Essential and non-essential paediatric surgery: implications for the future delivery of state health care in the UK.

BACKGROUND: Delivery of health care in the UK faces enormous challenges with the Department of Health driving significant financial cost savings to ensure viability of public health services. We have analysed and modelled the concept of 'essential' and 'non-essential' paediatric surgery linked to the delivery of children's surgery in the NHS in England. METHODS: Operation codes for surgical operations in newborns, children and adolescents were identified and Healthcare Resource Group tariffs-£Stg matched. Operations were designated as 'essential' or 'non-essential' based on the criteria-(1) life saving-neonatal surgery, emergency general surgery of childhood, cancer surgery; (2) debility if uncorrected; (3) aesthetics and (4) culture/attitude. Hospital Episode Statistics (HES) data were accessed and sampled for the total number of paediatric surgical operations-(age range 0-14 years) performed in NHS hospitals from 2009 to 2010. Annual costs (£) of both 'essential' and 'non-essential' operations were then calculated. RESULTS: The commonest 'essential' operations performed in children and adolescents in the year 2009-2010 was appendicectomy at a cost of over £51 million pounds. Costs of performing a selection of 'non-essential' paediatric surgery operations were >£14 million pounds/year. The NHs funds for example almost 11,000 paediatric circumcisions annually at a cost of >£8 million pounds-50% are performed for non-therapeutic reasons. CONCLUSIONS: Surgeons must engage and work actively with health care systems to ensure diminishing financial resources prioritise 'essential' operations for children. Commissioners must embrace evidence-based surgery. 'Essential' and 'non-essential' surgery has wide implications for the sustainability of the NHS and concepts herein developed can be applied to nations worldwide.

Does aggressive surgical resection improve survival in advanced stage 3 and 4 neuroblastoma? A systematic review and meta-analysis.

The role of surgery in the management of advanced staged neuroblastoma (NBL) is controversial. A systematic review and meta-analysis is reported to address robust evidence for curative "gross total tumor resection" (GTR) in Stage 3 and Stage 4 neuroblastoma. Studies were identified using Medline, Embase, and Cochrane databases using pre-specified search terms. Primary outcomes were 5-year overall (OS) and disease-free survival (DFS) after GTR and subtotal resection (STR) in Stage 3 or 4 NBL. Data were analyzed using Review Manager. The Mantel-Haenszel method and a random effects model was utilized to calculate odds ratios (95% CI). Fifteen studies (five Stage 3 and 13 Stage 4) met full inclusion criteria. The pooled odds ratio for 5 year OS in Stage 3 following GTR compared to STR was 2.4 (95% CI 1.19-4.85). In Stage 4 disease, the pooled odds ratio for 5 year overall survival (OS) following GTR compared to STR was 1.65 (95% CI 0.96-1.91); a pooled odds ratio for 5 year DFS following GTR compared to STR was 1.55 (95% CI 1.12-2.14). A clear survival benefit is shown for GTR over STR in Stage 3 NBL only. Though some advantage can be demonstrated for GTR as defined by DFS in Stage 4 NBL GTR did not significantly improve OS in Stage 4 disease.

Management and outcome of paediatric testicular tumours - A 20 year experience.

OBJECTIVE: To report a 20-year experience highlighting management and outcome(s) of paediatric testicular tumours. PATIENTS AND METHODS: All males (< 19 years) with an index diagnosis of testicular tumours during the era(s) 1998-2018 in North West England were identified. Data were collected regarding age at diagnosis, disease stage, surgical operations, tumour biology and outcome(s). RESULTS: A total of 34 male patients were identified. Median age at primary diagnosis was 94 months (range: 0-229 months). Eighteen tumours were benign and 16 malignant. Twenty cases (59%) were recorded in pre pubertal children and 14 (41%) in post pubertal males . In the pre pubertal group (0-11 years) - 15 cases of germ cell tumours (unrelated to germ cell neoplasia in situ - non-GCNIS derived) were recorded, including six yolk sac lesions, eight teratomas and one mixed teratoma/yolk sac tumour (pre-pubertal type). Four males with sex cord-stromal tumours included one juvenile granulosa cell tumour, two Sertoli cell tumours and one Leydig cell tumour. One miscellaneous type tumour notably a papillary cyst adenoma was also identified. In the post pubertal male cohort (>12 years) (n = 14) - four non-GCNIS derived tumours were identified (3 epidermoid cysts and one teratoma), eight cases of germ cell tumour derived from germ cell neoplasia in situ (GCNIS derived) included one teratoma, six with mixed germ cell tumours and one embryonal carcinoma. Two males had sex cord stromal tumours: (Leydig cell and granulosa cell biology). Twenty-eight patients underwent high radical inguinal orchidectomy(s) with one male also requiring retroperitoneal surgery to clear distant locoregional disease and a further single case thoracotomy and metastasectomy. Six patients had lesions suitable for 'testicular sparing' surgery. Six patients had metastatic disease at presentation (18%). Overall study survival was 97%. A single fatality occurred in an adolescent male with a mixed GCT harbouring liver, lung and para-aortic disease who died 48 months after initiating treatment. CONCLUSION: We highlight one of the largest study series of paediatric testicular tumours in the UK and Europe. Non-GCNIS derived tumours accounted for the most common tumour biology (56%). Survival for paediatric testicular tumours is reassuringly generally excellent. Delayed presentation however with a malignant testicular tumour may be associated with poor outcome(s).

Operative management of pediatric ovarian tumors and the challenge of fertility-preservation: Results from the UK CCLG Surgeons Cancer Group Nationwide Study.

BACKGROUND: Ovarian tumors in the pediatric age group are rare. A significant number of children with ovarian mass lesions present "out of hours "as surgical emergencies, and surgical management does not always involve a surgical oncologist. This multicenter study reports how the mode of clinical presentation may influence (i) operation (conventional open vs minimally invasive surgery (MIS)) and (ii) examines if young females presenting as surgical emergency(s) are more likely to undergo total oophorectomy or ovarian sparing surgery. METHODS: Retrospective multicenter study amongst UK pediatric surgical oncology centers. Females <16 years with diagnosis of ovarian tumor from 2006 to 2016 were included. Functional/neonatal ovarian cysts were excluded. RESULTS: Three hundred ten patients with ovarian tumors treated at 12 surgical oncology centers were identified. Mean age at surgery was 11 years [IQR 8-14]. Most common diagnosis were mature teratoma (57%, 177 cases), immature teratoma (10.9%, 34 cases) and cystadenoma (12%, 36 cases). Seventy percent (217) of cases were performed as open procedures. Thirty percent (94) of children underwent MIS. Tumors were significantly smaller in children who underwent MIS. Median tumor size in the laparoscopic group was 6 cm compared to 11 cm in the open group (p < 0.00001). Children who underwent MIS were significantly more likely to have ovary sparing surgery. CONCLUSION: This UK nationwide study demonstrates that ovary-sparing surgery and minimally invasive surgery are still infrequently deployed by pediatric surgeons in the UK. Patients with smaller tumors were more likely to undergo MIS, and more frequently underwent ovary-sparing surgery. In view of the implications on fertility and hormonal health caused by unilateral oophorectomy, it is time to review this current practise and agree consensus guidelines to reduce rates of unnecessary oophorectomy. LEVEL OF EVIDENCE STATEMENT: This is a level II evidence study. It is a retrospective multicentre collaborative study, which summarizes data from a national cohort of children.

Paediatric Horner's syndrome: is investigation for underlying malignancy always required?

OBJECTIVE: Horner's syndrome (HS) is characterised by a triad of ocular miosis, ptosis and anhidrosis. HS may be a subtle sign of occult pathology in otherwise asymptomatic children, neuroblastoma (NBL) being the the most common associated malignant tumour. Despite such knowledge, the incidence of underlying malignancy in children with HS remains unclear and robust evidence to guide best clinical practice is sparse. We performed a systematic review of the literature with the aim of identifying the incidence of NBL in children with HS of unknown aetiology, and establishing if screening for NBL should be routinely performed in this patient population. METHODS: Systematic review of the literature (PubMed and Ovid/Medline database, 1961-2018). RESULTS: The initial search identified 334 manuscripts, of which 8 studies were included in the final analysis. All reports were single-centre retrospective studies without control groups and included a total of 152 patients (age range 0-20 years). All studies investigated patients with HS but without previously established diagnosis. In the studies included, 17 out of a total of 152 patients were diagnosed with a space-occupying lesion. 12 out of the 152 patients were subsequently detected with NBL. CONCLUSION: HS in children may be the first sign of occult malignancy. We report the first systematic review that comprehensively investigates the incidence of malignancy in this unique patient cohort. We show that HS of unknown aetiology in children warrants further investigation(s) to exclude an underlying space-occupying lesion. This should include cross-sectional imaging of the brain, neck and thorax, plus urinary catecholamines for prompt diagnosis and treatment.

Non-cross-linked porcine acellular dermal matrix (Strattice Tissue Matrix) in pediatric reconstructive surgery.

BACKGROUND: A variety of prosthetic materials are used in the pediatric population for abdominal and chest wall reconstruction. Pediatric experience of non-cross-linked porcine acellular dermal matrix is limited to patients following liver transplantation. We review our outcomes in patients in whom this matrix was used. METHODS: A retrospective analysis of patients who underwent abdominal and chest wall reconstruction with a non-cross-linked porcine acellular dermal matrix (Strattice TM) was performed to assess clinical outcomes. RESULTS: The tissue matrix was used in thirteen patients over a three-year period. Eleven had abdominal wall reconstruction and two underwent chest wall reconstruction. Seven procedures were contaminated at the time of surgery. Median age at insertion was 8.1years (5days-18years) with a median weight of 20.6kg (1.9kg-99kg). The tissue matrix failed in one patient with no unanticipated adverse events. CONCLUSION: Future growth and need for reoperation requires special consideration in pediatric patients undergoing abdominal or thoracic wall reconstruction. Non-cross-linked porcine acellular dermal matrix can be safely used for abdominal and chest wall reconstruction in the pediatric population with a number of advantages over previously utilized materials. In our study, children have a favorable risk profile as compared to published adult series.

Gastrointestinal surgery in cystic fibrosis: a 20-year review.

OBJECTIVES: The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF). METHODS: Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental nutrition (TP), and time to full feeds (TFF). Outcome measures for DIOS were: age of onset, number of episodes, and need for laparotomy. RESULTS: Seventy-five of 376 neonates presented with MI. Fifty-four (92%) required laparotomy. Contrast enema decompression was attempted in nineteen. There were no post-operative deaths. Thirty-nine (72%) neonates with MI were managed with stomas. LOS was longer in those managed with stomas (p=0.001) and in complex MI (p=0.002). Thirty-five patients were treated for DIOS. Twenty-five patients were managed with gastrograffin. Ten patients underwent surgical management of DIOS. Overall, MI did not predispose to later development of DIOS. There was a significantly greater incidence of laparotomy for DIOS in children who had MI. CONCLUSION: The proportion of neonates with complex meconium ileus was high (49%) and may explain the infrequent utilisation of radiological decompression. Complex MI or management with stomas both significantly increase LOS. Re-laparotomy rate is high (22%) in MI irrespective of the type of management. DIOS is not a benign condition, particularly when the child has had previous abdominal surgery. Early referral to a surgical team is essential in these children.

Outcomes in distal hypospadias: a systematic review of the Mathieu and tubularized incised plate repairs.

AIM: To compare complication rates after distal hypospadias surgery using the tubularized incised plate (TIP) and Mathieu techniques. METHODS: For this meta-analysis, the Medline (1990-2009), Embase (1990-2009) and Cochrane databases were searched using the keyword 'hypospadias'. Inclusion criteria were: i) primary repairs; ii) distal hypospadias; iii) paediatric case series; iv) standard Mathieu and TIP techniques; v) complication data obtainable from the paper. Cases were only included in the denominator for each complication if explicitly identified in the paper. Fisher's exact was used for statistical analysis, with P < 0.05 considered significant. MAIN RESULTS: 129 papers were selected for full analysis; 23 papers met the inclusion criteria comprising 1872 TIP repairs and 1496 Mathieu repairs. We identified an increased incidence of urethral fistulae with the Mathieu technique (3.8% vs 5.3%, P = 0.028); however, this is lost on subgroup analysis. An increased incidence of meatal stenosis was identified in the TIP group (3.1% vs 0.7%, P < 0.001). CONCLUSIONS: There is no clear consensus on the ideal method of repair for distal hypospadias. Both the Mathieu and TIP techniques have been shown to have low rates of postoperative complications. Comparison between series is difficult because of a lack of consistency in reporting complications and follow up. We advocate uniform standards for reporting outcomes of hypospadias.