Two-meshes approach in posterior component separation with transversus abdominis release: the IMPACT study (Italian Multicentric Posterior-separation Abdominal Complex hernia Transversus-release).

BACKGROUND: Surgical management of large ventral hernias (VH) has remained a challenge. Various techniques like anterior component separation and posterior component separation (PCS) with transversus abdominis release (TAR) have been employed. Despite the initial success, the long-term efficacy of TAR is not yet comprehensively studied. Authors aimed to investigate the early-, medium-, and long-term outcomes and health-related quality of life (QoL) in patients treated with PCS and TAR. METHODS: This multicenter retrospective study analyzed data of 308 patients who underwent open PCS with TAR for primary or recurrent complex abdominal hernias between 2015 and 2020. The primary endpoint was the rate of hernia recurrence (HR) and mesh bulging (MB) at 3, 6, 12, 24, and 36 months. Secondary outcomes included surgical site events and QoL, assessed using EuraHS-QoL score. RESULTS: The average follow-up was 38.3 ± 12.7 months. The overall HR rate was 3.5% and the MB rate was 4.7%. Most of the recurrences were detected by clinical and ultrasound examination. QoL metrics showed improvement post-surgery. CONCLUSIONS: This study supports the long-term efficacy of PCS with TAR in the treatment of large and complex VH, with a low recurrence rate and an improvement in QoL. Further research is needed for a more in-depth understanding of these outcomes and the factors affecting them.

Narcolepsies, update in 2023.

Narcolepsy type 1 (NT1) and type 2 (NT2), also known as narcolepsy with and without cataplexy, are sleep disorders that benefited from major scientific advances over the last two decades. NT1 is caused by the loss of hypothalamic neurons producing orexin/hypocretin, a neurotransmitter regulating sleep and wake, which can be measured in the cerebrospinal fluid (CSF). A low CSF level of hypocretin-1/orexin-A is a highly specific and sensitive biomarker, sufficient to diagnose NT1. Orexin-deficiency is responsible for the main NT1 symptoms: sleepiness, cataplexy, disrupted nocturnal sleep, sleep-related hallucinations, and sleep paralysis. In the absence of a lumbar puncture, the diagnosis is based on neurophysiological tests (nocturnal and diurnal) and the presence of the pathognomonic symptom cataplexy. In the revised version of the International Classification of sleep Disorders, 3rd edition (ICSD-3-TR), a sleep onset rapid eye movement sleep (REM) period (SOREMP) (i.e. rapid occurrence of REM sleep) during the previous polysomnography may replace the diurnal multiple sleep latency test, when clear-cut cataplexy is present. A nocturnal SOREMP is very specific but not sensitive enough, and the diagnosis of cataplexy is usually based on clinical interview. It is thus of crucial importance to define typical versus atypical cataplectic attacks, and a list of clinical features and related degrees of certainty is proposed in this paper (expert opinion). The time frame of at least three months of evolution of sleepiness to diagnose NT1 was removed in the ICSD-3-TR, when clear-cut cataplexy or orexin-deficiency are established. However, it was kept for NT2 diagnosis, a less well-characterized disorder with unknown clinical course and absence of biolo biomarkers; sleep deprivation, shift working and substances intake being major differential diagnoses. Treatment of narcolepsy is nowadays only symptomatic, but the upcoming arrival of non-peptide orexin receptor-2 agonists should be a revolution in the management of these rare sleep diseases.

IPOM plus versus IPOM standard in incisional hernia repair: results of a prospective multicenter trial.

PURPOSE: Laparoscopic ventral hernia repair is a well-established technique with satisfying outcomes even at long term for the treatment of incisional and ventral hernia. However, the literature debate is still ongoing regarding the preferred surgical technique. Nowadays, two approaches are commonly adopted: the intraperitoneal onlay mesh repair (sIPOM) and the intraperitoneal onlay mesh reinforcement with defect closure before mesh placement (pIPOM). The aim of this prospective analysis is to compare the postoperative outcomes of patients treated for incisional hernia (IH) with sIPOM and pIPOM after 36 months follow-up in terms of recurrence, quality of life and wound events. METHODS: Patients receiving pIPOM and sIPOM for IH were actively followed up for 36 months. At the outpatient clinic, hernia recurrence (HR), mesh bulging (MB), quality of life with the Gastrointestinal Quality of Life Index (GIQLI) and wound events were assessed. RESULTS: Between January 2015 and January 2019, 98 patients underwent a pIPOM and 89 underwent an sIPOM. At 36 months, nine patients (4 in pIPOM and 5 in sIPOM) experienced an HR, while MB was recorded in four patients in pIPOM and nine in sIPOM. No statistically significant difference could be identified also in terms of final GIQLI score and wound events. CONCLUSIONS: LVHR with or without fascial closure, also in our study, provides satisfactory results in terms of safety and efficacy. The discordant results in the literature are probably related to independent variables such as the type of mesh, the type of suture and closure technique. Therefore, was the funeral of sIPOM done too early? STUDY DATASET IS AVAILABLE ON CLINICALTRIALS. GOV ID: NCT05712213.

A comparative blind study between skin biopsy and seed amplification assay to disclose pathological α-synuclein in RBD.

To compare the diagnostic accuracy of the immunofluorescence (IF) technique and aSyn-seed amplification assay (aSyn-SAA) of skin and cerebrospinal fluid (CSF) in disclosing pathological α-syn in idiopathic idiopathic REM sleep behavior disorder (iRBD) as early phase of a synucleinopathy. We prospectively recruited 41 patients with iRBD and 40 matched clinical controls including RBD associated with type 1 Narcolepsy (RBD-NT1, 21 patients), iatrogenic causes (2 pt) or OSAS (6 pt) and 11 patients with peripheral neuropathies. IF from samples taken by skin biopsy and aSyn-SAA from skin and CSF samples were analysed blinded to the clinical diagnosis. IF showed a good diagnostic accuracy (89%) that was lower in the case of skin and CSF-based aSyn-SAA (70% and 69%, respectively) because of a lower sensitivity and specificity. However, IF showed a significant agreement with CSF aSyn-SAA. In conclusion, our data may favor the use of skin biopsy and aSyn-SAA as diagnostic tools for a synucleinopathy in iRBD.

Is absorbable mesh useful in preventing parastomal hernia after emergency surgery? The PARTHENOPE study.

PURPOSE: Colostomy is a frequent event in oncological or inflammatory bowel diseases. Its related morbidity includes retraction, infection and parastomal hernia (PH), which is a quite common late complication. Several surgical options are available for PH repair, the majority including mesh. However, results are often disappointing with relevant recurrence rates, up to 33%. The study aim was to assess the feasibility and effectiveness of prophylactic biosynthetic mesh (BIO-A®, polyglycolide-trimethylene carbonate copolymer) placed during colostomy fashioning, in reducing PH. A prospective randomized controlled double-blind trial was conducted from January 2014 to December 2019 to compare conventional end-colostomy with end-colostomy reinforced with BIO-A mesh in ante-rectus position in patients undergoing colon diversion in emergency surgery. METHODS: Patients were clinically followed up at 3, 6, and 12 months and received a CT scan at 6 and 12 months. The postoperative morbidity and wound events were also evaluated. RESULTS: 55 patients receiving conventional colostomy considered as Control Group and 55 patients receiving BIO-A mesh supported colostomy (Mesh Group) were included in the study. At 12 months, the incidence of PH was 9 (12.7%) and 24 (43.6%) in the Mesh Group and Control Group, respectively (p < 0.05). Postoperative morbidity was similar between Mesh Group and Control Group (7 [12.7%] vs 4 [7.3%], respectively; p = 0.340). The multivariable analysis showed that not using a mesh (p = 0.042), age > 70 years (p = 0.041), diabetes (p < 0.001), colon dilation > 7 cm (p < 0.0001) and COPD (p = 0.009) were all related with postoperative PH. CONCLUSIONS: The prophylactic BIO-A mesh positioning during colostomy is an effective procedure reducing PH incidence at a 1 years follow-up guaranteeing low postoperative morbidity. STUDY DATASET IS AVAILABLE ON CLINICALTRIALS. GOV ID: NCT04436887.

Consistent skin α-synuclein positivity in REM sleep behavior disorder - A two center two-to-four-year follow-up study.

OBJECTIVE/METHODS: Phosphorylated alpha-synuclein (p-syn) in dermal nerves of patients with isolated REM sleep behavior disorder (iRBD) is detectable by immunofluorescence-labeling. Skin-biopsy-p-syn-positivity was recently postulated to be a prodromal marker of Parkinson's disease (PD) or related synucleinopathies. Here, we provide two-to four-year clinical and skin biopsy follow-up data of 33 iRBD patients, whose skin biopsy findings at baseline were reported in 2017. RESULTS: Follow-up biopsies were available from 25 patients (18 positive at baseline) and showed consistent findings over time in 24 patients. One patient converted from skin-biopsy-negativity to -positivity. P-syn-positivity was observed in iRBD patients who still had a normal FP-CIT-SPECT two years later. Clinically, five of the 23 at baseline skin-biopsy-positive patients (21.7%) had converted to PD or dementia with Lewy bodies at follow-up, but none of the skin-biopsy-negative patients. CONCLUSIONS: Dermal p-syn in iRBD is most probably an early consistent marker of synucleinopathy and may support other indicators of conversion to manifest disease state.

Prophylactic sublay non-absorbable mesh positioning following midline laparotomy in a clean-contaminated field: randomized clinical trial (PROMETHEUS).

BACKGROUND: Incisional hernia is a frequent postoperative complication after midline laparotomy. Prophylactic mesh augmentation in abdominal wall closure after elective surgery is recommended, but its role in emergency surgery is less well defined. METHODS: This prospective randomized trial evaluated the incidence of incisional hernia in patients undergoing urgent midline laparotomy for clean-contaminated surgery. Closure using a slowly absorbable running suture was compared with closure using an additional sublay mesh (Parietex ProGrip™). Patients were randomized just before abdominal wall closure using computer-generated permuted blocks. Patients, care providers, staff collecting data, and those assessing the endpoints were all blinded to the group allocation. Patients were followed up for 24 months by means of clinical and ultrasonographic evaluations. RESULTS: From January 2015 to June 2018, 200 patients were randomized: 100 to primary closure (control group) and 100 to Parietex ProGrip™ mesh-supported closure (mesh group). Eight patients in the control group and six in the mesh group were lost to follow-up. By 24 months after surgery, 21 patients in the control group and six in the mesh group had developed incisional hernia (P = 0.002). There was no difference between groups in the incidence of haematoma (2 versus 5; P = 0.248) and superficial wound infection (4 versus 5; P = 0.733). Multivariable analysis confirmed the role of mesh in preventing incisional hernia (odds ratio 0.11, 95 per cent c.i. 0.03 to 0.37; P < 0.001). One patient in the mesh group required mesh removal because of deep infection. CONCLUSION: Prophylactic mesh-augmented abdominal wall closure after urgent laparotomy in clean-contaminated wounds is safe and effective in reducing the incidence of incisional hernia. Registration number: NCT04436887 (http://www.clinicaltrials.gov).

Objective rest-activity cycle analysis by actigraphy identifies isolated rapid eye movement sleep behavior disorder.

BACKGROUND AND PURPOSE: Isolated rapid eye movement (REM) sleep behavior disorder (iRBD) is characterized by abnormal behaviours during REM sleep. Several studies showed that iRBD is a prodromal stage of synucleinopathies. Therefore, identifying iRBD in the general population is of utmost importance. In this study, we explore whether the assessment of rest-activity rhythm features can distinguish patients with iRBD from patients with disorders characterized by other pathological motor activity during sleep and healthy controls. METHODS: Nineteen patients with video-polysomnographic diagnosis of iRBD, 39 patients with other disorders with motor activity during sleep [19 with restless leg syndrome (RLS) and 20 with untreated sleep apnea syndrome (SAS)] and 16 healthy controls underwent 2-week actigraphy and video-polysomnography, and completed REM sleep behavior disorder screening questionnaires. Non-parametric analyses were applied to assess the rest-activity rhythm features. RESULTS: Patients with iRBD showed lower sleep efficiency, increased estimated wake after sleep onset and increased frequency of prolonged activity bouts compared to those with RLS and controls, while no difference emerged compared with SAS patients. Moreover, patients with iRBD presented increased occurrence of estimated nap in comparison to those with RLS, those with SAS and controls. The I < O, a 24-h measure that expresses the relationship between nocturnal and diurnal motor activity intensity, distinguished patients with iRBD from those with RLS, those with SAS and controls, with an area under the curve greater than that of REM sleep behavior disorder screening questionnaires. An I < O of 98.32 shows the best balance between sensitivity (63.2%) and specificity (89.1%). DISCUSSION: The I < O index distinguished iRBD patients from those with other pathological motor activity during sleep and controls, confirming its use as an objective measure suitable to screen large at-risk populations.

Safety and efficacy of prophylactic resorbable biosynthetic mesh following midline laparotomy in clean/contemned field: preliminary results of a randomized double blind prospective trial.

BACKGROUND: Incisional hernia (IH) is one of the most common sequelae of laparotomy. MATERIALS AND METHODS: We present a double-blind randomized study examining feasibility, safety and incisional hernia rate using a prophylactic Bio-A biosynthetic stripe (Gore) in a sub-lay position after midline laparotomy in patients undergoing operations in clean-contaminated and contaminated field. One hundred patients who underwent a midline laparotomy of at least 10 cm in a clean-contaminated and contaminated field were considered. Patients were divided into two groups: [Group A closed in double layer using PDS 0 with WL/SL of 1:4; Group B closure in double layer using PDS 0 and sub-lay positioning a 3 cm-wide BIO A (Gore) strip extended for the entire length of the incision]. The primary objective of the study was to identify IH rate in the two groups at 1- and 2-year follow-up. Secondary objective was to identify any differences in the two groups in terms of post-operative pain, morbidity and mortality. RESULTS: Out of a total of 100 patients included in the study, a 2-year follow-up was possible for 47 patients in group A and 45 in group B. The incidence of IH was 11/47 in group A (22%) and 3/45 in group B (6%) [p < 0.01]. Furthermore, no statistically significant difference was noted about post-operative morbidity and pain related to the wall closure method. CONCLUSIONS: The prophylactic use of a BIO-A biosynthetic stripe (Gore) showed a statistically significant reduction in the incisional hernia rate in patients who underwent clean-contaminated and contaminated surgery.

Red Flags for early referral of people with symptoms suggestive of narcolepsy: a report from a national multidisciplinary panel.

OBJECTIVE: Narcolepsy is a lifelong disease, manifesting with excessive daytime sleepiness and cataplexy, arising between childhood and young adulthood. The diagnosis is typically made after a long delay that burdens the disease severity. The aim of the project, promoted by the "Associazione Italiana Narcolettici e Ipersonni" is to develop Red Flags to detect symptoms for early referral, targeting non-sleep medicine specialists, general practitioners, and pediatricians. MATERIALS AND METHODS: A multidisciplinary panel, including patients, public institutions, and representatives of national scientific societies of specialties possibly involved in the diagnostic process of suspected narcolepsy, was convened. The project was accomplished in three phases. Phase 1: Sleep experts shaped clinical pictures of narcolepsy in pediatric and adult patients. On the basis of these pictures, Red Flags were drafted. Phase 2: Representatives of the scientific societies and patients filled in a form to identify barriers to the diagnosis of narcolepsy. Phase 3: The panel produced suggestions for the implementation of Red Flags. RESULTS: Red Flags were produced representing three clinical pictures of narcolepsy in pediatric patients ((1) usual sleep symptoms, (2) unusual sleep symptoms, (3) endocrinological signs) and two in adult patients ((1) usual sleep symptoms, (2) unusual sleep symptoms). Inadequate knowledge of symptoms at onset by medical doctors turned out to be the main reported barrier to diagnosis. CONCLUSIONS: This report will hopefully enhance knowledge and awareness of narcolepsy among non-specialists in sleep medicine in order to reduce the diagnostic delay that burdens patients in Italy. Similar initiatives could be promoted across Europe.

Spectral electroencephalography profile of rapid eye movement sleep at sleep onset in narcolepsy type 1.

BACKGROUND AND PURPOSE: The sleep-onset rapid eye movement (REM) period (SOREMP), the hallmark of narcolepsy, may be a specific state and not the simple anticipation of REM sleep. METHODS: We analyzed the electroencephalographic spectral content in untreated patients with narcolepsy type 1 (NT1) during the sleep-onset period (SOP) and during nocturnal REM sleep in two consecutive nocturnal recordings from 31 patients with NT1 (mean age 34 ± 15 years, 18 males) and a single nocturnal recording from 36 controls (mean age 38 ± 13 years, 21 males). The SOP was defined as the first 10 min starting at the beginning of the first epoch of any sleep stage, and further divided into two consecutive 5-min periods (SOP-1 and SOP-2); 1 min of artifact-free quiet wakefulness after lights-off was identified as well as 5 min of REM sleep in the middle of the night and another 5 min during the last REM sleep period. Electroencephalographic spectral analysis was performed using the C3/A2 channel. RESULTS: The SOP-1 and, more strikingly, SOP-2 had significantly less delta and sigma activity in patients with NT1 in the SOREMP condition versus both controls and patients with NT1 without SOREMP. SOP-2 also showed less theta and alpha activity. Conversely, sigma and beta activity were more represented during SOREMP compared with the nocturnal REM period in patients with NT1. CONCLUSIONS: The analysis of the SOP supports the concept that SOREMP is a different state compared with both nocturnal REM sleep and non-REM sleep onset.

Age-related differences in sleep-dependent consolidation of motor skills in patients with narcolepsy type 1.

OBJECTIVE: The influence of post-training sleep on the consolidation process of procedural (ie, visual and motor) knowledge has shown to be less effective in patients with chronic sleep disorders compared with healthy subjects. To ascertain whether the influence of the altered architecture of sleep in patients with narcolepsy type 1 (ie, with cataplexy: NT1) also varies with age, we compared the performance values of 16 children (aged from nine to 14 years) and 16 adults (aged from 24 to 51 years) on finger tapping task (FTT) after daytime and nighttime periods of sleep in the 24 hours following training. METHODS: All patients, who were drug-free and underwent continuous polysomnographic recordings, could take one or more naps after the training session (at 10 a.m.) until one hour before the first retrieval session (at 6 p.m.) and had an undisturbed period of nighttime sleep from about 10 p.m. to two hours before the second retrieval session (again at 10 a.m.). RESULTS: The pattern of sleep-dependent consolidation was significantly different in the two groups of patients: while performance accuracy was higher in adults compared with children at each session, performance speed improved after daytime sleep in children and after nighttime sleep in adults. The improvement in performance speed, although not related with any sleep parameters in both groups, was positively correlated with the daytime and nighttime total sleep time (TST) in children with greater consolidation gain. CONCLUSION: The interaction between time of day and age in the time course of consolidation of new motor skills discloses a different role of daytime sleep (active in children, simply protective from interferences in adults) in NT1 patients and suggests a flexible use of napping in the educational context.

Combined brain voxel-based morphometry and diffusion tensor imaging study in idiopathic restless legs syndrome patients.

BACKGROUND AND PURPOSE: The aim of this study was to evaluate the presence of abnormalities in the brain of patients with restless legs syndrome (RLS) using voxel-based morphometry and diffusion tensor imaging (DTI). METHODS: Twenty patients and twenty controls were studied. Voxel-based morphometry analysis was performed using statistical parametric mapping (SPM8) and FSL-VBM software tools. For voxel-wise analysis of DTI, tract-based spatial statistics (TBSS) and SPM8 were used. RESULTS: Applying an appropriate threshold of probability, no significant results were found either in comparison or in correlation analyses. CONCLUSIONS: Our data argue against clear structural or microstructural abnormalities in the brain of patients with idiopathic RLS, suggesting a prevalent role of functional or metabolic impairment.

Development of a disability scale for myotonic dystrophy type 1.

OBJECTIVES: Myotonic dystrophy type 1 (DM1) is a multisystem disorder. Many tests in the literature have evaluated single aspects of DM1 patients, mainly focusing on muscular impairment, without an overall quantification of the different disease-specific neurological features. We developed and validated a new functional scale for DM1 patients based on neuromuscular impairment (NI) and disability. MATERIALS AND METHODS: Thirty-three patients were tested in basal condition, 18 were re-evaluated after therapeutic intervention with mexiletine, and 13 at one year follow-up without treatment. The scale includes 21 ordinal items in four areas: neuropsychology, motricity, myotonia and daily life activities. We evaluated inter- and intra-observer reliability (intraclass correlation coefficient, ICC and Spearman correlations, respectively), internal consistency (Cronbach's alpha), external validity (Spearman correlations between each area and other clinical and objective measurements and scales), and sensitivity to clinical changes after treatment or at follow-up. RESULTS: Our analysis provided good results for inter-observer agreement (ICC = 0.72-0.97), intra-observer reliability, and internal consistency for all areas (Cronbach's α > 0.73). Total score and single area subscores were significantly correlated to objective measurements, disease duration and multisystem involvement. Finally, the scale was sensitive to clinical changes disclosing a significant improvement after treatment in the items assessing myotonia, and also to disease progression showing a significant worsening in all areas but myotonia in untreated patients. DISCUSSION: Our scale provides a new practical measure to evaluate NI and disability of DM1 patients. Further longitudinal studies are warranted to confirm its reliability in tracking disease progression and severity over a longer period of time.