The 2018 ISDE achalasia guidelines.

Achalasia is a relatively rare primary motor esophageal disorder, characterized by absence of relaxations of the lower esophageal sphincter and of peristalsis along the esophageal body. As a result, patients typically present with dysphagia, regurgitation and occasionally chest pain, pulmonary complication and malnutrition. New diagnostic methodologies and therapeutic techniques have been recently added to the armamentarium for treating achalasia. With the aim to offer clinicians and patients an up-to-date framework for making informed decisions on the management of this disease, the International Society for Diseases of the Esophagus Guidelines proposed and endorsed the Esophageal Achalasia Guidelines (I-GOAL). The guidelines were prepared according the Appraisal of Guidelines for Research and Evaluation (AGREE-REX) tool, accredited for guideline production by NICE UK. A systematic literature search was performed and the quality of evidence and the strength of recommendations were graded according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Given the relative rarity of this disease and the paucity of high-level evidence in the literature, this process was integrated with a three-step process of anonymous voting on each statement (DELPHI). Only statements with an approval rate >80% were accepted in the guidelines. Fifty-one experts from 11 countries and 3 representatives from patient support associations participated to the preparations of the guidelines. These guidelines deal specifically with the following achalasia issues: Diagnostic workup, Definition of the disease, Severity of presentation, Medical treatment, Botulinum Toxin injection, Pneumatic dilatation, POEM, Other endoscopic treatments, Laparoscopic myotomy, Definition of recurrence, Follow up and risk of cancer, Management of end stage achalasia, Treatment options for failure, Achalasia in children, Achalasia secondary to Chagas' disease.

Effects and efficacy of laparoscopic fundoplication in children with GERD: a prospective, multicenter study.

INTRODUCTION: Laparoscopic antireflux surgery (LARS) in children primarily aims to decrease reflux events and reduce reflux symptoms in children with therapy-resistant gastroesophageal reflux disease (GERD). The aim was to objectively assess the effect and efficacy of LARS in pediatric GERD patients and to identify parameters associated with failure of LARS. METHODS: Twenty-five children with GERD [12 males, median age 6 (2-18) years] were included prospectively. Reflux-specific questionnaires, stationary manometry, 24-h multichannel intraluminal impedance pH monitoring (MII-pH monitoring) and a 13C-labeled Na-octanoate breath test were used for clinical assessment before and 3 months after LARS. RESULTS: After LARS, three of 25 patients had persisting/recurrent reflux symptoms (one also had persistent pathological acid exposure on MII-pH monitoring). New-onset dysphagia was present in three patients after LARS. Total acid exposure time (AET) (8.5-0.8 %; p < 0.0001) and total number of reflux episodes (p < 0.001) significantly decreased and lower esophageal sphincter (LES) resting pressure significantly increased (10-24 mmHg, p < 0.0001) after LARS. LES relaxation, peristaltic contractions and gastric emptying time did not change. The total number of reflux episodes on MII-pH monitoring before LARS was a significant predictor for the effect of the procedure on reflux reduction (p < 0.0001). CONCLUSIONS: In children with therapy-resistant GERD, LARS significantly reduces reflux symptoms, total acid exposure time (AET) and number of acidic as well as weakly acidic reflux episodes. LES resting pressure increases after LARS, but esophageal function and gastric emptying are not affected. LARS showed better reflux reduction in children with a higher number of reflux episodes on preoperative MII-pH monitoring.

Effect of Vagus Nerve Integrity on Short and Long-Term Efficacy of Antireflux Surgery.

OBJECTIVES: Vagus nerve injury is a feared complication of antireflux surgery (ARS) that may negatively affect reflux control. The aim of the present prospective study was to evaluate short-term and long-term impact of vagus nerve injury, evaluated by pancreatic polypeptide response to insulin-induced hypoglycemia (PP-IH), on the outcome of ARS. METHODS: In the period from 1990 until 2000, 125 patients with gastroesophageal reflux disease (GERD) underwent ARS at a single center. Before and 6 months after surgery, vagus nerve integrity testing (PP-IH), 24-h pH-monitoring, gastric emptying, and reflux-associated symptoms were evaluated. In 2014, 14-25 years after surgery, 110 patients were contacted again for evaluation of long-term symptomatic outcome using two validated questionnaires (Gastrointestinal Symptom Rating Scale (GSRS) and GERD-Health Related Quality of Life (HRQL)). RESULTS: Short-term follow-up: vagus nerve injury (PP peak ≤47 pmol/l) was observed in 23 patients (18%) 6 months after fundoplication. In both groups, a comparable decrease in reflux parameters and symptoms was observed at 6-month follow-up. Postoperative gastric emptying was significantly delayed in the vagus nerve injury group compared with the vagus nerve intact group. Long-term follow-up: patients with vagus nerve injury showed significantly less effective reflux control and a higher re-operation rate. CONCLUSIONS: Vagus nerve injury occurs in up to 20% of patients after ARS. Reflux control 6 months after surgery was not affected by vagus nerve injury. However, long-term follow-up showed a negative effect on reflux symptom control and re-operation rate in patients with vagus nerve injury.

[Ancillary diagnostic imaging is undesirable if there is evidence of appendicitis]. / Aanvullend beeldvormend onderzoek is ongewenst bij aanwijzingen voor appendicitis.

CT and ultrasound have been advocated to improve the diagnostic accuracy and management of appendicitis. However, these were single-centre studies, performed by specialised radiologists and with a low level of evidence. Most of the literature shows no increase in diagnostic accuracy or decrease of negative appendectomies if radiographic imaging techniques are applied. However these techniques do lead to disadvantages such as radiation load and unnecessary prolongation of the diagnostic phase. Therefore, CT or ultrasound are of no use in the diagnosis and management of acute appendicitis.

Impact of laparoscopic antireflux surgery on belching in pediatric GERD patients.

BACKGROUND: Laparoscopic antireflux surgery (LARS) is a well-established treatment option for children with proton pomp inhibitor (PPI)-resistant gastroesophageal reflux disease (GERD). Besides preventing reflux of gastric fluid and solid content, LARS may also impair the ability of the stomach to vent intragastric air (i.e. gastric belching) and induce gas-related complications, such as bloating and/or hyperflatulence. Furthermore, it was previously hypothesized that LARS induces a behavioral type of belching, not originating from the stomach, called supragastric belching. The aim of this study was to objectively evaluate the impact of LARS on gastric (GB) and supragastric belching (SGB) in children with GERD. METHODS: We performed a prospective, Dutch multicenter cohort study including 25 patients (12 males, median age 6 (range 2-18) years) with PPI-resistant GERD who were scheduled for LARS. Twenty-four-hour multichannel intraluminal impedance pH monitoring (MII-pH monitoring) was performed before and 3 months after fundoplication. Impedance pH tracings were analyzed for reflux episodes and GBs and SGBs. KEY RESULTS: LARS reduced acid exposure time from 8.5% (6.0-16.2%) to 0.8% (0.2-2.8%), p < 0.001. The number of GBs also significantly decreased after LARS (59 [43-77] VS 5 [2-12], p < 0.001). The number of air swallows remained unchanged after LARS. SGBs were infrequent before LARS with no change in the number of SGB observed after the procedure. Postoperative belching symptoms were associated with GBs, not with SGBs. CONCLUSION & INFERENCES: LARS significantly reduces the number of GBs in children with GERD, whereas the number of air swallows remains unchanged. Postoperative symptomatic belching is associated with GBs, but not with SGBs. These findings suggest that LARS does not induce the occurrence of SGBs in children, but longer follow-up is required.

Complete versus partial fundoplication in children with gastroesophageal reflux disease: results of a systematic review and meta-analysis.

Complete fundoplication (Nissen) has long been accepted as the gold standard surgical procedure in children with therapy-resistant gastroesophageal reflux disease (GERD); however, increasingly more evidence has become available for partial fundoplication as an alternative. The aim of this study was to perform a systematic review and meta-analysis comparing complete versus partial fundoplication in children with therapy-resistant GERD. PubMed (1960 to 2011), EMBASE (from 1980 to 2011), and the Cochrane Library (issue 3, 2011) were systematically searched according to the PRISMA statement. Results were pooled in meta-analyses and expressed as risk ratios (RRs). In total, eight original trials comparing complete to partial fundoplication were identified. Seven of these studies had a retrospective study design. Short-term (RR 0.64; p = 0.28) and long-term (RR 0.85; p = 0.42) postoperative reflux control was similar for complete and partial fundoplication. Complete fundoplication required significantly more endoscopic dilatations for severe dysphagia (RR 7.26; p = 0.007) than partial fundoplication. This systematic review and meta-analysis showed that reflux control is similar after both complete and partial fundoplication, while partial fundoplication significantly reduces the number of dilatations to treat severe dysphagia. However, because of the lack of a well-designed study, we have to be cautious in making definitive conclusions. To decide which type of fundoplication is the best practice in pediatric GERD patients, more randomized controlled trials comparing complete to partial fundoplication in children with GERD are warranted.

The 2018 ISDE achalasia guidelines

Achalasia is a relatively rare primary motor esophageal disorder, characterized by absence of relaxations of the lower esophageal sphincter and of peristalsis along the esophageal body. As a result, patients typically present with dysphagia, regurgitation and occasionally chest pain, pulmonary complication and malnutrition. New diagnostic methodologies and therapeutic techniques have been recently added to the armamentarium for treating achalasia. With the aim to offer clinicians and patients an up-to-date framework for making informed decisions on the management of this disease, the International Society for Diseases of the Esophagus Guidelines proposed and endorsed the Esophageal Achalasia Guidelines (I-GOAL). The guidelines were prepared according the Appraisal of Guidelines for Research and Evaluation (AGREE-REX) tool, accredited for guideline production by NICE UK. A systematic literature search was performed and the quality of evidence and the strength of recommendations were graded according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Given the relative rarity of this disease and the paucity of high-level evidence in the literature, this process was integrated with a three-step process of anonymous voting on each statement (DELPHI). Only statements with an approval rate >80% were accepted in the guidelines. Fifty-one experts from 11 countries and 3 representatives from patient support associations participated to the preparations of the guidelines. These guidelines deal specifically with the following achalasia issues: Diagnostic workup, Definition of the disease, Severity of presentation, Medical treatment, Botulinum Toxin injection, Pneumatic dilatation, POEM, Other endoscopic treatments, Laparoscopic myotomy, Definition of recurrence, Follow up and risk of cancer, Management of end stage achalasia, Treatment options for failure, Achalasia in children, Achalasia secondary to Chagas' disease.

Hirschsprung's disease in children with Down syndrome: a comparative study.

BACKGROUND AND AIM: There is controversy in the literature regarding the outcome after surgical treatment of Hirschsprung's disease in children with Down syndrome (DS). The aim of this study was to compare the outcome of our series of DS children with Hirschsprung's disease to our series of children without Down syndrome (NDS) with Hirschsprung's disease. The impact of laparoscopy within the DS group was analyzed. MATERIAL AND METHODS: Between March 1987 and August 2008, 149 children were operated on for Hirschsprung's disease. 20 children of this group were additionally diagnosed with Down syndrome. All children underwent either an open or a laparoscopic Duhamel procedure. We evaluated postoperative hospital stay, short-term complications and the incidence of enterocolitis, constipation and incontinence. RESULTS: 20 patients (13.4%) in this series had Down syndrome. There were no significant differences in the extent of aganglionosis between children with or without Down syndrome. There were no intra-operative complications and no conversions. Postoperative leak occurred significantly more often in children with DS (n=5, 25%) compared to NDS children (n=1, 0.7%; p<0.0001). Postoperative leakage-related abscess formation was higher in the DS group (n=3, 15%) compared to the NDS group (0%). Within the DS group there was no significant difference between open or laparoscopic Duhamel procedure with regard to these postoperative complications. Postoperative hospital stay was significantly longer in the DS group compared to the NDS group (p<0.05). In the DS group there was a slightly shorter postoperative stay after laparoscopic Duhamel procedure. Mean long-term follow-up was 5.1 years. One death occurred in the DS group 9 months postoperatively due to sepsis and cardiomyopathy. Severe constipation was present significantly more often in DS children (n=11, 55%) compared to NDS children (n=29, 22.3%; p<0.01). There was no difference in incontinence between DS and NDS children. Enterocolitis occurred more frequently in DS patients after operation (40 [31% NDS] vs. 9 [45% DS]; p=0.038). CONCLUSION: Compared to NDS children, children with DS have a higher rate of postoperative complications and a longer hospital stay. During long-term follow-up most patients with DS are severely constipated and have a higher incidence of enterocolitis.

Neurenteric cyst--a case report of this rare disorder.

Neurenteric cysts are rare congenital lesions that are believed to be the result of the split notochord syndrome. We report the clinical case of a 5-year-old boy presenting with vague gastrointestinal symptoms and fatigue, who had undergone resection of a small intestine duplication cyst as a newborn. Computed tomography revealed a mediastinal neurenteric cyst with partial destruction of several thoracic vertebrae. Resection of the tumor proved effective. Recognition of this disorder is important: because of its benign nature, the prognosis after surgical resection can be good. If the diagnosis is made in an early stage, unnecessary progressive destruction of surrounding structures may be prevented.