Boerhaave’s syndrome presenting with hematemesis: case report
SPMC J Health Care Serv. 2019;5(2):7 ARK: http://n2t.net/ark:/76951/jhcs2n4n8f
1Department of General Surgery, Southern Philippines Medical Center, JP Laurel Ave, Davao City, Philippines
Correspondence Delbrynth P Mitchao, email@example.com
Article editor Fitzgerald Arancel
Received 18 January 2019
Accepted 25 October 2019
Cite as Mitchao DP, Pagarigan BB III, Lobo RR, Espino RS, Cruz JR. Boerhaave’s syndrome presenting with hematemesis: case report. SPMC J Health Care Serv. 2019;5(2):7. http://n2t.net/ark:/76951/jhcs2n4n8f
Boerhaave’s syndrome (BS) is a rare condition of the gastrointestinal tract characterized by a spontaneous rupture of the esophagus—mostly along the distal third—due to increased intra-esophageal pressure.1
Esophageal ruptures have an estimated worldwide incidence of only 3.1 in 1,000,000 individuals annually. Of these esophageal ruptures, 15% are spontaneous perforations.2 3
BS has a 40% mortality rate despite any surgical intervention, and a 90% mortality rate if left untreated.3 4
However, it also has a 50% rate of being misdiagnosed because of indeterminate symptoms.5
Although the Mackler triad of lower thoracic or chest pain, subcutaneous emphysema, and vomiting is pathognomonic of BS,6 7 8
less than half of cases of BS present with this combination of symptoms.9 10
Other patients with BS who do not present with this combination of symptoms are often diagnosed late or misdiagnosed with other more common diseases.11
Most patients with BS present with rather atypical symptoms that mimic cardiogenic conditions.12
Depending on the degree of gastric-content leakage, level of rupture, and time since the onset of rupture, patients would tend to present with dysphagia, dysphonia, and pain in the neck, chest, epigastric region, or upper abdomen.3
The definitive diagnosis of BS is made through computed tomography scans and esophageal contrast studies.13 14
The therapeutic management of BS usually involves surgery, and less invasive procedures are warranted only in some cases.5
The management of BS may include pleural/mediastinal decontamination or drainage, debridement or resection of necrotic esophageal tissue, repair of perforation, gastrointestinal decompression, distal enteral feeding access, or any combination of these procedures.15
BS may be managed conservatively using endoscopic placement of stents in cases where the leak is localized in the mediastinum on contrast studies.16
In cases where the patient is clinically unstable with signs of sepsis, or when there is an extension of the perforation into an adjacent body cavity, surgery is highly indicated.17
We report the case of a 46-year-old male who presented with clinical and radiographic signs of BS. The patient was initially managed with a conservative resection of the perforated esophagus 48 hours after the onset of symptoms, but eventually underwent subtotal esophagectomy and delayed esophageal reconstruction.
A 46 year-old male was referred to our institution due to persistent hematemesis. Twenty-four hours prior to admission, the patient had his first episode of vomiting of approximately 100 mL of fresh blood after drinking alcoholic beverage to the point of intoxication. Approximately eight hours prior to admission, he had a left-sided, retrosternal, non-radiating chest pain. This was associated with epigastric pain. Hematemesis persisted for around 15 more episodes, with an average of 30 mL per episode of vomitus, until admission. He went to a nearby hospital where he was diagnosed as having bleeding esophageal varices secondary to alcoholic liver disease. A chest radiograph done revealed minimal pleural effusion on the left hemithorax. He was transferred to our institution for persistent hematemesis and epigastric pain, and new-onset dyspnea.
The patient had been an alcoholic beverage drinker for approximately 30 years, consuming hard liquor almost every day, and drank to the point of intoxication on some occasions. He denied of any forceful retching or vomiting during or after his drinking episodes. He was also a smoker for 28 pack-years. He denied a history of recurrent epigastric pain or any comorbidities. His work prior to illness consisted mostly of heavy lifting in construction and carpentry.
Physical examination done on admission, 24 hours after the first episode of vomiting, revealed that the patient had elevated blood pressure at 140/100 mmHg, tachycardia at 123 beats per minute, and tachypnea at 24 cycles per minute. He had pale skin and palpebral conjunctivae. Examination of the chest and lungs revealed equal expansion, normal tactile fremitus, and clear breath sounds. Precordium was adynamic, and no abnormal heart sounds were appreciated. The abdomen was neither distended nor tender, but the bowel sounds were hypoactive. The rest of the physical examination findings were unremarkable. The patient was admitted in the Internal Medicine Department with an initial diagnosis of anemia secondary to upper gastrointestinal bleeding.
Diagnostic and therapeutic approaches
The patient had no further active vomiting upon admission. The admitting complete blood count showed anemia (98 g/L; normal range: 135 g/L to 175 g/L) and leukocytosis (19.46 x 103/µL) with predominance of neutrophils (91%). Serum calcium, magnesium and sodium levels were slightly low, while the results of the rest of the blood chemistry panel were unremarkable. Intravenous fluids, octreotide, and omeprazole were given to control the patient's symptoms.
An upper gastrointestinal endoscopy done 23 hours after admission revealed an ulcerated mass that extended 4 cm upwards from the posterior portion of the gastroesophageal junction. The mass had a necrotic base. The rest of the esophageal mucosa, the stomach, as well as the first and second parts of the duodenum, were unremarkable. At this point, the working diagnosis was ulcerated mass at the distal esophagus with extension to the gastroesophageal junction, and the patient was transferred to our service in the Surgery Department.
Our service documented new physical examination findings of crepitus on the left chest wall and decreased breath sounds on the left lower lung field. The presence of tachycardia and tachypnea, and laboratory findings of leukocytosis suggested that the patient had systemic inflammatory response syndrome. Given the patient's history and the initial course in the ward, we were already entertaining the possibility of esophageal rupture with signs of sepsis when we received the patient 30 hours after admission. We requested a repeat chest radiograph, and indeed, the findings of subcutaneous emphysema on the left cervical area and a progressing left pleural effusion confirmed our physical examination findings (Figure 1). We also did an abdominal CT scan, which revealed the presence of a distal esophageal tear with associated pneumomediastinum, and pleural effusion with compressive atelectasis on the left hemithorax (Figures 2 and 3) We treated the patient as having Boerhaave's syndrome with acute mediastinitis.
Anteroposterior chest x-ray taken 30 hours after admission, showing a meniscoid opacity in the left hemithorax obliterating the ipsilateral cardiac border, hemidiaphragm, and costophrenic sulcus. There is a subcutaneous emphysema visible in the left cervical region (A: red arrows). Lucencies characteristic of a pneumomediastinum (outlined in yellow) are seen from the lateral border of the superior vena cava, extending superiorly to the right paratracheal region, and from the left cardiac border, extending superiorly to the aortic knob, then up to the left paratracheal region (B).
Contrast CT [coronal (left column) and axial (right column) views] taken 46 hours after admission, showing the presence of subcutaneous emphysema (A: orange ring) in the left cervical area (A & B). There is an abnormal presence of air in the anterior mediastinum (D: yellow ring) and alongside the medial and lateral borders of the aortic arch (D: red arrows) (C & D). A wedge-shaped confluent opacity with air bronchograms (F: blue ring) is seen in the left lower lobe indicating the presence of a compressive atelectasis (E & F).
Contrast CT [sagittal (left column) and sequential axial (right column)] images showing a dilated esophagus and a thickened distal esophageal segment. Esophageal rupture is noted in the distal segment, showing air track extending from the esophagus into the mediastinum and full-thickness disruption of part of the esophageal wall (C, D, & E).
We started the patient on intravenous piperacillin-tazobactam and metronidazole, and we immediately performed an exploratory laparotomy via transhiatal approach. We made a midline incision from the subxiphoid level to the supraumbilical level. Intraabdominally, there were no signs of peritonitis. The stomach did not have any perforation, abscess formation or gastric content leak. The dissection to expose the esophageal hiatus was technically difficult—in the process, the splenic hilum was inadvertently injured, for which we had to do a splenectomy.
Upon exploring the distal esophagus and gastroesophageal junction, we could not find any visible or palpable tumor. Instead, we noted a perforated portion of the esophagus, extending from the gastroesophageal junction up to 4 cm proximally and involving more than 50% of the esophageal circumference. The edges of the perforation were necrotic. We also drained around 20 mL of suppurative fluid pooling at the posterior mediastinum.
We performed a cervical esophagostomy through the left side of the neck in order to divert the esophageal secretions and avoid further contamination of the mediastinum. We created a jejunostomy in order to temporarily institute enteral feeding, preserve gut function, and promote optimal wound healing. To drain the lungs and mediastinum, we placed one chest tube on the anterior mediastinum and another chest tube on the posterior mediastinum.
A conservative management approach did not seem feasible at this time, since the patient already showed signs of sepsis. We wanted to preserve as much of the normal anatomy of the esophagus as possible, so we only resected 12 cm of the upper gastrointestinal tract, starting from 5 cm above the necrotic portion of the perforated esophagus up to 3 cm below the gastroesophageal junction.(Figure 4) The branches of the vagus nerve innervating this portion of the esophagus and stomach were also resected.
Resected esophagus, measuring 12 cm in length, showing the anterior (left) and posterior (right) aspects.
We separately sealed the distal portion of the remaining esophagus and the proximal portion of the stomach with staple wires, and we decontaminated the mediastinal and pleural spaces by lavage. We then closed the hiatus with sutures to prevent herniation of the contents of the abdominal viscera into the chest.
Forty-eight hours after surgery, we did a comparative chest radiograph, which showed pulmonary congestion, further progression of pleural effusion on the left lower lung field, bilateral pneumonia, and regressing cervical subcutaneous emphysema. Despite the antibiotics and supportive management, the patient was persistently febrile and tachycardic until the 5th postoperative day. Complete blood counts (CBC) done within the 5 days following surgery revealed persistent leukocytosis (from 14.59 to 23.69 x 103/µL). We suspected that the patient had persistent mediastinitis, hence we decided to perform a repeat exploratory laparotomy.
We reopened the patient's previous midline incision. We reopened the hiatus and, upon accessing the mediastinum through the opening, we noted pus formation on the last 5 cm of the retained esophagus. We drained the pus collection at the posterior mediastinum. We performed a subtotal esophagectomy by resecting 6 cm of the retained esophagus starting from the portion at the level of the esophagostomy stoma. We then reclosed the hiatus, and then re-anchored the remaining free portion of the proximal esophagus to the skin.
Immediately after subtotal esophagectomy, the patient continued to have febrile episodes, tachypnea, and crackles on both lungs. A repeat chest radiograph still showed the pulmonary congestion that was initially present 48 hours after the first surgery and a progressing pleural effusion. We managed the patient as having acute lung injury, a common complication of esophagectomy. We continued the antibiotics, resumed incentive spirometry, and gave diuretics intermittently to help resolve the pulmonary congestion. After completion of a week's course of piperacillin-tazobactam and metronidazole, the patient was given intravenous meropenem. On the fourth day after subtotal esophagectomy, the laparotomy incision had purulent discharge. Gram-staining of the exudate revealed the presence of gram-positive cocci.
Over the next few days, the patient's fever lysed, his breathing improved, his surgical site wound started to heal, and his leukocyte levels gradually decreased from 37.26 to 14.85 x 103/µL. After the chest tubes attached to the anterior and posterior mediastinum were removed, a repeat chest radiograph done 10 days post-subtotal esophagectomy showed regression of the left pleural effusion and absence of pulmonary congestion. He was discharged two weeks after his second surgery with retained cervical esophagostomy and feeding jejunostomy, and was prescribed with oral antibiotics for one week.
The patient’s general health condition was deemed optimal for esophageal reconstruction on the fourth month after subtotal esophagectomy. For esophageal reconstruction, we initially planned to do a gastric pull-up via laparoscopic surgery. We prepared for an alternative approach--colonic interposition--should the stomach prove to be unviable for the reconstruction, hence we assessed the colon through colonoscopy prior to surgery.
Upon laparoscopy, we noted dense adhesions intra-abdominally, hence we proceeded to reopen the midline incision done on the two previous surgeries. We lysed the adhesions between the bowel and peritoneal wall, the bowel and stomach, and the stomach and liver, while carefully identifying and preserving the right gastric and right gastroepiploic arteries. We reopened the hiatus and bluntly dissected the substernal area in order to develop a cavity where the stomach could pass through from the abdomen up to the cervical area. We reaccessed the cervical area via the old esophagostomy incision in the left side of the neck and isolated the retained esophagus. We pulled the stomach up to the cervical incision through the substernal cavity and anastomosed the stomach and the remaining esophagus at the level of the incision. We anchored the stomach, now the neoesophagus, to the prevertebral fascia of the neck and performed a handsewn anastomosis between the stomach and the cervical esophagus using a single-layer, full-thickness interrupted stitch. We performed a layered closure of the neck incision. Because of the resection of the branches of the vagus nerve innervating the stomach during the first surgery, the patient may experience increase in pyloric sphincter tone and gastric stasis after the reconstructive surgery. Hence, we performed a pyloromyoplasty in order to improve gastric emptying and to prevent early postoperative complications of gastric stasis (i.e., aspiration pneumonia and gastric outlet obstruction) following substernal esophageal reconstruction. On the first day post-esophageal reconstruction, the patient had a few febrile episodes and complained of a non-productive cough. CBC results showed anemia (102 g/L) and leukocytosis (16.81 x 103/µL) with predominance of neutrophils (92%). Chest radiograph done revealed bilateral pneumonia and minimal pleural effusion on the left. After completing only six doses of intravenous cefoxitin, which was started prior to surgery, we shifted the patient to intravenous meropenem. Infection resolved on the tenth day after reconstruction, and the patient was subsequently discharged.
An upper gastrointestinal series done two weeks post-esophageal reconstruction showed an unobstructed flow of the barium solution through the neoesophagus down to the duodenum, with no evidence of unusual dilatations (Figure 5). We removed the feeding jejunostomy a month after the esophageal reconstruction surgery. Figure 6 shows a schematic representation of the patient's upper gastrointestinal tract at several stages in the therapeutic management.
Upper gastrointestinal series done two weeks post-esophageal reconstruction, showing an unobstructed flow of barium solution through the neoesophagus down to the duodenum, with no evidence of unusual dilatations.
Schematic representation of the upper gastrointestinal tract during several stages in the management of the patient. An esophageal tear was noted at the distal esophageal segment during computed tomography imaging prior to surgery, taken 46 hours after admission (A). Conservative resection of the distal esophagus and part of the stomach, and creation of a cervical esophagostomy and a feeding jejunostomy were done 56 hours after admission (B). Due to persistent mediastinitis, a subtotal esophagectomy was done four days after the first surgery (C). Four months after the subtotal esophagectomy, a delayed substernal esophageal reconstruction through esophagogastric anastomosis was done to reestablish the continuity of the gastrointestinal tract (D). Jejunostomy closure was done a month after the esophageal reconstruction.
Our patient presented with massive hematemesis—a rare occurrence in BS. However, the patient’s recent history of excessive alcoholic beverage drinking and his accompanying signs and symptoms of chest pain, tachycardia, tachypnea, and a later finding of subcutaneous emphysema, highly pointed to a diagnosis of BS. The patient underwent subtotal esophagectomy and partial gastrectomy and, after nutritional build-up, eventually had esophageal reconstruction.
The diagnosis of BS is often missed or delayed due to its rarity and its nonspecific symptoms that often mimic other less serious illnesses.
Several factors have been known to cause BS, with vomiting after excessive alcohol drinking--as in our patient--or after consuming a large meal as the most common culprit.5
In less than 5% of cases, BS may be associated with epileptic seizures, childbirth, weightlifting, abdominal trauma, compressed air injury, prolonged coughing—such as in asthma—and straining during parturition and defecation--all of which lead to increased intraluminal pressure against a closed glottis, due to failure of relaxation of the cricopharyngeus muscle, resulting in longitudinal esophageal perforation.2 3 18
There are also cases in which the rupture is associated with Barrett’s esophagus, gastroesophageal reflux disease, peptic stricture of the esophagus, paraesophageal hernia, esophageal dysmotility, or bleeding from a duodenal ulcer.6 19
Approximately 80% of BS cases occur among middle-aged males, but the syndrome can also occur among adults in the sixth or seventh decade of life.3 6 20
As in our patient, the most common site of rupture is the left posterior wall of the distal third of the esophagus, about two to three inches from the esophagogastric junction.3 5 15 21 22
This area of the esophagus has an anatomical weakness since it lacks an adjacent supporting connective tissue and has a thinner musculature.5 23
The clinical presentation of BS is non-specific but, as in our patient, the primary symptoms are chest pain and epigastric pain, often preceded by vomiting, and rarely hematemesis.20
When the esophagus ruptures, the pain is described as acute chest or lower thoracic pain, depending on the location of the rupture. This may be accompanied by epigastric pain. On physical examination, patients will have tachycardia and tachypnea. Breath sounds are diminished as pleural effusion develops, and the part of the lungs that are affected would depend on the site of esophageal perforation--middle or thoracic esophagus affects the right lung, while perforation on the distal esophagus affects the left lung. 19
There may be subcutaneous emphysema. The Mackler triad, as previously described, is pathognomonic for this disease.21
However, this triad is rare, and its absence may further delay the diagnosis. Very few cases have been reported wherein patients with BS initially present with hematemesis. Such presentation may prompt a different diagnosis and management approach as it mimics an upper gastrointestinal bleed.19 24 25
Because of the diversity of gastrointestinal and pulmonary signs and symptoms associated with BS, differentiating the syndrome from other disease entities can be clinically challenging. Mallory Weiss syndrome (MWS) is a non-transmural tear which often involves the mucosa or the submucosa of the gastroesophageal junction and cardia of the stomach, and rarely the distal esophagus, while BS is a transmural perforation that is purely esophageal in nature.6 26
Our patient initially presented with hematemesis, a most common manifestation of MWS, and extremely rare in BS.6 20
MWS may also present with melena and hematochezia. Heavy alcohol ingestion, as in our patient, is considered to be one of the most important predisposing factors for both BS and MWS.19
Another important consideration in patients presenting with hematemesis would be acute massive gastrointestinal bleeding as a complication of peptic ulcer disease. Gastrointestinal symptoms--such as epigastric pain, hematemesis and melena—are commonly present in perforated peptic ulcers.27
The epigastric pain--usually after an episode of retching and vomiting—may be severe and sharp, and may become generalized.27 28 29
As in those with perforated peptic ulcers, our patient initially complained of hematemesis. However, our patient’s epigastric pain was mild, and melena was absent. In contrast, BS usually results from retching and vomiting due to excessive alcoholic beverage drinking, while perforated peptic ulcers stem from a long-standing history of smoking and chronic use of non-steroidal anti-inflammatory drugs.28
Acute bleeding of esophageal and/or gastric varices, a fatal complication of liver cirrhosis, presents as hematemesis and melena. In cases of advanced liver cirrhosis, patients may also manifest with ascites, jaundice, and hematochezia.30
Alcohol abuse is one of the main risk factors of bleeding esophageal and gastric varices.31
Our patient is a chronic alcoholic beverage drinker who presented with hematemesis, but also complained of chest pain and epigastric pain; together, these symptoms are not commonly present in patients with acute variceal bleeding, but may be consistent with BS.
Blood tests are of little significance in the diagnosis of BS, except in ruling out other diseases (e.g., myocardial infarction, pancreatitis).1
Diagnostic imaging is highly important in identifying BS. This includes chest radiographs, chest CT scan and esophagography. The gold standard for the diagnosis of BS is the demonstration of perforation in an esophagogram using gastrografin as contrast agent.32 33
Chest radiographs and CT scan commonly show mediastinal and subcutaneous emphysema, pleural effusion, and pneumothorax. In 20% of the cases, the Naclerio V sign--a V-shaped air collection outlining the medial portion of the left hemidiaphragm and the left lower lateral mediastinal border--can also be seen on chest x-rays.34 35
These findings vary according to the location of the rupture. The pathognomonic radiographic findings usually appear later in the disease process,5
and incorrect timing of chest radiographs may lead to false negative results and delays in the diagnosis, such as in the case of our patient. An early chest radiograph of our patient showed a non-specific, minimal left pleural effusion. With disease progression, a repeat chest radiograph showed a progressing pleural effusion and new-onset subcutaneous emphysema, and an abdominal CT scan revealed a pneumomediastinum, confirming the diagnosis of BS.
Although treatment approach is closely related to the patient’s general condition and status, time interval from the onset of symptoms until diagnosis, size and location (thoracic, abdominal, or both) of the rupture, and extent of chest contamination, there is no consensus regarding the definitive treatment of BS.5 36
The ultimate goals of treatment are: sepsis control (adequate pleural/mediastinal drainage and antibiotic treatment) and perforation repair or reestablishment of the continuity of the gastrointestinal tract.5
There are three possible approaches to the management of BS: conservative, endoscopic, and surgical.1
Conservative management employs restricting oral intake, placing a nasogastric tube for drainage, performing thoracostomy—if needed—and administering intravenous fluids, broad spectrum antibiotics, and hyperalimentation.37
Conservative treatment can be provided to non-septic patients with small esophageal perforations.1
Endoscopic management of BS is useful in selected patients without evidence of systemic sepsis who are diagnosed early (less than 24 hours from the onset of symptoms), with smaller perforation defects, and with less than 50% involvement of the esophageal circumference.38
Endoluminal therapy for BS and subsequent esophageal leaks with the use of clips, sutures, and/or stents—with endoluminal closure of the perforation—has been successful as primary therapy for BS in several studies. 39 40 41
This patient was referred to our service more than 24 hours from the onset of symptoms, presenting with signs of mediastinitis and systemic sepsis, and had CT scan findings of a distal esophageal tear, pneumomediastinum, and pleural effusion. These findings require a surgical approach to the management of BS. Surgical management ranges from a less invasive thoracoscopic/laparoscopic debridement and mediastinal/pleural drainage to an open surgery with extensive resection and reconstruction of the esophagus.42
Primary esophageal repair with fundic reinforcement—the gold standard in the management of BS36
—should be initially considered if patients are treated within 24 hours and if the esophageal tissues are still viable.6
The approach is usually through thoracotomy with or without phrenolaparotomy.36
In perforations that are located in the distal esophagus, a transhiatal approach through a midline laparotomy is often indicated.37
For cases of delayed diagnosis (>24 hours), as in our patient, the vitality of the esophageal wall and the surrounding tissues are compromised, hence primary repair is not possible.6 36
The most common procedure that serves as an alternative to primary repair involves esophageal exclusion and diversion and/or esophagectomy with immediate or delayed reconstruction.43
Exclusion and diversion is a major consideration in patients who have lost more than 50% of the esophageal wall circumference, and who are unfit for surgery.38 44
Exclusion and diversion includes closure of the perforation by esophagogastric junction stapling, debridement and drainage, cervical esophagostomy (diversion of the cervical esophagus and creating a salivary fistula), and gastric decompression with a gastrostomy or jejunostomy.43 44
However, this option has been used less frequently due to the need for a second surgery and the difficulty in reestablishing the gastrointestinal tract continuity after the initial surgery. Esophagectomy is indicated in cases of widely necrotic esophagus. It has the advantage of eliminating the entire source of infection and inflammation, and of reestablishing the gastrointestinal tract continuity.36 43
Our patient underwent subtotal esophagectomy. Along with appropriate antibiotic coverage, this approach resulted in the remission of mediastinitis. The decision to perform a single-stage or delayed reconstruction of gastrointestinal tract continuity after esophagectomy must be made based on considerable judgment and expertise of the surgeon.43 45
Delayed reconstruction is advised in the setting of extensive mediastinal contamination, severe sepsis, and delayed diagnosis and treatment of BS, where patients may not be fit to tolerate the additional operative time in esophageal reconstruction.43 45
We decided to take this approach for our patient.
The reestablishment of the continuity of the gastrointestinal tract is a significant factor in the quality of life of patients after esophagectomy. Esophageal replacement may come from the stomach, the right colon, the left colon, or the jejunum.46
In the esophageal reconstruction that we performed, our patient’s stomach served as the neoesophagus. The stomach is the first and most reliable choice for esophageal reconstruction due to its robust blood supply and its proximity to the esophagus, and this approach only requires a single anastomosis to establish the gastrointestinal tract continuity.47
The use of the colon or the jejunum for interposition after esophagectomy is often a salvage procedure, that is, it is utilized only when the stomach is unavailable to serve as a conduit.46 48 49
Colonic or jejunal interposition requires multiple anastomoses making it time-consuming and extra challenging.50 51
For cases wherein the posterior mediastinum is obliterated due to a previous esophagectomy and esophageal diversion, the preferred approach is a substernal reconstruction (SR). SR is often used for delayed reconstruction, while the posterior mediastinal (PM) route is utilized for immediate reconstruction after esophagectomy.52 53
Compared to PM, SR has an advantage of providing easier access to postoperative strictures. SR also allows a better, tension-free anastomosis in cases where conduit length is shorter, as in the use of the stomach in our patient for reconstruction.52 54
When BS is diagnosed early, and when treatment is started within 24 hours from the onset of symptoms, mortality rate is less than 10%.55
In a study done in two centers in Europe involving 38 patients with BS, one death was observed among 22 patients (4.54%) with early diagnosis (<24 hours), whereas 3 fatalities were recorded among 16 patients (18.75%) with late diagnosis (>24 hours). The fatal outcomes were observed in 4 out of 25 septic patients (16%).55
Our patient underwent surgery about 48 hours after the onset of vomiting, The patient’s febrile episodes did not improve after the first surgery due to persistent mediastinitis, despite good compliance to antibiotics. The patient’s condition gradually improved only after the subtotal esophagectomy and the antibiotic shift.
In summary, we saw a middle-aged male who initially presented with hematemesis and was eventually diagnosed as having BS. He subsequently underwent subtotal esophagectomy and partial stomach resection. After nutritional build-up, our patient had esophageal reconstruction and anastomosis using a gastric pull-up approach. For patients diagnosed with BS, treatment should comprise sepsis control, repair of any perforation or resection of the affected esophageal segment, and reestablishment of the gastrointestinal tract continuity.
DPM, BBP, RRL and JRC contributed to the diagnostic and therapeutic care of the patient in this report. All authors acquired relevant patient data, and searched for and reviewed relevant medical literature used in this report. All authors wrote the original draft, performed the subsequent revisions, approved the final version, and agreed to be accountable for all aspects of this report.
We would like to thank Dr Michael N Jamiana, Dr Mark Edward Anthony M Maruya, and the residents and consultants of the Southern Philippines Medical Center (SPMC) Department of Surgery who helped us in the diagnostic and therapeutic management of the patient in this case report. We also thank the residents and consultants from the Department of Internal Medicine, Department of Anesthesiology, Department of Radiology, and Department of Pathology in SPMC who gave their inputs on the contents of this paper. We would also like to thank Dr Maria Theresa T Sanchez and Dr James II G Casuga of the Department of Radiology and Imaging Services in SPMC for their insights and assistance in obtaining the high-definition radiographic images used in this report.
Access and license
This is an Open Access article licensed under the Creative Commons
4.0 International License, which allows
others to share and adapt the work, provided that derivative works
bear appropriate citation to this original work and are not used for
commercial purposes. To view a copy of this license, visit
1. Spapen J, De Regt J, Nieboer K, Verfaillie G, Honoré PM, Spapen H. Boerhaave's Syndrome: Still a Diagnostic and Therapeutic Challenge in the 21st Century. Case Rep Crit Care. 2013;2013:161286.
2. Triadafilopoulos G. Boerhaave syndrome: effort rupture of the esophagus. 2018 Feb 21 [cited 2019 Dec 13]. In: UpToDate [Internet]. Illinois: UpToDate, Inc. c2019. Available from: https://www.uptodate.com/contents/boerhaave-syndrome-effort-rupture-of-the-esophagus#H111872919.
3. Turner AR, Turner SD. Boerhaave Syndrome [Updated 2019 Nov 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan [cited 2019 Dec 13]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430808/.
4. Connelly CL, Lamb PJ, Paterson-Brown S. Outcomes following Boerhaave's syndrome. Ann R Coll Surg Engl. 2013 Nov;95(8):557-60.
5. Han D, Huang Z, Xiang Z, Li H, Hang J. The role of operation in the treatment of Boerhaave’s syndrome. Biomed Res Int. 2018 Jun 28;2018.
6. Roy PK. Boerhaave Syndrome [Internet]. Medscape. 2018 Dec 6 [cited 2019 Dec 13]. Available from: https://emedicine.medscape.com/article/171683-overview.
7. Henderson J. What is the frequency of atypical presentations of Boerhaave’s syndrome? 1998 May [cited 2019 Dec 16]. In: Giuli R, Galmiche JP, Jamieson GG, Scarpignato C. The esophagogastric junction [Internet]. OESO Foundation. C2015. Available from: http://www.oeso.org/OESO/books/Vol_5_Eso_Junction/Articles/art431.html.
8. Nagji AS, Lau CL. Clinical Scenarios in Surgery: Decision Making and Operative Technique (Clinical Scenarios in Surgery Series), 1 Ed 9 Chapter 71. Esophageal Perforation. [Internet]. Available from: https://doctorlib.info/surgery/clinical-scenarios-surgery/71.html.
9. Loop FD, Groves LK. Esophageal perforations (collective review). Ann Thorac Surg 1970;10:571-87.
10. Pate JW, Walker WA, Cole FH Jr, Owen EW, Johnson WH. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg; 1989:47:689-92.
11. Wei L, Wang F, Chen S. A late diagnosed case of Spontaneous esophageal perforation in an elderly patient. Int J Clin Exp Med. 2015 Jul 15;8(7):11594-7.
12. Lieu MT, Layoun ME, Dai D, Soo Hoo GW, Betancourt J. Tension hydropneumothorax as the initial presentation of Boerhaave syndrome. Respir Med Case Rep. 2018 Jul 31;25:100-3.
13. Velasco Hernandez DN, Horiuchi HR, Rivaletto LA, Farina F, Viscuso M. Boerhaave’s syndrome with late presentation. Experience in an Argentine single center: case series. Ann Med Surg (Lond). 2019 Jul 13; 45:59-61.
14. Katzka DA. Esophageal disorders cause by medication, trauma, and infection. In: Feldan M, Friedman LS, Brandt LJ. Sleisenger and Fordtran’s gastrointestinal and liver disease. 10th ed. Philadelphia: Saunders, an imprint of Elsevier Inc.; 2016.
15. Sutcliffe RP, Forshaw MJ, Datta G, Rohatgi A, Strauss DC, Mason RC, Botha AJ. Surgical management of Boerhaave's syndrome in a tertiary oesophagogastric centre. Ann R Coll Surg Engl. 2009 Jul;91(5):374-80.
16. Munro A. What are the indications for conservative management of spontaneous esophageal perforations? In such cases, what is the best choice for nutritional support? 1998 May [cited 2019 Dec 16]. In: Giuli R, Galmiche JP, Jamieson GG, Scarpignato C. The esophagogastric junction [Internet]. OESO Foundation. C2015. Available from: http://www.oeso.org/OESO/books/Vol_5_Eso_Junction/Articles/art433.html
17.Mueller DK. Esophageal rupture treatment and management [Internet]. Medscape. 2019 Apr 9 [cited 2019 Dec 13]. Available from: https://emedicine.medscape.com/article/425410-treatmen.t.
18. Hecser L, Siklodi KP, Csiki G, Lungu MF, Jung H, Buda O. Boerhaave syndrome. A case report. Rom J Leg Med. 2011;19(4):283-6.
19. Dinic BR, Ilic G, Rajkovic ST, Stoimenov TJ. Boerhaave syndrome - case report. Sao Paulo Med J. 2016 Dec 12;135(1).
20. Zulueta MAG, Carreño DAV, Buitrago M. Two case reports of Boerhaave’s syndrome. Rev Colomb Gastroenterol. 2014;29:179-182.
21. Marshall MWB. Boerhaave syndrome: a case report. J Am Assoc Nurse Anesth. 2002 Aug;70(4):289-92.
22. Jaougon J, Mc Bride T, Delcambre F, Minniti A, Velly JF. Primary esophageal repar for Boerhaave’s syndrome whatever the free interval between perforation and treatment. Eur J Cardiothorac Surg. 2004 Apr 1;25(4):475-9.
23. D’Journo XB, Doddoli C, Avaro JP, Lienne P, Giovanni MA, Giudicelli R, et al. Long-term observation and functional state of the esophagus after primary repair of spontaneous esophageal rupture. Ann Thorac Surg. 2006 May; 81(5):1858-62.
24. Fikfak V, Gaur P, Kim MP. Endoscopic management of Boerhaave's syndrome presenting with hematemesis. J Surg Case Rep. 2014 Nov 1;2014(11):rju110.
25. Lee W, Siau K, Singh G. Boerhaave's syndrome presenting as an upper gastrointestinal bleed. BMJ Case Rep. 2013;2013.
26. Gayet B, Perniceni T. What is the relationship between the Mallory-Weiss syndrome and spontaneous rupture of the lower esophagus? 1998 May [cited 2019 Dec 13]. In: Giuli R, Galmiche JP, Jamieson GG, Scarpignato C. The esophagogastric junction [Internet]. OESO Foundation. C2015. Available from: http://www.oeso.org/OESO/books/Vol_5_Eso_Junction/Articles/art427.html.
27. Ezenkwele UA. Esophageal rupture and tears in emergency medicine clinical presentation. Medscape. Updated October 13, 2016.
28. Chung KT, Shelat VG. Perforated peptic ulcer - an update. World J Gastrointest Surg. 2017 Jan 27;9(1):1-12.
29. Søreide K, Thorsen K, Harrison EM, Bingener J, Møller MH, Ohene-Yeboah M, Søreide JA. Perforated peptic ulcer. Lancet. 2015 Sep 26;386(10000):1288-1298.
30. Kim YD. Management of acute variceal bleeding. Clin Endosc. 2014 Jul;47(4):308-14.
31. Runyon BA. Patient education: Esophageal varices (Beyond the Basics). 2018 Sep 4 [cited 2019 Dec 13]. In: UpToDate [Internet]. Illinois: UpToDate, Inc. c2019. Available from: https://www.uptodate.com/contents/esophageal-varices-beyond-the-basics/print.
32. Mikami M, Nakamura S, Koseki T, Kanemura T, Baba M, Fujikawa T, Kihara N, Kawahara Y, Nagaoka S. [A case of esophageal rupture confirmed by chest CT: characteristic changes in chest radiographs]. Nihon Kyobu Shikkan Gakkai Zasshi. 1989 May;27(5):630-4.
33. Sancheti MS, Fernandez FG. Surgical management of esophageal perforation. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas. 2015 Sep 1;20(3):234-250.
34. Bell DJ, Knipe H. Naclerio V sign [Internet]. Radiopaedia. [cited 2019 Dec 13]. Available from: https://radiopaedia.org/articles/naclerio-v-sign-1.
35.Sinha R. Naclerio’s V sign. Radiology. 2007 Oct 1;245(1).
36. Salo J, Sihvo E, Kauppi J, Räsänen J. Boerhaave's syndrome: lessons learned from 83 cases over three decades. Scand J Surg. 2013;102(4):271-3.
37. Graeber GM, Niezgoda JA, Burton NA, Collins, GJ, Zattchuk R. A comparison of patients with endoscopic esophageal perforations and patients with Boerhaave’s syndrome. Chest 1967 92(6):995-998.
38. Dickinson KJ, Buttar N, Wong Kee Song LM, Gostout CJ, Cassivi SD, Allen MS, Nichols FC, Shen KR, Wigle DA, Blackmon SH. Utility of endoscopic therapy in the management of Boerhaave syndrome. Endosc Int Open. 2016 Nov;4(11):E1146-E1150.
39. Musala C, Eisendrath P, Brasseur A, Vincent JL, Cappeliez S, Le Moine O, Devière J, Lemmers A. Successful treatment of Boerhaave syndrome with an over-the-scope clip. Endoscopy. 2015;47 Suppl 1 UCTN:E24-5.
40. Van Weyenberg SJ, Stam FJ, Marsman W. Successful endoscopic closure of spontaneous esophageal rupture (Boerhaave syndrome). Gastrointest Endosc. 2014 Jul;80(1):162.
41. Kobara H, Mori H, Rafiq K, Fujihara S, Nishiyama N, Kato K, Oryu M, Tani J, Miyoshi H, Masaki T. Successful endoscopic treatment of Boerhaave syndrome using an over-the-scope clip. Endoscopy. 2014;46 Suppl 1 UCTN:E82-3.
42. Haveman JW, Nieuwenhuijs VB, Kobold JP, van Dam GM, Plukker JT, Hofker HS. Adequate debridement and drainage of the mediastinum using open thoracotomy or video-assisted thoracoscopic surgery for Boerhaave's syndrome. Surg Endosc. 2011 Aug;25(8):2492-7.
43. Mavroudis CD, Kucharczuk JC. Acute management of esophageal perforation. Curr Surg Rep. 2014;2:34.
44. Rohatgi A, Papanikitas J, Sutcliffe R, Forshaw M, Mason R. The role of oesophageal diversion and exclusion in the management of oesophageal perforations. Int J Surg. 2009 Apr;7(2):142-4.
45. Raymond DP, Watson TJ. Esophageal diversion. Operative Techniques in Thoracic and Cardiovascular Surgery. 2008;13(2): 138–146.
46. Bakshi A, Sugarbaker DJ, Burt BM. Alternative conduits for esophageal replacement. Ann Cardiothorac Surg. 2017 Mar;6(2):137-143.
47. Poh M, Selber JC, Skoracki R, Walsh GL, Yu P. Technical challenges of total esophageal reconstruction using a supercharged jejunal flap. Ann Surg. 2011 Jun;253(6):1122-9.
48. Cense HA, Visser MR, van Sandick JW, de Boer AG, Lamme B, Obertop H, van Lanschot JJ. Quality of life after colon interposition by necessity for esophageal cancer replacement. J Surg Oncol. 2004 Oct 1;88(1):32-38.
49. Klink CD, Binnebösel M, Schneider M, Ophoff K, Schumpelick V, Jansen M. Operative outcome of colon interposition in the treatment of esophageal cancer: a 20-year experience. Surgery. 2010 Apr;147(4):491-6.
50. Zhang Z, Guo Y, Liang C, Feng H, Liu D. Free jejunum interposition as salvage surgery after cervical esophagus injury. J Thorac Dis. 2016 Jul;8(7):E513-6.
51. Flanagan JC, Batz R, Saboo SS, Nordeck SM, Abbara S, Kernstine K, Vasan V. Esophagectomy and gastric pull-through procedures: surgical techniques, imaging features, and potential complications. Radiographics. 2016 Jan-Feb;36(1):107-21.
52. Moremen JR, Ceppa DP, Rieger KM, Birdas TJ. Substernal reconstruction following esophagectomy: operation of last resort? J Thorac Dis. 2017 Dec;9(12):5040-45.
53. Boukerrouche A. Colon reconstruction and esophageal reconstructive surgery. Med Clin Rev. 2016;2:27.
54. Chen H, Lu JJ, Zhou J, Zhou X, Luo X, Liu Q, Tam J. Anterior versus posterior routes of reconstruction after esophagectomy: a comparative anatomic study. Ann Thorac Surg. 2009;87:400-4.
55. Schweigert M, Beattie R, Solymosi N, et al. Endoscopic stent insertion versus primary operative management for spontaneous rupture of the esophagus (Boerhaave syndrome): An international study comparing the outcome. Am Surg. 2013; 79(6): 634-640.
Copyright © 2019 DP Mitchao, et al.