Figures
- Figure 1
Chest radiograph of a patient with pPLCH. An endotracheal tube and bilateral intercostal chest drains are in situ with a residual right-sided pneumothorax. Extensive cystic changes and abnormal linear interstitial opacification can be seen.
- Figure 2
CT image of a patient with pPLCH with extensive cystic changes.
- Figure 3
a and b) Lung biopsy from the patient in figure 2 shows lung parenchyma that features conspicuous nodular aggregates, typically extending into airspaces, of variably sized medium and large cells, some multinucleated, featuring open nuclei that are focally clefted. Cells within the nodular infiltrates are highlighted under antisera to CD1a and S100 protein by immunohistochemical staining. Scale bars=50 μm.
Tables
- Table 1
Similarities and differences between PLCH in children and adults
Children Adults Sites involved Mostly as part of multisystem LCH Mostly isolated PLCH Smoking Not generally associated with smoking Nearly universally associated with smoking Pneumothoraces Less common as first presentation More common as first presentation Radiological findings Involvement of the costophrenic angle and lower lung fields Sparing of the costophrenic angle and lower lung fields - Table 2
Differential diagnoses of diffuse cystic lung disease in children [39–42]
Cystic lung disease Pulmonary manifestations Other potential features Genetic defect/association, if known Prevalence/onset Birt–Hogg–Dubé syndrome Characteristic CT findings of multiple, thin-walled, air-filled cysts; no interstitial lung disease Hair follicle tumours, renal tumours, family history of pneumothorax in 35%[39] Autosomal dominant, FLCN (folliculin gene) on chromosome 17 Rare and often diagnosed in early to mid-adulthood but has been reported in teenagers Lung cysts associated with FLNA mutation [43, 44] Multiple lung cysts Cerebral periventricular nodular heterotopia, cardiac valvular disease, skeletal abnormalities FLNA (filamin A gene) mutation Childhood cases Congenital cystic lung lesions Bronchogenic cysts/congenital pulmonary airway malformations Infection, bleeding, compression of other structures, concerns over malignancy Antenatally or may be detected later in childhood or in an adult Bronchopulmonary dysplasia Disruption in distal lung growth Preterm birth, complications associated with this Genetic contribution unclear Normally suspected from history of preterm birth or need for oxygen/ventilation Infective Staphylococcal infections may be associated with pneumatocele development May occur in association with hyper-IgE syndrome STAT3 and DOCK8 mutations associated with hyper-IgE syndrome [46] Can present in childhood Pleuropulmonary blastoma Can have cystic and solid components depending on subtype Most common paediatric pulmonary neoplasm DICER1 mutations [47] Children <6 years of age Down syndrome [45] Subpleural cystic areas Cardiac problems, developmental delay Trisomy 21 Can be suspected and diagnosed antenatally or shortly after birth
