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Review
. 2024 Nov 29:43:e2024076.
doi: 10.1590/1984-0462/2025/43/2024076. eCollection 2024.

Challenges and strategies: treating spontaneous pneumothorax in massive pulmonary langerhans cell histiocytosis in children

Letícia Helena Kaça do Carmo  1 , Luiz Augusto Marin Jaca  1 , Luiz Miguel Vicente Barreiros  1 , Gabriela Marengone Altizani  1 , Leticia Fontanini  1 , Maristella Bergamo Francisco Dos Reis  1 , Mauricio André Pereira da Silva  1 , Marcel Koenigkam Santos  1 , Monica Cypriano  2 , Ygor Aluísio Moura  2 , Henrique Lederman  2 , Carlos Alberto Scrideli  1 , Elvis Terci Valera  1
Affiliations
Review

Challenges and strategies: treating spontaneous pneumothorax in massive pulmonary langerhans cell histiocytosis in children

Letícia Helena Kaça do Carmo et al. Rev Paul Pediatr. .

Abstract
in English, Portuguese

Objective: The objective of this study was to report two cases of successive multiple spontaneous bilateral pneumothorax in children with massive lung involvement due to Langerhans cell histiocytosis (LCH), emphasizing the possibility of this differential diagnosis for the general pediatrician. Additionally, published cases describing pediatric patients with pulmonary manifestations of LCH were reviewed in the literature.

Case description: Case #1: A 3-year-old male patient with a sudden episode of spontaneous right-sided pneumothorax, surgically drained. After 2 months, he experienced two new episodes of contralateral pneumothorax. A pulmonary lymph node biopsy revealed the diagnosis of LCH. He underwent bilateral video-assisted thoracic surgery and mini-thoracotomy with mechanical pleurodesis, in addition to chemotherapy, requiring prolonged hospitalization. Case #2: A 4-year-old boy with progressive dyspnea and wheezing for 5 months. A pulmonary biopsy revealed LCH. He developed significant respiratory distress and right pneumothorax, requiring drainage. Silver nitrate pleurodesis and different chemotherapy regimens were performed. Both patients responded well to multiple chemotherapy treatments, surgeries, and intensive care support.

Comments: LCH is a challenging disease. Its clinical manifestation is variable, and pulmonary involvement occurs in about 10-15% of cases. We consider specialized surgical management and multidisciplinary support essential for the treatment of patients with massive pulmonary LCH. Although rare, massive pulmonary involvement by LCH should be considered in cases of recurrent pneumothorax in children.

Objetivo:: Relatar dois casos de pneumotórax bilateral espontâneo múltiplo sucessivo em crianças com envolvimento pulmonar maciço por histiocitose de células de Langerhans (HCL), alertando para a possibilidade desse diagnóstico diferencial para o pediatra geral. Ademais, casos publicados que descrevem pacientes pediátricos com manifestações pulmonares de HCL foram revisados na literatura.

Descrição do caso:: Caso 1: Paciente masculino, três anos, com episódio súbito pneumotórax espontâneo à direita, drenado cirurgicamente. Após dois meses, apresentou dois novos episódios de pneumotórax contralateral. Biópsia linfonodal pulmonar revelou diagnóstico de HCL. Foi submetido a cirurgia torácica assistida por vídeo (VATS) bilateral e minitoracotomia com pleurodese mecânica, além de tratamento quimioterápico, necessitando de internação prolongada. Caso 2: Menino, quatro anos, apresentando dispneia progressiva e sibilância por cinco meses. Biópsia pulmonar revelou HCL. Evoluiu com importante desconforto respiratório e pneumotórax direito, necessitando drenagem. Foi realizada pleurodese com nitrato de prata e diferentes esquemas de quimioterapia. Ambos os pacientes evoluíram bem após tratamentos quimioterápicos múltiplos, cirurgias e suporte intensivo.

Comentários:: A HCL é uma doença desafiadora. Sua manifestação clínica é variável, e o envolvimento pulmonar ocorre em cerca de 10–15% dos casos. Consideram-se o manejo cirúrgico especializado e o suporte multidisciplinar essenciais para o tratamento de pacientes com HCL pulmonar maciça. Ainda que raro, o envolvimento pulmonar maciço por HCL deve ser considerado em quadros de pneumotórax recorrentes em crianças.

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Conflict of interest statement

Conflict of interests: The authors declare that there is no conflict of interests.

Figures

Figure 1
Figure 1. (A) X-ray of admission showed a right pneumothorax. (B) X-ray after thoracostomy, showing pulmonary expansion and diffuse interstitial lung disease. (C) CT after thoracostomy, showing diffuse cystic lung disease. (D) Electropherogram showing the absence of mutation (blue column) at codon 600 of the BRAF gene (wild-type). (E) CT at the beginning of remission therapy, during new bilateral pneumothorax. (F) CT at the end of maintenance therapy shows good pulmonary expansion and reduced number and size of pulmonary cysts.
Figure 2
Figure 2. (A) An initial chest X-ray led to the diagnosis of pneumonia. (B) Chest CT showed multiple and disseminated pulmonary cysts throughout the lung parenchyma. (C) Chest X-ray after draining the right hemithorax. (D) A new chest X-ray depicting enlargement of the bubble on the left base, with mediastinal deviation to the right side.
See this image and copyright information in PMC

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