United States

Background
Fournier’s Gangrene is a type of necrotizing fasciitis that is rare and life-threatening if not treated promptly. It commonly affects the penis, scrotum, and perineal tissue in men between the ages 50-60. With fascial necrosis progressing as fast as 2-3 cm/hour, early and rapid diagnosis and treatment is critical in decreasing mortality and adverse outcomes. This case report presents a 22-year-old male patient with Fournier’s gangrene and describes the symptoms, physical examination, treatment plan and expected clinical course. 

Case Presentation
A 22-year-old male presented to the emergency room with nausea, vomiting and an evolving painful sore on the posterior medial thigh. He had a history of uncontrolled type 1 diabetes mellitus and was noncompliant with medicine. The patient’s vital signs at admission were as follows: BMI of 35.0, blood pressure of 141/84 mmHg, pulse rate of 126 bpm, respiratory rate of 18/min, temperature of 38.11C, and oxygen saturation of 97% on room air. His labs were significant for elevated glucose at 330 mg/dL with mild diabetic ketoacidosis and increase WBC count of 25.1 x 103/L with 81.4% neutrophil. In the ER the patient was started on empiric antibiotic therapy and initial differential diagnosis include medication intolerance, gastroenteritis and diabetic ketoacidosis. Two hours later, his temperature rose to 39.33C and blood pressure dropped to 107/58 mmHg. After another 5+ hours, general surgery was consulted for irrigation and debridement. Surgery was performed; skin and necrotic subcutaneous tissue was excision extending until healthy tissue was reached. This excision spread from the posterior medial thigh just below the buttock to the deep perineum and anterior medial thigh. Post-operation antibiotic regimen was altered to include clindamycin, vancomycin and piperacillin-tazobactam. After narrowing antibiotics and continuous clean wound dressing changes, a vacuum-assisted closure (VAC) was applied to the wound in the OR on post-operative day three. On post-operative day five, patient was discharged with 14 day course of oral Augmentin and home-health care for assisted dressing changes.

Discussion
This unusual age caused delayed treatment and missed diagnosis originally. This case presentation might change emergency and surgical physician’s differential diagnoses and plan of action for younger patients that present with worrying symptoms of Fournier’s Gangrene. By expanding the age range and keeping different diagnosis wide, this can keep younger populations from slipping through and being eliminated on initial presentation. When it comes to Fournier’s Gangrene, time is essential and earlier diagnosis can be the difference in limb loss or even mortality.


AMA Research Challenge 2023

A Case Report of Fournier’s Gangrene in an Unusual Age Group

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Morgagni hernias are a rare type of diaphragmatic hernias usually presenting as a congenital defect within the diaphragm at the foramen of Morgagni with a prevalence of 2-3% [1,6]. Although rare, these hernias are usually seen in infancy or early childhood but are uncommon within adulthood. Due to the location of the foramen of Morgagni, abdominal contents herniate anteriorly within the chest with the majority in the right chest (91%) and less commonly in the left (5%) or bilateral chests (4%) [5]. Patients often present with pulmonary symptoms including chest discomfort, shortness of breath, or pneumonia [6, 9]. Although most cases are found incidentally, due to the concern for incarceration these hernias are recommended for surgical repair [4]. Approaches for repair include laparotomy, thoracotomy, open thoracoabdominal approach, laparoscopy, video-assisted thoracoscopy (VATs) [2]. However, there seems to be very limited reports of a combined laparoscopic and VATs approach for the repair of a diaphragmatic hernia. Here we present a case of 76-year-old male who presented to our emergency department with shortness of breath, chest tightness and pain with oral intake found to have a Morgagni hernia with herniation of the omentum, small bowel, and colon into the left hemithorax. Using a combined laparoscopic and VATs approach, we were able to successfully repair his hernia with minimal complications demonstrating the combined approach as a safe and viable option.

Combined Video-Assisted Thoracoscopic and Laparoscopic Approach to a Morgagni Hernia Repair

Pancreaticoduodenectomy (PD) is a complex procedure with a high postoperative morbidity rate. Postoperative complications include pancreatic leak, intra-abdominal abscess, postpancreatectomy hemorrhage (PPH), and others. One of the most feared complications is PPH. This is a case of a patient status post robotic-assisted laparoscopic PD who suffered Gastroduodenal Artery (GDA) stump blowout leading to PPH and cardiac arrest. The Common Hepatic Artery (CHA) was subsequently embolized to control the PPH. This is the first report in existing literature to report embolization of CHA as a method of PPH management in the setting of a replaced left hepatic artery.

Coil embolization of Common Hepatic Artery as management of postpancreatectomy hemorrhage following Pancreaticoduodenectomy 


Background:
Small bowel obstruction (SBO) is a life-threatening condition that typically presents with abdominal pain, distention, nausea and/or vomiting in a patient with a history of abdominal/pelvic surgery. SBOs are rare in virgin abdomens but can be seen in patients with internal hernias such as a left paraduodenal hernia (PDH). A left PDH is a rare anomaly of midgut rotation that often goes undiagnosed until presenting as a SBO in adulthood. CT imaging is the best diagnostic modality for visualization of a left PDH. Effective surgical management poses a challenge due to its rarity.
Case Summary:
This is the case of a 35-year-old male with acute onset abdominal pain and no history of abdominal or pelvic surgery. Classic CT findings were central in making the diagnosis of SBO secondary to a left PDH. Definitive surgical management was achieved via laparotomy.
Conclusion:
In patients with a virgin abdomen who present with signs and symptoms concerning for SBO, a high index of suspicion for PDH should be maintained. In these cases, diagnosis and treatment necessitate knowledge of typical imaging features and surgical techniques.

Left Paraduodenal Hernia: A Rare Diagnosis in a Common ED Presentation

Chronic Venous Insufficiency is a prevalent condition characterized by dysfunctional venous hemodynamics, resulting in a spectrum of clinical manifestations such as venous leg ulcers (VLUs). Herein, we report the case of a symptomatic 54-year-old male with bilateral VLUs persisting for four years despite adherence to compression therapy. Comprehensive ultrasound examinations revealed bilateral lower extremity venous incompetence and reflux. Shortly thereafter, multipronged interventions comprising radiofrequency endovenous ablation, ultrasound-guided sclerotherapy, and endovenous laser ablation targeted the venous insufficiency; however, the recalcitrant VLUs persisted despite symptom improvement. Exploratory imaging elucidated previously undetected bilateral perforators, as a post-surgical hemodynamic equilibrium might have decreased local blood stagnation and enhanced perforator perfusion. Compellingly, the follow-up interventions resulted in complete ulcer healing and symptom resolution. Altogether, our case underscores the collective impact of care continuity, meticulous spatiotemporal vascular mapping, and a patient-tailored approach to treat recalcitrant VLUs.

Undetected Perforator Vein Incompetence Responsible for Recalcitrant Leg Ulcers

Background: Adrenal myelolipomas are benign, lipid-filled masses containing elements of interspersed myeloid cells and comprise approximately 3-6% of all adrenal masses. Epidemiologically, adrenal myelolipomas affect both sexes equally and are often discovered in middle age. These masses are typically unilateral and variable in size, with macroscopic fat as a diagnostic feature on imaging. Notably, patients with congenital adrenal hyperplasia (CAH) are more likely to present with large bilateral myelolipomas, and, as such, patients presenting with these features should be evaluated for concurrent CAH.
Case presentation: In this report, a 34-year-old male presented to the emergency department following an alleged assault on his abdomen. During his trauma evaluation, he received a CT scan of the abdomen, demonstrating large bilateral adrenal incidentalomas with pathognomonic features of macroscopic fat (Hounsfield unit -90 to -120) consistent with adrenal myelolipomas. The left and right adrenal masses were reported at 20 cm and 14 cm, respectively. On further questioning, he endorsed a past medical history of CAH and noncompliance with glucocorticoid and mineralocorticoid therapy associated with a chronic history of abdominal discomfort. On physical exam, palpable bilateral abdominal masses at the levels of adrenals were appreciated. This patient received appropriate lab and imaging to confirm the diagnosis of bilateral adrenal myelolipomas. He successfully underwent bilateral open adrenalectomy, is currently on maintenance glucocorticoid and fludrocortisone therapy, and is doing well one year post-operation.
Discussion: Adrenal myelolipomas are benign masses of mature adipocytes and scattered myeloid cells. They are the second most common benign adrenal masses following adrenal adenomas. While they are usually found unilaterally and incidentally, they can present bilaterally and symptomatically, especially in CAH, a group of genetic enzyme deficiencies involving steroidogenesis. There are three working hypotheses explaining adrenal myelolipoma's prevalence in CAH: 1) Increased adrenocorticotropic hormone (ACTH) leads to constitutive stimulation of the adrenal cortices, resulting in hyperplasia 2) Stress, infection, and trauma induce metaplastic transformation of the reticuloendothelial cells in the adrenal gland and 3) Granulocyte stimulating factor recruiting mesenchymal stem cells and circulating bone marrow which adrenocortical adipocytes are derived from.
The size and presence or severity of symptoms are essential in guiding management. Larger masses (> 4 cm) more commonly present with mass effects and possible complications of hemorrhage and, therefore, are surgically excised. Smaller masses and asymptomatic cases can be followed yearly with imaging. For masses larger than 10 cm, as in this patient, midline laparotomy is preferred to the laparoscopic surgical approach.

Large Bilateral Adrenal Myelolipomas in a 34-year old Male with Congenital Adrenal Hyperplasia

Bilateral synchronous testicular tumors are a relatively uncommon occurrence, especially when they involve germ cell tumors of different histology. In this context, we present a compelling case report of a male patient who was diagnosed with bilateral synchronous germ cell testicular tumors, with one being a seminoma and the other a non-seminomatous germ cell tumor (NSGCT). The coexistence of two distinct histological types, seminoma and NSGCT, necessitates a comprehensive diagnostic approach to accurately identify and characterize each tumor. This underscores the importance of clinical history, physical examination, imaging techniques, and histopathological analysis to establish an appropriate diagnosis. Careful consideration must be given to factors such as tumor stage, histological subtype, and individual patient characteristics to determine the most suitable treatment strategy. Treatment options may encompass a combination of surgery, chemotherapy, and radiation therapy, tailored to each tumor's specific characteristics and the patient's overall health. By highlighting this unique case, we aim to underscore the significance of meticulous evaluation and accurate diagnosis when confronted with bilateral synchronous testicular tumors of different histology.


Exceedingly Rare Bilateral Synchronous Germ Cell Testicular Tumors With Different Histopathological Features

Tumor lysis syndrome (TLS) occurs when tumor cells lyse and empty their contents into the bloodstream. TLS can occur in response to therapy or spontaneously, and it is most commonly seen in high-grade lymphomas, acute leukemias, and other rapidly proliferating tumors. For multiple myeloma (MM), the risk of RLS is less than one percent, and the risk of spontaneous TLS is even rarer. Therefore, we present a rare case of spontaneous TLS in MM. This case met the 2004 Cairo-Bishop laboratory criterion as well as the clinical TLS criterion for diagnosis and was treated with rasburicase as per guideline recommendations. We hope to educate others on such a presentation especially given the emergent nature of treatment and the increasing incidence of this disease.

A Rare Case of Spontaneous Tumor Lysis Syndrome in Multiple Myeloma

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare autoimmune disorder related to aquaporin-4 IgG (AQP4-IgG) antibody. Sometimes, NMOSD patients don't have this antibody, called seronegative NMOSD. Extremely rarely, this condition presents as a paraneoplastic syndrome. This presentation illustrates a case of paraneoplastic AQP4-IgG seronegative NMOSD.

A Rare Case of Paraneoplastic AQP4-IgG Negative Neuromyelitis Optica Spectrum Disorder

Factors 8 and 9 play pivotal roles in the activation of factor 10 in the coagulation cascade. In acquired factor 8 inhibitor, IgG autoantibodies bind to factor 8 molecules causing a functional factor 8 deficiency. Incidence is exceedingly rare at one to four per million per year. We discuss a case of severe acquired factor 8 defiency and discuss the diagnostic and treatment approach.

Diagnostic and Therapeutic Complexities in a Case of Severe Acquired Factor 8 
Deficiency

Background
This case presented with symptomatic anemia shortly after being diagnosed in NOV/2021 with Invasive Ductal Carcinoma in the left breast T4N2M0. She had chest pain, dry cough, epigastric pain, nausea and generalized fatiguability 3 weeks prior, no past medical history, but significant positive family history for non-Hodgkin Lymphoma. However, finding compatible blood units was very challenging and transfusion dependency was developed. The main approach is to reach clinical stability when she can receive the first cycle of neoadjuvant chemotherapy.

Methods
A complete blood profile, hemolysis indicators, Hb electrophoresis, CT scan of the abdomen was required to evaluate the case and bone marrow aspiration, biopsy, and immunohistochemistry study. Numerous PRBC units were given to support the patient under close monitoring with oral prednisolone, cyclophosphamide, and IVIg.
Result
She shows a hemolytic pattern with hemoglobin level at 6.3g/dl, with mild neutrophilia, normal platelets, reticulocytosis, indirect hyperbilirubinemia, positive direct Coomb’s test and high serum ferritin at 1045ng/L . However, the haptoglobin was not detected, MCV was normal, LDH level was not elevated at 170 U/L, normal vitamin B12, liver enzymes, urea and electrolytes and uric acid,. Her blood group was A+, the HB electrophoresis showed HbA 97.5%, HbA2 2.5% and CT scan of abdomen revealed mild hepatosplenomegaly. The peripheral blood film shows Anisocytosis, neutrophilia and normal platelet, and bone marrow biopsy showed no malignant infiltration. An immunohistochemistry study shows that the malignant cells are positive for CK (Creatine kinase) and Mammaglobin while they are negative for LCA, CD20, CD3 and GCDFP15.

Conclusion 
Autoimmune hemolytic anemia (AIHA) is recognized relatively rare with solid tumors as a paraneoplastic syndrome. However, in this case due to her family history, lymphoma must be excluded by bone marrow biopsy and immunohistochemistry study. Patient did respond well to prednisolone nevertheless complicated by diabetes mellitus and gouty arthritis and received numerous units of packed red blood cells, her anemia was corrected and patient was fit to

A case report of autoimmune hemolytic anemia in a newly diagnosed patient with breast cancer Challenging transfusion dependency that interferes with neoadjuvant chemotherapy

Next from AMA Research Challenge 2023

Combined Video-Assisted Thoracoscopic and Laparoscopic Approach to a Morgagni Hernia Repair