United States

Abstract Title: Unveiling the Cholestatic Phenotype: A Case of Autoimmune Hepatitis with Severe Hyperbilirubinemia 

Background: Autoimmune hepatitis (AIH) is defined by clinical, laboratory, and histologic findings as a hepatitic syndrome rather than a cholestatic syndrome. Cholestasis in AIH is typically due to associated primary biliary cirrhosis or primary sclerosing cholangitis. However, 8% cases of AIH have unexplained cholestasis (the Cholestatic-phenotype). In this case report, we described a patient who presented with cholestasis but was later diagnosed with AIH, responding well to steroid treatment. 

Case Presentation: A 52-year-old woman presented to the emergency department with a 15-day history of yellowing of the eyes and urine, accompanied by fatigue and pale-stools, following a brief febrile illness. Initial treatment with empirical antibiotics and supportive care at a local health center showed no improvement. Her medical history was unremarkable except for a similar episode of jaundice 4 years prior. Physical examination was normal, but laboratory tests revealed normocytic normochromic anemia, markedly elevated bilirubin (total 27 mg/dl, direct 15 mg/dl, indirect 12 mg/dl), and liver enzymes (AST-239 IU/L, ALT - 93 IU/L). Further evaluation showed a positive antinuclear antibody (ANA) profile with antibodies against SS-A, Scl-70 and Jo-1, but negative for anti-LKM, anti SMA and AMA. Liver ultrasound revealed altered liver echotexture without strictures or other abnormalities. Liver biopsy demonstrated features of auto-immune hepatitis(Figure 1), with interface hepatitis, cholestasis, chronic inflammatory infiltrates and portal fibrosis. Based on the Revised original scoring system for AIH, the patient had a pretreatment score of 15 (Simplifed scoring system score of 8) ,confirming the diagnosis. Treatment with azathioprine and prednisolone was initiated, with subsequent follow-up showing a positive response. Bilirubin levels normalized within two months. 

Discussion: For patients with cholestasis, especially with elevated bilirubin and positive ANA profile, AIH should be one of the differential diagnosis. Even if the classic serological markers like anti LKM and anti-SMA are negative, biopsy is the key to confirm the diagnosis in patients with such atypical presentation. The standard treatment for AIH is immunosuppressive therapy using steroids and steroid sparing agent such as azathioprine. Steroids such as prednisolone reduces the inflammation acutely and azathioprine is used for long-term chronic therapy to maintain the remission without the fear of steroid dependence.

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Unveiling the Cholestatic Phenotype: A Case of Autoimmune Hepatitis with Severe Hyperbilirubinemia

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Background:
Peripheral artery disease is the narrowing or blockage of arteries in the peripheral circulation, primarily caused by atherosclerosis. Treatment may initially be an exercise program and a focus on reducing risk factors such as quitting smoking and treating comorbid conditions. The next step is revascularization procedures, often starting with an endovascular approach (balloon angioplasty with or without stenting) and proceeding to surgical interventions if endovascular approach fails or is contraindicated. Surgical procedures include anatomical bypasses, such as aortofemoral bypass grafting, and extra-anatomical bypasses, such as axillofemoral bypass grafting (AXFBG).

Case Presentation:
A 67-year-old female with HTN, HLD, CVA with residual L sided deficits, PAD s/p L fem-AT bypass with rGSV (2020) with subsequent L CFA endarterectomy (2020), L groin debridement (2021) and recent LLE angiogram at OSH (2/5/24) who presented with acute on chronic LLE critical limb ischemia and found to have necrotic left great toe. CTA with occlusion of L common and external iliac artery with possible dissection in L CFA with reconstitution in L SFA. Preoperative diagnosis was left lower extremity ischemic ulceration. The patient underwent left axillary artery to proximal superficial femoral artery (axillofemoral) bypass 2/27/24. Patient was
recovering well after surgery until post op day 3. Patient developed symptoms of post ischemic compartment syndrome. Patient had pain out of proportion on left leg, extreme tenderness to touch, worse pain with passive extension, and some swelling in the left lower extremity. Palpable pulses were unchanged from before the surgery. Left dorsalis pedis pulse was not palpable and not detected with doppler at baseline. Ultimately, vascular surgery recommended left above knee amputation, which was done on 3/11/24.

Discussion:
Post-ischemic compartment syndrome is a surgical emergency caused by increased
intercompartmental pressure in the lower extremity that develops following revascularization procedures for acute limb ischemia. Surgical revascularization can cause the muscles of the extremity to develop edema due to fluid extravasation or inflammatory responses following an ischemia-reperfusion injury, resulting in rapid increase in ICP. Common procedures that cause this are embolectomy or arterial bypass. Inadequate backflow, high serum CK level, positive fluid balance, and advanced-stage acute limb ischemia are predictive factors for developing compartment syndrome. This patient had an amputation instead of a fasciotomy because the limb was already not being perfused well. If she had a fasciotomy, it would not have healed well and could have put the patient at risk for infection.

Axillofemoral Bypass and Post-Reperfusion Compartment Syndrome: Case Report

Biodegradable Temporizing Matrices (BTMs) are temporary, biocompatible, and resorbable scaffolds designed to temporarily occupy a defect site, facilitating tissue regeneration and neodermal growth in a surgical wound or defect site while minimizing the need for extensive tissue transfers or permanent implants. Spiradenomas are mostly benign skin tumors developing from sweat gland cells that typically require surgical excision as treatment, which can result in scarring and functional impairment, especially in functional joint areas like the ankle. While the application of BTMs in small- to moderate-sized defects resulting from malignant resections in functionally critical areas has shown promising results in preliminary studies, the use of BTM within the context of resection margins is an emerging concept that needs to be further studied. 
This case report presents the successful application of a BTM in the reconstruction of a soft tissue defect following wide local excision of a spiradenoma on the right ankle of a 64-year-old male patient, demonstrating the value of these matrices as an essential reconstructive tool in the management of soft tissue defects following malignant resections.

Biodegradable Temporizing Matrix (BTM) in Small Scale Reconstruction of Malignant Resections in Functional Areas: A Case Report

Introduction
Surgical drains are crucial in postoperative management to prevent complications such as fluid collections and abscess formation. However, they carry risks like migration or erosion into adjacent organs, leading to serious consequences like gastric outlet obstruction. This case highlights the complexities of managing a surgical drain eroding into the duodenum following interventions for gallstone pancreatitis.

Case Description
A 76-year-old male with a history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease, and recent gallstone pancreatitis presented to the ED with persistent vomiting and intolerance to oral intake. Initial assessment showed tachycardia, hypotension, and hypoxemia. He had undergone a laparoscopic cholecystectomy converted to open subtotal cholecystectomy and pancreatic necrosectomy 50 days earlier, with a Jackson-Pratt (JP) drain placed for purulent fluid management. He presented with worsening symptoms, including abdominal pain, purulent JP drain output, and a 30-pound weight loss. Severe protein-calorie malnutrition was noted, and total parenteral nutrition (TPN) was started. Lab findings indicated anemia of chronic disease.
CT imaging revealed gastric dilation, duodenal narrowing, and wall thickening suggesting gastric outlet obstruction from postoperative sequelae or pancreatitis inflammation. Additional findings included esophagitis, walled-off necrosis, and infected collections around the pancreas. Endoscopies on days 3 and 10 identified severe esophagitis and JP drain migration into the duodenum, causing obstruction. The drain was repositioned, resolving the obstruction and allowing diet progression.
By day 14, an upper GI study confirmed no duodenal leaks, enabling a transition to a regular diet and discharge on day 16. 

Discussion
This case underscores the complexities of managing surgical drain complications following extensive interventions for severe gallstone pancreatitis. The patient's history and surgical interventions predisposed him to drain-related complications due to extensive tissue manipulation and inflammatory responses. Endoscopic interventions were crucial for repositioning the drain and resolving the obstruction, highlighting advanced endoscopic techniques' evolving role.
Despite advancements, drain migration remains a concern, emphasizing the need for vigilant postoperative monitoring and early intervention. The patient's malnutrition and anemia added complexity to his recovery, with TPN playing a crucial role in optimizing outcomes.
Recent studies advocate for minimally invasive techniques for necrotizing pancreatitis, showing improved outcomes and reduced complications. Early intervention in pancreatic fluid collections may improve outcomes without increasing morbidity, challenging previous delayed intervention paradigms.
This case emphasizes conservative management and interdisciplinary teamwork in achieving favorable outcomes. Standardized protocols for prevention, early detection, and optimal management are crucial, along with continued research to refine techniques and establish evidence-based guidelines for high-risk scenarios.

Complexities of Managing Surgical Drain Erosion into the Duodenum Post-Gallstone Pancreatitis

Background
Roughly 1500 children are born with spina bifida (SB) each year within the United States. Of that 1500 about 78% of them have fecal incontinence attributed to neurogenic bowel dysfunction (NBD). The development of fecal incontinence and chronic constipation have been shown to be caused by the loss of motor and sensory function to the gastrointestinal tract. NBD has been shown to be a major problem for children and adults with SB, significantly decreasing quality of life. The treatment of NBD is multifactorial and is aimed to provide appropriately timed defecation. The treatment for NBD should start conservatively with a bowel regimen. Once conservative measures are exhausted there are surgical options available including the Malone antegrade continence enema (MACE) procedure or cecostomy. The MACE procedure has several indications including chronic constipation, NBD, anorectal abnormalities, and Hirschsprung disease. The MACE procedure involves dividing the appendix from the cecum, mobilizing it, bringing it most commonly to the umbilicus, leaving two centimeters of intra-abdominal stoma followed by division of the appendicular artery, and fixation of the stoma to the abdominal wall. Common complications of the MACE procedure include stoma stenosis, incontinence, mucosal prolapse, small bowel obstruction, and need for revision.
Case Presentation
This report describes a 31-year-old male who presented to the emergency department with symptoms of peritonitis. Patient has a significant history of SB with NBD and neurogenic bladder, as well as a significant surgical history of the MACE procedure as a child. On presentation, the patient had a computed tomography that was highly suggestive of small bowel obstruction, which promoted urgent surgical intervention. Diagnostic laparoscopy was negative, except for purulent fluid found within the abdominal cavity. Later additional history taking and clinical correlation revealed the patient likely attempted a self-enema while intoxicated, leading to the creation of a false lumen and the subsequent development of peritonitis.
Discussion
This case report is the first reported adult case of incidental false lumen creation causing peritonitis following the MACE procedure. There is no documented literature showing what the next steps should be in the adult population following perforation of the MACE procedure. This case report is aimed to present a rare and unique complication of the MACE procedure in the adult population. Additionally, we aim to prompt the need for further research of using known alternates to the MACE procedure in the adult population. This report also shows the need for continued life-long follow-up in these patients. 


Peritonitis Following Incidental False Lumen Creation in a Patient with an Appendicostomy: A Case Report

Supernumerary breast tissue is ectopic breast tissue that communicates with the breast skin, nipple or areola. Approximately 5.6% of the population exhibits supplementary breast tissue. To date, the National Comprehensive Cancer Network does not provide monitoring and management guidelines for ectopic breast DCIS. 

This report describes a woman in her 60’s with a left supernumerary nipple positioned at the inframammary fold with a recently discovered 2-cm palpable anomaly that was confirmed as an intraductal papilloma on histology. Upon surgical excision of the mass, pathology revealed a low grade ductal carcinoma in situ, estrogen receptor positive and progesterone receptor positive. The patient’s case was presented at a multidisciplinary tumor board. She was ultimately recommended for radiation therapy, followed by anti-hormone therapy.


Presentation of Ductal Carcinoma In Situ in a Supernumerary Nipple

Background
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) is a rare condition first described in 19561. It is characterized by clonal plasma cell proliferation and peripheral neuropathy due to increased inflammatory and angiogenic mediators. Misdiagnosis as Acute or Chronic Inflammatory Demyelinating Polyneuropathy (AIDP/CIDP) often occurs due to overlapping neuropathic features. We present a rare case of POEMS syndrome initially misdiagnosed as Guillain-Barre syndrome(GBS), highlighting diagnostic challenges of this rare condition.
Case Presentation
A 44-year-old male presented with fatigue, lower extremity weakness, numbness, and tingling for 1-2 months. Previously, he experienced abdominal pain and tested positive for enteroaggregative E. coli (EAEC) diarrhea. Lumbar puncture revealed elevated protein and normal cell count in cerebrospinal fluid (CSF), leading to a presumptive diagnosis of GBS. Intravenous immunoglobulin (IVIG) was administered with mild improvement. However, after discharge, his lower extremity numbness and weakness worsened and developed numbness and weakness of the upper extremities. Additionally, he exhibited hyperpigmentation on both hands and feet and double vision without papilledema. He was admitted to the hospital with a tentative diagnosis of CIDP and was treated with intravenous solumedrol and plasmapheresis without improvement.
Further tests and imaging were conducted: Thyroid function test indicated hypothyroidism, blood tests revealed normocytic anemia, thrombocytosis, negative HIV and hepatitis B/C panels. Vascular endothelial growth factor (VEGF) was elevated to 929 pg/mL.Immunofixation detected an M spike with IgA lambda and kappa monoclonal proteins. Abdominal CT showed splenomegaly, and a skeletal survey was negative for any bone lesions. Bone marrow biopsy revealed 10-15% plasma cell myeloma with plasmablastic features and atypical megakaryocytic hyperplasia. A formal diagnosis of POEMS syndrome was confirmed. Genomic pathology shows a missense mutation of exon 17 of CSF3R at amino acid position 755, converting wild-type arginine to glutamine. 
Following the diagnosis, weekly treatment with daratumumab, cyclophosphamide, bortezomib, and dexamethasone (CyBorD) was initiated, leading to improvement. The patient’s VEGF decreased to 226 pg/mL(not normalized), thrombocytosis resolved, and lower extremity weakness improved. Despite the improvement, he remained unable to ambulate and was discharged to acute rehabilitation for further functional recovery.
Discussion
This case illustrates classic signs of POEMS syndrome initially mimicking GBS, diagnosed months after symptoms onset. POEMS syndrome should be considered in differential diagnoses for patients presenting with peripheral neuropathy and monoclonal gammopathy, especially when unresponsive to IVIG and steroids. Early recognition is crucial, as treatment for POEMS differs significantly from AIDP/CIDP, and delayed treatment can lead to progressive, debilitating neuropathy, paralysis, and ultimately, cardiorespiratory failure2.



Reference

1 Crow RS. "Peripheral neuritis in myelomatosis." Br Med J. 1956;2(5000):802-804.

2 Dispenzieri, A. (2023). POEMS syndrome. In S. V. Rajkumar & R. F. Connor (Eds.), UpToDate. 





Diagnostic Pitfalls: POEMS Syndrome's GBS Mimicry


Background: Pancreatic cancer (PC) accounts for 3% of all cancers in the United States and 7% of all cancer-related deaths. Here, we presented a metastatic PC case with an excellent response to FOLFIRINOX therapy. 
Case Presentation: In June 2014, a 62-year-old Caucasian male sought medical attention due to ongoing abdominal pain since May of the same year. An ultrasound scan followed by a CT scan of the abdomen confirmed the presence of a 4.2 cm mass in the pancreatic head, indicating obstruction in both the pancreatic and bile ducts. Multiple metastatic lesions in the liver and numerous peripancreatic lymph nodes were also detected. Biopsy specimens from both the pancreas and liver confirmed the presence of pancreatic adenocarcinoma with metastasis. He was a smoker for 20 years and smoked a packet per day. 
Due to the advanced stage of his metastatic pancreatic adenocarcinoma, surgical resection was deemed inappropriate. He commenced treatment with FOLFIRINOX (leucovorin calcium, 5-fluorouracil, irinotecan hydrochloride, and oxaliplatin) in June 2014. After three months of therapy with FOLFIRINOX, a PET (positron emission tomography) scan conducted in September 2014 revealed the size of liver metastasis reduced by around 50- 65%. In September 2015, an Endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) of the pancreas came out negative for malignancy, which implies a significant positive response to FOLFIRINOX therapy.
The following six years, he had multiple follow-up scans with CT chest, abdomen, and pelvis, and they showed no disease progression.
In May 2022, a CT scan of the chest, abdomen, and pelvis revealed a notable increase in size and number of pulmonary nodules in both lungs and the hepatic lesions. Following this, he was evaluated for the ONKURE trial for better response.
Discussion: FOLFIRINOX response rate is 18.2% in metastatic pancreatic cancer patients and a complete pathologic response following FOLFIRINOX treatment is extremely rare. It has been reported in 4.5–5.9% of patients. This case report represented a remarkable response to FOLFIRINOX therapy in an advanced metastatic pancreatic cancer patient.

A Case of Excellent Response to FOLFIRINOX Therapy in a Stage IV Pancreatic Adenocarcinoma Patient

• Abstract Title 
• A Fatal case of Diffuse Large B Cell Lymphoma Presenting with Bilateral Necrotic Renal Masses. 
• Background 
• A 48-year-old woman with a history of chronic inflammatory demyelinating polyneuropathy presented to the hospital for failure to thrive. She experienced rapid decompensation during two consecutive hospitalizations; No diagnosis was able to be elucidated prior to patient expiring. 
• Case Presentation 
• A 48-year-old female with questionable past medical history of SLE and chronic inflammatory demyelinating polyneuropathy (CIDP), treated with weekly intravenous immunoglobulins (IVIG), presents to the hospital due to difficulty with activities of daily living, including eating and bathing. Family endorsed a thirty-pound weight loss with progressive dysphagia. CT Head showed right tentorium cerebellar lesion and CT abdomen and pelvis showed necrotic bilateral adrenal masses. Left retroperitoneal lymph node biopsy showed a benign reactive lymph node. Completed a Positron emission tomography (PET) scan depicting intensely avid bilateral adrenal masses with retroperitoneal and pelvic lymphadenopathy. 
• Three weeks after discharge, she presented back to hospital with worsening lethargy, microcytic anemia with hemoglobin of 6.9, hypotensive due to adrenal crisis responsive to fluids and stress dose steroids. New diffuse lower extremities purpuric lesions noted, for which biopsy showed leukocytoclastic vasculitis. Admitted to Medical Intensive Care Unit for worsening hyponatremia after fluid resuscitation and was started on 2% hypertonic saline. She was downgraded after resolution of hyponatremia and hypotension. She was again upgraded due to worsening fevers to 103F and started on broad spectrum antimicrobials: meropenem, vancomycin and fluconazole. Flow cytometry studies were negative. A large filling defect in the suprarenal inferior vena cava was noted and was started on apixaban. Despite all measures, the patient ultimately developed worsening hypoxic respiratory failure, was converted to comfort care measures and ultimately expired. 
• Pathology indicated Diffuse Large B-Cell Lymphoma returned after the patient expired. Initial differential diagnoses for this patient included lymphoma with negative biopsy due to steroid use as outpatient, Castleman disease, Rosai Dorfman disease or unspecified autoimmune disease. 
• Discussion 
• This case involves a young woman with controlled neurologic disease and multiple rheumatologic diagnoses. She presented with bilateral necrotic adrenal masses leading to adrenal insufficiency, which complicated her condition. Despite undergoing biopsy, no single diagnosis or syndrome was initially identified to explain the constellation of symptoms, and 
• unfortunately, the patient passed away from acute hypoxic respiratory failure. Diagnosis was limited by unusual symptom presentation and negative first biopsy. 



A Fatal case of Diffuse Large B Cell Lymphoma Presenting with Bilateral Necrotic Renal Masses

Background: Moyamoya disease is a rare, progressive vasculopathy that causes narrowing of intracranial arteries such as distal branches of the internal carotid artery (ICA) and proximal components of the middle anterior artery (MCA) and anterior cerebral artery (ACA). One of the ICA branches involved in Moyamoya syndrome include the anterior choroidal artery, which may develop an aneurysm, or more rarely like in this case, a pseudoaneurysm that may rupture and lead to a hemorrhagic stroke. 

Case Presentation: The patient is a 24 year-old-woman with a past medical history of sickle cell disease who presented to our neighboring hospital with left sided numbness. The patient reported an associated headache, nausea, and vomiting prior to the episode that prompted her to seek medical attention. She was transferred to our hospital due to a basal ganglia hemorrhage found on CT. Upon arrival at our emergency department, her vitals were stable and she was somnolent but AAOx3. Neurological exam was remarkable for left side hemiparesthesia and associated mild left side hemiparesis with motor strength of 4/5 in left upper and lower extremities. Patient was admitted to ICU level of care and neurology and neurointerventional radiology were consulted for further management. Brain MRI was remarkable for right thalamic intra-axial hematoma. Subsequent brain MRA was recommended and showed occluded bilateral ACA and MCA suspicious for Moyamoya syndrome. Digital subtraction angiography (DSA) was then performed showing a 4mm pseudoaneurysm of the right distal anterior choroidal artery which underwent successful embolization.

Discussion: The association between Moyamoya syndrome and sickle cell disease in the pediatric population has been well established, however, current literature is limited in regard to this disease in the adult population. Although other reports have described distal anterior choroidal artery aneurysms in Moyamoya syndrome, seldom to none have described a pseudoaneurysm of this artery in the context of Moyamoya syndrome and Sickle cell disease. Thus, our case report highlights an uncommon finding of Moyamoya syndrome whilst raising awareness of Moyamoya syndrome in adults with sickle cell anemia.

A Rare Anterior Choroidal Artery Pseudoaneurysm Secondary to Moyamoya Syndrome in a Patient with Sickle Cell Anemia

Background

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma, with an increased risk associated with Human Immunodeficiency Virus (HIV) infection. In the United States, Burkitt lymphoma often involves intra-abdominal organs or lymph nodes. Here, we present an interesting and exceedingly rare case of Burkitt lymphoma involving the heart in an HIV patient.

Case Presentation

A 40-year-old male presented with a newly noticed swelling in his right axilla. He reported a 40-pound weight loss along with occasional fever, night sweats, and abdominal pain. Physical examination revealed a 4 cm subcutaneous mass without skin changes. Vital signs and initial lab results were unremarkable. CT scans of the chest, abdomen, and pelvis revealed a 7-cm right axillary mass, marked diffuse thickening of the gastric and small bowel walls, and a large right atrial filling defect.
An excisional biopsy of the axillary mass confirmed Burkitt lymphoma. HIV serology was positive, with a viral load of 229,000 copies/ml, and LDH was elevated at 503 U/L. Peripheral blood smear showed no atypical cells, and bone marrow aspiration, biopsy, and flow cytometry were negative for lymphoma involvement. The patient was diagnosed with stage IIIBE Burkitt lymphoma and started on chemotherapy, including cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, etoposide, and cytarabine.

After one month, a follow-up CT scan showed a significant reduction in the size of the right axillary and atrial masses, as well as in the thickening of the small bowel and gastric walls. Given the significant treatment response of the intracardiac mass, the diagnosis was confirmed as Burkitt lymphoma with intracardiac involvement.

Discussion

Intracardiac Burkitt lymphoma is an extremely rare malignancy, with only 22 reported cases to date. As with other forms of Burkitt lymphoma, HIV infection increases the risk of developing intracardiac Burkitt lymphoma. The symptoms are often nonspecific and cardiopulmonary symptoms such as palpitations or dyspnea can occur in other types of Burkitt lymphoma as well.

Given the highly aggressive nature of intracardiac Burkitt lymphoma and its potential to cause arrhythmias or pericardial effusion, which can lead to life-threatening conditions, early diagnosis is crucial. However, the extreme rarity of the disease and the nonspecific nature of its symptoms often lead to a low level of awareness among physicians, increasing the likelihood of missed diagnoses. Therefore, it is essential for physicians to maintain a high index of suspicion for intracardiac involvement when diagnosing Burkitt lymphoma.


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