Fertility preservation service for children and young adults at high risk of infertility; the hub and spoke model.

PURPOSE: To evaluate the evolution of fertility preservation surgery in children and young adults at high risk of infertility from a single centre to a networked 'Hub and Spoke' service. METHODS: A case note review of patients referred for ovarian or testicular cryopreservation between Jan 2013 and Dec 2023. Demographic data, procurement numbers, and site of procurement were collected. Specialist feedback was obtained to identify the challenges faced. RESULTS: Over time, the number of referrals increased from 4 to 349 patients per year with the number of Spoke centres rising to 36 ovarian and 16 testicular. In 2013-2014; 100% of procurement was ovarian as compared to 2023; 51% ovarian, 49% testicular. Of the 395 referrals in 2021, 81% (n = 319) went on to have procurement and storage of tissue. Between 2013 and 2016, 96% of cases were performed at the Hub. In 2023, 53/349 (15%) cases were performed at the Hub with the remaining 296 (85%) procured at Spoke sites. Surgical issues such as access to theatre, variation and availability of surgical equipment, thermal injury to ovarian tissue and variation in the size of the testicular specimen were identified. CONCLUSION: The Hub and Spoke model successfully delivers treatment to patients close to home as safely possible within their local treatment centre.

Managing giant omphalocele: A systematic review of surgical techniques and outcomes.

AIM: We analysed closure techniques in the treatment of giant omphalocele. A challenging pathology where there lacks consensus. METHODS: Cochrane, MEDLINE and EMBASE were searched between 1 January 1992 and 31 December 2022 using terms and variations: omphalocele, exomphalos, giant, closure and outcome. Papers were selected using Preferred Reporting Items for Systematic review and Meta-Analyses 2020 criteria. Data collected included demographics, timing and technique of surgical repair, morbidity and mortality. RESULTS: We identified 342 papers; 34 met inclusion criteria with a total 356 neonates. Initial non-operative management was described in 26 papers (14 dressings, eight silo, four serial sac-ligation). Operative techniques by paper were as follows: Early closure: nine primary suture closure without patch, two primary closure with patch and four mixed methods. Delayed closure: five simple, four-component separation technique, four tissue expanders, one Botox/pneumoperitoneum and two with patch. Median number of procedures was two (1-6) in the early group versus three (1-4) in the delayed. The most favourable was early primary closure with biological patch. The most unfavourable was delayed closure with patch. Cumulative reported mortality remained high, mostly due to non-surgical causes. CONCLUSION: Definitions of giant omphalocele in the literature were heterogeneous with a variety of management approaches described.

Synthetic versus Biological Patches for CDH: A Comparison of Recurrence Rates and Adverse Events, Systematic Review, and Meta-Analysis.

OBJECTIVE: Our systematic review aims to compare recurrence rates and complications of biological versus synthetic patches for the repair of congenital diaphragmatic herniae. METHODS: Studies from January 1, 1980 to April 25, 2020, with patients under the age of 16 years and with a minimum 6-month follow-up, were included from MEDLINE, Embase, and Cochrane databases. Funnel plots for recurrence rates were constructed for biological and synthetic patches. Subgroup analysis was performed for recurrence rate at the 1-year time-point and data were gathered on individual adverse events from relevant studies. RESULTS: A total of 47 studies with 986 patients (226 biological, 760 synthetic) were included. Funnel plot analysis determined overall recurrence rates of 16.7% for synthetic and 30.3% for biological patches. Subgroup analysis of 493 and 146 patients with synthetic and biological patches, respectively, showed recurrence rates of 9.9 and 26%, respectively.The most commonly used patch types-PTFE (polytetrafluoroethylene) and SIS (small intestinal submucosa)-had 11.5 and 33.3% recurrence, respectively. Adhesive bowel obstruction rates ranged from 4 to 29% in studies that systematically reported it for synthetic, and 7 to 35% for biological patches. Gastroesophageal reflux rates ranged from 25 to 48% in studies that systematically reported it for synthetic, and 21 to 42% for biological patches. Pectus deformity rates were reported as high as 80% for synthetic patches. CONCLUSION: Biological patches appear to have higher recurrence rates than synthetic patches, while skeletal deformities are associated more commonly with synthetic patches. Results of biological patches are mainly using SIS and this may overestimate complications of current superior biological patches.

Gastrojejunal (GJ) tube feeding: developing a service and evaluating associated complications in a paediatric surgical centre.

INTRODUCTION: Gastrojejunal (GJ) tubes are becoming an established alternative method of delivering nutrition to children who do not tolerate gastric feeding. However, there is limited literature surrounding patient outcomes, the longevity of tubes or complications. We aim to highlight the development and evaluation of a service to provide children with GJ tube feeding. MATERIALS AND METHODS: A retrospective case-note review of children either undergoing an initial gastrostomy to gastrojejunal tube conversion or gastrojejunal tube replacement in our tertiary paediatric surgical centre between January 2015 and June 2018. RESULTS: 134 GJ feeding tubes were placed in 33 neurologically impaired children with a median age of 4.9 years (8 months-17 years) having a median 4 tube placements per child (1-11) within the study period. All tubes were 14 or 16 Fr 'AMT G-JET' tubes with a median replacement time of 174.9 days (13-504 days). The most common indication was foregut dysmotility in children with global developmental delay. The complication rate was 34.3% (46 tubes). In the study period, 2 patients (6.1%) reverted to oral feeding, 6 patients (18.2%) to gastric feeding and 25 children (75.7%) continued with jejunal feeding. No child required fundoplication. There were no procedure-related mortalities or mortality. CONCLUSIONS: GJ tube feeding is an effective and safe method of managing children with foregut dysmotility. Many patients do not require permanent jejunal feeding, and thus the reversibility of this method is an asset. A dedicated team is needed to co-ordinate tube replacements and provide efficient long-term jejunal feeding. LEVEL OF EVIDENCE: IV.

A Review on Safety and Outcomes of Mucous Fistula Refeeding in Neonates.

INTRODUCTION: The utility of mucous fistula refeeding (MFR) in neonates with short bowel syndrome is widely debated. Our purpose is to review MFR and outline methods, reported complications, and clinical outcomes (survival, weight gain, dependence on parenteral nutrition [PN], and time to enteral autonomy). MATERIALS AND METHODS: We performed a MEDLINE literature search and reference review from January 1980 to May 2020 for terms ("mucous fistula re-feeding" or "enteral re-feeding") and neonates. We included studies that utilized conventional MFR in the neonatal period. Non-English language articles were excluded. RESULTS: We identified 11 relevant articles. Internationally, there was no consensus on methods of MFR. A total of 197 neonates underwent MFR. Within a single study, four neonates developed major complications; however, the procedure was well tolerated without major complications in 10 of the 11 studies. A mortality of nine patients during MFR highlights the burden of disease within the study population; however, of these, only one was directly attributable to MFR. Minor complications were seldom quantified. Three studies demonstrated a higher rate of weight gain and shorter PN support versus controls. Neonates who underwent MFR had lower chance of anastomotic leak and quicker progression to full feed after reversal versus controls. The influence of microorganisms in MFR was only investigated in one study. CONCLUSION: Current evidence suggests benefits of MFR; however, an international consensus is yet to be reached on the optimal method. A large prospective study investigating the influence of MFR on the enteric system is required.