Pathophysiology of brain dysfunction due to sepsis remains poorly understood. Cerebral microcirculatory alterations may play a role; however, experimental data are scarce. This study sought to ...investigate whether the cerebral microcirculation is altered in a clinically relevant animal model of septic shock.
Fifteen anesthetized, invasively monitored, and mechanically ventilated female sheep were allocated to a sham procedure (n = 5) or sepsis (n = 10), in which peritonitis was induced by intra-abdominal injection of autologous faeces. Animals were observed until spontaneous death or for a maximum of 20 hours. In addition to global hemodynamic assessment, the microcirculation of the cerebral cortex was evaluated using Sidestream Dark-Field (SDF) videomicroscopy at baseline, 6 hours, 12 hours and at shock onset. At least five images of 20 seconds each from separate areas were recorded at each time point and stored under a random number to be analyzed, using a semi-quantitative method, by an investigator blinded to time and condition.
All septic animals developed a hyperdynamic state associated with organ dysfunction and, ultimately, septic shock. In the septic animals, there was a progressive decrease in cerebral total perfused vessel density (from 5.9 ± 0.9 at baseline to 4.8 ± 0.7 n/mm at shock onset, P = 0.009), functional capillary density (from 2.8 ± 0.4 to 2.1 ± 0.7 n/mm, P = 0.049), the proportion of small perfused vessels (from 95 ± 3 to 85 ± 8%, P = 0.02), and the total number of perfused capillaries (from 22.7 ± 2.7 to 17.5 ± 5.2 n/mm, P = 0.04). There were no significant changes in microcirculatory flow index over time. In sham animals, the cerebral microcirculation was unaltered during the study period.
In this model of peritonitis, the cerebral microcirculation was impaired during sepsis, with a significant reduction in perfused small vessels at the onset of septic shock. These alterations may play a role in the pathogenesis of septic encephalopathy.
Objectives
Children with Chiari malformation type I (CM‐I) have an increased risk of sleep apnea. The aim of the study was to describe the management of CM‐I patients in relation to symptoms, ...magnetic resonance imaging (MRI) findings and sleep apnea syndrome (SAS).
Methods
We performed a retrospective analysis of clinical charts of all 57 CM‐I patients seen between September 2013 and April 2017.
Results
A total of 45 patients had isolated CM‐I or associated co‐morbidity (CM‐Iia), 5 had craniosynostosis (CM‐Ics), and 7 a polymalformative syndrome (CM‐Ipm). The prevalence of SAS, defined as an apnea‐hypopnea index >1 event/h, was high in CM‐I ranging from 50% to 80% according to the CM‐I group. The prevalence of central sleep apnea (CSA) was low, with 5 (9%) patients having CSA and only 3 patients with CM‐Iia having isolated CSA. A total of 17 patients (30%) had foramen magnum decompression (FMD). Neither positive symptoms of CM‐I nor MRI findings alone, nor both combined were good indicators for FMD. No correlation was observed between the cerebellar tonsil descent and SAS in CM‐I. But all 5 patients with CSA had a FMD. The combination of MRI findings and/or symptoms of CM‐I together with moderate‐to‐severe SAS best discriminated patients who needed a FMD.
Conclusion
Our findings highlight the importance of a combined evaluation of symptoms, MRI and polygraphy results in the management of CM‐I patients.
Children with Chiari Malformation type II (CM-II) have an increased risk of sleep apnoea. The aim of the study was to describe the management of patients with CM-II in relation to sleep apnoea ...syndrome, clinical symptoms and magnetic resonance imaging (MRI) findings.
The paper reports 8 consecutive patients with CM-II followed between September 2013 and April 2017. The prevalence of sleep apnoea syndrome was high with 6 out of 8 patients having mild-to-severe sleep apnoea. Patients with severe sleep apnoea syndrome (3 patients) were treated with upper airway surgery and/or noninvasive ventilation.
Our findings highlight the importance of respiratory polygraphy in the management of patients with CM-ΙΙ. Poly(somno)graphy is recommended in the follow-up care of children with CM-II.
Introduction
The optimal surgical management of sylvian arachnoid cysts is debated. We present our experience in children who were treated endoscopically, focusing on the limits and complications of ...this approach.
Materials and methods
Seventeen children with a temporo-sylvian arachnoid cyst have been treated using a purely temporal endoscopic approach.
Results
In all but one case, the recognition of anatomical landmarks was obtained at the insertion of the endoscope. In one child, the orientation and opening of the basal cisterns were more difficult due to the thickness and opacity of the membranes. Nevertheless, it was possible to perform the cysto-cisternostomy endoscopically in all children with several stomies in 13. In one child, a venous bleeding occurred. There were no other intraoperative complications. There were no postoperative cerebrospinal fluid leaks. In two patients, a symptomatic subdural collection developed which required a transient subdural-peritoneal shunt. No subdural collection was noted on a delayed follow-up (mean, 23 months). Preoperative symptoms related to the mass effect exerted by the cyst resolved in all children. Two children presented a recurrence, 12 and 20 months after endoscopic surgery, respectively. At the redo-endoscopy, the stomies were found to be closed. Their reopening resulted in the disappearance of the symptoms.
Conclusions
An endoscopic approach can be used safely in the management of sylvian arachnoid cysts. An effective opening of the deep arachnoid membranes into the basal cisterns could be performed in all patients with the resolution of the preoperative symptoms. However, the long-term efficacy of the stoma needs to be assessed.
Abstract
Context
Endocrine complications are common in pediatric brain tumor patients.
Objective
To describe hypothalamic–pituitary–gonadal axis (HPGA) function in patients treated in childhood for a ...primary brain tumor more than 5 years earlier, in order to identify risk factors for HPGA impairment.
Methods
We retrospectively included 204 patients diagnosed with a primary brain tumor before 18 years of age and monitored at the pediatric endocrinology unit of the Necker Enfants-Malades University Hospital (Paris, France) between January 2010 and December 2015. Patients with pituitary adenoma or untreated glioma were excluded.
Results
Among patients with suprasellar glioma not treated by radiotherapy, the prevalence of advanced puberty was 65% overall and 70% when the diagnosis occurred before 5 years of age. Medulloblastoma chemotherapy caused gonadal toxicity in 70% of all patients and in 87.5% of those younger than 5 years at diagnosis. In the group with craniopharyngioma, 70% of patients had hypogonadotropic hypogonadism, which was consistently accompanied by growth hormone deficiency.
Conclusion
Tumor type, location, and treatment were the risk main factors for HPGA impairment. Awareness that onset can be delayed is essential to guide information of parents and patients, patient monitoring, and timely hormone replacement therapy.
Background
Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and ...treatment of CM1 in children.
Methods
A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale (“strongly disagree,” “disagree,” “agree,” “strongly agree”). Statements that were endorsed (“agree” or “strongly agree”) by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three).
Results
Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the “definition of radiological failure 24 month post-surgery.”
Conclusions
The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.
Ophthalmological involvement in anterior plagiocephaly (AP) due to unicoronal synostosis (UCS) raises management challenges. Two abnormalities of the extraocular muscles (EOM) are commonly reported ...in UCS without objective quantification: (1) excyclorotation of the eye and (2) malposition of the trochlea of the superior oblique muscle. Here we aimed to assess the positions of the EOM in AP, using geometric morphometrics based on MRI data.
Patient files were listed using Dr WareHouse, a dedicated big data search engine. We included all patients with AP managed between 2013 and 2018, with an available digital preoperative MRI. MRIs from age-matched controls without craniofacial conditions were also included. We defined 13 orbital and skull base landmarks in order to model the 3D position of the EOM. Cephalometric analyses and geometric morphometrics with Procrustes superimposition and principal component analysis were used with the aim of defining specific EOM anomalies in UCS.
We included 15 preoperative and 7 postoperative MRIs from patients with UCS and 24 MRIs from age-matched controls. Cephalometric analyses, Procrustes superimposition and distance computations showed a significant shape difference for the position of the trochlea of the superior oblique muscle and an excyclorotation of the EOM.
Our results confirm that UCS-associated anomalies of the superior oblique muscle function are associated with malposition of its trochlea in the roof of the orbit. This clinical anomaly supports the importance of MRI imaging in the surgical management of strabismus in patients with UCS.
Objective: This study aimed to assess the relevance of using multi-positional MRI (mMRI) to identify cranio-vertebral junction (CVJ) instability in pediatric patients with CVJ anomalies while ...determining objective mMRI criteria to detect this condition. Material and Methods: Data from children with CVJ anomalies who underwent a mMRI between 2017 and 2021 were retrospectively reviewed. Mobility assessment using mMRI involved: (1) morphometric analysis using hierarchical clustering on principal component analysis (HCPCA) to identify clusters of patients by considering their mobility similarities, assessed through delta (Δ) values of occipito-cervical parameters measured on mMRI; and (2) morphological analysis based on dynamic geometric CVJ models and analysis of displacement vectors between flexion and extension. Receiver operating characteristics (ROC) curves were generated for occipito-cervical parameters to establish instability cut-off values. (3) Additionally, an anatomical qualitative analysis of the CVJ was performed to identify morphological criteria of instability. Results: Forty-seven patients with CVJ anomalies were included (26 females, 21 males; mean age: 10.2 years 3–18). HCPCA identified 2 clusters: cluster №1 (stable patients, n = 39) and cluster №2 (unstable patients, n = 8). ΔpB-C2 (pB-C2 line delta) at ≥2.5 mm (AUC 0.98) and ΔBAI (Basion-axis Interval delta) ≥ 3 mm (AUC 0.97) predicted instability with 88% sensibility and 95% specificity and 88% sensitivity and 85% specificity, respectively. Geometric CVJ shape analysis differentiated patients along a continuum, from a low to a high CVJ motion that was characterized by a subluxation of C1 in the anterior direction. Qualitative analysis found correlations between instability and C2 anomalies, including fusions with C3 (body p = 0.032; posterior arch p = 0.045; inferior articular facets p = 0.012; lateral mass p = 0.029). Conclusions: We identified a cluster of pediatric patients with CVJ instability among a cohort of CVJ anomalies that were characterized by morphometric parameters with corresponding cut-off values that could serve as objective mMRI criteria. These findings warrant further validation through prospective case–control studies.
Purpose
Spinal lipoma of the filum terminale (LFT) is a congenital lumbosacral anomaly that can cause tethered cord syndrome. Purposes of this study are to clarify preoperative characteristics of ...LFT, to elucidate surgical effects, and to discuss the rationale of prophylactic surgery for LFT.
Methods
Medical data of 174 children (2008–2014) who underwent section of LFT were prospectively recorded for prevalence of symptoms, skin stigmas, and associated malformations, motivator of diagnosis, conus level, and surgical outcome. Mean age at surgery was 4.1 ± 4.2 years (37 days to 17.7 years).
Results
Ninety-four children (54.0 %) had skin stigmas and 60 (34.5 %) had certain perineal malformations. Seventy-nine children (45.4 %) were symptomatic. The most common motivator for diagnosis was skin stigmas (44.3 %), followed by associated malformations (33.3 %), and symptoms (20.1 %). The age at surgery was significantly older in symptomatic patients than in asymptomatic patients (
p
< 0.001). Surgery improved symptoms in 50 % of patients at 2.1-year follow-up period. Of 85 asymptomatic patients, all except one remained asymptomatic postoperatively and none of the symptomatic patients deteriorated. The presence of associated malformations and the conus level did not affect surgical outcome. Postoperative complications developed in nine patients (5.2 %): seven transient local problems, one definitive urological deterioration, and one transient respiratory problem.
Conclusions
Surgery for LFT was a simple and safe procedure. It improved half of symptomatic patients and stopped the deterioration of the others. Even if only one of the asymptomatic patients deteriorated at maximum follow-up, the role of prophylactic surgery remains a point of discussion.
Respiratory problems are common in Morquio‐A syndrome (MPS IVA) but objective data on sleep‐disordered breathing are scarce. The aim of our study was to review polygraphic (PG) findings and the need ...for noninvasive continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in children with MPS IVA. A retrospective review of the clinical charts and PG of 16 consecutive children (7 boys, mean age 10.5 ± 4.2 years) with MPS IVA seen over a period of 3 years was performed. The prevalence of obstructive sleep apnea (OSA) was 69% with only five patients, all younger than 10 years old, having a normal PG. Four patients had mild OSA (apnea‐hypopnea index AHI ≥1.5 and <5 events/hr), three patients had moderate OSA (AHI ≥5 and <10 events/hr), and three patients had severe OSA (AHI ≥ 10 events/hr). Among the 10 patients with OSA, 3 had prior adenoidectomy ± tonsillectomy and 6 were on enzyme replacement therapy. Only one patient had a central apnea index >5 events/hr despite prior cervico‐occipital decompression. Six patients, all older than 11 years old, were started on CPAP or NIV because of severe OSA (n = 4), nocturnal hypoventilation (n = 1), or impossibility to be weaned from NIV after an acute respiratory failure (n = 1). Prevalence of OSA is high in patients with MPS IVA, underlying the importance of a systematic screening for sleep‐disordered breathing. CPAP and NIV are efficient and well accepted for treating sleep‐disordered breathing.
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