Gallbladder Duplication: Case Report of a Rare Congenital Anomaly Treated by Single-Incision Laparoscopic Cholecystectomy in a Pediatric Patient

Introduction: Gallbladder duplication is a rare congenital anomaly. Preoperative diagnosis is essential for prevention of intra operative complications. Based on our review of the literature, this is the first description of gallbladder duplication treated with single-incision laparoscopic surgery (SILS) in a pediatric patient. Case Presentation: A 14 year-old girl presented to the pediatric surgery clinic with a 4-month history of right upper quadrant abdominal pain, nausea, and low- grade fevers. Preoperative imaging revealed gallbladder duplication. The single incision laparoscopic technique was employed. Intra operative ndings included two gallbladders that appeared to share a common wall. There were two parallel cystic ducts that inserted separately into the common hepatic duct. Gross pathology revealed two adjacent gallbladders separated by a thin septum, each with its own cystic duct. The postoperative course was unremarkable, and the patient was without symptoms at the 1 month follow-up visit. Conclusion: Gallbladder duplication is a rare congenital anomaly. This case report demonstrates successful treatment by excision using the single incision laparoscopic approach

Rare Presentation of High Grade Retroperitoneal Angiosarcoma in a Patient Status Post Endovascular Aortic Repair (EVAR)

INTRODUCTION: Primary angiosarcoma of the aorta is a rare malignancy arising from the aorta’s endothelial lining. The most common presentation is claudication, however in the presence of an endograft a tumor originating in the aneurysmal sac may remain undetected and mimic common graft pathology. Diagnosis is typically delayed due to vague clinical presentation and imaging being indistinguishable from common complications of endografts such as leaks, infections or hematomas. We report a case of primary angiosarcoma of the aorta after endovascular repair of an abdominal aortic aneurysm. This case report describes the sixth case in the literature of primary angiosarcoma arising after the placement of an endovascular stent. CASE PRESENTATION: A 75 year-old male with a previous history of endovascular aortic repair for a AAA six years prior presented to the emergency room with signs and symptoms concerning for bowel obstruction. He elicited abdominal pain, nausea, emesis, obstipation and was anemic and had leukocytosis. A CT angiogram of the abdomen and pelvis displayed a large mass encasing the infra-renal aorta, without active extravasation, that was externally compressing the second portion of the duodenum and causing a gastric outlet obstruction. The mass extended into both iliac arteries with extension into a known right iliac artery aneurysm. Review of the patient’s chart revealed that this mass was not present six months prior. Patient was treated non-operatively with placement of a nasogastric tube. Concerns for a Type I or III Endoleak delayed the eventual CT-guided biopsy of the mass. Pathology revealed a malignant spindled and pleomorphic mass with extensive hemorrhage and necrosis consistent with high grade angiosarcoma. Specimen tested positive for vimentin, CD68, ERG, and CD31 on immunohistochemical staining. Patient did not wish to undergo aggressive treatment of his condition and was discharged to a hospice facility where he expired days later. CONCLUSION: Angiosarcoma is a rare malignancy representing 2% of all soft tissue sarcomas; associated with a poor prognosis with a median survival time of seven to eight months in advanced tumors. Tumors larger than 5cm and those located in the retroperitoneum, such as in the case report that we present, are associated with a poor prognosis. Doxorubicin is the mainstay of treatment for locally advanced tumors. Paclitaxel has also been shown to be effective. We present this care report to increase cognizance of this rare entity in the setting of a previously placed aortic endograft and avoid unnecessary surgical interventions

Mixed Goblet Cell Carcinoid-Adenocarcinoma: A Case Series

INTRODUCTION: Mixed goblet cell carcinoid-adenocarcinoma (GCC) tumors are a group of rare heterogenous neoplasms of the appendix accounting for \u3c 5% of all primary appendiceal tumors. They are characterized as an intermediate between classic carcinoid tumors and appendiceal adenocarcinomas, exhibiting both neuroendocrine and glandular/mucinous morphology that most commonly presents in Caucasian females in the fifth and sixth decades. We present three cases of mixed GCC presenting as acute appendicitis. CASE PRESENTATION: Case #1 A 65-year-old male presented with RLQ pain, nausea, emesis, and leukocytosis. CT of the abdomen revealed perforated appendicitis. The patient underwent a laparoscopic appendectomy. Pathology revealed a high-grade adenocarcinoma ex goblet cell carcinoid, signet ring type extending through the muscularis propria into the mesoappendix measuring \u3e3cm. The patient subsequently underwent a colonoscopy that revealed diverticulosis, but was otherwise normal. Patient then underwent a right hemicolectomy and partial omentectomy. Pathology revealed normal ileal, omental, and colonic tissue without evidence of carcinoma. 0/12 nodes were positive. Case #2 A 49-year-old male presented with periumbilical pain, nausea, emesis, and leukocytosis. CT of the abdomen revealed appendicitis. The patient underwent a laparoscopic appendectomy. Pathology revealed a high-grade, poorly differentiated, adenocarcinoma ex goblet cell carcinoid invading through the muscularis propria into the periappendiceal soft tissue measuring 1.5cm. The patient subsequently underwent a colonoscopy that revealed diverticulosis, but was otherwise normal. Patient then underwent a laparoscopic right hemicolectomy. Pathology revealed normal ileal and colonic mucosa without evidence of carcinoma. 0/14 nodes were positive. Case #3 A 70-year-old female presented with periumbilical pain. CT of the abdomen revealed appendicitis. The patient underwent a laparoscopic appendectomy. Pathology revealed a high-grade adenocarcinoma ex goblet cell carcinoid extending through the muscularis propria into the mesoappendix measuring 4cm. Patient then underwent an exploratory laparotomy with a right hemicolectomy. Pathology revealed normal ileal and colonic mucosa without evidence of carcinoma. 0/21 nodes were positive. CONCLUSION: Mixed GCC tumors are rare tumors that tend to present at an advanced stage and most commonly spread via direct extension. Surgical resection with a right hemi-colectomy after an appendectomy has been shown to improve the prognosis. HIPEC and adjuvant chemotherapy are other therapeutic options, but have not been shown to improve survival. In the current age of increasing rates of managing acute appendicitis non-operatively we strongly encourage surgical appendectomy in patients older than 45 years of age to avoid missing this important diagnosis and the opportunity to treat it in a timely manner

Traumatic Stab Wound to the Chest: A Rare Cause of Pituitary Apoplexy

INTRODUCTION: Pituitary apoplexy is an uncommon condition that results from infarction and hemorrhage of an established pituitary adenoma. Based on our review of the literature, this is the first description of pituitary apoplexy presenting after a knife stab wound to the deltopectoral region. CASE PRESENTATION: A 44 year-old male presented to the trauma bay as a tier 1 trauma activation status post stabbing to the left deltopectoral groove. The patient was found to be hypotensive, tachycardic and actively exsanguinating from the stab wound. He was taken to the operating room where the wound was explored and the left cephalic vein was ligated. Patient tolerated the procedure well and was discharged two days post-operatively. Patient returned to the hospital on post-operative day four with a severe frontal headache. While in the emergency room, the patient quickly decompensated and was found to have ptosis on the left, abducens nerve palsy and anisocoria. MRI displayed a large pituitary macroadenoma with intra-mass hemorrhage filling the suprasellar cistern and invading the left cavernous sinus. Patient underwent an emergent transsphenoidal endoscopic pituitary resection. The post-operative course was unremarkable and the patient was found to have only mild symptoms of hypogonadism at his one month follow up which were successfully treated with hormone replacement. CONCLUSION: Pituitary apoplexy is a rare complication of a pituitary adenoma. This case report exhibits an extremely rare occurrence of pituitary apoplexy after a penetrating traumatic event to the chest that was successfully treated via transsphenoidal endoscopic pituitary resection

Rare Case of Hip Pain: Transiliac Lumbar Hernia

Bone grafts are essential to achieving a solid spinal fusion by encouraging osteoblastic activity and providing a scaffold to guide bone remodeling. The iliac crest, in particular, is a common site for autologous bone grafting and is still considered the gold standard due to its ease of access to cortical and cancellous bone.[1] However, autologous iliac crest bone grafting can be associated with significant morbidity especially when full thickness tricortical grafts are harvested. Common complications include donor site pain, hematoma, thigh dyesthesias, pelvic instability or fracture.[2] Gastrointestinal herniation on the other hand is a much rarer complication with an incidence of 5% to 9%.[3] Here we report a case of iliac graft site herniation presenting as hip pain and difficulty ambulating more than 15 years post operation

High-Flow Oxygen with Capping or Suctioning for Tracheostomy Decannulation

9 p.BACKGROUND When patients with a tracheostomy tube reach a stage in their care at which decannulation appears to be possible, it is common practice to cap the tracheostomy tube for 24 hours to see whether they can breathe on their own. Whether this approach to establishing patient readiness for decannulation leads to better outcomes than one based on the frequency of airway suctioning is unclear. METHODS In five intensive care units (ICUs), we enrolled conscious, critically ill adults who had a tracheostomy tube; patients were eligible after weaning from mechanical ventilation. In this unblinded trial, patients were randomly assigned either to undergo a 24-hour capping trial plus intermittent high-flow oxygen therapy (control group) or to receive continuous high-flow oxygen therapy with frequency of suctioning being the indicator of readiness for decannulation (intervention group). The primary outcome was the time to decannulation, compared by means of the log-rank test. Secondary outcomes included decannulation failure, weaning failure, respiratory infections, sepsis, multiorgan failure, durations of stay in the ICU and hospital, and deaths in the ICU and hospital. RESULTS The trial included 330 patients; the mean (±SD) age of the patients was 58.3±15.1 years, and 68.2% of the patients were men. A total of 161 patients were assigned to the control group and 169 to the intervention group. The time to decannulation was shorter in the intervention group than in the control group (median, 6 days [interquartile range, 5 to 7] vs. 13 days [interquartile range, 11 to 14]; absolute difference, 7 days [95% confidence interval, 5 to 9]). The incidence of pneumonia and tracheobronchitis was lower, and the duration of stay in the hospital shorter, in the intervention group than in the control group. Other secondary outcomes were similar in the two groups. CONCLUSIONS Basing the decision to decannulate on suctioning frequency plus continuous highflow oxygen therapy rather than on 24-hour capping trials plus intermittent highflow oxygen therapy reduced the time to decannulation, with no evidence of a between-group difference in the incidence of decannulation failure. (REDECAP ClinicalTrials.gov number, NCT02512744.

Population differentiation and historical demography of the threatened snowy plover Charadrius nivosus (Cassin, 1858)

Delineating conservation units is a complex and often controversial process that is particularly challenging for highly vagile species. Here, we reassess population genetic structure and identify those populations of highest conservation value in the threatened snowy plover (Charadrius nivosus, Cassin, 1858), a partial migrant shorebird endemic to the Americas. We use four categories of genetic data—mitochondrial DNA (mtDNA), microsatellites, Z-linked and autosomal single nucleotide polymorphisms (SNPs)—to: (1) assess subspecies delineation and examine population structure (2) compare the sensitivity of the different types of genetic data to detect spatial genetic patterns, and (3) reconstruct demographic history of the populations analysed. Delineation of two traditionally recognised subspecies was broadly supported by all data. In addition, microsatellite and SNPs but not mtDNA supported the recognition of Caribbean snowy plovers (C. n. tenuirostris) and Floridian populations (eastern C. n. nivosus) as distinct genetic lineage and deme, respectively. Low migration rates estimated from autosomal SNPs (m < 0.03) reflect a general paucity of exchange between genetic lineages. In contrast, we detected strong unidirectional migration (m = 0.26) from the western into the eastern nivosus deme. Within western nivosus, we found no genetic differentiation between coastal Pacific and inland populations. The correlation between geographic and genetic distances was weak but significant for all genetic data sets. All demes showed signatures of bottlenecks occurring during the past 1000 years. We conclude that at least four snowy plover conservation units are warranted: in addition to subspecies nivosus and occidentalis, a third unit comprises the Caribbean tenuirostris lineage and a fourth unit the distinct eastern nivosus deme

SARS-CoV-2 viral load in nasopharyngeal swabs is not an independent predictor of unfavorable outcome

The aim was to assess the ability of nasopharyngeal SARS-CoV-2 viral load at first patient’s hospital evaluation to predict unfavorable outcomes. We conducted a prospective cohort study including 321 adult patients with confirmed COVID-19 through RT-PCR in nasopharyngeal swabs. Quantitative Synthetic SARS-CoV-2 RNA cycle threshold values were used to calculate the viral load in log10 copies/mL. Disease severity at the end of follow up was categorized into mild, moderate, and severe. Primary endpoint was a composite of intensive care unit (ICU) admission and/or death (n = 85, 26.4%). Univariable and multivariable logistic regression analyses were performed. Nasopharyngeal SARS-CoV-2 viral load over the second quartile (≥ 7.35 log10 copies/mL, p = 0.003) and second tertile (≥ 8.27 log10 copies/mL, p = 0.01) were associated to unfavorable outcome in the unadjusted logistic regression analysis. However, in the final multivariable analysis, viral load was not independently associated with an unfavorable outcome. Five predictors were independently associated with increased odds of ICU admission and/or death: age ≥ 70 years, SpO2, neutrophils > 7.5 × 103/µL, lactate dehydrogenase ≥ 300 U/L, and C-reactive protein ≥ 100 mg/L. In summary, nasopharyngeal SARS-CoV-2 viral load on admission is generally high in patients with COVID-19, regardless of illness severity, but it cannot be used as an independent predictor of unfavorable clinical outcome

Dendritic cell deficiencies persist seven months after SARS-CoV-2 infection

Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV)-2 infection induces an exacerbated inflammation driven by innate immunity components. Dendritic cells (DCs) play a key role in the defense against viral infections, for instance plasmacytoid DCs (pDCs), have the capacity to produce vast amounts of interferon-alpha (IFN-α). In COVID-19 there is a deficit in DC numbers and IFN-α production, which has been associated with disease severity. In this work, we described that in addition to the DC deficiency, several DC activation and homing markers were altered in acute COVID-19 patients, which were associated with multiple inflammatory markers. Remarkably, previously hospitalized and nonhospitalized patients remained with decreased numbers of CD1c+ myeloid DCs and pDCs seven months after SARS-CoV-2 infection. Moreover, the expression of DC markers such as CD86 and CD4 were only restored in previously nonhospitalized patients, while no restoration of integrin β7 and indoleamine 2,3-dyoxigenase (IDO) levels were observed. These findings contribute to a better understanding of the immunological sequelae of COVID-19

GWAS for Systemic Sclerosis Identifies Multiple Risk Loci and Highlights Fibrotic and Vasculopathy Pathways

Systemic sclerosis (SSc) is an autoimmune disease that shows one of the highest mortality rates among rheumatic diseases. We perform a large genome-wide association study (GWAS), and meta-analysis with previous GWASs, in 26,679 individuals and identify 27 independent genome-wide associated signals, including 13 new risk loci. The novel associations nearly double the number of genome-wide hits reported for SSc thus far. We define 95% credible sets of less than 5 likely causal variants in 12 loci. Additionally, we identify specific SSc subtype-associated signals. Functional analysis of high-priority variants shows the potential function of SSc signals, with the identification of 43 robust target genes through HiChIP. Our results point towards molecular pathways potentially involved in vasculopathy and fibrosis, two main hallmarks in SSc, and highlight the spectrum of critical cell types for the disease. This work supports a better understanding of the genetic basis of SSc and provides directions for future functional experiments.Funding: This work was supported by Spanish Ministry of Economy and Competitiveness (grant ref. SAF2015-66761-P), Consejeria de Innovacion, Ciencia y Tecnologia, Junta de Andalucía (P12-BIO-1395), Ministerio de Educación, Cultura y Deporte through the program FPU, Juan de la Cierva fellowship (FJCI-2015-24028), Red de Investigación en Inflamación y Enfermadades Reumaticas (RIER) from Instituto de Salud Carlos III (RD16/0012/0013), and Scleroderma Research Foundation and NIH P50-HG007735 (to H.Y.C.). H.Y.C. is an Investigator of the Howard Hughes Medical Institute. PopGen 2.0 is supported by a grant from the German Ministry for Education and Research (01EY1103). M.D.M and S.A. are supported by grant DoD W81XWH-18-1-0423 and DoD W81XWH-16-1-0296, respectively