A systematic review of Sandifer syndrome in children with severe gastroesophageal reflux.

PURPOSE: Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to diagnosis. We aimed to systematically review available papers regarding SS. METHODS: After presenting our two cases of SS, we systematically reviewed articles published in MEDILINE/PubMed, Cochrane Library, and Web of Science. RESULTS: The meta-analysis included 54 reported cases and 2 of our own cases. Our results showed that all cases achieved symptom improvement with appropriate treatment for GER. Notably, 19 of the 56 cases exhibited anatomical anomalies, such as hiatal hernia and malrotation. Significantly more patients with than without anatomical anomalies required surgery (p < 0.001). However, 23 of the 29 patients without anatomical anomalies (79%) achieved symptom improvement without surgery. Patients who did not undergo surgery had a median (interquartile range) duration to symptom resolution of 1 (1-1) month. CONCLUSION: The primary care providers should keep SS in the differential diagnosis of patients presenting with abnormal posturing and no apparent neuromuscular disorders. Fundoplication may be effective especially for patients with anatomical anomalies or those whose symptoms do not improve after more than 1 month with nonsurgical treatment.

[Surgical strategy for hiatal hernias]. / Operative Strategie bei Hiatushernien.

The indications for surgical treatment of hiatus hernias differentiate between type I and types II, III and IV hernias. The indications for a type I hernia should include a proven reflux disease but the indications for surgical treatment of types II, III and IV hernias are mandatory due to the symptoms with problems in the passage of food and due to the sometimes very severe possible complications. The primary aims of surgery are the repositioning of the herniated contents and a hiatoplasty, which includes a surgical narrowing of the esophageal hiatus by suture implantation. In addition, depending on the clinical situation other procedures, such as hernia sac removal, mesh implantation, gastropexy and fundoplication can be considered. There are various approaches to the repair, all of which have individual advantages and disadvantages. An adaptation to the specific needs situation of the patient and the expertise of the surgeon is therefore essential.

Relief of Obstructive Shock with a Large Hiatal Hernia Occupying the Posterior Mediastinum by Urgent Endoscopy.

A 78-year-old man was admitted to our hospital with obstructive shock caused by a large hiatal hernia that occupied the posterior mediastinum. Tension gastro-duodenothorax was detected in his stomach and duodenum, and we performed urgent endoscopy to relieve shock. Large hiatal hernia occasionally leads to cardiac failure. This is the first reported use of urgent endoscopy to treat a large hiatal hernia.

Diagnostic Dilemma of Syncope: Esophageal Hiatal Hernia and High-risk Bundle-branch Block.

We herein report a complicated case of recurrent syncope accompanying bundle branch block and hiatal hernia of the esophagus. An 83-year-old woman presented with syncope. Echocardiography visualized the left atrium compressed by an esophageal hiatal hernia, which had potential to decrease the cardiac output. Although she underwent esophageal repair surgery, two months after the surgery, she presented to the emergency department again with complaints of syncope. At the return visit, her face was pale and her pulse rate was 30 beats per minute. Electrocardiography showed complete atrioventricular block. On reviewing the patient's previous electrocardiography findings, we found a record of trifascicular block. This case illustrates the importance of predicting atrioventricular blocks in patients with high-risk bundle-branch blocks. Keeping in mind high-risk bundle-branch blocks will help clinicians avoid anchoring bias due to a striking image masquerading as the true diagnosis.

Hiatal hernia reporting: time to remove subjectivity?

INTRODUCTION: The size of a hiatal hernia (HH) is a key determinant of the approach for surgical repair. However, endoscopists will often utilize subjective terms, such as "small," "medium," and "large," without any standardized objective correlations. The aim of this study was to identify HHs described using objective axial length measurements versus subjective size allocations and compare them to their corresponding manometry and barium swallow studies. METHODS AND PROCEDURES: Retrospective chart reviews were conducted on 93 patients diagnosed endoscopically with HHs between 2017 and 2021 at Newton-Wellesley Hospital. Information was collected regarding their HH subjective size assessment, axial length measurement (cm), manometry results, and barium swallow readings. Linear regression models were used to analyze the correlation between the objective endoscopic axial length measurements and manometry measurements. Ordered logistic regression models were used to correlate the ordinal endoscopic and barium swallow subjective size allocations with the continuous axial length measurements and manometry measurements. RESULTS: Of the 93 endoscopy reports, 42 included a subjective size estimate, 38 had axial length measurement, and 12 gave both. Of the 34 barium swallow reads, only one gave an objective HH size measurement. Axial length measurements were significantly correlated with the manometry measurements (R2 = 0.0957, p = 0.049). The endoscopic subjective size estimates were also closely related to the manometry measurements (R2 = 0.0543, p = 0.0164). Conversely, the subjective size estimates from barium swallow reads were not significantly correlated with the endoscopic axial length measurements (R2 = 0.0143, p = 0.366), endoscopic subjective size estimates (R2 = 0.0481, p = 0.0986), or the manometry measurements (R2 = 0.0418, p = 0.0738). Mesh placement was significantly correlated to pre-operative endoscopic axial length measurement (p = 0.0001), endoscopic subjective size estimate (p = 0.0301), and barium swallow read (p = 0.0211). However, mesh placement was not significantly correlated with pre-operative manometry measurements (0.2227). CONCLUSIONS: Endoscopic subjective size allocations and objective axial length measurements are associated with pre-operative objective measurements and intra-operative decisions, suggesting both can be used to guide clinical decision making. However, including axial length measurements in endoscopy reports can improve outcomes reporting.

Acute Mesentero-Axial Gastric Volvulus in the Setting of a Paraesophageal Hernia: A Rare Case Report.

Acute gastric volvulus is a surgical emergency that requires urgent intervention to prevent gastric ischemia and necrosis. Gastric volvulus manifests as an abnormal rotation or torsion of the stomach and may be associated with gastric outlet obstruction. This pathology can be classified as either mesentero-axial or organo-axial volvulus, depending on the axis of rotation. Similarly, it can be categorized as primary or secondary, depending on the etiology. We describe a case of a 63-year-old female with a history of peptic ulcer disease who presented with severe epigastric pain and vomiting of one-day duration. She was diagnosed with an acute mesentero-axial gastric volvulus, which was successfully reduced using a nasogastric tube.

Endoscopic measurement of hiatal hernias: is it reliable and does it have a clinical impact? Results from a large prospective database.

OBJECTIVES: The aim of our study was to compare the results of endoscopy and manometry with regard to the presence and longitudinal size of hiatal hernias using a cohort of patients with PPI-refractory reflux symptoms. In addition, we aimed to investigate the clinical relevance of hiatal hernias and their size in relation to the occurrence of GERD. METHODS: Five hundred patients with suspected GERD due to typical reflux symptoms (heartburn and regurgitation) and inadequate response to PPI therapy underwent standardized screening at a reflux center. After 2 weeks of PPI withdrawal, all patients underwent endoscopy, 24-h pH impedance measurement, and high-resolution manometry (HRM). Both endoscopy and HRM results were available for 487 patients. RESULTS: There was a high correlation between the endoscopic and manometric measured longitudinal size of hernias (rho = .768 (p < .001)). Absolute differences differ on a small effect basis (Cohen's d = 0.23). The presence of hernias increases significantly with the severity of GERD, regardless of whether the hernia was diagnosed by endoscopy or manometry. CONCLUSION: In summary, endoscopically and manometrically measured hiatal hernia size are highly significantly correlated. Patients with refractory reflux symptoms and a hernia size of 4 cm are very likely to have GERD. In the future, this finding could greatly simplify the diagnosis of GERD.

Being Caught in a Vicious Circle: An Interview Study of Individuals Suffering From Grade II-IV Hiatal Hernia.

Large paraesophageal hernias are related to life-threatening complications that warrant immediate surgery. Whether the long-standing chronic symptoms related to the disease in individuals without hernia incarceration motivate surgical treatment is still a subject for discussion. The aim of this study was to explore how individuals suffering from Grade II-IV hiatal hernia describe their symptoms and health, as well as how the disease affects their life. Semistructured interviews were performed with 22 individuals planning to undergo surgery for a large paraesophageal hernia. The data were analyzed using qualitative content analysis and resulted in one main theme "Being caught in a vicious circle" and six subthemes "Distressing and uncertain times," "The symptoms have seized control over my health," "Loss of energy and strength," "Strategies for managing daily life," "Loss of social life," and "Moments of hope despite failing health." Central to the participants' descriptions is their commitment to strategies for managing the ever-present and unpredictable symptoms that have seized control over their health. They were trapped in a hopeless and isolated existence, that is, a vicious circle, from which they were unable to escape. Despite the low incidence of volvulus and incarceration, the symptom burden and effect on general health motivate treatment in these individuals.

Research protocol for the Paraesophageal hernia symptom tool, a prospective multi-center cohort study to identify the need and threshold for surgery and assess the symptom response to surgery.

Large hiatus hernias with a significant paraesophageal component (types II-IV) have a range of insidious symptoms. Management of symptomatic hernias includes conservative treatment or surgery. Currently, there is no paraesophageal hernia disease-specific symptom questionnaire. As a result, many clinicians rely on the health-related quality of life questionnaires designed for gastro-esophageal reflux disease (GORD) to assess patients with hiatal hernias pre- and postoperatively. In view of this, a paraesophageal hernia symptom tool (POST) was designed. This POST questionnaire now requires validation and assessment of clinical utility. Twenty-one international sites will recruit patients with paraesophageal hernias to complete a series of questionnaires over a five-year period. There will be two cohorts of patients-patients with paraesophageal hernias undergoing surgery and patients managed conservatively. Patients are required to complete a validated GORD-HRQL, POST questionnaire, and satisfaction questionnaire preoperatively. Surgical cohorts will also complete questionnaires postoperatively at 4-6 weeks, 6 months, 12 months, and then annually for a total of 5 years. Conservatively managed patients will repeat questionnaires at 1 year. The first set of results will be released after 1 year with complete data published after a 5-year follow-up. The main results of the study will be patient's acceptance of the POST tool, clinical utility of the tool, assessment of the threshold for surgery, and patient symptom response to surgery. The study will validate the POST questionnaire and identify the relevance of the questionnaire in routine management of paraesophageal hernias.

[Surgical treatment of hiatal hernia complicated by short esophagus: a prospective study]. / Khirurgicheskoe lechenie gryzh pishchevodnogo otverstiya diafragmy, oslozhnennykh korotkim pishchevodom.

OBJECTIVE: To analyze immediate and long-term postoperative results in patients with hiatal hernia complicated by short esophagus. MATERIAL AND METHODS: We prospectively analyzed postoperative outcomes in 113 patients with hiatal hernia who underwent surgery between 2013 and 2021. The main group consisted of 54 patients with length of intra-abdominal segment of esophagus <4 cm who underwent Collis procedure or esophagus >4 cm and indications for Nissen fundoplication cuff. The control group consisted of 59 patients and indications for esophageal lengthening procedure only if length of intra-abdominal segment of esophagus was less than 2 cm. This surgery was started with anterolateral vagotomy, and Collis procedure was performed in case of ineffective vagotomy. Nissen fundoplication was performed for abdominal segment of esophagus >2 cm. RESULTS: In the main group, 17 (31.5%) patients with intra-abdominal segment of esophagus <4 cm required Collis procedure. In the control group, length of intra-abdominal segment of esophagus <2 cm was observed in 6 (10.2%) patients. In all cases, anterolateral vagotomy was performed. Surgery time was 189 (80-290) and 136 (90-320) min, respectively (p=0.001). Postoperative complications in the main group occurred in 8 (14.8%) patients, in the control group - 4 (6.8%) patients (p=0.281). One (1.7%) patient died in the control group. The follow-up period was 38 (12-66) months. In long-term period, recurrence developed in 2 (3.7%) and 11 (20%) patients, respectively (p=0.026). High satisfaction with postoperative outcomes was observed in 51 (94.4%) and 46 (79.3%) patients, respectively (p=0.038). CONCLUSION: Uncorrected shortening of the esophagus can be one of the main risk factors of recurrence in long-term period. Expanding the indications for Collis gastroplasty can reduce the incidence of poor outcomes without affecting the incidence of postoperative complications.

Comparison of upper gastrointestinal series and symptom questionnaires with intraoperative diagnosis of hiatal hernia during sleeve gastrectomy.

BACKGROUND: Hiatal hernias are common in bariatric surgery patients, but the utility of preoperative hiatal hernia diagnosis prior to sleeve gastrectomy (SG) is debated. OBJECTIVE: This study compared preoperative and intraoperative hiatal hernia detection rates in patients undergoing laparoscopic SG. SETTING: University hospital, United States. METHODS: As part of a randomized trial evaluating the role of routine crural inspection during SG, an initial cohort was prospectively studied to assess the correlation between preoperative upper gastrointestinal (UGI) series, reflux and dysphagia symptoms, and intraoperative hiatal hernia diagnosis. Preoperatively, patients completed the Gastroesophageal Reflux Disease Questionnaire (GerdQ), the Brief Esophageal Dysphagia Questionnaire (BEDQ), and a UGI series. Intraoperatively, patients with an anteriorly visible defect underwent hiatal hernia repair followed by SG. All others were randomized to standalone SG or posterior crural inspection with repair of any hiatal hernia identified prior to SG. RESULTS: Between November 2019 and June 2020, 100 patients (72 female patients) were enrolled. Preoperative UGI series identified hiatal hernia in 28% (26 of 93) of patients. Intraoperatively, hiatal hernia was diagnosed during initial inspection in 35 patients. Diagnosis was associated with older age, lower body mass index, and Black race but did not correlate with GerdQ or BEDQ. Using the standard conservative approach, compared with intraoperative diagnosis, sensitivity and specificity of the UGI series were 35.3% and 80.7%, respectively. Hiatal hernia was identified in an additional 34% (10 of 29) of patients randomized to posterior crural inspection. CONCLUSION: Hiatal hernias are highly prevalent in SG patients. However, GerdQ, BEDQ, and a UGI series unreliably identify hiatal hernia in the preoperative setting and should not influence intraoperative evaluation of the hiatus during SG.

Pseudo-hyperacute T Waves in a Giant Hiatal Hernia.

An 89-year-old woman with a giant hiatal hernia complained of persistent chest pain. An electrocardiogram (ECG) showed hyperacute T waves, suggesting the early phase of ST-elevation myocardial infarction. After endoscopic drainage for hiatal hernia, the chest pain disappeared, and the ECG abnormalities resolved. The present case illustrates that compression of the heart by a giant hiatal hernia can induce T wave elevation mimicking acute coronary syndrome.

A Massive Para-Esophageal Hernia: An Unusual Cause of Tension Physiology Leading to Cardiac Arrest.

Here, we describe the case of a 28-year-old man with history of super morbid obesity and Type IV para-esophageal hernia, who experienced cardiac arrest following incarceration and strangulation of his hernia sac. He required emergency surgery including an exploratory laparotomy and thoracotomy, with splenectomy, omentectomy, and partial gastrectomy. He was subsequently transferred to our institution due to the anticipated difficulty of restoring intestinal continuity. Continuity was established with a retrocolic Roux-en-Y reconstruction with a 90 cm Roux limb. Despite significant soft-tissue necrosis, he eventually recovered and was able to be transferred back to pursue rehabilitation closer to his home in a rural community.

Hiatal Hernia After Esophagectomy: A Single-Center Retrospective Analysis of a Rare Postoperative Phenomenon.

Development of a post-esophagectomy hiatal hernia (PEHH) is a rare, but problematic, sequela with the current reported prevalence ranging up to 20%. To determine the incidence rate of PEHH at our institution, a retrospective review of all transhiatal esophagectomies performed from 2012 to 2020 was conducted. Demographic, operative, and oncologic data were collected, rates of PEHH were calculated, and characteristics of subsequent repair were reviewed and analyzed. A total of 160 transhiatal esophagectomies were included, of which four patients (2.5%) developed a PEHH at a mean of 12 months postoperatively (range: 3-28 months) with symptomatology driving the diagnosis for three patients. The limited size of our study does not allow for statistically significant determinations regarding risk factors or method of repair. The true prevalence of a hiatal defect is likely higher than reported, as clinically asymptomatic patients are not captured in our current literature.

Giant Paraesophageal Hernia With Obstructing Splenic Flexure Mass in the Left Hemithorax.

Giant paraesophageal hernias contain greater than fifty percent of the stomach above the diaphragm. Over fifty percent of large bowel obstructions are due to colorectal adenocarcinoma. Here, we present a rare case of a 69-year-old female patient who developed a closed loop colonic obstruction caused by a colonic mass in the distal transverse colon within a giant paraesophageal hernia. We successfully performed emergent paraesophageal hernia reduction and mesh repair with extended right hemicolectomy and ileocolonic anastomosis. Emergent hernia repair via an abdominal approach can be used in this setting.