Congenital pulmonary lymphangiectasia: an unusual presentation of nonimmune hydrops in a preterm infant.

June 2012

Mele P, Sridhar S.

Source

Division of Neonatology, Department of Pediatrics, Stony Brook Long Island Children's Hospital, New York 11794, USA. Patricia.Mele@sbumed.org

Abstract

Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung. CPL can present antenatally as nonimmune hydrops with pleural effusions, which are frequently associated with polyhydraminos. CPL presents in the neonatal period with severe respiratory distress and can be complicated by chylothorax. Diagnosis is difficult, as radiographic findings are nonspecific. Lung biopsy is indicated in some severe cases to diagnose CPL. The majority of infants with this condition rarely survive the neonatal period and are often diagnosed at autopsy. The final diagnosis is confirmed histologically by lung biopsy or at postmortem. We report a rare case of CPL in a preterm female infant presenting with severe nonimmune Hydrops fetalis who survived the neonatal period, and report clinical follow-up to early childhood.

Lippincott, Williams, Wilkins

Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy.

Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy.

Neonatology. 2007

Akcakus M, Koklu E, Bilgin M, Kurtoglu S, Altunay L, Canpolat M, Budak N.
Division of Neonatology, Department of Paediatrics, School of Medicine, Erciyes University, Kayseri, Turkey.

Author Contacts
Esad Koklu, MDDivision of Neonatology, Department of PaediatricsSchool of Medicine, Erciyes UniversityTR-38039 Kayseri (Turkey)Tel. +90 352 437 4937, Fax +90 352 437 5825, E-Mail esad@erciyes.edu.tr

Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. Treatments for lymphangiectasia have included corticosteroids for patients with primary inflammatory conditions, dietary modifications, surgical resection for isolated lesions, octreotide, antiplasmin therapy and fibrin glue pleurodesis. However, there is no experience with pleurodesis by autologous blood therapy in the literature. We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of life and improved with pleurodesis by autologous blood therapy.

Karger

Chronic interstitial lung disease in children

Chronic interstitial lung disease in children
J Pediatr (Rio J). 2007 May/June

Paiva MA, Amaral SM.

Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil. mariaaparecida.paiva@gmail.com.

OBJECTIVES: To describe clinical and diagnostic features and the results of therapeutic conduct in a group of pediatric patients with chronic interstitial lung disease.

METHODS: A retrospective study of 25 immunocompetent patients, aged 2 months to 17 years, with chronic interstitial lung disease, admitted to the Pediatric Pulmonary Section, Department of Pediatrics, Hospital dos Servidores do Estado, over a 20-year period (1984-2004). A routine protocol for persistent chronic pneumonias was used and the patients with interstitial lung disease were selected. Clinical, laboratory and imaging data were analyzed.

RESULTS: Twenty-five patients were diagnosed with chronic interstitial lung disease, 13 were aged less than 2 years and 17 were male. Diagnoses were made based on history, physical examination and routine tests in one case, based on more complex tests in three cases and based on the results of invasive tests in 21 cases (20 by lung biopsy and one by bronchoalveolar lavage). Except for one patient with pulmonary lymphangiectasia, the long-term treatment (1 to 7 years) consisted of corticosteroid, in six cases associated with hydroxychloroquine. Four patients required home oxygen therapy. The authors followed the patients in the outpatient department (6 a 8 visits/year). Patient outcome was: good (15); regular, with mild sequelae (4); and poor, with severe sequelae (3). One patient was lost in the follow-up period and two died.

CONCLUSIONS: Chronic interstitial lung diseases in children are a group of rare pulmonary disorders, but a relevant one because of the possible progression to pulmonary fibrosis. Early diagnosis and a long-term, specialized treatment and follow-up are important for the patient outcome. Pediatricians should be aware of these diseases because in many cases diagnosis and treatment are overlooked.

PMID: 17551654 [PubMed - as supplied by publisher]

Congenital pulmonary lymphangiectasia: CT and pathologic findings.

J Thorac Imaging. 2004 Jan

Nobre LF, Muller NL, de Souza Junior AS, Marchiori E, Souza IV.

Department of Radiology, Universidade Federal de Santa Catarina, Florianopolis, Santa Catarina, Brasil.

Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood.

The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy.

The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.

Journal of Thoracic Imaging