Pneumatosis of the gastrointestinal tract was first reported in a cadaver in the year 1730 by Thorpe[5
] and Du vernoi[6
]. Later in the year 1876, pneumatosis intestinalis was reported in humans by Bang[7
]. Since then numerous cases of pneumatosis of the gastrointestinal tract have been described. In 1979, Jamart reported a review of 919 cases of pneumatosis intestinalis and found that pneumatosis of small bowel was predominantly subserous and that of colon was submucosal in location[8
]. Pneumatosis can involve any part of the gastrointestinal tract from the esophagus to the rectum, but has been reported more commonly in small bowel than large bowel. Cases of gastric pneumatosis have also been published but esophageal pneumatosis has been a rare occurrence.
On review of the available medical literature, we found eight cases (Table ) of esophageal pneumatosis reported till date. Interestingly, most of these cases including our case as well were reported in females except for two cases. Vanasin et al[9
], described the first case of esophageal pneumatosis in a 62 year old woman related to esophageal stricture. Katz et al[10
] reported three cases of esophageal pneumatosis related to fiberesophagoscopy. Fifth case was described by McKelvie et al[11
] in a 76-year-old man with infectious gastritis. Sixth case in a 74-year-old woman post duodeno-pancreatectomy was published in 1998 by Tixedor et al[12
]. Mclaughlin et al[13
] reported the seventh case of esophageal pneumatosis in a 77-year-old man who had underwent resection of cholangiocarcinoma. Yahng et al[14
] reported the eighth case in a 62-year-old woman who developed esophageal pneumatosis post chemotherapy with neutropenia.
Esophageal pneumatosis case reports
Pneumatosis may be idiopathic or may occur secondary to underlying pathological process. These secondary forms of pneumatosis account for about 85% of total cases[15
]. Pneumatosis intestinalis has been described in various medical conditions including necrotizing enterocolitis, pyloric stenosis, peptic ulcer disease, jejunoileal bypass, and intestinal obstruction. Besides being described in gastrointestinal disorders, it has also been reported in non-gastrointestinal disorders like asthma, chronic obstructive pulmonary disease, celiac disease, systemic lupus erythematosus, infectious enteritis, acquired immunodeficiency syndrome, primary immunodeficiency, leukemia, organ transplantation and bone marrow transplantation[3
Despite several cases reported in literature, pathogenesis of pneumatosis remains unclear. Two important theories proposed among several other proposed are mechanical and bacterial theory. According to mechanical theory, air present in the gastrointestinal tract lumen dissects into the walls of the gastrointestinal tract through a mucosal tear. Bacterial theory proposes that the air entering the walls of bowel is produced by gas forming bacteria in the bowel.
In our patient, we hypothesize that resection of the laryngocele followed by positive pressure ventilation may have predisposed to the formation of the pneumatosis, and hence supporting mechanical theory. Other possible etiology in our patient could be the underlying chronic bronchitis secondary to long standing smoking.
Typically it is an asymptomatic condition as in our patient. However may have a broad spectrum of presentation from an incidental finding to retrosternal chest pain, abdominal pain or septic shock. Symptoms may vary depending upon site of gastrointestinal tract involved.
Work up includes radiographs of the chest and the abdomen. CT, magnetic resonance imaging and barium studies[3
] can also be used, but CT has better sensitivity to detect intramural air[18
Management largely depends on the underlying etiology. Various treatment modalities have been utilized in the past including oxygen therapy and antibiotic use. Normobaric oxygen therapy, after its first experimental use in 1935, was used successfully for treatment of pneumatosis by Forgacs et al[20
]. Later in 1978, the use of hyperbaric oxygen was also shown to be beneficial by Masterson et al[21
]. Antibiotic therapy with Metronidazole has also been reported to be efficacious in literature supporting the bacterial theory of genesis[22
Algorithm for surgical management of pneumatosis has been suggested by Greenstein et al[23
] in cases of obstruction, elevated white cell count, advanced age and portal venous gas. Endoscopic modalities of therapy with cyst puncture and sclerotherapy have also been described[24
]. But with numerous case reports of success with conservative therapy, treatment of pneumatosis essentially remains non-surgical unless acute abdomen is suspected.