Pediatric Lung Transplantation: Perspectives for the Pathologist

Abstract

Lung transplantation offers life-saving and life-extending treatment for children and adolescents with congenital and acquired forms of pulmonary and pulmonary vascular disease, for whom medical therapy is ineffective or insufficient for sustained response. This review summarizes the pathology related to lung transplantation for the practicing pediatric pathologist and also highlights aspects of lung transplantation unique to the pediatric population. Clinical issues related to availability of organs, candidate eligibility, surgical technique, and postoperative monitoring are discussed. Pathologic evaluation of routine surveillance transbronchial biopsies requires attention to acute cellular rejection, opportunistic infection, and other forms of acute and resolving lung injury. These findings are correlated in some cases with endobronchial biopsies and bronchoalveolar lavage as adjunctive tools in surveillance. Open or thoracoscopic biopsies also have diagnostic utility in cases with acute or chronic graft deterioration of uncertain etiology. Future challenges in pediatric lung transplantation are similar to those in the adult population, with continued efforts focused on prolonging graft survival, prevention of bronchiolitis obliterans syndrome due to chronic cellular rejection, and evaluation of humoral rejection.

Pediatric Bronchoalveolar Lavage: Practical Considerations and Future Prospects

“…Midulla and colleagues [67], however, reported that HIV-1-infected children displayed elevated lymphocytes and eosinophils, in addition to albumin and fibronectin (which was even higher if the patient was Pneumocystispositive). Minimal literature exists at this time specifically addressing pediatric lung transplant recipients; however, findings appear to be similar to those reported in adults who have undergone lung transplant [68]. In addition to infection surveillance, BAL may be used to monitor rejection and patients at risk for obliterative bronchiolitis (OB), an indication of graft failure [69].…”

Antibody‐mediated rejection (AMR) in pediatric lung transplantation—Current state and future directions

“…More than five biopsies may be needed by the bronchoscopist to ensure that the sample meets these criteria for biopsy adequacy. Attaining lung biopsies in younger children is often challenging resulting in smaller biopsies relative to adolescent and adult biopsies with the size of required biopsy forceps being a potential limiting factor 24 . Similar biopsy adequacy criteria are routinely used for pediatric and adult biopsies with adequacy of smaller pediatric biopsies not being addressed by the ISHLT guidelines 23 .…”

“…Attaining lung biopsies in younger children is often challenging resulting in smaller biopsies relative to adolescent and adult biopsies with the size of required biopsy forceps being a potential limiting factor. 24 Similar biopsy adequacy criteria are routinely used for pediatric and adult biopsies with adequacy of smaller pediatric biopsies not being addressed by the ISHLT guidelines. 23 A comment on biopsy size and adequacy should be included in the pathologic evaluation report to inform the assignment of the final multidisciplinary diagnosis.…”