United States

Background: 
Lacrimal gland tumors are a rare malignancy with an incidence of about 1 per 1 million people. Epithelial tumors are the most common form representing 50-60% of all lacrimal gland tumors with carcinoma ex pleomorphic adenoma (CEPA) constituting 10% of the epithelial tumors. CEPA is a malignant tumor that arises from benign pleomorphic adenomas. Clinical features include rapid growth causing eyelid and orbital protrusion often accompanied by pain. Mean survival after diagnosis is three years. CEPA may progress to intracranial spreading along with possible metastatic malignancy to the lung, chest, and bone. These malignancies are treated aggressively with a combination of surgical resection, chemotherapy, and radiation. 

Case Presentation: 
A 79-year-old male with a history of right eye exenteration after a childhood injury initially presented to his PCP with a mass on his right lower eyelid. A biopsy was obtained, and pathological results were initially suspicious for squamous cell carcinoma. He was referred to ENT who excised the lesion with intraoperative pathology confirming a negative margin. Pathology results from the excision were consistent with a 3 cm high-grade carcinoma ex pleomorphic adenoma of the lacrimal gland. Three weeks after resection, PET/CT scan showed local recurrence at the inferior resection margin and metastatic disease in a 1B cervical lymph node. The patient underwent combination chemoradiation therapy with complete regression confirmed by PET/CT. Nine months after completing chemoradiation, repeat PET/CT showed two new lung nodules concerning for metastatic disease. The patient did not wish to undergo further testing or treatment and decided to transition to comfort care only. 

Discussion: 
CEPA is an incredibly rare and aggressive tumor about which little is known regarding the preceding oncogenic events in the literature, indicating the need for further research to improve treatments.

AMA Research Challenge 2022

Carcinoma Ex Pleomorphic Adenoma: An Extremely Rare Tumor of the Lacrimal Gland

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Charles Lopresto, DO

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poster

Aggressive natural killer cell leukemia (ANKL) is a rare neoplastic malignancy, especially in pediatric populations with very few cases reported in the literature. It commonly presents with a rapidly declining clinical course and has a median survival of two months. We report the case of a 15-year-old female who presented with fever, hepatosplenomegaly, hemophagocytosis, and disseminated intravascular coagulation (DIC). The patient was ultimately diagnosed with ANKL but died after developing multiorgan failure and DIC prior to the initiation of any treatment. In this case report, we review and discuss the literature concerning the diagnosis and treatment of ANKL in pediatric patients.

A Case Report on Aggressive Natural Killer Cell Leukemia in a Pediatric Patient 

A Sugar of 4: An Unlikely Presentation of Glycogen Storage Disease


Acute liver failure (ALF) is a dynamic clinical condition manifested either by an abrupt or indolent onset of a liver-based coagulopathy and biochemical evidence of hepatocellular injury resulting from rapid deterioration in liver cell function. Acetaminophen overdose is the most common cause of ALF in Western countries and its incidence appears to be increasing. Most acetaminophen overdose patients recover with early N-acetylcysteine (NAC) therapy and supportive care, but regulatory actions are needed to prevent future cases. However, we must consider alternative causes of hepatitis which include HBV infection (7%), autoimmune hepatitis (5%), ischemic hepatitis (4%), and various other causes (5%). We present a 16 y.o. who presented in Acute Liver Injury (ALI) with transaminitis progressing to ALF (INR 2.5, NH4 119 umol/L, ALT 2,812/AST 6,222 U/L). In this case presentation, we aim to highlight the importance of obtaining a thorough history and including the practice of excluding alternative causes. While our patient denied intentionally overdosing on acetaminophen, an acetaminophen level of 24 mcg/mL at ~24 hours is significant. Without the concomitant acute EBV hepatitis (EBV PCR pos, EBV Capsid Ag IgM 96.4, EBV IgG neg), she likely would have experienced less severe liver injury without progression to acute liver failure. The appropriate initiation of intravenous N-acetylcysteine and fomepizole likely played a critical role in halting the progression by blocking further metabolism of acetaminophen by CYP2E1. Perhaps most importantly in delayed presentations, fomepizole inhibits c-Jun-N-terminal Kinase (JNK) which mitigates liver inflammatory responses. NAC may help other causes of ALI/ALF not caused by acetaminophen.

Accidental Acetaminophen Overdose?

Abstract Title: An Interesting Presentation of Cutaneous Mastocytoma
Background
Mastocytosis is a clonal proliferative disorder in which mast cells accumulate in various organs, most commonly the skin. Mast cell degranulation releases inflammatory mediators, which induce edema, pain, and pruritus. 

Case Presentation
A previously healthy, two-month-old female presented to the emergency department (ED) for what appeared to be a blistering skin infection. Prior to arrival, the patient experienced a transient episode of “beet red” flushing, cheek swelling, and diarrhea. Immediately after, the area on the right ankle was indurated and violaceous with erythematous borders.

The patient was born via vaginal delivery at 39 weeks. Of note, the patient’s mother has a history of genital herpes simplex virus (HSV) and took valacyclovir one month prior to delivery. She reported no active lesions at the time of delivery. 

In the ED, physical exam revealed an approximately two-centimeter ovoid area of mild hyperpigmentation with an erythematous border on the lateral aspect of the right ankle. Overlying this area were multiple vesicles in a cluster, the largest approximately six millimeters in diameter.

HSV and general wound culture swabs of the ankle, as well as blood cultures, were collected. A punch biopsy of the lesion was sent for pathological review, which ruled out cutaneous HSV infection. Additional staining was performed to evaluate for Langerhans’ cell histiocytosis and mastocytosis. Histologic features and immunophenotype confirmed the diagnosis of mastocytosis. 

Discussion
Given the maternal history and concern for potentially fatal complications, the thought was that the patient could have HSV infection. When HSV infection was suspected, a Tzanck smear could have been an alternative diagnostic strategy; however, the sensitivity and accuracy can depend on the interpreter and disease incidence. Because of the relatively low incidence in this geographic region, it is likely that a negative Tzanck smear would have required confirmatory testing via punch biopsy.

Another strategy could have been a wound swab with viral polymerase chain reaction for HSV, a highly sensitive and specific test, but the involved hospital is not large enough to make these assays economically viable. Ultimately, performing a skin biopsy allowed a rapid and definitive diagnosis of this patient.

An Interesting Presentation of Cutaneous Mastocytoma

Hemifacial Spasms in the Pediatric Population: A Case Series

Isolated Upper Gastrointestinal Findings in Pediatric Patient Found to Have Crohn’s Disease

Introduction

Multisystem inflammatory syndrome in children rarely involves severe coronary dilation, multiple readmissions, or occurrence after COVID-19 vaccination. We describe the case of a boy aged 5 years with all three of these unusual features.

Case Presentation

A previously healthy 5-year-old male was hospitalized with fever for 4 days, sore throat, myalgias, abdominal pain, conjunctivitis, and rash. Symptom onset was 56 days after acute COVID-19 illness and 16 days after receiving one dose of the Pfizer COVID-19 vaccine. Workup was notable for elevated inflammatory markers, mildly elevated cardiac biomarkers with evidence of SARS-CoV-2 infection, including both SARS-COV2-PCR and anti-nucleocapsid antibody. Patient was diagnosed with MIS-C with Kawasaki features, and demonstrated clinical improvement after treatment with IVIG, steroids and aspirin. Echocardiography during the initial hospitalization was reassuring. On day 7 of MIS-C illness, the patient was discharged with low dose aspirin and prednisone.

A day after completing his course of prednisone, on day 10 of MIS-C illness, patient presented to the emergency room again with fever, fatigue, rash, conjunctivitis, and dry, cracked lips. Labs were notable for thrombocytosis, leukocytosis, and elevated CRP, ESR, and d-dimer. He was diagnosed with recrudescence of MIS-C with features of Kawasaki disease. He was started on IV methylprednisolone 1 mg/kg, and low dose aspirin. Echocardiography on day 11 was significant for newly dilated coronary arteries with no aneurysms, and patient was promptly started on his second course of IVIG, from which he remained afebrile. Repeat echocardiogram was not notable for any changes. On day 14, patient was discharged with a prednisolone taper and low dose aspirin. Until day 20 of illness, he took 1 mg/kg of prednisolone twice daily.

On day 21, patient’s prednisolone was tapered to 1 mg/kg once daily, and he presented with fevers, dry cracked lips, conjunctivitis and abdominal pain on day 23. Inflammatory markers had elevated since previous discharge. Echocardiography was significant for severe dilation to his coronary arteries, especially his right coronary artery (RCA) (4.5 mm) and left anterior descending artery (LAD) (6.7 mm). He began treatment for refractory MIS-C and was started on infliximab and methylprednisolone. He was also given enoxaparin for his coronary artery dilation. On day 25, echocardiography continued to show severe coronary artery dilation, most significant with the LAD. His inflammatory markers and clinical course improved, and on day 28, repeat echocardiography showed no significant change. Patient was discharged on enoxaparin, daily aspirin, and a 30-day steroid taper. He remains asymptomatic, and most recent echocardiogram remains stable with slight improvement. A month after his most recent discharge, his inflammatory markers were found to be increasing, and his steroid taper was adjusted accordingly.

Discussion:

Our patient was admitted three times for MIS-C refractory to initial treatment with intravenous immunoglobulin, corticosteroids and anti-inflammatory agents. Readmission for MIS-C is not common. Among the 457 cases of MIS-C at our institution, this is the only patient to require 2 readmissions for MIS-C. He also developed giant coronary aneurysms. A diagnosis of MIS-C after COVID-19 vaccination is rare. In a study of vaccination in the United States, only one case occurs per million vaccinated children between the ages of 12-20 were noted to have MIS-C after vaccination. Whether or not the patient should receive his second Pfizer vaccine was questioned. Given current CDC guidelines, no future COVID-19 vaccinations are planned until the patient has at least made a complete recovery from his cardiac complications.

Multiple MIS-C Readmissions and Giant Coronary Aneurysm after COVID-19 Illness and Vaccination

Thyrotoxic Periodic Paralysis in a Caucasian Adolescent Male

Background: Femoral nerve palsy can cause gait deficits and knee instability. For its treatment, nerve transfers are preferred. However, this approach is only applicable for a limited window of time. For patients outside of this window or when nerve transfers are not technically feasible, musculotendinous transfers represent a viable alternative. 

Case Presentation: A 39 years-old female suffered a complete transection of the femoral nerve within the pelvis during proximal thigh schwannoma resection. Three years postoperatively she presented with antalgic gait, atrophy of the quadriceps, 0/5 strength in knee extension, and loss of thigh sensation. EMG revealed complete femoral nerve palsy with no recruitment of the quadriceps, sartorius, and iliopsoas muscles. 
The patient elected to undergo an adductor magnus, gracilis, and TFL musculotendinous transfer to the quadriceps tendon. This approach aimed to balance forces by harvesting a hip flexor and hip adductor while preserving hip adductor function by leaving the adductor longus muscle intact. 
Intraoperatively, the gracilis tendon was weaved into the vastus medialis and medial quadriceps tendon. The adductor magnus was weaved into the rectus femoris and vastus intermedius tendons. The IT band was weaved into the vastus lateralis and rectus femoris. By 11 months post-op she had 4/5 in right knee extension and hip flexion strength. Gait analysis revealed active recruitment of the gracilis, TFL, and adductors with improved walking speed and gait symmetry without requiring assistive devices.

Discussion: 
Given the extent, proximal location, and chronicity of her femoral nerve injury, nerve transfers were not feasible for this patient. Therefore, we opted for a musculotendinous transfer. We used multiple leg muscles to attain sufficient knee extension strength. To simplify surgery and recovery we selected easily mobilized muscles with long tendons innervated by her healthy obturator nerve. This technique also had a shorter recovery time as reinnervation is not needed. 
Nonetheless, to relearn basic motor patterns with a new set of muscles and innervations, the patient underwent rehabilitation involving six weeks of immobilization followed by passive rage-of-motion movements and active strength-building exercises. Our subject’s EMG findings, lack of falls, and functional gait improvements further demonstrate these rehabilitation methods’ efficacy.

Adductor Magnus, Gracilis, and Tensor Fascia Latae Transfer Restores Knee Extension and Stability after Femoral Nerve Palsy

Background
The use of matrices in combination with tissue expanders has become increasingly common in implant-based immediate breast reconstruction. Matrices are often divided into either biological acellular dermal matrices (ADM) or synthetic meshes. Synthetic meshes are made from knitted absorbable or permanent fibers. Surgical mesh functions as a scaffold to facilitate natural tissue healing. To further enhance the scaffolding effect, the synthetic mesh can be enhanced with growth factors to promote better tissue integration. We present a technique that incorporates platelet rich plasma (PRP) into a synthetic mesh to promote healing and aesthetic outcomes in reconstruction, revision, and cosmetic breast surgeries. 

Methods
A retrospective chart analysis of patients who underwent a reconstructive, revision, or cosmetic breast surgery using synthetic mesh enhanced with PRP was performed. All patients were seen by a single plastic surgeon ranging from 2020 to 2021. Details regarding patient past medical history, operative technique, and postoperative outcomes were gathered from provider clinical notes.

Results
Twenty-one (37 breasts) patients underwent reconstruction, revision, or mastopexy procedures using Durasorb mesh enhanced with PRP. One patient developed a seroma (4.8%). One patient developed a hematoma (4.8%) in the early postoperative period. Six patients (28.6%) had necrotic flaps. Furthermore, histological analysis of the biological mesh showed excellent tissue incorporation into the native tissue compared to normal surgical mesh.

Conclusion
The long-term absorbable Durasorb mesh enhanced with PRP has potential as temporary tissue reinforcement in patients undergoing breast reconstruction, revision, or cosmetic surgery. However, further investigation is warranted. By utilizing PRP, it allows for the release of additional growth factors that enhance tissue integration.

Next from AMA Research Challenge 2022

A Case Report on Aggressive Natural Killer Cell Leukemia in a Pediatric Patient

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