Diseases of the Esophagus (2014) ••, ••–•• DOI: 10.1111/dote.12200
Case report
Spontaneous circumferential intramural esophageal dissection complicated with esophageal perforation and esophageal-pleural fistula: a case report and literature review R. Y. Zhu,1 T. T. Law,2 D. Tong,2 G. Tam,2 S. Law2 Yong Loo Lin School of Medicine, National University of Singapore, Singapore, and 2Division of Esophageal and Upper Gastrointestinal Surgery, Department of Surgery, University of Hong Kong, Queen Mary Hospital, Hong Kong 1
SUMMARY. Spontaneous intramural esophageal dissection (IED) is a rare disease entity. There are few reports of spontaneous IED requiring surgical treatment. Hereby, we report a 37-year-old gentleman who was diagnosed to have spontaneous extensive circumferential IED complicated with esophageal perforation, empyema, and esophageal-pleural fistula. Esophageal stenting and drainage of empyema were unsuccessful. Computed tomography and gastrografin contrast swallow demonstrated a leak to the pleural cavity, suggestive of esophageal-pleural fistula. Subsequently, a two-stage operation was performed: cervical esophagogastrostomy to bypass the perforated esophagus, followed by esophagectomy and decortication of the right lung. The patient recovered and was discharged home after a 3-week hospitalization. The management principles and recent published literature related to IED were reviewed.
INTRODUCTION Intramural esophageal dissection (IED) was first described by Marks and Keet in 1968.1 Other terms used to describe this clinical condition including intramucosal esophageal dissection, intramural esophageal rupture, esophageal apoplexy,2 and submucosal hematoma. IED is an uncommon but distinct clinical entity characterized by the separation of the mucosa and/or submucosa from deeper muscular layers. Spontaneous IED is classically seen in elderly women who are often taking anticoagulants, yet it has also been reported in young healthy individuals.3 In the latter case, it is usually precipitated by repeated retching or vomiting, which shares the same precipitating factors as Mallory–Weiss tear or Boerhaave’s syndrome.4,5 Other causes of IED include iatrogenic interventions (such as endoscopy) or foreign body impaction.5–7 The proposed pathophysiology of IED is that an esophageal mucosal break followed by an Address correspondence to: Professor Simon Law, MS, MA(Cantab), MBBChir., FRCSEd, FCSHK, FHKAM, FACS, Chief, Division of Esophageal and Upper Gastrointestinal Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong. Email: [email protected] Financial disclosure: Nil. © 2014 International Society for Diseases of the Esophagus
increase in intraesophageal pressure that causes separation of mucosa and/or submucosa from muscular layers. Mucosal break can be caused by trauma such as foreign body impaction or air insufflations such as endoscopic intervention; whereas bleeding within the submucosal layer may account for IED in patients taking anticoagulants. Common presenting symptoms include dysphagia, odynophagia, and chest pain whereas patients with submucosal hematoma may present with postprandial hematemesis.8 When the dissection confines to the submucosal plane, the risk of mediastinal contamination is low and the patient can be treated conservatively. However, if the dissection had transmural involvement with esophageal perforation, surgical treatment may be indicated.9–12 We report an unusual case of spontaneous extensive circumferential IED complicated by esophageal perforation, empyema, and esophageal-pleura fistula requiring esophagectomy as definitive treatment. Literatures on other therapeutic approaches for IED are reviewed. CASE REPORT A 37-year-old gentleman presented with a 2-week history of dyspnea and right-sided chest discomfort. 1
2
Diseases of the Esophagus
Fig. 1 Chest X-ray showing right lower and middle zone consolidation and loculated pleural effusion.
He did not have dysphagia or odynophagia. There was no history of vomiting or foreign body ingestion. He was febrile and tachycardic on admission. Physical examination demonstrated reduced breath sound and crepitations in the right chest. Laboratory tests revealed leukocytosis. Chest X-ray showed (A)
loculated right pleural effusion (Fig. 1). He was treated as pneumonia with antibiotics and pleural drainage. Subsequent computed tomography (CT) scan of the thorax and abdomen showed extensive circumferential IED extending from the cervical esophagus to the gastroesophageal junction (GEJ). There was no contrast extravasation. Oesophagogastroduodenoscopy (OGD) was performed and found a mucosal perforation at the middle third of the esophagus (29 cm). Patient was treated conservatively with nasogastric tube feeding and drainage of the right pleural effusion. He remained well, and reassessment CT scan 2 weeks later showed that the esophagus was dilated and with circumferential wall thickening (Fig. 2). There were rim enhancing subpulmonic pleural effusions loculated at the right lower hemithorax. Gastrografin contrast swallow demonstrated a dissected esophagus (Fig. 3a,b). An esophageal stent (Polyflex stent, Boston Scientific, Boston, MA, USA) was deployed under endoscopic and fluoroscopic guidance with an attempt to seal the perforation. However, he had persistent sepsis and an increased amount of turbid fluid from the chest drain was noted. Repeated OGD identified a new perforation site from 34 to 38 cm and that was distal to the distal end of the stent (GEJ at 43 cm). Another CT scan showed circumferential wall thickening of distal esophagus with paraesophageal air pockets distal to esophageal stent tracking to the right posterior pleura with pleural collection, suggestive of esophageal-pleural fistula (Fig. 4). (C)
(B)
Fig. 2 Computed tomography showing (A) extensive circumferential esophageal dissection extending from cervical esophagus to esophagogastric junction. (B) Esophageal dissection with true lumen (black arrow, nasogastric tube in situ) and false lumen (white arrow). (C) Loculated subpulmonic pleural effusion in right lower hemithorax with rim enhancement. © 2014 International Society for Diseases of the Esophagus
Spontaneous circumferential IED
3
(A)
Fig. 3 (A) Gastrografin swallow showing esophageal perforation and dissection into the submucosal space from the upper esophagus down to the esophagogastric junction. (B) White arrow: septum. Hash: true lumen. Asterisk: false lumen.
He was referred to our unit 1 month after stenting. OGD showed that the Polyflex stent was located at 24–32 cm, there was an ulcerative area at 34–35 cm communicating to the false lumen leading to a fistula © 2014 International Society for Diseases of the Esophagus
(Fig. 5). Gastrografin contrast swallow confirmed that the leakage site was at the right lower esophagus 5 cm inferior to the stent, and multiple contrast out-pouchings along mid and lower esophagus were
4
Diseases of the Esophagus
(B)
Fig. 3 Continued.
identified (Fig. 6). Given the long-standing history of esophageal perforation and contamination, esophageal exclusion with esophagogastrostomy bypass followed by second-stage esophagectomy was performed. During the first operation, the esophagus was excluded proximally at the cervical level via a left neck incision and stapled distally at the abdomen. A narrow gastric tube was created for reconstruction via the retrosternal route and cervical esophagogastrostomy was performed. A drainage tube was kept in the esophageal lumen for decompression. There was subclinical leakage at the esophagogastric anastomosis and that was treated conservatively. He underwent esophagectomy and decortication of the right lung 1 month later. The operation was difficult because of the dense adhesions in the right pleural cavity as a result of esophageal perforation and empyema thoracis. Postoperative course was uneventful. He was discharged home 3 weeks after the esophageal exclusion. The resected segment of esophagus measured 15 cm (length) × 6 cm (width). A 5 cm (length) × 3.5 cm (width) mucosal defect was noted in the middle third of esophagus (Fig. 7). Microscopically deep fissuring ulcers lined by inflamed granulation tissue were noted particularly in the lower third of the esophagus. The ulcers involved the muscularis propria layer. There was no evidence of viral infection or malignancy.
DISCUSSION IED is generally regarded as a benign condition that usually can be treated conservatively. IED is an intramural pathology most of the time; however, it can result in esophageal perforation. For IED patients without esophageal perforation, intravenous fluid, antiemetic and proton pump inhibitor with close monitoring were probably adequate treatments. When there is perforation, the treatment should follow the principle of esophageal perforation management. For hemodynamically stable patients, antibiotics, and chest drain insertion may be considered whereas for those with unstable cardiopulmonary vital signs and evidence of uncontrolled sepsis, surgery is essential for life saving. By far, there are few reports of IED requiring surgical treatment. Our patient had spontaneous IED but presented with atypical symptoms. The atypical presentation might cause a delay in diagnosis. For patients presented with typical symptoms including repeated vomiting, a recent history of foreign body ingestion or upper gastrointestinal tract instrumentation, a high index of suspicion can help in prompt diagnosis. Contrast swallow studies and CT scan with oral and intravenous contrasts are the preferred initial investigations.13 Contrast swallow study showing a double-barreled esophagus or a mucosal stripe sign is diagnostic of IED.5 It also allows the assessment of any associated esophageal perforation. There are different degrees of esophageal perforation as suggested by Skinner and Belsey,14 ranging from air leakage to contained perforation, free mediastinal leakage and free leakage with extension into the pleural space, which was seen in our patient. A concentric or eccentric esophageal wall thickening appeared in CT scan could represent submucosal hematoma in IED.15 There is no consensus on the treatment of IED with perforation. Endoscopic treatment options of esophageal perforation include the placement of self-expandable plastic stent (SEPS) and the use of endoscopic clips. While the use of SEPS is generally accepted as one of the treatment options in selected patients with Boerhaave’s syndrome,16–18 there is little evidence on the use of SEPS in the treatment of IED. Buscaglia reported the placement of SEPS in a case of iatrogenic IED occurring after stricture dilatation.19 However, in his report patient also underwent thoracotomy and lung decortication. Endoscopic treatment of spontaneous IED has been reported in some patients.20,21 Gluck reported the use of endoscopic dilatation of the esophageal stricture in two IED patients after spontaneous healing had occurred,20 while Bak reported endoscopic incision of the septum between true and false lumens using a needle-type diathermy knife.21 There has been only one report on successful endoscopic treatment in © 2014 International Society for Diseases of the Esophagus
Spontaneous circumferential IED
(A)
(C)
(E)
(B)
(D)
(F)
5
(G)
Fig. 4 (A–G) Computed tomography showing circumferential wall thickening of esophagus with paraesophageal air pockets (black arrows) distal to esophageal stent tracking to right posterior pleura with pleural collection noted suggesting presence of esophageal-pleural fistula.
circumferential spontaneous IED.22 Kim reported a case of successful endoscopic treatment for circumferential spontaneous IED by incision of the septum between the false and true lumens, transaction of the true esophageal wall, balloon dilatation, and metal stent insertion in a patient with underlying liver cirrhosis.22 One should note that esophageal perfora© 2014 International Society for Diseases of the Esophagus
tion did not occur in that reported case and the aim of stent insertion was to relieve dysphagia. Metal stent insertion was performed 16 months after the initial presentation. Endoscopic clips maybe used for the closure of perforations and fistulas. A device named Over-the-Scope-Clip (OTSC) (Ovesco Endoscopy AG, Tübingen, Germany) is designed for full
6
(A)
Diseases of the Esophagus
(B)
(C)
(D)
Fig. 5 (A,B) Oesophagogastroduodenoscopy showing esophageal stent. (C) Ulcerative area approximately 1 cm distal to stent. (D) Tight stricture at 37 cm.
thickness closure of perforations. The system consists of a nitinol alloy clip that is mounted on a transparent cap fitted to the tip of an endoscope. The clip is deployed when the edges of the perforation site are engaged within the cap. The concept is similar to the endoscopic band-ligation system for esophageal varices. Preliminary clinical data on OTSC closure of esophageal perforations and fistulas are encouraging. A multicenter European cohort study reported the
Fig. 6 Gastrografin swallow showing leakage at right lower esophagus, 5 cm inferior to stent (black arrow) with multiple filling out-pouchings.
result of OTSC in 36 patients with iatrogenic perforations of the gastrointestinal tract
Case report
Spontaneous circumferential intramural esophageal dissection complicated with esophageal perforation and esophageal-pleural fistula: a case report and literature review R. Y. Zhu,1 T. T. Law,2 D. Tong,2 G. Tam,2 S. Law2 Yong Loo Lin School of Medicine, National University of Singapore, Singapore, and 2Division of Esophageal and Upper Gastrointestinal Surgery, Department of Surgery, University of Hong Kong, Queen Mary Hospital, Hong Kong 1
SUMMARY. Spontaneous intramural esophageal dissection (IED) is a rare disease entity. There are few reports of spontaneous IED requiring surgical treatment. Hereby, we report a 37-year-old gentleman who was diagnosed to have spontaneous extensive circumferential IED complicated with esophageal perforation, empyema, and esophageal-pleural fistula. Esophageal stenting and drainage of empyema were unsuccessful. Computed tomography and gastrografin contrast swallow demonstrated a leak to the pleural cavity, suggestive of esophageal-pleural fistula. Subsequently, a two-stage operation was performed: cervical esophagogastrostomy to bypass the perforated esophagus, followed by esophagectomy and decortication of the right lung. The patient recovered and was discharged home after a 3-week hospitalization. The management principles and recent published literature related to IED were reviewed.
INTRODUCTION Intramural esophageal dissection (IED) was first described by Marks and Keet in 1968.1 Other terms used to describe this clinical condition including intramucosal esophageal dissection, intramural esophageal rupture, esophageal apoplexy,2 and submucosal hematoma. IED is an uncommon but distinct clinical entity characterized by the separation of the mucosa and/or submucosa from deeper muscular layers. Spontaneous IED is classically seen in elderly women who are often taking anticoagulants, yet it has also been reported in young healthy individuals.3 In the latter case, it is usually precipitated by repeated retching or vomiting, which shares the same precipitating factors as Mallory–Weiss tear or Boerhaave’s syndrome.4,5 Other causes of IED include iatrogenic interventions (such as endoscopy) or foreign body impaction.5–7 The proposed pathophysiology of IED is that an esophageal mucosal break followed by an Address correspondence to: Professor Simon Law, MS, MA(Cantab), MBBChir., FRCSEd, FCSHK, FHKAM, FACS, Chief, Division of Esophageal and Upper Gastrointestinal Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong. Email: [email protected] Financial disclosure: Nil. © 2014 International Society for Diseases of the Esophagus
increase in intraesophageal pressure that causes separation of mucosa and/or submucosa from muscular layers. Mucosal break can be caused by trauma such as foreign body impaction or air insufflations such as endoscopic intervention; whereas bleeding within the submucosal layer may account for IED in patients taking anticoagulants. Common presenting symptoms include dysphagia, odynophagia, and chest pain whereas patients with submucosal hematoma may present with postprandial hematemesis.8 When the dissection confines to the submucosal plane, the risk of mediastinal contamination is low and the patient can be treated conservatively. However, if the dissection had transmural involvement with esophageal perforation, surgical treatment may be indicated.9–12 We report an unusual case of spontaneous extensive circumferential IED complicated by esophageal perforation, empyema, and esophageal-pleura fistula requiring esophagectomy as definitive treatment. Literatures on other therapeutic approaches for IED are reviewed. CASE REPORT A 37-year-old gentleman presented with a 2-week history of dyspnea and right-sided chest discomfort. 1
2
Diseases of the Esophagus
Fig. 1 Chest X-ray showing right lower and middle zone consolidation and loculated pleural effusion.
He did not have dysphagia or odynophagia. There was no history of vomiting or foreign body ingestion. He was febrile and tachycardic on admission. Physical examination demonstrated reduced breath sound and crepitations in the right chest. Laboratory tests revealed leukocytosis. Chest X-ray showed (A)
loculated right pleural effusion (Fig. 1). He was treated as pneumonia with antibiotics and pleural drainage. Subsequent computed tomography (CT) scan of the thorax and abdomen showed extensive circumferential IED extending from the cervical esophagus to the gastroesophageal junction (GEJ). There was no contrast extravasation. Oesophagogastroduodenoscopy (OGD) was performed and found a mucosal perforation at the middle third of the esophagus (29 cm). Patient was treated conservatively with nasogastric tube feeding and drainage of the right pleural effusion. He remained well, and reassessment CT scan 2 weeks later showed that the esophagus was dilated and with circumferential wall thickening (Fig. 2). There were rim enhancing subpulmonic pleural effusions loculated at the right lower hemithorax. Gastrografin contrast swallow demonstrated a dissected esophagus (Fig. 3a,b). An esophageal stent (Polyflex stent, Boston Scientific, Boston, MA, USA) was deployed under endoscopic and fluoroscopic guidance with an attempt to seal the perforation. However, he had persistent sepsis and an increased amount of turbid fluid from the chest drain was noted. Repeated OGD identified a new perforation site from 34 to 38 cm and that was distal to the distal end of the stent (GEJ at 43 cm). Another CT scan showed circumferential wall thickening of distal esophagus with paraesophageal air pockets distal to esophageal stent tracking to the right posterior pleura with pleural collection, suggestive of esophageal-pleural fistula (Fig. 4). (C)
(B)
Fig. 2 Computed tomography showing (A) extensive circumferential esophageal dissection extending from cervical esophagus to esophagogastric junction. (B) Esophageal dissection with true lumen (black arrow, nasogastric tube in situ) and false lumen (white arrow). (C) Loculated subpulmonic pleural effusion in right lower hemithorax with rim enhancement. © 2014 International Society for Diseases of the Esophagus
Spontaneous circumferential IED
3
(A)
Fig. 3 (A) Gastrografin swallow showing esophageal perforation and dissection into the submucosal space from the upper esophagus down to the esophagogastric junction. (B) White arrow: septum. Hash: true lumen. Asterisk: false lumen.
He was referred to our unit 1 month after stenting. OGD showed that the Polyflex stent was located at 24–32 cm, there was an ulcerative area at 34–35 cm communicating to the false lumen leading to a fistula © 2014 International Society for Diseases of the Esophagus
(Fig. 5). Gastrografin contrast swallow confirmed that the leakage site was at the right lower esophagus 5 cm inferior to the stent, and multiple contrast out-pouchings along mid and lower esophagus were
4
Diseases of the Esophagus
(B)
Fig. 3 Continued.
identified (Fig. 6). Given the long-standing history of esophageal perforation and contamination, esophageal exclusion with esophagogastrostomy bypass followed by second-stage esophagectomy was performed. During the first operation, the esophagus was excluded proximally at the cervical level via a left neck incision and stapled distally at the abdomen. A narrow gastric tube was created for reconstruction via the retrosternal route and cervical esophagogastrostomy was performed. A drainage tube was kept in the esophageal lumen for decompression. There was subclinical leakage at the esophagogastric anastomosis and that was treated conservatively. He underwent esophagectomy and decortication of the right lung 1 month later. The operation was difficult because of the dense adhesions in the right pleural cavity as a result of esophageal perforation and empyema thoracis. Postoperative course was uneventful. He was discharged home 3 weeks after the esophageal exclusion. The resected segment of esophagus measured 15 cm (length) × 6 cm (width). A 5 cm (length) × 3.5 cm (width) mucosal defect was noted in the middle third of esophagus (Fig. 7). Microscopically deep fissuring ulcers lined by inflamed granulation tissue were noted particularly in the lower third of the esophagus. The ulcers involved the muscularis propria layer. There was no evidence of viral infection or malignancy.
DISCUSSION IED is generally regarded as a benign condition that usually can be treated conservatively. IED is an intramural pathology most of the time; however, it can result in esophageal perforation. For IED patients without esophageal perforation, intravenous fluid, antiemetic and proton pump inhibitor with close monitoring were probably adequate treatments. When there is perforation, the treatment should follow the principle of esophageal perforation management. For hemodynamically stable patients, antibiotics, and chest drain insertion may be considered whereas for those with unstable cardiopulmonary vital signs and evidence of uncontrolled sepsis, surgery is essential for life saving. By far, there are few reports of IED requiring surgical treatment. Our patient had spontaneous IED but presented with atypical symptoms. The atypical presentation might cause a delay in diagnosis. For patients presented with typical symptoms including repeated vomiting, a recent history of foreign body ingestion or upper gastrointestinal tract instrumentation, a high index of suspicion can help in prompt diagnosis. Contrast swallow studies and CT scan with oral and intravenous contrasts are the preferred initial investigations.13 Contrast swallow study showing a double-barreled esophagus or a mucosal stripe sign is diagnostic of IED.5 It also allows the assessment of any associated esophageal perforation. There are different degrees of esophageal perforation as suggested by Skinner and Belsey,14 ranging from air leakage to contained perforation, free mediastinal leakage and free leakage with extension into the pleural space, which was seen in our patient. A concentric or eccentric esophageal wall thickening appeared in CT scan could represent submucosal hematoma in IED.15 There is no consensus on the treatment of IED with perforation. Endoscopic treatment options of esophageal perforation include the placement of self-expandable plastic stent (SEPS) and the use of endoscopic clips. While the use of SEPS is generally accepted as one of the treatment options in selected patients with Boerhaave’s syndrome,16–18 there is little evidence on the use of SEPS in the treatment of IED. Buscaglia reported the placement of SEPS in a case of iatrogenic IED occurring after stricture dilatation.19 However, in his report patient also underwent thoracotomy and lung decortication. Endoscopic treatment of spontaneous IED has been reported in some patients.20,21 Gluck reported the use of endoscopic dilatation of the esophageal stricture in two IED patients after spontaneous healing had occurred,20 while Bak reported endoscopic incision of the septum between true and false lumens using a needle-type diathermy knife.21 There has been only one report on successful endoscopic treatment in © 2014 International Society for Diseases of the Esophagus
Spontaneous circumferential IED
(A)
(C)
(E)
(B)
(D)
(F)
5
(G)
Fig. 4 (A–G) Computed tomography showing circumferential wall thickening of esophagus with paraesophageal air pockets (black arrows) distal to esophageal stent tracking to right posterior pleura with pleural collection noted suggesting presence of esophageal-pleural fistula.
circumferential spontaneous IED.22 Kim reported a case of successful endoscopic treatment for circumferential spontaneous IED by incision of the septum between the false and true lumens, transaction of the true esophageal wall, balloon dilatation, and metal stent insertion in a patient with underlying liver cirrhosis.22 One should note that esophageal perfora© 2014 International Society for Diseases of the Esophagus
tion did not occur in that reported case and the aim of stent insertion was to relieve dysphagia. Metal stent insertion was performed 16 months after the initial presentation. Endoscopic clips maybe used for the closure of perforations and fistulas. A device named Over-the-Scope-Clip (OTSC) (Ovesco Endoscopy AG, Tübingen, Germany) is designed for full
6
(A)
Diseases of the Esophagus
(B)
(C)
(D)
Fig. 5 (A,B) Oesophagogastroduodenoscopy showing esophageal stent. (C) Ulcerative area approximately 1 cm distal to stent. (D) Tight stricture at 37 cm.
thickness closure of perforations. The system consists of a nitinol alloy clip that is mounted on a transparent cap fitted to the tip of an endoscope. The clip is deployed when the edges of the perforation site are engaged within the cap. The concept is similar to the endoscopic band-ligation system for esophageal varices. Preliminary clinical data on OTSC closure of esophageal perforations and fistulas are encouraging. A multicenter European cohort study reported the
Fig. 6 Gastrografin swallow showing leakage at right lower esophagus, 5 cm inferior to stent (black arrow) with multiple filling out-pouchings.
result of OTSC in 36 patients with iatrogenic perforations of the gastrointestinal tract
