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Children with compromised immune systems, whether due to primary or secondary immunodeficiencies, are susceptible to a broad spectrum of acute intrathoracic pathologies. These include infections, pulmonary edema, and malignancies. Pulmonary issues are common and perilous in this population, necessitating prompt and precise diagnosis for effective management. This review aims to provide an overview of such conditions, focusing on the imaging appearances of the most prevalent acute lung conditions affecting immunocompromised children. It emphasizes the critical importance of an integrated clinical and radiological approach when diagnosing these acute pulmonary disease states., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction., Objectives: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia., Materials and Methods: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed., Results: Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications., Conclusion: Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.

The term "systemic vasculitis" encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children.

Background: We describe radiographic changes in the ribs and scapulae seen in the first 6 months of life in children with ADA (adenosine deaminase) deficiency severe combined immundeficiency syndrome (SCIDS). We suggest that these changes are reversible with appropriate enzyme replacement therapy., Objective: The purpose of this study was to describe characteristic rib and scapular radiographic changes in infants with ADA-deficiency SCIDS., Materials and Methods: This was a retrospective review of chest radiographs of nine children with ADA-deficiency SCIDS performed in the first year of life by two experienced pediatric radiologists. A control cohort of unaffected children was used for comparison., Results: All children with ADA-deficiency SCIDS manifested unusual scapular spurring and anterior rib cupping. None of the control children manifested these changes., Conclusion: Characteristic and reversible scapular and rib changes in the correct clinical setting should suggest an early diagnosis of ADA deficiency, prompting appropriate diagnostic and therapeutic measures.

Background: Computer-aided detection (CAD) has been shown to increase the sensitivity for detection of pulmonary nodules in adults. This study reports initial findings utilizing a CAD system for the detection of pediatric pulmonary nodules., Objective: To assess the performance of CAD and pediatric radiologists in the detection of pediatric pulmonary nodules., Materials and Methods: CT scans from a series of pediatric patients with known primary tumors and lung nodules were analyzed by four radiologists and a commercially available CAD system. IRB approval was obtained. Sensitivities were calculated for detection according to nodule size and location., Results: In 24 children (age 3-18 years) 173 nodules were identified. Overall the sensitivity of CAD was 34%, but the sensitivity of CAD for detection of nodules 4.0 mm or larger was 80%. Overall radiologist sensitivity ranged from 68% to 79%. There were 0.9 CAD false-positives and 0.3-2.4 radiologist false-positives per study., Conclusion: CAD in our pediatric oncology patients had good sensitivity for detection of lung nodules 4 mm and larger with a low number of false-positives. However, the sensitivity was considerably less for nodules smaller than 4 mm.

Background: Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease in children. By definition it is a diagnosis of exclusion, and CT of the chest is primarily performed to exclude other causes. Previous studies have defined CT features suggestive of the diagnosis of IPAH, but these have all been limited to the adult population., Objective: Contrast-enhanced chest CT and high-resolution CT findings in IPAH were evaluated in an attempt to define features consistently seen in children with this condition., Materials and Methods: The chest CT scans performed at initial presentation were reviewed in 17 children with echocardiographic or angiographic evidence of IPAH., Result: There were nine boys and eight girls, ranging in age from 1 month to 17 years. The extrapulmonary findings included cardiomegaly with right-sided cardiac enlargement, which was seen in 13 children. The central pulmonary arteries were enlarged in 15 children, with peripheral enlargement in two. In six children this resulted in bronchial compression. In addition, mediastinal and hilar lymphadenopathy was noted in three children. Prominent intrapulmonary features included a peripheral vasculopathy, with enlarged tortuous vessels, seen in eight children. Ill-defined ground-glass centrilobular opacities were also noted in eight children, representing the most common parenchymal abnormality. Other findings included septal lines in five, diffuse ground-glass opacification in four and focal hyperlucent zones in three. Mosaic attenuation was seen in one child., Conclusion: A variety of imaging findings are identified in IPAH. Features particularly consistent with the diagnosis include peripheral vasculopathy and centrilobular opacities in the setting of cardiomegaly and central pulmonary arterial enlargement.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disorder of vascular endothelium that can result in the formation of pulmonary arteriovenous malformations (pAVMs). We observed that small pulmonary nodules, possibly representing early vascular malformations, are more common in these children than in the general population., Objective: To describe and characterize the small pulmonary nodules seen on chest CT examination in the lungs of children with HHT., Materials and Methods: We followed 19 children who had undergone chest CT as part of their initial diagnostic evaluation in the HHT clinic of a pediatric hospital. Chest CT scans were reviewed independently by two radiologists blinded to clinical data. Pulmonary nodules were analyzed according to lobar distribution, anatomic position in the lung (outer, mid, inner third), shape (round, linear, V- or Y-shaped, ill-defined), presence of overt AVMs, and nodule size (5 mm). We also reviewed a control group of chest CT scans performed on 25 age-matched children., Results: A total of 35 pulmonary nodules were identified on the CT scans in 15 of the 19 children. Multiple nodules were seen in eight children (42%), solitary nodules were seen in seven children (37%) and no nodules were seen in the remaining four children (21%). The nodules were found to be nonspecific with regard to multiple factors other than their anatomic position within the lung. The chest CT scans of 2 of the 25 age-matched controls revealed a total of only four nodules, a significantly lower total (P = 0.0001 vs. children with HHT)., Conclusion: Nonspecific nodules are commonly seen in children with HHT.

Background: Although pulmonary involvement occurs in the majority of children and adolescents with Wegener's granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients., Objective: To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG)., Materials and Methods: The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children., Results: The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01)., Conclusions: In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares.

Background: Recent reports have indicated that infants and young children have a higher sensitivity than older children and adults to radiation exposure and the potential for harmful side effects., Objective: To determine whether the present landmarks used in film positioning result in unnecessary radiation to non-thoracic structures on chest radiographs in the pediatric and neonatal population., Materials and Methods: Chest radiographs of 195 pediatric patients and 149 neonates were analyzed for extent of radiation field to non-thoracic regions. This was accomplished by delineating the most superior and inferior portions of the body included within the boundaries of collimation on each chest radiograph. The distance between the superior and inferior aspects of the lungs was measured and compared to the long axis of the radiation field on the radiograph. Radiographic reports were reviewed to determine whether valuable data were obtained from the imaging of these non-thoracic structures., Results: The ratio of radiation exposure to non-thoracic structures increases as the age of the patient decreases. Overall, 43% of the length of the chest radiograph was of non-thoracic structures, resulting in radiation exposure to these sites. No significant information was gained in a single case by including the neck. In 3% of the neonatal patients, a potentially significant comment was reported on the abdomen included on the chest radiograph., Conclusion: Present positioning techniques in neonatal and pediatric chest radiography result in unnecessary radiation exposure to non-thoracic structures. New landmarks for collimation should be sought to eliminate this problem.

Background: Severe acute respiratory syndrome (SARS) is a recently recognized condition of viral origin associated with substantial morbidity and mortality rates in adults. Little information is available on its radiologic manifestations in children., Objective: The goal of this study was to characterize the radiographic presentation of children with SARS., Materials and Methods: We abstracted data (n=62) on the radiologic appearance and course of SARS in pediatric patients with suspect (n=25) or probable (n=37) SARS, diagnosed in five hospital sites located in three cities: Toronto, Singapore, and Hong Kong. Available chest radiographs and thoracic CTs were reviewed for the presence of the following radiographic findings: airspace disease, air bronchograms, airways inflammation and peribronchial thickening, interstitial disease, pleural effusion, and hilar adenopathy., Results: A total of 62 patients (suspect=25, probable=37) were evaluated for SARS. Patient ages ranged from 5.5 months to 17 years and 11.5 months (average, 6 years and 10 months) with a female-to-male ratio of 32:30. Forty-one patients (66.1%) were in close contact with other probable, suspect, or quarantined cases; 10 patients (16.1%) had recently traveled to WHO-designated affected areas within 10 days; and 7 patients (11.2%) were transferred from other hospitals that had SARS patients. Three patients, who did not have close/hospital contact or travel history to affected areas, were classified as SARS cases based on their clinical signs and symptoms and on the fact that they were living in an endemic area. The most prominent clinical presentations were fever, with a temperature over 38 degrees C (100%), cough (62.9%), rhinorrhea (22.6%), myalgia (17.7%), chills (14.5%), and headache (11.3%). Other findings included sore throat (9.7%), gastrointestinal symptoms (9.7%), rigor (8.1%), and lethargy (6.5%). In general, fever and cough were the most common clinical presentations amongst younger pediatric SARS cases (age<10 years), whereas, in addition to these symptoms, headache, myalgia, sore throat, chills, and/or rigor were common in older patients (age>/=10 years). The chest radiographs of 35.5% of patients were normal. The most prominent radiological findings that were observed in the remaining patients were areas of consolidation (45.2%), often peripheral with multifocal lesions in 22.6%. Peribronchial thickening was noted on chest radiographs of 14.5% of patients. Pleural effusion was observed only in one patient (age 17 years and 11.5 months), whereas interstitial disease was not observed in any patient., Conclusion: In pediatric cases, SARS manifests with nonspecific radiographic features making radiological differentiation difficult, especially from other commonly encountered childhood respiratory viral illnesses causing airspace disease. The radiographic presentation of suspected SARS cases ranged from normal to mild perihilar peribronchial thickening. The radiographic presentations, as expected, were relatively more pronounced in the SARS probable cases.

Bronchial varices, which have rarely been described in the radiology literature, can be the result of pulmonary venous obstruction and may present with hemoptysis. This case is an illustration of this rare condition, which correlates CT findings with bronchoscopic findings. We also describe the findings on phase-contrast MR that demonstrated reversed diastolic flow in the branch pulmonary artery supplying the affected lung.

Background: Sternal fractures are rare injuries in children. The rarity of this injury is likely due to both the relative plasticity of the pediatric thorax and to the difficulty in establishing a radiographic diagnosis without dedicated views. Current literature suggests that this injury in young children is highly specific for abusive injury., Hypothesis: Sternal fractures are not highly specific for abusive injury., Materials and Methods: This was a retrospective radiographic and clinical chart review of all documented sternal fractures over an 11-year period at a large pediatric hospital., Results: Of 12 children with sternal fractures identified, four were < or = 2 years of age and eight were > or = 3 years of age. The mechanism of injury was suspicious for child abuse in two children. Both of these children were < or = 2 years of age. In one toddler, an unwitnessed injury resulted in extensive initial familial anxiety until abusive injury was excluded., Conclusion: Sternal fractures are unusual injuries, yet they, in themselves, are not highly specific for abusive injury.

Torsion of an accessory lobe of the liver and of the gallbladder is a rare etiology for acute abdominal pain in children and infants. We report a case of an 8-year-old girl who was admitted with acute epigastric pain and vomiting, after her brother had jumped on her back. Physical examination revealed an afebrile child with a nontender right upper quadrant (RUQ) mass. Color Doppler ultrasound and contrast-enhanced CT demonstrated a heterogeneous, avascular mass with displacement of a thickened-wall gallbladder. A contorted, congested accessory lobe of the liver and the gallbladder were resected at laparotomy. Imaging and operative findings are presented and a differential diagnosis is discussed in order to increase awareness of this rare condition.