Developing a core outcome set for the health outcomes for children and adults with congenital oesophageal atresia and/or tracheo-oesophageal fistula: OCELOT task group study protocol.

INTRODUCTION: Heterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions to standardise outcome reporting, facilitate meta-analysis and improve the relevance of research for patients and families. Our aim is to develop an internationally-agreed, comprehensive COS for OA-TOF, relevant from birth through to transition and adulthood. METHODS AND ANALYSIS: A long list of outcomes will be generated using (1) a systematic review of existing studies on OA-TOF and (2) qualitative research with children (patients), adults (patients) and families involving focus groups, semistructured interviews and self-reported outcome activity packs. A two-phase Delphi survey will then be completed by four key stakeholder groups: (1) patients (paediatric and adult); (2) families; (3) healthcare professionals; and (4) researchers. Phase I will include stakeholders individually rating the importance and relevance of each long-listed outcome using a 9-point Likert scale, with the option to suggest additional outcomes not already included. During phase II, stakeholders will review summarised results from phase I relative to their own initial score and then will be asked to rescore the outcome based on this information. Responses from phase II will be summarised using descriptive statistics and a predefined definition of consensus for inclusion or exclusion of outcomes. Following the Delphi process, stakeholder experts will be invited to review data at a consensus meeting and agree on a COS for OA-TOF. ETHICS AND DISSEMINATION: Ethical approval was sought through the Health Research Authority via the Integrated Research Application System, registration no. 297026. However, approval was deemed not to be required, so study sponsorship and oversight were provided by Alder Hey Children's NHS Foundation Trust. The study has been prospectively registered with the COMET Initiative. The study will be published in an open access forum.

International Core Outcome Set for Acute Simple Appendicitis in Children: Results of a Systematic Review, Delphi Study, and Focus Croups With Young People.

OBJECTIVE: To develop an international core outcome set (COS), a minimal collection of outcomes that should be measured and reported in all future clinical trials evaluating treatments of acute simple appendicitis in children. SUMMARY OF BACKGROUND DATA: A previous systematic review identified 115 outcomes in 60 trials and systematic reviews evaluating treatments for children with appendicitis, suggesting the need for a COS. METHODS: The development process consisted of 4 phases: (1) an updated systematic review identifying all previously reported outcomes, (2) a 2-stage international Delphi study in which parents with their children and surgeons rated these outcomes for inclusion in the COS, (3) focus groups with young people to identify missing outcomes, and (4) international expert meetings to ratify the final COS. RESULTS: The systematic review identified 129 outcomes which were mapped to 43 unique outcome terms for the Delphi survey. The first-round included 137 parents (8 countries) and 245 surgeons (10 countries), the second-round response rates were 61% and 85% respectively, with 10 outcomes emerging with consensus. After 2 young peoples' focus groups, 2 additional outcomes were added to the final COS (12): mortality, bowel obstruction, intraabdominal abscess, recurrent appendicitis, complicated appendicitis, return to baseline health, readmission, reoperation, unplanned appendectomy, adverse events related to treatment, major and minor complications. CONCLUSION: An evidence-informed COS based on international consensus, including patients and parents has been developed. This COS is recommended for all future studies evaluating treatment ofsimple appendicitis in children, to reduce heterogeneity between studies and facilitate data synthesis and evidence-based decision-making.

Home-Initiated-Programme-to-Prepare-for-Operation: evaluating the effect of an animation video on peri-operative anxiety in children: A randomised controlled trial.

BACKGROUND: Hospital admissions and surgical operations commonly trigger anxiety in young children. Despite employing numerous support measures in our hospital, such as a pre-operative play room, the encouragement of parental companionship during induction of anaesthesia and distraction therapy, allaying the anxiety of our young surgical patients remains a challenge. OBJECTIVES: To evaluate the effectiveness of a Home-Initiated-Programme-to-Prepare-for-Operation (HIPPO) on emotional manifestation and anxiety in children undergoing surgery. DESIGN, SETTING AND PATIENTS: One hundred and thirty children were randomly assigned to either control or intervention group between February 2018 and April 2019 in a tertiary paediatric hospital in Singapore. INTERVENTION: In addition to our standard pre-operative workflow, the intervention group received an additional home preparation kit consisting of an animated video on preoperative preparation and age-specific preoperative preparation activity sheets. MAIN OUTCOME MEASURES: The primary outcome was the Children's Emotional Manifestation Scale score to evaluate behaviour and emotion in the children before and during induction of anaesthesia. Secondary outcomes evaluated anxiety levels in parents and children, the child's behaviour and degree of co-operation using the State-Trait Anxiety Inventory scores, State-Trait Anxiety Inventory Children scores, the Induction Compliance Checklist scores, the Visual Analogue Scale scores for anxiety and the feedback questionnaire. RESULTS: The difference between the Children's Emotional Manifestation Scale score in control and intervention groups was not statistically significant. A promising difference was however observed in one of the secondary outcomes where the state-State-Trait Anxiety Inventory Children scores of 7 to 10-year olds in the intervention group almost reached significance; P = 0.067. CONCLUSION: Despite being a child-friendly, easily accessible and affordable tool for patient education, HIPPO did not reduce anxiety experienced by children in the pre-operative waiting area or during induction of anaesthesia. TRIAL REGISTRATION: ClinicalTrials.gov, identifier: NCT04271553.

Pediatric Solid Tumor Care and Multidisciplinary Tumor Boards in Low- and Middle-Income Countries in Southeast Asia.

PURPOSE: Pediatric solid tumors require coordinated multidisciplinary specialist care. However, expertise and resources to conduct multidisciplinary tumor boards (MDTBs) are lacking in low- and middle-income countries (LMICs). We aimed to profile the landscape of pediatric solid tumor care and practices and perceptions on MDTBs among pediatric solid tumor units (PSTUs) in Southeast Asian LMICs. METHODS: Using online surveys, availability of specialty manpower and MDTBs among PSTUs was first determined. From the subset of PSTUs with MDTBs, one pediatric surgeon and one pediatric oncologist from each center were queried using 5-point Likert scale questions adapted from published questionnaires. RESULTS: In 37 (80.4%) of 46 identified PSTUs, availability of pediatric-trained specialists was as follows: oncologists, 94.6%; surgeons, 91.9%; radiologists, 54.1%; pathologists, 40.5%; radiation oncologists, 29.7%; nuclear medicine physicians, 13.5%; and nurses, 81.1%. Availability of pediatric-trained surgeons, radiologists, and pathologists was significantly associated with the existence of MDTBs (P = .037, .005, and .022, respectively). Among 43 (89.6%) of 48 respondents from 24 PSTUs with MDTBs, 90.5% of oncologists reported > 50% oncology-dedicated workload versus 22.7% of surgeons. Views on benefits and barriers did not significantly differ between oncologists and surgeons. The majority agreed that MDTBs helped to improve accuracy of treatment recommendations and team competence. Complex cases, insufficient radiology and pathology preparation, and need for supplementary investigations were the top barriers. CONCLUSION: This first known profile of pediatric solid tumor care in Southeast Asia found that availability of pediatric-trained subspecialists was a significant prerequisite for pediatric MDTBs in this region. Most PSTUs lacked pediatric-trained pathologists and radiologists. Correspondingly, gaps in radiographic and pathologic diagnoses were the most common limitations for MDTBs. Greater emphasis on holistic multidisciplinary subspecialty development is needed to advance pediatric solid tumor care in Southeast Asia.

Time course response of inflammatory markers in pediatric appendicitis.

PURPOSE: We aim to evaluate the diagnostic value and time course response of the triple inflammatory markers: white blood cell count (WBC), neutrophil percentage (Neu), and C-reactive protein (CRP) in pediatric acute appendicitis. METHODS: A retrospective review of clinical data pertaining to 1391 patients admitted with suspicion for pediatric appendicitis from 2012 to 2017 was conducted. Triple inflammatory markers were acquired upon admission. Appendicitis was confirmed histologically post-appendectomy. The diagnostic value and time course response of these markers was trended in relation to the duration of abdominal pain on admission. RESULTS: 718 patients had histologically confirmed appendicitis. WBC and Neu demonstrate high sensitivity for early appendicitis at 94.6% and 80.0% at Day 1, while CRP demonstrates highest sensitivity of 97.9% at Day 4. The triple markers had poor overall diagnostic value when interpreted individually, however, had a high combined sensitivity of 99.7% and negative predictive value of 98.7% regardless of duration of disease. Overall negative appendectomy rate was 6.7% (n = 52). Among 19 patients with triple markers negative who underwent appendectomy, 17 (89.5%) were histologically normal. CONCLUSIONS: The triple inflammatory markers have limited diagnostic value when interpreted individually, but are strong discriminators of pediatric appendicitis when combined. Their high sensitivity and negative predictive value could potentially help patients avoid unnecessary admissions or costly imaging studies, and reduce negative appendectomy rates. In addition, their objective nature confers an advantage over existing clinical scoring systems which comprise subjective elements.

A simple classification of peritoneal contamination in perforated appendicitis predicts surgery-related complications.

AIM: Perforated appendicitis has poorer clinical outcomes compared to non-perforated appendicitis. However, accurate outcome comparisons in research and clinical audits are challenged by its wide spectrum of manifestation. Previous attempts at the classification of severity have been complex and difficult to reproduce. In our study, we used another institution's (Jones et al., TX, USA) previously described simple classification system of peritoneal contamination and examined its usefulness in predicting outcomes. METHODS: With ethical approval, we retrospectively reviewed the records of all paediatric patients operated at our institution for perforated appendicitis from 2016 to 2017. Patient demographics, intra-operative and histological findings, post-operative outcomes and length of stay were collected. Patients were categorised into group 1 (purulence in right lower quadrant only) and group 2 (contamination in two or more quadrants). Post-operative complications were defined as procedure-related (e.g. post-operative ileus, intra-abdominal abscess, visceral injury) and non-procedure-related (e.g. bronchospasm). Statistical analysis using χ2 tests for categorical data and Mann-Whitney U-tests for non-parametric continuous variables was performed, with a significance of P < 0.05. RESULTS: There were 134 eligible patients. We excluded 19 with incomplete data, leaving 115 for analysis, of which 69 (60%) were in group 2. Those in group 2 had a longer stay (P = 0.005) and more post-operative complications (P = 0.001), particularly procedure-related events (P = 0.006). There were no differences in age (P = 0.182), gender (P = 0.876), readmission rate (P = 0.317) and non-procedure-related post-operative complications (0.152). CONCLUSION: This simple classification of perforated appendicitis appears to differentiate clinical outcomes well, particularly for iatrogenic morbidity, making it useful for operative preparation and outcomes research.

Demographic factors affecting parental attitudes to clinical research in pediatric surgery: A pilot study.

PURPOSE: The purpose of this study was to evaluate demographic factors impacting parental attitudes to clinical research in pediatric surgery. METHODS: A prospective survey administered to caregivers accompanying children (pediatric surgical outpatient clinic or day surgery) using convenience sampling (September-November 2017) using a previously published survey with Likert scale was performed. Questions included demographics, parental willingness to enroll children in specified types of research, and beliefs regarding conduct of research. RESULTS: Eighty-four parents were surveyed (100 approached). No demographic factors significantly predicted research participation involving sample collection (urine, saliva, blood) or research requiring follow-up. However, mothers were less likely to agree to studies using common medications (p = 0.049) or common surgical procedures (p = 0.013) and less likely to agree to randomization involving surgery (assigning to common surgical procedure, p = 0.013; surgery vs no surgery, p = 0.031). University graduates were less likely to agree to randomization to surgery vs no surgery (p = 0.02). Beliefs regarding conduct of research were similar, except that non-university graduates were more likely to believe that privacy would be compromised (p = 0.003). Boys were deemed less likely to be too sick for participation (p = 0.03) and more likely to want to participate (p = 0.03). CONCLUSION: Behavioral and attitude differences in caregivers can inform strategies for recruitment among researchers. Impact of caregiver and child gender on responses requires further evaluation. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level II.

STING versus HIT technique of endoscopic treatment for vesicoureteral reflux: A systematic review and meta-analysis.

AIM: Our study aimed to compare the efficacy of two endoscopic techniques used for the correction of vesicoureteral reflux (VUR): subureteral transurethral injection (STING) and hydrodistension implantation technique (HIT). METHODS: A systematic review was conducted using MEDLINE, Google scholar, and Cochrane databases from 1984 to 2015. Meta-analysis of the selected studies was performed to compare the extent of reflux resolution following both techniques. RESULTS: Six observational studies met the inclusion criteria for content. These comprised 632 ureters treated by STING and 895 ureters treated by HIT procedure. All included studies utilized dextranomer/hyaluronic acid (Deflux) as the bulking agent. The overall resolution of VUR was significantly higher in HIT (82.5%) compared to STING (71.4%) [pooled odds ratio (OR)=0.54; 95% confidence interval (CI) 0.42-0.69; P<0.0001; I2=8%]. A subgroup analysis showed that HIT had better outcomes than STING for both lower grade (I-III) [OR=0.43; 95% CI 0.23-0.82; P=0.01; I2=0%] and high-grade VUR (IV-V) [OR=0.43; 95% CI 0.20-0.91; P=0.03; I2=0%]. However, there was no statistical difference in the requirement of additional injections between STING and HIT groups. CONCLUSION: HIT is superior to STING technique for resolution of VUR after Deflux injection. However, more randomized trials with longer follow-up are necessary to demonstrate the benefit of HIT compared to STING procedure. LEVEL OF EVIDENCE: Retrospective comparative studies - level III.

Undescended testis: Level of knowledge among potential referring health-care providers.

AIM: Studies report that most boys with undescended testis(UDT) are referred and operated beyond the recommended age of 1 year, possibly due to lack of awareness of treatment guidelines. We investigate the level of knowledge of UDT among potential referring health-care providers. METHOD: We devised a survey on the clinical features and appropriate management of UDT. Using convenience sampling, we approached health-care professionals with regular contact with paediatric patients and final year medical students. Respondents were allowed to remain anonymous. They were categorised according to specialty and level of experience/training. RESULTS: Of 1179 approached, 203 responded. Thirty-six (24%) of 149 qualified doctors had never seen a case of UDT. Median score was 6 (range 1-9). There was no significant difference in scores when comparing specialty. Mean scores decreased significantly in trend according to level of experience. When questioned regarding timings of referral and orchidopexy, 24% of qualified doctors would not refer until 9 months of age, and 66% thought orchidopexy should be done after 1 year old. Half would stop examining for UDT after 2 years old. CONCLUSIONS: Inexperience with UDT and outdated knowledge may contribute to delays in referral for UDT. Many would stop examining for UDT at 2 years old, placing undue reliance on accurate physical examination in early childhood and indicating lack of awareness of the ascending testis. Community health initiatives must emphasise recent changes in guidelines for management of UDT.

Undescended testis: 513 patients' characteristics, age at orchidopexy and patterns of referral.

OBJECTIVE: Undescended testis (UDT) affects 1-6% of males. Current recommendations are to correct maldescent by 1 year of age. We identify the population characteristics of children referred and managed for UDT, age at referral and orchidopexy, and patterns of referral. DESIGN, SETTING AND PATIENTS: Retrospective 5-year review of all patients operated for UDT from 2007 to 2011 in our institution. Patient demographics, neonatal diagnosis of UDT, age at referral, referral source and age at first orchidopexy were recorded. Data are reported as median (range). RESULTS: There were 513 boys with 576 undescended gonads; 450 (88%) had unilateral UDT. Congenital (present at birth) UDT was diagnosed in 287 (56%) children. Seventy-nine (15%) were premature births, 41 (8%) had associated major genitourinary abnormalities. Median age at referral was 1.1 (0-16.2) years; median age at first orchidopexy was 1.6 (0-17.2) years. When corrected for age, those with a history of prematurity and associated major genitourinary malformations were referred and operated on earlier. There was no difference in age at referral and orchidopexy when comparing unilateral versus bilateral maldescent, and palpability of UDT. Of those with congenital UDT, 70% were operated at beyond 1 year of age. Those referred from public tertiary hospitals were younger than those referred from community clinics (p<0.0001) and private healthcare institutions (p=0.003). CONCLUSIONS: Despite early diagnosis in many patients with UDT, most are referred and operated after 1 year of age, even in congenital UDT. Premature babies, those with major genitourinary anomalies, and those seen in public tertiary hospitals are referred earlier. Community health initiatives must emphasise prompt referral to allay the impact of delayed surgery.

Transanal endorectal pull-through versus transabdominal approach for Hirschsprung's disease: a systematic review and meta-analysis.

AIM: Transanal endorectal pull-through (TERPT) has become popular for single-stage treatment of Hirschsprung's disease. The benefits of TERPT over the conventional transabdominal approach (TAB) are still unclear. We performed a comprehensive meta-analysis comparing the clinical outcomes of TERPT and TAB. METHODS: Original articles published from 1998 to 2012 were searched from Medline, Embase, and Cochrane databases. Randomized controlled trials (RCT) and observational clinical studies (OCS) comparing TERPT and TAB were included. Outcomes evaluated included operative time, hospital stay and incidence of postoperative incontinence/soiling, constipation and enterocolitis. Pooled odds ratios (OR) were calculated for dichotomous variables; pooled mean differences (MD) were measured for continuous variables. RESULTS: Of 93 studies, 1 RCT and 11 OCS were included, comprising 444 cases of TERPT and 348 cases of TAB (215 Soave, 94 Duhamel, 24 Swenson, 15 Rehbein procedures). TERPT had shorter operative time (MD=-57.85 min; 95% confidence interval [CI], -83.11 to -32.60; P<0.00001) and hospital stay (MD=-7.06 days; 95% CI, -10.95 to -3.16; P=0.0004). TERPT had less postoperative incontinence/soiling (OR=0.58; 95% CI 0.37-0.90; P=0.01) and constipation (OR=0.49; 95% CI 0.30-0.81; P=0.005). There was no difference in incidence of postoperative enterocolitis. CONCLUSION: TERPT is superior to TAB in operative time, hospital stay, postoperative incontinence and constipation. However, more randomized controlled trials are necessary to verify the benefit of TERPT for Hirschsprung's disease.

Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification.

PURPOSE: We evaluated the incidence of congenital anomalies associated with anorectal malformations (ARMs) in relation to the anatomic type of ARM as defined by the Krickenbeck classification. METHODS: We reviewed 99 children with ARM in our institution from 2002 to 2011. Data were collected on patient demographics, type of ARM, and associated congenital anomalies, which were categorized according to organ systems. Statistical analysis was performed for comparison between groups using 'perineal fistula' as the base group. RESULTS: There were 62 (63%) male patients. The majority had perineal fistulas (35, 35%). Seventy-seven (78%) had at least one associated malformation. The most frequent malformations seen were genitourinary (28, 28%) and spinal anomalies (26, 26%). Those with rectovesical fistula had the highest proportion of genitourinary malformations (Odds Ratio [OR], 41.3; 95% confidence interval [CI], 4.7-363.4). Those with cloaca (OR, 49.5; 95% CI, 3.4-718.9) and those with rectovestibular fistula (OR, 12.4; 95% CI, 2.3-65.6) were most likely to have major spinal abnormalities, with tethered cord seen in all groups. The rectovestibular group was also most likely to have other associated malformations (OR, 8.6; 95% CI, 2.2-32.8). CONCLUSION: More than 75% of children with anorectal malformation have other associated malformations. Genitourinary anomalies are the most common. Major spinal anomalies are seen in all groups, affecting nearly half of those with rectovestibular fistula and those without fistula. The incidence of associated malformations in the rectovestibular group is higher than described in the literature. Thorough systematic evaluation of all infants with ARM should be done regardless of type of ARM.

The use of octyl cyanoacrylate (superglue) in hypospadias repair including its use as a fixator for urethral stents.

BACKGROUND: Octyl cyanoacrylate has been used for many years for simple skin closure, but its use in hypospadias repair and as a urethral stent fixator has not been previously reported. We report our experience. METHOD: A retrospective study was performed of all children undergoing hypospadias surgery from July 2007 to July 2011. Octyl cyanoacrylate was used for skin closure after placing a few sutures to align the skin edges. No other dressing was used. A urethral stent, if used, was glued to the penis and removed after one week. RESULTS: Seventy two procedures were performed on 37 patients. Eight distal hypospadias (22%) underwent single stage repair. Seventeen (46%) underwent staged repair for severe hypospadias, and twelve (32%) "hypospadiac cripples" underwent various salvage procedures. One patient (1/72) had partial dehiscence, and one stent dislodged. There were no wound infections. Four (4/25) tabularized urethroplasties developed a fistula (16%). CONCLUSION: Octyl cyanoacrylate is easy to apply on difficult hypospadias contours. It forms a strong, waterproof adhesive bond which separates from epithelial surfaces in 7 to 10 days and makes it an effective urethral stent fixator and an ideal dressing for hypospadias surgery.

Duhamel pull-through for Hirschsprung disease: a comparison of open and laparoscopic techniques.

PURPOSE: Various pull-through techniques, both open and laparoscopic, have been performed for Hirschsprung disease. Our study compares open and laparoscopic Duhamel pull-through. METHODS: After ethical approval, we reviewed all children (n = 181) with Hirschsprung disease admitted to our institution between 1999 and 2009. We excluded total colonic aganglionosis (n = 14), previous pull-through done elsewhere (n = 33), or follow-up performed abroad (n = 58). Open and laparoscopic pull-through were done in the same period according to surgeon preference. Data were analyzed using χ(2) or Mann-Whitney U test. RESULTS: Seventy-six children had a Duhamel pull-through for rectosigmoid aganglionosis. Operative time, time to full feeds, and length of hospital stay were similar in each group. OPEN (N = 41): Fifteen children (37%) required 33 further procedures. Fourteen had procedures for persistent constipation, including redo Duhamel (n = 2), stoma formation (n = 2), spur division (n = 2), and dilatation/stretch/Botox/rectal biopsy/manual evacuation (n = 23). Three children had other procedures (adhesiolysis [n = 2] and incisional hernia repair [n = 1]). LAPAROSCOPIC (N = 35): Fourteen children (40%) required 30 further procedures. Eleven had procedures for persistent constipation, including redo Duhamel (n = 1), stoma formation (n = 4), spur division (n = 9), and dilatation/stretch/rectal biopsy (n = 8). Three children had other procedures (adhesiolysis [n = 1] and incisional hernia repair [n = 2]). There were 4 conversions. CONCLUSION: Open and laparoscopic Duhamel pull-through have similar outcomes. We show that the techniques have comparable operative times and hospital stay.

Laparoscopic localization and microlaparotomy for focal isolated perforation in necrotizing enterocolitis: an alternative approach to a challenging problem.

BACKGROUND: Necrotizing enterocolitis has a wide clinical spectrum of manifestation. We report a novel method of managing focal isolated perforation in necrotizing enterocolitis by using diagnostic laparoscopy to localize the site of perforation and by making a microincision over the perforation to perform exteriorization or limited resection and primary anastomosis. METHODS: We included low-birth weight infants presenting with sudden clinical deterioration and pneumoperitoneum. Patients with gross abdominal wall signs were excluded on the probability that they had extensive disease. Diagnostic laparoscopy was performed using a 3.5-mm trocar and 1.9-mm telescope. A 1-cm incision was made over the site of perforation, and local surgical debridement was performed. RESULTS: There were 3 extremely low-birth weight patients (580, 700, and 780 g) and 1 larger infant (1.6 kg). In all cases, an isolated perforation was detected. None had widespread disease. The 3 smaller infants had exteriorization and enterostomies. The larger patient had resection and primary anastomosis. All patients recovered uneventfully. CONCLUSION: Diagnostic laparoscopy can be safely performed in extremely low-birth weight infants. It allows precise identification of the site of perforation to perform a limited microlaparotomy at this site, significantly reducing the surgical trauma of extensive bowel handling. We report a novel method of managing this vexing problem.

The predictive value of preoperative fluorine-18-L-3,4-dihydroxyphenylalanine positron emission tomography-computed tomography scans in children with congenital hyperinsulinism of infancy.

BACKGROUND/PURPOSE: In congenital hyperinsulinism (CHI) of infancy, the use of preoperative fluorine-18-L-3,4-dihydroxyphenylalanine-positron emission tomography-computed tomography ((18)F-DOPA-PET-CT) scan has recently been reported. The aim of this study was to evaluate the accuracy of this technique in discriminating between diffuse and focal CHI and the anatomical localization of focal lesions. METHODS: Between 2006 and 2010, (18)F-DOPA-PET scan was performed in 19 children with CHI (median age, 2 months; range, 1-12 months) who were not responding to medical therapy and underwent laparoscopic or open surgery. The findings of (18)F-DOPA-PET scan were correlated with histology. RESULTS: In 5 children, (18)F-DOPA-PET scan showed diffuse pancreatic uptake, confirmed at histology and supporting the genetic suspicion of diffuse disease. In 14 children, (18)F-DOPA-PET scan indicated focal pancreatic uptake, which corresponded to histology. However, in 5 patients (36%), (18)F-DOPA-PET scan was inaccurate in defining the location of the lesion (n = 3), size of the lesion (n = 1), or both location and size (n = 1), leading to an inaccurate pancreatic resection. CONCLUSIONS: Fluorine-18-L-3,4-dihydroxyphenylalanine-positron emission tomography-computed tomography scan discriminates between diffuse and focal forms of CHI. In focal forms, (18)F-DOPA-PET scan is useful in 2/3 of patients in defining the site and dimension of the focal lesion. Intraoperative histologic confirmation of complete focal lesion resection is needed.

Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TOF) can be repaired thoracoscopically, but this may cause hypercapnia, acidosis, and reduced cerebral oxygenation. We evaluated the effect of thoracoscopy in infants on cerebral oxygen saturation (cSO(2)), arterial blood gases, and carbon dioxide (CO(2)) absorption. METHODS: Eight infants underwent thoracoscopy (6 CDH and 2 EA/TOF). Serial arterial blood gases were taken. Regional cSO(2) was measured using near-infrared spectroscopy. Absorption of insufflated CO(2) was calculated from exhaled (13)CO(2)/(12)CO(2) ratio measured by mass spectrometry. RESULTS: CO(2) absorption increased during thoracoscopy with a maximum 29% ± 6% of exhaled CO(2) originating from the pneumothorax. Paco(2) increased from 9.4 ± 1.3 kPa at the start to 12.4 ± 1.0 intraoperatively and then decreased to 7.6 ± 1.2 kPa at end of operation. Arterial pH decreased from 7.19 ± 0.04 at the start to 7.05 ± 0.04 intraoperatively and then recovered to 7.28 ± 0.06 at end of operation. Cerebral hemoglobin oxygen saturation decreased from 87% ± 4% at the start to 75% ± 5% at end of operation. This had not recovered by 12 (74% ± 4%) or 24 hours (73% ± 3%) postoperatively. CONCLUSIONS: This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO(2). The effects of these phenomena on future brain development are unknown.

Surgical management of congenital hyperinsulinism of infancy.

Congenital hyperinsulinism of infancy (CHI) is characterized by inappropriate insulin secretion resulting in persistent hypoglycemia, which can lead to irreversible severe neurological damage in the infant. Many patients with CHI will respond to medical therapy, but surgery is necessary in those that do not. There are 2 main histologic subtypes, diffuse and focal, both of which may require different surgical strategies. Near-total pancreatectomy is the procedure of choice for diffuse CHI, whereas a localized resection is curative in focal CHI. Open surgery is the traditional approach to pancreatic resection. However, laparoscopy is increasingly used, particularly in localized resection for focal disease. We describe both methods of pancreatectomy.