Recurrent pneumomediastinum and pneumothorax in Langerhans cell histiocytosis

Yule, S. M.; Hamilton, J. R. L.; Windebank, Kevin

doi:10.1002/(sici)1096-911x(199708)29:2<139::aid-mpo13>3.0.co;2-a

Cited by 28 publications

(14 citation statements)

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“…Indeed, all deceased patients died within a median of 7 months (range: 4.6–13.7) after the PLCH diagnosis. For hospitalizations with pneumothorax, recurrences of pneumothorax were common, despite pleurodesis [ 19 , 20 , 25 , 26 ]. Optimal management for preventing pneumothorax recurrences has not been clearly established for children with LCH.…”

Section: Discussionmentioning

confidence: 99%

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Louet¹,

Barkaoui²,

Miron³

et al. 2020

Orphanet J Rare Dis

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Background Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. Methods Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure. Results The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine–corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging. Conclusions Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy.

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Section: Discussionmentioning

confidence: 99%

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Louet¹,

Barkaoui²,

Miron³

et al. 2020

Orphanet J Rare Dis

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“…Ventilation strategies to deal with the risk of recurrent air leaks must be considered in intensive care for children with respiratory failure. The use of ECMO (extracorporeal membrane oxygenation) to allow time for the benefits of chemotherapy or for lung transplantation has also been discussed [ 59 ]. Clearly, this is a difficult consideration when prognosis is often unpredictable.…”

Section: Managementmentioning

confidence: 99%

Paediatric pulmonary Langerhans cell histiocytosis

Barclay

Devaney

Bhatt

2020

Breathe

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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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“…L'hyperpression à glotte fermée peut être secondaire à une obstruction bronchique aigue, l'exemple de la crise d'asthme et l'inhalation de corps étranger [5, 6]; ou en cas de ventilation mécanique utilisant de grands volumes et/ou des valeurs élevées de pression en fin d'expiration; ou encore lors d'une toux quinteuse ou d'efforts de vomissement [7, 8]; et enfin lors d'une manœuvre de Valsalva comme la défécation, la manœuvre d'Hemlich ou de l'inhalation volontaire de cocaïne, de marijuana ou d'ecstasy [9, 10]. Un deuxième mécanisme du pneumomédiastin spontané fait appel à des lésions directes des parois alvéolaires [11, 12]. C'est le cas des pneumopathies bactériennes essentiellement à staphylocoques dorés, virales (grippe, rougeole, coqueluche) et parasitaires notamment le cas de la pneumocystose chez les patients immunodéprimés [11, 13].…”

Section: Discussionunclassified

“…C'est le cas des pneumopathies bactériennes essentiellement à staphylocoques dorés, virales (grippe, rougeole, coqueluche) et parasitaires notamment le cas de la pneumocystose chez les patients immunodéprimés [11, 13]. La miliaire tuberculeuse peut se compliquer aussi d'un pneumomédiastin ainsi que les pneumopathies infiltrantes diffuses au stade de fibrose [12]. Le mécanisme le plus fréquent dans notre série était celui de l'hyperpression à glotte fermé.…”

Section: Discussionunclassified

Pneumomédiastin spontané: à propos de 18 cas

Chaanoun¹,

Zaghba²,

Benjelloun³

et al. 2018

Pan Afr Med J

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Le pneumomédiastin spontané se définit par la présence d'air au niveau du médiastin en l'absence de cause traumatique ou iatrogène. Son diagnostic repose sur la radiographie thoracique. Le recours à d'autres examens paracliniques, tels que la tomodensitométrie thoracique ou la fibroscopie bronchique ou digestive, s'impose parfois. L'évolution est le plus souvent favorable. Nous rapportons 18 cas de pneumomédiastin spontané, colligés au service des maladies respiratoires du CHU Ibn Rochd de Casablanca entre 2006 et 2017. Il s'agit de 13 hommes et de cinq femmes dont la moyenne d'âge était de 24 ans. La symptomatologie clinique était dominée par la douleur thoracique rétrosternale. Les circonstances de survenue du pneumomédiastin étaient des quintes de toux dans sept cas, une crise d'asthme dans cinq cas, une consommation de narguilé et des vomissements Itératifs dans deux cas chacun, un accouchement et une exacerbation d'origine bactérienne de BPCO dans un cas chacun. L'évolution était favorable dans tous les cas avec une résorption spontanée du pneumomédiastin. Aucune récidive n'est survenue après un recul moyen de 3 ans.

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Recurrent pneumomediastinum and pneumothorax in Langerhans cell histiocytosis

Cited by 28 publications

References 13 publications

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Paediatric pulmonary Langerhans cell histiocytosis

Pneumomédiastin spontané: à propos de 18 cas

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