Savary Dilators Research Articles

Plummer-Vinson syndrome: Clinical, therapeutic and evolutionary aspects: a Moroccan case series

Plummer Vinson syndrome (PVS) is a rare entity characterized mainly by upper dysphagia, irondeficiency anemia, and the presence of an esophageal ring on endoscopic examination. We conducted a retrospective study over a period of 18 years describing the epidemiological and clinical profile, endoscopic management and the medium and long-term evolution. A total of 96 patients were enrolled, with a median age of 41,5 years and a clear female predominance (19/77). dysphagia was present in almost all our patients (97,9%), and an anemic syndrome in half the cases (52%). Female gender was significantly associated with PVS occurrence (p=0.002). The frequency of the disease was significantly higher after the age of 30 years with a peak in frequency in the 4th decade (p<0.034). Upper endoscopy found a membranous ring located in the cervical area in all cases. 1 patient had, in addition to the plummer vinson ring, a lower esophageal membranous called Schatzki ring with a small sliding hiatal hernia. PVS was associated with other autoimmune diseases mostly represented by celiac disease and autoimmune thyroiditis. Treatment was based on iron supplementation followed by endoscopic dilatation with Savary dilators in 91,6% (n=88) and balloons for 8,3% of cases (n=8). long-term outcome was favorable in 79.1% of patients with no clinical recurrence. 20,8% patients relapsed after a median of 29,2 months [2-72 months] requiring a second dilation. PVS was complicated by squamous cell carcinoma in 5,2% (n=5).

Endoscopic recannulation of long-segment, grade IV suprastomal tracheal stenosis: An operative technique

BackgroundLong-segment, grade IV suprastomal tracheal stenosis is rare and difficult to treat (Carpenter et al., 2022 [1]). Patients with grade IV stenosis have significant quality of life impairments since they are tracheostomy dependent and aphonic. Open airway surgery is often needed to improve tracheal patency, restore the patient's voice, and progress towards decannulation (Abouyared et al., 2017 [2]). However, not all patients are candidates for upfront open surgery (Abouyared et al., 2017; Shamji, 2018 [2,3]). Therefore, it is important to develop and refine endoscopic interventions to improve quality of life for these patients. MethodsWe describe a step-by-step endoscopic approach to the recannulation of long-segment, grade IV suprastomal tracheal stenosis. Briefly, our approach utilizes dual (proximal & distal) visualization of the stenosis prior to passing a 25 gauge needle through the stenosis to identify the proper trajectory for recannulation. Then a 16 gauge needle is passed in the same manner, and a wire is placed through the needle and into the distal airway. Once the airway is recannulated, the initial pinpoint opening is gradually widened in Seldinger fashion over the wire with Savary dilators followed by balloon dilation. Finally, a suprastomal L-stent (modified Montgomery T-Tube) is placed to reduce the risk of restenosis (Edwards et al., 2023 [4]). Case discussionA 39-year-old woman with a past medical history significant for poorly controlled type I diabetes mellitus and polysubstance abuse presented with tracheostomy dependence and aphonia. She was diagnosed with a long-segment, grade IV suprastomal tracheal stenosis and initially underwent endoscopic recannulation. This intervention restored her voice and allowed for optimization of her medical conditions before open airway surgery. ConclusionMost patients experience a significant improvement in their quality of life as their voice is typically restored following this procedure. Additionally, individuals who eventually require open airway surgery gain additional time for medical optimization. In our experience, this procedure represents a safe and effective means of extending the utility of traditional endoscopic airway interventions for the management of patients with grade IV stenosis.

S715 Comparison of Efficacy and Safety of Retrograde Balloon Pull-Through Technique vs Antegrade Savary Dilation for Benign Esophageal Strictures

S715 Comparison of Efficacy and Safety of Retrograde Balloon Pull-Through Technique vs Antegrade Savary Dilation for Benign Esophageal Strictures

Risk factors for total laryngectomy associated proximal esophageal stricture formation in head and neck cancer patients.

Development of esophageal strictures is common after the total laryngectomy of head and neck cancer patients. While endoscopic techniques like dilatation by balloon or Salvary bougies are well established, risk factors and pathophysiology for development of refractory strictures are less well understood. To evaluate risk factors associated with occurrence and recurrence of total-laryngectomy-associated esophageal strictures in head and neck cancer patients. We analyzed retrospectively a cohort of 170 head and neck squamous cell carcinoma patients, who underwent total laryngectomy between 2007 and 2017. The outcome measure was laryngectomy-associated proximal esophageal stricture needing an endoscopic dilatation by using a balloon or Savary dilators. Of the 170 patients in the cohort, 32 (18.8%) developed strictures. Mean time between surgery and first endoscopic intervention was 24.4 months. Significant predictive factors were age ≥ 65 (p=0.017), nodal status N> 1 (p=0.003), continued alcohol abuse after surgery (p=0.005) and diabetes mellitus (p=0.005). In a subgroup, 17 of 32 patients developed refractory strictures and needed more than three dilatations to relieve dysphagia. Postoperative mean (p=0.016) and maximum (p=0.015) C-reactive protein (CRP) were predictive for refractory strictures. Symptomatic strictures occurred in 18.8% of the cases. Age, nodal status N>1, continued alcohol abuse and diabetes mellitus were predictive factors. For refractory stenosis (>3 dilatations needed) mean and maximum postoperative CRP were predictive. This may indicate that systemic inflammatory response post-surgery is involved in the stricture formation process.

423. DYSPHAGIA AND RE-INTERVENTION AFTER MYOTOMY FOR ACHALASIA: A MATTER OF TECHNIQUE, OR A MATTER OF TIME?

Abstract Background Achalasia is an incurable neurodegenerative disease of the esophagus. Palliative treatment includes disruption of the lower esophageal sphincter via multiple techniques, including open Heller myotomy (OHM), laparoscopic Heller myotomy (LHM), and per-oral esophageal myotomy (POEM). There is a paucity of data comparing long-term outcomes between OHM, LHM, and POEM. We sought to evaluate the relationship of these methods with clinical outcome parameters using our institution’s experience. Methods Patients greater than 3 years removed from myotomy for achalasia were studied. Post-operative Eckardt Symptom (ES) score and daily proton pump inhibitor (PPI) usage was recorded. Patients underwent timed barium esophagram (TBE) as clinically indicated. Retention was defined as a persistent column >5 cm at 5 minutes. Post-operative interventions included Savary dilation, Botox injection, pneumatic dilation, surgical myotomy, and esophagectomy. Groups were stratified by surgery type (OHM, LHM, POEM) and by time since surgery (3–6 years, 6–10 years, >10 years). A logistic regression analysis was performed examining the relationship of surgery type, time, symptomatic/objective outcomes, and post-operative interventions. Results 172 patients had long-term data available. Mean follow up was 7.8 years (3.1–19.5 years). Nearly one-third of patients had a post-operative intervention (31%, 53/172). On regression analysis, OHM was associated with a higher risk of intervention (OR 9.4, p < 0.05). Barium retention was significantly associated with LHM (OR 15.9, p < 0.05) and the 3–6 year post-operative period (OR 11.1, p < 0.05). PPI usage was not associated with surgery type, but was associated with the 3–6 year post-operative period (OR 3.2, p < 0.05). There was no significant association of dysphagia with surgery type or time. Conclusion Post-operative barium retention and interventions are associated with specific myotomy techniques, but not time. PPI usage is associated with the early post-operative period, but not technique. Neither myotomy technique nor time alone are sufficient to predict risk of abnormal ES or dysphagia.

S2806 Biliary Stenting of Caustic Proximal Esophageal Stricture Complicated by Fistula From Prior Dilation

Introduction: Caustic ingestions can lead to recalcitrant strictures of the upper esophagus. Endoscopic treatment options include dilations and/or covered esophageal stents; however, a stricture at a high anatomic location renders traditional stenting difficult. Dilation has the risk of perforation and fistula development. We present a patient with complex, proximal and distal esophageal strictures from caustic ingestion who was managed surgically then salvaged endoscopically using biliary stents. Case Description/Methods: A 28-year-old woman with a history of caustic ingestion of bleach and was found to have airway injuries, severe proximal digestive tract injuries, and transmural gastric necrosis. She underwent a total gastrectomy and cervical esophagostomy followed by Roux esophagojejunostomy and stepwise reconstruction. She then had esophageal strictures initially treated with Savary dilation complicated by perforation and fistula formation between upper stricture and pleura. Endoscopy revealed a 3-mm lumen at the upper esophageal sphincter (UES)(Figure). Initial contrast injection revealed no passage into esophagus, with contrast passing to pleura. XP gastroscope was used to pass a guidewire through the proximal stricture and into the esophagus. A 10mm x 6cm biliary fully covered self-expanding metal stent (FCSEMS; Gore Viabil Biliary Endoprosthesis) was deployed across the stricture, covering the fistula. The patient subsequently had repeated endoscopies for stent migration and to reach the distal stricture for stenting. Discussion: Esophageal stents are not typically placed across the UES due to foreign body sensation. In this case, the anatomic location of the stricture and presence of fistula required stenting across the UES. We chose a biliary FCSEMS that had anti-migratory features (flanges and conformability) and did not foreshorten. Despite these measures, the stent did migrate multiple times requiring re-intervention over a period of months, eventually allowing access to the distal esophageal stricture after upper esophageal remodeling around the shape of the stent. At the time of presentation to advanced endoscopy, colonic interposition was a less preferred option due to lack of healthy pharyngeal or esophageal tissue for anastomosis. Through the use of biliary stents in the esophagus, we were able to simultaneously treat a difficult stricture and fistula, allowing eventual access to a distal stricture for endoscopic treatment.Figure 1.: Endoscopic and fluoroscopic imaging showing (A) proximal esophageal stricture from caustic ingestion, (B) contrast injection showing stricture with fistula to pleura prior to stenting, (C) contrast injection through biliary FCSEMS deployed across proximal stricture without evidence of leak, and (D) proximal endoscopic view of the biliary FCSEMS above the arytenoid and close to endotracheal tube in subsequent endoscopy.

Infectious transmission from Savary dilators is uncommon: retrospective review from a large tertiary academic center.

Infectious transmission from Savary dilators is uncommon: retrospective review from a large tertiary academic center.

The Outcome of Pediatric Esophageal Strictures Managed with Endoscopic Balloon Dilation in Saudi Arabia.

Background: Esophageal strictures can affect nutrition of infants and children impairing their weight gain. To our knowledge, this is the first article evaluating and comparing between the two methods of dilation in terms of outcome and one of few, if any, to assess both weight and height to evaluate the nutritional outcomes following dilation. To determine the safety, efficacy, and long-term effects of endoscopic dilation in managing pediatric esophageal strictures by assessing the clinical and nutritional outcomes. Methods: A retrospective study of 137 patients with esophageal strictures who underwent either endoscopic balloon dilatation or Savary dilatation, or both. Outcome parameters measured include the number of dilatations, nutritional status, and if symptoms had been relieved. Results: The most frequent cause of esophageal strictures was post-tracheoesophageal fistula repair (n = 51, 37.2%), and the majority were lower third strictures (n = 47, 34.3%). However, 8 cases (5.8%) had failed the dilation procedure. Savary dilatation had the highest number of complications. Overall, success rate was 79.6%. Higher success rate was for cases dilated by endoscopic balloon dilation (EBD) (n = 47, 90.4%). There was a statistically significant correlation between the success rate and the method of dilatation (P = .042). Statistically significant increment of weight was recorded for lower strictures (P = .001). Conclusion: EBD was associated with the highest success rate. Endoscopic dilatations are safe and efficient in managing pediatric esophageal strictures with improvement in both clinical and nutritional outcomes.

Patterson-Kelly Syndrome: A Single Centre Experience

<i>Background</i>: Patterson Kelly syndrome is a rare entity, presenting with a triad of Iron deficiency anemia, Dysphagia and Esophageal webs. The syndrome is extremely rare and its incidence is decreasing probably due to improvements in nutritional standards. The syndrome is enigmatic in the sense that not all patients may be having anemia, the syndrome is associated with various autoimmune conditions, webs are located in post-cricoid region only and there is increased prevalence of upper gastrointestinal malignancies in these patients. The symptoms respond to iron replacement but many patients require rupture of web for relief of dysphagia. Savary dilators, TTS balloon, electro-cautery have been variably used to break the web. <i>Objectives</i>: 1. To study the profile of Patterson Kelly syndrome at our center; 2. To study if the endoscope itself can be used as effective dilator for rupturing the web; 3. To study the effect of iron replacement in these patients; 4. To study the occurrence of upper gastrointestinal malignancies in this condition. <i>Material and methods</i>: We report our experience of sixteen patients with Patterson Kelly syndrome over last fifteen years. This is a retrospective, single center, observational study. All patients had demonstrable web on endoscopy. The endoscope itself was used as a dilator to break the web in more than half of the patients. The endoscope being rigid instrument can easily break the thin web during diagnostic endoscopy itself. If the opening was too narrow, TTS balloon or Savary Guilard dilators were used. One patient had a perforation while using endoscope as the dilator. Five patients required more than one sitting of dilatation for achieving relief of dysphagia or for recurrent web formation. Most patients received intravenous iron supplementation. Ten patients had complete relief of dysphagia without recurrence. One patient had concomitant esophageal squamous cell carcinoma in lower esophagus which became evident when the scope was passed distally, post dilatation of the web. <i>Conclusions</i>: Although rare, Patterson Kelly syndrome continues to exist and can be effectively treated by iron replacement, dilatation and rupture of esophageal webs using various methods. The web can be ruptured by careful maneuvering of the endoscope itself. Intravenous iron replacement can be used prior to the procedure which can ameliorate the symptoms and reduce the need for the dilatation or can make it easier. Patients need surveillance for upper gastrointestinal malignancies however exact guidelines in this regard are lacking.

3103 A Case of Multiple Peptic Strictures Requiring Multistage Dilatation: A Challenging Esophagus

INTRODUCTION: Peptic strictures are benign esophageal strictures that result from chronic gastroesophageal reflux disease (GERD). Endoscopic dilatation is the treatment of choice. However, some strictures are complicated and difficult to manage. We present a case of an elderly male with two benign esophageal strictures, which were dilated in multiple sessions. CASE DESCRIPTION/METHODS: A 66-year-old male with a history of GERD, heavy smoking, excessive use of NSAIDS and alcohol was admitted for chronic dysphagia to solids for the last two years worsening over the last few months without significant weight loss. Physical exam and initial laboratory data were unremarkable. Upper endoscopy showed a benign appearing stricture at 18 cm from the incisors which could not be traversed with a regular or a pediatric scope. Barium esophagogram showed two areas of stricture in the proximal and the distal esophagus. The proximal stricture was dilated using a balloon dilator through the scope in multiple sessions. Finally, a10 mm dilatation was achieved where the pediatric scope traversed the stricture to examine the distal esophagus, which revealed esophageal diverticulosis. It showed a complex stricture at 36 cm from the incisors which was traversed. Biopsies showed intra-epithelial lymphocytes consistent with GERD without any eosinophil or atypical cells. Fluoroscopy guided dilation was done using Savary dilators up to a 14 mm diameter in three sessions with clinical improvement. Cessation of smoking, heavy alcohol and NSAIDS use was advised, and a proton pump inhibitor was started. DISCUSSION: Stricture formation occurs in 7–23% of patients with reflux esophagitis and GERD. Long term acid exposure results in chronic esophagitis with subsequent fibrosis of the muscularis propria leading to a fibrotic narrowing of the esophagus with formation of diverticula. Risk factors include male gender, advanced age, GERD, heavy alcohol use and NSAIDS. Work up includes upper endoscopy and barium esophagogram. Differentials include eosinophilic esophagitis and lye strictures. Biopsies are mandatory to rule out malignancy. Treatment involves acid suppression and dilation using wire guided polyvinyl bougie dilators under fluoroscopic or endoscopic guidance and balloon dilators which are passed through the endoscope. Perforation and bleeding may occur in 0.5 % cases. Our case was challenging as malignancy at the distal stricture could not be ruled out until the proximal stricture was dilated enough for the endoscope to traverse.

1701 An Aggressive Case of Recurrent Esophageal Stricture in a Patient With Acute Esophageal Necrosis

INTRODUCTION: Esophageal strictures are a known complication of acute esophageal necrosis (AEN). Our patient presented with dysphagia and after EGD was found to have AEN complicated by an esophageal stricture requiring recurrent dilation. CASE DESCRIPTION/METHODS: 82 y/o male with PMH of HTN and prostate cancer was status post right sided orchiectomy for recurrent orchitis. After surgery, patient disclosed a 2-month history of dysphagia to solids. He reported food “getting stuck” and a 20 lb weight loss in 2 months. EGD showed white exudate in the proximal and mid esophagus that easily sloughed off. The mucosa was ulcerated with underlying granulation tissue (Figure 1). Brushings were negative for candida. Biopsy results from both proximal and distal esophagus showed marked necroinflammatory debris. Patient denied caustic ingestion. Serologic testing was normal. Patient was discharged on a liquid diet, pantoprazole 40 mg PO BID, sucralfate with EGD in 2 months. He returned 1 month later for worsening dysphagia. EGD revealed esophagitis in the mid and distal esophagus and a stricture at 36 cm (Figure 2). The white exudate had improved, but the mucosa was fibrosed. Using a Savary dilator, the stricture was dilated to 7 mm. Over the next 2 weeks, 3 EGD’s with dilation were required for recurrent stricture. On the 4th visit he reported retching and coffee ground emesis for 3 days. EGD revealed an esophageal tear at the stricture site (Figure 3). At the 5th visit, patient was asymptomatic and tolerating a clear liquid diet. EGD showed scarring and fibrosis of the esophageal mucosa and a stricture that was dilated to 12 mm. He will be followed closely due to concern for reformation of the stricture. DISCUSSION: AEN has been associated with infections, antibiotics, malignancy, prolonged vomiting etc. It is proposed that a low flow vascular state leaves the esophagus vulnerable to topical injury from acid reflux and pepsin. On EGD, a circumferential black discoloration with underlying friable hemorrhagic tissue is seen with a sharp transition point to normal appearing mucosa at the GE junction. The esophagus may have thick, white exudates that can be dislodged easily to reveal pink granulation tissue. Histology reveals necrosis of the esophageal mucosa and submucosa. Supportive care leads to endoscopic resolution in most patients. Esophageal stricture occurs in 25-40% of patients and requires dilation. Our patient required repetitive dilation as his stricture redeveloped rapidly. He may need PEG for nutritional support.

1769 Esophageal Candidiasis as an Unusual Cause of Upper Esophageal Stricture

INTRODUCTION: The location of an esophageal stricture often provides clues for its etiology. Upper esophageal strictures are most commonly associated with previous mediastinal radiation, caustic ingestion, or bullous dermatologic disorders. They are rarely attributed to infectious etiology. We present a case of an upper esophageal stricture that we believe is secondary to candida infection. CASE DESCRIPTION/METHODS: A 52-year-old female with past medical history significant for diabetes mellitus type 2 and invasive ductal carcinoma of right breast status post mastectomy and chemotherapy, now in remission and only on hormonal therapy, had presented to our gastroenterology clinic complaining of dysphagia with associated unintentional weight loss. Dysphagia had been present for several months prior and was progressive, with near inability to tolerate solids, and not associated with regurgitation or acid reflux symptoms. She had also reported a 15-pound weight loss during this time. Initial esophagogastroduodenoscopy revealed an upper esophageal stricture approximately 15cm from the incisors, preventing initial passage of standard upper endoscope. Neonatal scope was used which both traversed and dilated to 9mm. Past the stenosis, friable and desquamative- appearing mucosa was found. Upper esophageal biopsies were consistent with Candida esophagitis. Gastric cardia biopsy incidentally noted Helicobacter pylori. Patient was subsequently treated with oral fluconazole, triple therapy, and proton-pump inhibitor. Repeat follow-up esophagogastroduodenoscopy again demonstrated the initial upper esophageal stenosis, which could now be traversed with a standard upper endoscope. A Savary dilator was used to dilate the stricture to 39 Fr. Repeat esophageal and stomach biopsies were negative for Candida and H. Pylori, respectively. Subsequently, the patient reported improved symptoms without any dysphagia to solids or liquids. DISCUSSION: Candidal esophagitis is fairly common, however it is not typically known to cause upper esophageal strictures. This case highlights the need for physicians to maintain a broader differential and consider candida as a risk factor for developing stenotic lesions, even in immunocompetent patients and especially in the absence of other more well-known etiologies. Correct identification of etiology is necessary for appropriate treatment and preventing recurrence.

1787 Esophageal Stricture in Pregnancy Secondary to Stevens-Johnson Syndrome

INTRODUCTION: Stevens-Johnson syndrome (SJS) is a rare, potentially life-threatening, mucocutaneous reaction leading to extensive necrosis and detachment of the epidermis. SJS commonly follows a viral prodrome or drug hypersensitivity reaction with an incidence of 1 to 2 cases per million per year. The long-term sequelae include mucosal, pulmonary, cutaneous, vulvovaginal and rarely esophageal complications. We present a rare case involving a pregnant patient with multiple esophageal strictures due to SJS requiring repeat balloon dilations. CASE DESCRIPTION/METHODS: A 32 year old pregnant female with a history of drug induced SJS, complicated by multiple esophageal strictures and vaginal stenosis, presented with symptoms of recurrent, persistent solid food dysphagia and weight loss. Barium esophagram showed a mid-esophageal stricture measuring 5 mm in diameter and a distal stricture measuring 2 mm in diameter just proximal to the gastroesophageal junction. Due to the severity of symptoms, pregnancy, and the etiology of the stricture, the patient was started on a graded stepwise dilating approach by esophagogastroduodenoscopy (EGD). Repeat dilations were done by a “rule of 3” approach using a Savary dilator every two to three weeks. A maximal diameter of 10 to 11 mm was achieved and symptom relief improved throughout the patient’s pregnancy. The patient had no procedural complications and delivered a healthy baby at 39 weeks by cesarean section. DISCUSSION: Over 12,000 EGDs are performed annually on pregnant women and limited follow up data showed it as relatively safe for both the mother and fetus. SJS with esophageal involvement is exceptionally rare in adults with only a few reported cases and even fewer cases during pregnancy. Esophageal strictures secondary to SJS share many similarities to strictures caused by corrosive ingestion in both presentation and management. Corrosive ingestion strictures are treated by repeated dilation and one study showed a long term satisfaction of greater than 90%. In our case this was weighed against the higher incidence of iatrogenic injury to both the mother and fetus. Further research will need to be done to determine the long term complications in pregnant patients after repeat dilations.

1708 Case of Secondary Achalasia Presenting Several Years After Nissen Fundoplication With Improvement After Endoscopic Dilatation: “Rare but Not Forgotten”

INTRODUCTION: Secondary Achalasia is an esophageal motor disorder presenting with clinical, radiographic and manometric features that mimic those of primary achalasia except that it has a known etiology such as gastroesophageal junction (GEJ) tumor, antireflux surgery, gastric banding or gastrectomy. We present a rare case of secondary achalasia as a remote consequence of a laparoscopic Nissen fundoplication (LNF). CASE DESCRIPTION/METHODS: A 59 year old woman with a history of diabetes mellitus, gastroesophageal reflux disease (GERD), hiatal hernia status post LNF with hernia repair eight years ago, presented with dysphagia to solids and liquids with a 24-pound weight loss over the last 3 months. On presentation, physical exam and initial laboratory data were unremarkable. Computerized tomography of the chest showed esophageal dilatation with an obstruction at the GEJ. Upper endoscopy revealed a large amount of impacted food in the lower third of the esophagus which was removed with a Roth net, and the GEJ was traversed by the scope with significant resistance. On retroflexion evidence of a Nissen fundoplication was found at the GEJ with a tight appearing wrap. The GEJ was dilated using Savary dilators over a guide up to a diameter of 14 mm with remarkable improvement of the symptoms. An esophagogram done post dilatation showed transit of the barium contrast through the GEJ. DISCUSSION: GERD is the most common pathologic condition affecting the upper gastrointestinal tract. While most patients respond to medical therapy, LNF has become the gold standard for antireflux surgery in patients with medically refractory disease. Transient dysphagia occurs in 40% to 70% of patients after LNF in the early postoperative period due to edema, slipped, migrated or too tight fundoplication or a missed diagnosis of primary achalasia and nutcracker esophagus. However, it can also occur many years after the operation due to hiatal stenosis secondary to severe fibrotic reaction and anterior angulation of the GEJ. EGD is a good initial diagnostic tool and resistance or inability to pass a gastroscope through the EGJ should raise suspicion of secondary achalasia with a background history of LNF. GEJ tumor should always be ruled out with careful examination. Dilatation of the GEJ is done using wire guided Savary dilators or through the scope balloon dilators. Our case highlights the importance of an aggressive evaluation and careful interpretation of a patient presenting with dysphagia with a remote history of fundoplication.

1754 Plummer-Vinson Syndrome: A Time for a Redefinition and New Perspective

INTRODUCTION: Plummer-Vinson syndrome (PVS), or Kelly-Patterson syndrome, is a rare condition defined by the classic triad of dysphagia, iron-deficiency anemia, and esophageal webs, and dominated by Caucasian females. The pathophysiology remains unknown although iron deficiency is one theory as well as part of an autoimmune spectrum. We present two patients seen recently meeting criteria for PVS in whom new observations were made. CASE DESCRIPTION/METHODS: A 46-year-old Hispanic female with history of hypothyroidism presented with a Hgb of 3 and a ferritin of 16. She reported 2 weeks of fatigue, dyspnea, and constipation without overt bleeding, preceded by several months of dysphagia to solids. She was severely hypothyroid and placed on IV levothyroxine. Barium swallow suggested an esophageal web in the upper esophagus. At esophagogastroduodenoscopy (EGD) both adult and neonatal scopes could not be passed through a tight stenosis at the cricopharyngeus (Figure 1). This was dilated with Savary dilators to 8 mm. She was discharged home on iron supplements and follow up EGD with dilations scheduled. A 55-year-old Hispanic female with history of lupus, a positive ANA, Raynaud's, prior partial thyroidectomy, and chronic anemia was referred to GI clinic for evaluation of chronic dysphagia which had required multiple dilations at an outside facility over the past 18 months without improvement. She had a hgb of 6.7 with iron deficiency without any overt GI bleeding. EGD was performed and showed two benign appearing areas of intrinsic stenosis 15 and 20 cm from the incisors that were smooth and not ulcerated and could not be passed with the adult or pediatric endoscopes (Figure 2). Savary dilators were used to dilate to 11 mm diameter. Patient was placed on iron replacement and a fo llow up EGD was scheduled. DISCUSSION: Why PVS is only in females with accompanying iron deficiency anemia and a personal/family history of autoimmune phenomena and now is decreasing in recognition are not clear. The resolution of dysphagia with iron supplementation, could also be explained by the accompanying esophageal dilations restoring oral intake and hence improving iron absorption. Our cases highlight that PVS: 1) is not a true “esophageal web” but rather a tight fibrotic stricture at the level of the upper esophageal sphincter requiring aggressive dilation approach 2) occurs also in Hispanic females presenting with iron deficiency, dysphagia, and an autoimmune picture.

457 Predictor Factors for Dilation Treatment in Stricture Due to Eosinophilic Esophagitis

INTRODUCTION: Eosinophilic esophagitis (EOE) is one of the most common causes of dysphagia and food impaction in the last two decades. Medical therapy including proton pump inhibitors (PPI), topical steroids, and diet can help, but if an esophageal stricture is present, then dilation may be necessary. The safety and efficacy of stricture dilation in EOE have been shown, but there is limited data on factors that can predict if patients will require multiple dilations. The objectives of this study were to determine the frequency and predictors of requiring multiple dilations in patients with EOE. METHODS: A retrospective study from 2003-2018 was performed at a tertiary care center. Patients included in this study required a pathologic diagnosis of EOE (&gt;15 eosinophils per high power field), and required an esophageal stricture. The response was defined as the absence of symptoms. Adverse events included perforation. RESULTS: A total of 34 patients (21 men, 13 women) were included in the study. The median age was 43 years (range, 18-65). Follow-up time was 5.8 years. All patients underwent either a Savary dilation or pressure balloon dilation. Table 1 lists the characteristics of the strictures and balloon dilations. 8 patients required only one dilation session with 100% response rate, which defined. All patients were on PPI. 9 patients did not have adjunctive therapy, while 21 patients used topical steroids, 3 patients used 6-food elimination diet, and 1 patient used both topical steroids and 6-food elimination diet. 5 patients had an 8 mm stricture that was not able to be traversed with the endoscope, requiring a median of 2 sessions (range 2, 3) of dilation. Table 2 shows the characteristics of the strictures stratified by the number of sessions. Table 3 shows the number of sessions stratified by the length of the stricture. There were no perforations. CONCLUSION: Esophageal dilation has a definitive role in the management of EoE. It is indicated if the patients do not respond to pharmacological or dietary therapy. In this study, it seems the major predictor factor for frequent esophageal dilation is the length of the stricture. The treatment including corticosteroid inhaler or 6-food elimination is not helpful when the length of stricture more than 1 cm.

1756 Evolution of Esophageal Lichen Planus to Esophageal Leukoplakia

INTRODUCTION: To describe a case of esophageal lichen planus which appeared to evolve to leukoplakia (aka epidermoid metaplasia). CASE DESCRIPTION/METHODS: A 70 yo female with a prior history of lichen planus, presented from another institution for ongoing care related to proximal esophageal stricturing. The patient had well-documented lichen planus involving the oral and vaginal mucosae and axillary areas. Dysphagia had been ongoing for greater than a decade and prior endoscopic exams revealed the proximal esophagus to be of small caliber with proximal esophageal strictures/rings. Biopsies had shown nonspecific change. On some occasions an ultrathin gastroscope was required to traverse the proximal esophagus. The patient underwent numerous sessions of Savary dilation and given oral lichen planus, it was assumed that this process was also responsible for the proximal esophageal pathology. She was treated with periodic esophageal dilation and topical budesonide. Recently after the above therapy, dysphagia had resolved, and the patient was ingesting a normal diet. The esophagus was found to be of more normal caliber endoscopically (strictures were no longer evident) and prominent whitish proximal esophageal plaques were now noted (Figure 1). Proximal esophageal biopsies now revealed hyperorthokeratosis with hypergranulosis, but no inflammatory process or Civatte bodies (apoptotic keratinocytes). As such, recent endoscopic and pathologic findings were thought to be most consistent with esophageal leukoplakia/epidermoid metaplasia. Topical budesonide was continued for her oral lichen planus, but it was now recommended that it be administered in a "swish and spit" manner. Given the association of esophageal leukoplakia with squamous dysplasia and squamous cell carcinoma, the patient was placed in an endoscopic surveillance program. She has recently remained asymptomatic from the esophageal perspective. DISCUSSION: Lichen planus and leukoplakia (epidermoid metaplasia) involving the esophagus are distinct clinical, endoscopic and pathologic processes- both of which appear to have a female predominance, and potential association with squamous cell carcinoma. The esophageal disease in patient under discussion appeared to evolve from one of these processes to the other, raising the specter of an association between these disorders.

Revisional Procedures for Recurrent Symptoms After Heller Myotomy and Per-Oral Endoscopic Myotomy.

Background: Achalasia and other esophageal motility disorders are incurable diseases for which palliation and symptom relief are the goals. One of the many ways these diseases are treated is with either a Heller myotomy or, now more commonly, per-oral endoscopic myotomy (POEM). Unfortunately, symptoms persistence or recurrence is common. This review presents our current approach to these complex patients. Methods: Review of the literature pertaining to approaches to recurrent or persistent symptoms after myotomy for esophageal motility disorders and elucidation of our multidisciplinary approach to this patient group. Results: There are a myriad of causes of recurrent or persistent symptoms. These include incomplete myotomy, periesophageal scarring, reflux-induced stricture, obstructing fundoplication, functional dysphagia, and end-stage achalasia. Therapeutic options include redo myotomy (either Heller or POEM), botulinum toxin injection, pneumatic, balloon or Savary dilation, adhesiolysis, and fundoplication reversal or esophagectomy. Choice of approach is best done through multidisciplinary consensus. Conclusions: A multidisciplinary approach to patients with persistent and recurrent symptoms after myotomy can best tailor the therapeutic approach based on symptom causation.

Plummer Vinson Syndrome in a Young African American Female: A Rare Entity

Plummer Vinson syndrome (PVS), also known as Paterson-Brown-Kelly syndrome, is a rare clinical condition characterized by a triad of iron deficiency anemia, esophageal webs, and dysphagia. PVS has been rarely reported in African American females. In this case, however, we report a 26-year-old African American female with a 10-year history of iron deficiency anemia, who presented with severe fatigue and dysphagia. Her work-up revealed severe iron deficiency anemia, and upper endoscopy showed two esophageal rings. A 26-year-old African American female presented with severe fatigue, headache, and dysphagia for 5 months. The dysphagia was predominantly to solid foods compared to liquids. Patient also has a 10-year history of iron deficiency anemia and has stopped taking iron supplementations for the last 2 years. Physical examination revealed severe conjunctival pallor but was otherwise normal. Routine investigations revealed severe microcytic anemia with hemoglobin of 4.2 g/dL, mean corpuscular volume of 62.5 fL, and a ferritin level of 2 ng/dL. A modified barium swallow study showed two esophageal impressions in the cervical region, which were concerning for esophageal webs. Patient later underwent an esophagogastroduodenoscopy (EGD) which showed two upper esophageal rings; one semi-circumferential at 15 cm and a second circumferential at 17 cm from the incisors. A pediatric size scope was used to bypass the rings. She was initiated on iron replacement therapy with no symptomatic improvement of her dysphagia after 6 months of treatment. She then had a follow up EGD with wire-guided savary dilation with 8 mm dilator which resulted in an immediate improvement of her symptoms. Patient remained symptom free in her 2 months follow-up. PVS is characterized by iron deficiency anemia, esophageal webs, and dysphagia. It is predominantly seen in middle aged Caucasian females. Our case is unique because only a few cases of PVS have been reported in African American females. PVS is also a precancerous condition. Therefore, patients with this syndrome are at increased risk of developing gastrointestinal squamous cell carcinomas. Since nutritional deficiency is one of the major causes of iron deficiency anemia, the improvement in nutritional status of populations worldwide has led to a decreased incidence of PVS. Dysphagia in PVS usually responds to iron supplementation. However, resistant cases may require endoscopic dilatation of the webs as in our patient.1776_A Figure 1. EGD image Showing the first upper esophageal web, 15 cm from the incisors.1776_B Figure 2. EGD image Showing the second esophageal web, 17 cm from the incisors.1776_C Figure 3. Esophageal ring post wire-guided savary dilation with a satisfactory mucosal tear shown.

Efficacy and safety of lumen-apposing metal stent for benign gastrointestinal stricture.

Management of benign gastrointestinal (GI) strictures refractory to primary (balloon and savary dilation) and secondary (steroid injection, fully covered self-expanding metal stent, incision therapy) treatment modalities remains a challenge. Lumen-apposing metal stents (LAMSs), originally designed for the management of pancreatic fluid collections, are an attractive option for GI stricture because of their anti-migratory property, attributable to their saddle-shaped design. In this article, we reviewed 70 patients from 12 original studies who received LAMS for refractory (68/70) or treatment-naïve (2/70) benign GI stricture. The technical and clinical success rates were 98.6% (69/70) and 79.7% (55/69), respectively. Endoscopic placement, with or without fluoroscopic guidance, was generally successful, with only a minority requiring endoscopic ultrasound (EUS) guidance where the lumen was completely obscured. The majority of the strictures were short (≤1 cm), but comparable technical and clinical success was noted in isolated cases with long strictures, where 2 overlapping LAMSs were placed. For the overall population, a failure rate of 21.5% (14/69) was noted and was attributed to either lack of follow up, or to persistent or de novo symptoms requiring stent removal/exchange or surgical referral. One perforation (1.4%), five stent migration events (7.1%), two bleeding events (2.9%) and two de novo strictures proximal to the LAMS (2.9%) were reported for the entire study cohort. No mortality was attributable to LAMS placement. Although experience is still evolving, LAMS placement guided by esophagogastroduodenoscopy or EUS is a technically feasible and safe procedure with good clinical outcomes for benign refractory GI strictures.