Pneumomediastinum in inflammatory bowel disease Nino Mihatov, MD, and Andrew Z. Fenves, MD
A 28-year-old man with a history of inflammatory bowel disease (IBD) developed sudden-onset chest pain and dyspnea 9 days after esophagogastroduodenoscopy and colonoscopy. A chest radiograph demonstrated pneumomediastinum tracking along the left heart border. The spontaneous pneumomediastinum was presumed to be a complication of his severe colitis. The severity of our patient’s symptoms ultimately necessitated a subtotal colectomy, a decision unrelated to the pneumomediastinum. IBD-associated pneumomediastinum can be attributed to retroperitoneal air leakage from severe colitis and usually resolves with conservative management.
P
neumomediastinum, often associated with diseases of or traumatic injury to the airway, is a rare complication of inflammatory bowel disease (IBD). In IBD, pneumomediastinum is most frequently a procedural complication associated with esophageal microperforation during esophagogastroduodenoscopy or colonic perforation during colonoscopy (1). We present a patient with severe IBD who developed spontaneous pneumomediastinum. CASE REPORT A 28-year-old man with an 11-month history of IBD presented to an outside hospital with 6 weeks of fatigue, severe abdominal pain, diarrhea, and hematochezia. He was treated for an IBD flare with corticosteroids, mesalamine, and infliximab. He underwent a flexible sigmoidoscopy that demonstrated extensive rectal inflammation and ulceration suggestive of ulcerative colitis. After 3 weeks of persistent symptoms, he was transferred to our hospital for further management and surgical evaluation. Prior to his admission, he was taking mesalamine, prednisone, and hydrocortisone enemas. He was a graduate student who occasionally drank alcohol but had never smoked or used illicit substances. He had no known drug allergies. His family history was notable for colon cancer in his paternal grandfather and liver cancer in his maternal uncle. On admission to our hospital, he was afebrile, with a heart rate of 84 beats/minute, blood pressure of 100/60 mm Hg, and oxygen saturation of 94% on room air. He appeared uncomfortable and was vomiting. His cardiopulmonary examination disclosed no abnormalities. His abdomen was mildly distended and tender to palpation without organomegaly. Rectal examination revealed few external hemorrhoids without fissures or masses. Proc (Bayl Univ Med Cent) 2015;28(3):347–349
The patient’s hemoglobin was 8.1 g/dL and the hematocrit was 24.5%. His white blood cell count, platelet count, basic chemistries, prothrombin time, and partial thromboplastin time were normal. The serum albumin was 2.5 g/dL. The serum iron was 17 mcg/dL, iron binding capacity was 212 mcg/dL, and ferritin was 16 ng/mL. A chest radiograph was normal. On hospital day 2, the patient underwent an esophagogastroduodenoscopy that demonstrated a normal esophagus, stomach, and duodenum. Colonoscopy with biopsy revealed severe inflammation from the rectum to the transverse colon and discontinuously in the cecum in a pattern suggestive of Crohn’s disease. Corticosteroids and infliximab were continued. On day 11 of hospitalization, 9 days after the procedure, the patient developed sudden-onset chest pain and dyspnea. He was no longer vomiting and had not been retching or coughing. The chest pain worsened with positional changes and inspiration. He remained hemodynamically stable. Examination of the chest revealed palpable subcutaneous crepitus over both sternoclavicular joints. Lungs were clear to auscultation bilaterally. An anteroposterior chest radiograph showed pneumomediastinum tracking along the left cardiac border and superiorly in the bilateral soft tissues of the upper mediastinum and neck (Figure 1a). A barium swallow study revealed a normally distensible esophagus with no areas of abnormal contrast retention or extravasation (Figure 2). Computed tomography (CT) of the chest with oral contrast demonstrated contrast in the esophagus without obvious defect of the esophageal wall or extravasation of esophageal contrast (Figure 3). Further CT of the abdomen with oral and intravenous contrast demonstrated mild wall thickening of the distal descending and rectosigmoid colon with pericolonic stranding consistent with inflammatory bowel disease. The small bowel was normal. There was no evidence of toxic megacolon or retroperitoneal free air. With cessation of oral intake and intravenous broadspectrum antibiotics, the chest pain and dyspnea decreased. Radiographically, the patient had progressive reduction in the From the Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts. Corresponding author: Nino Mihatov, MD, Massachusetts General Hospital, Harvard Medical School, Department of Medicine, 55 Fruit Street, GRB 740, Boston, MA 02114 (e-mail: [email protected]). 347
a
b
Figure 1. Chest radiograph (a) at the onset of symptoms, with mediastinal air (white arrows) evident along the left heart border and upper mediastinum, and (b) 9 days later, with resolution of the previously demonstrated pneumomediastinum.
degree of mediastinal air with resolution by day 9 following the onset of symptoms (Figure 1b). The patient continued to have abdominal pain refractory to medical therapy. A repeat flexible sigmoidoscopy demonstrated worse colitis compared to the prior colonoscopy. The patient underwent an uncomplicated laparoscopic subtotal colectomy with ileostomy on hospital day 16. He was discharged on day 21. Pathology of the excised colon revealed severely active chronic colitis without dysplasia or granulomas. There was no evidence of colonic perforation.
Figure 2. Barium swallow study at the time of symptom onset demonstrating contrast in the esophagus and gastric fundus without evidence of contrast extravasation or esophageal wall defect. 348
DISCUSSION Pneumomediastinum is an overall rare finding characterized by free air within the mediastinum. Some principal causes include 1) blunt trauma to the chest; 2) increased alveolar pressure and rupture from coughing, straining, emesis, or positive pressure ventilation; 3) gas-forming organisms driving an infectious process in the neck, chest, or oropharynx; and 4) a complication from esophageal or colonic instrumentation and subsequent perforation (1–4). Spontaneous pneumomediastinum is defined by the absence of a clear inciting cause for mediastinal air accumulation. There are nine reported cases of spontaneous pneumomediastinum in association with inflammatory bowel disease, most commonly ulcerative colitis, and in the absence of evidence of perforation (5–12). In our patient who was not coughing or actively vomiting and had no underlying lung disease or perforation of a viscous, the spontaneous pneumomediastinum was thought to be a complication of his severe colitis. Symptoms of pneumomediastinum include dyspnea, chest pain, and neck pain. Examination often reveals subcutaneous crackling in the neck and cervical soft tissue reflective of free air (13). Pneumomediastinum can be diagnosed with plain chest radiographs and confirmatory CT. Identifying the cause of the pneumomediastinum can prove difficult. In IBD, severe inflammation facilitates forcible herniation of colonic mucosa, allowing microscopic perforations to develop that are permeable to air (7). The risk of colonic perforation in patients with ulcerative colitis, in particular, has been correlated to the severity of symptoms (14). The retroperitoneum communicates with the mediastinum and subcutaneous tissue through the visceral space, one of three described compartments of the neck (4). The visceral space follows the trachea and esophagus into the chest and creates a tract for air between the mediastinum and the neck. The space continues inferiorly through the diaphragmatic hiatus into the retroperitoneum.
Baylor University Medical Center Proceedings
Volume 28, Number 3
a
b
Figure 3. (a) Axial and (b) coronally reformatted CT images of the chest with intravenous contrast and oral contrast ingested at the time of image acquisition. Oral contrast is seen in the distal esophagus with no evidence of extravasation (black arrow). Mediastinal air is seen throughout the mediastinum, particularly along the heart border with extension to the soft tissue of the neck (white arrows).
The overall rarity of pneumomediastinum makes validating an optimal management strategy difficult. In general, management of pneumomediastinum requires surgical correction of any mucosal disruption, treatment of any infectious causes, and elimination of contributors to increased alveolar pressure such as bronchospasm or positive-pressure ventilation (4). In spontaneous pneumomediastinum, nonoperative management is often most effective (2, 15, 16). In IBD-associated pneumomediastinum, management of IBD symptoms has been reported to result in resolution of mediastinal air (6, 8, 11, 12). Although the decision to pursue surgery in this case was unrelated to the pneumomediastinum, persistent pneumomediastinum may reflect a severe degree of colitis that may necessitate surgical intervention. Pneumomediastinum should be considered in a patient with severe IBD symptoms who acutely develops pleuritic chest pain and dyspnea. An exhaustive search for a primary cause of pneumomediastinum, most notably colonic or esophageal perforation following instrumentation, should be undertaken. In the absence of a clear cause, pneumomediastinum can be attributed to retroperitoneal air leakage from severe colitis and should resolve with conservative management. 1.
2.
3.
Cappello M, Randazzo C, Peralta S, Cocorullo G. Subcutaneous emphysema, pneumomediastinum and pneumoperitoneum after diagnostic colonoscopy for ulcerative colitis: a rare but possible complication in patients with multiple risk factors. Int J Colorectal Dis 2011;26(3):393–394. Park NS, Choi JH, Lee DH, Kim YJ, Kim ES, Jung SW, Koo JS, Lee HS, Lee SW. Pneumoretroperitoneum, pneumomediastinum, pneumopericardium, and subcutaneous emphysema after colonoscopic examination. Gut Liver 2007;1(1):79–81. Dehal A, Tessier DJ. Intraperitoneal and extraperitoneal colonic perforation following diagnostic colonoscopy. JSLS 2014;18(1):136–141.
July 2015
4.
5.
6.
7. 8.
9. 10.
11.
12.
13. 14.
15. 16.
Maunder RJ, Pierson DJ, Hudson LD. Subcutaneous and mediastinal emphysema. Pathophysiology, diagnosis, and management. Arch Intern Med 1984;144(7):1447–1453. Mogan GR, Sachar DB, Bauer J, Salky B, Janowitz HD. Toxic megacolon in ulcerative colitis complicated by pneumomediastinum: report of two cases. Gastroenterology 1980;79(3):559–562. Annaházi A, Polyák I, Nagy F, Wittmann T, Molnár T. “Ulcerative crepitus”—a case with subcutaneous emphysema and pneumomediastinum without colonic perforation or toxic megacolon in ulcerative colitis successfully treated conservatively. J Crohns Colitis 2012;6(6):717–719. Cohen ME, Kleinman MS. Pneumomediastinum during relapse of ulcerative colitis. Am J Gastroenterol 1997;92(12):2306–2307. Martín Castillo A, Pons Miñano JA, Riquelme Riquelme J, Parrilla Paricio P. Retroperitoneal perforation, pneumomediastinum and subcutaneous emphysema in severe ulcerative colitis. Gastroenterol Hepatol 2003;26(4):275. Badina L, Ferrara G, Guastalla P, Barbi E. A precordial rub in a boy with a severe attack of ulcerative colitis. Pediatr Emerg Care 2014;30(4):268. Blanchet E, Kull E, Beau P. Pneumomediastinum without colonic perforation during a severe attack of ulcerative colitis. Gastroenterol Clin Biol 2001;25(12):1121–1123. Alvares JF, Dhawan PS, Tibrewala S, Shankaran K, Kulkarni SG, Rananavare R, Kalro RH. Retroperitoneal perforation in ulcerative colitis with mediastinal and subcutaneous emphysema. J Clin Gastroenterol 1997;25(2):453–455. Cooke AA, Deshpande AV, Shun A, O’Loughlin EV. Pneumomediastinum and subcutaneous emphysema in a child with ulcerative colitis. Pediatr Emerg Care 2010;26(2):129–131. Baumann MH, Sahn SA. Hamman’s sign revisited. Pneumothorax or pneumomediastinum? Chest 1992;102(4):1281–1282. De Dombal FT, Watts JM, Watkinson G, Goligher JC. Intraperitoneal perforation of the colon in ulcerative colitis. Proc R Soc Med 1965;58(9):713–715. Waye JD, Kahn O, Auerbach ME. Complications of colonoscopy and flexible sigmoidoscopy. Gastrointest Endosc Clin N Am 1996;6(2):343–377. Winek TG, Mosely HS, Grout G, Luallin D. Pneumoperitoneum and its association with ruptured abdominal viscus. Arch Surg 1988;123(6): 709–712.
Pneumomediastinum in inflammatory bowel disease
349
A 28-year-old man with a history of inflammatory bowel disease (IBD) developed sudden-onset chest pain and dyspnea 9 days after esophagogastroduodenoscopy and colonoscopy. A chest radiograph demonstrated pneumomediastinum tracking along the left heart border. The spontaneous pneumomediastinum was presumed to be a complication of his severe colitis. The severity of our patient’s symptoms ultimately necessitated a subtotal colectomy, a decision unrelated to the pneumomediastinum. IBD-associated pneumomediastinum can be attributed to retroperitoneal air leakage from severe colitis and usually resolves with conservative management.
P
neumomediastinum, often associated with diseases of or traumatic injury to the airway, is a rare complication of inflammatory bowel disease (IBD). In IBD, pneumomediastinum is most frequently a procedural complication associated with esophageal microperforation during esophagogastroduodenoscopy or colonic perforation during colonoscopy (1). We present a patient with severe IBD who developed spontaneous pneumomediastinum. CASE REPORT A 28-year-old man with an 11-month history of IBD presented to an outside hospital with 6 weeks of fatigue, severe abdominal pain, diarrhea, and hematochezia. He was treated for an IBD flare with corticosteroids, mesalamine, and infliximab. He underwent a flexible sigmoidoscopy that demonstrated extensive rectal inflammation and ulceration suggestive of ulcerative colitis. After 3 weeks of persistent symptoms, he was transferred to our hospital for further management and surgical evaluation. Prior to his admission, he was taking mesalamine, prednisone, and hydrocortisone enemas. He was a graduate student who occasionally drank alcohol but had never smoked or used illicit substances. He had no known drug allergies. His family history was notable for colon cancer in his paternal grandfather and liver cancer in his maternal uncle. On admission to our hospital, he was afebrile, with a heart rate of 84 beats/minute, blood pressure of 100/60 mm Hg, and oxygen saturation of 94% on room air. He appeared uncomfortable and was vomiting. His cardiopulmonary examination disclosed no abnormalities. His abdomen was mildly distended and tender to palpation without organomegaly. Rectal examination revealed few external hemorrhoids without fissures or masses. Proc (Bayl Univ Med Cent) 2015;28(3):347–349
The patient’s hemoglobin was 8.1 g/dL and the hematocrit was 24.5%. His white blood cell count, platelet count, basic chemistries, prothrombin time, and partial thromboplastin time were normal. The serum albumin was 2.5 g/dL. The serum iron was 17 mcg/dL, iron binding capacity was 212 mcg/dL, and ferritin was 16 ng/mL. A chest radiograph was normal. On hospital day 2, the patient underwent an esophagogastroduodenoscopy that demonstrated a normal esophagus, stomach, and duodenum. Colonoscopy with biopsy revealed severe inflammation from the rectum to the transverse colon and discontinuously in the cecum in a pattern suggestive of Crohn’s disease. Corticosteroids and infliximab were continued. On day 11 of hospitalization, 9 days after the procedure, the patient developed sudden-onset chest pain and dyspnea. He was no longer vomiting and had not been retching or coughing. The chest pain worsened with positional changes and inspiration. He remained hemodynamically stable. Examination of the chest revealed palpable subcutaneous crepitus over both sternoclavicular joints. Lungs were clear to auscultation bilaterally. An anteroposterior chest radiograph showed pneumomediastinum tracking along the left cardiac border and superiorly in the bilateral soft tissues of the upper mediastinum and neck (Figure 1a). A barium swallow study revealed a normally distensible esophagus with no areas of abnormal contrast retention or extravasation (Figure 2). Computed tomography (CT) of the chest with oral contrast demonstrated contrast in the esophagus without obvious defect of the esophageal wall or extravasation of esophageal contrast (Figure 3). Further CT of the abdomen with oral and intravenous contrast demonstrated mild wall thickening of the distal descending and rectosigmoid colon with pericolonic stranding consistent with inflammatory bowel disease. The small bowel was normal. There was no evidence of toxic megacolon or retroperitoneal free air. With cessation of oral intake and intravenous broadspectrum antibiotics, the chest pain and dyspnea decreased. Radiographically, the patient had progressive reduction in the From the Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts. Corresponding author: Nino Mihatov, MD, Massachusetts General Hospital, Harvard Medical School, Department of Medicine, 55 Fruit Street, GRB 740, Boston, MA 02114 (e-mail: [email protected]). 347
a
b
Figure 1. Chest radiograph (a) at the onset of symptoms, with mediastinal air (white arrows) evident along the left heart border and upper mediastinum, and (b) 9 days later, with resolution of the previously demonstrated pneumomediastinum.
degree of mediastinal air with resolution by day 9 following the onset of symptoms (Figure 1b). The patient continued to have abdominal pain refractory to medical therapy. A repeat flexible sigmoidoscopy demonstrated worse colitis compared to the prior colonoscopy. The patient underwent an uncomplicated laparoscopic subtotal colectomy with ileostomy on hospital day 16. He was discharged on day 21. Pathology of the excised colon revealed severely active chronic colitis without dysplasia or granulomas. There was no evidence of colonic perforation.
Figure 2. Barium swallow study at the time of symptom onset demonstrating contrast in the esophagus and gastric fundus without evidence of contrast extravasation or esophageal wall defect. 348
DISCUSSION Pneumomediastinum is an overall rare finding characterized by free air within the mediastinum. Some principal causes include 1) blunt trauma to the chest; 2) increased alveolar pressure and rupture from coughing, straining, emesis, or positive pressure ventilation; 3) gas-forming organisms driving an infectious process in the neck, chest, or oropharynx; and 4) a complication from esophageal or colonic instrumentation and subsequent perforation (1–4). Spontaneous pneumomediastinum is defined by the absence of a clear inciting cause for mediastinal air accumulation. There are nine reported cases of spontaneous pneumomediastinum in association with inflammatory bowel disease, most commonly ulcerative colitis, and in the absence of evidence of perforation (5–12). In our patient who was not coughing or actively vomiting and had no underlying lung disease or perforation of a viscous, the spontaneous pneumomediastinum was thought to be a complication of his severe colitis. Symptoms of pneumomediastinum include dyspnea, chest pain, and neck pain. Examination often reveals subcutaneous crackling in the neck and cervical soft tissue reflective of free air (13). Pneumomediastinum can be diagnosed with plain chest radiographs and confirmatory CT. Identifying the cause of the pneumomediastinum can prove difficult. In IBD, severe inflammation facilitates forcible herniation of colonic mucosa, allowing microscopic perforations to develop that are permeable to air (7). The risk of colonic perforation in patients with ulcerative colitis, in particular, has been correlated to the severity of symptoms (14). The retroperitoneum communicates with the mediastinum and subcutaneous tissue through the visceral space, one of three described compartments of the neck (4). The visceral space follows the trachea and esophagus into the chest and creates a tract for air between the mediastinum and the neck. The space continues inferiorly through the diaphragmatic hiatus into the retroperitoneum.
Baylor University Medical Center Proceedings
Volume 28, Number 3
a
b
Figure 3. (a) Axial and (b) coronally reformatted CT images of the chest with intravenous contrast and oral contrast ingested at the time of image acquisition. Oral contrast is seen in the distal esophagus with no evidence of extravasation (black arrow). Mediastinal air is seen throughout the mediastinum, particularly along the heart border with extension to the soft tissue of the neck (white arrows).
The overall rarity of pneumomediastinum makes validating an optimal management strategy difficult. In general, management of pneumomediastinum requires surgical correction of any mucosal disruption, treatment of any infectious causes, and elimination of contributors to increased alveolar pressure such as bronchospasm or positive-pressure ventilation (4). In spontaneous pneumomediastinum, nonoperative management is often most effective (2, 15, 16). In IBD-associated pneumomediastinum, management of IBD symptoms has been reported to result in resolution of mediastinal air (6, 8, 11, 12). Although the decision to pursue surgery in this case was unrelated to the pneumomediastinum, persistent pneumomediastinum may reflect a severe degree of colitis that may necessitate surgical intervention. Pneumomediastinum should be considered in a patient with severe IBD symptoms who acutely develops pleuritic chest pain and dyspnea. An exhaustive search for a primary cause of pneumomediastinum, most notably colonic or esophageal perforation following instrumentation, should be undertaken. In the absence of a clear cause, pneumomediastinum can be attributed to retroperitoneal air leakage from severe colitis and should resolve with conservative management. 1.
2.
3.
Cappello M, Randazzo C, Peralta S, Cocorullo G. Subcutaneous emphysema, pneumomediastinum and pneumoperitoneum after diagnostic colonoscopy for ulcerative colitis: a rare but possible complication in patients with multiple risk factors. Int J Colorectal Dis 2011;26(3):393–394. Park NS, Choi JH, Lee DH, Kim YJ, Kim ES, Jung SW, Koo JS, Lee HS, Lee SW. Pneumoretroperitoneum, pneumomediastinum, pneumopericardium, and subcutaneous emphysema after colonoscopic examination. Gut Liver 2007;1(1):79–81. Dehal A, Tessier DJ. Intraperitoneal and extraperitoneal colonic perforation following diagnostic colonoscopy. JSLS 2014;18(1):136–141.
July 2015
4.
5.
6.
7. 8.
9. 10.
11.
12.
13. 14.
15. 16.
Maunder RJ, Pierson DJ, Hudson LD. Subcutaneous and mediastinal emphysema. Pathophysiology, diagnosis, and management. Arch Intern Med 1984;144(7):1447–1453. Mogan GR, Sachar DB, Bauer J, Salky B, Janowitz HD. Toxic megacolon in ulcerative colitis complicated by pneumomediastinum: report of two cases. Gastroenterology 1980;79(3):559–562. Annaházi A, Polyák I, Nagy F, Wittmann T, Molnár T. “Ulcerative crepitus”—a case with subcutaneous emphysema and pneumomediastinum without colonic perforation or toxic megacolon in ulcerative colitis successfully treated conservatively. J Crohns Colitis 2012;6(6):717–719. Cohen ME, Kleinman MS. Pneumomediastinum during relapse of ulcerative colitis. Am J Gastroenterol 1997;92(12):2306–2307. Martín Castillo A, Pons Miñano JA, Riquelme Riquelme J, Parrilla Paricio P. Retroperitoneal perforation, pneumomediastinum and subcutaneous emphysema in severe ulcerative colitis. Gastroenterol Hepatol 2003;26(4):275. Badina L, Ferrara G, Guastalla P, Barbi E. A precordial rub in a boy with a severe attack of ulcerative colitis. Pediatr Emerg Care 2014;30(4):268. Blanchet E, Kull E, Beau P. Pneumomediastinum without colonic perforation during a severe attack of ulcerative colitis. Gastroenterol Clin Biol 2001;25(12):1121–1123. Alvares JF, Dhawan PS, Tibrewala S, Shankaran K, Kulkarni SG, Rananavare R, Kalro RH. Retroperitoneal perforation in ulcerative colitis with mediastinal and subcutaneous emphysema. J Clin Gastroenterol 1997;25(2):453–455. Cooke AA, Deshpande AV, Shun A, O’Loughlin EV. Pneumomediastinum and subcutaneous emphysema in a child with ulcerative colitis. Pediatr Emerg Care 2010;26(2):129–131. Baumann MH, Sahn SA. Hamman’s sign revisited. Pneumothorax or pneumomediastinum? Chest 1992;102(4):1281–1282. De Dombal FT, Watts JM, Watkinson G, Goligher JC. Intraperitoneal perforation of the colon in ulcerative colitis. Proc R Soc Med 1965;58(9):713–715. Waye JD, Kahn O, Auerbach ME. Complications of colonoscopy and flexible sigmoidoscopy. Gastrointest Endosc Clin N Am 1996;6(2):343–377. Winek TG, Mosely HS, Grout G, Luallin D. Pneumoperitoneum and its association with ruptured abdominal viscus. Arch Surg 1988;123(6): 709–712.
Pneumomediastinum in inflammatory bowel disease
349
