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1.
Orphanet J Rare Dis ; 17(1): 279, 2022 07 19.
Article in English | MEDLINE | ID: mdl-35854315

ABSTRACT

BACKGROUND: Alterations in the MYH7 gene can cause cardiac and skeletal myopathies. MYH7-related skeletal myopathies are extremely rare, and the vast majority of causal variants in the MYH7 gene are predicted to alter the rod domain of the of ß-cardiac myosin molecule, resulting in distal muscle weakness as the predominant manifestation. Here we describe two unrelated patients harboring an in-frame deletion in the MYH7 gene that is predicted to result in deletion of a single amino acid (p.Glu500del) in the head domain of ß-cardiac myosin. Both patients display an unusual skeletal myopathy phenotype with congenital axial stiffness and muscular hypertonus, but no cardiac involvement. RESULTS: Clinical data, MRI results and histopathological data were collected retrospectively in two unrelated boys (9 and 3.5 years old). Exome sequencing uncovered the same 3-bp in-frame deletion in exon 15 (c.1498_1500delGAG) of the MYH7 gene of both patients, a mutation which deletes a highly conserved glutamate residue (p.Glu500del) in the relay loop of the head domain of the ß-cardiac myosin heavy chain. The mutation occurred de novo in one patient, whereas mosaicism was detected in blood of the father of the second patient. Both boys presented with an unusual phenotype of prenatal polyhydramnios, congenital axial stiffness and muscular hypertonus. In one patient the phenotype evolved into an axial/proximal skeletal myopathy without distal involvement or cardiomyopathy, whereas the other patient exhibited predominantly stiffness and respiratory involvement. We review and compare all patients described in the literature who possess a variant predicted to alter the p.Glu500 residue in the ß-cardiac myosin head domain, and we provide in-silico analyses of potential effects on polypeptide function. CONCLUSION: The data presented here expand the phenotypic spectrum of mutations in the MYH7 gene and have implications for future diagnostics and therapeutic approaches.


Subject(s)
Muscular Diseases , Polyhydramnios , Amino Acids/metabolism , Cardiac Myosins/genetics , Cardiac Myosins/metabolism , Female , Humans , Muscle, Skeletal/metabolism , Muscular Diseases/genetics , Mutation , Myosin Heavy Chains/genetics , Myosin Heavy Chains/metabolism , Polyhydramnios/metabolism , Polyhydramnios/pathology , Retrospective Studies
2.
Hernia ; 25(2): 523-533, 2021 04.
Article in English | MEDLINE | ID: mdl-33599899

ABSTRACT

BACKGROUND: The optimal surgical treatment for lateral hernias of the abdominal wall remains unclear. The presented prospective study assesses for the first time in detail the clinical value of a totally endoscopic sublay (TES) technique for the repair of these hernias. METHODS: Twenty-four consecutive patients with a lateral abdominal wall hernia underwent TES repair. This technique is naturally combined with a transversus abdominis release maneuver to create a sufficient retromuscular/preperitoneal space that can accommodate, if necessary, a giant prosthetic mesh. RESULTS: The operations were successful in all but one patient who required open conversion because of dense intestinal adhesion. The mean defect width was 6.7 ± 3.9 cm. The mean defect area was 78.0 ± 102.4 cm2 (range 4-500 cm2). The mean mesh size used was 330.2 ± 165.4 cm2 (range 108-900 cm2). The mean operative time was 170.2 ± 73.8 min (range, 60-360 min). The mean visual analog scale score for pain at rest on the first day was 2.5 (range 1-4). The average postoperative stay was 3.4 days (range 2-7 days). No serious complications (Dindo-Clavien Grade 2-4) were seen within a mean follow-up period of 13.3 months. CONCLUSIONS: A totally endoscopic technique (TES) for the treatment of lateral hernias is described. The technique revealed to be reliable, safe and cost-effective. The first results are promising, but larger studies with longer follow-up periods are recommended to determine the real clinical value.


Subject(s)
Abdominal Wall , Hernia, Ventral , Abdominal Muscles/surgery , Abdominal Wall/surgery , Hernia, Ventral/surgery , Herniorrhaphy/adverse effects , Humans , Postoperative Complications , Prospective Studies , Surgical Mesh
3.
Surg Endosc ; 35(6): 2953-2964, 2021 06.
Article in English | MEDLINE | ID: mdl-32556698

ABSTRACT

INTRODUCTION: Immunosuppressive conditions and/or preoperative corticosteroid treatment have a negative influence on wound healing and can, therefore, lead to higher rates of surgical site infections (SSIs) and seromas. For inguinal hernia, no such studies have been carried out to date. METHODS: In an analysis of data from the Herniamed Registry, 2312 of 142,488 (1.6%) patients with primary unilateral inguinal hernia repair had an anamnestic history of an immunosuppressive condition and/or preoperative corticosteroid treatment. Using propensity score matching, 2297 (99.4%) pairs with comparative patient characteristics were formed. These were then compared using the following primary outcome criteria: intra- and postoperative complications, complication-related reoperations, recurrence at one-year follow-up, pain on exertion, pain at rest, and chronic pain requiring treatment at one-year follow-up. Of the 2297 matched pairs with primary unilateral inguinal hernia repair, 82.76% were male patients. 1010 (44.0%) were operated in laparo-endoscopic techniques (TEP, TAPP), 1225 (53.3%) in open techniques (Bassini, Shouldice, Lichtenstein, Plug, TIP, Gilbert, Desarda), and 62 (2.7%) in other techniques. RESULTS: The matched pair analysis results did not identify any disadvantage in terms of the outcome criteria for patients with an anamnestic history of immunosuppressive condition and/or preoperative corticosteroid treatment (yes vs no). In particular, no disadvantage was noted in the rate of surgical site infections (0.65% vs 0.70%; ns) or seromas (1.22% vs 1.57%; ns). The overall rates of postoperative complications were 3.40% vs 4.31% (p = ns) (plus 0.22% concordant events in five matched pairs). CONCLUSION: In primary unilateral inguinal hernia surgery, an immunosuppressive condition and/or preoperative corticosteroid treatment does not appear to have a negative influence on wound complications.


Subject(s)
Hernia, Inguinal , Adrenal Cortex Hormones/adverse effects , Hernia, Inguinal/surgery , Herniorrhaphy/adverse effects , Humans , Male , Reoperation , Risk Factors
4.
Hernia ; 24(3): 633-637, 2020 06.
Article in English | MEDLINE | ID: mdl-30900041

ABSTRACT

PURPOSE: Short-term effects of round ligament varicosity (RLV) in pregnancy have been investigated in small-scale studies. The long-term effects are unknown. This study aims to evaluate the risk of groin hernia manifestation after RLV in pregnancy, to delineate possible risk factors and to analyze the natural course of pregnancy and post-partum period with regard to RLV. METHODS: In a prospective analysis 28 pregnant women with RLV presented to the hernia clinic over 9 years. After clinical and ultrasound examination during pregnancy and publication of early results in 2013 a second structured follow-up was conducted. Demographic data, hernia-specific risk factors, comorbidities, pregnancy and birth-related data as well as post-partum period were documented without loss of follow-up. In these women, all pregnancies that occurred, including the ones without RLV, were analyzed. RESULTS: Median follow-up was 68 months (11.4-104.9). Only one groin hernia was found. No risk factors could be identified. After uncomplicated childbirth complaints subsided spontaneously in all but one patient within 4 weeks. Recurrence rates in subsequent pregnancies are up to 89%. CONCLUSION: Temporary RLV-induced dilation of the deep inguinal ring in pregnancy is not a common precursor for the development of inguinal hernias later in life. All findings support the theory that the hindrance of venous blood flow caused by the gravid uterus is an important contributing factor for RLV in pregnancy, which is self-limited but has a high risk of recurrence and is not an indication for surgery before or after delivery or for cesarean section.


Subject(s)
Hernia, Inguinal/etiology , Pregnancy Complications, Cardiovascular/etiology , Round Ligament of Uterus/blood supply , Varicose Veins/physiopathology , Adult , Dilatation, Pathologic/diagnosis , Dilatation, Pathologic/etiology , Female , Hernia, Inguinal/diagnosis , Hernia, Inguinal/surgery , Humans , Inguinal Canal/blood supply , Postpartum Period , Pregnancy/physiology , Pregnancy Complications, Cardiovascular/diagnosis , Prospective Studies , Risk Factors , Round Ligament of Uterus/diagnostic imaging , Self-Examination , Ultrasonography , Valsalva Maneuver , Varicose Veins/complications , Varicose Veins/diagnostic imaging , Young Adult
5.
Hernia ; 23(6): 1133-1140, 2019 Dec.
Article in English | MEDLINE | ID: mdl-31367964

ABSTRACT

PURPOSE: Knowledge of postoperative behavior of mesh implants used for hernia repair is generally limited to cases of recurrence, local complications or return to the previous operative field in other pathological conditions. Previous studies with MRI-visible mesh implants in different parts of the abdominal wall have led to variable findings with regard to mesh properties and mostly described a reduction in size over time with subsequently limited mesh overlap over hernia defects which could contribute to recurrence. We aimed to evaluate implant properties in a mechanically stable anatomical region after TAPP repair of primary unilateral inguinal hernias in men with clinical and MRI examinations 4 weeks and 1 year after surgery. METHODS: From 11/2015 to 01/2019, 23 men with primary, unilateral, inguinal hernias underwent TAPP repair with iron particle-loaded, MRI-visible mesh implants in a prospective cohort study. In 16 patients the operative outcome could be evaluated 4 weeks and 12 months after surgery by clinical examination and MRI evaluation with regard to postoperative course, possible adverse outcomes and radiological findings related to implant behavior-namely MRI-identifiability, mesh dislocation or reduction in surface area. RESULTS: All included patients had an uneventful postoperative clinical course. MRI after 4 weeks revealed one postoperative seroma, which resolved spontaneously. No recurrence was detected. Mesh implants could be accurately delineated in DIXON-IN studies and showed neither clinically nor statistically significant changes in size or position. CONCLUSION: 4 weeks and 1 year after a standardized TAPP procedure the mesh implant used in this study showed no tendency towards dislocation or reduction in size in this anatomical position. Its MRI visibility allows accurate delineation during the postoperative course by experienced radiologists in appropriate MRI protocols. Larger patient series are desirable to further support these findings. Shrinkage of implants in the groin as a reason for early recurrence may be overestimated.


Subject(s)
Hernia, Inguinal/surgery , Herniorrhaphy/methods , Surgical Mesh , Adult , Aged , Coated Materials, Biocompatible , Hernia, Inguinal/diagnosis , Hernia, Inguinal/diagnostic imaging , Humans , Iron Compounds , Laparoscopy , Magnetic Resonance Imaging , Male , Middle Aged , Prospective Studies , Young Adult
6.
Surg Endosc ; 33(11): 3511-3549, 2019 11.
Article in English | MEDLINE | ID: mdl-31292742

ABSTRACT

In 2014 the International Endohernia Society (IEHS) published the first international "Guidelines for laparoscopic treatment of ventral and incisional abdominal wall hernias". Guidelines reflect the currently best available evidence in diagnostics and therapy and give recommendations to help surgeons to standardize their techniques and to improve their results. However, science is a dynamic field which is continuously developing. Therefore, guidelines require regular updates to keep pace with the evolving literature. METHODS: For the development of the original guidelines all relevant literature published up to year 2012 was analyzed using the ranking of the Oxford Centre for Evidence-Based-Medicine. For the present update all of the previous authors were asked to evaluate the literature published during the recent years from 2012 to 2017 and revise their statements and recommendations given in the initial guidelines accordingly. In two Consensus Conferences (October 2017 Beijing, March 2018 Cologne) the updates were presented, discussed, and confirmed. To avoid redundancy, only new statements or recommendations are included in this paper. Therefore, for full understanding both of the guidelines, the original and the current, must be read. In addition, the new developments in repair of abdominal wall hernias like surgical techniques within the abdominal wall, release operations (transversus muscle release, component separation), Botox application, and robot-assisted repair methods were included. RESULTS: Due to an increase of the number of patients and further development of surgical techniques, repair of primary and secondary abdominal wall hernias attracts increasing interests of many surgeons. Whereas up to three decades ago hernia-related publications did not exceed 20 per year, currently this number is about 10-fold higher. Recent years are characterized by the advent of new techniques-minimal invasive techniques using robotics and laparoscopy, totally extraperitoneal repairs, novel myofascial release techniques for optimal closure of large defects, and Botox for relaxing the abdominal wall. Furthermore, a concomitant rectus diastasis was recognized as a significant risk factor for recurrence. Despite still insufficient evidence with respect to these new techniques it seemed to us necessary to include them in the update to stimulate surgeons to do research in these fields. CONCLUSION: Guidelines are recommendations based on best available evidence intended to help the surgeon to improve the quality of his daily work. However, science is a continuously evolving process, and as such guidelines should be updated about every 3 years. For a comprehensive reference, however, it is suggested to read both the initially guidelines published in 2014 together with the update. Moreover, the presented update includes also techniques which were not known 3 years before.


Subject(s)
Abdominal Wall/surgery , Hernia, Ventral/surgery , Herniorrhaphy/standards , Laparoscopy/standards , Evidence-Based Medicine , Herniorrhaphy/methods , Humans , Laparoscopy/methods , Societies, Medical
8.
Surg Endosc ; 33(10): 3069-3139, 2019 10.
Article in English | MEDLINE | ID: mdl-31250243

ABSTRACT

In 2014, the International Endohernia Society (IEHS) published the first international "Guidelines for laparoscopic treatment of ventral and incisional abdominal wall hernias." Guidelines reflect the currently best available evidence in diagnostics and therapy and give recommendations to help surgeons to standardize their techniques and to improve their results. However, science is a dynamic field which is continuously developing. Therefore, guidelines require regular updates to keep pace with the evolving literature. METHODS: For the development of the original guidelines, all relevant literature published up to year 2012 was analyzed using the ranking of the Oxford Centre for Evidence-Based Medicine. For the present update, all of the previous authors were asked to evaluate the literature published during the recent years from 2012 to 2017 and revise their statements and recommendations given in the initial guidelines accordingly. In two Consensus Conferences (October 2017 Beijing, March 2018 Cologne), the updates were presented, discussed, and confirmed. To avoid redundancy, only new statements or recommendations are included in this paper. Therefore, for full understanding both of the guidelines, the original and the current, must be read. In addition, the new developments in repair of abdominal wall hernias like surgical techniques within the abdominal wall, release operations (transversus muscle release, component separation), Botox application, and robot-assisted repair methods were included. RESULTS: Due to an increase of the number of patients and further development of surgical techniques, repair of primary and secondary abdominal wall hernias attracts increasing interests of many surgeons. Whereas up to three decades ago hernia-related publications did not exceed 20 per year, currently this number is about 10-fold higher. Recent years are characterized by the advent of new techniques-minimal invasive techniques using robotics and laparoscopy, totally extraperitoneal repairs, novel myofascial release techniques for optimal closure of large defects, and Botox for relaxing the abdominal wall. Furthermore, a concomitant rectus diastasis was recognized as a significant risk factor for recurrence. Despite insufficient evidence with respect to these new techniques, it seemed to us necessary to include them in the update to stimulate surgeons to do research in these fields. CONCLUSION: Guidelines are recommendations based on best available evidence intended to help the surgeon to improve the quality of his daily work. However, science is a continuously evolving process, and as such guidelines should be updated about every 3 years. For a comprehensive reference, however, it is suggested to read both the initial guidelines published in 2014 together with the update. Moreover, the presented update includes also techniques which were not known 3 years before.


Subject(s)
Hernia, Abdominal/surgery , Hernia, Ventral/surgery , Incisional Hernia/surgery , Laparoscopy , Hernia, Abdominal/diagnostic imaging , Hernia, Ventral/diagnostic imaging , Herniorrhaphy/methods , Herniorrhaphy/standards , Humans , Incisional Hernia/diagnostic imaging , Intraoperative Complications , Magnetic Resonance Imaging , Obesity/complications , Patient Positioning , Postoperative Complications , Recurrence , Robotic Surgical Procedures , Surgical Mesh , Tomography, X-Ray Computed
9.
Hernia ; 23(3): 417-428, 2019 06.
Article in English | MEDLINE | ID: mdl-31069580

ABSTRACT

BACKGROUND: Currently, three different techniques are favored for repair of an inguinal hernia: (1) The suture repair described by Shouldice. (2) An open mesh repair according to Lichtenstein. (3) Laparo-endoscopic techniques TAPP and TEP. The aim of the presented paper was to describe the ranking of the Transabdominal Preperitoneal Patch Plasty (TAPP) in comparison to the other techniques for inguinal hernia repair. METHODS: The manuscript is based on the experiences gained in more than 15,000 TAPPs and numerous own studies as well. The technique of TAPP is described in detail and also the results which can be achieved with special reference to primary unilateral inguinal hernias in male patients. Moreover, a systematic review of the literature is done for the comparison with the other techniques. RESULTS: According to own experiences, 98% of all patients with an inguinal hernia admitted for surgery to Marienhospital Stuttgart could be operated on using the TAPP technique. The recurrence rate and the rate of severe chronic pain in this setting were below 1%. Due to the limited quality of most of the published studies an evidence-based comparison which is the best of the currently most recommended techniques is questionable. Therefore, when comparing TAPP with TEP, no definite conclusion about superiority of one technique over the other is possible. Both techniques are safe and effective if properly performed. The guidelines recommend that the surgeon should use the technique he had learned best and is familiar with. The comparison between TAPP and the Shouldice repair shows less pain and a higher effectivity after TAPP. The recurrence rate after Lichtenstein repair and after TAPP is similar, but pain and recovery time are significantly less after TAPP. CONCLUSION: Analyzing the own abundant experiences and the reports in the literature, the TAPP technique has the potential to become the standard operative technique for repair of inguinal hernias in future. However, due to the low level of evidence of most of the studies definite conclusions are difficult to draw at this point of time.


Subject(s)
Hernia, Inguinal/surgery , Herniorrhaphy/methods , Humans , Laparoscopy , Surgical Mesh , Treatment Outcome
10.
JSLS ; 23(1)2019.
Article in English | MEDLINE | ID: mdl-30700965

ABSTRACT

BACKGROUND AND OBJECTIVES: The goal of the study was to evaluate retroperitoneal sarcomas with continuous growth into the scrotum through the inguinal canal with regard to diagnostic approach, surgical treatment, and outcome. The analysis is based on a comprehensively documented case and a complete systematic review of published literature. Potential pitfalls are highlighted. METHODS: We describe the case of a 57-year-old male Caucasian who presented with a swelling in the right groin. Suspecting a scrotal hernia, transabdominal preperitoneal plasty surgery was planned but intraoperatively a large retroperitoneal mass was revealed. After computed tomography scan and magnetic resonance imaging, a complete resection of the tumor was performed. Ten previously published cases describing the same pathology were retrieved from the PubMed database and analyzed systematically in a complete literature review. RESULTS: Histology showed a well-differentiated liposarcoma with tumor-free resection margins. Twenty-two months postoperatively, the patient is in complete clinical remission. CONCLUSION: Preoperative clinical suspicion of retroperitoneal involvement is paramount for developing of a surgical strategy and in unclear cases demands extended preoperative diagnostic workup. Following the appropriate patient management is crucial to prognosis.


Subject(s)
Hernia, Inguinal/complications , Hernia, Inguinal/surgery , Herniorrhaphy , Liposarcoma/pathology , Retroperitoneal Neoplasms/pathology , Hernia, Inguinal/diagnostic imaging , Humans , Inguinal Canal , Liposarcoma/diagnostic imaging , Liposarcoma/surgery , Magnetic Resonance Imaging , Male , Middle Aged , Retroperitoneal Neoplasms/diagnostic imaging , Retroperitoneal Neoplasms/surgery , Scrotum , Tomography, X-Ray Computed
11.
Surg Endosc ; 33(10): 3361-3369, 2019 10.
Article in English | MEDLINE | ID: mdl-30604264

ABSTRACT

BACKGROUND: For comparison of laparoscopic IPOM versus sublay technique for elective incisional hernia repair, the number of cases included in randomized controlled trials and meta-analyses is limited. Therefore, an urgent need for more comparative data persists. METHODS: In total, 9907 patients with an elective incisional hernia repair and 1-year follow-up were selected from the Herniamed Hernia Registry between September 1, 2009 and June 1, 2016. Using propensity score matching, 3965 (96.5%) matched pairs from 4110 laparoscopic IPOM and 5797 sublay operations were formed for comparison of the techniques. RESULTS: Comparison of laparoscopic IPOM versus open sublay revealed disadvantages for the sublay operation regarding postoperative surgical complications (3.4% vs. 10.5%; p < 0.001), complication-related reoperations (1.5% vs. 4.7%; p < 0.001), and postoperative general complications (2.5% vs. 3.7%; p = 0.004). The majority of surgical postoperative complications were surgical site infection, seroma, and bleeding. Laparoscopic IPOM had disadvantages in terms of intraoperative complications (2.3% vs. 1.3%; p < 0.001), mainly bleeding, bowel, and other organ injuries. No significant differences in the recurrence and pain rates at 1-year follow-up were observed. CONCLUSION: Laparoscopic IPOM was found to have advantages over the open sublay technique regarding the rates of both surgical and general postoperative complications as well as complication-related reoperations, but disadvantages regarding the rate of intraoperative complications.


Subject(s)
Herniorrhaphy/methods , Incisional Hernia/surgery , Laparoscopy , Postoperative Complications/epidemiology , Elective Surgical Procedures , Female , Follow-Up Studies , Humans , Intraoperative Complications/epidemiology , Male , Matched-Pair Analysis , Middle Aged , Pain/epidemiology , Propensity Score , Registries , Reoperation/statistics & numerical data , Seroma/epidemiology , Surgical Mesh
12.
Chirurg ; 89(8): 631-638, 2018 Aug.
Article in German | MEDLINE | ID: mdl-29931383

ABSTRACT

SURGICAL TECHNIQUES: The HerniaSurge guidelines have the highest evidence with respect to a strong recommendation for mesh-based surgical techniques. This evidence is equally valid for the Lichtenstein procedure as for the minimally invasive procedures TEP/TAPP. In the case of discrete symptomatic or asymptomatic inguinal hernias, watchful waiting can be an option, taking into account health status and social circumstances. Femoral hernias, on the other hand, should be treated promptly with mesh insertion. Also favored are laparoendoscopic techniques. The Shouldice repair achieves the least recurrences from the suturing procedures and may be an acceptable alternative when indicated or when the patient does not desire mesh reinforcement. In this case, a detailed patient education is necessary. MESH CHOICE: The complication potential of plastic meshes should be explained. The weight is no longer considered a suitable parameter for the classification of meshes and is no longer recommended for mesh selection. Large pore (>1-1.5 mm) monofilament implants have the best integration potential and should have a tear strength of approximately 16 Nm2. Traumatic mesh fixation is only recommended for large medial hernias (M3-EHS). Primarily not recommended are Plug & Patch, double-layered plastic implants (such as the PHS system) or other three-dimensional devices, as this could affect both the anterior and posterior planar layers and complicate the complementary surgical technique in the event of recurrence. In addition, the higher costs have to be considered. PERIOPERATIVE AND POSTOPERATIVE ASPECTS: Perioperative antibiotic prophylaxis in open repair procedures is recommended only in patients with an increased risk of infections. In laparoendoscopic procedures, antibiotic prophylaxis should not be performed or used with the utmost restraint. Careful preparation reduces chronic inguinal and testicular pain. In the case of interference of mesh and nerve, the nerve can be resected. A return to daily activity is recommended within 3-5 days. QUALITY ASSURANCE: The documentation of patient data should be done by establishing hernia registers for quality assurance and for the development of further treatment options. The implementation of the guidelines is supported by HerniaSurge.


Subject(s)
Hernia, Femoral , Hernia, Inguinal , Laparoscopy , Adult , Groin , Hernia, Inguinal/surgery , Herniorrhaphy , Humans , Practice Guidelines as Topic , Recurrence , Surgical Mesh
14.
Surg Endosc ; 32(7): 3295-3302, 2018 07.
Article in English | MEDLINE | ID: mdl-29340811

ABSTRACT

BACKGROUND: The mesh fixation technique used in repair of hiatal hernias and subxiphoid ventral and incisional hernias must meet strenuous requirements. In the literature, there are reports of life-threatening complications with cardiac tamponade and a high mortality rate on using tacks. The continuing practice of tack deployment for mesh fixation to the diaphragm and esophageal hiatus should be critically reviewed. METHODS: In a systematic search of the available literature in May 2017, 23 cases of severe penetrating cardiac complications were identified. The authors became aware of two other cases in which they acted as medical experts. Furthermore, the instructions for use issued by the manufacturers of the tacks were reviewed with regard to their deployment in the diaphragm. RESULTS: Twenty-three of 25 cases (92%) with severe cardiac injuries and subsequent cardiac tamponade were triggered by the use of tacks in the diaphragm. In six cases (24%), these related to ventral and incisional hernias with extension to the subxiphoid area, and in 19 cases (76%) to mesh-augmented hiatoplasty. Twelve of 25 (48%) patients died as a result of pericardial and/or heart muscle injury with cardiac tamponade despite heart surgery intervention. In the tack manufacturers' instructions for use, their deployment in the diaphragm, in particular in the vicinity of the heart, is contraindicated. Likewise, the existing guidelines urgently advise against the use of tacks in the diaphragm, recommending instead alternative fixation techniques. CONCLUSIONS: Tacks should not be used for mesh fixation in the diaphragm above the costal arch.


Subject(s)
Cardiac Tamponade/etiology , Diaphragm/surgery , Hernia, Hiatal/surgery , Herniorrhaphy/adverse effects , Herniorrhaphy/methods , Pericardium/injuries , Surgical Mesh , Adult , Aged , Aged, 80 and over , Female , Humans , Laparoscopy/methods , Male , Middle Aged
15.
J Neurol ; 265(1): 159-164, 2018 Jan.
Article in English | MEDLINE | ID: mdl-29181627

ABSTRACT

In this study, we performed a survey of infantile and late-onset Pompe disease (IOPD and LOPD) in Austria. Paediatric and neuromuscular centres were contacted to provide a set of anonymized clinical and genetic data of patients with IOPD and LOPD. The number of patients receiving enzyme replacement therapy (ERT) was obtained from the pharmaceutical company providing alglucosidase alfa. We found 25 patients in 24 families, 4 IOPD and 21 LOPD with a resulting prevalence of 1:350,914. The most frequent clinical manifestation in LOPD was a lower limb-girdle phenotype combined with axial weakness. Three patients were clinically pauci- or asymptomatic and were diagnosed because of persistent hyperCKemia. Diagnostic delay in LOPD was 7.4 ± 9.7 years. The most common mutation was c.-32-13T > G. All IOPD and 17 symptomatic LOPD patients are receiving ERT. Standardized follow-up was only available in six LOPD patients for the 6-min walk test (6minWT) and in ten for the forced vital capacity (FVC). Mean FVC did not decline (before ERT; 63.6 ± 39.7%; last evaluation during ERT: 61.9 ± 26.9%; P = 0.5) while there was a trend to decline in the mean distance covered by the 6minWT (before ERT: 373.5 ± 117.9 m; last evaluation during ERT: 308.5 ± 120.8 m; P = 0.077). The study shows a lower prevalence of Pompe disease in Austria than in other European countries and corroborates a limb-girdle phenotype with axial weakness as the most common clinical presentation, although asymptomatic hyperCKemia may be the first indication of LOPD.


Subject(s)
Enzyme Replacement Therapy/methods , Glycogen Storage Disease Type II , alpha-Glucosidases/genetics , Adolescent , Adult , Age of Onset , Aged , Austria/epidemiology , Child , Delayed Diagnosis , Female , Follow-Up Studies , Glycogen Storage Disease Type II/epidemiology , Glycogen Storage Disease Type II/genetics , Glycogen Storage Disease Type II/physiopathology , Glycogen Storage Disease Type II/therapy , Humans , Male , Middle Aged , Mutation/genetics , Retrospective Studies , Vital Capacity/physiology
16.
Surg Endosc ; 32(4): 1971-1983, 2018 04.
Article in English | MEDLINE | ID: mdl-29075969

ABSTRACT

BACKGROUND: In inguinal hernia repair, chronic pain must be expected in 10-12% of cases. Around one-quarter of patients (2-4%) experience severe pain requiring treatment. The risk factors for chronic pain reported in the literature include young age, female gender, perioperative pain, postoperative pain, recurrent hernia, open hernia repair, perioperative complications, and penetrating mesh fixation. This present analysis of data from the Herniamed Hernia Registry now investigates the influencing factors for chronic pain in male patients after primary, unilateral inguinal hernia repair in TAPP technique. METHODS: In total, 20,004 patients from the Herniamed Hernia Registry were included in uni- and multivariable analyses. For all patients, 1-year follow-up data were available. RESULTS: Multivariable analysis revealed that onset of pain at rest, on exertion, and requiring treatment was highly significantly influenced, in each case, by younger age (p < 0.001), preoperative pain (p < 0.001), smaller hernia defect (p < 0.001), and higher BMI (p < 0.001). Other influencing factors were postoperative complications (pain at rest p = 0.004 and pain on exertion p = 0.023) and penetrating compared with glue mesh fixation techniques (pain on exertion p = 0.037). CONCLUSIONS: The indication for inguinal hernia surgery should be very carefully considered in a young patient with a small hernia and preoperative pain.


Subject(s)
Chronic Pain/etiology , Hernia, Inguinal/surgery , Herniorrhaphy/methods , Pain, Postoperative/etiology , Adult , Follow-Up Studies , Humans , Male , Middle Aged , Multivariate Analysis , Registries , Risk Factors , Treatment Outcome
17.
Surg Endosc ; 32(5): 2222-2231, 2018 05.
Article in English | MEDLINE | ID: mdl-29075973

ABSTRACT

BACKGROUND: The reported range of seroma formation in the literature after TEP repair is between 0.5 and 12.2% and for TAPP between 3.0 and 8.0%. Significant clinical factors associated with seroma formation include old age, a large hernia defect, an extension of the hernia sac into the scrotum, as well as the presence of a residual indirect sac. Seroma formation is a frequent complication of laparoendoscopic mesh repair of moderate to large-size direct (medial) inguinal hernia defects. This present analysis of data from the Herniamed Hernia Registry now explores the influencing factors for seroma formation in male patients after TAPP repair of primary unilateral inguinal hernia. METHODS: In total, 20,004 male patients with TAPP repair of primary unilateral inguinal hernia were included in uni- and multivariable analysis. RESULTS: Univariable analysis revealed the highly significant impact of the fixation technique on the seroma rate (non-fixation 0.7% vs. tacks 2.1% vs. glue 3.9%; p < 0.001). Multivariable analysis showed that glue compared to tacks (OR 2.077 [1.650; 2.613]; p < 0.001) and non-fixation (OR 5.448 [4.056; 7.317]; p < 0.001) led to an increased seroma rate. A large hernia defect (III vs. I: OR 2.868 [1.815; 4.531]; p < 0.001; II vs. I: OR 2.157 [1.410; 3.300]; p < 0.001) presented a significantly higher risk of seroma formation. Likewise, medial compared to lateral inguinal hernias had a higher seroma rate (OR 1.272 [1.020; 1.585]; p = 0.032). CONCLUSIONS: Mesh fixation with tacks or glue, a larger hernia defect, and medial defect localization present a higher risk for seroma development in TAPP inguinal hernia repair.


Subject(s)
Hernia, Inguinal/surgery , Herniorrhaphy/adverse effects , Seroma/etiology , Seroma/prevention & control , Adult , Herniorrhaphy/methods , Humans , Incidence , Male , Middle Aged , Prospective Studies , Risk Factors , Surgical Mesh/adverse effects , Treatment Outcome
19.
Chirurg ; 88(4): 281-287, 2017 Apr.
Article in German | MEDLINE | ID: mdl-28303285

ABSTRACT

Transabdominal preperitoneal patch plasty (TAPP) is the most frequently used surgical technique for repair of an inguinal hernia in German hospitals (Federal Statistical Office, Wiesbaden). Since its initial description in 1992 by Arregui the technique has been further developed and completely standardized, so that TAPP is now suitable as an operation performed by trainee surgeons despite the complex technique. The individual operation stages have been described by many experts mainly based on their own experiences, training with other experts, participation in meetings with live or video demonstrations and reports in the literature. A scientific explanation for the individual operation steps, i.e. why a certain technique is given preference in comparison to other techniques, is not usually communicated. In this article for the first time not only the operation steps of TAPP are described in detail but also the scientific reasons (evidence) are given.


Subject(s)
Evidence-Based Medicine , Hernia, Inguinal/surgery , Herniorrhaphy/methods , Laparoscopy/methods , Dissection/methods , Hernia, Inguinal/diagnosis , Humans , Peritoneum/surgery , Pneumoperitoneum, Artificial/methods , Surgical Mesh
20.
Surg Endosc ; 31(10): 3872-3882, 2017 10.
Article in English | MEDLINE | ID: mdl-28160069

ABSTRACT

INTRODUCTION: The guidelines of the international hernia societies recommend laparo-endoscopic inguinal hernia repair for recurrent hernias after open primary repair. To date, no randomized trials have been conducted to compare the TEP vs TAPP outcome for recurrent inguinal hernia repair. A Swiss registry study identified only minor differences between the two techniques, thus suggesting the equivalence of the two procedures. MATERIALS AND METHODS: Between September 1, 2009 and August 31, 2013 data were entered into the Herniamed Registry on a total of 2246 patients with recurrent inguinal hernia repair following previous open primary operation in either TAPP (n = 1,464) or TEP technique (n = 782). RESULTS: Univariable and multivariable analysis did not find any significant difference between TEP and TAPP with regard to the intraoperative complications, complication-related reoperations, re-recurrences, pain at rest, pain on exertion, or chronic pain requiring treatment. The only difference identified was a significantly higher postoperative seroma rate after TAPP, which was influenced by the surgical technique, previous open primary operation and EHS-classification medial and responded to conservative treatment. CONCLUSION: TEP and TAPP are equivalent surgical techniques for recurrent inguinal hernia repair following previous open primary operation. The choice of technique should be tailored to the surgeon's expertise.


Subject(s)
Endoscopy/methods , Hernia, Inguinal/surgery , Surgical Mesh/adverse effects , Adult , Endoscopy/adverse effects , Humans , Intraoperative Complications/epidemiology , Intraoperative Complications/etiology , Male , Middle Aged , Pain/surgery , Pain Management , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Prospective Studies , Recurrence , Registries , Reoperation , Seroma/epidemiology , Seroma/etiology , Switzerland , Treatment Outcome
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