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1.
Pediatr Radiol ; 54(2): 199-207, 2024 02.
Artículo en Inglés | MEDLINE | ID: mdl-38191808

RESUMEN

BACKGROUND: Mutations in the T-Box 4 (TBX4) gene are a lesser-known cause of heritable pulmonary arterial hypertension (PAH). Patients with heritable PAH typically have worse outcomes when compared with patients with idiopathic PAH, yet little is known about the phenotypical presentation of this mutation. OBJECTIVE: This article reviews the pattern of chest CT findings in pediatric patients with PAH and TBX4 mutations and compares their radiographic presentation with those of age-matched patients with PAH but without TBX4 mutations. MATERIALS AND METHODS: A retrospective chart review of the pulmonary arterial hypertension database was performed. Pediatric patients with PAH-confirmed TBX4 mutations and an available high CT were included. Fifteen (9 females) patients met the inclusion criteria. Fourteen (8 females) age-matched controls with diagnosed PAH but without TBX4 mutations were also evaluated. The median age at diagnosis was 7.4 years (range: 0.1-16.4 years). Demographic information and clinical outcomes were collected. CTs of the chest were reviewed for multiple airway, parenchymal, and structural abnormalities (16 imaging findings in total). Chi-square tests were used to compare the prevalence of each imaging finding in the TBX4 cohort compared to the control group. RESULTS: Patients with TBX-4 mutations had increased presence of peripheral or subpleural irregularity (73% vs 0%, P < 0.01), cystic lucencies (67% vs 7%, P < 0.01), and linear or reticular opacity (53% vs 0%, P < 0.01) compared to the control group. Ground glass opacities, bronchiectasis, and centrilobular nodules were not significantly different between the two patient groups (P > 0.05). CONCLUSION: TBX4 mutations have distinct imaging phenotypes in pediatric patients with PAH. Compared to patients without this mutation, patients with TBX-4 genes typically present with peripheral or subpleural irregularity, cystic lucencies, and linear or reticular opacity.


Asunto(s)
Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Femenino , Humanos , Niño , Lactante , Preescolar , Adolescente , Estudios Retrospectivos , Arteria Pulmonar , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/genética , Hipertensión Pulmonar Primaria Familiar/genética , Mutación , Tomografía Computarizada por Rayos X , Proteínas de Dominio T Box/genética
2.
Dig Dis Sci ; 68(3): 877-888, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-35790702

RESUMEN

BACKGROUND: Internally penetrating Crohn's Disease complications, including abscesses and phlegmon, represent a high-risk Crohn's Disease phenotype. Anti-tumor-necrosis-factor-α (Anti-TNF) therapies are effective in treating penetrating Crohn's Disease and early initiation has shown unique benefits. However, timing of anti-TNF initiation in the setting of internally penetrating Crohn's Disease complications is typically heterogenous due to concern over precipitating serious infections. Recent studies demonstrate such an association may not exist. AIMS: We aimed to describe the multidisciplinary management of pediatric patients with internally penetrating Crohn's Disease complications, focusing on the utilization and timing of anti-TNF therapy relative to complication resolution and adverse events. METHODS: We performed a single-center retrospective cohort study of pediatric patients with internally penetrating Crohn's Disease complications from 2007 to 2021. The safety and effectiveness of anti-TNF therapy initiation prior to complication resolution was assessed by comparing rates of infectious and Crohn's Disease-related adverse events between those who received anti-TNF therapy prior to complication resolution, versus those who did not. RESULTS: Twenty-one patients with internally penetrating Crohn's Disease complications were identified. 7/21 received anti-TNF therapy prior to complication resolution. Infectious adverse events within 90 days of complication occurred in 0/7 patients initiating anti-TNF therapy prior to complication resolution and 10/14 patients who did not (p = 0.004). Crohn's Disease-related surgeries and hospitalizations within 1 year of complication occurred in 12/20 patients, with similar frequency between groups. CONCLUSIONS: Initiating anti-TNF therapy prior to internally penetrating Crohn's Disease complication resolution may be a safe and effective strategy to improve clinical outcomes.


Asunto(s)
Absceso Abdominal , Enfermedad de Crohn , Humanos , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/tratamiento farmacológico , Enfermedad de Crohn/cirugía , Infliximab/uso terapéutico , Inhibidores del Factor de Necrosis Tumoral/efectos adversos , Estudios Retrospectivos , Celulitis (Flemón)/tratamiento farmacológico , Celulitis (Flemón)/epidemiología , Celulitis (Flemón)/complicaciones , Factor de Necrosis Tumoral alfa , Absceso Abdominal/epidemiología , Absceso Abdominal/etiología , Necrosis
3.
Pediatr Radiol ; 53(10): 2069-2078, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37341726

RESUMEN

BACKGROUND: CT is the standard imaging technique to evaluate pediatric sinuses. Given the potential risks of radiation exposure in children, it is important to reduce pediatric CT dose and maintain image quality. OBJECTIVE: To study the utility of spectral shaping with tin filtration to improve dose efficiency for pediatric sinus CT exams. MATERIALS AND METHODS: A head phantom was scanned on a commercial dual-source CT using a conventional protocol (120 kV) and a proposed 100 kV with a 0.4-mm tin filter (Sn100 kV) protocol for comparison. Entrance point dose (EPD) of eye and parotid gland region was measured by an ion chamber. Sixty pediatric sinus CT exams (33 acquired with 120 kV, 27 acquired with Sn100 kV) were retrospectively collected. All patient images were objectively measured for image quality and blindly reviewed by 4 pediatric neuroradiologists for overall noise, overall diagnostic quality, and delineation of 4 critical paranasal sinus structures, using a 5-point Likert scale. RESULTS: Phantom CTDIvol from Sn100 kV is 4.35 mGy, compared to CTDIvol of 5.73 mGy from 120 kV at an identical noise level. EPD of sensitive organs decreases in Sn100 kV (e.g., right eye EPD 3.83±0.42 mGy), compared to 120 kV (5.26±0.24 mGy). Patients in the 2 protocol groups were age and weight (unpaired T test P>0.05) matched. The patient CTDIvol of Sn100 kV (4.45±0.47 mGy) is significantly lower than 120 kV (5.56±0.48 mGy, unpaired T test P<0.001). No statistically significant difference for any subjective readers' score (Wilcoxon test P>0.05) was found between the two groups, indicating proposed spectral shaping provides equivalent diagnostic image quality. CONCLUSION: Phantom and patient results demonstrate that spectral shaping can significantly reduce radiation dose for non-contrast pediatric sinus CT without compromising diagnostic quality.


Asunto(s)
Estaño , Tomografía Computarizada por Rayos X , Humanos , Niño , Tomografía Computarizada por Rayos X/métodos , Estudios Retrospectivos , Reducción Gradual de Medicamentos , Dosis de Radiación
4.
Pediatr Radiol ; 53(5): 885-891, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-36697721

RESUMEN

BACKGROUND: Modern CT scanners with lower radiation doses have resulted in large numbers of cardiac CTs being performed in children. As seen in adults, pediatric cardiac CT has the potential to demonstrate extracardiac variants and pathology that can occur in conjunction with congenital heart disease (CHD). Prior publications demonstrated a high incidence of extracardiac findings in various locations but the prevalence of urgent unexpected extracardiac findings in children is unknown. OBJECTIVE: The purpose of this study was to describe the incidence, distribution and clinical significance of the extracardiac findings on pediatric cardiac CT at a tertiary referral center. MATERIALS AND METHODS: We reviewed all reports (n = 648) for 554 children through young adults who received a cardiac CT study between Jan. 2, 2018, and March 10, 2020, at our tertiary referral pediatric hospital. We interrogated CT reports for extracardiac findings and categorized them by system (airway, pulmonary, abdomen, malpositioned lines and musculoskeletal). We then subclassified each of these findings by level of clinical importance based upon the need for intervention or treatment into low, medium or high importance. High-importance findings were confirmed with a focused chart review. If a patient had more than one CT with a persistent extracardiac finding, the finding was only counted once. RESULTS: We identified 562 individual extracardiac findings, with one or more extracardiac findings present in 91% of the study population. Extracardiac findings with high clinical importance, requiring urgent attention or intervention, were present in 10% (57/554) of cases. The most common location of extracardiac findings was pulmonary (50%; 280/562), followed by airway (22%; 125/562) and abdomen (9%; 52/562). CONCLUSION: Unexpected highly important extracardiac findings were found in 10% of patients. Therefore, extracardiac structures should be scrutinized for the timely identification of potentially highly important findings.


Asunto(s)
Cardiopatías Congénitas , Tomografía Computarizada por Rayos X , Recién Nacido , Humanos , Niño , Adulto Joven , Tomografía Computarizada por Rayos X/métodos , Cardiopatías Congénitas/diagnóstico por imagen , Corazón , Prevalencia , Estudios Retrospectivos
5.
Pediatr Radiol ; 53(9): 1874-1884, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37106091

RESUMEN

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.


Asunto(s)
Displasia Broncopulmonar , Cardiopatías Congénitas , Hipertensión Pulmonar , Venas Pulmonares , Estenosis de Vena Pulmonar , Recién Nacido , Lactante , Humanos , Masculino , Niño , Femenino , Estenosis de Vena Pulmonar/diagnóstico por imagen , Estenosis de Vena Pulmonar/complicaciones , Recien Nacido Prematuro , Venas Pulmonares/diagnóstico por imagen , Venas Pulmonares/anomalías , Cardiopatías Congénitas/complicaciones , Tomografía Computarizada por Rayos X , Pulmón/diagnóstico por imagen , Estudios Retrospectivos
6.
Pediatr Radiol ; 52(10): 1888-1894, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35648162

RESUMEN

Although rare in the pediatric population, pulmonary hypertension is a significant cause of morbidity and mortality in affected individuals. In addition to evaluating potential causes and severity of parenchymal lung diseases, non-contrast high-resolution CT of the chest can aid in the diagnosis of heritable and acquired causes. In addition to evaluating parenchymal lung disease, CT angiography can help to confirm findings of pulmonary hypertension using criteria similar to echocardiography, and provide detailed assessment of the pulmonary vascularity in specific causes.


Asunto(s)
Hipertensión Pulmonar , Enfermedades Pulmonares , Niño , Angiografía por Tomografía Computarizada , Ecocardiografía , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Enfermedades Pulmonares/complicaciones , Arteria Pulmonar/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos
7.
Pediatr Radiol ; 52(13): 2549-2556, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-35689704

RESUMEN

BACKGROUND: Germline mutation in bone morphogenetic protein type II (BMPR2) is the most common cause of idiopathic/heritable pulmonary hypertension in pediatric patients. Despite the discovery of this gene there are no known descriptions of the CT or CT angiography findings in these children. OBJECTIVE: To correlate the clinical presentation, pathology and chest CT findings in pediatric patients with pulmonary hypertension caused by mutations in the BMPR2 gene. MATERIALS AND METHODS: We performed a search to identify pediatric patients with a BMPR2 mutation and CT or CT angiography with the clinical history of pulmonary hypertension. Three pediatric radiologists reviewed the children's CT imaging findings and ranked the dominant findings in order of prevalence via consensus. RESULTS: We identified three children with pulmonary hypertension and confirmed germline BMPR2 mutations, two of whom had undergone lung biopsy. We then correlated the imaging findings with histopathology and clinical course. CONCLUSION: All of our patients with BMPR2 mutations demonstrated a distinct CT pattern of ground-glass nodules with a prominent central enhancing vessel/nodule. These findings correlated well with the pathological findings of plexogenic arteriopathy.


Asunto(s)
Hipertensión Pulmonar , Humanos , Niño , Hipertensión Pulmonar/genética , Mutación , Hipertensión Pulmonar Primaria Familiar , Tomografía Computarizada por Rayos X , Receptores de Proteínas Morfogenéticas Óseas de Tipo II/genética
8.
J Pediatr ; 236: 307-311, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-34022249

RESUMEN

We reviewed patients with Stevens-Johnson syndrome (SJS) evaluated at Children's Hospital Colorado and investigated the occurrence of bronchiolitis obliterans (BO). Approximately 9% of patients with SJS developed BO. Pediatricians should consider monitoring patients with SJS for BO, especially those with recurrent SJS and patients treated with mechanical ventilation.


Asunto(s)
Bronquiolitis Obliterante/complicaciones , Síndrome de Stevens-Johnson/complicaciones , Bronquiolitis Obliterante/diagnóstico por imagen , Niño , Femenino , Humanos , Pulmón/diagnóstico por imagen , Masculino , Respiración Artificial , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
10.
Pediatr Radiol ; 50(10): 1375-1380, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32696109

RESUMEN

BACKGROUND: As CT technology has advanced, techniques for pediatric cardiac CT in congenital heart disease have evolved from retrospective electrocardiography (ECG)-gating with relatively high radiation doses to lower-dose prospective ECG-gating and even single-beat gated scans. Despite these advances, coronary artery imaging in children remains challenging because of their small vessel size and high heart rates, often necessitating retrospective gating. OBJECTIVE: Evaluate coronary artery visualization in pediatric patients (<20 years) who underwent low-dose high-pitch ECG-triggered scans and stratify the probability of coronary artery visualization based upon heart rate and body surface area (BSA). MATERIALS AND METHODS: Two hundred eleven high-pitch ECG-triggered studies from April 2014 to November 2017 were reviewed by two pediatric cardiac imagers in this retrospective study. Patient age, gender, BSA, average heart rate, heart rate variability and use of general anesthesia were recorded as well as dose-length product (DLP) and volumetric CT dose index (CTDIvol). We assessed the coronary artery score using a 5-point scale, with score of ≥3 considered of diagnostic quality. We performed multivariate statistical analysis including logistic regression to analyze effects of heart rate and BSA. RESULTS: Patient age range was 1 day to 19 years (median age 3 years). Heart rate range was 49-188 beats per minute (bpm; median 122 bpm) and BSA range was 0.15-2.07 m2 (median 0.53 m2). The origin and proximal coronary arteries were confidently seen (score ≥3) in 61% of studies in this cohort. Coronary artery visualization scores further increased with increased BSA (P<0.002) and with decreased heart rate (P<0.001). At heart rates <100 bpm or in patients with BSA>0.58, adequate coronary artery visualization was present 72% of the time. CONCLUSION: While in many patients the coronary artery origins are visualized using high-pitch ECG-triggered technique, the importance of coronary artery visualization needs to be weighed with the radiation dose penalty in individual patients to achieve optimal imaging.


Asunto(s)
Superficie Corporal , Técnicas de Imagen Sincronizada Cardíacas/métodos , Vasos Coronarios/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Frecuencia Cardíaca , Tomografía Computarizada por Rayos X/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Adulto Joven
11.
J Pediatr ; 210: 106-111, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31005280

RESUMEN

OBJECTIVE: To describe the clinical features of children who presented to Children's Hospital Colorado (CHCO) with high-altitude pulmonary edema (HAPE). STUDY DESIGN: We performed a retrospective chart review in children discharged from CHCO (an elevation of 1668 m) with a clinical diagnosis of HAPE and a chest radiograph consistent with noncardiogenic pulmonary edema. Descriptive statistics were used to describe the demographics, presentations, and treatment strategies. RESULTS: From 2004 to 2014, 50 children presented to CHCO who were found to have a clinical diagnosis of HAPE and a chest radiograph consistent with noncardiogenic pulmonary edema. Most (72%) patients were male, and most (60%) of the children in the study were diagnosed with classic HAPE, 38% with re-entry HAPE, and 2% with high altitude resident pulmonary edema. Elevation at symptom presentation ranged from 1840 to 3536 m. Patients were treated with a variety of medications, including diuretics, steroids, and antibiotics. Four patients were newly diagnosed with structural heart findings: 2 patients with patent foramen ovale and 2 with atrial septal defects. Eleven patients had findings consistent with pulmonary hypertension at the time of echocardiography. CONCLUSIONS: HAPE symptoms may develop below 2500 m, so providers should not rule out HAPE based on elevation alone. Structural heart findings and pulmonary hypertension are associated with HAPE susceptibility and their presence may inform treatment. Inappropriate use of antibiotics and diuretics in children with HAPE suggest that further education of providers is warranted.


Asunto(s)
Mal de Altura/diagnóstico , Altitud , Hipertensión Pulmonar/diagnóstico , Edema Pulmonar/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Adulto Joven
12.
Pediatr Radiol ; 49(7): 869-875, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30927038

RESUMEN

BACKGROUND: The expression of the NKX2-1 gene and its encoded protein, thyroid transcription factor 1 (TTF-1), plays a role in pulmonary surfactant homeostasis and lung development. NKX2-1 mutations have been associated with neonatal respiratory distress, hypotonia, choreoathetosis and congenital hypothyroidism. These clinical findings have been coined brain-lung-thyroid syndrome, although not all three organs are always involved. While many of these children develop interstitial lung disease, no systematic review of chest high-resolution CT (HRCT) findings has been reported. OBJECTIVE: To summarize the clinical presentations, pathology and HRCT imaging findings of children with NKX2-1 mutations. MATERIALS AND METHODS: We identified six children with NKX2-1 mutations, deletions or duplications confirmed via genetic testing at our institution. Three pediatric radiologists reviewed the children's HRCT imaging findings and ranked the dominant findings in order of prevalence via consensus. We then correlated the imaging findings with histopathology and clinical course. RESULTS: All children in the study were heterozygous for NKX2-1 mutations, deletions or duplications. Ground-glass opacities were the most common imaging feature, present in all but one child. Consolidation was also a prevalent finding in 4/6 of the children. Architectural distortion was less common. CONCLUSION: HRCT findings of TTF-1 deficiency are heterogeneous and evolve over time. There is significant overlap between the HRCT findings of TTF-1 deficiency, other surfactant dysfunction mutations, and pulmonary interstitial glycogenosis. TTF-1 deficiency should be considered in term infants presenting with interstitial lung disease, especially if hypotonia or hypothyroidism is present.


Asunto(s)
Atetosis/diagnóstico por imagen , Atetosis/genética , Corea/diagnóstico por imagen , Corea/genética , Hipotiroidismo Congénito/diagnóstico por imagen , Hipotiroidismo Congénito/genética , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/genética , Síndrome de Dificultad Respiratoria del Recién Nacido/diagnóstico por imagen , Síndrome de Dificultad Respiratoria del Recién Nacido/genética , Factor Nuclear Tiroideo 1/genética , Tomografía Computarizada por Rayos X/métodos , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Factor Nuclear Tiroideo 1/deficiencia
13.
Pediatr Radiol ; 48(8): 1066-1072, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29687227

RESUMEN

BACKGROUND: Pulmonary interstitial glycogenosis is a form of childhood interstitial lung disease characterized by the histological finding of abundant glycogen-laden mesenchymal cells within the pulmonary interstitium. Patients present in the neonatal period with disproportionate respiratory distress. Often, pulmonary interstitial glycogenosis is accompanied by alveolar simplification complicating recognition and diagnosis. Despite the recognition of pulmonary interstitial glycogenosis as a distinct entity, only a few case reports describing imaging findings are found in the literature, with no published systematic review available. OBJECTIVE: The purpose of this review is to provide a review of CT findings of pulmonary interstitial glycogenosis with histological correlation to aid in early diagnosis and management. MATERIALS AND METHODS: A 10-year retrospective review was performed to identify pediatric patients <18 years who underwent biopsy and CT within the last 10 years at our institution. The inclusion criteria include patients who had a CT within 3 months of biopsy and pathology-proven pulmonary interstitial glycogenosis CTs that were evaluated by three radiologists using a standardized scoring system. RESULTS: Fifteen patients met inclusion criteria (9 male, 6 female). At the time of initial pre-biopsy CT, ages ranged from 2 weeks to 5 months. Pulmonary symptoms presented at birth in the majority of patients (n=13). Two patients presented in early infancy at 3 months (n=1) and 5 months (n=1). Ground glass opacities were the most common CT finding (n=14), which varied from diffuse to scattered. Cystic lucencies (n=11) were noted in the majority of patients as well. Interlobular septal thickening (n=10) and architectural distortion (n=8) were less common findings. CONCLUSION: The most common CT findings of pulmonary interstitial glycogenosis are ground glass opacities with cystic lucencies. While the imaging findings are distinct from the typical presentation of neuroendocrine hyperplasia of infancy, there is significant overlap of these findings with surfactant dysfunction mutations, entities that also present with respiratory distress in the neonatal period. Therefore, imaging findings in pulmonary interstitial glycogenosis are helpful in guiding the need for genetic testing and/or biopsy.


Asunto(s)
Enfermedad del Almacenamiento de Glucógeno/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Adolescente , Biopsia , Niño , Preescolar , Femenino , Enfermedad del Almacenamiento de Glucógeno/patología , Humanos , Lactante , Recién Nacido , Enfermedades Pulmonares Intersticiales/patología , Masculino
14.
Pediatr Radiol ; 48(12): 1745-1754, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-29955904

RESUMEN

BACKGROUND: Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare lung disease associated with significant air trapping. Although chest CT is crucial in establishing a diagnosis, CT and biopsy findings do not reveal airway abnormalities to explain the air trapping. OBJECTIVE: We compared lung and airway morphology obtained from chest CT scans in children with NEHI and control children. In the children with NEHI, we explored relationships between lung and airway shape and lung function. MATERIALS AND METHODS: We performed a retrospective review of children with NEHI who underwent clinical chest CT. We identified control children of similar size and age. We created lung masks and airway skeletons using semi-automated software and compared them using statistical shape modeling methods. Then we calculated a logistic regression model using lung and airway shape to differentiate NEHI from controls, and we compared shape model parameters to lung function measurements. RESULTS: Airway and lung shapes were statistically different between children with NEHI and controls. We noted a broad lung apex in the children with NEHI and a significantly increased apical anterior-posterior lung diameter. A logistic regression model including lung shape was 90% accurate in differentiating children with NEHI from controls. Correlation coefficients were significant between lung function values and lung and airway shape. CONCLUSION: Lung and airway shapes were different between children with NEHI and control children in this cohort. Children with NEHI had an increased anteroposterior diameter of their lungs that might be useful in the diagnostic criteria.


Asunto(s)
Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Células Neuroendocrinas/patología , Femenino , Humanos , Hiperplasia/diagnóstico por imagen , Lactante , Masculino , Interpretación de Imagen Radiográfica Asistida por Computador , Enfermedades Raras , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
15.
Pediatr Radiol ; 47(13): 1759-1765, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-28844075

RESUMEN

BACKGROUND: Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We found no reports of systematic review of high-resolution computed tomography (HRCT) findings in pediatric follicular bronchiolitis. OBJECTIVE: The purpose of this study was to describe the HRCT findings of follicular bronchiolitis in children and correlate these imaging findings with histopathology. MATERIALS AND METHODS: A 5-year retrospective review of all pathology-proven cases of follicular bronchiolitis was performed. Inclusion criteria were age <18 years and an HRCT within 6 months of lung biopsy. HRCTs were reviewed by three observers and scored using the system previously described by Brody et al. RESULTS: Six patients met the inclusion criteria with age range at HRCT of 7-82 months (median: 39.5 months). Pulmonary nodules (n=6) were the most common HRCT finding followed by focal consolidation (n=5), bronchiectasis (n=4) and lymphadenopathy (n=3). Tree and bud opacities and nodules on CT correlated with interstitial lymphocytic infiltrates and discrete lymphoid follicles on pathology. CONCLUSION: The salient HRCT findings of childhood follicular bronchiolitis are bilateral, lower lung zone predominant pulmonary nodules and bronchiectasis with infantile onset of symptoms. These characteristic HRCT findings help differentiate follicular bronchiolitis from other forms of infantile onset ILD.


Asunto(s)
Bronquiolitis/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Biopsia , Bronquiolitis/patología , Niño , Preescolar , Comorbilidad , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Masculino , Nódulos Pulmonares Múltiples/diagnóstico por imagen , Nódulos Pulmonares Múltiples/patología , Estudios Retrospectivos
19.
Pediatr Radiol ; 45(4): 562-9, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25301383

RESUMEN

BACKGROUND: Lymphangiectasia is a rarely encountered lymphatic dysplasia characterized by lymphatic dilation without proliferation. Although it can occur anywhere, the most common locations are the central conducting lymphatics and the pulmonary and intestinal lymphatic networks. Recent advances in lymphatic interventions have resulted in an increased reliance on imaging to characterize patterns of disease. OBJECTIVE: To describe the patient populations, underlying conditions, and imaging features of lymphangiectasia encountered at a tertiary pediatric institution over a 10-year period and correlate these with pathology and patient outcomes. MATERIALS AND METHODS: We retrospectively reviewed the pathology database from 2002 to 2012 to identify patients with pathologically or surgically proven lymphangiectasia who had undergone cross-sectional imaging. Medical records were reviewed for patient demographics, underlying conditions, treatment and outcome. RESULTS: Thirteen children were identified, ranging in age from 1 month to 16 years. Five had pulmonary lymphangiectasia, four intestinal and four diffuse involvement. Pulmonary imaging findings include diffuse or segmental interlobular septal thickening, pleural effusions and dilated mediastinal lymphatics. Intestinal imaging findings include focal or diffuse bowel wall thickening with central lymphatic dilation. Diffuse involvement included dilation of the central lymphatics and involvement of more than one organ system. Children with infantile presentation and diffuse pulmonary, intestinal or diffuse lymphatic abnormalities had a high mortality rate. Children with later presentations and segmental involvement demonstrated clinical improvement with occasional regression of disease. Three children with dilated central lymphatics on imaging underwent successful lymphatic duct ligation procedures with improved clinical course. CONCLUSION: Lymphangiectasia is a complex disorder with a spectrum of presentations, imaging appearances, treatments and outcomes. Cross-sectional imaging techniques distinguish segmental involvement of a single system (pulmonary or intestinal) from diffuse disease and may show dilated central conducting lymphatics, which may benefit from interventions such as ligation or occlusion.


Asunto(s)
Enfermedades Pulmonares/congénito , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia/congénito , Imagen por Resonancia Magnética/métodos , Tomografía Computarizada por Rayos X/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Enfermedades Pulmonares/diagnóstico , Linfangiectasia/diagnóstico , Masculino
20.
Pediatr Radiol ; 45(7): 1026-30, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25712453

RESUMEN

BACKGROUND: Lumbar spondylolysis, a unilateral or bilateral fracture at pars interarticularis, is a common cause of low back pain in children. The initial imaging study in the diagnosis of lumbar spondylolysis has historically been lumbar spine radiographs; however, radiographs can be equivocal or false-negative. Definitive diagnosis can be achieved with computed tomography (CT), but its use has been limited due to the dose of ionizing radiation to the patient. OBJECTIVE: By limiting the z-axis coverage to the relevant anatomy and optimizing the CT protocol, we are able to provide a definitive diagnosis of fractures of the pars interarticularis at comparable or lower radiation dose than commonly performed lumbar spine radiographs. As there is no gold standard for the diagnosis of spondylolysis besides surgery, we compared interobserver agreement and degree of confidence to determine which modality is preferable. MATERIALS AND METHODS: Sixty-two patients with low back pain ages 5-18 years were assessed for the presence of spondylolyis. Forty-seven patients were evaluated by radiography and 15 patients were evaluated by limited field-of-view CT. Both radiographic and CT examinations were assessed anonymously in random order for the presence or absence of spondylolyisis by six raters. Agreement was assessed among raters using a Fleiss Kappa statistic for multiple raters. RESULTS: CT provided a significantly higher level of agreement among raters than radiographs (P < 0.001). The overall Kappa for rater agreement with radiographs was 0.24, 0.34 and 0.40 for 2, 3 or 4 views, respectively, and 0.88 with CT. CONCLUSION: Interobserver agreement is significantly greater using limited z-axis coverage CT when compared with radiographs. Radiologist confidence improved significantly with CT compared to radiographs regardless of the number of views.


Asunto(s)
Dosis de Radiación , Espondilólisis/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Niño , Femenino , Humanos , Vértebras Lumbares/diagnóstico por imagen , Región Lumbosacra/diagnóstico por imagen , Masculino , Reproducibilidad de los Resultados
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