Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children.

PURPOSE: Choledochal cysts have traditionally been treated as an open procedure. However, recent publications from some large volume centres show that minimally invasive surgery has become their standard approach. Robotic surgical systems facilitate a surgeon's ability to undertake complex procedures. We present our experience of using robot assistance to treat choledochal cysts. METHODS: Children admitted with a choledochal cyst from March 2009 to Nov 2012 were included. There were 27 patients (20 females). Mean age 5.4 years (range 0.3-15.9). Mean weight 21.9 kg (range 5.9-78.8); 10 weighed <10 kg. The choledochal cysts were types 1c(12), 1f(10) and 4(5). The da vinci Surgical System was used for the robot-assisted resection of the choledochal cyst and hepaticojejunostomy. Roux loop was fashioned extra-corporeally. Mean follow-up is 2.7 years (range 0.9-4.7). RESULTS: 22 cases were completed successfully with robotic assistance. Five open conversions were for anatomical concerns or technical reasons. One child had three complications (omental hernia, anastomotic stricture and subsequent bile leak). Patients were on full feeds by a median of 5 days (range 3-6) and discharged after 6 days (range 4-7). CONCLUSION: Robotic-assisted resection of choledochal cysts in children is safe. They made a rapid recovery with a good cosmetic outcome.

Early and Long-term Complications of Robotic Assisted Laparoscopy in Infants and Children.

BACKGROUND: Robotic-assisted laparoscopy still lacks wide acceptance in infants and children. We developed the service and report the largest single institution experience of complications over a period of 11 years. METHODS: Between March 2006 and May 2017, consecutive infants and children who underwent robotic assisted laparoscopy under the care of two laparoscopic surgeons were studied. Data for patients, surgeons, year of surgery, operation, and timing, nature, grades of complications were assessed. RESULTS: A total of 601 robotic procedures (45 different types) were carried out in 539 patients. Of these 31 (5.8%) were converted, none for operative complications. These and another 4 with complicated co-morbidity were excluded, leaving 504 patients for further analysis. There were 60 (11.9%) complications in 57 (11.3%) patients. Mean (SD) age was 7.7 years -/+5.1 with the youngest being 4 weeks. Concomitant or bilateral robotic and non-robotic procedures took place in 8.1% and 13.3% of patients respectively. Significant medical co-morbidity and abdominal scarring were present in 29% and 14.9% of patients respectively. Complications occurred in theatre 1.6%, hospital 5.6%, 28 days 1.2%, and late 3.6%. Mean follow up was 7.6 years -/+ 3.1 SD. Over-all postoperative complication rate was 10.3%: CD grade I 6.5% (33), II 0.6% (3), and IIIa/b 3.2% (16) which included 1.4% (7) re-do surgery. Most (11/16) grade III occurred late. There were no bleeding, grade IV or V complications, surgical mortality, or technology related complications. CONCLUSIONS: Complications are low even during the learning phase and while developing the new technique. Most complications occurred early and were minor. Most high-grade complications presented late. LEVELS OF EVIDENCE: 2B.

Incidence of Isolated Biliary Atresia during the COVID Lockdown in Europe: Results from a Collaborative Project by RARE-Liver.

BACKGROUND: Biliary atresia (BA) is a rare cholangiopathy where one of the proposed aetiological mechanisms is an infectious viral trigger. Coronavirus disease-19 (COVID) lockdown restrictions were implemented to reduce the transmission of infections. Strictness of lockdown varied across European countries. This study aimed to investigate if there was an association between strictness of lockdown and change in isolated BA (IBA) incidence in Europe. METHODS: We approached European centres involved in the European Reference Network RARE-LIVER. We included IBA patients born between 2015 and June 2020. We calculated the number of IBA patients born per centre per month. The Stringency Index (SI) was used as lockdown strictness indicator. The association between percentage change of mean number of IBA patients born per month and the SI was assessed. RESULTS: We included 412 IBA patients from thirteen different centres. The median number of patients per month did not change: 6 (1-15) pre-lockdown and 7 (6-9) during lockdown (p = 0.34). There was an inverse association between SI and percentage change in IBA (B = -0.73, p = 0.03). Median age at Kasai portoenterostomy (days) did not differ between time periods (51 (9-179) vs. 53 (19-126), p = 0.73). CONCLUSION: In this European study, a stricter COVID-lockdown was seemingly accompanied by a simultaneous larger decrease in the number of IBA patients born per month in the lockdown. Results should be interpreted with caution due to the assumptions and limitations of the analysis.

Conversions in pediatric robot-assisted laparoscopic surgery.

BACKGROUND: New technology attracts necessary concerns regarding safety and effectiveness, including the risk and circumstances of conversions. This study analyses our 11-year experience of conversions from a dedicated pediatric robot-assisted laparoscopic surgery service. METHODS: Consecutive patients were evaluated from a prospective database for the period March 2006 to May 2017. Descriptive and quantitative data for conversions were analysed. Variables were investigated including year of surgery, patient age, operation type, surgeon, and experience. RESULTS: A total of 539 children underwent 601 procedures. There were 45 different types of procedures. Mean (± SD) age was 7.0 ± 5.2 years, and youngest 4 weeks old. There were 31 conversions (5.8%). Conversion rates were significantly higher in younger children, with rates of 10.0%, 6.1% and 4.2% for age groups 0-2, 2-6, and 6-18 years respectively (p = 0.01). There was a significant difference in conversion rates amongst procedures (p<0.001). Intravesical (9/26, 34.6%), liver cyst (2/8, 25.0%) and choledochal cyst (8/60, 13.3%) procedures were the highest. The most frequent reason for conversion was anesthetic related issues caused by dilated intestine and/or inadequate muscle relaxation (16/31, 52%). Three conversions were attributable to mechanical failures, none attributed to intraoperative complication. CONCLUSIONS: Conversion rates are low even in the learning phase and comparable favourably to conventional laparoscopy, supporting the safety and effectiveness of robot-assisted technology for advanced laparoscopy in children. The importance of optimization of surgical conditions cannot be overemphasised.

Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children less than 10 kg.

BACKGROUND/PURPOSE: Laparoscopic resection of choledochal cysts and hepaticojejunostomy have been described in children since 1995, but these can be technically demanding procedures. Robotic surgical systems can facilitate complex minimal-access procedures. In 2009, we made the transition from conventional laparoscopic to robotic-assisted choledochal cyst excision with hepaticojejunostomy. We present our experience in children weighing less than 10 kg. METHODS: During 2009, 5 children weighing less than 10 kg underwent robotic resections of choledochal cysts and hepaticojejunostomy using the da Vinci surgical system. The Roux loop was fashioned extracorporeally. Mean age was 1 year (range, 0.5-1.4), and mean weight was 8.5 kg (range, 7.6-9.5). All 5 had type 1c cysts, and 3 were very large. RESULTS: All 5 cases were treated successfully by robotic resection of the cyst and hepaticojejunostomy. Feeding was established by a median of 4 days (range, 3-6), and patients were discharged after a median of 6 days (range, 5-7) with no postoperative complications. CONCLUSION: The technique is safe and effective in children weighing less than 10 kg. The authors found ergonomic advantages in using robotic-assisted surgery for this complex minimal-access procedure.

Is cholecystectomy really an indication for concomitant splenectomy in mild hereditary spherocytosis?

BACKGROUND/AIM: The British Committee for Standards in Haematology currently recommends concomitant splenectomy in children with mild hereditary spherocytosis (HS) undergoing cholecystectomy for symptomatic gallstones. However, splenectomy is associated with a risk of life-threatening infection, particularly in young children. The aim of this study was to audit the outcome of the practice of uncoupling splenectomy and cholecystectomy in such patients. METHODS: Children referred with symptomatic gallstones complicating HS between April 1999 and April 2009 were prospectively identified and reviewed retrospectively. During this period, the policy was to undertake concomitant splenectomy only if indicated for haematological reasons and not simply because of planned cholecystectomy. RESULTS: A total of 16 patients (mean age 10.4, range 3.7 to 16 years, 11 women) with HS and symptomatic gallstones underwent cholecystectomy. Three patients subsequently required a splenectomy for haematological reasons 0.8-2.5 years after cholecystectomy; all three splenectomies were performed laparoscopically. There were no postoperative complications in the 16 patients; postoperative hospital stay was 1-3 days after either cholecystectomy or splenectomy. The 13 children with a retained spleen remain under regular review by a haematologist (median follow-up 4.6, range 0.5 to 10.6 years) and are well and transfusion independent. CONCLUSIONS: The advice to perform a concomitant splenectomy in children with mild HS undergoing cholecystectomy for symptomatic gallstones needs revisiting. In the era of minimal access surgery, the need for splenectomy in such children should be judged on its own merits.

Mesenchymal hamartoma of the liver: a systematic review.

Mesenchymal hamartoma of the liver is the second commonest benign liver tumor in children, yet its biology and pathogenesis are poorly understood. Cytogenetic studies have suggested that the tumor may be a neoplasm rather than a hamartoma. Typically, it presents as a large benign multicystic liver mass in a child younger than 3 years amenable to complete resection. However, its imaging characteristics are variable, ranging from a few large cysts to a solid mass occupying one or both lobes of the liver. In addition, the tumor occasionally contains angiomatous elements or is multifocal. Most tumors gradually increase in size, some reaching enormous proportions, which can make surgery challenging. Paradoxically, a few undergo incomplete spontaneous regression and, on rare occasions, others have shown malignant transformation to undifferentiated (embryonal) sarcoma. These unusual pathological and biological features must be taken into account when considering the management of affected individuals.