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2022 AMA Research Challenge

The rare presence of both Anti-TIF1-y and PL-7 antibodies in a case of Dermatomyositis.

ENDO 2021

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#### Event Details
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 **Speakers**
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Ricardo Correa, MD, EdD
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Stuart M. Greenstein, MD
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Stephanie Strohbeen, MD
 
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Jessica Li, MD/PhD Candidate
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Charles Lopresto, DO

### Sponsor
AMA would like to thank Laurel Road for sponsoring a $10,000 grand prize for the winner of the AMA Research Challenge. Laurel Road is not involved in the judging or scoring process to select the winner. The winner will be announced on Dec. 7 during the AMA Research Challenge finals. Payment of the grand prize will be directly from Laurel Road. See the AMA Research Challenge Official Rules for terms and conditions.

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Welcome to the largest national, multi-specialty research event featuring research from 750 medical students, residents and fellows, and international medical graduates.


Background: Rib fractures are a well-known risk of cardiopulmonary resuscitation (CPR) in children and adults alike. While fractures of the ribs commonly occur during CPR, herniation of the lung through the intercostal spaces is rare. This clinical vignette details the case of an elderly woman who experienced subcutaneous emphysema and lung herniation following manual CPR.
Methods: Case Report
Results: A 72-year-old woman with a history of hypertension, hyperlipidemia, chronic obstructive pulmonary disease, gastroesophageal reflux disease, cerebral vascular accident with a left basal ganglia infarct, bladder cancer with urostomy, depression, anxiety, and colon cancer was seen following two cardiac arrests at another facility. While performing CPR during the second cardiac arrest, the patient endured a rib fracture, left lung herniation, and diffuse subcutaneous emphysema that extended from the face to the torso. The patient was intubated and transferred to another hospital. Upon arrival, bilateral chest wall blow holes were placed and
bilateral wound vacuum-assisted closure devices were applied. Following partial resolution of the subcutaneous emphysema, the patient was placed under general anesthesia and underwent a flexible bronchoscopy and left video-assisted thoracoscopy. Thoracoscopy revealed lung herniation and penetrating injury as a result of the broken ribs piercing the entrapped lung. The incarcerated anterior upper left lobe and part of the lingula were reduced and the defect in the ribs was closed with two V-Loc sutures endoscopically. The patient experienced no acute issues post-operatively.
Conclusion: In the rare case that a patient experiences lung herniation following CPR, video-assisted thoracoscopy and endoscopic reduction of the incarcerated lung offers a plausible surgical treatment.

A Case of Intercostal Lung Herniation as a Result of Cardiopulmonary Resuscitation

Herein, we describe the successful transplantation of a brain-dead male donor with a remote history of pectus excavatum (PEX) repair. On computed tomography (CT), the ascending aorta appeared to be in close proximity to the metallic structures located in the sternum from the repair. Before harvesting the heart, visual and digital inspections were used to confirm the space between the sternum and ascending aorta where severe adhesions were found near the lower sternum. After the pericardium was opened, the subsequent recovery of the heart was performed as usual. At one year post-transplant, the recipient continues to have normal graft function.

History of Ravitch Procedure for Pectus Excavatum, Not a Contraindication for Heart Procurement

Coarctation of aorta associated with a sinus venosus atrial septal defect is rarely seen in neonates. Such a life-threatening presentation requires special surgical consideration. Patient improvement depends on emergent repair of the coarctation in order to reduce cardiac workload and increase recovery of left ventricular function. Complete repair at the first surgery is ideal and generally indicated, but in some circumstances may not be possible -- staged repair may be needed. We present a case of a two-month-old male who was referred for evaluation of a heart murmur and hypertension. Echocardiography revealed coarctation of the aorta, bicuspid aortic valve, sinus venosus atrial septal defect, doming of the pulmonic valve, and left ventricular dysfunction with EF of 67%. Ventricular dysfunction complicates any cardiopulmonary bypass with circulatory arrest necessary for a single-stage median sternotomy therefore, the patient had to undergo a staged-repair. The initial procedure done was an extended end-to-end repair of the coarctation of the aorta through a left posterolateral thoracotomy. The sinus venosus defect was later closed through a median sternotomy. The case is discussed, and the literature reviewed.

Management of Coarctation of the Aorta, Sinus Venosus Atrial Septal Defect, and Left Ventricular Dysfunction in an Infant

Background: Penile fractures are a well-documented but rare urological emergency. Typically, they are caused by a blunt injury during sexual intercourse. The fracture occurs upon rupture of the tunica albuginea and is accompanied by a distinct “pop”. Historically, surgical exploration has been the preferred treatment to repair the tunica albuginea to prevent sequelae such as erectile dysfunction, Peyronie’s disease, and voiding dysfunction. Recent case reports have demonstrated good functional outcomes with conservative management. We report a case of penile fracture where surgical exploration was not performed.
Case Presentation:
A 30 year-old male presented to the emergency department 1 week post suspected penile fracture. He reported having intercourse and heard a “pop” with immediate detumescence of his erection. He denied any difficulty with voiding following this trauma and was still able to sustain erections with pain. Patient noticed penile bruising the same day as the trauma and noticed left groin bruising 2 days post-injury. An MRI of the pelvis on the day of presentation confirmed rupture of the tunica albuginea of the penis with associated hematoma. Due to the delayed presentation, a shared decision making approach was utilized to discuss surgery vs conservative management. The patient opted to defer surgery and was advised to suspend sexual activity for 4 weeks and wait 4-6 weeks for resolution of the hematoma and bruising. On follow up one month post-injury, the patient noted healthy erections, with no pain or curvature. The physical exam was improved with a significant decrease in hematoma. Three month follow up also demonstrated healthy erections with no pain, curvature, or voiding dysfunction.

Discussion:
The most common cause of penile fracture is blunt trauma to the erect penis which causes a disruption of the tunica albuginea and rupture of the corpus cavernosum. Penile fractures are a well-documented but underreported urological emergency. The most preferred treatment is typically surgical exploration with corporal repair to decrease risk of penile curvature and erectile dysfunction from corporal scarring.

This patient’s management with conservative measures shows that penile fracture may not always necessitate surgical exploration. His fracture has resolved, and he is currently able to engage in sexual intercourse and maintain erections for the duration of intercourse without pain. We report this case to add to the growing body of literature that conservative management of penile fractures in the appropriately selected patient is an acceptable treatment option.

Case Report: A Case of Penile Fracture Resolution with Conversative Management

A Case of Cocaine-Induced Goodpasture Syndrome

Background

Endocarditis continues to be a predominant cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Given that patients with SLE are at risk for both nonbacterial thrombotic endocarditis (NBTE) and infective endocarditis, the distinction between etiologies is critical for providing appropriate therapy; however, this continues to pose a diagnostic challenge.


Case Presentation

An 18-year-old female with a past medical history of SLE with hemodialysis-dependent ESRD due to stage IV lupus nephritis presented with a 6-month history of progressive fatigue, dyspnea, and tachypnea. She was afebrile, tachycardic, and had a 1/6 rumbling diastolic murmur at the left lower sternal border. TTE revealed severe mitral stenosis and thickened mitral valve leaflets with severely restricted mobility and a mobile, echogenic mass on the atrial aspect of the anterior leaflet. Because of her severe valvular disease and accompanying symptoms, the patient underwent a mitral valve replacement. Heavy growth of S. pyogenes from the mitral valve tissue specimen was demonstrated via ¬MALDI-TOF identification. Antibiotic susceptibility testing showed sensitivity to penicillin, and she was continued on IV penicillin G for 6 weeks following mitral valve replacement.


Discussion

NBTE is a non-infectious form of endocarditis that is most commonly associated with advanced malignancy (80%), followed by SLE. The modified Duke criteria can be helpful in discerning between infectious and non-infectious etiologies of endocarditis, and the absence of typical organisms on blood culture offers support for the diagnosis of NBTE. NBTE lesions on echocardiography are usually located at the basal, middle, or tip of leaflets and are heterogeneous in echogenicity, meanwhile, infective endocarditis lesions are more likely to occur at the line of leaflet closure and are more homogeneous in appearance. Although the prevalence of NBTE is reported to be 40-60% by autopsy, it is only definitively diagnosed in 4-6% of patients with SLE due to the predominantly asymptomatic disease presentation. Given that asymptomatic patients with SLE are not routinely screened for cardiac disease with echocardiography, it is possible that sterile, non-infectious endocarditis may precede infectious endocarditis in these patients. Increased awareness of endocarditis in patients with SLE is paramount as early diagnosis and treatment reduce mortality.

Concomitant Infectious and Noninfectious Endocarditis in Systemic Lupus Erythematosus

COVID-19 Vaccination and Coagulopathies in the Midst of a Pandemic: A Double-Edged Sword

Background
Epithelial ovarian tumors such as ovarian serous cystadenoma make up approximately 19% of pediatric ovarian tumors and have a low occurrence rate in adolescent females. Ranging in size from 1 to greater than 30 cm, small ovarian serous cystadenomas, 1-3 cm, are typically incidental findings. Larger tumors often present with vague symptoms such as pelvic discomfort and bloating. 

Case Presentation
A 15-year-old Caucasian female presented to the emergency department via helivac following a vehicle accident in which the vehicle flipped and landed on her abdomen. She was transported to the nearest hospital, where a CT showed fractures at cervical level 7 through thoracic level 1. She was airlifted to the emergency department. On arrival, she had total paralysis of bilateral lower extremities. She was taken for a laminectomy and facetectomy. Following surgery, her abdomen was distended, which was new after her accident, according to family members. A CTA chest, abdomen, and pelvis revealed a 20-centimeter by 17-centimeter by 8.6-centimeter thin-walled water density abnormality (20 Hounsfield units) in the lower part of the pelvis and lower abdomen in contact with the urinary bladder, which displaced the uterus to the left side and compressed the distal abdominal aorta and both ureters. Pelvic ultrasound demonstrated a large unilocular anechoic cystic mass with thin walls which occupied the pelvic cavity entirely. The gynecologist performed a laparoscopic cystectomy and drained 1750 milliliters. Pathology soft tissue specimen measured 19 centimeters by 15 centimeters, and the thickness ranged from 0.1 to 0.5 centimeters. The final diagnosis was a benign left ovarian serous cystadenoma.

Discussion
Epithelial ovarian tumors such as ovarian serous cystadenoma make up approximately 19% of pediatric ovarian tumors and have a low occurrence rate in adolescent females. Ovarian serous cystadenomas are usually incidental findings. The sudden change in the patient's abdominal wall shape is likely due to the loss of intervention to her abdominal muscles secondary to spinal cord injury. It is unusual for an ovarian tumor of large size to be asymptomatic and subsequently discovered in an adolescent female in the setting of trauma.


Incidental finding in trauma setting of large ovarian serous cystadenoma in an adolescent female

Metastatic skin and breast nodules as first sign of gynecologic malignancy

Background 
Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a delayed type IV hypersensitivity reaction. It is an exfoliative mucocutaneous disease often caused by antibiotics or antiepileptics. Etiology of this disease is thought to be due to drug-specific CD8+ cytotoxic lymphocytes. These lymphocytes exocytose perforin and granzyme B, creating channels in the cell membrane & activating caspases, leading to apoptosis. Exact pathophysiological mechanisms are still speculative, but it is thought to be due to the interaction/binding of drug-associated antigens with MHC type I or cellular peptide, inducing the formation of an immunogenic compound. 

Case Presentation
A 74-year-old female was initially admitted for severe sepsis secondary to CAUTI and pyelonephritis with metabolic encephalopathy. She started piperacillin-tazobactam at this time. Four days later, she developed a maculopapular rash on her abdomen that spread diffusely and progressed to blisters involving the face, back, and bilateral upper and lower extremities, covering >30% of her body. Piperacillin-tazobactam was discontinued at this time and meropenem was initiated for pyelonephritis treatment. Therabond treatment was initiated along with fluids and electrolyte replacement. She completed pyelonephritis treatment with meropenem, and later levaquin. Her skin lesions continued to heal with conservative management. After two weeks, she was deemed suitable to discharge to an assisted living facility with daily Aquaphor dressing changes. 

Discussion
Among antibiotics, SJS/TEN is most commonly reported with aminopenicillins, sulfonamides and quinolones. Severe hypersensitivity reactions are reported less frequently with carboxypenicillins and ureidopenicillins; however, SJS/TEN and erythema multiforme have been reported with ticarcillin treatment. Aminopenicillins and ureidopenicillins share a similar biochemical structure; similar properties among antibiotics could cause a similar reaction. Piperacillin-tazobactam induced linear IgA bullous dermatosis (LABD) has been reported to present as SJS/TEN and was on the differential. On biopsy, LABD is a regenerating process compared to the degenerative nature of SJS/TEN. Atypical causes of SJS/TEN were ruled out: negative IgM for mycoplasma pneumoniae and negative ANA for potential autoimmune causes. Biopsies of the rash were taken and showed lymphocytic interface dermatitis and necrotic/degenerative keratinocytes, as well as epidermal blistering & sloughing. These findings, along with the patient’s history, were consistent with SJS/TEN.

The rare presence of both Anti-TIF1-y and PL-7 antibodies in a case of Dermatomyositis.

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