Foregut Cyst Research Articles

Assessment of the spectrum of enteric duplication cysts and their varied presentations

Enteric duplication cysts having an incidence of 1 in 4500 live births, are not often diagnosed antenatally. Patients who are eventually diagnosed, have a varying presentation, clinically and demographically, demographically and clinically. Classified as cystic and tubular, with the latter being radiologically difficult to detect on ultrasound. Differential diagnoses of mesenteric cysts and omental cysts. This prospective study was done over a two-year period, where patients who were diagnosed with enteric duplication cysts at Sir Padampat Institute of Neonatology and Child Institute, Jaipur were included. They were monitored from the time of diagnosis, assessing demographic data, preoperative status, surgical management, post operative treatment and follow up after discharge. Total14 patients were included, of which a majority were tubular variants of ileal duplication cyst. 3 patients had foregut duplication cysts (1 gastric, 2 oesophageal). The age ranged from 3 days to 10 years, with the average age of 2.2 years. Each child had a varying presentation, some of them presenting with complications like obstruction and perforation. The incidence was more in male, as compared to females (3.6:1 – male:female). Two children had associated anomalies (one had a congenital diaphragmatic hernia, and one had malrotation of the gut). In one child, a foregut duplication cyst was found to be communicating with a dilated segment of the jejunum, which was resected together. Enteric duplication cysts, can have a myriad of presentations, and other associated congenital anomalies. Further studies are required to identify attributing risk factors, regional incidence along with better awareness for antenatal screening.

Ciliated foregut cysts involving the hepatopancreaticobiliary system: a clinicopathological evaluation with focus on atypical features

AimsForegut cystic malformations are rare developmental abnormalities, which may involve the hepatopancreaticobiliary tract (HPBT). These cysts are composed of inner ciliated epithelium; subepithelial connective tissue layer; smooth muscle layer; and...

Neonatal Outcomes of Multiple Congenital Thoracic Lesions

Introduction: Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort. Methods: Retrospective chart review of patients born between September 1, 2013, and March 31, 2023, who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics. Results: Among 701 CTL patients, 74 (10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8 (12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n = 33, 44%). Bilateral CTL were found in 9 (12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n = 36, 49%). Genetic syndromes were confirmed in 3 (4%) and additional congenital anomalies in 9 (12.9%) patients, 5 of those had multiple congenital anomalies. Of 49 (65%) patients with multilobar CTL, 25 (51%) underwent bilobectomy and 24 (49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%. Conclusion: We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.

Variations in Epithelial Lining of Duplication Cysts Cannot be Explained by Known Theories.

Objectives. Duplication cysts are found in any part of the gastrointestinal tract from the oropharynx to anus. Although duplication cysts usually have similar epithelium with the adjacent organ, respiratory epithelium in the enteric duplication cysts is rarely reported. This study was performed to evaluate the variations in the epithelial lining of duplication cysts and its clinical implications. Methods. Patients diagnosed with duplication cysts between 2012 and 2022 were retrospectively reviewed to assess their histopathological results, clinical aspects, treatment options, and demographic characteristics. Results. Twenty-five patients were included. The mean age was 4 years, male-to-female ratio was 15:10. The localizations of duplication cysts were ileum (n = 12), duodenum(n = 4), stomach(n = 3), jejunum(n = 2), colon(n = 2), thoracoabdominal(n = 2), and rectum(n = 1). Most common presentation was abdominal pain (36%). Intestinal (48%) and gastric (40%) epithelia were the most common finding. Four patients (16%) had respiratory epithelium: two in foregut duplication cysts, one each in ileal, and rectal duplications. Twenty-four percentage of patients had associated anomalies. Surgical interventions were performed on 22 patients. Conclusion. Duplication cysts show a wide range of epithelial linings. Respiratory epithelium can be found in not only foregut duplication cysts but also midgut and hindgut duplication cysts. Although the presence of respiratory epithelium did not have any impact on clinical findings, none of the previous theories explain the presence of respiratory epithelium different from foregut duplications.

Video assisted thoracoscopy surgery a viable alternative for duplication cyst excision: a case report on foregut duplication cyst

Alimentary tract duplication is a relatively uncommon congenital abnormality. It can be found anywhere from the mouth to the anus and can be symptomatic or undetectable. Although congenital duplication can happen anywhere in the gastrointestinal tract, the ileum, oesophagus, and colon are the most common locations. The foregut, which includes the stomach, and first and second segments of the duodenum, is the source of one-third of all duplications. When foregut duplication involves the bronchial tree, respiratory symptoms are often present. Furthermore, excision through surgery is required for pathological examination of the resected specimen for definitive diagnosis. The most promising surgical approach for cyst excision has been thought to be open surgical resection through a posterolateral thoracotomy incision. However, video assisted thoracoscopy surgery has recently emerged as a viable surgical option for duplication cyst removal In paediatric surgery, video assisted thoracoscopy surgery has been established to facilitate the removal of mediastinal masses. However there is a limitation of research in the literature regarding the best surgical technique for oesophageal duplication cyst excision. In girls, foregut duplication is more common, especially in cases when bronchopulmonary involvement is present. While many duplications result in issues in early development, some are discovered by accident. When foregut duplication involves the bronchial tree, respiratory symptoms are often present. Haemoptysis and respiratory distress may be present in specific cases of the patient. Here, we described a 5-year-old male infant's case of foregut duplication with bronchial involvement and its management.

Laparoscopic resection of rare foregut duplication cyst lined by pseudostratified columnar epithelium in a pediatric patient

Laparoscopic resection of rare foregut duplication cyst lined by pseudostratified columnar epithelium in a pediatric patient

A rare cause of dysphagia due to retropharyngeal foregut duplication cyst: case report and review of the literature

BackgroundThe retropharyngeal space is a deep compartment of the head and neck region which extends from the base of the skull to the mediastinum, between the posterior pharyngeal wall and vertebral muscles, delimited laterally by carotid sheaths. Pathological processes of the retropharyngeal space are rarely encountered, generally are isolated and painless masses often cystic, and they usually originate from branchial arch anomalies, but only in rare cases, they turn out to be foregut duplication cysts. Foregut duplication cyst is rare congenital malformations arising along primitively derived alimentary tract during the first trimester in the developing embryo, mostly seen in the thorax and abdomen, with just few cases reported in the head and neck region. We report an extremely rare case of a foregut duplication cyst lined with respiratory epithelium located in the retropharyngeal space, at the level of the oropharynx, of an adult patient with dysphagia surgically treated, and we also made an analysis of the published literature about this very uncommon condition.Case presentationA 63-year-old male patient with chronic dysphagia was diagnosed with a retropharyngeal cystic lesion, which was surgically treated. Final pathologic evaluation confirmed the diagnosis of a rare foregut duplication cyst lined with respiratory epithelium. A review of the pertaining published literature about the head and neck foregut duplication cysts was made, with a particular emphasis on retropharyngeal ones.ConclusionsRetropharyngeal foregut duplication cysts are a very rare congenital cyst of the head and neck. Clinical symptoms such as dysphagia and dyspnea should be evaluated with fibrolaryngoscopy, and CT and MRI scans are of great significance for definitive diagnosis, which should include the possibility of a foregut duplication cyst in the differential diagnosis. Surgical excision is the elective treatment for this lesion, in order to prevent complications including infection and compression symptoms or eventually malignant transformation.

RARE FOREGUT CYST IN ORAL CAVITY

Foregut cysts are rare congenital choristomas arising from abnormal development of the foregut. Choristomas may comprise several different tissue types, including salivary glands (within the jawbone or gingival tissues), cartilaginous or bony lesions (mostly on the tongue), glial tissue (most common in the nasosinusal tract), or thyroid tissue. The aim of this report is to describe the unusual case of a neonate (2 months old) with a large congenital sublingual cystic lesion preventing breastfeeding, who was initially treated as a sublingual ranula using cryosurgery without success. An incisional biopsy was performed, which diagnosed a foregut cyst. The patient was referred to the Head and Neck Surgery team for enucleation, which resolved the lesion. The patient is under follow-up with no evidence of recurrence in six months. Foregut cysts are rare congenital choristomas arising from abnormal development of the foregut. Choristomas may comprise several different tissue types, including salivary glands (within the jawbone or gingival tissues), cartilaginous or bony lesions (mostly on the tongue), glial tissue (most common in the nasosinusal tract), or thyroid tissue. The aim of this report is to describe the unusual case of a neonate (2 months old) with a large congenital sublingual cystic lesion preventing breastfeeding, who was initially treated as a sublingual ranula using cryosurgery without success. An incisional biopsy was performed, which diagnosed a foregut cyst. The patient was referred to the Head and Neck Surgery team for enucleation, which resolved the lesion. The patient is under follow-up with no evidence of recurrence in six months.

Sperm protein antigen 17 and Sperm flagellar 1 cancer testis antigens are expressed in a rare case of ciliated foregut cyst of the common hepatic duct

Ciliated foregut cysts (CFCs) are frequently described in liver, pancreas and gallbladder and generally considered benign although one case of squamous cell metaplasia and five cases of squamous cell carcinoma arising from a ciliated hepatic foregut cyst have been reported. Here we explore two cancer-testis antigens (CTAs), Sperm protein antigen 17 (SPA17) and Sperm flagellar 1 (SPEF1) expression in a rare case of CFC of the common hepatic duct MATERIALS AND METHODS: 3µm-thick CFC sections were immunohistochemically treated with antibodies raised against human SPA17 or SPEF1. In silico Protein-Protein Interaction (PPI) network and differential protein expression were also investigated RESULTS: Immunohistochemistry revealed SPA17 and SPEF1 in the cytoplasm of ciliated epithelium. SPA17, but not SPEF1, was also detected in cilia. The PPI networks demonstrated that other CTAs are significantly predicted functional partners with SPA17 and SPEF1. The differential protein expression demonstrated that SPA17 was higher in breast cancer, cholangiocarcinoma, liver hepatocellular carcinoma, uterine corpus endometrial carcinoma, gastric adenocarcinoma, cervical squamous cell carcinoma, bladder urothelial carcinoma. SPEF1 expression was higher in breast cancer, cholangiocarcinoma, uterine corpus endometrial carcinoma and kidney renal papillary cell carcinoma CONCLUSIONS: Our study suggests that further characterization of SPA17 and SPEF1 in patients with CFCs might provide significant insights to understand the mechanisms underlying their potential to malignant transformation.

Aortic Wall Abrasion Caused by Needle Injury after Endoscopic Ultrasound-Guided Fine Needle Aspiration of a Mediastinal Hemangioma

Benign mediastinal cysts are challenging to diagnose. Although Endoscopic Ultrasound (EUS) and EUS-guided fine needle aspiration (FNA) can accurately diagnose mediastinal foregut cysts, little is known about their complications. This paper reports a rare case in which EUS-FNA performed on mediastinal hemangioma resulted in an aortic hematoma. A 29-year-old female patient was commissioned for EUS of an asymptomatic accidental mediastinal lesion. Chest CT revealed a 4.9×2.9×10.1 cm thin-walled cystic mass in the posterior mediastinum. EUS revealed a large, anechoic cystic lesion with a regular thin wall with negative Doppler. EUS-guided FNA was performed using a single-use 19-gauge aspiration needle (EZ Shot 3; Olympus, Tokyo, Japan), and approximately 70 cc of serous pinkish fluid was aspirated. The patient was in a stable condition with no signs of acute complication. One day after EUS-FNA, thoracoscopic resection for mediastinal mass was conducted. The purple and multi-loculated large cyst was removed. Upon removal, however, an aortic hematoma caused by a focal descending aortic wall injury was observed. After a few days of close observation, the patient was discharged upon stable 3D aorta angio CT findings. This paper reports a rare and severe complication of EUS-FNA, in which an aspiration needle caused a direct injury to the aorta. The injection must be performed carefully to avoid damaging the adjacent organs or digestive tract walls.

A rare case of ciliated foregut cyst of gall bladder

A rare case of ciliated foregut cyst of gall bladder

Successful management of a large lingual foregut duplication cyst with an ex-utero intrapartum treatment procedure

Successful management of a large lingual foregut duplication cyst with an ex-utero intrapartum treatment procedure

Patent hepatic ciliated foregut remnant resulting in an umbilicobiliary sinus tract, with gallbladder agenesis, in an 8-wk-old male French Bulldog.

Hepatic ciliated foregut remnants or cysts are congenital abnormalities resulting from retention of embryonic ciliated foregut within the liver. These structures are rarely reported in the human medical literature and have not been reported in the veterinary literature previously, to our knowledge. We describe here a case of an 8-wk-old male French Bulldog with a congenital patent hepatic ciliated foregut remnant resulting in an umbilicobiliary sinus tract. The dog also had concurrent gallbladder agenesis. The patient had yellow fluid discharging from the umbilicus, mimicking a patent urachus. Surgical exploration, removal, and histology provided a conclusive diagnosis of a hepatic foregut remnant and therapeutic resolution of the clinical signs. The histologic appearance of a hepatic foregut remnant is classical, namely a duct composed of 4 layers: an inner ciliated epithelial lining, loose connective tissue, smooth muscle, and a fibrous capsule.

HPB P45 Ciliated Hepatic Foregut Cysts: Not as Rare as Previously Believed

Abstract Background Ciliated hepatic foregut cysts (CHFCs) are uncommon cystic lesions within the liver. CHFCs can undergo a malignant transformation to form a primary squamous cell carcinoma of the liver. The true incidence and natural history of CHFCs is unknown and the risk of malignant transformation is unclear. We present a single centre’ s experience of CFHC management. Methods A retrospective review of a departmental database identified all patients with CHFCs over a 4 year time period. Patients with CHFCs confirmed on histological assessment or suspected on radiological imaging were included in this study. Clinical information regarding patient demographics, symptomatic presentation, surgical management and histopathological features were noted. The radiological characteristics of CHFCs were recorded and the malignant transformation rate was calculated Results 15 patients with CHFC were identified (7 histologically confirmed and 8 radiologically suspected cases). All patients were asymptomatic and the CHFCs were incidental findings.No CHFC developed an interval change in cyst features or underwent a malignant transformation during follow up. MRI serves as the most sensitive modality to diagnose CHFC Conclusions CHFCs may be more prevalent than previously reported. Definitive management should encompass a patient centred discussion regarding the merits of long term follow up with serial imaging versus resection on an individual basis once CHFC is diagnosed.

An unusual case of extrapulmonary sequestration presenting as vomiting in a neonate

Pulmonary sequestration is a rare, mostly sporadic disorder, with an incidence of 0.1–6% of all congenital lung malformations. It is also called as bronchopulmonary foregut malformation (BPFM) or accessory lung. A 16-day-old female child presented with vomiting and inability to take feeds. She was investigated further and differentials included foregut duplication cyst and BPFM. Surgical exploration confirmed the diagnosis of a BPFM. Delay in diagnosis may lead to complications such as torsion and sepsis.

Multi-time point imaging in 99m Tc-pertechnetate scintigraphy of ectopic gastric mucosa in duplication cysts: value-added services.

The aim of our study was to describe the scintigraphic patterns of 99m Tc-pertechnetate uptake in patients who were referred to the department of nuclear medicine for evaluating and diagnosing ectopic gastric mucosa in foregut and midgut duplication cysts. This hospital-based, retrospective cum prospective research spans a period of 8 years from April 2014 to January 2022. Previous hospital medical records were analyzed and subsequently, a database was prepared which included the age, sex, clinical indication of a 99m Tc-pertechnetate scan, and the planar and SPECT-computed tomography (CT) imaging findings. Postoperative histopathological reports were available for 21 patients. Dynamic and planar static imaging was performed. We included SPECT-CT in suspected duplication cysts to increase the sensitivity and specificity which is a tradeoff for a small amount of additional radiation exposure. A total of 69 patients were subjected to a 99m Tc-pertechnetate scan for suspected foregut or midgut duplication cysts. All were subjected to dynamic planar and delayed static images up to 24 h or until focal uptake of radiotracer was noted which corroborated the anatomical findings, whichever was earlier. SPECT-CT was performed along with the planar study in 31 patients which confirmed the findings. Previously performed CT scans were used for anatomical correlation in the remaining ones. Duplication cysts were localized in a total of 28 patients (19 foregut duplication cysts and 12 small bowel duplications - 3 patients had dual duplication cysts, both foregut, and midgut). Forty-one patients had no scintigraphic evidence of ectopic gastric mucosa. Of these 69 patients, histopathological diagnosis was available for 21 patients (22 lesions). The report was concordant with the scan findings in 15 patients (16 lesions) and 6 patients showed discordance in histopathological diagnosis and scan findings. In conclusion, multi-time point imaging is the key to diagnosing ectopic gastric mucosa of various sizes and in various locations. An abnormal radiotracer uptake in dynamic sequences, even before the appearance of the stomach in the region of the small bowel is indicative of intestinal duplication, and delayed radiotracer visualization in the region of the thorax is characteristic of intrathoracic foregut duplication cyst.

Robotic-Assisted Thoracoscopic (RATS) Enucleation of Esophageal Mesenchymal Tumors and Foregut Cysts.

Background: Esophageal mesenchymal tumors and foregut cysts are mostly benign lesions of the esophagus. Tumor enucleation is recommended for lesions with a risk of malignancy, or for the relief of clinical symptoms. Although robotic-assisted thoracoscopic enucleation of esophageal tumors and cysts has been demonstrated in sporadic case reports, its clinical role is yet to be elucidated. Methods: This study aimed to present the first case series in the literature for the perioperative and long-term clinical outcomes of robotic-assisted thoracoscopic enucleation. Results: A total of 19 patients who underwent robotic-assisted thoracoscopic enucleation of esophageal tumors and cysts from 2012 to 2019 were included in the study. The mean tumor/cyst size was 5.5 cm (1.5-22 cm). There were two cases shifting to minimally invasive esophagectomy (10.5%) due to intraoperative pathological confirmation of malignant gastrointestinal stromal tumors with mucosal invasion. Perioperative complication was detected in three (15.8%) cases, without 30-day or surgical mortality. There was no recurrence of tumor or symptoms in all patients during the clinical follow-up period (mean = 35 months). Conclusions: Robotic-assisted thoracoscopic enucleation of esophageal submucosal benign tumors is technically feasible and effective. Given its advantage in overcoming spatial limitations, it can become a widely accepted surgical option for such diseases.

543. THORACO-LAPAROSCOPIC EXCISION OF A FOREGUT DUPLICATION CYST WITH A PYLORO-DUODENAL COMMUNICATION

Abstract Foregut duplication cysts presents rarely in adults. Rarely, they can have an intraluminal communication. Traditionally, these cysts are excised via open surgical approaches. This video case describes the Thoraco-laparoscopic management of a 34-year old female patient who was diagnosed with foregut duplication cyst with a pyloro-duodenal communication. Successful excision of the foregut duplication cyst was achieved, through minimally invasive technique. Postoperatively, a right-sided pleural collection resulted, which was successfully managed with image-guided drainage. She is currently alive and asymptomatic at 1-year follow-up. Minimally invasive surgery is feasible and safe in the management of foregut duplication cysts with luminal communication.

Pediatric Hepatic Cystic Lesions: Differential Diagnosis and Multimodality Imaging Approach.

When a pediatric hepatic cystic lesion is identified at imaging, the differential diagnosis may be broad, including developmental, infectious, neoplastic, and posttraumatic or iatrogenic causes. The location of a cystic lesion and its number, size, composition, and relationship to the biliary system are features that help in narrowing the differential diagnosis. An incidentally detected simple hepatic cyst is the most commonly encountered. Ciliated foregut cysts are typically located in hepatic segment IVa. The presence of multiple cysts should raise suspicion for fibropolycystic liver disease, a group of related lesions-including biliary hamartoma and choledochal cyst-caused by abnormal embryologic development of the ductal plate. Communication of the cystic lesion with the biliary tree can confirm the diagnosis of choledochal cyst. In a neonate with jaundice, a cystic lesion at the porta hepatis should raise suspicion for choledochal cyst versus cystic biliary atresia. Hepatic abscess can appear cystlike, though typically with internal contents. In an immunocompromised child, multiple cystlike lesions should raise concern for fungal microabscesses. A complex cystic mass in a young child should raise suspicion for mesenchymal hamartoma, which can evolve into undifferentiated embryonal sarcoma if untreated. Hepatic hematoma and biloma can appear cystlike in children with a history of trauma or recent intervention. In neonates with an umbilical vein catheter (UVC), an intrahepatic cyst along the course of the UVC should raise concern for infusate extravasation. Familiarity with imaging findings and clinical features is essential for achieving accurate diagnosis of pediatric hepatic cystic lesions, which in turn can guide appropriate clinical management. Online supplemental material is available for this article. ©RSNA, 2022.

Hepatic Cysts: Reappraisal of the Classification, Terminology, Differential Diagnosis, and Clinicopathologic Characteristics in 258 Cases.

The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1cm cysts evaluated pathologically using updated criteria were classifiable as: I. Ductal plate malformation related (63%); that is, cystic bile duct hamartoma or not otherwise specified-type benign biliary cyst (35 with polycystic liver disease). These were female predominant (F/M=2.4), large (10cm), often multifocal with degenerative/inflammatory changes and frequently misclassified as "hepatobiliary cystadenoma." II. Neoplastic (13%); 27 (10.5%) had ovarian-type stroma (OTS) and qualified as mucinous cystic neoplasm (MCN) per World Health Organization (WHO). These were female, solitary, mean age 52, mean size 11cm, and 2 were associated with carcinoma (1 in situ and 1 microinvasive). There were 3 intraductal papillary neoplasms, 1 intraductal oncocytic papillary neoplasm, 1 cystic cholangiocarcinoma, and 2 cystic metastasis. III. Infectious/inflammatory (12%). These included 23 hydatid cysts (including 2 Echinococcus alveolaris both misdiagnosed preoperatively as cancer), nonspecific inflammatory cysts (abscesses, inflammatory cysts: 3.4%). IV. Congenital (7%). Mostly small (<3cm); choledochal cyst (5%), foregut cyst (2%). V. Miscellaneous (4%). In conclusion, hepatic cysts occur predominantly in women (3/1), are mostly (90%) non-neoplastic, and seldom (<2%) malignant. Cystic bile duct hamartomas and their relative not otherwise specified-type benign biliary cysts are frequently multifocal and often misdiagnosed as "cystadenoma/carcinoma." Defined by OTS, MCNs (the true "hepatobiliary cystadenoma/carcinoma") are solitary, constitute only 10.5% of hepatic cysts, and have a significantly different profile than the impression in the literature in that essentially all are perimenopausal females, and rarely associated with carcinoma (7%). Since MCNs can only be diagnosed by demonstration of OTS through complete microscopic examination, it is advisable to avoid the term "cystadenoma/cystadenocarcinoma" solely based on radiologic examination, and the following simplified terminology would be preferable in preoperative evaluation to avoid conflicts with the final pathologic diagnosis: (1) noncomplex (favor benign), (2) complex (in 3 subsets, as favor benign, cannot rule out malignancy, or favor malignancy), (3) malignant features.