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.

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.

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.

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.

Ethics in the management of conjoined twins.

In dealing with conjoined twins, we have adopted a pragmatic approach in balancing what is possible, parental wishes and the likely outcome for the children. We believe that separation where feasible should be undertaken but appreciate that a few parents may wish their children to remain joined. The advice that we give balances the very real risks of surgery against the type of life the twins will experience if they remain joined. In some types of union such as end to end ischiopagus, the twins will never stand and will be bedridden. With other types of union, independent walking is not possible. Such limitations on living seem intolerable to us, but we have left the final decision to parents.

Esophageal replacement: overcoming the need.

Three developments which have contributed to the declining necessity for esophageal replacement are improvement in the management of esophageal atresia, prevention of caustic injuries to the esophagus, and early antireflux surgery for intractable gastro-esophageal reflux. Despite these advances, replacement of the esophagus may still be necessary. The two most commonly used procedures for replacing the esophagus are colonic interposition and gastric transposition. Experience with 236 gastric transposition operations reveals a mortality of 2.5%, leak rate of 12%, and stricture of 20%. The follow-up shows a satisfaction of over 90%. New methods of overcoming the need for esophageal replacement are in progress with tissue engineering with a scaffold to produce a tubular graft to bridge the gap in the continuity of the esophagus.

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.

The history of paediatric surgery in the United Kingdom and the influence of the national health service on its development.

Details of the modern history of paediatric surgery in the United Kingdom with particular emphasis on the 3 main training centres in England in the 1960s to 1970s are discussed. The genesis of the National Health Service and of the British Association of Paediatric Surgeons and their influence on the establishment and sitting of regional centres, education and training, and centralization of rare conditions is highlighted.

Persistent ascites can be effectively treated by peritoneovenous shunts.

AIM: The aim the study was to review our experience and determine the effectiveness of peritoneovenous shunts in the management of persistent ascites. METHODS: A retrospective review of all patients who had a peritoneovenous shunt inserted for persistent ascites in our institution over 32 years (1977-2010) was performed. RESULTS: Thirty-one peritoneovenous shunts were inserted into 25 patients. Records from 22 were available. Median age was 34 months (range, 1 month-12 years), with median duration of ascites of 1.75 months (range, 3 weeks-2.5 years). Sixteen had previous abdominal surgery, whereas one had previous thoracic surgery. Other underlying pathologies included hepatitis (n = 2), lymphohistiocytosis, lymphatic hypoplasia, and carcinomatosis. One was regarded as idiopathic. Previous management consisted of paracentesis (n = 15), dietary modification (n = 11), diuretics (n = 9), and total parenteral nutrition (TPN) (n = 4). One underwent fetal drainage of ascites. No intraoperative complications occurred. Eight (36%) had postoperative complications, including shunt occlusion (n = 2), pulmonary edema (n = 2), infection (n = 2), and wound leakage (n = 1). One developed a varix following shunt removal. The ascites resolved after shunting in 20 (91%) of the 22 children. Four died from their underlying pathology. In children with malignancy, the shunt allowed prompt resumption in treatment schedule. CONCLUSION: This represents the largest series of children receiving peritoneovenous shunt for persistent ascites. It is a safe and effective treatment which should be considered early.

Foregut separation and tracheo-oesophageal malformations: the role of tracheal outgrowth, dorso-ventral patterning and programmed cell death.

Foregut division-the separation of dorsal (oesophageal) from ventral (tracheal) foregut components-is a crucial event in gastro-respiratory development, and frequently disturbed in clinical birth defects. Here, we examined three outstanding questions of foregut morphogenesis. The origin of the trachea is suggested to result either from respiratory outgrowth or progressive septation of the foregut tube. We found normal foregut lengthening despite failure of tracheo-oesophageal separation in Adriamycin-treated embryos, whereas active septation was observed only in normal foregut morphogenesis, indicating a primary role for septation. Dorso-ventral patterning of Nkx2.1 (ventral) and Sox2 (dorsal) expression is proposed to be critical for tracheo-oesophageal separation. However, normal dorso-ventral patterning of Nkx2.1 and Sox2 expression occurred in Adriamycin-treated embryos with defective foregut separation. In contrast, Shh expression shifts dynamically, ventral-to-dorsal, solely during normal morphogenesis, particularly implicating Shh in foregut morphogenesis. Dying cells localise to the fusing foregut epithelial ridges, with disturbance of this apoptotic pattern in Adriamycin, Shh and Nkx2.1 models. Strikingly, however, genetic suppression of apoptosis in the Apaf1 mutant did not prevent foregut separation, indicating that apoptosis is not required for tracheo-oesophageal morphogenesis. Epithelial remodelling during septation may cause loss of cell-cell or cell-matrix interactions, resulting in apoptosis (anoikis) as a secondary consequence.

Surgical outcomes of esophageal atresia without fistula for 24 years at a single institution.

PURPOSE: The aim of the study was to evaluate the surgical outcome of esophageal atresia (EA) without fistula for 24 years at a single tertiary center for pediatric surgery. METHODS: The study used a retrospective chart review of infants diagnosed with EA without fistula between 1981 and 2005. RESULTS: Of 33 patients with EA without fistula, 31 charts were available. Mean birth weight was 2327 g (range, 905-3390 g), and 71% were male. Most common associated anomalies were cardiac (n = 6; 19%) and renal (n = 5; 16%), followed by vertebral (n = 4; 13%) and anorectal (n = 2; 7%). The median initial esophageal gap was 5 vertebral bodies. Six had a primary repair, and 25 patients had esophageal replacement at a median age of 7 months. This involved gastric transposition in 20 (1 followed failed jejunal interposition), colonic interposition in 5, jejunal interposition in 1 (after a failed colonic), and repair at another center in 1. With a median review of 9 years, 21 patients had long-term sequelae with the need for multiple further surgical procedures including an antireflux procedure in 5. One patient died. CONCLUSIONS: Management of EA without fistula remains challenging. Most patients required staged treatment that included esophageal replacement. The frequency of late complications indicates the need for programmed long-term review.

Bacterial counts from hospital doctors' ties are higher than those from shirts.

Doctor ties are often contaminated with bacteria, and it has been suggested that they should not be worn. We have compared bacterial counts from the ties and shirt pockets of 50 doctors. Counts were higher (P = .002) from ties that were rarely, if ever, cleaned than from shirts that were washed every 2 days or more frequently. The results support the need for further research on unwashable clothing of hospital staff.

Gastric transposition in children.

PURPOSE: To analyze the outcome in 192 children (116 males, 76 females) undergoing transposition since 1981. METHODS: The most common indications for esophageal replacement included failed repair of different varieties of esophageal atresia (138), caustic injury (29), and peptic strictures (9). A total of 81% of the patients were referred from other hospitals (50% from other countries). Age at operation ranged from 7 days to 17 years. The gastric transposition was performed by using blunt mediastinal dissection in 98 patients, with an additional 90 patients undergoing lateral thoracotomy. The retrosternal position was used in 4 patients. RESULTS: There were no graft failures, including those who had previously had failed gastric tube or Scharli operations. Anastomotic leaks occurred in 12% (all but one resolved spontaneously). Anastomotic stricture, requiring dilation developed in 20%. Half of these patients had previously sustained caustic esophageal injury. There were 9 deaths in the group (4.6%). One death occurred intraoperatively, 5 in the early postoperative period, and there were 3 late deaths. In over 90% of our patients, the outcome was considered good to excellent in terms of absence of swallowing difficulties or other gastrointestinal symptoms. Many children preferred to eat small frequent meals. Poor outcome was particularly associated with multiple previous attempts at esophageal salvage. There was no deterioration in the function of the gastric transposition in those patients followed for more than 10 years. CONCLUSIONS: Gastric transposition for esophageal substitution is an acceptable procedure. It is attended by 4.6% mortality and a 12% leak rate. A total of 20% of the patients needed anastomotic dilation for stricture. In the long term, good function has been maintained. Gastric transposition compares favorably with other methods of esophageal replacement.

A historical vignette: gastroschisis.

In the Hunterian Museum at the Royal College of Surgeons of England in London, there are a series of fetal specimens of varying gestational ages collected by John Hunter (1728-1793). Among this collection is a fetus demonstrating a gastroschisis anomaly that had gone unrecognized for almost 200 years.

Oesophageal atresia.

Oesophageal atresia (OA) encompasses a group of congenital anomalies comprising of an interruption of the continuity of the oesophagus with or without a persistent communication with the trachea. In 86% of cases there is a distal tracheooesophageal fistula, in 7% there is no fistulous connection, while in 4% there is a tracheooesophageal fistula without atresia. OA occurs in 1 in 2500 live births. Infants with OA are unable to swallow saliva and are noted to have excessive salivation requiring repeated suctioning. Associated anomalies occur in 50% of cases, the majority involving one or more of the VACTERL association (vertebral, anorectal, cardiac, tracheooesophageal, renal and limb defects). The aetiology is largely unknown and is likely to be multifactorial, however, various clues have been uncovered in animal experiments particularly defects in the expression of the gene Sonic hedgehog (Shh). The vast majority of cases are sporadic and the recurrence risk for siblings is 1%. The diagnosis may be suspected prenatally by a small or absent stomach bubble on antenatal ultrasound scan at around 18 weeks gestation. The likelihood of an atresia is increased by the presence of polyhydramnios. A nasogastric tube should be passed at birth in all infants born to a mother with polyhydramnios as well as to infants who are excessively mucusy soon after delivery to establish or refute the diagnosis. In OA the tube will not progress beyond 10 cm from the mouth (confirmation is by plain X-ray of the chest and abdomen). Definitive management comprises disconnection of the tracheooesophageal fistula, closure of the tracheal defect and primary anastomosis of the oesophagus. Where there is a "long gap" between the ends of the oesophagus, delayed primary repair should be attempted. Only very rarely will an oesophageal replacement be required. Survival is directly related to birth weight and to the presence of a major cardiac defect. Infants weighing over 1500 g and having no major cardiac problem should have a near 100% survival, while the presence of one of the risk factors reduces survival to 80% and further to 30-50% in the presence of both risk factors.