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The vast majority of prenatally diagnosed congenital pulmonary malformations (CPM) remain asymptomatic at birth. The maximal value of the CPM volume ratio (CVRmax) predicts the risk of neonatal respiratory distress (NRD), and should allow for better assessment of the level of expertise needed at the delivery site. This study evaluated the level of maternity units currently chosen for the delivery of CPMs, and determined the impact of the choice of delivery site based on the CVRmax, with a threshold of 0.4 cm2. Data were extracted from the French prospective MALFPULM cohort, with inclusion between March 2015 and June 2018. The final study population consisted of 383 women. Deliveries in level 1 or 2 maternity units (n = 98, 25%) involved CPMs with lower CVRmax (p <0.001), causing fewer signs of prenatal compression (p = 0.025). Among the 62 children (16%) who presented with NRD, only seven (11%) were born in level 1 or 2 units (p = 0.0078). Choosing the maternity level according to the CVRmax would have increased the number of births in level 1 or 2 maternity hospitals by 70%. In these maternity units, the percentage of children with NRD would have increased from 8% in the actual distribution to 10% in the new strategy. Our results showed an overuse of level 3 maternity hospitals for the delivery of newborns with a prenatal diagnosis of CPM. The use of CVRmax should enable a reduction in the use of expertise centers without an adverse impact on newborns. [ABSTRACT FROM AUTHOR]

Background: Persistent thyroglossal duct leads to a fibrous cyst formation named thyroglossal cyst which is the most common form of congenital cyst and usually located in the midline neck below the hyoid bone. Rarely the thyroglossal cyst is associated with thyroid cancer. Although the Sistrunk procedure is often considered adequate, currently there is no clear consensus on the optimal management of thyroglossal duct cyst especially duct cyst carcinoma. In addition, there is no consensus about concurrent thyroidectomy in patients with thyroglossal cyst duct carcinoma. Case presentation: In this article, we present four patients with thyroglossal duct cyst and papillary thyroid cancer. Papillary thyroid cancer was located into the thyroglossal duct cyst (thyroglossal duct carcinoma) in two patients and two patients had thyroglossal duct cyst with thyroid carcinoma in thyroid tissue. Cervical lymph nodes were involved in one of the three patients. Sistrunk procedure and total thyroidectomy were performed for all. The thyroid lobes were not involved in patients with thyroid duct cyst carcinoma. Tumor or thyroglossal duct cyst recurrence did not occur in any of the patients in follow-up. Conclusion: The correlation between thyroglossal cyst and papillary thyroid carcinoma is high. In subjects with thyroglossal duct cyst, in addition to cyst assessment, thyroid gland and neck lymph nodes should be evaluated for malignancy. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study is to evaluate long-term neurodevelopmental and respiratory outcome after fetal therapy for fetal pleural effusion, congenital cystic adenomatoid malformation, and bronchopulmonary sequestration.Methods: Children ≥18 months of age underwent an assessment of neurologic, motor, and cognitive development. Medical records were reviewed to determine respiratory outcome. Behavioral outcome was assessed using the Child Behavioral Checklist.Results: Between 2001 and 2016, 63 fetuses with fetal hydrops secondary to thoracic abnormalities were treated at our center. Overall perinatal survival was 64% (40/63). Twenty-six children were included for follow-up (median age 55 months). Severe neurodevelopmental impairment (NDI) was detected in 15% (4/26). Three out of 4 children with severe NDI had associated causes contributing to the impairment. Overall adverse outcome, including perinatal mortality or NDI, was 55% (27/49). Fifteen percent (4/26) had severe respiratory sequelae. Parents did not report more behavioral problems than Dutch norms.Discussion: Our results suggest that severe NDI in this specific high-risk cohort occurs in 15%, which is above the range of the incidence of NDI reported in case series treated with other fetal therapies (5-10%). Large multicenter studies and an international web-based registry are warranted to prospectively gather outcome data at fixed time points. [ABSTRACT FROM AUTHOR]

Introduction: Congenital cystic adenomatoid malformation (CCAM), especially type-III, shares similar sonographic features with congenital lobar emphysema (CLE) in routine ultrasound scan. Thus, prenatal differentiation of CLE from a microcystic CCAM is challenging and difficult in practice. Discovery of molecular biomarkers has important clinical significance.Methods: We profiled gene expression in lung tissue from four CCAM type-III and five CLE subjects by microarray. A bioinformatic tool was used for signal pathways enrichment analysis. Further, quantitative reverse transcriptase PCR (qRT-PCR) was used to verify the results.Results: A total of 426 genes were identified to be significantly differentially expressed (fold-change >2.0, q value <0.05) between microcystic CCAM and CLE. Of these differentially expressed genes (DEGs), 392 were upregulated and 34 were downregulated in microcystic CCAM compared with CLE. Unsupervised clustering of the "expressed" genes could clearly delineate the CCAM and CLE samples. We also confirmed that eight randomly chose genes were differentially expressed at the mRNA level between CCAM and CLE.Conclusions: CCAM type-III and CLE have differential gene expression patterns. Our pilot study may gain a deeper understanding of the organogenetic origins and pathogenesis of these conditions. The suggestive candidates may serve as potential biomarkers for definitive diagnosis of congenital cystic lung lesions and eventually to treat them appropriately. [ABSTRACT FROM AUTHOR]

Congenital cystic adenomatoid malformation (CCAM) is a benign congenital tumour in which a part of the lung becomes polycystic. Case 1 was a 64‐year‐old male who was diagnosed with pneumonia, with multiple cysts in the right lower lung lobe, using chest computed tomography (CT). After treatment of the pneumonia, including Mycobacterium abscessus, a right lower lobectomy was performed. Case 2 was a 41‐year‐old male who had suffered from pneumonia many times since his youth. Polycystic and infiltrative shadows were observed on chest CT. After treatment of the pneumonia, a right lower lobectomy was performed. Pathologically, both the cases were diagnosed as CCAM type 1. Although CCAM in adults is very rare, it should be considered in the differential diagnosis of cases with repeated pneumonia due to suspected congenital cystic disease. CCAM is better detectable with chest CT and requires active surgical treatment. [ABSTRACT FROM AUTHOR]

Aim: Management of congenital pulmonary airway malformations (CPAM) is controversial, especially for asymptomatic patients. We aim to describe the clinical manifestations and management of CPAM at a tertiary paediatric hospital using a retrospective audit.Methods: Infants with CPAM were identified on the Fetal Diagnostic Unit database from 2007 to 2014. Information on antenatal and post-natal management was collected from medical record.Results: Thirty-five infants with antenatally diagnosed CPAM were included. Fetal CPAM volume ratio (CVR) was calculated from antenatal ultrasound measurement and used to categorise the infants into three groups of large (CVR ≥ 1.6, n = 8), medium (CVR of 0.5-1.6, n = 12) and small CPAM (CVR of ≤0.5, n = 15), respectively. Ten infants (10/35 = 29%) were symptomatic in the neonatal period. Overall, nine infants (26%) had surgical resection, among whom eight had large or medium-sized CPAM lesions as defined by the antenatal CVR. Three infants had neonatal emergency surgery and the remaining six had late elective surgery. Histology of eight cases showed CPAM, but one case showed congenital lobar emphysema. Criteria for surgery varied and included persistent symptoms after birth, complications during childhood and persistently abnormal chest X-ray. Most asymptomatic infants with CPAM were safely managed using a conservative approach, with no significant increase in late symptoms or complications.Conclusions: Conservative management of CPAM may be considered for infants/children who remain asymptomatic, especially those with a small lesion. For large and medium-sized CPAM, delineation using computed tomography is required, and surgery may be beneficial to prevent late symptoms and the risk of emergency surgery. [ABSTRACT FROM AUTHOR]

Wheezing and cough are common case scenarios that pediatricians encountered in their office practices. Although a bronchogenic cyst is an uncommon condition, it is essential to be considered in the differential diagnosis of a chronic cough and wheezing among young children who fail to respond to appropriate medical treatment. A 28-month-old girl was referred to our pediatric pulmonology clinic with persistent symptoms of a cough and wheeze unresponsive to standard asthma therapy. This presentation prompted us to undertake a detailed diagnostic evaluation. The evaluation exposed a cystic mass in the middle mediastinum compressing the trachea and left main bronchus. The cyst was excised and confirmed pathologically to be a benign bronchogenic cyst. Subsequently, the patient recovered well and had been free of respiratory symptoms during follow-up visits. This report highlights one of the rare causes of wheezing and cough in young children and emphasizes the importance of considering it in the differential diagnosis of a child presenting with refractory asthma-like symptoms. This is important for early diagnosis and management and to avoid unpredictable complications of this treatable condition. [ABSTRACT FROM AUTHOR]

Aim:  To define current management of congenital pulmonary airway malformation (CPAM).Methods:  A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire.Main Results:  Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively.Conclusion:  Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear. [ABSTRACT FROM AUTHOR]

Traumatic Pulmonary Pseudocysts (TPPs) are a rare consequence of thoracic trauma that is seen disproportionately in young adults and paediatric populations. In this case report, we detail a case of a TPP initially misdiagnosed on imaging as a Congenital Cystic Adenomatoid Malformation (CCAM). Conservative management and monitoring for resolution of TPP is highly effective as in most cases it self-resolves without issue or need for intervention. This contrasts with the surgical treatment often indicated for symptomatic CCAMs. Clearly, failure to recognize TPP as a rare but important differential of pulmonary cystic lesions in the context of trauma can lead to significant distress for the patient's family, over investigation and unnecessary or harmful interventions. We also review in this article the literature surrounding the radiological appearances, clinical features and management of both conditions. [ABSTRACT FROM AUTHOR]

The article describes the processes of collecting and adapting the diagnostic information necessary for use in automated analysis of three-dimensional images and surgical navigation. The work is carried out on the basis of the NMRC Obstetrics, Gynecology And Perinatology named after V.I. Kulakov of the Ministry of Health of the Russian Federation with the financial support of the Ministry of Science and Education of the Russian Federation (Agreement dated 03.10.2016 No. 14.607.21.0162, unique identifier RFMEFI60716X0162) The work is devoted to the features of collection, segmentation and description of the results of preoperative radiological diagnostics of newborn patients with congenital malformations of the lungs, such as bronchopulmonary sequestration (BS) and congenital cystic adenomatous malformation (CCAM). The goal of the work is the development of standards for the collection, classification and segmentation of various diagnostic information of congenital lung malformations in newborns necessary to use in automated three-dimensional image analysis and surgical navigation. In order to expand the scope of application, it was decided to supplement the data bank with information from the patient’s phenotypic chart, compiled by the clinical geneticist when examining the patient. According to the developed and implemented algorithms we collected and segmented 924 series of images belonging to 148 patients with lung anomalies and 356 series of normal lung. Available text descriptions of the series are reconstructed to the original developed standard. At present, using this data bank, a subsystem of neural network analysis and reconstruction of diagnostic images of newborn patients is being developed, as well as a surgical navigation system for performing endoscopic surgical manipulations on patients for congenital malformations of the lungs. [ABSTRACT FROM AUTHOR]

Introduction: Congenital cystic adenomatoid malformation (CCAM) is a rare anomaly. The mechanisms and the time at which the abnormality develops are still unclear. The malformation is characterized by the presence of single large or multiple but smaller cysts. Aim: To present the experience of our medical centre, the Polish Mother's Memorial Hospital - Research Institute. Material and methods: We analysed the medical records of 32 neonates hospitalized in 2008-2017 at the Department of Paediatric Surgery and Urology ICZMP due to pre- or postnatally diagnosed congenital cystic adenomatoid malformation. Results: In 2008-2017 32 children were hospitalized at our department due to prenatally diagnosed CCAM. An intrauterine procedure of thoracoamniotic shunting was performed in 13 fetuses. All newborns had chest computed tomography. Surgery had been performed in 21 children. Histopathological analysis of specimens prepared from the resected lung tissue confirmed type 1 CCAM in 4 cases, type 2 CCAM in 11 cases and type 3 CCAM in 1 case. Four patients were diagnosed with a bronchogenic cyst and one with extralobar pulmonary sequestration. Conclusions: Postnatal management of CCAM depends on the clinical status of the newborn. Newborns without clinical symptoms require radiological diagnostics to confirm the presence of malformation. Surgical treatment is recommended before the age of 10 months. Resection of the malformed lung fragment in patients with CCAM is the first-line treatment. It should be emphasized that despite the tremendous advances in diagnostic imaging, the final diagnosis of CCAM is made based on histopathological findings. [ABSTRACT FROM AUTHOR]

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-dayold male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM. [ABSTRACT FROM AUTHOR]

Objective: Congenital defects/diseases Background: Congenital cystic adenomatoid malformation (CCAM) is mostly reported from the second trimester of pregnancy. We report a case of a microcystic type of CCAM that was suggested by routine ultrasound examination at a gestational age of 12 weeks. Case Report: First-trimester ultrasound screening revealed the presence of a hyperechoic image that occupied the whole of the right lung, without no other any associated complications. The cardiac and aorta deviations with diaphragmatic eversion associated with a poly-hydramnios had subsequently appeared. The diagnosis of CCAM was confirmed histologically after termination of the pregnancy at 25 weeks of gestation. Conclusions: CCAM may occur at a very early stage of fetal lung development. [ABSTRACT FROM AUTHOR]

Background: Video-assisted thoracic surgery (VATS) pulmonary resection in children is a technically demanding procedure that requires a relatively long learning period. This study aimed to evaluate the serial improvement of quality metrics according to case volume experience in pediatric VATS pulmonary resection of congenital lung malformation (CLM). Methods VATS anatomical resection in CLM was attempted in 200 consecutive patients. The learning curve for the operative time was modeled by cumulative sum analysis. Quality metrics were used to measure technical achievement and efficiency outcomes. Results The median operative time was 95 min. The median length of hospital stay and chest tube indwelling time was 4 and 2 days, respectively. The improvement of operation time was observed persistently until 200 cases. However, two cut-off points, the 50th case and 110th case, were identified in the learning curve for operative time, and the 110th case was the turning point for stable outcomes with short operation time. Significant reduction of length of hospital stay and chest tube indwelling time was observed after 50 cases (p = .002 and p = .021, respectively). The complication rate decreased but continued at a low rate for entire study period and the interval decrease was not statistically significant. Conversion rate decreased significantly (p = .001), and technically challenging procedures were performed more frequently in later cases. Conclusions Improvements of quality metrics in operation time, conversion rate, length of hospital stay, and chest tube indwelling time were observed in proportion to case volume. Minimum experience of 50 is necessary for stable outcomes of pediatric VATS pulmonary resection. [ABSTRACT FROM AUTHOR]

We present a case of 6-month-old boy who presented with respiratory distress due to empyema, which was initially managed by the intercostal drain. Computed tomography scan confirmed the diagnosis of type 1 congenital cystic adenomatoid malformation (CCAM). Subsequently, there was the spontaneous resolution of empyema and CCAM prior to surgical intervention. Our case illustrates the postnatal spontaneous resolution of CCAM, which is rare. [ABSTRACT FROM AUTHOR]

Background: A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases.Methods: We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging. Patients followed up for at least 5 years were included.Results: 51 (43%) patients were managed surgically; 8 (6.7%) as an emergency during the neonatal period, 6 (5.1%) electively due to concerning features on CT scan, 20 (17%) following medical advice, 1 (0.8%) following a severe respiratory infection and in 5 (4.2%) the indication was unclear. The indication in 11 (9.2%) was recurrent respiratory infection and median age at surgery was 1.6 years (range 0.4 to 4.6 years). No cases of malignancy were identified on histological examination of resected lesions. 68 (57%) patients were managed conservatively for a median period of 9.9 years (range 5.2 years to 18 years). Seven (10%) were discharged, one was followed-up elsewhere (1.5%) and eight (11%) were lost to follow-up. In four patients (5.9%), the lesion resolved spontaneously. 52 (76%) continue to be followed-up and remain asymptomatic.Conclusions: This is one of the largest case series documenting the natural history of CPAMs and PS following a prenatal diagnosis and demonstrates that conservative management is a reasonable option in selected cases. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective To assess the effectiveness of laser surgery in fetuses with a cystic lung lesion with systemic arterial blood supply (hybrid lung lesion) at risk of perinatal death. Methods A cohort of five consecutive fetuses with a large hybrid lung lesion associated with hydrops and/or pleural effusion with severe lung compression was selected for percutaneous ultrasound-guided fetal laser ablation of the feeding artery ( FLAFA) before 32 weeks' gestation in a single tertiary national referral center in Queretaro, Mexico. The primary outcomes were survival and need for postnatal surgery. Results FLAFA was performed successfully in all cases at a median gestational age of 24.9 (range, 24.4-31.7) weeks. After fetal intervention, dimensions in both lungs increased and fluid effusions resolved in all cases. All cases were delivered liveborn at term at a median gestational age of 39.6 (range, 38.0-39.7) weeks, without respiratory morbidity or need for oxygen support, resulting in perinatal survival of 100%. During follow-up, three (60%) cases showed progressive regression of the entire lung mass and did not require postnatal surgery, whereas in two (40%) cases a progressive decrease in size of the mass was observed but a cystic portion of the lung mass persisted and postnatal lobectomy was required. Conclusion In fetuses with large hybrid lung lesions at risk of perinatal death, FLAFA is feasible and could improve survival and decrease the need for postnatal surgery. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective: To summarize the experience drawn from treatment of fetus with congenital cystic adenomatoid malformation (CCAM). Methods: This retrospective report has reviewed a clinical history of 115 patients with CCAM from September 2012 to June 2014. All cases were divided into two groups according to symptomatology: group A (n = 96, asymptomatic group) and group B (n = 19, symptomatic group). Major factors were compared between two groups. Numerical data were presented as the median (range). The difference between median was performed with ANOVA. Results: The mean gestational age of prenatal diagnosis was 24.3 weeks (range, 17–36). 49 cases with CCAM volume ratio (CVR) were retrospectively analyzed, with a median max CVR of 0.95 (range, 0.19–3.2), In the 115 cases, mean operation age was (3.7 ± 0.32) months old. Symptom is significantly associated with lesion size and chest radiographs (CXR) (p< 0.05). Compared with symptomatic CCAM patients, asymptomatic patients were associated with better clinical outcome. Conclusions: The value of CVR to evaluate prognosis showed no statistical significance. Although CXR are not reliable indicators to assess the lesion, it can predict the emergency situation. Due to potential trend of symptomatic and poor postoperative symptoms group, early postnatal CT scan and early surgical treatment are suggested. [ABSTRACT FROM PUBLISHER]

Aim The aim was to show rare cases of congenital cystic adenomatoid malformation (CCAM) and the manner of its surgical treatment with video-assisted thoracoscopic surgery (VATS). Methods Two male and one female child, 7, 4 and 3 years of age were treated for symptoms of cough and high temperature in district hospitals. In all three children laboratory blood tests and chest radiography were done. Auscultatory findings showed the presence of pneumonia. Children were treated with appropriate doses of antibiotics. After the rehabilitation of inflammation, they were sent to the University Clinical Center Sarajevo, where video-assisted thoracoscopic lobectomy (VATS) was indicated after computerized tomography (CT). Results Chest CT scan pointed to the CCAM and pulmonary sequestration (PS) changes to the lungs. This has required surgery lobectomy of an affected part of the lungs. In two children with PS, the aberrant systemic artery came from the most proximal part of aorta abdominals, the third patient did not have an anomalous artery. Conclusion VATS lobectomy is an alternative to the traditional thoracotomy for the treatment of CCAM and PS, however, it should be investigated in the future for its safety and effectiveness. [ABSTRACT FROM AUTHOR]

Background: Congenital pulmonary airway malformation (CPAM) is a relatively rare congenital anomaly with a wide spectrum of ultrasound features depending on the specific variety of CPAM. Antenatal ultrasound is a valuable, safe, nonionizing, cost-effective, widely available and easily reproducible imaging tool and is indispensable in the diagnosis of CPAM. In this paper, we aimed to report an atypical imaging presentation of CPAM type II in the second trimester, extensively involving all lobes of the left lung. Case Report: A 25-year-old G1P0A0 woman with a gestational age of around 22 weeks was referred for an anomaly scan. The antenatal ultrasound scan showed a single, live, intrauterine foetus corresponding to a gestational age of around 22 weeks and 4 days. There were multiple, anechoic structures noted within the pulmonary tissue in the left hemithorax, each measuring around 3 to 4 mm in diameter. The lesion was extending from the left lower lobe up to the apical (apicoposterior) segment of the left upper lobe. The ultrasound diagnosis of congenital pulmonary airway malformation type II was made. After explaining the condition and the poor prognosis to the patient, an informed consent was obtained after she opted for medical termination of pregnancy. Conclusions: Congenital pulmonary airway malformation (CPAM) is an uncommon foetal anomaly with a very wide range of ultrasound appearances depending on the specific type of CPAM. CPAM also has a wide spectrum of differential diagnoses and a variable prognosis. Antenatal ultrasound should always be the primary mode of diagnosis in CPAM. [ABSTRACT FROM AUTHOR]

Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, nonhereditary developmental anomalies of the lung with unknown etiology. CCAM is predominantly a disorder of infancy with the majority of the cases being diagnosed within the first 2 years of life. When CCAM presents in adults, it represents a diagnostic dilemma and requires careful evaluation. We here report a case of large solitary congenital pulmonary cystic adenomatoid malformation with infection and hemorrhage, which was diagnosed as encysted hydropneumothorax on computerized tomography scans but turned out to be infected pulmonary cystic adenomatoid malformation after surgical excision. [ABSTRACT FROM AUTHOR]

Background: Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies, the true incidence is yet to be determined. We carried out this prospective study with the aim to estimate its true incidence on a population basis.Methods: An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, postnatal outcomes, and other demographics were compared to those of patients with CPAM in the previous decades (1989-2008). The incidence of CPAM was calculated in different periods.Results: 66 CPAM patients were identified between 2009 and 2014 with 62 patients being detected by antenatal scan. In contrast, 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as ~1 in 27,400 and ~1 in 7200 live births, respectively (p = 0.024).Conclusion: With increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here, we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence. [ABSTRACT FROM AUTHOR]

This work was a retrospective audit of CCAM - 1994 to 2008. A total of 26 cases were identified. Mean gestational age at diagnosis was 20 weeks. All were unilateral and had serial scans. In 31% the lesion resolved, 8% decreased, 42% were unchanged and 4% increased in size. Only one foetus developed hydrops. All were born alive. Of 8 foetuses where the CCAM was thought to have resolved, 6 had persistent lesions (overall sensitivity and PPV of US 64% and 69%, respectively). Computerised tomography performed better than chest X-ray in detecting lesions postnatally (sensitivity 100% vs 88%, PPV 95% vs 78%). Seventeen cases required surgery, 4 conservative management and 2 were discharged following negative CXR; 12% had associated anomalies. Four babies had recurrent chest infections. CCAMs have a good overall prognosis. CT scan rather than CXR should be performed to confirm the resolution of antenatally diagnosed lesions. Surgical management is the preferred option over conservative management. [ABSTRACT FROM AUTHOR]

Introduction: Congenital Cystic Adenomatoid Malformation (CCAM) is an uncommon developmental deformity affecting the terminal respiratory structures. It is characterized by broncho pulmonary foregut malformations. The reason behind it is an arrest in lung development between 4th and 7th week of fetal life. Aim: The present study was conducted to assess the clinical and radiological profile and also to study the role of surgical intervention in patients with CCAM. Materials and Methods: All patients with clinical suspicion or provisional diagnosis of CCAM were included in the study. A clinical questionnaire was prepared to collect data. Computed Tomography (CT) chest with High Resolution Computed Tomography (HRCT) was done for all the patients. Patients were assessed by paediatric surgeon and eligible patients were operated. The procedure conducted was usually open thoracotomy under general anaesthesia. The affected lobes were removed and specimens were sent for histopathological analysis. All included patients were followed up prospectively to find out about their current level of health. Via telephonic interview they were asked about their overall growth, quality of life, activity, rate of respiratory infections and requirement of hospital admission. Results: Total 15 patients with diagnosis of CCAM were included in the study. Of them, 8 (53.3%) were male. The commonest presentation was cough 13(86%), sucking difficulty 11(73%), fever 9(60%), recurrent pneumonia 4(26%), hypoxia requiring oxygen supplementation 6(40%), others 2(12%). Thirteen patients required surgical intervention and underwent lobectomy. There were 2 cases of type I, one each of type II and III, 3 case of type IV while 5 were intermediate type. There was no procedure related mortality. The median duration of hospital stay and all were successfully discharged with median duration of stay 11 ± 16 days. Conclusion: The study concludes that if recognized early, surgic al removal of affected lung prevents the complications like recurrent pulmonary infections. The surgery is well tolerated without any post-operative mortality or morbidity. [ABSTRACT FROM AUTHOR]

Purpose: The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD).Materials and Methods: 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.Results: 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).Conclusions: 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. [ABSTRACT FROM AUTHOR]

Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, developmental, nonhereditary, hamartomatous pulmonary airway malformations. Etiology is unknown. It is a rare disease with an incidence of 1 in 11,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within the first 2 years of life. We report cases of three infants with CCAM (Stocker Type II and I) with brief review of literature. [ABSTRACT FROM AUTHOR]

Purpose Congenital cystic adenomatoid malformation (CCAM) of the lung in adults is very rare. We aimed to evaluate the clinicoradiological features of adult patients with CCAM. Methods Adult patients diagnosed with CCAM were evaluated for clinicoradiological presentation and management. Methods Nine patients were diagnosed with CCAM (four were incidentally diagnosed on chest imaging). Most cases appeared on computed tomography as thin-walled multiseptated cystic lesions. Two patients had another concurrent pulmonary developmental anomaly. Five patients were surgically treated and three conservatively. Conclusions CCAMs might be incidentally diagnosed on routine chest imaging in adults and can be safely treated with minimally invasive techniques. [ABSTRACT FROM AUTHOR]

Adenomatoid odontogenic tumor (AOT) is a relatively rare, benign, hamartomatous, and cystic odontogenic neoplasm that was first described more than a century ago. The lesion still continues to intrigue experts with its varied histomorphology and controversies regarding its development. The present article describes a case of cystic AOT with an unusual histomorphology associated with an impacted 44 in a 21-year-old male. [ABSTRACT FROM AUTHOR]

Background Congenital cystic diseases of the lung comprise a group of rare but closely related conditions with variable clinical features. This is a review of our experience with 64 cases outlining aspects of clinical features, diagnosis and management. Patients and methods The medical records of all infants and children with the diagnosis of congenital cystic diseases of the lung were retrospectively reviewed for age at diagnosis, sex, clinical features, diagnosis, treatment and outcome. The histology was obtained from the histopathology report. Results A total of 64 (39 males and 25 females) infants and children with congenital cystic diseases of the lung were treated. Their age at presentation ranged from 1 day to 15 years (mean 2.1 years). There were 23 cases of congenital lobar emphysema, 3 cases of pulmonary sequestration, 20 bronchogenic cysts and 18 cases of congenital cystic adenomatoid malformation. Twenty three (14 males and 9 females) patients had congenital lobar emphysema. Their age at presentation ranged from 1 day to 8 months (mean 3.1 months). The majority presented with variable degrees of respiratory distress symptoms and some of them were diagnosed because of repeated attacks of chest infection. Nine presented immediately after birth with respiratory distress symptoms, two of them had severe symptoms necessitating surgery within the first week after their delivery. In 19 patients the left upper lobe was affected and in the remaining 4, the right upper lobe was affected. Three patients (2males: 1female) had pulmonary sequestration. Two had extralobar pulmonary sequestration and the third had intralobar pulmonary sequestration. Twenty patients had bronchogenic cysts. There were 12 males and 8 females. Their age at diagnosis ranged from 2 months to 15 years (mean 4.2 years). Their clinical presentation was variable ranging from mild respiratory symptoms (2 patients), recurrent chest infection (11 patients) and respiratory distress (2 patients). In 5 patients, the cysts were discovered incidentally on routine chest x-ray. Two patients were born with large bronchogenic cysts and initially had mild respiratory distress but within two months, their symptoms increased necessitating thoracotomy and lobectomy. In 12 patients, the bronchogenic cysts were intra-lobar (right upper lobe 3, right lower lobe 4, right middle lobe 2, and left upper lobe 3). In 8, bronchogenic cysts were extra-lobar (right posterior mediastinum 4 and para-tracheal in 4). Eighteen patients had congenital cystic adenomatoid malformation. There were 11 males and 7 females. Their age at diagnosis ranged from 1 week to 12 years (mean 3.2 years). The presentation of these patients was also variable. The majority (14 patients) presented with repeated attacks of chest infection. In two patients, the diagnosis was confused with left congenital diaphragmatic hernia. Conclusions Congenital cystic diseases of the lung comprise a unique group of malformations with variable clinical presentation ranging from asymptomatic to those who present early with respiratory distress. Their common presentation is repeated chest infections. Awareness of this is important to obviate associated morbidity and mortality. Asymptomatic or mildly symptomatic congenital lobar emphysema can be treated conservatively, but the majority of patients with congenital cystic diseases of the lung require surgical excision. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Although there are reports of iatrogenic Horner syndrome in the adult population, pediatric cases are rare. The current report presents a case of acquired Horner syndrome that occurred after an epidural catheter was placed for pain control. Horner syndrome completely resolved after removing the catheter and no imaging or further work-up was necessary. [ABSTRACT FROM AUTHOR]

There is a wide variation in the management of infants with antenatally diagnosed lung malformations, with many paediatric surgeons and respiratory paediatricians recommending early investigations for all infants and a surgical excision for the majority of lesions, while others favour a conservative management for all asymptomatic infants. The benefits and risks of a surgical intervention have to be compared with the natural history of the untreated malformation and cohort studies from foetal diagnosis to adult life are required to provide the relevant evidence. Careful and repeated surveillance of identified foetuses is essential as recent advances in foetal medicine and surgery have improved the outcomes for the small minority, who are at risk of developing hydrops. [ABSTRACT FROM AUTHOR]

Introduction: Thoracoscopic lobectomy for congenital pulmonary airway malformation has been indicated from the neonatal period to adolescence. However, it is difficult to approach the pulmonary artery for lobectomy in congenital lung malformations with incomplete or absent interlobar fissures. Multidetector computed tomographic images and computed tomography pulmonary angiography gave us helpful information before the operation. We performed thoracoscopic lobectomy for congenital pulmonary airway malformations with absent interlobar fissures and adhesions in accordance with information from multidetector computed tomographic images. Case presentation: A 14-year-old Japanese girl received a diagnosis of congenital pulmonary airway malformation when she presented with pneumonia. Using multidetector computed tomography and three-dimensional reconstruction provides meticulous characterization of the anatomy in pediatric patients. We confirmed that her left A4+5 artery arose from her left pulmonary artery medial to A6. Her left pulmonary artery was divided just proximal to the A6 origin before the lobes were separated safely. We took advantage of using a stapler to divide the fissureless thick parenchyma. Perioperative diagnosis was congenital cystic adenomatoid malformation. Conclusions: We used preoperative multidetector computed tomography to outline the bronchovascular anatomy and guide hybrid video-assisted thoracoscopic surgery for a congenital cystic adenomatoid malformation in a fissureless left lung. [ABSTRACT FROM AUTHOR]

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Congenital cyst adenoid malformation (CCAM) is a rare congenital malformation occurring in approximately 1-4 in 100,000 births. It is classified into five subtypes with type 1 CCAM is most common subtype. The diagnosis of CCAM is usually made in infancy, and it is rare in adolescents and adults. We report a 15-year-old female, who presented in pediatric outpatient department with a history of recurrent cough since infancy. On the basis of clinical examination, provisional diagnosis of asthma was considered and patient was started on inhaled corticosteroid and long-term ß2 agonist. Lung function of the patient revealed low forced expiratory volume-1 s but without bronchodilator reversibility. Therefore, alternative diagnosis was suspected, and the patient was further evaluated with X-ray chest and high resolution computed tomography thorax. Based on radiological findings, a final diagnosis of CCAM was established. The case was highly unusual due to its atypical and late age of presentation. Acquaintance about this condition benefit clinician in making differential diagnosis of recurrent cough. [ABSTRACT FROM AUTHOR]

We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome). [ABSTRACT FROM AUTHOR]

The association of congenital pulmonary airway malformation (CPAM) with congenital heart disease is rare. We present the case of a 6-month-old child with atrial septal defect and pulmonary hypertension (PH) who presented with severe respiratory distress and hypoxia. The patient underwent right lobectomy for CPAM. With timely management, real-time monitoring, one lung ventilation, and adequate analgesia, we were able to extubate the child in the immediate postoperative period. We conclude that with meticulous planning and multidisciplinary team approach, such complex cases can be managed successfully. [ABSTRACT FROM AUTHOR]

This case report is of an infant born with a rare lung abnormality, Cystic Adenomatoid Malformation (CCAM). CCAM is a rare congenital lung malformation (incidence) representing 25% of congenital malformations and 95% of congenital lung lesions.(1,2) CCAM may be diagnosed in-utero by ultrasound examination, or after birth with the presentation of respiratory distress like symptoms. Differential diagnosis includes diaphragmatic hernia, pulmonary sequestration, bronchogenic cysts, and congenital lobar emphysema. Because most CCAM lesions are manageable with the proper assessment, diagnosis, and interventions. We report here a case of 45 day-old-male baby with features of respiratory distress since birth and X-ray chest suggestive of large cystic lesion of left lung CCAM. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 34-year-old pregnant woman, who was diagnosed with congenital hernia of the umbilical cord (CHUC), congenital cystic adenomatoid malformation (CCAM) at 26 weeks of gestation, and trisomy 13 at 27 weeks. Topics include the birth of a boy at 37 weeks by Caesarean section, with polydactyly, cleft lip and palate, and micropenis; prenatal ultrasonographic characteristics of CHUC; and use of ultrasonography and magnetic resonance imaging for prenatal diagnosis.

An abstract of the article "Prenatal ultrasound predictor factors in congenital cystic adenomatoid malformation," by A. Abascal Saiz and colleagues is presented.