Risk and relevance of open lung biopsy in pediatric ECMO patients: the Dutch experience

Houmes, Robert Jan; Kate, Chantal A. ten; Wildschut, Enno D.; Verdijk, Robert M.; Wijnen, René; Blaauw, Ivo de; Tibboel, Dick; Heijst, Arno van

doi:10.1016/j.jpedsurg.2016.11.031

Cited by 24 publications

(29 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Even when lung biopsy is considered essential, it is best performed in centres where there is a significant throughput of children undergoing these procedures for diffuse lung diseases. Lung biopsies can yield a confirmatory histological diagnosis in a large proportion of cases but can be associated with significant morbidity and some mortality [ 33 , 34 ].…”

Section: Diagnosismentioning

confidence: 99%

Paediatric pulmonary Langerhans cell histiocytosis

Barclay

Devaney

Bhatt

2020

Breathe

View full text Add to dashboard Cite

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.

show abstract

Section: Diagnosismentioning

confidence: 99%

Paediatric pulmonary Langerhans cell histiocytosis

Barclay

Devaney

Bhatt

2020

Breathe

View full text Add to dashboard Cite

show abstract

“… 1 , 4 , 7 , 8 , 19 , 23 , 28 , 29 , 37 Ideally, an open lung biopsy is performed before ECMO therapy has started or at least before a “second-run” ECMO therapy is considered. 7 , 14 , 27 , 28 On the other hand, ECMO therapy can provide an excellent bridge to lung transplantation in ACD/MPV patients with a mild phenotype. 12 , 36 To date, seven ACD/MPV patients receiving bilateral lung transplantation have been reported in the United States with a comparable five-year survival (56%) to infants transplanted for other indications at the St. Louis Children’s Hospital, Missouri.…”

Section: Treatmentmentioning

confidence: 99%

Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects

et al. 2018

Self Cite

View full text Add to dashboard Cite

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal disorder mainly involving the vascular development of the lungs. Since its first description, significant achievements in research have led to a better understanding of the underlying molecular mechanism of ACD/MPV and genetic studies have identified associations with genomic alterations in the locus of the transcription factor FOXF1. This in turn has increased the awareness among clinicians resulting in over 200 cases reported so far, including genotyping of patients in most recent reports. Collectively, this promoted a better stratification of the patient group, leading to new perspectives in research on the pathogenesis. Here, we provide an overview of the clinical aspects of ACD/MPV, including guidance for clinicians, and review the ongoing research into the complex molecular mechanism causing this severe lung disorder.

show abstract

“…Rates of blood loss and death did not differ in comparison with surgical intervention without ECMO. 7 In the case we presented here, the inability to wean the patient off ECMO support for several days suggested surgical intervention to be unavoidable. Thus, our patient's necrotizing pneumonia was successfully treated by surgical lobectomy while he was on ECMO support.…”

Section: Discussionmentioning

confidence: 80%