Acute esophageal necrosis and low-flow state.

Acute esophageal necrosis (AEN), also called black esophagus, is quite exceptional. Endoscopic findings show circumferential black discolouration of the esophagus with or without exudates. The etiology of AEN is presently unknown and is assumed to be multifactorial. Distal esophageal involvement with proximal extension ending sharply at the gastroesophageal junction is the most common presentation. The present case report describes the clinical and endoscopic evolution of black esophagus observed in a patient with significant peripheral vascular disease, who was presented to the intensive care unit at the Hopital Saint-Francois d'Assise (Quebec City, Quebec). Through an extensive review of the literature, common underlying clinical conditions of patients diagnosed with AEN have been identified.     

Black esophagus: a case series and literature review of acute esophageal necrosis

Black esophagus or acute esophageal necrosis (AEN) is a rare medical disorder which is characterized by a diffuse circumferential black esophageal mucosa. The majority of patients present with signs of upper gastrointestinal bleeding. Diagnosis is made based on esophagogastroduodenoscopy. Treatment consists of intravenous fluids, proton pomp inhibitors and additional therapies to treat the underlying illness. In this article we present five cases of patients with AEN and briefly review the literature of AEN.     

Delayed esophageal necrosis and perforation secondary to thoracic aortic rupture: a case report and review of the literature

Delayed esophageal necrosis secondary to aortic rupture is extremely rare but potentially fatal. Although diagnostic techniques have improved, survival remains rare. The clinical and imaging features have not been characterized, as no large series have been reported since the advent of computed tomography (CT) and endoscopy. Moreover, as delayed esophageal necrosis secondary to aortic rupture is rarely anticipated, diagnosis is usually delayed. We recently encountered a case of this complication and present this along with a literature review, in order to facilitate early recognition and treatment. In many cases, hemodynamics remain relatively stable despite aortic rupture and the long interval between onset and operation. Dysphagia is therefore an important symptom that may indicate compression of the esophagus and subsequent esophageal necrosis. Preoperative total obstruction of the esophagus on barium swallow, endoscopy, or CT is also an important feature suggesting ischemic change of the esophagus. Endoscopy often reveals an annular ulcer suggestive of an ischemic process leading to necrosis. Death generally occurs from severe mediastinitis occurring after graft repair of the aorta. We recommend performing postoperative endoscopy after aortic surgery when preoperative obstruction of esophagus is found. If endoscopy reveals annular necrosis, surgical treatment involving esophagectomy and omental translocation should be performed expediently before perforation occurs.     

Fatal Hemorrhage Complicating Carcinoma of the Esophagus

Four cases of esophageal carcinoma complicated by fatal hemorrhage are reported. All four patients had recently completed radiation therapy. An aortoesophageal fistula was present in two cases; fibrinoid necrosis of the esophageal arteries was present in the other two. The esophageal tumor was localized in two cases and had disappeared in one case. In one patient it had metastasized widely. Ninety-nine other reports of esophageal cancer and fatal hemorrhage are reviewed from the literature. Aortoesophageal fistula was the cause of hemorrhage in 78 cases. Occlusion of the vasa vasorum by thrombosis, inflammation, neoplastic cells or radiation injury appears to be the cause of aortic necrosis and fistula formation. Prompt surgical approach, if possible, should be used to control hemorrhage, as the primary tumor may be localized to the esophagus only.     

Black esophagus due to acute necrotizing esophagitis: four case reports

We describe four patients with acute esophageal necrosis who were admitted to hospital due to upper gastrointestinal bleeding. "Black esophagus" is endoscopically defined as diffuse dark pigmentation of the esophageal wall. The underlying conditions were ketoacidosis in three of the patients and diabetes mellitus in two. Three patients responded well to empirical supportive therapy and one patient died of coexisting illness rather than the esophageal status. Acute esophageal necrosis is a rare entity that should be considered in the differential diagnosis of upper gastrointestinal bleeding.     

Esophageal function in esophageal atresia

In order to determine the spectrum of esophageal dysfunction in repaired esophageal atresia, 14 patients were evaluated with esophageal manometry, intraluminal pH recording, and radiology. Nine patients had no difficulty in swallowing but six had symptoms suggestive of gastroesophageal (GE) reflux. On pH recording, six had evidence of GE reflux. Basal sphincter pressure was 22 mm Hg in both reflux and nonreflux patients. No patient had manometric evidence of peristalsis in the proximal esophagus, but six had peristalsis in the distal esophagus. On radiology all had a normal peristaltic stripping wave in the cervical esophagus, and peristalsis was absent in the proximal thoracic esophagus in all patients but present in the distal esophagus in five of the 10 patients studied. Esophageal dysfunction is present in all patients with repaired esophageal atresia even when symptoms are absent.Supported by grant AM 25731 from the National Institutes of Health.     

Chromoendoscopy is a valuable tool for screening of high-risk patients with head and neck cancer for early detection of esophageal cancer

BACKGROUND: The incidence of esophageal cancer is markedly increased in patients with head and neck cancer, and the presence of esophageal cancer is associated with reduced survival rates. AIMS: We investigated whether the results of screening for esophageal cancer in patients with head and neck cancer using chromoendoscopy would change the treatment of such patients. PATIENTS: 87 patients with head and neck cancer and known alcohol or nicotine abuse were screened for esophageal cancer. Methods: The patients underwent esophagogastroduodenoscopy and staining of the esophagus with 2% Lugol's solution. Biopsies were taken from unstained areas for histopathological assessment. RESULTS: Esophageal cancer was newly diagnosed in 10 patients (11.5%), including 2 with carcinoma in situ. There were dysplastic changes in 6 patients (7%) and an unknown Barrett esophagus in 4 patients (5%). In 36 patients (41%) unstained areas were associated with esophagitis. While unstained areas could not be detected in 17 patients, the histology was normal in 14 patients with unstained areas. In all the patients with newly detected invasive esophageal cancer, the treatment had to be changed from a curative neoadjuvant approach to palliative treatment. In 2 patients with carcinoma in situ mucosectomy was performed. In the cases with dysplastic areas and newly detected Barrett epithelium a careful follow-up regime was arranged. CONCLUSIONS: The staging of patients with head and neck cancer, and the risk factors for esophageal cancer should include chromoendoscopy of the esophagus as a standard procedure. Extended staging provides critical additional information, which helps to more safely distinguish future candidates for curative and palliative treatment.     

Acute esophageal necrosis

Acute esophageal necrosis (AEN) or “black esophagus” is a rare condition presented by patients with critical state of health and characterized by a darkened esophagus, usually the distal third, in upper digestive endoscopy. The main clinical manifestation is upper gastrointestinal bleeding and there may be abdominal pain, dysphagia, nausea, vomiting, fever and syncope associated. The diagnosis depends on clinical suspicion and performing endoscopy, the biopsy not being required. In this article we present a case of a patient who had lots of comorbidities and developed AEN during a post-operative period, and discuss the importance of AEN in an increasingly ageing population.     

Cardia-type metaplasia arising in the remnant esophagus after cardia resection

BACKGROUND: Specialized intestinalized metaplasia in the distal esophagus (Barrett's esophagus) is a recognized precursor of esophageal adenocarcinoma, but its pathogenesis is incompletely understood. The aim of this study was to investigate the mucosal effects of esophagogastrostomy, an artificial interface between esophageal squamous and gastric oxyntic epithelium. METHODS: EGD was performed in 14 consecutive patients (median age 63 years, range 26-71 years) who had undergone esophagogastrostomy from 3 to 88 months earlier. Biopsy specimens were obtained in 13 patients from the anastomosis and, when present, columnar epithelium in the remnant esophagus. RESULTS: In 10 patients, EGD demonstrated tongue-shaped segments of columnar epithelium extending from 0.3 to 7 cm into the remnant esophagus. Biopsy specimens revealed cardia-type mucosa in all patients, whether at the anastomosis or proximally in esophageal segments of columnar epithelium. Magnification endoscopy of cardia-type mucosa visualized a long-oval, tubular, or ridged surface pattern. In 3 cases, complete intestinal metaplasia was observed within the cardia-type mucosa. CONCLUSIONS: The frequent transformation of squamous epithelium into cardia-type mucosa in the distal remnant esophagus after esophagogastrostomy supports the concept that cardia-type mucosa is a reflux-induced metaplasia that may give rise to the subsequent development of specialized intestinalized metaplasia.     

Black esophagus: report of six cases and review of the literature, 1963–2003

Black esophagus is the uncommon endoscopic finding of extensive black discoloration of the esophageal mucosa, usually from acute esophageal necrosis. Six cases of black esophagus were seen at Mayo Clinic (Rochester, Minnesota, USA) from 1997 through 2003, and 46 cases were reported in the English-language literature from 1963 through 2003. We studied the demographics, clinical features, and outcomes of these 52 cases of black esophagus. Age and sex were known for 50 patients: the mean (SD) age was 65 years (19), and 42 patients (84%) were men. Symptoms were known for 51 patients: the most common symptom was upper gastrointestinal tract bleeding, occurring in 40 patients (78%). All 52 patients had at least one comorbid condition (with most having two or more), including duodenal ulcer in 17 (33%), cancer in 15 (29%), renal insufficiency in 15 (29%), and diabetes mellitus in 14 (28%). The suspected cause of black esophagus was reported for 40 patients: ischemia in 22 (55%); massive gastroesophageal reflux in seven (18%); and esophageal infection (Lactobacillus acidophilus, herpes simplex, Candida albicans) in four (10%). Most patients received supportive therapy, particularly acid suppression therapy. Of the 47 patients for whom outcomes were known, 17 (36%) died. There were no statistically significant differences between survivors and non-survivors. Black esophagus typically occurs in older men with at least one comorbid condition; a substantial number of patients die. Although the underlying mechanism leading to black esophagus is unknown, clinicians caring for patients with black esophagus should focus on optimizing perfusion, minimizing acid reflux, and treating esophageal infection if present.     

Vagal afferent dysfunction in naturally occurring canine esophageal motility disorder

Few studies have examined the vagal afferent innervation of the esophagus in naturally occurring esophageal motility disorders. The present study assessed the integrity of distension-sensitive vagal afferents innervating the esophagus in naturally occurring canine megaesophagus. In the dog, esophageal distension induces reflex inhibition of crural diaphragm electromyographic activity that is mediated by vagal afferents innervating esophageal mechanoreceptors. This reflex was measured during stepwise esophageal distension in six dogs with congenital idiopathic megaesophagus, two dogs with megaesophagus secondary to esophageal striated muscle disease, and eight matched controls. In contrast to control dogs, inhibition of crural electromyographic activity was not observed in megaesophagus dogs with esophageal distension within the control volume range. With esophageal distensions far in excess of the control volume range, inhibition of crural electromyographic activity was not observed in five of six dogs with congenital idiopathic megaeosphagus, while crural inhibition was observed in the two dogs with secondary megaesophagus. These findings indicate that a defect is present in the vagal afferent innervation to the esophagus in a majority of dogs with congenital idiopathic megaesophagus.The present study was funded by a Clive and Vera Ramaciotti Foundations research grant. Christopher T. Holland was the recipient of a Jean Walker Trust Fellowship from the University of Sydney.     

Tuberculous fistulas of the pharynx and esophagus

Four patients with tuberculous fistulas communicating with the pharynx or the esophagus are reported. In 1 patient, there was strong evidence to suggest primary involvement of the esophageal mucosa. The other 3 cases were related to involvement of the pharynx or the esophagus from adjacent tuberculous process, as confirmed by histopathological proof. The patients had varying degrees of symptoms, which in two dramatically responded to antituberculous therapy; the third patient needed surgery for complete cure and the last patient was lost to follow-up.     

Lugol staining pattern in background epithelium of patients with esophageal squamous cell carcinoma

BACKGROUND/AIMS: Squamous cell carcinoma of the esophagus often arises in the setting of chronic esophagitis. We investigated whether chronic esophagitis was associated with carcinogenesis in the esophageal squamous epithelium. METHODOLOGY: Videoendoscopy with Lugol staining was performed in 70 patients with invasive carcinoma of the esophagus. We especially focused the study on background epithelium of the esophagus, then background epithelium was classified into two groups according to differences in Lugol staining patterns. Following Lugol solution spraying, background epithelium showing uniform greenish-brown staining was defined as having a uniform pattern. In contrast, when multiple Lugol-unstained speckles were present throughout the esophagus, the pattern was defined as speckled. Furthermore, we also investigated whether glycogenic acanthosis is present or not in background epithelium. RESULTS: Chronic esophagitis was present in 11 of 70 patients (16%) with invasive carcinoma, indicating a speckled pattern in background epithelium following Lugol solution spraying. The remaining 84% of patients with invasive carcinoma showed normally uniform Lugol staining background epithelium. Glycogenic acanthosis was found in 65 (93%) of 70 patients. CONCLUSIONS: Approximately 80% of patients with esophageal squamous cell carcinoma showed normal Lugol staining of background epithelium. Field carcinogenesis is postulated to be not predominant in the development of esophageal squamous cell carcinoma in our Japanese subjects. In contrast, glycogenic acanthosis of the esophagus was associated with the background epithelium accompanied with esophageal squamous cell carcinoma.     

A case of acute esophageal necrosis (black esophagus) in a young man with Down syndrome

Acute esophageal necrosis, commonly referred to as ‘black esophagus?, is a rare clinical entity arising from a combination of ischemic insult, corrosive injury and decreased function of mucosal barrier systems and reparative mechanisms present in debilitating diseases. We describe the case of an 18-year-old man affected by Down syndrome, presenting with a streptococcus β-hemolytic group A infection of the upper airways. Although the patient was intensively treated with antibiotics, he developed a streptococcal toxic shock-like syndrome and died 5 days after admission. At autopsy, circumferential black discoloration of the esophageal mucosa that extended along the entire esophagus and ended abruptly at the esophageal-gastric junction was found. Neither ulceration nor esophageal perforation were present. Black esophagus is well known to the gastroenterology community, whereas it has been described only twice in the pathology literature. To the best of our knowledge, this is the first case ever reported complicating a streptococcal infection.     

Esophageal function in progressive systemic sclerosis.

A prospective study was performed in 13 consecutive patients with systemic progressive sclerosis (PSS). For the diagnosis of impaired esophageal peristalsis cineradiography and manometry are equally useful. Esophageal suction biopsy allows the diagnosis of esophagitis but not of scleroderma. Mild to severe esophageal involvement was observed in 12 patients. In only one patient the esophagus was virtually normal. Dysfunction of the esophageal body may occur early in the course of the disease while incompetence of the lower esophageal sphincter is observed on an average after 7 to 8 years. Both impairment of peristalsis and pressure of the lower esophageal sphincter may lead to delayed esophageal clearance. Relaxation of LES is normal even in the absence of primary peristalsis. Extensive esophageal damage including severe gastroesophageal reflux may be present in the absence of esophageal symptoms.     

Natural progression of the lower esophageal mucosal ring

Among 390 patients with endoscopically verified lower esophageal mucosal ring (LEMR), 22 cases were identified with previous or subsequent radiologic examinations of the esophagogastric region. Among these, it was found that 2 cases of LEMR had developed from a normal esophagus. In 3 patients, there was increasing stenosis of the LEMR. In 8 cases, the LEMR was transformed into an esophageal stricture. In 10 of the 13 cases, esophagitis of varying degree was present endoscopically. In the 9 patients exhibiting no change in the LEMR, only 1 patient had esophagitis. The data suggest that there is a potential progression from normal esophagus to lower esophageal ring to esophageal stricture that occurs in association with reflux esophagitis.     

Histopathological Analysis of Esophageal Mucosa in Patients with Achalasia

Background/AimsAchalasia is an esophageal motor disorder that leads to functional esophageal obstruction. Food stasis and bacterial fermentation can predispose an individual to esophageal mucosal inflammation, causing multifocal dysplasia and increasing the risk of developing esophageal squamous cell carcinoma. We aimed to evaluate esophageal mucosal alterations in achalasia patients and determine clinical factors associated with the histopathological findings.MethodsFrom 2009 to 2013, we obtained endoscopic biopsies from the lower and middle esophagus of 22 patients with achalasia and 17 controls. Patients’ clinical data and histological severity of esophagitis were retrospectively analyzed. Additionally, immunohistochemical staining for CD3, CD20, Ki-67, and p53 was conducted.ResultsThe median age of achalasia patients was 49.5 years (range, 27 to 82 years), and there were nine males (40.9%). The median symptom duration was 5.8 years (range, 1 to 33.5 years), and 10 patients (45%) underwent previous treatment (nine, balloon dilation; one, botulinum toxin injection). Achalasia patients had significantly more severe esophagitis than did controls (p=0.001, lower esophagus; p=0.008, middle esophagus), and the number of CD3-positive lymphocytes exceeded that of CD20-positive lymphocytes (p<0.001). Achalasia patients also had a higher esophageal Ki-67 proliferation index (p=0.048). Although statistically nonsignificant, p53 expression was only observed in achalasia patients. There was no association between the histological severity of esophagitis and other clinicopathological findings.ConclusionsAchalasia patients showed significantly severe histological esophagitis and a high Ki-67 proliferation index, indicating an increased risk of neoplastic progression. Therefore, careful endoscopic inspection is necessary for the early detection of superficial neoplasia in these patients. (Gut Liver 2021;15-722)     

High risk of malignancy in familial Barrett's esophagus: presentation of one family

Barrett's esophagus is an acquired condition fundamentally related to the presence of severe and prolonged pathologic acid and biliary gastro-esophageal reflux. However, genetic factors may also play a role in some cases. The aim of this study is to present 3 generations of a Spanish family with the largest number of members so far reported with Barrett's esophagus or esophageal adenocarcinoma. Of the 24 members of this family studied over 3 generations, 6 patients developed esophageal adenocarcinoma, 4 Barrett's esophagus, 6 clinical symptoms of gastro-esophageal reflux disease without Barrett's esophagus, and 8 were asymptomatic. In conclusion, patients with familial Barrett's esophagus get the disease more severely with a high rate of malignancy and, therefore, the endoscopic surveillance should be closer than in cases of nonfamilial Barrett's esophagus.