Quality of life outcomes after minimally invasive repair of pectus excavatum utilizing a new set of metallic bars and stabilizers

Background/PurposeThe aim of the study was to evaluate the postoperative quality of life (QoL) of patients who underwent minimally invasive repair of pectus excavatum (MIRPE) with a newly designed bar and bar stabilizers.MethodsWe conducted a prospective randomized study in which patients were operated either with standard perpendicular stabilizers (control group) or with the newly designed oblique stabilizers (intervention group). All patients were evaluated 6 months after the operation with the Pectus Excavatum Evaluation Questionnaire (PEEQ).ResultsThere were 16 patients in the control group and 14 in the intervention group. Mean age was 17 (SD: 3.3, range 14–27) years. There were no demographic differences between groups. Two patients in the control group and one in the intervention group were repaired with two bars instead of one. There was one reoperation in each group. There was a significant difference between the pre- and postoperative scores, in both groups, in the patient body image domain (control group: 9.5 to 3; p < 0.01; intervention group 10 to 3; p < 0.01), as well as in the psychosocial domain (control group: 13.5 to 24, p < 0.01; intervention group: 15 to 24, p < 0.01). With regards to the patients' perception of physical difficulties before and after MIRPE, the difference between pre- and postoperative scores was greater in the intervention group (8 to 12, p < 0.01) than in the control group (10 to 11, p = 0.04). The mean length of stay was 4.5 and 5 days in the intervention group and the control group, respectively.ConclusionOur study showed that patients who underwent MIRPE with the newly designed bars and stabilizers had non-inferior outcomes than patients reported in the literature who underwent MIRPE with standard bars and stabilizers. We found slightly better outcomes in patients in the intervention group compared to the control group, but larger studies will be needed to confirm if those differences are statistically significant.Level of evidenceII     

Management of functioning pediatric adrenal tumors

AimThe aim of this study is to present our experience in the management of hormonally active adrenal tumors in children.Material and methodsWe did a retrospective chart review of all children with hormonally active adrenal tumors evaluated at the endocrinology clinic and operated at our institution between 1983 and 2019.ResultsThere were 75 patients included in the study, 58 with adrenocortical tumors (ACTs) and 17 with pheochromocytomas (PCCs). Within the group of patients with ACTs, there were 41 females and 17 males. The mean age was 58.3 (SD: 87.9; range: 9–211) months. The clinical manifestation of the tumor's hormonal activity was virilization in 37 cases, Cushing syndrome in 5, and mixed in 16. A positive family history was present in 11 patients (18.9%). The mean tumor size was 48.2 (SD: 22.4; range: 7–120) mm. The pathological diagnosis was adenoma in 42 cases, carcinoma in 15 cases, and macronodular hyperplasia in 1. Median follow-up was 192 (range: 50–290) months. Tumor recurrence occurred in 6 patients (10.3%), and there were three disease-related deaths (5%). Within the group of patients with PCCs, there were 11 males and 6 females. The mean age was 146.7 (SD: 71.2; range: 60–216) months. A positive family history was present in 7 patients (41.2%). The mean tumor size was 36.6 (SD: 16.7; range: 7–120) mm. The pheochromocytoma was classified as benign in 15 cases and as malignant in 2. During a median follow-up of 180 (range: 127–300) months, recurrence was observed in 6 cases (35.3%) and disease-related death in 1 case (5.9%).ConclusionsProper diagnosis and management at our referral center were associated with a high cure rate, even in cases of malignant tumors. Familial surveillance is highly recommended.Level of evidenceLevel IV.     

Outcomes of gastrostomy placement with and without concomitant tracheostomy among ventilator dependent children

IntroductionSimultaneous gastrostomy tube (GT) and tracheostomy placement in young children offers potential benefit in limiting anesthetic exposure, but it is unknown whether combining these procedures introduces additional morbidity. This study compared outcomes after combined GT and tracheostomy placement versus GT placement alone among similar ventilator-dependent patients.MethodsVentilator-dependent children <2-years-old who underwent GT placement alone (MV-GT), simultaneous GT and tracheostomy placement (GT+T), and GT placement alone with a pre-existing tracheostomy (T-GT) were identified using 2012–2018 NSQIP-Pediatric Participant User Files. Multiple logistic regression models were used to compare outcomes while adjusting for other group differences.ResultsAmong 1100 children, 351 underwent MV-GT, 494 GT+T, and 255 T-GT. Major complications occurred in 23.6%, 17.0%, and 14.5% of the respective groups (p = 0.01). Major complications with GT+T were similar to T-GT (adjusted odds ratio [aOR]=1.19, 95%CI:0.78–1.83, p = 0.4) and lower than MV-GT (aOR=0.67, 95%CI:0.47–0.95, p = 0.02). Severe complications including mortality, cardiac arrest, and stroke were similar between the three groups (p = 0.8).ConclusionsChildren <2-years-old undergoing GT+T did not experience higher post-operative complications compared to children undergoing T-GT or MV-GT. Utilizing GT+T to limit anesthetic exposure may be reasonable within this high-risk population.Type of StudyTreatment StudyLevel of EvidenceLevel III     

ASO Visual Abstract: Will It Play in Peoria? A Pilot Study of a Robotic Skills Curriculum for Surgical Oncology Fellows

Annals of Surgical Oncology -     

Upper Extremity Overuse Injuries and Obesity After Spinal Cord Injury

Persons with spinal cord injury (SCI) are at high risk for developing neurogenic obesity due to muscle paralysis and obligatory sarcopenia, sympathetic blunting, anabolic deficiency, and blunted satiety. Persons with SCI are also at high risk for shoulder, elbow, wrist, and hand injuries, including neuromusculoskeletal pathologies and nociceptive pain, as human upper extremities are poorly designed to facilitate chronic weight-bearing activities, including manual wheelchair propulsion, transfers, self-care, and day-to-day activities. This article reviews current literature on the relationship between obesity and increased body weight with upper extremity overuse injuries, detailing pathology at the shoulders, elbows, and wrists that elicit pain and functional decline and stressing the importance of weight management to preserve function.     

Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251)

BackgroundThis is the first-in-human study of icenticaftor, an oral potentiator of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) channel. Restoration of CFTR activity has shown significant clinical benefits, but more studies are needed to address all CFTR mutations.MethodsSafety, pharmacodynamics/pharmacokinetics of icenticaftor were evaluated in a randomized, double-blind, placebo-controlled study in healthy volunteers. Efficacy was assessed in adult CF patients with ≥1 pre-specified CFTR Class III or IV mutation (150 and 450 mg bid), or homozygous for F508del mutation (450 mg bid). Primary efficacy endpoint was change from baseline in lung clearance index (LCI_2.5). Secondary endpoints included %predicted FEV₁ and sweat chloride level.ResultsClass IV mutations were present in 22 patients, Class III in 2 (both S549N), and 25 were homozygous for F508del. Icenticaftor was well-tolerated in healthy and CF subjects with no unexpected events or discontinuations in the CF groups. The most frequent study-drug related adverse events in CF patients were nausea (12.2%), headache (10.2%), and fatigue (6.1%). Icenticaftor 450 mg bid for 14 days showed significant improvements in all endpoints versus placebo in patients with Class III and IV mutations; mean %predicted FEV₁ increased by 6.46%, LCI_2.5 decreased by 1.13 points and sweat chloride decreased by 8.36 mmol/L. No significant efficacy was observed in patients homozygous for a single F508del.ConclusionsIcenticaftor was safe and well-tolerated in healthy volunteers and CF patients, and demonstrated clinically meaningful changes in lung function and sweat chloride level in CF patients with Class III and IV CFTR mutations.ClinicalTrials.gov: NCT02190604     

Pseudoangiosarcomatous squamous cell carcinoma: first case report on penis

Pseudoangiosarcomatous squamous cell carcinoma, also called pseudovascular, pseudoangiomatoid or adenoid pseudovascular carcinoma, is an uncommon and highly aggressive variant of squamous cell carcinoma. Histologically, it is characterized by proliferation of atypical keratinocytes with acantholysis and formation of pseudovascular spaces, forming anastomosed channels lined with neoplastic cells that invade the dermis. These cells are positive for cytokeratin and negative for vascular markers such as CD31 and CD34. There are few reports of this variant in the literature. Skin, breast, lung and vulva involvement have been described, but to the best of our knowledge, no cases involving the penis has been described. This study aims to describe the first case of angiosarcomatous squamous cell carcinoma of the penis. The patient presented with a painful lesion in the penis associated with urinary retention. Macroscopic findings exhibited an ulcerative vegetating lesion that involving the entire glans and part of the penile body, as well as infiltration of penile structures and scrotal skin. Microscopy shows atypical proliferation of sarcomatous keratinocyte pattern mimicking vascular spaces. Human papilloma virus (HPV) biomarkers and polymerase chain reaction (PCR) were all negative. Advanced penile squamous cell carcinoma with aggressive lymph node metastasis. This report presents the first case of penile pseudoangiosarcomatous squamous cell carcinoma, as an important differential diagnosis.     

The associations of medical marijuana policies with opioid‐related health care utilization

ObjectiveTo examine the associations between medical marijuana policies and opioid‐related hospitalizations and emergency department visits.Data SourcesWe utilized quarterly rates of hospital discharge data from the Healthcare Cost and Utilization Project''s (HCUP) Fast Stats Database from 2005 to 2016 along with state‐level sociodemographic data from US Census Bureau and Bureau of Labor Statistics and opioid‐related state health policy data from publicly available sources for the analysis.Study DesignAnalyses were carried out using a difference‐in‐differences regression approach. We estimate heterogeneous effects of medical marijuana policies such as initial policy, presence of active dispensary, and home cultivation on opioid‐related hospitalizations and emergency department visits related to opioids.Data Collection/Extraction MethodsPublicly available secondary data were collected, linked, and analyzed. Observations with missing values for explanatory variables were excluded from the analysis.Principal FindingsRegression results indicate that type of medical marijuana policy has varying effects on opioid‐related hospitalizations and emergency department visits. States that allow home cultivation of medical marijuana experienced significant positive associations with opioid‐related hospitalizations and emergency department visits, while no effect was observed with medical marijuana dispensaries. Moreover, recreational marijuana policies were positively associated with opioid‐related hospitalizations.ConclusionsThe findings indicate that the effects of medical marijuana policies on opioid‐related hospitalizations and emergency department visits vary depending on the type of medical marijuana policy. Our findings indicate that the implementation of home cultivation of marijuana is positively associated with hospitalizations and emergency department visits related to opioids, suggesting that easier access to marijuana among opioid users may result in adverse health conditions that need treatment.