Jonathan Sutcliffe

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.

Substance P and vasoactive intestinal peptide are reduced in right transverse colon in pediatric slow‐transit constipation

Background  Slow‐transit constipation (STC) is recognized in children but the etiology is unknown. Abnormalities in substance P (SP), vasoactive intestinal peptide (VIP) and nitric oxide (NO) have been implicated. The density of nerve fibers in circular muscle containing these transmitters was examined in colon from children with STC and compared to other pediatric and adult samples.

Slow-transit constipation in children: our experience

Constipation is a common problem in children, with childhood prevalence estimated at between 1 and 30%. It accounts for a significant percentage of referrals to paediatricians and paediatric gastroenterologists. It commonly runs in families, suggesting either an underlying genetic predisposition or common environmental factors, such as dietary exposure. The peak age for presentation of constipation is shortly after toilet training, when passage of hard stools can cause pain on defecation, which then triggers holding-on behaviour in the child. At the time of the next call to stool the toddler may try to prevent defecation by contraction of the pelvic floor muscles and anal sphincter. Unless the holding-on behaviour is quickly corrected by interventions to soften faeces and prevent further pain, the constipation can very rapidly become severe and chronic. Until recently, this mechanism was thought to be the only significant primary cause of constipation in childhood. In this review, we will summarise recent evidence to suggest that severe chronic constipation in children may also be due to slowed colonic transit.

Hirschsprung disease and anorectal malformation

Hirschsprung disease and Anorectal Malformations are congenital disorders presenting in neonates with distal intestinal obstruction. Hirschsprung disease is associated with a functional distal bowel obstruction resulting from the abnormal development of the enteric nervous system and ensuing aganglionosis of the distal gut. Anorectal Malformations comprise a spectrum of anatomical anomalies causing a mechanical bowel obstruction. Both conditions are frequently associated with congenital abnormalities/syndromes, which require careful assessment and evaluation. Surgical intervention is usually required for both conditions with careful preparation and meticulous technique. Long-term follow-up allows early identification and treatment of potentially debilitating symptoms, which include faecal incontinence.

Decrease in nerve fibre density in human sigmoid colon circular muscle occurs with growth but not aging.

Background  Studies in animals suggest that enteric neurons decrease in density or number with increasing age. Neurons containing nitric oxide (NO), vasoactive intestinal peptide (VIP) and Substance P (SP) have been implicated. In human large intestine, NO‐utilizing neurons decrease during childhood or early adulthood but it is not known if the innervation of the muscle changes. This study examined the density of nerve fibres containing these transmitters in sigmoid colon circular muscle from children and adults.

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.

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.

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.

Reduced distribution of pacemaking cells in dilated colon.

Interstitial cells of Cajal (ICC) act as pacemaker in gastrointestinal smooth muscle. In animals, small bowel dilatation produces a reduction in ICC numbers and in pacemaker function. With resolution of dilatation, ICC numbers and pacemaking function are partially restored. In human colonic disease states, dilatation is associated with dysmotility. The effect of dilatation on ICC distribution has not previously been examined in the human colon. Tissues from a neonate with colonic atresia and a 17-year-old adolescent with acquired megasigmoid were fixed, sectioned and incubated with anti cKit antibodies followed by fluorescent secondary antibodies. Distended and non-distended segments of colon were examined for ICC distribution using immunohistochemistry to c-Kit. Images were obtained with confocal microscopy. In both patients, there was a marked reduction in cKit-immunoreactive cells in the circular muscle and the myenteric plexus of the distended colon compared to the distal non-distended colon. Dilatation of the human colon is associated with a marked reduction in ICC. A resulting loss of pacemaker function could contribute to dysmotility associated with distension. Further studies assessing pacemaking function in human subjects and investigating reversibility of ICC disruption may allow new therapeutic strategies.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anomaly (aOR 5.17, 95% CI 1.9–14.1), abdominal wall erythema or discolouration at presentation (aOR 2.51, 95% CI 1.23–5.1), diagnosis of single intestinal perforation at laparotomy (aOR 3.1 95% CI 1.05–9.3), and necessity to perform a clip and drop procedure (aOR 30, 95% CI 3.9–237) were associated with increased 28-day mortality. These results can be used for national benchmarking and counselling of parents.