Lasting impact on children with an anorectal malformations with proper surgical preparation, respect for anatomic principles, and precise surgical management.

Optimal outcomes in the management of children with Anorectal Malformation (ARM) require careful surgical preparation and detailed understanding of the anatomic principles and operative setup. A clear understanding of operative anatomy and surgical principals guides decision making. Adherence to the principles of ARM repair, as well as the application of operative and imaging adjuncts, will yield the safest and most successful approach to ARM. In this review, we detail the surgical preparation, anatomic principles, and surgical management issues unique to ARM.

Management and early outcomes of children with appendicitis in the UK and Ireland during the COVID-19 pandemic: a survey of surgeons and observational study.

OBJECTIVES: Acute appendicitis is the most common surgical condition in children. In the UK, appendicectomy is the most common treatment with non-operative management unusual. Due to concerns about the risk of SARS-CoV-2 transmission during surgical procedures, surgeons were advised to consider non-operative treatment and avoid laparoscopy where possible. This study aims to report management and outcomes, to date, of children with appendicitis in the UK and Ireland during the COVID-19 pandemic. DESIGN: Survey of consultant surgeons who treat children with appendicitis that informed a prospective multicentre observational cohort study. SETTING: Data were collected from centres in the UK and Ireland for cases admitted between 1 April and 31 May 2020 (first 2 months of the COVID-19 pandemic) at both general surgical and specialist paediatric surgical centres. PARTICIPANTS: The study cohort includes 838 children with a clinical and/or radiological diagnosis of acute appendicitis of which 527 (63%) were male. MAIN OUTCOMES MEASURED: Primary outcome was treatment strategy used for acute appendicitis. Other outcomes reported include change in treatment strategy over time, use of diagnostic imaging and important patient outcomes to 30 days following hospital admission. RESULTS: From very early in the pandemic surgeons experienced a change in their management of children with appendicitis and almost all surgeons who responded to the survey anticipated further changes during the pandemic. Overall, 326/838 (39%) were initially treated non-operatively of whom 81/326 (25%) proceeded to appendicectomy within the initial hospital admission. Of cases treated initially surgically 243/512 (48%) were performed laparoscopically. Diagnostic imaging was used in 445/838 (53%) children. Cases treated non-operatively had a shorter hospital stay than those treated surgically but hospital readmissions within 30 days were similar between groups. In cases treated surgically the negative appendicectomy rate was 4.5%. There was a trend towards increased use of surgical treatment and from open to laparoscopic appendicectomy as the pandemic progressed. CONCLUSION: Non-operative treatment of appendicitis has been widely used for the first time in children in the UK and Ireland and is safe and effective in selected patients. Overall patient outcomes do not appear to have been adversely impacted by change in management during the pandemic thus far.

Improving outcomes in pilonidal sinus disease.

BACKGROUND: Optimal surgical treatment for pilonidal sinus disease remains controversial. Studies in children are uncommon with overall high postoperative recurrence and infection rates (20%). Our unit adopted a technique for excision of the pilonidal sinus with primary midline closure using a full-thickness flap. We present our results of this technique in the pediatric age group. METHODS: Single-center retrospective review and telephone follow-up was performed, including all children undergoing pilonidal sinus excision from 2005 to 2014 using the same operative technique and preoperative/postoperative care. RESULTS: 19 children were identified. Median age was 15years, and 47% were male. All had previously infected sinuses, with 5 requiring abscess drainage. Median follow-up was 13months (3.5-67months). Histology confirmed pilonidal sinus in 18/19 cases (one developmental malformation excluded from subsequent analysis). Recurrence requiring further surgery occurred in 4/18 (22%) patients at a median of 9months (range 8-36), wound dehiscence in 8/18 (44%), and wound infection in 5/18 (28%) cases. Laser hair removal of the natal cleft was performed in 7/18 (39%) patients with no further recurrence. CONCLUSION: This is the first report of this technique in children which showed a higher incidence of complications than a comparable adult study. Our study underlines the importance of accurate long-term outcomes when introducing new techniques to evaluate operation-associated morbidity. Our preliminary results of laser hair removal are promising but need to be evaluated with a prospective study.

Slow-transit constipation with concurrent upper gastrointestinal dysmotility and its response to transcutaneous electrical stimulation.

PURPOSE: Transcutaneous electrical stimulation (TES) speeds up colonic transit in children with slow-transit constipation (STC). This study examined if concurrent upper gastrointestinal dysmotility (UGD) affected response to TES. METHODS: Radio-nuclear transit studies (NTS) were performed before and after TES treatment of STC as part of a larger randomised controlled trial. UGD was defined as delayed gastric emptying and/or slow small bowel transit. Improvement was defined as increase of ≥1 Geometric Centre (median radiotracer position at each time [small bowel = 1, toilet = 6]). RESULTS: Forty-six subjects completed the trial, 34 had NTS after stimulation (21 M, 8-17 years, mean 11.3 years; symptoms >9 years). Active stimulation increased transit in >50% versus only 25% with sham (p = 0.04). Seventeen children also had UGD. In children with STC and either normal upper GI motility (NUGM) and UGD, NTS improved slightly after 1 month (57 vs. 60%; p = 0.9) and more after 2 months (88 vs. 40%; p = 0.07). However, mean transit rate significantly increased with NUGM, but not UGD (5.0 ± 0.2: 3.6 ± 0.6, p < 0.01). CONCLUSION: Transcutaneous electrical stimulation was beneficial for STC, with response weakly associated with UGD. As measured by NTS, STC children with NUGM responded slightly more, but with significantly greater increased transit compared to those with UGD. Higher numbers are needed to determine if the difference is important.

What is new in radiology and pathology of motility disorders in children?

Disorders affecting colorectal motility lead to significant morbidity in children with surgical conditions. Etiology is frequently unknown, which in turn makes treatment empiric and compromises outcome. A thorough understanding of the normal mechanisms of control and the ability to recognize and manage defects is an important goal for clinicians. This article reviews recent advances made in the investigation of children with colorectal motility disorders, including the role of transit studies (marker studies and scintigraphy), options for assessing anatomy (ultrasound, contrast enema, and sectional imaging) and the use of manometry, both anorectal and colonic. Current concepts in microscopic evaluation are outlined.

Gastrointestinal transit in children with chronic idiopathic constipation.

UNLABELLED: Chronic constipation in children is common and produces significant morbidity. Identification of the site of dysmotility in constipation may determine the cause and permit directed management. Scintigraphy differentiates constipated patients with anorectal hold-up from those with colonic slowing. Adults with colonic slowing demonstrate variation in the site of hold-up. However, in children with colonic slowing, variability in the site of hold-up has not been investigated. PURPOSE: The current study aimed to characterise colonic transit patterns in 64 children with chronic idiopathic constipation. METHODS: Scintigraphic images were grouped visually by their transit patterns. Intra-observer variation was assessed. Scintigraphic data were analysed quantitatively. RESULTS: Visual analysis of scintigraphy studies demonstrated normal transit (11/64), anorectal hold-up (7/64) and slow colonic transit (46/64). Transit characteristics in the slow transit group demonstrated three possible subgroups: pancolonic slowing (28/46), discrete hold-up in the transverse colon (10/46) and abnormal small and large bowel transit (8/46). Kappa testing demonstrated consistent characterisation (k = 0.79). Statistical analysis of scintigraphic data demonstrated highly significant differences from normal (P < 0.001) in the subgroups. CONCLUSION: Scintigraphy demonstrates three possible transit patterns in children with chronic constipation secondary to slow colonic transit.

24-Hour colonic manometry in pediatric slow transit constipation shows significant reductions in antegrade propagation.

BACKGROUND: The physiological basis of slow transit constipation (STC) in children remains poorly understood. We wished to examine pan-colonic motility in a group of children with severe chronic constipation refractory to conservative therapy. METHODS: We performed 24 h pan-colonic manometry in 18 children (13 boys, 11.6 +/- 0.9 yr, range 6.6-18.7 yr) with scintigraphically proven STC. A water-perfused, balloon tipped, 8-channel, silicone catheter with a 7.5 cm intersidehole distance was introduced through a previously formed appendicostomy. Comparison data were obtained from nasocolonic motility studies in 16 healthy young adult controls and per-appendicostomy motility studies in eight constipated children with anorectal retention and/or normal transit on scintigraphy (non-STC). RESULTS: Antegrade propagating sequences (PS) were significantly less frequent (P < 0.01) in subjects with STC (29 +/- 4 per 24 h) compared to adult (53 +/- 4 per 24 h) and non-STC (70 +/- 14 per 24 h) subjects. High amplitude propagating sequences (HAPS) were of a normal frequency in STC subjects. Retrograde propagating sequences were significantly more frequent (P < 0.05) in non-STC subjects compared to STC and adult subjects. High amplitude retrograde propagating sequences were only identified in the STC and non-STC pediatric groups. The normal increase in motility index associated with waking and ingestion of a meal was absent in STC subjects. CONCLUSIONS: Prolonged pancolonic manometry in children with STC showed significant impairment in antegrade propagating motor activity and failure to respond to normal physiological stimuli. Despite this, HAPS occurred with normal frequency. These findings suggest significant clinical differences between STC in children and adults.

Application of HapMap data to the evaluation of 8 candidate genes for pediatric slow transit constipation.

BACKGROUND: Slow transit constipation (STC) affects up to 3% of all children and is an increasingly recognized cause of chronic constipation in children. We conducted a pilot study to investigate whether genes encoding neurotransmitters (TAC1, TAC3, VIP, NOS1) and receptors (TACR1, TACR2, TACR3, KIT) could be responsible for STC. METHODS: One hundred seventeen tag single nucleotide polymorphisms (SNPs), distributed among the candidate genes, were selected from HapMap data and genotyped using Sequenom (San Diego, CA) technology in 35 affected families. Evaluation of association was performed by transmission disequilibrium test and multilocus analysis. RESULTS: Five SNPs (rs3771863, rs4580655, rs11722288, rs4563545, and rs3782221) in the TACR1, TACR3, KIT, and NOS1 genes were found to be potentially associated with STC, although the significance of these results does not withstand correction for multiple testing. CONCLUSIONS: Our data indicate that 5 SNPs in the NOS1, TACR1, TACR3, and KIT genes could be involved in STC, especially rs3771863 in intron 1 of TACR1, which showed the highest association.

The antegrade continence enema successfully treats idiopathic slow-transit constipation.

BACKGROUND: Antegrade continence enemas (ACEs) are successful for constipation and/or fecal incontinence caused by anorectal malformations or spina bifida but have been thought to be less successful in the treatment for patients with colonic dysmotility. We studied the long-term efficacy of ACE in a large group of patients with idiopathic slow-transit constipation (STC). METHODS: We identified 56 children with an appendicostomy for ACE with radiologically proven STC. An independent investigator (SKK) performed confidential telephone interviews. RESULTS: We assessed 42 of 56 children (31 boys) of mean age 13.1 years (range, 6.9-25). Mean follow-up was at 48 months (range, 3-118). Mean symptom duration before appendicostomy was 7.5 years (range, 1.4-17.4). Indications for appendicostomy were soiling (29/42), inadequate stool evacuation (7/42), and recurrent hospital admissions for nasogastric washouts (6/42). Both quality of life (Templeton quality of life [P < .0001]) and continence (modified Holschneider continence score [P < .0001]) improved with ACE. Soiling frequency decreased in 32 of 42 (11/32 completely continent). Thirty-seven of 42 children had reduced abdominal pain severity (P < .0001) and frequency (P < .0001). Complications included granulation tissue (33/42), stomal infection (18/42), and washout leakage (16/42). Fifteen of 42 children ceased using the appendicostomy (7/15 symptoms resolved). Thirty-five of 42 families felt that their aspirations had been met. CONCLUSIONS: Antegrade continence enemas were successful in 34 (81%) of 42 children with STC, contradicting views that ACEs are less effective in patients with colonic dysmotility.

Laparoscopic seromuscular colonic biopsies: a surgeon's experience.

BACKGROUND: Colonic dysmotility is a recognised cause of chronic constipation in children. Colonic dysmotility is better analysed by examination of the colonic muscle than rectal biopsy, which does not examine the defective area and has a low yield. We explored the role of laparoscopic colonic muscle biopsies to investigate children with intractable constipation. The authors describe the technique, its application, and results from a large series. METHODS: A retrospective review was conducted of all patients undergoing laparoscopic seromuscular colonic biopsies (hepatic flexure, mid-transverse colon, splenic flexure, and sigmoid colon) by a single surgeon for the investigation of chronic constipation over a 10-year period. Patient records were reviewed to determine the perforation frequency and management, postoperative recovery time and the frequency of an immunohistochemical abnormality. RESULTS: One hundred ninety-seven patients (118 boys) were investigated by laparoscopic biopsy during the period. The mean age was 8.0 +/- 4.0 years (range, 1.4-22.4). The patients took 28.7 +/- 13.6 hours (range, 8-120) to recover, with 37 (19%) having nausea and/or vomiting requiring antiemetics. Most patients (160/197, 81%) were discharged the following day. Six patients (3%) had a mucosal perforation recognised at operation (treated by an Endoloop) with no change in postoperative outcome. Two patients (1%) had an unrecognised mucosal perforation requiring laparoscopic reoperation and Endoloop closure (laparotomy/colostomy not required), with no further sequelae. Eight-six patients (44%) had a specific immunohistochemical neuropeptide anomaly (reduced substance P [84], reduced vasoactive intestinal peptide [2]). CONCLUSIONS: Laparoscopic biopsy is a valuable tool to investigate chronic constipation in children, allowing a pathological diagnosis to be made in many cases. The complications of the procedure are acceptably low with this technique.

Paediatric constipation for adult surgeons - article 2: new microscopic abnormalities and therapies.

Chronic constipation is a common condition in both adults and children. Children with chronic constipation frequently have symptoms that continue into adulthood. In the second part of the review we describe advances in the identification of abnormalities in the control of motility. The role of neurotransmitters in both paediatric and adult constipation is examined and the radical rethink of colonic dysmotility caused by the re-emergence of interstitial cells of Cajal is discussed. The recognition of chronic constipation as an heterogenous condition has led to the introduction of new therapies. Antegrade washouts through appendix stomas and an exciting new treatment with electrical interferential therapy may, in the future, result in a less invasive approach to the management of chronic constipation. An improved understanding of the assessment and management of chronic constipation in childhood is also likely to reduce the frequency and morbidity of chronic constipation in adults.

Paediatric constipation for adult surgeons--article 1: targeting the cause.

Constipation is very common in all age groups and can be resistant to standard therapies, producing chronic morbidity. Childhood onset constipation frequently produces symptoms that persist into adulthood. Recent advances have been made in the diagnosis and treatment of childhood constipation that could have application in adult practice. In the first part of the present review, the methods of differentiation of patients with a disorder of colonic motility from those with an anorectal hold-up are discussed. Slow transit constipation, with distinct features on colonic manometry and scintigraphy, has only recently been recognized in children. This diagnosis, together with a novel method of placement of a manometric catheter, is described. While the cause of slow transit constipation remains unclear, clinical features that differ between children and adults may provide an insight into the aetiology. The diagnosis of intestinal neuronal dysplasia is explained and the controversy surrounding the diagnosis outlined. We propose that the traditional histological criteria exclude many other clinically significant forms of dysplasia of the enteric nervous system and should be extended.