The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.

INTRODUCTION: Congenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology. METHOD: This retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran. RESULT: The average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients' type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04). CONCLUSION: Some CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.

Transesophageal endoscopic resection of mediastinal cysts (with video).

BACKGROUND AND AIMS: Primary mediastinal cysts are infrequent lesions derived from a variety of mediastinal organs or structures. Complete surgical resection is the treatment of choice even in asymptomatic patients to prevent severe adverse events (AEs) and to establish the diagnosis. Transesophageal endoscopic resection of benign mediastinal tumors has been proven feasible. The aim of this study was to evaluate the feasibility, safety, and efficacy of transesophageal endoscopic surgery for mediastinal cysts. METHODS: From January 2016 to May 2021, patients with mediastinal cysts who underwent transesophageal endoscopic resection were retrospectively included. Clinicopathologic characteristics, procedure-related parameters, AEs, and follow-up outcomes were analyzed. RESULTS: Ten patients with mediastinal cysts were included in this study. The mean cyst size was 3.3 ± 1.3 cm. Histopathology revealed 3 bronchogenic cysts (30.0%), 4 esophageal duplication cysts (40.0%), 2 gastroenteric cysts (20.0%), and 1 lymphatic cyst (10.0%). All procedures were performed uneventfully without conversion to traditional surgery. En-bloc resection was achieved in 6 patients (60.0%). Aggressive resection was avoided to prevent damage to the surrounding vital organs. Mean resection time and suture time were 58.0 ± 36.4 minutes and 5.4 ± 1.0 minutes, respectively. No major pneumothorax, bleeding, mucosal injury, or fistula occurred. One patient had a transient febrile episode (>38.5°C). Mean postoperative hospital stay was 2.7 ± .9 days. No residual or recurrent lesions were observed in any patient during a mean follow-up period of 29.8 ± 19.5 months. CONCLUSIONS: Transesophageal endoscopic surgery appears to be a feasible, safe, effective, and much less invasive approach for mediastinal cyst resection. Larger prospective studies are required to fully assess the efficacy and safety of this novel technique.

[Congenital Malformations of the Lung - an Overview]. / Angeborene Fehlbildungen der Lunge ­ eine Übersicht.

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.

Congenital Mediastinal Neuroenteric Cyst as a cause of respiratory distress since birth.

Mediastinal Neuroenteric Cyst is a rare congenital presentation in infants and is associated with a high mortality rate. It is a very uncommon benign lesion and usually develops from abnormal embryological development of the foregut. Till now, only 106 cases have been reported worldwide. In Pakistan only three cases have been published, with varying presentations. The clinical presentation and age at presentation vary from asymptomatic and coincidental finding on chest x-ray, to limb numbness or early presentation with severe symptoms like those in our case. In fact, it poses an important challenge for paediatricians. We present a rare case with emphasis on clinical presentation and diagnostic criteria.

Bronchogenic cyst presenting with respiratory distress in a neonate.

Bronchogenic cysts are considered rare airway anomalies that can have a significant impact on the well-being of infants. In this case report, we present a rare presentation of bronchogenic cyst presenting with early neonatal respiratory distress due to airway and vascular compression. Surgical excision was curative with an excellent prognosis.

Ectopic bronchogenic cyst of the gastric cardia considered to be a gastrointestinal stromal tumor before surgery: a case report.

BACKGROUND: We herein report a rare case of an ectopic bronchogenic cyst of the gastric cardia. The initial diagnosis was a gastrointestinal stromal tumor (GIST); however, postoperative pathologic examination confirmed that it was a bronchogenic cyst. CASE PRESENTATION: A 62-year-old woman visited our hospital for abdominal pain. The diagnosis prior to surgery was a GIST. Computed tomography imaging showed that the mass was located in the gastric cardia on the side of the lesser curvature. During the surgical exploration, it was noted that the tactility of the mass was not consistent with a GIST. Thus, we decided to perform local resection of the mass and part of the gastric wall without wedge resection. The pathological examination revealed a bronchogenic cyst. CONCLUSIONS: This case suggests that a bronchogenic cyst should be considered as a differential diagnosis of a GIST. It is also a unusual but necessary situation should be considered when explaining the etiology of a bronchogenic cyst.

CT and MRI characteristics for differentiating mediastinal Müllerian cysts from bronchogenic cysts.

AIM: To evaluate how computed tomography (CT) and magnetic resonance imaging (MRI) characteristics can be used to differentiate immunohistochemically confirmed mediastinal Müllerian cysts (MMCs) from bronchogenic cysts (BCs). MATERIALS AND METHODS: Sixteen patients with histopathologically and immunohistochemically confirmed mediastinal cysts (four with MMCs and 12 with BCs) were included in this study. CT and MRI images were reviewed retrospectively and the location, size, CT attenuation, and MRI signal intensity of the two pathologies were compared. RESULTS: On review of CT images, cysts could be located to the anterior mediastinum in four BCs, middle mediastinum in three MMCs and seven BCs, and posterior mediastinum in one MMC and one BC. Contact with a vertebral body was observed in 4/4 MMCs (100%) and 6/12 BCs (50%). The ratios of minimum-to-maximum diameter (0.57±0.09 versus 0.74±0.11, p<0.01), CT attenuation (7.8±6 versus 44.3±12 HU, p<0.01), and cyst-to-spinal cord signal intensity ratios (SIRs) on T1-weighted images (0.56±0.2 versus 1.31±0.4, p<0.01) were significantly lower for MMCs than BCs. No significant differences in maximum diameter, minimum diameter, and SIRs on T2-weighted images were found between MMCs and BCs. CONCLUSION: In characterising mediastinal cysts in a middle-aged female patient, contact with a vertebral body, flattened configuration, hypodensity on CT, and hypointensity compared to spinal cord on T1-weighted images are features that are specific to MMCs.

An unusual case of cardiac tamponade: Bronchogenic cyst infection due to Salmonella bredeney.

We present an unusual case of cardiac tamponade in a 17-year-old girl immunocompetent patient due to Salmonella enterica ssp. bredeney following infection of a bronchogenic cyst. The patient was admitted to hospital with pleuritic chest pain, dyspnoea and fever. Pulmonary angio-CT showed a bronchogenic cyst compressing the left atrium. The echocardiography showed diffuse pericardial effusion with right ventricular collapse consistent with cardiac tamponade. Pericardiocentesis was performed and microbiological cultures of the pericardial fluid became positive for Salmonella species confirmed later as bredeney subspecies by PCR. Empirical antibiotherapy was started with intravenous (IV) ceftriaxone. Bronchogenic cyst infection was suspected and confirmed by 18FDG PET CT. The patient was successfully treated by complete resection of the cyst and continuation of IV ceftriaxone followed by oral amoxicillin/clavulanate for a total duration of 6 weeks. She then completely recovered and didn't present any relapse after 6 months of follow up.

Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation.

Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.

[Bronchogenic Cyst of Stomach:Report of One Case].

Bronchogenic cysts are rare congenital cysts caused by anomalism of foregut in the embryonic stage.They locate most frequently in the mediastinum and are rarely seen in stomach.Here we report a case of gastric bronchogenic cysts that was diagnosed and treated in Peking Union Medical College Hospital from April 2 to 9,2018.

Bronchogenic Cyst in the Head and Neck Region.

OBJECTIVE: The objective of this study was to review the clinical characteristics and surgical treatment outcomes of patients with bronchogenic cysts in the head and neck region. METHODS: A retrospective chart review was performed at Chonnam National University Hwasun Hospital from January 2006 to May 2016. RESULTS: Eight patients with a final diagnosis of bronchogenic cyst in the head and neck region were identified based on their medical records. The most common location for the head and neck lymphatic malformations was the neck (4 patients), followed by the soft palate (1 patient), the posterior pharyngeal wall (1 patient), the floor of mouth (1 patient), and the arytenoid (1 patient). The size of the lymphatic malformations ranged from 1 to 6 cm. Bronchogenic cysts recurred in 2 patients. Both these patients were disease-free after the revision operation. No major complications resulting from the surgical intervention were observed. CONCLUSION: Bronchogenic cysts are rare congenital malformations and they can occur at various sites in the head and neck region. Bronchogenic cyst should be considered in the differential diagnosis of midline and lateral neck masses or intraoral cysts.

Intramural esophageal bronchogenic cyst mimicking achalasia in a toddler.

Bronchogenic cysts are congenital malformations of the tracheobronchial tree. We describe a 20-month-old male who presented with persistent non-bilious emesis; manometry and imaging were consistent with esophageal achalasia. During a planned laparoscopic Heller myotomy, an intramural bronchogenic cyst was discovered in the anterior esophagus at the level of the gastroesophageal junction and successfully resected with resolution of his symptoms.

Intrapericardial Development of a Bronchogenic Cyst - Case Report.

INTRODUCTION: Bronchogenic cysts, commonly located in the mediastinum or lung parenchyma, arise from abnormal budding of the tracheobronchial tree. Since an unknown percentage of asymptomatic adult patients with bronchogenic cysts remain undiagnosed, the true natural history ofthese cysts in adults is uncertain and the available series reflect a significant selection bias in favor of the symptomatic individual. CASE REPORT: We present the case of a 42-year-old female who presented with adull central chest pain, which gradually worsened overseveral weeks. After several tests performed (standard blood tests, standard X-Ray, ECG, angio-CT scan), we identified a mediastinal mass inferior to the carina and adjacent tothe left pleura, left atrium, pulmonary artery and esophagus. Considering the size of this mass and the possible complications, surgery was proposed and preformed. We were able to completely remove the cyst and the patient had no complications after surgery. DISCUSSIONS: It may be difficult to differentiate a bronchogenic cyst from other lesions (acquired cystic lesions, mediastinal masses). Serious complications from bronchogenic cysts are rare, but can include SVC syndrome, tracheal compression, pneumothorax, pleurisy and pneumonia. Surgical excision is recommended even for asymptomatic cysts, to prevent complications and operative difficulties. CONCLUSIONS: Bronchogenic cysts are rare and usually asymptomatic. Asymptomatic patients with bronchogenic cysts may become symptomatic cases, and in time may develop lifethreating complications. Definitive tissue diagnosis is usually available only after surgical excision. Based on our experience and after studying data from literature, we can conclude that bronchogenic cysts should be treated surgically and that a conservative approach is not recommended.

Neonatal respiratory distress syndrome revealing a cervical bronchogenic cyst: a case report.

BACKGROUND: Bronchogenic cyst is a congenital malformation, rarely located in the cervical region and almost never involved in a neonate with acute respiratory distress in the delivery room. CASE PRESENTATION: A female newborn with respiratory distress syndrome caused by a large left cervical mass. Intubation was difficult due to tracheal deviation. Magnetic resonance imaging confirmed a left cervical cyst displacing the trachea and esophagus laterally. Surgical excision was performed via a cervical approach on the 5th day, and pathological examination revealed a bronchogenic cyst. The patient's course was complicated by left vocal cord paralysis and necrotic lesions in the glottic and subglottic regions; she required a tracheostomy on the 13th day. Inflammatory stenosis in the subglottic region required balloon dilation once, 20 days later. Proximal esophageal stenosis induced transient upper airway obstruction with salivary stasis. Decannulation was performed at 2 months and the patient was discharged 10 days later. CONCLUSION: A bronchogenic cyst can exceptionally obstruct the airways in the neonatal period. Surgical excision is necessary, but postoperative complications may occur if the cyst is in close contact with the trachea and esophagus, including necrotic and stenotic lesions of the upper aerodigestive tract. In those situations, tracheostomy may be necessary for mechanical ventilation weaning and the initiation of oral feeding.

Completely intramural bronchogenic cyst of the cervical esophagus in a neonate.

Bronchogenic cysts are congenital cystic lesions of foregut origin, usually intrapulmonary or mediastinal, while esophageal cysts generally originate within the esophagus. To the best of our knowledge, this is the first report of a case of a completely intramural bronchogenic cyst of the cervical esophagus in a neonate.

[ASYMPTOMATIC ADULT RETROGASTRIC BRONCHOGENIC CYST: DIAGNOSIS AND TREATMENT CONSIDERATION BY ENDOSCOPIC ULTRASOUND FINE NEEDLE ASPIRATION (EUS-FNA)].

BACKGROUND: Adult bronchogenic cysts are rare. Retrogastric bronchogenic cysts are even rarer with only over 30 cases being reported in the English literature. These foregut cysts can be confused with cystic tumors. CASE PRESENTATION: We describe the case of a large gastric bronchogenic cyst in an asymptomatic 61-year-old woman who underwent an abdominal computerized tomography as part of a routine follow-up after lumpectomy due to breast cancer. Final diagnosis was made by endoscopic ultrasound fine needle aspiration (EUS-FNA) which led to conservative management of the patient who has remained asymptomatic over the last 8 years. CONCLUSIONS: Preoperative diagnosis of adult gastric bronchogenic cyst has been difficult to achieve. Our case highlights the importance of EUS in establishing the definitive diagnosis, enabling just follow-up with repeated imaging studies. The relevant literature is discussed.

[Analysis of clinical features of retroperitoneal bronchogenic cyst].

OBJECTIVE: To analyze the clinical features of retroperitoneal bronchogenic cyst. METHODS: The clinical data of 6 cases with retroperitoneal brochogenic cyst treated in Peking Union Medical College Hospital from April 1996 to October 2014 were retrospectively analyzed. The clinical manifestation, diagnosis, treatment and prognosis were analyzed. RESULTS: Of the patients, 1 was male and 5 were female aging from 31 to 50 years with a mean age of 38.3 years. Three cases were diagnosed from physical examination, 2 cases from upper abdominal pain and 1 case from left flank pain. The cysts located in the left adrenal region, between the liver and the pancreas, and anterior aspect of the tail of the pancreas were seen in 4 cases, 1 case and 1 case, respectively. The major diameter was from 5 cm to 13 cm, and the mean major diameter was 7 cm. Ultrasonography and CT scan could reveal cystic, cystic-solid or solid masses. Color doppler flow imaging showed no obvious blood flow, and contrast-enhanced CT scans showed no enhancement or no obvious enhancement. Six cases were all misdiagnosed preoperatively. They all underwent operations via retroperitoneal laparoscopic resection for 3 cases, laparotomy for 2 cases and open flank resection for 1 case respectively. The pathological diagnoses were all bronchogenic cysts. Three symptomatic patients became asymptomatic after operations. Five patients had been followed up. During the follow-up of 2 months to 15 years, no recurrence had been found with CT scan. CONCLUSIONS: Retroperitoneal bronchogenic cyst is rare and easily misdiagnosed. It should be considered in the differential diagnosis of a retroperitoneal mass. Most cysts are positioned in the left adrenal region and adjacent regions. Some cysts demonstrate soft tissue characteristics in image. After surgical removal, the patients have a good prognosis.