Coexistence of a retroesophageal right subclavian artery with a left maxillary artery medial to the mandibular nerve.

This case report describes the coexistence of a retroesophageal right subclavian artery and left maxillary artery which passed deep to the mandibular nerve. An 88-year-old woman died of acute heart failure, and the postmortem revealed that the right subclavian artery originated from the aortic arch as the last branch at the level of the fourth thoracic vertebra, then passed between the esophagus and the vertebral column. The artery then ascended right superiorly and passed behind the anterior scalene muscle. The right vertebral artery arose from the retroesophageal right subclavian artery and entered the transverse foramen of the sixth cervical vertebra. The left maxillary artery branched at the common trunk of the posterior deep temporal and the inferior alveolar arteries. The maxillary artery then turned anteromedially and branched to give the middle meningeal artery. The mandibular nerve gave off the buccal nerve, deep temporal nerve and a thick nerve just below the foramen ovale. The auriculotemporal nerve that branched from the thick nerve ran deep to the maxillary artery. The maxillary artery turned anteriorly, passing deep to the branches. The artery then split to give the buccal artery and the anterior deep temporal artery. In the pterygopalatine section, the maxillary artery branched off to form the common trunk of the infraorbital and sphenopalatine arteries and the posterior superior alveolar artery. It may be necessary to pay attention to the course of the maxillary artery and its relationship to the mandibular nerve branches, when a retroesophageal right subclavian artery is seen.

Loss of Endothelial TSPAN12 Promotes Fibrostenotic Eosinophilic Esophagitis via Endothelial Cell-Fibroblast Crosstalk.

BACKGROUND & AIMS: Eosinophilic esophagitis (EoE) can progress to fibrostenosis by unclear mechanisms. Herein, we investigated gene dysregulation in fibrostenotic EoE, its association with clinical parameters and specific pathways, and the functional consequences. METHODS: Esophageal biopsies from subjects with EoE were collected across 11 Consortium of Eosinophilic Gastrointestinal Disease Researchers sites (n = 311) and 2 independent replication cohorts (n = 83). Inclusion criteria for fibrostenotic EoE were endoscopic rings, stricture, and/or a history of dilation. Endoscopic, histologic, and molecular features were assessed by the EoE Endoscopic Reference Score, EoE Histology Scoring System, EoE Diagnostic Panel, and RNA sequencing. Esophageal endothelial TSPAN12 expression and functional effects on barrier integrity and gene expression were analyzed in vitro. RESULTS: TSPAN12 was the gene most correlated with fibrostenosis (r = -0.40, P < .001). TSPAN12 was lower in fibrostenotic EoE and correlated with EoE Endoscopic Reference Score, EoE Diagnostic Panel, and EoE Histology Scoring System (r = 0.34-0.47, P < .001). Lower TSPAN12 associated with smaller esophageal diameter (r = 0.44, P = .03), increased lamina propria fibrosis (r = -0.41, P < .001), and genes enriched in cell cycle-related pathways. Interleukin (IL)-13 reduced TSPAN12 expression in endothelial cells. Conversely, anti-IL-13 therapy increased TSPAN12 expression. TSPAN12 gene silencing increased endothelial cell permeability and dysregulated genes associated with extracellular matrix pathways. Endothelial cell-fibroblast crosstalk induced extracellular matrix changes relevant to esophageal remodeling. CONCLUSIONS: Patients with fibrostenotic EoE express decreased levels of endothelial TSPAN12. We propose that IL-13 decreases TSPAN12, likely contributing to the chronicity of EoE by promoting tissue remodeling through fibroblast-endothelial cell crosstalk.

Common pathogenesis for sirenomelia, OEIS complex, limb-body wall defect, and other malformations of caudal structures.

Decades of clinical, pathological, and epidemiological study and the recent application of advanced microarray and gene sequencing technologies have led to an understanding of the causes and pathogenesis of most recognized patterns of malformation. Still, there remain a number of patterns of malformation whose pathogenesis has not been established. Six such patterns of malformation are sirenomelia, VACTERL association, OEIS complex, limb-body wall defect (LBWD), urorectal septum malformation (URSM) sequence, and MURCS association, all of which predominantly affect caudal structures. On the basis of the overlap of the component malformations, the co-occurrence in individual fetuses, and the findings on fetal examination, a common pathogenesis is proposed for these patterns of malformation. The presence of a single artery in the umbilical cord provides a visible clue to the pathogenesis of all cases of sirenomelia and 30%-50% of cases of VACTERL association, OEIS complex, URSM sequence, and LBWD. The single artery is formed by a coalescence of arteries that supply the yolk sac, arises from the descending aorta high in the abdominal cavity, and redirects blood flow from the developing caudal structures of the embryo to the placenta. This phenomenon during embryogenesis is termed vitelline vascular steal.

Percutaneous transhepatic portal vein stenting as rescue treatment for recurrent oesophageal variceal bleeding in a 31-year-old woman with haepatocellular carcinoma in a non-cirrhotic liver.

A 31-year-old woman with hepatocellular carcinoma suffered from recurrent oesophageal variceal bleeding due to portal hypertension, which was caused by severe compression of the portal vein by metastatic lymph nodes. Endoscopic band ligation and pharmacological treatment did not suffice to prevent recurrence of variceal bleeding. Eventually, after the fifth variceal bleeding within 6 months, the patient was admitted to the intensive care unit in a haemodynamic shock. A Sengstaken-Blakemore tube was inserted and all treatment options were discussed, but only percutaneous transhepatic recanalisation of the portal vein with stent placement to reduce portal vein pressure was thought to be feasible with any chance to relieve portal vein pressure. After successful portal vein stenting, our patient did not have any recurrent bleeding in the remaining year of her life. We suggest that percutaneous transhepatic portal vein stenting may be a feasible and adequate last line treatment for complications of portal hypertension.

Effect of Venous Superdrainage on Colon Interposition for Esophageal Reconstruction.

When performing esophageal reconstruction, a colonic pedicle graft is chosen as the next candidate to the stomach because of complications arising from the operation time and vascular anastomosis. Vascular anastomosis is not necessarily required for pedicle grafts, but it is necessary to perform additional vascular anastomosis in some cases. We herein report a case of superdrainage in which anastomosis of the colonic vein and the right internal thoracic vein was effective against congestion. A 68-year-old man with thoracic esophageal cancer and pyloric antrum gastric cancer was referred to our hospital. Complete resection was performed with subtotal esophageal resection and total gastrectomy. We added superdrainage (right internal thoracic vein - ileocolic vein) to the colonic pedicle graft, which showed congestion, and performed esophageal reconstruction. Venous superdrainage using a colonic pedicle graft is effective for esophageal reconstruction.

Black esophagus: a syndrome of acute esophageal necrosis associated with active alcohol drinking.

Black esophagus, also known as acute esophageal necrosis (AEN) syndrome, is a rare entity characterized by patchy or diffuse circumferential black pigmentation of the esophageal mucosa from ischemic necrosis. It may present with life-threatening upper gastrointestinal hemorrhage resulting in high mortality in immunocompromised patients. Advanced age with multiple comorbidities compounded with compromised hemodynamic states are poor prognostic factors. Findings on laboratory work-up and radiological imaging are non-specific. After initial resuscitation, endoscopic evaluation and histological examination of esophageal biopsy are diagnostic. Early recognition and aggressive resuscitation are the fundamental principles for the management of AEN and better outcome of the disease. We report a case of a 56-year-old woman with diabetes mellitus, gastro-esophageal reflux disease, and active alcohol binging who presented with hematemesis and acute epigastric pain due to AEN. This case illustrates a rare etiology of AEN due to active alcohol drinking, which may be overlooked. Physician awareness about this etiology is important as early recognition and timely management may improve survival.

Genetic Mouse Models and Induced Pluripotent Stem Cells for Studying Tracheal-Esophageal Separation and Esophageal Development.

Esophagus and trachea arise from a common origin, the anterior foregut tube. The compartmentalization process of the foregut into the esophagus and trachea is still poorly understood. Esophageal atresia/tracheoesophageal fistula (EA/TEF) is one of the most common gastrointestinal congenital defects with an incidence rate of 1 in 2,500 births. EA/TEF is linked to the disruption of the compartmentalization process of the foregut tube. In EA/TEF patients, other organ anomalies and disorders have also been reported. Over the last two decades, animal models have shown the involvement of multiple signaling pathways and transcription factors in the development of the esophagus and trachea. Use of induced pluripotent stem cells (iPSCs) to understand organogenesis has been a valuable tool for mimicking gastrointestinal and respiratory organs. This review focuses on the signaling mechanisms involved in esophageal development and the use of iPSCs to model and understand it.

Distal contractile integral measurement and vascular compression in the esophagus: a problem unsolved?

BACKGROUND: Distal contractile integral (DCI) is influenced by factors other than esophageal smooth muscle contractility, such as intrabolus pressure and vascular and respiratory movements' artifacts. We aimed to determine the size of the contribution of pressures generated by vascular compression on the esophagus to the DCI measured in HRM recordings in symptomatic patients. METHODS: HRM manometry recordings obtained from 383 subjects referred to the GI motility laboratory at a tertiary center (2012-2016) were evaluated by visual inspection for evidence of strong vascular compression (SVC) of the esophagus. Clinical, demographic, manometric, and serologic data for Chagas disease were obtained. Subjects were classified, respectively, as asymptomatics (ASYM) or symptomatics (SYMP). DCI and SVC-DCI were measured, and the SVC-DCI/DCI ratio was expressed as a percentage and the difference between DCI and SVC-DCI (neat-DCI) was calculated. DCI, SVC-DCI, SVC-DCI/DCI % and neat-DCI from SYMP and ASYM were compared. RESULTS: SVC was conspicuous in 42 of 383 subjects (11%). In 33 subjects, SVC was detected only in supine position. SVC was localized in middle esophagus in 21 subjects (50%), in distal esophagus in 12 subjects (29%) and in both regions in 9 subjects (21%). In 9 subjects, SVC vanished from the swallowing window analysis (21%). CONCLUSIONS: SVC is a common finding in esophageal HRM study, particularly in the supine position. Occasionally, its contribution to DCI value is sufficiently great to masquerade esophageal hypocontractility. Different manometric protocols may be required in patients with SVC.

Evidence for Cytoprotective Effect of Carbon Monoxide Donor in the Development of Acute Esophagitis Leading to Acute Esophageal Epithelium Lesions.

Exposure to acidic gastric content due to malfunction of lower esophageal sphincter leads to acute reflux esophagitis (RE) leading to disruption of esophageal epithelial cells. Carbon monoxide (CO) produced by heme oxygenase (HMOX) activity or released from its donor, tricarbonyldichlororuthenium (II) dimer (CORM-2) was reported to protect gastric mucosa against acid-dependent non-steroidal anti-inflammatory drug-induced damage. Thus, we aimed to investigate if CO affects RE-induced esophageal epithelium lesions development. RE induced in Wistar rats by the ligation of a junction between pylorus and forestomach were pretreated i.g. with vehicle CORM-2; RuCl3; zinc protoporphyrin IX, or hemin. CORM-2 was combined with NG-nitro-L-arginine (L-NNA), indomethacin, capsazepine, or capsaicin-induced sensory nerve ablation. Esophageal lesion score (ELS), esophageal blood flow (EBF), and mucus production were determined by planimetry, laser flowmetry, histology. Esophageal Nrf-2, HMOXs, COXs, NOSs, TNF-α and its receptor, IL-1 family and IL-1 receptor antagonist (RA), NF-κB, HIF-1α, annexin-A1, suppressor of cytokine signaling (SOCS3), TRPV1, c-Jun, c-Fos mRNA/protein expressions, PGE2, 8-hydroxy-deoxyguanozine (8-OHdG) and serum COHb, TGF-ß1, TGF-ß2, IL-1ß, and IL-6 content were assessed by PCR, immunoblotting, immunohistochemistry, gas chromatography, ELISA or Luminex platform. Hemin or CORM-2 alone or combined with L-NNA or indomethacin decreased ELS. Capsazepine or capsaicin-induced denervation reversed CORM-2 effects. COHb blood content, esophageal HMOX-1, Nrf-2, TRPV1 protein, annexin-A1, HIF-1α, IL-1 family, NF-κB, c-Jun, c-Fos, SOCS3 mRNA expressions, and 8-OHdG levels were elevated while PGE2 concentration was decreased after RE. CO donor-maintained elevated mucosal TRPV1 protein, HIF-1 α, annexin-A1, IL-1RA, SOCS3 mRNA expression, or TGF-ß serum content, decreasing 8-OHdG level, and particular inflammatory markers expression/concentration. CORM-2 and Nrf-2/HMOX-1/CO pathway prevent esophageal mucosa against RE-induced lesions, DNA oxidation, and inflammatory response involving HIF-1α, annexin-A1, SOCS3, IL-1RA, TGF-ß-modulated pathways. Esophagoprotective and hyperemic CO effects are in part mediated by afferent sensory neurons and TRPV1 receptors activity with questionable COX/PGE2 or NO/NOS systems involvement.

Three-dimensional printing for surgical planning of a double aortic arch case.

BACKGROUND AND AIM: Over the past years, three-dimensional (3D) models of patient-specific anatomical conditions are being used to improve the comprehension and surgical management of a variety of diseases. It is an additional diagnostic tool that aids clinical decision-making. Furthermore, this technology is still not routinely used in the medical field since its availability is limited by cost and complex process. METHODS AND RESULTS: We describe a patient with a balanced-type double aortic arch encircling trachea and esophagus. Considering the clinical symptoms, surgical decompression of these structures and defined aortic arch reconstruction was indicated. The 3D printed model revealed narrowing of the left aortic arch at the junction of the descending thoracic aorta that did not clearly appear on the conventional images reconstruction. The left aortic arch was divided and the symptoms completely disappeared. No immediate or late complications occurred. CONCLUSION: 3D printed models can be helpful in surgical planning of congenital heart malformations. It should be strongly considered as an additional tool in complex cases.

Surface Microdialysis Detects Ischemia After Esophageal Resection-An Experimental Animal Study.

BACKGROUND: After an esophageal resection, continuity is commonly restored by a gastric tube reconstruction and an intrathoracic anastomosis to the remaining proximal esophagus. Ischemia of the anastomotic region is considered to play a pivotal role in anastomotic leakage. Microdialysis (µD) is an excellent method to measure local biochemical substances and parameters in a specific organ or compartment aiming at early detection of ischemia. This animal study evaluates ischemia of the gastric tube reconstruction using a novel method-µD on organ surfaces. This promising method may have the potential to detect an anastomotic leakage before clinical symptoms develop. METHODS: Anesthetized normoventilated pigs were used. Surface microdialysis (S-µD) catheters and an intraparenchymal oxygen tension catheter were placed on the stomach. A gastric tube was made and the gastroepiploic artery was divided halfway along the greater curvature to produce severe ischemia at the top of the gastric tube. µD data from four locations (gastric tube, ileum and peritoneal cavity) were recorded every 20 min during the experiment. Tissue samples from all catheter sites underwent histopathological analysis. Intraparenchymal oxygen partial pressure, systemic blood tests, and hemodynamic parameters were recorded. RESULTS: S-µD data showed values indicating severe ischemia at the top of the gastric tube and intermediate ischemia at the level of transection of the gastroepiploic artery. Ischemia was verified by histopathological analysis of tissue samples and intraparenchymal oxygen tension data. CONCLUSIONS: S-µD can detect and grade severity of local ischemia in real time, in an animal model.

The sub-classification of type B2 vessels according to the magnifying endoscopic classification of the Japan Esophageal Society.

OBJECTIVES: Guidelines for magnified endoscopic diagnosis of esophageal squamous cell carcinoma (SCC) have been proposed by the Japan Esophageal Society. Type B1, B2, and B3 reflect increasing tumor invasion depths (within mucosal epithelium or into lamina propria mucosa [T1a-EP/LPM], into muscularis mucosa or superficial invasion into submucosa [T1a-MM/T1b-SM1], and into submucosa [T1b-SM2], respectively). The diagnostic accuracy of type B1 and B3 is high, but accuracy of type B2 is low. We aimed to improve the diagnostic accuracy of type B2. METHODS: We retrospectively reviewed 248 SCC lesions treated with endoscopic submucosal dissection between January 2012 and July 2018 and identified the B2 lesions. The maximum diameter of the area presenting B2 was measured and evaluated in relation to tumor invasion, for which receiver-operating characteristic (ROC) curves were generated. The optimal area size for distinguishing T1a-EP/LPM from T1a-MM or deeper invasion was determined. RESULTS: There were 78 lesions with B2, of which 26 (33%) were T1a-MM or T1b-SM1 SCCs. ROC curve analysis indicated that the optimal cut-off for the target area showing B2 was 4 mm. The invasion depth (EP/LPM: MM/SM1: SM2) of B2 observed in an area with a diameter <4 mm (B2-Narrow) and those with diameter ≥4 mm (B2-Broad) was 46:11:1 and 1:15:4, respectively. To predict T1a-MM or deeper invasion, B2-Broad had a sensitivity, specificity, positive predictive value, and negative predictive value of 61%, 98%, 95%, and 79%, respectively. CONCLUSION: The diagnostic accuracy of type B2 was improved by evaluating the area of type B2.

Left-sided portal hypertension due to retroperitoneal fibrosis treated with an oesophagus preserving, modified Sugiura procedure.

Left-sided portal hypertension is a very uncommon condition and retroperitoneal fibrosis has rarely been reported as a cause. We present the case of a 77-year-old man with retroperitoneal fibrosis obstructing the splenic vein and causing recurrent episodes of upper gastrointestinal bleeding. Computed tomography showed a retroperitoneal mass as being responsible for the obstruction of the splenic vein, splenomegaly, and diffuse varices around the gastrosplenic and gastrohepatic ligaments. An oesophagus preserving, modified Sugiura procedure was performed with disconnection of the gastric vessels on the lesser curve of the stomach, preserving the pylorus branches of the nerves of Latarjet.

Evaluation of prognostic factor and nature of acute esophageal necrosis: Restropective multicenter study.

Acute esophageal necrosis (AEN) is a serious disease which can causes gastrointestinal bleeding and death. Although black color change is not essential factor of organ necrosis, AEN is also known as "black esophagus." Because of its rarity, there are limited studies regarding risk factors of mortality and recurrence. Thus, we conducted a multicenter retrospective study in order to evaluate the clinical characteristics of AEN. Method Clinical datum of AEN patients from 7 tertiary hospitals located in Daejeon-Choongcheong province were evaluated based on medical records. Our primary endpoint was risk factors for mortality and the secondary endpoint was risk factors for recurrence and clarifying whether "black esophagus" is a right terminology.Fourty one patients were enrolled. Thirty six patients were male, mean age was 69.5 years. Nine patients had died, and 4 patients showed recurrence. Sepsis and white color change in endoscopy were related to high mortality (Chi-Squared test, P < .05). Old age, high pulse rate, low hemoglobin, and low albumin were also related to high mortality. Unexpectedly, heavy drinking showed favorable a mortality. Septic condition and high pulse rate showed poor mortality in logistic regression test (P < .05). Coexisting duodenal ulcer was related to recurrence (Chi-Squared test, P < .05). There was no difference in the underlying condition except patients with a coexisting cancer and white-form displayed lower hemoglobin level. Conclusion: Our results imply that white color change, septic condition, high pulse rate, and low hemoglobin & albumin are poor prognostic factors in AEN. Further evaluation may help clarify the findings of our study.

[Preliminary evaluation of endoscopic selective varices devascularization in children].

Objective: To investigate the effect of the endoscopic selective varices devascularization (ESVD) for the esophageal gastric varices bleeding (EGVB) in children. Methods: The clinical data of the patients diagnosed with EGVB and treated with ESVD from January 2018 to March 2018 were retrospectively analyzed. The effects, safety and complications of ESVD were evaluated. Results: There were five patients (including 2 males and 3 females, age ranged from 4 to 7 years) in the study. No rebleeding was found at the first follow-up on one week post operation. Three patients were treated with the endo-therapy at the twice follow-up (3 months after surgery): 2 patients had ESVD again and 1 patient had resection under endoscopy due to stenosis caused by surgical scar. After the second procedure, there was no rebleeding but one patient had abdominal pain caused by mesenteric thrombosis, cured with low molecular weight heparin. Conclusion: The ESVD for EGVB is safe and effective, but the long-term curative effect should be further studied.

Efficacy of Novel Multispectral Imaging Device to Determine Anastomosis for Esophagogastrostomy.

BACKGROUND: Biomedical imaging devices that utilize the optical characteristics of hemoglobin (Hb) have become widespread. In the field of gastroenterology, there is a strong demand for devices that can apply this technique to surgical navigation. We aimed to introduce our novel multispectral device capable of intraoperatively performing quantitative imaging of the oxygen (O2) saturation and Hb amount of tissues noninvasively and in real time, and to examine its application for deciding the appropriate anastomosis point after subtotal or total esophagectomy. MATERIALS AND METHODS: A total of 39 patients with esophageal cancer were studied. Tissue O2 saturation and Hb amount of the gastric tube just before esophagogastric anastomosis were evaluated using a multispectral tissue quantitative imaging device. The anastomosis point was decided depending on the quantitative values and patterns of both the tissue O2 saturation and Hb amount. RESULTS: The device can instantaneously and noninvasively quantify and visualize the tissue O2 saturation and Hb amount using reflected light. The tissue Hb status could be classified into the following four types: good circulation type, congestion type, ischemia type, and mixed type of congestion and ischemia. Postoperative anastomotic failure occurred in 2 cases, and both were mixed cases. CONCLUSIONS: The method of quantitatively imaging the tissue O2 saturation and Hb level in real time and noninvasively using a multispectral device allows instantaneous determination of the anastomosis and related organ conditions, thereby contributing to determining the appropriate treatment direction.

Esophageal Blood Flow May Not Be Directly Influenced by Anastomotic Tension: An Exploratory Laser Doppler Study in Swine.

BACKGROUND: Anastomotic tension has been linked to leakage and stenosis in esophageal surgery in both adults and children. We aimed to determine the effects of esophageal topography, operative technique, and anastomotic tension on esophageal blood flow. MATERIALS AND METHODS: We divided and reanastomosed the esophagi at the carinal level with increasing levels of anastomotic tension in piglets (n = 10) and sham controls (n = 4). We examined esophageal blood flow pre- and postoperatively using laser Doppler flowmetry at the anastomosis and two predetermined proximal and distal points. Blood flow in relation to distance from the anastomosis was examined by multivariate linear regression. RESULTS: Thoracotomy alone did not influence perfusion at the carinal level in shams (Δ = 3.13 laser Doppler units, 95% confidence interval: -3.4 to 9.7, p = 0.2686). We constructed a (F[5,134] = 6.34, p < 0.0001) multinomial regression model based on distance to the anastomotic site and pre-/postoperative measurements (adjusted R 2 = 0.1624). Tissue blood flow was higher distant to the carina, but lower postoperatively and not influenced by the tension resulting from the extent of resection (F[1, 8] = 1.134, p = 0.318). CONCLUSION: Esophageal blood flow is higher at greater distances to the carinal level and hampered by esophageal division and reanastomosis. The extent of resection has less influence than previously assumed. Therefore, leakage and stenosis after esophageal anastomosis may not solely be caused by insufficient anastomotic blood flow.