Management of Esophageal Perforation: 28-Year Experience in a Major Referral Center

Esophageal perforation constitutes a surgical emergency. Despite its gravity, no single strategy has been described as sufficient to deal with most situations to date. The aim of this study was to assess the etiology, management, and outcome of esophageal perforation over a 28-year period, to characterize optimal treatment options in this severe disease. A retrospective clinical review of all patients treated for esophageal perforation at Ramón y Cajal Hospital between January 1987 and December 2015 was performed (n = 57). Iatrogenic injury was the most frequent cause of esophageal perforation (n = 32). Abdominal esophagus was the main location (23 patients; 40.4%). Eight patients (14%) were managed with antibiotics and parenteral nutrition. In seven patients (12.3%), an endoscopic stent was implanted. Surgical therapy was performed in 38 patients (66.7%). Morbidity and 90-day mortality rates were 61.4 and 28 per cent, respectively. Five patients were reoperated (8.8%). Median hospital stay was 23.5 days. The mortality rate was higher among patients with spontaneous and tumoral perforation (54.5 and 100%; P = 0.009), delayed diagnosis (>24 hours; P = 0.0001), and abdominal/thoracic location (37.5%; P = 0.05). No statistical differences were found between surgical and conservative/endoscopic management (31% vs 20%; P = 0.205) although hospital staying was longer in surgical group (36.30 days vs 15.63 days; P = 0.029). Esophageal perforation was associated with high morbidity and mortality rates. Global outcomes depend on etiology, site of perforation, and delay in diagnosis. An individualized approach for each patient should be chosen to prevent septic complications of this potentially fatal disease.

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Educational aspects of rare and orphan lung diseases

Respiratory Research ◽

10.1186/s12931-021-01676-1 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Tiago M. Alfaro ◽

Marlies S. Wijsenbeek ◽

Pippa Powell ◽

Daiana Stolz ◽

John R. Hurst ◽

...

Keyword(s):

Lung Diseases ◽

Healthcare Professionals ◽

Treatment Options ◽

Delayed Diagnosis ◽

Reference Network ◽

Delay In Diagnosis ◽

Positive Effects ◽

European Reference Network ◽

Patient Representatives ◽

Rare Lung Diseases

AbstractPeople with rare lung diseases often suffer the burden of delayed diagnosis, limited treatment options, and difficulties in finding expert physicians. One of the reasons for the delay in diagnosis is the limited training for healthcare practitioners on rare diseases. This review explores the main concerns and needs for education on rare lung diseases from the perspectives of both patients and professionals. Despite the increasing interest in rare lung disorders and some recent breakthrough developments on the management of several diseases, healthcare professionals, including general practitioners and hospital workers, receive little education on this topic. Nonetheless, many healthcare professionals show much interest in receiving further training, especially on diagnosis. Patients and families want easier access to high-quality education materials to help them manage their own disease. Well-educated patients are better equipped to deal with chronic diseases, but patient education can be challenging as patients’ individual health issues, and diverse backgrounds can create significant barriers. Raising more awareness for rare lung diseases and further development of patient-centred international expert networks like the European Reference Network on Rare Lung Diseases (ERN-LUNG), which includes both experts and patient representatives, are essential for improving care and education on rare lung diseases. Initiatives such as the Rare Disease Day, have been successful in increasing awareness for rare conditions. The development of online tools for accessing information has had positive effects and should be further supported and extended in the future.

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806 CERVICAL ESOPHAGEAL PERFORATION: MULTIMODAL APPROACH TO A RARE AND MORBID CONDITION

Diseases of the Esophagus ◽

10.1093/dote/doab052.806 ◽

2021 ◽

Vol 34 (Supplement_1) ◽

Author(s):

Chee Chuan Tang ◽

Kelvin Voon ◽

Nagalingam Premnath

Keyword(s):

Esophageal Perforation ◽

Treatment Options ◽

High Morbidity ◽

Contrast Study ◽

Oral Contrast ◽

Multimodal Approach ◽

Early Management ◽

Iatrogenic Perforation ◽

Diagnosis And Management ◽

Cervical Esophageal Perforation

Abstract Cervical esophageal perforation (CEP) is an uncommon but serious surgical condition. It is associated with a high morbidity and mortality if not managed timely, partly due to its close proximity with vital structures in the neck and mediastinum. The common causes include trauma, foreign body ingestion and iatrogenic perforation. The diagnosis and management of CEP remain challenging despite advances in surgery. Methods We present a series of 3 cases of CEPs, with multimodal approach for the management. Results In this series of 3 cases of CEPs with different presentations, tailored management strategies resulted with successful outcomes. Post-procedure/operative oral contrast study revealed no contrast leakage. Conclusion The diagnosis and management of CEP remain challenging despite advances in surgery. Contrasted CT scan and endoscopy are the mainstay of investigations. The treatment options of CEP range from conservative management to endoscopic intervention to surgical repair often with drainage procedures due to its frequent association with collections in the neck, pleural or mediastinal cavity. Prompt detection and early management with multimodal intervention ensure a better outcome in these patients.

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A case of esophageal perforation: Clinical and diagnostic management in emergency medicine

Emergency Care Journal ◽

10.4081/ecj.2017.6848 ◽

2018 ◽

Vol 13 (2) ◽

Cited By ~ 1

Author(s):

Marco Di Serafino ◽

Maurizio Martorano ◽

Pamela Delmonaco ◽

Chiara Gullotto ◽

Andrea Baglioni ◽

...

Keyword(s):

Foreign Body ◽

Esophageal Perforation ◽

Delayed Diagnosis ◽

Penetrating Trauma ◽

Foreign Body Ingestion ◽

Delay In Diagnosis ◽

Diagnostic Management ◽

Esophagus Perforation ◽

Boerhaave Syndrome ◽

Diagnostic Work

Esophageal perforation is a welldefined and severe clinical condition. The associated mortality rates range between 5% and 40% and are worsened by delayed diagnosis. Rapid diagnosis and therapy provide the best chance for survival; however, a delay in diagnosis is common, resulting in substantial morbidity and mortality. There are several aetiologies of esophagus perforation. Most esophageal ruptures are secondary to medical instrumentation. Other causes are Boerhaave syndrome, toxic ingestions and radiation, foreign body ingestion, penetrating trauma, and, rarely, blunt chest trauma. We reported the clinical management and the diagnostic work-up case of esophagus perforation due to the foreign body ingestion.

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A rare case of myelodysplastic syndrome with refractory thrombocytopenia

Hematology Reports ◽

10.4081/hr.2015.5897 ◽

2015 ◽

Vol 7 (3) ◽

Author(s):

Waqas Jehangir ◽

John Webb ◽

Shilpi Singh ◽

Sabrina Arshed ◽

Shuvendu Sen ◽

...

Keyword(s):

Rare Case ◽

Treatment Options ◽

Gene Mutations ◽

Mortality Rates ◽

Morbidity And Mortality ◽

Genetic Mutations ◽

Specific Gene ◽

High Morbidity ◽

Risk Patients ◽

Evidenced Based

Myelodysplastic syndromes (MDS) represent a variety of clonal abnormalities, possibly preleukemic and display numerous phenotypic manifestations. Specific mutations carry high morbidity and mortality rates due to cell line dysplasia. MDS commonly presents with symptoms related to anemia, and approximately two-thirds will develop thrombocytopenia, a rare, but potentially lethal complication that increases complexity in treatment and morbidity, and may be due to unique genetic mutations leading to refractory thrombocytopenia, ultimately leading to an overall reduction in survival. Careful identification and monitoring of this patient subdivision can significantly reduce morbidity and mortality, and potential identification of specific gene mutations and advances in treatment options will hopefully provide guidance on detecting at-risk patients in the future. We present a case of a man with MDS-U (karyotype 46, XY, del (20) (q11.2q13.3) (20) with no detected <em>JAK2</em> <em>V617F</em> mutation), who in despite of appropriate evidenced based treatment, continued to exhibit refractory thrombocytopenia.

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Management of spontaneous and iatrogenic perforations, leaks and fistulae of the upper gastrointestinal tract

Therapeutic Advances in Gastrointestinal Endoscopy ◽

10.1177/2631774519895845 ◽

2019 ◽

Vol 12 ◽

pp. 263177451989584

Author(s):

Jamal Al-Asiry ◽

Richard Lord ◽

Noor Mohammed

Keyword(s):

Surgical Repair ◽

Early Recognition ◽

Patient Specific ◽

Endoscopic Management ◽

Vacuum Therapy ◽

High Morbidity ◽

First Line ◽

Septic Complications ◽

The Past ◽

Upper Gastrointestinal

Upper gastrointestinal perforations, leaks and fistulae are relatively common occurrences with a growing number of these complications occuring as a result of therapeutic advancement and adoption of newer and bolder endoscopic therapies. Historically, these were predominantly managed surgically; however, owing to high morbidity and mortality associated with surgical repair, endoscopic options are preferable. Over the past decade, vast expansion in the endoscopic armamentarium for the management of perforations, leaks and fistulae has led to endoscopic management now being the first-line treatment. Here, we will review the endoscopic modalities including through-the-scope clips, over-the-scope clips, stents, vacuum therapy, endoscopic sutures and sealants. In addition, we will discuss nonendoscopic approach to management including early recognition of perforations, ways to reduce septic complications and format algorithms to guide therapy for different scenarios. However, it is important to stress that there is a lack of high-quality randomised studies to clearly guide management of such complications, resulting in a wide variation of approaches in management by specialists. Each case requires some degree of individualisation due to the potential array of problems encountered and patient-specific co-morbidities. In the future, more robust studies are clearly required to better guide specialist management.

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Delayed Diagnosis of Hidradenitis Suppurativa and Its Effect on Patients and Healthcare System

Dermatology ◽

10.1159/000508787 ◽

2020 ◽

Vol 236 (5) ◽

pp. 421-430 ◽

Cited By ~ 5

Author(s):

Georgios Kokolakis ◽

Kerstin Wolk ◽

Sylke Schneider-Burrus ◽

Stefanie Kalus ◽

Sebastian Barbus ◽

...

Keyword(s):

Healthcare System ◽

Inflammatory Disease ◽

Hidradenitis Suppurativa ◽

Severe Disease ◽

Delayed Diagnosis ◽

Chronic Inflammatory Disease ◽

Purulent Discharge ◽

Diagnosis Delay ◽

Cross Sectional ◽

Delay In Diagnosis

Background: Hidradenitis suppurativa (HS) is a neglected chronic inflammatory disease with long delay in diagnosis. Besides pain, purulent discharge, and destruction of skin architecture, HS patients experience metabolic, musculoskeletal, and psychological disorders. Objectives: To determine the delay in HS diagnosis and its consequences for patients and the healthcare system. Methods: This was a prospective, multicenter, epidemiologic, non-interventional cross-sectional trial carried out in Germany and based on self-reported questionnaires and medical examinations performed by dermatologists. In total, data of 394 adult HS patients were evaluated. Results: The average duration from manifestation of first symptoms until HS diagnosis was 10.0 ± 9.6 (mean ± SD) years. During this time, HS patients consulted on average more than 3 different physicians – most frequently general practitioners, dermatologists, surgeons, gynecologists – and faced more than 3 misdiagnoses. Diagnosis delay was longer in younger and non-smoking patients. In most cases, HS was correctly diagnosed by dermatologists. The longer the delay of diagnosis, the greater the disease severity at diagnosis. Delayed HS diagnosis was also associated with an increased number of surgically treated sites, concomitant diseases, and days of work missed. Conclusion: This study demonstrates an enormous delay in the diagnosis of HS, which results in more severe disease. It also shows for the first time that a delay in diagnosis of a chronic inflammatory disease leads to a higher number of concomitant systemic disorders. In addition to the impaired health status, delayed diagnosis of HS was associated with impairment of the professional life of affected people.

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Not Always Asthma: Clinical and Legal Consequences of Delayed Diagnosis of Laryngotracheal Stenosis

Case Reports in Otolaryngology ◽

10.1155/2014/325048 ◽

2014 ◽

Vol 2014 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Adam C. Nunn ◽

S. Ali R. Nouraei ◽

P. Jeremy George ◽

Guri S. Sandhu ◽

S. A. Reza Nouraei

Keyword(s):

Respiratory Symptoms ◽

Treatment Options ◽

Correct Diagnosis ◽

Delayed Diagnosis ◽

Rare Condition ◽

Laryngotracheal Stenosis ◽

Delay In Diagnosis ◽

Inflammatory Conditions ◽

Proper Interpretation ◽

Legal Consequences

Laryngotracheal stenosis (LTS) is a rare condition that occurs most commonly as a result of instrumentation of the airway but may also occur as a result of inflammatory conditions or idiopathically. Here, we present the case of a patient who developed LTS as a complication of granulomatosis with polyangiitis (GPA), which was misdiagnosed as asthma for 6 years. After an admission with respiratory symptoms that worsened to the extent that she required intubation, a previously well 14-year-old girl was diagnosed with GPA. Following immunosuppressive therapy, she made a good recovery and was discharged after 22 days. Over subsequent years, she developed dyspnoea and “wheeze” and a diagnosis of asthma was made. When she became pregnant, she was admitted to hospital with worsening respiratory symptoms, whereupon her “wheeze” was correctly identified as “stridor,” and subsequent investigations revealed a significant subglottic stenosis. The delay in diagnosis precluded the use of minimally invasive therapies, with the result that intermittent laser resection and open laryngotracheal reconstructive surgery were the only available treatment options. There were numerous points at which the correct diagnosis might have been made, either by proper interpretation of flow-volume loops or by calculation of the Empey or Expiratory Disproportion Indices from spirometry data.

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A Metabolomic Approach in Search of Neurobiomarkers of Perinatal Asphyxia: A Review of the Current Literature

Frontiers in Pediatrics ◽

10.3389/fped.2021.674585 ◽

2021 ◽

Vol 9 ◽

Author(s):

Marie Julie Debuf ◽

Katherine Carkeek ◽

Fiammetta Piersigilli

Keyword(s):

Complex Disease ◽

Perinatal Asphyxia ◽

Treatment Options ◽

Mortality Rates ◽

Current Evidence ◽

High Morbidity ◽

Severity Levels ◽

Ischemic Encephalopathy ◽

Metabolomic Approach

Perinatal asphyxia and the possible sequelae of hypoxic-ischemic encephalopathy (HIE), are associated with high morbidity and mortality rates. The use of therapeutic hypothermia (TH) commencing within the first 6 h of life—currently the only treatment validated for the management of HIE—has been proven to reduce the mortality rate and disability seen at follow up at 18 months. Although there have been attempts to identify neurobiomarkers assessing the severity levels in HIE; none have been validated in clinical use to date, and the lack thereof limits the optimal treatment for these vulnerable infants. Metabolomics is a promising field of the “omics technologies” that may: identify neurobiomarkers, help improve diagnosis, identify patients prone to developing HIE, and potentially improve targeted neuroprotection interventions. This review focuses on the current evidence of metabolomics, a novel tool which may prove to be a useful in the diagnosis, management and treatment options for this multifactorial complex disease. Some of the most promising metabolites analyzed are the group of acylcarnitines: Hydroxybutyrylcarnitine (Malonylcarnitine) [C3-DC (C4-OH)], Tetradecanoylcarnitine [C14], L-Palmitoylcarnitine [C16], Hexadecenoylcarnitine [C16:1], Stearoylcarnitine [C18], and Oleoylcarnitine [C18:1]. A metabolomic “fingerprint” or “index,” made up of 4 metabolites (succinate × glycerol/(β-hydroxybutyrate × O-phosphocholine)), seems promising in identifying neonates at risk of developing severe HIE.

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Evolution of treatment options for vein of Galen malformations

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.8.peds10231 ◽

2010 ◽

Vol 6 (5) ◽

pp. 444-451 ◽

Cited By ~ 53

Author(s):

Dhruv Khullar ◽

Ahmed M. I. Andeejani ◽

Ketan R. Bulsara

Keyword(s):

Mortality Rate ◽

Pediatric Population ◽

Treatment Options ◽

Mortality Rates ◽

Endovascular Embolization ◽

Outcome Data ◽

High Morbidity ◽

Vein Of Galen ◽

The Past ◽

Neurologically Normal

Object Vein of Galen aneurysmal malformations (VGAMs) continue to account for high morbidity and mortality rates in the pediatric population. Whereas in the past, mortality rates were nearly 100%, recent developments in endovascular embolization and improvements in neonatal care have improved prognoses. It is now possible that some patients can achieve normal neurological development following embolization of the VGAM. Access to the lesion can be gained via transarterial or transvenous routes. In this paper the authors review the pathophysiological characteristics of VGAM and discuss the evolution of treatment options. Methods A PubMed literature search was performed for vein of Galen malformation treatment options, beginning in the 1970s. A total of 22 papers were reviewed in full, and outcome data for 615 patients from 1983 to 2010 were compiled. Articles were reviewed if they focused primarily on the treatment of VGAM and reported outcomes for at least 5 treated patients. Results Of the 265 patient outcomes reported between 1983 and 2000, 200 received endovascular therapy. Of these patients 72% had a favorable outcome, and a 15% mortality rate was found. Microsurgery was found to have an 84.6% mortality rate. Furthermore, 76.7% of untreated patients died. More recently, endovascular embolization has become the mainstay of VGAM treatment. Of the 350 patients assessed between 2001 and 2010, 337 were treated endovascularly, mostly via the transarterial approach. Of these patients, 84.3% were found to have good or fair outcomes, and a 15.7% mortality rate was found. Neonates had the worst clinical outcomes following endovascular treatment, with a 35.6% mortality rate, whereas infants and children had significantly better outcomes, with mortality rates of 6.5% and 3.2%, respectively. Conclusions Endovascular embolization has considerably improved outcomes in patients with VGAM. In the past, the prognosis for patients with VGAM was dismal, and successful procedures were considered to be those that partially or completely obliterated the lesion, but did not necessarily improve the patient's symptoms. More recently, with the continued development and improvement of endovascular techniques, many patients are found to be neurologically normal on follow-up, and mortality rates have dropped substantially when compared with microsurgical treatment.

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Overview of Treatment Options for Critical Limb Ischaemia Patients

European Cardiology Review ◽

10.15420/ecr.2011.7.1.51 ◽

2011 ◽

Vol 7 (1) ◽

pp. 51 ◽

Cited By ~ 1

Author(s):

Frederic Baumann ◽

Nicolas Diehm ◽

◽

Keyword(s):

Treatment Options ◽

Critical Limb Ischaemia ◽

Treatment Modalities ◽

High Morbidity ◽

Limb Ischaemia ◽

Arterial Revascularisation ◽

Planning Treatment ◽

Treatment Concepts ◽

Peripheral Arterial ◽

Renal Origin

Patients with critical limb ischaemia (CLI) constitute a subgroup of patients with particularly severe peripheral arterial occlusive disease (PAD). Treatment modalities for these patients that often exhibit multilevel lesions and severe vascular calcifications are complicated due to multiple comorbidities, i.e. of cardiac and vascular but also of renal origin. These need to be taken into consideration while planning treatment options. Although CLI is associated with considerably high morbidity and mortality rates, the clinical outcome of patients being subjected to revascularisation has improved substantially in recent years. This is mainly due to improved secondary prevention strategies as well as dedicated endovascular innovations for this most challenging patient cohort. The aim of this article is to provide a discussion of the contemporary treatment concepts for CLI patients with a focus on arterial revascularisation.

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