Challenges faced in the management of complicated Boerhaave syndrome: a tertiary care center experience.

Spontaneous esophageal perforation is rare and is associated with high morbidity and mortality. A spectrum of various surgical modalities ranging from primary surgical repair to esophagectomy is available for its management. The optimal management of patients presenting late in a hemodynamically stable condition is not clearly defined in the literature. A retrospective review of all patients with Boerhaave syndrome managed by a single surgical team in a tertiary care center between 2008 and 2019 was performed (n = 16). Eleven patients were initially managed in the medical intensive care unit (MICU) as non-esophageal cause and 5 patients were referred after failed management (conservative/endoscopic). Demographics, clinical presentation, characteristics of perforation, initial diagnosis, and treatment were analyzed. All patients were males with a mean age of 42.2 years. A history of ethanol use was present in 6 patients. The median delay in diagnosis and referral was 16 days (range: 11-40 days). The common presenting symptoms were chest pain (n=11), dyspnoea (n=10), vomiting (n=4) and cough (n=2). The perforation was directed into right, left, and bilateral pleural cavities in 6, 8, and 2 patients respectively. The location of perforation was distal esophagus except for one patient. One patient was successfully treated with conservative management. The remaining patients underwent esophagectomy as a definitive surgical procedure. There was no significant postoperative morbidity and mortality. Esophagectomy can be done as a one-stage definitive procedure for patients with Boerhaave syndrome who present late in a hemodynamically stable condition with acceptable morbidity and good long term outcome.

Pathophysiology of Vomiting and Esophageal Perforation in Boerhaave's Syndrome.

BACKGROUND AND AIMS: Boerhaave's syndrome, involving esophagus rupture, is considered a pathological response to vomiting that may occur just before perforation. However, the mechanism of vomiting and occurrence of this disease have not been clearly demonstrated. METHODS: We identified patients with esophageal perforation between 1995 and 2017 and reviewed endoscopic findings at retching during upper gastrointestinal endoscopy. Finally, we proposed a theory for the underlying pathological mechanism. RESULTS: We retrospectively investigated 10 patients with esophageal perforation between 1995 and 2017. All patients presented after vomiting associated with large volumes of food and alcohol intake. Nine were treated by primary closure of the perforation and drainage of the thoracic cavity, and one was conservatively treated. In all cases, the perforations were longitudinal tears (1-4 cm) and located in the left of the esophagus, just above the gastric cardia. CONCLUSIONS: We hypothesize that vomiting occurred by retrograde propagation of gastrointestinal motor contraction from the jejunum to the gastric antrum, followed by prolapse of the gastric fornix mucosal into the esophagus. Subsequent esophageal perforation probably resulted from excessive prolapse due to strong contraction and destruction of the muscularis mucosa of the left side of abdominal esophagus, with longitudinal stretching of the whole left esophageal wall due to traction. We also propose that Boerhaave's syndrome is defined as "post-emetic esophageal perforation" to ensure broader recognition and more expedient diagnosis and treatment. Remaining conditions without any definite causes may be labeled "idiopathic" or "spontaneous" rupture of the esophagus.

Esophageal perforation in eosinophilic esophagitis: A systematic review on clinical presentation, management and outcomes.

There is evidence of an increased fragility in the inflamed esophagus of patients with eosinophilic esophagitis (EoE). We performed a systematic review on presentation, management and outcomes of and surgical interventions for esophageal perforation in these patients, by searching in the MEDLINE, Embase and Scopus databases. Of the 599 references identified, 41 full-papers and 9 abstract met the inclusion criteria. Overall, 76 esophageal perforation episodes in 70 individual patients aged between 9 and 65 years were reported. 51 patients had not been diagnosed with EoE at the time of perforation; 14 patients had an untreated disease and the remaining were non responsive to therapy. Acute or progressive pain after long-lasting dysphagia and food impaction was the most common symptom leading to diagnosis in 42 patients who presented with Boerhaave syndrome. Pushing impacted food into the stomach led to perforation in 5 cases. Eight episodes appeared after dilation. CT scans demonstrated perforation in 82.4% of patients. Conservative management (including esophageal stenting) was used in 67.1% patients. The 25 remaining patients underwent surgery. Recovery was uneventful in the vast majority of patients. No death was reported. Active inflammation due to undiagnosed or untreated EoE was present in most cases of esophageal perforation. Conservative treatment of perforation should always be considered in EoE.

A potential role of toll-like receptors, IFN-γ and the phosphatidylinositol 3-kinase pathway in the pathogenesis of acquired mediastinal lymphatic malformation.

Sarcoidosis is a multisystem disorder with non-caseating granulomas in various organs. The etiology of sarcoid granuloma formation is not clear and likely an antigen-induced process. We came across a previously treated sarcoidosis patient who presented with worsening dyspnea on exertion for several months and several days of difficulty swallowing. On Chest CT imaging, large posterior mediastinal mass was found that subsequently diagnosed as macrocystic lymphatic malformation after surgical resection. Pathophysiology of development of acquired lymphatic malformations in a sarcoidosis patient is currently not clear. We hypothesize there might be a complex interplay of Toll-like receptors, IFN-γ and the phosphatidylinositol 3-kinase pathway in the pathogenesis.

When appearances deceive: Echocardiographic changes due to common chest pathology.

Most indications for performing echocardiography focus on the evaluation of properties intrinsic to the heart. However, numerous extra-cardiac conditions indirectly convey changes to the echocardiographic appearance through alterations in the governing physiology. Pulmonary embolism increases pulmonary arterial pressure if a sufficient cross-sectional area of the pulmonary vascular bed is occluded. This may result in dilatation of the right ventricle and, in severe cases, concomitant early diastolic septal collapse into the left ventricle. Acute respiratory failure has been shown to yield a similar echocardiographic appearance in experimental conditions due to the resultant pulmonary vasoconstriction. Echocardiography in the presence of pulmonary disease can reveal underlying cardiac pathologies such as pulmonary hypertension that contribute to the clinical severity of respiratory distress. Positive pressure ventilation affects preload, afterload, and compliance of both ventricles. The echocardiographic net result cannot be uniformly anticipated, but provides information on the deciding physiology or pathophysiology. Mediastinal pathology including tumors, herniation of abdominal content, and pleural effusion can often be visualized directly with echocardiography. Mediastinal pathologies adjacent to the heart may compress the myocardium directly, thus facilitating echocardiographic and clinical signs of tamponade in the absence of pericardial effusion. In conclusion, many pathologies of extra-cardiac origin influence the echocardiographic appearance of the heart. These changes do not reflect properties of the myocardium but may well be mistaken for it. Hence, these conditions are essential knowledge to all physicians performing echocardiography across the spectrum from advanced cardiological diagnostics to rapid point-of-care focused cardiac ultrasonography.

[Boerhaave syndrome: update on physiopathology, diagnosis and early management]. / Syndrome de Boerhaave : mise au point sur la physiopathologie, le diagnostic et le traitement précoce.

Boerhaave syndrome is a rare condition, usually associated with a delay in diagnosis. It is fatal in the absence of therapy. The nonspecific nature of the clinical signs contributes to the poor outcome. This syndrome should be suspected in patients with severe chest or abdominal pain, associated with a history of vomiting, clinical signs of pneumomediastinum (subcutaneous emphysema, pleural effusion) and rapid clinical deterioration. Prompt diagnosis and initial management by the primary care physician and the emergency department physician are key elements of a better outcome. This article will review the pathogenesis, clinical manifestations, diagnosis and treatment of Boerhaave syndrome in adults.

Le syndrome de Boerhaave est une entité rare, de diagnostic difficile, dont l'évolution est potentiellement fatale en l'absence d'une prise en charge rapide. Le manque de spécificité des signes cliniques participe au retard diagnostique et au mauvais pronostic. Le syndrome doit être évoqué lors d'une symptomatologie thoracique ou abdominale aiguë, avec une anamnèse de vomissements, des signes cliniques de pneumomédiastin (emphysème sous-cutané, épanchements pleuraux) et l'apparition de signes de choc. Le pronostic étant conditionné par la rapidité du diagnostic et de la prise en charge, le rôle du médecin de premier recours et de l'urgentiste est crucial. Cet article a pour but de faire le point sur la physiopathologie, les manifestations cliniques, le diagnostic et le traitement de cette pathologie chez l'adulte.

Boerhaave's syndrome complicated by a Saccharomyces cerevisiae pleural empyema. Case report and review of the literature.

Objective and Importance Boerhaave's syndrome is a sudden and rare form of oesophageal rupture and is often complicated by local or systemic infection of the mediastinum or pleural cavity. Several micro-organisms are documented as cause of pleural empyema in patients with Boerhaave's syndrome. Intervention (& Technique) We report on a previously healthy 74-year-old male who was admitted at a regional hospital with severe retrosternal and abdominal pain after an episode of vigorous vomiting the morning after ingestion of large quantity of beer. A CT-scan confirmed the diagnosis of Boerhaave's syndrome, an oesophageal stent was placed and a left-sided pleural empyema necessitated chest tube drainage. Pleural fluid samples were cultured every two days and were positive for Proteus mirabilis on day 2 after admission and for Saccharomyces cerevisiae on day 8 after admission. Intravenous fluconazole 800 mg per day was added to the antibacterial treatment. Pleural fluid culture became negative for P. mirabilis on day 23 and for S. cerevisiae on day 13. Recurrent empyema necessitated intrapleural thrombolysis. The patient could be discharged from the ICU after 43 days, from the normal ward to a rehabilitation centre after an additional 13 days. Conclusion Pleural empyema caused by S. cerevisiae, commonly known as 'Brewers' yeast', has never been described in such patients. Our case illustrates that clinicians should be aware of infection with S. cerevisiae after oesophageal perforation, soon after ingestion of beer. Adequate antimycotic treatment was successful and led to negative culture of pleural fluid after 5 days.

A multicenter study on the utility and safety of EBUS-TBNA and EUS-B-FNA in children.

BACKGROUND AND AIM: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration (EUS-B-FNA) are useful modalities in the evaluation of mediastinal lymphadenopathy in adults; however, there is sparse data in children. The aim of this multicenter study is to describe the efficacy and safety of EBUS-TBNA and EUS-B-FNA in children with mediastinal lymphadenopathy of undefined etiology. METHODS: Retrospective analysis of consecutive pediatric (<18 years) subjects who underwent EBUS-TBNA or EUS-B-FNA for the evaluation of mediastinal lymphadenopathy. The demographic characteristics, indications, procedural details, pathological, cytological and microbiological diagnosis, diagnostic yield, and complications are presented. RESULTS: Of the 3,424 EBUS/EUS-B-FNA procedures, 67 (1.9%) were performed in the pediatric (3-17 years) population. Of these, 19 (28.4%) were performed in children ≤12 years of age. Overall, EBUS-TBNA and EUS-B-FNA were performed in 53 and 12 subjects, respectively. In two subjects, no significant lymph node was seen on EBUS. The procedure was performed under moderate sedation in spontaneously breathing subjects in 54 (80.6%) instances. An adequate sample was obtained in 60 (92.3%) subjects while a diagnostic sample was obtained in 37 (56.9%) of the 65 subjects. The diagnostic yield was not significantly different (P = 0.59) between EBUS-TBNA (58.5%) and EUS-B-FNA (50%). The sensitivity of EBUS-TBNA/EUS-B-FNA was 79.1% and led to a change in diagnosis in 28 (41.8%) subjects. Complications, all minor were encountered in six (8.9%) subjects. CONCLUSIONS: EBUS-TBNA and EUS-B-FNA are safe techniques with a good diagnostic yield in the evaluation of children with mediastinal lymphadenopathy. Pediatr Pulmonol. 2016;51:1031-1039. © 2016 Wiley Periodicals, Inc.

Hamman and Boerhaave syndromes - diagnostic dilemmas in a patient presenting with hyperemesis gravidarum: a case report.

INTRODUCTION: Hyperemesis gravidarum describes persistent vomiting leading to fluid and electrolyte imbalance. It is the commonest reason for admission in the first half of pregnancy. We describe a case of Hamman syndrome secondary to hyperemesis gravidarum. We also discuss Boerhaave syndrome: a particularly rare condition with only a handful of cases being described in the literature. CASE PRESENTATION: A 17 year old admitted with hyperemesis gravidarum was diagnosed with Hamman syndrome after complaining of chest pain due to the presence of subcutaneous emphysema and pneumomediastinum on chest radiograph. She was treated conservatively for potential ruptured oesophagus but then self-discharged against medical advice. CONCLUSION: Subcutaneous emphysema is an alarming finding in any pregnancy and should be treated in a timely and cautious manner. This case report adds weight to the previous literature advocating a conservative versus surgical approach to the management of a woman with Hamman syndrome secondary to hyperemesis gravidarum.

Chest pain and an abnormal chest X-ray--a case report.

Chest pain is a common symptom amongst patients presenting to the acute medical unit, and presents a diagnostic challenge. We present the case of a previously healthy 65 year old year old gentleman with chest pain and subsequent vomiting, treated initially as an acute coronary syndrome, despite normal ECG and troponin. Chest radiograph revealed left basal consolidation with an effusion, suggesting pneumonia. The absence of symptoms of respiratory infection along with a normal C-reactive protein level led to further investigation with CT. This revealed evidence of oesophageal rupture (Boerhaave's syndrome). Despite early surgical intervention our patient suffered a number of complications and continues his recovery in hospital three months post presentation.

The utility of esophagogastroduodenoscopy before endoscopic ultrasonography in patients undergoing endoscopic ultrasonography for pancreatico-biliary and mediastinal indications.

BACKGROUND: Oblique-viewing echoendoscopes may miss luminal lesions. There is no consensus on whether to routinely perform esophagogastroduodenoscopy (EGD) before endoscopic ultrasonography (EUS). Currently, practice patterns are variable and prospective data are needed. AIM: : To determine the proportion of clinically meaningful lesions detected when EGD is performed routinely before EUS. STUDY: This was a multicenter prospective cohort study conducted at tertiary referral center and large community practice. Patients undergoing EUS for pancreatico-biliary and mediastinal indications were enrolled. MAIN OUTCOMES: The primary outcome was the proportion of patients with a clinically meaningful lesion found on EGD. This was a combined outcome defined as any lesion that would alter medical management, or impact the subsequent EUS examination. RESULTS: Two hundred four patients were included in the final analysis. Clinically meaningful lesions were found on EGD in 45 patients [22.1%; 95% confidence interval (CI), 16.4-27.8]. Lesions that altered medical management were found in 32 patients (15.7%; 95% CI, 10.7-20.7). Lesions impacting the subsequent EUS examination were found in 20 patients (9.8%; 95% CI, 5.7-13.9). Clinically meaningful lesions found were (number of patients): esophagitis (14), ulcer (9), ring/stricture (7), large hiatal hernia (6), hyperplastic gastric polyp (5), Barrett esophagus (3), surgically altered anatomy (2), neoplastic lesion (2), subepithelial mass/GIST (1), stenosis (1), diverticulum (1), and fistula (1). CONCLUSIONS: EGD before EUS may detect enough clinically meaningful lesions to support the routine performance of EGD before EUS.

Ectopic parathyroid glands and their anatomical, clinical and surgical implications.

Ectopic parathyroid glands result from aberrant migration during early stages of development and lack of successful identification may lead to lack of success in parathyroid surgery. They constitute a common etiology of persistent or recurrent hyperparathyroidism, when they are missed at initial diagnosis. Their prevalence is about 2-43% in anatomical series and up to 16% and 14% in patients with primary and secondary hyperparathyroidism, respectively. Ectopic inferior parathyroids are most frequently found in the anterior mediastinum, in association with the thymus or the thyroid gland, while the most common position for ectopic superior parathyroids is the tracheoesophageal groove and retroesophageal region. Neck ultrasound and 99mTc Sestamibi scan are first-line imaging modalities, although with low sensitivity and specificity. However, their combination with modern techniques, such as single photon emission computed tomography (SPECT) alone or in combination with CT (SPECT/CT) increases their diagnostic accuracy. Fine needle-aspiration cytology of a lesion suspicious for parathyroid tissue and measurement of parathyroid hormone (PTH) in the aspired material further assist to the successful preoperative localization of ectopic glands. Common sites for surgical investigation are the upper thyroid pole and the upper vascular thyroid stalk behind the hypopharynx and cervical esophagus for the superior parathyroids, and the carotid artery bifurcation and the thymic tongue, for the inferior parathyroids. Radioguided minimally invasive parathyroidectomy after successful localization, assisted by rapid PTH measurement postoperatively, significantly improves surgical outcomes in patients with ectopic parathyroid adenomas.