What Proportion of Children With Complex Oesophageal Atresia Require Oesophageal Lengthening Procedures?

INTRODUCTION: Babies with oesophageal atresia (OA) who cannot achieve a primary anastomosis (complex OA) may be treated by attempted oesophageal lengthening. We contrast reported outcomes of lengthening with our experience of managing complex OA. PATIENTS AND METHODS: A consecutive series treated in an English regional centre was completed. Outcomes of interest were the rate of retention of the native oesophagus, complications requiring thoracotomy, rates of anastomotic leak, stricture, fundoplication, and mortality. Possible explanatory variables were the surgical techniques applied. RESULTS: 29/215 (13%) OA were complex, and 25/207 survived to repair. 14/25 (56%) had no distal fistula, pure OA, while 11/25 (44%) had a long gap with distal fistula. 18/25 (72%) had delayed primary anastomosis, while 7/25 (28%) required oesophageal replacement. However, 2 of the replacements were salvage procedures following failed traction. Only 4/207 (2%) of OA were potentially treatable by traction. Salvage surgery was required in 2/23 (9%) complex OA not subjected to lengthening. The native oesophagus was retained without utilising lengthening in 200/207 (97%). Amongst complex OA where traction techniques had not been attempted, the native oesophagus was retained in 18/23 (78%) of cases, with median time to oesophageal continuity of 77 days. There was no in hospital mortality following treatment of complex OA, and overall survival was identical to non-complex OA among cases surviving to anastomosis. DISCUSSION: Management of complex OA without lengthening procedures leads a similar rate of retention of the native oesophagus as reports describing lengthening, but with significantly less morbidity. We see little need for oesophageal lengthening in the management of complex OA. LEVEL OF EVIDENCE: IV.

Congenital diaphragmatic hernia survival in an English regional ECMO center.

Introduction: Congenital diaphragmatic hernia (CDH) remains a cause of neonatal death. Our aims are to describe contemporary rates of survival and the variables associated with this outcome, contrasting these with our study of two decades earlier and recent reports. Materials and methods: A retrospective review of all infants diagnosed in a regional center between January 2000 and December 2020 was performed. The outcome of interest was survival. Possible explanatory variables included side of defect, use of complex ventilatory or hemodynamic strategies (inhaled nitric oxide (iNO), high-frequency oscillatory ventilation (HFOV), extracorporeal membrane oxygenation (ECMO), and Prostin), presence of antenatal diagnosis, associated anomalies, birth weight, and gestation. Temporal changes were studied by measuring outcomes in each of four consecutive 63-month periods. Results: A total of 225 cases were diagnosed. Survival was 60% (134 of 225). Postnatal survival was 68% (134 of 198 liveborn), and postrepair survival was 84% (134 of 159 who survived to repair). Diagnosis was made antenatally in 66% of cases. Variables associated with mortality were the need for complex ventilatory strategies (iNO, HFOV, Prostin, and ECMO), antenatal diagnosis, right-sided defects, use of patch repair, associated anomalies, birth weight, and gestation. Survival has improved from our report of a prior decade and did not vary during the study period. Postnatal survival has improved despite fewer terminations. On multivariate analysis, the need for complex ventilation was the strongest predictor of death (OR=50, 95% CI 13 to 224, p<0.0001), and associated anomalies ceased to be predictive. Conclusions: Survival has improved from our earlier report, despite reduced numbers of terminations. This may be related to increased use of complex ventilatory strategies.

Pull through for Hirschsprung disease without planned rectal decompression is safe.

AIMS: Definitive surgery for Hirschsprung disease (HD) is typically deferred to allow rectal washouts. In contrast, we have performed pull through on the next available operating list following diagnostic biopsy. Our aims are to compare outcomes of surgery without planned decompression vs. surgery where the timing of pull through was deferred. METHODS: A consecutive series undergoing pull-through. Timing of surgery was at surgeon's discretion and classified as either no planned decompression surgery or other. Outcomes were i) complications graded >3b Clavien-Dindo (CD) within 30d, or ii) a need for a revisional pull through or iii) a post-pull-through stoma. We excluded total colonic aganglionosis. RESULTS: 156 children (116 boys) underwent pull-through. 71 had surgery without planned decompression. The indications for timing in the other 85 were: planned after home washouts (n = 28), planned after stoma (n = 29), diagnostic error (n = 19), prematurity (n = 3), late presentation (n = 2) and other (n = 4). The mean age at surgery in the surgery without planned decompression group was 18 days (range 1-49 days) vs. 310 days (range 14-4084 days). 5/72 (7%) undergoing surgery without planned decompression experienced a complication of ≥CD 3b, compared to 7/85 (8%) of delayed surgery (p = 0.8). 3/71 (4%) of the surgery without planned decompression group required a stoma following pull through compared to 11/83 (13%) of the delayed group (p = 0.059). 5/71 (7%) of the surgery without planned decompression group required revisional pull through compared to 13/85 (15%) of the delayed surgery group (p = 0.1). CONCLUSIONS: Surgery without planned rectal decompression for HD leads to no increase in significant complications, requirement for stoma or revision. Early definitive surgery is safe, and the use of pre-operative stoma or rectal washouts is not always necessary. LEVEL OF EVIDENCE: III.

Single institution experience of cloacal malformation.

INTRODUCTION: The aim of this study is to report on the outcomes of patients born with cloacal malformation, managed at a single institution more than the last 28 years. The focus of this study is the long term renal and colorectal outcomes. METHODS: Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed. RESULTS: Twenty-one patients fulfilled the inclusion criteria. Eleven long common channel (LCC) and ten short common channel (SCC) cloacae patients were identified. Median age at the time of primary reconstruction was 11 months in both groups. In the LCC group, seven (63.6%) patients underwent a Total Urogenital Mobilisation (TUM), and 4 (36.4%) required a vaginal replacement. 6/11 (54.5%) of patients required drainage of a hydrocolpos. In the SCC group, four patients required a TUM, two patients underwent mobilisation of the rectum and vagina alone, and three underwent rectal mobilisation alone. Two patients have required renal transplant for congenital renal dysplasia, and two have developed chronic renal failure associated with the sequalae of vesicoureteric reflux. Eleven (52.3%) of the patients manage their bowels with an antegrade continent enema (ACE), and two of the LCC cloaca are defunctioned with a colostomy. Clean intermittent catheterisation is performed by 12 (57%) of the patients, either per urethra or via a Mitrofanoff channel. CONCLUSION: The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence. LEVEL OF EVIDENCE: Level IV.

Functional outcomes, quality of life, sexual function, and fertility of adult patients undergoing ileo-anal pouch anastomosis in childhood.

BACKGROUND: We conducted a questionnaire-based study of pouch function, quality of life, sexual function, and fertility among patients who had undergone ileal pouch anal anastomosis (IPAA) in childhood and who are now more than 18 years old. METHODS: A consecutive series of patients were asked to complete the following questionnaires: Pouch function score (PFS), short form 36 (SF-36), International index of erectile function (IIEF) (males), and Female sexual function index (FSFI) (females). Fertility in females was also assessed. Data are quoted as a median (SD). RESULTS: Of 144 patients who had IPAA, 101 were eligible, and 70 responded. Mean age at surgery was 14 years, and mean current age is 26 years. Most patients had either ulcerative colitis or familial adenomatous polyposis. The questionnaire was completed by 38/70 (54%). Median PFS score was 6 (5). SF-36 were lower than previous reports. Median FSFI was 30 (7.6), 84% of possible maximum. Median IIEF was 69, 92% of possible maximum. Successful conception was reported in 5/7 women who had tried. However, there were several miscarriages and two surgical emergencies during pregnancies. CONCLUSIONS: IPAA can be performed in childhood with similar pouch function to reported adult series. Quality of life appears poorer, but sexual function is maintained. There may be an association with adverse events in pregnancy. LEVEL OF EVIDENCE: IV.

Surgeon-Level Variation in Outcome following Esophageal Atresia Repair Is Not Explained by Volume.

INTRODUCTION: To assess whether there is a difference in operative outcome for esophageal atresia (EA) depending on a surgeon's seniority as defined by years in consultant practice or number of cases performed. In addition a Clavien-Dindo score was used to sequentially analyze the outcome of each surgeon's EA procedure. MATERIALS AND METHODS: All repairs performed over 22 years in an English regional center were analyzed. Outcomes were: death, anastomotic leak, need for dilatation, need for more than three dilatations, need for fundoplication, and a Clavien-Dindo adverse outcome of ≥3b. Possible explanatory variables were: number of prior repairs by the surgeon, surgeon's years of consultant experience. We also examined the effect of variables intrinsic to the infant as possible confounding variables and as independent predictors of outcome. RESULTS: A total of 190 repairs were performed or supervised by 12 consultants. There was no significant association between consultant experience and any objective outcome. However, sequential analysis suggests there is variation between surgeons in the incidence of Clavien-Dindo events of ≥3b. Performance showed deterioration in one case. Mortality was explicable by cardiac and renal anomalies. CONCLUSION: There are surgeon-level variations in outcomes for the procedure of EA repair, but they are not explained by volume. Surgeon performance can deteriorate. Our study would not support the concept that patient outcomes could be improved by concentrating the provision of this surgery to fewer hospitals or surgeons.

Surgeon level variation in outcome of repair of congenital diaphragmatic hernia with particular reference to the management of recurrence.

INTRODUCTION: The aim of this study is to investigate firstly, the rate of recurrence following primary repair of a congenital diaphragmatic hernia (CDH) and secondly, the rate of recurrence following revisional surgical repair. The primary outcome is rate of recurrence. Secondary outcomes are to establish whether recurrence is related to surgeon, surgeon volume, side of defect, the use of a patch, or a thoracopscopic approach METHODS: All repairs performed in an English regional center over 22 years were recorded. Possible explanatory variables were whether the repair was itself of a recurrence, the surgeon's identity, the surgeon's volume of prior repairs, the side of the defect, the use of a patch. RESULTS: 198 repairs were performed; 170 primary repairs and 28 of recurrences. Failure occurred significantly more commonly among recurrences (32%) than primary repairs (11%), p = 0.005. Failure of the primary repair was significantly more common where a patch was used 8/34 (23%) rather than a sutured repair 10/136 (7%), p = 0.006, or where a thoracoscopic technique was used 4/13 (31%) rather than laparotomy 14/157 (9%) p = 0.01. Failure of the primary repair was unrelated to the identity of the surgeon (Χ2 = 5, p = 0.9) or the volume of prior repairs (t = 0.3, p = 0.6). However, failure of repair of a recurrence was significantly related to the surgeon's volume of prior repairs (t = 2.3, p = 0.01) and the identity of the surgeon (Χ2 = 17, p = 0.014), but not the use of a patch (Χ2 = 1.6, p = 0.2). CONCLUSIONS: Repair of a recurrence of a CDH has a higher probability of failure than the original repair and is related to both the identity of the surgeon and the prior volume of experience. There is a volume outcome relationship for the repair of recurrence, but not the primary repair of CDH. Our study suggests the repair of recurrence of CDH should be restricted to surgeons with proven outcomes for this procedure.

Commentary on long-term surgical and patient-reported outcomes of Hirschsprung disease.

This is a commentary on the manuscript titled "Long-Term Surgical and Patient-Reported Outcomes of Hirschsprung's Disease by Davidson J, Kyrklund K, Eaton S, et al.

Transition Care from Adolescence to Adulthood: A 10-Year Service Review of the Gynecological Implications for Young Women and Girls Born with Cloacal Anomalies.

STUDY OBJECTIVE: To establish the gynecological and reproductive outcomes for girls born with a cloacal anomaly, seen in a pediatric specialist cloaca clinic. DESIGN: Local approval was granted to conduct this review. Outcomes were retrospectively identified using healthcare records. PARTICIPANTS: Girls with known cloacal anomaly, seen in the cloaca clinic between 2009 and 2019, who had attained menarche or received gynecological input. RESULTS: Nine females met the inclusion criteria, who were 12-30 years old. The mean age of menarche was 12 years (SD = 1.29). Two developed obstructed menstruation, requiring surgical intervention. Vaginal stenosis affected all women. Three women underwent revision surgery, and 1 is awaiting surgery. None of the women have attempted a pregnancy, to our knowledge. CONCLUSION: Cloacal anomaly is a rare complex condition. Female individuals with cloacal anomaly require multidisciplinary gynecology specialist care throughout adolescence and adulthood. Provision of a dedicated gynecological service could improve the quality of life of these women.

Clinical guideline for retained button batteries.

OBJECTIVE: To design a clinical guideline for the emergency management of retained button batteries (RBBs) through analysis of UK National Health Service hospital guidelines and published literature. METHOD: 49 acute hospitals were contacted, and their guidelines were analysed. A consensus guideline was then created with multidisciplinary input. The final guideline was independently peer reviewed by the British Association of Otorhinolaryngology and Head and Neck Surgery (ENT UK) clinical guidelines committee. RESULTS: 40 (82%) trusts responded. 28 had a guideline for the management of a RBB in the aerodigestive tract. Significant variation between guidelines assessment, investigation and management of a RBB was identified. CONCLUSION: A single-page guideline was designed to improve frontline healthcare professional's immediate investigation and management of a RBB on presentation to emergency care. This has been published by ENT UK as a clinical guideline.

Severity of complications following restorative proctocolectomy in children is related to staging not diagnosis.

BACKGROUND: Restorative proctocolectomy (RPC) is performed using a variety of staged procedures for several diseases. Our aim was to assess whether the severity of complications, classified according to Clavien-Dindo, was related to the diagnosis or the procedure. METHODS: A consecutive series of children receiving an ileoanal pouch was prospectively recorded. Complications were scored by two blinded observers. Major complications were Clavien-Dindo ≥3b. Procedures were classified as: colectomy, proctectomy and pouch or proctocolectomy and pouch. Diagnoses were classified as: ulcerative colitis, familial adenomatous polyposis or other: idiopathic constipation, total colonic Hirschsprung's disease, juvenile polyposis, Crohn's colitis, fibrosing colonopathy or necrotising enterocolitis. RESULTS: 128 children underwent 191 procedures: 61 colectomies, 63 proctectomies and 67 proctocolectomies. 84 children had ulcerative colitis, 20 had FAP and 24 had other indications. Major complications were significantly more likely with proctocolectomy (16/67, 24%) than with either colectomy (4/61, 7%) or proctectomy (8/63, 13%), p = 0.01. There was no association between diagnosis and major complications: ulcerative colitis (18/133, 14%), FAP (5/20, 25%), other (5/38, 13%) p = 0.4. There was no increase in major complications following proctectomy if a major complication had occurred during prior colectomy. Overall, 15% of procedures experienced a major complication. 6/9 stoma related complications required operative intervention. CONCLUSIONS: The severity of complications after RPC in children is related to use of a two stage rather than three stage sequence of surgery, not the underlying diagnosis. TYPE OF STUDY: Case control study. LEVEL OF EVIDENCE: Level III.

Lung biopsy in children: when is it useful?

AIM: To provide a further insight into the usefulness of lung biopsy in children. METHODS: Lung biopsies in children from January 2007 to December 2017 were reviewed (n=39). The histology results were categorised as: definitive diagnosis, normal lung parenchyma, inconclusive. RESULTS: Lung biopsy provided a definitive diagnosis in 25 (64%) cases. A suspected diagnosis was confirmed in 16 (41%) and a new diagnosis was found in 9 (23%) children. Histology was inconclusive in 11 (28%) cases and normal in 3 (8%). Fifteen (38%) children had treatment altered due to the biopsy result. CONCLUSION: Lung biopsy mostly confirmed the suspected diagnosis and was associated with a low procedure related morbidity (n=1) and mortality (n=0). Importantly, the biopsy result identified a pathology which altered treatment in over one third of patients. However, in a number of cases the histology was inconclusive, therefore careful patient selection is recommended to maximise diagnostic yield.

Consensus standards of healthcare for adults and children with inflammatory bowel disease in the UK.

OBJECTIVE: Symptoms and clinical course during inflammatory bowel disease (IBD) vary among individuals. Personalised care is therefore essential to effective management, delivered by a strong patient-centred multidisciplinary team, working within a well-designed service. This study aimed to fully rewrite the UK Standards for the healthcare of adults and children with IBD, and to develop an IBD Service Benchmarking Tool to support current and future personalised care models. DESIGN: Led by IBD UK, a national multidisciplinary alliance of patients and nominated representatives from all major stakeholders in IBD care, Standards requirements were defined by survey data collated from 689 patients and 151 healthcare professionals. Standards were drafted and refined over three rounds of modified electronic-Delphi. RESULTS: Consensus was achieved for 59 Standards covering seven clinical domains; (1) design and delivery of the multidisciplinary IBD service; (2) prediagnostic referral pathways, protocols and timeframes; (3) holistic care of the newly diagnosed patient; (4) flare management to support patient empowerment, self-management and access to specialists where required; (5) surgery including appropriate expertise, preoperative information, psychological support and postoperative care; (6) inpatient medical care delivery (7) and ongoing long-term care in the outpatient department and primary care setting including shared care. Using these patient-centred Standards and informed by the IBD Quality Improvement Project (IBDQIP), this paper presents a national benchmarking framework. CONCLUSIONS: The Standards and Benchmarking Tool provide a framework for healthcare providers and patients to rate the quality of their service. This will recognise excellent care, and promote quality improvement, audit and service development in IBD.

Am I out of control? The application of statistical process control charts to children's surgery.

AIMS: To illustrate the construction of statistical control charts and show their potential application to analysis of outcomes in children's surgery. PATIENTS AND METHODS: Two datasets recording outcomes following esophageal atresia repair and intestinal resection for Crohn's disease maintained by the author were used to construct four types of charts. The effects of altering the target signal, the alarm signal and the limits are illustrated. The dilemmas in choice of target rate are described. Simulated data illustrate the advantages over hypothesis testing. RESULTS: The charts show the author's institutional leak rate for esophageal atresia repair may be within acceptable limits, but that this is dependent on the target set. The desirable target is contentious. The leak rate for anastomoses following intestinal resection for Crohn's disease leak is also within acceptable limits when compared to published experience, but may be deteriorating. The charts are able to detect deteriorating levels of performance well before hypothesis testing would suggest a systematic problem with outcomes. CONCLUSIONS: Statistical process control charts can provide surgeons with early warning of systematic poor performance. They are robust to volume-outcome influences, since the outcome is tested sequentially after each procedure or patient. They have application in a specialty with low frequencies of operations such as children's surgery. TYPE OF STUDY: Diagnostic test. LEVEL OF EVIDENCE: Level II.

Outcomes of colonic resection for chronic idiopathic constipation in childhood.

AIMS: The purpose of this study was to describe the outcomes of colonic resection for constipation in children. Three different types of resection are compared: pan-proctocolectomy with ileoanal pouch anastomosis (IPAA), total colectomy with ileorectal anastomosis (IR), and segmental resections and anastomosis (SR). PATIENTS AND METHODS: All colonic resections were prospectively recorded and their outcomes tabulated. Outcomes were classified as Good: anal defecation with no soiling; Intermediate: anal defecation with occasional soiling or need for ACE; Poor: a permanent stoma. All complications were also recorded. RESULTS: 22 children underwent colonic resection over a 20 year period. Mean follow up was 40 months. 18 had prior antegrade continent enema (ACE), and 12 had a prior stoma. 13 underwent IPAA, 6 IR, and 3 SR. 10 (45%) had a good outcome, 4 (18%) had an intermediate outcome, and 8 (36%) had a poor outcome. Resection restored 9/12 (75%) of children with a preexisting stoma to anal defecation. No procedure produced better outcome than the others in terms of results or complications. CONCLUSIONS: There may be a role for colonic resection in selected constipated children, but parents should be warned that there remains a significant possibility of a permanent stoma. Our study suggests that around two-fifths will be left with a permanent stoma. LEVEL OF EVIDENCE: Level IV.

Isolated ascites in a newborn with 'apple peel' jejunal atresia.

Isolated fetal ascites was diagnosed at 20 weeks in a primiparous woman with no significant medical history. Progressive fetal ascites worsened after 28 weeks and resulted in fetal hydroceles. Delivery was by caesarian section at 33 weeks, preceded by reduction of fetal ascites under ultrasound guidance. Following delivery, the baby required further reduction of abdominal fluid and endotracheal intubation to provide respiratory support. An extensive set of investigations, including metabolic and genetic screening, was performed; all results were negative. On day two of life, the baby developed bilious aspirates and an abdominal radiograph suggested intestinal obstruction. At laparotomy, an 'apple peel' jejunal atresia, abnormal mesentery with precarious blood supply and a proximal perforation were identified and the perforation 'sewn over'. The postoperative course was unremarkable, with Monogen feeds tolerated three weeks later. The baby continued to thrive at one year, tolerating increasing amount of long-chain fatty acids in diet.

Oesophageal replacement with stomach: A personal series and review of published experience.

AIM: To describe the outcomes of oesophageal replacement using stomach in children. METHODS: All children undergoing oesophageal replacement in a regional centre were prospectively recorded in a customised database and subjected to continual follow up. Complications within 30 days were classified as early, and all other complications were classified as late. Outcomes were related to a comprehensive analysis of published experience where studies were classified as having long-term follow up if the median duration exceeded 5 years. RESULTS: Ten children underwent oesophageal replacement using the stomach between 1998 and 2016. Indications were oesophageal atresia (6), caustic ingestion (2), foreign body ingestion (1) and oesophageal hamartoma (1). Two children died at 2 and 7 months after gastric transposition. All survivors are under review, with a median follow up of 8.5 years (range 3-14 years). Complications occurred in every case. Among survivors, three had early complications and eight had late complications. Early complications included anastomotic leak (2) and lung compression by stomach (1). Late complications were anaemia (8), anastomotic stricture (7), oesophagitis (5), dumping syndrome (2), perforation of a jejunostomy (1), para-gastric hiatal hernia (1), gastric outlet obstruction (1), Barrett's oesophagus (1), prolonged inability to swallow (1) and recurrent lower respiratory tract infections (1). Among 57 publications, only three achieved complete long-term follow up. The incidence of reported complications was higher when follow up was complete. CONCLUSIONS: Oesophageal replacement by gastric transposition in children leads to serious chronic morbidity. Published experience masks this because of incomplete and short follow up.