The structural and functional workings of KEOPS.

KEOPS (Kinase, Endopeptidase and Other Proteins of Small size) is a five-subunit protein complex that is highly conserved in eukaryotes and archaea and is essential for the fitness of cells and for animal development. In humans, mutations in KEOPS genes underlie Galloway-Mowat syndrome, which manifests in severe microcephaly and renal dysfunction that lead to childhood death. The Kae1 subunit of KEOPS catalyzes the universal and essential tRNA modification N6-threonylcarbamoyl adenosine (t6A), while the auxiliary subunits Cgi121, the kinase/ATPase Bud32, Pcc1 and Gon7 play a supporting role. Kae1 orthologs are also present in bacteria and mitochondria but function in distinct complexes with proteins that are not related in structure or function to the auxiliary subunits of KEOPS. Over the past 15 years since its discovery, extensive study in the KEOPS field has provided many answers towards understanding the roles that KEOPS plays in cells and in human disease and how KEOPS carries out these functions. In this review, we provide an overview into recent advances in the study of KEOPS and illuminate exciting future directions.

Congenital hiatal hernia segregating with a duplication in 9q22.31q22.32 in two families.

Congenital hiatal hernia (HH) is a rare congenital defect and is often described on a sporadic basis, but familial cases have also been reported. The mechanism of development is not well understood, and to our knowledge no specific genetic factors have been implicated to date. We report on seven individuals from two families with 9q22 duplication, who have variably associated features including congenital HH in four individuals. One family had an 1.09 Mb 9q22 duplication, and the other family had an overlapping 2.73 Mb 9q22 duplication. We review the genes in this region and discuss BARX1 (BarH-like homeobox gene 1) as a gene of interest.

Modern era surgical outcomes of elective and emergency giant paraesophageal hernia repair at a high-volume referral center.

INTRODUCTION: Repair of giant paraesophageal hernia (PEH) has historically been associated with significant morbidity and mortality such that elective repair is only offered to symptomatic patients. Recent reports suggest modern era outcomes have improved such that elective repair may now be safer than historically thought. Furthermore, the morbidity of emergency surgery may still be significant. These changes may have important implications for patient selection for elective repair. The objectives of this study were to determine and compare modern era surgical outcomes after elective and emergency repair of giant PEHs at a high-volume tertiary care center. METHODS: A retrospective review was conducted for all Type II-IV giant PEH repairs performed between 1 January 2012 and 31 December 2017. Type 1 hiatal hernias, fundoplication for reflux, and any co-surgery other than cholecystectomy were excluded from the final analysis. Baseline patient demographics, operative details, postoperative complications within 30 days and in-hospital or 30-day mortality were tabulated from the electronic medical record. Data were reported as median (interquartile range) unless otherwise specified. RESULTS: A total of 352 cases were reviewed, of which 204 met inclusion criteria (18 emergency, 186 elective). Eight had Type II PEH, 146 had Type III, and 50 had Type IV. Median length of stay was shorter in the elective group [1 (1) day elective vs. 5 (7) days emergency, p < 0.0001], and emergency patients were less likely to return directly to their original residence at discharge (13, 72% emergency vs 185, 99.4% elective, p < 0.0001). There were significantly more major complications (Clavien-Dindo score ≥ 3) in the emergency group (5, 28% emergency vs. 10, 5% elective, p = 0.005). There were no perioperative deaths in either group. Morbidity and mortality in both groups were less than historically reported. CONCLUSIONS: Informed consent discussions and patient selection for repair of giant PEHs should reflect modern era and institution-specific outcomes.

Asymmetrical elevation of esophagogastric junction pressure suggests hiatal repair contributes to antireflux surgery dysphagia.

The radial distribution of esophago-gastric junction (EGJ) pressures with regard to troublesome dysphagia (TDysph) after antireflux surgery is poorly understood. Before and after antireflux surgery, end-expiratory and peak-inspiratory EGJ pressures were measured at eight angles of 45° radial separation in patients with reflux disease. All 34 patients underwent posterior crural repair, then either 90° anterior (N = 13) or 360° fundoplication (N = 21). Dysphagia was assessed prospectively using a validated questionnaire (score range 0-45) and TDysph defined as a dysphagia score that was ≥5 above pre-op baseline. Compared with before surgery, for 90° fundoplication, end-expiratory EGJ pressures were highest in the left-anterolateral sectors, the position of the partial fundoplication. In other sectors, pressures were uniformly elevated. Compared with 90° fundoplication, radial pressures after 360° fundoplication were higher circumferentially (P = 0.004), with a posterior peak. Nine patients developed TDysph after surgery with a greater increase in end-expiratory and peak-inspiratory EGJ pressures (P = 0.03 and 0.03, respectively) and significantly higher inspiratory pressure at the point of maximal radial pressure asymmetry (P = 0.048), compared with 25 patients without TDysph. Circumferential elevation of end-expiratory EGJ pressure after 90° and 360° fundoplication suggests hiatal repair elevates EGJ pressure by extrinsic compression. The highly localized focal point of elevated EGJ pressure upon inspiration in patients with TDysph after surgery is indicative of a restrictive diaphragmatic hiatus in the presence of a fundoplication.

Homozygous splicing mutation in NUP133 causes Galloway-Mowat syndrome.

OBJECTIVE: Galloway-Mowat syndrome (GAMOS) is a neural and renal disorder, characterized by microcephaly, brain anomalies, and early onset nephrotic syndrome. Biallelic mutations in WDR73 and the 4 subunit genes of the KEOPS complex are reported to cause GAMOS. Furthermore, an identical homozygous NUP107 (nucleoporin 107kDa) mutation was identified in 4 GAMOS-like families, although biallelic NUP107 mutations were originally identified in steroid-resistant nephrotic syndrome. NUP107 and NUP133 (nucleoporin 133kDa) are interacting subunits of the nuclear pore complex in the nuclear envelope during interphase, and these proteins are also involved in centrosome positioning and spindle assembly during mitosis. METHODS: Linkage analysis and whole exome sequencing were performed in a previously reported GAMOS family with brain atrophy and steroid-resistant nephrotic syndrome. RESULTS: We identified a homozygous NUP133 mutation, c.3335-11T>A, which results in the insertion of 9bp of intronic sequence between exons 25 and 26 in the mutant transcript. NUP133 and NUP107 interaction was impaired by the NUP133 mutation based on an immunoprecipitation assay. Importantly, focal cortical dysplasia type IIa was recognized in the brain of an autopsied patient and focal segmental glomerulosclerosis was confirmed in the kidneys of the 3 examined patients. A nup133-knockdown zebrafish model exhibited microcephaly, fewer neuronal cells, underdeveloped glomeruli, and fusion of the foot processes of the podocytes, which mimicked human GAMOS features. nup133 morphants could be rescued by human wild-type NUP133 mRNA but not by mutant mRNA. INTERPRETATION: These data indicate that the biallelic NUP133 loss-of-function mutation causes GAMOS. Ann Neurol 2018;84:814-828.

The other explanation for dyspnea: giant paraesophageal hiatal hernia repair routinely improves pulmonary function.

Paraesophageal hiatal hernias (PEHs) are most commonly associated with gastrointestinal symptoms; less widely appreciated is their potentially important influence on respiratory function. We hypothesize that surgical repair of PEH will significantly improve not only gastrointestinal symptoms, but also preoperative dyspnea and spirometry scores. A prospective Institutional Review Board-approved database was used to review all patients undergoing PEH repair from 2000 to 2016. Patients with pre- and postoperative pulmonary function tests assessed by spirometry were included. Postoperative changes in spirometry measurements were compared to PEH size as reflected by the percentage of intrathoracic stomach observed on preoperative contrast studies. Patients were stratified according to improvement in forced expiratory volume in 1 second (FEV1). Patients with >12% ('significant') improvement in FEV1 after surgery were compared to the remaining patient population. In total, 299 patients met the inclusion criteria. Symptomatic improvement in respiratory function was noted in all patients after PEH repair. Age, gender, BMI, presenting symptoms, Charlson comorbidity index as well as preoperative comorbidities did not significantly impact the functional outcome. Spirometry results improved in 80% of the patients, 21% of whom showed an improvement of >20% compared to the preoperative level. 'Significant' improvement in respiratory function was seen in 122 of 299 (41%) patients. Patients presenting with moderate and severe preoperative pulmonary obstruction demonstrated 'significant' improvement in FEV1 in 48% and 40% of cases, respectively. Large PEHs, characterized by a percentage of intrathoracic stomach >75%, was strongly associated with 'significant' improvement in FEV1 (P = 0.001). PEHs can impact subjective and objective respiratory status and surgical repair can result in a significant improvement in dyspnea and pulmonary function score that is independent of preoperative pulmonary disease. Gastric herniation of more than 75% was associated with higher possibility for improvement of pulmonary function tests. Patients with persistent and unexplained dyspnea and coexistent PEH should be assessed by an experienced surgeon for consideration of elective repair.

Gender Specific Differences in Prevalence and Risk Factors for Gastro-Esophageal Reflux Disease.

BACKGROUND: Gender-related factors might play an important role in the development of reflux esophagitis (RE) and symptomatic gastro-esophageal reflux disease (GERD). We aimed to evaluate the prevalence and risk factors for RE and symptomatic GERD and determine whether gender specific differences exist. METHODS: This study was conducted on a health cohort consisting of 10,158 participants who underwent comprehensive health screening. Lifestyles and gastrointestinal symptoms were investigated using a self-reported structured questionnaire. Questionnaires about menstrual status were added for the women. RESULTS: The prevalence of RE in men was significantly higher than that in women (10.6% vs. 2.0%, P < 0.001); however, symptomatic GERD showed predominance in women (6.2% vs. 2.5%, P < 0.001). Although the prevalence of RE gradually increased with the duration of menopause stratified by decade (P = 0.007), that of symptomatic GERD rapidly increased across the menopausal transit in women. Apart from common risk factors of obesity and current smoking for RE, over 70 years of age in women and hiatal hernia and hypertriglyceridemia in men were significant risk factors. In symptomatic GERD, high somatization was a common risk factor. Excessive alcohol drinking was a significant risk factor in men, but not in women. CONCLUSION: This study showed a predominance of RE in men, but a predominance of symptomatic GERD in women. In women, dynamic increase in the prevalence of GERD is closely related to the menopause conditions and its duration. There are specific risk factors for RE and symptomatic GERD according to gender differences.

[Anatomical features and biomechanical properties of the diaphragm in pathogenesis of hiatal hernia]. / Anatomicheskie osobennosti i biomekhanicheskie svoistva diafragmy v patogeneze razvitiia gryzh pishchevodnogo otverstiia diafragmy.

OBJECTIVE: To study changes of diaphragm and esophageal-diaphragmatic junction depending on age and constitutional features. MATERIAL AND METHODS: We studied changes of diaphragm and esophageal-diaphragmatic junction depending on age and constitutional features by using of 40 cadaveric specimens (people aged 19-75 years). RESULTS: Esophageal-aortic ligament is observed rarer with age. This ligament is poorly developed in brachiomorphic body type while diaphragmatic-cardiac ligament is generally absent as a rule. This is a predisposing factor for weakening this area. It was revealed that reduced strength and elasticity (especially esophageal-aortic and esophageal-diaphragmatic ligaments) is one of the key factors in the development of hiatal hernia. It is especially relevant for brachiomorphic body type, the 2nd mature and elderly age. The 2nd mature period is associated with reduced diameter and kinking of great arteries, that leads to 1.5-2 times decrease of arterial capacity of the diaphragm. Therefore, hiatal hernia repair using own tissues may be insufficient and accompanied by recurrence in persons with brachiomorphic body type in the 2nd mature period. CONCLUSION: Analysis of biomechanical data and anatomical features of the diaphragm may be useful to predict recurrent hiatal hernia.

Long-term clinical outcomes after intrathoracic stomach surgery: a decade of longitudinal follow-up.

BACKGROUND: A subset of patients with large paraesophageal hernias have more than 75% of the stomach herniated above the diaphragm; such cases are referred to as intrathoracic stomach (ITS). Herein, we report longitudinal symptomatic outcomes over a decade after surgical ITS repair in a large patient cohort. METHODS: Patients who underwent surgical treatment for ITS from 01/2004 to 05/2016 were studied. Preoperative and follow-up data were prospectively collected. Patients completed a standardized symptom questionnaire 1 year postoperatively and at 2-year intervals thereafter. RESULTS: In total, 235 patients were reviewed. The mean age was 70.0 ± 11.6 years; 174 patients (74.0%) were women. Surgical procedures included 7 transthoracic repairs and 228 transabdominal repairs (222 laparoscopic, 2 open, 4 laparoscopic-to-open conversions). Anti-reflux procedures were performed in 173 patients (73.6%). 33 patients (14.0%) had mesh reinforcement of hiatal closure; 11 (4.7%) underwent Collis gastroplasty. Follow-up symptom questionnaires at 1, 3, 5, 7, 9, and 11 years were available for 81, 48, 47, 30, 33, and 38% of patients, respectively. Significant and lasting symptom improvement was reported at all follow-up time points. Mean satisfaction scores of 9.3, 9.1, 9.3, 9.0, 9.5, and 9.8 on a 1-10 scale were recorded at the aforementioned intervals. CONCLUSIONS: Long-term clinical outcomes confirm that laparoscopic ITS repair is safe and durable, and is associated with a high degree of patient satisfaction and symptom resolution.

Autologous augmentation of hiatal hernia repair with filtered platelet concentrate improves tissue remodeling in a swine model.

INTRODUCTION: Autologus augmentation of wound remodeling with platelet concentrate is a burgeoning field with promising results. We hypothesized that the addition of filtered platelet concentrate (fPC) to an acellular biologic graft would improve crural healing and tissue integrity in hiatal hernia repair. METHODS: Sixteen healthy Yorkshire female pigs were divided into three groups: hiatus repair (HR) (n = 7), HR with biologic graft (HRM; n = 8, and HR with biologic graft and fPC (fPC; n = 9). Surgeries were performed by a single surgeon. Animals were euthanized at 8 weeks, and the distal esophagus with hiatus was harvested en-block. Tissue was graded by a histopathologist on collagen deposition, vascularization, and inflammation at the graft-hiatal interface. Tensile strength testing was performed using the Teststar IIs (MTS), coupled with a strain extensometer (Epsilon). Samples of equal dimensions were preloaded to 1 N and deformed at a constant rate of 0.2 mm/s. Statistical analysis was performed via Kruskal-Wallis one-way analysis of variance. RESULTS: Aspirate analysis revealed a mean platelet count of 3 million platelets/1 mL of aspirate. Animals in the fPC group had significantly increased mean chronic inflammation (3.1 ± 1.1 vs. 1.8 ± 1.6, 1.2 ± 1.2, p = 0.04) compared to HR alone and HR + biologic graft. Vascular deposition did not differ between groups (p = 0.8). A trend toward increased collagen deposition was demonstrated for the fPC group (1.4 ± 1.1 vs. 2.0 ± 0.6 in HR group and 3.0 ± 1.2 in HRM group, p = 0.06). There was a statistically significant increase in tensile strength, yield force, and Young's modulus in the fPC group compared with HR and HR + biologic mesh (p < 0.01). CONCLUSION: A trend toward increased collagen deposition and vascularity of the fPC group was demonstrated. In addition, there was an increase in tensile strength and yield force in the fPC group. Use of autologous fPC appears a safe and promising adjunct to wound remodeling and healing in a swine model.

Accuracy of the Air Flow Sphincter Locator system in identifying the lower esophageal sphincter for placement of pH catheters.

The Air Flow Sphincter Locator (AFSL) is marketed as an alternative method to manometry for localizing the lower esophageal sphincter (LES) for pH probe placement. Such a system is desirable due to the additional time, cost, and discomfort associated with dual nasal intubation, but its accuracy has never been assessed. To assess the accuracy of the AFSL in localizing the LES. Fifty consecutive outpatients presenting for pH and manometry studies were included. The upper border of the LES was determined using HRM and the AFSL by two technicians independently. LES locations measured by technicians using AFSL versus manometry, as well as the manometrically determined LES locations by technicians versus MDs were compared. Differences in LES locations determined by HRM as read by MDs versus technicians were small; none were >3 cm, and 92% were within 2 cm. Comparison between LES locations determined by technicians using HRM versus the AFSL revealed that 52% had a difference of 2-3cm and 32% had a difference of >3 cm. Hiatal hernia was associated with a difference in LES location of >3 cm. Excluding patients with hiatal hernia, nonetheless, still produced a >3 cm difference in 24% of studies. Prior reports have suggested that a difference greater than +/-3 cm in pH probe placement is considered unacceptable for clinical studies. Based on our study, the AFSL placed the LES outside of this range in 32% of patients, and may be particularly inaccurate in the setting of a hiatal hernia. This suggests that the device may not be an acceptable alternative to manometry in determining LES location for pH probe placement.

[Rare causes of iatrogenic pericardial tamponade - 2 case reports].

Two rare causes of iatrogenic pericardial effusions are presented. In the first case, a 61-year-old woman who had undergone laparoscopic surgery for a diaphragmatic hernia was resuscitated without success the next day. As cause of death circulatory failure as a result of post-operative pulmonary embolism was reported. Autopsy results showed that the pericardium and the heart had been sewn to the diaphragm. The suture was torn from the tissue, which caused a hemorrhage into the pericardium and the chest cavity, so that death was diagnosed to be due to cardiac tamponade and hemothorax after an iatrogenic heart injury. In the second case, a 62-year-old man who had developed a massive incisional hernia after treatment of an abdominal gunshot wound underwent open herniotomy with mesh repair. Postoperatively, the man complained about increasing pain and shortness of breath. He was transferred to another hospital for further assessment, where a cardiac tamponade was diagnosed. Autopsy results showed that three of the plastic staples used to fix the mesh had perforated the diaphragm and the pericardium thus injuring the adjacent right ventricle with subsequent perforation and development of a hemopericardium.

Esophagogastric Junction Morphology and Distal Esophageal Acid Exposure.

BACKGROUND: The Chicago classification has recently added a morphological subclassification for the esophagogastric junction (EGJ). Our aim was to assess the distal esophageal acid exposure in patients with this new Chicago EGJ-type IIIa and IIIb classification. STUDY DESIGN: From a prospectively collected high-resolution manometry (HRM) database, we identified patients who underwent 24-h pH study between October 2011 and June 2015 and were diagnosed with EGJ-type III based on HRM. Chicago EGJ-type III is defined as the inter-peak nadir pressure ≤gastric pressure and a lower esophageal sphincter (LES)-crural diaphragm (CD) separation >2 cm [IIIa-pressure inversion point (PIP) remains at CD level and IIIb-PIP remains at LES level]. We classified the patients into reflux group [DeMeester score >14.72 or Fraction time pH (<4) > 4.2 %] and non-reflux group based on 24-h pH study. RESULTS: Fifty patients were identified that satisfied the study criteria, of which 37 patients (74 %) were EGJ-type IIIa. In those with EGJ-type IIIb, abdominal LES length (AL) in reflux group was significantly shorter than the non-reflux group (0.8 vs. 1.8, p < 0.05). EGJ-type IIIa patients showed significantly higher value for DeMeester score and Fraction time pH and more often had a positive pH study than EGJ-type IIIb patients (DeMeester score: 26.7 vs. 11.7, p < 0.05; Fraction time pH: 7.9 vs. 2.6, p < 0.05; positive pH study: 81.1 vs. 30.8 %, p < 0.001). Reflux was more common in LES-CD ≥ 3 cm than that in LES-CD < 3 cm (85 vs. 56.7 %, p < 0.05). CONCLUSION: A subset of patients with >2-cm LES-CD separation (type IIIb) maintain a physiological intra-abdominal location of the EGJ and are less likely to have reflux. A LES-CD ≥ 3 cm seems to discern a hiatus hernia of clinical significance.

The use of crural relaxing incisions with biologic mesh reinforcement during laparoscopic repair of complex hiatal hernias.

INTRODUCTION: Laparoscopic hiatal hernia repair has a better chance of success if the hiatus is closed without tension. This study attempts to answer the following questions: (1) What is the rate of hiatal hernia recurrence in patients who undergo hiatal closure with diaphragmatic relaxing incisions? (2) Can biologic mesh be safely substituted for synthetic mesh as coverage of the relaxing incisions? METHODS: We identified all patients who underwent laparoscopic hiatal hernia repair at our institution between 2007 and 2013 and reviewed their clinical records. Radiologic recurrence was identified by an experienced radiologist and defined as the presence of any abdominal contents located above the diaphragm on esophagram. Clinical recurrence was defined as little or no improvement in symptoms, the development of a new symptom, or the need for medical, endoscopic, or surgical treatment of postoperative symptoms. RESULTS: A minimum of 6 months of radiologic and clinical follow-up was available for 146 (40 %) patients, including 16 with relaxing incisions. There were 66 (45 %) recurrent hernias detected on esophagram. There was no difference in the rate of recurrent hiatal hernia among the three groups: Primary closure of the hiatus (21/36 [58 %]), primary closure with biologic mesh reinforcement (36/94 [38 %]), and relaxing incision with biologic mesh reinforcement (9/16 [56 %]; p = 0.428). Two reoperations were performed on patients who underwent left relaxing incisions and developed symptomatic diaphragmatic hernias through the left relaxing incisions. There were no complications associated with use of biologic mesh at the hiatus. CONCLUSIONS: Rate of recurrent hiatal hernia is similar between patients who undergo diaphragmatic relaxing incisions and patients who undergo primary hiatal closure. Relaxing incisions can be safely performed on either crus; however, biologic mesh should not be used to patch a left-sided relaxing incision due to the risk of developing a diaphragmatic hernia.

Clinical Conundrum: Killian-Jamieson Diverticulum with Paraesophageal Hernia.

Killian-Jamieson diverticulum is a outpouching of the lateral cervical esophageal wall adjacent to the insertion of the recurrent laryngeal to the larynx and is much less common in clinical practice than Zenkers Diverticulum. Surgical management of Killian-Jamieson diverticulum requires open transcervical diverticulectomy due to the proximity of the recurrent laryngeal nerve to the base of the pouch. We present a case of a Killian-Jamieson diverticulum associated with a concurrent large type III paraesophageal hernia causing significant solid-food dysphagia, post-prandial regurgitation of solid foods, and chronic cough managed with open transcervical diverticulectomy and laparoscopic paraesophageal hernia repair with Nissen fundoplication.

Hiatal hernia with upside-down stomach. Management of acute incarceration: case presentation and review of literature.

Upside-down stomach (UDS) represents the rarest type of hiatal hernia (<5%) and is characterized by herniation of the entire stomach or most gastric portions into the posterior mediastinum. We present here a very rare complication of such a condition which is incarceration of upside-down stomach. A 54 year-old female was admitted to the emergency department presenting signs of acute epigastric pain radiating into thorax. Computed tomography revealed a giant hiatal hernia with incarceration of the gastric trunk. Immediate operation for reduction of the incarcerated stomach and repair of the hiatal defect was performed. The patient was discharged without any complication and was followed up at the surgical outpatient department. The presented case confirms that differentiation of an acute epigastric or intrathoracic pain in adults should always exclude presence of hiatal hernia which in case of incarceration should be treated by prompt surgical management.

[СOMPARISON OF LAPAROSCOPIC CRURORAPHY AND PLASTY, USING LIGHTWEIGHT NET TRANSPLANT IN LARGE HIATAL HERNIAS: REMOTE RESULTS OF PROSPECTIVE RANDOMIZED INVESTIGATION].

Two procedures of laparoscopic plasty of large hiatal hernias (HH): cruroraphy (group I) and a two­layered plasty, using lightweight partially absorbable net (LPAN) Ultrapro (group II) were compared in prospective randomized investigation. The results of treat* ment were studied in terms from 24 to 27 mo, (24.4 ± 0.72) mo at average. The pure symptomatic anatomical recurrences rate, including those in conjunction with function* al recurrences, were trustworthily less in group II; duration of functional dysphagia (without stricture) did not differ in the groups trustworthily. Тhus, in large HH a two­lay* ered plasty conduction, using LPAN, permits to reduce the anatomical recurrences rate, not enhancing the complications rate, and it may be considered the operation of choice.

[THE KEY CAUSES OF SUCCESS OF VIDEOLAPAROSCOPIC OPERATIONS FOR GASTROESOPHAGEAL REFLUX DISEASE AND HIATAL HERNIA].

Results of operative treatment of 168 patients, suffering hiatal hernia and gastroesophageal reflux disease, in Clinic of Surgery and Endoscopy in 2007 ­ 2016 yrs, were analyzed. The key causes for the operation success, performed for hiatal hernia; gastroesophageal reflux disease were considered: the surgeons' learning curve, choice of method of fundoplication and cruroraphy, preoperative compliance of the patient to antisecretory preparations, rate of postoperative morbidity, psychological state of the patient, atypical symptoms, esophageal function and the reflux type present. Standardization of intervention maintenance the good intervention maintenance the good indexes of postonerative prognosis, including, the conversion rate ­ 0.6%, postoperative morbidity ­ 3%, duration of postoperative stationary treatment ­ 3 days at average

Cameron lesions in patients with hiatal hernias: prevalence, presentation, and treatment outcome.

Cameron lesions, as defined by erosions and ulcerations at the diaphragmatic hiatus, are found in the setting of gastrointestinal (GI) bleeding in patients with a hiatus hernia (HH). The study aim was to determine the epidemiology and clinical manifestations of Cameron lesions. We performed a retrospective cohort study evaluating consecutive patients undergoing upper endoscopy over a 2-year period. Endoscopy reports were systematically reviewed to determine the presence or absence of Cameron lesions and HH. Inpatient and outpatient records were reviewed to determine prevalence, risk factors, and outcome of medical treatment of Cameron lesions. Of 8260 upper endoscopic examinations, 1306 (20.2%) reported an HH. When categorized by size, 65.6% of HH were small (<3 cm), 23.0% moderate (3-4.9 cm), and 11.4% were large (≥5 cm). Of these, 43 patients (mean age 65.2 years, 49% female) had Cameron lesions, with a prevalence of 3.3% in the presence of HH. Prevalence was highest with large HH (12.8%). On univariate analysis, large HH, frequent non-steroidal anti-inflammatory drug (NSAID) use, GI bleeding (both occult and overt), and nadir hemoglobin level were significantly greater with Cameron lesions compared with HH without Cameron lesions (P ≤ 0.03). Large HH size and NSAID use were identified as independent risk factors for Cameron lesions on multivariate logistic regression analysis. Cameron lesions are more prevalent in the setting of large HH and NSAID use, can be associated with GI bleeding, and can respond to medical management.

Long-term outcome and quality of life after supercharged jejunal interposition for oesophageal replacement.

BACKGROUND: The consequences of major conduit necrosis following oesophagectomy are devastating. Jejunal interposition with vascular supercharging is an alternative reconstructive method if colon is unavailable. Aims of this study were to review the long-term outcome and quality of life of patients undergoing this surgery in our tertiary unit. METHODS: Patients undergoing oesophageal reconstruction with supercharged jejunum were identified and retrospective review of hospital notes performed. Each patient was then interviewed for follow up data and quality of life assessment using the EORTC QLQ-C30 questionnaire. RESULTS: Six patients (5 men) (median age 59 years (range 34-72) underwent supercharged pedicled jejunal (SPJ) interposition from May 2005-August 2010. Indications for surgery were loss of both gastric and colonic conduits following surgery for oesophageal cancer (n = 4), loss of gastric conduit and previous colectomy (n = 1) and lastly, gastric and colonic infarction in a strangulated paraoesophageal hernia (n = 1). Median time to reconstruction was 12 months [6-15 range]. There were no in-hospital deaths. Median postoperative stay was 46 days [13-118]. Three patients required surgical re-intervention for leak, sepsis and reflux, respectively. Median follow up was 6.5 years [range 7-102 months]. One patient died seven months following surgery due to respiratory complications. On follow up, 5 patients have an enteral diet without supplemental nutrition, maintaining weight and good quality of life scores. CONCLUSIONS: Supercharged jejunal interposition is a suitable alternative conduit for delayed oesophageal replacement in patients with otherwise limited reconstructive options. Good functional outcomes can be achieved despite formidable technical challenges in this group.