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.     

The Los Angeles and Savary–Miller systems for grading esophagitis: utilization and correlation with histology

The aim of the study is to determine the proportion of patients who have esophageal biopsy specimens taken for an endoscopic diagnosis of reflux esophagitis in which an endoscopic grade of esophagitis (Los Angeles [LA] or Savary–Miller [SM]) is communicated to the pathologist, and to evaluate the correlation between those endoscopic grades and histopathologic findings. We searched the database of Caris Diagnostics (a large, gastrointestinal pathology practice that receives specimens from community‐based endoscopy centers), and extracted data from all patients who had an endoscopy with esophageal biopsies submitted in a 12‐month period. There were esophageal biopsy specimens from 49 480 patients obtained during 58 986 endoscopies. The LA grade was provided in 5513 cases (27.9% of 19 778 with endoscopic esophagitis); the SM grade was stated in only 2416 cases (12.2%). A histopathologic diagnosis of erosive or ulcerative esophagitis was made significantly less often in LA grade A patients (3.2%) than in those with LA grades C (20.0%) and D (23.3%); erosive or ulcerative esophagitis was found in only 1.4% of patients with SM grade I and in 35.5% of cases with grade IV. Endoscopists who biopsy the esophagus of patients with reflux esophagitis usually do not communicate the grade of esophagitis to the pathologist. Although both the LA and SM grading systems are based on the presence of esophageal mucosal breaks (erosions or ulcers), in practice such breaks are documented in only a minority of esophageal biopsy specimens taken from patients with reflux esophagitis of any grade.     

Mucosal cancer of the esophagus that presented significant changes of endoscopic findings in repeated observations

We report our experience of a very unique case with superficial esophageal cancer presenting significant changes of endoscopic findings within 2 months. A 60‐year‐old man was referred to our hospital because of abrupt and severe chest pain. Upper gastrointestinal endoscopy demonstrated a shallow depressed lesion covered with whitish slough at the middle thoracic esophagus, which was identified as an unstained area by iodine dye spray. In the lower thoracic esophagus, we did not detect any abnormality during initial endoscopy. Although we diagnosed this lesion as atypical esophagitis, histological examination of the biopsy specimen confirmed squamous cell carcinoma. Furthermore, the endoscopic appearance showed dramatic changes over 2 months. The initial lesion at the middle thoracic esophagus gradually diminished, while the mucosa became slightly clouded in the lower thoracic esophagus. This cloudy area became unstained after iodine dye spray. The unstained area of the lower thoracic esophagus gradually spread. We performed four endoscopic mucosal resections separately in 4 days over a period of 5 months. All of the specimens were shown to be squamous cell carcinoma on histological examination. The patient is on endoscopic surveillance and over a period of 1 year, there has been no recurrence.     

Acute upper gastrointestinal bleeding from multiple Barrett's ulcers associated with metachronous multiple colorectal carcinomas

Although gastroesophageal reflux disease is sometimes associated with esophageal ulcer and/or mucosal erosion, acute upper gastrointestinal bleeding from an esophageal ulcer is uncommon. We report a case of acute gastrointestinal bleeding from one of multiple esophageal ulcers in extensive Barrett's esophagus in the postoperative period after low anterior resection performed for descending colon carcinoma. A 74-year-old Japanese woman had undergone sigmoid colon resection 6 years earlier. The patient had a history of repeated reflux esophagitis. She was referred to a local hospital for a simple health screening 5 years after surgery. The patient had noticed bloody stool and felt mild difficulty at defecation 2 weeks before admission. Lower gastrointestinal endoscopy performed at a local hospital revealed a type 2 tumor located approximately 15 cm from the anal verge, which was obviously the cause of the bloody stool and constipation. The patient was admitted to our hospital for surgical treatment. Ten days after the low anterior resection, upper gastrointestinal bleeding occurred. Upper gastrointestinal endoscopy revealed multiple ulcers in the lower esophagus, which had caused the bleeding. Endoscopic biopsy revealed that esophageal ulcer occurred in the Barrett's esophagus, extending 15 cm from the functional esophagogastric junction. This case highlights acute upper gastrointestinal bleeding from multiple Barrett's ulcers in extensive Barrett's epithelium occurring in the postoperative period of colorectal carcinoma, and indicates an association of Barrett's esophagus with metachronous multiple colon carcinoma.     

Difference in localization of esophageal mucosal breaks among grades of esophagitis

BACKGROUND: Gastroesophageal reflux occurs mainly during the daytime in patients with Los Angeles grade A esophagitis, but predominantly during the night in patients with grade C and D esophagitis. The purpose of the present paper was to investigate whether this difference in the pattern of gastroesophageal reflux influences the circumferential localization of erosions in the esophageal wall. METHODS: The subjects were 394 consecutive patients diagnosed endoscopically as having reflux esophagitis (grade A, n = 223; B, n = 93; C, n = 53; D, n = 25 cases). Their endoscopic films were reviewed retrospectively to determine the circumferential location of esophageal mucosal breaks, and also the prevalence and size of hiatal hernia (HH). RESULTS: The numbers of mucosal breaks analyzed in patients with grade A, B and C esophagitis were 321, 173 and 54, respectively. Patients with grade A and B esophagitis had longitudinal mucosal breaks mainly in the right-anterior wall of the lower esophagus, whereas patients with grade C esophagitis had transverse mucosal breaks mainly in the posterior wall. The prevalence and size of HH was significantly higher and larger, respectively, in patients with grade C or D esophagitis than in those with grade A and B esophagitis. CONCLUSION: The circumferential location of esophageal mucosal breaks differs significantly among different grades of esophagitis.     

Acute necrotizing esophagitis: role of nonsteroidal anti-inflammatory drugs

Background Acute necrotizing esophagitis (ANE) is a rarely described entity that is thought to be a cause of upper gastrointestinal (UGI) bleeding, The present study examined the incidence of ANE among patients with UGI bleeding, as well as the clinical features of ANE, and the coexisting illnesses and medication histories of ANE patients. Methods A retrospective analysis of clinical and endoscopic findings and the clinical course in 16 patients with ANE was carried out over a 3-year period. Results We observed 16 patients (6%) of ANE in 239 patients with UGI bleeding during the 3-year period. The average age of the patients was 62.5 years. The lesions predominantly affected the lower third of the esophagus, and hiatal hernia was the most common (63%) coexisting endoscopic finding. All patients had coexisting disease. Fifty percent of patients with ANE (eight patients) had taken nonsteroidal anti-inflammatory drugs (NSAIDs). ANE also occurred in four patients with diabetic ketoacidosis. Supportive therapy, including parenteral nutrition and administration of a proton pump inhibitor, was effective. Conclusions ANE is more common than has been previously reported, and it should be included in the differential diagnosis of UGI bleeding. ANE could be characterized as an “acute esophageal mucosal lesion,” particularly in aged patients with hiatal hernia and among those who consume NSAIDs.     

Update on endoscopic diagnosis, management and surveillance strategies of esophageal diseases

In the last few decades, upper gastrointestinal endoscopy has become the most complementary test for investigation of esophageal diseases. Its accessibility and safety guarantee wide clinical utilization in patients with suspected benign and malignant diseases of the esophagus. Recent technological advances in endoscopic imaging and tissue analysis obtained from the esophagus have been useful to better understand and manage highly relevant diseases such as gastroesophageal reflux disease, eosinophilic esophagitis and esophageal cancer. Using endoscopy to elucidate esophageal disorders in children has been another field of intensive and challenging research. This editorial highlights the latest advances in the endoscopic management of esophageal diseases, and focuses on Barrett’s esophagus, esophageal cancer, eosinophilic esophagitis, as well as esophageal disorders in the pediatric population.     

Esophageal atresia: metaplasia,Barrett

Barrett's esophagus is characterized by the replacement of squamous epithelium by columnar epithelium that is intestinal metaplasia‐positive or ‐negative in the distal esophagus. Gastroesophageal reflux disease, which is frequent and prolonged in esophageal atresia, probably plays a major role in the development of Barrett's esophagus through repeated mucosal damage. Long‐term acid exposure contributes to carcinogenesis in Barrett's esophagus of intestinal type, but its effect on gastric metaplasia is less well defined. Recent studies have suggest that metaplasia arises in about 15% of patients with esophageal atresia, with a lag time to developing metaplasia from initial surgical correction of about 10 years. Preliminary data from an ongoing multicenter study including 88 patients with esophageal atresia aged 15–19 years showed gastric metaplasia in 42% of patients (29 fundic and 7 cardial metaplasia), while one patient presented intestinal metaplasia. Esophageal mucosal abnormalities can be observed in esophageal atresia patients at endoscopy despite the absence of symptoms. Whether prolonged, aggressive, acid suppression is beneficial in these situations remains to be determined. Barrett's metaplasia can be removed by endoscopic mucosal resection or destroyed with endoscopic ablative techniques, such as photodynamic therapy, radiofrequency ablation, and cryotherapy. The risk of developing esophageal carcinoma is still a controversial issue as only a few clinical cases have been reported in young adults with esophageal atresia. As late complications of esophageal atresia, particularly esophagitis and Barrett's esophagus, are increasingly being recognized, long‐time systematic follow up of the esophageal mucosa including multistage biopsies is therefore required even in asymptomatic patients.     

AIDS病人消化道黏膜病变内镜及病理特征研究

目的研究艾滋病(AIDS)病人消化道黏膜病变的内镜及病理特征。方法对北京地坛医院1995-2009年期间收治的AIDS病人中,经临床、内镜、病理确诊并发食道、肠道黏膜病变的62例病例资料做回顾性分析。结果 62例中,31例食道黏膜病变病人胃镜像显示不同程度的黏膜弥漫性充血、水肿,点片状白斑、白色伪膜或条索样、絮状真菌团块(6例并发食管黏膜溃疡)。其中16例病人病理检测到真菌菌丝或孢子而被确诊真菌性食管炎。33例肠道黏膜病变病人(2例同时合并食道黏膜病变)结肠镜像显示,除2例黏膜未见明显异常外,余31例均有结肠黏膜病变。慢性结肠炎14例,慢性结肠炎伴结肠溃疡15例,阿米巴性溃疡和回盲部恶性淋巴瘤(非霍奇金淋巴瘤)各1例。病理学特点:急慢性食管、结肠炎,主要表现为黏膜组织疏松水肿,伴炎性细胞浸润,而溃疡除炎性病理改变外,伴有黏膜局灶、片状坏死,中性粒细胞浸润,呈非特异性炎症改变。结论 AIDS病人合并消化道黏膜病变肠道溃疡的发生率要高于食道。肠道黏膜病变以慢性结肠炎和结肠溃疡为主,同时可并发恶性肿瘤。     

Does magnifying endoscopy improve the diagnosis of erosive esophagitis?

Background and Aims:  Low-grade erosive esophagitis (i.e. Los Angeles grade A) is the most predominant type of esophagitis in Japan. It is unclear whether all the mucosal breaks detected by conventional endoscopy are indicative of esophageal mucosal erosion. Hospital-based, cross-sectional, cross-over, observational study was assigned to investigate the value of magnifying endoscopy for diagnosis of erosive esophagitis.
Methods:  From August to December 2006, 178 consecutive patients with upper gastrointestinal symptoms were enrolled at three university hospitals and one national medical center in western Japan. Before endoscopy, all participants were requested to answer questionnaires concerning their symptoms. Experienced endoscopists performed an endoscopic diagnosis of each patient first with a conventional standard view and then with a magnifying view. Endoscopic diagnostic concordance between conventional and magnifying endoscopic view for erosive esophagitis was calculated. Relationship between a variety of symptoms and erosive esophagitis was also evaluated.
Results:  Erosive esophagitis was identified using conventional and magnifying endoscopy in 14.6% and 17.4% of patients, respectively. Eleven false-negative and six false-positive diagnoses using conventional endoscopy occurred among the 178 enrolled patients. The weighted kappa value of diagnostic concordance for erosive esophagitis between the two endoscopic views was 0.76. The prevalence of erosive esophagitis in patients with reflux-, dysmotility-, and ulcer-like symptoms was 20.7%, 24.1%, and 15.2%, respectively.
Conclusions:  Magnifying endoscopy did not significantly improve the diagnostic sensitivity of erosive esophagitis over non-magnifying, conventional endoscopy. Erosive esophagitis was frequently identified in patients that did not have reflux symptoms.     

ACUTE ESOPHAGEAL NECROSIS

Acute esophageal necrosis (AEN) is defined as a diffuse blackened aspect of the esophagus observed by upper gastrointestinal endoscopy associated with the histopathological findings of necrosis of the esophageal mucosa. In general, the condition is present in severely compromised patients. Its cause remains unknown but is probably multifactorial. In the present case report we describe the clinical, endoscopic and histopathological characteristics of black esophagus observed in three patients and present a review of the literature.     

Intermittent Dysphagia for Solids Associated with a Multiringed Esophagus: Clinical Features and Response to Dilatation

The entity of the multiringed esophagus, generally presenting in adults as intermittent dysphagia for solids, is relatively uncommon and its pathogenesis is unknown. The goal of this study was to describe the demographic, clinical, and endoscopic features of patients presenting with this condition, their response to esophageal dilatation, and the relationship of multiple esophageal rings to eosinophilic esophagitis. Between 1989 and June 2004, 32 patients at this adult hospital fulfilled the following inclusion criteria: (1) intermittent dysphagia for solids, (2) multiple esophageal rings at endoscopy, and (3) esophageal dilatation(s) performed. Response to esophageal dilatation was measured by need for subsequent dilatations. Seventy-five percent of the patients were male. Median age at onset of dysphagia was 21 years and at presentation 36.5 years. All had multiple rings in the proximal or midesophagus on endoscopy and had undergone a total of 73 esophageal dilatations with no esophageal perforations. Median maximal dilator size was 15 mm; however, 16% developed significant esophageal mucosal tears even with 11-mm dilators. Sixty-six percent required repeat dilatation, with the median time interval before recurrence being 8 months. Eosinophilic esophagitis (mucosal eosinophil count > 20/HPF) was present in 50% of this cohort. From this study we conclude that a multiringed esophagus causing intermittent dysphagia occurs predominantly in young males, responds well to dilatation, but repeated dilatations are often necessary. Dilatation can lead to extensive mucosal tears and should be performed with caution. Eosinophilic esophagitis is commonly but not invariably associated with this entity. Frequent relapse of dysphagia highlights the need for effective pharmacotherapy.     

Gastrointestinal Hemorrhage Due to Gastrointestinal Mycobacterium avium intracellulare or Esophageal Candidiasis in Patients with the Acquired Immunodeficiency Syndrome

A 33-yr-old black intravenous drug abuser with the acquired immunodeficiency syndrome (AIDS) had a massive fatal upper gastrointestinal hemorrhage due to profound and diffuse esophageal ulceration from Candida , as demonstrated by postmortem examination. A 2-yr-old white male with congenitally acquired AIDS had a massive fatal esophageal bleed as a result of esophagitis from Candida albicans , as proven by pathologic examination and culture of endoscopic biopsies. A 27-yr-old black human immunodeficiency virus-sero-positive female died from massive lower gastrointestinal bleeding due to extensive small and large intestinal ulceration caused by Mycobacterium avium intracellulare , as proven by microscopic examination and myco-bacterial culture of intestinal tissue. These reports extend the clinical spectrum of these infections in AIDS patients by demonstrating that these infections can produce gastrointestinal bleeding.     

Œsophagite nécrosante aiguë : une complication rare

IntroductionAcute esophageal necrosis (AEN) is a rare medical disorder, which is characterized by a diffuse black esophageal mucosal during upper gastrointestinal endoscopy which is a highly recommended diagnostic tool. Its high mortality rate requires to be quickly evocated and an early management.Case reportWe report a case of a 93-year-old patient with upper gastrointestinal bleeding. The upper endoscopy shows a grade D AEN according to the Los Angeles classification. Treatment consists of a parenteral nutritional support and an intravenous proton pump inhibitors treatment, which increase chances of a favorable outcome on endoscopic controls at 2 and 6 weeks.ConclusionAEN has to be quickly evocated in a polyvascular and old patient with upper gastrointestinal bleeding. Our experience confirms that optimal and early management allow a esophageal complete healing at 6 weeks.     

急性非静脉曲张性上消化道出血临床分析

目的分析非静脉曲张性上消化道出血的临床特征。方法回顾分析我院消化内科2009年1月-2011年12月期间收治的经胃镜证实的301例非静脉曲张性上消化道出血病例,分析总结非静脉曲张性上消化道出血的常见病因及临床诊治情况。结果非静脉曲张性上消化道出血的常见病因依次为消化性溃疡、消化道肿瘤、急性胃黏膜病变,内科保守治疗的有效率为96.35%(290/301),内镜下止血成功率93.55%(58/62)。结论消化性溃疡是非静脉曲张性上消化道出血最常见的病因。在消化道出血的救治中,急诊内镜、选择性血管造影以及内外科的紧密配合与协作发挥着重要作用。     

Endoscopic resection of esophageal leiomyoma with elastic band ligation

Leiomyoma is the most frequent esophageal benign tumor. It represents 70% of these tumors and 1 to 8% of all esophageal tumors. The majority of cases are asymptomatic and are discovered by chance in endoscopic examinations, and a conservative management is adopted. An endoscopic or surgical treatment can be applied in symptomatic cases, basically depending on the size and the underlying layer. OBJECTIVE: To report the endoscopic resection of an esophageal leiomyoma by using an elastic band ligation. Case report: A 55 year-old man complained of prolonged upper dysphagia with solids during three months. An upper gastrointestinal endoscopy was performed and an elevated lesion of 1 cm in diameter was found in the esophagus, at 25 cm from the upper dental arcade, with smooth surface and adjacent areas of normal mucosa. It was interpreted as a submucosal lesion. A hypoechoic mucosal and submucosal formation with well delimited borders was observed in an endoscopic ultrasonography. The lesion was resected by using an endoscopic band ligation and a subsequent section with a polypectomy snare. The patient evolved uneventfully and was discharged in 24 hours. The endoscopic examinations during the follow-up showed the healing of the resulting scar. Pathologists diagnosis was esophageal leiomyoma. CONCLUSION: The elastic band ligation is a simple, safe, effective and cheap method to be taken into account for the treatment of mucosal and submucosal esophageal lesions.     

Acute esophageal necrosis: a rare syndrome

Background Acute esophageal necrosis, which presents as a black esophagus on endoscopy, is a rare disorder that is poorly described in the medical literature. In this study, we analyze all cases reported to date to define risk factors, clinical presentation, endoscopic features, histologic appearance, treatment, complications, outcome and etiopathogenesis of the disease and to describe a distinct medical syndrome and propose a staging system. Methods We searched Medline and PubMed from January 1965 to February 2006 for English-language articles using the key words “acute esophageal necrosis,” “necrotizing esophagitis,” and “black esophagus.” Results A total of 88 patients were reported in the literature during the 40 years, 70 men and 16 women with an average age of 67 years. Patients were generally admitted for gastrointestinal bleeding and cardiovascular event/shock. Patients presented with hematemesis and melena in more than 70% of the cases. Upper endoscopy showed black, diffusely necrotic esophageal mucosa predominantly affecting the distal third of the organ. Necrosis was confirmed histologically in most cases. Complications included strictures or stenoses, mediastinitis/abscesses, and perforations. Overall mortality was 31.8%. Conclusions This study provides a structured approach to identifying risk factors, diagnosis, and pathogenesis of the acute esophageal necrosis. Risk factors include age, male sex, cardiovascular disease, hemodynamic compromise, gastric outlet obstruction, alcohol ingestion, malnutrition, diabetes, renal insufficiency, hypoxemia, hypercoagulable state, and trauma. Mechanism of damage is usually multifactorial secondary to ischemic compromise, acute gastric outlet obstruction, and malnutrition. Overall, acute esophageal necrosis should be viewed as a poor prognostic factor, associated with high mortality from the underlying clinical disease.     

返流性食管炎的内镜探讨

本文报告近５年我院内镜检出返流性食管炎２３７例，临床症状主要为烧心、上腹痛、腹胀、呕血、黑便及吞咽困难。食管裂孔疝和十二指肠球部病变为主要的消化系统伴随疾病。内镜表现食管粘膜红斑者占９．７％。孤立性或非融合性糜烂者占２９．１％。环周性或融合性糜烂者占３４．２％、溃疡形成者占２７．０％，且有３．４％发生食管下端狭窄。资料提示本病我国并不少见，发病明显与贲门功能不全和十二指肠病变有关。     

Gastroesophageal Reflux Disease in the Elderly: More Severe Disease That Requires Aggressive Therapy

Objective: To evaluate gastroesophageal reflux disease in the elderly (people ≥ 60 yr). Methods: Basal gastric-acid secretion was prospectively determined in 228 consecutive patients with symptomatic gastroesophageal reflux disease who had upper gastrointestinal endoscopy and were diagnosed with either pyrosis alone (n = 98), erosive esophagitis (n = 87), or Barrett's esophagus (n = 43). Results: Patients ≥ 60 yr (n = 66) had significantly more esophageal mucosal disease (erosive esophagitis, Barrett's esophagus) than patients < 60 yr (n = 162)- 81% versus 47% ( p = 0.000002, Fisher's exact test). Furthermore, 87% of patients ≥ 70 yr had esophageal mucosal disease. For each decade from < 30 yr to ≥ 70 yr, there was a significant increase in esophageal mucosal disease ( p = 0.002; X ² test, 23.96); however, there were no significant differences in severity of pyrosis symptoms or in mean basal acid output for each decade. When 146 of the 228 patients with gastroesophageal reflux disease were given enough ranitidine (mean, 630 mg/d; range, 300–3000 mg/d) for the relief of all pyrosis symptoms and healing of all esophageal mucosal disease, there were no significant differences in ranitidine therapy between each decade. Conclusions: Elderly patients with pyrosis symptoms severe enough to require upper gastrointestinal endoscopy have gastroesophageal reflux disease with more esophageal mucosal disease (erosive esophagitis, Barrett's esophagus) than patients < 60 yr, and like younger patients, may require markedly increased doses of ranitidine as large as 2400 mg/d for effective therapy.     

Endoscopic findings and esophageal cancer incidence among Fanconi Anemia patients participating in an endoscopic surveillance program

Background and aims

The primary clinical characteristics of Fanconi Anemia (FA) include typical physical features, progressive bone marrow failure, and an increased incidence of neoplasms, including esophageal carcinoma. Currently, there are no data regarding endoscopic findings or the interval time to malignancy in these patients. Data about the contribution of Human Papilloma Virus (HPV) to esophageal carcinoma is conflicting. Our objective is to document the upper gastrointestinal (GI) findings at baseline, document cancer incidence, and evaluate the role of HPV among these cancers.