Eicosapentaenoic acid administration ameliorates the progression of liver fibrosis after laparoscopic Kasai portoenterostomy.

PURPOSE: Biliary atresia (BA) poses a persistent challenge characterized by ongoing liver inflammation and subsequent fibrosis even after the clearance of jaundice (COJ). This study aimed to evaluate the therapeutic potential of eicosapentaenoic acid (EPA) in alleviating liver inflammation and limiting fibrosis during the post-COJ phase of BA. METHODS: Among the BA patients undergoing laparoscopic Kasai portoenterostomy (lapKP) between December 2016 and October 2021, EPA (20-40 mg/kg/day) was administered orally to those whose parents consented. The study included patients from January 2014 to October 2021, classifying them into two groups: EPA-treated (Group E) and untreated (Group N). Their liver fibrosis and clinical course at 1 and 2 years post-lapKP were compared. RESULTS: Group E consisted of 25 patients, while Group N comprised 32 patients. Twenty-one patients in Group E and 25 patients in Group N achieved COJ (p = 0.74). Among jaundice-free patients at 1 and 2 years post-lapKP, Group E exhibited significantly lower M2BPGi levels and platelet counts, and Group E showed a significant reduction in Aminotransferase-to-Platelet Ratio Index (APRI) at 2 years post-lapKP. CONCLUSION: Although EPA administration did not improve COJ, it attenuated the progression of liver fibrosis during the 2 years following lapKP in jaundice-free patients. (200/200Words).

First pediatric pelvic surgery with the Senhance® robotic surgical system: A case series.

The Senhance® robotic system (Senhance [Asensus Surgical Inc., Naderhan, NC, USA]) is a new surgical assistive robot following the da Vinci Surgical System that has been demonstrated to be safe and efficacious. Herein, we report the first case series of pediatric pelvic surgery using Senhance. Two anorectoplasties and one rectal pull-through coloanal anastomosis for rectal stenosis were performed in three children (5-9 months, 7-9 kg) using a 10-mm three-dimensional (3D) 4K camera and 3 and 5 mm forceps operated with Senhance. None of the patients had intraoperative complications or a good postoperative course. Pediatric pelvic surgery with Senhance could be performed precisely and safely with a small body cavity. With its beautiful 3D images, motion of forceps with reduced tremor, and availability of 3-mm forceps, Senhance may be better suited for children compared with other models.

Developing an Effective Off-the-job Training Model and an Automated Evaluation System for Thoracoscopic Esophageal Atresia Surgery.

BACKGROUND: Pediatric minimally invasive surgery requires advanced technical skills. Off-the-job training (OJT), especially when using disease-specific models, is an effective method of acquiring surgical skills. To achieve effective OJT, it is necessary to provide objective and appropriate skill assessment feedback to trainees. We aimed to construct a system that automatically evaluates surgical skills based on forceps movement using deep learning (DL). METHODS: Using our original esophageal atresia OJT model, participants were tasked with performing esophageal anastomosis. All tasks were recorded for image analysis. Based on manual objective skill assessments, each participant's surgical skills were categorized into two groups: good and poor. The motion of the forceps in both groups was used as training data. Employing this training data, we constructed an automated system that recognized the movement of forceps and determined the quality of the surgical technique. RESULTS: Thirteen participants were assigned to the good skill group and 32 to the poor skill group. These cases were validated using an automated skill assessment system. This system showed a precision of 75%, a specificity of 94%, and an area under the receiver operating characteristic curve of 0.81. CONCLUSIONS: We constructed a system that automatically evaluated the quality of surgical techniques based on the movement of forceps using DL. Artificial intelligence diagnostics further revealed the procedures important for suture manipulation. LEVELS OF EVIDENCE: Level IV.

Cholestasis after Kasai operation predicts portal hypertension in native liver survivors of biliary atresia: a multicenter study.

PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.

Duodenal duplication cyst at the second part of the duodenum with congenital duodenal position anomaly completely resected by laparoscopic partial duodenectomy: a case report.

BACKGROUND: Duodenal duplication cysts (DDC) are rare duplications of the alimentary tract. Their treatment depends on their size and location. A radical treatment is total resection, if possible. However, partial excision, puncture, and marsupialization can be selected to prevent surgical injury to the pancreaticobiliary tract despite the risk of recurrence. There are some reports of pancreaticoduodenectomy for DDC because of the risk of recurrent symptoms and malignancy. However, this is considered excessively invasive for DDC, particularly in pediatric cases, because of its extremely low rate of malignancy and high morbidity and mortality rates. We encountered a case of DDC with a congenital duodenal position anomaly occurring in the second part of the duodenum. Taking advantage of the congenital duodenal position anomaly, the DDC was completely resected without injuring the pancreaticobiliary duct. CASE PRESENTATION: A 6-year-old boy was diagnosed with a duodenal duplication cyst with obstruction. There was a congenital duodenal position anomaly. The distal second part of the duodenum was the dorsal side of the proximal second part of the duodenum and ascended upward from the proximal second part of the duodenum. The third and fourth parts of the duodenum ran downward to the left and posterior parts of the portal vein, forming the ligament of Treitz. Complete laparoscopic resection of the duodenal duplication cyst and the second to fourth parts of the duodenum, and duodenojejunostomy with retrocolic reconstruction was performed because the duodenum was easily mobilized to the ligament of Treitz owing to the duodenal position anomaly. The duodenojejunostomy with retrocolic reconstruction achieved a more physiologically normal appearance compared to what would have been achieved with a Roux-en-Y reconstruction. The patient was discharged on postoperative day 12 without any complications. CONCLUSIONS: The procedure used in this case might not be easily applied in all laparoscopy cases. However, it could be an option for duodenal duplication cysts with congenital duodenal position anomalies.

A systematic review of Sandifer syndrome in children with severe gastroesophageal reflux.

PURPOSE: Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to diagnosis. We aimed to systematically review available papers regarding SS. METHODS: After presenting our two cases of SS, we systematically reviewed articles published in MEDILINE/PubMed, Cochrane Library, and Web of Science. RESULTS: The meta-analysis included 54 reported cases and 2 of our own cases. Our results showed that all cases achieved symptom improvement with appropriate treatment for GER. Notably, 19 of the 56 cases exhibited anatomical anomalies, such as hiatal hernia and malrotation. Significantly more patients with than without anatomical anomalies required surgery (p < 0.001). However, 23 of the 29 patients without anatomical anomalies (79%) achieved symptom improvement without surgery. Patients who did not undergo surgery had a median (interquartile range) duration to symptom resolution of 1 (1-1) month. CONCLUSION: The primary care providers should keep SS in the differential diagnosis of patients presenting with abnormal posturing and no apparent neuromuscular disorders. Fundoplication may be effective especially for patients with anatomical anomalies or those whose symptoms do not improve after more than 1 month with nonsurgical treatment.

Scoring system for diagnosis and pretreatment risk assessment of neuroblastoma using urinary biomarker combinations.

The urinary catecholamine metabolites, homovanillic acid (HVA) and vanillylmandelic acid (VMA), are used for the adjunctive diagnosis of neuroblastomas. We aimed to develop a scoring system for the diagnosis and pretreatment risk assessment of neuroblastoma, incorporating age and other urinary catecholamine metabolite combinations. Urine samples from 227 controls (227 samples) and 68 patients with neuroblastoma (228 samples) were evaluated. First, the catecholamine metabolites vanillactic acid (VLA) and 3-methoxytyramine sulfate (MTS) were identified as urinary marker candidates through comprehensive analysis using liquid chromatography-mass spectrometry. The concentrations of these marker candidates and conventional markers were then compared among controls, patients, and numerous risk groups to develop a scoring system. Participants were classified into four groups: control, low risk, intermediate risk, and high risk, and the proportional odds model was fitted using the L2-penalized maximum likelihood method, incorporating age on a monthly scale for adjustment. This scoring model using the novel urine catecholamine metabolite combinations, VLA and MTS, had greater area under the curve values than the model using HVA and VMA for diagnosis (0.978 vs. 0.964), pretreatment risk assessment (low and intermediate risk vs. high risk: 0.866 vs. 0.724; low risk vs. intermediate and high risk: 0.871 vs. 0.680), and prognostic factors (MYCN status: 0.741 vs. 0.369, histology: 0.932 vs. 0.747). The new system also had greater accuracy in detecting missing high-risk neuroblastomas, and in predicting the pretreatment risk at the time of screening. The new scoring system employing VLA and MTS has the potential to replace the conventional adjunctive diagnostic method using HVA and VMA.

Laparoscopic Fundoplication in Patients with Ventriculoperitoneal Shunts: A Systematic Review and Our Experience.

Background: Data on the outcomes of laparoscopic fundoplication (LF) in patients with ventriculoperitoneal (VP) shunts are limited. Materials and Methods: We retrospectively evaluated the demographic characteristics and outcomes of patients who underwent LF at our institutions between 2014 and 2022. Then, we systematically reviewed articles in MEDILINE/PubMed, Cochrane Library, and Web of Science. Results: There was no significant difference in terms of the outcomes between patients with VP shunt (n = 10) and those without (n = 96) at our institutions. None of the patients presented with shunt trouble after LF. The meta-analysis included four retrospective studies and our institutional data. In total, 605 patients (55 with VP shunt) underwent LF. Furthermore, 2 (3.6%) of 55 patients (1 with infection and 1 with occlusion) had shunt troubles. The conversion and complication rates, operative time, and length of hospital stay did not significantly differ between patients with VP shunt and those without. Conclusions: LF can be safely performed on children with VP shunts and is associated with a low risk of shunt troubles. The Clinical Trial Registration number is 2022-387.

Surgical Strategies for Neonates with Prenatally Diagnosed Congenital Biliary Dilatation.

BACKGROUND: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. METHODS: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. RESULTS: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. CONCLUSIONS: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. LEVEL OF EVIDENCE: Level IV.

Update on aortopexy and posterior tracheopexy for tracheomalacia in patients with esophageal atresia.

Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea's lateral and anterior-posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.

Preoperative management comprising tube irrigation using a trans-anal indwelling tube for infants with hirschsprung disease can allow single-stage radical surgery.

BACKGROUND: Preoperative management of Hirschsprung's disease (HD) is currently being conducted with the goal of performing single-stage radical surgery without ileostomy. METHODS: We retrospectively reviewed HD cases between 2013 and 2022, as well as their outcomes related to preoperative management. RESULTS: Thirty-nine patients with HD were included in this study, including short-segment HD (30 cases), long-segment HD (4 cases), and total colonic aganglionosis (5 cases). Among these 39 patients, 95% (37 of 39 patients) underwent single-stage radical surgery after management with glycerin enema use (n = 13), irrigation with tube insertion each time irrigation was performed (n = 13), and irrigation using a tube placed in the bowel (n = 11). CONCLUSIONS: Preoperative management of patients with HD allowed for single-stage surgery of long-segment HD and total colonic aganglionosis. Cases that could be managed without performing an emergency enterostomy during the neonatal period were managed with irrigation until radical surgery was performed.

Robotic versus laparoscopic radical surgery for pediatric congenital biliary dilatation: a comparison of surgical outcomes of a single surgeon's initial experience.

PURPOSE: This study aimed to clarify the validity of robot-assisted surgery (RAS) for pediatric patients with congenital biliary dilatation (CBD). METHODS: We retrospectively compared RAS and laparoscopic surgery (LS) for pediatric CBD performed by the same certified surgeon between 2016 and 2022. RESULTS: We included 6 RAS and 12 LS cases in this study. One case of RAS with laparotomy was excluded from the analysis. The patients in the two groups had comparable ages and body weights. The median surgery duration, the suture time per stitch, and the time to drain removal were 385 min, 145 s, and 5 days in the RAS group and 370 min (p = 0.28), 177 s (p = 0.03), and 6 days (p = 0.03) in the LS group, respectively. The time to create the Roux-en-Y limb was significantly longer in the RAS group. Postoperative complications occurred in one RAS case and in four LS cases. CONCLUSIONS: Less anastomotic time per stitch and less time to drain removal suggest that RAS may contribute to accurate suturing and fine intra-pancreatic bile duct dissection. In addition, RAS requiring large movements of forceps in a large surgical field, such as Roux-en-Y creation, is inferior to LS.

Refractory esophageal anastomotic stricture after esophageal atresia surgery improved with retrograde balloon dilatation through gastrostomy followed by laparoscopic fundoplication: a case report.

BACKGROUND: An esophageal anastomotic stricture (EAS) after an esophageal atresia surgery occurs in approximately 4-60% of the cases, and its first-line therapy includes balloon dilatation. Oral balloon dilatation cannot be performed in some EAS cases; conversely, even if dilatation is possible, these strictures recur in some cases, necessitating a surgical procedure for repairing the stenosis. However, these procedures are invasive and have short- and long-term complications. If an EAS recurs repeatedly after multiple balloon dilations, gastroesophageal reflux disease (GERD) may be the underlying cause. A fundoplication procedure may be effective for treating a refractory EAS, as in the present case. CASE PRESENTATION: A neonatal patient with type D esophageal atresia underwent thoracoscopic esophago-esophageal anastomosis at the age of 1 day, and her postoperative course was uneventful. Thereafter, the patient underwent gastrostomy for poor oral intake at the age of 3 months. After gastrostomy, the patient presented with a complete obstructive EAS. Balloon dilatation via the oral route was attempted; however, a guidewire could not be inserted into the EAS site. Hence, retrograde balloon dilatation via gastrostomy was performed successfully. However, the EAS recurred easily thereafter, and laparoscopic anti-reflux surgery was performed to prevent GERD. The anti-reflux surgery cured the otherwise refractory EAS and prevented its recurrence. CONCLUSIONS: Retrograde balloon dilatation is another treatment option for an EAS. When an EAS recurs soon after dilatation, the patient must be evaluated for GERD; if severe GERD is observed, an appropriate anti-reflux surgery is required before dilating the EAS.

Case Report: Retropancreatic fascia hernia protruding into the thoracic cavity through a Bochdalek hernia.

Retropancreatic fascia hernia is a novel internal hernia originating from the retropancreatic fascial defect, which subsequently expands toward the dorsal aspect of the pancreatic body and migrates into the retroperitoneal space. We encountered a rare case of concomitant retropancreatic fascia and Bochdalek hernias. Here, we describe the imaging characteristics of this hernia type and its surgical strategies.

Case report: Laparoscopic gastrojejunostomy for duodenal atresia with situs inversus and preduodenal portal vein: a report of two cases.

Congenital duodenal atresia with situs inversus is occasionally accompanied by a preduodenal portal vein (PDPV), which is incidentally diagnosed during surgery. Duodenoduodenostomy is the most common and effective treatment. However, some patients require other anastomoses. Here, we present two cases of laparoscopic gastrojejunostomy for congenital duodenal atresia with situs inversus and PDPV and describe the reason for selecting gastrojejunostomy. The optimal surgical strategy is patient specific and should be determined based on the patient's general and physical condition.

Preoperative Contrast Examinations Help Determine the Appropriate Cervical Approach for Congenital Gross Type C Esophageal Atresia: A Report of Two Cases.

BACKGROUND Kluth demonstrated that esophageal atresia/tracheoesophageal fistula (EA/TEF) has several anatomical variations and thus requires a preoperative imaging study to determine the surgical strategy. We routinely perform a contrast examination with iodixanol to assess the location of the TEF and the upper end of the esophageal pouch to determine the most appropriate approach. We herein present two cases of type C EA/TEF who successfully underwent radical surgery by a cervical approach based on the information from the contrast examination. CASE REPORT Case 1 was a Japanese boy suspected of type C EA/TEF after birth. A contrast examination with iodixanol showed that a TEF was at the second thoracic vertebra (Th2), as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach; the postoperative course was uneventful. Case 2 was also a Japanese boy suspected of type C EA/TEF. A contrast examination showed that the TEF was at Th1-2, as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach. The patient suffered from congenital tracheal stenosis and required tracheoplasty. However, there were no apparent complications after the surgery. CONCLUSIONS Here, we used the imaging information to adopt the cervical approach in type C EA/TEF cases and concluded that routine preoperative contrast examinations helped assess the TEF location and upper end of the esophageal pouch without significant complications.

Anastomotic time was associated with postoperative complications: a cumulative sum analysis of thoracoscopic repair of tracheoesophageal fistula in a single surgeon's experience.

PURPOSE: This study aimed to evaluate the learning curve of thoracoscopic repair of tracheoesophageal fistula (TEF) by a single surgeon using a cumulative sum (CUSUM) analysis. METHODS: Prospective clinical data of consecutive Gross type-C TEF repairs performed by a pediatric surgeon from 2010 to 2020 were recorded. CUSUM charts for anastomosis and operating times were generated. The learning curves were compared with the effect of accumulation based on case experience. RESULTS: For 33 consecutive cases, the mean operative and anastomosis times were 139 ± 39 min and 3137 ± 1110 s, respectively. Significant transitions beyond the learning phase for total operating and anastomosis times were observed at cases 13 and 17. Both the total operating time and anastomosis time were significantly faster in the proficiency improvement phase than in the initial learning phase. Postoperative complications significantly decreased after the initial anastomosis learning phase but not after the initial total operating learning phase. CONCLUSIONS: Thoracoscopic repair of TEF is considered safe and feasible after 13 cases, where the surgeon can improve their proficiency with the total operation procedure, and 17 cases, which will enable the surgeon to achieve proficiency in anastomosis. Postoperative complications significantly decreased after gaining familiarity with the anastomosis procedure through the learning phase.

A new type of retropancreatic fascia hernia in the supramesocolic space preoperatively misdiagnosed as a diaphragmatic hernia: report of two cases.

BACKGROUND: We encountered two cases of a new type of retroperitoneal hernia. We herein report the unique features of these cases. CASE PRESENTATION: Case 1: A Japanese girl was born at a gestational age of 37 weeks, weighing 2550 g. She underwent laparotomic left diaphragmatic hernia repair for a left Bochdalek hernia at the age of one day. The postoperative course was uneventful; however, chest radiography at the age of 35 days revealed bowel gas in the mediastinum, while computed tomography exhibited intestinal prolapses from the medial side of the mesh into the thoracic cavity. Reoperation was performed at the age of 77 days, showing that the defect hole was not at the diaphragm but in the absence of retropancreatic fascia, which was connected to the posterior mediastinum from the supramesocolic space. The mediastinum space was closed with a suturing spine and artificial mesh, and the defect hole in the pancreatic body was sutured. Case 2: A Japanese boy was born at a gestational age of 40 weeks, weighing 3502 g. He was diagnosed with a left diaphragmatic hernia at birth and underwent laparotomy at the age of two days. Operative findings showed no defect hole in the diaphragm, and no intestine was observed in the abdominal cavity. After close observation of the abdominal cavity, the intestine was found around the pancreatic body, and manual reduction of the intestine was performed. The defect hole existed in the absence of the retropancreatic fascia, which was connected to the extra-pleural space. The defect hole in the pancreatic body was sutured and closed with a non-absorbable thread. CONCLUSIONS: We assumed that our cases were a new type of retroperitoneal hernia, which we named "retropancreatic fascia hernia".

Management of Congenital and Postoperative Chylothorax: Use of Thoracoscopic Lymphatic Leak Ligations with Intraoperative ICG Lymphangiography.

BACKGROUND: Congenital chylothorax (CCT) and postoperative chylothorax (POCT) are rare and difficult to treat. We report our treatment strategy and outcomes for chylothorax, including thoracoscopic surgery with indocyanine-green (ICG) near-infrared fluorescence lymphangiography. METHODS: A retrospective review of patients with CCT and POCT from 2014 to 2021 was performed. After definitive diagnosis, conservative treatments with octreotide, followed by intravenous steroids as needed, were performed. Patients who were refractory to conservative treatment were transferred to surgical treatment, consisting of thoracoscopic lymphatic leak ligations using ICG intraoperative lymphangiography. The effectiveness of conservative and surgical treatment was then examined. RESULTS: We included 19 cases of CCT and 31 cases of POCT. The 31 POCT patients included 23 of 84 postoperative patients with congenital diaphragmatic hernia (CDH), 7 of 54 postoperative patients with esophageal atresia (EA), and 1 of 3 postoperative patients with lymphatic malformation. The efficacy of conservative treatment was 12/19 for CCT, 22/23 for CDH, and 4/7 for EA. Surgical intervention was performed in 10 patients, and the rate of resolution of chylothorax within 3 weeks after surgery was 90%. CONCLUSION: Thoracoscopic lymphatic leak ligations with intraoperative ICG lymphangiography are feasible and useful in patients with chylothorax refractory to conservative treatment. LEVEL OF EVIDENCE: Level IV.