Clinico-radiologic features of pleuroparenchymal fibroelastosis in children.

Authors:
Haithuy N Nguyen
Haithuy N Nguyen
University of Texas Health Science Center at Houston
Shailendra Das
Shailendra Das
Pediatric Pulmonary Section
Maria C Gazzaneo
Maria C Gazzaneo
Baylor College of Medicine
United States
Ernestina Melicoff
Ernestina Melicoff
National Institute of Neurological Disorders and Stroke
United States
George B Mallory
George B Mallory
Baylor College of Medicine
Karen W Eldin
Karen W Eldin
Texas Children's Hospital
United States

Pediatr Radiol 2019 Apr 19. Epub 2019 Apr 19.

Department of Pediatric Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

Background: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies.

Objective: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis.

Materials And Methods: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018. Two pediatric radiologists evaluated the chest imaging studies for the presence or absence of published radiologic findings of PPFE in adults, including platythorax, pneumothorax, upper lobe predominant pleural and septal thickening, and bronchiectasis. Platythorax indices were calculated from the normal chest CT exams of eight age- and gender-matched individuals obtained via the radiology search engine.

Results: The mean presentation age was 12.9 years (range: 7-16 years). Seven of the eight had a history of chemotherapy and radiation therapy for cancer. Three of the eight had undergone bone marrow transplantation and none had undergone lung transplantation. The mean time between chemotherapy, radiation therapy, and/or bone marrow transplantation and the presentation of PPFE was 8.4 years (range: 5.6-12.1 years). Most of the patients presented with dyspnea (63%), cough (50%) and/or pneumothorax (38%). The mean percentage of predicted FEV1 (forced expiratory volume in one second) was 14.1 (range: 7.7-27.5). All eight patients demonstrated platythorax, bronchiectasis, pleural and septal thickening (upper lobes in four, upper and lower lobes in four) and six had pneumothorax. Five underwent lung biopsies, four of whom developed pneumothoraces.

Conclusion: Clinical and radiologic findings of pediatric PPFE are similar to those in adults, although a majority of the former have a history of treated cancer. Clinical presentation of restrictive lung disease, dyspnea, cough or spontaneous pneumothorax years after treatment for childhood cancer combined with platythorax, upper lobe pleural and septal thickening and traction bronchiectasis on chest CT establishes a presumptive diagnosis of PPFE.

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http://link.springer.com/10.1007/s00247-019-04405-5
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April 2019
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References

(Supplied by CrossRef)
Article in Kokyu
R Amitani et al.
Kokyu 1992
Article in Chest
SK Frankel et al.
Chest 2004
Article in Arch Pathol Lab Med
SK Cheng et al.
Arch Pathol Lab Med 2016
Article in Am J Respir Crit Care Med
WD Travis et al.
Am J Respir Crit Care Med 2013
Article in Eur Respir J
T Nakatani et al.
Eur Respir J 2015
Article in Hum Pathol
JN Rosenbaum et al.
Hum Pathol 2015
Article in Eur Respir J
TL Reddy et al.
Eur Respir J 2012
Article in Mod Pathol
JH Thüsen von der et al.
Mod Pathol 2011
Article in Eur Respir J
C Beynat-Mouterde et al.
Eur Respir J 2014
Article in Eur Respir J
P Camus et al.
Eur Respir J 2014
Article in Diagn Interv Radiol
F Mariani et al.
Diagn Interv Radiol 2016

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