Thoracoscopic external traction suture elongation for the management of long-gap oesophageal atresia: a two-centre experience.

AIM: Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. METHOD: Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). RESULTS: From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6 days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52 months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. CONCLUSION: In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.

Controlled aspiration of large paediatric ovarian cystic tumours.

AIM: Cystic ovarian masses in children may be physiological or neoplastic. It is mandatory that suspected neoplastic lesions are resected without tumour spillage. However, a large midline incision is cosmetically unappealing incision to young women. Here we describe our experience of using controlled drainage without spillage that allows a cosmetic pfannenstiel approach without compromising oncological principles. METHOD: All girls treated with large ovarian cystic masses since 2008 in our centre were identified and data was collected prospectively. A small pfannenstiel incision was performed followed by peritoneal washings; tissue glue was used to stick an Opsite™ dressing to the cyst surface and fluid drained so there was no leakage back into the patient. Once aspirated the cyst was delivered and an ovarian preserving cystectomy was performed where possible. RESULTS: Twenty-three girls (median age 14.5 years (8.1 to 16.5 years) were included. Pre-operative MRI scan showed a complex lesions with median volume of 1169 ml (range 252-7077 ml). At surgery 22/23 cysts were intact and removed without spillage. HISTOLOGY: mature teratoma (11), serous cyst (3), mucinous cyst adenocarcinoma (2), mucinous cystadenoma (5), Sertoli-Leydig tumour, sclerosing stromal tumour. One girl with pre-operative rupture of a mucinous adenocarcinoma subsequently died. Ovarian sparing cystectomy was performed in 17/23 girls. All other patients are well without evidence of recurrence. CONCLUSION: This is the largest series in children and adolescents using controlled drainage of cystic ovarian tumours. Though there were a range of diagnoses we have shown that these can be removed safely with a cosmetic pfannenstiel approach while following oncological principles.

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.

A single centre matched pair series comparing minimally invasive and open surgery for the resection of pediatric renal tumours.

INTRODUCTION: Minimally invasive nephrectomy (MIN)for the resection of pediatric renal tumors is controversial. We compared patients who underwent laparoscopic MIN according to our unit protocol with matched patients who underwent open surgery (ON). METHOD: A single centre retrospective analysis of prospectively collected data for MIN. Tumor volume was calculated from pre-operative imaging and volume matched ON patients were identified from the regional tumor database. Demographics, complications, operative time, length of stay, number of nodes resected, status of margins, radiotherapy (RT)requirement, length of follow-up and outcomes were analysed. RESULTS: Fourteen MINs performed between September 2016 and 11/2019 (M:F 5:9; median age 37 (range 2-73)months) were matched with ON controls (7:7 M:F, median age 42 (1-74)months). Diagnoses in the MIN group were Wilms Tumour (WT)in11 and one each of mesoblastic nephroma (MN), malignant rhabdoid tumour of the kidney (MRTK), nephroblastomatosis and in the ON group were 13 WT and 1 MN. Median operating time for MIN was 180 (125-300)minutes, with 4 conversions and 155 (1-3-480)minutes in ON. There were no intra-operative ruptures. Three tumors in each group had positive margins. A median of 4 (0-6) nodes were removed during MIN and 4 (2-22) during ON. Median length of stay (LoS)was 4 (2-15)days following MIN and 4 (2-9) following MIN. Six ON and 3 MIN patients required radiotherapy. Differences were not significant. Median follow up of 30 (6-44)months for MIN and 73 (34-109)months for ON patients was significantly different. There have been no local recurrences but the child with MRTK died following brain tumor recurrence. CONCLUSION: MIN for pediatric renal tumors has similar short term outcomes to ON in carefully selected patients.

Detection of Circulating and Disseminated Neuroblastoma Cells Using the ImageStream Flow Cytometer for Use as Predictive and Pharmacodynamic Biomarkers.

PURPOSE: Circulating tumor cells (CTCs) serve as noninvasive tumor biomarkers in many types of cancer. Our aim was to detect CTCs from patients with neuroblastoma for use as predictive and pharmacodynamic biomarkers. EXPERIMENTAL DESIGN: We collected matched blood and bone marrow samples from 40 patients with neuroblastoma to detect GD2 +/CD45- neuroblastoma CTCs from blood and disseminated tumor cells (DTCs) from bone marrow using the Imagestream Imaging flow cytometer (ISx). In six cases, circulating free DNA (cfDNA) extracted from plasma isolated from the CTC sample was analyzed by high-density single-nucleotide polymorphism (SNP) arrays. RESULTS: CTCs were detected in 26 of 42 blood samples (1-264/mL) and DTCs in 25 of 35 bone marrow samples (57-291,544/mL). Higher numbers of CTCs in patients with newly diagnosed, high-risk neuroblastoma correlated with failure to obtain a complete bone marrow (BM) metastatic response after induction chemotherapy (P < 0.01). Ex vivo Nutlin-3 (MDM2 inhibitor) treatment of blood and BM increased p53 and p21 expression in CTCs and DTCs compared with DMSO controls. In five of six cases, cfDNA analyzed by SNP arrays revealed copy number abnormalities associated with neuroblastoma. CONCLUSIONS: This is the first study to show that CTCs and DTCs are detectable in neuroblastoma using the ISx, with concurrently extracted cfDNA used for copy number profiling, and may be useful as pharmacodynamic biomarkers in early-phase clinical trials. Further investigation is required to determine whether CTC numbers are predictive biomarkers of BM response to first-line induction chemotherapy.

Aortoesophageal fistula after endovascular aortic aneurysm repair of a mycotic thoracic aneurysm.

Mycotic aneurysms constitute a small proportion of aortic aneurysms. Endovascular repair of mycotic aneurysms has been applied with good short-term and midterm results. However, the uncommon aortoenteric fistula formation remains a potentially fatal complication when repairing such infective aneurysms. We present the case of an 80-year-old woman with thoracic and abdominal aortic mycotic aneurysms, which were successfully treated with endografting. However, the patient presented 3 months later with upper gastrointestinal bleeding secondary to erosion of the thoracic graft into the oesophagus. The patient was treated conservatively due to the high risk of surgical repair. There is currently little exposure to the management of mycotic aortic aneurysms. If suspected, imaging of the entire vasculature will aid initial diagnosis and highlight the extent of the disease process, allowing for efficient management. Aortic endografting for mycotic thoracic aneurysms is a high-risk procedure yet is still an appropriate intervention. Aortoenteric fistulae pose a rare but severe complication of aortic endografting in this setting.