Following the PRISMA Extension for scoping reviews, we meticulously searched MEDLINE and EMBASE for all peer-reviewed articles relevant to 'Blue rubber bleb nevus syndrome' dating from the inception of those databases through December 28, 2021.
A collection of ninety-nine articles was compiled, encompassing three observational studies and a substantial 101 cases culled from case reports and series. The efficacy of sirolimus in BRBNS was evaluated in only one prospective study, while multiple observational studies suffered from limited sample sizes. A frequent observation in clinical presentations was anemia (50.5%) and melena (26.5%). While skin findings served as a clue for BRBNS, only 574 percent presented vascular malformation. A clinical basis overwhelmingly formed the diagnostic process, genetic sequencing revealing BRBNS in a mere 1% of the cases. In the context of BRBNS, vascular malformations presented a significant variation in anatomical distribution, with the oral cavity exhibiting the highest prevalence (559%), followed by the small intestine (495%), colon and rectum (356%), and the stomach (267%).
Despite its underappreciated role, adult BRBNS could be the underlying cause of the treatment-resistant condition of microcytic anemia or concealed gastrointestinal bleeding. Subsequent research efforts are crucial for establishing a unified comprehension of diagnosis and treatment in adults affected by BRBNS. The utility of genetic testing in diagnosing adult BRBNS and the patient characteristics likely to respond to sirolimus, a potential curative agent, are subjects that require further exploration.
In cases of underrecognition, adult BRBNS may be a cause of refractory microcytic anemia or covert gastrointestinal bleeding. In order to develop a unified understanding of diagnosis and treatment approaches for adult BRBNS, further research is critical. The elucidation of genetic testing's utility in adult BRBNS diagnosis, along with the identification of patient attributes primed to respond positively to sirolimus, a potentially curative agent, still needs to be accomplished.
Awake surgery, a method for treating gliomas, has been globally embraced and accepted as a standard neurosurgical practice. Nevertheless, its primary use centers on restoring speech and basic motor functions; intraoperative applications for recovering more complex brain functions are, however, still under development. The upkeep of these functions is critical to enabling postoperative patients to regain their normal social routines. This review article examines the preservation of spatial attention and higher-order motor functions, exploring their neural correlates and the practical application of awake surgical procedures facilitated by purposeful tasks. For assessing spatial attention, the line bisection task stands as a standard, yet complementary methods like exploratory tasks are valuable, dictated by the specific location of the relevant brain structures. Two tasks were developed to promote higher-order motor functioning: 1) the PEG & COIN task, which assesses the skill of grasping and approaching, and 2) the sponge-control task, which determines movement based on somatosensory awareness. Although scientific knowledge and evidence remain confined in this neurosurgical field, we predict that expanding our research on higher brain functions and developing precise and efficient intraoperative assessments will ultimately conserve the quality of life for patients.
Awake surgery enables a more precise evaluation of language function and other neurological functions difficult to assess using conventional electrophysiological examinations. The success of awake surgery hinges on a coordinated effort by anesthesiologists and rehabilitation physicians, assessing both motor and language functions, and on the seamless sharing of information during the perioperative phase. The methodologies of surgical preparation and anesthesia carry certain unique aspects which necessitate a comprehensive grasp. To maintain a secure airway, supraglottic airway devices are indispensable, and the ventilation's availability must be confirmed upon positioning the patient. For optimal intraoperative neurological evaluation, the preoperative neurological assessment is indispensable, encompassing the decision of the simplest evaluation method and its disclosure to the patient before the surgery. A motor function assessment meticulously examines minute movements, with no bearing on the surgical act. A thorough evaluation of language function typically incorporates the analysis of visual naming and auditory comprehension.
For hemifacial spasm (HFS) patients undergoing microvascular decompression (MVD), brainstem auditory evoked potentials (BAEPs) and abnormal muscle responses (AMRs) monitoring is a standard procedure. Intraoperative wave V findings in BAEP monitoring do not always reliably correlate with postoperative hearing outcomes. Conversely, if an alarm signal, as clear and imperative as a modification in wave V, is detected, the surgeon must either end the surgical procedure or inject artificial cerebrospinal fluid into the eighth cranial nerve. Auditory function maintenance during MVD of HFS mandates the execution of BAEP monitoring. AMR monitoring proves valuable in identifying the offending vessels causing pressure on the facial nerve and confirming the decompression procedure's completion during the operation. Real-time adjustments to AMR's onset latency and amplitude are sometimes made during the operation of the implicated vessels. reactive oxygen intermediates These findings provide surgeons with the means to discover the offending vessels. Post-decompression, any lingering AMRs exhibiting a 50% or greater amplitude decrease from baseline levels are indicators of postoperative HFS loss in the long term. Following dural exposure, should AMRs vanish, ongoing AMR monitoring is essential as the reoccurrence of AMRs is frequently noted.
To effectively locate the focus area in patients with MRI-positive lesions, intraoperative electrocorticography (ECoG) proves to be an important monitoring procedure. Previous investigations have underscored the practical application of intraoperative electrocorticography (ECoG), especially for children diagnosed with focal cortical dysplasia. A detailed intraoperative ECoG monitoring methodology, used during the resection of a focal cortical dysplasia in a 2-year-old boy, will be presented, resulting in a seizure-free outcome. UCLTRO1938 The intraoperative ECoG, despite its clinical significance, has several associated problems. These involve the possibility of the surgical focus being determined by interictal spikes instead of the seizure onset zone, and the pervasive impact of the anesthetic conditions. Subsequently, we must be mindful of its boundaries. The significance of interictal high-frequency oscillations as a biomarker in epilepsy surgery has been increasingly acknowledged. Intraoperative ECoG monitoring advancements are indispensable for the near future.
Surgical procedures on the spine or spinal cord present a risk of damaging the nerve roots and the spinal column, possibly triggering profound neurological impairments. Surgical positioning, mechanical compression, and tumor resection are just a few instances where intraoperative monitoring is essential for ensuring the integrity of nerve function. This system's capacity for early detection of neuronal injury allows surgeons to avoid postoperative complications. Considering compatibility with the disease, surgical procedure, and lesion localization is crucial for choosing the right monitoring systems. The significance of monitoring and the timing of stimulation are crucial for the team to conduct a safe surgery. Cases from our hospital showcase the different intraoperative monitoring methods and potential problems encountered in spine and spinal cord surgeries, presented in this paper.
Preventing complications from disturbed blood flow in cerebrovascular disease is paramount in both surgical and endovascular treatments, thus requiring intraoperative monitoring. In revascularization surgeries, such as bypass procedures, carotid endarterectomies, and aneurysm clipping, monitoring is a standard practice. Blood flow in both the intracranial and extracranial areas is targeted for normalization by revascularization, but this process necessarily requires interrupting cerebral blood flow, even temporarily. Generalizing the effects of impeded cerebral blood flow on circulation and function is not possible due to the mediating role of collateral circulation and the diversity of patient presentations. Monitoring is indispensable for comprehending the dynamic shifts during the operative procedure. medicinal and edible plants It serves a critical role in revascularization procedures, verifying the sufficiency of re-established cerebral blood flow. Neurological dysfunction is revealed through changes in monitoring waveforms, but in some cases, clipping procedures may fail to display waveforms, thereby leading to the persistence of neurological impairment. Despite these challenges, this approach can successfully identify the surgical procedure that triggered the problem, thereby improving the success rate of future surgical procedures.
Sufficient tumor removal and preservation of neural function during vestibular schwannoma surgery are ensured by intraoperative neuromonitoring, which is indispensable for securing long-term outcomes. Quantitative and real-time assessment of facial nerve function is facilitated by repetitive direct stimulation during intraoperative continuous facial nerve monitoring. The hearing function of the ABR and, subsequently, CNAP, is continuously assessed via close monitoring. Electromyographic readings of masseter and extraocular muscles, along with SEP, MEP, and neuromonitoring of lower cranial nerves, are employed as necessary. Our neuromonitoring techniques for vestibular schwannoma surgery, along with an illustrative video, are presented in this article.
Invasive brain tumors, particularly gliomas, commonly sprout in the eloquent brain regions associated with language and motor activities. Ensuring the safety of the procedure while maximizing tumor removal and preserving neurological function is the primary objective in brain tumor resection.