Objectives: To determine the value of open lung biopsy (OLB) in terms of di
agnosis, morbidity, mortality, and benefits in immunocompromised children w
ith pulmonary involvement.
Study design: We retrospectively reviewed 36 OLBs performed in 32 immunocom
promised children between 1985 and 1998. Seventeen biopsies were performed
in patients with primary immunodeficiency syndromes and 19 in patients with
secondary immunodeficiency syndromes. Twenty-eight biopsies were performed
because of a lack of response to ongoing antimicrobial treatments with neg
ative or positive findings on bronchoalveolar lavage (BAL) and a deteriorat
ing clinical or radiologic course, and 8 biopsies were performed because of
persistent chest x-ray infiltrates.
Results: Diffuse pulmonary infiltrates were observed on chest x-ray in 28 c
ases, hyperinflation in 3 cases, and nodular infiltrates in 5 cases. A hist
opathologic diagnosis was possible for all 36 OLBs. Specific diagnosis was
obtained in 22 (61%) (12 infectious agents, 6 tumors, 4 bronchiolitis oblit
erans) and non-specific diagnosis in 14 (399/0). Fungi were the main infect
ious agents (8 of 12). For the diagnosed infections, BAL provided 4 true-po
sitive, 3 false-positive, and 6 false-negative results. Specific treatment
was changed in 77% of cases, providing real benefits in 12 (33%) cases. The
morbidity and overall mortality rates were 31% and 33%, respectively. The
mortality rate was significantly higher in the first 30 days after OLB in p
atients receiving ventilatory assistance (58%).
Conclusions: OLB in immunocompromised children with deteriorating clinical
or radiologic course is a sensitive diagnostic tool.