Ventral Foregut Research Articles

487. A RARE CASE OF BRONCHOGENIC CYST ESOPHAGUS

Abstract Introduction Bronchogenic cyst is a relatively rare congenital malformation that develops from abnormal budding of the ventral foregut during the early stage of gestation. Histologically, the cyst wall is covered by respiratory-type ciliated epithelium that may include cartilage and bronchial glands. Case report We present a 20 yrs male with chest discomfort during swallowing for 6 months. Evaluated with Upper GI scopy showed smooth mucosal elevation 30-35 cm from incisors. CECT Chest Showed features of Intramural cystic lesion of size 7x7cm.Patient was evaluated and taken up for surgery. Robotic Thoracoscopic cyst excison was done. Intraoperatively 7x7cm cystic lesion in lower esophagus infra carinally. Cyst has no communication with mucosa. Post operatively patient recovered well, Drin removed on 3 rd Post operative day and Patient was discharged on 4 th day. Histopathology showed the cyst wall is covered by respiratory-type ciliated epithelium that may include cartilage and bronchial glands https://1drv.ms/v/s!AklfEcoVwf9chCmLN-FSXBA2yV3B

Expansion of ventral foregut is linked to changes in the enhancer landscape for organ-specific differentiation

Cell proliferation is fundamental for almost all stages of development and differentiation that require an increase in cell number. Although cell cycle phase has been associated with differentiation, the actual process of proliferation has not been considered as having a specific role. Here we exploit human embryonic stem cell-derived endodermal progenitors that we find are an in vitro model for the ventral foregut. These cells exhibit expansion-dependent increases in differentiation efficiency to pancreatic progenitors that are linked to organ-specific enhancer priming at the level of chromatin accessibility and the decommissioning of lineage-inappropriate enhancers. Our findings suggest that cell proliferation in embryonic development is about more than tissue expansion; it is required to ensure equilibration of gene regulatory networks allowing cells to become primed for future differentiation. Expansion of lineage-specific intermediates may therefore be an important step in achieving high-fidelity in vitro differentiation.

SOX2 and SOX21 in Lung Epithelial Differentiation and Repair.

The lung originates from the ventral foregut and develops into an intricate branched structure of airways, alveoli, vessels and support tissue. As the lung develops, cells become specified and differentiate into the various cell lineages. This process is controlled by specific transcription factors, such as the SRY-related HMG-box genes SOX2 and SOX21, that are activated or repressed through intrinsic and extrinsic signals. Disturbances in any of these processes during the development of the lung may lead to various pediatric lung disorders, such as Congenital Diaphragmatic Hernia (CDH), Congenital Pulmonary Airway Malformation (CPAM) and Broncho-Pulmonary Dysplasia (BPD). Changes in the composition of the airways and the alveoli may result in reduced respiratory function and eventually lead to chronic lung disorders. In this concise review, we describe different intrinsic and extrinsic cellular processes required for proper differentiation of the epithelium during development and regeneration, and the influence of the microenvironment on this process with special focus on SOX2 and SOX21.

Infected Bronchogenic Cyst with Mycobacterium tuberculosis as a Cause of Respiratory Distress: A Case Report

Bronchogenic cyst is a common primary cyst of the mediastinum, and it is usually located in the middle mediastinum and intrapulmonary regions. Bronchogenic cysts are lesions of congenital origin that occur due to abnormal budding from the primitive ventral foregut. They are common in the pediatric age group and have symptoms of respiratory distress, recurrent cough, wheezing, and stridor. Definitive management involves surgical excision. We herein report our experience of a bronchogenic cyst complicated by Mycobacterium tuberculosis infection in an 8-month-old infant. The patient underwent a right posterolateral thoracotomy, and an intrapulmonary bronchogenic cyst filled with purulent fluid was excised. The patient was given antituberculous medication, and the post-operative outcome was good.

The homeodomain transcription factor Ventx2 regulates respiratory progenitor cell number and differentiation timing during Xenopus lung development.

Ventx2 is an Antennapedia superfamily/NK-like subclass homeodomain transcription factor best known for its roles in the regulation of early dorsoventral patterning during Xenopus gastrulation and in the maintenance of neural crest multipotency. In this work we characterize the spatiotemporal expression pattern of ventx2 in progenitor cells of the Xenopus respiratory system epithelium. We find that ventx2 is directly induced by BMP signaling in the ventral foregut prior to nkx2-1, the earliest epithelial marker of the respiratory lineage. Functional studies demonstrate that Ventx2 regulates the number of Nkx2-1/Sox9+ respiratory progenitor cells induced during foregut development, the timing and level of surfactant protein gene expression, and proper tracheal-esophageal separation. Our data suggest that Ventx2 regulates the balance of respiratory progenitor cell expansion and differentiation. While the ventx gene family has been lost from the mouse genome during evolution, humans have retained a ventx2-like gene (VENTX). Finally, we discuss how our findings might suggest a possible function of VENTX in human respiratory progenitor cells.

Intra-abdominal ectopic bronchogenic cyst with a mucinous neoplasm harboring a GNAS mutation: A case report

BACKGROUNDBronchogenic cysts are congenital cysts caused by abnormal sprouting from the ventral foregut during fetal life. They usually occur in the mediastinum or lung, but there are very rare cases of ectopic bronchogenic cysts that develop in the abdominal cavity. A unique intra-abdominal ectopic bronchogenic cyst with a mucinous neoplasm that was producing carcinoembryonic antigen (CEA), harboring a GNAS mutation, is reported. The present case may contribute to clarifying the mechanism of tumorigenesis and malignant transformation of ectopic bronchogenic cysts.CASE SUMMARYIn 2007, a man in his 50s was incidentally found to have an intra-abdominal cystic mass, 8 cm in diameter. Surgical resection was recommended, but he preferred to remain under observation. In 2020, his serum CEA level increased to 26.7 ng/mL, and abdominal computed tomography showed a 15 cm × 12 cm, multifocal, cystic mass located predominantly on the lesser curvature of the stomach. Since malignancy could not be ruled out, he finally underwent surgical resection. Histologically, the cystic wall was lined by ciliated columnar epithelium, accompanied by bronchial gland-like tissue, bronchial cartilage, and smooth muscle. Part of the cyst consisted of atypical columnar epithelium with an MIB-1 index of 5% and positive for CEA. Moreover, a GNAS mutation (p.R201C) was detected in the atypical epithelium, leading to a diagnosis of an ectopic bronchogenic cyst with a low-grade mucinous neoplasm. The patient is currently undergoing outpatient follow-up without recurrence.CONCLUSIONAn extremely rare case of an abdominal bronchogenic cyst with a low-grade mucinous neoplasm harboring a GNAS mutation was reported.

January 2022 medical image of the month: bronchial obstruction due to pledget in airway following foregut cyst resection

No abstract available. Article truncated after 150 words. A 37-year-old woman complaining of chest pain and cough underwent resection of a mediastinal foregut duplication cyst complicated by a 10-day hospitalization with a prolonged air leak. Seven years later, she presented with worsening cough and shortness of breath, complaining of similar symptoms intermittently in the 7 years between her surgery and presentation. Chest CT showed a hyperattenuating lesion obstructing the medial basal segmental airways, with bronchoscopy revealing suture and a pledget obstructing the medial basal segmental right lower lobe bronchus. The pledget and suture were successfully removed. Repeat bronchoscopy several months later showed no residual airway foreign body, although medial basal subsegmental bronchial stenosis prevented advancement of the bronchoscope distally; this finding correlated with the CT impression of airway stenosis or occlusion in this region on the follow up CT. Bronchogenic cysts result from abnormal lung budding and development of the ventral foregut during the first trimester (1). Many …

Ectopic bronchogenic cyst arising from the diaphragm: a rare case report and literature review

BackgroundBronchogenic cysts can be caused by errors in the growth of the ventral foregut. Localization of the bronchogenic cyst (BC) varies depending on the level of the abnormal budding. They are usually located in the lungs and mediastinum. BCs of the diaphragm are a rare form of this abnormality.Case presentationA 66-year-old woman coughs and expectorates. CT scan evaluation revealed a soft tissue shadow of 6 × 5 cm in the left lung. Under thoracoscopic surgery, we found that the mass originated from the diaphragm away from the lung tissue, we completely removed the mass and the pathological result was diagnosed as BC.ConclusionsThe prognosis of ectopic BC is usually optimistic for benign tumors, as long as the tumor is completely removed.

Molecular crosstalk in tracheal development and its recurrence in adult tissue regeneration.

The trachea is a rigid air duct with some mobility, which comprises the upper region of the respiratory tract and delivers inhaled air to alveoli for gas exchange. During development, the tracheal primordium is first established at the ventral anterior foregut by interactions between the epithelium and mesenchyme through various signaling pathways, such as Wnt, Bmp, retinoic acid, Shh, and Fgf, and then segregates from digestive organs. Abnormalities in this crosstalk result in lethal congenital diseases, such as tracheal agenesis. Interestingly, these molecular mechanisms also play roles in tissue regeneration in adulthood, although it remains less understood compared with their roles in embryonic development. In this review, we discuss cellular and molecular mechanisms of trachea development that regulate the morphogenesis of this simple tubular structure and identities of individual differentiated cells. We also discuss how the facultative regeneration capacity of the epithelium is established during development and maintained in adulthood.

Presternal bronchogenic cyst: A rare presentation

Among the various congenital anomalies of the respiratory system, the extrapulmonary bronchogenic cyst is a rare pathologic lesion. The bronchogenic cyst is a developmental anomaly, occurring due to malformation of ventral foregut during the first 6 weeks of the intrauterine life. The cystic abnormality presents in infancy, children, and often in young adults. A case of 27-year-old male presented with subcutaneous swelling in the presternal area. The nodular swelling was excised and subsequently sent for surgical pathology examination. The cystic swelling was diagnosed on histopathology as a cutaneous bronchogenic cyst.

PS01.090: LAPAROSCOPIC EXCISION OF A PARAESOPHAGEAL BRONCHOGENIC CYST IN A 9-YEAR-OLD MALE

Abstract Background Bronchogenic cyst is a malformation of the ventral foregut. It is ofen asymptomatic and discovered as an incidental finding. In children, it comprises approximately 6% of all mediastinal masses. However, paraesophageal bronchogenic cyst is a rare and has been reported only three cases in children. We report a case of a 9-year-old male with an asymptomatic paraesophageal bronchogenic cyst. Methods Retrospective chart review. Results A 9-year-old male was detected to have retrocardiac mass lesion (2.7 × 1.9 × 1.2 cm), which was detected incidentally on an abdominal computed tomography done for acute appendicitis. No enhancement was noticed after contrast administration. Magnetic resonance imaging revealed a well-circumscribed cyst adjacent to the esophagus just above the hiatus. T1-weighted image showed a low intensity mass and the T2-weighted image showed a high intensity mass. Endoscopic ultrasonography examination of the esophagus demonstrated a cystic mass, with low-echo density. The echo also showed disruption of the fourth layer of the esophageal wall. With these findings, esophageal duplication was suspected. A laparoscopic operation was performed via 4 ports plus one puncture. 5mm laparoscope (30°angle) was inserted in the umbilical port, three 5mm trocars were placed and 3mm grasping forceps was inserted. The cyst was identified in the intra-abdominal esophageal part. Lesion removal was performed with a coagulator hook. A complete excision of the cyst was achieved without perforating of the esophagus. The postoperative courses was uneventful with home discharging on day six. The pathological diagnosis was a bronchogenic cyst because of finding a ciliated respiratory epithelium and cartilage and mucus-secreting glands. Conclusion Paraesophageal bronchogenic cyst is rare. However, it should be considered in the differential diagnoses for mediastinal masses at any age. Laparoscopy is a safe and effective method to deal with masses of posterior mediastinum even in children. Disclosure All authors have declared no conflicts of interest.

Intradiaphragmatic Bronchogenic Cysts: A Case Report and Literature Review

Bronchogenic cysts are a congenital developmental abnormality arising from the ventral foregut and are found most commonly in the mediastinum. However, several case reports indicated that bronchogenic cysts occur rarely within the diaphragm. Mubang et al. reported a case of intradiaphragmatic bronchogenic cyst and summarized 21 cases previously reported in the English literature. Most cases had several clinical symptoms, including back and/or chest pain, cough, etc. Here, we report a case of intradiaphragmatic bronchogenic cyst detected incidentally on chest computed tomography (CT) in an adult male patient without any clinical symptoms.

Formation and reduction of provisional ventral foregut diverticulum in the embryos of Anguilliformes as the recapitulation of lungs

We described the development and subsequent reduction of ventral foregut diverticulum in the early ontogenesis of Anguilliformes in plankton samples collected in the coastal waters of central Vietnam. The diverticulum is a provisional structure derived from the ventral-lateral area of the caudal esophagus. It develops as a protrusion from the esophagus wall and consists of one cell layer. An increase in the volume of the diverticulum results from the proliferation of these cells and transformation of their shape. It was found that the development of the ventral diverticulum in eels is similar to the early stages of lungs formation in other vertebrates. The formation of ventral diverticulum of the esophagus was recorded in all studied embryos. The time and the extent of its development differs between species and can serve as an additional morphological character in the taxonomic identification of eels at early stages of their ontogeny.

Osr1 functions downstream of Hedgehog pathway to regulate foregut development

During early fetal development, paracrine Hedgehog (HH) ligands secreted from the foregut epithelium activate Gli transcription factors in the surrounding mesenchyme to coordinate formation of the respiratory system, digestive track and the cardiovascular network. Although disruptions to this process can lead to devastating congenital defects, the underlying mechanisms and downstream targets, are poorly understood. We show that the zinc finger transcription factor Osr1 is a novel HH target as Osr1 expression in the foregut mesenchyme depends on HH signaling and the effector of HH pathway Gli3 binds to a conserved genomic loci near Osr1 promoter region. Molecular analysis of mouse germline Osr1 mutants reveals multiple functions of Osr1 during foregut development. Osr1 mutants exhibit fewer lung progenitors in the ventral foregut. Osr is then required for the proper branching of the primary lung buds, with mutants exhibiting miss-located lung lobes. Finally, Osr1 is essential for proper mesenchymal differentiation including pulmonary arteries, esophageal and tracheal smooth muscle as well as tracheal cartilage rings. Tissue specific conditional knockouts in combination with lineage tracing indicate that Osr1 is required cell autonomously in the foregut mesenchyme. We conclude that Osr1 is a novel downstream target of HH pathway, required for lung specification, branching morphogenesis and foregut mesenchymal differentiation.

The gastropod foregut — evolution viewed through a developmental lens

Comparative data on the developing gastropod foregut suggest that this multicomponent feeding complex consists of two developmental modules. Modularity is revealed by delayed development of the buccal cavity and radular sac (“ventral module”) relative to the dorsal food channel (“dorsal module”) in gastropods with feeding larvae compared with those that may have never had a feeding larval stage. If nonfeeding larvae like those of extant patellogastropods and vetigastropods are ancestral for gastropods, then the uncoupling and heterochronic offset of dorsal and ventral foregut modules allowed the post-metamorphic dorsal food channel to be co-opted as a simple but functional esophagus for feeding larvae. Furthermore, by reducing energy cost per ovum, the heterochronic offset may have given mothers the evolutionary option of increasing fecundity or investing in protective egg encapsulation material. A second developmental innovation was spatial separation of the dorsal and ventral foregut modules, as illustrated by distal foregut development in buccinid neogastropods and venom gland development in cone snails. Spatial uncoupling may have enhanced the evolvability of gastropod foreguts by allowing phenotypic variants of ventral module components to be selected within post-metamorphic ecological settings, without needing to be first tested for compatibility with larval feeding. Finally, we describe a case in which foregut modularity has helped facilitate a highly derived life history in which encapsulated embryos ingest nurse eggs.

Intrapulmonary bronchogenic cyst

Bronchogenic cysts are congenital anomaly caused by abnormal bronchial development from the primitive ventral foregut, which arises from cells isolated from the main pulmonary branching when lung bud separates from the primitive gut. We are reporting a case of 14-year-old boy who presented with nonspecific chest pain and cough.

A Retinoic Acid-Hedgehog Cascade Coordinates Mesoderm-Inducing Signals and Endoderm Competence during Lung Specification

Organogenesis of the trachea and lungs requires a complex series of mesoderm-endoderm interactions mediated by WNT, BMP, retinoic acid (RA), and hedgehog (Hh), but how these pathways interact ina gene regulatory network is less clear. Using Xenopus embryology, mouse genetics, and human ES cell cultures, we identified a conserved signaling cascade that initiates respiratory lineage specification. We show that RA has multiple roles; first RA pre-patterns the lateral plate mesoderm and then itpromotes Hh ligand expression in the foregut endoderm. Hh subsequently signals back to the pre-patterned mesoderm to promote expression of the lung-inducing ligands Wnt2/2b and Bmp4. Finally, RA regulates the competence of the endoderm to activate the Nkx2-1+ respiratory program in response to these mesodermal WNT and BMP signals. These data provide insights into early lung development and a paradigm for how mesenchymal signals are coordinated with epithelial competence during organogenesis.

Differentiation of Mouse Embryonic Stem Cells into Ventral Foregut Precursors.

Anterior definitive endoderm (ADE), the ventral foregut precursor, is both an important embryonic signaling center and a unique multipotent precursor of liver, pancreas, and other organs. Here, a method is described for the differentiation of mouse embryonic stem cells (mESCs) to definitive endoderm with pronounced anterior character. ADE-containing cultures can be produced in vitro by suspension (embryoid body) culture or in a serum-free adherent monolayer culture. ESC-derived ADE cells are committed to endodermal fates and can undergo further differentiation in vitro towards ventral foregut derivatives.

Lung endoderm morphogenesis: gasping for form and function.

The respiratory endoderm develops from a small cluster of cells located on the ventral anterior foregut. This population of progenitors generates the myriad epithelial lineages required for proper lung function in adults through a complex and delicately balanced series of developmental events controlled by many critical signaling and transcription factor pathways. In the past decade, understanding of this process has grown enormously, helped in part by cell lineage fate analysis and deep sequencing of the transcriptomes of various progenitors and differentiated cell types. This review explores how these new techniques, coupled with more traditional approaches, have provided a detailed picture of development of the epithelial lineages in the lung and insight into how aberrant development can lead to lung disease.

Bronchogenic Cyst in the Liver Mimicking Metastasis of Colorectal Carcinoma: A Case Report and Review

Introduction: Carcinomas of the colon and rectum are the third most common cancer entity in the world and bear a high risk of synchronous (25%) or metachronous (50%) hepatic tumor seeding. For therapeutic decisions the differential diagnosis between benign and malignant hepatic lesions in the computer tomography scan is of major importance. We herein discuss congenital hepatic cysts derived from the primitive foregut as potential differential diagnosis for hepatic metastases from a colorectal primary tumor. Case presentation: A caucasian, 56-year old female patient with the initial diagnosis of an adenocarcinoma of the rectum had initially been treated by an anterior rectum resection and hemihepatectomy due to synchronous hepatic metastases (pT3 pN1(2/13), G2, pM1(HEP), L1, V0, pR0) in a curative intention. The follow-up staging after 2 years showed recurrent liver metastases. A local resection of the hepatic foci was performed. One of three lesions was classified as a classical ventral foregut derived bronchogenic cyst located subcapsularly in the liver. A follow-up resection of pulmonary metastases was performed 13 months later. The patient is still alive and healthy 68 months after the primary operation. Conclusions: For the diagnosis and treatment of hepatic metastases of colorectal carcinomas it is very useful to know potential differential diagnoses in radiographic imaging. Foregut derived cysts can be histologically subclassified into Ciliated Hepatic Foregut Cysts and Bronchogenic Cysts. While Ciliated Hepatic Foregut Cysts are known to be located in the liver, we present the rare case of a Bronchogenic Cyst, which was located in the liver parenchyma.