Contemporary report of surgical outcomes after single-stage total pancreatectomy: A 10-year experience.

BACKGROUND: Surgeons rarely perform elective total pancreatectomy (TP). Our study seeks to report surgical outcomes in a contemporary series of single-stage (SS) TP patients. METHODS: Between the years 2013 to 2023 we conducted a retrospective review of 60 consecutive patients who underwent SSTP. Demographics, pathology, treatment-related variables, and survival were recorded and analyzed. RESULTS: SSTP consisted of 3% (60/1859) of elective pancreas resections conducted. Patient median age was 68 years. Ninety percent of these patients (n = 54) underwent SSTP for pancreatic ductal adenocarcinoma (PDAC). Conversion from a planned partial pancreatectomy to TP occurred intraoperatively in 31 (52%) patients. Fifty-nine patients (98%) underwent an R0 resection. Median length of hospital stay was 6 days. The majority of morbidities were minor, with 27% patients (n = 16) developing severe complications (Clavien-Dindo ≥3). Thirty and ninety-day mortality rates were 1.67% (one patient) and 5% (three patients), respectively. Median survival for the entire cohort was 24.4 months; 22.7 months for PDAC patients, with 1-, 3-, and 5-year survival of 68%, 43%, and 16%, respectively. No mortality occurred in non-PDAC patients (n = 6). CONCLUSION: Elective single-stage total pancreatectomy can be a safe and appropriate treatment option. SSTP should be in the armamentarium of surgeons performing pancreatic resection.

Anatomic shoulder arthroplasty in Walch type C glenoid deformity: mid- to long-term outcomes.

BACKGROUND: Hypoplastic glenoid morphology in the setting of glenohumeral osteoarthritis is a rare yet complex surgical problem. Treatment of this patient population with anatomic total shoulder arthroplasty (aTSA) remains controversial. Furthermore, there is no gold-standard approach, with limited guidance for surgeons on the need for glenoid version correction in the setting of a dysplastic glenoid. The purpose of this study was to evaluate mid- to long-term outcomes and reoperation rates of aTSA for the treatment of primary glenohumeral osteoarthritis with Walch type C glenoid deformity. METHODS: This observational, retrospective cohort study identified patients with a Walch type C glenoid who underwent aTSA at 2 institutions between 2007 and 2016. Patients were contacted to complete updated patient-reported outcome measures at a minimum of 5.5 years postoperatively. The outcome measures collected included the American Shoulder and Elbow Surgeons (ASES) score and Single Assessment Numeric Evaluation (SANE) score. Secondary outcomes included any additional surgical procedures on the operative shoulder, patient satisfaction, and willingness to undergo aTSA again. RESULTS: In total, 30 patients met the inclusion criteria, of whom 26 (86.7%) were able to be contacted to undergo final outcome evaluations. The mean age at the time of surgery was 61.3 years (range, 40.9-75.5 years), and 20 patients (76.9%) were men. The mean follow-up period was 8.5 years (range, 5.5-11.3 years) after surgery. Treatment was performed with an augmented component in 9 patients and with a standard component in 17. Of the 17 patients with non-augmented components, 9 underwent partial correction with asymmetrical reaming, 3 received a mini-inset glenoid component, and 2 had an anteriorly offset humeral component. At final follow-up, patients had a mean ASES score of 83.6 ± 16.7, ASES pain score of 24.7 ± 20.8, SANE score of 80.4 ± 20.9, and patient satisfaction rate of 84.1%. No statistically significant differences in any outcome measure were observed between patients with augmented glenoid components and those with non-augmented glenoid components. One revision to reverse shoulder arthroplasty was performed for instability at 7 years postoperatively after a traumatic dislocation. All patients reported that they would be willing to undergo the same surgical procedure again. DISCUSSION: Despite variance in glenoid reconstructive approach, aTSA provides satisfactory and sustained improvements in patient-reported outcomes in patients with glenoid dysplasia and primary glenohumeral osteoarthritis with a low revision rate at a mean of 8.5 years. Anatomic shoulder arthroplasty should remain a surgical option in patients with Walch type C glenoid deformity.

Implementation of an opioid reduction toolkit in pancreatectomy patients significantly increases patient awareness of safe practice and decreases amount prescribed and consumed.

BACKGROUND: Postoperative opioid abuse following surgery is a major concern. This study sought to create an opioid reduction toolkit to reduce the number of narcotics prescribed and consumed while increasing awareness of safe disposal in pancreatectomy patients. METHODS: Prescription, consumption, and refill request data for postoperative opioids were collected from patients receiving an open pancreatectomy before and after the implementation of an opioid reduction toolkit. Outcomes included safe disposal practice awareness for unused medication. RESULTS: 159 patients were included in the study: 24 in the pre-intervention and 135 in the post-intervention group. No significant demographic or clinical differences existed between groups. Median morphine milliequivalents (MMEs) prescribed were significantly reduced from 225 (225-310) to 75 (75-113) in the post-intervention group (p < 0.0001). Median MMEs consumed were significantly reduced from 109 (111-207) to 15 (0-75), p < 0.0001), as well. Refill request rates remained equivalent during the study (Pre: 17% v Post: 13%, p = 0.9) while patient awareness of safe disposal increased (Pre: 25% v Post: 62%, p < 0.0001). DISCUSSION: An opioid reduction toolkit significantly reduced the number of postoperative opioids prescribed and consumed after open pancreatectomy, while refill request rates remained the same and patients' awareness of safe disposal increased.

Genetic heterogeneity of heritable ectopic mineralization disorders in a large international cohort.

PURPOSE: Heritable ectopic mineralization disorders comprise a group of conditions with a broad range of clinical manifestations in nonskeletal connective tissues. We report the genetic findings from a large international cohort of 478 patients afflicted with ectopic mineralization. METHODS: Sequence variations were identified using a next-generation sequencing panel consisting of 29 genes reported in association with ectopic mineralization. The pathogenicity of select splicing and missense variants was analyzed in experimental systems in vitro and in vivo. RESULTS: A total of 872 variants of unknown significance as well as likely pathogenic and pathogenic variants were disclosed in 25 genes. A total of 159 distinct variants were identified in 425 patients in ABCC6, the gene responsible for pseudoxanthoma elasticum, a heritable multisystem ectopic mineralization disorder. The interpretation of variant pathogenicity relying on bioinformatic predictions did not provide a consensus. Our in vitro and in vivo functional assessment of 14 ABCC6 variants highlighted this dilemma and provided unambiguous interpretations to their pathogenicity. CONCLUSION: The results expand the ABCC6 variant repertoire, shed new light on the genetic heterogeneity of heritable ectopic mineralization disorders, and provide evidence that functional characterization in appropriate experimental systems is necessary to determine the pathogenicity of genetic variants.

Rare occurrence of pseudomyxoma peritonei (PMP) syndrome arising from a malignant transformed ovarian primary mature cystic teratoma treated by cytoreductive surgery and HIPEC: a case report.

BACKGROUND: Pseudomyxoma peritonei (PMP) syndrome is a disease process that typically occurs from ruptured appendiceal mucocele neoplasms. PMP syndrome arising from malignant transformation of an ovarian primary mature cystic teratoma (MCT) is a pathogenesis rarely encountered. CASE PRESENTATION: Herein, we report a 28-year-old patient evaluated and treated for a right ovarian mass and large volume symptomatic abdominopelvic mucinous ascites. Molecular profiling and genetic analysis revealed mutations in ATM, GNAS, and KRAS proteins while IHC demonstrated gastrointestinal-specific staining for CK20, CDX2, CK7, and SATB2. Peritoneal cytology showed paucicellular mucin. Diffuse peritoneal adenomucinosis (DPAM) variant of PMP arising from a ruptured ovarian primary MCT after malignant transformation to a low-grade appendiceal-like mucinous neoplasm was ultimately confirmed. Treatment included staged therapeutic tumor debulking and right salpingo-oophorectomy followed by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). CONCLUSIONS: Our report builds upon the existing literature supporting this aggressive treatment option reserved for advanced abdominal malignancies utilized in this patient with a rare clinical entity.

Mid- to long-term outcomes of augmented and nonaugmented anatomic shoulder arthroplasty in Walch B3 glenoids.

BACKGROUND: In cases of severe osteoarthritis, posterior glenoid wear leads to acquired retroversion of the glenoid. Surgical treatment of glenoids with acquired retroversion and posterior humeral subluxation with anatomic total shoulder arthroplasty (aTSA) is controversial. The purpose of this study was to determine mid- to long-term outcomes and reoperation rates of augmented and nonaugmented aTSA for treatment of glenohumeral osteoarthritis with Walch B3 glenoid deformity. METHODS: This observational cohort study reviewed patients with a Walch B3 glenoid undergoing aTSA at a single institution between 2007 and 2014. Patients were contacted to complete updated patient-reported outcome measures at a minimum of 6 years postoperatively. Outcome measures collected included the American Shoulder and Elbow Surgeons Standardized Shoulder Assessment Form (ASES) score and Single Alpha Numeric Evaluation (SANE) score. Secondary outcomes included any additional surgery on the operative shoulder, patient satisfaction, and willingness to undergo aTSA again. RESULTS: Thirty-nine patients met inclusion criteria, and thirty-five (89.7%) were able to be contacted for final outcomes evaluation. Mean follow-up was 8.7 years (range 6-13) after surgery. Sixteen patients were treated with an augmented glenoid component, and 19 patients were treated with a standard glenoid component. Of those with standard components, all were partially corrected with asymmetric reaming. At final follow-up, there were no statistically significant differences between those with augmented and standard glenoid components for mean ASES score (93.3 vs. 85.7, P = .217), ASES pain score (47.2 vs. 41.6, P = .161), SANE score (87.8 vs. 86.0, P = .692), and percentage patient satisfaction (95.6% vs. 96.8%, P = .735), forward elevation (148° vs. 149°, P = .852), or external rotation (36° vs. 39°, P = .202). No patient in either group had undergone revision surgery of the operative shoulder over the study period and all patients stated that they would undergo the same surgery again. DISCUSSION: Both augmented and standard aTSA can provide satisfactory and sustained improvements in patient-reported outcomes in patients with acquired glenoid retroversion due to glenohumeral osteoarthritis. Despite a trend toward alternative treatment options, anatomic shoulder arthroplasty should remain a surgical consideration even in the setting of a Walch B3 glenoid deformity.

Functional Assessment of Missense Variants in the ABCC6 Gene Implicated in Pseudoxanthoma Elasticum, a Heritable Ectopic Mineralization Disorder.

Pseudoxanthoma elasticum, a heritable multisystem ectopic mineralization disorder, is caused by inactivating mutations in the ABCC6 gene. The encoded protein, ABCC6, a transmembrane transporter, has a specialized efflux function in hepatocytes by contributing to plasma levels of inorganic pyrophosphate, a potent inhibitor of mineralization in soft connective tissues. Reduced plasma inorganic pyrophosphate levels underlie the ectopic mineralization in pseudoxanthoma elasticum. In this study, we characterized the pathogenicity of three human ABCC6 missense variants using an adenovirus-mediated liver-specific ABCC6 transgene expression system in an Abcc6-/- mouse model of pseudoxanthoma elasticum. Variants p.L420V and p.R1064W were found benign because they had abundance and plasma membrane localization in hepatocytes similar to the wild-type human ABCC6 transgene, normalized plasma inorganic pyrophosphate levels, and prevented mineralization in the dermal sheath of vibrissae in muzzle skin, a phenotypic hallmark in the Abcc6-/- mice. In contrast, p.S400F was shown to be pathogenic because it failed to normalize plasma inorganic pyrophosphate levels and had no effect on ectopic mineralization despite its normal expression and proper localization in hepatocytes. These results showed that adenovirus-mediated hepatic ABCC6 expression in Abcc6-/- mice can provide a model system to effectively elucidate the multifaceted functional consequences of human ABCC6 missense variants identified in patients with pseudoxanthoma elasticum.