Cystic Adenomatoid Malformation Research Articles

VP14.04: Fetal and neonatal outcomes of antenatally diagnosed congenital pulmonary airway malformations: a systematic review

VP14.04: Fetal and neonatal outcomes of antenatally diagnosed congenital pulmonary airway malformations: a systematic review

Thoracoscopic segmentectomy for a large previously undiagnosed CPAM presenting as a spontaneous pneumothorax: A case report.

IntroductionIncidentally found congenital pulmonary airway malformations (CPAM) in older children are extremely rare and have traditionally been managed with minimally invasive versus open lobectomy of the affected lobe.Presentation of caseIn this report, we present a 11-year-old male who presented with a recurrent spontaneous pneumothorax and was found to have a large symptomatic CPAM confined to a single segment of the right lower lobe. The patient was successfully treated with thoracoscopic segmentectomy without any residual disease seen on follow up imaging.DiscussionMinimally invasive thoracoscopic approach has many advantages over open approach including better pain control, reduced hospital length of stay, and decreased intraoperative blood loss. With increasing use of minimally invasive approaches, lung-sparing surgery has demonstrated to be a viable and an attractive option for definitive resection of CPAM, without compromising resection margins and/or future lung function.ConclusionThis report demonstrates that minimally invasive lung-sparing surgical treatment of a large CPAM is feasible in older children.

Congenital cystic adenomatoid malformation associated with right aortic arch with mirror image branching and absence of left pulmonary artery in an adult

BackgroundCongenital cystic adenomatoid malformation is an uncommon developmental anomaly that is diagnosed prenatally or during early childhood and is rare to present in adulthood. Type 2 congenital cystic adenomatoid malformation is often associated with other anomalies such as congenital cardiac anomalies. We present a case of type 2 congenital cystic adenomatoid malformation in an adult male associated with absence of left pulmonary artery and right aortic arch with mirror image branching. To our knowledge, this is the first case report with the combination of all three anomalies.Case presentationA 33-year-old male presented to the Department of Pulmonary Medicine with high-grade fever, chills and rigor, breathlessness, and productive cough for a duration of one week. He had multiple similar self-limiting episodes in the past which did not require hospitalization. The plain radiograph showed right aortic arch and a small left hilum. CT thorax showed multiple cystic lesions of size 0.5–2.0 cm in the apico-posterior segment of the left upper lobe; anterior, posterior, and lateral segments of the left lower lobe; and the apical segment of the right upper lobe with a maximal cyst wall thickness of 2 mm. Volume of left lung was reduced with trans-thoracic herniation of the medial segment of the right middle lobe; bronchiectatic changes were seen in the apical, anterior, and posterior segments of the right upper lobe; and fibrotic strands were seen in the apical segment of the right lower lobe and the apico-posterior segment of the right upper lobe. Mediastinal window showed right-sided aortic arch with mirror image branching. The pulmonary trunk was seen to continue as the right pulmonary artery with absent left pulmonary artery.ConclusionsThough congenital cystic adenomatoid malformation is a disease of infancy and childhood, it should also be considered in adults presenting with recurrent lower respiratory tract infections and/or pneumothorax. Radiologists must be aware of the types of congenital cystic adenomatous malformation (CCAM) and their associations, as type 2 CCAM has a high association with congenital cardiac disease. Proximal interruption of pulmonary artery should be considered if the pulmonary trunk continues as either right or left pulmonary artery.

In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience

BackgroundSevere primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). ObjectivesTo compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. MethodA retrospective analysis was performed for all fetuses treated by TAS, between the years 2004–2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. Results38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. ConclusionTAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.

To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM. This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center-Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth. Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM. CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Thoracic MDCT findings of a combined congenital lung lesion: Bronchial atresia associated with congenital pulmonary airway malformation.

To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children. All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics. Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.

Prenatal ultrasound measurements of mild congenital pulmonary airway malformation and long-term prognosis: A retrospective cohort study.

To investigate the relationship between mild congenital pulmonary airway malformation (CPAM) and its long-term prognosis in childhood and to explore whether surgery is necessary. We conducted a retrospective cohort of fetuses with mild CPAM diagnosed prenatally with available long-term outcomes in childhood from 2004 to 2016. The patients were divided into two groups according to the fetal CPAM-to-volume ratio (CVR) of less than 1.0 and 1.0-1.6. The primary outcome was a postnatal composite outcome including CPAM-associated respiratory symptoms and surgical resection of the lesion. The secondary outcomes included neonatal asphyxia, perinatal morbidity and mortality. Forty-two fetuses were identified as having CVR <1.0 or CVR-1.0-1.6 respectively (n=37 vs n=5; 88.1% vs 11.9%), with the median duration of follow up 2.15years (0.3-10.8years). Of 42 patients, 32 (76%) remained asymptomatic without recurrent respiratory symptoms or surgical resection; the other 10 with CVR <1.0 had respiratory symptoms. Of 10 symptomatic cases, five recovered after expectant treatment, and five underwent resection, for an increase in lesion size and recurrent respiratory infection. Patients with CVR <1.0 still need to be closely observed after birth. Conservative management is a reasonable option in asymptomatic cases, but surgery might be necessary in some.

Congenital cystic adenomatoid malformation: a case report

Congenital cystic adenomatoid malformation (CCAM) is a rare condition defined by multiple cysts produced in the lung that occur during the fetal period, with respiratory distress as presenting symptoms. Untreated CCAM may lead to repeated lung infection and pneumothorax. Many surgical techniques have been used to treat CCAM. However, those techniques showed various results. Moreover, less studies were performed to evaluate the effect of those surgical techniques in treating CCAM patients. We reported a management of a rare pediatric case of CCAM referred to the Department of Thoracic and Cardiovascular Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito General Hospital. The patient was a 29-day-old male baby who presented with pneumothorax on the right lung due to CCAM. The plan of treatment for the patient was lobectomy until pneumonectomy on the affected lung. During the thoracotomy procedure, we found that all lobes in the right lung were covered with fibrous tissue. Based on this finding, a decortication procedure to remove the fibrous tissue continued by a bullectomy procedure with the insertion of a chest tube were performed. The patient continuously showed improvement in breathing and wound healing, thus making the patient discharged from the hospital on the nineteenth postoperative day. The cause of CCAM is thought to be congenital abnormalities of the bronchiole epithelium that produce multiple cysts. Due to its rarity and lack of research on CCAM, many CCAM patients are misdiagnosed/ underdiagnosed. Common surgical methods used in this patient are lobectomy with continuation until pneumonectomy or bilobectomy, if necessary, to prevent recurrence. Parenchymal saving methods can be considered because they have the same outcome as lobectomy. As performed in this patient, thoracotomy decortication continued with bullectomy is adequately capable of alleviating respiratory distress symptoms and is thus described as successful.

Exencephaly with Cystic Adenomatoid Malformation- A Review of a Rare Case

Exencephaly is a rare malformation of the cranium which is characterized by absence of skull, cranium cavity and a scalp with protruding brain tissue outside the skull. The prognosis for infants born with this malformation is extremely poor. Exencephaly has been associated with several other congenital anomalies. We present a case of exencephaly associated with cystic adenomatoid malformation in autopsy findings in a17weeks female abortus. Keywords: Exencephaly, acrania, cystic adenomatoid malformation.

The etiology of diffuse cystic lung diseases: an analysis of 1010 consecutive cases in a LAM clinic

BackgroundThe differential diagnosis of diffuse cystic lung disease (DCLD) is a clinical challenge. We wish to analyze the distribution of the etiology of DCLD based on data from a single lymphangioleiomyomatosis (LAM) clinic.MethodsAll DCLD patients at the LAM Clinic of Peking Union Medical College Hospital between January 2006 and December 2019 were analyzed. Information on the demographic, clinical, radiological, and pathological features was collected.ResultsA total of 1010 patients with DCLD on CT scan were evaluated. A sum of 711(70.4%) patients were diagnosed with definite or probable LAM. Other diagnoses included Birt–Hogg–Dubé syndrome (46), Sjogren's syndrome (38), pulmonary Langerhans cell histiocytosis (14), lung tumors (3), Castleman disease (2), antineutrophil cytoplasmic antibody-associated vasculitis (2), systemic lupus erythematosus (1), Marfan syndrome (1), amyloidosis (1), congenital cystic adenomatoid malformation of the lung (1), and pleuroparenchymal fibroelastosis (1). In the 38 patients diagnosed with Sjogren's syndrome, 2 were diagnosed with light-chain deposition disease, 2 were diagnosed with amyloidosis and 1 was diagnosed with lymphocytic interstitial pneumonia. One hundred and eighty-nine patients (18.7%) were undiagnosed. Lung biopsy results were available in 27 patients in the undiagnosed DCLD group but did not provide a diagnosis.ConclusionApproximately 70% of DCLD patients in our LAM clinic had LAM. The common differential diagnoses included Birt–Hogg–Dubé syndrome, Sjogren’s syndrome, and pulmonary Langerhans cell histiocytosis. Detailed clinical information and laboratory, genetic, and pathological investigations provide correct diagnoses in most patients with DCLD.

A Rare Pediatric Case Report of a Hybrid Lesion of Congenital Pulmonary Airway Malformation and Bronchopulmonary Sequestration

Congenital malformations of the lungs are rare. Congenital pulmonary airway malformation and bronchopulmonary sequestrations are commoner malformations amongst them. Co-existence of both in a single lesion is extremely rare. Such hybrid lesions or rather malformations have been sparsely reported. We report a similar lesion in an eleven years old male who presented with hemoptysis and diagnosed to have a hybrid lesion.

Tension pneumothorax in a toddler with congenital cystic adenomatoid malformation

Abstract Congenital cystic adenomatoid malformations (CCAM) are the most common type of congenital parenchymal lung malformations in neonates as well as in children; presenting with sudden onset of respiratory distress, recurrent pulmonary infections, pneumothorax or haemoptysis. However they can be asymptomatic too. Here we are reporting a case of spontaneous pneumothorax occurring in a female toddler and challenges in its management.

Characterization of the Hyperintense Bronchus Sign as a Fetal MRI Marker of Airway Obstruction.

Background Fetal MRI-based differential diagnosis of congenital lung malformations is difficult because of the paucity of well-described imaging markers. Purpose To characterize the hyperintense bronchus sign (HBS) in in vivo fetal MRI of congenital lung malformation cases. Materials and Methods In this retrospective two-center study, fetal MRI scans obtained in fetuses with congenital lung malformations at US (January 2002 to September 2018) were reviewed for the HBS, a tubular or branching hyperintense structure within a lung lesion on T2-weighted images. The frequency of the HBS and respective gestational ages in weeks and days were analyzed. Areas under the curve (AUCs), 95% CIs, and P values of the HBS regarding airway obstruction, as found in histopathologic and postnatal CT findings as the reference standards, were calculated for different gestational ages. Results A total of 177 fetuses with congenital lung malformations (95 male fetuses) and 248 fetal MRI scans obtained at a median gestational age of 25.6 weeks (interquartile range, 8.9 weeks) were included. The HBS was found in 79% (53 of 67) of fetuses with bronchial atresia, 71% (39 of 55) with bronchopulmonary sequestration (BPS), 43% (three of seven) with hybrid lesion, 15% (six of 40) with congenital cystic adenomatoid malformation, and 13% (one of eight) with bronchogenic cyst at a median gestational age of 24.9 weeks (interquartile range, 9.7 weeks). HBS on MRI scans at any gestational age had an AUC of 0.76 (95% CI: 0.70, 0.83; P = .04) for the presence of isolated or BPS-associated airway obstruction at histopathologic analysis and postnatal CT. The AUC of HBS on fetal MRI scans obtained until gestational age of 26 weeks (AUC, 0.83; 95% CI: 0.75, 0.91; P < .001) was significantly higher (P = .045) than that for fetal MRI scans obtained after gestational age 26 weeks (AUC, 0.69; 95% CI: 0.57, 0.80; P = .004). Conclusion The hyperintense bronchus sign is a frequently detectable feature at fetal MRI and is associated with airway obstruction particularly before gestational age 26 weeks. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Dubinsky in this issue.

Surgery in congenital lung malformations: the evolution from thoracotomy to VATS, 10-year experience in a single center

BackgroundCongenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment.We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery.MethodsSurgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated.ResultsA total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy.ConclusionsCongenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers.

Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis

Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.

English

The differential diagnosis of the cystic lung disease is extensive, ranging from an isolated incidental lung cyst to multiple diffuse lung cysts with other varied associated lung abnormalities. High resolution computed tomography (HRCT) thorax is the imaging modality of choice in the evaluation of these diseases, by improving the characterisation of the lung cysts (number, size, shape, distribution, wall thickness and regularity), and associated pulmonary and extrapulmonary abnormalities (like ground glass densities, nodules, interstitial fibrosis, mediastinal lymphadenopathy). After differentiating from the common cyst mimickers (cavity, centrilobular emphysema and cystic bronchiectasis), they should be grouped by location into subpleural cysts (paraseptal emphysema, bulla and honeycombing) and intraparenchymal cysts. Intraparenchymal cysts are in turn categorised into solitary / localised cysts (incidental cyst, congenital pulmonary airway malformations, intrapulmonary bronchogenic cyst and hydatid cyst) and multiple diffuse cysts. The next step will be the categorisation of these multiple diffuse cysts into those without other lung abnormalities (lymphangioleiomyomatosis and Birt-Hogg-Dube syndrome) and those with other lung abnormalities such as predominant ground glass densities (Pneumocystis jirovecii pneumonia, desquamative interstitial pneumonia, lymphoid interstitial pneumonia, subacute hypersensitivity pneumonitis and pneumatoceles) or predominant nodules (pulmonary Langerhans cell histiocytosis, amyloidosis, light chain deposition disease, follicular bronchiolitis, recurrent respiratory papillomatosis, cystic tuberculosis, cystic lung metastasis, sarcoidosis, and pulmonary mesenchymal cystic hamartomas). We conclude that this orderly radiologic approach in a given HRCT chest study of a cystic lung disease (in addition to correlation with clinical and laboratory findings), can lead us to a specific diagnosis in majority of instances, thereby optimising their treatment management, without the need of an invasive biopsy. KEY WORDS HRCT Thorax, Lung Cysts, Ground Glass Densities, Lung Nodules

Narrative review of congenital lung lesions.

This article reviews the contemporary diagnosis and management of antenatally diagnosed congenital lung lesions. These anomalies, which include congenital pulmonary airway malformation (CPAM) (formerly congenital cystic adenomatoid malformation), bronchopulmonary sequestration (BPS), bronchogenic cyst, and congenital lobar emphysema (CLE), are relatively rare but are increasingly encountered by clinicians because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment of these lesions throughout pregnancy remains the norm rather than the exception. Perinatal management strategies may differ based on initial size and growth patterns of these masses until delivery. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, maternal steroids have become standard of care in the management of larger lesions at risk for nonimmune hydrops. As a result, fetal surgical procedures, including open resection, thoracoamniotic shunting, and ex utero intrapartum treatment (EXIT), are less uncommonly performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for postnatal lung resection on an elective basis. Many children are good candidates for minimally invasive (thoracoscopic) surgical approaches as an alternative to resection by thoracotomy. In the vast majority of cases, the overall prognosis remains excellent.

Application and effects of an early childhood education machine on analgesia and sedation in children after cardiothoracic surgery

ObjectiveTo study the effect of an early childhood education machine on sedation and analgesia in children after cardiothoracic surgery.MethodsA prospective randomized controlled study was conducted in a provincial hospital in China. Fifty-two patients (aged from 1 to 5 years) underwent cardiothoracic surgery (including: ventricular septal defect, patent ductus arteriosus, atrial septal defect, pulmonary stenosis, pulmonary sequestration and congenital cystic adenomatoid lung malformation) were divided into the study group (n = 26) and the control group (n = 26). The patients in the study group underwent intervention with an early childhood education machine (uniform type) in addition to routine standard treatment and nursing, while the patients in the control group only received routine standard treatment and nursing. Richmond agitation sedation score (RASS) and face, legs, activity, cry, consolability (FLACC) score of all of the patients were evaluated, and the negative emotions (self-rating anxiety scale (SAS) score and self-rating depression scale (SDS) score) of the parents of the two groups were compared.ResultsThere was no significant difference in the general clinical data between the two groups. The RASS and FLACC scores in the study group were significantly lower than those in the control group, and the SAS and SDS scores of the parents in the study group were significantly lower than those in the control group.ConclusionThe application of an early childhood education machine for children after cardiothoracic surgery can effectively reduce postoperative agitation, improve sedation and analgesia of the patients, and ease the pessimistic mood of the patients’ parents.

Perinatal Outcome in Fetuses with Dislodged Thoraco-Amniotic Shunts

Objective: Fetal thoraco-amniotic shunts (TASs) can dislodge in utero, migrating internally into the fetal thorax or externally into the amniotic cavity. Our objective was to evaluate the perinatal and long-term outcome of fetuses with TAS dislodgement and conduct a review of the literature. Methods: This is a retrospective review of all TAS inserted for primary pleural effusions and macrocystic congenital pulmonary airway malformations (CPAMs) in a tertiary fetal medicine center (1991–2020). Antenatal history, procedural factors, and perinatal and long-term outcomes were reviewed in all fetuses with dislodged shunts and compared to fetuses with shunts that did not dislodge. Results: Of 211 TAS inserted at a mean gestational age of 27.8 weeks ± 5.47 (17.4–38.1 weeks), 187 (89%) were inserted for pleural effusions and 24 (11%) for macrocystic CPAMs. Shunts dislodged in 18 fetuses (8.5%), 17 (94%) of which were for pleural effusions. Shunts migrated into the chest wall/amniotic cavity or into the thorax among 7/18 (39%) and 11/18 (61%) fetuses, respectively. Eleven (61%) fetuses were initially hydropic, which resolved in 8 (72%) cases. Effusions were bilateral in 9 (50%), amnioreduction was required in 6 (33%), and fetal rotation in 8 cases (44%). Four (22%) fetuses underwent repeat shunting, 12 (67%) neonates required ventilatory support, and 2 (11%) neonates required chest tubes. There was no significant difference in technical factors or outcomes between infants with shunts that dislodged and those that did not. Among 11 intrathoracic shunts, 2 (18%) were removed postnatally and the remainder are in situ without any shunt-related or respiratory complications over a follow-up period of 9 months to 22 years. Conclusion: TAS dislodged antenatally in 8.5% of fetuses, with 2/3 of shunts migrating into the thorax, and nearly 25% requiring re-shunting. Retained intrathoracic shunts were well tolerated and may not necessarily require surgical removal after birth.

An adult with episodic retrosternal chest pain: an unusual presentation of congenital pulmonary airway malformation \u2013 case report

BackgroundCongenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous connection presenting as episodic retrosternal chest pain on exertion in an adult has not been described.Case presentationA 21-year-old female, non-smoker, presented with a 4-year history of sharp, episodic, retrosternal chest pains brought on with exercise. A contrast-enhanced computed tomography (CT) scan showed a grossly overinflated left lower lobe with partial anomalous pulmonary venous drainage into the left hemi-azygos vein. Lobectomy, the recommended treatment of choice, carried out thoracoscopically, was curative with satisfactory mid-term results. Histology confirmed type-II congenital pulmonary airway malformation.ConclusionsCPAM can present in young adults with unusual symptoms of chest pain on exertion. When suspected a contrast-enhanced CT scan is the gold standard for establishing the diagnosis. An anatomical lung resection is curative with satisfactory medium term results.