The not‐so‐rare absent RAIR: Internal anal sphincter achalasia in a review of 1072 children with constipation undergoing high‐resolution anorectal manometry

Baaleman, Desiree F.; Malamisura, Monica; Benninga, Marc A.; Bali, Neetu; Vaz, Karla; Yacob, Desale; Lorenzo, Carlo Di; Lu, Peter L.

doi:10.1111/nmo.14028

Cited by 18 publications

(25 citation statements)

References 33 publications

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“…Previous studies have confirmed that the presence of RAIR depends on intramural pathways [9,14]. For example, RA in patients with HD is due to the congenital absence of ganglion cells in the colon or rectum, which prevents the reflexive relaxation of the internal anal sphincter (IAS) after rectal distention [30].…”

Section: Discussionmentioning

confidence: 95%

“…There are many possible causes of RA including megarectum, IASA, severely low anal resting pressure, post rectal resection or coloanal anastomosis, rectal ischemia, rectal prolapse, chronic constipation (CC), fecal incontinence, systemic sclerosis (SSc), diabetic neuropathy, myelomeningocele, and Chagas disease [9,10,12,25,[29][30][31][32]. Examination-related reasons contributing to RA include displacements of catheters, artifacts, insufficient rectal balloon inflation, or stool impaction [14]. In our study, the organic causes for RA were excluded by reviewing detailed medical history, imaging examination, and other data when patients were enrolled.…”

Section: Discussionmentioning

confidence: 99%

“…We found that RA is not rarely observed on HR-ARM in adult patients with FC. A recent study reported that children with IASA showed more severe constipation and usually do not respond to laxatives [14]. Most of these studies have focused on children with constipation, and few studies focused on RA in adult patients with FC [15,16].…”

Section: Introductionmentioning

confidence: 99%

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Clinical Characteristics of Adult Functional Constipation Patients with Rectoanal Areflexia and Their Response to Biofeedback Therapy

Wang

Shah

et al. 2023

Diagnostics

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Background: The London Classification for anorectal physiological dysfunction specifically proposes rectoanal areflexia (RA), which means the absence of a rectoanal inhibitory reflex (RAIR) based on a manometric diagnosis. Although RA is not observed in healthy people, it can be found in adult patients with functional constipation (FC). This study describes the clinical manifestations of adult patients with FC and RA and their response to biofeedback therapy (BFT). Methods: This retrospective study reviewed the reports of high-resolution anorectal manometry (HR-ARM) and the efficacy of BFT in adult patients with FC. In addition, the Constipation Scoring System (CSS) scale, Patient Assessment of Constipation Symptoms (PAC-SYM) scale, Patient Assessment of Constipation Quality of Life (PAC-QOL) scale, Zung’s Self-Rating Anxiety Scale (SAS), Zung’s Self-Rating Depression Scale (SDS), balloon expulsion test (BET), and the use of laxatives were assessed. Results: A total of 257 adult patients diagnosed with FC were divided into the RA group (n = 89) and the RAIR group (n = 168). In the RA and RAIR groups, 60 (67.4%) and 117 (69.6%) patients, respectively, had dyssynergic defecation (DD) during simulated defecation. Type II pattern of dyssynergia was most frequently observed in both groups. Compared with the RA group, the RAIR group showed a higher CSS score, physical discomfort score, and prevalence of inadequate relaxation of the anal sphincter (p < 0.001, p = 0.036, and p = 0.017, respectively). The anxiety and depression scores were not different between the two groups. The proportion of patients using volumetric and stimulant laxatives and their combination was significantly higher in FC patients with RA, whereas the efficacy of BFT was significantly lower (p = 0.005, p < 0.001, p = 0.045, and p = 0.010, respectively). Conclusion: Adult FC patients with RA may suffer more severe constipation and have a lower efficacy of BFT compared with those with RAIR.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

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Clinical Characteristics of Adult Functional Constipation Patients with Rectoanal Areflexia and Their Response to Biofeedback Therapy

Wang

Shah

et al. 2023

Diagnostics

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“…Internal anal sphincter achalasia was also one of the causes of constipation, which was considered to be ultrashort-segment megacolon ( 10 , 11 ). However, recent studies have found that internal anal sphincter achalasia was different from HSCR in clinical manifestations, pathophysiology and treatment ( 12 , 13 ). The diagnostic of internal anal sphincter achalasia is determined by the following criteria: anorectal inhibitory reflex is negative, the internal anal sphincter has ganglion cells, the rectal mucosa is negative for acetylcholinesterase staining, and no surgical treatment is performed ( 11 , 14 ).…”

Section: Discussionmentioning

confidence: 99%

Treatment of postoperative intestinal dysfunction of hirschsprung's disease based on the principle of “anorectal balance”

Tian

Ma²,

Deng³

et al. 2022

Front. Surg.

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PurposeRadical surgery is the most effective treatment for Hirschsprung's disease. However, some children still have symptoms of intestinal dysfunction such as constipation, abdominal distension, and recurrent enterocolitis after operation. The purpose of this study was to evaluate treatment outcomes of postoperative intestinal dysfunction in children with Hirschsprung's disease by using the principle of “anorectal balance”.MethodsThe clinical data of postoperative intestinal dysfunction in children with Hirschsprung's disease in the single treatment group from July 2019 to July 2021 were retrospectively analyzed. All the enrolled children underwent botulinum toxin injection (2.5 U/kg); 3 to 6 months later, the injection was performed again; the children who had received more than two botulinum toxin injections underwent the internal sphincter myectomy. Anorectal manometry was performed routinely after operation, and abdominal distension and defecation were recorded.ResultsA total of thirty children with postoperative intestinal dysfunction underwent radical surgery for Hirschsprung's disease were included in this study. Symptoms of constipation, abdominal distension and enterocolitis were improved after botulinum toxin injections in most children compared to before surgery (P < 0.01). After re-injection of botulinum toxin in twelve children, the frequency of defecation increased, the anal resting pressure decreased, and the clinical symptoms were relieved again (P < 0.05). Eleven children underwent internal sphincter myectomy, and the symptoms of constipation, abdominal distension and enterocolitis were significantly improved after the operation (P < 0.01).ConclusionBotulinum toxin injection and internal sphincter myectomy based on the principle of “anorectal balance” can effectively reduce the resting pressure of the anus and relieve intestinal dysfunction, and have satisfactory clinical effect.

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“…Notably, specific aspects of the paediatric age include a distinct anatomical and physiological background, and a substantial role for early-life programming (10,11), hence the relevance of studying children, instead of translating directly from adult gastroenterology. This also refers to the application of established diagnostic techniques (such as manometry and pH-impedance) to uncover normal parameters and characterize pathophysiology in childhood (12,13).…”

Section: Diseases Of the Gastrointestinal Tract Neuro-gastroenterolog...mentioning

confidence: 99%

Making Research Flourish Through ESPGHAN

Gasparetto

Strisciuglio

Assa

et al. 2021

J. pediatr. gastroenterol. nutr.

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Recent research breakthroughs have emerged from applied basic research throughout all scientific areas, including adult and paediatric gastroenterology, hepatology and nutrition (PGHAN). The research landscape within the European Society of Paediatric Gastroenterology and Nutrition (ESPGHAN) is also inevitably changing from clinical research to studies involving applied laboratory research. This position paper aims to depict the current status quo of basic science and translational research within ESPGHAN, and to delineate how the society could invest in research in the present and future time. The paper also explores which research areas in the field of PGHAN represent the current and future priorities, and what type of support is needed across the ESPGHAN working groups (WGs) and special interest groups (SIGs) to fulfil their research goals.

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The not‐so‐rare absent RAIR: Internal anal sphincter achalasia in a review of 1072 children with constipation undergoing high‐resolution anorectal manometry

Abstract: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Cited by 18 publications

References 33 publications

Clinical Characteristics of Adult Functional Constipation Patients with Rectoanal Areflexia and Their Response to Biofeedback Therapy

Clinical Characteristics of Adult Functional Constipation Patients with Rectoanal Areflexia and Their Response to Biofeedback Therapy

Treatment of postoperative intestinal dysfunction of hirschsprung's disease based on the principle of “anorectal balance”

Making Research Flourish Through ESPGHAN

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