Operative Planning of Chest Wall Reconstructions Illustrated by a Large Defect in a Child.

Reconstruction of large chest wall defects is challenging. Here we discuss the process of decision-making in planning chest wall reconstruction, considering the requirements of tumor removal, stabilization of the chest wall, and soft tissue coverage, illustrated by a case of a hemi-chest wall defect in a child. Ewing sarcoma measuring 10 × 9 × 13 cm was resected in a 9-year-old boy, followed by stabilization using a Gore-Tex patch. Due to extension of the oncologic resection far into the superomedial quadrant of the chest, tension-free coverage with a classical latissimus-dorsi flap could not be achieved. Integrating the serratus-anterior muscle into the flap creating a chimeric latissimus-dorsi/serratus-anterior flap allowed for excellent soft tissue coverage of the foreign body. As the skin could be preserved, careful incision planning was necessary to allow for best possible exposure during oncologic resection and flap harvest, while ensuring skin vascularization impaired by underlying tumor resection. Two vertical skin incisions were chosen, one presternal and a second in the mid-axillary fold delineating a large bipedicled skin flap. Postoperative recovery was excellent. Solid skin vascularization and adequate soft tissue coverage of the alloplastic material allowed for the patient to receive two cycles of postoperative radiotherapy without developing wound dehiscence. Careful interdisciplinary planning of skin incisions allowed for good exposure for tumor resection and flap harvest while preserving skin vascularization. Choosing a chimeric latissimus-dorsi/serratus-anterior flap provided larger coverage than a classical latissimus-dorsi flap with minimal additional donor site morbidity. Taken together, we here present a pragmatic solution to a complex problem.

Effect of Centralization on Surgical Outcome of Children Operated for Liver Tumors in Switzerland: A Retrospective Comparative Study.

BACKGROUND: Pediatric liver surgery is complex, and complications are not uncommon. Centralization of highly specialized surgery has been shown to improve quality of care. In 2012, pediatric liver surgery was centralized in Switzerland in one national center. This study analyses results before and after centralization. METHODS: Retrospective monocentric comparative study. Analysis of medical records of children (0-16 years) operated for any liver tumor between 1 January 2001 and 31 December 2020. Forty-one patients were included: 14 before centralization (before 1 January 2012) and 27 after centralization (after 1 January 2012). Epidemiological, pre-, intra-, and post-operative data were collected. Fischer's exact and t-test were used to compare groups. RESULTS: The two cohorts were homogeneous. Operating time was reduced, although not significantly, from 366 to 277 min. Length of postoperative stay and mortality were not statistically different between groups. Yet, after centralization, overall postoperative complication rate decreased significantly from 57% to 15% (p = 0.01), Clavien > III complications decreased from 50% to 7% (p < 0.01), and hepatic recurrences were also significantly reduced (40% to 5%, p = 0.03). CONCLUSION: Centralization of the surgical management of liver tumors in Switzerland has improved quality of care in our center by significantly reducing postoperative complications and hepatic recurrence.

A Role of brachytherapy in bilateral Wilms tumors: A long-term follow-up of three highly selected cases and literature review.

PURPOSE: To describe experience with partial nephrectomy combined with brachytherapy as part of the local management of bilateral Wilms tumor (WT) including a review of the available literature. RESULTS (METHODS AND CASE DESCRIPTION): Between 2011 and 2014, three highly selected patients (age nine months, 16 months, and 4 years) with bilateral WT (two synchronous and one metachronous) underwent enucleation and perioperative brachytherapy to the tumor bed. With a minimum follow-up of 5 years, all three patients are in continuous complete remission with preserved kidney function. CONCLUSIONS: Although nephron sparing surgery aiming at tumor free-margins remains the gold standard for bilateral WT, tumor enucleation followed by brachytherapy may be considered in carefully selected patients at high risk for end-stage kidney failure. Given the rarity and complexity of the procedure, concentration of care of such patients is mandatory.

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry.

INTRODUCTION: Hepatoportoenterostomy (HPE) is the first-line treatment for biliary atresia (BA) patients. This study aims to describe perioperative complications after HPE and to analyze their impact on outcome. MATERIALS AND METHODS: Patients with HPE (Swiss National Biliary Atresia Registry, 1994-2017) were retrospectively analyzed. Perioperative complications were defined as complications occurring up to 30 days after surgery. Surgical complications were defined as directly related to the surgical act; medical complications were defined as any other deviation from the uneventful postoperative course. RESULTS: Sixty-two patients were included. Median age at HPE was 63 days (18-126). Twenty six patients out of 62 (42%) had ≥ 1 complications: 6/62 (10%) surgical, 24/62 (39%) medical, that is, we observed 7 surgical and 28 medical complications. As for medical complications, cholangitis was the most frequent: 19/28 (68%). Lower gestational age at birth correlated with more overall complications (p = 0.02). Age, weight at HPE, syndromic BA, and postoperative steroid administration were not significantly correlated. There were no perioperative deaths. Perioperative complications did not correlate with overall survival (p = 0.14) and survival with native liver (p = 0.55). CONCLUSION: HPE is often associated with perioperative medical complications. Lower gestational age at birth was significantly associated with more complications. Perioperative complications had no impact on overall outcome.

International survey on anticoagulation and antiplatelet strategies after pediatric liver transplantation.

In pediatric LT, anticoagulants and antiplatelet agents are regularly used to reduce the risk of vascular thrombosis. As evidence for optimal strategy is lacking, local practices vary greatly. The present survey aimed to compile an international overview of anticoagulation and antiplatelet strategies in pediatric LT. An online survey was sent to 98 pediatric LT centers in North and South America, Europe, Asia, and Australia. Twenty-four centers answered the survey. 20/24 (83%) use some sort of anticoagulation and antiplatelet therapy, yielding 20 different strategies. Perioperative vascular problems, size of the hepatic artery, and patient weight were the most frequent determinants of changes in anticoagulant and antiplatelet strategy. Early HAT rates were reported to be 5% or less in 79% of responding centers. Anticoagulation and antiplatelet strategies were not significantly associated with early HAT rates (P = 0.63), or with the number of pediatric LTs performed per year and center (P = 0.92). Internationally, there is a wide variety in anticoagulation and antiplatelet strategies after pediatric LT. Efforts must be made to design a prospective multicentric trial to identify the optimal antithrombotic strategy.

Perioperative Complications in Neonatal Surgery: Biliary Atresia and Choledochal Malformations.

Biliary atresia (BA) and congenital choledochal malformations (CCM) are rare. Both pathologies must (BA) or may (CCM) be operated during the neonatal period or early infancy. We briefly describe the classical operative techniques for both pathologies, followed by the most common and severe perioperative complications encountered during and up to 30 days after surgery in children operated for BA and CCM early in life. For patients with BA, intestinal complications represent the most common and hazardous perioperative surgical complications. Cholangitis is the most frequently encountered medical complication post hepato-porto-enterostomy. For CCM, it seems that neonates encounter little perioperative complications; however, reports are scarce; bile leak and/or cholangitis are the most reported. Overall, for patients with CCM, the literature is ambivalent whether more perioperative complications occur in the younger or in the older patient, and whether these occur more frequently in those symptomatic or asymptomatic at operation. It is difficult to give clear advice for when children with asymptomatic CCM should be operated, and benefits and risks must be carefully counterbalanced. Perioperative mortality for both BA and CCM is low and is reported to be around 1 to 2%.

Microsurgery for "wrist" arteriovenous fistula creation in children: a retrospective cohort study.

INTRODUCTION: Creating functional arteriovenous fistulae (AVF) at the wrist is challenging in paediatric patients because of the small calibre of the blood vessels. METHODS: We report our experience with AVF surgery in children younger than 15 years of age using microsurgical techniques. Twenty-five patients underwent AVF surgery between 2003 and 2015 (20 for haemodialysis, 4 for plasmapheresis and 1 for parenteral nutrition). Median (range) age was 9 (2-15) years and median weight was 24 (8-61) kg. RESULTS: The one-month occlusion rate was 8%. The primary and secondary patency rates at 1, 2, 3 years were: 60%, 49%, 42%, and 82%, 72%, 54%, respectively. The median (range) maturation time was 4.53 (0.5-11.2) months. We found no statistically significant effect of patient age, body weight, type of AVF and indication for AVF creation on the primary and secondary patency rates. CONCLUSIONS: Microsurgical AVF creation at the wrist can be performed with satisfactory results and should be the preferred technique in the paediatric population.

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study.

INTRODUCTION: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood. METHODS: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively. RESULTS: Between January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0-17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up. CONCLUSION: Surgery for CM carries a significant short- and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.

The timing of surgery of antenatally diagnosed choledochal malformations: A descriptive analysis of a 26-year nationwide cohort.

INTRODUCTION: Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms. METHODS: Using the Netherlands Study group on CHoledochal Cyst/malformation (NeSCHoc) we retrospectively collected demographic, biochemical and surgical data from all Dutch patients with an antenatally detected CM. RESULTS: Between 1989 and 2014, antenatally suspected CM was confirmed in 17 patients at a median age of 10days (1day-2months). Four patients developed symptoms directly after birth (24%). Thirteen patients (76%) remained asymptomatic. Two of these progressed to symptoms before surgical intervention at 0.7 and 2.1months resp. Postoperatively, four patients developed short-term complications and three developed long-term complications. Patients <5.6kg (the series median) showed more short-term complications (66%) when compared to patients >5.6kg (0%, p=0.02). CONCLUSION: When not symptomatic within the first days of life, the majority of children with antenatally detected CM remains asymptomatic. Surgery might safely be delayed to the age of 6months or a weight of 6kg. Postponing surgery in the clinically and biochemical asymptomatic patient might decrease the complication rate. LEVELS OF EVIDENCE: Level III.

Surgical treatment of childhood hepatoblastoma in the Netherlands (1990-2013).

BACKGROUND: Achievement of complete surgical resection plays a key role in the successful treatment of children with hepatoblastoma. The aim of this study is to assess the surgical outcomes after partial liver resections for hepatoblastoma, focusing on postoperative complications, resection margins, 30-day mortality, and long-term survival. METHOD: Chart reviews were carried out on all patients treated for hepatoblastoma in the Netherlands between 1990 and 2013. RESULTS: A total of 103 patients were included, of whom 94 underwent surgery. Partial hepatectomy was performed in 76 patients and 18 patients received a liver transplant as a primary procedure. In 42 of 73 (58 %) patients, one or more complications were reported. In 3 patients, information regarding complications was not available. Hemorrhage necessitating blood transfusion occurred in 33 (45 %) patients and 9 (12 %) patients developed biliary complications, of whom 8 needed one or more additional surgical interventions. Overall, 5-year disease-specific survival was 82, 92 % in the group of patients who underwent partial hepatectomy, and 77 % in the group of patients who underwent liver transplantation. CONCLUSIONS: Partial hepatectomy after chemotherapy in children with hepatoblastoma offers good chances of survival. This type of major surgery is associated with a high rate of surgical complications (58 %), which is not detrimental to survival.

Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons.

BACKGROUND: Choledochal malformation (CM) is a rare medical condition of which 80% are diagnosed in pediatric patients. There are several important controversies regarding diagnostic workup, management, and follow-up in these pediatric patients. To assess preferences and practices of Dutch pediatric surgeons regarding the diagnostic procedures, management, and follow-up of children with CM we conducted an electronic survey. METHODS: A questionnaire was sent to all the pediatric surgeons working in the academic centers and the only community hospital with a pediatric surgery service. The questionnaire included, items regarding incidence, diagnostic workup, interval between diagnosis and surgery, surgical techniques, and follow-up. We also assessed whether personal exposure influenced the preferences and practices. RESULTS: Overall 22 out of the 31 (71%) Dutch pediatric surgeons returned the questionnaire. Total 15 out of 22 (68%) encountered CM up to 2 times/y, whereas 7 out of 22 (32%) encountered it more than 2 times/y. Indications for surgery were significantly different between surgeons who encountered CM > 2 time/y versus those who did not: 6/6 (100%) of surgeons encountering CM > 2 times/y considered the presence of an asymptomatic CM an indication for surgery versus 5/14 (36%) of the pediatric surgeons who encountered a CM up to 2 times/y (p = 0.01). Overall 12 out of the 22 (55%) respondents preferred surgery between 6 months and 2 years of age. The amount of exposure did not differ in preferred age at surgery or surgical technique. In the symptomatic child 10/22 (45%) of respondents preferred surgery within 3 months. Overall 7/22 (32%) favored laparoscopic resection. Hepaticojejunostomy with Roux-en-Y reconstruction was the preferred reconstruction for all the respondents. One-third stated that they never performed a parenchyma resection. Follow-up was limited to 10 years in almost half of the respondents. CONCLUSION: Dutch pediatric surgeons demonstrate a wide variety of opinions regarding diagnostic workup, treatment, and follow-up of CM. While most surgeons encounter CM up to 2 times/y, there is an association between exposure and several of the outcome parameters. Some of the answers are not in line with the expert opinion. This demonstrates that there is a need for evidence-based (inter)national guidelines regarding the diagnostic approach, management, and follow-up.

Nephron sparing surgery (NSS) for unilateral wilms tumor (UWT): the SIOP 2001 experience.

BACKGROUND: Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors. AIM: Inventory of the current SIOP NSS-experience. PROCEDURES: 2,800 patients with a unilateral, localized or metastatic and an unequivocal surgical technique recorded were included. All had neo-adjuvant chemotherapy and delayed surgery. In 91 (3%) NSS was performed and in 2709 TN. Data was retrieved from the SIOP WT 2001 database. RESULTS: NSS group contained 65% stage I tumours and the TN group 48%. Tumor volume (at diagnosis and surgery) was significantly smaller in the NSS group. Within stage III, after NSS, 7/12 (58%) had positive margins (M +), 5 with tumor negative lymph nodes (LN-). After TN, 355/712 (55%) had M + , 182 were LN-. Treatment of M+ in the NSS group resulted in two conversions to TN (one combined with radiotherapy), three patients had radiotherapy only and in two patients local therapy, if given, was not recorded. After NSS, four recurrences occurred. For localized disease the 5-year overall (OS) and event free survival (EFS) in NSS group was 100 and 94.8 (95% CI:89.9-99.9), respectively, while OS and EFS in the TN group were 94.4 (95% CI: 93.2-95.5, log-rank test P = 0.06) and 86.5 (95% CI:85.0-88.1, log-rank test P = 0.06), respectively. CONCLUSIONS: NSS was only performed in 3% of patients with uWT. Despite excellent survival with few relapses, the gain of nephrons needs to be weighed against the risk to induce stage III with intensified therapy.

Endoscopic management of biliary complications after partial liver resection in children.

BACKGROUND: Biliary complications after liver surgery are difficult to manage. Endoscopic retrograde cholangiopancreatography (ERCP) with stenting of the common bile duct is not commonly practiced in children for this purpose. The aim of this retrospective review is to evaluate the role of ERCP as both a diagnostic and a therapeutic tool in the management of biliary complications after liver resection in children. PATIENTS AND METHODS: The charts of all patients from 0 to 18 years old who underwent partial liver resection in a tertiary children's hospital in Amsterdam, the Netherlands, between 2000 and 2010 were retrospectively reviewed. RESULTS: Forty-five children (median age: 3.6 years, range: 2 months-17 years) underwent partial liver resection. Post-operative biliary complications occurred in 13 children. Ten patients were suffering from bile leakage. Eight of them underwent ERCP with stent placement after which leakage stopped in 5 patients. Three patients presented with a post-operative biliary tract stricture. ERCP with dilation and stent placement was performed in 2 of them, which solved the problem in one patient. ERCP demonstrated the nature (bile leak and/or biliary tract stricture(s)), extent, and location of the lesion in 8 of 10 children. There were no serious procedure related complications. Rescue procedures in the other patients included hepaticojejunostomy and liver transplant. CONCLUSION: ERCP with stenting of the common bile duct has a diagnostic and therapeutic role in the management of bile leaks after partial liver resection in children. The value of ERCP in the management of a stricture of the biliary tract is less conclusive.

Memory CD4(+)CCR5(+) T cells are abundantly present in the gut of newborn infants to facilitate mother-to-child transmission of HIV-1.

Despite potential clinical importance, target cells for mother-to-child transmission of HIV-1 have not yet been identified. Cord blood-derived CD4(+) T cells are largely naive and do not express CCR5, the mandatory coreceptor for transmitted HIV-1 R5 strains in infants. In the present study, we demonstrate that in the human fetal and infant gut mucosa, there is already a large subset of mucosal memory CD4(+)CCR5(+) T cells with predominantly a Th1 and Th17 phenotype. Using next-generation sequencing of the TCRß chain, clonally expanded T cells as a hallmark for memory development predominated in the gut mucosa (30%), whereas few were found in the lymph nodes (1%) and none in cord blood (0%). The gut mucosal fetal and infant CD4(+) T cells were highly susceptible to HIV-1 without any prestimulation; pol proviral DNA levels were similar to infected phytohemagglutinin-stimulated adult PBMCs. In conclusion, in the present study, we show that extensive adaptive immunity is present before birth and the gut mucosa is the preferential site for memory CD4(+) T cells. These CD4(+)CCR5(+) T cells in the infant mucosa provide a large pool of susceptible cells for ingested HIV-1 at birth and during breastfeeding, indicating a mucosal route of mother-to-child transmission that can be targeted in prevention strategies.

[A boy with an abdominal mass]. / Een jongen met een zwelling in de buik.

A 11-year-old boy presented with an abdominal mass in the right upper abdomen. As a newborn he was analyzed for this mass, diagnosed as an adrenal hemorrhage. Abdominal X-ray and CT revealed a fetus-in-fetu, which was removed surgically.

The role of plain radiography in paediatric wrist trauma.

OBJECTIVES: Acute wrist trauma in children is one of the most frequent reasons for visiting the emergency department (ED). Radiographic imaging in children with wrist trauma is mostly performed routinely to confirm or rule out a fracture. The aim of this study was to determine how many radiographs of the wrist show a fracture in children following wrist trauma. METHODS: A retrospective cohort study was performed in three Dutch hospitals from 2009-2010. Data were extracted from patient records and radiographic reports. RESULTS: Of the 1,223 children who presented at the ED after a wrist trauma, 51 % had a wrist fracture. The peak incidence of having a wrist fracture was at the age of 10 years; 65 % of the children younger than 10 years of age had a wrist fracture. Of all the patients without a wrist fracture, 74 % were older than 10 years of age. CONCLUSION: Almost half of the paediatric patients with a trauma of the wrist had normal radiographs. The development of a clinical decision rule to determine when a radiograph of the wrist is indicated following acute wrist trauma is needed. This could likely reduce the number of radiographs. MAIN MESSAGES: • Fifty-one percent of the children with wrist trauma have a wrist fracture. • Peak incidence of having a wrist fracture is at the age of 10 years. • Sixty-five percent of the children younger than 10 years of age had a wrist fracture. • Of all the patients without a wrist fracture, 74 % were older than 10 years of age. • The development of a clinical decision rule to reduce the number of radiographs is needed.

[Dysphagia after introduction of solid food: typical presentation of congenital oesophageal stenosis]. / Dysfagie na introductie van vast voedsel: typische presentatie van congenitale slokdarmstenose.

BACKGROUND: Congenital oesophageal stenosis is a rare cause of food passage symptoms in infants. It has a typical presentation with symptoms of dysphagia of solid food, starting at the time of introducing supplementary feeding. CASE DESCRIPTION: We present a 6-month-old girl, who started spitting and coughing and had a slower growth rate after the introduction of solid food. Using upper gastrointestinal tract radiography, oesophagogastroscopy and histopathological examination, a congenital oesophageal stenosis due to tracheobronchial remnants was demonstrated. The stenosis was surgically removed. This case description is typical for congenital oesophageal stenosis. CONCLUSION: Early recognition of the typical presentation of congenital oesophageal stenosis can prevent unnecessary investigation and delay in treatment. Surgical resection of the stenotic oesophageal segment usually results in full recovery.

The failure rate of nonoperative management in children with splenic or liver injury with contrast blush on computed tomography: a systematic review.

PURPOSE: Nonoperative management (NOM) is the treatment of choice for hemodymically stable pediatric patients with spleen or liver trauma. The aim of this study was to assess the failure rate of NOM in children with blunt liver and/or splenic injury when a contrast blush is present on a computed tomography (CT) scan. METHODS: A systematic review of the literature published between 1985 and 2009 was performed by searching the EMBASE and MEDLINE database for English and German articles. Articles were eligible if they reported the failure rate of NOM with or without angioembolization (AE) in pediatric patients with splenic and/or liver injuries with a contrast blush on CT and included 2 or more trauma patients. Two reviewers independently assessed the eligibility and the quality of the articles and performed the data extraction. Interrater differences were resolved by discussion. RESULTS: Nine studies were included describing 117 pediatric patients. The median sample size was 5 (range, 2-44). Seven studies (including 71 patients) reported a total of 16 patients with failure after NOM without AE. Failure rates across these studies ranged from 4.5% to 100%; the pooled percentage was 28.2% (95% confidence interval, 8.9%-61.3%). The failure percentages after NOM with or without AE ranged from 0 to 100%; the pooled percentage was 21% (95% confidence interval, 7.5%-46.8%). Two studies (including 46 patients) reported a total of 3 patients (6.5%) with failure after NOM with primary AE. CONCLUSION: Despite the current low level of evidence on failure rate of NOM when a contrast blush is present on CT, we emphasize that there is a significant number of patients in whom NOM fails. We therefore recommend that the management of splenic and hepatic injury in children should not only be based on the physiologic response but should include consideration of the presence of a contrast blush.

The use of the Statscan digital X-ray unit in paediatric polytrauma.

We present a 3-year review of clinical paediatric experience with the Statscan (Lodox Systems, Johannesburg, South Africa), a low-dose, digital, whole-body, slit-scanning X-ray machine. While focusing on the role of the unit in paediatric polytrauma, insight into its applications in other paediatric settings is provided.