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Background Awake craniotomy with direct cortical stimulation and mapping is the gold standard for resection of lesions near eloquent brain areas, as it can maximize the extent of resection while minimizing the risk of neurological damage. In contrast to the adult population, only small series of awake craniotomies have been reported in children. Aims The aim of our study is to establish the feasibility of awake craniotomy in the pediatric population. Methods We performed a retrospective observational study of children undergoing a supratentorial awake craniotomy between January 2009 and April 2019 in a pediatric tertiary care center. Our primary outcome was feasibility of awake craniotomy, defined as the ability to complete the procedure without conversion to general anesthesia. Our secondary outcomes were the incidence of serious intraoperative complications and the mapping completion rate. Results Thirty procedures were performed in 28 children: 12 females and 16 males. The median age was 14 years (range 7-17). The primary diagnosis was tumor (83.3%), epilepsy (13.3%), and arterio-venous malformation (3.3%). The anesthetic techniques were asleep-awake-asleep (96.7%) and conscious sedation (3.3%), all cases supplemented with scalp block and pin-site infiltration. Awake craniotomy was feasible in 29 cases (96.7%), one patient converted to general anesthesia due to agitation. Serious complications occurred in six patients: agitation (6.7%), seizures (3.3%), increased intracranial pressure (3.3%), respiratory depression (3.3%), and bradycardia (3.3%). All complications were quickly resolved and without major consequences. Cortical mapping was completed in 96.6% cases. New neurological deficits occurred in six patients (20%)-moderate in one case and mild in 5-being all absent at 6 months of follow-up. Conclusion Awake craniotomy with intraoperative mapping can be successfully performed in children. Adequate patient selection and close cooperation between neurosurgeons, anesthesiologists, neuropsychologists, and neurophysiologists is paramount. Further studies are needed to determine the best anesthetic technique in this population group. more...

The indications for operating on lesions in or near areas of cortical eloquence balance the benefit of resection with the risk of permanent neurological deficit. In adults, awake craniotomy has become a versatile tool in tumor, epilepsy and functional neurosurgery, permitting intra-operative stimulation mapping particularly for language, sensory and motor cortical pathways. This allows for maximal tumor resection with considerable reduction in the risk of post-operative speech and motor deficits. We report our experience of awake craniotomy and cortical stimulation for epilepsy and supratentorial tumors located in and around eloquent areas in a pediatric population (n=10, five females). The presenting symptom was mainly seizures and all children had normal neurological examinations. Neuroimaging showed lesions in the left opercular (n=4) and precentral or peri-sylvian regions (n=6). Three right-sided and seven left-sided awake craniotomies were performed. Two patients had a history of prior craniotomy. All patients had intra-operative mapping for either speech or motor or both using cortical stimulation. The surgical goal for tumor patients was gross total resection, while for all epilepsy procedures, focal cortical resections were completed without any difficulty. None of the patients had permanent post-operative neurologic deficits. The patient with an epileptic focus over the speech area in the left frontal lobe had a mild word finding difficulty post-operatively but this improved progressively. Follow-up ranged from 6 to 27 months. Pediatric awake craniotomy with intra-operative mapping is a precise, safe and reliable method allowing for resection of lesions in eloquent areas. Further validations on larger number of patients will be needed to verify the utility of this technique in the pediatric population. more...

A series of life-threatening nonanesthetic-related MH reactions in a child was the inspiration for a proactive, novel solution allowing for early prehospital, potentially lifesaving intravenous dantrolene administration. Multidisciplinary collaboration is essential and parent education must be comprehensive and ongoing. This case underlines the importance of considering nonanesthetic MH susceptibility in the child who has a history of unspecified myopathy and who presents with fever and total body stiffness. more...

Myogenic and neural control of intestinal transit were investigated in a model of distension-induced peristalsis. A comparison was made between the electrical and mechanical activities and outflow of contents observed in control mice and in W/W v mice, which lack the interstitial cells of Cajal associated with Auerbach's plexus. Distension caused a periodic appearance of increased motor activity due to stimulation of enteric nerves in both control and W/W v mice. Excitation was primarily delivered by cholinergic nerves, whereas periodic inhibition was mediated by neuronal nitric oxide. In control mice, outflow was driven by propagating slow-wave activity and was only in the aboral direction. Outflow only occurred when slow waves carried sufficient action potentials to cause phasic intraluminal pressure increases of ≥ 1 cmH2O through direct stimulation of the musculature or by distension-induced neurally mediated activation. In W/W v mice, outflow was associated with propagating action potentials that occurred due to either neural stimulation or direct muscle stimulation. Action potential propagation and outflow occurred in both oral and aboral directions. In summary, in both control and W/W v mice, distension induced periodic motor activity through stimulation of the enteric nervous system. Intraluminal contents were not moved in front of such motor activity. Rather, within such periods of activity that occurred concurrently throughout an entire segment, pulsatile outflow was directed by individual propagating slow waves with superimposed action potentials in control tissue, and by propagating action potentials in W/W v mice, which lack interstitial cells of Cajal. more...

The role of interstitial cells of Cajal associated with Auerbach's plexus (ICC-AP) in the pathophysiology of inflammation-induced abnormalities in gut motor activity is poorly understood. Therefore we applied a well-described model of inflammation (infection by Trichinella spiralis) to the mouse small intestine where the structure and function of ICC-AP are best known. Electron microscopic evaluation revealed that 1 to 3 days after infection, selective and patchy damage to the ICC processes occurred, thereby disrupting contacts between these ICC and smooth muscle cells as well as ICC and nerves, which was associated with disordered electrical activity and abnormal peristalsis. Ten to 15 days after infection, damage to ICC-AP was maximal and now involving the cell body and major processes. Marked synthetic activity and regrowth of their processes occurred from day 3 onward and recovery was completed at day 40 after infection. No changes to the network of ICC-AP were seen with c-Kit immunohistochemistry. From day 1 after infection, macrophages infiltrated the AP area, making close contact including peg-and-socket-like junctions with smooth muscle cells and ICC-AP but up to day 6 after infection without any sign of phagocytosis. By day 6 after infection, lymphocytes entered the musculature forming close contacts with ICC-AP. This was not associated with damage to ICC-AP but with proliferation of rough endoplasmic reticulum. From day 23 onward, immune cells withdrew from the musculature except macrophages, resulting in a markedly increased population of macrophages in the AP area at day 60 after infection. more...

The relationship between the development of the enteric nervous system and interstitial cells of Cajal (ICC) in the human small intestine was investigated in a full-term infant who presented with intestinal pseudo-obstruction. Immunohistochemistry revealed absence of enteric nerves and ganglia but abundant c-Kit immunoreactivity associated with Auerbach's plexus (ICC-AP). However, c-Kit immunoreactivity associated with the deep muscular plexus (ICC-DMP) and intermuscular ICC was absent. Electron microscopy showed ICC-AP with a normal ultrastructure; ICC-DMP were seen but were severely injured, suggesting degeneration. In vitro recording of intestinal muscle showed slow wave activity as well as response to cholinergic stimulation. Fluoroscopic examination of the small bowel showed a variety of motor patterns, including rhythmic, propagating contractions. In conclusion, total absence of enteric nerves was associated with absence of normal ICC-DMP. However, a normal musculature, including a network of ICC-AP, allowed for generation of rhythmic, propagating contractile activity, suggesting the presence of functional motor activity. more...

Background & Aims: Interstitial cells of Cajal (ICC) play an important role in the control of gastrointestinal motility. We aimed to determine a potential role for ICC in the pathophysiology of inflammation-induced motor disorders. Methods: Effects of Trichinella spiralis infection on electrical pacemaker activity, the structure of ICC associated with Auerbach's plexus, and in vivo motor patterns were studied in the mouse small intestine. Results: Between days 1 and 15 after infection, structural damage occurred in the network of ICC, particularly in the processes connecting ICC to each other and to smooth muscle cells. This was associated with desynchronization of electrical pacemaker activity. Abnormal slow wave activity occurred, including doubling of frequency and electrical quiescence, leading to the development of ectopic pacemaker activity in vivo. In vivo motor patterns in the small intestine changed from consistent peristaltic contractile activity in control animals to periods of quiescence alternating with both orally and aborally propagating contractile activity in the presence of inflammation. Sixty days after infection, all parameters studied had returned to normal values. Conclusions: Inflammation-induced alterations in the network of ICC of the small intestine associated with Auerbach's plexus lead to disorganization of motor patterns. Because of the strong temporal correlation between damage to the ICC network, electrical uncoupling, the appearance of ectopic pacemaker activity, and the occurrence of retrograde peristalsis, it is concluded that ICC can play a major role in inflammation-induced motor disturbances. GASTROENTEROLOGY 2000;119:1590-1599 more...

Background & Aims: Interstitial cells of Cajal (ICC) have been linked to the generation of intestinal pacemaker activity, but their role in in vivo motor dysfunction is unclear. In this study, we investigated the hypothesis that ICC play a role in the generation of distention-induced peristalsis using W/W v mice that lack ICC associated with Auerbach's plexus. Methods: Radiological observations were made of the movement of contrast fluid through the proximal small intestine. Electrical activities were recorded in the external muscle layers. In addition, intraluminal pressure changes were recorded in isolated intestinal segments. Results: In control mice, after gavage of 0.5 mL of barium sulfate in the stomach, the contrast fluid moved through the proximal small intestine in peristaltic waves at ~47 times a minute, propagating aborally at ~2 cm/s. Electrical slow waves and intraluminal pressure waves were synchronized at similar frequencies and propagation velocities. In W/W v mice, such regular peristaltic waves were not observed. Action potentials and contractions appeared random, and contents moved back and forth in an irregular manner. The net propulsive effect of contractile activity in W/W v mutant mice was much weaker than that in controls. Conclusions: Slow wave controlled peristalsis occurs in the normal proximal small intestine upon gastric emptying of a semiliquid. This motor pattern is absent in W/W v mice that lack ICC. GASTROENTEROLOGY 1998;114:724-736 more...