United States

Background
An allergy to mammalian meat and its derived projects due to an IgE antibody to galactose-alpha-1,3-galactose. In the United States, almost exclusively due the tick bite of Amyblyomma Americanum, The Lone Star tick. The allergy to only be limited to red meat, but the epitope of the antigen has been increasingly found in an increased food. Amblyomma Americanum is found most prevalently in the Southeastern United States. 3-6 hours after eating red meat (e.g. beef, lamb), patients begin to suffer integumentary symptoms such as rash, hives, or swelling, GI symptoms such as vomiting or cramping, respiratory symptoms such as wheezing, SOB, stridor, throat clearing, or hypotension
Case Presentation
We report 3 cases of pediatric patients who present with only non-anaphylactic symptoms of Alpha-gal syndrome. A nine-year old female presented to the peds GI clinic with 4.5 year history of progressively worsening abdominal pain, emesis, and diarrhea with no relief from Prevacid or Lansoprazole. Normal PEx, CMP, CBC w/ diff., EGD, and negative celiac. A14 yo F presented to Peds GI clinic w/ 4 year history of diarrhea, nausea, and abdominal pain, uncontrolled by Ranitidine, Zantac, and Prilosec. Normal US, HIDA Scan PEx, CBC w/ diff, CMP, EGD, negative H. Pylori and pregnancy test. 6 yo M presented to Peds GI clinic with history of chronic, intermittent abdominal pain, constipation, and dyschezia. Eliminated gluten and dairy, which led to transient symptom relief, but did not persist. Normal PEx, CBC w/diff., CMP, TSH, negative celiac. 
Discussion
The patients observed in this study were of varying ages and had a different set of symptoms, whether it be emesis, diarrhea, or constipation, with the only commonality being abdominal pain
Regardless of their backgrounds, when these patients were found to have an elevated alpha-gal IgE test and to go a meat avoidance test, their abdominal pain and most of their symptoms globally resolved. Some of these patients had abdominal symptoms to foods such as ice-cream; carb moiety galactose-alpha-1,3-galactose is present in cow’s and goat’s milk, which can appear as a milk intolerance. These patients took on average at least 2-3 years to diagnose, given that they did not have delayed anaphylaxis.


2022 AMA Research Challenge

Three pediatric patients with atypical non-anaphylactic presentations of Alpha-gal Syndrome

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poster

Background: 
Pancreatic pseudocysts occur relatively rarely in children as sequelae of pancreatitis. Giant pseudocysts measure over 10 cm in diameter, and pseudocysts 20 cm or over have rarely been reported in children, and optimal management is not clearly defined. We present a case of a child with a giant pancreatic pseudocyst after idiopathic necrotizing pancreatitis, and discuss management and clinical course. 
Case Presentation:
A 12-year-old otherwise healthy male presented with 4-day history of abdominal pain radiating to back, constipation, and vomiting. He was found to have SARS-CoV-2 infection without respiratory symptoms and acute necrotizing pancreatitis. His clinical course was mild. He was treated supportively and discharged after a 5-day hospital course with clinical improvement. 
Three weeks afterwards, the patient developed progressive early satiety, postprandial vomiting, and weight loss. The upper abdomen was distended with a visible, palpable mass. MRI showed a mature pancreatic pseudocyst measuring 20 x 11.3 x 16.1 cm without significant solid internal debris. Endoscopic ultrasound examination (EUS) with cystgastrostomy creation was performed with lumen apposing metal stent (LAMS) placement, and 2.5 L of brown fluid was drained. The patient tolerated the procedure well with abdominal mass resolution during the procedure. The following day, his abdomen was soft and non-tender to palpation with symptom resolution. He was discharged with plan for repeat CT to assess pseudocyst resolution and endoscopic cystgastrostomy stent removal if drainage was complete in 1 month. 
Patient presented 3 weeks after cystgastrostomy with abdominal pain and vomiting. Repeat endoscopy was performed and found stent occlusion with necrotic tissue. He underwent necrosectomy and LAMS replacement with two plastic pigtail stents for prolonged drainage with symptom resolution. Patient is asymptomatic with plans for repeat imaging in 3 months and stent removal if drainage is complete. 
Discussion: 
This case illustrates unusual development of a symptomatic giant pediatric pancreatic pseudocyst after idiopathic necrotizing pancreatitis, requiring intervention rather than conservative treatment. EUS drainage by cystgastrostomy stent insertion is an accepted treatment though few pediatric gastroenterologists are trained to perform this. Therefore, patients and families must travel or transfer to centers with appropriate expertise and resources.

Unusual Giant Pancreatic Pseudocyst Development in a Pediatric Patient after Necrotizing Pancreatitis

Background:
Breast cancer is the second leading cause of cancer mortality, and the most common malignancy in women after skin cancer. Appropriate management of breast problems has significant implications for women’s health. Awareness of potential pitfalls in diagnostic work-ups is critical. In this case we demonstrate how cognitive bias impacted the clinical course of a patient with a breast mass.
Case Presentation:
A previously healthy 45-year-old female presents with weakness, hypertension, hyperglycemia, and a 7-kilogram weight increase within the past month concerning for Cushing’s syndrome. Serum lab studies showed elevated adrenocorticotrophic hormone (207 pg/mL) and cortisol (70.1 µg/dL). After dexamethasone suppression testing, serum cortisol levels remained elevated. 
Brain magnetic resonance imaging was negative for pituitary abnormalities. Abdominal computerized tomography (CT) showed bilateral adrenal hyperplasia. Chest CT performed for evaluation of dyspnea, showed a 2.9 cm anterior mediastinal mass. Core needle biopsy of the thymic lesion identified a T1N1 atypical carcinoid tumor. Subsequent sternotomy and thymectomy was performed for ectopic Cushing’s and plan was for clinical and for imaging surveillance.
A follow-up DOTATE PET CT performed two months later demonstrated a DOTATE-avid breast mass in the upper outer left breast. On ultrasound core needle biopsy of the left breast mass pathology reported triple negative T1N0 invasive ductal carcinoma. After 3-months of adjuvant chemotherapy with Taxotere and Cytoxan, follow-up PET CT showed the breast mass decreased in size, however a new left breast mass was identified. Ultrasound wire-localization was performed to facilitate surgical excision of left breast masses. Surgical pathology for both breast masses revealed neuroendocrine tumors. Subsequent, re-evaluation of the core needle breast biopsy specimen clarified the lesion was metastatic atypical carcinoid.
Discussion:
Despite a recent history of carcinoid tumor, metastatic disease was prematurely excluded from the differential. Our case highlights the importance of prior medical and surgical history in the appropriate diagnosis and management of mammary tumors. Additionally, it highlights the importance of accurate pathology in pre- and post-surgical disease management, and radiology-pathology concordance. Finally, our case reinforces the value of a broad differential in diagnostic work-up.

Atypical Mammary Mass in a 45-year-old Female

Background: 
Small bowel neoplasms account for less than 5% of all gastrointestinal malignancies. Leiomyosarcoma of small bowel is an extremely rare (1.2 cases per million per year) type of tumor arising from smooth muscle which is difficult to diagnose with vague signs and non-specific symptoms that occur late in the clinical course. Intussusception is characterized by invagination of the proximal segment of small bowel into the adjacent distal segment leading to obstruction and in adults it is usually due to a pathological lead point in the intestine such as neoplasm. Here we present a case of intussusception as an unusual presentation of a rare leiomyosarcoma of the jejunum and its valuable, minimally invasive surgical management. 
Case: A 60-year-old diabetic male presented with a three-month history of intermittent abdominal pain, melena, and weight loss. Abdominal CT scan revealed a solid mass leading to intussusception in mid-jejunum with mesenteric lymphadenopathy. Patient underwent diagnostic laparoscopy which confirmed a solid mass within the intussuscepted loop of small intestine with no evidence of metastasis. The involved loop of jejunum and its mesentery containing the tumor were laparoscopically resected, followed by primary intracorporeal anastomosis. Pathologic evaluation revealed a 7.5 cm x 5 cm tumor with clear surgical margins, high mitotic rate, extensive necrosis, and reactive mesenteric lymphadenopathy. The tumor demonstrated immunohistochemical reactivity to Smoothelin, Desmin, Vimentin, Ki-67 with 90% nuclei positivity and focal SMA, consistent with leiomyosarcoma. Postoperative recovery was unremarkable with minimal pain and only three days of hospital stay. 
Discussion: 
Leiomyosarcoma of the small bowel is an extremely rare tumor and often presents late with poor prognosis. These tumors are known for being radioresistant with a high propensity for local recurrence and metastasis. Hence, complete surgical resection is often associated with higher survival and quality of life. The unusual presentation of intussusception in this patient led to early diagnosis and prompt surgical intervention. Total laparoscopic resection of the tumor with intracorporeal anastomosis in this patient was safe, feasible, and offered minimal postoperative pain, short hospital stay, and fast recovery. 


Intussusception as a Rare Presentation of Leiomyosarcoma of Small Bowel

Abstract Title:
Is median arcuate ligament syndrome a differential diagnosis of epigastric abdominal pain?
Background
Median arcuate ligament syndrome (MALS) is caused by the compression of the celiac artery by the tendinous arch of the diaphragm at the T12-L1 level. It is a diagnosis of exclusion in patients with epigastric abdominal pain, weight loss, nausea, or vomiting. It is considered when the workup for epigastric pain is negative after baseline investigations of upper GI endoscopy, CT abdomen, etc. When the diagnosis is unclear despite these investigations, MALS needs to be considered as a differential diagnosis.
Case Presentation
A 32-year-old male patient presented with epigastric abdominal pain for 15 years, aggravated in the past two weeks. The abdominal pain was mainly post-prandial in occurrence with associated nausea. Upper GI endoscopy and ultrasound of the abdomen were non-contributory to arriving at a diagnosis. A CT angiogram suggested by the vascular surgeon revealed stenosis of the celiac artery for a length of 5.1 mm with arcuate angulation and a ‘hooked’ appearance. This was caused by a 5.2 mm thick median arcuate ligament. The patient subsequently underwent the
Ó 2022 American Medical Association. All rights reserved.
‘Median arcuate ligament release procedure’ by laparotomy. Post-operatively, the improvement in stenosis was confirmed with duplex ultrasound.
Discussion
The median arcuate ligament is the tendinous arch that connects the right and left crus of the diaphragm on either side of the aortic hiatus. Formed by the fascia covering the upper part of the psoas major muscle, the ligament is attached medially to the sides of L1 and L2 and laterally to the transverse process of L1. MALS is caused by the compression of the celiac artery by the median arcuate ligament. Current literature proposes two mechanisms for symptoms in MALS – mesenteric ischemia due to arterial compression and celiac nerve compression. MALS is more common in individuals with substance abuse or psychiatric disorders. A thickness of more than 4 mm of the median arcuate ligament and a celiac trunk compression persisting through end- inspiration are considered significant for diagnosing MALS. Treatment involves operative decompression of the celiac artery. It remains to be addressed through upcoming research on the benefit of surgery in MALS.

Is median arcuate ligament syndrome a differential diagnosis of epigastric abdominal pain?

Laparoscopic Management of a Rare Mesenteric Gastrointestinal Stromal Tumor

Background: 
Gallstone ileus is a rare form of bowel obstruction caused by the passing of a gallstone through a biliary-enteric fistula with subsequent bowel impaction. This fistula is usually created between the gallbladder and duodenum due to chronic cholecystitis. Patients typically present with signs and symptoms of bowel obstruction and radiological evidence of biliary-enteric fistula. Although accounting for less than 0.1% of all mechanical bowel obstructions, gallstone ileus is associated with relatively high mortality rates ranging from 12% to 27%. In addition to enterolithotomy, surgical management may include cholecystectomy and fistula closure, depending on the clinical presentation. Here we present a case of gallstone ileus which was laparoscopically managed by enterolithotomy only.

Case Presentation: 
A 74-year-old female with past medical history of hypertension and hyperlipidemia presented with worsening abdominal pain, nausea, and vomiting for two days. On physical examination, abdominal distension and generalized abdominal tenderness was appreciated. Initial laboratory results demonstrated serum lipase of 1,260 U/L. Abdominal CT revealed Rigler’s triad, consisting of pneumobilia, small bowel obstruction, and an ectopic calcified gallstone. The patient underwent laparoscopic enterolithotomy, during which the impacted gallstone was removed and the enterotomy was repaired primarily. There was evidence of significant scarring in the upper abdomen from a previous inflammatory process with no signs of acute infection. Pathological evaluation demonstrated a 3 x 2.5 x 2 cm cholesterol gallstone. Postoperative course was unremarkable, and the patient was discharged home two days after surgery.

Discussion: 
Surgical management of gallstone ileus has engendered controversy around whether to perform a cholecystectomy and fistula closure during the enterolithotomy, on a later date, or not at all. Furthermore, while the current standard is laparotomy, there are increasing reports of successful laparoscopic management. As represented in this case, laparoscopic management of gallstone ileus by enterolithotomy alone is a safe and effective alternative offering less postoperative pain, faster recovery, and lower morbidity.

Laparoscopic Management of Gallstone Ileus by Enterolithotomy alone

Background: Rib fractures are a common traumatic injury, occuring in 10% of patients with blunt chest wall trauma. Currently, medical management is the standard of care for rib fractures, including pain control, oxygen supplementation, ventilation if required, and pulmonary rehabilitation. Despite advancements in critical care and technology, medically managed patients continue to experience several acute and chronic complications. However, within the last 20 years, muscle sparing and minimally invasive techniques such as surgical stabilization of rib fractures (SSRF) have emerged, leading to improved outcomes in patients with rib fractures. Typically, SSRF is performed on the third through eighth ribs due to their contribution to chest wall stability, while the first, second, eleventh and twelfth ribs are not plated.
Case Presentation:
Here, we present the case of a 63 year old female presenting as a tier 2 trauma who sustained multiple rib fractures after falling from a horse. The patient was initially managed in the ICU medically with pulmonary care and pain control, but experienced refractory pain and difficulty mobilizing under this treatment regimen. The patient was offered SSRF to reduce the displaced lateral and anterior fractures and elected to pursue surgical treatment in order to shorten her recovery time. Of note, the patient was noted to have an anatomical variant in which the axillary artery was visualized above the 2nd rib. 
Discussion:
Patient specific risk fractures including overall bone health and comorbidities should influence treatment decisions when deciding between medical or surgical treatment approaches. However, in the past two decades, surgical stabilization of rib fractures, particularly in patients with flail injury and minimal comorbidities, has gained traction as a viable therapeutic alternative. Surgical stabilization of the second rib is considered more technically challenging due to the additional exposure required in the area and the surrounding vascular anatomy. Given the dramatic fracture pattern noted in this patient, the second rib was deemed the best target for initial plate placement, and resulted in a significant decrease in chest wall deformity and pain experienced by the patient in the post-operative period. 



Leaning Into The Future: Normalizing The Application Of Surgical Stabilization Of Rib Fractures.

Introduction/Objective
Inhaled halogenated compounds are commonly used as induction and maintenance anesthetic agents that are highly efficacious and can be easily administered through vaporization. Hepatotoxicity is a known side effect of halogenated anesthetics, most classically associated with halothane. Of the halogenated anesthetic class, sevoflurane is generally considered to have the lowest risk of hepatotoxicity with many sources describing it as a safe alternative to other halogenated anesthetics. In this report, we describe a case of sevoflurane-associated liver failure in a patient undergoing kidney transplantation that led to the subsequent loss of the donor kidney.

Case Presentation
A 65-year-old patient with stage 5 ESRD, congenitally malformed non-functional right kidney, and hypertension presented to our tertiary care center for kidney transplantation. The operation was 7 hours in duration and was without intraoperative complication. Anesthetic regimen included induction with propofol, fentanyl, and rocuronium and patient was maintained with sevoflurane for the duration of the operation. Five hours postoperatively, patient became increasingly somnolent, developed laboratory evidence of metabolic acidosis, and was transferred to the intensive care. Patient then developed a clinically evident coagulopathy with increased serosanguinous drain output that prompted emergent exploratory laparotomy. Two days later, patient returned to the OR for abdominal re-exploration. The donor kidney was dusky, edematous, without a doppler signal, and was subsequently explanted. Following explantation, patient had a prolonged and complicated hospital stay, but was eventually discharged to an inpatient rehabilitation facility on long term dialysis. 

Discussion
Acute liver failure (ALF) is a potentially-life threatening alteration in hepatic function. The development of coagulopathy and encephalopathy with evidence of hepatic dysfunction on laboratory evaluation are the minimum diagnostic criteria. The differential diagnosis of acute liver failure is broad and includes infection, congenital or genetic conditions, ischemia, and drug-induced etiologies. A high index of suspicion is required to make the diagnosis of drug-induced liver failure (DILF), particularly for drugs that are infrequently associated with hepatotoxicity, such as sevoflurane. Risk factors for developing DILF from sevoflurane include recent surgical procedure with sevoflurane anesthetic, previous exposure to sevoflurane, renal dysfunction, and immunocompromised status. Identification of the causative agent and discontinuing hepatotoxic medications are key components in the management of DILF along with supportive care.

Conclusion
We describe a case of acute fulminant hepatitis secondary to sevoflurane exposure in a patient undergoing renal transplantation which ultimately led to the loss of the donor kidney. Our study adds to the limited number of case reports describing possible sevoflurane-induced hepatotoxicity and reinforces the importance of close postoperative monitoring of patients following kidney transplantation. 


Sevoflurane Associated Liver Failure in Kidney Transplantation Patient

Spiradenocarcinoma is an extremely rare, highly aggressive malignant sweat gland tumor with an estimated prevalence of 0.005% of all cutaneous carcinomas. Due to its rarity, there are no currently established guidelines regarding therapeutic management of this malignancy, with limited data available on the epidemiological features, diagnosis, or prognosis. Historically, this malignancy has shown an inconsistent response to chemotherapy and radiation, leaving patients and clinicians with relatively no options for metastatic or unresectable disease. We describe a clinical presentation of recurrent spiradenocarcinoma with distant metastases in a 42 year old female with a 10 year history of a previously stable mass on her posterior shoulder. The mass underwent spontaneous transformation and began rapidly enlarging, which had prompted her to seek medical care. Surgical excision was performed and histopathology confirmed the diagnosis. Imaging showed sites of distant metastasis and palliative care was provided in the form of debulking surgery one year later when chemotherapy failed to halt the progression disease in her axilla. The patient reported improved quality of life at her surgical oncology follow up and is currently undergoing a novel multimodal therapeutic approach using surgery, chemotherapy, and radiation for treatment of her metastatic disease, which has shown promising evidence in reduction of previously identified metastatic sites.

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Three pediatric patients with atypical non-anaphylactic presentations of Alpha-gal Syndrome

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Unusual Giant Pancreatic Pseudocyst Development in a Pediatric Patient after Necrotizing Pancreatitis

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