Assessing Risk of Severe Complications after Endoscopic Transnasal Transsphenoidal Surgery: A Comparison of Frailty, American Society of Anesthesiologists, and Comorbidity Scores.

Objective This study aimed to improve age-independent risk stratification for patients undergoing endoscopic transnasal transsphenoidal (TNTS) approach to pituitary mass resection by investigating the associations between frailty, American Society of Anesthesiologists (ASA), and comorbidity scores with severe complications following TNTS. Design This study is a retrospective review. Setting This review was conducted utilizing the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database. Participants A total of 680 cases of TNTS identified from 2010 to 2013 were included in this study. Main Outcome Measures The modified frailty index (mFI) was calculated to quantify frailty. ASA and Charlson's comorbidity index (CCI) scores were obtained as physiologic status and comorbidity-based prognostic markers. Severe complications were separated into intensive care unit (ICU)-level complications, defined by Clavien-Dindo grade IV (CDIV) criteria, and mortality. Results Overall, 24 CDIV complications (3.5%) and 6 deaths (0.9%) were recorded. Scores for mFI ( p = 0.01, R 2 = 0.97) and ASA ( p = 0.04., R 2 = 0.87) were significantly correlated with CDIV complications. ASA scores were significantly correlated with mortality ( p = 0.03, R 2 = 0.87), as well as independently associated with CDIV complication by multivariable regression models (odds ratio [OR] = 2.96, 95% confidence interval [CI]: 1.35-6.83, p < 0.01), while mFI was not. CCI was not significantly associated with CDIV complications or mortality. A multivariable regression model incorporating ASA had a lower Akaike's Information Criteria (AIC; 188.55) than a model incorporating mFI (195.99). Conclusion Frailty and physiologic status, as measured by mFI and ASA scores respectively, both correlate with ICU-level complications after TNTS. ASA scores demonstrate greater clinical utility than mFI scores; however, as they are more easily generated, uniquely correlated with mortality and independently associated with ICU-level complication risk on multivariable regression analysis.

PIK3CA mutation in a case of CTNNB1-mutant sinonasal glomangiopericytoma.

Glomangiopericytomas are rare, primary sinonasal tumors. The existing literature is mostly limited to reports describing the clinicopathologic characteristics of these tumors. Comprehensive genetic characterization of glomangiopericytomas remains lacking. Whole-exome sequencing of a case of glomangiopericytoma was performed under an institutional review board-approved protocol. A 69-yr-old female underwent surgical resection of a glomangiopericytoma. Whole-exome sequencing revealed somatic mutations in CTNNB1 and PIK3CA, the former previously associated with this pathology but the latter not described. Concurrent dysregulation of Wnt/ß-catenin and PI3K/AKT/mTOR signaling, secondary to mutations in these two oncogenes, may be amenable to targeted treatment with existing clinically approved drugs. Genomic characterization of glomangiopericytomas remains lacking. This study reports novel coexistence of PIK3CA and CTNNB1 mutations in a case of glomangiopericytoma that may offer insight into the pathogenesis and potential for targeted medical therapies of this rare tumor.

Bilateral congenital cholesteatoma: A case report and review of the literature.

OBJECTIVES: Congenital cholesteatoma (CC) occurs less commonly than acquired cholesteatoma (AC), and bilateral CC (BCC) is even more rare with only 38 such cases having been reported in the past 42 years. Because of the rarity of this condition, providers confronted with cases of BCC may find it difficult to treat while balancing complete removal of disease, optimal hearing outcomes, and minimized surgical burden in the pediatric patient. This review alerts physicians that BCC occurs, highlights past presentations and management strategies, describes the considerations in treatment and offers an algorithm helpful in the management of BCC. METHODS: Review of a single case with extensive review of published reports from 1975 to 2017 pertaining to management of BCC. RESULTS: A five-year-old boy presented with bilateral congenital cholesteatoma. Tympanomastoidectomy was performed to remove cholesteatoma in the left ear then in the right ear months later. Ossicular chain reconstruction was deferred in both cases. Second look procedures revealed persistent cholesteatoma in both ears. In the descriptions of the 38 published BCC cases, the extent and location of the CC varied widely as did the approach to management. In the 18 cases that had descriptions of surgical management, four had second look procedures. In the 16 reports that described extent of cholesteatoma, 12 had the first of two or more operations on the ear with more extensive cholesteatoma. CONCLUSIONS: Risks are increased for recidivism/recurrence and hearing impairment in children with BCC compared to children with unilateral CC. We present a novel algorithm for management of BCC that recommends surgery for cholesteatoma removal first in the more severely affected ear and delayed OCR for both ears. Simultaneous surgery may be considered in certain cases.