Congenital Cystic Malformation Research Articles

Efficacy of Da Vinci Robot-assisted Thoracoscopic Surgery in Children With Congenital Cystic Adenomatiod Malformation

BackgroundSurgical intervention is advisable for both asymptomatic and symptomatic CCAM children. This study aims to compare and analyze the efficacy of thoracoscopic and Da Vinci robot-assisted procedures in the management of CCAM among pediatric patients. MethodsThe clinical data of 188 pediatric patients diagnosed with CCAM and admitted to the Children's Hospital, Zhejiang University School of Medicine, from April 2019 to April 2023 were retrospectively analyzed. The Clavien-Dindo classification was employed for the systematic categorization of postoperative complications. ResultsThe demographic and clinical characteristics of the patients were comparable between the two groups. Postoperative outcomes, such as the chest tube indwelling rate (92.6% vs 36.2%, p < 0.001∗), chest tube duration (2.0 (2.0–3.0) days vs 1.0 (1.0–2.0) days, p < 0.001∗), and length of postoperative hospital stay (6.0 (5.0–7.0) days vs 5.0 (5.0–6.0) days, p < 0.001∗), favored RATS over VATS. Additionally, there was no significant difference in complications between the two group, but the p-value is in a critical state. Ⅲa complications (mainly composed of postoperative thoracentesis procedures) manifesting as a higher rate in the RATS, nearly double that observed in the VATS. ConclusionsRobot-assisted thoracoscopic lung resection is demonstrated to be safe and feasible, with notable advantages in short-term postoperative clinical outcomes. Nevertheless, the practicality and long-term benefits of this technique necessitate further refinement and dedicated study. Level of EvidenceLEVEL III.

Abstract #1388241: A Rare Case of an Intrathyroidal Bronchogenic Cyst

Bronchogenic cysts are uncommon congenital cystic malformations that arise due to dysplasia during ventral foregut development of the respiratory tract. They are mostly asymptomatic but can grow in size, causing compressive symptoms secondary to infection or malignancy. Bronchogenic cysts are usually located in the mediastinum or intrapulmonary regions, although they can rarely present in ectopic extrathoracic locations such as the thyroid. Here we report a rare case of an intrathyroidal bronchogenic cyst. A 48-year-old woman was incidentally found to have a thyroid mass on magnetic resonance imaging (MRI) neck by her neurologist during workup for migraines. An initial thyroid ultrasound showed a 4.7 cm left thyroid cyst with no concerning features, and a repeat follow up ultrasound 6 months later reported no change in its size. TSH, free T3, and free T4 were unremarkable. However, the patient began to endorse fullness in her neck that progressed to compressive symptoms, including dysphagia with solids and liquids, shortness of breath, and mild dysphonia. Endocrinology and otolaryngology (ENT) were consulted. ENT performed a nasopharyngolaryngoscopy, which was unremarkable for masses. A fine-needle aspiration (FNA) drained 25 mL of cystic fluid with non-diagnostic pathology with a virtually acellular specimen. FNA for parathyroid hormone level was low at 6 pg/mL (10 to 55 pg/mL), not suggesting parathyroid tissue. Compressive symptoms initially improved after FNA, but within 1 month her symptoms returned. Repeat thyroid ultrasound after FNA showed a 2.6 x 2.0 x 3.8 cm cystic nodule with regular margins. The patient opted for thyroid lobectomy as opposed to repeat aspirations. Her thyroid lobectomy was uncomplicated. The surgical biopsy was consistent with an intrathyroidal bronchogenic cyst. Her symptoms resolved after surgery and at her 3-month follow appointment, she continued to do well with resolution of symptoms. Bronchogenic cysts are quite rare in the thyroid. To our knowledge, there have only been 6 cases of intrathyroidal bronchogenic cysts previously reported. Bronchogenic cysts can grow in size causing compressive symptoms, with simple aspiration having a high rate of recurrence. Infection or, less commonly, transformation into malignant tumors are possible complications. Treatment for asymptomatic bronchogenic cysts remains controversial. However, once symptomatic, surgical resection is recommended over observation. Prognosis is generally excellent without recurrence for full resections.

Prenatal diagnosis of cystic hygroma cases in a tertiary centre and retrospective analysis of pregnancy results

The aim of this study is to retrospectively examine invasive diagnostic methods, structural anomalies accompanying cystic hygroma, and pregnancy outcomes in cystic hygroma cases admitted to a tertiary centre. The population of the study consisted of 29 live foetuses with cystic hygroma in the foetal neck only in the first or second trimester. In the study, pregnant women who applied to our centre were included. Amniocentesis or chorionic villus sampling was performed for genetic analysis according to the weeks of the pregnant women who were diagnosed with cystic hygroma by ultrasound examination by two clinicians experienced in foetal anomaly. Of the pregnant women included in the study, 10 had normal karyotype, 12 had abnormal karyotype and 13 had structural abnormality. It is very important to provide genetic counselling to the families of foetuses with cystic hygroma with a multidisciplinary team approach consisting of neonatologists, paediatric surgeons and experienced sonographers. Implications for rehabilitation What is already known on this subject? Cystic hygroma, also known as cystic lymphangioma, is a congenital cystic malformation often seen in the first trimester, which occurs in the foetal neck due to the failure of the connections between the cervical lymphatic vessels and the jugular venous system to develop normally. Cystic hygroma may be isolated, but highly associated with foetal aneuploidy, hydrops fetalis, abnormal foetal nuchal translucency. What do the results of this study add? Invasive prenatal diagnostic tests (CVS or amniocentesis) should be performed in all patients with cystic hygroma, as cystic hygromas can be diagnosed by first trimester foetal genetic sonogram screening and are largely accompanied by chromosomal abnormalities. What are the implications of these findings for clinical practice and/or further research? In foetuses with cystic hygroma, foetal karyotyping, detailed sonography and their documentation, genetic counselling is important to families of cystic hygroma foetuses with a multidisciplinary team approach consisting of neonatologists, paediatric surgeons and maternal foetal medicine specialists, since there is a high risk for aneuploidy and foetal malformation.

Late referral of fetal congenital cystic adenomatoid malformation in COVID-19 pregnant women: Diagnostic and management dilemma from limited resource setting country: Case report

we reported a case of late congenital cystic malformation (CCAM) diagnosis with problematic COVID-19 infected condition during delivery and dilemmatic neonatal management. A 24-year-old pregnant woman, second pregnancy, came very late in pregnancy (38 weeks gestational age), misdiagnosed with congenital diaphragmatic hernia. Our ultrasound examination revealed congenital cystic malformation type-1 in the right lung, with some of the normal lung lobes. The Left side lung was normal. CCAM volume ratio (CVR) 2,79 cm. Due to COVID-19 infection, our multidisciplinary team decided to perform quick chest tube insertion during the ex-utero intrapartum therapy (EXIT) procedure to avoid pneumothorax complications continued with intubation for the newborn. Neonatal middle right lung lobectomy was done after optimal condition at 20 days old with pathology anatomic result of CCAM type 1. The diagnosis, type, and prognosis of CCAM should be established earlier to make enough time for the better-prepared multidisciplinary management of the newborn.

"Prenataly Diagnosed Bilateral Congenital Cystic Adenoid Malformation, Class 2, a Case Report"

Slagjana Simeonova Krstevska1*, Vesna Naunova2, Lazo Jovcevski2, Toni Risteski2, Igor Samardziski1, Sofija Zlateska1, Aneta Sima1, Daniel Milkovski1, Ivo Kjaev1, Vlatko Girevski1 and Darko Andonovski3 Author Affiliations 1University clinic for obstetrics and gynecology, Macedonia 2University clinic for pediatric surgery, Macedonia 3PHU “Dr Andonovski” Probishtip, N. Macedonia Received: March 12, 2022 | Published: March 24, 2022 Corresponding author: Slagjana Simeonova Krstevska, University clinic for obstetrics and gynecology, Macedonia DOI: 10.26717/BJSTR.2022.42.006813

Surgical Repair of Atrial Septal Defect and Lung Lobe Resection for Pulmonary Sequestration and Cystic Malformation in One Stage: A Case Report

Incidence of congenital cystic malformation of lungs ranges from 1 in 11,000 to 1 in 35,000 live birth. Concurrence of Congenital Pulmonary Airway Malformation (CPAM) with congenital heart disease is even infrequent. Even rarer, is the incidence of congenital pulmonary vascular and cystic parenchymal lesions with cardiac defects. The non functioning lung tissue is separated from bronchial tree and vascularised by aberrant artery from the systemic circulation. Simultaneous repair of cardiac and pulmonary defects, though challenging, but are preferable because of surgery being done in single stage with better cosmesis. A 10-yearold female child presented to the hospital with recurrent chest infections and respiratory distress while running since five years of age. The patient underwent atrial septal defect closure and right lower lobectomy for CPAM in one stage through midline sternotomy. We conclude that such complex cases can be managed successfully with meticulous planning and multidisciplinary team approach.

Mediastinal and retroperitoneal transdiaphragmatic bronchogenic cyst with gastric mucosa

Congenital cystic lung lesions are rare lung malformations. They are mostly diagnosed by a routine antenatal ultrasound scanning. Clinical symptoms ranging from asymptomatic condition to life-threatening course. We presented a case of congenital cystic lung malformation, the case demonstrated a 20- month- old boy who was reported to our hospital because he was diagnosed prenatally with a right-sided cystic lung mass. He was asymptomatic. A diagnosis of mediastinal retroperitoneal bronchogenic cyst was made radiologically. He underwent a successful right thoracotomy and the transdiaphragmatic cystic mass was completely resected. Histologic examination revealed bronchogenic cyst with gastric mucosa. Early diagnosis improves outcomes in infants with congenital cystic lung anomalies. Surgical management is the treatment of choice for congenital cystic lung malformations.

Esophageal Duplication Cysts in 97 Adult Patients: A Systematic Review.

Esophageal duplication cysts are a rare congenital cystic malformation from faulty intrauterine recanalization of the esophagus during the 4-8th weeks of development. They account for 20% of all gastrointestinal duplication cysts and commonly involve the distal esophagus. Presenting symptoms may be related to size and location. Following the PRISMA guidelines, a systematic review was performed by searching published literature in various databases. Data from 97 reported case reports were pooled to present a descriptive and statistical analysis. Patient population was composed of 51(52.5%) males and 46 (47.5%) females, and mean ages was 42.3years (18-77). Distal cysts were the most prevalent. Seventy-nine (81.4%) patients were symptomatic; common symptoms included dysphagia, chest pain, cough and weight loss. Fifteen (15.5%) patients were treated conservatively and 75 (84.5%) by surgical treatment, among them thoracotomy in 30 (30.9%) patients and VATS in 17 (17.5%) patients. Mean length of hospital stay was 8.6days (range: 1-26days). One fatality was registered. Location, unlike size, was not found to influence presenting symptoms or treatment employed. Frequency of conservative treatment was not significantly different between symptomatic and asymptomatic patients. Open approaches were associated with longer stays than their minimally invasive counterparts. Esophageal duplication cysts remain rare in adults and are frequently located in the distal esophagus. Larger cysts are more likely to cause symptoms. Various surgical techniques may successfully be employed in the treatment of this pathology. Minimally invasive procedures have a shorter hospital stay.

Hybrid Congenital Lung Malformation with Difficulty in Diagnosis and Treatment: Congenital Cystic Adenoid Malformation and Pulmonary Sequestration Co-existence

Hybrid Congenital Lung Malformation with Difficulty in Diagnosis and Treatment: Congenital Cystic Adenoid Malformation and Pulmonary Sequestration Co-existence

Routine betamethasone therapy of non-hydropic fetuses with CPAM - the way to improve perinatal outcome?

Authors discuss novel approach to the management of fetal congenital pulmonary cystic malformation (CPAM) and possible benefit of routine administration of betamethasone, which is currently recommended only for severe cases. The article presents authors' own experience with antenatally diagnosed CPAM and describes 4 cases of prenatally diagnosed CPAM without hydrops treated by two doses of betamethasone at 21-31 weeks of gestation with the aim of improving the perinatal prognosis by effect on not only mortality but also postnatal morbidity. Article also summarizes current knowledge on all aspects of the prenatal CPAM focusing on its treatment options. Data were obtained from the literature search based on the PubMed and Scopus database with additional search of particular articles from reference list of the selected publications.All 4 patients from the case reports showed regression of the pulmonary mass after maternal administration of betamethasone with 100 % survival rate. Even though it is not possible to say if the CPAM regression was due to the betamethasone treatment, we did not observe any complication in relation to the treatment and all cases survived until discharge. During the literature search, we did not find any data on betamethasone administration in non-hydropic fetuses with CPAM in relation to the overall perinatal and postnatal morbidity, neither data comparing the outcome between the treated versus observed only fetuses.Routine betamethasone treatment should be discussed in antenatally diagnosed CPAM cases without fetal hydrops in order to reduce the perinatal morbidity associated with CPAM (Tab. 1, Ref. 47). Keywords: betamethasone, CPAM (congenital pulmonary adenomatoid malformation), fetal therapy.

A CASE OF CHRONIC PULMONARY ASPERGILLOSIS IN A HEALTHY IMMUNOCOMPETENT PATIENT

SESSION TITLE: Chest Infections: Find the Fungus SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Chronic pulmonary aspergillosis (CPA), and specifically its most common form – chronic cavitary pulmonary aspergillosis (CCPA) - is an uncommon pulmonary disease (1). It may be seen in either patients are who are immunocompromised or patients who are immunocompetent with underlying lung disease. We present a case of CCPA complicated by recurrent hemoptysis necessitating surgical resection in a healthy 19-year-old female without known underlying lung disease. CASE PRESENTATION: A 19 year-old female presented with one month of hemoptysis. She was healthy, without any known medical problems until age 17 when she was found to have a mycetoma and cavitary pneumonia. She underwent an extensive workup, including HIV and Quantiferon Gold testing, at that time which was negative and was treated with antibiotics. She was unable to complete a prolonged course of voriconazole due to financial constraints. Interval imaging demonstrated multiple cavitary lesions with air fluid levels and soft tissue density consistent with aspergilloma. She was asymptomatic between age 17 and 19, when she began to experience hemoptysis. The patient presented to our hospital with persistent hemoptysis. She underwent robotic video-assisted thorascopic surgery (VATS) with right lower lobectomy. The parenchyma was noted to contain numerous cystic cavities. Histological examination demonstrated free-floating aspergillus hyphae within a cyst wall. Although not definitive, the aspergilloma may have formed in an area of interlobar pulmonary sequestration or congenital cystic adenoid malformation. Five days following surgery, the patient had no further hemoptysis and was discharged home. DISCUSSION: CCPA is a common form of CPA, which also encompasses aspergillus nodules, simple aspergilloma, and subacute invasive aspergillosis (SAIA) or chronic necrotizing pulmonary aspergillosis. Left untreated, CCPA may progress to chronic fibrosing pulmonary aspergillosis (CFPA), which is characterized by extensive pulmonary fibrosis. It is exceedingly unusual for CCPA to develop in immunocompetent individuals without underlying pulmonary disease. Our patient is a young and healthy female who had no prior respiratory symptoms who presented at the age of 17 with an already formed aspergilloma. Oral antifungal therapy is considered standard of care for treatment of CCPA (1). Response is slow and requires a minimum of four months. Hemoptysis may necessitate surgical resection in those with adequate pulmonary function. CONCLUSIONS: CCPA may occur in imunnocompetent patients with no prior history of lung disease. The possibility of the presence of a previously unknown and asymptomatic congenital malformation may increase the risk for the development of CPA. Reference #1: Denning DW, Cadranel J, Beigelman-Aubry C, et al. Chronic pulmonary aspergillosis: rationale and clinical guidelines for diagnosis and management. Eur Respir J 2016;47:45-68. DISCLOSURES: No relevant relationships by Craig Fryman, source=Web Response No relevant relationships by Sameer Khanijo, source=Web Response No relevant relationships by Kelvin Wong, source=Web Response

Type I regressed pleuropulmonary blastoma in a 10-year-old boy.

Pleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystectomy was performed. Microscopic examination confirmed the presence of PPB type Ir. Patient was managed surgically alone with no added chemotherapy, as there was no overall survival benefit. PPB Ir has an overall favorable clinical outcome. Limited follow-up data are available due to the rarity of the lesion and the overlap with other congenital cystic lung malformations.

Врожденная кистозная мальформация легких у новорожденных

Врожденная кистозная мальформация легких у новорожденных

The usefulness of 3D-CT simulation and intraoperative navigation in pediatric minimally invasive surgery

Abstract Background/aims The aim of this study was to evaluate the usefulness of 3D-CT simulation in pediatric minimally invasive surgery. Methodology adenomatoidPreoperative 3D-CT simulation was performed using data from contrast enhancement MDCT and SYNAPSE VINCENT software. The images of 3D CT simulation were installed to iPad, and were used as intra-operative images. From 2011 to 2015, three cases who underwent minimally invasive surgery were enrolled. (hepatoblastoma (n = 1), Congenital cystic aednomatoid malformation (CCAM) (n = 1) and mediastinal tumor (n = 1)). Results Case 1: 1y-6m male with hepatoblastoma (PRETEXT I, S5) underwent laparoscopic surgery after the preoperative chemotherapy (CDDP 4 course). On preoperative 3D-CT simulation, the positional relationship between the lesion, vasculature and bile duct had been described well. And the laparoscopic tumor resection (partial hepatectomy) was safely performed. Case 2: 11 m female with Congenital Cystic Adenomatoid Malformation (CCAM) underwent thoracoscopy-assisted lobectomy. On preoperative 3D-CT simulation, the positional relation ship between the lesion, vasculature and bronchus had been described. Furthermore, 3D-CT described the aberrant-V2 which was arisen from inferior pulmonary vein to drain the right upper lobe. And the thoracoscopy-assisted resection of right upper lobe was safely performed. Case 3: 6-years-old male with mediastinal cyst (e.g. bronchogenic cyst) underwent completely-thoracoscopic tumorectomy. On preoperative 3D-CT simulation, the positional relationship among the lesion, bronchi, cardiovascular system, and esophagus. Conclusions Preoperative 3D-CT simulation and intraoperative navigation for pediatric minimally invasive surgery were useful and feasible.

Neonate with congenital cystic adenoid malformation of lung for lobectomy: Anesthesia concerns.

Neonate with congenital cystic adenoid malformation of lung for lobectomy: Anesthesia concerns.

Congenital Pulmonary Malformations in Children: Diagnosis and Management in a Pediatric Department in a Low Source Country

Congenital pulmonary malformations (CPM) comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia and congenital lobar emphysema.Socioeconomic, demographic, and hospital factors were determinants of survival of CPM. Antenatal diagnosis should be developed in order to improve management and prognosis of CPM in country with low resource.

Long-term outcomes of congenital lung malformations

Congenital lung malformations comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia, and congenital lobar emphysema. These anomalies are detected with increasing frequency by pre-natal sonography, but may also present for the first time with symptoms in childhood or later life. When symptomatic, there is little controversy that resection is indicated, which is usually curative. When a lesion is asymptomatic there is greater debate regarding the benefit of resection versus continued observation. This article provides an overview of the spectrum of disorders, the management options available and the long-term outcomes associated with each treatment option.

Complicated pneumothorax and congenital lung cystic malformation

Congenital cystic adenomatoid malformation, also named congenital pulmonary airway malformation (CPAM), is a congenital lung abnormality which is uncommon in adults. The usual radiological appearance of CPAM is a cystic space-occupying lesion. We present one case of CPAM with unusual clinical and radiological findings, a complicated spontaneous pneumothorax with intracystic haemorrhage with successful conservative initial treatment, despite acute haemodynamic instability.

Lung abscess in congenital cystic malformation during early infancy

Congenital Pulmonary Airway Malformation (CPAM) previously known as Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway disease is a developmental, non-hereditary hamartomatous abnormality of lung of unknown cause. Lung abscess is a rare condition and much rarer in early infancy. If CPAM is suspected as the cause of repeated pulmonary infection limited to single lobe, patient should be treated early with surgical removal of the affected lobe as Type I CPAM the most frequent subtype, is associated with risk of malignant transformation. We present a 52days old child presented with lung abscess in CPAM.

Congenital Cystic Lung Malformation Presenting in a Previously Asymptomatic Thirty-Year-Old Patient

This case is a rare presentation in an adult in his thirties of an uncommon disease normally detected in utero or shortly thereafter. It posed a diagnostic dilemma for our surgical and medical teams initially.