Lung ultrasound to predict the duration of respiratory support in newborn infants with respiratory distress.

AIM: Lung ultrasound (LU) and clinical parameters evaluated during the first postnatal hour potentially predict the length of CPAP therapy in newborns with respiratory distress. METHODS: In a single-centre, prospective observational pilot study, 130 newborns ≥36 weeks gestational age were assessed using standardised LU at 30 and 60 min postnatally. Various clinical parameters were evaluated influencing CPAP duration (<1 vs. ≥1 h) using univariate and multivariate analyses. RESULTS: Lung ultrasound score >5, FiO2 > 0.21 and respiratory acidosis 30 min postnatally were associated with CPAP ≥1 h. Our model showed good diagnostic quality (ROC AUC = 0.87) and was confirmed by classification and regression tree (CART) analysis. Additional LU findings like double lung point and pleural line abnormalities were frequently observed, with good interrater reliability for LU interpretation (ICC = 0.76-0.77). CONCLUSION: Newborns with postnatal respiratory distress at risk for prolonged CPAP therapy can be identified based on specific LU and clinical parameters assessed 30 min postnatally. Despite the need for validation in an independent sample, these findings may lay the groundwork for a prediction tool. LU proved feasible and reliable for assessing respiratory status in this population, highlighting potential utility in clinical practice.

Feasibility and Implementation of a 4D Free-Breathing Variable Density Stack-of-Stars Functional Magnetic Resonance Urography in Young Children Without Sedation.

BACKGROUND: Functional magnetic resonance urography (MRU) is well established in the diagnostic workup of urinary tract anomalies in children, providing comprehensive morphological and functional information. However, dynamic contrast-enhanced images acquired in the standard Cartesian k-space manner are prone to motion artifacts. A newly introduced 4D high spatiotemporal resolution dynamic contrast-enhanced magnetic resonance imaging based on variable density elliptical centric radial stack-of-stars sharing technique has shown improved image quality regarding motions under free breathing. OBJECTIVE: The aims of this study were to implement this 4D free-breathing sequence for functional MRU and to compare its image quality and analyzability with standard breath-hold Cartesian MRU. MATERIALS AND METHODS: We retrospectively evaluated all functional 4D MRU performed without general anesthesia between September 2021 and December 2022 and compared them with matched pairs (age, affected kidney, diagnosis) of standard Cartesian MRU between 2016 and 2022. Image analysis was performed by 2 radiologists independently regarding the following criteria using a 4-point Likert scale, with 4 being the best: overall image quality, diagnostic confidence, respiratory motion artifacts, as well as sharpness and contrast of aorta, kidneys, and ureters. We also measured vertical kidney motion due to respiratory motion and compared the variance for each kidney using F test. Finally, both radiologists calculated the volume, split renal volume (vDRF), split renal Patlak function (pDRF), and split renal function considering the volume and Patlak function (vpDRF) for each kidney. Values were compared using Bland-Altman plots and F test. RESULTS: Forty children (20 for 4D free-breathing and standard breath-hold, respectively) were enrolled. Ten children of each group were examined using feed-and-sleep technique (median age: 4D, 3.3 months; standard, 4.2 months), 10 were awake (median age: 4D, 8.9 years; standard, 8.6 years). Overall image quality, diagnostic confidence, respiratory motion artifacts, as well as sharpness and contrast of the aorta, kidneys, and ureters were rated significantly better for 4D free-breathing compared with standard breath-hold by both readers ( P ranging from <0.0001 to 0.005). Vertical kidney motion was significantly reduced in 4D free-breathing for the right and the left kidney (both P < 0.001). There was a significantly smaller variance concerning the differences between the 2 readers for vpDRF in 4D MRU ( P = 0.0003). In contrast, no significant difference could be demonstrated for volume ( P = 0.05), vDRF ( P = 0.93), and pDRF ( P = 0.14). CONCLUSIONS: We demonstrated the feasibility of applying a 4D free-breathing variable density stack-of-stars imaging for functional MRU in young pediatric patients with improved image quality, fewer motion artifacts, and improved functional analyzability.

Local MRI before and after Tumor Resection in Neuroblastoma: Impact of Residual Disease on Event Free Survival.

(1) Background: The study aimed to investigate the influence of MRI-defined residual disease on local tumor control after resection of neuroblastic tumors in patients without routine adjuvant radiotherapy. (2) Methods: Patients, who underwent tumor resection between 2009 and 2019 and received a pre- and postoperative MRI, were included in this retrospective single-center study. Measurement of residual disease (RD) was performed using standardized criteria. Primary endpoint was the local or combined (local and metastatic) event free survival (EFS). (3) Results: Forty-one patients (20 female) with median age of 39 months were analyzed. Risk group analysis showed eleven low-, eight intermediate-, and twenty-two high-risk patients (LR, IR, HR). RD was found in 16 cases by MRI. A local or combined relapse or progression was found in nine patients of whom eight patients had RD (p = 0.0004). From the six patients with local or combined relapse in the HR group, five had RD (p = 0.005). Only one of 25 patients without RD had a local event. Mean EFS (month) was significantly higher if MRI showed no residual tumor (81 ± 5 vs. 43 ± 9; p = 0.0014) for the total cohort and the HR subgroup (62 ± 7 vs. 31 ± 11; p = 0.016). (4) Conclusions: In our series, evidence of residual tumor, detectable by MRI, was associated with insufficient local control, resulting in relapses or local progression in 50% of patients. Only one of the patients without residual tumor had a local relapse.

Relationship Between Neonatal Brain Injury and Objective Measures of Head Trauma: A Case-Control Study.

BACKGROUND AND OBJECTIVES: Neonatal brain injury is a common and devastating diagnosis conferring lifelong challenges for children and families. The role of mechanical forces applied to the head, often referred to as "birth trauma," are often considered although evidence for this association is lacking. The objective of this study was to investigate the association between common types of neonatal brain injury and scalp swelling using a novel method to quantify scalp swelling as an unbiased proxy for mechanical forces applied to the head. METHODS: Case-control study using population-based, prospectively collected tertiary care center databases and healthy controls from the Human Connectome Development Project. Included were infants born 32-42 weeks gestational age and MRI in the first 9 days. Outcomes categories included healthy neonates, hypoxic ischemic encephalopathy (HIE) with or without brain injury, or stroke (ischemic or hemorrhagic). Volume of scalp swelling was objectively quantified by a novel imaging method blinded to brain injury. Variables included mode of delivery and use of instrumentation. Statistical tests included Kruskal-Wallis test, chi square, and multivariable and multinomial logistic regression. RESULTS: There were 309 infants included (55% male): 72 healthy controls, 77 HIE without brain injury on MRI, 78 HIE with brain injury, and 82 with stroke (60 ischemic, 22 hemorrhagic). Scalp swelling was present in 126 (40.8%, 95% confidence interval [CI] 35.2%-46.5%) with no difference in proportions between outcome groups. Compared to healthy controls, median volume was higher in those with HIE without brain injury (17.5 mL, 95% CI 6.8-28.2), HIE with brain injury (12.1 mL, 95% CI 5.5-18.6), but not ischemic stroke (4.7 mL, 95% CI -1.2-10.6) nor hemorrhagic stroke (8.3 mL, 95% CI -2.2-18.8). Scalp swelling was associated with instrumented delivery (OR 2.1, 95% CI 1.0-4.1), but not associated with increased odds of brain injury in those with HIE (OR 1.5, 95% CI 0.76-3.30). Scalp swelling measures were highly reliable (ICC = 0.97). DISCUSSION: "Birth trauma" quantified by scalp swelling volume was more common in infants with difficult deliveries, but not associated with greater odds of brain injury due to hypoxia or stroke. These results may help parents and practitioners to dissociate the appearance of trauma with the risk of brain injury.

The role of surgery in the treatment of neuroblastoma metastases at rare sites.

PURPOSE: Treatment of neuroblastoma metastases usually consists of chemotherapy and irradiation. However, in selected cases, surgical treatment is also indicated. In this study, we present three cases of patients with neuroblastoma metastases at rare sites that underwent surgery. MATERIALS AND METHODS: We retrospectively analyzed data of patients who underwent surgery for neuroblastoma at our department of Pediatric Surgery and Pediatric Urology at the University Children's Hospital in Tuebingen and selected those patients who had surgery explicitly for a metastasis. RESULTS: Between 2002 and 2020, 277 children underwent surgical treatment for neuroblastoma. Three cases with metastases at exceptional sites are presented here after therapy according to protocols. One patient had a penile metastasis and received surgery including a plastic reconstruction. The patient showed no signs of erectile or urinary dysfunction at follow-up. Another patient had a metastasis in the proximal ulna, which remained vital even after exhausted treatment after two relapses. Afterward there was no restriction of movement of the extremity. The third patient had, amongst others, metastases to the pancreatic body and to the liver. Both were surgically removed during primary tumor resection. This patient died after local tumor relapse. The other two patients showed no evidence of tumor relapse after a follow-up of 18 and 17 months, respectively. CONCLUSION: Although children with neuroblastoma often present with metastases, there is no recommendation for surgical treatment other than diagnostic biopsies. In case of persistence of metastasis or after exhaustion of high-risk therapy, surgical resection must be considered.

Enhanced Image Processing Using Complex Averaging in Diffusion-Weighted Imaging of the Prostate: The Impact on Image Quality and Lesion Detectability.

Diffusion-weighted images of the prostate can suffer from a "hazy" background in low signal-intensity areas. We hypothesize that enhanced image processing (EIP) using complex averaging reduces artifacts, noise, and distortion in conventionally acquired diffusion-weighted images and synthesized high b-value images, thus leading to higher image quality and better detection of potentially malignant lesions. Conventional DWI trace images with a b-value of 1000 s/mm2 (b1000), calculated images with a b-value of 2000 s/mm2 (cb2000), and ADC maps of 3T multiparametric prostate MRIs in 53 patients (age 68.8 ± 10 years) were retrospectively evaluated. Standard images were compared to images using EIP. In the standard images, 36 lesions were detected in the peripheral zone and 20 in the transition zone. In 13 patients, EIP led to the detection of 8 additional lesions and the upgrading of 6 lesions; 6 of these patients were diagnosed with prostate carcinoma Gleason 7 or 8. EIP improved qualitative ratings for overall image quality and lesion detectability. Artifacts were significantly reduced in the cb2000 images. Quantitative measurements for lesion detectability expressed as an SI ratio were significantly improved. EIP using complex averaging led to image quality improvements in acquired and synthesized DWI, potentially resulting in elevated diagnostic accuracy and management changes.

Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy.

PURPOSE: Mendelian etiologies for acute encephalopathies in previously healthy children are poorly understood, with the exception of RAN binding protein 2 (RANBP2)-associated acute necrotizing encephalopathy subtype 1 (ANE1). We provide clinical, genetic, and neuroradiological evidence that biallelic variants in ribonuclease inhibitor (RNH1) confer susceptibility to a distinctive ANE subtype. METHODS: This study aimed to evaluate clinical data, neuroradiological studies, genomic sequencing, and protein immunoblotting results in 8 children from 4 families who experienced acute febrile encephalopathy. RESULTS: All 8 healthy children became acutely encephalopathic during a viral/febrile illness and received a variety of immune modulation treatments. Long-term outcomes varied from death to severe neurologic deficits to normal outcomes. The neuroradiological findings overlapped with ANE but had distinguishing features. All affected children had biallelic predicted damaging variants in RNH1: a subset that was studied had undetectable RNH1 protein. Incomplete penetrance of the RNH1 variants was evident in 1 family. CONCLUSION: Biallelic variants in RNH1 confer susceptibility to a subtype of ANE (ANE2) in previously healthy children. Intensive immunological treatments may alter outcomes. Genomic sequencing in children with unexplained acute febrile encephalopathy can detect underlying genetic etiologies, such as RNH1, and improve outcomes in the probands and at-risk siblings.

Thoracic lymphatic anomalies in patients with univentricular hearts: correlation of morphologic findings in isotropic T2-weighted MRI with the outcome after fontan palliation.

Objectives: In this study we examined the correlation between the extent of thoracic lymphatic anomalies in patients after surgical palliation by total cavopulmonary connection (TCPC) and their outcome in terms of clinical and laboratory parameters. Materials and methods: We prospectively examined 33 patients after TCPC with an isotropic heavily T2-weighted MRI sequence on a 3.0 T scanner. Examinations were performed after a solid meal, slice thickness of 0.6 mm, TR of 2400 ms, TE of 692 ms, FoV of 460 mm, covering thoracic and abdominal regions. Findings of the lymphatic system were correlated with clinical and laboratory parameters obtained at the annual routine check-up. Results: Eight patients (group 1) showed type 4 lymphatic abnormalities. Twentyfive patients (group 2) presented less severe anomalies (type 1-3). In the treadmill CPET, group 2 reached step 7.0;6.0/8.0 vs. 6.0;3.5/6.8 in group 1 (p = 0.006*) and a distance of 775;638/854 m vs. 513;315/661 m (p = 0.006*). In the laboratory examinations, group 2 showed significantly lower levels of AST, ALT and stool calprotectin as compared to group 1. There were no significant differences in NT-pro-BNP, total protein, IgG, lymphocytes or platelets, but trends. A history of ascites showed 5/8 patients in group 1 vs. 4/25 patients in group 2 (p = 0.02*), PLE occurred in 4/8 patient in group 1 vs. 1/25 patients in group 2 (p = 0.008*). Conclusion: In the long-term follow-up after TCPC, patients with severe thoracic and cervical lymphatic abnormalities showed restrictions in exercise capacity, higher liver enzymes and an increased rate of symptoms of imminent Fontan-failure such as ascites and PLE.

Identifying Genetic Susceptibility in Neonates With Hypoxic-Ischemic Encephalopathy: A Retrospective Case Series.

Neonatal hypoxic-ischemic encephalopathy is a clinical phenomenon that often results from perinatal asphyxia. To mitigate secondary neurologic injury, prompt initial assessment and diagnosis is needed to identify patients eligible for therapeutic hypothermia. However, occasionally neonates present with a clinical picture of hypoxic-ischemic encephalopathy without significant risk factors for perinatal asphyxia. We hypothesized that in patients with genetic abnormalities, the clinical manifestation of those abnormalities may overlap with hypoxic-ischemic encephalopathy criteria, potentially contributing to a causal misattribution. We reviewed 210 charts of infants meeting local protocol criteria for moderate to severe hypoxic-ischemic encephalopathy in neonatal intensive care units in Calgary, Alberta. All patients that met criteria for therapeutic hypothermia were eligible for the study. Data were collected surrounding pregnancy and birth histories, as well as any available genetic or metabolic testing including microarray, gene panels, whole-exome sequencing, and newborn metabolic screens. Twenty-eight patients had genetic testing such as microarray, whole-exome sequencing, or a gene panel, because of clinical suspicion. Ten of 28 patients had genetic mutations, including CDKL5, pyruvate dehydrogenase, CFTR, CYP21A2, ISY1, KIF1A, KCNQ2, SCN9A, MTFMT, and NPHP1. All patients lacked significant risk factors to support a moderate to severe hypoxic-ischemic encephalopathy diagnosis. Treatment was changed in 2 patients because of confirmed genetic etiology. This study demonstrates the importance of identifying genetic comorbidities as potential contributors to a hypoxic-ischemic encephalopathy phenotype in neonates. Early identification of clinical factors that support an alternate diagnosis should be considered when the patient's clinical picture is not typical of hypoxic-ischemic encephalopathy and could aid in both treatment decisions and outcome prognostication.

[Whole-body MRI in cancer predisposition syndromes]. / Ganzkörper-MRT bei Tumorprädispositionssyndromen.

BACKGROUND: In recent decades, whole-body magnetic resonance imaging (WB-MRI) has become established as the modality of choice for the diagnosis, staging, and follow-up of oncological diseases as well as for the screening of cancer predisposition syndromes, such as Li-Fraumeni syndrome. METHODS: As a comprehensive imaging modality without ionizing radiation, WB-MRI can be used repetitively and because of its excellent soft tissue contrast and high resolution provides early and precise detection of pathologies. This article discusses the technical requirements, some examination strategies and the clinical significance of typical findings of WB-MRI in patients with cancer predisposition syndromes.

AI Denoising Improves Image Quality and Radiological Workflows in Pediatric Ultra-Low-Dose Thorax Computed Tomography Scans.

(1) This study evaluates the impact of an AI denoising algorithm on image quality, diagnostic accuracy, and radiological workflows in pediatric chest ultra-low-dose CT (ULDCT). (2) Methods: 100 consecutive pediatric thorax ULDCT were included and reconstructed using weighted filtered back projection (wFBP), iterative reconstruction (ADMIRE 2), and AI denoising (PixelShine). Place-consistent noise measurements were used to compare objective image quality. Eight blinded readers independently rated the subjective image quality on a Likert scale (1 = worst to 5 = best). Each reader wrote a semiquantitative report to evaluate disease severity using a severity score with six common pathologies. The time to diagnosis was measured for each reader to compare the possible workflow benefits. Properly corrected mixed-effects analysis with post-hoc subgroup tests were used. Spearman's correlation coefficient measured inter-reader agreement for the subjective image quality analysis and the severity score sheets. (3) Results: The highest noise was measured for wFBP, followed by ADMIRE 2, and PixelShine (76.9 ± 9.62 vs. 43.4 ± 4.45 vs. 34.8 ± 3.27 HU; each p < 0.001). The highest subjective image quality was measured for PixelShine, followed by ADMIRE 2, and wFBP (4 (4−5) vs. 3 (4−5) vs. 3 (2−4), each p < 0.001) with good inter-rater agreement (r ≥ 0.790; p ≤ 0.001). In diagnostic accuracy analysis, there was a good inter-rater agreement between the severity scores (r ≥ 0.764; p < 0.001) without significant differences between severity score items per reconstruction mode (F (5.71; 566) = 0.792; p = 0.570). The shortest time to diagnosis was measured for the PixelShine datasets, followed by ADMIRE 2, and wFBP (2.28 ± 1.56 vs. 2.45 ± 1.90 vs. 2.66 ± 2.31 min; F (1.000; 99.00) = 268.1; p < 0.001). (4) Conclusions: AI denoising significantly improves image quality in pediatric thorax ULDCT without compromising the diagnostic confidence and reduces the time to diagnosis substantially.

Neuroprotection care bundle implementation is associated with improved long-term neurodevelopmental outcomes in extremely premature infants.

OBJECTIVE: To study the impact of an evidence-based neuroprotection care (NPC) bundle on long-term neurodevelopmental impairment (NDI) in infants born extremely premature. STUDY DESIGN: An NPC bundle targeting predefined risk factors for acute brain injury in extremely preterm infants was implemented. We compared the incidence of composite outcome of death or severe neurodevelopmental impairment (sNDI) at 21 months adjusted age pre and post bundle implementation. RESULTS: Adjusting for confounding factors, NPC bundle implementation associated with a significant reduction in death or sNDI (aOR, 0.34; 95% CI 0.17-0.68; P = 0.002), mortality (aOR, 0.31; 95% CI (0.12-0.79); P = 0.015), sNDI (aOR, 0.37; 95% CI: 0.12-0.94; P = 0.039), any motor, language, or cognitive composite score <70 (aOR, 0.48; 95% CI: 0.26-0.90; P = 0.021). CONCLUSION: Implementation of NPC bundle targeting predefined risk factors is associated with a reduction in mortality or sNDI in extremely preterm infants.

Results after skin traction for femur shaft fractures in children below the age of four years.

PURPOSE: Nonsurgical management has been identified as the treatment of choice for femoral shaft fractures in children below four years of age. For various reasons, the surgical approach has become increasingly popular in recent years. The aim of this study is to report results after vertical skin traction and analyze the benefits of this technique as well as to point out advantages compared with surgery in this age group. METHODS: The authors performed a retrospective data analysis, including all patients with femoral shaft fractures below the age of four who were treated with vertical skin traction at our institution between January 2006 and December 2016. RESULTS: Skin traction for a femoral shaft fracture was performed for 36 patients (mean age 1.6 years; 1 day-3.5 years). The mean duration of traction was 18.5 days (14-30). Complications included soft tissue affections (n = 5), which all healed spontaneously. Consolidation was observed in all fractures. Initial axial deviations and shortening did not change during traction until consolidation (p > 0.05), and no relevant torsion deformity occurred (p = 0.01). Patients gained full weight-bearing within 12.3 days (7-40) following end of traction. At the final follow-up, after a mean of 29.3 months (12-192), leg-length discrepancy (mean 7.1 mm; 5-20) was found on radiograms in nine cases, and axial deviations (mean 7.7°; 5-25) were documented in seven cases. None of the patients had limitations in daily activities or sports. CONCLUSION: Skin traction is a technically easy, safe, and non-invasive treatment modality for femoral shaft fractures in children below the age of four years. Strong results are obtained benefited from a high potential of growth-related correction, and in principle no anesthesia is needed. A prolonged hospitalization and socio-economic factors maintain the ongoing debate in comparison with the surgical approach. LEVEL OF EVIDENCE: Level III, retrospective.

Ageing is associated with maladaptive immune response and worse outcome after traumatic brain injury.

Traumatic brain injury is increasingly common in older individuals. Older age is one of the strongest predictors for poor prognosis after brain trauma, a phenomenon driven by the presence of extra-cranial comorbidities as well as pre-existent pathologies associated with cognitive impairment and brain volume loss (such as cerebrovascular disease or age-related neurodegeneration). Furthermore, ageing is associated with a dysregulated immune response, which includes attenuated responses to infection and vaccination, and a failure to resolve inflammation leading to chronic inflammatory states. In traumatic brain injury, where the immune response is imperative for the clearance of cellular debris and survey of the injured milieu, an appropriate self-limiting response is vital to promote recovery. Currently, our understanding of age-related factors that contribute to the outcome is limited; but a more complete understanding is essential for the development of tailored therapeutic strategies to mitigate the consequences of traumatic brain injury. Here we show greater functional deficits, white matter abnormalities and worse long-term outcomes in aged compared with young C57BL/6J mice after either moderate or severe traumatic brain injury. These effects are associated with altered systemic, meningeal and brain tissue immune response. Importantly, the impaired acute systemic immune response in the mice was similar to the findings observed in our clinical cohort. Traumatic brain-injured patient cohort over 70 years of age showed lower monocyte and lymphocyte counts compared with those under 45 years. In mice, traumatic brain injury was associated with alterations in peripheral immune subsets, which differed in aged compared with adult mice. There was a significant increase in transcription of immune and inflammatory genes in the meninges post-traumatic brain injury, including monocyte/leucocyte-recruiting chemokines. Immune cells were recruited to the region of the dural injury, with a significantly higher number of CD11b+ myeloid cells in aged compared with the adult mice. In brain tissue, when compared with the young adult mice, we observed a more pronounced and widespread reactive astrogliosis 1 month after trauma in aged mice, sustained by an early and persistent induction of proinflammatory astrocytic state. These findings provide important insights regarding age-related exacerbation of neurological damage after brain trauma.

Feeding during therapeutic hypothermia is safe and may improve outcomes in newborns with perinatal asphyxia.

OBJECTIVE: We assessed the impact of early enteral feeding introduction during therapeutic hypothermia on time to reach full enteral feeding (FEF) and other feeding related outcomes in infants born at ≥35 weeks gestational age and diagnosed with moderate to severe Hypoxic-Ischemic Encephalopathy. METHODS: A prospective cohort with historical control study, conducted on infants admitted to the Alberta Children's Hospital level III NICU in Calgary between January 2013 and December 2018. Infants were divided into 2 groups: (1) unfed group (UG), which was kept nil per os during the 72 h of therapeutic Hypothermia (TH), with subsequent introduction of feeding and gradual increase to FEF; (2) fed group (FG), which received feeding at 10 mL/kg/day during TH then increased gradually to FEF. Groups were compared for time to FEF and the type of milk they were being fed on discharge. Other gut related health risks such as NEC and sepsis were examined. RESULTS: During the study period, 146 infants received therapeutic hypothermia, of whom 75 in the UG and 71 in the FG. The FG compared to the UG received the first feed sooner after TH initiation (median 57 vs. 86.5 h, p < .001), reached FEF earlier (median 6 vs. 8 days, p = .012), had a higher rate of being fully fed in the first week of life (70 vs. 53%, p < .035), was kept NPO for shorter duration (median 2 vs. 4 days, p < .001), and had a higher rate of breast milk feeding at discharge (41 vs. 13%, p < .001). There were no cases of necrotizing enterocolitis or late onset sepsis in either group during the hospital stay. CONCLUSION: Minimal enteral feeding during therapeutic hypothermia appears to be safe and leads to a shorter time to FEF and higher rates of breast milk feeding at discharge.

Pearls & Oy-sters: Cerebral Abscess Secondary to Pulmonary Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant condition that is linked to a myriad of neurologic complications arising from vascular malformations of the brain, spinal cord, and lungs. Our case describes a previously healthy 3-year-old male who presented to hospital with fever of unknown origin and was found to have a brain abscess stemming from a pulmonary arteriovenous malformation (PAVM). This etiology was identified after a period of diagnostic delay; the medical team was suspicious for a proximal embolic source due to the presence of multiple tiny infarcts seen on MRI of the brain, but transthoracic echocardiogram and head and neck angiogram were unremarkable. Fortunately, an enhanced CT of the chest was performed, identifying a moderately sized PAVM. PAVMs are associated with intracranial abscesses due to shunting and loss of the normal filtering effects of the lung capillary bed. Impaired pulmonary filtration can permit paradoxical thromboemboli and septic microemboli to enter systemic circulation, predisposing patients with PAVMs to cerebral abscess and ischemic stroke. Screening for PAVMs with contrast-enhanced echocardiogram or enhanced CT of the chest may be considered in patients with cryptogenic brain abscess or recurrent embolic stroke of unknown origin. PAVMs are often associated with hereditary hemorrhagic telangiectasia (HHT). As many features of HHT have delayed clinical manifestation, genetic testing for HHT should be considered in all people with PAVM, even in the absence of other clinical features. In our case, genetic testing returned positive, confirming a new diagnosis of HHT type 1.

Safely Inserting Neonatal Chest Drains.

BACKGROUND: Inserting a chest drain for a left-sided neonatal pneumothorax carries a risk of penetrating the pericardium. We identified reference ranges for the chest wall thickness (CWT) and distance between the pericardium and parietal pleura to improve safety of chest tube insertion. METHOD: We prospectively measured the CWT using ultrasound in 20 neonates (body weight [BW] 640-2,700 g, age <10 days) at the usual site of puncture in the 4th and 5th intercostal space (ICS). Furthermore, we measured the minimal distance between the parietal pleura and the cardiac silhouette in 131 neonatal chest X-rays (birth weight, 420-4,930 g [divided into 11 weight groups]; age <10 days). Both data sets were transformed into weight-dependent percentiles (Ps). We considered the difference between the sum of P 2.5 for the CWT plus P 2.5 for pleura-heart distance minus P 97.5 for the CWT as a safe corridor for placing the tip of the needle. RESULTS: At both ICSs, curves for the above metrics did not cross, indicating a narrow but safe corridor for each BW with at least 97.5% probability. This safety corridor was 4.6-5.2 mm wide for the 4th and 2.8-3.4 mm for the 5th ICS. CONCLUSION: These data offer a reference for left-sided chest drain insertion for BW <2,700 g, which may help to improve safety of the procedure.