Multiple thoracic and abdominal foregut duplication cysts: A case report.

BACKGROUND: Congenital enteric duplication cysts are tubular or cystic structures that normally lie alongside the gastrointestinal (GI) tract. Enteric duplication cysts are typically solitary lesions that occur anywhere near the GI tract from the neck to the rectum, but having multiple duplication cysts is rare, and presentation within the pancreas is extremely rare. CASE SUMMARY: We herein demonstrate a case of esophageal, gastric, and gastric-type duplication cyst of the pancreas in a seventeen-month-old girl who presented with failure to thrive, abdominal pain, vomiting, hematemesis, and melena since the age of three months. The cysts were excised by thoracoscopy and laparoscopy in the same setting. To our knowledge, no such case has been published. CONCLUSION: Enteric duplications can occur throughout the entire alimentary tract. When they occur in the pancreas, they present a formidable challenge in both diagnosis and treatment. Due to the risk of complications and malignant transformation, surgical removal is the recommended treatment of all duplication cysts.

Thoracoabdominal duplication accompanied by intestinal malrotation: a case report and literature review of a rare congenital anomaly in an infant.

Introduction and importance: Thoracoabdominal duplication and intestinal malrotation are extremely rare congenital alimentary tract anomalies that can manifest in any segment of the gastrointestinal tract. Still, tubular duplications are an even rarer subset of alimentary tract duplications. Misdiagnosis could occur and this will yield devastating ramifications. Therefore, consideration in the clinical settings is warranted to aid in conducting timely therapeutic interventions. Case presentation: In this article, we illustrate the overwhelmingly rare occurrence of thoracoabdominal duplication coexistent with intestinal malrotation in a 7-month-old male whose primary complaint was chronic dyspnoea since birth that progressed to involve cough and fever. Imaging analysis revealed a significant intrathoracic fluid-filled cyst. Clinical discussion: The intestinal malrotation was treated through Ladd's procedure, and surgical excision of the duplicated segments was accomplished. The subsequent analysis of the resected specimens via means of histopathology utilizing Hematoxylin and Eosin dyes established the definitive diagnosis of a foregut duplication cyst. Conclusion: Thoracoabdominal duplication is one of the most crucial topics in the field of Paediatric Surgery. It is exceptionally rare in occurrence, and the scarcity of available resources that document and describe this topic is evident in the published literature. The authors must opt to document, study, and broaden awareness regarding this life-threatening pathology so that they can circumvent the resultant complications by means of early detection and the performance of apt surgical interventions. Upon careful review of the available literature, we can state that ours is the first-ever case documented from their country regarding this topic and this co-incidence.

Mixed Heterotopic Gastrointestinal/Respiratory Oral Cysts in Newborns: From Prenatal Diagnosis to Histopathological and Therapeutic Management: A Case Report and Literature Review.

Fetal lingual tumors are very rare, and their early prenatal diagnosis is important for defining the subsequent therapeutic strategy. In this study, we aimed to describe a case of a congenital septate lingual cyst and perform an extensive literature review on two main databases (PubMed, Web of Science), analyzing the clinical manifestations, the imaging appearance, the differential diagnosis, and particularities regarding the treatment of these tumors. The electronic search revealed 17 articles with 18 cases of mixed heterotopic gastrointestinal/respiratory oral epithelial cysts that met the eligibility criteria and were included in this review. The clinical case was diagnosed prenatally during second-trimester screening. On the eighth day of life, the fetus underwent an MRI of the head, which revealed an expansive cystic process on the ventral side of the tongue with the greatest diameter of 21.7 mm, containing a septum of 1 mm inside. On the 13th day of life, surgery was performed under general anesthesia, and the lingual cystic formation was completely excised. The postoperative evolution was favorable. The histopathological examination revealed a heterotopic gastric/respiratory-mixed epithelial cyst with non-keratinized respiratory, gastric squamous, and foveolar epithelium. The lingual cyst diagnosed prenatally is an accidental discovery, the differential diagnosis of which can include several pathologies with different degrees of severity but with a generally good prognosis.

Retroperitoneal Foregut Duplication Cyst Containing Gastric, Respiratory, and Pancreatic Tissue Misinterpreted as an Adrenal Mass on Imaging: A Case Report and Review of the Literature.

Foregut duplication cyst is most commonly seen in the posterior mediastinum without communication with adjacent organs or presence of other malformations and typically shows ectopic gastric or respiratory epithelium. The finding of ectopic pancreatic tissue is extremely rare. A 15-year-old female patient presented with elevated blood pressure, intermittent right flank pain for 18 months. An abdominal MRI revealed a 4 cm right cystic retroperitoneal mass, initially thought to arise from the right adrenal gland. Intraoperatively, the mass was centered in the retroperitoneum between the right adrenal gland and kidney, without attachments to these organs and no communication with other structures, and it was completely excised. Gross examination revealed a 3.5 cm well-circumscribed solid and cystic mass with orange-white cut surface and cloudy fluid surrounded by variable amounts of adipose tissue. On microscopic examination, the cysts were lined by respiratory and gastric antral/oxyntic-type mucosa, surrounded by muscularis mucosae, submucosal tissue, muscularis propria, and perienteric tissue. Within the muscular propria, exocrine pancreatic tissue was also noted. The patient is well with 7-months of follow up. This case is the first one reporting gastric, respiratory, and pancreatic epithelia.

Imaging Findings of Adenocarcinoma Arising from Lingual Foregut Duplication Cyst.

Lingual foregut duplication cysts are rare developmental anomalies with varied symptoms, and adenocarcinomas may develop from them. Radiological evaluations, including computed tomography and magnetic resonance imaging, can help diagnose these lesions and determine their extent. Abnormal enhancement should raise the suspicion of malignancy of a lingual foregut duplication cyst. Early detection and complete surgical resection of lingual foregut duplication cysts are necessary to prevent malignant transformations. Complete tumor resection with adjuvant radiotherapy or radiochemotherapy is recommended. The teaching point is that mucinous adenocarcinoma can develop from a lingual foregut duplication cyst, and radiological studies may aid in the diagnosis. Teaching Point: Adenocarcinomas can develop from lingual foregut duplication cysts, which are rare developmental anomalies, and radiologic studies may aid in the diagnosis.

Successful thoracoscopic excision of complex supracardiac middle mediastinal bronchogenic cyst in an infant.

Introduction: We report the first case of complex supracardiac middle mediastinal foregut duplication lesion compressing and adherent to the heart, managed successfully thoracoscopically in an infant, in an innovative way. Materials and Surgical Technique: A 1-year-old girl was diagnosed as having supracardiac middle mediastinal foregut duplication lesion. It was completely overlying and adherent to her heart giving transmitted pulsations to the lesion, causing indentation over the left atrium and surrounded by all vital structures such as aorta, pulmonary artery, bronchi and phrenic nerve. After proper counselling of parents and relatives, the patient was posted for surgery. After proper positioning, thoracoscopic access was gained, difficulty here was neither bulge nor surface marking of the lesion was seen in thoracic cavity anywhere, considering the anatomical relations. The mediastinal pleura was opened, through a very narrow window for accessing the lesion, which was surrounded by vital structures in the middle mediastinum. A gentle dissection of the lesion was done to relieve it from adjacent adhered thoracic vitals structures successfully. Histopathological examination confirmed it as a bronchogenic cyst. Discussion: Foregut duplication cyst, especially bronchogenic cysts, have been reported at various locations, however, supracardiac middle mediastinal bronchogenic cyst completely sitting on the heart, adherent to it causing compression of the left atrium and left bronchus and surrounded completely by the aorta, pulmonary artery and bronchus, has not been reported till date. Also, successful thoracoscopic excision of such rarely located lesion moving with heart, in a 1-year-old girl, has not been reported yet.

Heterotopic gastrointestinal cyst within the submandibular space in an adult: A case report and review of the literature.

Heterotopic gastrointestinal cysts (HGIC) are rare congenital cysts that arise anywhere along the gastrointestinal tract. HGICs are infrequently reported in the oral cavity; 0.3% of HGICs are reported in the tongue and even more rarely in the submandibular space. Oral HCIGs are more common in children with only 13 reported cases in adults. In the present report, we discuss the differential diagnoses of the submandibular space lesions and describe a rare case of a very large submandibular space HGIC in an adult patient.

Thoracoscopic Resection of a Foregut Duplication Cyst with the Use of a 5-mm Stapling Device in an Infant-A Case Report.

Esophageal foregut duplication cysts are a rare congenital anomaly predominantly diagnosed in children. With possible growth foregut duplication cysts may cause compression on thoracic or mediastinal structures, respectively. Due to the presence of ectopic gastric mucosa and its potential malignant alteration resection of foregut duplication cysts is recommended. More recently, the use of a thoracoscopic approach for resection has shown to be an advantageous alternative to a conventional open approach. A case of a complete thoracoscopic resection of an esophageal foregut duplication cyst using a 5-mm stapling device is presented.

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.

Lingual Choristoma with Gastric Epithelium Revisited.

The term 'choristoma' refers to normal appearing tissue in an abnormal location. We describe a case of choristoma with gastric epithelium of the dorsal tongue in a pediatric patient. Lingual choristomas are uncommon cystic or solid lesions which may demonstrate different types of tissue (e.g. gastric epithelium, respiratory epithelium, osseous and neuroglial tissue) histologically. Choristomas with gastric epithelium, also known as heterotropic gastric mucosa or foregut duplication cysts, are thought to arise from pluripotential cells of the embryonic foregut. They most frequently involve the anterior two-thirds of the tongue. Most patients are asymptomatic, but larger lesions may lead to feeding and breathing difficulties. Pathologic evaluation and surgical excision remain the mainstay of diagnosis and treatment, respectively. The pathologic characteristics of other congenital tongue lesions are also discussed.

Multiple Esophageal Duplication Cysts in a Toddler: A Rare Scenario.

The foregut duplication cysts are rare congenital malformation and are usually "solitary" midline noncommunicating mediastinal structures. We present a 2-year-old asymptomatic boy having multiple noncommunicating foregut cysts in the neck as well as the thorax.

Thoracoscopic resection of foregut duplication cyst in a neonate.

Foregut duplication is more common in girls, particularly if there is bronchopulmonary involvement. The incidence of oesophageal duplication cyst is estimated to be one in 8200 live births with male prevalence. Most duplications are benign, but the presence of ectopic gastric mucosa and the potential for malignant degeneration remain a concern. A newborn female, antenatally diagnosed with right-sided thoracic mass, was diagnosed with a foregut duplication cyst of size 4.1 cm × 3.7 cm × 8 cm in the posterior mediastinum. Thoracoscopic resection was done on day of life 14. The postoperative recovery was uneventful and histopathology confirmed the diagnosis. A literature search revealed only a few cases of an early thoracoscopic intervention, and ours is the earliest reported. Thoracoscopy in the neonatal period is safe and effective.

Two's Company: Multiple Thoracic Lesions on Prenatal US and MRI.

BACKGROUND: Congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), and CPAM-BPS hybrid lesions are most commonly solitary; however, >1 lung congenital lung lesion may occur. OBJECTIVES: To assess the frequency of multiple congenital thoracic anomalies at a high-volume referral center; determine prenatal ultrasound (US) and magnetic resonance imaging (MRI) features of these multifocal congenital lung lesions that may allow prenatal detection; and determine the most common distribution or site of origin. METHODS: Database searches were performed from August 2008 to May 2019 for prenatally evaluated cases that had a final postnatal surgical diagnosis of >1 congenital lung lesion or a lung lesion associated with foregut duplication cyst (FDC). Lesion location, size, echotexture, and signal characteristics were assessed on prenatal imaging and correlated with postnatal computed tomographic angiography and surgical pathology. -Results: Of 539 neonates that underwent surgery for a thoracic lesion, 35 (6.5%) had >1 thoracic abnormality. Multiple discrete lung lesions were present in 19 cases, and a lung lesion associated with an FDC was present in 16. Multifocal lung lesions were bilateral in 3 cases; unilateral, multilobar in 12; and, unilobar multisegmental in 4. Median total CPAM volume/head circumference ratio for multifocal lung lesions on US was 0.66 (range, 0.16-1.80). Prenatal recognition of multifocal lung lesions occurred in 7/19 cases (36.8%). Lesion combinations were CPAM-CPAM in 10 cases, CPAM-BPS in 5, CPAM-hybrid in 2, hybrid-hybrid in 1, and hybrid-BPS in 1. Of 5 unilateral, multifocal lung lesions, multifocality was prenatally established through identification of a band of normal intervening lung or intrinsic differences in lesion imaging features. CONCLUSIONS: Although less common, multiple thoracic abnormalities can be detected prenatally. Of multifocal lung lesions, the most common combination was CPAM-CPAM, with a unilateral, multilobar distribution. Prenatal recognition is important for pregnancy counseling and postnatal surgical management.

Foregut duplications in the superior mediastinum: beware of a common wall with the tracheo-bronchial tree.

BACKGROUND: Foregut duplication cysts (FD) with a common muscular wall with the oesophagus are well recognised. Our case series highlights the existence of a common wall between superior mediastinal FD and the tracheo-bronchial tree. MATERIALS AND METHODS: Over the last 15 years, we have thoracoscopically resected 41 FD. Five cases were identified to have a common wall with the tracheo-bronchial tree at operation. The clinical history, radiology, findings at operation and pathology were evaluated to highlight learning points. RESULTS: Five superior mediastinal cysts with a common wall were identified, with two antenatally and three postnatally diagnosed. All three postnatal cases and one antenatal case presented with symptoms of respiratory compromise and stridor. Only one neonate was relatively asymptomatic before resection. CT similarities in all cases were: separation of trachea and oesophagus by the cyst, oesophageal deviation to the right or compression and compression of trachea/bronchus. Thoracoscopically, two cysts were resected without injury to the airway, while three had inadvertent tracheo-bronchial injury. Repair of the tracheal injury was possible in one case thoracoscopically, while two cases required conversions, as adequate oxygenation could not be maintained despite on-table endotracheal tube advancement beyond the injury and thoracoscopic manoeuvres. All cases had excellent outcomes at follow-up (median 25months, range 4-39months) with resolution of symptoms and no recurrences. CONCLUSIONS: Our report highlights the existence of a common wall between the superior mediastinal FD and the tracheo-bronchial tree. Thoracoscopic resections are feasible including repair of inadvertent airway injury, provided adequate oxygenation can be maintained.

Two synchronous congenital neck masses with facial nerve weakness.

OBJECTIVE: To describe a case of a complex neck mass with final pathology significant for two synchronous lesions, a type 1 branchial cleft cyst and an enteric duplication cyst, and to discuss its management given the unique presentation.

Heterotopic Gastrointestinal Cyst of the Oral Cavity Radiology-Pathology Correlation.

Heterotopic gastrointestinal cyst of the oral cavity is a rare congenital lesion that may arise from ectopic undifferentiated endodermal cells. Imaging, particularly MRI, is useful for surgical planning. On MRI, the cysts typically demonstrate high signal on T2-weighted sequences and variable signal on T1-weighted sequences, which can resemble other conditions, such as dermoids. On histology, the appearance of these lesions can be variable, and may include stratified squamous, simple and ciliated columnar, as well as foveolar and intestinal-type epithelia, often surrounding by smooth muscle. Complete surgical excision is the treatment of choice.

Foregut Duplication Cysts in Children.

BACKGROUND AND OBJECTIVES: Duplications of the alimentary tract are rare anomalies. We report our experience with foregut duplication cysts including their clinical presentation, diagnostic modalities, and surgical management. METHODS: We report a 20-year retrospective review of all foregut duplication cysts managed at our institution. RESULTS: Twelve patients with 13 foregut duplication cysts were identified. The ages of the children at the time of surgery ranged from infancy to adolescence, with a mean age of 7.2 years. Half of the patients presented with abdominal pain and vomiting, and the remaining either had respiratory distress or were asymptomatic. All resections were performed electively. Two of the 11 patients had other congenital anomalies, including a congenital pulmonary airway malformation and coarctation of the aorta. One patient had prenatal diagnosis by ultrasonography. Nine patients underwent complete successful excision with no complications. Three patients whose symptoms resolved during hospitalization remained under observation because of parental preference. CONCLUSIONS: Foregut malformation in children may present with a variety of symptoms or can be found incidentally. The decision and timing of surgery is based on the clinical presentation. Surgical intervention in asymptomatic patients should be based on a thorough discussion with the parents.

Foregut duplication cyst of the floor of mouth in a neonate: case report.

Congenital oral masses may interfere with vital functions such as respiration and deglutition in neonates. We report a congenital heterotopic oral gastrointestinal cyst that caused failure to thrive. A working knowledge of the differential diagnosis and pathophysiology of this can guide the clinician in its evaluation and management.

Foregut duplication cyst associated with esophageal atresia-one-stage neonatal surgical repair.

Foregut duplication cysts can occur anywhere along the foregut-derived structures. Its association with esophageal atresia (EA) and tracheoesophageal fistula (TEF) is exceptionally rare, the majority of cases being diagnosed at an older age due to symptoms or complications. We present the third reported case in the literature, when diagnosis and treatment were done at the time of repair for EA-TEF.