Sickle cell disease (SCD) is a type of inherited disorder of hemoglobin characterized by recurring vaso-occlusive pain crises that affect many organ systems. Although it is uncommon, involvement of the orofacial region, such as the mandible area and jaw, can lead to a mandibular osteomyelitis, which can cause serious morbidity and diagnostic uncertainty. We describe a 17-year-old male Saudi patient with SCD who initially complained of bodily ache before developing fever, facial swelling, frontoparietal head swelling, and elevated inflammatory markers. The image resembled an odontogenic abscess along with frontoparietal head collection. However, the patient's clinical history, painful crises, elevated inflammatory markers, and high Hb S% along with further radiological study were all compatible with a mandibular osteomyelitis along with acute soft head syndrome. Vigorous supportive care with exchange transfusions to lower Hb S%, antibiotic support, and multidisciplinary consultation was all part of the management. The patient was steadily getting better, and after 1 month from starting management, all of the facial and head swelling had completely disappeared. In conclusion, this case highlights the presentation of two uncommon SCD manifestations: mandibular osteomyelitis and acute soft head syndrome in young SCD patient. Additionally, it demonstrated how crucial it is to keep mandibular crisis (osteomyelitis) as a differential diagnosis for orofacial pain in SCD patients and how lowering Hb S% is important for treating such instances.
Epstein-Barr virus-associated gastric cancer (EBVaGC) is a relatively rare clinical subtype. Despite its generally favorable prognosis, preoperative diagnosis remains challenging, and no established consensus exists regarding postoperative adjuvant treatment strategies for early-stage patients with high-risk pathological factors. This study reports a female case of early-stage EBVaGC of the lymphoepithelioma-like carcinoma subtype complicated with perineural invasion (a high-risk factor), and systematically explores the etiology, pathological features, key diagnostic points, individualized adjuvant therapy options, and prognosis of EBVaGC. The aim is to improve clinicians' understanding of this gastric cancer subtype, reduce its misdiagnosis rate, and accelerate the development of standardized diagnostic and treatment pathways. A case of a 60-year-old female patient admitted with "abdominal pain and distension for more than 1 year" was reported, and postoperative pathology confirmed EBVaGC. The clinical manifestations and diagnostic and treatment process were analyzed in combination with a review of relevant literature from recent years. Preoperative gastroscopic biopsy revealed poorly differentiated adenocarcinoma of the gastric mucosa. The patient underwent laparoscopic radical subtotal gastrectomy with lymph node dissection. Postoperative pathology confirmed EBVaGC. After symptomatic treatment, the patient improved and was discharged from the hospital. One month after surgery, no disease progression was observed and the SOX regimen was administered. The patient tolerated the treatment well, and the next cycle of chemotherapy is currently planned. EBVaGC lacks specific clinical manifestations, and laboratory findings may be within normal ranges at the time of onset. Reliance solely on conventional gastroscopy and histopathological biopsy can easily lead to misdiagnosis and inappropriate treatment. Epstein-Barr virus-encoded RNA in situ hybridization is the core diagnostic modality for definitive diagnosis. This subtype generally has a favorable prognosis. However, for patients with high-risk pathological factors such as perineural invasion and tumor invasion into the muscularis propria, standardized postoperative individualized adjuvant therapy should be administered based on tumor stage and molecular profiles.
Nonbacterial thrombotic endocarditis (NBTE) is a rare condition characterized by sterile fibrin-platelet vegetations on cardiac valves, typically associated with hypercoagulable states. Isolated tricuspid valve (TV) involvement during pregnancy is exceedingly rare, with no previously reported cases of fatal maternal and fetal outcome in the published literature. We report a 31-year-old woman (gravida 3) with a complex medical history including human immunodeficiency virus infection, chronic kidney disease, recurrent venous thromboembolism, and thrombocytopenia, who presented at 9 weeks of gestation with a large, mobile TV mass. Initial workup revealed elevated IgM antiphospholipid antibodies, a positive dilute Russell's viper venom time, negative blood cultures, and a concurrent pulmonary embolism. A transthoracic echocardiogram identified a 2.2 × 1.6 cm irregular hyperechoic mass on the anterior tricuspid leaflet. Despite therapeutic anticoagulation with enoxaparin and aspirin, the mass enlarged to 3.5 × 2.7 cm by 13 weeks of gestation. Following multidisciplinary discussion and shared decision-making with the patient, AngioVac-assisted percutaneous aspiration thrombectomy was performed at 14 weeks and 4 days of gestation. Histopathology confirmed sterile fibrin thrombi with histiocytic infiltration consistent with NBTE. Fetal demise was identified on postoperative day 2. Subsequently, the patient developed encephalopathy and massive hemoptysis, raising concern for catastrophic antiphospholipid syndrome. Despite aggressive resuscitation including venovenous extracorporeal membrane oxygenation, continuous renal replacement therapy, and maximum vasopressor support, the patient died. This is the first reported case of fatal maternal and fetal outcomes associated with TV NBTE during pregnancy. The case underscores the challenges of evaluation and management of cardiac masses in immunocompromised pregnant patients, the limitations of APS classification criteria in the peripartum setting, and the importance of a multidisciplinary cardio-obstetric approach.
Atrial fibrillation (AF) is the most common arrhythmia in clinical practice. Catheter ablation, particularly pulmonary vein isolation, reduces AF recurrence by 50% and is more effective than antiarrhythmic drugs. To better understand this condition, we present a case of a patient with persistent AF admitted to our department. The planned treatment strategy was a one-stop procedure combining catheter radiofrequency ablation with left atrial appendage occlusion (LAAO). Although AF, atrial flutter (AFL), and supraventricular tachycardia (SVT) exhibit distinct electrocardiographic characteristics, they often share overlapping triggers and common electrophysiological substrates within the atrial myocardium. AF is frequently initiated by ectopic triggers originating from the pulmonary veins, typical AFL is maintained by a macro-reentrant circuit around the cavotricuspid isthmus, and atrioventricular nodal reentrant tachycardia (AVNRT), the most common form of SVT, involves dual conduction pathways within the atrioventricular node. Transcatheter intervention provides an integrated therapeutic strategy that enables sequential targeting of these anatomically and electrophysiologically distinct substrates within a single electroanatomical mapping procedure, thereby offering a comprehensive curative approach while avoiding redundant interventions. However, the patient developed AFL and dual-pathway SVT during the procedure. Despite these challenges, radiofrequency ablation was performed successfully, followed by satisfactory LAAO.
Extracorporeal membrane oxygenation (ECMO) is increasingly utilized for refractory respiratory failure in sickle cell disease (SCD), yet registry data indicate in-hospital survival of only 40% in adults, with particular concerns regarding hemolysis, thrombosis, and bleeding processes already pathologically amplified in SCD. A targeted hematologic strategy using combined therapeutic plasma exchange (PLEX) and red cell exchange (RCE) may offer an alternative approach, but high-quality outcome data remain limited. We report a 37-year-old male with homozygous SCD (HbSS genotype) and glucose-6-phosphate dehydrogenase (G6PD) deficiency who presented with severe vaso-occlusive crisis that progressed to two cardiac arrests, shock requiring four vasopressors, severe acute respiratory distress syndrome (PaO2/FiO2 56 mm Hg), biventricular failure, and multiorgan dysfunction. Laboratory findings included marked lactate dehydrogenase elevation (7,169 U/L, > 25 × upper limit of normal) and severe thrombocytopenia (platelet count 37 × 103/µL). A multidisciplinary team considered ECMO but elected to pursue combined RCE and daily PLEX as salvage therapy. Seven sessions of daily PLEX combined with intermittent RCE reduced the hemoglobin S fraction from 73% to 18%. Vasopressor requirements decreased within 24 h, with complete independence by day 8. Inflammatory markers declined substantially within 72 h. The patient was extubated on day 10 and discharged from the intensive care unit on day 14 without neurological deficit. This case demonstrates that combined RCE and daily PLEX may represent a viable salvage strategy for post-arrest refractory cardiorespiratory failure in SCD, potentially sparing the need for ECMO in carefully selected patients. Prospective validation is essential.
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a relatively uncommon autoimmune disease, predominantly causing kidney or lung injury. Pituitary dysfunction is exceedingly rare in microscopic polyangiitis (MPA). Rupture or hemorrhage of renal artery aneurysms is also scarcely reported in AAV, especially in MPA. In this study, we describe a 68-year-old woman who presented with shortness of breath at rest, polydipsia, and polyuria. In addition to an increased p-ANCA titer (1:10) and the presence of anti-myeloperoxidase antibodies, T1-weighted magnetic resonance imaging of the head revealed pituitary injury. A diagnosis of MPA accompanied by pulmonary intestinal disease, central diabetes insipidus, and hypophysitis was confirmed. The patient responded to intravenous methylprednisolone and immunoglobulin. However, 1 week after admission, she complained of a sudden onset of sharp pain in her right waist area, accompanied by weakness, nausea, syncope, and hypotension. The hemoglobin level decreased from 95 to 47 g/L and abdominal computed tomography revealed a large perirenal hematoma surrounding the right kidney. The patient was immediately administered methylprednisolone pulse therapy (500 mg/day for 3 days) and cyclophosphamide (CTX; 0.6 g), followed by fluid resuscitation, blood transfusion, hemostatic therapy, and infusion of fresh frozen plasma. Arterial angiography demonstrated active bleeding of the interlobular artery of the right kidney, and selective arterial embolization was performed. Fortunately, she responded well to glucocorticoid and CTX therapy and did not relapse during the 5-year follow-up. Pituitary involvement accompanied by spontaneous rupture of a renal aneurysm is an extremely rare complication of MPA and has not been reported before.
Massive intra-abdominal tumors are associated with major perioperative anesthetic and surgical challenges due to respiratory compromise, hemodynamic instability, difficult positioning, fluid shifts, and postoperative complications. We present the case of a 65-year-old woman with a giant ovarian tumor weighing approximately 21 kg who underwent successful surgical resection following multidisciplinary perioperative management. A massive abdominal cystic lesion occupying most of the abdominal cavity, compressing abdominal organs, and associated with thoracic abnormalities was identified. Surgical treatment included hysterectomy, omentectomy, appendectomy, and cystectomy. The novelty of this case lies in the extreme tumor size, the severe positional hemodynamic instability caused by inferior vena cava compression, and the intraoperative confirmation of increased intra-abdominal pressure requiring immediate adaptation of anesthetic and surgical management. Despite the complexity of the case, the patient had a favorable perioperative course. This case highlights the importance of careful anesthetic planning, respiratory optimization, invasive monitoring, and multidisciplinary collaboration in the management of giant abdominal tumors.
Stroke is a leading cause of severe long-term disability, and foot drop is a common post-stroke gait impairment that may reduce toe clearance, increasing the risk of toe contacts and, in turn, trip-related falls. This case series evaluated the novel combination of transcranial direct current stimulation (tDCS), functional electrical stimulation (FES) to the tibialis anterior, and physical therapy training to improve minimum toe clearance (MTC) in individuals with chronic stroke. The rationale for this combined approach was that tDCS may prime the motor cortex and enhance neuroplastic responsiveness, while FES provides task-specific peripheral stimulation to the dorsiflexors during gait-related training, potentially increasing MTC and minimizing toe drag. Four individuals with chronic stroke participated in an 8-week treatment program with sessions twice a week. Assessments were conducted at baseline, 4 weeks, and 8 weeks. Participant 1 started with tDCS alone for 4 weeks, then added FES for the remaining 4 weeks. Participant 2 began with FES for the first 4 weeks, and tDCS was added for the remaining 4 weeks. Participants 3 and 4 received both tDCS and FES throughout the entire 8-week program. Participants 1 (58-year-old female) and 4 (42-year-old male) showed no active ankle dorsiflexion at baseline, while participants 2 (66-year-old male) and 3 (36-year-old male) had a grade of 2 out of 5 for ankle dorsiflexion strength at baseline. Toe clearance and functional outcomes were collected at each assessment. Participants 1 and 4 demonstrated no meaningful change in MTC, whereas participants 2 and 3 both showed modest improvements. Among these responders, MTC increased by an average of 0.63 cm. These findings suggest that combining cortical neuromodulation with localized FES and physical therapy may improve toe clearance in select individuals with chronic stroke. However, because tibialis anterior activation was not directly measured, it remains unclear whether the observed changes were due to improved dorsiflexor activation or other compensatory gait strategies.
Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive skeletal dysplasia characterized by disproportionate short stature, skeletal abnormalities, craniofacial and dental anomalies, and a high prevalence of congenital cardiovascular disease. Pregnancy in patients with EVC syndrome presents unique anesthetic challenges due to altered airway anatomy, abnormal spinal anatomy affecting neuraxial techniques, restrictive pulmonary physiology, potential cardiac dysfunction, and resultant hemodynamic complications. Evidence guiding anesthetic management for this population remains limited. We present the case of a 23-year-old pregnant patient with a heterozygous EVC2 gene variant, who presented for repeat cesarean section. After comprehensive pre-anesthetic evaluation, neuraxial anesthesia with spinal blockade was selected to avoid airway manipulation and minimize hemodynamic stress. Adequate surgical anesthesia was achieved with hyperbaric bupivacaine, and the patient remained hemodynamically stable throughout the intraoperative and postoperative periods without complications. This case highlights the importance of individualized anesthetic planning in patients with rare skeletal dysplasia undergoing cesarean section. Careful assessment of airway, spine, and cardiac function allows safe use of neuraxial anesthesia and may reduce perioperative morbidity to ensure best operative outcomes.
