Congenital Cystic Lung Disease Research Articles

Congenital cystic diseases of the lung

Congenital cystic diseases of the lung are rare but their incidence is increasing, especially with the widespread use of antenatal ultrasound. Close relationships between airway and respiratory segments and vascular structures during embryonic development can lead to malformations. It should be remembered that congenital cystic lung diseases have the potential to be life-threatening and the possibility of missed diagnosis is high. Surgical resection yields good results in symptomatic lesions, with lobectomy being the most commonly performed surgical approach. Some centers recommend surgical resection even in asymptomatic patients to prevent infection and exclude malignancy.

Nine surgical cases of childhood congenital cystic lung disease

Nine surgical cases of childhood congenital cystic lung disease

Traumatic Pulmonary Pseudocyst Mimicking a Congenital Cystic Lung Disease

Traumatic pulmonary pseudocyst (TPP) is a rare entity that occurs following a trauma to the chest. It usually presents as multiple cystic lesions on thoracic imaging. It is treated conservatively and tends to completely resolve after few months. In the absence of striking signs of trauma such as rib fractures, TPP can be mistaken for other cystic lung diseases. We present a case of TPP in a 17-year-old male who was seen for mild hemoptysis after falling off a cliff. The extent of his right lower lobe cystic lesions along with the lack of major signs of trauma led to an incorrect diagnosis of congenital pulmonary airway malformation. The patient was considered for lobectomy, which he refused. Imaging of the chest repeated one and three years later showed complete resolution of the lesions.

Congenital Pulmonary Airway Malformation (CPAM): A Case Report

Congenital pulmonary airway malformation (CPAM) is a very rare congenital cystic lung disease in children. Frequent respiratory tract infections (RTI) are major concerns in these patients. When a child having recurrent episodis of RTI, CPAM could be the underlying pathology. Surgical excision is recommended to make a definite diagnosis and exclude hidden malignancies and is also the treatment of choice. Here in, we report a 6 months old girl presenting with an acute respiratory infection for the first time. An routine USG of abdomen incidentally showed multiple cystic lessions in the left lower lung. CT scan of chest also revealed similar lessions in some area. After receiving treatment for pneumonia surgical excision was performed and she was doing well after surgeryBangladesh J Child Health 2017; VOL 41 (2) :129-134

Two Cases of Congenital Cystic Lung Diseases (CCLDs) With Fetal Hydrops, for Which Thoracoamniotic Shunting Was Performed

Two Cases of Congenital Cystic Lung Diseases (CCLDs) With Fetal Hydrops, for Which Thoracoamniotic Shunting Was Performed

Thoracoscopic Lobectomy in Infants and Children Utilizing a 5 mm Stapling Device.

Purpose: Thoracoscopic lobectomy for congenital cystic lung disease has become an accepted and in many institutions the preferred technique. However, the technical challenges are many. Previous endoscopic staplers (12 mm) used commonly in adults are too large for use in infants This study evaluates the safety and efficacy of using a 5 mm stapling device to seal and divide major pulmonary structures.Methods: From July 2014 to March 2016, 26 patients of age 6 weeks to 13 months underwent thoracoscopic lobectomy for CPAM or sequestration. Weights ranged from 3.2 to 11.4 kg. There were 7 upper lobectomies, 2 middle, and 17 lower lobectomies. In each case, the 5 mm stapler (Justright Surgical; Louisville, Colorado) was the primary device for vessel and bronchial sealing and division. It is 4.8 mm in diameter with an anvil length of 25 mm and lays down four rows of staples and divides between them. A 3 mm sealing device was used for dissection and to take smaller segmental vessels as necessary. Stump lines were evaluated for bleeding or air leak in all cases.Results: All procedures were accomplished successfully thoracoscopically. The stapler was used on the main lobar artery cases and vein in 24 cases, a large systemic sequestration vessel in 5 cases, and the bronchus in all 26. The stapler was also used to complete the minor fissure in 1 case and the major fissure in 1 case. A total of 96 staple loads were fired. Operative times ranged from 35 to 135 minutes. There was no significant bleeding of any vascular stump. In 1 case, the edge of the bronchial stump had to be reinforced, this was thought to be secondary to too much tissue being enclosed in the jaws. There were no postoperative complications.Conclusion: The use of a 5 mm stapling device appears to be safe and effective in thoracoscopic lobectomy in infants. It allows for safe management of major pulmonary vessels and bronchi in the confined chest of an infant through a single 5 mm port.

Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.

The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Fetal hydrops was visualized using MRI in 9.2% of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8% of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04±1.71 vs. 0.98±0.50, P<0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37±1.28 to 1.14±0.84 in CCAM and from 1.08±0.47 to 0.46±0.64 in non-CCAM). An estimated 8-9% of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.

Thoracoscopic Vs open resection of congenital cystic lung disease- utilization and outcomes in 1120 children in the United States

PurposeTo determine if utilization of thoracoscopic resection of congenital cystic lung disease (CLD) is increasing and if this approach is associated with improved outcomes using a large national sample. MethodsChildren ≤20years old who underwent resection of a congenital cystic adenomatoid malformation, bronchopulmonary sequestration, or bronchogenic cyst were identified from the Healthcare Cost and Utilization Project Kids' Inpatient Database (2009, 2012) and Nationwide Inpatient Sample (2008, 2010–2011). Patient characteristics and outcomes were compared between thoracoscopic and open approaches using univariate and multivariable analyses stratified by magnitude of resection. ResultsThoracoscopic resection was used in 39.4% of 1120 children who underwent resection of CLD. Utilization of the thoracoscopic approach increased from 32.2% in 2008 to 48.2% in 2012. Use of thoracoscopy was lower in lobectomy than segmental resection (32.5 vs 48.4%, p<.001). Newborns, those with comorbid congenital conditions, and those with respiratory infections also had lower rates of thoracoscopy. After stratifying by magnitude of resection and adjusting for patient complexity, complication rates and postoperative length of stay were similar between thoracoscopic and open approaches. ConclusionUtilization of thoracoscopic resection for CLD in the United States is increasing with time. After adjusting for patient complexity, there is no difference in postoperative length of stay or complications between thoracoscopic and open lobectomy and sub-lobar resection.

Clinical Features of Congenital Cystic Lung Diseases: A Report on a Nationwide Multicenter Study in Japan.

The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p < 0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

Epithelial inactivation of Yy1 abrogates lung branching morphogenesis.

Yin Yang 1 (YY1) is a multifunctional zinc-finger-containing transcription factor that plays crucial roles in numerous biological processes by selectively activating or repressing transcription, depending upon promoter contextual differences and specific protein interactions. In mice, Yy1 null mutants die early in gestation whereas Yy1 hypomorphs die at birth from lung defects. We studied how the epithelial-specific inactivation of Yy1 impacts on lung development. The Yy1 mutation in lung epithelium resulted in neonatal death due to respiratory failure. It impaired tracheal cartilage formation, altered cell differentiation, abrogated lung branching and caused airway dilation similar to that seen in human congenital cystic lung diseases. The cystic lung phenotype in Yy1 mutants can be partly explained by the reduced expression of Shh, a transcriptional target of YY1, in lung endoderm, and the subsequent derepression of mesenchymal Fgf10 expression. Accordingly, SHH supplementation partially rescued the lung phenotype in vitro. Analysis of human lung tissues revealed decreased YY1 expression in children with pleuropulmonary blastoma (PPB), a rare pediatric lung tumor arising during fetal development and associated with DICER1 mutations. No evidence for a potential genetic interplay between murine Dicer and Yy1 genes during lung morphogenesis was observed. However, the cystic lung phenotype resulting from the epithelial inactivation of Dicer function mimics the Yy1 lung malformations with similar changes in Shh and Fgf10 expression. Together, our data demonstrate the crucial requirement for YY1 in lung morphogenesis and identify Yy1 mutant mice as a potential model for studying the genetic basis of PPB.

Risk of respiratory syncytial virus infection in infants with congenital cystic lung disease.

Congenital cystic lung disease (CCLD), which includes congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and congenital lobar emphysema, has been reported to increase the risk of recurrent respiratory infection. In particular, respiratory syncytial virus (RSV) causes severe lower respiratory tract disease in high-risk infants. The objective of this study was to investigate the risk of severe RSV infection in infants with CCLD. Infants antenatally diagnosed as having CCLD and admitted to a neonatal intensive care unit at the National Center for Child Health and Development in Tokyo between September 2002 and October 2011 were included in this study. We investigated retrospectively whether the infants were hospitalized with RSV infection by 24 months of age using their medical records. Forty-eight infants were antenatally diagnosed as having CCLD. Of the 48 infants, four (8.3%) were hospitalized with RSV infection by 24 months of age. Infants with CCLD have increased risk of severe RSV infection.

Cystic lung disease: systematic, stepwise diagnosis.

Once cystic lung disease is confirmed on computed tomography, one can arrive at the likely diagnosis in most cases by taking a systematic, stepwise approach based on the clinical and radiographic features. Here, we describe the features of cystic lung disease that point to lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary angerhans cell histiocytosis, interstitial pneumonia, congenital cystic lung disease, pulmonary infection, and systemic disease.

Congenital cystic lung disease: prenatal ultrasound and postnatal multidetector computer tomography evaluation. Correlation with surgical and pathological data

The aim of this study was to evaluate the diagnostic accuracy of postnatal multidetector computed tomography (MDCT) compared with prenatal ultrasound (US), surgical findings, and histology, in 33 patients with congenital cystic lung disease. Thirty-three patients, 17 males and 16 females, were evaluated by MDCT. Twenty-seven of these patients underwent prenatal US between week 18 and 22, and between week 32 and 35 of gestation. Lung lobectomy, segmentectomy, atypical resection, lesion resection were performed in 31 patients and surgical specimens were analysed. Prenatal US and MDCT correctly diagnosed 76.9 and 94 % of the lesions, respectively. Disagreement occurred in six lesions with prenatal US and in two lesions with MDCT. No statistically significant differences were observed between the two techniques (P = 0.122). As most surgeons consider the surgical resection of these lesions mandatory, our study underscores the essential role of imaging, in particular CT, in providing invaluable preoperative information on congenital cystic lung diseases recognised in uterus.

Successful use of caudal anesthesia and light sevoflurane mask ventilation for inguinal hernia repair in an infant with multiple large intrapulmonary cysts.

Successful use of caudal anesthesia and light sevoflurane mask ventilation for inguinal hernia repair in an infant with multiple large intrapulmonary cysts.

Thoracoscopic Segmentectomy for Congenital and Acquired Pulmonary Disease: A Case for Lung-Sparing Surgery

Over the last 15 years thoracoscopic lobectomy for congenital and acquired lesions has become an accepted modality in pediatric thoracic surgery. There is still debate about the need to perform a complete lobectomy for some of these lesions, and some advocate observation rather than resection, despite possible long-term complications of untreated lesions. High-resolution computed tomography (CT) scans and physical findings at the time of surgery, along with new advanced techniques, now allow for discrete partial anatomic resections, which may preserve normal lung. This study evaluates the feasibility and early results using these techniques in selected cases. With institutional review board approval, the records of all patients undergoing thoracoscopic lung resection were reviewed. From January 2006 to December 2012, 23 patients, ranging from 1 month to 16 years of age and weighing 3.8-42 kg, underwent thoracoscopy for planned resection. Pathology was congenital cystic lung disease in 19 patients, bronchiectasis in 3 patients, and arteriovenous malformation in 1 patient. In each case findings on CT scan and at the time of surgery warranted consideration of lung-preserving surgery. Procedures were performed through three ports using single lung ventilation and CO2 insufflation to achieve lung collapse. The LigaSure™ device (Covidien, Norwalk, CT) was the primary instrument used to seal and divide the lung parenchyma and seal vessels. All procedures were completed successfully thoracoscopically. An anatomic segmental resection was achieved in 22 of 23 cases. Operative time ranged from 30 to 300 minutes (mean, 120 minutes). Segmental resections included the left upper lobe apical/posterior (n=4), lingula (n=3), left lower lobe superior (n=5), medial or posterior basal (n=3), right middle lobe medial (n=1), right upper lobe apical (n=1), right lower lobe superior (n=4), and posterior basal (n=2). Two patients had more than one segment excised. Chest tubes were left in for 24 hours in 16 cases, 48 hours in 4 cases, and 5 days in 1 case. Hospital stay ranged from 1 to 6 days (mean, 2 days). Follow-up CT scans obtained at 1-6 years (mean, 28 months) show no residual disease in 20 of 21 patients. One patient underwent a nonanatomic resection and had evidence of recurrent congenital pulmonary airway malformation at the 4-year follow-up. This patient underwent a secondary thoracoscopic resection. Thoracoscopic lung-conserving therapy is technically feasible and safe in infants and children. The magnification provided by a thoracoscopic approach makes identification of segmental anatomic planes easier, aiding in safe dissection and resection. Anatomic resection appears to be associated with a low morbidity. It may be appropriate in the case of bilateral or extensive disease or in cases where the diseased tissue is clearly limited to an anatomic segment. Continued long-term follow-up is needed.

Pulmonary Invasive Mucinous Adenocarcinoma in an Asymptomatic 23-Year-Old Male: Presentation with a Large Cavitary Mass

Pulmonary invasive mucinous adenocarcinoma (PIMA) is a variant of invasive adenocarcinoma of the lung (1). The incidence of this tumor is known to be rising (2). The tumor cells demonstrate goblet cell histology with copious intracytoplasmic mucin material (1, 2). Imaging may show a broad spectrum of findings, but lobar or segmental consolidation is found in the majority (1, 3-5). This case presented as a large mass-like consolidation with multiple internal air-cysts and/or pseudo-cavitations which mimicked complicated congenital cystic lung disease in a patient with relatively young age to consider lung cancer. Therefore, we report a case of PIMA presenting as a large cavitary mass in a 23-year-old male with clinical and radiological findings, and a review of the literature. CASE REPORT

Surgical treatment for patients with congenital cystic lung disease under 5 years old

胎児期に先天性肺嚢胞性疾患を指摘される機会が増えた．CCAMや肺分画症の治療は一般的に外科的切除であるが，生後自然退縮する例があり無症候性症例に対する治療方針は確立されていない．幼児以下の先天性肺嚢胞性疾患8例を後向きに検討した．5例は胎児期の超音波検査で発見され，2例は胎児治療を受けた．3例は生後感染を契機に発見された．8例中7例に外科的切除が行われた．胎児治療の2例はいずれも呼吸状態の悪化により緊急手術（日齢5日と16日）が行われた．全例目立った合併症なく退院となり，呼吸器関連後遺症，再発，悪性腫瘍の発生も認めない．先天性肺嚢胞性疾患に対する手術は幼児以下でも安全に施行し得る．有症候性症例や縮小傾向のない無症候性症例には可及的早期に手術を行うことが望ましく，胎児治療を要した症例は出生後早期の手術を念頭に置いて周産期管理を行う必要があると考えられた．

Final diagnosis in patients with congenital cystic lung disease detected by fetal ultrasonography

Pediatrics InternationalVolume 53, Issue 1 p. 131-132 Final diagnosis in patients with congenital cystic lung disease detected by fetal ultrasonography Gen Kuratsuji, Corresponding Author Gen Kuratsuji Departments of PediatricsGen Kuratsuji, MD, Division of Neonatology, Department of Pediatrics, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku,160-8582 Tokyo, Japan. Email: kuragen@wd6.so-net.ne.jpSearch for more papers by this authorIsamu Hokuto, Isamu Hokuto Departments of PediatricsSearch for more papers by this authorMasataka Higuchi, Masataka Higuchi Departments of PediatricsSearch for more papers by this authorGoro Koinuma, Goro Koinuma Departments of PediatricsSearch for more papers by this authorYasuhide Morikawa, Yasuhide Morikawa Pediatric SurgerySearch for more papers by this authorKen Hoshino, Ken Hoshino Pediatric SurgerySearch for more papers by this authorMamoru Tanaka, Mamoru Tanaka Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, JapanSearch for more papers by this authorKei Miyakoshi, Kei Miyakoshi Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, JapanSearch for more papers by this authorKazushige Ikeda, Kazushige Ikeda Departments of PediatricsSearch for more papers by this author Gen Kuratsuji, Corresponding Author Gen Kuratsuji Departments of PediatricsGen Kuratsuji, MD, Division of Neonatology, Department of Pediatrics, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku,160-8582 Tokyo, Japan. Email: kuragen@wd6.so-net.ne.jpSearch for more papers by this authorIsamu Hokuto, Isamu Hokuto Departments of PediatricsSearch for more papers by this authorMasataka Higuchi, Masataka Higuchi Departments of PediatricsSearch for more papers by this authorGoro Koinuma, Goro Koinuma Departments of PediatricsSearch for more papers by this authorYasuhide Morikawa, Yasuhide Morikawa Pediatric SurgerySearch for more papers by this authorKen Hoshino, Ken Hoshino Pediatric SurgerySearch for more papers by this authorMamoru Tanaka, Mamoru Tanaka Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, JapanSearch for more papers by this authorKei Miyakoshi, Kei Miyakoshi Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, JapanSearch for more papers by this authorKazushige Ikeda, Kazushige Ikeda Departments of PediatricsSearch for more papers by this author First published: 15 March 2011 https://doi.org/10.1111/j.1442-200X.2010.03301.xCitations: 3Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume53, Issue1February 2011Pages 131-132 RelatedInformation

Simple Congenital Cystic Adenomatoid Malformation with a Feeding Artery -A case report-

CCAM with no other anomalies such as sequestration receives its blood supply from the pulmonary artery. Our case presented with a simple CCAM and no other anomalies but with a feeding artery. Although preoperative evaluation may not show feeding arteries, they may exist in congenital cystic lung diseases.

Thoracoscopic lobectomy for congenital cystic lung diseases in neonates and small infants

There are still few reports on thoracoscopic lobectomy in neonates. The rate of prenatally diagnosed congenital cystic lung diseases is increasing, and such diseases appear to be a good indication for thoracoscopic lobectomy. We performed a prospective trial of thoracoscopic lobectomy for all congenital cystic lung diseases in children. Complete thoracoscopic lobectomy was performed by employing carbon dioxide insufflation without single lung ventilation. The results were analyzed, comparing patients with pre- and postnatal diagnoses. Seven patients were prenatally diagnosed with congenital pulmonary airway malformations (CPAM) between January 2008 and August 2009, and all but one underwent surgery during the neonatal period (prenatal group). Nine patients underwent surgery after infection subsided due to lesions (7 CPAM, 2 intrapulmonary sequestration) postnatally identified by infection presence (median 2 years; range 15 days to 14 years) (postnatal group). The prenatal group showed a significantly lower rate of adhesions, shorter operation time, and smaller volume of blood loss. There were two conversions in the postnatal group. There were two postoperative complications (persistent air leak and phrenic nerve paralysis) in the prenatal group. The esthetic results were good. Thoracoscopic lobectomy for congenital cystic lung diseases in neonates was practicable, with good esthetic results. Dissection was easier with significantly less blood loss in patients without adhesions, even in neonates. However, this procedure necessitated highly skilled endoscopic maneuvers within a small working space.