Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt : a case report

Gorham–Stout disease (GSD) is a rare bone disease characterized by massive osteolysis and lymphatic proliferation. The origin of the condition is unknown, and no established treatment protocol exists. Massive pleural effusion is a frequent complication of GSD in the thoracic region. We present the case of a 23-year-old male with thoracic GSD, subsequent paraparesis, and life-threatening pleural effusion. The patient was managed by a multidisciplinary team with a good recovery. The pleural effusion was successfully treated with a pleuro-peritoneal shunt. This is the first report of the use of this mini-invasive technique in the management of pleural effusion related to GSD. Further, we present the potential role of interleukin-6 and bone resorption markers in the measurement of the disease activity.publishedVersionPeer reviewe

Computed tomography -defined sarcopenia is associated with long-term survival among patients undergoing open thoracic aortic reconstruction

Background and objective: As markers of sarcopenia, psoas muscle areas and indexes measured from computed tomography images have been found to predict long-term mortality in cardiothoracic as well as other surgical cohorts. Our objective was to investigate the association between psoas muscle status, taking into account muscle density in addition to area, and survival among patients undergoing open thoracic aortic reconstruction. Methods: This was a retrospective registry study of a total of 451 patients treated with open surgery for thoracic aortic pathology. Psoas muscle area and density were measured from preoperative computed tomography images at the L3 and L4 lumbar levels. In addition, lean psoas muscle area was calculated by averaging sex-specific values of psoas muscle area and density. The association between mortality and psoas muscle status was analyzed with adjusted Cox-regression analysis. Results: The median age of the study population was 63 (interquartile range (IQR): 53–70) years. The majority were male (74.7%, n = 337) and underwent elective procedures (58.1% n = 262). Surgery of the ascending aorta was carried out in 90% of the patients, and 15% (n = 67) had concomitant coronary artery bypass surgery. Aortic dissection was present in 34.6% (n = 156) patients. Median follow-up time was 4.3 years (IQR: 2.2–7.4). During the follow-up, 106 patients (23.5%) died, with 55.7% of deaths occurring within the first four postoperative weeks. Psoas muscle parameters were not associated with perioperative mortality, but significant independent associations with long-term mortality were observed for psoas muscle area, density, and lean psoas muscle area with hazard ratios (HRs) of 0.63 (95% confidence interval (CI): 0.45–0.88), 0.62 (95% CI: 0.46–0.83), and 0.47 (95% CI: 0.32–0.69), respectively (all per 1-SD increase). Conclusions: Psoas muscle sarcopenia status is associated with long-term mortality after open thoracic aortic surgery.Peer reviewe

Non-small Cell Lung Cancer with Special Reference to Treatment Decisions and Staging by Molecular Methods, Immunohistochemistry and Computed Tomography

Keuhkosyöpä on yleisin sypäkuolemien aiheuttaja maailmassa. Arviolta kolmasosa potilaista voidaan leikata toteamisvaiheessa, ja parhaassakin tapauksessa jopa kolmasosasalla leikatuista tauti uusii viiden vuoden kuluessa. Tupakointi aiheuttaa 80-90% keuhkosyövistä. Vanhusväestön kasvaessa lisääntyy myös keuhkosyöpään sairastuvien riskiryhmä. Uusilla leikkaustekniikoilla voidaan tietyissä tapauksissa välttää keuhkon poisto, iäkkäitä potilaita on leikattu parantavasti ja hyvin tuloksin, ja uusia menetelmiä tutkitaan jotta ne potilaat joilla on korkea riski saada uusimiseen voitaisiin tunnistaa. Tämä erityisesti siksi että yhdistämällä solunsalpaajahoito leikkaukseen on pienissä aineistoissa julkaistu hyviä tuloksia. Tutkimuksessa selvitettiin keuhkoputkea muovaavan (bronkoplastisen) tekniikan soveltuvuutta keuhkosyövän hoidossa, yli 75-vuotiaiden keuhkosyöpäpotilaiden leikkaustuloksia, syöpäsolujen jakautumisen estävän p53 geenin toimimattomuuden aiheuttavan mutaation ennusteellista merkitystä, mikroskooppisia imusolmukkeiden etäpesäkkeitä havaitsevan immunohistokemiallisen värjäustekiinkan antamaa lisätietoa levinneisyydestä, sekä tietokonekerroskuvauksen luotettavuutta imusolmukelevinneisyyden arvioimiseksi. Tietyissä tapauksissa keuhkon ylälohkon kasvain, joka leviää pääkeuhkoputkeen, voidaan poistaa luotettavasti bronkoplastisella menetelmällä välttäen koko keuhkon poisto. Yli 75-vuotiailla, ja jopa yli 80-vuotiailla on mielekästä pyrkiä parantavaan keuhkosyöpäleikkaukseen, mutta lohkonpoistoa suuremmissa toimenpiteissä leikkaushaitan riski kasvaa huomattavasti. Mutaation vuoksi toimimaton p53 geeni oli yhteydessä huonoon ennusteeseen adenokarsinooma-solutyypin keuhkosyövässä, mutta ei epidermoidityypissä. Immunohistokemia ei osoittanut mikroskooppista levinneisyyttä imusolmukkeisiin mutta silti syöpä saattoi levitä agressiivisesti. Tietokonekerroskuvauksella ei voi täysin luotettavasti arvioida imusolmukelevinneisyyttä, vaikka leikattavuus voidaankin ennustaa suurella varmuudella. Kirurgian kehitys mahdollistaa keuhkonpoiston välttämisen joskin vain tietyissä tapauksissa. Vanhustenkin keuhkosyöpäkirurgiaan tulee suhtautua aktiivisesti mutta harkiten. Geenimutaatioiden osoittamisen kasvainkudoksessa, ja syöpäsoluihin sitoutuvien vasta-ainevärjäysten (immunohistokemia) käyttöä on aiheellista tutkia lisää, että korkean uusitumisriskin omaavat potilaat voidaan tunnistaa, erityisesti kun syövän yhdistelmähoidoissa on tapahtunut kehitystä. Tietokonekerroskuvaus ei ole täysin luotettava menetelmä imusolmukelevinneisyyden arvioimiseksi, vaan tämä tulee selvittää leikkauksen yhteydessä järjestelmällisen imusolmukepoiston avulla.Lung cancer is the commonest cause of cancer deaths world-wide. The elderly population is increasing, and consequently so are the numbers of those at risk of developing lung cancer due to cumulative life-time exposure to cigarette smoke. The majority of patients are beyond curative resection when diagnosed by reason of disseminated cancer or smoking-related co-morbidity. Until recently there has been no effective systemic treatment for lung cancer. Developments in multimodality therapy have now shown promising results in several small series of patients. This has underlined the importance of being able to detect those patients who have a poor prognosis even after a curative resection. The presence of lymph node metastases is the most important prognostic factor in lung cancer. However, even if the tumor is limited to the lung, and no nodal metastases are histologically detectable, approximately one third of patients will relapse with cancer within five years of a complete resection. Molecular methods which detect genetic mutations in the tumor may predict cancer cell aggression, while immunohistochemical methods can detect occult metastases of one to three cells in apparently histologically normal lymph nodes. Systematic perioperative lymph node dissection has become an essential part of accurate surgical staging. Bronchoplastic resections undertaken to circumvent pneumonectomy in lung cancer patients, and considered radical, resulted in a 40% cancer-related 5-year survival. In patients 75 years old or older who underwent surgery for lung cancer, a 56% relative 5-year survival compared to an age- and sex-matched standard population was achieved. Thirteen patients who were 80 years old or older were included in the study. While the mortality did not differ, the complication rate was significantly higher for procedures beyond lobectomy. Only stage IA patients had a cumulative 5-year survival which diverged from that of the other stages combined. After surgery, 88% of the patients were able to return to their homes. The presence of a genetic mutation of the p53 tumor supressor gene was determined by molecular methods in resected epidermoid lung carcinoma and adenocarcinoma patients. In multivariate analysis, a p53 mutation proved to be a statistically significant adverse prognostic factor in adenocarcinoma patients, but not in epidermoid carcinoma. The usefulness of immunostaining, using cytokeratin and Ber-EP4 antibodies, in detecting occult metastases in systematically removed intrapulmonary and mediastinal nodes which were normal upon histological examination was studied. Immunohistochemistry did not detect occult metastases, even when systemic metastatic disease was present. The lymph node stage, as determined by preoperative CT, was compared to the surgical-pathological stage in lung cancer patients who underwent systematic perioperative lymph node dissection. The lymph node status accorded with the surgical pathological stage in 69% of cases. Unsuspected mediastinal node metastases were found at surgery in 15% of patients. Bronchoplastic resection is an adequate oncological surgical procedure which can be used to obviate pneumonectomy in selected cases. Radical lung cancer surgery is justified in elderly patients, and a good postoperative course of events can be expected, but the extent of the resection should be carefully and individually considered. Molecular staging and the prognostic effect of a genetic p53 mutation, especially in adenocarcinoma, should be further studied in order to identify resected patients with a poor prognosis. The absence of detectable regional lymph node metastases, even when immunohistochemically sought, does not ensure a good postoperative prognosis. CT is not reliable for the accurate preoperative staging of intrathoracic lymph nodes

Inspiratory training and immediate lung recovery after resective pulmonary surgery : A randomized clinical trial

Background: Prompt and uneventful recovery after resective pulmonary surgery benefits patients by decreasing length and total costs of hospital stay. Postoperative physiotherapy has been shown to be advantageous for patient recovery in several studies and lately inspiratory muscle training (IMT) physiotherapy has been used also in thoracic patients. This randomized controlled trial intended to evaluate whether IMT is an efficient and feasible method of physiotherapy compared to water bottle positive expiratory physiotherapy (PEP) immediately after lung resections. Methods: Forty-two patients were randomly allocated into two intervention groups: water bottle PEP (n=20) and IMT group (n=22). Patients were given physiotherapeutic guidance once a day and patients were also instructed to do independent exercises. Measurements of pulmonary function were compared between the treatment groups according to intention to treat by using two-way repeated measures analysis of variances at three time points (preoperative, first postoperative day, and second postoperative day). Walking distance was measured at first and second postoperative day and similarly, evaluation of postoperative air leak during exercises was performed. Physiotherapy was modified or temporarily interrupted, if necessary, because of the air leak. Results: Postoperative pulmonary function tests were equal between the intervention groups. Air leak was relatively common after lung resections: 31% of all patients had mild or moderate/severe air leak at first postoperative day and 14% of all patients had mild to severe air leak at second postoperative day respectively. There were no statistically significant differences in occurrence of air leak between intervention groups, but water resistance had to be reduced or physiotherapy discontinued significantly more often among the water bottle PEP group patients (P=0.01). Walking distance improved slightly faster in the IMT group between the first and the second postoperative day when compared to the water bottle PEP group, but the difference between the groups was not statistically significant. Conclusions: IMT physiotherapy is equally effective to water bottle PEP training in postoperative physiotherapy after lung resection surgery evaluated with pulmonary function tests and walking distance. In addition, IMT physiotherapy is safe and more feasible form of physiotherapy during postoperative air leak compared to water bottle PEP.publishedVersionPeer reviewe

Treatment of right-sided aortic arch aneurysms with aberrant left subclavian artery with Kommerell's diverticulum using the frozen elephant trunk technique.

OBJECTIVES The ideal treatment for aneuryms of aberrant left subclavian arteries with Kommerell's diverticulum arising from right aortic arches remains open. METHODS Between January 2015 and December 2020, 5 patients with aneurysms from a right-sided aortic arch with aberrant left subclavian artery and Kommerell's diverticulum underwent repair by using the frozen elephant trunk technique in 3 aortic centres. Patients' characteristics were retrospectively reviewed and the surgical procedure and outcomes are presented. RESULTS The median age of the 2 male and 3 female patients was 59 (range from 49 to 63) years. The median operative times were as follows: surgery 405 min (range from 335 to 534), cardiopulmonary bypass time 244 min (range from 208 to 280) and aortic clamp time 120 min (from 71 to 184). The mean core temperature was 25.94°C (from 24 to 28). The intensive care unit stay was 4 days (range from 1 to 8) and the in-hospital stay 21 days (from 16 to 34). All patients were discharged and we observed no stroke or spinal cord ischaemia postoperatively. During the median follow-up time of 1003 days (range from 450 to 2306), 3 patients required subsequent thoracic endovascular distal stent graft extension. CONCLUSIONS The frozen elephant trunk technique is a good treatment option for patients with aneuryms of an aberrant left subclavian artery with Kommerell's diverticulum arising from right aortic arches. Secondary stent graft extension is a frequently needed component of the treatment concept

A randomized trial comparing inspiratory training and positive pressure training in immediate lung recovery after minor pleuro-pulmonary surgery

Background: Two respiratory physiotherapy modalities were compared in a randomized controlled trial on patients undergoing minor pleuro-pulmonary surgery. Methods: Forty-five patients were randomly allocated into positive expiratory pressure (PEP) therapy (n=23) and inspiratory muscle training (IMT) groups (n=22). Individualized group specific physiotherapeutic guidance was administered preoperatively, and once a day postoperatively. Patients also performed independent exercises and kept a logbook. Pain was assessed on a numerical reference scale (NRS). Volumetric pulmonary function values and walking distance were recorded preoperatively, and on first (POD1) and second postoperative days (POD2). Pre- and postoperative values were compared using two-way repeated measures analysis of variance. Results: Patient characteristics and pleuro-pulmonary interventions were similar between the groups. Thoracotomy was performed in 14/45 and video assisted surgery (VATS) in 31/45 of cases. Preoperative volumetric pulmonary functions were normal or slightly decreased in 29/45, and fell significantly (P<0.001) on the first postoperative day (POD1) and improved but remained significantly lower on the second postoperative day. The recovery of mean FEV1, FIV1 and FIVC values was greater in the IMT than in the PEP group between POD1 and POD2, but without significant difference. The corresponding relative to preoperative values were higher in the IMT group, with a significant difference in FEV1 (P=0.045). Also relative PEF and FIV1 values seemed to be slightly higher in the IMT compared to the PEP group, but not significantly. Average NRS values for pain were lower in the IMT group (P=0.010) but only on POD1. Air leak was noted in 4/45 patients, two in each group, on POD1, and two in PEP groups and one in IMT group on POD2. Mean measured walking distances between groups did not differ. Mean hospital stay was 4 days in the PEP group and 3 days in the IMT group. There was no hospital mortality. Conclusions: Pulmonary function values decreased significantly after minor lung resections, supporting rehabilitative respiratory physiotherapy to avoid postoperative pulmonary complications (PPCs). Both PEP and IMT training were well tolerated and equally efficient when comparing spirometry values at three time points. IMT appeared advantageous regarding relative FEV1 recovery and immediate postoperative pain.publishedVersionPeer reviewe

Early and midterm results of frozen elephant trunk operation with Evita open stent-graft in patients with Marfan syndrome : results of a multicentre study

Background: Endovascular treatment of patients with Marfan syndrome (MFS) is not recommended. Hybrid procedures such as frozen elephant trunk (FET), which combines stent-graft deployment with an integrated non-stented fabric graft for proximal grafting and suturing, have not been previously evaluated. The aim of this study was to assess the safety and feasibility of FET operation in patients with MFS. Methods: Patients enrolled in the International E-vita Open Registry (IEOR) who underwent FET procedure between January 2001 and February 2020 meeting Ghent criteria for MFS were included in the study. Early and midterm results were retrospectively analyzed. Preoperative, postoperative and follow-up computed tomography angiography scans were analysed. Results: We analyzed 37 patients [mean age 38 ± 11 years, 65% men]. Acute or chronic aortic dissection was present in 35 (95%) patients (14 and 21 patients respectively). Two (5%) patients had an aneurysm without dissection. Malperfusion syndrome was present in 4 patients. Twenty-nine (78%) patients had history of aortic surgical interventions. The 30-day and in-hospital mortality amounted to 8 and 14% respectively. False lumen exclusion was present in 73% in stented segment in last postoperative CT. The overall 5-year survival was 71% and freedom from reintervention downstream was 58% at 5 years. Of the nine patients who required reintervention for distal aortic disease, one patient died. Conclusions: FET operation for patients with MFS can be performed with acceptable mortality and morbidity. In long-term follow-up no reinterventions on the aortic arch were required. FET allows for easier second stage operations providing platform for surgical and endovascular reinterventions.publishedVersionPeer reviewe