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1.
World Neurosurg ; 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38960310

ABSTRACT

OBJECTIVE: Intracranial cavernous malformations (CMs) are benign vascular lesions associated with hemorrhage, seizures, and corresponding neurological deficits. Recent evidence shows that frailty predicts neurosurgical adverse outcomes with superior discrimination compared to greater patient age. Therefore, we utilized the Risk Analysis Index (RAI) to predict adverse outcomes following cavernous malformations resection (CMR). METHODS: This retrospective study utilized the Nationwide Inpatient Sample (NIS) to identify patients who underwent craniotomy for CMR (2019-2020). Multivariate analysis used RAI to assess the ability of frailty to predict non-home discharge (NHD), extended length of stay (eLOS) and postoperative adverse outcomes. Receiver operating characteristic (ROC) curve analysis evaluated the discriminatory accuracy of RAI for prediction of NHD. RESULTS: 1200 CMR patients were identified. Mean patient age was 38 ± 1.2 years, 53.3% (N = 640) were female, and 58.3% (N = 700) had private insurance. Patients were stratified into four frailty tiers based on RAI scores, "robust" (0-20, R): N = 905 (80.8%), "normal" (21-30, N), N = 110 (9.8%), "frail" (31-40, F) N= 25 (2.2%), and "very frail" (41+, VF): 80 (7.1%). Increasing frailty was associated with eLOS and higher rates of NHD (p< 0.05). The RAI demonstrated strong discriminatory accuracy (C-statistic = 0.722) for prediction of NHD following CMR in AUROC. CONCLUSION: Preoperative frailty independently predicts adverse outcomes, including eLOS and NHD in patients undergoing resection of cranial CMs. Integrating RAI into preoperative frailty risk assessment may optimize risk stratification and improve patient selection and reallocate perioperative management resources for better patient outcomes.

2.
Heliyon ; 10(12): e32690, 2024 Jun 30.
Article in English | MEDLINE | ID: mdl-38952376

ABSTRACT

Objective: To obtain insight into the molecular process implicated in venous malformations (VMs) and identify potential targets for treatment of VMs, this study profiled the gene expression pattern in VMs, investigated alterations of syndecan-1 (SDC1) expression in VMs, and tested the hypothesis that aberrant SDC1 expression triggers abnormal angiogenesis and VM development. Methods: Microarray analysis was performed to identify differentially expressed genes (DEGs) on a transcriptome-wide level in VMs and conjunctive normal. Gene Ontology molecular functional analysis and Kyoto Encyclopedia of Genes and Genomes pathway analysis were carried out to establish enhancement of biological signaling pathways involved in VMs. Among the DEGs, we focused on SDC1, which is involved in matrix remodeling, cell proliferation and invasion, and angiogenesis. SDC1 expression in VMs was verified by qRT-PCR, western blotting, and immunohistochemistry. Loss-of-function of SDC1 was achieved in human umbilical vein endothelial cells (HUVECs) by siRNA to investigate the roles of SDC1 in cell migration, invasion, and angiogenesis. Results: Compared with control tissue, the transcriptome study identified 274 upregulated DEGs and 3 downregulated DEGs. The transcript and protein levels of SDC1 were significantly decreased in VMs compared with normal tissue. Inhibition of SDC1 enhanced HUVEC migration, invasion, and angiogenesis. Conclusion: Our genome-wide microarray analysis suggests the involvement of numerous genes in VMs. Among them, SDC1 plays a substantial role in the process of angiogenesis and development of VMs. SDC1 may represent a potential target for a molecular therapy for VMs.

3.
BMC Pediatr ; 24(1): 435, 2024 Jul 06.
Article in English | MEDLINE | ID: mdl-38971736

ABSTRACT

INTRODUCTION: Congenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology. METHOD: This retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran. RESULT: The average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients' type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04). CONCLUSION: Some CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.


Subject(s)
Cystic Adenomatoid Malformation of Lung, Congenital , Humans , Retrospective Studies , Iran/epidemiology , Female , Male , Infant , Cystic Adenomatoid Malformation of Lung, Congenital/diagnostic imaging , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Cystic Adenomatoid Malformation of Lung, Congenital/epidemiology , Child, Preschool , Lung/abnormalities , Lung/diagnostic imaging , Pulmonary Emphysema/congenital , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/epidemiology , Pulmonary Emphysema/diagnosis , Child , Bronchopulmonary Sequestration/diagnostic imaging , Bronchopulmonary Sequestration/epidemiology , Lung Diseases/congenital , Lung Diseases/epidemiology , Lung Diseases/diagnostic imaging , Lung Diseases/diagnosis , Bronchogenic Cyst/diagnostic imaging , Bronchogenic Cyst/epidemiology , Bronchogenic Cyst/diagnosis , Bronchogenic Cyst/congenital , Infant, Newborn , Prevalence
4.
Pediatr Radiol ; 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38980353

ABSTRACT

Vascular anomalies develop during fetal life and can be detected on prenatal ultrasonography and fetal magnetic resonance imaging. Diagnosis of lymphatic, venous, and arteriovenous malformations, as well as congenital hemangiomas and other congenital vascular tumors, may be challenging. The benign vascular anomalies may be difficult to differentiate from malignancies with a similar appearance. In this manuscript, we present a succinct overview of the congenital vascular anomalies that may present in fetal or neonatal life.

5.
Biosci Rep ; 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38980708

ABSTRACT

KRIT1 is a 75 kDa scaffolding protein which regulates endothelial cell phenotype by limiting the response to inflammatory stimuli and maintaining a quiescent and stable endothelial barrier. Loss of function mutations in KRIT1 lead to the development of cerebral cavernous malformations (CCM), a disease marked by the formation of abnormal blood vessels which exhibit a loss of barrier function, increased endothelial proliferation, and altered gene expression. While many advances have been made in our understanding of how KRIT1, and the functionally related proteins CCM2 and PDCD10, contribute to the regulation of blood vessels and the vascular barrier, some important open questions remain. In addition, KRIT1 is widely expressed and KRIT1 and the other CCM proteins have been shown to play important roles in non-endothelial cell types and tissues, which may or may not be related to their role as pathogenic originators of CCM. In this review, we discuss some of the unsettled questions regarding the role of KRIT1 in vascular physiology and discuss recent advances that suggest this ubiquitously expressed protein may have a role beyond the endothelial cell.

6.
Surg Neurol Int ; 15: 206, 2024.
Article in English | MEDLINE | ID: mdl-38974548

ABSTRACT

Background: Spontaneous obliteration of untreated cerebral arteriovenous malformations (AVMs) is rare, occurring in <1% of cases, and is even less common in pediatric populations. The mechanisms driving spontaneous regression of brain AVMs remain poorly understood, and long-term surveillance in pediatric patients is infrequently documented. Case Description: The authors reported a remarkably rare instance of spontaneous thrombosis in a pial AVM accompanied by a large intranidal aneurysm in a 10-month-old infant, initially presenting with a nocturnal seizure. Diagnostic imaging revealed a ruptured intranidal aneurysm causing acute hemorrhage in the left anterior interhemispheric subdural space, extending into adjacent areas. Further, magnetic resonance imaging (MRI) and magnetic resonance angiography delineated the AVM in the left superior frontal gyrus, associated with a thrombosed aneurysm and surrounding edema. Cerebral angiography confirmed the AVM's origin from the left anterior cerebral artery, displaying early venous drainage and small, indirect feeders not amenable to endovascular treatment. Over time, serial imaging showed the aneurysm's transition from partial to complete thrombosis. Subsequent MRIs and angiographic assessments up to age 10 confirmed complete resolution of the AVM and aneurysm, with focal hyperemia persisted until age 16, when recurrent AVM was identified. Conclusion: We document a rare spontaneous regression of a pial AVM with an intranidal aneurysm influenced by specific vascular factors. Despite this, spontaneous thrombosis should not replace vigilant long-term monitoring in pediatric neurovascular care.

7.
Front Pediatr ; 12: 1393891, 2024.
Article in English | MEDLINE | ID: mdl-38978842

ABSTRACT

Pancreatitis, in general, is a high-morbidity condition. Genetic conditions and anatomic variants are sometimes seen, especially in children, where biliary etiologies and alcohol are less common than in adults. The decision to intervene, the combined operative-endoscopic strategy, and the timing pose unique challenges. We report the case of a 10-year-old boy with PRSS1 mutation and pancreatic duct duplication, discussing the management and reviewing the recent reports in the Literature.

8.
Reprod Toxicol ; 128: 108655, 2024 Jul 06.
Article in English | MEDLINE | ID: mdl-38972362

ABSTRACT

Observational studies have suggested an association between air pollutants and congenital malformations; however, conclusions are inconsistent and the causal associations have not been elucidated. In this study, based on publicly available genetic data, a two-sample Mendelian randomization (MR) was applied to explore the associations between particulate matter 2.5 (PM2.5), NOX, NO2 levels and 11 congenital malformations. Inverse variance weighted (IVW), MR-Egger and weighted median were used as analytical methods, with IVW being the main method. A series of sensitivity analyses were used to verify the robustness of the results. For significant associations, multivariable MR (MVMR) was utilized to explore possible mediating effects. The IVW results showed that PM2.5 was associated with congenital malformations of digestive system (OR = 7.72, 95 %CI = 2.33-25.54, P = 8.11E-4) and multiple systems (OR = 8.63, 95 %CI = 1.02-73.43, P = 0.048) risks; NOX was associated with circulatory system (OR = 4.65, 95 %CI = 1.15-18.86, P = 0.031) and cardiac septal defects (OR = 14.09, 95 %CI = 1.62-122.59, P = 0.017) risks; NO2 was correlated with digestive system (OR = 27.12, 95 %CI = 1.81-407.07, P = 0.017) and cardiac septal defects (OR = 22.57, 95 %CI = 2.50-203.45, P = 0.005) risks. Further MVMR analyses suggest that there may be interactions in the effects of these air pollutants on congenital malformations. In conclusion, this study demonstrated a causal association between air pollution and congenital malformations from a genetic perspective.

9.
Article in English | MEDLINE | ID: mdl-38951201

ABSTRACT

OBJECTIVES: Lymphatic malformations (LMs) are abnormal lymphatic vessels with cystic characteristics, categorized as macrocystic, microcystic, or a combination of both. They represent the second most common vascular malformations, and their management involves multidisciplinary approaches based on clinical assessments and imaging studies. LMs manifest as a challenge to medical professionals in the head and neck, posing functional and aesthetic concerns. Our systematic review aims to compare the efficacy of sclerotherapy and surgery for LMs, identifying optimal treatment modalities for each scenario. METHODS: We searched four electronic databases for related studies. Data were extracted from the included studies. We calculated the pooled rate ratios with 95% confidence intervals (CIs). The I2 test was used to detect heterogeneity. The inclusion of the studies required the following prerequisites: 1- Studies focusing on any lymphatic malformations in the head and neck, whether microcystic, macrocystic, or a mix of both; 2- Studies performed on more than ten patients; 3- All interventions used as surgery, sclerotherapy, or both. RESULTS: We included 58 studies in our systematic review, of which 45 were eligible for the meta-analysis. For macrocystic LMs, sodium tetradecyl sulfate (STS) mixed with ethanol and excision achieved the highest complete response rates at (92.9%) and (92.5%), respectively. Surgical excision showed the lowest poor response rate. Polidocanol microfoam had the highest poor response rate (11.1%). In microcystic LMs, combining sclerotherapy with excision showed the highest complete response rate (70.3%) and the lowest poor response rate (1.3%). Picibanil had the lowest complete response rate (9.1%) and the highest rate of poor response (61.4%). In mixed LMs, surgical excision had the highest complete response rate (70.3%). CONCLUSION: Both surgical excision and STS combined with ethanol are highly effective for treating macrocystic LMs, achieving similar complete response rates. The combination of sclerotherapy and surgical excision demonstrated the best outcomes in microcystic LMs. Surgical excision demonstrates superior efficacy over sclerotherapy for mixed LMs. These findings suggest that excision is generally more effective in achieving complete and excellent responses across all LM subtypes. Further high-quality studies are necessary to standardize and optimize treatment protocols.

10.
Cureus ; 16(5): e61320, 2024 May.
Article in English | MEDLINE | ID: mdl-38947675

ABSTRACT

Coronary arteriovenous fistulas (CAVFs) are congenital or acquired communications between the coronary arteries and coronary venous system, and they can also include other cardiac structures or vasculature. We discuss a case of a large fistula between the left main coronary artery and the right atrium in a geriatric patient with a history of gastrointestinal arteriovenous malformations (AVM). The occurrence of CAVFs, an uncommon cardiac irregularity, is particularly infrequent among older adults. Typically, it is discovered by chance when investigating symptoms such as shortness of breath or chest pain, where coronary angiography is necessary to determine the most effective treatment strategy. This case highlights the possible utility of evaluating CAVFs in patients with a history of gastrointestinal AVM who similarly present with clinical symptoms of high-output heart failure. Once identified, this could simplify the treatment approach and improve communication between healthcare providers to minimize the risk of harm to the patient.

11.
Cureus ; 16(4): e58538, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38957816

ABSTRACT

Klippel-Feil syndrome (KFS) is a rare congenital disorder characterized by the fusion of cervical vertebrae, limiting neck mobility, and often presenting with clinical manifestations such as neck pain, stiffness, and neurological deficits. While the classical presentation of KFS includes a "clinical triad" comprising a shortened neck, a low posterior hairline, and limited cervical motion, not all patients exhibit all three features. This case report presents an 81-year-old male with the complete KFS triad and underscores the diagnostic challenges and management strategies associated with this condition. Despite the rarity of KFS, understanding it is crucial for clinicians due to its profound implications on patient management and quality of life. This case emphasizes the importance of clinical suspicion in Internal Medicine, showcasing how an isolated presentation may often be a manifestation of an underlying congenital condition.

12.
Emerg Radiol ; 2024 Jul 11.
Article in English | MEDLINE | ID: mdl-38987491

ABSTRACT

OBJECTIVE: To compare events of recurrent swelling between treated and untreated patients with macrocystic lymphatic malformations of the head and neck not involving the airway. The frequency and timing of emergency department (ED) visits related to the event were analysed to provide data on efficacy and ideal timing of treatment. METHODS: A 5-year retrospective review of a hospital database was conducted reviewing 35 patients (15 female, 20 male; mean age 3.9 years) with macrocystic lymphatic malformations of the head and neck not involving the airway. Patients treated with oral medications were excluded. A survival analysis was performed comparing the incidence of recurrent swelling of the malformation. A Cox regression analysis was conducted using age, gender, diameter of lymphatic malformation at presentation, and echogenicity on US as covariates. Fisher's test and mean comparisons were performed to correlate the populations baselines and the number and frequency of ED visits between the 2 groups. RESULTS: Thirteen patients underwent sclerotherapy soon after initial presentation and 22 elected for observation. The two baseline populations differed at presentation with the treatment group being younger (1.4 ± 2.4 vs. 5.4 ± 6.3 years, p = 0.03) and with larger lesions (5.7 ± 2.7 vs. 4.0 ± 1.7 cm p = 0.03). Mean follow-up time was 2.7 years. Survival analysis showed 1 or multiple recurrences affected 16 patients in the untreated group and 3 patients in the treated group. (p = 0.04). Age, gender, diameter of the lesion at presentation and increased echogenicity on US were not predictive factors of recurrence. Although the probability of visiting the ED at least once did not differ between the two groups (p = 0.42), patients from the non-treatment group were more likely to visit the ED more than once (p = 0.03). CONCLUSIONS: Sclerotherapy treatment may reduce the chance of recurrent swelling or an event after initial presentation to the ED.

13.
J Med Case Rep ; 18(1): 313, 2024 Jul 08.
Article in English | MEDLINE | ID: mdl-38973008

ABSTRACT

BACKGROUND: Pulmonary arteriovenous malformations are a relatively uncommon medical condition, affecting roughly 1 in every 2500 individuals. Of those suffering from pulmonary arteriovenous malformations, 80% have an underlying genetic condition: hereditary hemorrhagic telangiectasia. CASE PRESENTATION: We present the case of a 20-year-old Pakistani male with a history of persistent slower-onset frontal headaches that increased in severity within the course of the day. His hemoglobin was 18 g/dl, indicating polycythemia, for which he had undergone seven venesections in a month previously. His physical examination was unremarkable. His computed tomography scan depicted multiple dilated tortuous vessels with branching linear opacities in the right lower lobe of the lungs. The multiple feeding arteries were supplied by the right main pulmonary artery, and the large draining veins led to the right inferior pulmonary vein. This was identified as a diffuse pulmonary arteriovenous malformation. He was recommended for a right pulmonary artery angiogram. It showed multiple tortuous vessels with a nidus and large draining veins-features of a diffuse arteriovenous malformation in the right lower lobe of the lung consistent with the computed tomography scan. Embolization of two of these vessels feeding the arteriovenous malformation was conducted, using Amplatzer Vascular plug 2, whereas multiple pushable coils (five coils) were used for embolizing the third feeding vessel. This achieved 70-80% successful embolization of right pulmonary AVM; however, some residual flow was still seen in the arteriovenous malformation given the complexity of the lesion. Immediately after, his oxygen saturation improved from 78% to 96%. CONCLUSION: Diffuse pulmonary arteriovenous malformations, as seen in this patient, are rare, accounting for less than 5% of total pulmonary arteriovenous malformations diagnosed. The patient presented with a complaint of progressive frontal headaches, which can be attributed to low oxygen saturation or the presence of a cerebral arteriovenous malformation. There was no history of hereditary hemorrhagic telangiectasia in the patient's family. Furthermore, although most patients with hereditary hemorrhagic telangiectasia and hence pulmonary arteriovenous malformation have complaints of iron-deficiency anemia, our patient in contrast was suffering from polycythemia. This can be explained as a compensatory mechanism in hypoxemic conditions. Moreover, the patient had no complaint of hemoptysis or epistaxis, giving a varied presentation in comparison with a typical pulmonary arteriovenous malformation.


Subject(s)
Arteriovenous Malformations , Embolization, Therapeutic , Headache , Polycythemia , Pulmonary Artery , Pulmonary Veins , Humans , Male , Polycythemia/complications , Pulmonary Veins/abnormalities , Pulmonary Veins/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Young Adult , Arteriovenous Malformations/complications , Arteriovenous Malformations/diagnostic imaging , Headache/etiology , Tomography, X-Ray Computed , Treatment Outcome , Arteriovenous Fistula
14.
Epilepsy Behav ; 157: 109924, 2024 Jun 29.
Article in English | MEDLINE | ID: mdl-38945077

ABSTRACT

OBJECTIVE: This review aims to summarize existing evidence on the adverse pregnancy outcomes and seizure control effects of using lamotrigine (LTG) monotherapy in pregnancy women with epilepsy (WWE) during pregnancy. METHODS: A comprehensive search was conducted in various databases including Cochrane, Web of Science, CBM, PubMed, Embase, CNKI, and Pregnancy Registration Center databases to identify relevant studies. The search was concluded up to January 2024. Studies comparing LTG with other antiseizure medications (ASMs) for treating epilepsy in pregnant women were included, with no language or regional restrictions. RESULTS: A total of 19 studies were included for analysis, with 16 studies reporting adverse pregnancy outcomes and 6 studies reporting seizure control outcomes. Meta-analysis showed that compared to monotherapy with carbamazepine (CBZ), sodium valproate (VPA), and levetiracetam (LEV), LTG monotherapy had a slightly weaker ability to control seizures during pregnancy, with ORs and 95 %CIs of 0.65 (0.57-0.75; CBZ), 0.50 (0.32-0.79; VPA), and 0.55 (0.36-0.84; LEV). Regarding adverse pregnancy outcomes, the occurrence rate of LTG monotherapy was significantly lower than that of CBZ, VPA, phenytoin (PHT), and phenobarbital (PHB), with ORs and 95 %CIs ranging from 0.30 (0.25-0.35; VPA) to 0.68 (0.56-0.81; CBZ). CONCLUSION: Based on meta-analysis, LTG and LEV appear to be preferred medications for controlling seizures during pregnancy. This review provides further support for the use of LTG monotherapy in pregnant WWE, building upon existing evidence for clinical practitioners.

15.
World Neurosurg ; 2024 Jun 28.
Article in English | MEDLINE | ID: mdl-38945210

ABSTRACT

Cerebrofacial venous metameric syndrome (CVMS) is a complex low-flow vascular malformation affecting bone and soft tissues, including brain, dura mater, and eye. We show images of CVMS in an 18-month-old boy presenting facial venous malformations, developmental venous anomalies, dural sinus malformations, and dilated great cerebral vein, suggesting a vein of Galen aneurysmal malformation. Although Sturge-Weber syndrome is the most known form of CVMS, its presentations are variable and include several venous malformations. Recognizing the various manifestations of CVMS is necessary for adequate screening, treatment, and follow-up.

16.
J Vasc Surg Venous Lymphat Disord ; : 101938, 2024 Jun 27.
Article in English | MEDLINE | ID: mdl-38944113

ABSTRACT

OBJECTIVE: The aim of this study is to share our experience in treating patients with LMs over a span of 14 years, evaluating its efficacy and safety, particularly with the use of ethanol as sclerosant of choice. METHODS: A retrospective review of pediatric patients diagnosed and later treated for LMs between 2008 and 2022 was conducted. We collected patient demographics, LM characteristics, treatment strategies and outcomes, including response to treatment and complications. RESULTS: The cohort included 36 patients (24 males), first presenting clinically at a median age of 5 months (range 0-12 years). LMs were macrocystic (17), microcystic (3), and mixed types (16). In most patients (22) the malformation involved the cervicofacial area. Twenty-five patients underwent 54 procedures, averaging 2 procedures per patient (range 1-13). Sclerotherapy resulted in 90% of patients exhibiting some response of the LM (p=0.005). Ethanol was used in most procedures (31) and proved most efficacious, facilitating partial or complete response of the malformations in all cases compared to 72% with other sclerosants (p=0.06). Sclerotherapy exhibited low complication rates among all sclerosants used (7%, p=0.74). CONCLUSION: Sclerotherapy is a safe and effective intervention for pediatric LMs. Ethanol demonstrated comparable efficacy and safety to other sclerosants, highlighting its potential as a preferred treatment option. This study supports the tailored use of sclerotherapy, guided by a thorough understanding of the risks and benefits, to provide optimized care for patients with LMs.

17.
Children (Basel) ; 11(6)2024 May 25.
Article in English | MEDLINE | ID: mdl-38929218

ABSTRACT

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.

18.
Life (Basel) ; 14(6)2024 May 23.
Article in English | MEDLINE | ID: mdl-38929654

ABSTRACT

This narrative review aims to summarise the classification of vascular anomalies, their clinical presentation, and their radiological features to propose a diagnostic algorithm to approach patients with suspected soft tissue vascular anomalies of the extremities. The management of vascular anomalies necessitates a multidisciplinary approach. Clinical presentation and physical examination are sufficient in most cases to achieve a correct diagnosis. This is especially true for small congenital lesions of the skin and subcutaneous tissue. Imaging is used for accurate characterization of these lesions, especially in cases of atypical or vague clinical presentation, and to assess extension in cases of lesions that are larger and localized in deeper tissues.

19.
Med Int (Lond) ; 4(4): 42, 2024.
Article in English | MEDLINE | ID: mdl-38912418

ABSTRACT

The present systematic review aimed to identify all the available literature on awake craniotomy (AC) in patients with arteriovenous malformation (AVM) in order to evaluate its safety, risks, benefits and effectiveness. All available literature on AC in patients with AVM was collected and evaluated in an aim to provide a better understanding of its safety, associated risks and benefits. A systematic search for studies employing AC in patients with AVM was conducted using the PubMed, Scopus and ScienceDirect databases without restrictions on the year of publication, language, or study design, from inception up to May 30, 2021. A total of 11 studies published between 2004 and 2021 with 106 patients who underwent ACs were considered eligible. The rate of complete resection was 93% [95% confidence interval (CI), 82 to 100%; I2 0%]. The intraoperative complication rate was 21% (95% CI, 1 to 41%; I2 55%) and the post-operative complication rate was 33% (95% CI, 19 to 48%; I2 40%). During follow-up, the complication rate was 6% (95% CI, 1 to 10%; I2 30%). The post-operative complication rate was higher in the Spetzler-Martin grade (SMG) III-V group (31%; 95% CI, 21 to 42%; I2 46%) than in the SMG I-II group (12%; 95% CI, 2 to 22%; I2 0%). Similarly, the follow-up complication rate was higher in the SMG III-V group (9%; 95% CI, 2 to 16%; I2 34%) than in the SMG I-II group (0%; 95% CI, 0 to 4%; I2 0%). On the whole, the present study provides preliminary evidence to indicate that AC is a possible and useful option for the resection of AVM in selected patients. Well-designed future studies with long-term follow-up are required however, to investigate various aspects of safety and provide solid data for AC in patients with AVM.

20.
Psychiatry Res ; 339: 116050, 2024 Jun 21.
Article in English | MEDLINE | ID: mdl-38914040

ABSTRACT

Previous research examining bipolar-disorder (BD) and pregnancy/neonatal outcomes yielded mixed results, were mostly derived from Western countries and rarely delineated effect between disorder and mood-stabilizers. This population-based study identified women age 15-50 years who delivered first/singleton child in 2003-2018 in Hong Kong, utilizing territory-wide medical-record database of public healthcare services. Propensity-score weighted logistic-regression analyses adjusted for confounders were employed to examine risk of adverse pregnancy, delivery and neonatal outcomes associated with BD and mood-stabilizers (lithium, anticonvulsants and antipsychotics). Exploratory unadjusted-analyses were conducted to assess risk for congenital-malformations. Of 465,069 women, 302 had BD-diagnosis, including 168 redeemed ≥ 1 prescription of mood-stabilizers during pregnancy (treated-BD) and 134 gestationally-unexposed to mood-stabilizers (untreated-BD). BD was significantly-associated with increased risk of gestational-diabetes (adjusted-odds-ratio: 1.75 [95 % CI: 1.15-2.70]) and maternal somatic hospitalization ≤ 90 days post-discharge from index-delivery (2.12 [1.19-3.90]). In treatment status-stratified analyses, treated-BD women exhibited significantly-increased rate of gestational-diabetes (2.09 [1.21-3.70]) relative to controls (non-BD and gestationally-unexposed to mood-stabilizers). No significant association of BD or mood-stabilizers with other adverse outcomes was observed. Overall, our findings indicate that BD and mood-stabilizers are not associated with most adverse pregnancy, delivery and neonatal outcomes. Further research clarifying comparative safety of individual mood-stabilizing agents on pregnancy/neonatal outcomes is required.

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