emphysematous gastritis , which was first described by fraenkel in 1889 ( 1 ) , is a rare disease characterized by the presence of air within the wall of the stomach and diffuse gastric wall inflammation by gas - forming bacteria , which may be also life - threatening due to systemic toxicity .
predisposing factors include the ingestion of corrosives , alcohol abuse , recent abdominal surgery , diabetes , and immunocompromised conditions ( 1 - 3 ) .
since 1889 , only 51 cases have been reported worldwide ( 1 - 8 ) .
mucormycosis is a rare fungal infection that usually involves the nasopharynx ( 9 ) . among the several forms ,
gastrointestinal mucormycosis is very rare , and the manifestations range from colonization of peptic ulcers to infiltrative disease with vascular invasion and dissemination ( 9 ) . commonly associated conditions include diabetes mellitus , lymphoma , leukemia , renal disease , septicemia , malnutrition , and long - term treatment with steroids and antibiotics ( 10 ) . among the reported cases of emphysematous gastritis , fungal organisms such as candida species have been isolated rarely ( 4 , 11 - 15 ) .
however , emphysematous gastritis associated with mucormycotic infection is extremely rare , and only one case has been reported in the english literature ( 15 ) .
here we report a case of emphysematous gastritis , diagnosed by computed tomography ( ct ) and confirmed by histopathologic findings associated with invasive gastric mucormycosis that showed angioinvasion , necrosis , ultimately disseminated to the colon and liver , and was fatal despite medical and surgical treatment . to our knowledge , this is the first report of a patient with emphysematous gastritis associated with invasive gastric mucormycosis in korea .
a 43-yr old man was admitted to the emergency room because of diffuse abdominal pain , indigestion , and poor oral intake for 4 days . he had had a 1-yr history of hypertension ,
there was also a history of excessive ingestion of alcohol and heavy smoking for 20 yr , but there was no history of peptic ulcer disease .
his blood pressure was 160/100 mmhg , with a body temperature of 36.2 , pulse rate of 98/min , and respiration rate of 26/min .
abdominal examination revealed decreased bowel sounds and severe tenderness in the epigastrium , with rigidity and rebound tenderness .
hematological and biochemical examinations on admission showed hemoglobin 13.9 g / dl ( normal : 13 - 18 ) , white blood cell 11.7710/l ( 3.6 - 11 ) , platelet 21010/l ( 150 - 450 ) , blood sugar 816 mg / dl ( 70 - 110 ) , aspartate aminotransferase 148 iu / l ( 5 - 38 ) , alanine aminotransferase 76
iu / l ( 4 - 43 ) , lactate dehydrogenase 1,320 iu / l ( 180 - 460 ) , amylase 6 u / l ( 45 - 108 ) , blood urea nitrogen 37.7 mg / dl ( 8 - 23 ) , and creatinine 1.6 mg / dl ( 0.5 - 1.2 ) , and blood acetone - positive .
chest radiography revealed collapse and consolidation in the basal part of the left lower lobe , suggesting pneumonia .
abdominal ct scan revealed gastric wall thickening with a collection of dirty air bubbles and a small amount of pneumoperitoneum ( fig .
operation findings showed nearly total necrosis of stomach wall with dark green colored pus collection around the stomach .
grossly , the stomach had a large area of necrosis with black discoloration of the mucosa ( fig .
the remaining viable stomach wall showed large numbers of neutrophilis in the edematous submucosa and muscle layer .
there were several gas - filled bubbles in the submucosa and muscle layer associated with numerous scattered or colonies of gram - negative bacilli , consistent with emphysematous gastritis ( fig .
1 ) . at the same time , there were numerous broad - based , non - septate , right angular branched fungal hyphae , morphologically consistent with mucormycosis , detected on hematoxyline and eosin stain as well as silver stains throughout both necrotic and viable stomach wall ( fig .
culture of pus in the peritoneum revealed klebsiella pneumonia , staphylococcus aureus , and pseudomonas aeruginosa .
amphotericin b infusion was added after operation . on the fifth hospital day , there was pus discharge from the wound site .
however , the patient remained to have non - toxic appearance with stable vital signs . on the seventh hospital day
, he complained of diarrhea and vague abdominal discomfort . over the following 2 days ,
follow - up ct revealed an approximately 14.012.012.0 cm - sized , irregularly shaped air - containing fluid collection at the gastrectomy site , suggesting air - forming postoperative abscess and anastomotic site leakage .
reoperation was performed , and the operative findings showed multiple perforations with necrosis in the transverse colon , necrotic change in the liver , hematoma with fluid collection in the peritoneal cavity , and leakage in the anastomotic site .
rouxen - y esophagojejunostomy with cecocolostomy and segmental resection of the large intestine were performed . the submitted pathological specimen consisted of segments of colon , small intestine , and a portion of liver .
the gross examination of the segment of colon showed multiple perforations , dirty serosa , and necrotic mucosa with greenish black exudates .
microscopic examination of the colon and small intestine showed ischemic necrosis of mucosa with acute and chronic inflammation , and foreign body reaction .
microscopic examination of the liver also showed totally necrotic parenchyma with many angioinvasive mucormycotic fungal hyphae .
auscultation of the lungs revealed decreased breathing sounds with rale on both lung fields , and saturation of arterial blood gas analysis was decreased .
he underwent intubation and ventilation . on the 21st - hospital day , the patient expired .
clinically this condition is divided into gastric emphysema and emphysematous gastritis ( 3 , 5 , 12 ) .
these two conditions should be differentiated because they are characterized by different clinical symptoms , possible etiology , radiologic findings , treatment , and prognosis ( 3 , 5 , 12 ) . in gastric emphysema , air enters the stomach wall from the lumen , from the peritoneal surface , or from its connections with the esophagus and duodenum , resulting from barotraumas in the absence of bacterial infection ( 12 ) .
radiologically , in contrast to emphysematous gastritis , a more linear distribution of gas in the gastric wall is characteristic of gastric emphysema ( 3 ) .
the symptom of acute abdomen as seen in patients with emphysematous gastritis is usually absent in patients with gastric emphysema ( 5 ) . in general ,
gastric emphysema is asymptomatic ; its course is usually benign and resolves spontaneously without treatment ( 3 , 5 , 12 ) . on the other hand , emphysematous gastritis , the gas formed in situ by gas - forming bacteria invading the gastric wall , results in a necrotizing inflammation of the gastric wall . in 1889
, fraenkel first reported a young man who died after several attacks of severe abdominal pain , blood vomitus , and diarrhea ( 1 ) . at autopsy ,
originally , the stomach wall is well protected from bacterial infection by the close connection between cells , acidic ph , and good blood supply ( 3 , 12 ) .
the predisposing factors , leading to the breakdown of these defenses , are ingestion of corrosives , alcohol abuse , diabetes , recent abdominal surgery , gastroenteritis , immunocompromised conditions , and treatment with non - steroidal anti - inflammatory drugs ( 1 - 3 ) . in the present case ,
the patient had a history of alcohol abuse , which might have altered the unstirred mucous layer , and diabetes due to chronic pancreatitis , which might have led to a systemic predisposition to infection .
patients with emphysematous gastritis typically develop acute abdominal pain , as was the case in our patient , usually developing 1 week after initiation , accompanied by diarrhea , nausea , vomiting , and occasionally hematemesis and melena ( 1 , 4 , 12 ) .
physical examination of the abdomen reveals epigastric tenderness , distension , and decreased bowel sounds ( 4 , 12 ) .
abdominal computed tomography , the best imaging modality to establish the diagnosis , reveals gastric wall thickening and intramural gas ( 1 , 3 , 12 ) .
in contrast to gastric emphysema , the gas in the wall is in the form of irregular mottled bubbles or spots , especially around the fundus and the greater curvature , and remains in place despite the position of the body or absorption through the gastric tube ( 3 , 4 , 12 ) .
organisms that have been cultured from stomach aspirates , blood , and peritoneal fluid include streptococci , e. coli , p. aeruginosa , clostridium .
perfringens , and s. aureus ( 1 , 4 ) . among the previously reported cases , those associated with fungal infection were rare ( 4
, 11 - 15 ) . of these , most cases were candida species ( 4 , 11 , 12 , 15 ) .
pathologically , the gas forming organisms in emphysematous gastritis have been shown to infiltrate the stomach wall diffusely and there are edematous stomach wall - containing gas bubbles ( 1 , 6 ) . the therapeutic approach for emphysematous gastritis included initial stabilization of the septic condition through vigorous fluid resuscitation and early empirical parenteral antibiotic therapy with a broad range of antibiotics covering gram - negative and anaerobic bacteria ( 2 , 3 , 12 , 14 ) .
specific therapy should be modified according to the results of the gastric fluid culture and sensitivity testing of the isolated organism ( 12 ) .
indication for emergency surgery include deterioration despite optimal medical management , involvement of a large portion of or the entire stomach , presence of gastric infarction , or perforation ( 3 , 4 ) . despite meticulous treatment ,
the mortality rate of emphysematous gastritis is about 60% , and the morbidity near 21% ( 2 ) .
mucormycosis is a rare , opportunistic fungal infection that occurs almost exclusively in immunocompromised hosts such as patients with diabetes , leukemia , lymphoma , renal disease , septicemia , burns , malnutrition , and following long - term treatment with steroids and antibiotics ( 10 ) .
clinical manifestations of mucormycosis can be categorized as rhinocerebral , pulmonary , disseminated , gastrointestinal , and cutaneous ( 9 ) . among these ,
rhinocerebral and pulmonary disease are the most common forms , and gastrointestinal involvement is very rare , accounting for only 7% of all cases ( 16 ) .
other associated conditions are immunosuppressed conditions such as renal failure , hematological malignancies , cirrhosis , and solid organ transplantation ( 16 ) .
it is believed that infection of the alimentary tract is acquired through direct ingestion of and invasion by fungal spores .
the gastrointestinal organ most frequently involved is the stomach , followed by the colon , small intestine , and esophagus ( 16 ) .
when they invade , the lesion extends and marked surrounding induration develops , with a shaggy , velvety discolored surface or large plaqe - like areas of green and blacked eschar ( 16 ) .
they also invade blood vessels and thus tend to cause extensive thrombosis , necrosis , and , ultimately , dissemination ( 10 ) .
association with emphysematous gastritis is an extremely rare , and only one such case has been reported ( 15 ) .
mucormycosis is not gas - forming , therefore this infection is not a direct cause of emphysematous gastritis .
gastric wall necrosis caused by bacterial infection may be predisposed to the invasion by mucormycosis . alternatively , it is possible that invasive mucormycosis with subsequent gastric wall necrosis led to secondary bacterial invasion and intramural gas production ( 15 ) .
successful management of mucormycosis includes aggressive metabolic support , amphotericin b , and surgical debridement of all necrotic involved tissue ( 15 , 16 ) . as in emphysematous gastritis , the prognosis of gastrointestinal mucormycosis is poor .
forty - two cases of gastric mucormycosis have been described in the literature thus for , with a mortality above 98% ( 15 ) .
we report an extremely rare case of emphysematous gastritis associated with invasive gastric mucormycosis in a 43-yr - old man . heavy alcohol abuse and diabetes mellitus were the predisposing factors .
ct is the diagnostic procedure of choice of emphysematous gastritis and helps in differentiating with gastric emphysema .
moreover , the gastric mucormycosis showed angioinvasion , necrosis , and ultimately , disseminate to colon and liver . in the case of mucormycosis , biopsy of involved areas
this patient also had a fatal outcome despite antibiotic and antifungal therapy and surgery . increased awareness of these disease entities may facilitate early diagnosis , prompt medical therapy , and appropriate surgical intervention , and ultimately improve survival rates .
 emphysematous gastritis is a rare form of phlegmonous gastritis , characterized by air in the wall of the stomach due to invasion by gas - forming microorganisms . 
 the most commonly involved microorganisms are streptococci , escherichia coli , pseudomonas aeruginosa , clostrodium perfrigens and staphylococcus aureus . 
 gastrointestinal mucormycosis is another rare condition , which is most frequently occurs in the stomach . because emphysematous gastritis associated with invasive gastric mucormycosis is an extremely rare clinical condition and both are 
 life - threatening diseases , early precise diagnosis and early treatment should be done to avoid mortality . 
 herein we present an extremely rare case of emphysematous gastritis associated with invasive gastric mucormycosis . 
 a 43-yr - old man , suffering from alcoholism and diabetes , has experienced diffuse abdominal pain for 4 days . 
 abdominal computed tomography scan demonstrated gas within the stomach wall . 
 a histologic examination of the total gastrectomy specimen showed several gas - filled bubbles in the wall , along with numerous fungal hyphae throughout the necrotic stomach wall . 
 he died of multiorgan failure secondary to disseminated mucormycosis , despite the intensive medical therapy . 
INTRODUCTION
CASE REPORT
DISCUSSION