Leonel Rodriguez

Antegrade colonic enemas and intestinal diversion are highly effective in the management of children with intractable constipation

PURPOSE Intractable constipation in children is an uncommon but debilitating condition. When medical therapy fails, surgery is warranted; but the optimal surgical approach has not been clearly defined. We reviewed our experience with operative management of intractable constipation to identify predictors of success and to compare outcomes after 3 surgical approaches: antegrade continence enema (ACE), enteral diversion, and primary resection. METHODS A retrospective review of pediatric patients undergoing ACE, diversion, or resection for intractable, idiopathic constipation from 1994 to 2007 was performed. Satisfactory outcome was defined as minimal fecal soiling and passage of stool at least every other day (ACE, resection) or functional enterostomy without abdominal distension (diversion). RESULTS Forty-four patients (range = 1-26 years, mean = 9 years) were included. Sixteen patients underwent ACE, 19 underwent primary diversion (5 ileostomy, 14 colostomy), and 9 had primary colonic resections. Satisfactory outcomes were achieved in 63%, 95%, and 22%, respectively. Of the 19 patients diverted, 14 had intestinal continuity reestablished at a mean of 27 months postdiversion, with all of these having a satisfactory outcome at an average follow-up of 56 months. Five patients underwent closure of the enterostomy without resection, whereas the remainder underwent resection of dysmotile colon based on preoperative colonic manometry studies. Of those undergoing ACE procedures, age younger than 12 years was a predictor of success, whereas preoperative colonic manometry was not predictive of outcome. Second manometry 1 year post-ACE showed improvement in all patients tested. On retrospective review, patient noncompliance contributed to ACE failure. CONCLUSIONS Antegrade continence enema and enteral diversion are very effective initial procedures in the management of intractable constipation. Greater than 90% of diverted patients have an excellent outcome after the eventual restoration of intestinal continuity. Colon resection should not be offered as initial therapy, as it is associated with nearly 80% failure rate and the frequent need for additional surgery.

Factors associated with successful decrease and discontinuation of antegrade continence enemas (ACE) in children with defecation disorders: a study evaluating the effect of ACE on colon motility.

Background  Antegrade continence enemas (ACE) have been used in the treatment of defecation disorders in children; little is known on their effect on colon motility and the utility of the colon manometry (CM) predicting long‐term ACE outcomes.

Clinical presentation, response to therapy, and outcome of gastroparesis in children.

Objectives: The aims of the present study was to define the clinical features, response to therapy, and outcome of pediatric gastroparesis. Methods: Retrospective review of 230 children with gastroparesis. Demographics, gastric emptying times, symptoms, response to medications, and outcome were determined for each of 3 groups (infants, children, and adolescents). Results: Mean age was 9 years, with boys predominating among infants and girls among adolescents. Postviral gastroparesis occurred in 18% and mitochondrial dysfunction (MD) in 8%. Symptoms varied with age, with children experiencing more vomiting and adolescents reporting more nausea and abdominal pain. The addition of promotility drugs was an effective therapy. Overall rates of symptom resolution were 22% at 6 months, 53% at 18 months, and 61% at 36 months, with median time to resolution of 14 months. Factors associated with symptom resolution included younger age, male sex, postviral gastroparesis, shorter duration of symptoms, response to addition of promotility therapy, and absence of MD. In multivariate analysis, longer duration of symptoms and MD both predicted lower rates of resolution, whereas younger age and response to addition of promotility therapy predicted a higher rate. Conclusions: Pediatric gastroparesis is a complex condition with variable symptomatology and outcome depending on multiple parameters. Understanding the clinical features and response to therapy will improve our diagnosis and treatment of this disorder.

Endoscopic intrapyloric injection of botulinum toxin A in the treatment of children with gastroparesis: a retrospective, open-label study

BACKGROUND Botulinum toxin A has been used in children to treat spastic disorders and recently for GI conditions. Open-label studies in adults with gastroparesis have reported an improvement in symptoms and gastric emptying after endoscopic intrapyloric botulinum injections (IPBIs), although placebo-controlled trials have shown conflicting results. Only a single case report of IPBI is available in children. OBJECTIVE To determine the long-term clinical outcomes and predictive factors for IPBI response in children with gastroparesis refractory to medical therapy. DESIGN Retrospective review. SETTING Single tertiary care center. PATIENTS Children with refractory gastroparesis symptoms undergoing IPBIs. INTERVENTIONS IPBIs. MAIN OUTCOME MEASUREMENTS Clinical improvement and predictive factors for response. RESULTS A total of 70 injections were given to 47 patients (mean age 9.98 ± 6.5 years; 23 female patients) with follow-up in 45 patients. IPBI failed in 15 patients and was successful in 30 patients. The median duration of response to the first IPBI was 3.0 months (95% CI, 1.2-4.8). A total of 29 patients received a single IPBI, and 18 received multiple IPBIs. Older age and vomiting predicted response to initial IPBI, and male sex predicted response to repeat IPBI. Only 1 patient reported exacerbation of vomiting after IPBI resolving within a week. LIMITATIONS The open-label and retrospective nature of the study. CONCLUSION IPBI is safe and may be effective in the management of children with symptoms of gastroparesis. Subgroups identifying who responded to the first IPBI include older patients and those presenting with vomiting, whereas male patients responded better to repeat IPBIs.

Longitudinal and radial characteristics of intra-anal pressures in children using 3D high-definition anorectal manometry: new observations.

OBJECTIVES:The pathophysiology of fecal incontinence is not well understood. Standard or high-resolution anorectal manometry (ARM) provides simple two-dimensional (2D) intra-anal pressure measurements and do not identify radial asymmetry or localize abnormal sphincter function. 3D high-definition ARM (HDARM) has 256 pressure sensors distributed circumferentially and provides a detailed topographical and 3D pressure gradient representation of the anal canal. The objective of this study was to use HDARM to characterize intra-anal pressure profiles in children during rest and squeeze.METHODS:HDARM manometric tracings of 30 children with constipation referred for ARM were reviewed. 2D pressure profiles using high-resolution manometry were used to measure the length of the high-pressure zone (HPZ). The HPZ was divided into four equal segments from the anal verge to adjust for the variable sphincter length. Longitudinal and radial measurements of the HPZ during rest and squeeze (anterior, left, posterior, right quadrants of the HPZ) were taken along each segment in 2D and 3D topographical views. A 3D reconstruction combining all patients was then constructed.RESULTS:Mean age was 149.3±1.8 months and mean HPZ length was 3.0±0.1 cm. Using 2D manometry, the mean peak HPZ pressure at rest was 72.0±2.5 mm Hg, and was located in the second segment of the HPZ. The mean peak HPZ pressure at squeeze was 202.9±13.1, and was located in the second segment. 3D measurement demonstrated both longitudinal and radial asymmetry along the anterior, left, posterior, and right quadrants of the HPZ. Left and right quadrant pressures were higher than anterior and posterior pressures at the anal verge and segment 1 during rest and squeeze. Anterior pressures were lower than posterior pressures longitudinally and radially in segments 2, 3, and 4 both during rest and squeeze. Our findings also suggest that in pediatrics it may be necessary to adjust pressure measurement to the anal canal length to get a more accurate picture.CONCLUSIONS:3D HDARM allows for a detailed characterization of intra-anal pressures. 3D topographic pressure measurements demonstrate longitudinal and radial asymmetry of the anal canal at rest and during squeeze. This is the first time longitudinal and radial asymmetry of the anal canal has been described in children. 3D HDARM may allow for a better understanding of the mechanisms of fecal continence in children.

An ANMS-NASPGHAN consensus document on anorectal and colonic manometry in children.

Over the last few years, the study of the colon and anorectal function has experienced great technical advances that have facilitated the performance of the tests and have allowed a more detailed characterization of reflexes and motor patterns. As a result, we have achieved a much better understanding of the pathophysiology of children with defecation problems. Anorectal and colonic manometry are now commonly used in all major pediatric referral centers as diagnostic tools and to guide the management of children with intractable constipation and fecal incontinence, particularly when a surgical intervention is being considered.

Internal anal sphincter relaxation associated with bisacodyl-induced colonic high amplitude propagating contractions in children with constipation: a colo-anal reflex?

Background  Describe the association of internal anal sphincter (IAS) relaxation with colonic high‐ amplitude peristaltic contractions (HAPCs).

Pediatric rumination subtypes: A study using high-resolution esophageal manometry with impedance.

The differential diagnosis of intractable reflux in children includes rumination syndrome, but confirming the diagnosis using antroduodenal manometry is invasive, is costly, and requires anesthesia. High‐resolution esophageal manometry with impedance (HRM‐MII) overcomes these limitations, and the goal of this study is to validate the use of HRM‐MII as a diagnostic tool for rumination and to describe the subtypes of pediatric rumination.

Prospective evaluation of same day versus next day colon manometry results in children with medical refractory constipation

We evaluated the change in colon manometry (CM) parameters and interpretation comparing results when the study is performed the same day after the motility catheter is placed under anesthesia or the following day.

Use of Clostridium botulinum toxin in gastrointestinal motility disorders in children.

More than a century has elapsed since the identification of Clostridia neurotoxins as the cause of paralytic diseases. Clostridium botulinum is a heterogeneous group of Gram-positive, rod-shaped, spore-forming, obligate anaerobic bacteria that produce a potent neurotoxin. Eight different Clostridium botulinum neurotoxins have been described (A-H) and 5 of those cause disease in humans. These toxins cause paralysis by blocking the presynaptic release of acetylcholine at the neuromuscular junction. Advantage can be taken of this blockade to alleviate muscle spams due to excessive neural activity of central origin or to weaken a muscle for treatment purposes. In therapeutic applications, minute quantities of botulinum neurotoxin type A are injected directly into selected muscles. The Food and Drug Administration first approved botulinum toxin (BT) type A in 1989 for the treatment of strabismus and blepharospasm associated with dystonia in patients 12 years of age or older. Ever since, therapeutic applications of BT have expanded to other systems, including the gastrointestinal tract. Although only a single fatality has been reported to our knowledge with use of BT for gastroenterological conditions, there are significant complications ranging from minor pain, rash and allergic reactions to pneumothorax, bowel perforation and significant paralysis of tissues surrounding the injection (including vocal cord paralysis and dysphagia). This editorial describes the clinical experience and evidence for the use BT in gastrointestinal motility disorders in children.