Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Sacco, Oliviero; Moscatelli, Andrea; Conte, Massimo; Grasso, Chiara; Magnano, Gian Michele; Sementa, Angela Rita; Martelli, Alberto M.; Rossi, Giovanni

doi:10.1002/pbc.25912

Cited by 8 publications

(7 citation statements)

References 19 publications

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“…ECMO has been shown to be a valid alternative as a bridge for lung transplantation in PLCH. 15 It was not considered as an alternative treatment in our case because of severe deterioration of the patient.…”

Section: Discussionmentioning

confidence: 95%

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

Soyer

Özyüksel

Boybeyi

et al. 2019

European J Pediatr Surg Rep

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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.

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Section: Discussionmentioning

confidence: 95%

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

Soyer

Özyüksel

Boybeyi

et al. 2019

European J Pediatr Surg Rep

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“…pneumothoraces and respiratory failure), leading to the need for intensive care support. There are cases in the literature of isolated pPLCH being associated with rapid deterioration, and consideration of treatments such as ECMO and lung transplantation [ 63 ]. Children with this condition require careful surveillance as the outcome can be difficult to predict.…”

Section: Prognosismentioning

confidence: 99%

Paediatric pulmonary Langerhans cell histiocytosis

Barclay

Devaney

Bhatt

2020

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Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.

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Section: Discussionmentioning

confidence: 99%

“…Pulmonary LCH is extremely rare in children and usually occurs as part of a multisystem disorder [3][4][5] with broad range of symptoms at presentation. 4,5 The median age of diagnosis of all LCH in children is 5.9 years in the UK. 6 Pulmonary disease is present in up to 50% of children with multisystem LCH 3 and the mean age of presentation in children has been reported as 11.9 months.…”

Section: Discussionmentioning

confidence: 99%

“…Pneumothoraces that occur in children with pPLCH can be fatal 4 and hence positive-pressure ventilation should be used cautiously. [3][4][5] Reports of ECMO use are limited in pPLCH with previous reports describing its use prior to lung transplantation 5 or to reduce high inspiratory pressures in a patient with known pPLCH and recurrent pneumothoraces. 4 ECMO is a life-saving tool provided by a limited number of centres in the UK.…”

Section: Discussionmentioning

confidence: 99%

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Extracorporeal membrane oxygenation: Bridging therapy in paediatric pulmonary Langerhans cell histiocytosis

Bhatt

Semple

Slater

et al. 2021

J Paediatrics Child Health

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Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Cited by 8 publications

References 19 publications

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

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

Paediatric pulmonary Langerhans cell histiocytosis

Extracorporeal membrane oxygenation: Bridging therapy in paediatric pulmonary Langerhans cell histiocytosis

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