[Acute soft Tissue Infection with impending Loss of Finger in amniotic Band Syndrome of a 22-years-old Patient with palmoplantar Keratoderma congenital Alopecia Syndrome Type II]. / Akute Weichteilinfektion mit drohendem Fingerverlust bei Schnürfurchensyndrom einer 22-jährigen Patientin mit palmoplantarem Keratoderm-kongenitalem Alopezie-Syndrom Typ II.

We report on the treatment of a 22-year-old female patient with an acute soft tissue infection in the area of an amniotic band due to palmoplantar keratoderma congenital alopecia syndrome (PPKCA) type II, a very rare genodermatosis with less than 20 cases described in literature. An acute soft tissue infection distal from the pre-existing constriction ring with hyperkeratosis on the right small finger led to a decompensation of the venous and lymphatic drain with imminent loss of the finger. Due to urgent surgical treatment with decompression and debridement of the dorsal soft tissue infection, microsurgical circular resection of the constriction ring and primary wound closure the finger could be preserved. After soft tissue consolidation and hand therapy, the patient achieved free movement of the small finger with subjective freedom of symptoms and good aesthetic results.

Propensity matched comparison of extrapleural pneumonectomy and pleurectomy/decortication for mesothelioma patients.

OBJECTIVES: The objective of this retrospective study was to assess perioperative outcomes, overall survival and freedom from recurrence after induction chemotherapy followed by extrapleural pneumonectomy (EPP) or pleurectomy/decortication (P/D) in patients with mesothelioma in a propensity score matched analysis. METHODS: Between September 1999 and August 2015, 167 patients received multimodality treatment (platinum-based chemotherapy followed by EPP [ n = 141] or P/D [ n = 26]). We performed 2:1 propensity score matching for gender, laterality, epithelioid histological subtype and International Mesothelioma Interest Group (iMig) stage (52 EPP and 26 P/D). RESULTS: Postoperative major morbidity (48% vs 58%, P = 0.5) was similar in both groups; however, the complication profile and severity were different and favoured P/D; the 90-day mortality (8% vs 0%, P = 0.3) rate was lower in P/D although not statistically significant. Prolonged air leak (≥10 days) occurred in 15 patients (58%) undergoing P/D. The intensive care unit stay was significantly longer after EPP ( P = 0.001). Freedom from recurrence was similar for both groups (EPP: median 15 months, 95% confidence interval [CI]: 10-21; P/D: 13 months, 95% CI: 11-17) ( P = 0.2). Overall survival was significantly longer for patients undergoing P/D (median 32 months, 95% CI: 29-35) compared to EPP (23 months, 95% CI: 21-25) ( P = 0.031), but in the P/D group many cases were censored (73%) and the follow-up time was relatively short. CONCLUSIONS: P/D and EPP seem to have similar rates of major morbidity, although the profile of complications is different and more severe after EPP. Freedom from recurrence is comparable in both groups whereas improved overall survival needs to be confirmed in a large patient group with longer follow-up.

Relapse pattern and second-line treatment following multimodality treatment for malignant pleural mesothelioma.

OBJECTIVES: To analyse the relapse pattern and influence of second-line treatment after recurrence of malignant pleural mesothelioma (MPM) in patients who had previously undergone multimodality treatment. METHODS: Between September 1999 and December 2013, 136 patients underwent macroscopic complete resection (MCR) by extrapleural pneumonectomy after induction chemotherapy for MPM. We analysed 106 patients who presented with recurrent disease until October 2014. Data were retrieved from our mesothelioma database, with additional information regarding precise localization gathered by reviewing the imaging and medical records. RESULTS: The overall recurrence rate was 78% (106/136 patients). The median freedom from recurrence was 9 months after surgery [95% confidence interval (95% CI) 7-10]. Local recurrence only was observed in 33 patients (31%), distant metastases only in 27 patients (26%) and simultaneous distant and local recurrence in 46 patients (43%). Local recurrence was observed significantly less frequently in patients having received adjuvant radiotherapy (19 vs 47%, P = 0.003), but there was no significant impact on overall survival (OS) [radiation: 22 months (95% CI 19-24); no-radiation: 23 months (95% CI 18-27), P = 0.6]. The median OS was 22 months (95% CI 21-24), median post-recurrence survival (PRS) was 7 months (95% CI 5-9) and patients with local recurrence only survived significantly longer (12 months, 95% CI 8-16) compared with patients with distant recurrence only (5 months, 95% CI 2-8) or distant plus local relapse (6 months, 95% CI 3-9; P = 0.04). A total of 78 patients received a second-line therapy after tumour recurrence: chemotherapy (n = 48), local radiotherapy (n = 9), surgery (n = 10) or a combination thereof (n = 11). Patients undergoing second-line treatment survived significantly longer compared with patients not receiving therapy (P < 0.0005). The median PRS after surgery was significantly longer than that of patients receiving chemo-, radio- or chemo-radiotherapy (P = 0.04). CONCLUSIONS: Local recurrence of MPM remains the most frequent type of relapse even after multimodality treatment including MCR. In the present cohort, active treatment seems beneficial to the patient since surgical excision of local tumour relapse has good long-term outcome in selected patients. Thus, second-line treatment may prolong PRS; however, these results need to be confirmed in a prospective manner.

Repeated lung volume reduction surgery is successful in selected patients.

OBJECTIVES: Lung volume reduction surgery (LVRS) improves dyspnoea, quality of life and may even prolong survival in carefully selected patients with end-stage emphysema. The benefit may be sustained for several years and vanishes with the natural progression of the disease. Data on repeated surgical treatment of emphysema are scarce. The aim of this study was to evaluate the safety, effects and outcomes of repeated LVRS (Re-LVRS) in patients no longer benefiting from their initial LVRS. METHODS: Between June 2002 and December 2013, 22 patients (9 females) with advanced emphysema underwent Re-LVRS at a median of 60 months (25-196) after their initial LVRS. While initial LVRS was performed thoracoscopically as a bilateral procedure, Re-LVRS was performed unilaterally by a video-assisted thoracoscopic technique in 19 patients and, due to adhesions, by thoracotomy in 3 patients. Pulmonary function test (PFT) was performed at 3 and 12 months postoperatively. RESULTS: Lung function at Re-LVRS was similar to that prior to the first LVRS. The 90-day mortality rate was 0%. The first patient died 15 months postoperatively. The median hospitalization time after Re-LVRS was significantly longer compared with the initial LVRS [14 days, interquartile range (IQR): 11-19, vs 9 days, IQR: 8-14; P = 0.017]. The most frequent complication was prolonged air leak with a median drainage time of 11 days (IQR: 6-13); reoperations due to persistent air leak were necessary in 7 patients (32%). Five patients (23%) had no complications. Lung function and Medical Research Council (MRC) score improved significantly for up to 12 months after Re-LVRS, with results similar to those after initial bilateral LVRS. The average increase in the forced expiratory volume in 1 s (FEV1) was 25% (a 7% increase over the predicted value or 0.18 l) at 3 months, and the mean reduction in hyperinflation, assessed by relative decrease in RV/TLC (residual volume/total lung capacity), was 12% at 3 months (a decrease of 8% in absolute ratios). The mean MRC breathlessness score decreased significantly after 3 months (from 3.7 to 2.2). CONCLUSIONS: Re-LVRS can be performed successfully in carefully selected patients as a palliative treatment. It may be performed as a bridge to transplantation or in patients with newly diagnosed intrapulmonary nodules or during elective cardiac surgery. Morbidity is acceptable and outcomes may be satisfactory with significantly improved lung function and reduced dyspnoea for at least 12 months postoperatively.