CASE REPORT
A Bleeding Esophagopulmonary Fistula: Rare Complication of Stasis Ulcer in Refractory Achalasia Jeremy T.H. Tan, FRACS,* Nagendra N. Dudi-Venkata, MBBS,w Shelbin I. Neelankavil, MS,* and Lucas H.A. Sanders, FRACS, FCS (SA)zy
Abstract: Achalasia refractory to Heller cardiomyotomy is a rare problem. Complications arising from long-standing achalasia include perforation because of stasis ulcers and malignancy. Here, we report a case of refractory achalasia presenting with recurrent hematemesis because of a perforated stasis ulcer leading to a bleeding esophagopulmonary fistula. Key Words: achalasia, stasis ulcer, esphagopulmonary fistula, cardiomyotomy, esophagectomy
(Surg Laparosc Endosc Percutan Tech 2014;24:e77–e79)
A
chalasia is a rare condition caused by a progressive neuronal degeneration in the mesenteric plexus of Auerbach, resulting in a nonrelaxing, hypertensive lower esophageal sphincter and aperistalsis of the body of the esophagus. Patients mostly present with complaints of progressive dysphagia, both to solids and liquids, and regurgitation. Achalasia can be treated by recurrent endoscopic pneumatic dilatations, endoscopic botulinum toxin (botox) injections, or surgical myotomy, either laparoscopically or by open Heller cardiomyotomy. In refractory cases, esophagectomy has also been described. Complications of long-standing achalasia include lung abscess, pneumonia, stasis ulcer, and malignancy. Here, we report a case of a 47-year-old man with refractory achalasia who had recurrent hematemesis because of a perforated stasis ulcer due to a rare, and previously undescribed, complication of the esophagopulmonary fistula.
The patient underwent an uneventful laparoscopic myotomy 5 cm proximal and 2 cm distal to the gastroesophageal junction (GOJ). A posterior 270-degree fundoplication was also carried out to reduce gastroesophageal reflux. Postoperative swallow performed on day 3, however, still showed very sluggish emptying of the distal esophagus, in addition to the known sigmoid megaesophagus (Fig. 1). The patient subsequently tolerated a nourishing fluid diet and thus was discharged with a plan to assess esophageal emptying over the course of next few weeks with an expected improvement in swallowing because of the reduction in postoperative swelling at the GOJ. However, the patient presented to the emergency department of our hospital 4 weeks postoperatively with an episode of hematemesis. After commencing intravenous proton-pump inhibitor infusion, an upper gastrointestinal endoscopy was performed. It demonstrated a 2 cm ulcer in the distal esophagus about 3 cm proximal to the GOJ that was thought to be a stasis/stercoral ulcer. As there was no active bleeding, it was decided not to treat it endoscopically at that time. Mild resistance was encountered on further advancement of the endoscope into the stomach. The next evening, he had another large hematemesis and was taken for an emergency endoscopy where the ulcer was again visualized, with some bleeding at the edges. It was treated with an adrenaline injection and gold-probe diathermy with good immediate effect. He was admitted to the intensive care unit (ICU), intubated and ventilated postprocedure to prevent aspiration. Two days later, while still sedated and ventilated in ICU, he had another large hematemesis. Computed tomography angiogram
CASE REPORT A 47-year-old man was referred to us with recurrent achalasia. He had undergone a cardiomyotomy through a left thoracotomy 30 years ago, but over the past 10 years he presented biennially for the dilatation of his lower esophagus. He tended to present late with his esophagus full of food, requiring several days of fasting before a successful endoscopic dilatation could be performed. After due consideration of his history and presenting complaints, discussion with his gastroenterologist and the patient, it was felt that a laparoscopic Heller cardiomyotomy should be offered as an option. Received for publication October 30, 2012; accepted December 10, 2012. From the *Department of General Surgery, Sir Charles Gairdner Hospital, Hospital Ave, Nedlands; zDepartment of Cardiothoracic Surgery, Lung Institute of Western Australia, Sir Charles Gairdner Hospital, Hospital Ave, Nedlands; wDepartment of General Surgery, St. John of God Hospital, Subiaco; and ySchool of Surgery, University of Western Australia, Crawley, WA. The authors declare no conflicts of interest. Reprints: Nagendra N. Dudi-Venkata, MBBS, Department of General Surgery, St. John of God Hospital, 12 Salvado Road, Subiaco, Perth, Western Australia - 6008 (e-mail: [email protected]). Copyright r 2014 by Lippincott Williams & Wilkins
Surg Laparosc Endosc Percutan Tech
FIGURE 1. Contrast swallow showing gastroesophageal junction after “failed” laparoscopic Heller cardiomyotomy.
Volume 24, Number 2, April 2014
www.surgical-laparoscopy.com |
e77
Tan et al
Surg Laparosc Endosc Percutan Tech
FIGURE 2. Chest x-ray during the time of combined hematemesis and hemoptysis. was done but it did not reveal any site of active bleeding. A repeat urgent endoscopy showed an intermittently bleeding Dieulafoytype lesion between the GOJ and the ulcer. This was clipped endoscopically. At about this time, blood was seen on endotracheal tube (ETT) suctioning as well. It was assumed that some blood had spilled around the cuff of the ETT. Chest x-ray after procedure revealed collapse/consolidation of the left lower lobe consistent with aspiration (Fig. 2). The patient remained stable over the next 3 days but on the fourth day, he again had a large hematemesis, and his hemoglobin fell to 73 g/L with even more blood coming out of his ETT. Given
Volume 24, Number 2, April 2014
that he had failed 2 endoscopic attempts at treatment and a negative computed tomography angiogram, a decision was made to perform a distal esophagectomy through a left thoracoabdominal incision. On operating table, upper gastrointestinal endoscopy showed a markedly dilated esophagus full of blood and clot. The ulcer could not be visualized. With the patient in a supine position, a left thoracoabdominal incision was made through the seventh left intercostal space. The medial border of the left lung was found to be tightly adherent to the left lateral surface of the distal esophagus. As the lung was being dissected off the esophagus, a perforation was found from the esophagus to the left lower lobe of the lung at the site of the distal esophageal ulcer. The stomach and the GOJ were mobilized, preserving the right gastroepiploic vessels; the duodenum was kocherized and a pyloroplasty performed. The distal esophagus could only be mobilized comfortably to the level of the left inferior pulmonary vein because of the density of the adhesions. This point was about 3 cm proximal to the ulcer perforation site. The stomach was tubularized with a linear cutter and the distal esophagus was transected as proximally as possible. A handsewn, interrupted, end-to-side esophagogastrostomy was then performed, ensuring a wide anastomosis between the esophagus and the stomach. The defect in the lung initially showed an air leak and had bleeding edges, but both of these settled down spontaneously by the end of the operation and did not require any suturing or stapling for control. A feeding jejunostomy was inserted, the chest and the abdominal cavities were drained, and the incision was closed. The patient went back to ICU with the double-lumen ETT in situ, which was changed to a single-lumen ETT the following day. There was no further bleeding from his esophagus or lung thereafter. He made a slow recovery and required a tracheostomy tube as a part of the process but was able to be discharged to the ward after a total of 14 days in the ICU. In the ward, he progressed to decannulation and was tolerating a puree diet at the time of discharge. A contrast swallow before commencement of the fluids
FIGURE 3. Contrast swallow following distal esophagectomy.
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2014 Lippincott Williams & Wilkins
Surg Laparosc Endosc Percutan Tech
Volume 24, Number 2, April 2014
showed free flow of contrast into the stomach and small bowel, without any evidence of leak (Fig. 3). Histopathology of the resected specimen showed an area of ulceration wherein the mucosa was replaced by inflamed granulation tissue. An area of transmural necrosis was also present, in keeping with focal perforation. There were dilated vessels in the wall. There was no dysplasia or malignancy. At review in the outpatient clinic 6 weeks postoperatively, he was tolerating normal food and had gained 12 kg in weight. His bowels were working normally and there were minimal reflux symptoms.
DISCUSSION Achalasia cardia can be a long-standing condition and at times could be refractory to various modalities of conservative treatment including laparoscopic myotomy. Some of the causes of ulceration in achalasia include stasis ulcers, pill esophagitis, and malignancies.1 There is only 1 other case report in the literature of perforation of a stasis ulcer in achalasia where the patient had a free perforation into the mediastinum and she succumbed to pneumonia despite operative intervention.2 Our case is unusual because, due to the previous left thoracotomy, the lung was adherent to the esophagus and thus a possible free perforation ended up being an esophagopulmonary fistula. Bleeding from the ulcer thus led to the blood coming up orally and from the ETT and showed a consolidated appearance of the left lower lobe on radiologic imaging (Fig. 2). Moreover, in our patient, there was no evidence of malignancy. In regards to the distal esophageal resections, especially for distal esophageal adenocarcinomas, they are nowadays performed by an Ivor Lewis (Lewis-Tanner) 2stage laparotomy and right thoracotomy approach. However, in this case, because of the marked consolidation of
r
2014 Lippincott Williams & Wilkins
A Bleeding Esophagopulmonary Fistula
the left lung, it was felt that the patient would not have tolerated a single left lung ventilation if a right thoracotomy was going to be performed. It was thus performed through a left thoracoabdominal approach, which also turned out to be better for dissecting the adherent left lung off the esophagus. The patient tolerated single right lung ventilation very well during the operation. The question of how much esophagus needs to be resected in cases of refractory achalasia has also been studied. Miller et al3 showed that functional outcome is similar for cervical versus thoracic anastomoses after esophageal resection for achalasia. We were only able to resect the distal 8 cm of esophagus in our patient, but so far he has had reasonable food tolerance. He has been told to sit up for 30 minutes after each meal to allow food to drain by gravity. In conclusion, in the modern laparoscopic era, refractory achalasia following Heller cardiomyotomy is a rare condition. This case also demonstrates a rare and serious complication of the long-standing achalasia after repair. Recognition of this condition and urgent aggressive treatment with consideration of an esophagectomy is pivotal to prevent further complications of the disease.
REFERENCES 1. Kikendall JW. Pill esophagitis. J Clin Gastroenterol. 1999; 28:298–305. 2. Banait VS, Sandur V, Murugesh M, et al. Spontaneous esophageal perforation in a patient with achalasia cardia and rheumatoid arthritis. Indian J Gastroenterol. 2006;25:160–161. 3. Miller DL, Allen MS, Trastek VF, et al. Esophageal resection for recurrent achalasia. Ann Thorac Surg. 1995;60:922–926.
www.surgical-laparoscopy.com |
e79
A Bleeding Esophagopulmonary Fistula: Rare Complication of Stasis Ulcer in Refractory Achalasia Jeremy T.H. Tan, FRACS,* Nagendra N. Dudi-Venkata, MBBS,w Shelbin I. Neelankavil, MS,* and Lucas H.A. Sanders, FRACS, FCS (SA)zy
Abstract: Achalasia refractory to Heller cardiomyotomy is a rare problem. Complications arising from long-standing achalasia include perforation because of stasis ulcers and malignancy. Here, we report a case of refractory achalasia presenting with recurrent hematemesis because of a perforated stasis ulcer leading to a bleeding esophagopulmonary fistula. Key Words: achalasia, stasis ulcer, esphagopulmonary fistula, cardiomyotomy, esophagectomy
(Surg Laparosc Endosc Percutan Tech 2014;24:e77–e79)
A
chalasia is a rare condition caused by a progressive neuronal degeneration in the mesenteric plexus of Auerbach, resulting in a nonrelaxing, hypertensive lower esophageal sphincter and aperistalsis of the body of the esophagus. Patients mostly present with complaints of progressive dysphagia, both to solids and liquids, and regurgitation. Achalasia can be treated by recurrent endoscopic pneumatic dilatations, endoscopic botulinum toxin (botox) injections, or surgical myotomy, either laparoscopically or by open Heller cardiomyotomy. In refractory cases, esophagectomy has also been described. Complications of long-standing achalasia include lung abscess, pneumonia, stasis ulcer, and malignancy. Here, we report a case of a 47-year-old man with refractory achalasia who had recurrent hematemesis because of a perforated stasis ulcer due to a rare, and previously undescribed, complication of the esophagopulmonary fistula.
The patient underwent an uneventful laparoscopic myotomy 5 cm proximal and 2 cm distal to the gastroesophageal junction (GOJ). A posterior 270-degree fundoplication was also carried out to reduce gastroesophageal reflux. Postoperative swallow performed on day 3, however, still showed very sluggish emptying of the distal esophagus, in addition to the known sigmoid megaesophagus (Fig. 1). The patient subsequently tolerated a nourishing fluid diet and thus was discharged with a plan to assess esophageal emptying over the course of next few weeks with an expected improvement in swallowing because of the reduction in postoperative swelling at the GOJ. However, the patient presented to the emergency department of our hospital 4 weeks postoperatively with an episode of hematemesis. After commencing intravenous proton-pump inhibitor infusion, an upper gastrointestinal endoscopy was performed. It demonstrated a 2 cm ulcer in the distal esophagus about 3 cm proximal to the GOJ that was thought to be a stasis/stercoral ulcer. As there was no active bleeding, it was decided not to treat it endoscopically at that time. Mild resistance was encountered on further advancement of the endoscope into the stomach. The next evening, he had another large hematemesis and was taken for an emergency endoscopy where the ulcer was again visualized, with some bleeding at the edges. It was treated with an adrenaline injection and gold-probe diathermy with good immediate effect. He was admitted to the intensive care unit (ICU), intubated and ventilated postprocedure to prevent aspiration. Two days later, while still sedated and ventilated in ICU, he had another large hematemesis. Computed tomography angiogram
CASE REPORT A 47-year-old man was referred to us with recurrent achalasia. He had undergone a cardiomyotomy through a left thoracotomy 30 years ago, but over the past 10 years he presented biennially for the dilatation of his lower esophagus. He tended to present late with his esophagus full of food, requiring several days of fasting before a successful endoscopic dilatation could be performed. After due consideration of his history and presenting complaints, discussion with his gastroenterologist and the patient, it was felt that a laparoscopic Heller cardiomyotomy should be offered as an option. Received for publication October 30, 2012; accepted December 10, 2012. From the *Department of General Surgery, Sir Charles Gairdner Hospital, Hospital Ave, Nedlands; zDepartment of Cardiothoracic Surgery, Lung Institute of Western Australia, Sir Charles Gairdner Hospital, Hospital Ave, Nedlands; wDepartment of General Surgery, St. John of God Hospital, Subiaco; and ySchool of Surgery, University of Western Australia, Crawley, WA. The authors declare no conflicts of interest. Reprints: Nagendra N. Dudi-Venkata, MBBS, Department of General Surgery, St. John of God Hospital, 12 Salvado Road, Subiaco, Perth, Western Australia - 6008 (e-mail: [email protected]). Copyright r 2014 by Lippincott Williams & Wilkins
Surg Laparosc Endosc Percutan Tech
FIGURE 1. Contrast swallow showing gastroesophageal junction after “failed” laparoscopic Heller cardiomyotomy.
Volume 24, Number 2, April 2014
www.surgical-laparoscopy.com |
e77
Tan et al
Surg Laparosc Endosc Percutan Tech
FIGURE 2. Chest x-ray during the time of combined hematemesis and hemoptysis. was done but it did not reveal any site of active bleeding. A repeat urgent endoscopy showed an intermittently bleeding Dieulafoytype lesion between the GOJ and the ulcer. This was clipped endoscopically. At about this time, blood was seen on endotracheal tube (ETT) suctioning as well. It was assumed that some blood had spilled around the cuff of the ETT. Chest x-ray after procedure revealed collapse/consolidation of the left lower lobe consistent with aspiration (Fig. 2). The patient remained stable over the next 3 days but on the fourth day, he again had a large hematemesis, and his hemoglobin fell to 73 g/L with even more blood coming out of his ETT. Given
Volume 24, Number 2, April 2014
that he had failed 2 endoscopic attempts at treatment and a negative computed tomography angiogram, a decision was made to perform a distal esophagectomy through a left thoracoabdominal incision. On operating table, upper gastrointestinal endoscopy showed a markedly dilated esophagus full of blood and clot. The ulcer could not be visualized. With the patient in a supine position, a left thoracoabdominal incision was made through the seventh left intercostal space. The medial border of the left lung was found to be tightly adherent to the left lateral surface of the distal esophagus. As the lung was being dissected off the esophagus, a perforation was found from the esophagus to the left lower lobe of the lung at the site of the distal esophageal ulcer. The stomach and the GOJ were mobilized, preserving the right gastroepiploic vessels; the duodenum was kocherized and a pyloroplasty performed. The distal esophagus could only be mobilized comfortably to the level of the left inferior pulmonary vein because of the density of the adhesions. This point was about 3 cm proximal to the ulcer perforation site. The stomach was tubularized with a linear cutter and the distal esophagus was transected as proximally as possible. A handsewn, interrupted, end-to-side esophagogastrostomy was then performed, ensuring a wide anastomosis between the esophagus and the stomach. The defect in the lung initially showed an air leak and had bleeding edges, but both of these settled down spontaneously by the end of the operation and did not require any suturing or stapling for control. A feeding jejunostomy was inserted, the chest and the abdominal cavities were drained, and the incision was closed. The patient went back to ICU with the double-lumen ETT in situ, which was changed to a single-lumen ETT the following day. There was no further bleeding from his esophagus or lung thereafter. He made a slow recovery and required a tracheostomy tube as a part of the process but was able to be discharged to the ward after a total of 14 days in the ICU. In the ward, he progressed to decannulation and was tolerating a puree diet at the time of discharge. A contrast swallow before commencement of the fluids
FIGURE 3. Contrast swallow following distal esophagectomy.
e78 | www.surgical-laparoscopy.com
r
2014 Lippincott Williams & Wilkins
Surg Laparosc Endosc Percutan Tech
Volume 24, Number 2, April 2014
showed free flow of contrast into the stomach and small bowel, without any evidence of leak (Fig. 3). Histopathology of the resected specimen showed an area of ulceration wherein the mucosa was replaced by inflamed granulation tissue. An area of transmural necrosis was also present, in keeping with focal perforation. There were dilated vessels in the wall. There was no dysplasia or malignancy. At review in the outpatient clinic 6 weeks postoperatively, he was tolerating normal food and had gained 12 kg in weight. His bowels were working normally and there were minimal reflux symptoms.
DISCUSSION Achalasia cardia can be a long-standing condition and at times could be refractory to various modalities of conservative treatment including laparoscopic myotomy. Some of the causes of ulceration in achalasia include stasis ulcers, pill esophagitis, and malignancies.1 There is only 1 other case report in the literature of perforation of a stasis ulcer in achalasia where the patient had a free perforation into the mediastinum and she succumbed to pneumonia despite operative intervention.2 Our case is unusual because, due to the previous left thoracotomy, the lung was adherent to the esophagus and thus a possible free perforation ended up being an esophagopulmonary fistula. Bleeding from the ulcer thus led to the blood coming up orally and from the ETT and showed a consolidated appearance of the left lower lobe on radiologic imaging (Fig. 2). Moreover, in our patient, there was no evidence of malignancy. In regards to the distal esophageal resections, especially for distal esophageal adenocarcinomas, they are nowadays performed by an Ivor Lewis (Lewis-Tanner) 2stage laparotomy and right thoracotomy approach. However, in this case, because of the marked consolidation of
r
2014 Lippincott Williams & Wilkins
A Bleeding Esophagopulmonary Fistula
the left lung, it was felt that the patient would not have tolerated a single left lung ventilation if a right thoracotomy was going to be performed. It was thus performed through a left thoracoabdominal approach, which also turned out to be better for dissecting the adherent left lung off the esophagus. The patient tolerated single right lung ventilation very well during the operation. The question of how much esophagus needs to be resected in cases of refractory achalasia has also been studied. Miller et al3 showed that functional outcome is similar for cervical versus thoracic anastomoses after esophageal resection for achalasia. We were only able to resect the distal 8 cm of esophagus in our patient, but so far he has had reasonable food tolerance. He has been told to sit up for 30 minutes after each meal to allow food to drain by gravity. In conclusion, in the modern laparoscopic era, refractory achalasia following Heller cardiomyotomy is a rare condition. This case also demonstrates a rare and serious complication of the long-standing achalasia after repair. Recognition of this condition and urgent aggressive treatment with consideration of an esophagectomy is pivotal to prevent further complications of the disease.
REFERENCES 1. Kikendall JW. Pill esophagitis. J Clin Gastroenterol. 1999; 28:298–305. 2. Banait VS, Sandur V, Murugesh M, et al. Spontaneous esophageal perforation in a patient with achalasia cardia and rheumatoid arthritis. Indian J Gastroenterol. 2006;25:160–161. 3. Miller DL, Allen MS, Trastek VF, et al. Esophageal resection for recurrent achalasia. Ann Thorac Surg. 1995;60:922–926.
www.surgical-laparoscopy.com |
e79
