Ann Aspirot

Characterization of Esophageal Motility Following Esophageal Atresia Repair Using High-Resolution Esophageal Manometry

Background: Esophageal dysmotility, a considerable issue following esophageal atresia (EA) repair, has been reported but has not been precisely described and characterized. Using high-resolution esophageal manometry (HREM), we characterized the esophageal motility patterns in children with repaired EA and compared these patterns of dysmotility with symptomatology. Methods: HREM was performed as an outpatient procedure in patients with repaired EA. The tracings were analyzed using the software provided by the company and were then reviewed visually. Charts were reviewed for medical/surgical histories and symptoms were assessed by a standardized questionnaire. Results: Forty patients (25 boys, 15 girls) with a median age of 8 years (11 months–18 years) underwent an HREM. Thirty-five patients had type C EA and 5 had type A EA. Only 7 patients were asymptomatic at the time of the examination. HREM results were abnormal in all of the patients. Three different esophageal motility patterns were derived from HREM tracing analysis: aperistalsis (15 patients, 38%), pressurization (6 patients, 15%), and distal contractions (19 patients, 47%). Distal contractions pattern was found exclusively in type C EA. Dysphagia was encountered in the 3 groups. Gastroesophageal reflux disease-related symptoms predominated in the aperistalsis group. Conclusions: HREM improves our understanding and allows precise characterization of esophageal dysmotility in patients who have undergone EA repair.

Antegrade enemas for defecation disorders: do they improve the colonic motility?

PURPOSE The aim of the study was to describe the changes in colonic motility occurring after chronic antegrade enema use in children and young adults. METHODS Colonic manometry tracings of patients who had used antegrade enemas for at least 6 months and were being evaluated for possible discontinuation of this treatment were retrospective reviewed. RESULTS Seven patients (median age of 12 years, range 3-15 years) met our inclusion criteria. Four patients had idiopathic constipation, 2 had tethered cord, and 1 had Hirschsprung disease. Colonic manometry before the use of antegrade enemas showed dysmotility in 6 (86%) children, mostly in the distal colon. None of the patients underwent colonic resection between the 2 studies. All the patients had colonic manometry repeated between 14 and 46 months after the creation of the cecostomy. All patients with abnormal colonic manometry improved with the use of antegrade enema with a complete normalization of colonic motility in 5 (83%) patients. CONCLUSION Use of antegrade enema alone, without diversion or resection, may improve colonic motility.

A study of calretinin in Hirschsprung pathology, particularly in total colonic aganglionosis

INTRODUCTION Calretinin, a calcium-binding protein, has been reported to be an important new marker in Hirschsprungs disease (HD). The aim is to study the diagnostic value of Calretinin in total colonic aganglionosis (TA), prematurity, and superficial biopsy when nerve hyperplasia may not be accessed by ACE activity. METHODS Records of patients diagnosed with HD at our institution from 1985 to 2010 were studied and patients with TA identified. We examined tissue samples from those TA, partial colectomies for HD, biopsies for suspicion of HD, and rectal tissue from aborted fetuses. Immunohistochemical analysis of Calretinin was compared with ACE gold standard method in all cases. RESULTS In the majority of the cases, the diagnosis was ascertained by ACE activity and Calretinin staining. However, in 9 cases, the diagnosis was possible with Calretinin staining but not with ACE: in 4 TA because of the absence of nerve hyperplasia, and in 5 cases because the biopsies were too superficial to examine the nerve hyperplasia. In addition, Calretinin was expressed in the gut as early as 22 gestational weeks. CONCLUSION The use of Calretinin staining may be superior to ACE activity, particularly in the context of TA, superficial biopsies, and prematurity, allowing earlier diagnosis.

Effect of amoxicillin/clavulanate on gastrointestinal motility in children.

Aim: The aim of the present study was to evaluate the effect of amoxicillin/clavulanate (A/C) on gastrointestinal motility. Methods: Twenty consecutive pediatric patients referred for antroduodenal manometry received 20 mg/kg of A/C into the small bowel lumen. In 10 patients (group A), A/C was given 1 hour after and in 10 (group B), 1 hour before ingestion of a meal. Characteristics of the migrating motor complex, including presence, frequency, amplitude, and propagation of duodenal phase III and phase I duration and phase II motility index (MI), were evaluated 30 minutes before and after A/C administration. Results: There were no statistically significant differences in age and sex between the 2 groups. Manometry studies were considered normal in 8 patients in each group. In group A, 2 patients developed duodenal phase III after receiving A/C, and no significant difference was found in the MI before and after the drug administration. In group B, 9 patients developed duodenal phase III (P < 0.05 vs group A). All phase III occurred within a few minutes from the medication administration. Most duodenal phase III contractions were preceded by an antral component during fasting but never after the medication was administered in either of the 2 groups (P < 0.001 vs fasting). In group B, the duration of duodenal phase I was shorter after drug administration (P < 0.05). There was no significant difference in duodenal phase II MI before and after A/C administration for the 2 study groups. Conclusions: In children, administration of A/C directly into the small bowel before a meal induces phase III-type contractions in the duodenum, with characteristics similar to those present in the fasting state. These data suggest the possible use of A/C as a prokinetic agent. Further studies are needed to clarify its specific mechanism of action and the group of patients most likely to benefit from its use.

Esophageal dysmotility is present before surgery in isolated tracheoesophageal fistula.

ABSTRACT After surgical correction of esophageal atresia with or without tracheoesophageal fistula, esophageal body motility dysfunction has been reported in nearly all patients. Using high-resolution esophageal manometry before surgical repair in 2 children with isolated tracheoesophageal fistula, we sought to determine whether dysmotility was present before any surgical insult to test the hypothesis that dysmotility associated with esophageal atresia with or without tracheoesophageal fistula is related to intrinsic primary factors linked to abnormal development of the esophagus. Both had an abnormal esophageal motility: one exhibited hypomotility with distal contraction, whereas the other showed a complete aperistalsis pattern. This suggests that esophageal dysmotility is congenital in nature rather than secondary to surgery.

Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula

OBJECTIVE To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA). METHODS All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively. RESULTS Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%. CONCLUSION ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.

Do some adolescents with rumination syndrome have "supragastric vomiting"?

JPGN Volume 50, ome is defined by the R umination syndr Rome III criteria as repeated painless regurgitation and rechewing or expulsion of food within minutes of starting a meal (1). It is a phenomenon considered normal in ruminant animals, but its occurrence in humans is always pathologic. This syndrome had been reported in the past as being prevalent among infants and adults with developmental disabilities (2,3), but more recently its recognition has increased among children, adolescents, and adults with normal intelligence (4,5). The pathophysiology of rumination remains incompletely understood. The aim of this article is to describe 2 cases of adolescent girls with rumination syndrome in which we identified a new pathophysiologic mechanism similar to what has been described in supragastric belching (6). We termed it ‘‘supragastric vomiting’’ and to our knowledge it has not been described before.

A survey-based assessment of the Canadian pediatric surgery workforce.

BACKGROUND There is significant lack of information regarding the Canadian pediatric surgery workforce. METHODS An IRB-approved survey aimed at assessing workforce issues was administered to pediatric surgeons and pediatric surgery chiefs in Canada in 2012. RESULTS The survey was completed by 98% of practicing surgeons and 13 of the 18 division chiefs. Only 6% of surgeons are older than 60 years, and only a fifth anticipate retirement over the next decade. The workforce is stable, with 82% of surgeons unlikely to change current positions. Surgical volume showed essentially no growth during the 5-year period 2006-2010. The majority of surgeons felt they were performing the right number or too few cases and anticipated minimal or no future growth in their individual practices or that of their group. Based on anticipated vacancies, the best estimate is a need for 20 new pediatric surgeons over the next decade. This need is significantly surpassed by the current output from the Canadian training programs. CONCLUSIONS The Canadian pediatric surgery workforce is currently saturated. The mismatch between the number of graduating trainees and the available positions over the next decade has significant repercussions for current surgery and pediatric surgery residents wishing to practice in Canada.

Esophageal Motor Disorders: Achalasia, Diffuse Esophageal Spasm, Nonspecific Motor Disorders, Eosinophilic Esophagitis

The esophagus, which plays a primary role in food transport, is a collapsible organ with three main sections: upper esophageal sphincter (UES), esophageal body, and lower esophageal sphincter (LES). This chapter details basic pathophysiologic aspects of four disorders of esophageal motility, esophageal achalasia, esophageal spasm, nutcracker esophagus, and eosinophilic esophagitis and briefly describes nonspecific esophageal motility disorders. Each disorder causes different eating and digestive symptoms (dysphagia, abdominal and chest pain, heart burn, regurgitation of digested food) and may appear to mimic GERD or esophageal reflux. In some disorders, such as achalasia, much of the literature is based on the adult population, while pediatric information is noted only in case and retrospective studies. On the other hand, eosinophilic esophagitis is more prevalent in the pediatric population and may be related to food and environmental allergens. Diffuse esophageal spasm and nutcracker esophagus are benign and very rare in the pediatric population.

High-resolution Esophageal Manometry Patterns in Children and Adolescents With Rumination Syndrome

Background: Rumination is defined by effortless regurgitation within seconds or minutes of ingested food. The aim of this study was to determine the high-resolution esophageal manometry (HREM) pattern in children with rumination syndrome. Methods: HREM was evaluated in 15 pediatric patients with rumination syndrome according to the Rome criteria and compared with 15 controls. Primary rumination was defined as a clinical rumination episode associated with a rise of gastric pressure above 30 mmHg. Secondary rumination was defined as a clinical rumination episode associated with a rise of gastric pressure above 30 mmHg during a transient lower esophageal sphincter relaxation (TLESR). Results: Ninety-two episodes of rumination were demonstrated during HREM study in 12 of the 15 patients (80%; 1–29 episodes per patient; median intragastric pressure 49.6 mmHg). Primary rumination occurred in 3 patients and secondary rumination in 5 patients. One patient had primary and secondary rumination episodes. In 3 patients, classification of rumination episodes was not possible due to repetitive swallowing leading to lower esophageal sphincter relaxation. In the control group, no episodes of rumination occurred. The sensitivity and the specificity of the HREM study (association of a clinical rumination episode with a rise in gastric pressure >30 mmHg) to confirm the diagnosis of rumination were 80% and 100%, respectively. Conclusions: HREM allows confirming diagnosis of rumination syndrome and to differentiate between primary and secondary rumination in the presence of objective rumination episodes. Further research is needed to study whether HREM results may influence treatment and outcome of children with rumination syndrome.