AME Case Reports最新文献

Background: The Nuss procedure is a minimally invasive surgical procedure for treating pectus excavatum. Recently, its application has been extended to flail chest.

Case description: A 72-year-old woman presented to the emergency department with ventricular fibrillation caused by acute myocardial infarction. The patient underwent cardiac catheterization and coronary artery stenting following cardiopulmonary resuscitation (CPR). The patient was referred to Fukaya Red Cross Hospital owing to dyspnea caused by a flail chest following CPR. Being an elderly woman, the supporting tissues of the thorax had weakened, and she had a pronounced flail chest, a labored breathing pattern, and severe respiratory distress. Although there is the disadvantage that cardiac massage would become difficult, the cardiologist determined that cardiac function had stabilized after stent placement and that the likelihood of cardiac massage being necessary was extremely low, and therefore it was determined that repair using the Nuss method would be preferable. Two Nuss bars were inserted. Following the procedure, her respiratory condition improved, and she was discharged. Eight months later, the collapse of the anterior chest wall had resolved, and the Nuss bars were removed. However, the patient developed bilateral pneumothoraces post-removal. Thoracic drainage was performed, leading to improvement, and she was subsequently discharged.

Conclusions: This is a rare case of using the Nuss procedure to treat flail chest following CPR. Despite the occurrence of bilateral pneumothorax after Nuss bar removal, the patient's respiratory condition significantly improved, and she had a favorable prognosis.

Background: Acute myeloid leukemia (AML) harboring mixed lineage leukemia (MLL) rearrangement typically presents with high malignancy, poor remission rates, susceptibility to relapse, and a dismal prognosis. For these patients, achieving rapid remission followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an important strategy, underscoring the importance of choosing the initial induction regimen. Mitoxantrone liposome (L-MIT) is a modified drug formulation that enhances anti-tumor activity and reduces toxicity. At present, there is no report on the efficacy and safety of the MEA regimen consisting of L-MIT combined with etoposide and cytarabine in patients with AML with MLL rearrangement.

Case description: We described four patients with MLL-rearranged AML who received L-MIT combined with etoposide and cytarabine (MEA) as induction therapy, evaluating efficacy and safety after one treatment cycle. Three patients achieved complete remission (CR), including two who reached minimal residual disease (MRD) negativity, and another patient achieved partial remission (PR). Currently, three patients received allo-HSCT, while one was in consolidation chemotherapy due to advanced age and financial limitations. The main adverse event was manageable myelosuppression, with one case remaining febrile without signs of infection, while the other three experienced varying degrees of infections. Gastrointestinal side effects were mild, with no liver or kidney damage, obvious cardiac toxicity, infusion reactions, or skin discoloration.

Conclusions: The L-MIT-based MEA regimen showed promising efficacy with a favorable safety profile in patients with MLL-rearranged AML, suggesting that the MEA regimen could be one of the preferred therapeutic options for this population.

Background: Adrenocortical cancer is a rare malignancy with high risk of relapse and disease progression. The approaches to the treatment of relapse cases are not sufficiently clearly defined by current clinical guidelines.

Case description: Male patient O., born in 1967, has been suffering from the cancer of right adrenal gland since 2020. The patient underwent right-sided epinephrectomy in April 2020. Later he got several courses of chemotherapy with the mixed results. Then he underwent laparoscopic cytoreductive assisted excision of the recurrent neoplasm of the right adrenal gland on 04 April 2024, and received everolimus 10 mg per day postoperatively. There was evidence of recurrence of adrenocortical cancer of the adrenal gland with invasion of the fibrous capsule without signs of tumor growth at the edges of the resection. According to immunohistochemistry data, there was a decrease in phosphatase and tensin homolog deleted on chromosome 10 (PTEN) expression, an increase in Ki-67 expression, and a lack of programmed death-ligand 1 (PD-L1) expression. According to multispiral computed tomography data from May 2024 they found an increase in cells in the lungs by 2 mm, an increase in cells in the liver by 18 and 6 mm, an increase in paracaval lymph nodes by 12 mm. The given case of relapse and progression of the disease is of interest, because the removal of part of the liver during cytoreductive surgery and preservation of the kidney improve the quality of life. Due to limited resources hyperthermic intraperitoneal chemotherapy (HIPEC) and other methods of intraoperative chemotherapy were not applied.

Conclusions: Cytoreductive surgery is an important option for complex treatment in the advanced stages of adrenocortical cancer.

Background: Autoimmune enteropathy (AIE) is a rare autoimmune disorder characterized primarily by villous atrophy of the small intestinal mucosa. This article presents a case of adult-onset AIE featuring intractable diarrhea and severe malnutrition, along with a review of the existing literature to summarize its clinical characteristics, aiming to provide insights for the diagnosis and management of AIE.

Case description: A 60-year-old male was admitted due to intermittent abdominal pain, diarrhea, and weight loss. Laboratory tests revealed gliadin immunoglobulin A (IgA) (+) and immunoglobulin G (IgG) (+), but tissue transglutaminase IgA (-) and IgG (-). Endoscopy revealed that the mucous membrane of the small intestine was congested and edematous, the villi were atrophied and prone to bleeding after biopsy, and some of the villi exhibited a white mossy appearance. Pathologically, the villi of many segments of the small intestine presented diffuse and obvious atrophy, and there was lymphocytic infiltration in the intestinal glands. After exclusionary diagnosis, the patient was considered to have AIE. After effective initial treatment with corticosteroid, the patient exhibited recurrent symptoms and poor nutritional status, and eventually died of sepsis.

Conclusions: For patients with refractory diarrhea and intestinal villous atrophy, a comprehensive diagnosis should be made based on medication history, epidemiological factors, gluten dietary response, serological markers, and histopathological findings. Early intervention with corticosteroids combined with nutritional support is critical, while vigilance against severe adverse events such as life-threatening infections is essential.

Background: Fungemia has become an increasingly significant cause of morbidity and mortality in hospitalized patients. Sepsis is generally recognized as a hyperdynamic condition, commonly characterized by tachycardia as part of the systemic inflammatory response. However, in rare instances, patients may exhibit a paradoxical response in the form of bradycardia. This atypical presentation should prompt heightened vigilance and consideration of an underlying threat.

Case description: A case series of 3 patients who had unexplained bradycardia with septic shock. Their blood culture showed fungal growth. Fortunately, within a few days of starting antifungal therapy, their bradycardia showed marked improvement. This improvement supports the assumption of relation between bradycardia and fungemia. Data collections were extended continuously over a 24-hour period. To ensure the accuracy and reliability of the recorded heart rate, the average of the three lowest recorded values was calculated. This approach was employed to minimize the potential impact of any isolated, coincidentally bradycardia readings that might not reflect the patient's overall condition.

Conclusions: This study serves to underscore the paramount importance of maintaining a high index of suspicion for fungal pathogens in septic patients presenting with bradycardia, advocating for heightened clinical vigilance and a proactive approach to diagnostic and therapeutic decision-making. Furthermore, it underscores the urgent imperative for robust clinical research endeavors aimed at elucidating the efficacy and therapeutic impact of early antifungal interventions on the clinical outcomes in the context of sepsis.

Background: Proximal tibiofibular joint instability (PTJI) is a rare condition, particularly in total knee arthroplasty (TKA) revision, with only one prior case reported. This case highlights the importance of considering PTJI in patients with chronic lateral knee pain and instability following TKA and demonstrates a novel stabilization approach for managing this challenging condition.

Case description: A 73-year-old female with a history of multiple knee surgeries presented to our clinic due to increasing lateral knee pain radiating along the syndesmosis, aggravated by activities like squatting. Clinical examination revealed anteroposterior subluxation of the fibular head, suggesting PTJI. Radiological findings confirmed tibial component loosening, and the diagnosis of aseptic loosening with PTJI was made. Given her complex surgical history, a two-stage revision was planned. The first stage involved prosthesis removal, bacteriological sampling, and spacer placement. The second stage included revision TKA and proximal tibiofibular joint (PTFJ) stabilization using a Twin Tail Tight-Rope™ system and Arthrex endobutton, preserving joint mobility and restoring functional stability. A hinged revision knee prosthesis was then implanted. The patient had an uneventful recovery. At 1-year follow-up, the pain score was 1/10 and she achieved good mobility. Radiographs showed satisfactory implant positioning. The Western Ontario and McMaster Universities Arthritis Index (WOMAC) score of 15/96 indicated a successful outcome.

Conclusions: PTJI is rare and is not typically associated with TKA. This case report highlights a unique presentation of PTJI in a multi-revised TKA, raising concerns about the potential role of repeated proximal tibial resections in ligamentous instability of the PTFJ. The specific localization of pain and its resolution following PTFJ fixation support this hypothesis. Therefore, careful assessment of the PTFJ should be considered in TKA revisions, particularly in cases involving extensive lateral tibial resections.

Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm characterized by the abnormal proliferation of Langerhans cells, which can infiltrate various tissues throughout the body, leading to a spectrum of organ damage. This study aims to explore the clinical characteristics, imaging manifestations, and pathological features of the disease to enhance clinical diagnosis and understanding of such conditions.

Case description: This study reviews the clinical data of four patients diagnosed with LCH at The First Affiliated Hospital of Hunan University of Traditional Chinese Medicine. All patients met the diagnostic criteria outlined in the LCH guidelines by the American Society of Hematology, with Langerhans cells expressing CD1a, S-100, and Langerin (CD207).

Conclusions: LCH can involve various organs and systems, presenting with diverse clinical manifestations; in particular, rib Langerhans cell histiocytosis (RLCH) primarily manifests as asymptomatic or mildly painful bone swelling. Computerized tomography (CT) imaging of RLCH typically reveals localized masses and focal bone destruction, with or without surrounding soft tissue invasion. Conversely, magnetic resonance imaging provides a clearer assessment of lesion size and the extent of adjacent soft tissue involvement, offering advantages in guiding surgical excision. Diagnosis requires correlation with pathological and immunohistochemical results. For single-system, single-site (SS-s LCH), R0 surgical resection is feasible; however, single-system, multi-site (SS-m LCH) cases necessitate combined chemotherapy or targeted therapies. Overall, the treatment outcomes for this disease remain reasonably favorable.

Background: Acute bilateral basal ganglia hemorrhage is a rare and life-threatening condition, often associated with high morbidity and mortality. Timely diagnosis and intervention are crucial to improving outcomes. This report presents a case successfully managed with minimally invasive surgery (MIS).

Case description: A 44-year-old male, previously independent in daily activities with a medical history of hypertension, presented to the emergency department with severely altered consciousness [Glasgow Coma Scale (GCS) score 6]. Immediate intubation and admission to the intensive care unit (ICU) were initiated. Computed tomography (CT) angiography confirmed no evidence of vascular abnormalities or aneurysms. Tubular retractor-assisted microsurgical evacuation was performed to remove the hematoma and reduce intracranial pressure (ICP). The patient demonstrated neurological improvement, including spontaneous eye opening by postoperative day 3 and discharge from the ICU on day 10, highlighting the effectiveness of the intervention. He was subsequently discharged from the hospital on day 15 and transferred to a rehabilitation center for further recovery. Bilateral basal ganglia hemorrhage is rare, and its management often poses challenges due to high morbidity and mortality rates.

Conclusions: This case underscores the importance of prompt diagnosis and intervention in bilateral basal ganglia hemorrhage. MIS offers a viable therapeutic option that may enhance recovery while reducing surgical risks.

Background: There are few cases that report the presence of lupus anticoagulant and antiphospholipid antibodies between Evans syndrome and the presence of lupus anticoagulant. However, differentiating between primary and secondary causes of Evans syndrome remains challenging.

Case description: A 31-year-old female with a medical history of autism spectrum disorder, antiphospholipid syndrome (APS), complete systemic lupus erythematosus (SLE), and Evans syndrome was admitted for cholecystitis requiring laparoscopic cholecystectomy. In the setting of her acute illness, her thrombocytopenia worsened. Her hospital course was then complicated by the development of a left adrenal hematoma and venous thrombosis. Anticoagulation therapy was conservatively held due to concern for bleeding risk with the hematoma. However, given persistent thrombocytopenia, she subsequently underwent a platelet transfusion and was started on a 5-day course of intravenous immunoglobulin (IVIG). A venous Doppler ultrasound revealed a new occlusive deep venous thrombosis in the right axillary and brachial veins and occlusive venous thrombosis in the left cephalic veins while on the conservative anticoagulation regimen. This led to the immediate restart of anticoagulation therapy with close monitoring of coagulation labs. Her platelets improved Eltrombopag and IVIG up to 50,000 platelets/mcL.

Conclusions: This case aims to highlight the challenges of managing thrombocytopenia in a patient with multiple autoimmune conditions on opposite ends of the coagulopathic spectrum in the inpatient setting.

Background: Lung transplant recipients on immunosuppression are at increased risk for aggressive opportunistic infections, including invasive aspergillosis (IA). Distal septic embolization from angio-IA with involvement of the small and large bowel is extremely uncommon, and clinical manifestations of bowel ischemia and perforation may be masked due to immunosuppression.

Case description: We present herein the first reported case of peritonitis secondary to jejunal and cecal perforation secondary to angio-IA after lung transplantation (LT) in a 62-year-old male. The patient was admitted to the hospital for acute cellular rejection requiring high-dose immunosuppression. His course was complicated by respiratory failure secondary to IA, with resulting multiorgan system dysfunction during which time peritonitis was noted on examination and cross-sectional imaging demonstrated pneumatosis, portal venous gas, and pneumoperitoneum. The patient required emergent surgical intervention and underwent an exploratory laparotomy, jejunal resection, right hemicolectomy, and end ileostomy with colonic mucus fistula. Final pathologic analysis of resected specimens demonstrated angio-IA in both the jejunal and cecal segments with associated transmural ischemic necrosis.

Conclusions: Bowel perforation secondary to angio-IA is a very rare but serious complication after LT that is associated with high morbidity and mortality. Evaluation requires a high index of suspicion in the setting of immunosuppression and often concomitant extraintestinal pathologies. Early, aggressive surgical intervention is necessary.