I. de Blaauw

Laparoscopic Treatment of Intestinal Malrotation in Children

Purpose Intestinal malrotation is a congenital intestinal rotation anomaly, which can be treated by either laparotomy or laparoscopy. Our hypothesis is that laparoscopic treatment leads to less small bowel obstruction because of the fewer adhesions in comparison to laparotomy, without increasing the risk of recurrent volvulus. We analyzed the outcome of patients who had a correction for intestinal malrotation after the introduction of laparoscopy. Methods All patients between 0 and 18 years who underwent a surgical procedure for malrotation in the Radboudumc Amalia Childrens Hospital, Nijmegen, the Netherlands, between January 2004 and December 2011 were retrospectively reviewed for duration of operation, perioperative complications, length of hospital stay, and rate of redo surgery for intestinal volvulus or obstruction. Results A total of 83 patients were included of which 33 had a laparoscopic procedure and 50 had a laparotomy for suspected malrotation. Operating time was 63 minutes for the laparoscopic procedure versus 76 minutes for laparotomy (p = 0.588). Significantly more complications were found in the laparotomy group (11 vs. 35%, p = 0.047). However, one patient (aged 4 months) in the laparoscopy group developed an early (< 24 hours) recurrent volvulus. Length of hospital stay was significantly longer after a laparotomy (9 vs. 16 days, p = 0.002). Three (17%) patients in the laparoscopy group needed redo surgery compared with six (9%) in the laparotomy group (p = 0.400). No late volvulus occurred in both groups. After laparotomy, redo surgery because of the small bowel obstruction was more frequent (5 vs. 0%), although this was not statistically significant. Conclusion In both the laparoscopy and laparotomy group, no cases of long-term recurrent volvulus were seen. After laparotomy, more patients developed a late small bowel obstruction because of the adhesions for which redo surgery was needed. In the laparotomy group, the number of complications was significantly higher and the length of hospital stay was significantly longer. Comparing laparoscopy and laparotomy for the treatment of malrotation, no difference exists for the long-term risk of recurrent volvulus. In children aged 6 months or older with suspicion of intestinal malrotation but not presenting with an acute abdomen or hemodynamically instability, laparoscopy should be considered as a first approach to diagnose and subsequently treat intestinal malrotation.

Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations

PURPOSE Cloacal malformations are the most complex type of anorectal malformation in females. This study aimed to report quality of life (QoL) of patients with a cloacal malformation for the first time in literature. MATERIALS AND METHODS Female patients with an anorectal malformation participating in a follow-up program for congenital malformation survivors aged 5 or older were eligible for this study. QoL was assessed with the PedsQL™ 4.0 inventory. Scores of patients with a cloacal malformation (CM) were compared with those of female patients with rectoperineal or rectovestibular fistulas (RP/RV) and with reference data. RESULTS A total of 59 patients (67% response rate; 13 patients with cloacal malformation) were included, QoL was assessed by patient self-report at median age of 12years (8-13), and by parent proxy-report at median age of 8years (5-12). There were no differences between groups regarding the presence of associated anomalies, with also no differences regarding anomalies in the urinary tract (CM vs. RP/RV=31% vs. 15%, p=0.237). Scores of the cloacal malformations group were similar to those of the comparison group, except the proxy-reported scores on school functioning (60.0 vs. 80.0, p=0.003). Proxy-reported scores of cloacal malformation patients were significantly lower than reference values on total QoL-score, psychosocial health, and emotional and school performance. Patients (irrespective of type of ARM) who suffered from fecal soiling reported significantly lower scores with regard to psychosocial health (71.7 vs. 81.7, p=0.034) and its subscale school performance (65.0 vs. 80.0, p<0.001). QoL-scores reported by cloacal malformation patients did not differ significantly from the reference values of the healthy population. Parents of cloacal malformation patients reported significantly lower total QoL, emotional and school performances, as well as a lower general psychosocial health for their children relative to reference data of healthy children. CONCLUSION Patients with cloacal malformations and females with less complex anorectal malformations report similar QoL. Parents of cloacal malformation patients report more problems on several psychosocial domains relative to the healthy reference group. To monitor these matters, long-term follow-up protocols should contain multidisciplinary treatment including periodical assessment of psychosocial well-being.

Clinical Outcome of Cloacal Exstrophy, Current Status, and a Change in Surgical Management

INTRODUCTION Cloacal exstrophy is a complex and rare congenital malformation. Because of improvements in medicine, operative techniques, and perioperative management the survival rates are now approaching 100%. Currently, treatment is focused on improving quality of life. Since 1974, we encountered 20 patients with cloacal exstrophy in our hospital. The aim of this study is to evaluate our clinical experience and outcome during the last 39 years. PATIENTS AND METHODS A retrospective study of the medical records was performed. We evaluated anatomical status, phenotype and genotype at birth, subsequent surgical treatment and current gastrointestinal, urinary, spinal, genital, and gender status and outcome. RESULTS The records of all 20 patients who were treated in our center were included in this study. Of the 20 patients, 18 were primary patients, 2 referred. Six patients died within the first year of life. Median age of the surviving 14 patients was 25 years (range, 4-39 years) at time of evaluation. At the last follow-up, 11 patients had an endileostomy or endcolostomy. Three patients had a pull-through; two of them were continent for feces. Two patients were continent for urine; one with a sinus urogenitalis and one after urethra reconstruction. Eleven patients are incontinent; six of them had an incontinent urinary deviation (Bricker deviation). Of the other five incontinent patients, two had a urethral reconstruction, one vesicovaginal anastomosis, one perineotomy, and one epispadias bladder. Urinary continence was unknown in one patient. Of the surviving patients, nine have 46 XY karyotype and five 46 XX karyotype. Initially, six XY patients underwent external genital reconstruction with reassignment to female gender; however, one died postoperatively. One declared to feel like a man at age of 15 years. Remaining four XY patients underwent male external genital reconstruction. All XX patients underwent vagina or vulva reconstruction, except for one who still has aplasia vaginae. All patients were born with varying types of spinal dysraphism. DISCUSSION Despite medical efforts and development in treatment and support, satisfaction in outcome of cloacal exstrophy remains a challenge. Although reconstruction may be very difficult, male genital reconstruction in 46 XY patients has been given preference for more than a decade. A specialized multidisciplinary team should provide lifelong colorectal, urological, sexual, and psychosocial support to these patients, taking into account their age and phase of life.

Redo pull-through surgery in Hirschsprung disease: Short-term clinical outcome

INTRODUCTION Although surgery is effective in most patients with Hirschsprung disease (HD), some have persistent obstructive symptoms. Additional medical treatment is generally sufficient, but a small fraction of these patients needs secondary surgery. Series on redo surgery are scarce. Aim of this study is to evaluate complications and clinical outcome of patients in need of redo surgery for HD. MATERIALS AND METHODS Sixteen patients underwent redo endorectal pull-through surgery in our center between 2007 and 2015. Medical records were reviewed and demographics, indication for redo surgery, surgical procedures, complications, and clinical outcome were scored. RESULTS The median age at the time of redo was 4.6years (range: 2months-21years). Median follow-up after redo was 3years (range: 9months-7years). Before redo surgery, all patients (100%) had obstructive symptoms, one patient had recurrent enterocolitis, and four patients were fecally incontinent despite adequate attempts of bowel management. Surgical procedure consisted of a transanal endorectal pull-through (TERPT) in all patients, with additional laparotomy in 7 (44%) and protective stoma in 8 patients (50%). Complications within 30days after redo surgery were anastomotic dehiscence (3; 19%), wound abscess (2; 13%), rectovaginal fistula (1; 7%) or enterocutaneous fistula (1; 7%). During follow-up, nine patients needed additional surgery, mainly to close the stoma. At final follow-up there were no patients with stenosis, obstructive symptoms, remaining rectovaginal fistula, or small bowel obstruction. Only one patient experienced enterocolitis. Six patients (43%) reported soiling or fecal incontinence. CONCLUSION TERPT for redo surgery for HD is effective in resolving sustained severe obstructive symptoms after primary surgery, but the outcome is complicated by a relatively high rate of soiling and fecal incontinence.

Duhamel versus transanal endorectal pull through (TERPT) for the surgical treatment of Hirschsprung’s disease

For the surgical treatment of Hirschsprung’s disease, several surgical techniques are used to resect the distal aganglionic colon. Two frequently used techniques are the Duhamel procedure and the transanal endorectal pull-through procedure. During the ‘8th Pediatric Colorectal Course’ in Nijmegen, November 2015, a workshop was organized to share experiences of both techniques by several experts in the field and to discuss (long term) outcomes. Specifically, the objective of the meeting was to discuss the main controversies in relation to the technical execution of both procedures in order to make an initial assessment of the limitations of available evidence for clinical decision-making and to formulate a set of preliminary recommendations for current clinical care and future research.

Perioperative Nutritional Management in Congenital Perineal and Vestibular Fistulas: A Systematic Review

BACKGROUND Surgical advancements have led to improved outcomes for children with congenital anorectal malformations with vestibular and perineal fistulas. However, the effect of perioperative nutritional management is debated and guidelines have not yet been established. OBJECTIVE The study aims to give an overview of available published evidence, regarding the impact of different perioperative nutritional management protocols on surgical outcome. METHODS A systematic literature review was conducted using PubMed, Embase, Cochrane Library, and CINAHL databases. All original articles concerning perioperative nutrition in children with vestibular and perineal fistulas were included. Methodological quality was assessed with the Rangel score. Included studies were subdivided into two groups: early enteral nutrition and prolonged fasting with or without parenteral nutrition. RESULTS The database search resulted in 768 publications. Nine studies were eligible for inclusion. Wound complications were present in 56 of the 1,557 patients (4%) in whom this was assessed, and were more frequently seen in the prolonged fasting group (2 vs. 10%, p < 0.0001). Regarding the long-term outcome, constipation (grade II-III) was seen in 4% of the early feeding group, compared with 13% in the prolonged fasting group (p < 0.0001). CONCLUSION This systematic review presents an overview of studies reporting on perioperative nutritional management in children with perineal and vestibular fistulas. Although study quality is low and study heterogeneity may also influence our results, early enteral feeding seems to be the preferable postoperative feeding strategy. Both early wound complications as well as long-term complications, in terms of clinically relevant constipation, seem to be lower in the early enteral feeding group. However, a prospective randomized, multicentered trial should be initiated to draw definitive conclusions regarding this matter.

The Challenges of the European Anorectal Malformations-Net Registry

Anorectal malformations (ARM) have a low prevalence, patients need specialized surgical care, and in many cases, patients born with ARM even need life-long aftercare. Due to its low prevalence most patients are still treated in low-volume pediatric surgical centers without any adequate monitoring of the outcome. Data on prevalence, comparison of different surgical techniques, and prospective outcome measurements are still scarce and difficult to interpret. In 2010, a consortium was founded (ARM-Net consortium) including several European pediatric surgical centers to collaborate more in research and share knowledge on ARM. One of the structures started by the consortium was an ARM-Net registry for the inclusion of all future patients treated in these centers. With this review, we report the structure of the ARM-Net registry, some of the results, and discuss the challenges we faced and still face after its introduction in 2010.

The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC): rationale, infrastructure, and initial steps

Each year, in the USA, approximately 800 babies are born with anorectal malformations (ARM) and roughly the same number with Hirschsprung disease (HD) [1, 2]. Surgical reconstruction and the associated care are important factors in the health of these patients and their quality of life. Despite successful surgical repair, more than 60% of HD and ARM patients will suffer from bowel disorders such as severe constipation and fecal incontinence [3–8], which have detrimental long-term effects on quality of life [7]. Two specific areas in which research is needed are longterm outcomes and disease-specific standardized definitions. The effect of various surgical and medical treatment methods on long-term postoperative outcomes for ARMs and HD is not well understood. In addition, there are no standard definitions for medical treatment failure, appropriate preoperative evaluation, and indications for surgery in cases of functional constipation [9]. Critical barriers to research in these areas include highly variable clinical care, a wide spectrum of poorly defined clinical phenotypes, small sample sizes, a lack of coordinated multicenter effort, incomplete data collection, inconsistent clinical definitions, and a lack of established longterm outcome measures. Current guidelines for surgical treatment, postoperative care, and clinical outcomes for these disorders are based primarily on expert opinion and retrospective studies performed at single institutions. Without common definitions and standardized outcome measures applied consistently across multiple centers, the results from these single-center studies may not be generalizable. As a result, there is no clear best practice for many aspects of care, and practice varies widely between institutions. This report describes the development, organization, and work plan of an international consortium, the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC; http://pcplc .org). The purpose of the PCPLC is to provide the infrastructure needed to conduct rigorous multicenter studies that can overcome many of the previous barriers to research.

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

Total colonic aganglionosis (TCA) is a severe form of Hirschsprungs 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.

“Rectal ulcer: consider Hirschsprung’s disease” Report of the Colorectal Club meeting, Milan, June 14–15, 2015

This year the meeting of the Pediatric Colorectal Club was held in Milan. The yearly meeting of pediatric surgeons dedicated to colorectal surgery was founded to bring together surgeons with this special interest. The key focus has always been: Hirschsprung’s disease and (congenital) anorectal malformations. This year more than 200 pediatric surgeons from all over the world came to Milan many of whom gave oral or poster presentations. Anorectal malformations are rare and often associated with urogenital anomalies. One of the keynote speakers emphasized that only a small number of children develop renal failure, particularly those born with urogenital anomalies. The potential risk factors were discussed, and it was emphasized that urinary tract infections in the early years of life need to be prevented. Remarkably, despite a high percentage of urinary reflux, this was not an additional risk factor. It was pointed out that dysplastic or other congenital malformations of the urinary tract have more effect on kidney function. The timing of transplant surgery was discussed, particularly in some of the complex cases. Here was agreement that a thorough preoperative workup is essential before any reconstruction is performed in these cases, e.g., cloacal malformations Research on the genetics of anorectal malformations, which is slowly moving forward, was another topic covered. Multiple genes and multiple environmental factors are involved. A large series of rare variants of genes have been screened but found not to play a major role. It seems that the new focus of research will become the interaction of genes and environmental factors despite the fact that this area is the most difficult to study. Another associated anomaly in patients born with an anorectal malformation is the presence of a tethered cord. It is still a matter of debate what clinical investigations are best for determining the presence of a tethered cord and what management strategy is most effective. The value of ultrasound in the neonate was discussed and seems not to be sensitive enough; some still advocate its use as a screening tool, others prefer MRI but it was not agreed at what age MRI should be performed and whether it was appropriate for asymptomatic patients. There was a lively discussion about whether surgery should be offered to the children with a tethered cord: It was clear that there is no consensus among pediatric surgical units in this regard. There was also disagreement about the timing of surgery: Should it be preventive or only performed when the child becomes symptomatic? Unluckily, there were no pediatric neurosurgeons to contribute to the discussion, and suggestions were made to have them invited to one of the future colorectal meetings. A recently founded European group of pediatric surgeons (ARM-net.eu) is collecting a lot of data on the subject, and this may have impact on the future policy regarding tethered cord. Prof. Alberto Pena, one of the founding fathers of the Colorectal Club and probably the most senior colleague in this field, stated that even at the end of his career, he still finds it difficult to understand the concept of tethered cord. Several papers that were presented related to the transition of children born with an anorectal malformation from pediatric medical services to adult services. The need & I. de Blaauw ivo.deblaauw@radboudumc.nl