PDF(Pubmed)
Abstract：
UNASSIGNED: To elaborate the clinical characteristics of congenital pulmonary lymphangiectasia in a neonate with hydrops fetalis. This could be an alert in considering it as a differential diagnosis for neonates with acute respiratory failure.
UNASSIGNED: We reviewed and analyzed single-center registry patients who underwent cadaveric autopsies in the Department of Pathology at Children\'s Hospital from January 1, 2010 to December 31, 2021. We aimed to explore the perinatal clinical manifestations associated with congenital pulmonary lymphangiectasis (CPL). Literature was reviewed to summarize the common features of CPL in pregnancy from individual cases, and to facilitate prenatal and intrapartum diagnosis prognosis, and assessment of medical emergencies.
UNASSIGNED: Thirty-four patients were included, and the main causes of death were intrauterine infection (n = 6), severe pneumonia (n = 11), spontaneous pneumothorax (n = 3), hemorrhagic shock (n = 2), CPL (n = 1), and other non-respiratory failure manifestations (n = 12). The manifestations of respiratory distress in CPL were different from those of intrauterine infections and respiratory failure due to parenchymal lung lesions. These include prenatal presentation of fetal edema, postnatal presentation of uncorrectable respiratory failure with severe hypoproteinemia, pneumothorax and interstitial emphysema on imaging, and poor response to treatment with surfactant-like substances. Thus, when the pregnancy tests reveal fetal edema and postnatal presentation of acute, respiratory distress, the diagnosis of CPL should be considered first, and corresponding medical care should be implemented to improve the survival rate.
UNASSIGNED: CPL is a rare pulmonary defect, and its perinatal clinical manifestations can often be neglected. For children with prenatal fetal edema who die after birth due to progressive respiratory distress, a timely autopsy is of utmost importance to clarify the etiology, improve understanding of CPL, and diagnose early to allow for proper prenatal and postnatal care.
UNASSIGNED: We reviewed and analyzed single-center registry patients who underwent cadaveric autopsies in the Department of Pathology at Children\'s Hospital from January 1, 2010 to December 31, 2021. We aimed to explore the perinatal clinical manifestations associated with congenital pulmonary lymphangiectasis (CPL). Literature was reviewed to summarize the common features of CPL in pregnancy from individual cases, and to facilitate prenatal and intrapartum diagnosis prognosis, and assessment of medical emergencies.
UNASSIGNED: Thirty-four patients were included, and the main causes of death were intrauterine infection (n = 6), severe pneumonia (n = 11), spontaneous pneumothorax (n = 3), hemorrhagic shock (n = 2), CPL (n = 1), and other non-respiratory failure manifestations (n = 12). The manifestations of respiratory distress in CPL were different from those of intrauterine infections and respiratory failure due to parenchymal lung lesions. These include prenatal presentation of fetal edema, postnatal presentation of uncorrectable respiratory failure with severe hypoproteinemia, pneumothorax and interstitial emphysema on imaging, and poor response to treatment with surfactant-like substances. Thus, when the pregnancy tests reveal fetal edema and postnatal presentation of acute, respiratory distress, the diagnosis of CPL should be considered first, and corresponding medical care should be implemented to improve the survival rate.
UNASSIGNED: CPL is a rare pulmonary defect, and its perinatal clinical manifestations can often be neglected. For children with prenatal fetal edema who die after birth due to progressive respiratory distress, a timely autopsy is of utmost importance to clarify the etiology, improve understanding of CPL, and diagnose early to allow for proper prenatal and postnatal care.
摘要：
■阐述胎儿水肿新生儿先天性肺淋巴管扩张症的临床特征。这可能是将其视为急性呼吸衰竭新生儿的鉴别诊断的警报。
我们回顾并分析了2010年1月1日至2021年12月31日在儿童医院病理科接受尸体尸检的单中心登记患者。我们旨在探讨与先天性肺淋巴管扩张症（CPL）相关的围产期临床表现。文献复习,从个别病例中总结妊娠合并妊娠的共同特点,并促进产前和产时诊断预后，和医疗紧急情况的评估。
■包括34名患者，死亡原因主要为宫内感染(n=6),重症肺炎（n=11），自发性气胸（n=3），失血性休克（n=2），CPL（n=1），和其他非呼吸衰竭表现（n=12）。CPL中呼吸窘迫的表现与宫内感染和肺实质病变引起的呼吸衰竭不同。这些包括胎儿水肿的产前表现，产后出现严重低蛋白血症的不可纠正的呼吸衰竭，气胸和间质性肺气肿成像，和对表面活性剂样物质的治疗反应差。因此，当妊娠试验显示胎儿水肿和产后出现急性时，呼吸窘迫,应首先考虑CPL的诊断，并实施相应的医疗护理，以提高生存率。
■CPL是一种罕见的肺缺损，其围产期临床表现常被忽视。对于因进行性呼吸窘迫而在出生后死亡的产前胎儿水肿的儿童，及时的尸检对于明确病因至关重要，提高对CPL的理解，早期诊断，以便适当的产前和产后护理。
我们回顾并分析了2010年1月1日至2021年12月31日在儿童医院病理科接受尸体尸检的单中心登记患者。我们旨在探讨与先天性肺淋巴管扩张症（CPL）相关的围产期临床表现。文献复习,从个别病例中总结妊娠合并妊娠的共同特点,并促进产前和产时诊断预后，和医疗紧急情况的评估。
■包括34名患者，死亡原因主要为宫内感染(n=6),重症肺炎（n=11），自发性气胸（n=3），失血性休克（n=2），CPL（n=1），和其他非呼吸衰竭表现（n=12）。CPL中呼吸窘迫的表现与宫内感染和肺实质病变引起的呼吸衰竭不同。这些包括胎儿水肿的产前表现，产后出现严重低蛋白血症的不可纠正的呼吸衰竭，气胸和间质性肺气肿成像，和对表面活性剂样物质的治疗反应差。因此，当妊娠试验显示胎儿水肿和产后出现急性时，呼吸窘迫,应首先考虑CPL的诊断，并实施相应的医疗护理，以提高生存率。
■CPL是一种罕见的肺缺损，其围产期临床表现常被忽视。对于因进行性呼吸窘迫而在出生后死亡的产前胎儿水肿的儿童，及时的尸检对于明确病因至关重要，提高对CPL的理解，早期诊断，以便适当的产前和产后护理。
