Dilated Distal Esophagus Research Articles

Herpetic oesophagitis in an immunocompetent infant with gastroesophageal reflux

A 7-month-old boy presented to our emergency department because of persistent vomiting with streaks of blood despite esomeprazole treatment for gastroesophageal reflux for 3 months. Additionally, he refused solid food and showed failure to thrive (decrease of two standard deviation scores for weight-for-age). The boy's mother was known to have recurrent herpes labialis. Abdominal ultrasound showed no pyloric hypertrophy, but a barium swallow radiogram revealed a dilated distal oesophagus with a pinpoint stenosis (figure A). Oesophagogastroscopy showed extensive inflammation with whitish exudate and friable mucosa of the distal part of the oesophagus (figure B; video), as well as stenosis of the lower oesophageal sphincter. During oesophagogastroscopy, the stenosis was dilated to 8 mm and multiple biopsies were taken. Histopathological examination revealed features typical for herpes simplex virus oesophagitis (HSVE; figure C), which was confirmed by herpes simplex virus (HSV) immunohistochemistry (figure D). Moreover, HSV-1 was detected by PCR analysis. Additional laboratory tests revealed normal levels of T, B, and NK lymphocyte subpopulations and immunoglobulins. The patient was treated with valaciclovir (30 mg/kg per day in two doses) for 10 days, high-dose omeprazole (2 mg/kg per day in one dose), and nasogastric tube feeding. 2 weeks after initiation of treatment, the patient was symptom free and gained weight. 4 weeks after valaciclovir treatment, oesophagogastroscopy showed remarkable improvement with complete disappearance of mucosal inflammation, but clearly visible was a mild sliding hiatal hernia. Nasogastric tube feeding was discontinued after the endoscopy and omeprazole treatment was continued to treat reflux oesophagitis.

New evidence defining the pathology and pathogenesis of lower esophageal sphincter damage

SummaryBackgroundPresent diagnosis and management of gastroesophageal reflux disease (GERD) has resulted in a dramatic increase in the incidence of esophageal adenocarcinoma. This is due to failure to identify pathologic changes of early GERD; at present, pathology is limited to management of Barrett esophagus (BE).MethodsConvincing evidence have confirmed that cardiac mucosa distal to the squamocolumnar junction in the endoscopically normal person is a metaplastic GERD-induced esophageal epithelium, and not a normal proximal gastric epithelium.ResultsWhen cardiac mucosa is recognized as a metaplastic esophageal epithelium, it becomes self-evident that the present endoscopic definition of the gastro-esophageal junction is incorrect, and there exists a dilated distal esophagus (DDE) in what is incorrectly termed the “gastric cardia” presently mistaken for proximal stomach. It also becomes clear that the length of the DDE correlates with the presence and severity of GERD and represents the pathology of the entire spectrum of GERD. Further, it allows recognition that the DDE, measured as the gap between esophageal squamous epithelium and gastric oxyntic mucosa that is composed of cardiac mucosa, represents the pathologic anatomy of damage to the abdominal segment of the lower esophageal sphincter (LES).ConclusionThe new understanding of the significance of cardiac mucosa provides a new and highly accurate histologic method of assessment of LES damage, the primary cause of GERD. This opens a new door to complete histologic assessment of GERD from its etiologic standpoint and to new research that permit early diagnosis of GERD at its outset. Ultimately, such early diagnosis has the potential to reverse the increasing trend of esophageal adenocarcinoma.

1821 Post-Fundoplication Esophageal Bezoar Resolved With Endoscopic Instillation and Oral Ingestion of Cola

INTRODUCTION: We present a difficult bezoar-like esophageal food impaction which occurred 6 months after repair of a giant Type IV hiatal hernia. CASE DESCRIPTION/METHODS: The patient is an active 90-year-old female who had a giant Type IV paraoesophageal hernia containing stomach and colon for many years. She underwent repair with mesh and a loose Nissen fundoplication (58 Fr). Pre-operative esophageal manometry was not performed. Post-operative esophagram showed intact wrap, with “3 mm minimum diameter central luminal channel through the fundoplication.” Preserved esophageal motility was also noted. Recovery was uneventful. Six months later, she presented to the ED complaining of sensation of food stuck in her throat. She was tolerating secretions and tiny sips of water. Barium esophagram revealed “dilated distal esophagus, with contrast pooling above retained material at the GE junction”. EGD was performed under general anesthesia. The distal esophagus was completely obstructed with food. Despite use of multiple tools including net, rat-tooth forceps and pronged graspers, it could not be cleared. Using small instillations of cola through a 60 cc syringe, we were able to “tunnel” through the impaction and create a lumen into the stomach. Retroflexion showed a small hiatal hernia. After more than 2 hours, there was still a significant amount of food adherent to the walls of the esophagus. The patient was extubated and admitted to the hospital. She was instructed to drink small sips of cola and ice chips upright in bed. She ingested 2 L of cola over the next 8 hours without difficulty. EGD the next day noted complete passage of food. There was no frank ring or stricture. Fundoplication was intact with a small(2 cm) hiatal hernia. She was discharged the same day on soft diet. At follow up with thoracic surgery she had no further dysphagia episodes. She was instructed to drink cola once a day for maintenance. DISCUSSION: Although the patient had a loose wrap and no known dysmotility, post-op diameter of the esophageal lumen was fairly small (3 mm). If patient was not adhering to post-fundoplication diet, this likely precipitated “packing” of food into the distal esophagus and remnant small hernia. Removal of esophageal food impaction is most commonly done by pushing food into the stomach, or removing it mechanically. The use of proteolytic enzymes e.g. papain is not recommended due to perforation risk. One can consider adding cola to the arsenal of tools for safely treating difficult esophageal food impactions.

1839 From the Lap to the Lung: The Esophagus Is the Answer

INTRODUCTION: Esophageal pathologies are significant yet under-reported complications of laparoscopic adjustable gastric banding (LAGB). We report a case of LAGB-associated esophageal dilatation and dysmotility that resulted in multifocal aspiration pneumonia (PNA) and a lung abscess 8 years after LAGB. CASE DESCRIPTION/METHODS: A 48-year-old morbidly obese man who underwent LAGB in 2011 presented with productive cough, dyspnea, and pleuritic chest pain for 3 weeks. He also reported intermittent postprandial gastric reflux and regurgitation of food since his LAGB, which he had been managing with lifestyle modifications. In the Emergency Room, he was febrile to 102F with leukocytosis to 16.8k/uL. CT chest revealed a fluid-filled esophagus (Figure 1) and a thick-walled cavity in the right lower lobe with an air-fluid level, consistent with a lung abscess (Figure 2). Additional consolidations were seen in the bilateral upper lobes. An esophagram was done due to a suspicion for aspiration PNA, and this confirmed a dilated distal esophagus (Figure 3) with impaired peristalsis and nonpropulsive contractions indicative of dysmotility. The patient’s esophageal pathologies and multifocal PNA were determined to be complications from his LAGB. He completed 3 weeks of antibiotics and was discharged with bariatric follow up for revision or removal of his LAGB. DISCUSSION: LAGB has several advantages over other bariatric procedures including band adjustability, reversibility, and small surgical risk. Despite this, the prevalence of LAGB has declined significantly due to several long-term complications including esophageal pathologies. In fact, one study reported esophageal dysmotility in 68% and esophageal dilatation in 25% of patients 5-7 years after LAGB. Esophageal dilatation is thought to occur from an overly tight band and in patients who overeat and use their distal esophagus as a food reservoir. This results in unsynchronized esophageal contractions and, thus, dysmotility. These patients may report reflux, vomiting, and regurgitation, which put them at high risk for aspiration PNA and, more rarely, complications such as lung abscesses. Patients who present with aspiration PNA or lung abscesses require antibiotics, but esophageal pathology alone should lead to consideration of band deflation or removal in order to prevent future recurrences. This case of LAGB-associated esophageal dysfunction highlights the importance of long-term monitoring of LAGB patients, especially in the setting of continued reflux or regurgitation.

How the pathologist can aid in the assessment of gastroesophageal reflux disease.

To provide new concepts regarding the early pathologic changes of gastroesophageal reflux disease (GERD) that are associated with damage to the lower esophageal sphincter (LES). A body of evidence exists that cardiac mucosa is a metaplastic esophageal epithelium rather than a normal gastric epithelium. Recent studies in asymptomatic volunteers suggest a potential mechanism for cardiac metaplasia in the squamous epithelium of the esophagus. The concept that cardiac mucosa is esophageal, not gastric, suggests that the widely accepted endoscopic definition of the gastroesophageal junction (GEJ) is incorrect. I propose that the true GEJ is the proximal extent of gastric oxyntic epithelium. If there is cardiac mucosa lining proximal rugal folds, that cardiac mucosa-lined region is the dilated distal esophagus, not the proximal stomach. The dilated distal esophagus is the pathologic expression of damage to the abdominal segment of the LES. This concept suggests a new test for measuring damage to the abdominal LES and a new understanding of the disease of GERD based on the measured amount of LES damage. This opens the door to new research and change in objectives in the management of reflux disease from control of symptoms to prevention of complications such as Barrett's esophagus and adenocarcinoma.

A case report of achalasia

Achalasia is characterized by esophageal nonperistaltic contraction and incomplete relaxation of the lower esophageal sphincter. The most common symptom are dysphagia, regurgitation, and heartburn. A 48 years old male was admitted into HAM General Hospital Medan with achief complaint of dysphagia since adulthood. The patient had to drink a lot of water to help swallow solid or soft food. Complaint worsened in the last three months followed by odynophagia, nausea, and vomiting undigested, retained food. The patient also complained about heartburn and was previously diagnosed with a variant of angina pectoris but his symptoms didn’t improve with medication. The patient had a history of weight loss but no anorexia, no prior history of corrosive ingestion. The patient was an active smoker. Physical examination revealed no abnormality. Oesophagogram showed dilated distal esophagus with rat tail appearance. Gastroscopy revealed dilatation on the lower third of the esophagus. Computed Tomography revealed dilatation of distal esophagus. Based on the results of the exams, we concluded the diagnosis as achalasia. The patient was treated with calcium channel blocker and proton pump inhibitor. The patient showed clinical improvement after treated and was discharged. The patient was planned for once a month follow up in the outpatient clinic.

Functional performance in late-onset GM2 gangliosidosis (Tay-Sachs and Sandhoff diseases), longitudinal data over 3 consecutive years

Anatomically, the normal state in the human adult is an esophagus that is entirely tubular and has a sharp transition to the saccular stomach where it forms an acute angle of His. Histologically, the normal esophagus is lined entirely by stratified squamous epithelium, transitioning at the gastroesophageal junction to gastric oxyntic epithelium, which lines the entire proximal stomach. Cardiac epithelium is normally absent. Its presence indicates the abnormal state of the dilated distal esophagus that results from damage to the abdominal segment of the lower esophageal sphincter. It is the early stage of gastroesophageal reflux disease (GERD) that is presently ignored and mistaken as normal proximal stomach. This normal state is not recognized at present time by the myth that 1–4 mm of cardiac epithelium normally exists between the distal end of the squamous epithelium and the proximal limit of gastric oxyntic epithelium. Evidence that eradicates this myth includes the facts: (1) The cardiac epithelium is absent in some people. (2) The presence of cardiac epithelium in the dilated distal esophagus is associated with the presence of submucosal esophageal glands. (3) Its extent varies from 0 to <15 mm in persons without GERD and increases up to 30 mm in patients with GERD. (4) The presence of any cardiac epithelium is associated with abnormal acid exposure, reflux esophagitis, and manometric abnormalities in the lower esophageal sphincter. (5) Cardiac epithelium extends into the thoracic esophagus as visible columnar-lined esophagus (CLE) in patients with severe GERD associated with severe lower esophageal sphincter damage. (6) The morphologic, immunohistochemical, and molecular features of cardiac epithelium in the dilated distal esophagus and visible CLE are identical and different than those of distal gastric epithelium. Eradication of the myth that cardiac epithelium is normally absent is essential to the understanding of the pathologic changes associated with the earliest stage of GERD. Existence of the myth at present prevents this understanding.

Spontaneous Intramural Hematoma of the Esophagus: An Unusual Case of Dysphagia and Mediastinal Mass

Introduction: Intramural esophageal hematoma (IEH) is an uncommon but potentially lethal cause of chest pain with accompanying dysphagia. While the majority of cases can be treated with conservative management, we present a case of a spontaneous IEH that later progressed to a symptomatic fibrotic, scared hematoma requiring surgical intervention. Case Report: A 41-year-old female presented to the ED with two days of progressively worsening, severe, non-radiating abdominal pain. She has associated nausea and non-bloody emesis. She denies having heartburn, dysphagia, distention or a change in her bowel habits. Labs show a mild leukocytosis of 10.7; otherwise chemistries, LFTs and lipase are all within normal limits. A KUB shows possible ileus and RUQ ultrasound was normal. CT shows a large mass in the posterior mediastinum with areas of high attenuation, measuring 11.4 x 7.8 x 5.6 in maximal cross sectional diameter (Fig. 1). It extends through the esophageal hiatus with a mass effect on the GE junction, proximal stomach and left atrium. EUS demonstrated an esophageal intramural mass in the wall of the esophagus with associated pericardial effusion. Fine needle aspiration of the mass was significant for gross blood along with inflammatory cells and abundant fibrotic debris (Fig. 2-3). At this time thoracic surgery was consulted to evaluate for potential surgical management of the mass. The patient underwent a left thoracotomy with exploration of the mediastinum where they found a fibrosed hematoma, which was excised. The patient remained in the hospital and was discharged several days later with resolution of symptoms.Figure: CT Chest showing dilated distal esophagus with Intramural Nodule.Figure: EGD showing intramural hematoma.Figure: EUS demonstrating intramural lesion found in the lower third of esophagus appearing to originate from within muscularis propia.Discussion: Intramural esophageal hematoma (IEH) is a rare cause of progressive, chest pain. The majority of cases are due to the use of anticoagulation, trauma, foreign body ingestion, or complications of instrumentation6. While the majority of cases resolve spontaneously, there is scarce literature dictating the management of persistent fibrotic hematomas. Surgical options include both open thoracotomy and video-assisted thorascopic surgery. We present this case in hopes to increase the breadth of knowledge regarding complicated IEH and demonstrate the room for future therapeutic endoscopic interventions opposed to invasive surgery. Finally, we hope to portray a case of a non-resolving IEH to elude on the potential benefit of early reimaging in patients with persistent symptoms.

Achalasia after Bariatric Roux-en-Y Gastric Bypass Surgery Reversal

Introduction: Achalasia is rare esophageal motility disorder that leads to loss of peristalsis in the distal esophagus and a failure of lower esophageal sphincter (LES) relaxation. It usually manifests as dysphagia for solids and liquids, and regurgitation of undigested food or saliva. The development of esophageal motility disorders including achalasia following bariatric surgery is still controversial and differ according to the type of surgery. Many cases of achalasia as a consequence of gastric band placement have been described. However, the development of achalasia and other motility disorders after Roux-en-Y gastric bypass (RYGB) are rare and only reported in some case reports. Case report: 70-year-old female patient with past medical history of morbid obesity presented to with complains of persistent nausea and regurgitation for the last 1 month. Her surgical history was significant for a laparoscopic cholecystectomy in addition to a laparoscopic Roux-en-Y gastric bypass (RYGB) approximately 2 years ago. Her surgery was revised 2 months prior to her presentation. Physical examination showed the wound of her recent surgery with no evidence of discharge or poor healing. Initial laboratory workup including complete blood count, comprehensive metabolic panel, and stool analysis were unremarkable. CT-scan of the abdomen and pelvis with contrast showed enteric contrast within the dilated distal esophagus, and was suspicious for mild stricture at gastro-esophageal sphincter (GES). Esophagogastroduodenoscopy showed a tortuous and dilated esophagus with large amounts of thick secretions. No strictures or stenosis was noted. Barium study was done afterward and showed persistent narrowing of the GES with a moderately dilated esophagus proximally and tertiary esophageal contractions. It was suggestive of achalasia. An outpatient manometry study revealed high LES pressure with abnormal relaxation, in addition to aperistalsis and those findings were consistent with type II achalasia. She was referred to surgery for possible laparoscopic heller's myotomy. Conclusion: We are presenting a rare case of achalasia after RYGB. As of our best knowledge there are only 3 cases reported for achalasia after RYGB and this is the first case described after revision of RYGB. The exact pathophysiology is not clearly understood, but local trauma due to surgery may play a role. An important consideration in similar cases is to rule out stenosis of the gastrojejunostomy.Figure 1Figure 2Figure 3

A New Pathologic Assessment of Gastroesophageal Reflux Disease: The Squamo-Oxyntic Gap.

Diagnosis of gastroesophageal reflux disease (GORD) is delayed by the lack of uniform histopathologic criteria for diagnosis. The only practical value of pathology is the assessment of columnar lined esophagus (CLO). As a result, GORD is treated with acid suppressive drug therapy until there is a failure to control symptoms and/or advanced adenocarcinoma develops. The reasons why there is a failure of pathologic diagnosis are two false dogmas that result in two widely believed fundamental errors. These are the belief that cardiac epithelium normally lines the proximal stomach (1) and that the gastroesophageal junction (GOJ) is defined by the proximal limit of rugal folds (2). When these false dogmas are eradicated by existing powerful evidence, the pathology of GERD falls into the following stages, all defined by histology: (a) The normal state where the esophageal squamous epithelium transitions at the GOJ to gastric oxyntic epithelium with no intervening cardiac epithelium; (b) cardiac metaplasia of the squamous epithelium due to exposure to gastric juice results in cephalad movement of the squamo-columnar junction (SCJ). This creates the squamo-oxyntic gap and the dilated distal esophagus, which is distal to the endoscopic GOJ. The length of the squamo-oxyntic gap in the dilated distal esophagus is concordant with the shortening of the abdominal segment of the lower esophageal sphincter (LOS); (c) in the early stages, the gap is <5 mm and the LOS retains its competence. Reflux is uncommon and patients are asymptomatic; (d) the squamo-oxyntic gap increases in length, concordant with the amount of shortening of the LOS, which becomes increasingly incompetent. At a gap length of 5-15 mm, reflux is sufficient to cause symptoms, but in most patients, symptoms are controllable and the patients are normal at endoscopy. The gap is entirely within the dilated distal esophagus, which is mistaken by present criteria for proximal stomach. (e) The last stage of GORD is when the squamo-oxyntic gap is >15 mm. In these patients, reflux is severe with increasingly uncontrollable symptoms and columnar lined esophagus, both irreversible states.Understanding this pathophysiology of GORD by these new histologic criteria will allow diagnosis at the earliest and eminently reversible stages of the disease. This can open the door to new methods of treatment that will have the potential to prevent progression to the irreversible phase of GORD, including columnar lined esophagus. If successful, this will effectively prevent progression to adenocarcinoma.

Kritische Betrachtung neuer Konzepte zur endoskopischen Anatomie des ösphagogastralen Übergangs im Sinne des Denkens von Parmenides und Martin Heidegger

Current endoscopic anatomy interposes the gastric cardia between the tubular oesophagus and the proximal stomach. In contrast to that, recent evidence unfolds a different view. Using "PubMed" and "Scopus" searches, we examined if the novel understanding regarding the cardia goes in line with the concept of unfolding, as described by Heidegger based on the ancient didactic poetry of Parmenides. What has been taken as gastric cardia in fact represents reflux-damaged, dilated, columnar lined oesophagus (CLO): dilated distal oesophagus (DDO). Due to its macroscopic gastric appearance it cannot be discriminated from the stomach by endoscopy. Differentiation between DDE and proximal stomach requires the histopathology of measured multi-level biopsies obtained from the DDO and the proximal stomach. Cardaic, onxytocardiac mucosa and intestinal metaplasia (IM; Barrett's oesophagus) define CLO and thus the oesophageal location, while oxyntic mucosa (OM) of the proximal stomach verifies a gastric biopsy location. Endoscopically visible CLO and DDO define the morphological manifestation of reflux: the squamo-oxyntic gap (SOG). Biopsies obtained from the level of the diaphragmatic impressions allow differentiation between an enlarged hiatus with normal anatomic content (CLO; oesophagus) vs. hernia with abnormal content (OM; stomach). Non-dysplastic Barrett's oesophagus exists in 10 %-17 % of asymptomatic and in 20 %-100 % (with increasing CLO length) of reflux symptom-positive individuals (annual cancer risk: 0.2 %-0.7 %). These data justify biopsy of an endoscopically normal appearing squamocolumnar junction for the exclusion of Barrett's oesophagus and cancer risk. In the absence of contraindications, cancer risk-based therapy of dysplastic Barrett's oesophagus includes radiofrequency ablation (RFA) ± endoscopic resection. The perception of the cardia as reflux damaged DDO mirrors the concept of unfolding, as described by the interpretation of the didactic poem of Parmenides by Heidegger. Our data recommend to omit the term "cardia" and allocate morphology either to the oesophagus (CLO, DDO) or to the proximal stomach or indicate that allocation is impossible (i. e.. tumour-induced). Future studies will have to test the value of this novel concept for diagnosis, treatment of gastro-oesophageal reflux disease and cancer prevention.

Fetal stomach paracentesis in combined duodenal and esophageal atresia

Fetuses with concomitant duodenal atresia (DA) and esophageal atresia (EA) might develop in utero gastric rupture as well as neonatal respiratory complication due to dilated stomach and duodenum. Our patient with the typical "double bubble" appearance was highly suspected to have DA in the second trimester. Follow-up examinations revealed a massively dilated stomach and duodenum with a dilated distal esophagus, indicating concomitant DA and EA. With advancing pregnancy, the fetal abdomen progressively increased in size by retention of fluid in the closed loop of DA and EA. To avoid gastric perforation, prenatal stomach paracentesis using an ultrasound-guided needle was performed three times until delivery. A male neonate born at 37weeks gestation showed no respiratory complication. Perinatal clinical features and operative findings revealed combined DA and EA (gross type A). He was successfully managed with duodenoduodenostomy, followed by esophago-esophagostomy. On fetal sonography, the marked "double bubble" appearance and the cystic structure presenting peristalsis-like movement above the diaphragm were indicative of concomitant DA and EA. Fetal stomach paracentesis could contribute to the improvement of perinatal outcomes in fetuses with this pathological condition.

Dysarthria as the Initial Manifestation of Esophageal Adenocarcinoma

Purpose: The majority of patients with esophageal cancer typically present with dysphagia, weight loss, and/or odynophagia at the time of diagnosis. Half of all newly diagnosed cases are unresectable or have distant metastasis with involvement of the lungs, liver, and/or adrenal glands. Brain metastases from esophageal carcinoma are very rare. In the available literature, the incidence of brain metastasis has been shown to be at 0-2% in clinical studies and 0-5.1% in autopsy studies. We report a case of a 65-year-old Caucasian man presenting with a 7-day history of progressive dysarthria. He denied any other complaint and had no other physical exam findings. The patient did admit to using chewing tobacco on and off for the past 20 years. His medical history was positive for being treated for prostate cancer 4 years prior with radiation. The initial head computed tomography (CT) demonstrated a left frontal lobe mass measuring 25 mm with metastatic characteristics (Figure 1). A wide search for a primary source of the cancer was undertaken and a dilated distal esophagus just proximal to the gastroesophageal junction with wall thickening was noted on CT scan. Endoscopy demonstrated a circumferential distal esophageal ulcerated mass extending 5 cm to the proximal part of the stomach. Biopsy of the mass proved to be positive for moderately differentiated invasive adenocarcinoma which was Gastric HER-2 NEU positive. Due to the rare occurrence of brain metastasis from esophageal cancer and given the history of previously treated prostate cancer the patient underwent a brain biopsy which also confirmed metastatic adenocarcinoma. The lesion was unable to be completely resected due to worsening dysarthria during the procedure. Despite the low incidence of brain metastasis from esophageal cancer, when other more common sources are excluded, one must keep esophageal carcinoma as a possible primary lesion.Figure

Review on novel concepts of columnar lined esophagus

Columnar lined esophagus (CLE) is a marker for gastroesophageal reflux and associates with an increased cancer risk among those with Barrett's esophagus. Recent studies fostered the development of integrated CLE concepts. Using PubMed, we conducted a review of studies on novel histopathological concepts of nondysplastic CLE. Two histopathological concepts-the squamo-oxyntic gap (SOG) and the dilated distal esophagus (DDE), currently model our novel understanding of CLE. As a consequence of reflux, SOG interposes between the squamous lined esophagus and the oxyntic mucosa of the proximal stomach. Thus the SOG describes the histopathology of CLE within the tubular esophagus and the DDE, which is known to develop at the cost of a shortened lower esophageal sphincter and foster increased acid gastric reflux. Histopathological studies of the lower end of the esophagus indicate, that the DDE is reflux damaged, dilated, gastric type folds forming esophagus and cannot be differentiated from proximal stomach by endoscopy. While the endoscopically visible squamocolumnar junction (SCJ) defines the proximal limit of the SOG, the assessment of the distal limit requires the histopathology of measured multilevel biopsies. Within the SOG, CLE types distribute along a distinct zonation with intestinal metaplasia (IM; Barrett's esophagus) and/or cardiac mucosa (CM) at the SCJ and oxyntocardiac mucosa (OCM) within the distal portion of the SOG. The zonation follows the pH-gradient across the distal esophagus. Diagnosis of SOG and DDE includes endoscopy, histopathology of measured multi-level biopsies from the distal esophagus, function, and radiologic tests. CM and OCM do not require treatment and are surveilled in 5 year intervals, unless they associate with life quality impairing symptoms, which demand medical or surgical therapy. In the presence of an increased cancer risk profile, it is justified to consider radiofrequency ablation (RFA) of IM within clinical studies in order to prevent the progression to dysplasia and cancer. Dysplasia justifies RFA ± endoscopic resection. SOG and DDE represent novel concepts fusing the morphological and functional aspects of CLE. Future studies should examine the impact of SOG and DDE for monitoring and management of gastroesophageal reflux disease (GERD).

Histologic definition of gastro-esophageal reflux disease

To review recent data supporting the development of new histology-based definitions of gastro-esophageal reflux disease (GERD). Three precisely definable columnar epithelial types--cardiac, oxyntocardiac and intestinal--may be interposed between esophageal squamous epithelium and gastric oxyntic (acid secreting) mucosa. This enables definition of a new histologic concept: the squamo-oxyntic gap. The squamo-oxyntic gap is zero or very small in autopsies performed on patients without evidence of GERD. The gap progressively increases in length with the severity of GERD, indicating that the squamo-oxyntic gap is a marker for chronic GERD. The distal part of the gap lines gastric-type rugal folds and, therefore, is distal to the present endoscopic definition of the gastro-esophageal junction. I contend that this distal gap segment (which has esophageal submucosal glands) is actually the dilated distal esophagus; this is the pathologic correlate of destruction of the abdominal segment of the lower esophageal sphincter. The dilated distal esophagus is mistaken for 'gastric cardia' by present endoscopic definitions. I believe that these data support the adoption of novel histologic definitions of GERD as follows: the presence of any squamo-oxyntic gap defines GERD; the length of the gap is a measure of severity of chronic GERD; and the presence of intestinal metaplasia in the gap defines Barrett esophagus and cancer risk.

Reply to letter from Sebastian F. Schoppmann et al. regarding ‘Radial esophageal acid exposure and the dilated distal esophagus’

Reply to letter from Sebastian F. Schoppmann et al. regarding ‘Radial esophageal acid exposure and the dilated distal esophagus’

Radial esophageal acid exposure and the dilated distal esophagus

With interest we read the article by Ohara et al. [1] published in the recent issue of the Journal of Gastroenterology. Using a novel catheter-mounted multiprobe uni-level pH monitoring system, the authors compared radial acid exposure 2 cm above the manometric lower esophageal sphincter in asymptomatic controls (n = 4), patients with non-erosive reflux disease (NERD, n = 5), and persons with reflux esophagitis (n = 10). Patients with NERD and those with esophagitis showed significantly prolonged acid exposure times on the right side of the distal esophagus (in continuation of the small curvature of the stomach), when compared with findings on the left side (great curvature side of the stomach). Furthermore, this and other recent studies have shown increased severity of endoscopically visible reflux-induced changes on the right versus the left side of the distal esophagus [2]. In addition, three-dimensional (3D) high-resolution manometry has revealed decreased competency within the right-sided portion of the lower esophageal sphincter pressure zone, when compared with the left side [3]. Taken together, the above data and the study by Ohara et al. indicate that the right side of the lower portion of the esophagus is prone to acid/reflux-exposure and reflux injury, when compared with the left side. Recent anatomical, histopathological, and physiological studies have examined the pathogenesis of gastroesophageal reflux disease (GERD) [4]. Repeated overeatinginduced gastric dilatations extend into the distal esophagus. As a consequence, the lower portion of the distal esophagus dilates and is exposed to the gastric contents. Reflux inflames the esophagus (esophagitis), weakens the lower esophageal sphincter, and stimulates the replacement of the normal mucosa by columnar lined esophagus (CLE): this is the dilated distal esophagus (DDE) [4]. Combination of the data obtained by the above investigations [2–4] and the study by Ohara et al. [1] justifies the suggestion that the development of the DDE predominantly commences within the right-side portion of the distal esophagus. These important findings should serve as the basis for future endoscopic and histopathological studies and should help to further elucidate the pathogenesis of GERD [5, 6]. In addition, biopsies directed to the right-side portion of the distal esophagus and the squamocolumnar junction (SCJ) could contribute to increasing the yield for the assessment of premalignant reflux-induced lesions, i.e., Barrett’s esophagus (±dysplasia) [2]. Finally, novel understanding gained from such studies could help to improve anti-reflux surgery, i.e., the placement of a fundic wrap with the inclusion of the ‘‘weak’’ right-side portion of the suprajunctional distal esophagus [7]. The authors are kindly asked to comment on the above suggestions.

Gastroesophageal Intussusception with Megaesophagus in a Hedgehog (Atelerix Albiventris)

A 220-g, 3-month-old intact male hedgehog (Atelerix albiventris) was evaluated for a 3-day history of increased respiratory effort, vocalization, and salivation. Survey and positive-contrast radiographs suggested gastroesophageal intussusception and hiatal hernia as possible differential diagnoses. On endoscopic evaluation, a dilated distal esophagus and everted stomach with fluid regurgitation were observed. The intraesophageal portion of the stomach was returned to the normal anatomic position by the introduced endoscopic tip. The hedgehog died after an episode of severe vomiting 2 days after initial presentation. On necropsy, gross findings included megaesophagus and everted gastric cardia into the distal esophagus; the lower esophageal sphincter was located in the normal anatomic position. Histopathologic examination of the esophagus revealed diffuse ulcers with submucosal hemorrhage and severe inflammation, suggestive of reflux esophagitis. To the authors' knowledge, this is the first case of gastroesophageal intussusception with megaesophagus reported in a hedgehog.

The cardia: Esophageal or gastric? Critical reviewing the anatomy and histopathology of the esophagogastric junction

Discrepancy exists regarding the anatomical allocation of the cardia: esophageal or gastric. With this review we aimed to clarify this issue. Using PUB MED, Scopus and Google we analyzed the recent literature (1889-2012) regarding the "esophageal" vs. the "gastric" cardia. The synonymous use of the term cardia to describe the anti reflux mechanism within the distal portion of the esophagus and the proximal segment of the stomach nourished the misunderstanding, that the cardia represents a normal anatomical structure interposed between the tubular esophagus and the body of the stomach. Anatomical, histopathological and physiological studies revealed that what has been taken for gastric cardia in fact represents reflux damaged dilated distal esophagus (DDE). Since DDE is covered by columnar lined esophagus (CLE) it cannot be differentiated from the proximal stomach during regular endoscopy. However, the histopathology of multi level biopsies obtained from the endoscopically suspected esophagogastric junction (EGJ) serves to allocate the origin of the columnar lined foregut, esophageal (cardiac, oxyntocardiac mucosa, intestinal metaplasia) vs. gastric (oxyntic mucosa). Neither the esophagus nor the stomach contains a "cardia". The recent misconceptions regarding the foregut anatomy explain, why the innermost coverage of the reflux damaged esophagus is termed "cardiac mucosa". Thus the term should be reserved to name the histopathology of cardiac and oxyntocardiac mucosa, which develop due to gastroesophageal reflux within the distal esophagus.

The Dilated Distal Esophagus

Present management algorithms for patients with gastroesophageal reflux disease (GERD) limit endoscopy to patients with advanced disease. When endoscopy is performed, biopsy is limited to patients who have a visible columnar-lined esophagus. Biopsy is not recommended for patients whose endoscopy is normal. This algorithm results in the failure to evaluate patients with early stages of GERD at a pathologic level. We report 714 patients with normal endoscopic findings irrespective of symptoms who had adequate biopsies taken from the squamocolumnar junction and the area 1-cm distal to this from mucosa that had rugal folds. Concurrent biopsies were also taken from the gastric body and/or antrum. All patients had a gap between their esophageal squamous epithelium and gastric oxyntic mucosa in the junctional region composed of oxyntocardiac ± cardiac ± intestinal epithelia. A total of 643 (90.1%) patients had no significant pathology in the gastric antrum and/or body, indicating that the squamooxyntic gap was an isolated abnormality in this region in all but 71 (9.9%) patients. The gap contained only oxyntocardiac epithelium in 71 (9.9%) patients, cardiac mucosa without intestinal metaplasia in 482 (67.5%) patients, and intestinal metaplasia in 161 (22.6%) patients. The pathologic interpretation of biopsies taken from the gastroesophageal junction is confusing and has significant interobserver variation. This results from lack of agreement as to whether these biopsies originate in the proximal stomach ("gastric cardia") or in the esophagus. We provide evidence that the presence of oxyntocardiac ± cardiac ± intestinal epithelia in biopsies from patients who are endoscopically normal is diagnostic of a dilated GERD-damaged distal esophagus. The dilated distal esophagus is the pathologic manifestation of destruction of the abdominal segment of the lower esophageal sphincter. Its presence is the pathologic basis of early GERD, which is missed if patients who are endoscopically normal are not biopsied, as is the present recommendation. Its recognition allows for accurate and evidence-based interpretation of biopsies from this region and removes the present confusion and permits the development of a reproducible pathologic diagnostic method.