested variants of relevant mutations. The question of pre-radiotherapy screening for radiosensitivity syndromes and diseases requires further study, as current data are limited and do not provide enough insight into the significance of different genetic variants.
•Scalp AVMs are rare pathologies that account for 8% of all cranial AVMs.•Pulsatile mass constitutes the most common presenting symptom of sAVM.•Cerebral Angiogram is crucial in assessing sAVM and is ...an essential planning tool.•A combined endovascular and surgical approach is highly encouraged for sAVM.
Arteriovenous malformation of the scalp (sAVM) is a rare pathology that accounts for 8% of all cranial arteriovenous malformations. It is believed to be congenital; however, few reported were secondary to trauma. They are managed either via surgical resection, endovascular embolization, or combined approach. Cases with bilateral dual extracranial feeders are yet significantly scarce incidents among this rare pathology. We report a case of sAVM with dual bilateral feeders and review the literature.
We report a case of a 24-year-old male who presented with a spontaneous para-midline enlarging mass over five years associated with headache. Imaging, including angiography, showed a subcutaneous vascular lesion measuring 2.4x4.2 cm supplied by bilateral superficial temporal arteries and occipital arteries. The patient underwent endovascular subtotal embolization with subsequent surgical excision with excellent recovery.
The rare occurrence of sAVMs requires a high index of suspicion and angiographic images to assess blood supply for preoperative embolization. A combined management approach is associated with good outcome and decrease the risk of recurrence.
The reconstruction technique and materials used for endoscopic skull base surgery (ESBS) are important factors in preventing cerebrospinal fluid (CSF) leak, a notable complication following this ...surgery. Visualizing the status of reconstruction early in the postoperative course can help determine the risk of postoperative CSF leak. Here, we aimed to determine if the radiological status of reconstruction post endonasal endoscopic surgery can predict postoperative CSF leak.
This retrospective study included patients who had undergone ESBS between 2015 and 2020. An early computed tomography (CT) scan (obtained within 24 hours of surgery) was utilized to evaluate the reconstruction and postoperative radiological changes, and its findings were correlated with the occurrence of postoperative CSF leaks.
Our study included 11 (12.7%) out of 86 patients with CSF leaks. The type of reconstruction, construction material, and type of nasal packing were not identified as significant risk factors for CSF leaks. The location of the fat graft (placed properly vs. displaced out of the surgical cavity) was significantly associated with CSF leak (P = 0.001). All patients with a displaced solid reconstruction (n = 5), displaced septal flap (n = 6), signs of air continuation (n = 2), or significantly increased amount of air (n = 5) presented with a CSF leak (P < 0.001).
Early postoperative CT scan is predictive of CSF leak. Displacement of the fat graft in early postoperative CT was the most important factor in predicting CSF leak. In this patient group, paying attention to radiological predictors of CSF leaks is important, supported by clinical findings.
Abstract
OBJECTIVE
This observational cross-sectional multicenter study aimed to evaluate the longitudinal impact of the coronavirus disease 2019 (COVID-19) pandemic on neurosurgical practice.
...METHODS
We included 29 participating neurosurgeons in centers from all geographical regions in the Kingdom of Saudi Arabia. The study period, which was between March 5, 2020 and May 20, 2020, was divided into three equal periods to determine the longitudinal effect of COVID-19 measures on neurosurgical practice over time.
RESULTS
During the 11-week study period, 474 neurosurgical interventions were performed. The median number of neurosurgical procedures per day was 5.5 (interquartile range IQR: 3.5–8). The number of cases declined from 72 in the first week and plateaued at the 30’s range in subsequent weeks. The most and least number of performed procedures were oncology (129 27.2%) and functional procedures (6 1.3%), respectively. Emergency (Priority 1) cases were more frequent than non-urgent (Priority 4) cases (178 37.6% vs. 74 15.6%, respectively). In our series, there were three positive COVID-19 cases. There was a significant among-period difference in the length of hospital stay, which dropped from a median stay of 7 days (IQR: 4 – 18) to 6 (IQR: 3 - 13) to 5 days (IQR: 2 - 8). There was no significant among-period difference with respect to institution type, complications, or mortality.
CONCLUSION
Our study demonstrated that the COVID-19 pandemic decreased the number of procedures performed in neurosurgery practice. The load of emergency neurosurgery procedures did not change throughout the three periods, which reflects the need to designate ample resources to cover emergencies. Notably, with strict screening for COVID -19 infections, neurosurgical procedures could be safely performed during the early pandemic phase. We recommend to restart performing neurosurgical procedures once the pandemic gets stabilized to avoid possible post-pandemic health-care system intolerable overload.
The accelerated growth of endoscopic endonasal skull base surgery has led to an abundance of highly cited works that have helped shape the field into its current state. Highlighting these works can ...serve as a guide for trainees and facilitate evidence-based clinical decision making and operative techniques.
Elsevier's Scopus database was used to generate a list of the 100 most cited articles on endoscopic endonasal skull base surgery in September 2022. Results were categorized based on pathology, approach, study design, and study objective. A citation per year analysis was conducted to highlight later publications that may not have had the time to accumulate as many citations as older publications.
The average number of citations for the 100 most cited articles was 210 per article (range 104–1285). Publication dates ranged from 1997 to 2019, with the highest number of articles among the 100 most cited being published in 2008 (15 articles). The journal with the greatest number of publications was Neurosurgery (30 articles). Of the 100 most cited articles, 77 were published between 2000 and 2010. Moreover, 40 of the 100 articles reported surgical outcomes and related complications, and the most common pathology was pituitary adenoma (25 articles).
This article provides a list of highly influential articles in the field of endoscopic endonasal skull base surgery, highlighting its rapid evolution over the last 3 decades and demonstrating its leap from small descriptive series confined to certain pathologies to larger cohorts exploring possible boundaries and other pathologies.
Cerebrospinal fluid (CSF) leak remains the primary concern of endoscopic skull base surgery (ESBS). Pneumocephalus seen in postoperative images has been linked to CSF leak in some reports; however, ...few studies have looked at the extent to which it is indicative of CSF leak. In the current study, we aim to examine the size and location of post-ESBS pneumocephalus in the early postoperative period and determine their association with postoperative CSF leak.
Patients undergoing ESBS in a 5-year period were included. All patients underwent brain computed tomography scan within the first 24 postoperative hours. Computed tomography scans were reviewed by a neurosurgeon and a radiologist and have been classified based on the size and location of pneumocephalus. Patients were followed in the postoperative period for clinical signs of CSF leak and managed accordingly.
Out of 120 patients, 86 patients met the inclusion criteria. Thirty-five patients (41%) had no pneumocephalus on day one postoperative imaging, while 51 patients (59%) had pneumocephalus with different sizes and distributions. Eleven of 86 patients developed CSF leak. Of the 11 patients, 5 patients (45%) had grade 4 pneumocephalus (P value = 0.02). Patients with multiple locations of pneumocephalus were more likely to develop CSF leak (P value = 0.01).
In post-ESBS patients, both the volume and location of the pneumocephalus are potentially predictive of CSF leak. In patients with a larger volume of intra-axial air and/or multiple air locations, an impending CSF leak should be anticipated.
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