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Achalasia is a rare esophageal motility disorder. Much of the literature is based on the adult population. In adults, guidance of therapeutic approach by manometric findings has led to improvement in patient outcome. Promising results have been achieved with novel therapies such as PerOral Endoscopic Myotomy (POEM). Areas covered: In this review, we provide an overview of the novel diagnostic and therapeutic tools for achalasia management and in what way they will relate to the future management of pediatric achalasia. We performed a PubMed and EMBASE search of English literature on achalasia using the keywords 'children', 'achalasia', 'pneumatic dilation', 'myotomy' and 'POEM'. Cohort studies < 10 cases and studies describing patients ≥ 20 years were excluded. Data regarding patient characteristics, treatment outcome and adverse events were extracted and presented descriptively, or pooled when possible. Expert commentary: Available data report that pneumatic dilation and laparoscopic Heller's myotomy are effective in children, with certain studies suggesting lower success rates in pneumatic dilation. POEM is increasingly used in the pediatric setting with promising short-term results. Gastro-esophageal reflux disease (GERD) may occur post-achalasia intervention due to disruption of the LES and therefore requires diligent follow-up, especially in children treated with POEM.

Objectives: Pediatric achalasia is a rare neurodegenerative disorder of the esophagus that requires treatment. Different diagnostic and treatment modalities are available, but there are no data that show how children can best be diagnosed and treated. We aimed to identify current practices regarding the diagnostic and therapeutic approach toward children with achalasia. Methods: Information on the current practice regarding the management of pediatric achalasia was collected by an online-based survey sent to members of the European and North American Societies for Pediatric Gastroenterology Hepatology and Nutrition involved in pediatric achalasia care. Results: The survey was completed by 38 centers from 24 countries. Within these centers, 108 children were diagnosed with achalasia in the last year (median 2, range 0–15). Achalasia was primarily managed by a pediatric gastroenterologist (76%) and involved a multidisciplinary team in 84% of centers, also including a surgeon (87%), radiologist (61%), dietician (37%), speech pathologist (8%), and psychologist (5%). Medical history taking and physical examination were considered most important to establish the diagnosis (50%), followed by (a combination of) manometry (45%) or contrast swallow (21%). Treatment of first choice was Heller myotomy (58%), followed by pneumatic dilation (46%) and peroral endoscopic myotomy (29%). Conclusion: This study shows a great heterogeneity in the management of pediatric achalasia amongst different centers worldwide. These findings stress the need for well-designed intervention trials in children with achalasia. Given the rarity of this disease, we recommend that achalasia care should be managed in centers with access to appropriate diagnostic and treatment modalities.

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3D imaging can be used for objective measurement of facial asymmetry in HNRMS survivors. The raw asymmetry index correlated with a clinical assessment of facial deformity. Comparisons between treatment groups seemed inappropriate given the differences in facial asymmetry between British and Dutch controls. In future studies, pretreatment images could act as matched controls for posttreatment evaluation.

Objectives: After surgical repair, up to 70% of esophageal atresia (EA) patients suffer from gastroesophageal reflux disease (GERD). The ESPGHAN/NASPGHAN guidelines on management of gastrointestinal complications in EA patients were published in 2016. Yet, the implementation of recommendations on GERD management remains poor. We aimed to assess GERD management in EA patients in more detail, to identify management inconsistencies, gaps in current knowledge, and future directions for research. Methods: A digital questionnaire on GERD management in EA patients was sent to all members of the ESPGHAN EA working group and members of the International network of esophageal atresia (INoEA). Results: Forty responses were received. Thirty-five (87.5%) clinicians routinely prescribed acid suppressive therapy for 1–24 (median 12) months. A fundoplication was considered by 90.0% of clinicians in case of refractory GERD with persistent symptoms despite maximal acid suppressive therapy and in 92.5% of clinicians in case of GERD with presence of esophagitis on EGD. Half of clinicians referred patients with recurrent strictures or dependence on transpyloric feeds. Up to 25.0% of clinicians also referred all long-gap EA patients for fundoplication, those with long-term need of acid suppressants, recurrent chest infections and feedings difficulties. Conclusions: Respondents’ opinions on the optimal duration for routine acid suppressive therapy and indications for fundoplication in EA patients varied widely. To improve evidence-based care for EA patients, future prospective multicenter outcome studies should compare different diagnostic and treatment regimes for GERD in patients with EA. Complications of therapy should be one of the main outcome measures in such trials.

Summary Up to 45% of esophageal atresia (EA) patients undergo fundoplication during childhood. Their esophageal dysmotility may predispose to worse fundoplication outcomes compared with patients without EA. We therefore compared fundoplication outcomes and symptoms pre- and post-fundoplication in EA patients with matched patients without EA. A retrospective review of patients with- and without EA who underwent a fundoplication was performed between 2006 and 2017. Therapeutic success was defined as complete sustained resolution of symptoms that were the reason to perform fundoplication. Fundoplication indications of 39 EA patients (49% male; median age 1.1 [0.1–17.0] yrs) and 39 non-EA patients (46% male; median age 1.3 [0.3–17.0] yrs) included respiratory symptoms, brief resolved unexplained events, typical symptoms of gastroesophageal reflux disease, recurrent strictures and respiratory problems. Post-fundoplication, therapeutic success was achieved in 5 (13%) EA patients versus 29 (74%) non-EA patients (P&lt;0.001). Despite therapeutic success, all 5 (13%) EA patients developed postoperative sustained symptoms/complications versus 12 (31%) non-EA patients. Eleven (28%) EA patients versus 3 (8%) non-EA patients did not achieve any therapeutic success (P=0.036). Remaining patients achieved partial therapeutic success. EA patients suffered significantly more often from postoperative sustained dysphagia (41% vs. 13%; P=0.039), gagging (33% vs. 23%; P&lt;0.001) and bloating (40% vs. 17%; P=0.022). Fundoplication outcomes in EA patients are poor and EA patients are more susceptible to post-fundoplication sustained symptoms and complications compared with patients without EA. The decision to perform fundoplication in EA patients with proven gastroesophageal reflux disease needs to be made with caution after thorough multidisciplinary evaluation.