Descriptive Analysis of Pre-existing Data on Eosinophilic Esophagitis and Associated Morbidities in Cleveland Clinic Abu Dhabi, United Arab Emirates.

BACKGROUND: Data on eosinophilic gastrointestinal disorders (EGIDs) are limited in the United Arab Emirates. OBJECTIVE: This study aimed to describe the distribution of eosinophilic gastritis/gastroenteritis (EoG/EGE) and eosinophilic esophagitis (EoE) among patients whose data are recorded on the Cleveland Clinic Abu Dhabi (CCAD) systems. METHODS: Anonymized aggregate data were obtained from the Clinerion Patient Network Explorer (PNEx®) network installed at the CCAD on patients diagnosed with EoG/EGE (n=17, of whom 11 (64.7%) were females) or with EoE (n=118, of whom 35 (29.7%) were females) between 2015 and 2022; 63 (53.4%) were diagnosed between 2020 and 2022. RESULTS: The vast majority of EoE patients visited the gastroenterology clinic (111, 94.1%), followed by the primary care (70, 59.3%), the emergency medicine (60, 50.8%), and the immunology (59, 50%) units. A total of 84 patients with EoE also presented with another type-2 inflammatory condition including allergic rhinitis, food allergy, and atopic dermatitis. Proton pump inhibitors and corticosteroids were widely prescribed, and four patients with EoE have been commenced on biologics since 2020. However, heterogeneity of medication use was identified, warranting local clinical practice guidelines. CONCLUSION: A referral network could be established with local and regional reach, which relies on a multidisciplinary approach for the management of EoE and associated type 2 co-morbidities. More studies are needed to better depict the clinical picture of patients with EGIDs in general and EoE, in particular, in terms of diagnosis, pharmacological treatment, non-medical interventions, disease course, and patient-reported outcomes.

Management of pharyngocutaneous fistula following laryngectomy with autologous fat graft: A case report and literature review.

INTRODUCTION AND SIGNIFICANCE: Pharyngocutaneous fistula (PCF) is a serious complication following total laryngectomy, significantly impacting patients' quality of life and treatment costs. Management is complex, with potential for recurrence after surgical intervention. CASE PRESENTATION: We present a case of PCF that developed following laryngectomy. Initial conservative treatment failed to resolve the fistula. A pectoralis major myofascial flap was then attempted, but the fistula recurred. Finally, autologous fat grafting was performed with excellent results. CLINICAL DISCUSSION: Conservative management should be the first-line approach for PCF, as most cases respond favorably. However, if the fistula persists, alternative methods should be explored to achieve optimal outcomes. Our case highlights the importance of not persisting with a failed approach. Autologous fat grafting offers a promising alternative with demonstrably positive outcomes. CONCLUSION: Pharyngocutaneous fistula presents a significant challenge in post-laryngectomy care. Surgical repair can be complex with potential complications. Our case demonstrates the effectiveness of autologous fat grafting as a successful treatment modality.

Expert Recommendations on the Diagnosis of Eosinophilic Esophagitis in the United Arab Emirates.

Eosinophilic esophagitis (EoE) is a chronic, progressive, type 2 inflammatory esophageal disease presenting as dysphagia to solid food and non-obstructive food impaction. Knowledge gaps exist in its diagnosis and management. These expert recommendations focused on the diagnosis of EoE in the United Arab Emirates. An electronic search of PubMed and Embase databases was used to gather evidence from systematic reviews, randomized controlled trials, consensus papers, and expert opinions from the last five years on the diagnosis of EoE. The evidence was graded using the Oxford system. Literature search findings were shared with the expert panel. A 5-point scale (strongly agree, agree, neither agree nor disagree, disagree, and strongly disagree) was used, and a concordance rate of >75% among experts indicated agreement. Using a modified Delphi technique, 18 qualified experts provided 17 recommendations. Eleven statements achieved high agreement, four got moderate agreement, and two got low agreement. Challenges exist in diagnosing EoE, particularly in children. Esophageal biopsies were crucial in diagnosis, irrespective of visible mucosal changes. Further research on diagnostic tools like endoscopic mucosal impedance and biomarkers is needed. Diagnosis relies on esophageal biopsies and symptom-histology correlation; however, tools like EoE assessment questionnaires and endoscopic mucosal impedance could enhance the accuracy and efficiency of EoE diagnosis. The diagnosis of EoE is challenging since the symptoms seldom correlate with the histological findings. Currently, diagnosis is based on patient symptoms and endoscopic and histological findings. Further research into mucosal impedance tests and the role of biomarkers is needed to facilitate diagnosis.

Modeling Liver Organogenesis by Recreating Three-Dimensional Collective Cell Migration: A Role for TGFß Pathway.

Three-dimensional (3D) collective cell migration (CCM) is critical for improving liver cell therapies, eliciting mechanisms of liver disease, and modeling human liver development and organogenesis. Mechanisms of CCM differ in 2D vs. 3D systems, and existing models are limited to 2D or transwell-based systems, suggesting there is a need for improved 3D models of CCM. To recreate liver 3D CCM, we engineered in vitro 3D models based upon a morphogenetic transition that occurs during liver organogenesis, which occurs rapidly between E8.5 and E9.5 (mouse). During this morphogenetic transition, 3D CCM exhibits co-migration (multiple cell types), thick-strand interactions with surrounding septum transversum mesenchyme (STM), branching morphogenesis, and 3D interstitial migration. Here, we engineer several 3D in vitro culture systems, each of which mimics one of these processes in vitro. In mixed spheroids bearing both liver cells and uniquely MRC-5 (fetal lung) fibroblasts, we observed evidence of co-migration, and a significant increase in length and number of liver spheroid protrusions, which was highly sensitive to transforming growth factor beta 1 (TGFß1) stimulation. In MRC-5-conditioned medium (M-CM) experiments, we observed dose-dependent branching morphogenesis associated with an upregulation of Twist1, which was inhibited by a broad TGFß inhibitor. In models in which liver spheroids and MRC-5 spheroids were co-cultured, we observed complex strand morphogenesis, whereby thin, linear, 3D liver cell strands attach to the MRC-5 spheroid, anchor and thicken to form permanent and thick anchoring contacts between the two spheroids. In these spheroid co-culture models, we also observed spheroid fusion and strong evidence for interstitial migration. In conclusion, we present several novel cultivation systems that recreate distinct features of liver 3D CCM. These methodologies will greatly improve our molecular, cellular, and tissue-scale understanding of liver organogenesis, liver diseases like cancer, and liver cell therapy, and will also serve as a tool to bridge conventional 2D studies and preclinical in vivo studies.

Colopancreatic Fistula: An Uncommon Complication of Recurrent Acute Pancreatitis.

Colonic complications, including colopancreatic fistulas (CPFs), are uncommon after acute and chronic pancreatitis. However, they have been reported and are serious. CPFs are less likely to close spontaneously and are associated with a higher risk of complications. Therefore, more definitive treatment is required that includes surgical and endoscopic options. We present a case of a 62-year-old male patient with a history of heavy alcohol intake and recurrent acute pancreatitis who presented with a 6-month history of watery diarrhea and abdominal pain. His abdominal imaging showed a possible connection between the colon and the pancreas. A further multidisciplinary workup by the gastroenterology and surgery teams, including endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, and colonoscopy, resulted in a diagnosis of CPF. A distal pancreatectomy and left hemicolectomy were performed, and the diagnosis of CPF was confirmed intraoperatively. The patient showed improvement afterward.

Intrahepatic Cholangiocarcinoma Masquerading as Acute Fatty Liver of Pregnancy: A Case Report and Review of the Literature.

Cholangiocarcinoma (CCA) is an uncommon cancer and accounts only for 3% of all gastrointestinal malignancies. In this report, we present a case of an intrahepatic cholangiocarcinoma masquerading as acute fatty liver of pregnancy (AFLP). A 38-year-old female who is 36-week pregnant presented with a 1-week history of headache, nausea, vomiting, and right upper abdominal pain, along with hepatomegaly. Laboratory investigations were remarkable for mild leukocytosis, hyperbilirubinemia, proteinuria, and elevated transaminases and prothrombin time. Ultrasound of the liver revealed hepatomegaly, fatty infiltration, and a right hepatic lobe mass. Based on the overall picture, AFLP was suspected, and the patient underwent delivery by Cesarean section. However, bilirubin and liver enzyme levels gradually increased after delivery. MRI revealed a large dominant hepatic mass along with multiple satellite lesions in both lobes. Biopsy revealed the presence of intrahepatic CCA. CCA presenting during pregnancy is extremely rare with only 9 other cases reported in the literature. Therefore, the signs and symptoms can be easily confused with other more common disorders that occur during pregnancy.

Severe chronic diarrhoea secondary to primary lymph node gastrinoma.

The existence of primary lymph node (LN) gastrinoma is questionable and controversial. In fact, the presence of gastrinoma in such uncommon site raises the possibility of metastasis from another occult primary site. An extensive evaluation and careful follow-up is always warranted. A female aged 48 years presented with chronic abdominal pain and watery diarrhoea. Her serum gastrin and chromogranin were elevated, and an underlying gastrinoma was suspected. Further evaluation with an octreotide scan, an endoscopic ultrasound and a secretin stimulation test confirmed the diagnosis. Further evaluation for multiple endocrine neoplasia-1 syndrome was negative. She underwent a surgical enucleation near the head of the pancreas. No other lesions were found after careful exploration of the gastrinoma triangle. Histology showed a LN with a neuroendocrine tumour that tested positively with gastrin and chromogranin stains. Her symptoms resolved postoperatively, her serum gastrin normalised and a repeated octreotide scan was negative.

Gastric Metastases from Lung Adenocarcinoma Causing Gastrointestinal Bleeding.

Metastases to the stomach are rare. They are commonly asymptomatic, and the diagnosis is usually established during autopsy. We present a patient known to have stage IV lung adenocarcinoma who presented with melena and shock. Endoscopy revealed multiple gastric nodules, which were proved to be metastatic deposits from her lung cancer. The possibility of gastric metastases should be kept in mind in patients presenting with gastrointestinal bleeding. Endoscopy and biopsy remain the gold standard for diagnostic testing in such patients.

Perforated Bladder as a Cause of Abdominal Ascites in a Patient Presenting with Acute Onset Abdominal Pain.

Bladder perforation, especially when atraumatic, is a rare cause of ascites and is often difficult to differentiate from other causes of abdominal pain and ascites in the acute setting.  A 43-year-old Caucasian woman with a history of multiple sclerosis presented with acute abdominal pain. A computed tomography (CT) scan of her abdomen and pelvis without contrast revealed ascites, acute kidney injury (AKI) was noted on laboratory workup, and very little urine was drained by Foley catheter. Over the next several days, the patient's clinical condition deteriorated with no definitive diagnosis. A repeat CT of her abdomen and pelvis without contrast showed worsening ascites. She underwent paracentesis, which revealed a markedly elevated ascitic fluid creatinine consistent with bladder rupture. She then underwent an urgent cystogram to confirm the diagnosis, and the urologic consultant recommended conservative management with a Foley catheter to allow for bladder healing. Conservative treatment failed however, and she underwent a surgical repair with drain placement which was followed by an improvement in her clinical condition. This case illustrates a unique presentation of a young woman with multiple sclerosis whose bladder perforation presented as abdominal pain and ascites. The multidisciplinary approach required here highlights the difficulty in reaching this diagnosis which is often undermined in patients who lack a history of traumatic injury. Such delays led to a complicated hospital course for our patient. Maintaining a broad differential for abdominal pain and ascites is essential.

Hepatic hydrothorax: clinical review and update on consensus guidelines.

Hepatic Hydrothorax (HH) is defined as a pleural effusion greater than 500 ml in association with cirrhosis and portal hypertension. It is an uncommon complication of cirrhosis, most frequently seen in association with decompensated liver disease. The development of HH remains incompletely understood and involves a complex pathophysiological process with the most acceptable explanation being the passage of the ascetic fluid through small diaphragmatic defects. Given the limited capacity of the pleural space, even the modest pleural effusion can result in significant respiratory symptoms. The diagnosis of HH should be suspected in any patient with established cirrhosis and portal hypertension presenting with unilateral pleural effusion especially on the right side. Diagnostic thoracentesis should be performed in all patients with suspected HH to confirm the diagnosis and rule out infection and alternative diagnoses. Spontaneous bacterial empyema and spontaneous bacterial pleuritis can complicate HH and increase morbidity and mortality. HH can be difficult to treat and in our review below we will list the therapeutic modalities awaiting the evaluation for the only definitive therapy, which is liver transplantation.

Massive upper gastrointestinal hemorrhage due to invasive hepatocellular carcinoma and hepato-gastric fistula.

A 36-year-old male Asian immigrant with a history of hepatitis B and hepatitis C related unresectable hepatocellular carcinoma in the left lobe of the liver presented with hematemesis and severe anemia. He was diagnosed with a liver mass that was resected 8 years ago described as a benign tumor in his home country. He had received trans-arterial chemoembolization (TACE) four months ago after subsequent diagnosis of unresectable hepatoma, and currently was receiving chemotherapy with Sorafenib. After resuscitation, a contrast enhanced computerized tomography was performed which showed fistulization of hepatocellular carcinoma into adjacent stomach. This finding was confirmed during endoscopy with direct visualization of the fistulous opening. Hepatocellular carcinoma (HCC) invading the gastrointestinal (GI) tract is rare. We present a case and literature review of HCC with local invasion of the stomach causing massive upper GI bleeding after receiving TACE.

Role of endoscopic ultrasound and endoscopic retrograde cholangiopancreatography in isolated pancreatic metastasis from lung cancer.

A case is reported of a 50-year-old woman with a history of small-cell lung cancer admitted with pancreatic head lesions, discovered during investigation for obstructive jaundice. Endoscopic ultrasound assisted fine needle aspiration of the pancreatic mass was consistent with small cell carcinoma, presenting as an isolated metastasis from the previously diagnosed lung cancer. Endoscopic retrograde cholangiopancreatography (ERCP) showed extrinsic compression and a bile duct stricture, requiring sphincterotomy and stent insertion. This case highlights that acute pancreatitis and biliary obstruction can occur as a manifestation of small cell lung cancer metastasizing to the pancreas. EUS is a safe, low risk and rapid diagnostic tool in such cases, and ERCP with stenting offers a safe and effective treatment option.