A rare case report: Left middle lobectomy in recurrent hemoptysis and chest infections of Kartagener syndrome patient.

INTRODUCTION: Kartagener syndrome is rare, with an incidence of 1 in 32,000 live births. It consists of a triad of bronchiectasis, situs inversus, and sinusitis. Normally, the middle lobe is part of the right lung, but due to situs inversus, the middle lobe is part of the left lung, making it prone to bronchiectasis and infections. CASE REPORT: We present a unique case of a 16-year-old adolescent with a known history of Kartagener syndrome who presented with recurrent chest infections and hemoptysis refractory to conservative management. He was diagnosed with bronchiectasis of the left middle lobe through a computed tomography (CT) scan and subsequently underwent a posterolateral thoracotomy and left middle lobectomy. This is a rare finding with limited literature available on such cases, to the best of our knowledge. DISCUSSION: Conservative treatment is usually the first line of approach. However, in cases of recurrent chest infections and hemoptysis, surgical management is considered to prevent the infection from spreading to the healthy lung and to avoid life-threatening complications when medical therapy fails. Surgical intervention, while more invasive, can provide a definitive solution and improve the patient's quality of life. CONCLUSION: Early diagnosis of Kartagener syndrome is crucial for determining the appropriate management course. In patients presenting with recurrent hemoptysis and chest infections, surgical resection is an effective treatment approach to prevent complications and enhance long-term outcomes.

Intrapulmonary schwannoma presenting as an asymptomatic lung mass: a case report.

BACKGROUND: Schwannomas are solitary well-circumscribed encapsulated benign tumors that exhibit Schwann cell differentiation, and arise directly from myelinated peripheral or central nerves. Although they are usually asymptomatic and found incidentally, schwannomas can cause symptoms due to compression of nearby structures which, depending on the location, can make clinical presentations widely variable. Despite their rarity, schwannomas have been documented in a number of locations including the limbs, cerebellopontine angle, posterior mediastinum, and, far more infrequently, the lungs. CASE PRESENTATION: In this article, we report an incidental finding of an intrapulmonary schwannoma in a 59-year-old Pakistani woman who was grossly asymptomatic upon presentation to the cardiothoracic surgery clinic. An [18F]fluorodeoxyglucose positron emission tomography/computed tomography scan revealed a lobulated soft-tissue lesion measuring 23 mm × 23 mm in the lower lobe of the right lung. A computed tomography-guided core biopsy of the mass was performed, which revealed a benign spindle cell lesion based on histopathological examination and immunohistochemical staining. The mass was surgically resected via a right lower lobectomy, and subsequently confirmed to be an encapsulated neoplastic lesion composed of well-differentiated Schwann cells. There were no short- or long-term complications, morbidities, or recurrences based on 1-year follow-up. CONCLUSION: This report underscores the predominantly asymptomatic nature of schwannomas and reemphasizes the efficacy of surgical resection as a safe and curative procedure for a tumor of this nature. Albeit very rare, intrapulmonary schwannomas can be considered a differential diagnosis when encountering solitary asymptomatic pulmonary nodules or masses.

Operating Ethically: A Review of Surgical Ethics in Pakistan and Recommendations for the Way Forward.

Medical ethics underpin the moral framework that delineates the professional relationship between physicians and their patients and thereby is an integral part of making patient-centric healthcare decisions. The concept of ethics is deeply embedded in the field of surgery as surgeons confront a myriad of dilemmas as a part of their routine, whether it be in a preoperative or postoperative environment. The current review aims to describe the state of surgical ethics in Pakistan, with the intent of encouraging dialogues about the ethical considerations relevant to the field surgery that will identify actionable areas for improvement. While most surgeons are aware of the traditional principles of ethics and their practice, their surgical and clinical decisions may fall short of these standards because of time constraints and prevailing cultural and religious beliefs and taboos. The rigorous application of ethical principles in areas of patient-related communication, such as consenting, trainee education, palliative and end-of-life care, and surgical innovation and research, will have significant implications for patients, surgeons, and society. Our review has identified the lack of formal bioethics education and insufficient oversight and ethical regulations to be at the core of inadequate ethical practices in Pakistan and has highlighted actionable areas to be addressed in the future.

Left Pulmonary Artery Aneurysm Secondary to Metastatic Lung Sarcoma: A Case Report.

Aneurysms are characterized by focal dilation of the blood vessel wall due to weakening. The involvement of two layers of the vessel wall is classified as a pseudoaneurysm while the involvement of all three layers is called a true aneurysm. Involvement of neoplastic lesions is rare, but the few reported cases have been associated with pulmonary artery pseudoaneurysms as opposed to true pulmonary artery aneurysms (PAAs). Our case of a true left PAA of a patient with metastatic sarcoma of the lung shows an association that has previously not been reported to the best of our knowledge.

A rare case of diffuse ectasia in the coronary arteries and proximal aneurysm in the left anterior descending artery: A case report.

INTRODUCTION: Coronary artery aneurysm is defined as dilatation of a segment of coronary artery, greater than 1.5 times the diameter of the largest normal coronary artery of the patient. Major cause of coronary artery aneurysm is coronary artery disease. Coronary artery aneurysm maybe an incidental finding during coronary angiography or it may present with symptoms including chest pain, myocardial infarction or even death. CASE PRESENTATION: We present a rare case of a 59-year-old gentleman, who had, previously, underwent multiple left heart catheterizations and percutaneous coronary interventions. He presented to us with symptomatic diffuse ectasia of the coronary arteries with proximal aneurysm in the Left Anterior Descending Artery. DISCUSSION: Coronary artery aneurysms are very rare with an incidence of about 0.02 %-0.04 % and a prevalence of 0.3 %-12 %. Right Coronary Artery is the principal site coronary artery aneurysms with a predominance of 83.8 %. The occurrence of multiple aneurysms is a very rare finding. About 73 % patients present with single aneurysms while only 27 % present with multiple aneurysms. Coronary artery aneurysms predispose to various complications including compression of the chambers, thrombosis and rupture. CONCLUSION: From the presented case it can be concluded that even though, conservative management is available, surgical repair is the gold standard treatment, especially in the presence of multiple, symptomatic and large aneurysms in order to avoid fatal ischemic complications.

Case report: Aorto-left atrial fistula-A rare complication of native aortic valve endocarditis.

BACKGROUND: Aorto-cavitary fistula is a rare complication of infective endocarditis. Multimodal imaging is commonly required to assess the severity and extent of infection because of the complex pathology of the valvular and paravalvular apparatus in endocarditis. CASE PRESENTATION: We present an unusual case of a middle-aged man with recent history of meningoencephalitis who developed infective endocarditis complicated by ruptured abscess in inter-valvular fibrosa between aortic and mitral valve resulting in free communication or fistula formation between aorta and left atrium. Patient underwent double valve replacement (aortic and mitral) along with repair of the aorta. CONCLUSIONS: Our case highlights recognition of this rare clinical presentation of aorto-left atrial fistula in infective endocarditis and the diagnostic role of transesophageal echocardiography in good clinical outcome with aggressive and timely management.

Foreign body aspiration presenting as a chronic lung disease: a unique finding in an elderly patient.

Foreign body aspiration is a serious clinical manifestation in the elderly and has a significant potential to cause life-endangering harm. In this unique report, we highlight the case of a seventy-year-old conscious male, who reported with complaints of chronic cough initially diagnosed as chronic bronchitis; however, on radiological examination, the infectious nidus was identified as a 5 cm long metallic nail in the right lower lung.

An unusual case of multiple aspergillomas in the right lower lobe of the lung: A case report.

INTRODUCTION AND IMPORTANCE: Aspergilloma is a pathological condition that affects the pulmonary tissues, and it is usually associated with Chronic Pulmonary Aspergillosis, Aspergillus fumigatus being the most common causative pathogen. The presence of multiple asperillomas in a single lung lobe is an unusual manifestation of Aspergilloma and therefore very little literature can be found regarding this. CASE PRESENTATION: Below we report a rare case of numerous pulmonary cavities containing aspergillomas, about 15-16, as an atypical presentation of chronic pulmonary aspergillosis, all of which were present in a single lung lobe of the patient. CLINICAL DISCUSSION: The most common clinical presentation of pulmonary aspergilloma is hemoptysis. It can manifest as a single aspergilloma or multifocal aspergillomas, which usually occur in unilateral lung cavities or bilateral lung cavities. The predominant site being the upper lung lobe, therefore, the appearance of several aspergillomas in a single lung lobe, with unusual manifestation in the lower lobe, is a very rare finding. Surgical resection is considered gold standard treatment in the form of lobectomy. According to various reports, the patient mortality rate after surgical intervention for aspergilloma is reported to be 0 %-22.6 %. CONCLUSION: From the discussed case report, the conclusion can be made that even though, surgical resection holds a certain amount of risk, since there no effective alternate treatment yet, hence surgical resection of the affected segment still remains the Gold-standard treatment in both single and multiple aspergillomas.

Coronary artery to pulmonary artery fistula: Catheter or scalpel? A case report.

INTRODUCTION: Coronary artery fistula (CAF) is an abnormal connection between coronary artery and a major vessel or cardiac chamber with left to right shunt having an incidence of 0.002 % in recent literature. Fistulous communication of coronary artery with pulmonary artery (PA) is a rare subtype and comprises of about 17 % of all the CAF cases. CASE PRESENTATION: We report a case of a middle-aged gentleman, known case of asymptomatic CAF for the last 20 years. He presented to us with 6 months history of chest pain on exertion. On coronory angiogram he was diagnosed to have a preexisting CAF of proximal LAD to main PA and severe coronary artery disease in left anterior descending coronary artery (LAD). He was managed surgically and underwent ligation of the fistula along with coronary artery bypass grafting (CABG). CLINICAL DISCUSSION: Management of CAF is medical, percutaneous or open-heart surgery. Due to rarity of the disease no international guidelines exists and treatment is controversial. Complications of CAF include endocarditis, early atherosclerosis, rupture, hemopericardium, pulmonary hypertension and myocardial ischemia, hence early correction is warranted. Our case emphasizes on the natural course of this rare disease and how to change management plan accordingly in the better interest of patient. CONCLUSION: Our case presents the natural course and management of a rare congenital cardiac disease. Surgery was chosen as an appropriate option due to CAD involving proximal LAD and concomitant coronary artery to PA fistula.

Atypical presentation of a rare gestational trophoblastic neoplasm: A case report.

A 34-year-old woman had a history of 3 miscarriages and presented with symptoms of an unproductive cough and rising serum beta-human chorionic gonadotropin (b-hCG) levels. There were no gynaecological symptoms. A diagnosis of Choriocarcinoma with pulmonary metastasis was made. In Pakistan, the incidence of Choriocarcinoma remains exceedingly rare, accounting for less than 0.3% of all gynaecologic tumours. Though rare, if managed appropriately, it has a reasonably good prognosis, as demonstrated by the outcome of the current case.

Radial artery coronary bypass grafting: Surgical outcomes of an unexplored innovation in a developing country.

OBJECTIVE: To explore postoperative outcomes, particularly prolonged length of hospital stay, in radial artery coronary artery bypass graft patients in a tertiary-care setting. METHODS: The pilot prospective cohort study was conducted at the Aga Khan University Hospital, Karachi, from September 2019 to September 2020, and comprised adult patients of either gender due to undergo coronary artery bypass grafting for coronary artery disease involving two or more vessels. The subjects were approached for the use of their radial artery as a conduit. Prolonged length of hospital stay was defined as postoperative stay >9 days. Multivariable logistic regression was used to identify independent predictors of the length of hospital stay. Data was analysed using SPSS 21. RESULTS: Of the 97 patients, 84(86.6%) were males. The overall mean age of the sample was 58.33±8.34 years. Mean length of hospital stay was 8.10±2.37 days, and 23(23.7%) patients had prolonged stay. Higher age was a significant predictor of prolonged hospital stay (p<0.05). Besides, 23(23.7%) patients developed acute kidney injury. There was no incidence of wound, infection or deep venous thrombosis, while 1(1.03%) patient had to be reopened due to excessive postoperative bleeding, and it represented the lone mortality. CONCLUSIONS: Patient age was found to be a significant predictor of prolonged hospital stay in patients undergoing radial artery coronary artery bypass graft, while almost a quarter of the sample was affected by acute kidney injury.

Pulmonary Adenoid Cystic Carcinoma Presenting Late With Intrapericardial Extension: Case Report.

Adenoid cystic carcinoma, also known as cylindroma, is one of the rare and unexplored clinical presentations of lung cancer, for which existing knowledge is scarce. This case report discusses a presentation of this tumor in the right lung, which subsequently extended to the left atrium through the right superior pulmonary vein. The extension of this rare tumor into the left atrium makes this case both uniquely distinctive and clinically relevant. The management strategy opted for this case was a right posterolateral thoracotomy and right pneumonectomy with partial resection of the left atrium. The desired outcome of this report is to shed light on the unusual clinical pathophysiology, register its atypical extensions, and navigate surgeons who may encounter this manifestation in the future.

Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature.

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS. CASE PRESENTATION: A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery. CONCLUSION: In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences.

Peer-taught virtual research workshops for surgical residents: Protocol for a novel and sustainable solution to improving surgical research in Pakistan.

OBJECTIVE: To generate a protocol describing the methodology for a study assessing the effectiveness of a comprehensive series of peer-taught online research workshops for surgical residents across Pakistan in terms of improving research-related knowledge and skills, and long-term research involvement and output. METHODS: The quasi-experimental study will consist of a series of six online research workshops conducted over Zoom for surgical residents across Pakistan. An online sign-up form will be circulated on social media to current surgical residents throughout Pakistan. Curricular content covered in the workshops will be developed in collaboration with experienced research faculty at the Departments of Surgery and Community Health Sciences at the Aga Khan University Hospital, Karachi. Facilitators of the workshops will be current surgical residents with a solid track record of research involvement at the hospital. Improvement in research-related knowledge and skills will be assessed through pre- and post-tests for each workshop. A post-series feedback form will assess satisfaction with the organisation, content relevance, peer-teaching and online delivery of the workshops. Long-term research involvement and output will be assessed by follow-up surveys at 6 months and 1 year post-workshop. All data-collection will be done via Google Forms. For ethical purposes, informed consent will be taken from participants prior to enrolment; data will be collected using a unique identifier number to maintain anonymity; and the only incentive provided to participants will be a Certificate of Completion for the research workshop series. RESULTS: We expect that surgical trainees participating in the series of six research workshops will demonstrate a highly significant percentage improvement (p<0.001) in the pre- and post-tests for each of the six core research competencies taught. On post-series feedback, we expect the majority of participants (>80%) to rate the overall organisation, effectiveness of online mode of instruction, and relevance to surgical training of the workshops as excellent/good. We also expect that most participants (>80%) would strongly agree/agree that peer-education is an effective model of teaching in research workshops. Finally, we expect statistically significant improvement (p<0.05) in research involvement and output in terms of publications as assessed in the 6 months and 1year post-workshop follow-up surveys. CONCLUSIONS: In light of the ever-growing need for academic surgeons in Pakistan, this research protocol details a comprehensive strategy for research capacity-building among surgical trainees across the country. By equipping trainees with the knowledge and skills to conduct high-quality research, virtual research workshops provide a novel, grassroots-level and sustainable solution for addressing the surgical research crisis in Pakistan.

Impact of COVID-19 on Cardiovascular Disease Presentation, Emergency Department Triage and Inpatient Cardiology Services in a Low- to Middle-Income Country - Perspective from a Tertiary Care Hospital of Pakistan.

Aims: To identify the changes in cardiovascular disease presentation, emergency room triage and inpatient diagnostic and therapeutic pathways. Methods: We conducted a retrospective cohort study at the Aga Khan University Hospital, Karachi. We collected data for patients presenting to the emergency department with cardiovascular symptoms between March-July 2019 (pre-COVID period) and March-July 2020 (COVID period). The comparison was made to quantify the differences in demographics, clinical characteristics, admission, diagnostic and therapeutic procedures, and in-hospital mortality between the two periods. Results: Of 2976 patients presenting with cardiac complaints to the emergency department (ED), 2041(69%) patients presented during the pre-COVID period, and 935 (31%) patients presented during the COVID period. There was significant reduction in acute coronary syndrome (ACS) (8% [95% CI 4-11], p < 0.001) and heart failure (↓6% [95% CI 3-8], p < 0.001). A striking surge was noted in Type II Myocardial injury (↑18% [95% CI 20-15], p < 0.001) during the pandemic. There was reduction in cardiovascular admissions (coronary care unit p < 0.01, coronary step-down unit p = 0.03), cardiovascular imaging (p < 0.001), and procedures (percutaneous coronary intervention p = 0.04 and coronary angiography p = 0.02). No significant difference was noted in mortality (4.7% vs. 3.7%). The percentage of patients presenting from rural areas declined significantly during the COVID period (18% vs. 14%, p = 0.01). In the subgroup analysis of sex, we noticed a falling trend of intervention performed in females during the COVID period (8.2% male vs. 3.3 % female). Conclusions: This study shows a significant decline in patients presenting with Type I myocardial infarction (MI) and a decrease in cardiovascular imaging and procedures during the COVID period. There was a significant increase noted in Type II MI.

Ruptured mycotic reverse saphenous vein graft aneurysm: A late complication of mediastinitis.

Reverse saphenous vein graft aneurysm (RSVGA) is a rare complication that occurs after coronary bypass grafting surgery. Mycotic aneurysm of reverse saphenous vein graft is even more rare. We describe the case of a 76-year-old man who underwent coronary artery bypass grafting 10 years back and had mediastinitis four to five months after surgery. He presented with chest pain in July 2017 and was diagnosed as NSTEMI. Angiography showed left main coronary artery disease (90%) with aneurysm of reverse saphenous vein graft to right posterior descending artery(RPDA). Emergent percutaneous coronary intervention (PCI) of the left main artery was performed as the patient suffered ventricular arrhythmia for which cardiopulmonary resuscitation (CPR) was done. After CPR, haematoma appeared on the left side of the sternum subcutaneously. CT scan of the chest was conducted which showed a pseudoaneurysm measuring 35 mm in the mid-segment of reverse saphenous vein graft (RSVG) to the right posterior descending artery (RPDA) with a surrounding mediastinal haematoma communicating with subcutaneous haematoma, so RSVGA to RPDA was coiled. As blood culture grew candida, antifungal medication was started. When the patient's condition stabilised he was discharged.

Wet labs: A useful tool in training surgical residents in a third world country.

•Wet labs are a useful, cost-effective and safe tool in teaching of Cardiothoracic Surgery residents.•In a third world country where advance real life simulators are not available.•It improves resident's tissue handling and surgical skills.•Allows faculty members to give continuous feedback to their residents.

A rare case of constrictive pericarditis as initial manifestation of paediatric anaplastic large cell lymphoma requiring urgent pericardiectomy.

Constrictive pericarditis (CP) is a rare end stage inflammatory disorder affecting both parietal and visceral pericardium leading to a right heart failure. Malignancy is the least common cause of CP. Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of all Non-Hodgkin lymphomas in children. Very few case reports have reported ALCL that is involving the heart and only two have been published involving pericardium but all were managed medically. We present an interesting case of an 11 year old child who presented with an effusive CP that required urgent Pericardiectomy for managing right heart failure. His histopathology was positive for ALK + ALCL.

Solitary fibrous pleural tumor. A rare and challenging case.

INTRODUCTION: Solitary fibrous tumor of the pleura (SFTP) is a rare tumor originating from mesenchymal tissue underlying the mesothelial pleural layer with only a limited number of reported cases. Benign and malignant SFTP usually appear as a well-defined, homogeneous, and rounded mass on imaging. Complete en bloc is usually the treatment of choice. CASE PRESENTATION: 44 years old gentleman presented with the unintentional weight loss and chest discomfort for 6 months. A CT scan of chest showed a 30 × 20 × 20 cm heterogeneously enhancing soft tissue mass involving the entire right hemithorax. PET scan showed a localized disease. Therefore, a complete right upper lobectomy and complete removal of mass was done, that resulted in complete expansion of the middle and lower lobe. The patient remains stable till date. Biopsy revealed a fibrous pleural tumor. CONCLUSION: Complete surgical resection of the tumor is usually sufficient, but there are reported cases with recurrence. Wedge resection for complete excision can be carried out for tumors arising from visceral pleura. Extra pleural excision can be done without chest wall resection in tumors arising from the parietal pleura.

When surgeons are rarer than resources - our experience with improving access to thoracic surgery in an urban setting: A special report.

With progressive globalisation enabled by technology, there is an increased interest in finding viable solutions to the myriad health problems faced by developing countries. In countries like Pakistan, occasionally the challenge is not a dearth of material resources but rather unavailability of expertise. The current paper was planned to share a model that was successfully implemented in the urban setting of Karachi, Pakistan, from 2012 onwards which significantly improved access to thoracic surgery for underprivileged individuals. Our model focussed on a qualified thoracic surgeon reviving a defunct thoracic surgical unit thereby optimising the use of resources already available in the community. The key to efficient outcomes was direct managerial control by the surgeon who first educated himself in the various processes involved. The model, with its challenges and solutions, has good potential foradaptation in other urban settings in the developing world..