Congenital Pulmonary Lymphangiectasia Research Articles

Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.

Linfangiectasia pulmonar congênita unilateral: um relato de caso

Pulmonary lymphangiectasia is a rare developmental disorder in which lymphatic vessels proliferate and dilate. Based on pathogenesis and clinical phenotype, it can be classified as: primary or congenital pulmonary lymphangiectasia (CPL), or secondary. The diagnosis is made by clinical characteristic and imaging and histopathological exams features. Lung biopsy is considered the gold standard for the diagnosis of CPL and is also useful to distinguish between interstitial lung diseases or other forms of pulmonary lymphatic dilation. The definitive treatment of the isolated form can be curative with resection of the affected area. In diffuse form, the treatment is palliative and aims to limit the production and accumulation of lymph in the pulmonary serosa. Currently, the conservative treatment is recommended for chylothorax correction. This article aims to report the case of a patient with a diagnosis of congenital pulmonary lymphangiectasia with good evolution, emphasizing the importance of early diagnosis and therapeutic approach, and to compare with what has been described in the literature.

Pulmonary lymphangiectasia a rare cause of chylothorax: A case report

Background: Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the pulmonary lymphatic vessels. PL should be considered in the differential diagnosis in infants with pleural effusion, especially in the presence of chylothorax. Case Presentation: A 3-week-old neonate presented with tachypnea, cough, and reluctance to feed. Work-up found bilateral chylous pleural effusion. The patient was initially managed conservatively with bilateral tube thoracostomies and octreotide, however output from chest tubes did not subside. A left thoracotomy was performed with wedge biopsy of the apical segment of the left inferior lung lobe and multiple titanium clips were used to control thoracic duct. Postoperatively the patient continued to have high chest tube output, despite placement of clips. Histology of the biopsy confirmed the diagnosis of congenital pulmonary lymphangiectasia. Conclusion: Pulmonary lymphangiectasia is a rare cause of chylothorax. The treatment is often challenging as found in the index case.

Congenital Pulmonary Lymphangiectasia impersonating pulmonary interstitial emphysema in an extremely low gestational age neonate

Background: The purpose of this case report is to illustrate the clinical course of an extremely rare case of Congenital Pulmonary Lymphangiectasia (CPL) presenting as an early pulmonary interstitial emphysema (PIE) complicating respiratory distress syndrome, in an extremely low gestational age neonate (ELGAN) along with a short review of the literature. Case presentation: This 24 weeks ELGAN male with radiologic changes consistent with pulmonary interstitial emphysema (PIE) complicating respiratory distress syndrome which later progressed to bilateral cystic lung changes, expired at 23 days of age. Maternal history was complicated by E. coli urinary tract infection and Group-B Strep chorioamnionitis. The infant remained intubated throughout the hospital course and received antibiotics initially and terminally. His tracheal aspirate cultures also grew Mycoplasma hominis, and Ureaplasma urealyticum, treated with azithromycin. An autopsy revealed diffuse bilateral congenital lymphangiectasia. Postmortem blood and lung tissue cultures were positive for methicillin-resistant Staphylococcus aureus (MRSA). Conclusion: Despite its extreme rarity, the authors recommend considering congenital pulmonary lymphangiectasia in the differential diagnosis, in an extremely preterm neonate, presenting with early pulmonary interstitial emphysema (PIE), and respiratory failure refractory to surfactant, antibiotic therapy, and complex ventilator management.

Congenital interstitial lung diseases: What the anesthesiologist needs to know.

Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Some diseases have a very poor prognosis, whereas others have a benign course with appropriate treatment. The current manuscript reviews congenital interstitial lung diseases that typically affect neonates and young children and may be encountered by the pediatric anesthesiologist.

Pulmonary lymphangiectasia: A case report

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. Here we present a case of pulmonary lymphangiectasia in an one month old child as incidental finding on autopsy.

Congenital pulmonary lymphangiectasia in an extremely low birth weight: a case report

Abstract Objectives Congenital pulmonary lymphangiectasia (CPL) is a rare but fatal disorder of the lung. Case presentation We describe the case of an extremely low birth weight (ELBW) infant who presented with severe respiratory distress and recurrent bilateral pneumothorax. He died on day 17. The post-mortem examination of the lungs showed numerous cystic spaces, ranging from 1 to 2 mm in size, in the visceral pleura, in the thickened interlobular septum and hilum. A diagnosis of primary CPL was made. Conclusions We intend to underline that CPL, albeit rare, must be one of the differential diagnoses in infants with severe neonatal respiratory distress not responding to intensive care. Chronic interstitial lung diseases are a challenging diagnostic clinical problem, which requires a multidisciplinary approach. Histological lung examination may be useful to demonstrate the presence of CPL.

Congenital Pulmonary Lymphangiectasia: A Disorder not only of Fetoneonates.

Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung, characterized by dilation of pulmonary subpleural, interlobar, perivascular and peribronchial lymphatics. The incidence of CPL among stillborn and neonates was estimated to be <1%. The etiology of CPL is unknown. However, it has been suspected to be of a genetic background. Recent basic studies revealed that it might be caused by the FOXC2, Vegfr-3 and integrin α9β1gene mutations. A clinical diagnosis of CPL can be made much easier in full-term neonates who present with respiratory distress, pleural (especially chylous) effusions with or without generalized edema. In infancy, the diagnosis seems to be more difficult due to the nonspecific respiratory symptoms like persistent tachypnea, cough and wheeze. Lung biopsy with subsequent histological and immunohistochemical studies is the golden diagnostic method of CPL. Immunohistochemical staining for endothelial cell markers CD31, CD34 and D2-40 confirms lymphatic origin. Therapeutic strategies include supportive, nutritional, investigational, aggressively interventional and surgical regimens, most of which have shown promising outcomes. Although CPL was once regarded as a disorder of very poor prognosis in neonatal onset cases, teenager and adult patients have shown good outcomes upon long-term follow-up.Die angeborene pulmonale Lymphangiektasie (CPL) ist eine seltene Entwicklungsstörung der Lunge, die durch eine Dilatation der pulmonalen subpleuralen, interlobären, perivaskulären und peribronchialen Lymphgefäße charakterisiert ist. Die Inzidenz der CPL bei Totgeburten und Neugeborenen wird <1% geschätzt. Die Ätiologie der CPL ist unbekannt. Allerdings wird ein genetischer Hintergrund vermutet. Neuere Grundlagenstudien zeigten, dass die CPL durch FOXC2, Vegfr-3 und Integrin α9β1-Genmutationen verursacht sein könnte. Die klinische Diagnose der CPL ist sehr viel einfacher in Reifgeborenen zu stellen, die Atemnot, Pleuraergüsse (vor allem chylöse) mit und ohne generalisiertem Ödem aufweisen. In der frühen Kindheit ist die Diagnose aufgrund der unspezifischen respiratorischen Symptomatik wie persistierende Tachypnoen, Husten oder Röcheln schwerer zu stellen. Die Lungenbiopsie mit anschließenden histologischen und immunhistochemischen Untersuchungen ist der Goldstandard für die Diagnose der CPL. Die immunhistochemische Färbung der Endothelzellmarker CD31, CD34 und D2-40 bestätigt den lymphatischen Ursprung. Die Behandlungsstrategien umfassen unterstützende, alimentäre, in Erprobung befindliche, aggressiv-interventionelle und chirurgische Behandlungspläne, von denen die meisten ermutigende Ergebnisse zeigten. Obwohl die CPL einst bei Fällen mit Ausbruch im Neugeborenenalter als Erkrankung mit sehr schlechter Prognose galt, zeigen Teenager und erwachsene Patienten in der Langzeit-Nachbeobachtung gute Verläufe.

Congenital pulmonary lymphangiectasia and early mortality after stage 1 reconstruction procedures.

Pulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions. An institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. During that period, 1669 stage 1 procedures were performed. Autopsy lung specimens were reviewed by a paediatric pathologist. Patients who died of suspected technical issues were excluded. A total of 54 patients were included, and of these seven cases (8.5%) of pulmonary lymphangiectasia were identified. The mean estimated gestational age was 38.2±2.4 weeks, and the mean birth weight was 3.0±0.6 kg. The median interval between surgery and death was 1 day (with a range from 0 to 18 days). The atrial septum was intact in one patient (14.3%), restrictive in three patients (42.9%), and unrestrictive in three patients (42.9%). Pulmonary lymphangiectasia may develop in hypoplastic left heart syndrome with or without a restrictive atrial septum. As standard prenatal diagnostic evaluations and treatment methods for pulmonary lymphangiectasia are limited, this may be an important contributor to early and late mortality following stage 1 reconstruction for hypoplastic left heart syndrome.

Congenital pulmonary lymphangiectasis mimicking congenital pulmonary interstitial emphysema in a patient with congenital diaphragmatic hernia

Congenital pulmonary lymphangiectasis is a rare abnormality of the lung characterized by dilated pulmonary lymphatic vessels. The diagnosis of congenital pulmonary lymphangiectasis is challenging due to its unspecific presentation, as well as possible radiological imitation of interstitial emphysema. We present a patient where diagnosis was hampered by the extremely rare combination of pulmonary lymphangiectasis and congenital diaphragmatic hernia.

Clinical Features of Neonates with Hydrops Fetalis.

The objective of this study was to evaluate the clinical characteristics of neonates with hydrops fetalis to improve recognition of the disease. The clinical data of 10 neonates with hydrops fetalis were retrospectively studied. Prenatal characteristics, causes, clinical features, and prognosis were explored. Eight neonates presenting abnormal nonstress test suffered from severe neonatal asphyxia at birth and were resuscitated by endotracheal intubation. Nine had skin edema, eight had pleural effusions with one unilateral and seven bilateral. Six had ascites, eight had polyhydramnios, one had multiple malformations and one had chromosome abnormalities. One survived and nine died. Six died due to resuscitation failure in delivery room, two died due to giving up after 1 day and one died due to the treatment failure after 6 months. Causes of hydrops fetalis were a congenital diaphragmatic hemangioma, recurrent atrial premature beats, genetic syndrome suspicious, Down syndrome, congenital pulmonary lymphangiectasia, anemia, paroxysmal supraventricular tachycardia, placental chorioangioma, and idiopathic edema. The prognosis varied because of different etiologies of hydrops fetalis. Severe cases frequently had skin edema and high rate of asphyxia at birth and difficult resuscitation. Timely intrauterine interventions were helpful for successful resuscitation. A well-prepared resuscitation team and the effectiveness of resuscitation could correlate to increasing survival rate.

English

Congenital high airway obstruction syndrome (CHAOS) results in a predictable constellation of findings: large echogenic lungs flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops. 1 The finding of CHAOS on prenatal ultrasound examination is diagnostic of complete or near-complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia. A greater understanding of the natural history of CHAOS may permit improved prenatal and perinatal management. INTRODUCTION: Congenital chest malformations are rare, and may involve the lung parenchyma, bronchi, arterial supply, and venous drainage. They include congenital cystic adenomatoid malformation (CCAM), congenital diaphragmatic hernia (CDH), bronchopulmonary sequestration (BPS), congenital hydrothorax, and congenital lobar emphysema. Rarer entities include congenital high airway obstruction syndrome (CHAOS), congenital bronchogenic cyst, bronchial atresia, pulmonary Arterio Venous malformation (PAVM), congenital pulmonary lymphangiectasia, pulmo- nary hypoplasia-aplasia, mediastinal teratoma, and mediastinal lymphangioma.2 Sometimes there may be compromise in normal pulmonary development, causing hypoplastic lung formation. Pulmonary abnormalities are not mutually exclusive, since abnormalities frequently occur together as hybrid conditions. MATERIALS AND METHODS: Here we present a case of a fetus post-delivery found to have large hyperechoic lungs, flattened diaphragm and hydrops in the prenatal scan, which form a triad in the detection of CHAOS. A brief review of literature is discussed. CASE PRESENTATION: In our case we had a twenty six years old pregnant lady with twenty two weeks history of amenorrohea who had come for a routine antenatal scan. She was the mother of a two year old child which was born normally and was doing well. On antenatal ultrasound of the present pregnancy, the fetus was found to be of approximately twenty weeks gestation. The fetus was found to have large hyperechoic lungs, flattened diaphragm and hydrops which form a triad in the detection of CHAOS. (Fig: 1) The patient and her husband were counseled regarding the risks involved on progression of the fetus till birth. The parents agreed to terminate the pregnancy voluntarily. A dead male fetus was delivered and sent for autopsy subsequently. An interesting finding noted during dissection of neck and thoracic organs was an abrupt ending of the trachea approximately 11 mm caudal to the lower end of larynx. (Fig: 2 & Fig: 3). The subsequent histopathology showing swollen cells confirmed the diagnosis (Fig: 4)

Lymphatic function is required prenatally for lung inflation at birth.

Mammals must inflate their lungs and breathe within minutes of birth to survive. A key regulator of neonatal lung inflation is pulmonary surfactant, a lipoprotein complex which increases lung compliance by reducing alveolar surface tension (Morgan, 1971). Whether other developmental processes also alter lung mechanics in preparation for birth is unknown. We identify prenatal lymphatic function as an unexpected requirement for neonatal lung inflation and respiration. Mice lacking lymphatic vessels, due either to loss of the lymphangiogenic factor CCBE1 or VEGFR3 function, appear cyanotic and die shortly after birth due to failure of lung inflation. Failure of lung inflation is not due to reduced surfactant levels or altered development of the lung but is associated with an elevated wet/dry ratio consistent with edema. Embryonic studies reveal active lymphatic function in the late gestation lung, and significantly reduced total lung compliance in late gestation embryos that lack lymphatics. These findings reveal that lymphatic vascular function plays a previously unrecognized mechanical role in the developing lung that prepares it for inflation at birth. They explain respiratory failure in infants with congenital pulmonary lymphangiectasia, and suggest that inadequate late gestation lymphatic function may also contribute to respiratory failure in premature infants.

Idiopathic Congenital Chylothorax Presented with Hydrops Fetalis

Idiopathic Congenital Chylothorax Presented with Hydrops Fetalis Although congenital chylothorax is a rare cause of hydrops fetalis with an estimated prevalence of 1/10.000-15.000, it is the most common type of pleural effusion in fetus and newborn. If congenital chylothorax is diagnosed in foetal life with hydrops fetalis, it has high mortality rates. Congenital chylothorax may occur alone. Hovewer it has been also reported with other lymphatic anomalies, congenital heart diseases, Turner, Down and Noonan syndromes, congenital pulmonary lymphangiectasia, cytic hygroma and H-type tracheo-oesophageal fistula. Herein, we report a case of idiopathic congenital chylothorax presented with hydrops fetalis. Chylothorax should be considered in the differential diagnosis of newborns with hydrops fetalis. Early diagnosis and treatment of congenital chylothorax with hydrops fetalis may improve the survival rates of the these newborns.

P06.11: Full term hydrops fetalis caused by congenital pulmonary lymphangiectasia: a case report

<scp>P06</scp>.11: Full term hydrops fetalis caused by congenital pulmonary lymphangiectasia: a case report

Childhood Pneumonectomies: Two Decades' Experience of a Referral Center

We aimed to review the pre- and postoperative characteristics of children undergoing pneumonectomy, with special emphasis on the management of surgical complications, in comparison with the current literature findings. A total of 20 patients who underwent pneumonectomy for various etiologies from 1988 to 2011 were investigated retrospectively with respect to the presenting findings, preoperative evaluation, surgical information, postoperative follow-up, and outcome. A total of 11 girls and 9 boys with a median age of 8 years (0.5-17 years) presented with symptoms of productive cough (n = 9), recurrent chest infections (n = 9), nonproductive cough (n = 1), respiratory distress (n = 1) with a median duration of 2 years (0.16 to 12 years). Medical histories revealed chest infection (n = 11), foreign body aspiration (n = 3), aspiration pneumonia (n = 2), tuberculosis (n = 2), caustic aspiration (n = 1), and congenital lung pathology (n = 1). Clinical evaluation provided diagnoses of bronchiectasis (n = 16), total atelectasis (n = 2), bronchopleural fistula (BPF) (n = 1), and cystic lung disease (n = 1). Pneumonectomy was performed on the right in 6 and left in 14 of the cases. Pericardial (n = 1) and esophageal (n = 1) laceration were the perioperative (11.1%), massive hemorrhage (n = 1) and chylothorax (n = 1) the postoperative (11.1%), and scoliosis (n = 1) and BPF (n = 1) the long-term (11.1%) complications. Pathological evaluation revealed bronchiectasis (n = 14), consisting of three cases with additional sign of foreign body, chronic inflammation (n = 4), atelectasis with fibrous pleural thickening (n = 1), and congenital pulmonary lymphangiectasia (n = 1). A total of 14 cases were free of symptoms and 5 had significant improvement in general condition during the follow-up, for a median duration of 2 years (1 to 10). The one with the diagnosis of Becker muscular dystrophy died 2 years after pneumonectomy because of respiratory failure. Correct selection of indications, careful preoperative preparation with eradication of infection, meticulous performance of surgical and anesthetic techniques, early detection and management of complications, and long-term follow-up including pulmonary rehabilitation are essential parameters to reduce morbidity and mortality rates in childhood pneumonectomy. Better compensatory lung growth and improvement in development after the operation will facilitate better health and improved life quality in children.

Congenital Pulmonary Lymphangiectasia

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.

Revisiting the Histopathologic Spectrum of Congenital Pulmonary Developmental Disorders

A retrospective analysis of lobectomy, pneumonectomy, cystectomy specimens (10 years) and stillbirth/neonatal autopsies (5 years) was carried out to analyze the histologic spectrum of congenital pulmonary developmental disorders/malformations. The autopsy data was analyzed to identify the lesions that are more diffuse and not amenable to surgical management. A total of 166 cases of pulmonary developmental disorders/ malformation were found. Out of 2,155 stillbirth/neonatal autopsies, there were 105 cases of pulmonary hypoplasia, 2 cases of congenital pulmonary lymphangiectasis, 2 cases of extralobar sequestration, and 3 cases of congenital pulmonary airway malformation (CPAM). Among the surgical specimens, there were 21 cases of CPAM, 11 cases of intralobar sequestration, 5 cases of congenital lobar emphysema and 17 cases of bronchogenic cyst. All these malformations have been described in reference to the latest updated classification of developmental disorders/malformations.

Lymphangiectasies pulmonaires congénitales : une cause rare de détresse respiratoire néonatale

Congenital pulmonary lymphangiectasis (CPL) is a rare condition in neonates characterized by abnormal dilatation of the lymphatics draining the interstitial and subpleural spaces of the lungs. Diagnosis is difficult in the neonatal period because respiratory features and radiological findings are not specific of the disease. Definitive diagnosis of CPL can be made only by pathologic examinations. We report a case of a male near-term neonate presenting with severe respiratory distress at birth. The initial chest X-ray showed frosted glass-like infiltrates with air bronchogram suggesting a maternofetal infection or respiratory distress syndrome. The infant required mechanical ventilation and chest tube insertion for right then bilateral pneumothorax. The child died 15 days later in spite of optimal high-frequency ventilation, bilateral pneumothorax drainage, and hemodynamic support. Autopsy revealed features consistent with the diagnosis of CPL. Although CPL is very rare, we should be aware that it is a possible cause of severe unexplained respiratory distress in neonates.

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return

Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlob ular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.