Improvements in Incontinence with Self-Management in Patients with Anorectal Malformations.

UNLABELLED: There are limited data available in children with anorectal malformation (ARM) regarding the use of transanal colonic irrigation delivered with the Peristeen system (Coloplast Denmark A/S, Humlebaek, Denmark). To our knowledge no study has combined the element of controlled evacuation with self-management strategies. Our center began offering this management regimen 5 years ago to patients suffering from fecal incontinence. The aim of this study was to appraise the results of this approach in children from 4 to 18 years with incontinence and fecal soiling secondary to ARM. MATERIAL AND METHODS: Bowel management was initiated with the help of hydrosonography to evaluate bowel motility and the volume of the enema. The Peristeen irrigation system was used. Anorectal irrigation was repeated every 24, 48, or 72 hours. A personal schedule was developed based on every patient's individual preferences. Irrigations were self-administered by the patient while sitting on the toilet. Patients were controlled for soiling, time needed for irrigation, time interval between irrigations 6 and 12 months after start of therapy, with further yearly follow-ups. RESULTS: A total of 40 patients aged between 4 and 18 years were evaluated. After 12 months of therapy, 32 patients were free of symptoms of soiling. Six patients were soiling occasionally. Two patients did not follow the therapeutic regime. The average time needed for irrigation was 35 minutes, with the lower limit of 12 minutes and the upper limit of 60 minutes, irrigations where done every 24 hours in 12 patients. Overall 25 patients irrigated twice every 48 and 72 hours to achieve a constant 7-day rhythm. One patient irrigated every 5 days. At follow-up after 2, 3, and 4 years success rates were stable. CONCLUSION: We suggest that colonic irrigation should be combined with self-management strategies in children with ARM. Adherence to therapy can be enhanced with the use of an individualized irrigation schedule. The amount of time required for the irrigation can be significantly reduced. Therefore, when establishing colonic irrigation in children and adolescents a focus should be placed on time-saving measures and self-regulation.

[Treatment for fecal incontinence in patients with anorectal malformations. Introduction of a therapeutic approach]. / Therapie der Inkontinenz am Beispiel anorektaler Fehlbildungen Vorstellung eines Behandlungskonzepts.

UNLABELLED: Fecal incontinence is a serious problem that may lead to social segregation and psychological problems. Patients with anorectal malformations frequently suffer fecal incontinence even with an excellent anatomic repair. In these patients an effective management program with enemas can improve their quality of life. We want to present our experience with bowel management and anorectal irrigation as treatment for stool incontinence. MATERIAL AND METHODS: Patients who presented with soiling regardless of the type of anomaly were included in the study. The diagnostic program comprised a careful clinical history, physical examination, exact classification of the malformation and stool protocol. All patients suffering from true fecal incontinence were included in a bowel management program. These patients received oral polyethylenglykol to evacuate stool impaction. Than anorectal irrigation was initiated and repeated every 24 or 48 hours. Patients were controlled for soiling, time needed for irrigation and time interval between irrigations 6 and 12 months after start oftherapy. RESULTS: 40 patients aged 4 to 54 with a mean age of 15,95 years were evaluated. 12 months after start of therapy 32 patients were free of symptoms of soiling. 6 patients were soiling occasionally once or twice per week. 2 patients did not follow the therapeutic regime and therefore did not show an improved condition concerning soiling in the long run. The average time needed for irrigation was 45 minutes, irrigations where done every 24 hours in 12 patients. 25 patients irrigated twice every 48 and 72 hours to achieve a constant seven day rhythm. One patient irrigated every five days. DISCUSSION: Patients born with anorectal malformation and suffering from stool incontinece can be kept clean of stool if they are subjected to an adequate treatment.

Regional deep hyperthermia for salvage treatment of children and adolescents with refractory or recurrent non-testicular malignant germ-cell tumours: an open-label, non-randomised, single-institution, phase 2 study.

BACKGROUND: Although the survival of children and adolescents with malignant germ-cell tumours has improved greatly in recent years, the outcome remains poor for those with refractory or recurrent malignant germ-cell tumours. We aimed to determine whether objective tumour response could be achieved in patients with refractory or recurrent malignant germ-cell tumours with PEI-regional deep hyperthermia as salvage treatment. METHODS: Patients with refractory or recurrent non-testicular malignant germ-cell tumours after standard cisplatin-based chemotherapy were treated prospectively with PEI chemotherapy (cisplatin 40 mg/m(2), delivered intravenously on days 1 and 4; etoposide 100 mg/m(2), intravenously on days 1-4; and ifosfamide 1800 mg/m(2), intravenously on days 1-4) plus simultaneous 1-h regional deep hyperthermia (41-43°C) on days 1 and 4. Patients received three to four treatment courses at 21-day intervals until residual tumour resection was possible; they subsequently received one or two additional courses of PEI-regional deep hyperthermia. Local radiotherapy was given for incompletely resected tumours. Chemotherapy and hyperthermia toxic effects were assessed using WHO grading. The primary endpoint was the proportion of patients who had an objective response as assessed with Response Evaluation Criteria in Solid Tumors version 1.0 guidelines. Secondary endpoints were the event-free survival and overall survival after 5 years. This ongoing PEI-regional deep hyperthermia study (Hyper-PEI protocol) is registered at the German Cancer Society, number 50-2732. FINDINGS: 44 patients aged 7 months to 21 years (median 2 years 7 months) with refractory or recurrent malignant germ-cell tumours (nine patients with poor response, 23 patients with first relapse, 12 patients with multiple relapses) were included in this study. We identified 34 yolk sac tumours, eight embryonal carcinomas, one choriocarcinoma, and one dysgerminoma by histology analysis. Of the 35 patients who had sufficient clinical and radiographical data available for response assessment, 30 (86%) had an objective response to treatment (16 patients had complete remission and 14 had partial remission). 5-year event-free survival was 62% (95% CI 45-75), and 5-year overall survival was 72% (95% CI 55-83). The median follow-up of surviving patients was 82 months (range 9-195). WHO grade 3-4 neutropenia and thrombocytopenia occurred in all 181 chemotherapy cycles. Granulocytopenic fever, which required intercurrent hospital admission, was noted in 29 (66%) of 44 patients after 53 (29%) of 181 courses. Five patients experienced treatment-related grade-3 acute renal toxic effects. INTERPRETATION: A multimodal strategy integrating PEI-regional deep hyperthermia and tumour resection with or without radiation can successfully treat children and adolescents with refractory or recurrent malignant non-testicular germ-cell tumours. The long-term prognosis of patients with poor response or after first relapse was almost similar to those receiving first-line treatment. This strategy merits further investigation. FUNDING: Deutsche Krebshilfe eV, Bonn, Elterninitiative Kinderkrebsklinik Düsseldorf eV, the Barbara and Hubertus-Trettnerstiftung, and the Marie Quendt Fund.

Hirschsprung disease in the older child: diagnostic strategies.

BACKGROUND: Hirschsprung disease is usually diagnosed in patients who are younger than 1 year; but in some individuals it is found later in childhood. OBJECTIVE: This retrospective study is focused on clinical signs and symptoms of Hirschsprung disease in older children. MATERIALS AND METHODS: Patients with Hirschsprung disease were included in the study if they were older than 14 months at the time of diagnosis. RESULTS: Ten patients older than 14 months were diagnosed with Hirschsprung disease; 7 were males and 3 females. In all, 60% had a positive history of recurrent gastrointestinal infection with vomiting and hospitalization. In 6 patients, the final diagnosis was delayed because of unspecific findings in contrast enema. Rectal biopsy confirmed the diagnosis in all patients. CONCLUSION: Contrast enema is not a specific method for diagnosing Hirschsprung disease. Rectal biopsy alone confirms the diagnosis and provides a clear indication for surgery.

Peritoneal taurolidine lavage in children with localised peritonitis due to appendicitis.

Despite aggressive surgical treatment, rational antibiotic therapy, and modern intensive care, generalised peritonitis remains a major threat in the paediatric age group. Several adjuvant strategies such as peritoneal saline lavage and peritoneal drainage have been utilised. Taurolidine, derived from the amino acid taurine, has bactericidic, antiendotoxic, and antiinflammatory properties. It has been introduced previously for intraoperative peritoneal lavage in treating peritonitis in adults. The aim of our study was to evaluate the effect of peritoneal taurolidine lavage on the clinical course and serological inflammation markers in children with perforated appendicitis and localised peritonitis. A series of 27 children presenting with appendicitis between January 1999 and July 2001 were included in the study after parental informed consent. All patients underwent open appendectomy. Taurolidine peritoneal lavage was applied in 15 randomly selected children (eight girls and seven boys; mean age 10 years and 10 months). Twelve children received saline peritoneal lavage and served as the control group (six girls and six boys; mean age 9 years and 7 months). Blood was taken preoperatively and on postoperative days 1, 3, 7, and 14. Full blood cell count, C-reactive protein, endotoxin, interleukin-1, interleukin-6, soluble interleukin-2 receptor, tumour necrosis factor alpha, and procalcitonin were investigated to evaluate the serological course of inflammation. Both groups initially presented with severe inflammation as evidenced clinically and serologically. The clinical postoperative course was uneventful in 13/15 patients in the treatment group and 10/12 patients in the control group. The remaining patients presented complications: intraperitoneal abscess or early postoperative bowel obstruction. With regard to the serological inflammatory parameters, no significant differences were found between the two groups except for the soluble interleukin-2-receptor on the 7th postoperative day. In conclusion, the expected reduction of endotoxin levels and inflammatory activity in the treatment group was not evident. A significant advantage of adjuvant peritoneal taurolidine lavage in the surgical therapy of children with localised peritonitis due to appendicitis could not be shown in our study.