The functional disturbances driving “out-of-proportion” dyspnoea in patients with fibrosing interstitial lung disease (f-ILD) showing only mild restrictive abnormalities remain poorly understood. ...Eighteen patients (10 with idiopathic pulmonary fibrosis) showing preserved spirometry and mildly reduced total lung capacity (≥70% predicted) and 18 controls underwent an incremental cardiopulmonary exercise test with measurements of operating lung volumes and Borg dyspnoea scores. Patients’ lower exercise tolerance was associated with higher ventilation (V̇E)/carbon dioxide (V̇CO2) compared with controls (V̇E/V̇CO2 nadir=35 ± 3 versus 29 ± 2; p < 0.001). Patients showed higher tidal volume/inspiratory capacity and lower inspiratory reserve volume at a given exercise intensity, reporting higher dyspnoea scores as a function of both work rate and V̇E. Steeper dyspnoea-work rate slopes were associated with lower lung diffusing capacity, higher V̇E/V̇CO2, and lower peak O2 uptake (p < 0.05). Heightened ventilatory demands in the setting of progressively lower capacity for tidal volume expansion on exertion largely explain higher-than-expected dyspnoea in f-ILD patients with largely preserved dynamic and “static” lung volumes at rest.
•The physiological abnormalities driving “out-of-proportion” dyspnea in mild f-ILD remain unclear.•Despite normal spirometry and total lung capacity typically above 70% predicted, patients showed moderate impairment in DLCO•Exertional dyspnea was related to heightened ventilatory demands and progressively lower capacity for tidal volume expansion•Exertional dyspnea and exercise intolerance were associated with lower DLCO, excess ventilation, and inspiratory constraints•These findings can causally link mild f-ILD to “out-of-proportion” dyspnoea, exposing potential therapeutic targets
Aneurysmal subarachnoid hemorrhage (SAH) is rare in neonates. The authors present a unique report of a neonate with SAH from anterior inferior cerebellar artery (AICA) aneurysm rupture that was ...successfully treated with Onyx embolization. This case report demonstrates the utility of Onyx embolization for posterior circulation aneurysms in neonates and the successful management of SAH in this population.
Teratomas account for 18-20% of all intracranial germ cell tumors and mostly occur in the pineal region with only a few cases of pediatric sellar and suprasellar teratomas described in the ...literature. Here, we present a case of a child with an intracranial mature teratoma with pancreatic features causing vasospasm and subsequent stroke, found to be positive for CDKN2A-an independent variant associated with malignancy and small vessel disease leading to stroke.
Following pulmonary embolism (PE), a third of patients develop persistent dyspnea, which is commonly termed the post-PE syndrome. The neurophysiological underpinnings of exertional dyspnea in ...patients with post-PE syndrome without pulmonary hypertension (PH) are unclear. Thus, the current study determined if abnormally high inspiratory neural drive (IND) due, in part, to residual pulmonary gas-exchange abnormalities, was linked to heightened exertional dyspnea and exercise limitation, in such patients. Fourteen participants with post-PE syndrome (without resting PH) and 14 age-, sex-, and body mass index-matched healthy controls undertook pulmonary function testing and a symptom-limited cycle cardiopulmonary exercise test with measurements of IND (diaphragmatic electromyography), ventilatory requirements for CO
(V̇e/V̇co
), and perceived dyspnea intensity (modified Borg 0-10 scale). Post-PE (vs. control) had a reduced resting transfer coefficient for carbon monoxide (
: 84 ± 15 vs. 104 ± 14%pred,
< 0.001) and peak oxygen uptake (V̇o
) (76 ± 14 vs. 124 ± 28%pred,
< 0.001). IND and V̇e/V̇co
were higher in post-PE than controls at standardized submaximal work rates (
< 0.05). Dyspnea increased similarly in both groups as a function of increasing IND but was higher in post-PE at standardized submaximal work rates (
< 0.05). High IND was associated with low
(
= -0.484,
< 0.001), high V̇e/V̇co
nadir (
= 0.453,
< 0.001), and low V̇o
(
= -0.523,
< 0.001). In patients with post-PE syndrome, exercise IND was higher than controls and was associated with greater dyspnea intensity. The heightened IND and dyspnea in post-PE, in turn, were strongly associated with low resting
and high exercise V̇e/V̇co
, which suggest important pulmonary gas-exchange abnormalities in this patient population.
This study is the first to show that increased exertional dyspnea in patients with post-pulmonary embolism (PE) syndrome, without overt pulmonary hypertension, was strongly associated with elevated inspiratory neural drive (IND) to the diaphragm during exercise, compared with healthy controls. The greater IND was associated with impairments in pulmonary gas exchange and significant deconditioning. Our results help to explain why many patients with post-PE syndrome report significant dyspnea at relatively low levels of physical activity.
Localizing neurologic function within the brain remains a significant challenge in clinical neurosurgery. Invasive mapping with direct electrocortical stimulation currently is the clinical gold ...standard but is impractical in young or cognitively delayed patients who are unable to reliably perform tasks. Resting state functional magnetic resonance imaging non-invasively identifies resting state networks without the need for task performance, hence, is well suited to pediatric patients. We compared sensorimotor network localization by resting state fMRI to cortical stimulation sensory and motor mapping in 16 pediatric patients aged 3.1 to 18.6 years. All had medically refractory epilepsy that required invasive electrographic monitoring and stimulation mapping. The resting state fMRI data were analyzed using a previously trained machine learning classifier that has previously been evaluated in adults. We report comparable functional localization by resting state fMRI compared to stimulation mapping. These results provide strong evidence for the utility of resting state functional imaging in the localization of sensorimotor cortex across a wide range of pediatric patients.
BACKGROUNDThe precise mechanisms driving poor exercise tolerance in patients with fibrotic interstitial lung diseases (fibrotic ILDs) showing a severe impairment in single-breath lung diffusing ...capacity for carbon monoxide (DLCO < 40% predicted) are not fully understood. Rather than only reflecting impaired O2 transfer, a severely impaired DLCO may signal deranged integrative physiologic adjustments to exercise that jointly increase the burden of exertional symptoms in fibrotic ILD.METHODSSixty-seven subjects (46 with idiopathic pulmonary fibrosis, 24 showing DLCO < 40%) and 22 controls underwent pulmonary function tests and an incremental cardiopulmonary exercise test with serial measurements of operating lung volumes and 0-10 Borg dyspnea and leg discomfort scores.RESULTSSubjects from the DLCO < 40% group showed lower spirometric values, more severe restriction, and lower alveolar volume and transfer coefficient compared to controls and participants with less impaired DLCO (P < .05). Peak work rate was ∼45% (vs controls) and ∼20% (vs DLCO > 40%) lower in the former group, being associated with lower (and flatter) O2 pulse, an earlier lactate (anaerobic) threshold, heightened submaximal ventilation, and lower SpO2 . Moreover, critically high inspiratory constrains were reached at lower exercise intensities in the DLCO < 40% group (P < .05). In association with the greatest leg discomfort scores, they reported the highest dyspnea scores at a given work rate. Between-group differences lessened or disappeared when dyspnea intensity was related to indexes of increased demand-capacity imbalance, that is, decreasing submaximal, dynamic ventilatory reserve, and inspiratory reserve volume/total lung capacity (P > .05).CONCLUSIONSA severely reduced DLCO in fibrotic ILD signals multiple interconnected derangements (cardiovascular impairment, an early shift to anaerobic metabolism, excess ventilation, inspiratory constraints, and hypoxemia) that ultimately lead to limiting respiratory (dyspnea) and peripheral (leg discomfort) symptoms. DLCO < 40%, therefore, might help in clinical decision-making to indicate the patient with fibrotic ILD who might derive particular benefit from pharmacologic and non-pharmacologic interventions aimed at lessening these systemic abnormalities.
OBJECTIVE Endoscope-assisted methods for treatment of craniosynostosis have reported benefits over open calvarial vault reconstruction. In this paper, the authors evaluated 2 methods for ...endoscope-assisted correction of sagittal synostosis: wide vertex suturectomy and barrel stave osteotomies (WVS+BSO) and narrow vertex suturectomy (NVS). METHODS The authors evaluated patients with nonsyndromic sagittal synostosis treated with either wide vertex suturectomy (4-6 cm) and barrel stave osteotomies (WVS+BSO) or narrow vertex suturectomy (NVS) (approximately 2 cm) between October 2006 and July 2013. Prospectively collected data included patient age, sex, operative time, estimated blood loss (EBL), postoperative hemoglobin level, number of transfusions, complications, and cephalic index. Fourteen patients in the NVS group were age matched to 14 patients in the WVS+BSO group. Descriptive statistics were calculated, and Student t-tests were used to compare prospectively obtained data from the WVS+BSO group with the NVS group in a series of univariate analyses. RESULTS The mean age at surgery was 3.9 months for WVS+BSO and 3.8 months for NVS. The mean operative time for patients undergoing NVS was 59.0 minutes, significantly less than the 83.4-minute operative time for patients undergoing WVS+BSO (p < 0.05). The differences in mean EBL (NVS: 25.4 ml; WVS+BSO: 27.5 ml), mean postoperative hemoglobin level (NVS: 8.6 g/dl; WVS+BSO: 8.0 g/dl), mean preoperative cephalic index (NVS: 69.9; WVS+BSO: 68.2), and mean cephalic index at 1 year of age (NVS: 78.1; WVS+BSO: 77.2) were not statistically significant. CONCLUSIONS The NVS and WVS+BSO produced nearly identical clinical results, as cephalic index at 1 year of age was similar between the 2 approaches. However, the NVS required fewer procedural steps and significantly less operative time than the WVS+BSO. The NVS group obtained the final cephalic index in a similar amount of time postoperatively as the WVS+BSO group. Complications, transfusion rates, and EBL were not different between the 2 techniques.
BACKGROUND:Preoperative severity and postoperative success for patients with sagittal synostosis are measured by cephalic index, but this metric does not describe the appropriateness of euryon ...location. The authors hypothesize that cephalic index in patients with sagittal synostosis is an inaccurate measure of scaphocephaly.
METHODS:Preoperative and 1-year postoperative cranial computed tomographic scans of children with sagittal synostosis treated before 6 months of age by either total calvarial reconstruction or endoscope-assisted craniectomy and helmet therapy (n = 10 for each) were reviewed retrospectively. The location of euryons in age-matched controls was measured as a fraction of the glabella-opisthocranion distance (horizontal point of maximum width) and as the fraction of the nasion-vertex vertical distance (vertical point of maximum width). Cephalic index at this ideal location (normative cephalic index) and traditional cephalic index were determined in all patients.
RESULTS:Ideal euryon location from preoperative controls was 56 percent by the horizontal point of maximum width and 56 percent by the vertical point of maximum width. Normative cephalic index (0.60) was significantly less than traditional cephalic index (0.66) in patients preoperatively (p < 0.001) and remained smaller postoperatively (0.68 versus 0.73) for patients who underwent open reconstruction (p < 0.001). Patients treated endoscopically also had a smaller normative cephalic index (0.71) than traditional cephalic index (0.76) postoperatively (p < 0.001).
CONCLUSIONS:Anterocaudal displacement of euryon in patients with sagittal synostosis influences cephalic index. Normative cephalic index, assessed at ideal euryon location, is a more accurate measure of preoperative severity and postoperative outcome.
CLINICAL QUESTION/LEVEL OF EVIDENCE:Diagnostic, III.
OBJECT The authors present a retrospective cohort study examining complications in patients undergoing surgery for craniosynostosis using both minimally invasive endoscopic and open approaches. ...METHODS Over the past 10 years, 295 nonsyndromic patients (140 undergoing endoscopic procedures and 155 undergoing open procedures) and 33 syndromic patients (endoscopic procedures in 10 and open procedures in 23) met the authors' criteria. Variables analyzed included age at surgery, presence of a preexisting CSF shunt, skin incision method, estimated blood loss, transfusions of packed red blood cells, use of intravenous steroids or tranexamic acid, intraoperative durotomies, procedure length, and length of hospital stay. Complications were classified as either surgically or medically related. RESULTS In the nonsyndromic endoscopic group, the authors experienced 3 (2.1%) surgical and 5 (3.6%) medical complications. In the nonsyndromic open group, there were 2 (1.3%) surgical and 7 (4.5%) medical complications. Intraoperative durotomies occurred in 5 (3.6%) endoscopic and 12 (7.8%) open cases, were repaired primarily, and did not result in reoperations for CSF leakage. Similar complication rates were seen in syndromic cases. There was no death or permanent morbidity. Additionally, endoscopic procedures were associated with significantly decreased estimated blood loss, transfusions, procedure length, and length of hospital stay compared with open procedures. CONCLUSIONS Rates of intraoperative durotomies and surgical and medical complications were comparable between endoscopic and open techniques. This is the largest direct comparison to date between endoscopic and open interventions for synostosis, and the results are in agreement with previous series that endoscopic surgery confers distinct advantages over open surgery in appropriate patient populations.
Intraventricular hemorrhage (IVH) and progressive posthemorrhagic ventricular dilation (PPHVD) may result in significant neurological morbidity in preterm infants. At present, there is no consensus ...regarding the optimal timing or type of neurosurgical procedure to best treat PPHVD. Conflicting data exist regarding the relative risks and benefits of two commonly used temporizing neurosurgical procedures (TNPs), ventricular access devices (VADs or ventricular reservoirs) versus ventriculosubgaleal (VSG) shunts. This study was designed to address this issue.
This is a single-center, 10-year retrospective review of all preterm infants admitted to the St. Louis Children's Hospital neonatal intensive care unit (NICU) with Papile Grade III-IV IVH. The development of PPHVD and the requirement for and type of TNP were recorded. Rates of TNP complication, ventriculoperitoneal (VP) shunt implantation, shunt infection, and mortality rates were used to compare the efficacy and limitations of each TNP type.
Over this 10-year interval, 325 preterm infants with Grade III-IV IVH were identified, with trends showing an increasing number of affected infants annually, and an increasing number of TNPs were required annually. Ninety-five (29.2%) of the 325 infants underwent a TNP for PPHVD (65 VADs, 30 VSG shunts). The rate of permanent VP shunt implantation for all TNPs was 72.6% (69 of 95 infants). Forty-nine (75.4%) of the 65 infants treated with VADs and 20 (66.7%) of the 30 treated with VSG shunts required VP shunts (p = 0.38). There was no statistical difference between VAD or VSG shunt with regard to TNP-related infection (p = 0.57), need for TNP revision (p = 0.16), subsequent shunt infection (p = 0.77), shunt revision rate (p = 0.58), or mortality rate (p = 0.24).
Rates of IVH and PPHVD observed at the authors' center have increased over time. In contrast to recent literature, the results from the current study did not demonstrate a difference in complication rate or requirement for permanent VP shunt placement between VADs and VSG shunts. Definitive conclusions will require a larger, prospective trial.
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