Outcomes in Hirschsprung's disease with coexisting learning disability.

This study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung's disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala's BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44-60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0-23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known: • Hirschsprung's disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments. • The outcomes for these patients specifically have been poorly described in the literature. What is New: • Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment. • Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

Long-term surgical and patient-reported outcomes of Hirschsprung Disease.

BACKGROUND: Information is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe long-term outcomes across multiple domains, completing a core outcome set through to adulthood. METHODS: HSCR patients operated at a single center over a 35-year period (1978-2013) were studied. Patients completed detailed questionnaires on bowel and urologic function, and HRQOL. Patients with learning disability (LD) were excluded. Outcomes were compared to normative data. Data are reported as median [IQR] or mean (SD). RESULTS: 186 patients (median age 28 [18-32] years; 135 males) completed surveys. Bowel function was reduced (BFS 17 [14-19] vs. 19 [19-20], p < 0•0001;η2 = 0•22). Prevalence and severity of fecal soiling and fecal awareness improved with age (p < 0•05 for both). Urinary incontinence was more frequent than controls, most of all in 13-26y females (65% vs. 31%,p = 0•003). In adults, this correlated independently with constipation symptoms (OR 3.18 [1.4-7.5],p = 0.008). HRQoL outcomes strongly correlated with functional outcome: 42% of children demonstrated clinically significant reductions in overall PedsQL score, and poor bowel outcome was strongly associated with impaired QOL (B = 22•7 [12•7-32•7],p < 0•001). In adults, GIQLI scores were more often impacted in patients with extended segment disease. SF-36 scores were reduced relative to population level data in most domains, with large effect sizes noted for females in General Health (g = 1.19) and Social Wellbeing (g = 0.8). CONCLUSION: Functional impairment is common after pull-through, but bowel function improves with age. Clustering of poor functional outcomes across multiple domains identifies a need for early recognition and long-term support for these patients.

Laparoscopic Ladd's procedure for malrotation in infants and children is still a controversial approach.

BACKGROUND: Open Ladd's procedure is the gold standard for the correction of intestinal malrotation and laparoscopic approach remains controversial. This study aimed to evaluate our experience in laparoscopic management of malrotation. METHODS: Single center retrospective study including patients who underwent a laparoscopic assessment of intestinal malrotation with correction if appropriate between 2007 and 2017. RESULTS: Sixty-five patients (median age 7 months) had a laparoscopic assessment with and without correction of malrotation. Forty-five (69%) were symptomatic, including 16 (25%) with a midgut volvulus. The procedure was completed laparoscopically in 55 (86%) patients in 110 min (30-190). Conversions happened more frequently at the beginning of the experience. With a follow-up of 12.5 months (8 days-5.3 years), morbidity rate was 15% and 4 (6%) patients underwent a redo surgery, all in the first 5 months after surgery, compared with 3/53 (6%) in a contemporaneous group undergoing open Ladd's. CONCLUSION: This is the largest series reported so far of the laparoscopic management of malrotation. Laparoscopic Ladd's procedure is reliable but still exposes to open conversion which may be in part owing to a learning curve. A low conversion threshold is important in cases with volvulus. The redo rate is similar to that of the open procedure. LEVEL OF EVIDENCE: Level III retrospective comparative treatment study.

Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients.

BACKGROUND/PURPOSE: Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. METHODS: We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P<0.05. RESULTS: In our institution, 12 consecutive patients aged 17-month-old (22 days-7 years), underwent laparoscopic patch repair of MH, with one conversion to laparotomy. No recurrence or significant complication occurred over a follow-up period of 8 months (1-48 months). Thirty-six articles were included from literature review and were combined with the current series. All were retrospective case reports or series, of which 6 were comparative cohorts with both MIS and open repairs. A total of 296 patients from 37 series were ultimately used for analysis: 80 had open repair (4 patch) and 216 had MIS repair (32 patch), with a patch rate of 12%. There were 13 recurrences (4%): no difference between open and MIS repairs (4/80 vs 9/216, p=0.75); recurrence rate following primary repair was 13/260 (5%), but no recurrence occurred with 36 patch repairs. Meta-analysis showed no difference in recurrence between open and MIS repair (p=0.83), whereas patch repair was associated with 14% less recurrence compared with primary repair, although it did not reach statistical significance (p=0.12). There were 13 complications (5%): no difference between open and MIS repairs (5/80 vs 8/216, p=0.35). One small bowel obstruction occurred in a patient who had laparoscopic patch repair. CONCLUSION: In MH, recurrence and complication rates are comparable between MIS and open repairs. Use of patch appeared to confer additional benefit in reducing recurrence. TYPE OF STUDY: Systematic review LEVEL OF EVIDENCE: 3A.

Laparoscopic Enteropexy for Prolapsing Stoma: A Case Series Describing a Novel Technique.

BACKGROUND: Ileostomy prolapse has a cumulative risk of 11% and represents a significant complication with associated morbidity, which may result in multiple admissions and procedures requiring a general anesthetic. We have developed a laparoscopic technique for managing stoma prolapse-so-called laparoscopic enteropexy for prolapsing stoma (LEPS). METHODS: Retrospective analysis of a prospectively maintained departmental surgical logbook was performed alongside clinical case history review for patients undergoing LEPS. Primary outcome was defined as recurrent prolapse. Secondary outcomes were any postoperative complication or complication occurring at the time of stoma closure. RESULTS: A total of 15 LEPS procedures were performed on 14 patients with stoma prolapse-13 were patients with ileostomy and 1 with a loop colostomy. Median operative time was 75 minutes (range 50-95). Median postoperative stay was 1 day (1-4 days). There was one return to theater for a small bowel intussusception on the second postoperative day wherein taking down of the bowel and repeat LEPS were necessary. There was one recurrence of prolapse in a separate patient (1/14 [7%]). Three patients have since had their stoma closed without complication. CONCLUSION: We describe here the initial case series of our LEPS procedure for managing stoma prolapse. This is a reproducible and technically straightforward laparoscopic procedure with an excellent success rate in preventing further prolapse.

Anatomy and physiology of the peritoneum.

The peritoneum is commonly encountered in abdominal surgery. The development and rotation of the primitive gut tube lead to the normal adult arrangement of the peritoneal cavity, which forms bloodless planes allowing the retroperitoneal portions of the bowel to be safely mobilised. The arrangement of the peritoneum also forms spaces in which infected fluid or pus can collect. The microcirculation of peritoneal fluid is now well understood, and the large absorptive surface of the peritoneum can be exploited in peritoneal dialysis. The absorption of gas by the peritoneum following abdominal surgery is faster in neonates than in older children, and understanding this process contributes to the interpretation of post-operative radiographs.

Role of simulation in training the next generation of endoscopists.

The use of simulation based training in endoscopy has been increasingly described, simulation has the potential reduce the harm caused to patients by novices performing procedures, increase efficiency by reducing the time needed to train in the clinical environment and increase the opportunity to repeatedly practice rare procedures as well as allowing the assessment of performance. Simulators can consist of mechanical devices, employ cadaveric animal tissue or use virtual reality technology. Simulators have been used to teach upper and lower gastrointestinal endoscopy as well as interventional procedures. This review reviews the currently available endoscopic simulators, and the evidence for their efficacy, demonstrating that the ability of simulators to differentiate between novice and expert endoscopists is well established. There is limited evidence for improved patient outcome as a result of simulation training. We also consider how the environment within which a simulation is placed can be manipulated to alter the learning achieved, broadening the scope of simulation to develop communication as well as technical skills. Finally the implications for future practice are considered; technology is likely improve the fidelity of simulators, increasing the potential for simulation to improve patient outcomes. The impact of the simulation environment, and the correct place of simulation within the training curriculum are both issues which need addressing.

Troublesome knowledge in pediatric surgical trainees: a qualitative study.

BACKGROUND: Meyer and Land (2003) describe threshold concepts as being "akin to a portal, opening up a new and previously inaccessible way of thinking about something." As a consequence, threshold concepts have a transformational potential and may lead to an associated change in identity. The successful completion of pediatric surgical training in the United Kingdom is a lengthy and complex professional journey in which trainees emerge as consultants with a professional identity. We sought to explore how "threshold concepts" applied to pediatric surgical training with a view to identifying elements that were "troublesome." METHODS: Semistructured interviews were conducted. Transcripts were generated from audio recordings and thematically analyzed by the authors. Constant comparison was used to refine themes. Participants were purposively recruited across all years of training. A total of 8 pediatric surgical trainees participated in the study. Approval from obtained from the Human Research Ethics committee. RESULTS: Although there is overlap between themes, analysis revealed "troublesome" areas of training related to knowledge (breadth and rarity of some conditions), clinical judgment (shifting expectation of independence), technical skills (accessing opportunities), transitions between roles (increasing responsibility and remoteness of support), relationships with trainers, and the effect of negative experiences. CONCLUSIONS: Viewing trainees' experiences of surgical training through the lens of "threshold concepts" provides insight to the importance of viewing the curriculum in a holistic way. Negative experiences in training were an important catalyst for development, inducing a fundamental change in perception, which might be characterized as a rupture of a "meaning frame." Trainees in pediatric surgery can be viewed as moving to a mature specialist identity via a transitional state-liminality, from entry into specialist training.

Chronically symptomatic patients with undetectable gall bladder on ultrasonography could benefit from early cholecystectomy.

90 percent of symptomatic patients undergoing cholecystectomy have cholelithiasis with 10% categorized as asymptomatic cholecystitis. In both instances, the gallbladder is evident on ultrasonography. In children with symptomatic biliary dyspepsia, the decision to proceed to cholecystectomy is made difficult if choleliths are not seen on ultrasonography. This decision is made even more difficult if the gallbladder itself is not seen on repeated imaging. In a cohort of 54 cholecystectomies, 3 cases, with recurrent right upper quadrant pain and undetectable gallbladders on repeat ultrasonography, were identified. After prolonged observation all underwent successful cholecystectomy. Histology demonstrated a markedly fibrotic and thickened gallbladder in all. Given this experience, we suggest that nonvisibility of the gallbladder, in fact, maybe be a feature of a chronic acalculous cholecystitis. We advise consideration of cholecystectomy for chronic biliary dyspepsia where repeat ultrasonography fails to demonstrate a gallbladder.

Risk of hernia occurrence where division of an indirect inguinal sac without ligation is undertaken.

We sought to ascertain the risk of inguinal hernia occurrence when division of the processus vaginalis is undertaken without ligation, in the context of laparoscopic Fowler-Stephens orchidopexy. A cohort of patients with intra-abdominal testes subjected to a two-stage Fowler-Stephens procedure was reviewed. Analysis of a 68-month period between November 2005 and August 2011 was performed. A comprehensive search of the literature was undertaken, and these data were compared with previously published studies of patients undergoing orchidopexy or herniotomy where the peritoneal defect was not closed. The procedure was undertaken as previously described, with a conventional first stage using a three-port technique. No attempt was made to approximate the peritoneal margins of the processus vaginalis/hernia or close the internal ring at the second stage. In our own experience 17 patients with undescended testes (2 with bilateral cases) underwent laparoscopically assisted, gubernaculum-sparing, Fowler-Stephens orchidopexy. Median age at first operation was 1.86 years (range, 1-9 years). All 17 patients had successful surgery with all 19 testes palpable within the scrotum at postoperative assessment at 3 and 6 months. No direct, indirect, or incisional hernias were noted at a mean follow-up of 2.7 years (standard deviation 1.71). Our experience and reports in the literature do support simple division of the indirect hernia sac as a tenable alternative to ligation. The result of this limited review would support a prospective randomized trial comparing ligation with simple division of hernia sacs.

Does thoracoscopic congenital diaphragmatic hernia repair cause a significant intraoperative acidosis when compared to an open abdominal approach?

PURPOSE: Thoracoscopic congenital diaphragmatic hernia (CDH) repair is increasingly reported. A significant intraoperative acidosis secondary to the pneumocarbia, as well as an increased recurrence rate, are possible concerns. Our aim was to review our early experience of the technique. METHODS: A prospective and retrospective data collection was carried out on all patients undergoing either an open or thoracoscopic CDH repair for a 4-year period. Preoperative blood gas values were identified at various stages of the operative procedure. A pH of 7.2 was considered to be a significant acidosis. The duration of surgery, complications, and recurrence rates were also recorded. Data were analyzed using the Mann-Whitney U test, and a P value of .05 or less was considered significant. RESULTS: Twenty-two patients were included. One death occurred before surgery. Twelve patients underwent thoracoscopic repair (8 neonatal), and 9 underwent open repair (8 neonatal). There were 9 left-sided defects in the thoracoscopic group and 9 in the open group. Operative time was longer in the thoracoscopic group compared to the open group (median, 135 vs 93.5 minutes; P = .02). Neonates undergoing thoracoscopic repair were heavier compared to the open group (median, 3.9 vs 2.9 kg; P = .05), and their preoperative requirements for ventilation and inotropes were comparable. However, the association between those patients who required preoperative inotropes and those who required a patch repair was statistically significant P = .03. Two patients in each group developed an intraoperative acidosis. A further patient in the thoracoscopic group had a severe acidosis present at the beginning of surgery. There was no statistical difference in pH values or recurrence rate between the 2 groups. All recurrences were in patients requiring patch repairs. No postoperative mortality occurred. CONCLUSIONS: We present our early experience of thoracoscopic CDH repair. Our results from thoracoscopic repair appear similar to the open procedure performed over the same period. No clear difference in intraoperative pH or recurrence rate has been demonstrated in our series. There is a need for a multicenter prospective study to establish the longer term outcome of this technique.