A rare case of Boerhaave syndrome with cervico-thoracic esophageal junction rupture causing bilateral empyema; case report from Nepal

Abstract

Introduction and importance

Boerhaave syndrome is a rare finding where spontaneous rupture of esophagus occurs, usually in distal segment. It is a life threatening condition that requires urgent surgical intervention.

Case presentation

We present a case of 70 years male who presented with pleural effusion and later empyema following spontaneous rupture of cervico-thoracic junction of esophagus who was managed successfully with primary surgical repair.

Clinical discussion

Although Boerhaave syndrome is tricky to diagnose but it should be considered in all cases with a combination of gastrointestinal and pulmonary signs and symptoms.

Conclusion

Clinical correlation along with imaging such as HRCT chest or gastrografin study is required to come to a diagnosis, however surgical intervention should not be delayed to reduce the mortality.

Highlights

• •Boerhaave syndrome is a rare finding where spontaneous rupture of esophagus occurs, usually in distal segment.
• •Cervico-thoracic junction rupture is rare finding leading to boerhaave syndrome
• •All cases with a combination of gastrointestinal symptoms and pulmonary signs and symptoms should raise suspicion.
• •Early surgical intervention saves lives.

1. Introduction

Boerhaave syndrome consists of spontaneous longitudinal transmural rupture of the esophagus, usually in its distal part [1]. It is many times an incidental finding as there are no specific symptoms of it. The classic triad of Mackler (vomiting, lower thoracic pain and subcutaneous emphysema) is present in less than 50 % of cases [1] which makes it difficult to diagnose. Esophageal rupture may lead to either of mediastinal inflammation and subsequent pleural rupture that may cause pleural effusion, subcutaneous emphysema along with sepsis.

Patients may present with excruciating retrosternal chest pain, severe retching and vomiting.

It is a life threatening condition that requires urgent surgical intervention and requires a multidisciplinary approach for better outcome. In this case report we discuss about this rare condition that could be difficult to diagnose. This case report has been reported in line with the SCARE 2020 standards [2].

2. Presentation of case

We report a case of a 70 year old male with hypertension, diabetes mellitus and chronic kidney disease presenting with sudden severe bouts of vomiting and had visited a local hospital with the suspicion of foreign body (bone) impaction in the food pipe. Basic clinical examination and xray of neck (AP and Lateral) was done and was discharged on finding no gross abnormalities, ruling out foreign body impaction. After 5 days, he was admitted as he presented with fever, headache and persistent neck pain. USG neck showed a simple cyst of thyroid and UGI Endoscopy revealed erosive antral gastritis for which medical management was done and was discharged.

Patient visited our hospital on 7th day of incident, for chest pain and right sided pleural effusion was seen on chest x ray. He had raised WBC 16880 cm³/mm, urea (53 mg/dl), creatinine (1.3), Hb 10.3 g%, LDH 269 μ/l, serum albumin 2 g/dl and GRBS 524 mg/dl. Echocardiography showed normal LV systolic function with EF 60 % and mild TR. USG Abdomen revealed cholelithiasis and collection in bilateral pleural cavity. USG Chest showed collapsed consolidation of bilateral lungs.

Therapeutic tapping was done from the right lung. He later complained of left sided chest pain and had a gradual increase in shortness of breath. Chest x ray revealed bilateral pleural effusion, more on the left side. Initial USG guided diagnostic pleural fluid tapping from left lung had 500 ml of turbid fluid, which showed WBC of 16,500 cells/cumm, 90 % neutrophils, ADA 45, LDH 8815, protein 4.2, gram stain 8–10 pus cells and culture result awaited. Later as there was no improvement, bilateral chest tube drain was placed which drained about 800 ml and 900 ml in the first 24 h from right and left side respectively. Next day the patient complained of dysphagia and persistent chest pain for which HRCT chest was done, on which esophageal perforation was suspected. HRCT Chest reported hydropneumothorax with bilateral lower lobe collapse, thickening of the wall of upper thoracic esophagus with extraluminal air and collection in the paraesophageal region. Gastrografin swallow test initially showed no leak whereas undiluted urografin showed contrast leak from posterolateral aspect of esophagus (Fig. 1).

Fig. 1.

Multiple radiological images of chest X ray, CT scan and gastrografin study with demonstration of pleural effusion, empyema and leak through esophagus respectively.

3. Case management

Patient was put under intravenous antibiotics with clindamycin, meropenem and levofloxacin along with iv paracetamol for analgesia, iv PPIs. TPN 1000 kcal @ 63 ml/h was started as he was kept nil per oral. Open surgical exploration and repair was done. A J shaped incision of about 3 cm was given over left lower cervical region along with the anterior aspect of sternocleidomastoid muscle. Platysma flap was taken out and sternocleidomastoid muscle was retracted. Blunt dissection of esophageal adventitia was made, dissected, followed by debridement and freshening of the margins of the perforation. Longitudinal tear of size 0.5 × 0.5 cm at posterior surface of the cervico-esophageal junction was noted. Muscle fibers both up and down the perforation were cut longitudinally and repair of perforation was made with 3-0 vicryl suture, interrupted suture. Flap was placed over the repair and sutured circumferentially with interrupted 3-0 vicryl suture and single layer closure was done. Corrugated drain was kept and muscles and skin closure was done. Feeding jejunostomy was placed. Nasogastric tube was kept on free drain for 3 days, and feeding jejunostomy free drain for 24 h followed by iv DNS 500 ml over 24 h followed by 300 kcal on next day and gradually increment from feeding jejunostomy tube. Iv antibiotics were continued for the full course. Patient was shifted to the ward and on 12th day was discharged to home.

4. Discussion

Boerhaave syndrome is most frequent among men in the sixth and seventh decades. Our patient was also in his 7th decade of his life. The rupture can occur in the cervical or intra-abdominal esophagus and most frequently occurs in the lower third of the esophagus and in the left lateral position, due to anatomic weakness in this area [3]. The rupture less commonly occurs in the subdiaphragmatic or upper thoracic area. In our case perforation was on the cervicothoracic junction of the esophagus making it a rare finding.

There can be unusual features including a delayed presentation and position during the time of presentation. In our case, almost after 5 days he had respiratory symptoms where it led to pleural effusion and empyema. As the patient was hemodynamically stable and had a history of emesis 5 days prior to presentation with normal x-ray and endoscopy findings, Boerhaave's syndrome was not considered initially. Initial problem may have been a sub-clinical Boerhaave syndrome as only pleural effusion and empyema resulted from the leak.

The term spontaneous perforation of the esophagus, although commonly used, is an inappropriate term, as the rupture is rarely spontaneous and almost invariably follows barotrauma from a sudden post-emetic rise in esophageal pressure. We think that a positive history of severe bouts of vomiting with no other presentations initially could be the cause of perforation in our case.

Spontaneous rupture usually occurs in a longitudinal fashion and varies in size from 0.6 cm to 8.9 cm long, with the left side more commonly affected than the right (90 %) [4]. In cases of perforation of the middle third of the esophagus, pleural effusion is present on the right side, while in cases of rupture of the distal esophagus, pleural effusion is present on the left side [5]. There was initial right sided effusion in our case followed by left sided massive effusion which was managed by bilateral chest drain insertion and drainage. Longitudinal tear noticed during surgery and massive effusion on left side may indicate this to be a spontaneous rupture as seen in cervical segments.

There should be a high index of suspicion in patients presenting with vomiting and pulmonary symptoms with no other obvious probable cause. Clinical correlation along with imaging such as HRCT chest or gastrografin study is required to come to a diagnosis. Results of upper gastrointestinal studies such as contrast medium swallows usually show extravasation but may have false-negative results in up to 20 % of patients [6]. When diluted contrast was used, normal study results were yielded whereas upon use of undiluted urografin, positive results for leak were demonstrated thus we must be aware of false negative results.

The major differential diagnoses include spontaneous pneumothorax, gastric ulcer or bowel perforation with free air in the abdomen, Mallory-Weiss tear, diaphragmatic rupture, pneumonia, pancreatitis, and acute pericarditis.

Esophageal rupture may lead to mediastinitis and peritonitis, usually presenting with sepsis causing multiple organ failure syndrome. Thus there is a high mortality rate. Even if treated promptly, the mortality approaches 50 %, usually related to sepsis, mediastinitis, pericarditis, pneumonitis, or empyema [6]. If treatment is not started within 24 h from the onset of symptoms, the mortality rate is 25 %; after 24 h, it is 65 %; and after 48 h, it is 75 %–89 % [7].

When the diagnosis of cervical perforation is within 24 h from injury, surgical repair of the perforation has better outcome. Tissue flaps from sternohyoid, sternothyroid or sternocleidomastoid muscle may be used to reinforce the cervical esophageal perforation repair site [8]. If diagnosis is made beyond 24 h of injury, primary repair may not be feasible due to severe contamination and inflammation. In these patients, an esophageal T-tube insertion has been advocated which helps to create a controlled esophageal fistula that allows to drain the contamination [9]. Diversion through cervical esophagostomy creation can be other modality of surgery. Minimally invasive techniques such as endoscopic insertion of self-expanding metallic stents (SEMS), drug eluting self-expandable stents, endoscopic clips such as hemoclip and fibrin glue injection are also getting popular. The drug eluting stent must be at least 4 cm longer than the size of the damage. The stent must be able to cover an area of at least 2 cm from the proximal and the distal of the perforation [10]. However, placement of a stent has been found to be ineffective in the event of an injury on the proximal of the cervical esophagus or on the gastroesophageal junction, segment injuries longer than 6 cm, and an anastomotic leakage found in the more distal conduit section [11]. Esophagectomy, is very rarely used today. Endoscopic vacuum sponge therapy has been introduced recently but the roles and success of these new devices are unclear.

5. Conclusion

Boerhaave syndrome might be tricky to diagnose but it should be considered in all cases with a combination of gastrointestinal symptoms (especially sudden epigastric pain and multiple bouts of severe vomiting) and pulmonary signs and symptoms (shortness of breath and chest heaviness). There are diagnostic and therapeutic challenges but surgical intervention should not be delayed to reduce the mortality.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The authors institute provided ethical approval for this case study.

Funding

N/A.

Guarantor

Bikash Khadka.

Research registration number

N/A.

CRediT authorship contribution statement

• Bikash Khadka: Conceptualization, Wrote manuscript

• Kishor Khanal: Revised manuscript

• Puja Dahal: Collected data, Wrote manuscript

• Hemant Adhikari: Revised manuscript.

Declaration of competing interest

N/A.