United States

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. 


2022 AMA Research Challenge

Intussusception as a Rare Presentation of Leiomyosarcoma of Small Bowel

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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.

Spiradenocarcinoma: Challenges in Therapeutic Management of Rare Cancers

Sudden Dislodgement of Fecalith from Giant Jejunal Diverticulum causing Small Bowel Obstruction

Background: Benign pneumoperitoneum is a common finding after endoscopic procedures, associated with rates as high as 25% after PEG placement. However, tension pneumoperitoneum following PEG placement has not been reported in the literature. 
Case presentation: Here we describe the case of a 31-year old female with history significant for suspected myotonic dystrophy and tracheostomy placement who developed tension pneumoperitoneum shortly after PEG tube placement. She required immediate diagnostic laparoscopy during which no perforation or solid organ injury was discovered. After decompression of the intra-abdominal free air, she recovered well within 24 hours with no further sequella.
Conclusions: Tension pneumoperitoneum is a possible complication of PEG placement.

Tension Pneumoperitoneum after Percutaneous Endoscopic Gastrostomy (PEG) Tube Placement: A Case Report

Abstract Title:
A Case of COVID-19 Vasculitis Complicated by Hemophagocytic Lymphohistiocytosis

Background
Hemophagocytic lymphohistiocytosis (HLH) is an acute and rapidly progressive systemic inflammatory disorder characterized by cytopenia, excessive cytokine production, and hyperferritinemia resulting in multi-organ failure. Primary HLH is mainly due to genetic inheritance whereas secondary HLH is due to malignancy, infection, or autoimmune stimulus. COVID-19 can involve all organ systems, including the circulatory system, causing thrombosis and vascular dysfunction leading to cutaneous vasculitis. Severe COVID-19 infection causes cytokine hyperinflammation, triggering an intense immune response that may be the mechanism of HLH development. We chose this case to review the clinical picture of HLH development in a patient with a history of COVID-19 vasculitis and its severe outcomes.

Case Presentation
A 67-year-old male presented with bilateral lower extremity ulcerated lesions. Patient had COVID-19 infection seven months prior and was treated with steroids, antibiotics, and oxygen support without complication. However, one month later he developed bilateral lower extremity vasculitis. Antibody panel was inconclusive and skin biopsy of the lesions showed medium and small vessel vasculitis secondary to COVID-19 thrombotic vasculopathy. Subsequently oral steroid therapy, rituximab and IVIG infusion were given. However, the lesions progressively worsened and the patient went into septic shock requiring vasopressors and stress dose of corticosteroids. He developed pancytopenia with ANC nadir of 900, thrombocytopenia (PLT: 29k), LDH of 1300 U/L, ferritin 20,000 ng/nL. Bone marrow aspirate revealed mostly non-viable cells, scattered megakaryocytes, and scattered macrophages with hemophagocytosis. Based on the clinical history and morphological findings, hemophagocytic lymphohistiocytosis was diagnosed. The patient received filgrastim and dexamethasone, however, he clinically deteriorated and passed within four days of diagnosis.

Discussion
COVID-19 induces a wide range of clinical symptoms, including vasculitis. Hemophagocytic lymphohistiocytosis is a very rare condition with high mortality and should be considered in the differential diagnosis of patients with prolonged non-resolving fever and multisystem involvement. Due to its rarity and variable presentation, a high degree of clinical suspicion is warranted to diagnose HLH in the setting of COVID-19 vasculitis. We hope that presenting this case will result in earlier diagnosis and intervention to prevent long-term morbidity and mortality as seen in our case.

A Case of COVID-19 Vasculitis Complicated by Hemophagocytic Lymphohistiocytosis

Background
The differential for transaminitis in oncology patients includes ischemia, toxic ingestion, metastases, new malignancy, infection, vascular occlusion, autoimmune, and drug-induced etiologies. Temozolomide, an alkylating agent used first line with concurrent radiation to treat advanced gliomas is associated with improved patient overall and progression-free survival. Temozolamide has no hepatic metabolism and is rarely implicated in drug-induced living injury (DILI) (0.5% cases of a 1000 patient prospective study of DILI). Histologic assessment of temozolamide-associated DILI demonstrated bile duct loss and cholestatic or mixed liver injury pattern. 

Case Presentation
A 62-year-old male with a past medical history of urinary retention with a chronic foley, grade III astrocytoma status-post craniotomy with tumor resection undergoing chemoradiation presented to the ED after outpatient labs demonstrated new liver function test (LFT) abnormalities from a normal baseline 5-days prior. ALT=755, AST=300, ALP=166 and R-factor=13.4 consistent with hepatocellular liver injury. The patient endorsed drinking 2 standard drinks 4 times weekly for 20 years and denied tobacco use, drug use, tattoos, needle exposures, recent travel, or sick contacts. Workup was notable for negative alcohol, salicylate, and acetaminophen levels. Hepatitis A, B, and C serologies, EBV, HSV, VZV, CMV were negative. Anti-nuclear, anti-smooth muscle, and anti-LKM antibodies were negative. Ferritin and ceruloplasmin levels were normal. Right upper quadrant ultrasound demonstrated mild hepatomegaly and hepatic steatosis without focal masses and patent portal veins with forward flow and a normal gallbladder. Temozolamide was considered the likeliest cause of the patient’s transaminitis given the negative workup and 1-day time interval of LFT abnormalities from chemotherapy administration with rapid resolution (by >50% in 2 days and to baseline in 2 weeks) following therapy discontinuation. As such, chemotherapy was discontinued, and the patient continued outpatient radiation without issue.

Discussion
This case illustrates the importance of generating a wide differential for oncology patients presenting with transaminitis and an appreciation for idiosyncratic drug reactions. Alkylating agents like temozolamide while classically associated with cholestatic patterns of liver injury can also be implicated in hepatocellular liver injury such as in this rare case. However, DILI is a diagnosis of exclusion that requires a broad negative workup.


Intussusception as a Rare Presentation of Leiomyosarcoma of Small Bowel

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