Management of chronic intractable constipation in children.

BACKGROUND: Chronic intractable constipation (CIC) is a debilitating disease that is challenging to manage. Treatment options in children include medications, enemas, and surgical management in selected cases. METHOD: We reviewed medical records of pediatric patients diagnosed as having CIC at Tufts Medical Center from 2005 to 2012. Demographic variables, diagnostic procedures, and medical and surgical outcomes were collected. Clinical outcome was defined using the Rome III criteria. RESULTS: A total of 14 patients were included in the study (10 boys). The age range was 10 to 21 years. All of the patients had the diagnosis of CIC. Eleven patients had cecostomy placement. During the follow-up period, 10 patients underwent total abdominal colectomy with ileorectal anastomosis, 1 had total colectomy with ileostomy, and 1 had partial colectomy with colorectal anastomosis. Successful clinical outcome was reported in 7 patients with 3 patients reporting persistent fecal incontinence. Colonic motility studies were performed on 12 patients (colonic neuropathy in 11 patients and normal study in 1 patient). Defecography was consistent with isolated pelvic floor dysfunction in 1 patient, abnormal motility and anatomy in 1 patient, pelvic floor dysfunction and abnormal motility in 2 patients, and found abnormal motility only in 5. Defecography study was normal in 5 patients. All of the patients with abnormal colonic manometry underwent a surgical procedure. CONCLUSIONS: Anorectal manometry, colonic manometry, and defecography help in understanding the pathophysiology of defecation disorders in children. The majority of patients with abnormal colonic manometry underwent TAC-IRA. There was no statistical correlation between individual investigations (anorectal manometry, colonic manometry, and defecography) with surgical intervention (P > 0.35). TAC-IRA may be safe and useful intervention in a subset of patients when other treatment options have failed.

Phenylephrine as a simulated intravascular epidural test dose in pediatrics: a pilot study.

BACKGROUND: A test dose is used to detect intravascular injection during neuraxial block in pediatrics. Accidental intravascular epidural local anesthetic injection might be unrecognized in anesthetized children leading to potential life-threatening complications. In children, sevoflurane anesthesia blunts the hemodynamic response when intravascular cathecolamines are administered. No studies have explored the hemodynamics and the criteria for a positive test dose result following phenylephrine in sevoflurane anesthetized children. METHODS: Healthy children undergoing minor procedures were randomly assigned to receive intravenous placebo, or 5 µg∙kg(-1) phenylephrine (n = 11/group) during sevoflurane anesthesia. Hemodynamic response was assessed using electrocardiography, pulse oxymetry and non-invasive blood pressure monitoring for 5 min following drug administration in anesthetized patients. RESULTS: All patients receiving phenylephrine showed a decreased heart rate (HR) but not all of them met the positive criterion for test dose response. Overall, at 1 min, patients receiving phenylephrine showed a 25% decrease in HR from the baseline while an increase in blood pressure was noticed in 54% of patients receiving phenylephrine. DISCUSSION: Phenylephrine might be a future indicator of positive intravascular test dose. Further investigation is needed to find out the phenylephrine dose that elicits a reliable hemodynamic response and whether phenylephrine needs to be dose age-adjusted in order to appreciate relevant hemodynamic changes in children receiving neuraxial blocks undergoing general anesthesia.

Colonic hyperactivity results in frequent fecal soiling in a subset of children after surgery for Hirschsprung disease.

BACKGROUND AND AIM: Fecal soiling is a challenging problem in some children after pull-through surgery for Hirschsprung disease (HSCR). The prevailing perception is that soiling results from overflow incontinence; however, its treatment with laxatives yields mixed results. Colonic manometry studies are reported to be normal in most patients in this population. The interpretation of these findings does not support the physiology of fecal overflow incontinence in these children. The aim of the present study was to define the physiology underlying daily, frequent fecal soiling in children after surgery for HSCR using manometric techniques. PATIENTS AND METHODS: Four pediatric motility centers in the United States participated in the study; medical records and manometric tracings (anorectal and colonic) of children (n = 59; 6.5 years; 48 boys) who had pull-through surgery for HSCR and presented with daily, frequent fecal soiling were examined. Children referred for evaluation of constipation who had normal colonic manometry served as controls (n = 25; 6.7 years; 12 boys). The patients with HSCR were divided into 2 groups (Hirschsprung disease groups 1 and 2 [HD1, HD2]) based on the absence or presence of high-amplitude propagated contractions (HAPCs). A control group that included children with chronic constipation was also studied. We compared the mean HAPC frequency between the HD2 and control groups. RESULTS: HD1 included 21 patients who had no HAPCs in fasting or postprandial periods. HD2 included 38 patients who had an average of 0.07 HPACs/min while fasting and 0.13/min in the postprandial state. In this subset the number of HAPCs in the fasting state (P = 0.04) and the postprandial state (P < 0.001) was greater when compared with controls. Additionally, there was a significant increase in HAPCs/min from the fasting to the postprandial state (P = 0.01). In the HD2 group 40% had colonic hyperactivity. CONCLUSIONS: Daily, frequent fecal soiling after pull-through surgery for HSCR may be due to colonic hyperactivity in some children. It is imperative that this unique subset be identified because the management strategy would include avoidance of laxatives, contrary to standard current practice.

Long-term outcome of congenital intestinal pseudoobstruction.

We evaluated 85 children with congenital chronic intestinal pseudoobstruction (CIP) over the past 10 years. Twelve (14%) were born prematurely. One had a family history of CIP. Six had systemic diseases. Thirty-five (41%) had urinary bladder involvement. Manometric features were consistent with myopathy in 32, neuropathy in 48, and mixed disease in 5. Of 48 patients with neuropathy, 6 had urinary bladder involvement (12.5%) (P < 0.0001 vs myopathy), and 10 had malrotation (21%) (P = NS vs myopathy). Upon referral, 53 (62%) were dependent on partial or total parenteral nutrition (PN). At the time of chart review (median 25 months after evaluation), 22 patients had died, 14 of whom were on total PN, 13 of them died because of PN-related complications and 1 died of sepsis. Three others died of sepsis while on partial PN (P = 0.007 vs mortality in patients fed enterally) and five died after small bowel transplantation. In conclusion, in children with congenital CIP, the risk for prematurity is increased twofold, the majority of cases are sporadic, abnormal bladder function is more common in myopathic CIP, and complications related to parenteral nutrition are the main cause of death in children with CIP.

Management of intractable constipation with antegrade enemas in neurologically intact children.

OBJECTIVES: To assess the benefit of antegrade enemas in children with severe constipation who were referred to a tertiary care center. METHODS: From 1997 to 1999, 12 children (9 male, aged 8.7 +/- 4.4 years) underwent cecostomy placement. All children were neurologically normal and had been extensively examined to rule out organic causes of constipation. Follow-up included a questionnaire to interview caregivers 13.1 +/- 8.5 months after cecostomy placement. RESULTS: For all children, antegrade enemas led to improvement in the number of bowel movements / week (7.1 versus 1.4, P < 0.005), number of soiling accidents / week (1.0 versus 4.7, P < 0.01), abdominal pain score (0.9 versus 2.9, P < 0.005), emotional health score (3.6 versus 1.9, P < 0.005), overall health score (3.6 versus 1.7, P < 0.005), number of medications used for constipation (0.8 versus 4.0, P < 0.005), number of missed school days / month (1.5 versus 7.5, P < 0.02), and number of physician office visits / year (9.2 versus 24.0, P < 0.05). Irrigation solutions used for the antegrade enemas included polyethylene glycol (67%), saline and glycerin solution (25%), and phosphate enema (8%), administered everyday in seven children and every other day in five children. Adverse events included skin breakdown and granulation tissue in one patient, leakage of irrigation solution in one patient, and dislodging of the tube in two patients. Five patients discontinued the use of antegrade enemas within a mean of 14.6 +/- 9.1 months after beginning treatment. CONCLUSION: Antegrade enemas through a cecostomy are a safe and satisfactory option for children who are neurologically intact and who have severe constipation that does not respond to medical treatment.