Citations

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

Background: The vertical rectus abdominis myocutaneous flap has been used to reliably reconstruct posterior soft-tissue defects following resection of high sacral tumors, providing skin and soft-tissue bulk. Current literature examining the use of these flaps for high sacral reconstruction is limited. The purpose of this study was to report the authors’ institution's outcome on the use of the vertical rectus abdominis myocutaneous flap to reconstruct a high sacral defect following an oncologic resection. Methods: Eighty-seven patients (29 female and 58 male) underwent vertical rectus abdominis myocutaneous flap surgery to reconstruct a posterior wound following high sacral tumor resection from 1994 to 2016. The mean age and body mass index were 52 years and 27.8 kg/m2, respectively. The mean follow-up was 6 years. Results: Sixty-eight patients (79 percent) sustained a complication, most commonly a wound complication [n = 41 (47 percent)]. Twenty patients (23 percent) sustained a donor-site wound complication, most commonly a wound dehiscence (n = 10). Complications resulted in a reoperation in 44 patients (51 percent), with total flap failure occurring in one (1 percent). Preoperative radiotherapy and obesity were found to increase the risk of wound complications (p = 0.004 and p = 0.02, respectively) and deep infection (p = 0.03 and p = 0.02, respectively). Conclusions: Complications were common following vertical rectus abdominis myocutaneous flap surgery; however, total flap loss was not. Patients with obesity and preoperative radiotherapy should be cautioned on their increased risk of complications. Currently, the vertical rectus abdominis myocutaneous flap is the authors’ preferred means of soft-tissue reconstruction following high sacral tumor resection. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Abstract Purpose: In phase I testing, alisertib tablets with irinotecan and temozolomide showed significant antitumor activity in patients with neuroblastoma. This study sought to confirm activity of this regimen; evaluate an alisertib oral solution; and evaluate biomarkers of clinical outcomes. Patients and Methods: We conducted a two-stage phase II trial of alisertib tablets (60 mg/m2/dose × 7 days), irinotecan (50 mg/m2/dose i.v. × 5 days), and temozolomide (100 mg/m2/dose orally × 5 days) in patients with relapsed or refractory neuroblastoma. The primary endpoint was best objective response. A separate cohort was treated with alisertib at 45 mg/m2 using oral solution instead of tablets. Exploratory analyses sought to identify predictors of toxicity, response, and progression-free survival (PFS) using pooled data from phase I, phase II, and oral solution cohorts. Results: Twenty and 12 eligible patients were treated in the phase II and oral solution cohorts, respectively. Hematologic toxicities were the most common adverse events. In phase II, partial responses were observed in 19 evaluable patients (21%). The estimated PFS at 1 year was 34%. In the oral solution cohort, 3 patients (25%) had first cycle dose-limiting toxicity (DLT). Alisertib oral solution at 45 mg/m2 had significantly higher median Cmax and exposure compared with tablets at 60 mg/m2. Higher alisertib trough concentration was associated with first cycle DLT, whereas MYCN amplification was associated with inferior PFS. Conclusions: This combination shows antitumor activity, particularly in patients with MYCN nonamplified tumors. Data on an alisertib oral solution expand the population able to be treated with this agent.

Ehlers-Danlos Syndrome is a connective tissue disorder that results in joint and generalized tissue hyperlaxity, predisposing patients to early onset arthritis. An institutional database spanning 1999–2016 was reviewed for all patients with symptomatic carpometacarpal arthritis and coexisting Ehlers-Danlos Syndrome, resulting in 14 thumbs amongst nine patients followed clinically for a median of 5.9 years (range 1–15). Patients managed non-operatively demonstrated stable subjective pain and trended towards decreased range of motion over time, whereas patients managed operatively had significant improvements in pain and trended towards increased grip strength. Our findings suggest that patients with Ehlers-Danlos-associated carpometacarpal arthritis can be successfully managed both operatively and non-operatively and that patients with pain refractory to conservative therapy may benefit from relief and increased hand strength following surgical intervention. Level of evidence: IV

* There are limited motion-sparing salvage options for patients with pancarpal wrist arthritis. Total wrist arthroplasty is one option that has long been used in an attempt to preserve motion while relieving pain and wrist dysfunction; however, enthusiasm for the procedure has been tempered by high rates of complications. * In an attempt to minimize or eliminate these complications, recent implants have been redesigned with a more semiconstrained arc of motion and ellipsoidal articulation. Furthermore, the distal component has undergone many different modifications to improve bone growth into the implant. * Short-term small series have shown promising improvements in terms of the rate of complications as compared with previous-generation designs.

An analysis was conducted of 325 consecutive metacarpophalangeal arthroplasties prospectively collected using a single institution’s total joints registry over a 14-year time period to characterize long-term radiographic and functional outcomes. Patients were followed for a mean of 7.2 years (2–18) or until revision. The 5-, 10- and 15-year survival free from revision were 98%, 95% and 95%, respectively. The 5-, 10- and 15-year survival rates free from radiographic implant fracture were 93%, 58% and 35%, respectively. The 5-, 10- and 15-year survival rates free from coronal plane deformity greater than 10° were 81%, 37% and 17%, respectively. Patients had significant improvements in their postoperative pain levels and metacarpophalangeal arc of motion. Neither implant fracture, nor coronal plane deformity >10° had a significant association with worse function. Overall, pain relief and functional improvement are reliable, though silicone implants do not protect from progression of coronal plane deformity and have a high fracture rate. Level of evidence: IV