Prognostic factors for pediatric patients with severe intestinal motility disorders: a single institution's experience.

PURPOSE: To identify the prognostic factors for pediatric severe intestinal motility disorder (IMD). METHODS: We reviewed the medical records of patients with severe IMD, who required total parenteral nutrition (TPN) for ≥ 60 days at our institution between April, 1984 and March, 2023, examining their characteristics to identify prognostic factors. RESULTS: The types of IMD in the 14 patients enrolled in this study were as follows: isolated hypoganglionosis (IHG, n = 6), extensive aganglionosis (EAG: n = 6), and chronic idiopathic intestinal pseudo-obstruction (CIIP, n = 2). There was no significant difference in mortality among the three types of severe IMD. Weaning-off TPN and the use of the colon were not significant prognostic factors, but cholestasis was a significant prognostic factor (p = 0.005). There was a high mortality rate (50%), with the major causes of death being intestinal failure-associated liver disease (IFALD) following hepatic failure, and catheter-related blood stream infection (CRBSI). One IHG patient underwent small bowel transplantation but died of acute rejection. CONCLUSION: Severe IMD is still associated with a high mortality rate and cholestasis predicts the prognosis. Thus, preventing or improving IFALD and CRBSI caused by long-term TPN is important for reducing the mortality rate.

Congenital Risk Factors for Chronic Kidney Disease in Patients With Persistent Cloaca: Results From a Nationwide Survey in Japan.

BACKGROUND: We conducted a nationwide survey of persistent cloaca (PC) to investigate the renal function outcomes and factors affecting chronic kidney disease (CKD) in patients with PC. METHOD: Information from 466 patients with PC was obtained via a questionnaire in this study. The 290 patients (62.2%) with renal function data were classified into 2 groups based on their estimated glomerular filtration rate: advanced CKD group (<30 mL/min/1.73 m2 [or post-renal replacement therapy]) and non-advanced CKD group (≥30 mL/min/1.73 m2). Univariate and multivariate analyses were performed to identify risk factors for CKD that may affect the renal function, including renal and urinary tract malformations, associated anomalies, and urinary tract treatment. The advanced CKD group was divided into two groups based on age to evaluate age-related differences (younger- and older-age CKD groups). RESULTS: A regression analysis revealed that congenital renal malformations (odds ratio [OR]: 14.06, 95% confidence interval [CI]:3.07-131.65, p < 0.0001), urinary tract obstruction (OR:4.28, 95%CI:1.12-24.23, p < 0.05), and sacral agenesis (OR:4.54, 95% CI:0.84-30.67, p < 0.05) were significantly associated with advanced CKD. In the univariate analysis of factors affecting the renal prognosis, clean intermittent catheterization (CIC) (OR:4.18, 95%CI:1.21-16.45, p = 0.015), vesicostomy (OR:3.65, 95%CI:1.11-12.98, p = 0.019), and surgery for vesicoureteral reflux (OR:5.43, 95%CI:1.41-22.73, p = 0.006) were significantly associated with advanced CKD. Based on the univariate analysis, hydrometrocolpos was significantly more prevalent in the older-age CKD group compared to the younger-age CKD group (p < 0.05). CONCLUSION: CKD development in patients with PC is influenced by a complex interplay of factors, including renal malformations and neurogenic bladder dysfunction due to spinal anomalies. LEVEL OF EVIDENCE: III (Study of Diagnostic Test, Study of nonconsecutive patients, and/or without a universally applied "gold" standard).

Inflammation patterns in early post-operative cholangitis predict long-term outcomes in biliary atresia: a potential role of non-suppurative cholangitis.

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

An ultrasound-guided supraclavicular approach for tunneled central venous catheter insertion can be safely performed by junior residents.

PURPOSE: Ultrasound-guided supraclavicular catheterization (UGSC) of the brachiocephalic vein (BCV) for long-term tunneled central venous catheter (tCVC) insertion may be safer than the internal jugular vein approach due to its superior field of view. We examined the clinical outcomes of tCVC insertions performed by junior residents through UGSC of the BCV. PATIENTS AND METHODS: From January 2018 to December 2023, we assessed clinical outcomes and compared the experience levels of surgeons conducting tCVC insertions. Surgeons were categorized into three groups: junior residency (JR), senior residency (SR), and board-certified pediatric surgeons (BCPS). RESULTS: 177 tCVC insertions were done on 146 patients. Intraoperative complications included 6 cases of arterial puncture, 1 case of pneumothorax, 1 case of over insertion of catheter tip, and 1 case of suspected hemothorax. Distribution across groups was as follows: 28 cases (15.8%) in JR group, 92 (52.0%) in SR group, and 57 (32.2%) in BCPS group. Although the JR group exhibited longer operation times than the BCPS group, no significant differences in intraoperative complications were noted. CONCLUSION: Junior residents can safely perform UGSC for tCVC insertion. However, careful consideration of complications such as arterial or thoracic puncture is essential and case selection should be based on experience.

Impact of hepatocyte growth factor on the colonic morphology and gut microbiome in short bowel syndrome rat model.

PURPOSE: This study aimed to investigate the impact of hepatocyte growth factor (HGF) on colonic morphology and gut microbiota in a rat model of short bowel syndrome (SBS). METHODS: SD rats underwent jugular vein catheterization for total parenteral nutrition (TPN) and 90% small bowel resection [TPN + SBS (control group) or TPN + SBS + intravenous HGF (0.3 mg/kg/day, HGF group)]. Rats were harvested on day 7. Colonic morphology, gut microflora, tight junction, and Toll-like receptor-4 (TLR4) were evaluated. RESULTS: No significant differences were observed in the colonic morphological assessment. No significant differences were observed in the expression of tight junction-related genes in the proximal colon. However, the claudin-1 expression tended to increase and the claudin-3 expression tended to decrease in the distal colon of the HGF group. The Verrucomicrobiota in the gut microflora of the colon tended to increase in the HGF group. The abundance of most LPS-producing microbiota was lower in the HGF group than in the control group. The gene expression of TLR4 was significantly downregulated in the distal colon of the HGF group. CONCLUSION: HGF may enhance the mucus barrier through the tight junctions or gut microbiome in the distal colon.

Ovarian endometrioma: a report of a pediatric case diagnosed prior to menstruation.

BACKGROUND: Ovarian endometriomas (OEs) are rarely found in the pediatric population, especially before menstruation. We report a 6-year-old girl who was postoperatively diagnosed with OE before menstruation. CASE PRESENTATION: A 6-year-old girl presented to a local pediatrician with abdominal pain and vomiting. Abdominal ultrasonography revealed a multilocular cystic lesion to the left of the bladder. Magnetic resonance imaging (MRI) revealed similar findings, with the contents of the cyst showing a low signal on T1-weighted imaging and a high signal on T2-weighted imaging. The patient was referred to our institution for further examination. Enhanced computed tomography (CT) showed a multilocular cystic lesion sized 56 × 44 × 30 mm with partial calcification. The left ovarian vein was dilated, suggesting the origin of the tumor to be the left ovary. Extirpation of the lesion was performed under laparoscopic assistance. Pathological findings indicated an ovarian endometrioma. To our knowledge, this is the youngest report of an OE diagnosed in a patient prior to menstruation. CONCLUSIONS: OEs in children before menstruation are extremely rare; thus, the long-term prognosis is yet to be determined.

Comparison of Transumbilical Laparoscopy-Assisted Appendectomy with Conventional Three-Port Laparoscopic Appendectomy Performed by Pediatric Surgeons in Training for Appendicitis in Children.

Background: Transumbilical laparoscopy-assisted appendectomy (TULAA) is the technique of choice for all types of appendicitis. However, the technique is challenging for trainees to learn in comparison with performing conventional three-port laparoscopic appendectomy (CTPLA) in children. We aimed to compare the surgical outcomes of children with appendicitis treated by TULAA versus CTPLA performed by pediatric surgeons in training (PSITs). Materials and Methods: This retrospective study analyzed pediatric patients with acute appendicitis treated with CTPLA or TULAA between April 2016 and December 2022. Operative time (OT: minutes), pneumoperitoneum time (PT: minutes), blood loss (milliliter), length of hospital stay (days), and surgical site infection rate were compared between the two groups. Operative outcomes were also analyzed according to type of appendicitis such as uncomplicated and complicated cases. Results: Two hundred twenty-five laparoscopic appendectomies were performed by CTPLA (n = 94) or TULAA (n = 131). All cases were performed by PSITs and there was no open conversion cases. TULAA had a shorter OT (67.0 ± 28.4 versus 78.3 ± 21.7; P < .01) and PT (26.1 ± 17.4 versus 52.5 ± 22.1 min; P < .01). The surgical site infection rate was slightly higher in the TULAA group, but the difference was not statistically significant. In uncomplicated appendicitis (n = 164), significant differences between the CTPLA and TULAA groups were observed in OT (CTPLA versus TULAA: 70.7 ± 14.9 versus 59.1 ± 21.6, P < .01) and PT (CTPLA versus TULAA: 43.6 ± 13.1 versus 20.4 ± 13.6, P < .01). With regard to postoperative complications, only surgical site infection was significantly different between the CTPLA and TULAA groups (CTPLA: 0.0% versus TULAA: 8.2%, P < .05). In complicated cases (n = 61), there were significant differences between the groups in PT (CTPLA versus TULAA: 73.4 ± 24.9 versus 42.3 ± 17.2, P < .01) and length of hospital stay (CTPLA versus TULAA: 7.0 ± 1.3 versus 8.9 ± 4.7, P < .05). Conclusions: TULAA had a shorter OT and PT than CTPLA. TULAA for PSITs shows similar safety and feasibility to CTPLA for not only uncomplicated cases but also complicated cases.

Successful Laparoscopic Hepaticojejunostomy for Infant Congenital Biliary Dilatation with both Aberrant Right Hepatic Artery and Bile Duct from the Caudate Region.

A boy with congenital hydronephrosis underwent ultrasonography every month for follow-up. At 4 months of age, ultrasonography incidentally revealed congenital biliary dilatation (5-cm type Ia). We performed laparoscopic extrahepatic bile duct resection and hepaticojejunostomy. After dissecting the dilated common bile duct (CBD), we found that the arcading-like shaped right hepatic artery (RHA) coursed in front of the CBD. Additionally, a tiny duct was identified below the main hepatic duct. At first, we thought it was a lymphatic vessel and dissected it from the main hepatic duct. However, bile flow out was recognized after dissecting the tiny duct. Finally, we confirmed it as an aberrant bile duct from the caudate region. We anastomosed the bile duct from the caudate region and main hepatic duct in a double-barrel fashion and performed hepaticojejunostomy below the RHA. The postoperative course was uneventful. Ultrasonography showed no intrahepatic ductal dilatation including the caudate lobe.

Evaluation of skill acquisition characteristics depending on the size of a dry box.

BACKGROUND: Few studies have analyzed the effect of the size of the working space in training on the acquisition of endoscopic skills. In this study, adult- and infant-sized dry boxes (DBs) were used to verify how the size of the working space in training affects forceps manipulation and learning curve. MATERIAL AND METHODS: Seventy-two medical students were enrolled. The task was peg transfer. The training environment was divided into adult- and infant-sized DBs. Skill evaluations were also divided into adult- and infant-sized DBs (four groups in total). The forceps manipulation characteristics and task completion time were compared before and after training. RESULTS: Regarding skill evaluations using adult-sized DBs, there were no significant differences between the infant- and adult-sized DB-trained groups. Regarding skill evaluations using infant-sized DBs, there were no significant differences between the groups before training. After training, there was no significant difference in the total path length or average acceleration of the forceps between the groups. However, the infant-sized DB-trained group had a significantly faster average forceps velocity and faster task completion time than the adult-sized DB-trained group. CONCLUSION: Training with a small DB is more efficient in acquiring smoother and faster forceps manipulation in a small working space.

Endosurgery workshop to improve the confidence of novice pediatric surgeons in performing laparoscopic hepaticojejunostomy.

PURPOSE: This study assessed the efficacy of a high-impact, short-term workshop in honing the laparoscopic hepaticojejunostomy technical skills and self-confidence of novice pediatric surgeons, focusing on vertical needle driving and knot tying. METHODS: Lectures, hands-on sessions, pre- and post-workshop evaluations, and training using porcine models were conducted to refine basic and advanced skills. The "hepaticojejunostomy simulator" was used for comparative analysis of precision in pre- and post-workshop vertical needle driving and knot tying. Participants self-evaluated their skills and confidence on a 5-point scale. RESULTS: After the workshop, eight inexperienced pediatric surgeons demonstrated a significant improvement in hepaticojejunostomy suturing task completion rates and needle-driving precision at the jejunum and hepatic duct. However, the A-Lap Mini Endoscopic Surgery Skill Assessment System indicated no significant improvements in most assessed parameters, except for the full-layer closure score (p = 0.03). However, a significant increase in participants' confidence levels in performing laparoscopic hepaticojejunostomy was observed. CONCLUSION: The workshop augmented technical proficiency and confidence in young pediatric surgeons. The combination of lectures, practical exposure, and model training is an effective educational strategy in pediatric surgical instruction.

Safe and secure laparoscopy-assisted jejunostomy tube placement using a percutaneous loop needle device in an infant.

ABSTRACT: We, herein, report a surgical technique for laparoscopy-assisted jejunostomy tube placement in an infant using a loop needle device to fix the jejunum and abdominal wall. A 3-year-old boy with Down's syndrome underwent nutritional management by gastrostomy due to oral feeding difficulty after radical surgery for congenital duodenal stenosis and following bile duct stenosis. However, intractable gastrostomy site leakage emerged; hence, laparoscopy-assisted gastrostomy takedown and simultaneous laparoscopy-assisted jejunostomy tube placement were planned. After laparoscopy-assisted gastrostomy closure was performed, the jejunum was extracted through the umbilical trocar wound. A jejunostomy tube kit was inserted at the left side of the umbilicus. After tube insertion into the jejunum, the jejunostomy tube was wrapped with four interrupted sutures using the Witzel technique. Suture threads were extracted by percutaneous insertion of a loop needle device, and then, the jejunum was fixed to the abdominal wall. The post-operative course was uneventful. Percutaneous insertion of a loop needle device is useful for fixing a jejunostomy tube to the abdominal wall using the Witzel technique. Our technique is safe, secure and expected to reduce the risk of dislodgement of tube and peritonitis associated with jejunostomy tube placement.

Clinical outcome and neurological development of patients with biliary atresia associated with a bleeding tendency: a single institution experience.

PURPOSE: We compared the clinical features of patients with biliary atresia (BA) associated with a bleeding tendency (BT) at the time of the diagnosis with those of patients without a bleeding tendency (NBT). METHODS: The patients' background characteristics, age in days at the first visit, Kasai portoenterostomy (KPE), and postoperative course were retrospectively analyzed. RESULTS: Nine of the 93 BA patients (9.7%) showed a BT, including 7 with intracranial hemorrhaging (ICH), 1 with gastrointestinal bleeding, and 1 with a prothrombin time (PT) of 0%. The age at the first visit was 62 ± 12 days old for BT patients and 53 ± 27 days old for NBT patients (p = 0.4); the age at KPE was 77 ± 9 days old for BT patients and 65 ± 24 days old for NBT patients (p = 0.2); the time from the first visit to surgery was 13 ± 7 days for BT patients and 11 ± 10 days for NBT patients (p = 0.5); and the native liver survival rate was 56% for BT patients and 58% for NBT patients (p = 1), with no significant difference in any of the parameters. The neurological outcomes of survivors of ICH were favorable. CONCLUSIONS: Appropriate BT correction allowed early KPE even after ICH, resulting in native liver survival rates comparable to those of NBT patients without significant neurological complications.

Notable Clinical Differences Between Neonatal and Post-Neonatal Intestinal Malrotation: A Multicenter Review in Southern Japan.

BACKGROUND: Most cases of intestinal malrotation appear in neonates with bilious vomiting due to midgut volvulus, whereas in cases that develop beyond infancy, the initial symptoms vary. This study investigated the clinical features of these two populations and identified issues that should be considered in daily practice. METHODS: A retrospective chart review was conducted from January 1, 2010, to December 31, 2022. Data on patients with intestinal malrotation were collected in an anonymized fashion from five pediatric surgical hub facilities in the Southern Kyushu and Okinawa areas of Japan. RESULTS: Of the 80 subjects, 57 (71.3%) were neonates (Group N) and 23 (28.7%) were infants and schoolchildren (Group I). The frequencies of initial symptoms, such as abdominal distention (Group N: 19.3% vs. Group I: 13.0%), bilious vomiting (59.6% vs. 43.5%), and hematochezia (8.8% vs. 21.7%), were not skewed by the age of onset (p = 0.535, 0.087, and 0.141, respectively). Midgut volvulus was significantly more frequent in Group N (71.9% [41/57] vs. 34.8% [8/23]; p = 0.005), while the degree of torsion was greater in group I (median 360° [interquartile range: 180-360°] vs. 450° [360-540°]; p = 0.029). Although the bowel resection rate was equivalent (7.0% [4/57] vs. 4.3% [1/23]; p = 1.000), half of the patients in Group N presented with 180° torsion. The neonatal intestine has been highlighted as being more susceptible to ischemia than that in older children. CONCLUSIONS: The incidence of midgut volvulus is higher in neonates than in older children. Even relatively mild torsion can cause ischemic bowel changes during the neonatal period. LEVEL OF EVIDENCE: LEVEL III.

Potential mechanisms underlying the effect of hepatocyte growth factor on liver injury in short bowel syndrome model rats.

PURPOSE: The purpose of this study was to investigate the autophagy associated with apoptosis in hepatic damage in the short bowel syndrome rat model. METHODS: SD rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. Animals were divided into two groups: TPN plus SBS (Control group) or TPN plus SBS plus intravenous administration of HGF (HGF group). On day 7, the rats were harvested, and hepatocellular injury was evaluated. RESULTS: In an SBS rat model, hepatic steatosis and lobular inflammation were histologically suppressed in the HGF group (p < 0.01). The expression of tumor necrosis factor-α in the HGF group tend to be higher than that in the control group (p = 0.13). The gene expression of transforming Growth Factor-ß in the HGF group was suppressed compared to the control group (p < 0.01). HGF treatment may have an antiapoptotic effect via the intrinsic pathway by caspase 9. Protein expressions of Rubicon (p = 0.03) and p62 (p < 0.01) in the HGF group were found to have increased compared to those in the control group. CONCLUSION: The inhibitory effect of HGF on hepatic steatosis remains unclear, and further studies focusing on the mechanisms of fat accumulation are needed.

Change over time in the postoperative defecation function in female patients with anovestibular fistula at a single institution: focus on the comparison of anal transposition with anterior sagittal anorectoplasty.

PURPOSE: Anovestibular fistula (AVF) is the most common type of ARM in female patients. The present study investigated changes over time in the postoperative defecation function of female patients with AVF. METHODS: Patient data were collected from 1984 to 2021. Eighty-eight female patients with AVF were enrolled. Patients' characteristics and the long-term outcome of defecation function were reviewed and analyzed retrospectively. The bowel function was evaluated according to the Japan Society of ARM Study Group evacuation score (ES). RESULTS: Thirty-eight patients underwent anal transposition (AT), and 8 underwent anterior sagittal anorectoplasty (ASARP). The total evacuation score (ES) in AVF patients reached "excellent" at nine years old, regardless of the operative procedure. The constipation scores with AT showed better improvement than those with ASARP, but soiling scores in the ASARP group showed better improvement than those in the AT group. The postoperative complications did not affect the postoperative bowel function in AVF patients. CONCLUSION: Most patients with AVF eventually achieved a satisfactory total ES. Given the difference in defecation score transition depending on the operative procedure or postoperative complications, it may be important to perform long-term defecation management via surgical procedures.

How many cases do instructor class pediatric surgeons need to experience to be an independent operator in performing advanced endoscopic surgery? A nationwide survey to establish an ideal curriculum for pediatric endoscopic surgery in Japan.

PURPOSE: To ensure the safe spread of pediatric endoscopic surgery, it is essential to build a training curriculum, and a survey of the current situation in Japan is necessary. The present study assessed an efficient training curriculum by clarifying instructor class pediatric surgeons' experiences, including autonomy when performing advanced endoscopic surgeries. METHODS: An online nationwide questionnaire survey was conducted among pediatric surgeons who had Endoscopic Surgical Skill Qualification (ESSQ) and board-certified instructors who had skills comparable to ESSQ. We assessed participants' training experience, opinions concerning the ideal training curriculum, and the correlation between surgical experience and the level of autonomy. The Zwisch scale was used to assess autonomy. RESULTS: Fifty-two participants responded to the survey (response rate: 86.7%). Only 57.7% of the respondents felt that they had received sufficient endoscopic surgery training. Most respondents considered an educational curriculum for endoscopic surgery including off-the-job training essential during the training period. Autonomy had been acquired after experiencing two to three cases for most advanced endoscopic surgeries. CONCLUSION: This first nationwide survey in Japan showed that instructor class pediatric surgeons acquired autonomy after experiencing two to three for most advanced endoscopic surgeries. Our findings suggest that training, especially off-the-job training, has been insufficient.

Proctoring System of Pediatric Laparoscopic Surgery for Choledochal Cyst.

Background: Endoscopic surgery also has been becoming widespread in the field of pediatric surgery. However, most disease treated by pediatric surgery in a single institution are small number of cases. Besides, the variety of operative procedures that need to be performed in this field is quite wide. For these reasons, pediatric surgeons have limited opportunities to perform endoscopic surgery. Therefore, it is difficult to introduce advanced endoscopic surgery at a single local hospital. To educate pediatric surgeons in local hospitals, for widespread advanced pediatric endoscopic surgery safely, and to eliminate the need for patient centralization, we have introduced a proctoring system. We compared the surgical results of our institution, a center hospital, with other local institutions, to investigate the feasibility of our proctoring system. Methods: The experienced pediatric surgeon of our institution visits local hospitals to provide onsite coaching and supervises pediatric surgeons on the learning curve. All patients who underwent laparoscopic cyst excision and hepaticojejunostomy for choledochal cysts, one of the advanced pediatric endoscopic surgeries was retrospectively reviewed. Results: Thirty-four cases were evaluated (14 cases in our institution, 20 cases in 9 other institutions). The procedures of all 34 cases were performed by surgeons with 0-2 cases of experience in the procedure. There were no open conversion cases. There was no significant difference in the operative date. There was 1 case (6.7%) of postoperative complications during hospitalization at our institution and 3 cases (14.3%) at other institutions (P = .47). Two cases of late complications (13.3%) occurred at our institution, whereas 6 cases (28.6%) occurred at other institutions (P = .28). Conclusion: With the proctoring system, the performance and completion of advanced pediatric endoscopic surgery at local institutions was feasible. This has important implications given the ever-growing demand for pediatric endoscopic surgery and the increasing need for competent pediatric endoscopic surgeons.

Case report: Minimal tissue damage and low coagulation liver resection for hepatoblastoma using indocyanine green fluorescence and water-jet dissector.

Near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in pediatric surgery as it has in general surgery. In addition, a water-jet dissector (WJD) has been successfully introduced in adult hepatic surgery. Tissue structures are dissected selectively and gently by the WJD. However, there have been no reports of hepatic resection for pediatric patients using a WJD. We applied NIR fluorescence imaging with ICG to visualize the resection line of the liver and used a WJD for liver parenchyma dissection in pediatric hepatoblastoma. The patient was a 3-year-old girl with a large liver tumor. Enhanced computed tomography revealed a liver tumor (maximum diameter: 120 mm) in the right lobe and three small lung metastases. The liver tumor was diagnosed as hepatoblastoma (PRETEXT 2) based on an open biopsy. We performed right hepatectomy after neoadjuvant chemotherapy. The right lobe was mobilized from the diaphragm, and then intraoperative ultrasound was performed to detect the localization of the tumor and its proximity to the vascular structures. We detected the right hepatic artery (RHA), right portal vein (RPV), and right hepatic vein (RHV). The middle hepatic vein was not involved. After ligation of the RHA and RPV to selectively control the right lobe inflow, ICG was administered intravenously and observed by an NIR endoscope. The resection line was clearly visualized by overlaying images in comparison to conventional demarcation line detection. Then, we used a WJD to dissect the parenchyma. Small vessels were divided from parenchymal tissue and were clearly visible. We resected them after clamping with metal clips. Finally, the RHV was transected by a linear stapler, and right hepatectomy was completed with 25 ml of blood loss. There was no postoperative hemorrhage. We performed hepaticojejunostomy because of stricture of the common bile duct on postoperative day 302. The patient was discharged after adjuvant chemotherapy. NIR imaging clearly showed the resection line. The WJD automatically separated, and thus made visible, the more resistant duct and vessel structures from the parenchyma. The combined use of NIR imaging and WJD was useful for pediatric hepatectomy.

The clinical features of patients who underwent bladder augmentation of cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

PURPOSE: Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. RESULTS: BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6-9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0-20.5] years and the serum creatinine level was 0.44 [0.36-0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. CONCLUSION: The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.

Posterior sagittal anorecto-urethro-vagino-plasty in the late period was associated with the long-term bowel function in patients with persistent cloaca: results of a nationwide survey in Japan.

PURPOSE: After conducting a nationwide survey of persistent cloaca (PC), we assessed whether or not the timing of definitive anorectoplasty affects the long-term bowel function of patients with PC. METHODS: Patient information was obtained via questionnaire, and a total of 169 PC patients who underwent posterior sagittal anorectourethrovaginoplasty (PSARUVP) were enrolled in this study. Patients were classified into 2 groups based on their operative period, which was analyzed by the area under the receiver operating characteristic curve: the early group (EG) underwent anorectoplasty at ≤ 18 months old (n = 106), and the late group (LG) underwent anorectoplasty at > 18 months old (n = 63). The bowel function was evaluated using the evacuation score of the Japan Society of Anorectal Malformation Study Group. We also examined the postoperative results of vaginoplasty. RESULTS: The total evacuation score was significantly higher in the EG than in the LG (5.2 ± 1.7 vs. 4.2 ± 1.8, p = 0.003). The frequency of bowel movement and the constipation scores were significantly higher in the EG than in the LG (1.4 ± 0.6 vs. 1.2 ± 0.7, p < 0.05, 2.4 ± 1.0 vs. 2.1 ± 1.0, p < 0.05, respectively). Postoperative vaginal stenosis was observed in 18 cases (10.7%), of which 16 could be reconstructed transperineally. CONCLUSION: PSARUVP should be performed in early infancy and facilitate vaginal reconstruction.