Assessment of jejunal interposition perfusion using indocyanine green.

INTRODUCTION: Jejunal interposition (JI) is an option for oesophageal replacement in children; ensuring good graft perfusion is essential. We report three cases where Indocyanine Green (ICG) with Near-Infrared Fluorescence (NIRF) was used to assess perfusion during graft selection, passage into the chest and anastomotic assessment. This extra assessment may reduce risk of anastomotic leak and/or stricture. METHODS: We describe the technique and salient features of all patients who have undergone ICG/NIRF-assisted JI in our centre. Patient demographics, indications for surgery, intra-operative plan, video of NIR perfusion assessment, complications and outcomes were reviewed. RESULTS: ICG/NIRF was used in three patients (2M:1F) at a dose of 0.2 mg/kg. ICG/NIRF imaging helped select the jejunal graft and confirmed perfusion after division of segmental arteries. Perfusion was assessed before and after passing the graft through the diaphragmatic hiatus and before and after making the oesophago-jejunal anastomosis. Intrathoracic assessment at the end of the procedure confirmed good perfusion of mesentery and intrathoracic bowel. In two patients, the reassurance contributed to successful procedures. In the third patient, graft selection was satisfactory, but borderline perfusion on clinical assessment after passing the graft to the chest, confirmed by ICG/NIRF meant the graft was abandoned. CONCLUSIONS: ICG/NIRF imaging was feasible and augmented our subjective assessment of graft perfusion, giving greater confidence during graft preparation, movement, and anastomosis. In addition, the imaging helped us abandon one graft. This series demonstrates the feasibility and benefit of ICG/NIR use in JI surgery. Further studies are required to optimise ICG use in this setting.

Esophago-Vascular Fistulae in Children: Five Survivors, Literature Review, and Proposal for Management.

INTRODUCTION: Esophago-vascular fistulae in children are almost uniformly fatal with death occurring by exsanguination. We present a single centre series of five surviving patients, a proposal for management and literature review. MATERIALS AND METHODS: Patients were identified from surgical logbooks, surgeon recollection and discharge coding data. Demographics, symptoms, co-morbidities, radiology, management and follow up details were recorded. RESULTS: Five patients (1M, 4F) were identified. Four were aorto-esophageal and one caroto-esophageal. Median age at initial presentation was 44 (8-177) months. Four patients had cross sectional imaging prior to surgery. Median time from presentation to combined entero-vascular surgery was 15 (0-419) days. Four patients required repair on cardio-pulmonary bypass with four undergoing staged surgical procedures. All required combined esophageal and cardio-vascular surgery. Length of PICU stay following combined surgery was 4 (2-60) days and overall hospital stay was 53 (15-84) days. Median follow up was 51 (17-61) months. Two patients had esophageal atresia and trachea-esophageal fistula managed as neonates. Three had no co-morbidities. Four had esophageal foreign bodies:1 esophageal stent, 2 button batteries, 1 chicken bone. One patient had a complication following colonic interposition. Four patients required an esophagostomy at the time of definitive surgery. All patients were alive and well at last follow up with one having successful reconnection surgery. CONCLUSION: In this series, outcomes were favourable. Multidisciplinary discussion and surgery are mandatory. If hemorrhage is controlled at presentation, then survival to discharge is possible but the magnitude of surgical intervention is both significant and very high risk. LEVEL OF EVIDENCE: Level 3.

A single centre experience using internal traction sutures in managing long gap oesophageal atresia.

AIM: Thoracoscopically placed internal traction sutures (ITS) for the initial management of long gap oesophageal atresia (LGOA), not amenable to primary anastomosis, was first described in 2015. Here we describe our experience using ITS both thoracoscopically and at thoracotomy where the gap between upper and lower oesophagus is too wide for primary anastomosis. METHOD: The case notes of all infants treated with ITS for oesophageal atresia (01/10/2015 to 01/12/2019) were reviewed. Gaps considered too wide for an anastomosis had ITS placed between the two pouches as described by Patkowski in 2015. All patients were gastrostomy fed.  Patients returned to theatre with an expectation to complete the anastomosis or re-tighten the traction sutures. RESULTS: Seven patients (4 OA, 1 OA with proximal fistula, 2 OA/distal TOF) median birthweight 2.28 kg (1.2-3.6 kg) were managed using ITS. Median gap length 4.5 (3-9) vertebral bodies.  ITS were placed thoracoscopically in 5 cases and at thoracotomy in 2 at median 46 days (1-120) old. In all cases, ITS was associated with significant intra-thoracic adhesions. Five patients leaked from the traction sutures. Four patients had a delayed primary anastomosis performed at thoracotomy and 3 required a cervical oesophagostomy. The median length of stay was 159 days (98-282).  All patients started thoracoscopically eventually required thoracotomy. CONCLUSION: The use of ITS in our department was associated with significant complications, particularly intra-thoracic leaks and adhesions.  In our hands ITS did not improve the feasibility of thoracoscopic repair for LGOA and has been abandoned by us. LEVEL OF EVIDENCE: Level IV Case Series.

Congenitally Malformed Hearts: Aspects of Teaching and Research Involving Medical Students.

To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. This article describes the experiences of the latest cohort of students on this programme, who undertook varying research projects using the Birmingham Cardiac Archive, with the guidance of Professor Anderson. The projects outlined include various aspects of isomerism, encompassing both the cardiac and abdominal manifestations, as well as details of congenitally corrected transposition of the great arteries and prenatally diagnosed right aortic arch and double arch. These studies all aimed to increase the knowledge base of their respective cardiac malformations and provide a basis for further research.

Exomphalos major: Conservative management using Manuka honey dressings and an outreach surgical nursing team.

INTRODUCTION: Controversy exists over the best dressing for conservative management of exomphalos major. Here we describe our experience of using Manuka Honey. METHODS: Our regimen involved covering the sac with Manuka honey (Advancis Medical™) wrapped with gauze and crepe bandage. Initially, dressings were changed 3 times a week and then twice weekly until full epithelialisation. Babies went home after reaching full feeds, with our outreach nurses continuing dressings in clinic until the parents were trained to do them alone. Only patients needing management of co-morbidities were transferred to our unit. Patients would be reviewed by video consultation. Data was prospectively collected. RESULTS: From 2011-2019, 24 consecutive patients (11:13 M:F; median gestation 37 weeks, birth weight 3.1 kg) with exomphalos major were managed with honey dressings. Fourteen babies had significant associated anomalies of which 10 died of problems unrelated to the exomphalos. Time to full feeds 6 (2-58) days; time to discharge 21(7-66) days if no associated anomalies; time to epithelialisation 73 (27-199) days. Dressings were well tolerated. Definitive closure occurred at 17(11-38) months and was uneventful. No patient required fundoplication and all patients were orally fed. Only one patient developed a clinically significant infection. CONCLUSION: This is the largest report of using Manuka honey for the management of exomphalos major. Benefits include early feeding, early discharge and a 'normalisation' of the neonatal period. Key to our success was the surgical outreach service supporting parents doing the dressings, first at the local hospital and then at home.

Characterization of the upper pouch tracheo-oesophageal fistula in oesophageal atresia.

AIM: A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. METHODS: A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. RESULTS: Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28-39) weeks, and median birth weight was 1647g (range 1100-3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. CONCLUSION: Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature. LEVEL OF EVIDENCE: level IV.

A review of pregnancies complicated by congenital sacrococcygeal teratoma in the West Midlands region over an 18-year period: population-based, cohort study.

OBJECTIVES: To describe the epidemiology and outcomes of sacrococcygeal teratoma (SCT) and identify the factors affecting prognosis in a population-based cohort. METHODS: Analyses of fetal SCTs from a population-based congenital anomaly register between 1995 and 2012, linked to regional datasets. A systematic literature review was performed for published studies on perinatal SCT (1995 to 2012). RESULTS: Thirty-six confirmed SCT cases were identified, giving a total prevalence of 0.30 per 10 000 births (95%CI 0.20-0.39). Twenty-three cases (63.9%) were diagnosed prenatally. There were six false positive prenatal diagnoses, and the positive predictive value of ultrasound for SCT was 79.3%. Secondary complications in prenatally diagnosed cases were polyhydramnios (27.2%), fetal hydrops (9.1%) and rapidly growing tumour (54.0%). The perinatal (PNMR) and infant mortality rates were 333.3 per 1000 births and 285.7 per 1000 live births, respectively. All stillbirths and infant deaths occurred in cases diagnosed prenatally. Factors associated with higher PNMR in registerable births were solid, vascular tumour composition (1000), polyhydramnios (667), premature delivery (667) and rapidly growing tumour (454). In the systematic review, prenatal hydrops fetalis and prematurity were the most morbid association in SCT. CONCLUSION: Prenatal ultrasound was relatively sensitive and specific in diagnosing SCT with good survival rates in live-born cases.

Gastrostomy insertion in the 21st century: PEG or laparoscopic? Report from a large single-centre series.

PURPOSE: To determine whether laparoscopic-assisted gastrostomy (LAG) has superseded percutaneous endoscopic gastrostomy (PEG) based on the clinical outcomes. METHODS: A retrospective study was undertaken for the period January 06-December 09. Demographic and clinical outcomes were recorded and the two groups were compared. RESULTS: 164 patients were studied (PEG, n = 107; LAG, n = 57). 93.5 % of PEG patients required two general anaesthetics compared with 8 % of LAG patients. Median time to using the gastrostomy was 24 (range 0-168) h in PEG and 0 (0-96) h in LAG patients (p < 0.001). Major complications occurred in 15/107 (14 %) of PEG and 2/57 (3.5 %) of LAG patients (p = 0.05). Re-operation rate following complications was 18/107 (16.8 %) for PEG and 3/57 (5.2 %) for LAG (p = 0.05). Minor complications arose in 41/107 (38 %) of PEG and 32/57 (56 %) of LAG (p = 0.05). Post-operative hospital stay was 2 (1-40) days for PEG and 2 (0-20) days for LAG (p = 0.01). The day-case rate was 0/107 for PEG and 5/57 (9 %) for LAG. There was no gastrostomy-related mortality in the series. CONCLUSION: LAG requires fewer anaesthetics, is associated with shorter time to feeding, shortened hospital stay and has a reduced risk of major complications. LAG is a very good alternative to the PEG in children.

Management of gastroesophageal reflux associated with malrotation in children.

AIM: Children being investigated for gastroesophageal reflux (GOR) have a high incidence of malrotation. Current literature suggests these patients should be managed with a combined antireflux and Ladd's procedure. We review our experience, the largest series to date, of performing an elective Ladd's procedure as the first-line intervention. METHOD: Retrospective case note review of 20 children with significant symptoms of GOR and an incidental finding of malrotation. Children presenting immediately with bilious vomiting were excluded. All patients underwent a Ladd's procedure as their sole primary operative intervention. RESULTS: Median age at operation was 7 months (21 days-12 years). Fifteen patients (75%) had evidence of reflux on barium contrast study. All children were followed up for at least 6 months. Eighteen (90%) had resolution or significant improvement of their symptoms postsurgery. Only 3 have not managed to tolerate a full oral diet, all unrelated to GOR. None of our series required an antireflux procedure. CONCLUSION: In children with debilitating vomiting necessitating surgical management, a contrast study is imperative in the work up. The high incidence of GOR and the significant improvement after correction of malrotation show the relationship between delayed gastric emptying and GOR. We suggest that when an abnormally placed duodenojejunal flexure is found, a Ladd's procedure alone is sufficient and may obviate the need for a more invasive antireflux procedure.

Conservative management of exomphalos major with silver dressings: are they safe?

Historically, some dressings used in exomphalos major were associated with toxicity. These have been abandoned in favor of safer dressings. Silver toxicity has not been described following the use of silver dressings in infants. We, however, found disconcerting serum silver levels in 2 consecutive patients during treatment with silver salt containing dressings.

Appendicitis in children: a comparative study between a specialist paediatric centre and a district general hospital.

PURPOSE: The study aimed to compare paediatric appendicectomy practice in a specialist paediatric centre (SPC) with a district general hospital (DGH). METHODS: This was a retrospective study of children younger than 16 years treated between January 1, 2005, and September 30, 2007. RESULTS: Two hundred seven patients (SPC) and 264 (DGH) had an operation for suspected appendicitis. Thirty-one percent of SPC patients were female vs 41% in the DGH (P = .03). Median age (range) was 10.3 years (1.2-15.9 years) in the SPC and 11.8 (3.3-16.0 years) in the DGH (P < or = .0001). The negative appendicectomy rate was 4% at the SPC and 20% at the DGH (P < or = .0001). Perforated appendicitis was found in 37% of children at the SPC compared with only 18% at the DGH (P < or = .0001). Median (range) length of stay was 5 days at the SPC (1-21 days) compared with 2 days at the DGH (1-21 days) (P < or = .0001). CONCLUSION: Our findings have important implications for local practice in our 2 centres but may also have wider implications for the national organisation of the surgical care of children and for the training of general surgeons.

The role of imaging in children with suspected appendicitis: the UK perspective.

The diagnosis of uncomplicated acute appendicitis is often straightforward, allowing timely appendicectomy without the need for expensive tests or imaging. Repeated clinical examination by an experienced surgeon has traditionally been the key to making the diagnosis in both straightforward and difficult cases. Nonetheless, all surgeons will remove some normal appendices. Sometimes it can be particularly difficult to make the diagnosis, especially in the child under 5 years of age, in teenage girls, in young women and in the elderly. When difficult to make, the diagnosis may be significantly delayed and since the pathology is progressive, the patient may suffer potentially avoidable complications. This paper looks at two potential roles for imaging. Firstly, can imaging, applied selectively, help make the difficult diagnosis less difficult and so reduce delays and morbidity? Secondly, could imaging all patients with suspected appendicitis reduce the number of normal appendices removed from children who seem to have all the signs and symptoms of straightforward uncomplicated acute appendicitis but who actually have presumed self-resolving non-appendiceal pathology? The answer to these questions may depend on three factors that are not entirely independent: a surgical unit's current audited negative appendicectomy rate, population base/case mix and the expertise of the examining surgeon. Individual surgeons and some surgical units, by policy, use modern imaging techniques with quite different frequencies that may be appropriate depending on these three factors. This article argues that a careful history and repeated clinical examination is the key to making the diagnosis, with imaging, primarily ultrasonography, being used in patients with a palpable mass or in those having had 48 h of hospital observation without progress. In Europe, imaging has played a limited role in the investigation of the child with suspected appendicitis with the diagnosis relying on repeated examination by an experienced clinician. Ongoing changes in surgical training in the UK may affect the acquisition of clinical expertise that is crucial to this clinical management. High-quality surgical training and surgical audit are needed to monitor the delivery of care and to ensure that the care pathway being used is appropriate for the local resources and population.

Chemosensitivity in pediatric dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of fibrohistiocytic spectrum and uncommonly presents in infancy and childhood. The standard treatment is wide-margin surgical excision, but this is difficult to achieve in large tumors and may result in unacceptable cosmetic, structural, and functional consequences. The authors describe a child with recurring DFSP who, after extensive surgery, responded to treatment with vinblastine and oral methotrexate. This is the first reported case in literature of a chemotherapeutic response in pediatric DFSP.