An unusual case of metastatic trophoblastic neoplasm presenting with diffuse cystic lung disease and pulmonary artery pseudoaneurysms in a teenager.

Diffuse cystic lung diseases (DCLDs) are a diverse group of lung disorders characterized by the presence of multiple air filled cysts within the lung tissue. These cysts are thin walled and surrounded by normal lung tissue. In adults, DCLD can be associated with various conditions such as lymphangioleiomyomatosis (LAM), Langerhans cell histiocytosis, cancers, and more. In children, DCLD is often linked to lung developmental abnormalities, with bronchopulmonary dysplasia being a common cause. Patients with pulmonary cysts are typically asymptomatic, but some may experience mild symptoms or pneumothorax. While DCLD in children is rarely due to malignancy, metastatic lung disease can be a cause. It is important for clinicians to be aware of the possibility of metastatic lung disease when encountering DCLD.

Complications of lymphangioleiomyomatosis in pregnancy: a case report and review of the literature.

Lymphangioleiomyomatosis is a rare cystic lung disease primarily affecting premenopausal females and may be exacerbated by pregnancy. We conducted a literature review of lymphangioleiomyomatosis during pregnancy with a specific focus on related maternal morbidity and obstetrical outcomes. We also report a case of lymphangioleiomyomatosis that presented as an acute spontaneous pneumothorax in the third trimester of pregnancy, followed by significant maternal morbidity. A 37-year-old primigravid woman who presented at 29 weeks 5 days gestation with chest pain was diagnosed with spontaneous pneumothorax. Further imaging demonstrated cystic lung lesions and renal angiomyolipomas. She developed severe abdominal pain concerning for placental abruption that led to an urgent cesarean delivery at 30 weeks 2 days gestation. Her course was complicated by recurrent pneumothorax, superimposed preeclampsia, and significant ileus and bowel dilation complicated by bowel perforation. For patients with a clinical suspicion of lymphangioleiomyomatosis in pregnancy, prompt recognition, diagnosis, and referral to appropriate multidisciplinary subspecialists is critical to mitigate complications and optimize outcomes both during and after pregnancy.

A hybrid lesion of intralobar sequestration with mixed features of CPAM type I and type II unmasked following SARS-CoV-2 infection: Case report and literature review.

Introduction: Hybrid lesions of intralobar sequestration (ILS) associated with congenital pulmonary airway malformation (CPAM) is rare and could be undetected by prenatal ultrasound. Some of the cases are discovered incidentally or following lung infection in late childhood or adulthood. Case presentation: 17-year-old female developed chest pain, non-productive cough, low grade fever, and sore throat several weeks following SARS-CoV-2 infection. CT angiogram revealed a large lobulated cystic mass with celiac arterial supply in the posterior right lower lobe that was diagnostic for pulmonary sequestration. Gradually she recovered from all respiratory symptoms after a course of multiple antibiotic treatment for symptom relief. In order to prevent recurrent infection and malignancy, she underwent right lower lung mass resection approximately 3 months later. Discussion and conclusion: Pathological examination confirmed a hybrid lesion of ILS with mixed features of CPAM type I and type II. The hallmark morphological features of SARS-CoV-2 infection were not identified except for those of superimposed acute and chronic bronchopneumonia, abscesses formation and fibrosis within the lesion. This is the first case report of a hybrid lesion of ILS associated with CPAM type I and type II, unmasked following SARS-CoV-2 infection. By using the term of hybrid lesion to report this case is to efficiently correlate the terminology and nomenclature applied in the literature currently for multidisciplinary communication between radiology, pulmonary, surgery and pathology.

Congenital lobar emphysema case report: A frequently misdiagnosed disease.

Introduction and importance: Congenital Lobar Emphysema is a rare cystic lesion of the lung which may be misdiagnosed and managed as pneumonia or pneumothorax. Case presentation: We presented a case of a congenital cystic lung malformation, the case demonstrated a 29-day-old boy who presented with respiratory distress. He was initially diagnosed as pneumonia and was given unnecessary antibiotic treatment in another hospital without improvement. Subsequently, the diagnosis of congenital lobar emphysema of the left upper lobe was made on the basis of the clinical and radiologic features. The condition wasn't detected before birth. He underwent a successful left thoracotomy with left upper lobe lobectomy. Clinical discussion: Congenital Lobar Emphysema poses a challenge in diagnosis. It may mimic other causes of respiratory distress. Conclusion: Congenital Lobar Emphysema requires a high index of clinical and radiological suspicion to make an early diagnosis so that timely treatment will be offered.

Thoracoscopic surgery for congenital lung malformations: Does previous infection really matter?

BACKGROUND/ PURPOSE: Elective resection of congenital lung malformations (CLMs) is still debatable. The two main risks are malignant transformation and recurrent pulmonary infections. Our study aimed to assess the effect of previous pulmonary infection on the intraoperative and postoperative courses of thoracoscopic surgery for CLMs. METHODS: This is a retrospective study including all thoracoscopic lung resections for CLMs between 2010 and 2019. Ninety patients were included. There was a history of previous pulmonary infection in 28 patients (group A) and no such history in 62 patients (group B). RESULTS: The median age at operation for group A was 20.4 months (IQR:14.9-41.4) versus 15.1 months (IQR:9.7-20.8) in group B (p = 0.006). There were 10 conversions (35.7%) in group A and 8 (12.9%) in group B (p = 0.02). The operative time was significantly shorter in group B (p<0.002). In group A, 32.1% of patients experienced postoperative fever versus 11.3% of group B (p = 0.03), with higher antibiotics requirement (28.6% versus 6.5% respectively, p = 0.007). However, no significant differences were found in terms of postoperative complications (p = 0.99). CONCLUSION: Earlier intervention for CLMs before the development of pulmonary infection carries higher chances for the success of the thoracoscopic approach with shorter operative time and more uneventful postoperative courses.

A rare cause of recurrent hemopneumothorax.

Background: Angiosarcoma is a rare and aggressive tumor of vascular endothelial origin. Pulmonary metastasis can lead to potential life-threatening complications, such as bleeding and pneumothorax.Methods: We report on a 82-year-old male with major hemoptysis, recurrent hemopneumothorax and cystic lung lesions.Results: Although initial diagnostic findings were misleading, thoracoscopic exploration revealed pulmonary and pleural metastasized angiosarcoma.Conclusion: The presence of hemoptysis or (recurrent) hemopneumothorax associated with cystic lung lesions should alert the clinician of possible pulmonary metastasized angiosarcoma.

Secuestro pulmonar en adultos: reporte de tres casos y revisión de la literatura / Adult bronchopulmonary sequestration: report of three cases and review of the literature

Resumen El secuestro pulmonar es una enfermedad congénita infrecuente caracterizada por la presencia de una porción de parénquima pulmonar que recibe vascularización independiente de la circulación sistémica. Se presentan tres casos de secuestro pulmonar intralobares, de presentación tardía, uno de ellos asociado a una malformación adenomatosa quística y en los cuales el manejo se completó por videotoracoscopia: dos de ellos con lobectomía total y uno con lobectomía sublobar. Se trata de una enfermedad de presentación clínica variable y cuyo tratamiento es, en la mayoría de los casos, quirúrgico, requiriéndose usualmente resecciones lobares, las cuales pueden llevarse a cabo de manera eficaz y segura por videotoracoscopia.

Abstract Pulmonary sequestration is an infrequent congenital disease characterized by the presence of a portion of pulmonary parenchyma that receives vascularization independent of the systemic circulation. We present three cases of intralobar pulmonary sequestration of late presentation, one of them associated with a cystic adenomatous malformation and in which the management was completed by videothoracoscopy: two of them with total lobectomy and the other with sublobar lobectomy. It is a disease of variable clinical presentation and whose treatment is, in most cases, surgical, usually requiring lobar resections, which can be carried out effectively and safely by video-assisted thoracoscopy.

Genome-wide copy number variations in congenital cystic lung disease / 临床儿科杂志

Objective To explore the potentially relevant copy number variations (CNVs) in congenital cystic lung diseases (CCLD). Methods Clinical data of 16 patients diagnosed with CCLD and CNVs results were retrospectively analyzed. Results Of 16 cases, 12 were males and 4 were females aged between 2 months and 12 years and 6 months. Of 16 cases, 10 cases were bronchogenic cyst, 4 cases were pulmonary sequestration, 2 cases were congenital cystic adenomatoid malformation, 1 case was congenital lobar emphysema and one case was not classified. These cases presented mainly with fever, cough, and sputum without specificity. Six CNVs with unknown clinical significance were found in two patients. Abnormal amplification of HDAC8 gene was found in 4 patients diagnosed with BC.Conclusions CCLD is less likely to be associated with the CNVs;HDAC8 gene may be related to bronchogenic cyst.

Congenital Cystic Lung Lesions: Evolution From In-utero Detection to Pathology Diagnosis-A Multidisciplinary Approach.

Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings. An 8-year retrospective review of the perinatal and pathology database of a single tertiary care center identified 42 cases of congenital cystic lung lesions of which 36 had known prenatal ultrasound and prenatal course available. Final pathologic diagnoses were 15 CPAM (41%), 7 BPS (19%), and 9 hybrid BPS and CPAM lesions (25%). Five cases with bronchial atresia were also identified (either in isolation or associated with CPAM or BPS). The overall characteristics of these lesions by prenatal ultrasound, postnatal imaging, and ultimate histopathologic diagnosis are described.

Congenital lung lesions: Postnatal management and outcome.

Antenatal diagnosis of lung lesion has become more accurate resulting in dilemma and controversies of its antenatal and postnatal management. Majority of antenatally diagnosed congenital lung lesions are asymptomatic in the neonatal age group. Large lung lesions cause respiratory compromise and inevitably require urgent investigations and surgery. The congenital lung lesion presenting with hydrops requires careful postnatal management of lung hypoplasia and persistent pulmonary hypertension. Preoperative stabilization with gentle ventilation with permissive hypercapnia and delayed surgery similar to congenital diaphragmatic hernia management has been shown to result in good outcome. The diagnostic investigations and surgical management of the asymptomatic lung lesions remain controversial. Postnatal management and outcome of congenital cystic lung lesions are discussed.

Congenital cystic lesions of the lungs: The perils of misdiagnosis - A single-center experience.

BACKGROUND: A majority of cystic lesions in the western world are detected antenatally, whereas, the diagnosis in our setup occurs once the child becomes symptomatic. Surgical management is primarily dictated by the presence of symptoms, recurrent infection, and rarely by the potential risk of malignant transformation. MATERIALS AND METHODS: A retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from January 2000 through June 2011 for antenatal diagnosis, presentation, diagnostic modalities, treatment, and complications. RESULTS: Forty cystic lung lesions were identified. Only 8% were antenatally detected. Out of 40, the final diagnosis was congenital cystic adenomatoid malformation in 19, congenital lobar emphysema in 11, and bronchogenic cysts and pulmonary sequestration in five each. Of these, 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center. Postoperative morbidity in the form of bronchopleural fistula, pneumothorax, and non-expansion of the residual lung was noted in 10% of the patients. CONCLUSION: Antenatal diagnosis of these lesions is still uncommon in third world countries. Prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion, due to incorrect diagnosis.

Congenital cystic lesions of the lung.

Congenital cystic lesions of the lung are present in 1 in 10,000-35,000 births and present as a spectrum of anomalies. Majority of these cystic lesions comprise congenital cystic adenomatoid malformations, pulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most of these lesions are nowadays detected antenatally, however some will present either in the newborn or during later childhood. A review of the aetiology, classification, natural history, investigations, and treatment of congenital cystic lung lesions is discussed.

[Congenital cystic adenomatoid malformations of the lung: diagnosis, treatment, pathophysiological hypothesis]. / Malformations adénomatoïdes kystiques du poumon : diagnostic, prise en charge, hypothèses physiopathologiques.

Congenital cystic adenomatoid malformations (CCAM) of the lung are the most frequent congenital lung malformations. Their diagnosis is based on histological features. CCAM consist of bronchopulmonary cystic lesions which are classified according to the presence and cysts size. Type I CCAM are composed of large cysts (>2 cm) lined by a columnar pseudostratified epithelium. Type II CCAM contain multiple small cystic lesions (<1 cm) lined by a flattened cuboidal epithelium. Type III CCAM are more solid and contain immature structures resembling the pseudoglandular stage of lung development. Ultrasonography (US) allows early detection during the second trimester of pregnancy as cystic, and/or hyperechoic fetal lung lesions. Although most CCAM remain asymptomatic, CCAM can cause polyhydramnios or fetal hydrops, respiratory distress at birth, infections and pneumothoraces during infancy, and may give rise to malignancies. Serial US allow detection of complications, and planification of delivery. Complicated forms require an urgent treatment. In fetuses with a macrocystic life-threatening lesion, a thoraco-amniotic shunt can be placed. Microcystic compressive forms may respond to prenatal steroids. Post-natal symptomatic lesions require early surgery. The treatment of asymptomatic forms remains controversial. Some recommend a non-operative approach with a long-term clinical and radiological following, whereas other favour a preventive surgical excision. The origin of CCAM remains unknown. Recent advances suggest a transient and focal abnormality in lung development which may result from an airway obstruction. This article reviews the diagnosis, treatment, and pathophysiology of CCAM.

Cystic and cavitary lung lesions in children: radiologic findings with pathologic correlation.

A number of diseases produce focal or multiple thin-walled or thick-walled air- or fluid-containing cysts or cavitary lung lesions in both infants and children. In infants and children, there is a spectrum of focal or multifocal cystic and cavitary lung lesions including congenital lobar emphysema, congenital cystic adenomatoid malformation, pleuropulmonary blastoma, bronchogenic cyst, pulmonary sequestration, Langerhans cell histiocytosis, airway diseases, infectious diseases (bacterial infection, fungal infection, etc.), hydatid cysts, destroid lung, and traumatic pseudocyst. For the evaluation of cystic or cavitary lung lesion in infants and children, imaging plays an important role in accurate early diagnosis and optimal patient management. Therefore, a practical imaging approach based on the most sensitive and least invasive imaging modality in an efficient and cost-effective manner is paramount. We reviewed the conventional radiographs and computed tomography findings of the most common cystic and cavitary lung lesions in infants and children.

Congenital cystic lung lesions in 52 children / 中华实用儿科临床杂志

Objective To investigate the diagnosis,operation and outcomes of congenital cystic lung lesions in children.Methods Fifty-two children with congenital cystic lung lesions were collected.The clinical data s were analyzed including manifestations,lesion characteristics,imaging,histopathology,diagnosis,surgical treatment methods and short-term pulmonary functions.Results There were 33 male and 19 female,aged from 1 day to 159 months,their median age was 3.67 months.Five cases were neonates.There were 42 cases of bronchogenic cyst,6 cases of pulmonary sequestration,3 cases of congenital lobar emphysema and 1 case of congenital cystic adenomatoid malformation.The cases commonly manifested by lung infections.Of 52 cases,61.5％ were diagnosed after the first onset of lung infection,21.1％ for repeated lung infections and 17.3％ with no symptom.Right lower lung was the most common lesion site and the cases accounted about for 36.5％.Multiple gas cysts were the most common imaging findings accounting about for 42.3％.The pathologic examination of all cases showed bronchogenic cysts.The missed diagnosis rate was 17.3％.The misdiagnosis rate was 24.9％,which of bronchogenic cyst cases was 19.2％.All cases received the lobectomy without death.Conclusions The preoperative diagnosis of congenital cystic lung lesions is mostly based on imaging findings.Clinical manifestations are useful for identifying early the lesions.The preoperative diagnosis may be easily missed or incorrectly done.Pathological findings contribute to make a definite diagnosis.The surgery effect is satisfactory and the outcome is good.

Congenital cystic lung lesions / 临床儿科杂志

Congenital cystic lung lesions are a group of congenital lung diseases with low incidence. These include congenital cystic adenomatoid malformation,bronchogenic cyst,congenital lobar emphysema and pulmonary sequestration. These malformations occur during the period of lung development stimulated by various factors. Manifestations of the diseases are very similar,but the pathogenesis and pathology are very different. Congenital cystic adenomatoid malformations are thought to be the results of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements and without development of alveoli. There are 5 pathological types of congenital cystic adenomatoid malformations. Bronchogenic cysts are the results of abnormal budding from a segment of the tracheobronchial tree during embryo development,and the buds with no communication with normal tracheobronchial tree. Congenital lobar emphysema is a term reserved for hyperinflation of alveoli from idiopathic reasons or extrinsic compression,as well as pathological changes of the bronchial wall. Pulmonary sequestrations account for parts of nonfunctioning lung tissue that mostly do not communicate with normal bronchoalveolar tree and vascularized by a systemic artery,two types(intralobar and extralobar sequestration)are described.