Acute Liver Failure With Liver Enzymes >5,000 in Sickle Cell Disease.

Sickle cell disease is a hemoglobinopathy often complicated by painful vaso-occlusive episodes, acute chest syndrome, stroke, and myocardial infarction. Sickle cell intrahepatic cholestasis (SCIC) is a rare and potentially fatal complication of sickle cell disease. SCIC is thought to involve progressive hepatic injury due to sickling within sinusoids. We present the case of a young patient with SCIC and acute liver failure, requiring prompt treatment with exchange transfusion. Our case describes features that should raise suspicion for hepatic failure in SCIC and highlights exchange transfusion as a successful management approach in similar patients with an otherwise high risk of mortality.

Choledocholithiasis Can Present with Marked Transaminases Elevation: Systematic Review and Meta-Analysis.

BACKGROUND: Extreme transaminase elevation > 1000 international units per liter (IU/L) is typically caused by hepatocellular injury due to ischemia, drugs, or viral infection. Acute choledocholithiasis can also present with marked transaminase elevation mimicking severe hepatocellular injury, contrary to the presumed cholestatic pattern. METHODS: We searched PubMed/Medline, EMBASE, Cochrane Library, and Google Scholar for studies reporting the proportion of marked elevation of alanine aminotransferase (ALT) or aspartate aminotransferase (AST) > 1000 IU/L in patients with common bile duct (CBD) stones. A proportion meta-analysis with a corresponding 95% confidence interval (CI) was used to pool the proportion of patients with extreme transaminase elevation. I2 was used to examine heterogeneity. We used CMA software utilizing a random effect model for statistical analysis. RESULTS: Three studies (n = 1328 patients) were included in our analysis. The reported frequency of ALT or AST > 1000 IU/L in choledocholithiasis patients ranged between 6 and 9.6%, with pooled frequency of 7.8% (95% CI 5.5-10.8%, I2 61%). The frequency of patients with ALT or AST > 500 IU/L was higher, ranging between 28 and 47%, with pooled frequency of 33.1% (95% CI 25.3-42%, I2 88%). CONCLUSION: This is the first meta-analysis to study prevalence of severe hepatocellular injury in patients with CBD stones. Results revealed that approximately one-third of patients with choledocholithiasis present with ALT or AST > 500 IU/L. Furthermore, levels > 1000 IU/L are not uncommon. An elaborate work-up for alternative etiologies of severe transaminase elevation is likely unwarranted in cases with clear evidence of choledocholithiasis.

Etiologies and Outcomes of Transaminase Elevation > 1000 IU/L: A Systematic Review and Meta-Analysis.

BACKGROUND: Among liver injury causes, few result in marked elevation of liver enzymes to a level > 1,000 international units per liter (IU/L). This review summarizes common etiologies of marked transaminase elevation and associated prognostic factors. METHODS: We performed a comprehensive search on PubMed, EMBASE, Cochrane Library, and Google Scholar from inception through December 2022 using MOOSE guidelines for studies reporting frequency of etiologies of marked transaminase elevation. We used a proportion meta-analysis to pool frequencies with corresponding 95% confidence interval (CI). I2 was used to adjudicate heterogeneity. We used CMA software for statistical analysis. RESULTS: Seven relevant studies (n = 1608 patients) were included. The pooled frequency of ischemic hepatitis was 51% (95% CI 42-60%, I2 = 91%), viral hepatitis was 13.1% (95% CI 9.7-17.6%, I2 = 80%), toxins or drug-induced liver injury (DILI) was 13% (95% CI 8-18%, I2 = 85%), and pancreaticobiliary-related injury was 7.8% (95% CI 4.4-13.6%, I2 = 89%). Mortality was significantly higher in ischemic hepatitis versus other causes of marked transaminase elevation, with an odds ratio of 21 (95% CI 9.9-44.8, P value < 0.0001, I2 = 64% Q 11.1). DISCUSSION: This is the first meta-analysis to examine etiologies of marked transaminase elevation > 1000 IU/L. Liver ischemia is the most common cause, while other causes include DILI or toxins, viral hepatitis, and biliary pathologies. We found biliary pathologies to be the fourth most common cause. This is clinically relevant as it has been traditionally linked to a cholestatic pattern of liver injury. Being aware of this presentation may help prevent delayed or missed diagnoses and unnecessary testing.

Transcervical and robotic-assisted thoracoscopic resection of a substernal goiter.

Several genetic and environmental factors contribute to the development of multinodular goitre. A transcervical surgical resection is recommended for larger goitres, though a minority of cases may require sternotomy or thoracotomy. We present a case of a posterior substernal goitre that was resected with combined transcervical and robotically assisted thoracic approaches. A woman in her 30s with an enlarging thyroid goitre elected to proceed with surgical resection. CT imaging demonstrated significant extension of the goitre into the posterior mediastinum and a staged approach was decided on. Both the initial transcervical thyroidectomy and the subsequent robotically assisted resection of the mediastinal portion were successful, without major complications. While most substernal goitres can be resected transcervically, certain rare anatomic features, such as extension into the posterior mediastinum, warrant consideration of a thoracic approach. Specifically, a robotic-assisted resection poses several advantages over traditional, more invasive approaches.

Association of comorbidity score with perioperative outcomes following transoral robotic surgery: National analysis.

BACKGROUND: The association of comorbidities with perioperative outcomes after transoral robotic surgery (TORS) is not well-defined in the literature. METHODS: Using the National Cancer Database, 4004 patients with T1-T2 oropharyngeal cancer between 2010 and 2017 were stratified based on their Charlson-Deyo Comorbidity Class (CDCC). Thirty-day unplanned readmissions, 30-day mortality, and 90-day mortality were compared using chi-square test and logistic regression. Hospital length of stay (LOS) was compared using the Kruskal-Wallis test. RESULTS: LOS was greater for patients with CDCC 2 or 3 compared to CDCC 0 or 1 (p < 0.001). Increasing age and CDCC ≥3 were associated with 30-day mortality (CDCC ≥3: odds ratio [OR] 5.55, 95% confidence interval [CI] 1.59-19.45). CDCC ≥3 (OR 2.61, 95%CI 1.09-6.27) was significantly associated with 30-day readmissions. CONCLUSION: This national analysis demonstrates greater rates of unplanned 30-day readmissions, longer hospitalizations, and increased 30- and 90-day mortality after TORS in patients with CDCC ≥3.

Laceration of Aberrant Internal Carotid Artery Following Myringotomy: A Case Report and Review of Literature.

INTRODUCTION: The presence of an aberrant internal carotid artery (ICA) in the middle ear is rare. Patients may be asymptomatic or complain of conductive hearing loss, otalgia, pulsatile tinnitus, or aural fullness. Otoscopic exam findings can include a pulsating erythematous lesion on the tympanic membrane (TM). It may be misdiagnosed as a glomus tumor, hemangioma, or serous otitis media, or go unrecognized until surgical exploration. Early recognition is important as intraoperative discovery carries risk of iatrogenic injury, hemorrhage and subsequent neurologic sequelae. Prevention requires adequate preoperative suspicion and can be confirmed with radiologic examination via computed tomography (CT) scan or magnetic resonance angiography (MRA). Management of iatrogenic injury of an aberrant ICA can include packing, vessel embolization and/or surgical ligation. PATIENT CASE: We report the case of an aberrant ICA injury in a pediatric patient undergoing a myringotomy with tube placement, who sustained neurologic deficits that eventually resolved following treatment with packing and coil embolization. DISCUSSION AND CONCLUSIONS: An aberrant ICA can cause life-threatening complications without prior diagnosis in a routine myringotomy. Suspicious exam findings should prompt temporal bone CT to rule out aberrant ICA or other vascular pathology of the middle ear prior to surgery. In the case of iatrogenic injury of an aberrant ICA, there is no consensus in existing literature on optimal management. We reviewed 37 studies to compare therapeutic options and subsequent outcomes. Though complications are rare regardless of management, cases in which solely packing was utilized demonstrated an increased incidence of hemiparesis, aphasia, hearing loss, re-bleeding, and delayed pseudoaneurysm, as compared to an approach coupling packing with embolization or ligation, both of which have comparable outcomes.