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Background: Early diagnosis and treatment of under-recognized retroperitoneal fibrosis (RPF) are essential before reaching the poorly responsive fibrotic stage. Although most patients respond to medical therapy, relapses and unresponsiveness are common. However, open surgery in medically resistant patients is associated with major adverse clinical events. Methods: This is a single-centre longitudinal study of optimal medical therapy (OMT) vs. endovascular aneurysm repair (EVAR) in patients presenting with RPF to our tertiary referral vascular centre. Out of 22,349 aortic referrals, we performed 1,555 aortic interventions over twenty years. Amongst them, 1,006 were EVAR, TEVAR and BEVAR. Seventeen patients (1.09%) had documented peri-aortic RPF. Results: Out of the 17 RPF patients, 11 received OMT only, while 6 underwent EVAR after the failure of OMT. 82% (n = 14) were male, and the median follow-up was 62.7 months (IQR: 28.2-106). Nine (52%) had immunoglobulin G4-related disease (4 OMT vs. 5 EVAR). EVAR patients had 100% technical success without perioperative mortality. Furthermore, all the EVAR patients were symptom-free following the intervention. Pre-operative aortic RPF index (maximum peri-aortic soft tissue diameter/maximum aortic diameter) was higher in the EVAR than in OMT. However, there was a significant decrement in the aortic RPF index following EVAR (P = 0.04). Conclusion: We believe that when optimal medical therapy fails in RPF, EVAR provides a promising outcome. Further studies are recommended to establish the role of endovascular repair.
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Background: Skeletal dysplasia's cause significant neurological symptoms and disrupt the development of many bones and cartilages in the body. Skeletal dysplasia, although a common presentation in paediatric population, rarely presents in older age group. Case presentation: This case presents a unique incidental finding of skeletal dysplasia in a fifty-year-old male patient who presented with osteoarthritis. Eventual workup uncloaked the presence of cleidocranial dysplasia and spondyloepiphyseal dysplasia. The patient in this case had both dysplasias at the same time. Discussion: Cleidocranial dysplasia and Spondyloepiphyseal dysplasia are two uncommon autosomal dominant dysplasia's that are often diagnosed in early life and can have serious consequences, including death. It is critical to diagnose a child early in life. Radiology findings from a thorough skeletal examination aid in the early detection of numerous dysplasia's, which helps improving quality of life and allowing for effective treatment. Conclusion: The novelty of our presented case lies in the rare presentation of CCD and SED occurring concurrently at an older age with accompanying collateral abnormalities usually emerging more commonly in infants. Early diagnosis is thus essential for optimal management.
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Introduction: Mixed connective tissue disease (MCTD) is a rare autoimmune condition characterized by Scleroderma, Polymyositis, and Systemic Lupus Erythematous (SLE). Though a possible relationship between COVID-19 and autoimmune diseases has been recently reported, its pathophysiological mechanism behind flares in Lupus Nephritis (LN), a complication of SLE, remains unknown. Case presentation: A 22-year-old COVID-19 positive female presented with anemia, bilateral pitting edema, periorbital swelling, and posterior cervical lymphadenitis. Further inspection revealed lower abdominal striae, hepatosplenomegaly, and hyperpigmented skin nodules. Complete blood counts showed elevated inflammatory markers and excessively high protein creatinine ratio. Antinuclear antibody titers were elevated (anti-smith and U1 small nuclear ribonucleoprotein) and Rheumatoid Factor was positive. She was diagnosed with MCTD associated with a flare of LN. To control her lupus flare, a lower dose of steroids was initially administered, in addition to oral hydroxychloroquine and intravenous cyclophosphamide. Her condition steadily improved and was discharged on oral steroid maintenance medication. Discussion: We present a rare phenomenon of newly diagnosed LN, a complication of SLE, with MCTD in a PCR-confirmed COVID-19 patient. The diagnostic conundrum and treatment hurdles should be carefully addressed when patients present with lupus and COVID-19 pneumonia, with further exploration of the immuno-pathophysiology of COVID-19 infection in multi-systemic organ dysfunction in autoimmune disorders. Conclusion: In COVID-19 patients with LN and acute renal injury, it is critical to promptly and cautiously treat symptomatic flares associated with autoimmune disorders such as SLE and MCTD that may have gone unnoticed to prevent morbidity from an additional respiratory infection.
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Introduction: Acute appendicitis is one of the leading causes of acute abdominal pain and surgical emergency. Stump appendicitis is a known complication of appendectomy whereby a retained appendiceal tip serves as a nidus for recurrent bouts of inflammation. Nevertheless, full-blown appendicitis of the vermiform appendix after a prior appendectomy remains a diagnostic conundrum. Case presentation: A 45-year-old woman presented with a six-month history of right iliac fossa pain. Pertinently, she had undergone a prior open appendectomy twelve years ago. Further investigative workup revealed full-blown appendicitis, which was not attributable to a retained appendiceal stump. A subsequent laparoscopic appendectomy was performed, and the resultant specimen was sent for further evaluation, confirming the diagnosis of recurrent appendicitis. Clinical discussion: Acute appendicitis is one of the most common life-threatening abdominal surgical emergencies worldwide, with 300000 appendectomies performed annually in the United States alone. Stump and chronic appendicitis are two separate and exceedingly rare clinical entities that may present simultaneously and develop serious complications unless promptly recognized and appropriately managed. The present paper prompts the clinicians to distinguish amongst the two at the initial surgery in order to thwart further exacerbations. Conclusion: While stump appendicitis is a rare but well-characterized complication of a prior appendectomy, full-blown appendicitis of vermiform appendix remains elusive. It is therefore imperative to distinguish between a duplicated and a recurrent appendix at the initial operative procedure to facilitate optimal patient management.
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Introduction: Pancreatic pseudocysts remain a feared complication of acute or chronic pancreatitis and are often characterized by collections of fluids due to underlying damage to the pancreatic ducts, culminating in a walled-off region bereft of an epithelial layer but surrounded by granulation tissue. While fungal infections of pancreatic pseudocysts are rarely encountered, candida albicans remains the most frequently implicated organism. Case presentation: A 55-year-old male presented with pain in the left-hypochondriac region, accompanied by non-bilious emesis and nausea. Interestingly, the patient also tested positive for a COVID-19 infection. Investigative workup divulged enhancing pancreatic walls with a radiologic impression consistent with a pancreatic pseudocyst. An ultrasound-guided external drainage was performed; the drainage was conducted unremarkably, with the resultant fluid collection revealing the presence of Candida Glabrata. The patient was commenced on antifungal therapy and continues to do well to date. Discussion: Infectious ailments of pancreatic pseudocysts remain a widely known complication of acute pancreatitis. While it is rare, fungal infection is a crucial consideration for patients with pancreatic pseudocysts, especially in the context of a lack of an adequate response to antibiotics, deterioration, comorbidities, and immunocompromised states. Conclusion: Rapid identification of the microbe responsible for pancreatic pseudocyst infection is vital for time-sensitive treatment and a more rapid recovery, curbing associated morbidity and mortality.