Citations

Abstract Introduction Hepatopulmonary syndrome (HPS) is a rare condition where liver disease causes intrapulmonary vascular dilation, subsequently leading to intrapulmonary shunting and thus hypoxemia. Although diagnosis can be challenging, classical HPS features include dyspnea, platypnea, and orthodeoxia, which aid diagnosis. Timely diagnosis of HPS is crucial to provide adequate treatment, which ultimately involves supportive care while awaiting liver transplantation. This is a case of a patient who has a known history of alcoholic cirrhosis who presented to the pulmonology clinic with the chief complaint of progressive dyspnea. Investigations established the diagnosis of HPS, and the patient was referred for liver transplantation. Case A 65-year-old female with a past medical history of significant alcohol use, alcoholic cirrhosis, portal hypertension, recurrent upper gastrointestinal bleeding and a 40-pack-year history. She presented to the pulmonology clinic due to progressive dyspnea on exertion, which she had been experiencing for three years. Furthermore, home pulse oximetry readings were in the 80s, and she denied any cough, orthopnea, or peripheral edema. The patient had a previous echocardiogram with a bubble study, which identified concern for left-to-right shunting. Initially, it was perceived to be intracardiac; however, right heart catheterization was completed, and there was no evidence of an intracardiac shunt. Additionally, pulmonary pressures were normal, favouring intrapulmonary shunting. Physical examination was significant for orthodeoxia with oxygen saturation of 89 percent while upright, and 97 percent while supine, and the patient reported feeling less breathless when lying down. Pulmonary function testing was significant for a substantial reduction in diffusion capacity. Given the constellation of symptoms, including platypnea, orthodeoxia, and evidence of intrapulmonary shunting, with a background history of alcoholic cirrhosis, the patient was diagnosed with HPS and was referred for a liver transplant evaluation. Discussion This case identifies the importance of evaluation for HPS in patients with underlying liver disease and persistent shortness of breath. The constellation of liver disease, hypoxemia, and concern for intrapulmonary vascular dilation were telling for this patient. Currently, there is no medical intervention to cure HPS, and treatment revolves around supportive care and supplemental oxygenation while awaiting liver transplantation. After liver transplantation, patients will often experience improved oxygenation due to the correction of the intrapulmonary vasodilation, which in turn corrects the ventilation-perfusion mismatch. Although early postoperative mortality is slightly higher, long-term survival for HPS patients post-transplant is like that of non—HPS transplant recipients. Therefore, early diagnosis and referral for transplant evaluation are critical to optimize patient outcomes. This abstract is funded by: NONE

SUMMARY Given their malignant potential, resection of esophageal granular cell tumors (GCTs) is often undertaken, yet the optimal technique is unknown. We present a large series of dedicated endoscopic resection using band ligation (EMR-B) of esophageal GCTs. Patients diagnosed with esophageal GCTs between 2002 and 2019 were identified using a prospectively collected pathology database. Endoscopic reports were reviewed, and patients who underwent dedicated EMR-B of esophageal GCTs were included. Medical records were queried for demographics, findings, adverse events, and follow-up. We identified 21 patients who underwent dedicated EMR-B for previously identified esophageal GCT. Median age was 39 years; 16 (76%) were female. Eight (38%) had preceding signs or symptoms, potentially attributable to the GCT. Upon endoscopic evaluation, 12 (57%) were found in the distal esophagus. Endoscopic ultrasound was used in 15 cases (71%). Median lesion size was 7 mm, interquartile range 4 mm—8 mm. The largest lesion was 12 mm. A total of 20 (95%) had en bloc resection confirmed with pathologic examination. The only patient with tumor extending to the resection margin underwent surveillance endoscopy that showed no residual tumor. No patients experienced bleeding, perforation, or stricturing in our series. No patients have had known recurrence of their esophageal GCT. EMR-B of esophageal GCT achieves complete histopathologic resection with minimal adverse events. EMR-B is safe and effective and seems prudent compared with observation for what could be an aggressive and malignant tumor. EMR-B should be considered first-line therapy when resecting esophageal GCT up to 12 mm in diameter.