Outcomes in adulthood of gastric transposition for complex and long gap esophageal atresia.

BACKGROUND: Long term outcomes of gastric transposition (GT) for complex esophageal atresia (EA) are poorly reported. We aimed to perform comprehensive long term follow up of adults who had been treated with GT for EA as children. METHODS: Consecutive patients who underwent GT for EA in childhood aged >18 years old were identified alongside age matched patients who had primary repair (PR). Type of EA, comorbidities and details of surgery were recorded. Telephone interviews included medical history, current symptoms - including gastrointestinal symptom rating scale (GSRS), morbidity and health related quality of life (HRQoL) using gastrointestinal quality of life index (GIQLI). RESULTS: 32 participants were interviewed in each group (mean age 29 years). BMI (19.9 ±â€¯3.5) was significantly lower (p = 0.0006) in GT group. 6/32 (19%) still required supplementary feeding. Adult morbidity included anastomotic stricture (34%), chronic respiratory disease (28%), dumping symptoms (25%), anemia (47%) and depression (19%). 3 patients required major revision surgery. Participants in both groups report regular upper gastrointestinal symptoms (GSRS: GT = 2.1, PR = 2.0) and were more symptomatic than the normal population (1.4) but not statistically different from each other. HRQoL (GIQLI = 113) was lower than after PR (122) but not significantly different (p = 0.29) and the normal population (125). 23% of GT participants had higher than normal HRQoL. CONCLUSIONS: GT for EA is associated with significant morbidity and symptoms, including issues previously unreported in adulthood such as mental health problems. This mandates long term follow up and quality transition of these patients into adult care. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level III.

Loop colostomies are safe in anorectal malformations.

AIM OF THE STUDY: Divided colostomy (DC) has been recommended in anorectal malformations (ARMs) with previously reported advantages of decreasing overflow into the distal limb and urinary tract infections (UTIs). Skin bridge loop colostomy (LC) is a technically easier alternative without an increase in these complications. We report our institutional experience of LC in ARM. METHODS: Retrospective study (Institution-approved Clinical Audit) reviewing the clinical records of all patients with ARM undergoing stoma formation in a single UK tertiary pediatric surgical center (2000-2015). Data collected included type of ARM, associated anomalies, type and level of colostomy, time to stoma closure, complications and UTIs. RESULTS: One hundred and eighty-two (95 female) patients underwent colostomy formation for ARM. The vast majority (171/ 94%) underwent LC; 9 (5%) had a divided colostomy (DC) and 2 (1%) had no available data. The spectrum of defects in girls included rectovestibular (62/65%), rectovaginal (4/4%) and cloaca (29/31%). In boys, 71 (82%) had a fistula to the urinary tract and 16 (18%) presented with a perineal fistula. Urological abnormalities coexisted in 87 (47.8%) patients. Thirty five (21%) patients developed UTIs. Among the 19 girls who developed UTI, 8 had rectovestibular fistula and 11 had cloaca. Of the 16 boys who developed UTI, 14 had a fistula to the urinary tract and 11 had an independent urological abnormality. The mean time from stoma formation to stoma closure was 10 (3-52) months. Complications were reported in 22 (12%) LCs. Fifteen patients (9%) developed a stoma prolapse following LC with 10 (6%) requiring surgical revision. CONCLUSIONS: This is the largest reported series of outcomes following LC for ARM. LC is easier to perform and to close, requiring minimal surgical access, with comparable complications and outcomes to those published for DC. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: III.

Primary versus Staged Closure of Exomphalos Major: Cardiac Anomalies Do Not Affect Outcome.

AIM: The objective of the study is to describe management of exomphalos major and investigate the effect of congenital cardiac anomalies. METHODS: A single-center retrospective review (with audit approval) was performed of neonates with exomphalos major (fascial defect ≥ 5cm ± liver herniation) between 2004 and 2014.Demographic and operative data were collected and outcomes compared between infants who had primary or staged closure. Data, median (range), were analyzed appropriately. RESULTS: A total of 22 patients were included, 20 with liver herniation and 1 with pentalogy of Cantrell. Gestational age was 38 (30-40) weeks, birth weight 2.7 (1.4-4.6) kg, and 13 (60%) were male. Two were managed conservatively due to severe comorbidities, 5 underwent primary closure, and 15 had application of Prolene (Ethicon Inc) mesh silo and serial reduction. Five died, including two managed conservatively, none primarily of the exomphalos. Survivors were followed up for 38 months (2-71). Cardiac anomalies were present in 20 (91%) patients: 8 had minor and 12 major anomalies. Twelve (55%) patients had other anomalies. Primary closure was associated with shorter length of stay (13 vs. 85 days, p = 0.02), but infants had similar lengths of intensive care stay, duration of parenteral feeds, and time to full feeds. Infants with cardiac anomalies had shorter times to full closure (28 vs. 62 days, p = 0.03), but other outcomes were similar. CONCLUSION: Infants whose defect can be closed primarily have a shorter length of stay, but other outcomes are similar. Infants with more significant abdominovisceral disproportion are managed with staged closure; the presence of major cardiac anomalies does not affect surgical outcome.

Long-term outcomes of Heller's myotomy and balloon dilatation in childhood achalasia.

Achalasia is a rare esophageal motility disorder: its optimal treatment in children is still a matter of debate. Records of children treated for achalasia, over an 18-year period, were reviewed.Forty-eight children (median age at diagnosis 10 years; range 3-17 years) were identified. Twenty-eight patients were initially treated with Heller's myotomy (HM) and 20 with balloon dilatation (BD). At last follow-up (median 3 years; range 1-5.5 years), 43.8% (21/48) of children were symptom free. The number of asymptomatic children was significantly higher among those treated initially with HM compared to BD (HM 15/28, 53.6% BD 6/20, 30%, p < 0.05). All children who underwent BD required HM due to symptom recurrence. The median (range) total number of procedures was significantly higher in the BD group (BD 3 (1-7); HM 1 (1-5); p < 0.05) with a shorter time to the second intervention (BD 14 months, 95%CI 4-24; HM 58 months, 95%CI 38-79; p < 0.05). Of 108 procedures, esophageal perforation occurred in two children after HM (two out of 48 HM procedures in total, 4%) and one child after BD (1/60, 1.7%). CONCLUSION: Less than half of children with achalasia are symptom free after initial treatment with either BD or HM. HM, however, when performed as first procedure, provided longer symptom-free period and reduced need for subsequent intervention. What is Known: • Balloon dilatation (BD) and Heller's myotomy (HM) are safe and effective treatment options for achalasia. • Controversy, however, exists regarding the most effective initial therapeutic approach. What is New: • HM with or without fundoplication may represent the initial therapeutic approach of choice. • Initial BD may negatively affect the outcome of a subsequent HM.

Oesophageal atresia is correctable and survivable in infants less than 1 kg.

INTRODUCTION: Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of <1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients. METHODS: A retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality. RESULTS: Of 349 patients with OA across the 22-year period, 9 ELBW patients were identified (<1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died-two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died-one from cardiac disease the others from conditions indicative of their prematurity-necrotising enterocolitis and intraventricular haemorrhage. CONCLUSIONS: 50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants <800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes.

The extramucosal interrupted end-to-end intestinal anastomosis in infants and children; a single surgeon 21year experience.

BACKGROUND/PURPOSE: To report outcomes of a standardised technique for intestinal anastomosis in infants and children. METHODS: Data were prospectively collected on all paediatric intestinal anastomosis by a single surgeon over a 21year period. Anastomoses were constructed using an end-to-end extramucosal technique with interrupted polypropylene sutures. Demographic and clinical data were recorded. RESULTS: Six-hundred and thirteen anastomoses were constructed in 550 patients. Median age at time of anastomosis was 6months (range 1day-226months). The most common reason for anastomosis was stoma closure (n=271, 49%). For those patients that required multiple anastomoses the most common pathology was acute NEC (n=22/41, 54%). One-hundred and one (18.4%) patients passed stool within 24hours of surgery, 175 (31.8%) between 24-48 hours and 95 (17.3%) between 48-72 hours. Anastomotic complications occurred in 7 patients (1.3%) including anastomotic leakage (n=5, 0.9%) and anastomotic stricture (n=2, 0.4%). The majority of anastomotic leakages (80%) followed resection of acute NEC. CONCLUSIONS: The interrupted extramucosal anastomosis is safe and effective. The return of bowel function is rapid and the complication rate acceptable. We recommend this technique be used for all intestinal anastomoses in children and infants.

Esophageal Atresia: Improved Outcome in High-Risk Groups Revisited.

Objectives Improved survival in infants with esophageal atresia (EA) with a birth weight < 1,500 g or a major cardiac anomaly has been reported when compared with the original Spitz classification proposed in 1994. Aim We reviewed outcome data for infants born over the last decade in our institution to update previously reported survival statistics. Materials and Methods The records of all neonates (n = 200) with a diagnosis of EA managed in a single institution between 2001 and 2011 were reviewed and compared with data from the original Spitz study and the subsequent reported cohort from the same institution. Data were obtained on birth weight, presence of a major cardiac anomaly, and survival. Differences in survival were compared using the Yates-corrected chi-square test. Local ethical study approval was obtained. Results Infants born over the last decade had a comparable overall survival rate of 93% (186/200) versus 92.6% (174/188) in the previously reported cohort (1993-2004). We demonstrate an improved survival as compared to the Spitz cohort (87.6%, 326 /372, p = 0.06) and a statistically significant improvement in survival in Group II (p = 0.01). Within this group, 12/51 neonates had a birth weight < 1,500 g and 39/51 had major cardiac anomalies. Of interest, of the nine deaths in Group II, eight were in the subgroup with major cardiac anomalies. Conclusion The survival of neonates in Group II has significantly improved. Mortalities within this group were predominantly in the subgroup with major cardiac anomalies suggesting birth weight is of less significance than in previous years reflecting recent advances in neonatal care. We propose an updated prognostic classification that makes a distinction between cardiac and low-birth-weight infants.

Early reported rectal sensation predicts continence in anorectal anomalies.

BACKGROUND: Straining at stool is an automatic reflex in babies and implies the presence of rectal sensation. We hypothesised that early reported rectal sensation would predict future continence in children with anorectal anomalies. AIM OF THE STUDY: The aim of this study is to determine if early straining at stool was a useful predictor of future continence in infants born with high anorectal malformations. METHODS: A retrospective case note review of prospectively collected clinical information was performed with institutional review board approval. All patients with intermediate/high anorectal malformation operated on by a single surgeon from 1984 to 2010 were included. After stoma closure, parents were asked: The responses were noted within the first year of stoma closure and then all patients were followed up until they were at least 3 ½years old and continence could be assessed using the Krickenbeck outcome classification. Data were compared using Fisher's exact test and sensitivity, specificity and positive predictive value (PPV) were calculated. MAIN RESULTS: Forty-eight patients were included in the study. Sixteen (33%) were female (12 cloacal malformation, 3 rectovaginal fistula, 1 rectal atresia) and 32 (66%) were male (6 rectovesical fistulae, 22 rectourethral fistulae, 4 no fistula). Median follow-up was 9.7years (range 3.5-17.9). Twenty-one children were noted by their parents to exhibit early straining at stool after stoma closure. Twenty of them achieved long term continence. The sensitivity of early straining as a predictor for long term continence was 77%, specificity 95% and positive predictive value 95%. CONCLUSION: The presence of early rectal sensation reported by parents is a good predictor of long term continence. This allows more informed discussion with families in the early years of life.

Classification and clinical evaluation.

Conjoined twins represent a great challenge for most pediatric specialists including pediatric surgeons, anesthetists, neonatologists, urologists, neurosurgeons, and orthopedic surgeons. This anomaly can be classified according to the type of twins׳ fusion. Various organs can be fused making the separation difficult. Conjoined twins are usually diagnosed antenatally by ultrasound. Detailed fetal echocardiography is necessary to counsel the parents during pregnancy. Postnatally, the majority of the conjoined twins can be thoroughly investigated using various imaging techniques. This allows careful planning of the operation. However, in approximately one-third of the patients an urgent operation is required at birth without a complete assessment of the joining. This is associated with a poorer outcome.

Planning the operation.

The management of conjoined twins falls into three distinct groups-non-operative, emergency separation and elective separation. Planning meetings involving all the personnel who will be required during the operation are held. The radiological findings are presented and the anaesthetic, nursing and intensive care requirements are highlighted.

Theatre setup.

The theatre staff would have been made aware of the special requirements for the operation at the planning meetings. Two sets of scrubs nurses will be required but only one set will be needed for the actual separation. The second set of scrub nurses will be needed only when both twins have survived the separation and the second twin is moved to another operating room for closure of the wound.

The separation procedure.

The various stages of the separation are carefully planned but despite this, variations which will change the schedule of the procedure may exist. In general the operation commences on the opposite side from the main procedure and then the twins are turned for the remainder of the operation. Each type of conjoined twin is different but basically thoracopagus involves the hearts, omphalopagus involves the liver and small intestine and ischiopagus involves the large intestine and genito-urinary system. Our results are presented together with interesting cases from which lessons have been learned.

Comparison of minimally invasive and open gastric transposition in children.

BACKGROUND: Gastric transposition is an established method of esophageal replacement in children, and the use of minimally invasive techniques avoids the trauma of open access. The objective of this study was to compare outcomes of minimally invasive versus open gastric transposition in children. MATERIALS AND METHODS: All cases of attempted laparoscopic-assisted gastric transposition at Great Ormond Street Hospital (GOSH), London, United Kingdom, between 2003 and 2012 were retrospectively reviewed. A comprehensive literature search was completed on MEDLINE for minimally invasive gastric transposition in children, and postoperative outcomes were collated. The outcomes from the retrospective review (single-center, GOSH) and the literature search (multicenter) were compared with those of the largest study on open gastric transposition consisting of 192 cases performed at GOSH. RESULTS: In this retrospective review of 19 patients (mean age, 3.5 years; range, 0.4-15 years), the indications were long-gap esophageal atresia, postoperative, caustic, and idiopathic esophageal stricture, and esophageal dysmotility. Three cases were converted to laparotomy and excluded from subsequent analysis. There were one anastomotic leak, two strictures, and no deaths in this series. The literature search yielded a further 50 cases for comparison. Single-center (n=16) and multicenter (n=66) comparison of minimally invasive versus open technique (n=192) showed no difference in leak (6.3% and 16.7%, respectively, versus 12.0%; P=.701 and P=.398), stricture (12.5% and 15.2% versus 20.8%; P=.535 and P=.370), and mortality rates (0% and 1.5% versus 4.7%; P=1.000 and P=.461). CONCLUSION: Minimally invasive gastric transposition is a safe and acceptable alternative to open surgery in children.

Currarino's syndrome in twins presenting as neonatal intestinal obstruction--identical presentation in non-identical twins.

We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.

Measured gap length and outcomes in oesophageal atresia.

AIMS: Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) is the most common congenital anomaly of the oesophagus. There is limited literature suggesting a linear relationship between increasing gap length and the incidence of all major complications. We sought to assess whether measured gap length at the time of surgery was related to outcomes in our patients. METHODS: All patients with a diagnosis of OA +/- TOF who underwent repair under a single surgeon between 1983 and 2012 were included. The length between the oesophageal pouches was measured at the time of surgery. Patients were then divided into three groups; short ≤1cm, intermediate >1-≤2cm and long >2-≤5cm. Outcome measures were anastomotic leak, strictures requiring dilatation, gastrooesophageal reflux disease (GORD) and need for fundoplication. RESULTS: 122 patients were included in the study. The outcomes for patients with short (n=53), intermediate (n=51) and long gaps (n=18) were as follows: anastomotic leak - 1.9%, 2%, 5.5% (P=0.66), strictures requiring dilatation - 32%, 33%, 50% (P=0.67), GORD - 51%, 59%, 72% (P=0.58) and need for fundoplication - 11%, 20%, 44% (*P=0.02). There were no deaths related to the repair. CONCLUSIONS: Measured gap length at the time of surgery did not have a linear relationship with leak or stricture rate. Our experience suggests that when primary repair is possible absolute gap length is irrelevant to the development of post-operative complications. There is however a significant increase in the need for fundoplication in those with a long gap.

The outcome of critically ill neonates undergoing laparotomy for necrotising enterocolitis in the neonatal intensive care unit: a 10-year review.

PURPOSE: To evaluate outcomes in critically ill neonates with necrotising enterocolitis (NEC) undergoing a laparotomy in the neonatal intensive care unit (NICU). METHODS: This is a retrospective review of neonates diagnosed with NEC who underwent a laparotomy on NICU between 2001 and 2011. Demographic, diagnostic, operative and outcome data were analysed. Nonparametric comparison was used. Data are reported as median (range). RESULTS: 221 infants with NEC were referred for surgical evaluation; 182 (82%) underwent surgery; 15 (8%) required a laparotomy on NICU. Five had NEC totalis, 4 multifocal disease and 6 focal disease. Five had an open and close laparotomy, 8 stoma with/without bowel resection and 2 bowel resection and primary anastomosis. Ten (67%) died at a median of 6.5-hours (2-72) postoperatively; 2 died at 72 and 264-days. The 30-day mortality rate was higher (p=0.01) among infants undergoing a laparotomy on NICU (10/15; 67%) than in theatre (54/167; 32%). There was no significant difference in mean Paediatric Index of Mortality 2 Scores between survivors and nonsurvivors (p=0.55). Three (20%) infants remain alive with no or minimal disability at 1.4 (0.5-7.5) years. CONCLUSION: Laparotomy for NEC on NICU is a treatment option for neonates who are too unstable to transfer to theatre. However, with 67% dying within 6.5-hours and a further 13% after months in hospital, we must consider whether surgery is always in their best interests. Development of a prediction model to help distinguish those at highest risk of long-term morbidity and mortality could help with decision making in this difficult situation.

Early experience of thoracoscopic aortopexy for severe tracheomalacia in infants after esophageal atresia and tracheo-esophageal fistula repair.

BACKGROUND: Aortopexy is the most effective treatment for severe tracheomalacia associated with esophageal atresia with distal tracheo-esophageal fistula (EA/TOF). In the last few years, the thoracoscopic approach has been proposed, but the number of patients treated is limited. The purpose of this study is to review our initial experience with thoracoscopic aortopexy. PATIENTS AND METHODS: A retrospective review of medical records was performed on EA/TOF patients undergoing thoracoscopic aortopexy at the Great Ormond Street Hospital for Sick Children (London, United Kingdom) from January 2009 to May 2012. Patient demographics, indication, perioperative course, and long-term results when available were noted. RESULTS: Four patients underwent a successful thoracoscopic aortopexy, with no operation being converted. No morbidity or mortality was associated with the procedure. Length of postoperative stay ranged from 2 to 4 days. All patients were relieved of their symptoms, and no recurrence was noted. CONCLUSIONS: Thoracoscopic aortopexy is a feasible and successful treatment for severe tracheomalacia in EA/TOF patients. The complication rate may be lower than after the open procedure and is more satisfactory in terms of cosmetic appearance. However, we need a larger and prospective study with a longer follow-up to confirm these preliminary results.

Is early delivery beneficial in gastroschisis?

PURPOSE: Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD). METHODS: A retrospective review of gastroschisis (1992-2012) linked fetal/neonatal data at 2 tertiary referral centers was performed. Primary outcomes were ENT and length of hospital stay (LOS). Data (median [range]) were analyzed using parametric/non-parametric tests, positive/negative predictive values, and regression analysis. RESULTS: Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p<0.0001) and LOS (p<0.0001) were reduced with increasing gestational age. IABD persisted to last scan in 92 patients, 68 (74%) simple (intact/uncompromised bowel), 24 (26%) complex. IABD or EABD diameter in complex patients was not significantly greater than simple gastroschisis. Combined IABD/EABD was present in 22 patients (14 simple, 8 complex). When present at <30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from <30weeks. CONCLUSION: Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at <37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis.