Long-term functional outcomes and quality of life in patients with Hirschsprung's disease.

BACKGROUND: It is unclear whether functional outcomes improve or deteriorate with age following surgery for Hirschsprung's disease. The aim of this cross-sectional study was to determine the long-term functional outcomes and quality of life (QoL) in patients with Hirschsprung's disease. METHODS: Patients with pathologically proven Hirschsprung's disease older than 7 years were included. Patients with a permanent stoma or intellectual disability were excluded. Functional outcomes were assessed according to the Rome IV criteria using the Defaecation and Faecal Continence questionnaire. QoL was assessed by means of the Child Health Questionnaire Child Form 87 or World Health Organization Quality of Life questionnaire 100. Reference data from healthy controls were available for comparison. RESULTS: Of 619 patients invited, 346 (55·9 per cent) responded, with a median age of 18 (range 8-45) years. The prevalence of constipation was comparable in paediatric and adult patients (both 22·0 per cent), and in patients and controls. Compared with controls, adults with Hirschsprung's disease significantly more often experienced straining (50·3 versus 36·1 per cent; P = 0·011) and incomplete evacuation (47·4 versus 27·2 per cent; P < 0·001). The prevalence of faecal incontinence, most commonly soiling, was lower in adults than children with Hirschsprung's disease (16·8 versus 37·6 per cent; P < 0·001), but remained higher than in controls (16·8 versus 6·1 per cent; P = 0·003). Patients with poor functional outcomes scored significantly lower in several QoL domains. CONCLUSION: This study has shown that functional outcomes are better in adults than children, but symptoms of constipation and soiling persist in a substantial group of adults with Hirschsprung's disease. The persistence of defaecation problems is an indication that continuous care is necessary in this specific group of patients.

Long-term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands.

AIM: Total colonic aganglionosis (TCA) is a severe form of Hirschsprung's disease (HD) associated with a high morbidity. This study assessed long-term functional outcome and quality of life (QoL) of patients with TCA in a national consecutive cohort. METHODS: Surgical and demographic characteristics in the medical records of all patients (n = 53) diagnosed with TCA between 1995 and 2015 were reviewed. Functional outcome of all nonsyndromal patients, aged ≥ 4 years (n = 35), was assessed using a questionnaire and in medical records. Generic and disease-specific QoL were assessed using standardized validated questionnaires. RESULTS: Of 35 patients eligible for follow-up, 18 (51%) responded to the questionnaires. They were aged 4-19 years. A Duhamel procedure was performed in 67% of these patients and a Rehbein procedure was performed in 33%. In the questionnaire, 65% of the patients reported constipation, 47% faecal incontinence and 53% soiling. Moreover, 18% of patients used bowel management (flushing or laxatives) and 29% had an adapted diet only. Children and adolescents with TCA had worse perception of their general health and were more limited by bodily pain and discomfort compared with healthy peers. Their quality of life is influenced most by frequent complaints of diarrhoea and other physical symptoms. CONCLUSION: Children and adolescents with TCA report lower health-related QoL compared with healthy peers, especially in the physical domain. We suggest standardized follow-up and prospective longitudinal future research on functionality and QoL of these patients.

Early Consequences of Pectus Excavatum Surgery on Self-Esteem and General Quality of Life.

BACKGROUND: An early observation after chest wall correction is direct inspection from the PE patient of their "new" thorax. Changes in self-perception may give raise to other psychological adaptations. The aim of this study was to evaluate the early changes in the fields of self-esteem, body image and QoL. METHODS: Prospective observational longitudinal multicenter cohort study. Self-esteem, emotional limitations and general health were assessed using the Child Health Questionnaire (CHQ) in patients under 18 and the World Health Organization Quality of Life Questionnaire-bref (WHOQOL-bref) was used for body image, psychological domain and overall QoL in patients over 16 years of age. Measurements were taken before surgery (T1) and 6 weeks (T2), and 6 months thereafter (T3). RESULTS: Scores on post-operative self-esteem were significantly higher compared with scores pre-operatively (p < 0.007). Also body image, psychological domain and emotional limitations showed significant improvement, respectively p < 0.001, p < 0.001, and p < 0.016. Significant improvement in the first three components was mainly achieved in the first 6 weeks post-operative. In emotional limitation, however, the largest change was between 6 weeks and 6 months. Overall quality of life in the WHOQOL-bref and general health domain in the CHQ showed no significant improvement in relation to the pre-operative scores. CONCLUSION: Post-operative PE patients after Nuss procedure showed an improved body image, increased self-esteem and increased psychological resilience in the first 6 months, with the most marked change in the first 6 weeks. Also emotional limitations changed significantly over time. The changes were not large enough to influence general QoL or general health significantly.

Transitional health care for patients with Hirschsprung disease and anorectal malformations.

BACKGROUND: Hirschsprung disease (HD) and anorectal malformations (ARM) are congenital disorders with potentially lifelong consequences. Although follow-up is performed in most pediatric patients, transfer to adult health care is often problematic. This study assesses transitional care with the help of questionnaires in consultation with adult patients. METHODS: This study was conducted in an outpatient clinic of a pediatric surgical center in the Netherlands. All patients born and treated for ARM or HD before 1992 were invited to visit our clinic. Patients completed questionnaires concerning disease-specific functioning and quality of life at an initial visit to in response to which individual treatment plans were modified. Patients were reviewed 1 year later. RESULTS: Twenty-seven patients (17 ARM and 10 HD), mean age 27.9 years (range 17-64 years) of the 168 invited visited the transitional clinic (17%). Passive fecal incontinence was reported by 7/27, other defecatory problems, including urge incontinence and incomplete evacuation in 17/27 and anal or abdominal pain reported by 9/27. Quality of life was lower than a matched population. Only 13/27 returned for repeat assessment at 1 year; however, a further 8 reported that that their problems had resolved. In those attending follow-up, negative thoughts and feelings about their condition had decreased and one more patient was fully continent. There was no change in quality of life, bowel function or pain recorded. Twelve out of thirteen patients reported that they had found the transitional clinic satisfactory. CONCLUSIONS: The transitional outpatient clinic provides care adapted to the needs and wishes of adult HD and ARM patients. It is a novel addition to quality of care of patients with complex congenital disorders.

The effect of taurolidine on the time-to-positivity of blood cultures.

Background: Taurolidine containing lock solutions (TL) are a promising method for the prevention of central line associated bloodstream infections. Per accident, the TL may not always be aspirated from the central venous catheter (CVC) before blood cultures are obtained. The TL could, unintentionally, end up in a blood culture vial, possibly altering the results. The aim of this study was to investigate the effect of the TLs on the detection of microbial growth in blood culture vials. Methods: Different lock solutions (taurolidine-citrate-heparin (TCHL), taurolidine, heparin, citrate or NaCl) were added to BD BACTECTM blood culture vials (Plus Aerobic/F, Lytic/10 Anaerobic/F or Peds Plus/F) before spiking with Staphylococcus aureus (ATCC 29213 or a clinical strain) or Escherichia coli (ATCC 25922 or a clinical strain) in the presence and absence of blood. Subsequently, blood culture vials were incubated in the BD BACTEC FX instrument with Time-to-positivity (TTP) as primary outcome. In addition, the effect of the TCHL on a variety of other micro-organisms was tested. Discussion: In the presence of taurolidine, the TTP was considerably delayed or vials even remained negative as compared to vials containing heparin, citrate or NaCl. This effect was dose-dependent. The delayed TTP was much less pronounced in the presence of blood, but still notable. Conclusion: This study stresses the clinical importance of discarding TLs from the CVC before obtaining a blood culture.

Satisfaction of Paediatric Oncology Patients, Survivors, and Nurses with the Position of Their Totally Implantable Venous Access Port (SPACE-Study).

BACKGROUND: To compare paediatric oncologic vascular access ports located on the anterior thoracic wall to ports on the lower lateral thoracic wall, in terms of perceived port-related hindrance and scar-quality. METHODS: A cross-sectional survey study including paediatric oncology patients (≥8-<19 yrs), caregivers (in patients <8 yrs), survivors (>22 yrs with only anterior ports) and nurses of the Princess Máxima Center, the Netherlands, was performed. The survey consisted of questions regarding satisfaction, hindrance during daily life, and port position preference. For survivors, scar-quality was assessed using the validated Patient and Observer Scar Assessment Scale (POSAS 2.0); a high score (i.e., a displeasing scar) was defined as a score higher than the third quartile of the median for that question. RESULTS: In total, 147 participants were included; 83 patients/caregivers, 31 survivors, and 33 nurses. Overall, 81 % was satisfied with the position of their port. Satisfaction, hindrance and complications did not differ between anterior and lower lateral ports. For the anterior position, minimal pressure on the port during daily life was a mentioned reason to prefer this position. For the lower lateral position, less visibility of the scar and easiest access were mentioned. Of all survivors with an anterior port scar, one in five had a displeasing scar and all scars observed were widened. Female patients preferred a lower lateral port, and scar-quality was better for left-sided port scars. CONCLUSION: The port position should be chosen together with patients/caregivers based on the (dis-)advantages of each position, as identified by this study. LEVEL OF EVIDENCE: II.

Tracked ultrasound registration for intraoperative navigation during pediatric bone tumor resections with soft tissue components: a porcine cadaver study.

PURPOSE: Resection of pediatric osteosarcoma in the extremities with soft tissue involvement presents surgical challenges due to difficult visualization and palpation of the tumor. Therefore, an adequate image-guided surgery (IGS) system is required for more accurate tumor resection. The use of a 3D model in combination with intraoperative tracked ultrasound (iUS) may enhance surgical decision making. This study evaluates the clinical feasibility of iUS as a surgical tool using a porcine cadaver model. METHODS: First, a 3D model of the porcine lower limb was created based on preoperative scans. Second, the bone surface of the tibia was automatically detected with an iUS by a sweep on the skin. The bone surface of the preoperative 3D model was then matched with the bone surface detected by the iUS. Ten artificial targets were used to calculate the target registration error (TRE). Intraoperative performance of iUS IGS was evaluated by six pediatric surgeons and two pediatric oncologic orthopedists. Finally, user experience was assessed with a post-procedural questionnaire. RESULTS: Eight registration procedures were performed with a mean TRE of 6.78 ± 1.33 mm. The surgeons agreed about the willingness for clinical implementation in their current clinical practice. They mentioned the additional clinical value of iUS in combination with the 3D model for the localization of the soft tissue components of the tumor. The concept of the proposed IGS system is considered feasible by the clinical panel, but the large TRE and degree of automation need to be addressed in further work. CONCLUSION: The participating pediatric surgeons and orthopedists were convinced of the clinical value of the interaction between the iUS and the 3D model. Further research is required to improve the surgical accuracy and degree of automation of iUS-based registration systems for the surgical management of pediatric osteosarcoma.

An abnormal screening mammogram causes more anxiety than a palpable lump in benign breast disease.

Being recalled for further diagnostic procedures after an abnormal screening mammogram (ASM) can evoke a high state anxiety with lowered quality of life (QoL). We examined whether these adverse psychological consequences are found in all women with benign breast disease (BBD) or are particular to women referred after ASM. In addition, the influence of the anxiety as a personality characteristic (trait anxiety) was studied. Between September 2002 and February 2010 we performed a prospective longitudinal study in six Dutch hospitals. Women referred after ASM or with a palpable lump in the breast (PL), who were subsequently diagnosed with BBD, were included. Before diagnosis (at referral) and during follow-up, questionnaires were completed examining trait anxiety (at referral), state anxiety, depressive symptoms (at referral, one, three and 6 months after diagnosis), and QoL (at referral and 12 months). Women referred after ASM (N=363) were compared with women with PL (N=401). A similar state anxiety score was found in both groups, but a lower psychological QoL score at 12 months was seen in the ASM group. In women with not-high trait anxiety those in the ASM group were more anxious with more depressive symptoms at referral, and reported impaired psychological QoL at referral and at 12 months compared with the PL group. No differences were found between ASM and PL in women with high trait anxiety, but this group scored unfavorably on anxiety, depressive symptoms and QoL compared with women with not-high trait anxiety. ASM evokes more anxiety and depressive symptoms and lowered QoL compared with women referred with PL, especially in women who are not prone to anxiety. Women should be fully informed properly about the risks and benefits of breast cancer screening programs. We recommend identifying women at risk of reduced QoL using a psychometric test.

Distress and post-traumatic stress in parents of patients with congenital gastrointestinal malformations: a cross-sectional cohort study.

BACKGROUND: Congenital gastrointestinal malformation (CGIM) require neonatal surgical treatment and may lead to disease-specific sequelae, which have a potential psychological impact on parents. The aim of this study is to assess distress and symptoms of post-traumatic stress disorder (PTSD) in parents of patients with CGIM. In this cross-sectional study, seventy-nine parents (47 mothers and 32 fathers) of 53 patients with CGIM completed the Distress Thermometer for Parents (DT-P) and the Self Rating Scale for Posttraumatic Stress Disorders (SRS-PTSD) as part of the multidisciplinary follow-up of their children (aged 5-35 months). Group differences were tested between parents and representative Dutch reference groups with regard to rates of (clinical) distress and PTSD, and severity of overall distress and PTSD, for mothers and fathers separately. Mixed model regression models were used to study factors associated with the risk of (clinical) distress, PTSD and with severity of symptoms of PTSD (intrusion, avoidance and hyperarousal). RESULTS: Prevalence of clinical distress was comparable to reference groups for mothers (46%) and fathers (34%). There was no difference in severity of overall distress between both mothers as well as fathers and reference groups. Prevalence of PTSD was significantly higher in mothers (23%) compared to the reference group (5.3%) (OR = 5.51, p < 0.001), not in fathers (6.3% vs 2.2.%). Symptoms of intrusion were commonly reported by all the parents (75%). Longer total length of child's hospital stay was associated with more severe symptoms of intrusion, avoidance and hyperarousal. Child's length of follow-up was negatively associated with severity of intrusion. CONCLUSIONS: Having a child with CGIM has a huge impact on parents, demonstrated by a higher prevalence of PTSD in mothers, but not fathers, compared to parents in the general population. Monitoring of symptoms of PTSD of parents in follow-up is necessary.

The efficacy of taurolidine containing lock solutions for the prevention of central-venous-catheter-related bloodstream infections: a systematic review and meta-analysis.

The incidence of central venous catheter (CVC)-related bloodstream infections is high in patients requiring a long-term CVC. Therefore, infection prevention is of the utmost importance. The aim of this study was to provide an updated overview of randomized controlled trials (RCTs) comparing the efficacy of taurolidine containing lock solutions (TL) to other lock solutions for the prevention of CVC-related bloodstream infections in all patient populations. On 15th February 2021, PubMed, Embase and The Cochrane Library were searched for RCTs comparing the efficacy of TLs for the prevention of CVC-related bloodstream infections with other lock solutions. Exclusion criteria were non-RCTs, studies describing <10 patients and studies using TLs as treatment. Risk of bias was evaluated using the Cochrane Risk of Bias 2 tool. A random effects model was used to pool individual study incidence rate ratios (IRRs). Subgroup analyses were performed based on the following factors: CVC indication, comparator lock and bacterial isolates cultured. A total of 14 articles were included in the qualitative synthesis describing 1219 haemodialysis, total parenteral nutrition and oncology patients. The pooled IRR estimated for all patient groups together (nine studies; 918 patients) was 0.30 (95% confidence interval 0.19-0.46), favouring the TLs. Adverse events (10 studies; 867 patients) were mild and scarce. The quality of the evidence was limited due to a high risk of bias and indirectness of evidence. The use of TLs might be promising for the prevention of CVC-related bloodstream infections. Large-scale RCTs are needed to draw firm conclusions on the efficacy of TLs.

In transit metastases in children, adolescents and young adults with localized rhabdomyosarcoma of the distal extremities: Analysis of the EpSSG RMS 2005 study.

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.

Effect of abnormal screening mammogram on quality of life.

BACKGROUND: Screening for breast cancer reduces breast cancer-related mortality. Advantages of screening are explained clearly, but its disadvantages are underrepresented in consent folders. METHODS: In September 2002 a prospective, longitudinal study started concerning quality of life (QoL) in women with breast disease. Between September 2002 and January 2007, 385 women with an abnormal screening mammogram were included. Of these, 152 women were diagnosed with breast cancer (BC group) and 233 had a false-positive result (FP group). Questionnaires concerning anxiety (State and Trait Anxiety Inventory) and QoL (World Health Organization Quality of Life assessment instrument 100) were completed before diagnosis, and 1, 3, 6 and 12 months later. RESULTS: The BC group was significantly older (60.2 versus 57.3 years; P < 0.001); significantly more histological biopsies were needed in the FP group (P < 0.001). Almost 60 per cent of the FP group revisited the outpatient clinic in the first year. Trait anxiety had a profound influence on QoL. Women in the FP group with a high score on trait anxiety had lowest QoL on all measurements (P < 0.001). They also reported more feelings of anxiety compared with women in the FP group with a lower trait anxiety score, and women in the BC group with a low trait anxiety score (P < 0.001). CONCLUSION: Women with a false-positive diagnosis of screen-detected breast cancer had a low QoL and feelings of anxiety, especially when they scored high on trait anxiety. This effect lasted for at least 1 year.

Presacral masses in children: presentation, aetiology and risk of malignancy.

AIM: The risk of malignant changes in presacral tumours in children was investigated in relation to age at diagnosis, type of presentation and origin of the tumour. METHOD: A retrospective review was carried out in 17 patients surgically treated for congenital presacral masses over a 22-year period. RESULTS: Constipation was the main symptom in 14 (82%) of 17 patients. The lesions were evident on digital examination in 14 patients. Mature teratoma (n = 9, 64%) was the most common lesion, including three malignancies. Currarino syndrome was diagnosed in 10 (71%) patients. Two unclassified variant HLXB9 gene mutations were found in five (29%) patients who underwent genetic testing. CONCLUSION: Congenital presacral tumours in children were mostly mature teratomas, either as sacrococcygeal teratomas or as part of the Currarino syndrome. The risk of malignancy in patients older than 1 year necessitates early surgical resection.

Systematic review and meta-analysis concerning near-infrared imaging with fluorescent agents to identify the sentinel lymph node in oncology patients.

Sentinel node procedures (SNP) are performed with the use of tracer-agents, mainly radio-colloid and/or blue dye. Fluorescent agents have emerged as a new tracer-agent to identify the SLN intra-operatively with near-infrared imaging. Our aim is to compare the detection rate of fluorescent agents to current "golden standards" (blue dye and/or radio-colloid) for the SNP by means of a systematic review and meta-analysis without any restrictions based on tumor type. A systematic search in PubMed, Embase and The Cochrane Library was performed. Articles that compared the detection rates of fluorescent agents with radio-colloid and/or blue dye were included. Meta-analyses were performed for breast, gynecological and dermatological cancer using a random effects model. In total 6195 articles were screened which resulted in a final inclusion of 55 articles. All studies used indocyanine green (ICG) as fluorescent agent. Meta-analyses comparing ICG with blue dye showed a significant and clinically relevant difference in detection rate in favor of ICG, for both breast, dermatological and gynecological cancer. Meta-analyses comparing ICG with radio-colloid did not show any significant differences, with the exception of ICG versus radio-colloid + blue dye for the bilateral SLN detection in gynecological cancer. Near-infrared fluorescence imaging using ICG provides a higher detection rate compared to blue dye for the SNP in a range of different tumor types. SLN detection rates of ICG are comparable to radio-colloid. Due to their complementary characteristics in terms of spatial resolution and transdermal sensitivity, we suggest to use a combination of both ICG and a radio-colloid.

The impact of perioperative care on complications and short term outcome in ARM type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown. METHODS: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered. RESULTS: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation. CONCLUSIONS: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: III.

Splenic function after angioembolization for splenic trauma in children and adults: A systematic review.

PURPOSE: Splenic artery embolization (SAE), proximal or distal, is becoming the standard of care for traumatic splenic injury. Theoretically the immunological function of the spleen may be preserved, but this has not yet been proven. A parameter for measuring the remaining splenic function must therefore be determined in order to decide whether or not vaccinations and/or antibiotic prophylaxis are necessary to prevent an overwhelming post-splenectomy infection (OPSI). METHODS: A systematic review of the literature was performed July 2015 by searching the Embase and Medline databases. Articles were eligible if they described at least two trauma patients and the subject was splenic function. Description of procedure and/or success rate of SAE was not necessary for inclusion. Two reviewers independently assessed the eligibility and the quality of the articles and performed the data extraction. RESULTS: Twelve studies were included, eleven with adult patients and one focusing on children. All studies used different parameters to assess splenic function. None of them reported a OPSI after splenic embolization. Eleven studies found a preserved splenic function after SAE, in both adults and children. CONCLUSION: All but one studies on the long term effects of SAE indicate a preserved splenic function. However, there is still no single parameter or test available which can demonstrate that unequivocally.

Outcome in anorectal malformation type rectovesical fistula: a nationwide cohort study in The Netherlands.

PURPOSE: Outcomes of patients with an ARM-type rectovesical fistula are scarcely reported in medical literature. This study evaluates associated congenital anomalies and long-term colorectal and urological outcome in this group of ARM-patients. METHODS: A retrospective Dutch cohort study on patients treated between 1983 and 2014 was performed. Associated congenital anomalies were documented, and colorectal and urological outcome recorded at five and ten years of follow-up. RESULTS: Eighteen patients were included, with a mean follow-up of 10.8years. Associated congenital anomalies were observed in 89% of the patients, 61% considered a VACTERL-association. Total sacral agenesis was present in 17% of our patients. At five and ten years follow-up voluntary bowel movements were described in 80% and 50%, constipation in 80% and 87%, and soiling in 42% and 63% of the patients, respectively. Bowel management was needed in 90% and one patient had a definitive colostomy. PSARP was the surgical reconstructive procedure in 83%. Urological outcome showed 14 patients (81%) to be continent. No kidney transplantations were needed. CONCLUSION: In our national cohort of ARM-patients type rectovesical fistula that included a significant proportion of patients with major sacral anomalies, the vast majority remained reliant on bowel management to be clean after ten years follow-up, despite "modern" PSARP-repair. Continence for urine is achieved in the majority of patients, and end-stage kidney failure is rare.

Quality of life and health status in breast carcinoma.

Quality of life is of increasing importance in clinical oncology studies. When analysing publications concerning quality of life in breast cancer, however, the majority of the articles appear to study health status and not quality of life. Therefore five recommendations were formulated to apply reading a 'quality of life' article. With the use of these recommendations an article can be evaluated and the clinical significance can be assessed.