Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2011 Dec 1:2011:bcr0920114734.
doi: 10.1136/bcr.09.2011.4734.

Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

Dayang Anita Abdul Aziz  1 , Nur Afdzillah Abdul Rahman, Swee Fong Tang, Hasniah Abdul Latif, Faizah Mohd Zaki, Zulfiqar Mohd Annuar, Hamidah Alias, Zarina Abdul Latiff
Affiliations
Case Reports

Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

Dayang Anita Abdul Aziz et al. BMJ Case Rep. .

Abstract

Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.

PubMed Disclaimer

Conflict of interest statement

Competing interests None.

Figures

Figure 1
Figure 1
Chest x-ray of patient with pulmonary langerhans cells histiocytosis; note the bizarre honeycomb appearance brought by bilateral diffuse cystic changes on the lungs with left-sided pneumothorax.
Figure 2
Figure 2
CT thorax of pulmonary langerhans cells histiocytosis presurgery. Note the extensive bullae with compressed lung tissues and evidence of pneumothorax.
Figure 3
Figure 3
CT thorax of pulmonary langerhans cells histiocytosis post pleurectomy. Note both lung tissues have expanded. A small residual pneumothorax is seen on the apical left zone (patient remained asymptomatic despite this finding).
Figure 4
Figure 4
CT thorax of the same patient at 6 months post surgery. Note both lung tissues have expanded further.
Figure 5
Figure 5
Appearance of right lung langerhans cells histiocytosis at surgery.
Figure 6
Figure 6
Appearance of left lung langerhans cells histiocytosis at surgery. Note lesions in the left lung is less severe than right.
See this image and copyright information in PMC

References

    1. Vassallo R, Ryu JH, Colby TV, et al. Pulmonary langerhans cell histiocytosis. N Eng J Med 2000;342:1969–78 - PubMed
    1. Al-Trabolsi HA, Alshehri M, Al-Shomrani A, et al. “Primary” pulmonary Langerhans cell histiocytosis in a two-year-old child: case report and literature review. J Pediatr Hematol Oncol 2006;28:79–81 - PubMed
    1. Aricò M, Girschikofsky M, Généreau T, et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 2003;39:2341–8 - PubMed
    1. Favara BE, Steele A. Langerhans cell histiocytosis of lymph nodes: a morphological assessment of 43 biopsies. Pediatr Pathol Lab Med 1997;17:769–87 - PubMed
    1. Travis WD, Borok Z, Roum JH, et al. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases. Am J Surg Pathol 1993;17:971–86 - PubMed

Publication types