Portal hypertension caused by cirrhosis is the most common etiology of esophageal varices. However, abnormalities of the splenoportal axis in the absence of liver disease may also cause portal hypertension resulting in varices. We report a rare case of esophageal variceal bleed in a noncirrhotic patient with isolated splenomegaly secondary to chronic granulocyte colony stimulating factor (G-CSF) therapy. The patient is a 26-year-old male with Cohen syndrome who required long-term G-CSF treatment for chronic neutropenia. He presented with large volume hematemesis and pancytopenia in the setting of known splenomegaly with no evidence of cirrhosis. An urgent EGD revealed active variceal bleeding and portal hypertensive gastropathy. The patient was appropriately resuscitated and underwent a successful transjugular intrahepatic portosystemic shunt and CT-guided coil placement for the bleeding varices. We are the first to report variceal bleed as a complication of long-term G-CSF use, a life-threatening consequence that requires urgent intervention.

Introduction: Point of care ultrasound is a burgeoning tool in clinical medicine and its utility has been demonstrated in a variety of contexts. It may be especially useful in rural areas where access to other imaging equipment (such as CT) is limited. However, there exists debate about the utility of teaching ultrasound theory and technique to medical undergraduates, particularly those in their first two years of study. This study evaluated the efficacy of teaching undergraduate-tailored ultrasound training sessions to first and second-year medical students at the Northern Ontario School of Medicine (NOSM), a rural-focused medical institution. Methods: Sixty students participated in tailored ultrasound teaching sessions that involved both lecture and hands-on components. Participating students were assessed following each session, as well as at study completion, in terms of ultrasound knowledge, anatomy, pathology, orientation, and interpretation of computerized tomography (CT) scans (transferability). Participants’ performance was measured against a control group of their peers. Program evaluation was completed using Likert-type scales to determine participant comfort with ultrasound before and after the training, and areas of strength and improvement. Results: Participating students showed statistically significant improvement in ultrasound interpretation and anatomical orientation with trends toward improved anatomy and pathology knowledge, and ability to interpret computerized tomography (CT) scans compared to controls. Students participating in the course expressed improved comfort with ultrasound techniques and desire for future integration of ultrasound into their training, but noted that increasing frequency of training sessions might have improved retention and confidence. Conclusion: Results suggest that using an undergraduate-focused and system-specific ultrasound training course yields retention in ultrasound interpretation ability and objective improvement in relational anatomy knowledge. Trends toward improvement in general anatomy, pathology and CT interpretation suggest areas of future study.

Background Portal hypertension caused by cirrhosis is the most common etiology of esophageal varices. However, abnormalities of the spleno-portal axis in the absence of liver disease may also cause portal hypertension resulting in varices. We report a rare case of esophageal variceal bleed in a non-cirrhotic patient with isolated splenomegaly secondary to chronic G-CSF therapy. Aims This report outlines the case of a patient with Cohen Syndrome (CS) who presented with an upper gastrointestinal (GI) bleed in the setting of previously documented splenomegaly and portal hypertension. We expand on the clinical investigations, diagnosis, treatment plan and hospital course of this patient. Methods Case report, review of literature. Results A 26-year old male with previously diagnosed CS presented with large volume hematemesis and pancytopenia. CS is a rare autosomal recessive disorder. In our patient this manifested with congenital neutropenia, microcephaly, retinal dystrophy and global developmental delay. He required long term G-CSF therapy to manage chronic neutropenia and subsequently developed splenomegaly, a known side effect. The most recent MRI identified stable splenomegaly with a craniocaudal length of 23 cm, normal liver size and no radiographic evidence of cirrhosis. The imaging was also significant for gastroesophageal and splenorenal varices but no ascites or recanalization of the umbilical vein. A recent liver biopsy had shown mild pericellular fibrosis with no active liver disease or cirrhosis. In the past, the patient had declined EGD, therapeutic splenectomy or assessment of hepatic venous pressure gradient through invasive venography. His liver enzymes, bilirubin and albumin had always been within normal limits. The patient had no history of GI bleeding. Previous investigations for hematologic malignancies or myelodysplastic syndrome had been negative. Upon admission, an urgent EGD revealed active variceal bleeding in the esophagus and portal gastropathy. Given the extent of his congenital orofacial abnormalities a variceal band ligator could not be passed for appropriate intervention. The patient was transferred to the Intensive Care Unit and managed with intravenous proton pump inhibitor, octreotide, as well as transfusions of packed red blood cells, platelets and fresh frozen plasma. Within the next 48 hours, the patient underwent successful transjugular intrahepatic portosystemic shunt and CT-guided coil placements for the bleeding varices. Conclusions This is a rare case of variceal bleed in a non-cirrhotic patient with portal hypertension from iatrogenic splenomegaly. While there are previous reports of spontaneous splenic rupture secondary to G-CSF therapy we are the first to report variceal bleed as a complication. This is a life-threatening consequence that requires urgent intervention and intensive care. Funding Agencies None