Pediatric Endoscope Research Articles

Implementation of a Microbiological Surveillance Protocol in a Portuguese Tertiary Care Academic Endoscopy Unit

Introduction: International societies recommend microbiological surveillance of endoscopes to reduce the incidence of endoscope-associated infections, particularly for duodenoscopes. However, surveillance protocols are not internationally standardized, both regarding sample collection, processing, and culture. This study aims to provide a framework protocol encompassing the experience of a tertiary large volume endoscopy center and the microbiology laboratory for collecting and culturing of endoscope samples for microbiological surveillance. Methods: A sample collection and processing protocol was designed as a result of a cooperation between the Endoscopy Center of the Gastroenterology Department and the Microbiology Laboratory of the Department of Clinical Pathology. This protocol reflects international recommendations in this topic and the human and technological resources of the involved departments. Results: The established protocol for collecting samples varies according to the type and model of endoscope. The specimens are collected as sterile saline liquid samples, as well as swabs (with and without transport media). Together with the collection of samples from the endoscope, samples from the final rinse water as well as the water bottle are also collected. For duodenoscopes and curvilinear echoendoscopes, we perform microbiological surveillance every 3 months; for gastroscopes and colonoscopes, at least, once a year; and for specific endoscopes, such as the pediatric or dual-channel therapeutic endoscopes, enteroscopes, or radial echoendoscopes, every 6 months. Conclusion: Endoscopy units should have detailed protocols for microbiological surveillance of endoscopes. These protocols should be drawn up by a multidisciplinary team that includes endoscopy nurses, gastroenterologists, microbiologists, and the antimicrobial stewardship team, following international recommendations, adapted to each institution resources.

In Vivo Raman Spectroscopy Reveals Biochemical Composition of the Esophageal Tissue in Pediatric Eosinophilic Esophagitis.

Biochemical alterations in the esophagus of patients with eosinophilic esophagitis (EoE) are poorly understood. We used Raman spectroscopy through a pediatric endoscope to identify key Raman features reflective of the esophageal biochemical composition to differentiate between children with EoE from non-EoE controls and between children with active (aEoE) and inactive EoE (iEoE). Spectral measurements were obtained using a customized pediatric endoscope-compatible fiber-optic Raman probe in real time during an esophagogastroduodenoscopy. Chemometric analysis was performed to identify key Raman features associated with EoE. Pearson correlation analysis was used to assess relationship between the key Raman features and EoE activity indices. Their diagnostic utility was ascertained using the receiver operator characteristic curve analysis. Forty-three children were included in the study (EoE = 32 [74%] and non-EoE control = 11 [26%]; aEoE = 20 [63%] and iEoE = 12 [37%]). Raman intensities assigned to lipids, proteins, and glycogen:protein ratio accurately distinguished children with EoE from those without EoE and aEoE from iEoE. They significantly correlated with EoE activity indices. The Raman peak ratio for lipids had 90.6% sensitivity, 100% specificity, and an area under the curve of 0.95 to differentiate children with EoE from non-EoE controls. The glycogen:protein ratio had 70% sensitivity, 91.7% specificity, and an area under the curve of 0.75 to distinguish children with aEoE from iEoE. Real-time intraendoscopy Raman spectroscopy is an effective method for identifying spectral markers reflective of the esophageal biochemical composition in children with EoE. This technique may aid in the diagnosis and monitoring of EoE and help to elucidate EoE pathogenesis.

A multifaceted comparative analysis of image and video technologies in gastrointestinal endoscope and their clinical applications.

This paper presents a comprehensive exploration of endoscopic technologies in clinical applications across seven tables, each focusing on a unique facet of the medical field. The discourse begins with a detailed analysis of pediatric endoscopes, highlighting their diagnostic capabilities in various conditions. It then delves into the specifications and applications of globally recognized capsule endoscopy devices. Additionally, the paper incorporates an analysis of advanced imaging techniques, such as Narrow Band Imaging (NBI), Flexible Spectral Imaging Color Enhancement (FICE), and i-scan, which are increasingly being integrated into ultrathin gastrointestinal (GI) endoscopes. Factors like technological capabilities, light source, camera technology, and computational constraints are evaluated to understand their compatibility with these advanced imaging techniques, each offering unique advantages and challenges in clinical settings. NBI, for instance, is lauded for its user-friendly, real-time enhanced imaging capabilities, making it effective for early detection of conditions like colorectal cancer and Barrett's esophagus. Conversely, FICE and i-scan offer high customizability and are compatible with a broader range of endoscope models. The paper further delves into innovative advances in movement control for Nasojejunal (NJ) feeding tube endoscopy, elucidating the potential of AI and other novel strategies. A review of the technologies and methodologies enhancing endoscopic procedure control and diagnostic precision follows, emphasizing image and video technologies in pediatric endoscopy, capsule endoscopes, ultrathin endoscopes, and their clinical applications. Finally, a comparative analysis of leading real-time video monitoring endoscopes in clinical practices underscores the continuous advancements in the field of endoscopy, ensuring improved diagnostics and precision in surgical procedures. Collectively, the comparative analysis presented in this paper highlights the remarkable diversity and continuous evolution of endoscopic technologies, underlining their crucial role in diagnosing and treating an array of medical conditions, thereby fostering advancements in patient care and clinical outcomes.

Eustachian Tube Dysfunction in Children with Adenoid Hypertrophy: The Effect of Intranasal Azelastine-Fluticasone Spray Treatment on Middle Ear Ventilation and Adenoid Tissue.

Eustachian tube dysfunction (ETD) is frequent in children with adenoid hypertrophy (AH). Although the most common treatment of AH is surgical removal of adenoid tissue, numerous studies have reported the efficacy of intranasal steroids. The effects of the intranasal steroid and azelastine combination on AH and ETD have not been reported before. In this study, we tried to determine the effects of 3-month intranasal Azelastine-Fluticasone dipropionate combination (Aze-Flu) treatment in children with ETD and AH. 100 children who had open mouth sleep, snoring, and sleep apnea and were diagnosed with AH and ETD participated in this study. The mean age was 7.73 ± 2.37 (4-14 years). The rates of adenoid tissue hypertrophy and choanal occlusion were evaluated using a rigid pediatric nasal endoscope and reassessed after 3 months of Aze-Flu nasal spray treatment. The function of the Eustachian tube (ET) was evaluated before and after medical treatment using the Eustachian tube score, the Eustachian dysfunction test-7 (ETS-7) and tubomanometry (TMM). The results were evaluated in 100 patients with AH and ETD. The adenoid tissue to choana rate was 82% before treatment and decreased to 37% after treatment. The ETS-7 test score was 6.36 before treatment and increased to 9.72 at the end of 3 months. Both the regression of the adenoid tissue and the improvement in the Eustachian function scores were statistically significant (p < 0.05). AH significantly increases the frequency of ETD. In this study, it was observed that Aze-Flu treatment was significantly effective in both regression of the adenoid tissue and Eustachian tube dysfunction. We believe that it can be applied as an initial therapy in children with AH and associated ETD.

S2415 A Common Procedure Leads to the Diagnosis of an Uncommon Cause of Dysphagia

Introduction: Epithelioid angiosarcoma is a rare and aggressive soft-tissue endothelial cell neoplasm that accounts for less than 1% of sarcomas and rarely occurs in the gastrointestinal tract. We report a case of a 41-year-old female without prior medical history who presented with progressive dysphagia and unintentional weight loss. Case Description/Methods: Initial upper endoscopy found to have a narrowed gastroesophageal junction (GEJ) that required balloon dilation to 10mm and the use of a pediatric upper endoscope to traverse. Only thickened gastric folds at the cardia were seen, which were biopsied. Histology revealed chronic active moderate gastritis with Helicobacter pylori and patient was treated with triple therapy. However, due to ongoing dysphagia a contrast enhanced computed tomography (CT) of the chest was obtained and demonstrated thickening of the distal esophageal wall. Next, an endoscopic ultrasound (EUS) was performed and demonstrated unusual thickening of the distal esophageal wall. Fine needle biopsy (FNB) revealed high-grade esophageal epithelioid angiosarcoma of the distal esophagus. She was treated with neoadjuvant chemo- and radiotherapy followed by a robotic laparoscopic Ivor Lewis esophagectomy. The patient has been disease free after 1 month of follow up (Figure). Discussion: This case highlights the importance of timely recognition and diagnosis of dysphagia caused by pseudo-obstruction. Timely CT and EUS/FNB should be performed to evaluate for less common entities.Figure 1.: Case image.

Endoscopic Retrograde Cholangiopancreatography in Children: Feasibility, Success, and Safety with Standard Adult Endoscopes and Accessories.

PurposeThe role of endoscopic retrograde cholangiopancreatography (ERCP) in the management of hepatobiliary and pancreatic diseases in the pediatric population was not well defined until recently. Our aim was to determine the feasibility, outcomes, and safety of ERCP in a local pediatric population, particularly using standard adult endoscopes and accessories.MethodsThis retrospective study was conducted at the National Hospital of Sri Lanka. Pediatric patients (aged <16 years) who underwent ERCP from January 2015 to December 2020 were included in the study. Data, including patient demographics, indications for the procedure, technical details, and associated complications, were collected from the internal database and patient records maintained at the hospital.ResultsThe study included 62 patients who underwent a total of 98 ERCP procedures. All the procedures were performed by adult gastroenterologists using standard adult endoscopes and accessories. The mean age was 11.01±3.47 years. Pancreatic diseases were the major indications for most of the procedures (n=81, 82.7%), with chronic pancreatitis being the most common. Seventeen procedures (17.3%) were carried out for biliary diseases. Overall cannulation and technical success rates were 87.8% and 85.7%, respectively. Stent placement was the most common therapeutic intervention (n=66; 67.4%). Post-ERCP pancreatitis was the most common complication, occurring in eight patients (8.2%).ConclusionERCP can be successfully and safely performed in pediatric populations using standard adult endoscopes and accessories with complications similar to those of adults. Adult ERCP services can be offered to most pediatric patients without additional costs of pediatric endoscopes and accessories.

External Sphincter Injection of Onabotulinum-A Toxin in Treatment of Children with Bladder- Sphincteric Dyssynergia

Abstract Background: The first application of Onabotulinum-A Toxin in urology was its injection into the urinary sphincter to treat neurogenic detrusor-sphincter dyssynergia in quadri-plegic men. Since that first report by Dyskatra et al., in 1988, the results of focal Onabotulinum-A Toxininjections into the sphincter, the bladder wall and lately into the prostate have raised the interest of the urology community in this promising new therapeutic modality. This is an evidence-based review of the current indications, techniques and outcomes of Ona-botulinum-A Toxin injections into the urethral sphincter. Aim of Study: We evaluated the effect of detrusor injection of onabotulinum-A toxin into the urethral urinary sphincter in children with bladder-external sphincter dyssynergia to decrease urethral resistance and improve voiding. Material and Methods: Prospective treatment was per-formed in 25 children 4 to 15 years old (mean age 7) with bladder-neck dyssynergia using onabotulinum-A toxin. Pre-operatively all children were evaluated by ultrasound, voiding cystourethrography, excretory urography, magnetic resonance imaging and urodynamic studies, including pressure flow, electromyography and uroflowmetry. Using a rigid pediatric endoscope and a 4Fr injection needle 50 to 100 IU onabotu-linum-A toxin was injected into the external sphincter at the 5 and 7 o'clock positions. Follow-up was 6 to 15 months. Repeat injections every 6 months were given according to the response with a maximum of 3 injections. Results: Immediately after onabotulinum-A toxin injection all except 1 patient were able to void without catheterization. No acute complications occurred. Postoperatively post-void residual urine decreased by 30%, detrusor leak point pressure decreased significantly by a mean ± SD of 66±18 vs 37±4cm H2O and uroflowmetry showed a marked increase in maximum urine flow of 7±2 vs 17.3±8ml per second. Conclusions: Urethral sphincter onabotulinum-A toxin injection could be considered a reliable treatment modality in children with nonneurogenic neurogenic bladder after the failure of conservative therapy.

Tu1028 POST-SURGICAL OR SPONTANEOUS RECALCITRANT LEAK MANAGEMANT USING NOVEL APPROACHES: ENDOSCOPIC DUPLICATION OF GUT VIA EXTRALUMINAL STENT (EDGES) & SUTURE OBTRUDED COVERED STENTS (SOCS)

Gastrointestinal fistulas, leaks, & perforations are associated with high morbidity and mortality as well as prolonged hospitalization. In 30-40% of patients the current management strategies such as wait & watch, transcutaneous drainage and endoscopic closure fail to resolve the symptoms adequately. We envisioned novel minimally invasive approaches to manage these defects. EDGES: used for recalcitrant fistulas with a para-luminal abscess cavity in the proximity of the GI tract. A pediatric upper endoscope was advanced through the fistula into the extra-luminal space. Under direct endoscopic view an intentional perforation was created to connect the distal most end of the abscess cavity to the gut lumen. A fully covered metal stent (Gore Viabil 10x100mm) was deployed from the gut lumen, through the fistula, across the abscess cavity, and back through the new perforation into the gut lumen. This approach prevented the entry of gut contents into the abscess to eventually allow the healing (Figure 1-4). SOCS: used in patients with recalcitrant fistulas with an abscess cavity that was not in the proximity of the GI tract to accomplish EDGES. A fully covered metal stent (Gore Viabil 10x100mm) was deployed ex-vivo. One of the ends of the stent was obtruded by proline suture & tested for complete sealing. Using a push or pull technique the obtruded end of the stent was passed from the lumen, through the fistula, into the abscess cavity & the open end remained in the gut lumen plugging the fistulous opening (Figure 5). We assessed the outcome of patients who underwent these techniques between April 2018 to Nov. 2019. The underlying cause of the defect and clinical follow-up as well as endoscopic & radiographic data was collected. We defined an endoscopic response as absence of a leak under endoscopic or fluoroscopic assessment of the GI defect. A total of 15 patients (5 female), mean age of 48 years were managed using EDGES in 6 patients & SOCS in 9 patients. Overall success of these novel approaches was 80%. Mean time to response was 89.5 days with an average follow-up of 166.3 days. EDGES was successful in 83% cases and SOCS was successful in 78% cases. 7(47%) patients had IBD, and one had malignancy; 5(33%) defects were gastric, 3(20%) were jejunal, 3(20%) were ileal, and 4(26.7%) were colonic. The average number of procedures prior to EDGES/SOCS was 5.4. Of the 3 patients who did not respond to endoscopic therapy, two patients failed therapy due to stent migration. Our novel, unconventional EDGES and SOCS approaches are promising to manage recalcitrant leaks. A consideration should be given to these approaches to avert high-risk surgery in a subset of patients.

Predicting pediatric esophageal wall thickness: An EUS study.

Background and Objective:EUS has been shown in two small series to be capable of documenting increases in the total esophageal wall thickness (TWT) in children and adults with eosinophilic esophagitis (EoE). To apply EUS-derived TWT in clinical situations or in scientific investigations in pediatric EoE, measurements of esophageal TWT in children of differing ages and heights are required.Materials and Methods:Thirty patients (18M: 12F, 7 months to 20 years and 10 months) with a history of esophageal symptoms, but no endoscopic or histologic criteria of EoE were studied using a through the scope 20 MHZ Olympus Ultrasound miniprobe UM-3R (Olympus America, Center Valley Pa 18034) through a GIF Q180 or 160 (Olympus) standard pediatric upper endoscope. The mucosa, the mucosa plus submucosa, and the TWT were measured in the mid- and distal esophagus immediately before taking diagnostic biopsies.Results:Measurements from both sites showed a statistically significant increase in TWT as a function of age (P < 0.001) and height (P < 0.001), as did the individual layers. The width of the mucosa and the submucosa were equivalent and together, they contributed more than half of the entire TWT. There were no significant differences between the means of the mid- and distal esophageal measurements. A multiple regression equation that can predict TWT based on age, with 95% confidence limits, is presented.Conclusions:EUS has demonstrated that esophageal TWT in a cohort of control children correlates with height and with age and has provided insights into the organization of the esophageal wall. Esophageal TWT values obtained by EUS can now be interpreted to recognize esophageal wall thickening throughout childhood.

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.

Nasendoscopic Findings of Velopharyngeal Sphincter in Operated Cleft Palate Patients: Is It Different than Normal Population.

Objective This study was aimed for nasendoscopic assessment of velopharyngeal sphinteric closure in patients with operated cleft palate and to compare it with normal population. Design A cross-sectional study was done in a tertiary cleft care center in 30 patients with operated cleft palate after a minimum of 6 months of their surgery and 30 randomly selected volunteers with normal speech. Both groups were one-time evaluated by three observers using 70 degree rigid nasendoscope and/or pediatric fiber optic endoscope. Velopharyngeal sphincter closure characteristics in terms of pattern of closure, dominant element involved in the closure, degree of palatal movement, and completeness of the closure were evaluated, recorded, and compared between the groups. Results In both groups, the most common pattern of closure is coronal and soft palate is the dominant mobile element in velopharyngeal closure. All normal subjects showed complete closure of the sphincter with good soft palate movement. But only 50% of the operated patients with cleft showed complete closure and even less than them had good movements of the soft palate. Conclusions Although the pattern of the closure in the operated patients is similar to the normal subjects, the movement of the soft palate and completeness of the velopharyngeal sphincter closure still remain the problem in the operated palate patients.

The interobserver agreement of optical features used to differentiate benign from neoplastic biliary lesions assessed at balloon-assisted cholangioscopy.

Balloon-assisted cholangioscopy allows mucosal assessment of the biliary tree with pediatric endoscopes. No validated optical criteria exist to differentiate benign from neoplastic biliary lesions. We aimed to identify, validate, and revalidate optical features differentiating benign from neoplastic biliary lesions. Furthermore, we aimed to determine whether cholangioscopic appearance allows endoscopists to accurately differentiate benign from neoplastic biliary lesions. Baseline: from 44 de-identified balloon-assisted cholangioscopy videos, a blinded investigator analyzed potential optical features distinguishing benign from neoplastic biliary lesions. during the initial "teaching phase," 20 endoscopists viewed video clips of 11 optical features identified in the baseline study. At the subsequent "test phase," 20 further video clips were assessed by the endoscopists blinded to clinical details and questionnaires completed for the presence or absence of optical features, favored diagnosis and diagnostic confidence. Revalidation: The six identified optical features from the validation study with at least moderate agreement were revalidated the same way 12months later assessing 20 new lesions. Baseline: 11 optical features were found to differentiate benign from neoplastic biliary lesions. Validation and revalidation: six optical features demonstrated at least moderate interobserver agreement (irregular margin, dark mucosa, adherent mucous, papillary projections, tubular, or branched/disorganized surface structures). Endoscopists correctly diagnosed lesions as benign in 89% and neoplastic in 83%. When highly confident, endoscopists correctly diagnosed 96% of benign and 87% neoplastic lesions. Six features were validated and revalidated to differentiate benign from neoplastic biliary lesions. When highly confident with a diagnosis, endoscopists usually differentiate benign from neoplastic biliary lesions.

Cannot intubate and cannot ventilate scenario in an infant for airway assessment.

Sir, Cannot intubate and cannot ventilate scenario, in general, is rare, especially in infant age group, but results in calamitous events if it occurs. Here, we report the management of a case of cannot intubate and cannot ventilate in an infant with a history of stridor. Case A 6-month-old girl was brought by an ambulance to our tertiary care hospital with difficulty in breathing and severe stridor. It was decided to perform airway assessment by ENT surgeon to determine the possible causes of stridor due to failure of improvement despite maximum medications given by pediatrician. Preoperative assessment showed an infant with mixed stridor requiring about 4 L of oxygen to maintain oxygen saturation above 90%. Suprasternal and intercostal recessions with conducted sounds could be heard on the chest. Chest X-ray was normal. No premedication was given and she was shifted to the operation theatre. Supraglottic airway assessment was done awake with topical anesthesia using flexible fiberoptic endoscope which was normal. Subglottic endoscopic examination was decided under general anesthesia. She was induced with sevoflurane to maintain spontaneous ventilation for dynamic airway assessment. The spontaneous ventilation was assisted with great difficulty after loss of consciousness. The ENT surgeon used pediatric rigid endoscope. It revealed subglottic mass occluding 90% of the area, and it was not possible to bypass the mass with the smallest endoscope available (size 2.7 mm) [Figure 1]. The infant started to desaturate after endoscopic manipulations with difficulty to ventilate. Intubation failed with the smallest tube size 2. Ventilation was tried but was not effective. The saturation and heart rate started to drop with the scenario of cannot intubate and cannot ventilate. Atropine was given and external cardiac massage started as saturation dropped to less than 50% and heart rate fell to less than 60 beats per minute. Emergency tracheostomy was done successfully in 7 min and saturation improved to 99%. Infant regained consciousness and spontaneous breathing. Computed tomography was done which revealed a small hypodense area measuring 0.5 cm in diameter in the region of glottis. LASER marsupialization of the subglottic cyst was done successfully after 2 days from emergency tracheostomy.Figure 1: Subglottic mass obstructing 90% of the airwayUnfortunately, there is no definitive algorithm to follow for difficult airway in case of patients aged less than 1 year of age. Difficult airway society (DAS) published algorithm of cannot intubate and cannot ventilate in a paralyzed anesthetized child aged 1–8 years in 2015.[1] In the present case, we skipped insertion of supraglottic devices, percutaneous transtracheal jet ventilation (PTJV), rigid bronchoscopy ventilation, and percutaneous cannula cricothyroidotomy (PCC) for different reasons. Supraglottic devices cannot be used due to distal mechanical obstruction. PTJV is relatively contraindicated due to near total airway obstruction below vocal cord.[2] In addition to risk of serious complications such as air embolism, extensive cervical emphysema, pneumothorax, and damage to the esophagus occur if jet ventilation is tried with misplaced cannula.[3] Rigid bronchoscopy ventilation was impossible as the smallest endoscope of size 2.7 mm could not bypass the mass. PCC is not easy in infants. Moreover, it is challenging to obtain a proper angle to insert PCC without the risk of perforation of posterior tracheal wall.[4] Emergent tracheostomy should be considered as the first step if an ENT surgeon is available.[5] The management of cannot intubate, cannot ventilate scenario needs vast experience in airway assessment, unshaken confidence, with extreme cooperation between anesthetists and ENT surgeons. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Linitis Plastica Presenting as Dysphagia Thought to Be Pseudoachalasia

A 79 yr old M with past medical history of Barrett's esophagus s/p ablation presents with progressive dysphagia to solid foods. EGD showed stenosis at the GEJ, no tumor seen, but firm stricture. Biopsies showed unremarkable mucosa, negative for H. Pylori, but concern for extrinsic compression and congestive gastropathy. CT did not show any masses. Barium esophagram showed tapering of esophagus to the GEJ with a ‘bird's beak appearance. Manometry was confounded by a second high pressure zone beneath LES concerning for probe coiling. Manometry did not clearly meet any Chicago criteria. Possible diagnosis of achalasia type II questioned due to elements of preserved peristalsis. Repeat EGD again demonstrated severe stenosis, and a dilated esophagus. Biopsies were repeated which demonstrated no abnormalities. The patient was referred to thoracic surgery for surgical correction of achalasia. Third EGD with sequential dilation 21-33Fr dilators. Subsequently during admission for TIA, he had worsening dysphagia and CT chest demonstrated a mild thickening of the distal esophagus, previously seen but not reported. Repeat EGD again noted a marked narrowing that could not be traversed with standard endoscope but crossed with pediatric endoscope. In the stomach there were diffusely thickened folds and poor distensibility. EUS demonstrated irregular thickening of the mucosal layers and submucosal layers measuring 12mm thickness extending from the GEJ throughout the stomach to antrum. No lymphadenopathy noted. Biopsies demonstrated poorly differentiated carcinoma, immunostains were positive for AE1/3 and negative for CD45. Ki67 is positive in ˜70% tumor cells. PET scan showed widespread bone metastasis and hypermetabolic uptake in relation to the gastric mucosa. We describe a rare case of linitis plastica presenting as pseudoachalasia. However, the accuracy of endoscopy ranges widely, depending on the gross tumor growth pattern and the anatomic location of the tumor, with a sensitivity of only 33-73% observed in linitis plastica patients. Imaging and early endoscopy were concerning for achalasia, particularly in the setting of pathognomonic presence of “bird's beak” on barium swallow. Was questioned by manometry, however study had some uncertainty due to concern for possible coiling of the probe. This case serves to highlight the importance of EUS when endoscopy, imaging, and manometry are inconsistent.2969_A Figure 1 No Caption available.2969_B Figure 2 No Caption available.

Unusual Presentation of Boerhaaveʼs Syndrome: Self-Limiting Spontaneous Perforation of the Mid-Esophagus

Spontaneous rupture of the esophagus as described by Dr. Boerhaave in 1724, is associated with high mortality, 36 to 40%. We report a patient who suffered an unusual spontaneous perforation of the midesophagus and survived with conservative therapy. An 84-year-old man presented with persistent chest pain; 2 hours earlier while eating pork he felt that it got stuck in his lower chest. He noted that anything he drank came back up though he could swallow his saliva. He had no history of heartburn, chronic cough, or regurgitation. His dentition was good. There was no history of hematemesis, melena, fever, or chills. There was no significant surgical history. Examination: bp-155/76, p-83, T-36.8, r-18/min, wt-120 lbs. He was alert and oriented. The neck, heart, lungs, and abdomen were unremarkable. Labs: WBC-7,100, Hg-15.3. At EGD, immediately upon entering the esophagus, a deep linear rent with exposed muscle was seen leading down to two lumens (photo A). A pediatric endoscope passed through a lumen into the stomach which appeared normal with no injury at the cardia. CT scan demonstrated a large hiatal hernia and a significant amount of gas within the mediastinum, but no effusion. Water-soluble contrast esophagogram showed extravasation of contrast in the mid esophagus slightly inferior to the aortic arch (photo B). The patient was treated conservatively with intravenous antibiotics. On day 6, repeat esophagogram revealed a smooth focal narrowing at the level of the aortic arch but no contrast extravasation (photo C). The patient was discharged on day 11 tolerating clear liquids and off antibiotics. The Dutch admiral described by Dr. Boerhaave had a history of being a glutton. In that era, it was common to induce vomiting to allow immediate indulgence in more food. The admiral was found to have olive oil and roast duck in his left pleural cavity. Spontaneous rupture of the esophagus is rare and occurs predominantly in the distal esophagus. In the past it was almost universally fatal. The first drainage of mediastenitis was recorded in 1941. Successful surgical closure of esophageal perforation was performed by Dr.Barrett in 1947. We described a patient who had an even more unique presentation: spontaneous perforation of the mid-esophagus. Surprisingly he did very well with just conservative therapy and was discharged after only eleven days.1808_A Figure 1. Esophageal wall rent with perforation1808_B Figure 2. Contrast esophagogram with contrast extravasation (day 0)1808_C Figure 3. Contrast esophagogram without contrast extravasation (day 6)

An Unusual and Rare Case of Esophageal Stricture as a Result of Systemic Chemotherapy for Solid Malignancy

Esophageal stricture secondary to isolated chemotherapy is exceptionally rare. We present the case of a patient receiving adjuvant chemotherapy who subsequently developed acute, severe esophageal stricture. A 34 year old male with a diagnosis of metastatic testicular seminoma presented to the hospital following a left orchiectomy for initiation of chemotherapy. He was started on Etoposide and Cisplatin chemotherapy and received an initial five days of therapy. Two weeks following the first cycle of chemotherapy, the patient developed symptoms of dysphagia and odynophagia, and all other review of systems was negative. He had no prior history of caustic ingestions or radiation therapy. The primary team noted oral thrush which was empirically treated with antifungal therapy. As symptoms persisted throughout the second and third cycles of therapy, a gastroenterology consult was called. On exam, patients vitals were stable, physical exam was unremarkable with resolution of thrush, and initial labs were consistent with primary malignancy. Gastrograffin esophogram revealed a narrowing in the distal third of the esophagus. CT scan revealed esophageal distension to the level of the GE junction. On EGD, a site of moderate stenosis measuring 1cm x 9mm was found 29 cm from the incisors which was traversed with mild resistance. An additional site of stenosis was found 30 cm from the incisors. A severe stenosis with a luminal diameter of 3 mm was found 6 cm further. A pediatric endoscope was used due to severity of stenosis. Histopathologic examination revealed nonspecific esophagitis. Cytology was negative for malignancy. No fungal elements were noted. Patient was offered but refused treatment with endoscopic dilation. The patient opted for a laproscopic PEG tube placement to optimize nutrition. The acute onset of esophageal stricture, without any prior radiation treatment or secondary causes of dysphagia led to the diagnosis of esophageal stricture secondary to use of systemic chemotherapy. We suspect esophageal stricture as complication of systemic chemotherapy as the patient had no history of long standing acid reflux or known exposure to established nonpeptic causes of stricture. This unexpected adverse outcome is likely caused by an interplay of mucosal damage associated with chemotherapy and the rapidly proliferating cells that line the gastrointestinal tract. These findings should prompt further investigation into the pathways of such a rare isolated injury.Figure: Gastrograffin esophogram showing generalized distal narrowing of the distal esophagus.Figure: Endoscopic view of severe stenotic stricture at 36 cm from incisors measure 3 mm in inner diameter.Figure: Endoscopic view of esophagus with visualization of stricture and friable mucosa.

Airflow Shape Is Associated With the Pharyngeal Structure Causing OSA

OSA results from the collapse of different pharyngeal structures (soft palate, tongue, lateral walls, and epiglottis). The structure involved in collapse has been shown to impact non-CPAP OSA treatment. Different inspiratory airflow shapes are also observed among patients with OSA. We hypothesized that inspiratory flow shape reflects the underlying pharyngeal structure involved in airway collapse. Subjects with OSA were studied with a pediatric endoscope and simultaneous nasal flow and pharyngeal pressure recordings during natural sleep. The mechanism causing collapse was classified as tongue-related, isolated palatal, lateral walls, or epiglottis. Flow shape was classified according to the degree of negative effort dependence (NED), defined as the percent reduction in inspiratory flow from peak to plateau. Thirty-one subjects with OSA (mean apnea-hypopnea index score ± SD, 54 ± 27 events/h) who were 50 ± 9 years of age were studied. NED was associated with the structure causing collapse (P< .001). Tongue-related obstruction (n= 13) was associated with a smallamount of NED (median, 19; interquartile range [IQR], 14%-25%). Moderate NED was found among subjects with isolated palatal collapse (median, 45; IQR, 39%-52%; n= 8) andlateral wall collapse (median, 50; IQR, 44%-64%; n= 8). The epiglottis was associated with severe NED (median, 89; IQR, 78%-91%) and abrupt discontinuities in inspiratory flow (n= 9). Inspiratory flow shape is influenced by the pharyngeal structure causing collapse. Flow shape analysis may be used as a noninvasive tool to help determine the pharyngeal structure causing collapse.

G-Tube Guided Endoscopic Retrograde Cholangiopancreatography; A Lifesaving Procedure

Introduction: Acute cholangitis is a consequence of obstruction and infection of the biliary tract. Endoscopic retrograde cholangiopancreatography (ERCP) is key in the management of choice as acute cholangitis as stone extraction and/or stent insertion establishes biliary drainage and relief of infection. Anatomical variation following Roux-en-Y gastric bypass surgery or patients with esophageal obstructions like in our case are prone to make the early intervention by ERCP a challenge . We describe a case report of septic cholangitis in a patient whom gastrostomy tube (G-tube) access allowed the performance of successful ERCP which was otherwise impossible. Case report: 75-year-old male with multiple comorbidities and esophageal cancer status post G-tube placement for malnutrition who presented with cholangitis. On admission, the patient had fever, nausea and decrease in level of activity. His initial evaluation showed that the patient had a sepsis with a fever of 99.6, leukopenia with WBC of 2.0, elevation in the liver enzymes with alkaline phosphatase of 238, alanine aminotransferase of 48, total bilirubin 1.2 and lactic acid of 3.2. His infectious workup included blood cultures that grew Enterobacter cloacae. His CT scan showed stone in the common bile duct. MRCP demonstrated a 1.3 cm obstructive distal common bile duct stone with extrahepatic and intrahepatic biliary dilatation. ERCP was unsuccessful due to a partially obstructing esophageal mass. After changing to a small pediatric endoscope, the scope was able to be passing the mass and a showed a good visualization of the ampulla but therapeutic intervention was not successful. Patient was brought back for a second attempt using his G-tube. A wire was passed under fluoroscopy adjacent to the G-tube then the G-tube was removed. Then a stent anchoring system was used with 3 anchors being placed. Subsequently, an axios stent (lumen-apposing self-expandable metallic stent) was placed under combined direct vision and fluoro. The stent was then sutured down in order to prevent migration. Following placemen of the stent balloon dilatation was performed inside the stent up to 15 mm in size. Following dilatation over a jag wire the ERCP scope was passed through the stent and used to cannulate the ampulla. A sphincterotomy was performed and the stone was removed with a balloon catheter. Following completion of the stone removal the Axios stent was then removed and a G-tube was replaced and confirmed with contrast for position. The patient tolerated the procedure without any complication, his bilirubin normalized and the patient discharged in stable condition the next day on a total course of 14 days of antibiotics. Conclusion: In cholangitis patient with limited access, G-tube sites provide a useful access value to examine the gastrointestinal track for therapeutic intervention.

A Case of Plummer-Vinson Syndrome and Celiac Disease

Introduction: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and cervical esophageal web. Celiac disease and Plummer-Vinson syndrome are both known to be associated with iron deficiency. There are very few reported cases of both being seen in the same patient. We report a case of a female found to have Plummer-Vinson syndrome along with celiac disase. Case report: We present a case of a 48 year old female with a long standing history of iron deficiency anemia. She had been following up with her primary care physician for a number of years for iron deficiency anemia and had extensive workup done. She required intermittent blood transfusions and was on B12 and Iron. She had been seen by gastroenterology 5 years prior to this visit, at that time it was recommended she have an upper endoscopy and colonoscopy which she refused due to being pregnant at the time. She was told to follow up with gastroenterology for an upper endoscopy and colonoscopy after her pregnancy was over. Years later she re-presented with anemia and dysphagia. An upper endoscopy and colonoscopy were scheduled. Upper endoscopy demonstrated a benign appearing intrinsic stenosis 24 cm from the incisors. A pediatric endoscope was required to traverse the web-like stenosis in the proximal esophagus. Duodenal biopsies were taken to check for celiac disease. Colonoscopy showed the entire colon to be normal. Duodenal biopsies were consistent with immune-mediated injury such as gluten sensitivity. She was thought to have Plummer-Vinson syndrome in the setting of iron deficiency anemia secondary to celiac disease. It was recommended that the patient adhere to a gluten free diet. The patient was scheduled for upper endoscopy for esophageal dilation but was again lost to follow-up. Conclusion: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and a cervical esophageal web. Celiac disease and Plummer Vinson syndrome are rarely seen coinciding together in the same patient. Important point to remember is that if a patient is thought to have Plummer-Vinson syndrome they should be worked up for celiac disease as a cause of iron deficiency anemia. This case demonstrates this importance of keeping in mind that Plummer-Vinson syndrome and celiac disease can coexist in the same patient.

A Case of End-Stage Eosinophilic Esophagitis

Eosinophillic esophagitis (EoE) is an increasingly recognized cause of refractory heartburn and dysphagia. It is characterized by a chronic, immune/antigen-mediated esophageal dysfunction associated with eosinophil-predominant inflammation on histology. A diagnosis of EoE is often delayed because of symptomatic overlap with GERD and patients may present with severe complications such as strictures or esophageal fibrosis. An otherwise healthy 29-year-old Caucasian male with history of esophageal strictures requiring intermittent dilations presented with severe chest pain and food impaction sensation after eating a meal. Workup for cardiac cases of chest pain was negative. CT chest was concerning for possible esophageal micro-perforation. Empiric antibiotics were started for mediastinitis. Upper endoscopy revealed small caliber esophagus starting at 25 cm preventing the passage of an adult scope. A pediatric upper endoscope was passed with difficulty through the esophagus revealing a deep mucosal tear in the esophagus without any food bolus. Esophagus was noted to be narrow through the gastroesophageal junction. Distinct ring-furrow pattern suggestive of EoE was noted. Esophageal biopsies showed squamous mucosa with moderate to marked basal cell hyperplasia and increased numbers of intraepithelial eosinophils (> 45 per high power field). He was discharged home with plans for frequent serial dilations along with oral inhaled corticosteroids. Our patient had several prior endoscopies with dilations but was never diagnosed or treated for eosinophilic esophagitis. He has now progressed to end-stage eosinophilic esophagitis with a severely stenotic esophagus. He will likely have to be on a modified diet throughout his life.Figure 1Figure 2EoE is a relatively rare disease with an estimated prevalence of 25.9/100,000 patients affecting males more than females. Patients present with non-specific symptoms including heartburn, chest pain, dysphagia or epigastric pain, which often leads to a delay in diagnosis. Early identification and treatment of symptoms result in fewer complications. Prior studies have demonstrated a direct correlation with delay in diagnosis and prevalence of fibrotic features on endoscopy. A high degree of suspicion is required to diagnose this condition in patients who have persistent symptoms despite optimal PPI therapy and have concomitant allergies. Earlier diagnosis and treatment can prevent complications of esophageal fibrosis and stricture formation.