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.

Fluoroscopic balloon dilation of esophageal atresia anastomotic strictures in children and young adults: single-center study of 103 consecutive patients from 1999 to 2011.

PURPOSE: To determine whether fluoroscopic balloon dilation (FBD) is a safe and effective method of treating esophageal anastomotic stricture after surgical repair in an unselected patient population. MATERIALS AND METHODS: With ethics committee approval, records for 103 consecutive patients who underwent FBD with our interventional radiology service (1999-2011) were reviewed retrospectively. Patients underwent diagnostic contrast material-enhanced study prior to the first dilation. Dilations were performed by using general anesthesia. Outcomes were number and/or frequency of dilations, clinical effectiveness and response to dilations, esophageal perforation, requirement for surgery, and mortality. Data were expressed as mean ± standard deviation (with range). Comparisons were conducted by using the Fisher exact test and log-rank test. The significance level was set at P < .05. RESULTS: One hundred three patients (61 male patients, 59%) underwent 378 FBD sessions (median, two dilations per patient; range, 1-40 dilations). The median age at first FBD was 2.2 years (range, 0.1-19.5 years). The balloon catheter diameters ranged from 4 to 20 mm. FBD was successful in 93 patients (90%): 44 (47%) after single dilation and 49 (53%) after multiple dilations. There was no difference in the proportion of patients who required one dilation and were younger than 1 year versus those who were 1 year of age and older (P > .99; odds ratio, 1.07 [range, 0.43-2.66]). Ten patients (10%) required further procedures: Three underwent stent placement, three underwent esophageal stricture resection, and four underwent esophageal reconstruction. Four esophageal perforations (1%) developed after FBD. Antireflux surgery was performed in 18 patients (17%). There were no deaths. CONCLUSION: FBD for anastomotic strictures after esophageal atresia repair is feasible and acceptably safe and provides relief of symptoms in most patients (90%); however, about half require more than one dilation, and surgery is best predicted if more than 10 dilations are required.

Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial.

OBJECTIVE: We aimed to evaluate the effect of thoracoscopy in neonates on intraoperative arterial blood gases, compared with open surgery. BACKGROUND: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be repaired thoracoscopically, but this may cause hypercapnia and acidosis, which are potentially harmful. METHODS: This was a pilot randomized controlled trial. The target number of 20 neonates (weight > 1.6 kg) were randomized to either open (5 CDH, 5 EA/TEF) or thoracoscopic (5 CDH, 5 EA/TEF) repair. Arterial blood gases were measured every 30 minutes intraoperatively, and compared by multilevel modeling, presented as mean and difference (95% confidence interval) from these predictions. RESULTS: Overall, the intraoperative PaCO2 was 61 mm Hg in open and 83 mm Hg [difference 22 mm Hg (2 to 42); P = 0.036] in thoracoscopy and the pH was 7.24 in open and 7.13 [difference -0.11 (-0.20 to -0.01); P = 0.025] in thoracoscopy. The duration of hypercapnia and acidosis was longer in thoracoscopy compared with that in open. For patients with CDH, thoracoscopy was associated with a significant increase in intraoperative hypercapnia [open 68 mm Hg; thoracoscopy 96 mm Hg; difference 28 mm Hg (8 to 48); P = 0.008] and severe acidosis [open 7.21; thoracoscopy 7.08; difference -0.13 (-0.24 to -0.02); P = 0.018]. No significant difference in PaCO2, pH, or PaO2 was observed in patients undergoing thoracoscopic repair of EA/TEF. CONCLUSIONS: This pilot randomized controlled trial shows that thoracoscopic repair of CDH is associated with prolonged and severe intraoperative hypercapnia and acidosis, compared with open surgery. These findings do not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH, calling into question the safety of this practice. The effect of thoracoscopy on blood gases during repair of EA/TEF in neonates requires further evaluation. (ClinicalTrials.gov Identifier: NCT01467245).

Recurrence rate of Morgagni diaphragmatic hernia following laparoscopic repair.

PURPOSE: Morgagni diaphragmatic hernia can be repaired laparoscopically. The aim of this study is to evaluate the outcome of this minimally invasive approach. METHODS: A retrospective review was conducted on all consecutive children who underwent repair of Morgagni hernia from January 2002 to December 2011 in our hospital. Data are expressed as median (range). RESULTS: There were 12 children with Morgagni hernia. Age at surgery was 7.5 months (2-125). Associated malformations were present in 7 children (58 %). All children underwent initial laparoscopic approach. Two children (16 %) underwent conversion to open surgery. The hernia was closed primarily in 11 children (92 %), using a polyester patch in 1 (8 %). There were no intraoperative or immediate postoperative complications. Five children (42 %), all repaired initially without a patch, had a recurrence of the Morgagni hernia. The repair of the recurrent hernia was performed laparoscopically in four out of the five children, and a patch was used in two patients with no further recurrences or complications. CONCLUSIONS: There is a high rate of recurrence after laparoscopic Morgagni hernia repair. This is exclusively associated with laparoscopic repair without patch, and it is in contrast with the low recurrence rate reported previously. More frequent use of patch may be beneficial.

Cost-effectiveness of laparoscopic versus open pyloromyotomy.

BACKGROUND: Infantile hypertrophic pyloric stenosis can be corrected by either open (OP) or laparoscopic pyloromyotomy (LP). LP may provide clinical benefits of reduced time to postoperative full feeds and reduced postoperative inpatient stay, but the cost effectiveness is not known. Our aim was to compare the cost effectiveness of laparoscopic and open pyloromyotomy. METHODS: OP and LP were compared in a multicenter randomized double-blind controlled trial, for which the primary outcomes were time to full feeds and time to discharge. In order to undertake a detailed cost analysis, we assigned costs, calculated on an individual patient basis, to laboratory costs, imaging, medical staff, medication, ward, operative, and outpatient appointments for 74 patients recruited from one of the participating centers. Data (mean ± SEM) were compared using linear regression analysis, adjusting for the minimization criteria used in the trial. RESULTS: Operation costs were similar between the two groups ($3,276 ± $244 LP versus $3,535 ± $152 OP). A shorter time to full feeds and shorter hospital stay in LP versus OP patients resulted in a highly significant difference in ward costs ($2,650 ± $126 LP versus $3,398 ± $126 OP; P = .001) and a small difference in other costs. Overall, LP patients were $1,263 (95% confidence interval $395-$2,130; P = .005) less expensive to treat than OP patients. Sensitivity analyses of laparoscopic hardware usage and of incomplete pyloromyotomy indicated that LP was consistently less expensive than OP. CONCLUSIONS: LP is a cost-effective alternative to OP as it delivers improved clinical outcome at a lower price.

Outcomes following laparoscopic inguinal hernia repair in infants compared with older children.

PURPOSE: Indications for laparoscopic inguinal hernia repair in infants and children remain controversial. The purpose of this study is to compare clinical features and outcome of laparoscopic inguinal hernia repair in infants with older children. METHODS: Retrospective single centre review of all patients <16 years of age (n = 380) undergoing laparoscopic inguinal hernia repair over a 5-year period (Jan 2005-Dec 2009). Outcomes were compared between infants (≤ 12 months of age) with older children (1-15 years). RESULTS: There was a trend towards higher recurrence rate in older children than in infants (4 % vs. 1 %; p = 0.17). Total complications and complications requiring surgery were similar in both age groups. There was one testicular atrophy in an infant who had an incarcerated inguinal hernia. The incidence of bilateral inguinal hernia and contralateral patent processus vaginalis (PPV) were both significantly higher in infants (total 61 % compared with 35 % in older children). CONCLUSIONS: Laparoscopic inguinal hernia repair in infants is safe and carries acceptable complication and recurrence rates. The laparoscopic approach may be particularly beneficial in infants since it allows simultaneous closure of a contralateral PPV and bilateral herniae. The outcome of laparoscopic inguinal hernia repair in older children requires further evaluation.

Clinical outcome of a randomized controlled blinded trial of open versus laparoscopic Nissen fundoplication in infants and children.

OBJECTIVE: To compare the clinical outcome and endocrine response in children who were randomized to open or laparoscopic Nissen fundoplication using minimization. BACKGROUND: It is assumed that laparoscopic surgery is associated with less pain, quicker recovery and dampened endocrine response. Few randomized studies have been performed in children. METHODS: Parents gave informed consent, and this study was approved and registered (ClinicalTrials.gov Identifier: NCT00231543). Anesthesia, postoperative analgesia and feeding were standardized. Parents and staff were blinded to allocation. Blood was taken for markers of endocrine response. RESULTS: Twenty open and 19 laparoscopic patients were comparable with respect to age, weight, neurological status, and presence of congenital anomalies. Median time to full feeds was 2 days in both groups (P = 0.85); hospital stay was 4.5 days in the open group versus 5.0 days in the laparoscopic group (P = 0.57). Pain was adequately managed in both groups and there was no difference in morphine requirements. Median follow-up was 22 (range 12-34) months. Dysphagia, recurrence and need for redo fundoplication were not different between groups; retching was higher after open surgery (56% vs. 6%; P = 0.003). Insulin levels decreased at 24 hours, and was 54% lower (P = 0.02) after laparoscopy. Cortisol was elevated immediately postoperative, but was 42% lower (P = 0.02) after laparoscopy. CONCLUSIONS: There was no difference in the postoperative analgesia requirements and recovery. Laparoscopy decreased insulin levels to a greater extent, but caused less of a response in cortisol. Early postoperative outcome confirmed equal efficacy, but fewer children with retching after laparoscopy.

Aortopexy as treatment for tracheo-bronchomalacia in children: an 18-year single-center experience.

OBJECTIVES: To define the factors influencing the outcome of aortopexy as management of tracheo-bronchomalacia. DESIGN: A retrospective, single-center, observational, cohort study. SETTINGS: Surgical services in a tertiary care hospital. PATIENTS: One hundred five children who underwent an aortopexy for tracheo-bronchomalacia between 1990 and 2008. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Comorbidity (categorized into three groups), surgical approach, and location of malacia were reviewed and analyzed in relation to mortality, need for a second intervention, complications, time to extubation after surgery, intensive care unit stay, and clinical improvement. Median age at surgery was 24 wks (1 wk-541 wks). Two surgical approaches were used: median sternotomy (n = 46) and left anterior parasternal (n = 59). Long-term (>2 yrs) follow-up was available for 73 patients (median = 7.0 yrs [2-18 yrs]); 73% were asymptomatic, 18% had minor symptoms, and 9% needed either ventilation or tracheostomy. The overall mortality rate was 9%, of which one-third was airway-related. Multivariable analysis revealed that major comorbidities were a significant risk factor both for mortality and the need for further procedures (re-do surgery, tracheostomy, internal stents) in contrast to surgical approach and involvement of the bronchus. Intensive care unit stay and days of ventilation after surgery were also significantly higher in patients with major comorbidities. CONCLUSIONS: Aortopexy proved to be an effective treatment for most cases of tracheo-bronchomalacia, but major comorbidity was associated with an adverse outcome. Our data suggest that aortopexy should be considered in most cases of severe tracheo-bronchomalacia.

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.

Peritoneal drainage or laparotomy for neonatal bowel perforation? A randomized controlled trial.

OBJECTIVE: To determine whether primary peritoneal drainage improves survival and outcome of extremely low birth weight (ELBW) infants with intestinal perforation. SUMMARY BACKGROUND DATA: Optimal surgical management of ELBW infants with intestinal perforation is unknown. METHODS: An international multicenter randomized controlled trial was performed between 2002 and 2006. Inclusion criteria were birthweight >or=1000 g and pneumoperitoneum on x-ray (necrotizing enterocolitis or isolated perforation). Patients were randomized to peritoneal drain or laparotomy, minimizing differences in weight, gestation, ventilation, inotropes, platelets, country, and on-site surgical facilities. Patients randomized to drain were allowed to have a delayed laparotomy after at least 12 hours of no clinical improvement. RESULTS: Sixty-nine patients were randomized (35 drain, 34 laparotomy); 1 subsequently withdrew consent. Six-month survival was 18/35 (51.4%) with a drain and 21/33 (63.6%) with laparotomy (P = 0.3; difference 12% 95% CI, -11, 34%). Cox regression analysis showed no significant difference between groups (hazard ratio for primary drain 1.6; P = 0.3; 95% CI, 0.7-3.4). Delayed laparotomy was performed in 26/35 (74%) patients after a median of 2.5 days (range, 0.4-21) and did not improve 6-month survival compared with primary laparotomy (relative risk of mortality 1.4; P = 0.4; 95% CI, 0.6-3.4). Drain was effective as a definitive treatment in only 4/35 (11%) surviving neonates, the rest either had a delayed laparotomy or died. CONCLUSIONS: Seventy-four percent of neonates treated with primary peritoneal drainage required delayed laparotomy. There were no significant differences in outcomes between the 2 randomization groups. Primary peritoneal drainage is ineffective as either a temporising measure or definitive treatment. If a drain is inserted, a timely "rescue" laparotomy should be considered. Trial registration number ISRCTN18282954; http://isrctn.org/

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.

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.

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.

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.

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.

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.

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.