Congenital pulmonary lymphangiectasis resulting in pleural effusions managed by thoracoamniotic shunting

Singh, Paul; Ahmed, Fatimah

doi:10.1016/j.epsc.2013.05.003

Cited by 6 publications

(5 citation statements)

References 35 publications

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“…CPL associated with systemic lymphangiectasia seems to have a slightly better prognosis than isolated CPL. The combination of CPL with hydrops fetalis, bilateral chylothorax or the immediate onset of severe respiratory distress at birth has the worst prognosis [6] . Recently, Faul et al also divided the lymphangiectasis into the primary (congenital) and secondary forms to differentiate it from the lymphangiomatosis, and they noted that the primary form presents in neonates and is usually fatal.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary lymphangiectasia: A case report

Subba¹,

Sathawane²,

Swami³

2021

Int. J. Clin. Diagn. Pathol.

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

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Section: Discussionmentioning

confidence: 99%

Pulmonary lymphangiectasia: A case report

Subba¹,

Sathawane²,

Swami³

2021

Int. J. Clin. Diagn. Pathol.

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“…In the postneonatal period, affected infants may present with chronic lung disease characterized by recurrent cough, wheezing, frequent respiratory exacerbations and even congestive heart failure. [3][4][5][6] PL is usually diagnosed during the prenatal and/or neonatal period or in children or adults when presenting with milder course but can be difficult, as many of the respiratory symptoms and radiologic findings are nonspecific. 3,4 During the prenatal period, obstetric fetal ultrasound is crucial in the antenatal diagnosis of PL and that all causes leading to hydrops featalis have to be considered.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Shadow on Prenatal Sonogram

Lee

Al-Wahab

Ambat

2015

Clin Pediatr (Phila)

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An 11-day-old male infant was referred to be admitted to the neonatal intensive care unit (NICU) from the general pediatric clinic with a 1-day history of progressively worsening respiratory distress. Over the past 24 hours, he developed fast breathing and some grunting respirations. He had no nasal congestion, cough, fever, or known sick contact. His mother also reported that he had become fussy, had decreased oral intake, and had taken longer to finish his bottle.The infant was the second child born of nonconsanguineous parentage, to a 24 year-old-woman at 39 weeks by normal spontaneous vaginal delivery with a birthweight of 3.01 kg. Antenatal ultrasound performed at 24 weeks' gestation revealed a pulmonary shadow in the right lung, which persisted on follow-up ultrasound at 28 weeks. Otherwise, there were no other known pregnancy complications. He was admitted to the NICU after birth for evaluation of possible pleural effusion, which was not found on serial chest radiography. He was discharged home on day 5 without any respiratory symptoms.Vital signs on admission were as follows: temperature 98.6°F, pulse 143 beats/min, respiratory rate 67 to 75 breaths/min, blood pressure 87-93/49-61 mm Hg, and oxygen saturation of >90% while receiving oxygen by nasal cannula. He was grunting with subcostal, intercostal retractions, nasal flaring, and decreased air entry on the right hemithorax. The rest of the physical examination findings displayed absence of murmur, abdominal distention, hepatosplenomegaly, dysmorphic features, or birth defects.Chest radiograph showed diffuse reticular pulmonary opacity with large right pleural effusion (Figure 1).Initial laboratory studies included an arterial blood gas with pH of 7.33, a P CO2 of 45 mm Hg, calculated bicarbonate of 23.6 mEq/L, and a base excess of −2.2. The white blood cell count (WBC) was 8.8 × 10 3 /µL, with 49% neutrophils, 35% lymphocytes, and 12% monocytes. The C-reactive protein level was 0.4 mg/L. Thoracentesis performed on admission followed by ultrasound-guided placement of right chest tube drained yellow, turbid fluid. Pleural fluid analysis was consistent 604599C PJXXX10.

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“…However, CPL was considered to be a result of a failure of normal regression of the lymphatics after the 18-20th week of gestation [28]. CPL was also described to be associated with chromosomal anomalies, and it was found to be concurrent with genetic disorders and congenital heart disease in conjunction with a variety of congenital heart defects, such as total anomalous pulmonary venous return [16], stenosis of the pulmonary and mitral valves, hypoplastic left heart, cor triatriatum, atresia of the pulmonary veins and atrioventricular canal defects [27]. It is hypothesized that cardiac lesions somehow interfere with the normal regression of the lymphatic tissue elements during the midsecond trimester [11].…”

Section: Etiologymentioning

confidence: 99%

“…Barker et al [2] described infants and children with CPL and normal birth weight but initial poor weight gain between 3 months and the first year of life, who eventually improved. Antenatally, CPL is most commonly associated with chylous pleural effusions, and sometimes with chylopericardium, chylous ascites, hydrops fetalis, or generalized lymphedema [27]. Postnatally, newborns with CPL present with respiratory distress, which often requires mechanical ventilation support [27], and children with CPL may present with respiratory difficulties, and even congestive heart failure [4].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Antenatally, CPL is most commonly associated with chylous pleural effusions, and sometimes with chylopericardium, chylous ascites, hydrops fetalis, or generalized lymphedema [27]. Postnatally, newborns with CPL present with respiratory distress, which often requires mechanical ventilation support [27], and children with CPL may present with respiratory difficulties, and even congestive heart failure [4]. Apart from circulatory presentations, some infants with CPL develop gastroesophageal reflux, symptoms of gastroesophageal reflux may include a sensation of warmth or burning rising up to the neck area (heartburn or pyrosis), swallowing difficulties (dysphagia), and chest pain [5].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

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Congenital Pulmonary Lymphangiectasia: A Disorder not only of Fetoneonates

Yuan

2017

Klin Padiatr

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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.

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Congenital pulmonary lymphangiectasis resulting in pleural effusions managed by thoracoamniotic shunting

Cited by 6 publications

References 35 publications

Pulmonary lymphangiectasia: A case report

Pulmonary lymphangiectasia: A case report

Pulmonary Shadow on Prenatal Sonogram

Congenital Pulmonary Lymphangiectasia: A Disorder not only of Fetoneonates

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