Hematemesis, especially when severe enough to cause hemodynamic instability, typically occurs in patients with hepatobiliary pathology associated with portal hypertension and varices. However, hematemesis can rarely be the presenting symptom of a wandering spleen (WS), via the development of varices from sinistral portal hypertension. This case report describes a 14-year-old previously healthy male with acute hematemesis, who was found to have a WS and variceal hemorrhage that required emergent resuscitation and splenectomy.Hematemesis because of variceal bleeding is a common diagnosis in adults with cirrhosis or other causes of generalized (right- and left-sided) portal hypertension. In children and adolescents, hematemesis is most frequently because of Mallory-Weiss tears or peptic ulcer disease. Severe pediatric upper gastrointestinal bleeds are most often because of varices.1 At all ages, varices usually result from generalized portal hypertension, but they can also result from isolated sinistral (also called left-sided or segmental) portal hypertension (SPH). One rare etiology of SPH is torsion of a WS. This case of a 14-year-old male with hemodynamically significant hematemesis as his presenting symptom represents an exceptionally rare manifestation of WS.A 14-year-old previously healthy male presented to a community hospital emergency department for acute hematemesis. After 2 days of treating upper respiratory symptoms with expectorants and albuterol, he awoke that morning feeling unwell. He walked to his bathroom, fell without losing consciousness, and began having nonbloody emesis that progressed to repeated bright red hematemesis. When asked, he remembered 1 black stool the previous day. He denied previous episodes of abdominal pain, blood in vomit or stool, sick contacts, travel, new foods, alcohol consumption, and caustic ingestions. He reported very limited NSAID use in the previous week for overuse shoulder pain from baseball.On presentation, he had normal vital signs, mild epigastric tenderness without peritoneal signs, and heme-positive dark stool. His initial hemoglobin (Hb) was 8.8 g/dL, MCV 89 fL, platelets 148 K/uL, INR 1.3, PTT 26.6 seconds, albumin 3.0 g/dL, and otherwise normal complete metabolic panel (including liver function tests) and lipase. He had 100 mL of clot-containing emesis and received a normal saline bolus, 1 unit of packed red blood cells (PRBC), and intravenous pantoprazole and octreotide.On transfer to a tertiary care pediatric hospital, he maintained normal vital signs, reported mild right upper quadrant and epigastric tenderness, and had repeat Hb 8.4 g/dL. He continued to receive pantoprazole and octreotide infusions and was closely monitored while kept NPO. He remained asymptomatic and hemodynamically stable for nearly 12 hours on hospital day (HD) #1. Given his age, previous health, and clinical stability, the initial clinical impression was that he had a Mallory-Weiss tear, NSAID-induced gastritis, or peptic ulcer disease, so octreotide was stopped.However, later that evening, he had sudden-onset hematemesis, pallor, diaphoresis, altered mentation, hypotension (systolic pressures 70s), and tachycardia (pulse 110s). Hemoglobin had fallen to 7.3 g/dL. He was emergently given fluid boluses and transferred to the ICU for hemorrhagic shock, with activation of the massive transfusion protocol for 9 units of PRBC, 8 units of fresh-frozen plasma, and 5 units of platelets. His Hb nadir was 6.6 g/dL. Abdominal Doppler ultrasound revealed splenomegaly (maximum dimension 12.7 cm) and dilation of the portal vein and splenic hilar veins, which is suspicious for portal hypertension. He was intubated for airway protection and underwent esophagogastroduodenoscopy on HD#2, with epinephrine injection and clipping of a solitary raised erythematous lesion in the gastric cardia, thought to be an arteriovenous malformation or varix given the ultrasound findings (Fig 1). General surgery specialists, who were consulted intraprocedurally, raised concern for possible WS given the ultrasound findings and possible varix, and recommended computerized tomography to better characterize the gastric lesion and its potential causes. Octreotide infusion was restarted. Computerized tomography of the abdomen and pelvis revealed hepatomegaly (maximum dimension 22 cm), dilatation of the portal vein (15 mm diameter), increasing splenomegaly (18 cm), and gastric submucosal, perigastric, and perisplenic varices. Magnetic resonance angiography and venography and cholangiopancreatography on HD#3 ruled out thrombosis but revealed interval displacement and posterior rotation of the spleen in the left hemipelvis, which is consistent with WS (Fig 2).After discussing the case with interventional radiology specialists for consideration of partial splenic artery embolization to reduce splenic blood flow and splenomegaly, surgical specialists instead recommended a splenopexy versus splenectomy. However, before this could occur, the patient had recurrent hemodynamically significant hematemesis on HD#5, retriggering the massive transfusion protocol for 3 units of PRBC and 2 units of platelets and prompting emergent exploratory laparotomy, splenectomy, and gastrotomy with suture ligation of bleeding fundal gastric varices. He was extubated on HD#6, given postoperative prophylactic antibiotic therapy and initiated on enteral feedings on HD#10 after a fluoroscopic upper gastrointestinal series showed no leak at the gastrotomy site. He was discharged from the hospital on twice daily omeprazole on HD#14 and received guidance regarding postsplenectomy vaccinations. Final pathology revealed splenomegaly with extensive congestion of red pulp, neutrophilic inflammation, and lymph nodes with medullary hemorrhage. Eighteen months later, he continued to recover well without complications.WS is a very rare phenomenon, with an estimated incidence of <0.5%.2,3 WS can be congenital or acquired. Congenital WS results from failure of the dorsal mesogastrium to form and fuse appropriately during embryogenesis, leading to a long splenic vascular pedicle and failure of multiple suspensory ligaments to form and attach the spleen in the left upper quadrant.4,5 Acquired WS can result from risk factors like splenomegaly, abdominal wall laxity, and hormonal changes of pregnancy leading to ligament laxity.6 Other suspected risk factors include trauma, history of diaphragmatic hernia repair, history of malaria, and benign hematologic diseases.7Because of inadequate ligamentous attachments, a WS can move throughout the patient’s abdomen and pelvis and can twist on its vascular pedicle. When such torsion occurs, complications can include splenic infarction and hemorrhage, splenic vein thrombosis, gastric volvulus with potential gastric rupture, acute pancreatitis, small bowel obstruction, spontaneous hemoperitoneum, splenorenal collaterals, and varices.7–13 Varices result from increased pressure in the splenic venous system, leading to isolated SPH and high venous pressures in collateral drainage systems, namely the left portal venous system. This high pressure produces gastric varices, typically without esophageal varices because of their distinct drainage systems.14 This contrasts with varices caused by generalized portal hypertension, which begins as right-sided venous hypertension (from hepatobiliary disease like cirrhosis from any cause, portal vein thrombosis, or congestive hepatopathy from constrictive pericarditis or restrictive cardiomyopathies) and inevitably leads to left-sided venous hypertension. Isolated SPH in adults is usually caused by pancreatitis, pancreatic (pseudo)cysts, or pancreatic tumors, leading to splenic vein occlusion or thrombosis.14We found scant literature on causes of SPH specific to children or adolescents, but 1 report identified reactive lymphadenopathy in a 10-year-old girl as the etiology for splenic vein compression and SPH causing gastric variceal bleeding with hematemesis.15We conducted an extensive literature review on PubMed using the key words “wandering spleen” and “varices.” We identified additional articles from the reference lists of the initial articles. This yielded 27 articles in English, French, Japanese, and Spanish. Only 7 definitive cases of WS were found in which the patient presented with hematemesis.Most of the hundreds of reported cases of WS occurred without varices in patients who presented with abdominal pain and/or mass and were then incidentally discovered to have WS on imaging. The majority of reports involved children or women in their third to fifth decade. The patients typically presented with intermittent abdominal pain from transient self-resolving splenic torsion (often treated with splenopexy), or with acute abdomen from unresolved torsion leading to splenic infarction or other intrabdominal complications (requiring splenectomy).6,16–19One of the first reports of varices as a WS complication was published in 1980 about a 14-year-old girl who presented with epigastric pain and early satiety and was found to have gastric torsion and slow gastric variceal bleeding with melena because of a WS.9,20 A few other case reports mention gastric varices that led to melena without hematemesis,11,20,21 including one in which a pregnant patient presented with acute pancreatitis and then after cesarean delivery developed hemodynamically significant lower gastrointestinal bleeding from a gastric ulcer amid gastric varices, requiring splenectomy.11Of those cases that report varices as a complication, fewer than 10 were found reporting variceal bleeding significant enough to produce hematemesis as the presenting symptom.8–10,17,22–27 Those patients generally had several commonalities: women age 20 to 50 years (many of whom had previously given birth to children), gastric fundal varices without esophageal varices, and overall clinical stability. A single previous case of a pediatric patient presenting with hematemesis was found: a 16-year-old girl who was too unstable for endoscopy but was found to have varices in the gastric wall and spleen during emergency laparotomy.25 Our case may be the first report in a male, and the second pediatric (<18 years) report, of WS presenting as hematemesis because of variceal bleeding from SPH. Our report differs from the previous reports by its involvement of a previously healthy male adolescent, variceal bleeding in the gastric cardia in addition to the typical fundus, and presentation with hematemesis significant enough to cause hemorrhagic shock.Several articles discuss the indications and outcomes of splenopexy versus splenectomy as treatment of WS. In the absence of clinical instability and splenic complications, splenopexy is generally preferred to avoid the risk of postsplenectomy sepsis. Although many reports confirm that splenectomy can lead to eventual resolution of varices, it is unclear whether splenopexy can.28–30 In our case, splenectomy was performed because of the patient’s clinical instability. We anticipate that any future endoscopic evaluations will demonstrate resolution of his varices.This case of a 14-year-old male with hematemesis illustrates that variceal bleeding is an etiology of hematemesis that cannot be discounted regardless of age, or the absence of hepatobiliary comorbidities commonly associated with portal hypertension, because varices can result from occult SPH. WS is a very rare entity that typically presents in children or adult women with abdominal pain and/or mass, but it can produce varices that can cause significant hematemesis and hemorrhagic shock. Identification of WS should prompt examination for associated varices so that appropriate interventions can be made to reduce the risk of potentially fatal hemorrhage.
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Mar 18, 2024
Rehana Rehman + 3