A severe presentation of acute generalized exanthematous pustulosis with non-infectious circulatory shock in an adolescent.

We present a severe case of acute generalized exanthematous pustulosis (AGEP) secondary to trimethoprim-sulfamethoxazole complicated by non-infectious circulatory shock in a 16-year-old boy. Hemodynamic instability has been reported as a complication of AGEP in adults, but is rarely observed in pediatric patients. The patient we present demonstrated characteristic cutaneous findings of AGEP including isolated non-follicular, sterile pustules on a background of erythema with involvement at intertriginous areas and subsequently developed non-infectious circulatory shock. This case expands the spectrum of possible clinical presentations for AGEP in pediatric patients.

Concurrent Chondrodysplasia Punctata Type 2 (Conradi-Hunermann-Happle Syndrome) and Ichthyosis Vulgaris in Teenaged Twin Girls.

We present concurrent X-linked chondrodysplasia punctata and ichthyosis vulgaris in adolescent fraternal twin girls, notable for initial presentation with dry skin in adolescence, characterized by dark-brown scale typical of ichthyosis vulgaris and blaschkolinear, atrophic, scaly plaques. This constellation of findings prompted further genetic investigation. Using a multigene approach to examine 39 genes associated with congenital ichthyosis, next-generation sequencing revealed a novel heterozygous missense mutation at a mutational hotspot in the EBP gene c.439C>T (p.R147C) in conjunction with a single nonsense mutation in the FLG gene (p.R501X) in both sisters. These individuals highlight the clinical variability of Conradi-Hunermann-Happle syndrome, illustrate the possibility of co-occurrence of rare and common forms of ichthyosis, and demonstrate the utility of multigene analysis.

Allergan Style 410 Implants for Breast Reconstruction: A Prospective Study in Efficacy, Safety, and Symmetry.

BACKGROUND: Implant-based breast reconstruction is performed with both saline and silicone. In 2001, a study was conducted in which a novel implant made of highly cohesive silicone gel in anatomical shape was used. It differs from others because it holds its anatomical shape and forces tissue to conform to the implant. METHODS: Two hundred twelve consecutive patients who underwent reconstruction with Allergan 410 cohesive gel anatomical implants were enrolled prospectively over a 12-year period. Complications and satisfaction rates were recorded. RESULTS: Average age of the patients was 48.5 years and average body mass index was 24.1 kg/m. Bilateral reconstruction was performed in 83 percent of patients. Long-term follow-up was achieved in all patients and averaged 3.3 years (range, 0.5 to 10.2 years). The overall complication rate was 19.8 percent; most were minor complications, such as rippling (9.4 percent) and asymmetry (4.2 percent). Major complications included infection (2.4 percent), malposition (1.4 percent), capsular contracture (0.9 percent), seroma (0.5 percent), extrusion (0.5 percent), and implant rupture (0.5 percent). The rate of implant-related reoperation, excluding cancer recurrence, was 9.0 percent, consisting of implant replacement (5.7 percent), implant removal (1.9 percent), and implant repositioning (1.4 percent). Patient satisfaction averaged 4.83 (range, 2 to 5) on a five-point scale. Surgeon satisfaction averaged 4.9 (range, 2 to 5). CONCLUSIONS: Allergan 410 cohesive gel anatomical implants have a favorable risk profile in reconstruction, with excellent patient and surgeon satisfaction. This novel implant allows for a paradigm shift in implant-based breast reconstruction. Surgeons can now use an implant to help shape the final contour of the breast mound rather than rely on mastectomy flaps and suture techniques to create aesthetic contours needed to create an attractive breast reconstruction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Calciphylaxis: Controversies in Pathogenesis, Diagnosis and Treatment.

Calcific uremic arteriolopathy, otherwise known as calciphylaxis, is a rare disease characterized by skin ulceration and tissue necrosis, likely the result of vascular calcification with accompanying intimal hypertrophy and small vessel thrombosis. Although most often associated with end-stage renal disease, it has also been seen in a number of other disorders (collectively referred to as nonuremic calciphylaxis). The purpose of this review is to summarize and analyze the currently available literature regarding the pathophysiology, risk factors, clinical presentation, diagnostic features and treatment modalities for this exceptionally uncommon illness. A series of recommended treatments is proposed for optimal treatment of calciphylaxis lesions.

Pyoderma gangrenosum (PG) associated with levamisole-adulterated cocaine: Clinical, serologic, and histopathologic findings in a cohort of patients.

BACKGROUND: Recently, isolated reports of pyoderma gangrenosum (PG) secondary to levamisole-contaminated cocaine have been described, with similar serologic findings to the vasculopathic presentation. OBJECTIVE: We sought to describe clinical, histopathological, and serologic findings in 8 patients with PG associated with levamisole-contaminated cocaine. METHODS: Eight consecutive patients presenting with this disease spanning the period from 2011 to 2015 were included for the cohort. Observed variables included: lesion distribution, morphology, serologic titers, and histopathologic evaluation for vasculitis and vasculopathy. RESULTS: All patients reported cocaine exposure prior to the onset of lesions resembling PG. Lesions appeared primarily on the upper (6 of 8 patients) and lower (all 8 patients) extremities. Most patients demonstrated elevated titers for p-ANCA and antiphospholipid antibodies, and a diffuse dermal infiltrate dominated by neutrophils was seen in all biopsy specimens. Lesions improved or remained stable with conservative management or short courses of steroids, and recurrence was only noted on re-exposure to adulterated cocaine. LIMITATIONS: The study is limited by sample size. CONCLUSIONS: PG may occur after exposure to levamisole-adulterated cocaine. Clinical and histopathological findings resemble those seen in conventional forms of PG, whereas serologic findings mirror those seen in other levamisole-associated vasculopathic or vasculitic eruptions. Cocaine avoidance represents a cornerstone of management in these patients.

Analysis of the American Society of Anesthesiologists Physical Status Classification System and Caprini Risk Assessment Model in Predicting Venous Thromboembolic Outcomes in Plastic Surgery Patients.

BACKGROUND: Venous thromboembolism (VTE) can be a fatal outcome of plastic surgery. Risk assessment models attempt to determine a patient's risk, yet few studies have compared different models in plastic surgery patients. OBJECTIVE: The authors investigated preoperative ASA physical status and 2005 Caprini scores to determine which model was more predictive of VTE. METHODS: A retrospective chart review examined 1801 patients undergoing contouring and reconstructive procedures from January 2008 to January 2012. Patients were grouped into risk tiers for ASA scores (1-2 = low, 3+ = high) with 2 cutoffs for Caprini scores (1-4 = low, 5+ high; 1-5 = low, 6+ = high), then re-stratified into 3 tiers using Caprini score cutoffs (1-4 = low, 5-8 = high, 9+ = highest; 1-5 = low, 6-8 = high, 9+ = highest). Median scores of VTE patients were compared to those without VTE. Odds ratio and chi-squared analyses were performed. RESULTS: Of the 1598 patients included in the study, 1.50% developed VTE. Median ASA scores differed significantly between comparison groups but Caprini scores did not vary regardless of cutoff. When examining the 2-tiered Caprini scores, using low risk = 1-5 showed a significant relationship between risk tier and DVT development (P = 0.0266). CONCLUSION: The ASA system yielded the highest odds ratio of VTE development between low and high-risk patients. The Caprini model captured more patients with VTE in its high-risk category. Combining the two models for a more heuristic approach to preoperative care may identify patients at higher risk. LEVEL OF EVIDENCE: 4 Risk.

Evaluation of the American Society of Anesthesiologists Physical Status classification system in risk assessment for plastic and reconstructive surgery patients.

BACKGROUND: The American Society of Anesthesiologists Physical Status (ASA-PS) classification is a ranking system that quantifies patient health before anesthesia and surgery. Some surgical disciplines apply the ASA-PS to gauge a patient's likelihood of developing postoperative complications. OBJECTIVE: In this study, the authors analyze whether ASA-PS scores can successfully predict risk for postoperative complications in plastic and reconstructive operations. METHODS: The authors retrospectively reviewed the charts of 1801 patient procedures and selected for inclusion 1794 complex plastic and reconstructive operations that took place at 1 of several academic medical institutions between January 2008 and January 2012. ASA-PS scores, patient comorbidities, and postoperative complications were analyzed. Percentile data were treated with tests for proportions. Nonpercentile data were analyzed through comparison of means (t test). Low-risk (ASA 1-2) and high-risk (ASA 3+) groups were compared with simple odds ratios. RESULTS: For the 1430 women and 364 men in the patient cohort (average age, 49.5 years), the overall complication rate was 27.7%. When patients with complications were compared to those without, body mass index, operation time, recent major surgery, diabetes, hypertension, renal disease, cancer, and oral contraceptive use were statistically significant. After high-risk (n = 398) and low-risk (n = 1396) groups were identified, infection, delayed wound healing, deep vein thrombosis, and overall complications had significantly increased incidence in the high risk group. Notably, deep vein thrombosis displayed the highest odds ratio (4.17) and a complication rate increase from 0.93% to 3.77%. CONCLUSIONS: ASA-PS scores can be used either as substitutes for or as adjuncts to questionnaire-based risk assessment methods in plastic surgery. In addition to deducing significant findings for deep vein thrombosis incidence, ASA-PS scores hold important predictive associations for multiple non-venous thromboembolism complications, providing a broader measurement for postoperative complication risks. LEVEL OF EVIDENCE: 4.

Application of the Caprini risk assessment model in evaluation of non-venous thromboembolism complications in plastic and reconstructive surgery patients.

BACKGROUND: The Caprini Risk Assessment Model is used to categorize patient risk for venous thromboembolism (VTE) events; its predictive associations have been repeatedly corroborated. Calculating scores involves consideration of systemic factors that may predict other postoperative complications. OBJECTIVE: This study investigates whether Caprini scores can be applied to non-VTE complications. METHODS: The authors undertook a retrospective chart review of 1598 encounters for a series of complex reconstructive and body contouring operations at an academic medical institution. Input variables included Caprini score components, patient comorbidities, and prophylactic use of antithrombotic drugs. Output variables were postoperative complications. Tests for proportions were performed on percentile data. Nonpercentile data were treated with comparison of means (t test). Odds ratios for complications were calculated for stratified risk groups and compared. RESULTS: The overall complication rate was 28.03%. Deep vein thrombosis (DVT) incidence was 1.50%. Differences in age, body mass index (BMI), operation time, hypertension, diabetes, renal disease, and cancer were statistically significant between patients who experienced complications and those who did not. For DVT versus DVT-free patients, differences in sex, BMI, operation time, smoking status, diabetes, hypertension, and prior DVT were significant. Caprini scores identified 628 encounters as low risk (0-4) and 970 as high risk (>5). Dehiscence, infection, necrosis, seroma, hematoma, and overall complication rate significantly increased the incidence for the high-risk group. CONCLUSIONS: Caprini scores can be used as valuable predictors for some non-VTE postoperative complications (dehiscence, infection, seroma, hematoma, and necrosis). In addition to VTE events, clinicians should pay special attention to clinical signs indicative of the complications listed above when dealing with high-risk, high-Caprini score patients.