Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

2006

Wilson RD,
Pawel B,
Bebbington M,
Johnson MP,
Lim FY,
Stamilio D,
Silber A,
Zakii E,
Flake AW.
The Center for Fetal Diagnosis and Treatment, Department of Surgery at The Children's Hospital of Philadelphia/University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4399, USA. wilsonrd@email.chop.edu

OBJECTIVE: Case report and literature review for congenital pulmonary lymphangiectasis (CPL)

CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL.

DISCUSSION: Review of pathology, clinical course, and genetics of CPL. CONCLUSION: This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.

Wiley Interscience

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Related Abstract

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Pleural effusions in the neonate.

2006

Rocha G,
Fernandes P,
Rocha P,
Quintas C,
Martins T,
Proenca E.

Division of Neonatology, Department of Pediatrics, Hospital de Sao Joao, University Hospital, Porto, Portugal. gusrocha@oninet.pt

INTRODUCTION: Pleural effusions are rare in the neonate and may be associated to several clinical conditions. Only a few series of pleural effusions in the fetus and newborn are described in the literature. AIM: This study was undertaken to determine more accurately the causes and prognostic significance of pleural effusions in a population of high-risk neonates.

MATERIALS AND METHODS: A retrospective chart review of 62 neonates admitted to the neonatal intensive care unit of six medical centers in the north of Portugal, between 1997 and 2004, that presented the diagnosis of pleural effusion.

RESULTS: 33M/29F newborns; preterms 47 (76%); GA 33 (25-40) wk; BW 1830 (660-4270) g; C-section 39 (63%). Pleural effusions were congenital in 20 (32%) newborns and acquired in 42 (68%). Congenital pleural effusions occurred as fetal hydrops in 11 (18%) patients and as chylothorax in 9 (15%). In four cases of hydrops, the cause was a congenital chylothorax. Congenital chylothorax (n=13) was the most common (65%) congenital pleural effusion in this study. The incidence of congenital chylothorax was 1:8.600 deliveries and male:female ratio was 2:1. Mortality occurred in five newborns due to pulmonary hypoplasia. Traumatic (iatrogenic) were the most frequent (n=31) acquired pleural effusions. These included 8 (13%) cases of hemothorax and 8 (13%) cases of total parenteral nutrition leakage. Pleural effusions after intra-thoracic surgery were mainly (79%) chylothoraces. There were 11 (26%) non-iatrogenic acquired pleural effusions. No mortality was associated with acquired pleural effusions.

CONCLUSIONS: Congenital pleural effusions usually occur as hydrops or congenital chylothorax. Traumatic (iatrogenic) are the most frequent acquired pleural effusions in a tertiary NICU. Pleural effusions after intra-thoracic surgery are mainly chylothoraces. Non-iatrogenic acquired pleural effusions are associated to several clinical conditions, and mortality is usually associated to the underlying condition.

Keywords:
Chylothorax, hydrops, newborn, pleural effusion

Meta Press