Monolayer Modification of Spherical Amorphous Silica by Clay Nanosheets.

Clay-silica nanocomposite materials (CSiN) were prepared by an electrostatic interaction between negatively charged clay nanosheets and positively charged spherical silica, which was modified with an alkyl ammonium group by silane coupling. By optimization of the preparation conditions, 84% coverage of the silica surface by the clay nanosheets was achieved. Adsorption experiments using cationic porphyrin dyes on the CSiN revealed that the clay nanosheet covers the spherical silica as a single layer and does not detach from the silica surface under aqueous conditions. In addition, it turned out that the cationic porphyrin dye did not penetrate the space between the silica surface and the clay nanosheet. Porphyrin molecules were adsorbed only at the outer surface of the clay nanosheet without molecular aggregation even under the high-density adsorption conditions. By combining spherical silica and clay nanosheets, it is possible to prepare novel hybrid materials where the surface can act as a unique adsorption field for dyes.

A sutureless overlapped anastomosis technique using linear staplers with reinforced bioabsorbable material in robotic right colectomy with intracorporeal anastomosis.

AIM: Creation of an overlapped anastomosis using handsewn sutures for common enterotomy is very popular in robotic right colectomy (RRC) with intracorpareal anastomosis (IA). The aim of this study is to present a simple method for constructing a sutureless overlapped anastomosis using a 60 mm linear stapler with a reinforced bioabsorbable material in RRC with IA. METHOD: The distal ileum and proximal colon were put in overlapping positions. Enterotomies were created 2 cm proximal to the ileal stump and 8 cm distal to the colonic stump on the antimesenteric side. Subsequently, a 60 mm linear stapler with a reinforced bioabsorbable material was inserted into each lumen and fired. Finally, the bowel was elevated while holding the bioabsorbable material, and the common enterotomy was grasped with the robotic instrument in the middle and closed using a linear stapler with a reinforced bioabsorbable material. RESULTS: This technique was applied to 10 patients with tumours of the caecum, ascending colon, or transverse colon. The median operating time, anastomosis construction time, blood loss, and postoperative stay were 281 min (range 228-459 min), 12 min (range 11-17 min), 10 mL (range 0-110 mL), and 10 days (range 8-15 days), respectively. No adverse intraoperative events were observed. Postoperatively, one patient developed chylous ascites, but there were no other complications. CONCLUSION: The simple technique for constructing a sutureless overlapped anastomosis using a 60 mm linear stapler with a reinforced bioabsorbable material in robotic right colectomy with intracorporeal anastomosis appears to be safe and feasible.

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.

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.

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.

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.

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.

Predictive factors of bowel resection for midgut volvulus based on an analysis of bi-center experiences in southern Japan.

PURPOSE: Midgut volvulus is an urgent disease often occurring in neonates. This study clarified the clinical features of midgut volvulus and evaluated predictors to avoid bowel resection. METHODS: This bi-center retrospective study enrolled 48 patients who underwent surgery for intestinal malrotation between 2010 and 2022. Patients' background characteristics and preoperative imaging findings were reviewed. RESULTS: Midgut volvulus was recognized in 32 patients (66.7%), and 6 (12.5%) underwent bowel resection. Based on a receiver operating curve analysis of bowel resection, the cut-off value of the body weight at birth and at operation were 1984 g [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.52-0.99] and 2418 g (AUC 0.70, 95% CI 0.46-0.94), respectively. The cut-off value of intestinal torsion was 540° (AUC 0.76, 95% CI 0.57-0.95), and that of the time from the onset to the diagnosis was 12 h (AUC 0.85, 95% CI 0.72-0.98). For midgut volvulus with an intestinal torsion > 540°, the most sensitive preoperative imaging test was ultrasonography (75%) Patients with bloody stool tended to undergo bowel resection. CONCLUSIONS: Patients with a low body weight and bloody stool should be confirmed to have whirlpool sign by ultrasonography and scheduled for surgery as soon as possible.

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 complete tumor resection for pulmonary mucoepidermoid carcinoma by thoracoscopic right upper lobectomy with intraoperative bronchoscopy: A pediatric case report.

INTRODUCTION AND IMPORTANCE: Mucoepidermoid carcinoma presents as an exophytic endobronchial mass that induces obstructive symptoms often followed by distal collapse atelectasis of the lung parenchyma. CASE PRESENTATION: A six-year-old girl had recurrent bacterial pneumonia and atelectasis of the right upper lobe. Computed tomography revealed a 30-mm mass in the anterior segment of the right upper lobe with an obstructed trachea and peripheral atelectasis. A minor salivary gland tumor was suspected, so thoracoscopic right upper lobectomy (RUL) was performed. Intraoperative bronchoscopy showed no protrusion of the tumor into the tracheal lumen. We confirmed that there was no injury to the middle lobe branch and no residual tumor via bronchoscopy before transection of the trachel bronchus of the right upper lobe. The histological type was low-grade mucoepidermoid carcinoma. The postoperative course was uneventful, and no recurrence was evident after one year. CLINICAL DISCUSSION: Primary pulmonary cancers in children are extremely rare. Mucoepidermoid carcinoma is the most common disease in pediatric primary lung tumors but remains relatively rare. Mucoepidermoid carcinoma of the tracheobronchial tree sometimes requires sleeve resection. Intraoperative bronchoscopy helped determine the exact position of the tumor. The value of intraoperative bronchoscopy for sparing the lung parenchyma and preserving as much of the respiratory function as possible. Intraoperative bronchoscopy should be actively performed in cases of pediatric lobectomy, especially those involving tracheobronchial tumors. CONCLUSION: Intraoperative bronchoscopy allowed for complete RUL without residual tumor or injury of the middle lobe bronchus.

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.

Abdominal wall defect repair with component separation technique for giant omphalocele with previous relaxing incisions on the abdominal skin.

BACKGROUND: The repair of large abdominal wall defects that cannot be closed primarily is quite challenging. The component separation technique (CST) is a surgical approach using autologous tissue to close large abdominal wall defects. The CST requires extensive dissection between the abdominal skin and the anterior sheath of the rectus abdominis muscle. Subsequently, incisions are made at both sides of the external oblique aponeurosis, releasing the external oblique muscle from the internal oblique muscle, and then the right and left rectus abdominis muscles are brought together in the midline for defect closure. However, impairment of blood flow in the abdominal wall skin and necrotic changes are recognized as potential complications. CASE PRESENTATION: The CST was performed in a 4-year-old boy with a large ventral hernia who had undergone skin closure with abdominal wall relaxing incisions for the primary treatment of giant omphalocele in the neonatal period. Given his history of incisions on the abdominal wall, he was speculated to be at high risk for postoperative skin ischemia. Dissection was therefore kept to a minimum to preserve the blood supply from the superior and inferior epigastric arteries and perforating branches of those arteries through the rectus abdominis muscle. In addition, care was taken to adjust the muscle relaxant dosage while monitoring the intravesical pressure, ensuring that it did not exceed 20 mmHg to avoid impaired circulation in the abdominal wall caused by abdominal compartment syndrome. He was discharged 23 days after the surgery without any complications, and neither recurrence of the ventral hernia nor bowel obstruction was observed in 4 years. CONCLUSIONS: A giant omphalocele with primary skin closure was treated by applying the CST. The procedure can be performed safely while preserving the blood flow to the abdominal wall, even in patients with a history of relaxing incisions on the abdominal skin. The CST is expected to be effective for repairing the large abdominal wall defects seen in giant omphalocele when primary closure is not possible.

Cases of pediatric intra-abdominal solid organ injury induced by blunt trauma experienced over a 15-year period at two centers in Japan.

Objective: The present study attempts to clarify the clinical features of pediatric intra-abdominal solid organ injury at two institutions. Methods: The injured organ, patient age, sex, injury grade, imaging findings, intervention, length of hospital stay, and complications were retrospectively reviewed using medical records at two centers from 2007 to 2021. Results: There were 25 cases of liver injury, 9 of splenic injury, 8 of pancreatic injury, and 5 of renal injury. The mean age of all patients was 8.6±3.8 years old, with no difference between organ injury types. Radiological intervention was performed in four cases of liver injury (16.0%) and one case of splenic injury (11.1%), and surgery was performed in two cases of liver injury (8.0%) and three cases of pancreatic injury (37.5%). All other cases were treated conservatively. Complications included adhesive ileus in one case of liver injury (4.0%), splenic atrophy in one case of splenic injury (11.1%), pseudocysts in three cases of pancreatic injury (37.5%), atrophy of the pancreatic parenchyma in one case of pancreatic injury (12.5%), and urinoma in one case of renal injury (20.0%). No mortalities were observed. Conclusion: Pediatric patients with blunt trauma had favorable outcomes at two pediatric trauma centers covering a broad medical area, including remote islands.

An analysis of the correlation between the efficacy of training using a high-fidelity disease-specific simulator and the clinical outcomes of laparoscopic surgery for congenital biliary dilatation in pediatric patients.

PURPOSE: The present study clarified the efficacy of repeating laparoscopic surgery training using a disease-specific simulator and investigated the clinical outcomes of laparoscopic surgery for congenital biliary dilatation (CBD) in pediatric patients after training. METHODS: A high-fidelity laparoscopic hepaticojejunostomy simulator was used. Four pediatric surgeons performed practice laparoscopic hepaticojejunostomy three times using the simulator. The details of forceps manipulation during the task were analyzed. The clinical outcomes of 13 CBD cases treated with laparoscopic surgery in our institution were also evaluated based on medical records. RESULTS: The time required to complete the task became significantly shorter each successive time (1st: 1062.18 ± 346.79 s vs. 3rd: 717.44 ± 260.80 s, p = 0.039). There were no significant differences in the total path length of the right forceps (1st: 55.56 ± 23.21 m vs. 3rd: 28.25 ± 17.01 m, p = 0.17), total path length of the left forceps (1st: 47.79 ± 20.79 m vs. 3rd: 31.83 ± 17.62 m, p = 0.17), average velocity of the right forceps (1st: 58.78 ± 21.29 mm/s vs.44.98 ± 10.25 mm/s, p = 0.47), or the average velocity of the left forceps (1st: 50.39 ± 19.25 mm/s vs. 52.26 ± 19.59 mm/s, p = 0.78). Regarding the clinical outcome, all CBD patients underwent laparoscopic surgery performed by practiced pediatric surgeons who had no experience. The operative time was 545.53 ± 91.01 min, and the blood loss was 24.2 ± 25.8 ml. There were no cases of open conversion, intraoperative adverse events, or anastomotic leakage. CONCLUSION: Disease-specific simulator training significantly decreased the task performance time by improving the forceps manipulation economy. In addition, simulator training may improve the operative safety and quality of laparoscopic hepaticojejunostomy in pediatric CBD patients.

Thoracoscopic repair of neonatal left diaphragmatic hernia with sac combined with both extralobar pulmonary sequestration and congenital pulmonary airway malformation.

Intradiaphragmatic extralobar pulmonary sequestration (IDEPS) is considered to be very rare among extralobar pulmonary sequestration (EPS), and IDEPS combined with congenital diaphragmatic hernia (CDH) with sac is extremely rare. The patient was a neonatal girl. Left-sided CDH with a hernia sac was diagnosed based on computed tomography (CT). Thoracoscopic repair was planned for the 5th day after birth. Left CDH with sac was recognized on the posterolateral side and isolated EPS was recognized on the sac. The hernia sac, including the EPS, was resected, and the diaphragm defect was closed. The resected specimen consisted of fibrous connective tissue and pulmonary sequestration (PS) connected with the sac. It was diagnosed as the hybrid form, with features of both type 2 congenital pulmonary airway malformation (CPAM) and EPS. The postoperative course was uneventful. At 1 year and 9 mo after the operation, no recurrence of herniation or laterality of the diaphragm was recognized.

Feasibility of Laparoscopic Fundoplication Without Removing the Preceding Gastrostomy in Severely Neurologically Impaired Patients: A Multicenter Evaluation of the Traction Technique.

Purpose: Severely neurologically impaired patients sometimes require anti-reflux surgery with preceding gastrostomy. We apply a traction technique for laparoscopic fundoplication (LF) without gastrostomy takedown (GTD) in such cases. We conducted a multicenter review to assess the feasibility of our approach. Materials and Methods: In brief, the traction technique involves left-lateral-traction of the stomach body, right-lateral-traction of the round ligament of the liver, and elevation of the left liver lobe to create a sufficient field for manipulating the forceps. Patients who underwent LF with Nissen's procedures in 2010-2022 were retrospectively reviewed. Data were analyzed by a one-way analysis of variance. Results: The operative approaches included the traction technique (n = 16; Group 1), GTD and reconstruction (n = 5; Group 2), and LF followed by gastrostomy (n = 92; Group 3). In comparison with Group 1, significant differences were only found in pneumoperitoneum time (Group 1 versus Group 2 versus Group 3: 174.4 minutes versus 250.4 minutes versus 179.5 minutes; P = .0179). Operating time (222.7 minutes versus 303.0 minutes versus 239.7 minutes; P = .0743), duration to full-strength enteral nutrition (10.4 days versus 17.2 days versus 11.0 days; P = .0806), and length of hospital stay (17.2 days versus 31.0 days versus 18.5 days; P = .3247) were equivalent. No re-fundoplication was required in Group 1. Conclusion: The traction technique secures the operative quality and outcome of LF without GTD.

Late Occurrence of the Tubo-Ovarian Abscess after Appendectomy for Perforated Appendicitis in a Virginal Adolescent Girl.

BACKGROUND: Tubo-ovarian abscess (TOA) is generally seen in sexually active women. It is rarely reported in virginal adolescent girls. CASE: A 12-year-old virginal girl was referred to us for repeated fever and right lower abdominal pain. She had undergone an appendectomy for a perforated appendix with abscess 5 years previously. Laparoscopic surgery revealed pelvic adhesions associated with TOA in the right pelvis. At 2 months after laparoscopic drainage, she underwent resection of the affected tube with wedge resection of the ipsilateral ovary due to the recurrence of TOA. SUMMARY AND CONCLUSION: Late occurrence of TOA should be considered in the differential diagnosis of repeated abdominal pain and fever in virginal adolescent girls with a history of appendectomy for complicated appendicitis, even if the history is remote.

Case report: Severe hepatic fibrosis induced by chronic cholestasis of congenital biliary dilation treated by laparoscopic surgery after immunonutrition support- An infantile case.

Introduction: In some patients with congenital biliary dilation (CBD), biliary cirrhosis has been reported to rapidly progress from the neonatal period to the infantile period. We herein report an infantile case of CBD that showed severe biliary cirrhosis at the diagnosis, which was treated by laparoscopic surgery. Case presentation: A 16-month-old girl underwent conservative therapy for liver dysfunction and cholangitis on a remote island of our prefecture. She was transferred to our hospital after the detection of a huge dilated common bile duct on imaging at the previous hospital. Contrast-enhanced computed tomography showed a dilated common bile duct (maximum diameter: 5 cm), thus suggesting CBD. However, her laboratory data on admission showed a poor nutritional status and severe liver dysfunction (Alb, 2.5 mg/dl; AST, 79 IU/L; ALT, 43 IU/L; γ-GTP, 491 mg/dl; D-bil, 0.3 mg/dl; CHE, 90 IU/L; NH3, 123 µg/dl). We initially performed laparoscopic exploration and bile drainage via the gallbladder, noting severe hepatic fibrosis resembling end-stage liver cirrhosis. After placing a drainage tube in the gallbladder, cholangiography was performed. Cholangiography showed Todani type IVa CBD with pancreaticobiliary maljunction. Contrast agent flowing into the duodenum could not be confirmed. The patient received liver-supporting therapy and nutritional support for 7 weeks before definitive surgery. Following the improvement of the hepatic synthetic capacity (Alb, 4.0 mg/dl; AST, 82 IU/L; ALT, 78 IU/L; γ-GTP, 157 mg/dl; D-bil, 0.2 mg/dl; CHE, 232 IU/L; NH3, 75 µg/dl), we performed extrahepatic bile duct excision and hepaticojejunostomy laparoscopically. Laparoscopic surgery was successfully performed along with liver biopsy. Histopathologically, the liver specimen showed chronic hepatitis and fibrosis (F3A2). Biliary scintigraphy showed good bile excretion at postoperative day 15. The postoperative course uneventful, and the patient was discharged on the 23rd day after surgery. A needle liver biopsy six months later showed mild improvement of chronic hepatitis and fibrosis (F2-3A1). The patient was regularly followed at the outpatient clinic. Conclusions: Severe liver fibrosis was suspected to be continuous cholestasis of CBD after birth. CBD with severe liver fibrosis may avoid liver transplantation by two-stage surgery with hepatoprotection therapy and immunonutritional support.