Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

PURPOSE: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. METHODS: A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. RESULTS: After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. CONCLUSIONS: A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Prospective switch study comparing two irrigation systems for transanal irrigation in children.

BACKGROUND AND STUDY AIMS: Transanal irrigation (TAI) is used in children to treat constipation and incontinence. Belgium has 2 systems available: Colotip® (cheaper, however not designed for TAI) or Peristeen®. PATIENTS AND METHODS: This patient-control switch study is the first to compare 2 TAI systems. Children regularly using Colotip® for TAI were asked to participate, after consent, a visual analogue scale (VAS) rating the system and a 2-week diary (fecal continence, self-reliance, time spent on the toilet, pain, Bristol stool scale, irrigation volume and frequency of enema) were completed. Non-parametric statistics were used. RESULTS: Out of 26 children using Colotip®, 18 (69%) children participated and 5 refused (fear n=1, satisfaction Colotip® system n=7). Of these 18 children (interquartile range: 3-18 years, median 12.5 years, 9 girls) 5 patients stopped Peristeen® (pain n=1, fear n=1 and balloon loss n=3) and 2 were lost from follow up. Dropouts and included patients showed no statistical difference. In the 11 remaining patients, pseudo-continence (p 0.015), independence (p 0.01) and VAS score (p 0.007) were significantly better with Peristeen®, no difference was found in time spent on the toilet (p 0.288) and presence of pain (p 0.785). CONCLUSIONS: In children Peristeen® offered significantly higher pseudo-continence and independency. 30% refused participation because of satisfaction with the Colotip® and 30% spina bifida patients reported rectal balloon loss due to sphincter hypotony. To diminish Peristeen® failure, a test-catheter could be of value. Considering Colotip® satisfaction, both systems should be available. Patient selection for Peristeen® needs further research.

Not all pediatric intestinal polyps are alike.

BACKGROUND/AIMS: In childhood, clinical presentation of intes- tinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identify exceptions. Rare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. METHODS: Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient files were checked for clinical manifestations and histo- pathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. RESULTS: Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. CONCLUSION: Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies.

Rectal red blood loss in a healthy toddler is not always a juvenile polyp.

Aim Heterotopic gastric mucosa is a well-known congenital anomaly in Meckel's diverticula and duplication cysts. Solitary heterotopic gastric mucosa in the rectum is a rare and frequently overlooked abnormality. Starting from a patient history, the literature is searched and all cases reported over the past 20 years are reviewed and compared to a summary of the older cases. Differences between adult and childhood presentation are outlined and our patient is compared with prior reported cases. Case A 3-year-old girl presented with recurrent rectal blood loss caused by heterotopic gastric mucosa without duplication cyst. She was endoscopically treated with two-stage endoscopic surgical dissection (ESD). Up to now, rectal heterotopic gastric mucosa has been reported in 34 adults and 24 children, including this patient. There is an overall male dominance (69%). Presenting complaints in children were recurrent fresh blood loss per anum (96%), pain (46%), perineal ulcers (25%), diarrhoea (8%) and one patient had an ano-cutaneous fistula. Endoscopy revealed a mucosal elevation with a slightly different aspect (33%), a polyp (42%) and a solitary ulcer (25%). Endoscopy in adults reveals more frequently polyps compared to children. Treatment in childhood is mainly surgical where adults are more frequently treated with endoscopic techniques. Conclusion In a child with recurrent rectal bleeding in good general health, it is important to withhold heterotopic gastric mucosa in the differential diagnosis and take sufficient biopsies during endoscopy.

Routine surgical pathology in general surgery.

BACKGROUND: Although pathological analysis provides the definitive diagnosis for most resection specimens, recent evidence suggests that such analysis may be omitted for certain routine samples. This was a retrospective analysis of the value of routine histopathological examination performed in daily general surgical practice. METHODS: All specimens from routine appendicectomies, cholecystectomies, haemorrhoidectomies and inguinal hernia repairs performed between 1993 and 2002 were included. The analysis included a comparison of histological and macroscopic diagnoses, review of preoperative and peroperative findings, and an evaluation of the consequences of routine histopathological assessment on patient management and costs. RESULTS: With the exception of hernia specimens, the rate of submission for routine pathological evaluation was 100 per cent. No hernia sac specimen from more than 2000 interventions revealed aberrant histological findings. Of 311 haemorrhoidectomy specimens three showed malignancy, all of which had a suspicious macroscopic appearance. Of 1465 appendices, only one (0.1 per cent) had a potentially relevant histological diagnosis that was not suspected macroscopically. Among 1523 cholecystectomy specimens, all adenomas (0.6 per cent) and carcinomas (0.4 per cent) were suspected macroscopically or developed in association with a known disease. CONCLUSION: The rarity of incidental histological findings relevant to patient management, especially in the absence of macroscopic abnormalities, suggests that routine histological examination of certain specimens may be omitted. A more elementary role for macroscopic examination of the specimen by the surgeon and the pathologist is proposed.

[Iatrogenic perforation of the gallbladder during laparoscopic cholecystectomy does not influence the prognosis. Prospective study]. / L'effraction de la vésicule lors des cholécystectomies laparoscopiques n'influence pas la morbidité. Etude prospective.

BACKGROUND: Perforation of the gallbladder and spillage of gallstones frequently occur in laparoscopic cholecystectomy. As stones may be lost and as spilled bile is known to be contaminated, influence on morbidity may be expected. AIMS: To evaluate the immediate and late consequences on morbidity of peroperative gallbladder perforation during laparoscopic cholecystectomy (LC) in an universitary hospital center. PATIENTS AND METHODS: One hundred and twenty one LC were prospectively evaluated with a mean follow-up of 30 months. Elective operations on 30 men and 91 women with a mean age of 56.4 years (18-85) were carried out for symptomatic cholecystolithiasis in 97 cases (80%), and in 24 cases for complicated cholecystolithiasis. The "french technique" was used for all LC, with systematic intra-operative cholangiography and ultra Sonography. Thirty-seven (30.5%) LC were performed by surgical trainees, 84 LC by confirmed surgeons. The consequences of ultra-operative gallbladder perforation were evaluated in the immediate postoperative period, especially for septic complications, and thereafter, patients were followed up 1, 6, 12 and 24 months postoperatively. RESULTS: Ultra-operative gallbladder perforation occurred in 24 cases (20%), in 83.3% during gallbladder dissection. Gallstone spillage occurred six times, and all spilled stones were removed. Gallbladder perforation was more frequent (but non significant) in acute cholecystitis (25 vs 19%, ns). A clear correlation to the skill and experience of the surgeon is shown (32.4 vs 14.2%, P =0.01). Gallbladder perforation is accompanied by an elevated (nonsignificant) postoperative morbidity (16.6 vs 7.2%, P =0.62) which is, in fact related to older patient and more acute cholecystitis in this group. No reoperations were necessary. One and two years follow-up revealed no long-term complications specially due to lost gallstones. CONCLUSION: Peroperative gallbladder perforation during LC carries no morbidity, provided a total and complete recuperation of gallstones spilled and local treatment of bile contamination with local irrigation and antibiotics. This complication is correlated to the surgeon's skill and experience.

Giant diverticulum of the colon: report of two new cases and review of the literature.

Diverticulosis coli affects more than one in three individuals older than 65 in the Western world. Giant diverticulum of the colon is an extremely rare complication of diverticular disease; only 113 cases, mostly situated in the colon sigmoideum, have been reported in the world literature. Two new cases of giant diverticulum of the colon sigmoideum, with totally different clinical presentation, diagnosis, and management, are reported-one being the cause of chronic anemia and the other presenting as an acute abdomen. Based on a review of the literature, an update on symptomatology, diagnosis, pathogenesis, and therapeutical options of this rare disorder is provided.

[Use of prosthesis in inguinal hernia repair]. / Place des prothèses dans les cures de hernies inguinales.

AIMS: To evaluate the use of mesh in the evolution of hernia repair technique during a period of twelve years. PATIENTS--METHODS: From january 1991, to december 2002, 1306 patients 1136 men, 190 women, with inguinal hernia, with a mean age of 47.4 years old (15-98) have had an elective surgical repair. 1097 (83.9 per cent) were unilateral. 209 (16.1 per cent) were recurrent hernia. Clinical data, operative technique have been prospectively evaluated: Four techniques were used: Shouldice procedure, Stoppa operation and laparoscopic repair (TEP) in 1992 and Lichtenstein technique since 1993. RESULTS: During the study, meshes were used in 65.7 per cent of the patients respectively 33.3 per cent for Lichtenstein, 26.9 per cent for laparoscopic repair and only 5.3 per cent for Stoppa open procedure. During the study, prosthesis rate increased from 9.1 to 85.4 per cent for primary hernia and from 10 to 100 per cent for recurrent hernias. To day, Stoppa procedure is not yet performed. Herniorraphies decrease from 91.9 to 14.6 per cent. CONCLUSIONS: "Tension free" techniques represent 85.4 per cent of all procedures at the end of the study, specially Lichtenstein operation (65 per cent) which seems to become the new "Gold Standart" in inguinal hernia repair.

Congenital short-bowel; a case study and review of the literature.

A congenital short bowel (CSB) is a rare entity in pediatric surgery. We present the case of a newborn boy with a total small intestinal length of 47 cm, malrotation and gastroesophageal reflux, who is 19 months old at the time of this report. Main treatment steps were Ladd's procedure, a fundoplication and long-term parenteral nutrition. We suggest that missing physiological herniation of the gut into the coelomic cavity may impair normal intestinal growth and rotation and lead to congenital short bowel. Review of all cases reported in the literature shows a considerable mortality of 88%. The limiting factor seems to be reduced motility of the short small bowel causing functional obstruction and liver failure.