Vertebral anomalies and VACTERL association in pontine tegmental cap dysplasia: a paediatric case report.

This case report highlights vertebral segmental anomalies and the fact that the child presented has a rare neurologic condition called pontine tegmental cap dysplasia. Additionally, this case aims to educate learners in developing a differential diagnosis for vertebral and cardiac anomalies such as VACTERL syndromes and common syndromes associated with butterfly vertebrae in children and adolescents.

Novel Approach in Cardiac Sarcoidosis: A Case Report Highlighting Abatacept as a Promising Treatment Option.

Cardiac sarcoidosis (CS) is a rare auto-immune disorder where immune cells form granulomas in the heart that may lead to potential arrhythmias and heart failure. Due to the low prevalence of CS, the management remains challenging, requiring a multidisciplinary approach. In addition to the management of the resulting arrhythmias and heart failure, corticosteroids and immunosuppressants are used as anti-inflammatories to prevent disease progression. Immunosuppressive regimens for the treatment of CS are not yet well established. Abatacept has been approved for rheumatoid arthritis and psoriatic arthritis and both are mainly Th1-driven autoimmune diseases. Even though there are several different drugs used to treat corticosteroid-dependent sarcoidosis, abatacept may represent a unique option as its side effects differ from other drugs like methotrexate, azathioprine, or mycophenolate, especially bone marrow and liver toxicity. We present the case of a 52-year-old CS patient treated with abatacept after the failure of methotrexate and mycophenolate mofetil. Our patient had a history of stage D heart failure with reduced ejection fraction (HFrEF) with ejection fraction (EF) of 15-20%, nonischemic cardiomyopathy (NICM) s/p left heart catheterization (LHC), CS diagnosed by positron emission tomography (PET), status post implantable cardioverter-defibrillator (ICD) implantation, lung sarcoid, paroxysmal atrial fibrillation, and aflutter, who followed with cardiology, rheumatology, and pulmonology. He had multiple admissions for heart failure exacerbations. The patient was initially diagnosed with pulmonary sarcoidosis after which he completed a small course of steroids. CT chest showed lymphadenopathy; however, endobronchial ultrasound (EBUS) did not show evidence of pulmonary sarcoidosis. During an admission for heart failure about four years later, cardiac PET CT showed CS, and rheumatology was brought on board. The patient initially refused steroids and steroid-sparing agents. At subsequent visits, the patient was amenable to medication and was started on methotrexate 10mg weekly. However, given worsening chronic kidney disorder, methotrexate was discontinued and mycophenolate 200mg daily was started. A couple of weeks after mycophenolate was started, the patient felt like "his throat was closing up" and his stomach was cramping, which was thought to be an allergic response to the mycophenolate so it was discontinued. He then received an abatacept infusion which he tolerated well. Currently, our patient has been referred for heart transplantation.

A near-miss diagnosis of necrotizing breast fasciitis complicated by atrial fibrillation secondary to septic focus: a case report and brief review of literature.

Necrotizing fasciitis of the breast is a rare, life-threatening entity characterized by a rapidly aggressive infection of the soft tissue. There are few literature reports on necrotizing fasciitis at the level of the breast tissue as the most common locations are within the abdominal wall or extremities, but this entity can lead to sepsis and systemic multiorgan failure if not adequately managed. Here, we report a case that highlights the course of a 68-year-old African American female with a past medical history of hypertension, hyperlipidemia, and poorly controlled diabetes mellitus, who presented with the complaint of a painful right breast abscess with intermittent, purulent drainage. An initial point-of-care ultrasound displayed an area of induration of the right breast as well as soft tissue edema with no identifiable fluid collection. A subsequent CT abdomen and pelvis was obtained given new onset abdominal pain, which demonstrated incidental findings of inflammatory changes and subcutaneous emphysema along with colonic diverticulosis. Surgical intervention was immediately sought for which she underwent debridement and exploration of the right breast with findings that were consistent with necrotizing transformation. The patient was sent back to the OR for an additional surgical debridement the next day. Of note, the patient had post-op atrial fibrillation with rapid ventricular response and had to be admitted to the ICU for conversion to sinus rhythm. She returned to sinus rhythm and was transferred back to medicine before application of a negative pressure wound dressing on discharge. The patient was transitioned from Enoxaparin to Apixaban for anticoagulation control in the setting of atrial fibrillation before being discharged to a Skilled Nursing Facility with long-term antibiotics. This case highlights the difficulty and significance in establishing a prompt diagnosis for necrotizing fasciitis.

Visual Outcome of Plasma Exchange in Optic Neuritis Associated With Flu Vaccination: A Case Report.

Post-flu-vaccination optic neuritis is an extremely rare condition with an incidence ranging from 0.003 cases to 0.89 per 100 000 population. The exact pathophysiology is not clearly defined. Most of the patients with post-flu-vaccination optic neuritis tend to present with progressive worsening of vision in 2-3 weeks post-vaccine administration. A prompt fundus examination supplemented with MRI imaging of the orbit is required to establish the diagnosis. On diagnosis, early initiation of high-dose oral or IV steroids is recommended to prevent optic atrophy or worsening of vision. Most patients tend to have complete recovery of vision when started on steroids. However, if the patient continues to have worsening symptoms while being treated with a high dose of steroids, plasmapheresis (PLEX) is an effective intervention.

A Case of Fibrillary Glomerulonephritis.

Fibrillary glomerulonephritis (FGN) is a very rare manifestation of glomerulonephritis characterized by the presence of deposits of randomly oriented microfibrils (10-30 nm size) in the glomeruli and visible on electron microscopy. Our patient is a 63-year-old African American male who presented with a past medical history of cirrhosis; he was initially suspected to have hepatorenal syndrome, but on kidney biopsy, and was diagnosed with FGN. Possible multiple myeloma was suspected due to its strong association with FGN and an elevated serum kappa-lambda ratio in the patient. This was confirmed by bone biopsy to be smoldering myeloma.