ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI An Unusual Cause of Pneumoperitoneum Jesús Cañete-Gómez, Juan J. Segura-Sampedro, and Julio Reguera-Rosal Department of General Surgery, University Hospital Virgen del Rocío, Sevilla, Spain
Question: A 59-yearold man was admitted the hospital with fever (39 C), nausea, and vomiting. He reported mild, diffuse abdominal pain for a week. He has received last chemotherapy owing to Philadelphia chromosome–positive acute lymphoblastic leukemia (Phþ ALL) 4 days before (vincristine and dexamethasone), and now presents general health deterioration. Physical examination revealed a respiratory rate of 23 breaths per minute, a heart rate of 82 bpm, and a temperature of 38.6 C with a blood pressure of 144/90 mmHg. Abdominal examination revealed decreased bowel sounds and severe diffuse abdominal pain, with rigidity and rebound tenderness. There was no evidence of hepatosplenomegaly or masslike lesions. Laboratory studies included a white blood cell count of 920/mm3; neutrophil count of 85%; hemoglobin 8.9 g/dL; and platelet count, 89000/mm3. Computed tomography showed pneumoperitoneum at the anterior abdominal cavity, surrounding the stomach and spleen, with pneumatosis of the gastric wall and perforation. Widespread hypodense splenic parenchyma contrast captures not likely owing to thrombosis of the splenic artery was suspected; we were unable to rule out gastric artery thrombosis. There is a difference of hepatic perfusion between left and right hepatic lobes (Figure A, B). What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The author discloses no conflicts. © 2014 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.05.041
Gastroenterology 2014;147:e3–e5
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 2: Splenic and Gastric Mucormycosis Resulting in Gastric Perforation Owing to Left Gastric and Splenic Artery Thrombosis
Laparotomy revealed gastric necrosis with multiple perforations in the gastric fundus with pneumatosis of the gastric wall (Figure C) and splenic artery thrombosis with massive splenic infarction (Figure D). we performed total gastrectomy (Figure E) with splenectomy. Microscopic examination revealed a transverse section of the arterial vessel of the gastric wall with the lumen obliterated and extensive tissue destruction and disintegration of the layer with infiltration of numerous broad-based, nonseptate, right angular branched fungal hyphae, morphologically consistent with mucormycosis (Figure F; stain, hematoxylin and eosin; original magnification, 400). Mucormycosis is a life-threatening infection caused by fungi of the subphylum Mucoromycotina. Traditional risk factors for the development of invasive mucormycosis include diabetes, defects in host phagocytes, corticosteroid use, organ or stem cell transplantation, and increased levels of available serum iron. The factors predisposing to disseminated disease are not well understood, but include cancer chemotherapy, immunosuppressive therapy, corticosteroid therapy, renal failure, and a prolonged postoperative course.1 Mucormycosis can affect any organ, but the most common presentations involve either the nasal sinuses, orbit, and brain (rhino-orbital-cerebral). The stomach is the most common site of gastrointestinal mucormycosis, followed by the colon and ileum. Symptoms of gastrointestinal mucormycosis vary and depend on the affected site. Nonspecific abdominal pain and distention associated with nausea and vomiting are the most common symptoms. Fever and hematochezia may also occur. The diagnosis may be made by biopsy of the suspected area during surgery or endoscopy.2 Diagnosis can only be established once the fungi are identified. Only then, appropriate therapy can be initiated. Thus, this case highlights the need to maintain a high index of suspicion for invasive fungal infections, including mucormycosis, in patients who are being treated with cancer chemotherapy and who present with disease that crosses tissue planes. Primary antifungal therapy for mucormycosis should be based on a polyene antifungal agent, because this drug class is by far the most active against the relevant pathogens. Most experts prefer to use lipid formulations of amphotericin B, which can be administered at higher doses and with less toxicity than amphotericin B deoxycholate. Antifungal therapy alone is typically inadequate to control mucormycosis, and surgery to debulk the fungal infection and/or resect all infected tissue is often required to effect cure. Aside from the resistance of some fungal strains to amphotericin B, several hallmark features of mucormycosis—including angioinvasion, thrombosis, and tissue necrosis—result in poor penetration of anti-infective agents to the site of infection. Furthermore, in multiple case series, patients who did not undergo surgical debridement of mucormycosis had a far higher mortality rate than patients who underwent surgery.3 We treated our patient successfully with gastrectomy and splenectomy and the aggressive use of intravenous amphotericin B when gastrointestinal mucormycosis was confirmed. e4
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Finally, immunosuppressive medications, particularly corticosteroids, should be dose reduced or stopped if at all possible.
References 1. 2. 3.
Spellberg B, Edwards J Jr, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556–569. Gutierrez O, Cantalapiedra A, Tabuyo MI, et al. Emphysematous gastritis and severe aplastic anemia. Hematol J 2003; 4:82–84. Spellberg B, Walsh TJ, Kontoyiannis DP, et al. Recent advances in the management of mucormycosis: from bench to bedside. Clin Infect Dis 2009;48:1743–1751.
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Question: A 59-yearold man was admitted the hospital with fever (39 C), nausea, and vomiting. He reported mild, diffuse abdominal pain for a week. He has received last chemotherapy owing to Philadelphia chromosome–positive acute lymphoblastic leukemia (Phþ ALL) 4 days before (vincristine and dexamethasone), and now presents general health deterioration. Physical examination revealed a respiratory rate of 23 breaths per minute, a heart rate of 82 bpm, and a temperature of 38.6 C with a blood pressure of 144/90 mmHg. Abdominal examination revealed decreased bowel sounds and severe diffuse abdominal pain, with rigidity and rebound tenderness. There was no evidence of hepatosplenomegaly or masslike lesions. Laboratory studies included a white blood cell count of 920/mm3; neutrophil count of 85%; hemoglobin 8.9 g/dL; and platelet count, 89000/mm3. Computed tomography showed pneumoperitoneum at the anterior abdominal cavity, surrounding the stomach and spleen, with pneumatosis of the gastric wall and perforation. Widespread hypodense splenic parenchyma contrast captures not likely owing to thrombosis of the splenic artery was suspected; we were unable to rule out gastric artery thrombosis. There is a difference of hepatic perfusion between left and right hepatic lobes (Figure A, B). What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The author discloses no conflicts. © 2014 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.05.041
Gastroenterology 2014;147:e3–e5
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 2: Splenic and Gastric Mucormycosis Resulting in Gastric Perforation Owing to Left Gastric and Splenic Artery Thrombosis
Laparotomy revealed gastric necrosis with multiple perforations in the gastric fundus with pneumatosis of the gastric wall (Figure C) and splenic artery thrombosis with massive splenic infarction (Figure D). we performed total gastrectomy (Figure E) with splenectomy. Microscopic examination revealed a transverse section of the arterial vessel of the gastric wall with the lumen obliterated and extensive tissue destruction and disintegration of the layer with infiltration of numerous broad-based, nonseptate, right angular branched fungal hyphae, morphologically consistent with mucormycosis (Figure F; stain, hematoxylin and eosin; original magnification, 400). Mucormycosis is a life-threatening infection caused by fungi of the subphylum Mucoromycotina. Traditional risk factors for the development of invasive mucormycosis include diabetes, defects in host phagocytes, corticosteroid use, organ or stem cell transplantation, and increased levels of available serum iron. The factors predisposing to disseminated disease are not well understood, but include cancer chemotherapy, immunosuppressive therapy, corticosteroid therapy, renal failure, and a prolonged postoperative course.1 Mucormycosis can affect any organ, but the most common presentations involve either the nasal sinuses, orbit, and brain (rhino-orbital-cerebral). The stomach is the most common site of gastrointestinal mucormycosis, followed by the colon and ileum. Symptoms of gastrointestinal mucormycosis vary and depend on the affected site. Nonspecific abdominal pain and distention associated with nausea and vomiting are the most common symptoms. Fever and hematochezia may also occur. The diagnosis may be made by biopsy of the suspected area during surgery or endoscopy.2 Diagnosis can only be established once the fungi are identified. Only then, appropriate therapy can be initiated. Thus, this case highlights the need to maintain a high index of suspicion for invasive fungal infections, including mucormycosis, in patients who are being treated with cancer chemotherapy and who present with disease that crosses tissue planes. Primary antifungal therapy for mucormycosis should be based on a polyene antifungal agent, because this drug class is by far the most active against the relevant pathogens. Most experts prefer to use lipid formulations of amphotericin B, which can be administered at higher doses and with less toxicity than amphotericin B deoxycholate. Antifungal therapy alone is typically inadequate to control mucormycosis, and surgery to debulk the fungal infection and/or resect all infected tissue is often required to effect cure. Aside from the resistance of some fungal strains to amphotericin B, several hallmark features of mucormycosis—including angioinvasion, thrombosis, and tissue necrosis—result in poor penetration of anti-infective agents to the site of infection. Furthermore, in multiple case series, patients who did not undergo surgical debridement of mucormycosis had a far higher mortality rate than patients who underwent surgery.3 We treated our patient successfully with gastrectomy and splenectomy and the aggressive use of intravenous amphotericin B when gastrointestinal mucormycosis was confirmed. e4
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Finally, immunosuppressive medications, particularly corticosteroids, should be dose reduced or stopped if at all possible.
References 1. 2. 3.
Spellberg B, Edwards J Jr, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556–569. Gutierrez O, Cantalapiedra A, Tabuyo MI, et al. Emphysematous gastritis and severe aplastic anemia. Hematol J 2003; 4:82–84. Spellberg B, Walsh TJ, Kontoyiannis DP, et al. Recent advances in the management of mucormycosis: from bench to bedside. Clin Infect Dis 2009;48:1743–1751.
e5
