Ductal stenting with bilateral pulmonary artery banding as a life-saving management for hypoplastic left heart syndrome with congenital esophageal atresia: A case series.

We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions. Learning objective: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.

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.

Characteristics of intussusception in the period of arbitrary Rotavirus vaccination.

BACKGROUND: In November 2011, rotavirus (RV) vaccine was launched in Japan as a voluntary vaccination to prevent RV-associated gastroenterocolitis. We examined the characteristics of intussusception following RV vaccination in our two centers. METHODS: We investigated intussusception patients <16 years old from January 2006 to September 2020. Patients were categorized according to the period (before [Group A] or after the introduction of arbitrary RV vaccination [Group B]). The patient characteristics and treatment of intussusception were retrospectively investigated. RESULTS: During the study period, 560 patients (group A, n = 233; group B, n = 327) were identified. The distribution of patients who were 0-6 months old was not significantly different between the groups (group A, n = 12, 5.2%; group B, n = 18, 5.5%). Among these 18 patients in Group B, 7 were vaccinated against RV, and 10 were not. One patient was excluded due to incomplete data. On comparing patients with and without RV vaccination, the mean age at the onset of intussusception was 3.3 ± 0.4 versus 4.0 ± 0.3 months (P = 0.19), the mean interval from the onset to treatment was 7.5 ± 2.4 versus 16.0 ± 2.2 h (P = 0.03), the time of the contrast enema for treatment was 9.1 ± 3.3 versus 7.7 ± 2.8 min (P = 0.76), and the final pressure of the contrast enema was 92.5 ± 4.4 versus 92.2 ± 4.4 cmH2 O (P = 0.97). CONCLUSIONS: Arbitrary RV vaccination did not influence the age distribution of intussusception, and the interval from the onset to treatment was significantly shorter in the patients with RV vaccination than in those without it. Recognizing the presence of intussusception following RV vaccination enables accurate treatment.

High insertion of the right diaphragm complicated with congenital diaphragmatic hernia: A case report of rare thoracoscopic findings.

We encountered a case of high insertion of the right diaphragm complicated with congenital diaphragmatic hernia that was diagnosed based on thoracoscopic findings. A full-term male baby was suspected of having right congenital diaphragmatic hernia or diaphragmatic eventration on postnatal imaging. He only had episodes of mild but prolonged symptoms following upper respiratory tract infection and his course was otherwise uneventful during outpatient monitoring. At 1 year old, the elevated liver volume remained large, which might eventually interfere with his lung growth, so thoracoscopic exploration was planned. Thoracoscopy revealed liver prolapse from a diaphragmatic defect. In addition, the anterior to lateral inserted part of the diaphragm was high, with the anterior part reaching the fourth rib. We repaired only the diaphragmatic defect without repositioning the diaphragm, and the postoperative course was uneventful. High insertion of the diaphragm should be considered as a differential diagnosis of congenital diaphragmatic eventration.

Ambidextrous needle driving and knot tying helps perform secure laparoscopic hepaticojejunostomy of choledochal cyst (with video).

Ieiri and colleagues report an ingenious technique for performing ambidextrous suturing in laparoscopic hepaticojejunostomy for cases with small hepatic ducts. Surgeons who devote professional attention to minimally invasive surgery should take care to practice extensively with their non-dominant as well as dominant hand to improve the quality of laparoscopic surgery.

Rex Shunt for Portal Vein Thrombosis After Pediatric Living Donor Liver Transplantation.

BACKGROUND Portal vein thrombosis (PVT) after pediatric liver transplantation (LT) is a common but grave complication which could eventually result in life-threatening portal hypertension. A "Rex" shunt between the superior mesenteric vein and the Rex recess of the liver has been reported to be a treatment option for extrahepatic portal vein obstruction; however, its application to living donor liver transplantation (LDLT) is limited due to the availability of appropriate vein grafts. In this study, we retrospectively evaluated the effectiveness of Rex shunt as an option for the treatment of PVT after pediatric LDLT. CASE REPORT Three children underwent the Rex shunt for early (n=2) and late (n=1) PVT after LDLT using the greater saphenous vein (n=2) and the external iliac vein (n=1) from the parents who previously donated their livers. Two of the 3 children are free from symptoms with patent shunt grafts at 14 years after the procedures. One child died at 30 days after LDLT due to repeated episodes of PVT, which finally led to hepatic infarction. CONCLUSIONS The Rex shunt is feasible to treat PVT after LDLT. However, additional surgical insults to the living donor need further discussion.

Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex.

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended. Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage. Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex.

Navigation surgery using indocyanine green fluorescent imaging for hepatoblastoma patients.

BACKGROUND: Technology for detecting liver tumors and identifying the bile ducts using indocyanine green (ICG) has recently been developed. However, the usefulness and limitations of ICG navigation surgery for hepatoblastoma (HB) have not been fully clarified. We herein report our experiences with surgical navigation using ICG for in HB patients. METHODS: In 5 HB patients, 10 ICG navigation surgeries were performed using a 10-mm infrared fluorescence imaging scope after the injection of 0.5 mg/kg ICG intravenously. The surgical and clinical features were collected retrospectively. RESULTS: Navigation surgery using ICG was performed for primary liver tumors in 4 cases, and the timing of ICG injection was 90.5 ± 33.7 h before the operation. All tumors exhibited intense fluorescence from the liver surface. ICG navigation for the primary liver tumor was useful for detecting the residual tumor at the stump and invasion to the diaphragm during surgery. Six lung surgeries using ICG navigation were performed. The timing of ICG injection was 21.8 ± 3.4 h before the operation. The size of the metastatic tumor was 7.4 ± 4.1 mm (1.2-15 mm). Of 11 metastatic tumors detected by computed tomography (CT), 10-including the smallest tumor (1.2 mm)-were able to be detected by ICG from the lung surface. The depth of the 10 ICG-positive tumors from the lung surface was 0.9 ± 1.9 mm (0-6 mm), and the depth of the single ICG-negative tumor was 12 mm. One lesion not detected by CT showed ICG false positivity. CONCLUSION: Navigation surgery using ICG for patients with HB was useful for identifying tumors and confirming complete resection. However, in ICG navigation surgery, we must be aware of the limitations with regard to the tumor size and the depth from the surface.

Efficacy of Prophylactic Negative Pressure Wound Therapy After Pediatric Liver Transplant.

OBJECTIVES: Wound dehiscence is a common surgical complication, especially among pediatric liver transplant recipients in our center. In 2013, we introduced negative pressure wound therapy as a preventive treatment. We herein report the clinical outcomes of this intervention. MATERIALS AND METHODS: We conducted a retrospective review of the 26 pediatric liver transplant recipients in our center since 2011. We excluded 1 girl whose wound could not be closed due to bowel edema. The first 13 of the 25 remaining patients were treated with conventional wound management (conventional group). The latter 12 were treated with prophylactic negative pressure wound therapy (prophylactic group). Incidences of surgical complications and patient characteristics were compared between groups. RESULTS: Wound dehiscence occurred in 7 of the 13 patients in the conventional group and 3 of the 12 patients in the prophylactic group. When restricted to dehiscence that required surgical debridement, there were 6 cases in the conventional group and no cases in the prophylactic group. Although background data showed that liver insufficiency in the prophylactic group was more severe, this group had a lower incidence of wound dehiscence (P = .015). CONCLUSIONS: Prophylactic negative pressure wound therapy is thought to be effective for preventing wound dehiscence among pediatric liver transplant recipients.

Comparison of biliary atresia with and without intracranial hemorrhage.

BACKGROUND/PURPOSE: Intracranial hemorrhage (ICH) is a severe complication of biliary atresia (BA). We aimed to compare the clinical data of BA patients with and without ICH. METHODS: Sixty-three BA patients who underwent Kasai portoenterostomy were included in this study. We retrospectively reviewed their clinical records, and compared the ICH and non-ICH groups. RESULTS: ICH occurred in seven patients (11.1%). The patients with ICH were significantly older at the time of Kasai portoenterostomy (median age: 90.0 vs 65.5 days). The hepatobiliary enzyme levels of the patients with ICH were significantly lower in comparison to the patients without ICH (T-Bil 6.7 vs 9.8 mg/dl; AST 95 vs 194 U/L; ALT 44 vs 114 U/L). On the other hand, the coagulation test values of the patients with ICH were significantly higher in comparison to the patients without ICH (PT 50.0 vs 12.4 s; APTT 200.0 vs 36.9 s). Although the survival rates did not differ to a statistically significant extent, persistent neurological sequelae occurred in two patients in the ICH group. CONCLUSIONS: The hepatobiliary enzyme levels of the patients with ICH were significantly lower than those without ICH. However, coagulopathy was found to be significantly more progressive in patients with ICH. LEVELS OF EVIDENCE: Level III.

Liver graft-to-spleen volume ratio as a useful predictive factor of the early graft function in children and young adults transplanted for biliary atresia: a retrospective study.

A graft volume/standard liver volume ratio (GV/SLV) > 35% or graft/recipient weight ratio (GRWR) > 0.8% has been considered as a standard criteria of graft selection. Even if the graft size meets these selection criteria, small-for-size syndrome can still occur depending on the portal venous flow (PVF). The aim of this study was to identify other factors contributing to portal hyperperfusion and the post-transplant course, focusing on the graft volume-to-spleen volume ratio (GV/SV). Thirty-seven BA patients who underwent living donor liver transplantation were reviewed retrospectively. First, we evaluated the preoperative factors contributing to portal hyperperfusion. Second, we evaluated the factors contributing to post-transplant complications, such as thrombocytopenia, hyperbilirubinemia, and coagulopathy. The GV/SLV was >35% in all cases; however, portal hyperperfusion (≥250 ml/min/100 g graft) was found in 12 recipients (35.3%). Furthermore, although the GRWR was >0.8% in over 90% of cases, portal hyperperfusion was found in 10 recipients (32.3%). In contrast, the GV/SV showed a significant correlation with the PVF after reperfusion. If the GV/SV was <0.88, about 80% of recipients developed portal hyperperfusion. Furthermore, the GV/SV also showed a significant correlation with post-transplant persistent thrombocytopenia and hyperbilirubinemia. The GV/SV < 0.88 predicts portal hyperperfusion, post-transplant persistent thrombocytopenia, and hyperbilirubinemia.

The incidence of chylous ascites after liver transplantation and the proposal of a diagnostic and management protocol.

BACKGROUND: No protocol has been established for the diagnosis and management of chylous ascites after liver transplantation (LT). In this study, we retrospectively reviewed our cases of posttransplant chylous ascites (PTCA) and aimed to propose a diagnostic and management protocol. PATIENTS AND METHODS: We retrospectively reviewed the clinical records of 96 LT recipients who underwent LT at our department. The incidence of PTCA and the associated risk factors were analyzed and our protocol for chylous ascites was evaluated. RESULTS: PTCA occurred in 6 (6.3%) patients (mean age: 10.7±11.0years) at a mean of 10.8±3.6days after LT. The primary disease in all of PTCA cases was biliary atresia (BA). The periportal lymphadnopathy was an independent risk factor for PTCA. In all cases PTCA successfully resolved according to our protocol. Octreotide was administered in 4 of our 6 PTCA cases. The mean postoperative hospital stay was 40.2±8.4days, which was similar to that of cases without PTCA. CONCLUSIONS: The incidence of PTCA in LT patients, especially in those with BA, is relatively high. Our diagnostic criteria and our management protocol were helpful for patients with refractory ascites after LT. TYPE OF STUDY: Diagnostic test: Level II. Treatment study: Level III.

In vivo and ex vivo methods of growing a liver bud through tissue connection.

Cell-based therapy has been proposed as an alternative to orthotopic liver transplantation. The novel transplantation of an in vitro-generated liver bud might have therapeutic potential. In vivo and ex vivo methods for growing a liver bud are essential for paving the way for the clinical translation of liver bud transplantation. We herein report a novel transplantation method for liver buds that are grown in vivo involving orthotopic transplantation on the transected parenchyma of the liver, which showed long engraftment and marked growth in comparison to heterotopic transplantation. Furthermore, this study demonstrates a method for rapidly fabricating scalable liver-like tissue by fusing hundreds of liver bud-like spheroids using a 3D bioprinter. Its system to fix the shape of the 3D tissue with the needle-array system enabled the fabrication of elaborate geometry and the immediate execution of culture circulation after 3D printing-thereby avoiding an ischemic environment ex vivo. The ex vivo-fabricated human liver-like tissue exhibited self-tissue organization ex vivo and engraftment on the liver of nude rats. These achievements conclusively show both in vivo and ex vivo methods for growing in vitro-generated liver buds. These methods provide a new approach for in vitro-generated liver organoids transplantation.

Umbilical hernia: Influence of adhesive strapping on outcome.

BACKGROUND: Adhesive strapping for umbilical hernia has been re-evaluated as a promising treatment. We evaluated the influence of adhesive strapping on the outcome of umbilical hernia. METHODS: We retrospectively evaluated patients with umbilical hernia referred to the present institution from April 2011 to December 2015. Patients who were treated with adhesive strapping were compared with an observation alone group. The adhesive strapping group was also subdivided into two groups: the cure group and the treatment failure group. RESULTS: A total of 212 patients with umbilical hernia were referred to the present institution. Eighty-nine patients were treated with adhesive strapping, while 27 had observation only. The cure rate in the adhesive strapping group was significantly higher than that in the observation group. The duration of treatment of the adhesive strapping group was significantly shorter than that of the observation group. In the adhesive strapping group, the patients in the cure group were treated significantly earlier than those in the treatment failure group (P < 0.001). Furthermore, even in cases of umbilical hernia non-closure, surgical repair was easier after adhesive strapping. CONCLUSION: Adhesive strapping represents a promising treatment for umbilical hernia. To achieve the best results, adhesive strapping should be initiated as early as possible.