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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.

2022 AMA Research Challenge

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

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Charles Lopresto, DO

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poster

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.

Piperacillin-tazobactam induced toxic epidermal necrolysis

Introduction
COVID19 infection can manifest as a broad spectrum of phenotypes ranging from asymptomatic cases to pneumonia, acute respiratory distress syndrome and death. It has been reported that elderly and immunosuppressed individuals are more prone to the severe manifestations of COVID19. Here we report a case of COVID19 infection in a person with Brutton’s agammaglobulinemia. 

Case presentation
A 41-year-old male with a past medical history of Bruton agammaglobulinemia on chronic IVIg therapy and bronchiectasis secondary to repeated respiratory infections and chronic obstructive pulmonary disease presented to our intensive care unit as a transfer from an outside hospital in hypoxic respiratory failure. The patient reported a subjective fever, dry cough, and lightheadedness, and on admission to the ICU, was found to be SARS-CoV-2 positive. On the physical exam, we saw a cachectic male with dampened breath sounds bilaterally with no audible wheezes or rhonchi. He was satting at 50% on room air in the field and had visibly increased work of breathing. The patient’s IgG and IgM were tested on ICU day 2 and were negative for a humoral response against SARS-CoV-2 despite being fully vaccinated. IVIG was initiated, and the patient received respiratory support from HFNC to BiPAP. After becoming stable, he was transferred to the hospital floor, where he continued to experience shortness of breath. He was placed on BiPAP, and sputum cultures were obtained. Sputum cultures grew gram-positive cocci in pairs, chains, and clusters and scant gram-positive rods, which were all presumed to be normal respiratory flora. Streptococcus pneumonia and Legionella pneumophila urine antigen tests were both negative. 
Labs showed elevated inflammatory markers with concurrent mixed radiological findings such as bilateral patchy ground-glass suggestive of COVID infection with features of bronchiectasis/ pneumonia exacerbation. In the pretext of immunosuppression with risk of non-clearance of infection, the patient was started on broad-spectrum antibiotics- ceftin, ceftriaxone, piperacillin/tazobactam, along with dexamethasone, tiotropium bromide, albuterol, fluconazole furoate/vilanterol. 
The patient was shifted back and forth in ICU and general medical floor due to unstable oxygen saturation with respiratory distress. Due to the progression in lung opacities, he was started on Meropenem for 14 days. Recent antibody workup showed a mild surge in COVID IgG (0.28) but was insufficient to mount appropriate IgG antibodies to SARS-CoV-2. He was finally shifted to the floor on AirVo with 40L O2/min. He was gradually weaned off of oxygen to 4L O2/min via NC within a week, showing a remarkable recovery in the background of chronically marked immunosuppression. 

Conclusion
T cell response is important for immune protection for the virus and it has been reported that the number of cytotoxic T cells expressing activation markers such as HLA-DR and CD38 increases during infection. Like in our case, since a patient with Brutton’s agammaglobulinemia could recover from the infection shows that human immune system could use multiple paths to counteract viral infection and a normal T cell immunity can be sufficient to curb the infection.


Response to COVID-19 in a Patient with Bruton Agammaglobulinemia: Humoral immunity may not be necessary to overcome COVID-19 infection

TB or not TB?
An uncommon presentation of a common disease

BACKGROUND:
● Tuberculous dactylitis accounts for 4-8% of skeletal tuberculosis. It is relatively
common in children aged 1-6 years and adults aged 20-50 years. It is rare in
children over 10 years old. It is 3x more common in men than women.
● The usual presenting feature is painless swelling of fingers. A single digit, usually
middle or index finger, is involved. The proximal phalanx is most often involved. Toe
involvement is less common. Multiple sites are involved in 25% to 30% of cases.
Joint involvement may/may not be present. Fistulas and sinuses occur in neglected
cases.

CASE PRESENTATION:
The patient, a 20 year old female with 8 months of amenorrhea, was admitted to the
hospital with chief complaints of multiple episodes of vomiting and streaky hematemesis
since 1 day.
The patient also had gradually progressive painful swelling of the left index finger since
1 month, associated with low grade fever.
No history of:
-similar episodes in the past
-past medical and surgical illness
-tuberculosis or tuberculosis contact
-trauma/unknown bite over hand
Examination
General Examination:
-Pallor
-Hyperpigmented knuckles
-Angular cheilitis
-Multiple enlarged cervical lymph nodes
Local examination: Erythematous, painful swelling of the left index finger with full ROM.
Investigations:
Hb: 8.2 gm%
ESR,CRP: Elevated
Left hand X-ray: suspicious for osteomyelitis
Local USG: s/o index finger tenosynovitis with local abscess
Management
Surgical drainage f/b gene Xpert and culture sensitivity which revealed Mycobacterium
tuberculosis.
Lymph node FNAC confirmed necrotic cervical lymphadenopathy, s/o MTb.
Rx: Antituberculous regimen
Final Diagnosis
Tuberculous dactylitis and tuberculous cervical lymphadenopathy.

DISCUSSION:
Tuberculous dactylitis is an uncommon form of extra-pulmonary tuberculosis involving
the small bones of the hand or the foot. Tuberculous dactylitis is quite uncommon
beyond 6 years of age after the formation of the epiphyseal centers.
The bones of the hands are more affected than the bones of the feet, manifesting as
soft tissue swelling and periostitis.
Thus, in order to diagnose this condition in adults, eliciting a detailed history and
entertaining a high index of suspicion is extremely important, as a delay in
diagnosis can have serious consequences for the patient.

TB or not TB? An uncommon presentation of tuberculous dactylitis

Authors:
Xavier C Cortez, Emily Finlayson, Susan Smith, Catherine Lau, Ralph Gonzales, Thomas Chi, Harnoor Jolly

Background:
Prolonged length of stay (LOS) after surgery has been shown to worsen patient recovery and utilize health system resources unnecessarily. Logistical and social support barriers to optimizing surgical care have been under-examined. The primary objective of this study was to implement a social needs screening tool amongst patients undergoing surgery and measure its impact on LOS and total direct cost.

Methods:
This was a single institution prospective cohort study where staff members utilized a social needs screening tool for patients undergoing surgery in seven surgical departments (Plastics, Vascular, Neurosurgery, OHNS, Ophthalmology, Orthopedics, Urology) from July 1, 2020 to June 30, 2021. Patient ability to obtain mandatory COVID testing, transportation to and from the hospital for surgery, availability of a post-operative caregiver, and current mood were queried during screening. Referral to Social Work Services was conducted if the patient reported a resource deficiency. LOS and total direct cost were collected for each encounter and compared to a control cohort of patients from the same departments from January 1, 2019 to February 28, 2020 that did not undergo social needs screening.

Results:
3284 patients were screen-eligible and were compared to 3360 control encounters. 190 patients screened positive and were provided social work support. There were no significant differences in age, gender, and race between the screened and control cohorts. Screen-positive patients were more likely to have government insurance as their primary payor (p<0.05). Median and average LOS was lower in the intervention (1, 2.67 days) compared to the control cohort (2, 3.58 days). Median and average direct cost was lower in the intervention ($11,661; $20,406) compared to the control ($18,193; $30,084) cohort.

Conclusion:
The implementation of a social needs screening tool to proactively address logistical barriers to care reduces LOS and total direct cost for surgical patients, optimizing comprehensive healthcare delivery.

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

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