Congenital unilobar pulmonary lymphangiectasis

Rettwitz-Volk, Werner; Schlößer, Rolf; Ahrens, Philip; Hörlin, Albrecht

doi:10.1002/(sici)1099-0496(199904)27:4<290::aid-ppul12>3.0.co;2-f

Cited by 21 publications

(3 citation statements)

References 7 publications

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“…CPL is usually bilateral but congenital unilobar pulmonary lymphangiectasia has been reported (9). The etiology of CPL is unclear, but one hypothesis suggests that the large lymphatic vessels of the lung that normally form at 9-16 weeks of gestation do not undergo normal regression at 20 weeks in these cases (10).…”

Section: Discussionmentioning

confidence: 99%

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

et al. 2014

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Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.Graphical Abstract

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Section: Discussionmentioning

confidence: 99%

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

et al. 2014

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“…Our cases show mild thickening of interstitium of the alveolar walls by collagen, fibroblasts, and some inflammatory cells. Since such interstitial nonspecific changes can be seen in normal lungs or other case reports of CPL (1, 7, 8), it can easily be differentiated from other interstitial lung disease, such as interstitial pneumonia. In the case 1, the ante-mortem clinical data of this neonate could not be obtained.…”

Section: Discussionmentioning

confidence: 99%

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases

et al. 2007

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Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

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“…Congenital dilatation of the pulmonary lymphatic vessels is the key feature of this disease and has been classified according to its presence in other organs and the existence of associated malformations [3]. In the majority of cases, cyanosis is present shortly after birth, though diagnosis can be delayed for several weeks in cases of unilobar presentation [4]. Mortality is higher if both lungs are affected [5], and early foetal signs can be those of non-immune hydrops foetalis [6].…”

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confidence: 99%

Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis

Hoehn

William²,

McPhaden³

et al. 2006

European Respiratory Journal

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Abnormal growth and development of lymphatic pulmonary structures leads to severe hypoxia in congenital pulmonary lymphangiectasis (CPL).This case study aims to determine the cellular source and topographical distribution of the nitric oxide synthases in CPL. It studies the post mortem tissue of a term newborn with the clinical course and histological findings of CPL and three controls without pulmonary pathology.It was found that endothelial cells of pulmonary arteries and lymphatic structures stained significantly more for endothelial nitric oxide synthase protein in the CPL patient compared to the controls.The authors conclude that synthesis of endothelial nitric oxide synthase is upregulated in vascular and lymphatic endothelial cells in congenital pulmonary lymphangiectasis.

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Congenital unilobar pulmonary lymphangiectasis

Cited by 21 publications

References 7 publications

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases

Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis

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