A Case Report on Subcutaneous Emphysema and Pneumomediastinum Following a Routine Dental Procedure.

Subcutaneous emphysema (SE) and pneumomediastinum are rare complications of air beneath the skin layers and in the mediastinal space, respectively, following routine dental procedures. A few cases exist in the literature. A 53-year-old female presented to the emergency department shortly after a cavity filling, with marked swelling of her right orbit, face, and neck. Physical examination and computed tomography (CT) revealed subcutaneous emphysema and pneumomediastinum. The patient was treated with prophylactic antibiotics for one week and Peridex rinse twice daily. Subcutaneous emphysema and pneumomediastinum cases have been associated with potentially life-threatening sequelae and infections. Although these conditions are almost exclusively benign and self-limiting, physicians should consider the associated fatal complications and manage accordingly. Dental providers should be able to recognize this complication and provide patients with appropriate guidance.

Facial Nerve Palsy in a Five-Month-Old Infant.

Facial nerve palsy is a common neurological disorder, and the etiology is categorized as either congenital or acquired. Even after extensive workup, a vast majority of cases are deemed idiopathic. Treatment of acquired facial nerve palsy in pediatrics is essential to prevent long-term aesthetic and functional complications. The prognosis is favorable in pediatric patients and those treated with corticosteroids.

Inability to Ambulate in a Pediatric Patient Secondary to an Aneurysmal Bone Cyst With Associated Pathological Fracture.

Bone pain in pediatric patients is exceedingly common, with etiologies ranging from benign lesions such as fibrous dysplasia and enchondromas to potentially devastating, life-threatening malignancies such as Ewing's sarcoma or osteosarcoma. Given the low yield of physical examination and routine laboratory workup, pediatric patients with bone pain or an inability to ambulate warrant further workup. The initial workup should consist of imaging with radiography. A large majority of patients will have a resolution of symptoms without intervention and will have normal imaging. When radiographic imaging has suspicious findings, expert consultation is warranted because differentiating between benign and malignant processes on imaging can be challenging. Here we present a case of a six-year-old male with progressive worsening leg pain who was found to have a rare aneurysmal bone cyst with an associated pathologic fracture requiring open reduction and internal fixation.

Appendicitis in a Six-Month-Old Child With Coincidental Pneumonia.

Appendicitis is an exceedingly uncommon diagnosis in infancy and, thus, is typically not considered a differential diagnosis for this population. Its atypical presentation, with a wide range of clinical manifestations, creates a diagnostic challenge for physicians. Along with this, a patient's inability to articulate their pain adds another layer of diagnostic challenge. Here, we present the case of a six-month-old infant with a complicated hospital course of pneumonia and subsequent ileus, who was later found to have appendicitis with a surrounding abscess.

Infant With Recurrent Vomiting and Poor Weight Gain Secondary to an Aberrant Subclavian Artery.

Failure to thrive (FTT) is a term commonly used to characterize slower-than-expected weight gain. While inadequate caloric intake is the predominant cause, failure to thrive is a manifestation of undernutrition often resulting from the interplay of multiple etiologies. This case highlights the diagnosis and management of an infant who presented with recurrent large-volume emesis and poor weight gain secondary to esophageal compression from an aberrant right subclavian artery (ARSA).

A Five-Month-Old Boy With Hypotonia, Electrolyte Derangements, and Failure to Thrive.

Failure to thrive in the setting of profound hypotonia and multiple electrolyte derangements is a challenging constellation of findings that offers a broad differential diagnosis for providers to consider. Initial management should focus on the stabilization of the patient and correction of potential life-threatening electrolyte derangements. Once completed, the diagnosis should be sought, and in this case, many were considered and ultimately ruled out with thorough history and physical examination. Laboratory abnormalities revealed the final diagnosis of pseudohypoaldosteronism and connected the case. With proper treatment, our patient had a resolution of laboratory anomalies along with improved growth and tone.

Congenital lobar emphysema: A rare cause of respiratory failure in neonates.

Congenital lobar overinflation, also known as congenital lobar emphysema (CLE), is an uncommon (1/20,000-30,000 live births) abnormality characterized by hyperinflation of one or more pulmonary lobes, usually with contralateral displacement of the mediastinum. While the etiology of most cases of CLE is poorly understood and labeled idiopathic, some cases are thought to be due to an intrinsic or extrinsic bronchial wall abnormality causing a ball valve mechanism with resultant hyperinflation of the affected lobe. CLE tends to have a predilection for males presenting insidiously in the first 6 months of life and have respiratory distress and progressive failure, with 50% of cases being asymptomatic at birth. Acquired forms of lobar emphysema are similar but are secondary to prolonged exposure to oxygen and positive pressure ventilation in premature infants. Clinical presentation is variable, ranging from wheezing, increased respiratory effort, cyanosis, feeding difficulties, and reflux or respiratory failure. Chest radiography (CXR) is the initial imaging obtained for any neonate with respiratory distress, which can aid in diagnosis showing overinflation, while computerized tomography (CT) remains the gold standard for confirmatory diagnosis. Treatment is variable and based on clinical severity. Some cases can be managed conservatively, while more severe cases require surgical intervention with lobectomy. Here, we present a case of a neonate in respiratory distress soon after birth, had initial improvement with supportive care, and was found to be secondary to CLE.

Diffuse alveolar hemorrhage in a pediatric patient with systemic lupus erythematosus.

A 15-year-old female patient was initially transferred due to symptomatic anemia following months of menorrhagia, fatigue, dyspnea, and weight loss. Early during her hospital course, pulmonary complications occurred with the development of respiratory failure secondary to bilateral pleural effusions. She was managed with bi-level pressure support ventilation alternating with a high-flow nasal cannula. An extensive workup resulted in a diagnosis of systemic lupus erythematosus: C3 level was 9 mg/dl (81-157 mg/dl) and her C4 level was 2 mg/dl (12-39 mg/dl); ANA titer was 1:5120 in a homogenous pattern. Scl-70 was qualitatively positive, quantitative anti-dsDNA level was 1044 IU/ml (<150 IU/ml), hemoglobin at presentation was 6.3 g/dl (11.8-15.7 g/dl), and her albumin level was 2.8 g/dl (3.5-5.0 g/dl). The patient was started on prednisone 60 mg daily and then transitioned to cyclophosphamide. Her symptoms improved initially; however, several days into admission she developed acute respiratory decompensation with blood-tinged sputum. Her hemoglobin levels, which had stabilized, began decreasing from 9.5 to 7.4 g/dl. Chest radiography showed new bilateral hazy infiltrates, and computerized tomography of the chest showed bilateral pleural effusions and parenchymal disease. A bronchoscopy confirmed a diagnosis of diffuse alveolar hemorrhage. The patient was treated with plasma exchange therapy and her corticosteroids were restarted. She completed a course of steroids and at initial pulmonology follow-up and showed stable pulmonary status with an improvement of her symptoms.

Quantitative single photon emission computed tomography derived standardized uptake values on 99mTc-PYP scan in patients with suspected ATTR cardiac amyloidosis.

Technetium-99 pyrophosphate scintigraphy (99mTc-PYP) provides qualitative and semiquantitative diagnosis of ATTR cardiac amyloidosis (ATTR-CA) using the Perugini scoring system and heart/contralateral heart ratio (H/CL) on planar imaging. Standardized uptake values (SUV) with quantitative single photon emission computed tomography (xSPECT/CT) can offer superior diagnostic accuracy and quantification through precise myocardial contouring that enhances assessment of ATTR-CA burden. We examined the correlation of xSPECT/CT SUVs with Perugini score and H/CL ratio. We also assessed SUV correlation with cardiac magnetic resonance (CMR), echocardiographic, and baseline clinical characteristics. Retrospective review of 78 patients with suspected ATTR-CA that underwent 99mTc-PYP scintigraphy with xSPECT/CT. Patients were grouped off Perugini score (Grade 0-1 and Grade 2-3), H/CL ratio (≥ 1.5 and < 1.5). Two cohorts were also created: myocardium SUVmax > 1.88 and ≤ 1.88 at 1-hour based off an AUC curve with 1.88 showing the greatest sensitivity and specificity. Cardiac SUV retention index was calculated as [SUVmax myocardium/SUVmax vertebrae] × SUVmax paraspinal muscle. Primary outcome was myocardium SUVmax at 1-hour correlation with Perugini grades, H/CL ratio, CMR, and echocardiographic data. Higher Perugini Grades corresponded with higher myocardium SUVmax values, especially when comparing Perugini Grade 3 to Grade 2 and 1 (3.03 ± 2.1 vs 0.59 ± 0.97 and 0.09 ± 0.2, P < 0.001). Additionally, patients with H/CL ≥ 1.5 had significantly higher myocardium SUVmax compared to patients with H/CL ≤ 1.5 (2.92 ± 2.18 vs 0.35 ± 0.60, P < 0.01). Myocardium SUVmax at 1-hour strongly correlated with ECV (r = 0.91, P = 0.001), pre-contrast T1 map values (r = 0.66, P = 0.037), and left ventricle mass index (r = 0.80, P = 0.002) on CMR. SUVs derived from 99mTc-PYP scintigraphy with xSPECT/CT provides a discriminatory and quantitative method to diagnose and assess ATTR-CA burden. These findings strongly correlate with CMR.

Clitoromegaly Secondary to Exogenous Androgen Exposure From Paternal Skin to Skin Transfer.

Clitoromegaly is the abnormal enlargement of the clitoris. Its etiology is often divided into congenital and acquired causes, leading to a differential diagnosis that is quite broad. Workup often includes serum hormone testing, imaging studies, and sometimes an investigation into genetic and nonhormonal causes, which can be obtained from a detailed patient history. Exposure to exogenous or endogenous androgens can directly stimulate and enlarge the clitoris, resulting in early virilization. Transdermal testosterone gel can be transferred from the skin of an adult to the skin of a child. Topical testosterone gel is an approved therapy for the treatment of hypoandrogenism in males. While it offers a convenient means of treatment, there is a concern about unintentional exposure to females and children from skin contact with the application site. Here, we report a case of an infant who presented to a pediatric endocrinology clinic for clitoromegaly that was possibly due to exogenous exposure to testosterone.

A 14-Year-Old Female With Several-Week History of Rash, Worsening Diffuse Arthralgias, and Fevers.

Macrophage activation syndrome (MAS) as the initial presentation of systemic juvenile idiopathic arthritis (sJIA) is an uncommon and difficult diagnosis to ascertain. However, it remains critical to establish the diagnosis since MAS is a potentially life-threatening systemic inflammatory condition. Prompt recognition can lead to early initiation of treatment with corticosteroids and overall improved outcomes. Here, we present a case of a 14-year-old female with MAS as the initial manifestation of sJIA.

Out of this world: Elizabethkingia miricola complicated urinary tract infection in a patient with associated pubic symphysis osteomyelitis and pyomyositis.

Elizabethkingia miricola (E. miricola) is a gram-negative rod initially isolated from condensation at the Russian Mir space station. In the literature, there are few cases of human isolates that have been identified, with only one prior case of E. miricola urinary tract infection (UTI). Here we report a case of a patient with a chronic suprapubic catheter that was found to have E. miricola UTI with fistulization between the bladder and pubic symphysis, leading to osteomyelitis and surrounding pyomyositis. He was placed on Tigecycline based on susceptibility profile, underwent bilateral nephrostomy tube placement and discharged home with close outpatient follow-up. With the increasing use of novel detection methods, accurate identification and antimicrobial susceptibility testing is necessary for this multidrug resistant organism and others like it.

Recurrent spontaneous pneumothorax in a 15-year-old female associated with electronic cigarettes.

Pneumothorax as a sequela of vaping is a relatively recent complication being described in the literature. Smoking has classically been associated with an increased risk of pneumothorax, and emerging evidence is showing that electronic cigarettes (e-cigarettes) likely carry some of the same risks. Since e-cigarettes increased in popularity, especially among the adolescent population, there has been a reported increased incidence of lung injury, including pneumothorax. We present a case of a 15-year-old female with a history of e-cigarette use admitted for recurrent pneumothorax with failure to re-expand requiring surgical intervention.

Acute Ischemic Stroke in an Eight-Year-Old Male With Elevated Factor VIII Activity and SARS-CoV-2 Antibodies.

Acute ischemic stroke (AIS) is a significant source of morbidity and mortality and is one of the top causes of death in the United States. Of these patients, most are elderly individuals, compared to a limited proportion of cases seen in pediatrics. AIS is classically associated with age-dependent atherosclerotic disease processes secondary to comorbidities such as diabetes and hypertension. When considering the pediatric population, stroke is far less common and often requires workup of other underlying etiologies that create a hypercoagulable state. Here we present a case of an eight-year-old male with a left middle cerebral artery (MCA) ischemic stroke in the setting of increased factor VIII activity and SARS-CoV-2 antibodies.

A Case of Acalvaria in a Full Term, Live Born Male Infant.

Acalvaria is a rare cranial vault defect characterized by the presence of the cerebellum and cerebral cortex with the absence of the calvarium above the orbits, intact facial structures, and the presence of dura mater. Unfortunately, this diagnosis comes with a dim prognosis that is not compatible with life long-term. First-trimester diagnosis with ultrasonography can establish the diagnosis. If imaging is equivocal, advanced imaging with fetal MRI has a role to aid in distinguishing between similar cranial vault defects that fall on the spectrum including anencephaly, exencephaly, and acrania. We present the case of a term male infant with known acalvaria diagnosed incidentally on prenatal ultrasound that was delivered by cesarean section to a G3P3 mother at the time of delivery with two prior uncomplicated pregnancies. Maternal history was rather insignificant except for gestational diabetes and gestational hypertension well-controlled without medication. After initiation of comfort measures only, the infant expired on day of life two.

Pre-existing cardiovascular disease increases risk of atrial arrhythmia and mortality in cancer patients treated with Ibrutinib.

BACKGROUND: Ibrutinib is a Bruton's tyrosine kinase inhibitor used in the treatment of hematological malignancies. The most common cardiotoxicity associated with ibrutinib is atrial arrhythmia (atrial fibrillation and flutter). It is known that patients with cardiovascular disease (CVD) are at an increased risk for developing atrial arrhythmia. However, the rate of atrial arrhythmia in patients with pre-existing CVD treated with ibrutinib is unknown. OBJECTIVE: This study examined whether patients with pre-existing CVD are at a higher risk for developing atrial arrhythmias compared to those without prior CVD. METHODS: A single-institution retrospective chart review of patients with no prior history of atrial arrhythmia treated with ibrutinib from 2012 to 2020 was performed. Patients were grouped into two cohorts: those with CVD (known history of coronary artery disease, heart failure, pulmonary hypertension, at least moderate valvular heart disease, or device implantation) and those without CVD. The primary outcome was incidence of atrial arrhythmia, and the secondary outcomes were all-cause mortality, risk of bleeding, and discontinuation of ibrutinib. The predictors of atrial arrhythmia (namely atrial fibrillation) were assessed using logistic regression. A Cox-Proportional Hazard model was created for mortality. RESULTS: Patients were followed for a median of 1.1 years. Among 217 patients treated with ibrutinib, the rate of new-onset atrial arrhythmia was nearly threefold higher in the cohort with CVD compared to the cohort without CVD (17% vs 7%, p = 0.02). Patients with CVD also demonstrated increased adjusted all-cause mortality (OR 1.9, 95% CI 1.06-3.41, p = 0.01) and decreased survival probability (43% vs 54%, p = 0.04) compared to those without CVD over the follow-up period. There were no differences in risk of bleeding or discontinuation between the two cohorts. CONCLUSIONS: Pre-existing cardiovascular disease was associated with significantly higher rates of atrial arrhythmia and mortality in patients with hematological malignancies managed with ibrutinib.

A Novel Technique to Detect Femoral Shaft Perforation during Direct Anterior Total Hip Arthroplasty.

Despite its popularity, the direct anterior approach for hip arthroplasty is not without complications. Intraoperative femoral shaft perforation using this approach ranges from 0.8% to 7%. A missed perforation can lead to fracture with the need for further surgery if not detected intraoperatively. We describe a reproducible and cost-effective technique using a plastic Yankauer suction handle to help identify proximal femoral perforations during direct anterior total hip arthroplasty. Careful attention to the visual, tactile, and auditory feedback provided by the suction handle can help ensure the cortical continuity of the proximal femur. Familiarity with relevant surgical anatomy, improving surgical technique, and scrutinizing implant positioning helps minimize the risk of complications during the direct anterior approach.

HSP90 Inhibition and Cellular Stress Elicits Phenotypic Plasticity in Hematopoietic Differentiation.

Cancer cells exist in a state of Darwinian selection using mechanisms that produce changes in gene expression through genetic and epigenetic alteration to facilitate their survival. Cellular plasticity, or the ability to alter cellular phenotype, can assist in survival of premalignant cells as they progress to full malignancy by providing another mechanism of adaptation. The connection between cellular stress and the progression of cancer has been established, although the details of the mechanisms have yet to be fully elucidated. The molecular chaperone HSP90 is often upregulated in cancers as they progress, presumably to allow cancer cells to deal with misfolded proteins and cellular stress associated with transformation. The objective of this work is to test the hypothesis that inhibition of HSP90 results in increased cell plasticity in mammalian systems that can confer a greater adaptability to selective pressures. The approach used is a murine in vitro model system of hematopoietic differentiation that utilizes a murine hematopoietic stem cell line, erythroid myeloid lymphoid (EML) clone 1, during their maturation from stem cells to granulocytic progenitors. During the differentiation protocol, 80%-90% of the cells die when placed in medium where the major growth factor is granulocyte-macrophage-colony stimulating factor. Using this selection point model, EML cells exhibit increases in cellular plasticity when they are better able to adapt to this medium and survive. Increases in cellular plasticity were found to occur upon exposure to geldanamycin to inhibit HSP90, when subjected to various forms of cellular stress, or inhibition of histone acetylation. Furthermore, we provide evidence that the cellular plasticity associated with inhibition of HSP90 in this model involves epigenetic mechanisms and is dependent upon high levels of stem cell factor signaling. This work provides evidence for a role of HSP90 and cellular stress in inducing phenotypic plasticity in mammalian systems that has new implications for cellular stress in progression and evolution of cancer.