CC BY 4.0 · European J Pediatr Surg Rep 2019; 07(01): e8-e11
DOI: 10.1055/s-0039-1688771
Case Report
Georg Thieme Verlag KG Stuttgart · New York

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

1  Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Gül Özyüksel
1  Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
1  Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Kübra Çakmakkaya
2  Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Sinan Yavuz
3  Department of Pediatric Intensive Care, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Bilgehan Yalçın
4  Department of Pediatric Oncology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Diclehan Orhan
5  Department of Pediatric Pathology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Ebru Yalçın
6  Department of Pediatric Pulmonology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Deniz Doğru
6  Department of Pediatric Pulmonology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Benan Bayrakçı
3  Department of Pediatric Intensive Care, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Nural Kiper
6  Department of Pediatric Pulmonology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
,
Canan Akyüz
4  Department of Pediatric Oncology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
› Author Affiliations
Further Information

Publication History

13 December 2018

31 March 2019

Publication Date:
23 May 2019 (online)

  

Abstract

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.

Note

This case report is presented in the Joint Congress of 36th National Congress of Turkish Association of Pediatric Surgeons and International Pediatric Endosurgery Group (IPEG), Middle East Chapter, October 24 to 27, 2018, İzmir, Turkey.