Anomalies In Pediatric Patients Research Articles

Airway Anomalies in Pediatric Patients After Surgery for Congenital Heart Disease: Single-Center Retrospective Cohort Study, Taiwan 2017-2020.

Airway anomalies increase risk of morbidity and mortality in postoperative pediatric patients with congenital heart disease (CHD). We aimed to identify airway anomalies and the association with intermediate outcomes in patients undergoing surgery for CHD. Single-center, hospital-based retrospective study in Taiwan, 2017-2020. A tertiary referral hospital in Taiwan. All pediatric patients who underwent surgery for CHD and were admitted to the PICU and had data about airway evaluation by cardiopulmonary CT scan or bronchoscopy. None. Among 820 CHD patients identified as having undergone airway evaluation in the PICU, 185 (22.6%) were diagnosed with airway anomalies, including structural lesions in 146 of 185 (78.9%), and dynamic problems were seen in 87 of 185 (47.0%). In this population, the explanatory factors associated with greater odds (odds ratio [OR]) of airway anomaly were premature birth (OR, 1.90; p = 0.002), genetic syndromes (OR, 2.60; p < 0.001), and in those with preoperative ventilator use (OR, 4.28; p < 0.001). In comparison to those without airway anomalies, the presence of airway anomalies was associated with higher hospital mortality (11.4% vs. 2.7%; p < 0.001), prolonged intubation days (8 d [1-27 d] vs. 1 d [1-5 d]; p < 0.001), longer PICU length of stay (23 d [8-81 d] vs. 7 d [4-18 d]; p < 0.001), and greater hazard of intermediate mortality (adjusted hazard ratio, 2.60; p = 0.001). In our single-center retrospective study, 2017-2020, between one-in-five and one-in-four of our postoperative CHD patients undergoing an airway evaluation had airway anomalies. Factors associated with greater odds of airway anomaly included, those with premature birth, or genetic syndromes, and preoperative ventilator use. Overall, in patients undergoing airway evaluation, the finding of an airway anomalies was associated with longer postoperative intubation duration and greater hazard of intermediate mortality.

Assessment of Cardiac Anomalies in Pediatric Patients with Pectus Carinatum

Background: Pectus carinatum (PC) is an anterior thoracic visible deformity manifested by protrusion of the sternum. It can be an isolated chest deformity or it can be seen together with genetic pathologies. Methods: We analyzed the cardiac findings of 40 children who were diagnosed with pectus carinatum at Kahramanmaras Sutcu Imam University’s Thoracic Surgery Outpatient Clinic between 1 February 2021 and 1 February 2022 and 97 healthy controls in similar age groups. Results: The Pectus Carinatum patients had higher rates of cardiac malposition, MVP (Mitral valve prolapse), MI (mitral insufficiency), TVP (tricuspid valve prolapse), CHD (congenital heart disease), and aortic valve pathology. Conclusıons: Our study showed that the prevalence of cardiac pathologies in the pediatric PC patients was higher than that in the control group consisting of healthy children. Thus, it may be recommend the referral of pediatric PC patients to cardiology outpatient clinics for the early diagnosis of potential cardiac pathologies.

Prevalence of Developmental Dental Anomalies in Pediatric Patients and an Assessment of Students’ Knowledge in Two Mexican Dental Schools: A Cross-Sectional Study

Dental anomalies (DAs) in children refer to conditions affecting the structure, number, shape, and size of teeth. This study aimed to assess the prevalence of these anomalies in both primary and permanent dentitions among two Mexican pediatric populations aged 2-14 years. Additionally, the level of knowledge about DAs among students pursuing a degree in dentistry in both universities was determined. A cross‑sectional study was conducted on children making their first visit to the Pediatric Dental Clinics of the Universities of Nuevo León and San Luis Potosí in México between March and May 2017. The prevalence of DAs was estimated. In addition, a questionnaire comprising 20 closed items regarding DAs was administered to 100 undergraduate students to evaluate their level of knowledge. Ninety-four children (54 boys and 42 girls; mean age 9.73±2.1 years) were included. Twenty-two patients (23.4%) exhibited at least one DA in their primary or permanent teeth. The most common DAs were in size (macrodontia) and structure (enamel hypoplasia). Agenesis occurred three times, and there were two cases of mesiodens. Regarding gender, 10 patients were girls (45.4%), and 12 were boys (54.6%). The prevalence of DAs in permanent teeth (57.5%) was higher than that in primary teeth (42.5%, p=0.031). Only two patients presented DAs in both primary and permanent teeth. No tooth-shaped anomalies were detected. Regarding the level of knowledge among the university students, the percentage of correct answers was 61.6%. Our findings emphasize the importance of encouraging parents to initiate pediatric dental visits early. Additionally, the study highlights the significance of conducting a thorough evaluation of the patients.

Late Presentation of Coarctation of the Aorta in an 11-Year-Old Male Child: A Case Study

Introduction: Coarctation of the aorta is a condition characterized by a narrowed segment of the aorta due to thickening of the artery wall. This case study highlights the late diagnosis of coarctation of the aorta in an 11-year-old male child who presented with symptoms initially suggestive of a respiratory infection. Case study: An 11-year-old male presented to the outpatient department with chief complaints of fever, cough, and poor appetite. He developed two episodes of epistaxis and hypertension with soft systolic murmur. Echocardiography showed severe coarctation of the aorta. Discussion: Coarctation of the aorta is a relatively common congenital heart defect that can remain asymptomatic until later in childhood or adulthood. It is the seventh most common type of CHD. The delay in the diagnosis of coarctation of the aorta underscores the challenges of recognizing cardiac anomalies in pediatric patients presenting with nonspecific complaints. Conclusion: Healthcare providers must remain vigilant and thorough in their assessment to prevent late diagnoses and associated complications in pediatric patients.

Prevalence of Anatomical Anomalies in Pediatric Patients Hospitalized with UTIs in a Tertiary Care Center's Urology and Nephrology Ward

Background: Urinary tract infections (UTIs) are a significant pediatric health concern, often linked to anatomical anomalies of the urinary tract. Understanding the prevalence and characteristics of these anomalies in children with UTIs is crucial for effective diagnosis and management. Objective: This study aimed to investigate the prevalence and nature of anatomical anomalies in children diagnosed with UTIs, focusing on age and gender distribution, and to identify common anomalies associated with UTIs in pediatric patients. Methods: A retrospective cross-sectional study was conducted at the Children's Medical Center in Tehran, from March to September 2022. The study included pediatric patients admitted with UTIs, excluding those over 18 years of age. Convenience sampling was used to target at least 70 patients with definitive anatomical anomalies. Data collection involved reviewing medical records and laboratory results, focusing on variables such as age, sex, paraclinical tests, and types of anatomical anomalies. Statistical analysis was performed using SPSS software version 25, employing Fisher’s exact or χ2 tests for categorical variables, and the student’s t-test, ANOVA, and Chi-Square tests for continuous variables. Results: The average age of patients was 29.61 months (SD = 35.28), with a median of 12.50 months (IQR = 36). The average number of UTI episodes was 3.43 (SD = 2.31), ranging from 1 to 10 episodes. Bilateral hydronephrosis was observed in 15 patients (21.7%), while neurogenic bladder and Reflux Grade 4 or 5 were identified in 11 (15.9%) and 14 (20.3%) patients, respectively. VCUG was performed in 63 patients (91.3%). Unilateral hydronephrosis was the most common imaging finding, observed in 33 patients (47.8%). Conclusion: The study concludes that children around 29 months of age, particularly females, are more prone to have anatomical anomalies associated with UTIs. Bilateral hydronephrosis was a prevalent anomaly. However, limitations such as potential data unavailability or misinterpretation, errors in laboratory data, and patient comorbidities must be considered when interpreting these findings.

Clinical effectiveness and safety of sirolimus in pediatric patients with complex vascular anomalies: necessitating personalized and comprehensive approaches.

Managing complex vascular anomalies in pediatric care requires comprehensive approaches. Sirolimus, an mTOR inhibitor with immunosuppressive and anti-angiogenic properties, offers promise. We evaluated sirolimus's effectiveness and safety in pediatric patients with complex vascular anomalies at a tertiary children's hospital. Our study included 20 patients, aged 1 month to 19 years, with diverse vascular anomalies resistant to conventional therapies or located in high-risk areas precluding surgery. The evaluation of response encompassed measuring the reduction in the size of the targeted vascular or lymphatic lesions as observed on radiologic imaging, along with considering improvements reported by the patients. Patients used sirolimus for a median of 2.1 years, ranging from 0.6-4.3 years. Results indicated that 60% of patients achieved complete or partial response (CR/PR), whereas 40% had stable disease (SD). Notably, no disease progression occurred. Lesion size assessment was complex, yet patients' self-reported improvements were considered. Three patients reinitiated sirolimus after discontinuation due to worsening lesions. Sirolimus treatment demonstrated good tolerability, with minor complications except for one case of Pneumocystis jiroveci pneumonia. Group comparisons based on response highlighted better outcomes in patients with vascular tumors (CR/PR group 58.0% vs. SD group 0.0%, P = 0.015) or localized measurable lesions (83.3% vs. 12.5%, P = 0.005). Our study underscores sirolimus's potential for treating complex vascular anomalies in pediatric patients. Challenges associated with optimal treatment duration and concurrent interventions necessitate a comprehensive approach and genetic testing to optimize outcomes.

Management of urachal anomalies in pediatric patients: A comparison of treatment strategies between pediatric urology and general surgery

Persistence of embryonic urachal structures due to a failure of the urachus to involute into the median umbilical ligament is known as a urachal anomaly (UA). UAs may remain asymptomatic or lead to abdominal pain and recurrent infections. Management of UAs in pediatric patients has historically lacked a clear consensus between conservative and surgical management. While both urologists and general surgeons manage this pathology, a comparison of management style and outcomes between these specialties has not been published to our knowledge. To (1) evaluate trends in management of UAs among pediatric urologists and general surgeons across three tertiary care children's hospitals and (2) identify factors that place patients at higher risk for requiring surgery. All patients diagnosed with a UA from 2016 to 2020at our multi-site institution were identified by ICD-10 code Q64.4 "malformation of the urachus" and retrospectively reviewed. Patient demographics, treatment specialty, remnant subtype, and management strategy were recorded. Data was dichotomized between both urology and general surgery as well as between surgical and nonsurgical intervention to identify and compare management strategies. Overall, 143 patients diagnosed with UAs were identified. Of these patients, 74 were treated by urology and 69 were treated by general surgery. Patients who were treated by urology were significantly more likely to receive conservative treatment (66.2% treated conservatively vs. 33.8% treated surgically), while patients treated by general surgery were significantly more likely to undergo surgery (84.1% treated surgically vs. 15.9% treated conservatively, p<.0001). Though, urology was more likely to treat patients who presented incidentally (p<.01), and general surgery was more likely to treat patients who presented with an infected remnant (p<.01). Patients of male sex were more likely overall to receive surgery compared to female patients (p<.01). Management of UAs by urologists was more conservative than general surgeons. However, both specialties treat distinctly different patient presentations, with urology managing more incidental remnants and general surgery operating on more emergent, infected urachi. Limitations of the study included its retrospective nature and the insufficient reporting of urachal remnant subtypes and presence of infection among patients. Management strategies of UAs differ among urology and general surgery, but surgical and conservative treatments are necessary to appropriately treat their distinct patient populations. This study provides valuable insight into current practices of UA management and may help to inform future treatment.

Root Anomalies after Combined Chemotherapy Treatment for Acute Lymphoblastic Leukemia during Childhood: A Case Report‬‬‬‬‬‬‬‬‬‬‬‬‬

Dental abnormalities have been sporadically reported in patients treated as children for acute lymphoblastic leukemia (ALL) with multi-agent chemotherapy, with and without radiation. Reports included delayed dental development, microdontia, hypoplasia, agenesis, V-shaped roots, shortened roots, and cervical root resorption. These anomalies in pediatric patients require regular dental follow-up and sometimes highly complex treatment. We describe a well-documented case of a 19-year-old male who presented with such anomalies 12 years after treatment with several chemotherapy agents for ALL, and discuss the resultant disruption of normal tooth development that led to the ensuing abnormalities.

Current management strategies of urachal anomalies in pediatric patients: A scoping review

IntroductionManagement of urachal anomalies in pediatric patients has historically lacked a clear consensus between conservative and surgical management. We aimed to review and summarize the literature on the diagnosis, symptoms, and evolution in the management of urachal anomalies in pediatric patients.MethodsWe performed a scoping literature review of PubMed/Medline and WebOfScience from January 2000 to February 2022.Results32 publications were selected for inclusion in this analysis. 1,438 unique studies were identified with 32 studies meeting inclusion criteria. 15/32 studies discussed both conservative and surgical management, 14/32 studies discussed only surgical management outcomes, and 3/32 studies discussed diagnostic methods. The studies discussing conservative management supported the treatment of urachal anomalies with an initial conservative approach, which includes watchful waiting, repeated ultrasounds, lesion measurement, and antibiotic use. 5/32 of the included studies identified patients that were converted from conservative to surgical management with conversion rates ranging from 12.5% to 43.5% per study. 14/20 converted patients were identified to have a urachal cyst and 13/20 had a persistent infection.ConclusionsStrong evidence exists that supports initial conservative management over surgical management of pediatric urachal anomalies. However, predictive factors for determining which patients will require surgical management remain elusive. Treatment algorithms can potentially be developed once carefully developed prospective studies delineate statistically significant patient factors which necessitate surgical management over observation.

Prevalence of dental anomalies in pediatric patients at Dental and Oral Hospital of Universitas Muhammadiyah Yogyakarta

Background: A dental abnormality is a deviation from the normal shape and structure of the teeth due to interference during growth and development. Various kinds of abnormalities occur, such as anomalies in the size, shape, position, number, and structure of the teeth. These conditions cause problems in the arch length and occlusion of the maxilla and mandible. Purpose: This study aimed to describe the prevalence of developmental dental anomalies in pediatric patients at the Dental and Oral Hospital of Universitas Muhammadiyah Yogyakarta (UMY) and its networks (Qatrunnada Kindergarten, Budi Mulia Dua Taman Siswa Kindergarten, and Muhammadiyah Sapen Pusat Primary School). Methods: A descriptive observational study with a cross-sectional design was conducted. There were 10,714 pediatric patients included. Results: The prevalence of developmental dental anomalies in pediatric patients at Dental and Oral Hospital, UMY and its network was 0.30%. The prevalence of mesiodens, hypodontia, and fusion dental anomalies were 0.14%, 0.056%, and 0.028%, respectively. There was a 0.019% prevalence of microdontia, peg tooth, and amelogenesis imperfecta. The prevalence of taurodontia and gemination was 0.009%. Conclusion: Dental anomalies occurred more frequently in male pediatric patients, and mesiodens was the most prevalent.

Dental Caries and Dental Anomalies in Children Undergoing Chemotherapy for Malignant Diseases.

The study aims to evaluate the prevalence of dental caries and anomalies in pediatric patients undergoing chemotherapy or those who have completed chemotherapy. A total of 250 pediatric patients within the age group of 6 months to 17 years, either admitted to hospitals for chemotherapy or on follow-up, were included as a part of the study. Complete oral examination inclusive of the diet history, oral hygiene methods, past dental history, the decayed, missing, and filled teeth (DMFT), def score, and any dental anomaly was diagnosed clinically and radiographically using an orthopantomogram. The samples were further categorized under type of malignancies and duration of chemotherapeutic drug usage groups (from 6 months to 10 years and more than 10 years) to establish a correlation of these variables with the prevalence of dental caries and dental anomalies. Among all patients, 108 (43.2%) had completed the treatment (chemotherapy), while 142 (56.8%) were undergoing the treatment. Forty-three (17.2%) patients showed positive findings for dental anomalies. The present study confirms the strong positive correlation between long-term exposure to chemotherapeutic agents and the prevalence of dental anomalies and dental caries in children. Talekar AL, Musale PK, Kothare SS. Dental Caries and Dental Anomalies in Children Undergoing Chemotherapy for Malignant Diseases. Int J Clin Pediatr Dent 2022;15(4):428-432.

Magnetic Resonance Imaging Findings and Accompanying Malformations in Pediatric Patients with Gray Matter Heterotopia

AbstractObjective: The aim of the study is to classify gray matter heterotopias detected in pediatric patients according to magnetic resonance imaging findings and to define the accompanying cerebral malformations.Material and Methods: Images of all pediatric patients who were detected to have heterotopia in brain magnetic resonance imaging between January 2012 and June 2020 were retrospectively evaluated. The type, location of heterotopia, and accompanying cerebral anomalies were analyzed.Results: A total of 42 patients, 22 male, and 20 female, with a mean age of 7.80±4.53 years (2-16 years) with gray matter heterotopia were included in the study. Of the patients 33 (78.6%) had subependymal, 7 (16.7%) had subcortical, and 2 (4.7%) had band heterotopia. Twenty-four patients had epilepsy.Conclusion: According to our study, subependymal heterotopia was most common in pediatric patients. The subependymal heterotopias were most commonly located in the trigon region of the lateral ventricles. All of the subcortical heterotopies were located in the frontal and unifocal. Band heterotopias were located symmetrically in the subcortical region of the bilateral cerebral hemispheres. The main accompanying anomalies were Chiari II malformation, and corpus callosum agenesis. Defining the type of heterotopia and associated anomalies in pediatric patients is important for patient management.

Testicular, Epididymal and Vasal Anomalies in Pediatric Patients with Cryptorchid Testes and Testes with Communicating Hydrocele.

The goal of this study was to determine the prevalence of the testicular, epididymal, and vasal anomalies (TEVA) in cryptorchid and communicating hydrocele pediatric patients. Six hundred and ninety-one prepubertal boys underwent inguinal exploration for 741 undescended (UDT) or hydrocele testes. Two hundred and fifty-five TEVA were detected in 154 UDT boys, compared to 32 defects in 24 hydrocele patients (p < 0.001). The TEVA were more frequent in bilateral UDT (p = 0.009). Multiple defects were observed more frequently in the intra-abdominal testicles (p = 0.028). A correlation was found between the testicular atrophy index (TAI) and the incidence and number of TEVA in the UDT boys (p < 0.001). The smaller the testis (higher TAI), the more the defects that appeared in it and the higher the frequency of their appearance. Another correlation was established between testis position and the incidence and number of TEVA (p < 0.001). The higher the testis position, the more the defects that appeared in it and the higher the frequency of their appearance. A correlation was established between the position and the volume of the affected testis (p < 0.001). The higher the gonad position, the more severe the atrophy observed in it. The TEVA were more frequent in the UDT boys than in the hydrocele patients. We revealed that the risk of abnormal fusion between the testis, epididymis, and vas deferens is connected with the testis position (intra-abdominal testes) and bilateral non-descent.

The diagnostic accuracy of ultrasound and upper gastrointestinal contrast studies for locating atresia/stenosis and intestinal malrotation and detecting annular pancreas in pediatric patients with duodenal atresia/stenosis.

This study aimed to evaluate the diagnostic performance of ultrasound to locate atresia/stenosis and other abdominal anomalies in pediatric patients with duodenal atresia/stenosis, including intestinal malrotation and annular pancreas. We classified 36 retrospective cases of duodenal atresia/stenosis based on intestinal malrotation status, and evaluated the diagnostic performance of ultrasound/upper gastrointestinal contrast studies to locate atresia/stenosis and intestinal malrotation, as well as ultrasound for detecting additional anomalies such as annular pancreas. The incidence of annular pancreas was compared between groups using Fisher's exact test. Atresia/stenosis was correctly located by ultrasound in 33 (91.7%) cases and by upper gastrointestinal contrast study in 36 (100%) cases. Of the eight cases with intestinal malrotation, five and two were correctly diagnosed by ultrasound and upper gastrointestinal contrast study, respectively. Ultrasound correctly diagnosed annular pancreas in 6/14 cases. The incidence of annular pancreas was significantly different between the groups (present/absent in groups with vs. without intestinal malrotation: 6/2 vs. 8/20, P = 0.036). Ultrasound has a relatively high capability in locating atresia/stenosis. However, some cases are misdiagnosed. In clinical practice, upper gastrointestinal contrast studies should be used complementarily during diagnosis. Additional anomalies may not be detected by preoperative examinations; therefore, surgeons should carefully evaluate for additional anomalies during surgery, especially coexisting intestinal malrotation and annular pancreas.

Multi-detector computed tomography (MDCT) as a diagnostic tool in assessment of thoracic aortic anomalies in pediatric patients

BackgroundA wide variety of congenital thoracic aortic variants and pathological anomalies could be assessed recently in diagnostic and interventional radiology. Multi-detector computed tomography (MDCT) is one of the most important non-invasive diagnostic tools for their detection. The aim of the study was to evaluate role of MDCT scanning for diagnosis of thoracic aortic anatomic variants and diseases in pediatric patients.ResultsThirty patients (15 male and 15 female), mean age (8.49 ± 20.29 months) were diagnosed with different thoracic aortic anomalies by MDCT then confirmed by surgical results. MDCT was more sensitive than echocardiography in detection of hypo plastic arch, vascular rings, interrupted aortic arch anomalies, and aortic coarctation. Both MDCT and echocardiography showed 100% sensitivity in their detection of TGA, TOF, and PDA. MDCT detected 6 cases of right-sided aortic arch while echo missed 2 cases. Different aortic arch branching patterns and coronary origin were better demonstrated by MDCT.Conclusion320-Multi-detector computed tomography is a reliable tool for optimal detection of thoracic aortic anomalies and preoperative planning.

The Application of Retrospective ECG Gated 320-MSCT Angiography in Diagnosis of Congenital Pulmonary Arterial Anomalies in Pediatrics

Congenital pulmonary arteries anomalies included a wide-ranged spectrum of pathology that is usually associated with other congenital heart diseases. These anomalies can be classified into several major categories, which show considerable overlap among those categories. The aim of this study is to evaluate the role of multi-slice computed tomography (MSCT) angiography in assessment of pulmonary arteries anomalies. This study included 76 patients (42 males and 34 females) with male to female ratio 55.2% to 44.7%. The age of the patients ranged from 4 days to 15 years. The studied cases for pulmonary arteries anomalies were subclassified to anomalies of the caliber, origin and development (conotruncal anomalies). We compared MSCT findings with the data collected by cardiac catheterization and/or operation in 38 patients. 320-MSCT diagnosed cases of pulmonary arterial anomalies with 99% sensitivity, 99.8% specificity, 99% PPV, 99.8% NPV and 93.4% accuracy. This study concluded that MSCT is a non-invasive imaging modality that has a significant value in the evaluation of the congenital pulmonary arteries anomalies and its associated extracardiac anomalies in pediatric patients as well as assessment of post-operative complications. It is superior to ECHO in evaluating the pulmonary artery anomalies specially the pulmonary artery branches which are obscured by aerated lung. Also, there is good outcome of the cases owing to early and accurate diagnosis of the cases and post-operative follow up.

Two-Stage Approach for Unstable Pediatric Craniocervical Junction Anomalies with a Halo Vest and Delayed Occipitocervical Fusion: Technical Note, Case Series, and Literature Review

Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes. A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis. Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from-3.26 mm to-6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41). The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

Marfan syndrome in children: correlation between musculoskeletal features and cardiac Z-score

We investigated the correlation between the musculoskeletal features and the cardiovascular anomalies in pediatric patients affected by Marfan syndrome, in order to identify possible orthopedic deformities that could be a warning sign for severe aortic dilatation. Moreover, we analyzed the role of the orthopedic aspects in the early diagnosis of the disease in a pediatric population. Seventy-two patients from 3 to 14 years of age, underwent interdisciplinary evaluation that included an orthopedic and cardiological examination. At the orthopedic examination, we analyzed the musculoskeletal features included in the systemic score of the revised Ghent criteria. Cardiological evaluation included a transthoracic echocardiography with definition of the cardiac Z-score, which is an index that evaluates aortic diameter. A statistical analysis was performed. We identified a statistically significant correlation between the presence of pectus excavatum and cardiac Z-score ≥3 (P = 0.022). Clinically, this data means that pectus excavatum is frequently observed in patients with larger aortic root diameter. On the contrary, no statistically significant correlation was found between the other investigated musculoskeletal features and a pathological Z-score. In the pediatric population, the diagnosis of Marfan syndrome remains difficult because many clinical manifestations are age-dependent and the Ghent criteria, usually used for adults, are not reliable in children. Our results show that the presence of pectus excavatum could help in the early identification of patients at greater risk of developing possibly fatal aortic disease. However, it is always indicated to screen all patients with Marfan syndrome for cardiac abnormalities, even in absence of pectus excavatum.

MDCT angiographic findings of various congenital pulmonary artery anomalies in pediatric patients

BackgroundCongenital pulmonary artery anomalies are variable and need proper diagnosis and treatment. CT angiography with multiplanar reconstruction has the main role in the assessment of these anomalies and this non-invasive method should be the method of choice for preoperative planning and postoperative follow up.The aim of the study is to assess the value of MDCT in the detection of pulmonary arteries anomalies in the pediatric population with complex congenital heart disease in conjunction with echocardiography as an alternative to conventional angiography and to determine the superiority of MDCT in the assessment of other abnormalities such as airway anomalies.ResultsIn our retrospective study, 52 patients (28 male and 24 females, aged 1 day to 4 years: mean age 2 years) were examined with contrast-enhanced CT. CT examinations were done using a 128-section CT scanner (Siemens Somatom Definition AS) using non-ionic iodinated contrast media. 2D and 3D reconstructions were performed. The correlation was made with echocardiograms. All imaging studies were reviewed. The echo was done to all patients. Surgery and/or catheter angiography performed to all patients, their findings were reviewed and compared to CTA findings. Other abnormalities such as congenital airway anomalies are detected using axial MDCT images and reconstructed imaging techniques.MDCT was accurate in revealing pulmonary artery anomalies. The commonest pulmonary artery anomaly was atresia, stenosis then hypoplasia. These anomalies may be isolated or associated with other congenital heart diseases. In the current study, MDCT could diagnose all cases of pulmonary arterial anomalies with 96% sensitivity, 100% specificity, 98% accuracy, 100% positive, and 94% negative predictive values. CT scans provide accurate information to assess complex spatial relationships of vascular airway compression frequently associated with CHD in the pediatric population.ConclusionMDCT scanner can be an alternative to diagnostic conventional angiography for the non-invasive assessment of the pulmonary artery. Higher quality multiplanar and 3D reconstruction achieved by the MDCT scanners offer a rapid, reliable and non-invasive technique that can be used for the evaluation and preoperative assessment of thoracic vascular and extra-vascular anatomy in infants and children with suspected congenital heart disease. CT technologies are constantly developing collaboration between radiologists, pediatric cardiologists, and anesthesiologists, which is essential for improving CT performance.

Diagnostic Accuracy of Noncontrast Self-navigated Free-breathing MR Angiography versus CT Angiography: A Prospective Study in Pediatric Patients with Suspected Anomalous Coronary Arteries

To evaluate the diagnostic accuracy of a prototype noncontrast, free-breathing, self-navigated 3D (SN3D) MR angiography (MRA) technique for the assessment of coronary artery anatomy in children with known or suspected coronary anomalies, using CT angiography (CTA) as the reference standard. Twenty-one children (15 male, 12.3 ± 2.6 years) were prospectively enrolled between July 2014 and August 2016 in this IRB-approved, HIPAA-compliant study. Patients underwent same-day unenhanced SN3D-MRA and contrast-enhanced CTA. Two observers rated the visualization of coronary artery segments and diagnostic confidence on a 3-point scale and assessed coronary arteries for anomalous origin, as well as interarterial and intramural course. Sensitivity, specificity, positive (PPV) and negative predictive values (NPV) of SN3D-MRA for the detection of coronary artery abnormalities were calculated. Interobserver agreement was assessed using Intraclass Correlation Coefficients (ICC). Fourteen children showed coronary artery abnormalities on CTA. The visualization of coronary segments was rated significantly higher for CTA compared to MRA (p <0.015), except for the left main coronary artery (p = 0.301), with good to excellent interobserver agreement (ICC = 0.62-0.94). Diagnostic confidence was higher for CTA (p = 0.046). Sensitivity, specificity, PPV, and NPV of MRA were 92%, 92%, 96%, and 87% for the detection of coronary artery anomalies, 85%, 85%, 74%, and 92% for high origin, 71%, 92%, 82%, and 87% for interarterial, and 41%, 96%, 87%, and 80% for intramural course. Noncontrast SN3D-MRA is highly accurate for the detection of coronary artery anomalies in pediatric patients while diagnostic confidence and coronary artery visualization remain superior with CTA.