Contemporary Management of Achalasia by Pediatric Surgeons: A Survey of the International Pediatric Endosurgical Group

Gould, Joanna L.; Rentea, Rebecca M.; Peter, Shawn D. St.

doi:10.1089/lap.2016.0085

Cited by 15 publications

(6 citation statements)

References 11 publications

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“…Esophageal manometry was available in most centers but was only considered by half to be the most important diagnostic investigation. These findings are consistent with a survey conducted by the International Pediatric Endosurgical group, where 73% requested a manometry before intervention (14). Access to and experience with HRM remains a limitation in pediatric practice despite the fact that HRM is now considered the best test for diagnosing and subtyping achalasia in adult patients.…”

Section: Discussionsupporting

confidence: 90%

Clinical Management of Pediatric Achalasia

Lennep

Wijk

Omari

et al. 2019

J. pediatr. gastroenterol. nutr.

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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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Section: Discussionsupporting

confidence: 90%

Clinical Management of Pediatric Achalasia

Lennep

Wijk

Omari

et al. 2019

J. pediatr. gastroenterol. nutr.

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“…[23] The current lack of evidence supporting one treatment approach over another, combined with the rarity of achalasia in children, has led to wide variation in practice. [24] Well-designed multi-institutional studies are needed to improve treatment and prevent the adverse impact this disease has on a child's long-term quality of life. [16]…”

Section: Esophageal Achalasiamentioning

confidence: 99%

Pediatric enteric neuropathies: diagnosis and current management

Westfal¹,

Goldstein

2017

Current Opinion in Pediatrics

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Purpose of Review Neurointestinal diseases are increasingly recognized as causes of significant gastrointestinal morbidity in children. This review highlights the most common pediatric enteric neuropathies and their diagnosis and management, emphasizing insights and discoveries from the most recent literature available. Recent Findings The embryologic and histopathologic causes of enteric neuropathies are varied. They range from congenital aganglionosis in Hirschsprung disease, to autoimmune-mediated loss of neuronal subtypes in esophageal achalasia and Chagas disease, to degenerative neuropathies in some cases of chronic intestinal pseudo-obstruction and gastroparesis. Increased awareness of the clinical presentation and diagnostic evaluation of these conditions is essential as it allows for earlier initiation of treatment and improved outcomes. Most current therapies, which include medical management, neurostimulation, and operative intervention, aim to minimize the symptoms caused by these conditions. The evidence base for many of these treatments in children is poor, and multi-institutional prospective studies are needed. An innovative therapy on the horizon involves using neuronal stem cell transplantation to treat the underlying pathology by replacing the missing or damaged neurons in these diseases. Summary While recent advances in basic and clinical neurogastroenterology have significantly improved our awareness and understanding of enteric neuropathies, the efficacy of current treatment approaches is limited. The development of novel therapies, including pharmacologic modulators of neurointestinal function, neurostimulation to enhance gut motility, and neuronal cell-based therapies, is essential to improve the long-term outcomes in children with these disorders.

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“…LHM for achalasia was first described in 1991 by Shimi et al 28 It was later introduced as a treatment for children by Holcomb et al 29 Since then, many authors have demonstrated that LHM is a safe and effective treatment even in pediatric age. [30][31][32][33][34] A recent survey among International Pediatric Endosurgical Group members outlined LHM as preferred technique in the treatment of pediatric EA. Results were consistent across the patient ages, and 94% of responders performed a concomitant partial gastric fundoplication.…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic Heller–Dor Is a Persistently Effective Treatment for Achalasia Even in Pediatric Patients: A 25-Year Experience at a Single Tertiary Center

et al. 2023

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Background Esophageal achalasia (EA) is a rare primary motility disorder in any age group, and particularly rare in the pediatric population, with a reported incidence of 0.18 per 100,000 children a year. EA in pediatric age is currently treated in the same way as in adults, but this approach is based on only a few studies on small case series. The aim of this retrospective study was to assess the long-term outcome of the laparoscopic Heller–Dor (LHD) procedure when performed in pediatric patients with EA at our university hospital. Materials and Methods We considered children and adolescents younger than 16 years old diagnosed with EA and treated with LHD between 1996 and 2022. Clinical data were prospectively collected in an ongoing database. Symptoms were recorded and their severity was calculated using the Eckardt score. Barium swallow, esophageal manometry (conventional or high-resolution), and endoscopy were performed before and after the surgical procedure. Results During the study period, 40 children with a median age of 14 years (interquartile range [IQR]: 11–15) underwent LHD. At a median follow-up of 10.5 years (IQR: 4.5–13.9), a good outcome was achieved in 36/40 patients (90%). Two of the four patients whose surgical procedure failed underwent complementary pneumatic dilations successfully, thus increasing the overall success rate to 95%. A previous endoscopic treatment (in five patients) did not affect the final outcome (p = 0.49). An intraoperative mucosal lesion was detected in only one patient (2.5%) and was repaired at the time without further consequences. During the follow-up, 22 patients underwent endoscopy, and 17 had pH monitoring as well: only 2 of these patients showed reflux esophagitis at endoscopy (one of them with abnormal findings on pH monitoring), amounting to a 9.1% rate of instrumentally confirmed postoperative reflux. Conclusion LHD is a safe and persistently effective treatment for EA in pediatric age, with a success rate comparable with what is usually obtained in adults, and better than what has been reported to date in the pediatric literature. Adding a fundoplication certainly helps ensure an optimal long-term control of any gastroesophageal reflux induced by the myotomy.

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Contemporary Management of Achalasia by Pediatric Surgeons: A Survey of the International Pediatric Endosurgical Group

Abstract: Given the infrequency of achalasia in children, there are a range of treatment plans among pediatric surgeons. We have identified current practices as a first step in developing more standard treatment pathways.

Cited by 15 publications

References 11 publications

Clinical Management of Pediatric Achalasia

Clinical Management of Pediatric Achalasia

Pediatric enteric neuropathies: diagnosis and current management

Laparoscopic Heller–Dor Is a Persistently Effective Treatment for Achalasia Even in Pediatric Patients: A 25-Year Experience at a Single Tertiary Center

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