Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Jan;7(1):e8-e11.
doi: 10.1055/s-0039-1688771. Epub 2019 May 23.

Bilateral Pulmonary Langerhans's Cell Histiocytosis Is Surgical Challenge in Children: A Case Report

Free PMC article
Case Reports

Bilateral Pulmonary Langerhans's Cell Histiocytosis Is Surgical Challenge in Children: A Case Report

Tutku Soyer et al. European J Pediatr Surg Rep. .
Free PMC article


Background Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report The patient was admitted to the emergency department with severe respiratory distress and SP. After chest tube insertion, biopsy from neck mass revealed Langerhans's cell histiocytosis. Chemotherapy including vinblastine and prednisone was initiated. Due to persistent respiratory difficulty and air leaks, talc pleurodesis and thoracoscopic bullae excision with pleural decortication were performed. Two months after the admission, due to nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO. Conclusion Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be considered as last resort of treatment alternative in persistent cases.

Keywords: bilateral; children; pulmonary Langerhans's cell histiocytosis.

Conflict of interest statement

Conflict of Interest None.


Fig. 1
Fig. 1
( A ) Chest X-ray of the patient showing pneumothorax on right side. ( B ) Computed tomography revealing bilateral cysts and ground glass appearance.
Fig. 2
Fig. 2
Thoracoscopic image of cystic lesion.

Similar articles

See all similar articles


    1. Favara B E, Feller A C, Pauli M et al. Contemporary classification of histiocytic disorders. Med Pediatr Oncol. 1997;29(03):157–166. - PubMed
    1. Vassallo R, Ryu J H, Schroeder D R, Decker P A, Limper A H. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. 2002;346(07):484–490. - PubMed
    1. Alston R D, Tatevossian R G, McNally R J, Kelsey A, Birch J M, Eden T O. Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatr Blood Cancer. 2007;48(05):555–560. - PubMed
    1. Suri H S, Yi E S, Nowakowski G S, Vassallo R. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 2012;7:16. - PMC - PubMed
    1. Schulze J, Kitz R, Grüttner H P, Schmidt H, Zielen S. Severe isolated pulmonary Langerhans cell histiocytosis in a 6-year-old girl. Eur J Pediatr. 2004;163(06):320–322. - PubMed

Publication types