Initial development and validation of a fecal incontinence-specific quality of life measure.

BACKGROUND/PURPOSE: Fecal incontinence is a prevalent pediatric condition with psychosocial impacts on both children and their caregivers. We sought to develop and validate the Cincinnati Fecal Incontinence Scale (CINCY-FIS) as a psychometrically valid measure to assess the quality of life and caregiver impacts of pediatric fecal incontinence. METHODS: Items were generated through review of previous measures, expert consensus, and pilot testing with feedback from 8 families. Initial study measures were completed by 222 caregivers. Following item reduction, 18 items were subjected to confirmatory factor analysis. Convergent and criterion validity were assessed using correlation. Reliability was established using internal consistency statistics and test-retest reliability at baseline and 2-week follow-up. RESULTS: A five factor first-order structure with two higher-order factors demonstrated acceptable fit to the data, was consistent with a priori hypotheses, and was more parsimonious than the alternative model. Convergent validity and criterion-related validity were established for all of the CINCY-FIS scales. Reliability was high and consistent across both measurement occasions. CONCLUSIONS: The CINCY-FIS is a reliable and valid assessment of pediatric fecal incontinence-specific quality of life and parenting stress. The score is highly sensitive to patient changes making it suitable for both clinical and research purposes. TYPE OF STUDY: Prospective observational. LEVEL OF EVIDENCE: Study of Diagnostic Test Level II.

Spontaneous Rupture of Splenic Hemangioma in a Neonate.

Spleen vascular tumors such as hemangiomas, albeit rare, can present during neonatal period with unexplained circulatory shock. We present a case of a newborn with refractory hypovolemic shock and acute abdomen that underwent emergency splenectomy due to spontaneous rupture of a splenic hemangioma.

Surgical outcomes, bowel habits and quality of life in young patients after ileoanal anastomosis for ulcerative colitis.

PURPOSE: We aim to investigate the postoperative outcomes, bowel habits and quality of life (QoL) of younger pediatric ulcerative colitis (UC) patients following surgical intervention compared to an older pediatric population. METHODS: Medical records of UC patients after colectomy with ileoanal reconstruction (2002-2013) at our institution were reviewed. Patients/parents completed a QoL, bowel habits and disease course questionnaire. Surgical outcomes, bowel habits and QoL were reported comparing the younger (≤11years old, n=26) to older (>11years old, n=38) cohorts. RESULTS: The mean age at colectomy was 7.04±0.63years vs 14.71±0.32years in the two groups. Patients had a significant (P<0.001) reduction in stooling frequency after surgery in both age groups and had favorable rates of fecal continence. The frequency of pouchitis and postoperative small bowel obstruction was similar in both cohorts. Dehydration was slightly increased in the younger population but not significant. Anastomotic leak and stricture rates were slightly reduced in younger patients. Postoperative QoL was favorable and similar regardless of age at surgery. CONCLUSIONS: Colectomy with ileoanal anastomosis for young children (≤11years old) with UC is without increased complications relative to older patients and maintains a postoperative QoL and stool patterns.

The association of the severity of anorectal malformations and intestinal malrotation.

INTRODUCTION: Intestinal malrotation is a known association of anorectal malformations (ARM). Exact incidence, prognosis and surgical implications related to ARM are unknown. The aim of this study was to identify relevant associations between ARM and the presence of malrotation. METHODS: Records of patients from two referral centers were retrospectively analyzed looking for malrotation associated to ARM and its management, as well as factors for functional prognosis. RESULTS: 40 patients out of 2572 with ARM (1.6%) were found to have malrotation. Females were more commonly affected, and severe malformations were more frequent (cloaca, covered cloacal exstrophy in females and rectoprostatic and rectobladder neck fistula in males). Factors significantly associated with malrotation included Müllerian or Wolffian duct anomalies (P<0.05), while fecal continence status, presence of constipation, and use of laxatives or enemas were not. Detecting and correcting malrotation early on or at the time of colostomy creation represented a protective factor against additional surgeries for bowel obstruction and volvulus (P<0.001). Removal of the appendix during malrotation treatment required constructing a neoappendicostomy using a cecal flap in 9 out of 14 patients needing antegrade enema administration. CONCLUSIONS: Malrotation presence in patients with ARM has the same frequency as in the general population, but it is more common in severe malformations. Surgeons treating these patients should address the malrotation at the time of colostomy opening if detected. The appendix should be preserved for potential future use as an appendicostomy for antegrade administration of enemas.

Health-Related Quality of Life and Parental Stress in Children With Fecal Incontinence: A Normative Comparison.

OBJECTIVE: The aim of the present study was to describe the quality of life and parenting stress associated with a child with fecal incontinence (FI). METHODS: Female caregivers (n = 170) of children of 3 to 12 years age with FI completed a broad and general measure of quality of life and a measure of parenting stress. Results were compared with proxy reports for a normative sample of healthy children. RESULTS: Caregivers of children with FI reported significantly impaired quality of life for their children and increased parenting stress in all of the respective domains relative to healthy controls. Impairments reported by caregivers were large in magnitude. Similarly, rates of parenting stress were at or greater than the 98th percentile for caregivers of children with FI. CONCLUSIONS: Children with fecal incontinence and their families are in need of interventions targeting their quality of life and the stress associated with caregiving. FI appears to be particularly stressful for caregivers who may be in need of support beyond medical management of their child's bowel. Moreover, additional refinements in disease-specific quality of life assessment are needed in this population. Such refinement would allow for more precise measurement of the quality of life processes that are unique to FI.

Enterolito de sulfato de bario en una paciente con malformación anorrectal. Notas sobre el colostograma distal apropiado / Barium sulfate enterolith in a patient with anorectal malformation. Remarks on the appropriate distal colostogram

Resumen: Presentación del caso: Paciente femenino de 2 años de edad con malformación Anorrectal y fístula rectovestibular a quién se realizó colostograma distal con sulfato de bario provocando concreción lítica de bario en el fondo de saco del colon distal al no enjuagar el contenido. Ante la imposibilidad de retirar el enterolito a través del estoma disfuncional de la derivación intestinal decidimos realizar la extracción al momento de hacer la anorrectoplastía sagital posterior con riesgo aumentado de infección, dehiscencia de herida y lesión de estructuras adyacentes. Enfatizamos la necesidad de recordar a la comunidad médica radiológica y pediátrica de la adecuada realización de este tipo de estudios siempre con medio de contraste hidrosoluble, y aprovechamos para recapitular la técnica de realización del mismo.

Abstract: Case presentation: 2 year-old female patient with anorectal malformation with recto-vestibular fistula to whom a distal colostogram with barium sulfate was done, thus provoking lithic concretion in the distal sac. Since it was deemed impossible to extract the enterolith trough de distal stoma we decided to perform the anorectoplasty and extract the lit at that moment knowing the increased surgical risks such as infection, dehiscence and damage of adjacent structures. We stress the need to remind the radiological and pediatric medical communities to always perform this kind of imaging studies with water-soluble contrast agents and we also annotate the technique for doing so.

Laparoscopic approach in the management of anorectal malformations.

Seventeen years have passed since the first description of the laparoscopic approach for anorectal malformation and approximately 68 articles have been published on the subject. In this review article, we aim to describe the advantages as well as the indications and contraindications of this approach when dealing with each specific type of anorectal malformation, according to what has been described in the literature and to our own experience. The ideal and undisputable indication for laparoscopy remains for cases in which the abdomen needs to be entered to repair the malformation. Only 10% of male patients with anorectal malformation are born with a recto-bladder neck fistula that requires an abdominal approach, this represents an ideal indication for laparoscopy. In females, only the complex cloacae with a common channel length greater than 3 cm are the ones that require a laparotomy; they represent about 30% of the cloacae. However, the repair of this type of cloacae also requires sophisticated and technically demanding maneuvers that have never been done laparoscopically. In cases of recto-urethral prostatic fistulas the malformation can be repaired either way: laparoscopically or posterior sagitally. In all other malformations: recto-perineal fistula, recto-urethral bulbar fistula, anorectal malformation without fistula, rectal atresia, recto-vestibular fistula; no justification for laparoscopy could be found; and in some cases, laparoscopy is contraindicated. In the published reports, there is no evidence supporting the idea that laparoscopic repair results in better functional results when compared with non-laparoscopic operation; there is a tendency to omit information relevant to bowel control such as the characteristics of the sacrum and the presence or absence of tethered cord; and most authors do not compare results between comparable malformations.