The principles of enhanced recovery after percutaneous endoscopic gastrostomy (ERaPEG): a UK tertiary center experience.

PURPOSE: Enhanced recovery after surgery (ERAS) pathways have been shown to improve surgical outcomes and patient satisfaction. The aim of the study was to assess whether the implementation of a perioperative enhanced recovery after percutaneous endoscopic gastrostomy (ERaPEG) pathway based on ERAS principles was safe, satisfactory to parents and improved outcomes. METHODS: Following a quality improvement project, a multimodal ERaPEG pathway was introduced as standard practice within the department and children undergoing elective same-day admission percutaneous endoscopic gastrostomy (PEG) at a single UK tertiary center were prospectively enrolled. Exclusion criteria were patients undergoing other concurrent procedures and those who underwent a laparoscopic assisted/open procedure. Data included patient demographics, underlying diagnosis, indication, length of stay (LOS) and 30-day readmission. Parental experience and satisfaction were determined using a questionnaire including 5-point Likert scales. A retrospective cohort was used for comparison. Data were analyzed using Chi-Square test and Mann-Whitney U tests. RESULTS: Ninety-five patients met the inclusion criteria: 50 pre and 45 post the implementation of ERaPEG. Median age was 3 and 2 years, respectively. Neurodisability was the underlying diagnosis in most patients (84%-pre-ERaPEG; 76%-post-ERaPEG). Most common PEG indication was medication/nutritional supplementation (52%-pre-ERaPEG; 51%-post-ERaPEG). The LOS significantly decreased from a median of 51.5 h (pre-ERaPEG) to 32 h (post-ERaPEG) (p < 0.001). Thirty-day readmission rates were similar (6% vs 11%). Most parents felt that the educational material was easy to access and understand. Post-operatively the majority of parents (≥ 80%) were confident in managing the gastrostomy device, setting up/giving the feeds and also felt that the LOS was appropriate. CONCLUSION: This study shows that the implementation of an ERaPEG pathway significantly reduced LOS following PEG. In addition, the pathway was satisfactory to parents and offered the benefit of improved resource utilization.

Long-gap esophageal atresia: gastric transposition or esophageal lengthening with delayed primary anastomosis? A systematic review.

PURPOSE: This study aims to evaluate different surgical approaches to long-gap esophageal atresia (LGEA) with or without tracheoesophageal fistula (TEF) is unclear. METHODS: A systematic literature review was done comparing gastric transposition versus esophageal lengthening with delayed primary anastomosis in infants with LGEA+/-TEF. The primary outcome was time to full oral feeds. Secondary outcomes were time to full enteric feeds, need for further surgery, growth, mortality, and postoperative adverse events. RESULTS: No comparative studies were found. However, the literature was re-interrogated for non-comparative studies. Four hundred thirty-eight articles were identified and screened, and 18 met the inclusion criteria. All were case series. Forty-three infants underwent gastric transposition, and 106 had esophageal lengthening with delayed primary anastomosis. One study on gastric transposition reported time to full oral feeds, and one study in each group reported growth. Time to full enteric feeds was reported in one study in each group. 30% of infants had further surgery following gastric transposition, including hiatus hernia repair (5/43, 12%) and esophageal dilation (7/43, 16%). Following esophageal lengthening, 62/106 (58%) had anti-reflux surgery, 58/106 (55%) esophageal dilatation and 11/106 (10%) esophageal stricture resection. Anastomotic complications occurred in 13/43 (30%), gastrointestinal in 16/43 (37%), respiratory in 17/43 (40%), and nerve injury in 2/43 (5%) of the gastric transposition group. In the esophageal lengthening group, anastomotic complications occurred in 68/106 (64%), gastrointestinal in 62/106 (58%), respiratory in 6/106 (6%), and none sustained nerve injury. Each group had one death due to a cause not directly related to the surgical procedure. CONCLUSIONS: This systematic review highlights the morbidity associated with both surgical procedures and the variety in reporting outcomes.

Hepatic benign and malignant masses in children: a single UK tertiary centre experience.

PURPOSE: The aim of this study was to review the incidence, spectrum and investigation of liver masses presenting to our tertiary liver unit. METHODS: A retrospective single-centre cohort study of paediatric patients (age < 16 years) diagnosed with a liver mass who were investigated at a UK paediatric hepatobiliary tertiary referral centre. Data relating to the clinical findings at presentation, source of referral, diagnostic investigations and histopathology findings were recorded and analysed. RESULTS: 107 patients were identified between July 2012 and July 2019 (55 females). 69 (64%) patients had benign liver lesions, whereas the remaining 38 (36%) were found to have malignant masses. The liver lesions were most commonly detected incidentally (33%) with the second most common presentation being with palpable mass (29%). 82 (77%) patients were referred from other hospitals. All patients underwent USS, 74% MRI, 36% CT, 5% CEUS, 1% PET and 5% HIDA scan with 47% ultimately undergoing biopsy. 72% of patients had AFP measured. The most common benign liver masses were haemangioma 33 (48%), hepatic cyst 17 (25%) and FNH 12 (19%). Amongst the malignant lesions, 29 (76%) were hepatoblastoma and 3 (8%) were hepatocellular carcinoma. CONCLUSIONS: This large review series demonstrates that just over 1/3 of patients presenting to a paediatric tertiary referral centre with a focal liver lesion had neoplastic liver lesions, despite the published literature commonly stating that 2/3 of paediatric liver masses are malignant.

Paediatric inflammatory multisystem syndrome: temporally associated with SARS-CoV-2 (PIMS-TS) - A mimic of intra-abdominal malignancy.

We report the case of a school-aged boy who presented with clinical features suggesting acute appendicitis. However, further imaging which included CT, demonstrated an inflammatory mass involving the transverse colon raising the suspicion of lymphoma. He then developed intestinal obstruction, and in view of the rapid progression of the disease, he was thought to have non-Hodgkin's lymphoma. He underwent an open excisional biopsy, which revealed a necroinflammatory process and no suggestion of lymphoma or an alternative malignancy or specific diagnosis. His steroid treatment was stopped, and he made a good recovery postoperatively. Positive COVID-19 antibodies, positive response to steroids, results and clinical features were consistent with paediatric inflammatory multisystem syndrome (PIMS-TS), with extensive investigation not offering an alternative diagnosis.While PIMS-TS is a relatively new entity, we believe that this case highlights the importance of it being considered a differential diagnosis of a child presenting with an inflammatory mass.

Enterocolic lymphocytic phlebitis: a rare pathology in children.

Enterocolic lymphocytic phlebitis (ELP) is a rare pathology, usually seen in the adult population. The condition affects the venous vasculature of the intestine sparing the arteries and lymphatics. It is commonly identified histologically after surgical resection making the preoperative diagnosis a clinical challenge. As the condition can lead to intestinal ischaemia and necrosis, surgical resection of the affected segment is the treatment of choice. We report the case of a 9-year-old girl who presented with a 2-day history of lower abdominal pain and anorexia. The initial diagnosis of appendicitis was made clinically and the patient underwent a diagnostic laparoscopy . The appendix was macroscopically normal and there were no other obvious intra-abdominal pathologies to account for her presenting complaints . The appendix was removed, as per the preoperative discussion and consent from parents. She recovered well and was discharged home the following day. The histological examination of the appendix demonstrated ELP. We describe this rare clinical entity in a child especially in view of the scarce published literature in the paediatric population.

Pediatric Surgery during the COVID-19 Pandemic: An International Survey of Current Practice.

INTRODUCTION: Understanding the challenges experienced by pediatric surgeons in the early phases of the pandemic may help identify key issues and focus research. MATERIALS AND METHODS: Two pediatric surgeons from each of the 10 countries most affected by COVID-19 were surveyed over a 10-day period. Data were obtained regarding service provision, infection control, specific surgical conditions, and the surgical workforce. RESULTS: Twenty pediatric surgeons responded. All centers had postponed non-emergency surgery and clinics for nonurgent conditions with virtual consultations being undertaken in 90% of centers. A majority (65%) of centers had not yet knowingly operated on a positive patient. Minimal access surgery was performed in 75% centers but a further 75% had reduced or stopped upper gastrointestinal endoscopy. The management of simple appendicitis was unchanged in 70% centers, patients with intussusception were being referred for radiological reduction in all centers and definitive pull-through surgery for Hirschsprung patients was performed by 95% where washouts were successful. Timing of surgery for reducible neonatal inguinal hernias had changed in 55% of centers and the management of urgent feeding gastrostomy referrals and of inflammatory bowel disease patients failing with biological therapy varied considerably. CONCLUSION: Service provision has been severely affected by COVID-19 leading to an inevitable increase in untreated surgical pathology. Better understanding of extrapulmonary infectivity, the risk of asymptomatic carriage in children, and the reliability of testing for surgical scenarios may allow appropriate use of conventional surgery, including laparoscopy and endoscopy, and rational development of the novel care pathways needed during the pandemic.

Upper Gastrointestinal Bleeding in Children: A Tertiary United Kingdom Children's Hospital Experience.

The aim of this study was to review the aetiology, presentation and management of these patients with upper gastrointestinal bleeding (UGIB) at a tertiary children's unit in the United Kingdom. This was a retrospective single-institution study on children (<16 years) who presented with acute UGIB over a period of 5 years using known International Classification of Diseases (ICD) codes. A total of 32 children (17 males, 15 females) were identified with a total median age at presentation of 5.5 years. The majority (24/32) of patients presented as an emergency. A total of 19/32 presented with isolated haematemesis, 8/32 with isolated melaena and 5/32 with a combination of melaena and haematemesis. On admission, the mean haemoglobin of patients who presented with isolated haematemesis was 11 g/dL, those with isolated melaena 9.3 g/dL and those with a combination 7.8 g/dL. Blood transfusion was required in 3/19 with haematemesis and 3/5 with haematemesis and melaena. A total of 19/32 underwent upper gastrointestinal endoscopy. Endoscopic findings were oesophageal varices (5/19) of which 4 required banding; bleeding gastric ulcer (1/19) requiring clips, haemospray and adrenaline; gastric vascular malformation (1/19) treated with Argon plasma coagulation therapy; duodenal ulcer (3/19) which required surgery in two cases; oesophagitis (5/19); and gastritis +/- duodenitis (3/19). A total of 13/32 patients did not undergo endoscopy and the presumed aetiology was a Mallory-Weiss tear (4/13); ingestion of foreign body (2/13); gastritis (3/13); viral illness (1/13); unknown (2/13). While UGIB is uncommon in children, the morbidity associated with it is very significant. Melaena, dropping haemoglobin, and requirement for a blood transfusion appear to be significant markers of an underlying cause of UGIB that requires therapeutic intervention. A multi-disciplinary team comprising gastroenterologists and surgeons is essential.

Pancreatic tumours in children: diagnosis, treatment and outcome.

PURPOSE: Pancreatic tumours in children are exceedingly rare and as a result constitute a diagnostic and therapeutic challenge to paediatric surgeons. We reviewed our experience with these rare entities. METHODS: Retrospective single institution study on all paediatric pancreatic tumours over a period of 38 years (1973-2011) and literature review. We recorded data relating to the clinical features at presentation, diagnostic evaluation, treatment and outcome. RESULTS: Fourteen patients were identified (eight male). The most common symptoms at presentation were abdominal pain, anorexia and vomiting. Two cases were discovered incidentally. There were 12 primary and 2 metastatic tumours. Tumour types were solid pseudopapillary neoplasm (n = 6), insulinoma (n = 3), pancreatoblastoma (n = 1), congenital pancreatic cyst (n = 1), Burkitt lymphoma of the pancreas (n = 1) and metastatic lesions of other primary tumours (n = 2). Twelve were treated with surgical resection including 2 laparoscopically. Post-surgical complications included acute pancreatitis (n = 2) and pancreatic pseudocyst (n = 2). There was one death from a metastatic tumour and treatment is ongoing in one patient. The remainder has survived. CONCLUSION: Paediatric pancreatic tumours are rare entities and are usually benign. Clinical symptoms are often non-specific and presentation may be late due to tumour inactivity in case of endocrine neoplasms. For most tumours, surgical resection is the optimal treatment which may be successfully performed laparoscopically when the lesion is in the body or tail of the pancreas. Long-term outcome is generally good.

Peristeen (ⓒ) transanal irrigation system for paediatric faecal incontinence: a single centre experience.

Aim. To evaluate the efficacy of the Peristeen (Ⓒ) transanal irrigation system when treating faecal incontinence in children due to chronic idiopathic constipation. Methods. A retrospective study was conducted of the first cohort of patients affected with faecal incontinence and referred to our centre for Peristeen (Ⓒ) transanal irrigation treatment between January 2010 and December 2012. Patients with neurogenic bowel disturbance were excluded. A previously described and validated faecal continence scoring system was used to assess bowel function and social problems before and after treatment with Peristeen (Ⓒ) . Results. 13 patients were referred for Peristeen (Ⓒ) transanal irrigation during the study period. Mean time of using Peristeen (Ⓒ) was 12.6 months (±0.6 months) and mean length of follow-up was 21.2 months (±0.9 months). All patients were noted to have an improvement in their faecal continence score, with a mean improvement from 9.7 ± 1.4 to 14.8 ± 2.7 (P = 0.0008) and a reduction in episodes of soiling and increasing in quality of life scores. Conclusion. In this initial study, Peristeen (Ⓒ) appears to be a safe and effective bowel management system, which improves bowel function and quality of life in children affected with faecal incontinence as a result of chronic idiopathic constipation, Hirschsprung's disease, and anorectal malformations.

Sarcoidosis: clinical mimicry of pulmonary embolism.

Sarcoidosis can manifest with clinical signs suggestive of pulmonary embolism (PE). A 36-year-old male patient presented with a 2-day history of left-sided pleuritic chest pain and dyspnoea. He was hypoxicand tachypnoeic, and initial blood tests showed a positive plasma D-dimer. Subsequent arterial blood gas showed respiratory alkalosis with type 1 respiratory failure. He was suspected to have a PE, and CT pulmonary angiogram (CTPA) was urgently arranged. This latter investigation did not show any impairment in pulmonary arterial blood flow but revealed bilateral hilar and mediastinal lymphadenopathy suggesting sarcoidosis. Serum calcium and ACE were also elevated. The patient was treated conservatively and discharged after 2 days with out-patient endobronchial ultrasound and clinic follow-up arranged.

An ACE diagnosis.

Gaucher's disease is not commonly considered in the differential diagnosis of adult patients with hepatosplenomegaly and increased serum ACE. A 19-year-old girl presented with recurrent epigastric and left hypochondrial pain over a period of 9 years, associated with episodes of nausea and diarrhoea. She was extensively investigated and found to have splenomegaly and raised serum ACE. A screen for haematological disorders was negative. She reported an insect bite during an overseas holiday preceding her symptoms. She was therefore also screened for infectious causes of hepatosplenomegaly but without success. Later on in life, she reported joint pain and discomfort. Sarcoidosis was thought to be the putative cause on more than one occasion. However, the presence of splenomegaly and her relatively young age, led the rheumatologist to the correct diagnosis.