Analysis of gap length as a predictor of surgical outcomes in esophageal atresia with distal fistula: a single center experience.

PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.

Factors predicting surgical difficulties in congenital biliary dilatation in pediatric patients.

BACKGROUND: The effects of disease classification and the patient's preoperative condition on the difficulty of performing a laparotomy for pediatric congenital biliary dilatation (CBD) have not been fully elucidated. METHODS: The present study retrospectively analyzed 46 pediatric CBD laparotomies performed at the study center between March 2010 and December 2021 and predictors of operative time. The patients were separated into a short operative time group (SOT) (≤ 360 min, n = 27) and a long operative time group (LOT) (> 360 min, n = 19). RESULTS: The preoperative AST and ALT values were higher, and the bile duct anastomosis diameter was larger, in the LOT. Correlation analysis demonstrated that the maximum cyst diameter, preoperative neutrophil-to-lymphocyte ratio, AST, ALT, AMY, and bile duct anastomosis diameter correlated positively with operative time. Multivariate analysis identified the maximal cyst diameter, preoperative AST, and bile duct anastomosis diameter as significant factors affecting surgical time. Postoperatively, intrapancreatic stones and paralytic ileus were observed in one patient each in the SOT, and mild bile leakage was observed in one patient in the LOT. CONCLUSIONS: The maximum cyst diameter, preoperative AST, and bile duct anastomosis diameter have the potential to predict the difficulty of performing a pediatric CBD laparotomy.

Tracheobronchial rupture in children due to chest compression.

A tracheobronchial rupture can be lethal. Its etiology in children varies and includes blunt trauma and iatrogenic injury. Most of the latter are associated with tracheal intubation, with other, iatrogenic causes scarcely being reported. We herein reported the first case of tracheobronchial rupture caused by chest compression during cardiopulmonary resuscitation. The present case highlights the importance of close follow-up after cardiopulmonary resuscitation, even if the patients are not intubated.

Simulated slide tracheoplasty for congenital tracheal stenosis using three-dimensional printed models.

PURPOSE: The slide tracheoplasty (STP) is the standard treatment for severe congenital tracheal stenosis (CTS). Understanding the features of the tracheal stenosis in each case and choosing an appropriate incision design are very important for successfully executing the procedure. The present study aimed to evaluate the advantages of three-dimensional (3D) printed models of the trachea for improving CTS. METHODS: Three-D tracheal models were created using computed tomography (CT) data from ten patients undergoing STP for CTS. Simulated surgery was performed using the hollow models after reinforcing with them with a coating of gum spray. Clinical outcomes, including patient survival, postoperative surgical interventions, and time required for STP, were compared with the corresponding values in the last ten patients before the introduction of 3D model simulations. RESULTS: All ten patients for whom simulated surgery using a 3D tracheal model were conducted achieved good airway patency after their STP. The surgeons reported feeling that the 3D model simulations were highly effective although there was no significant difference in the clinical outcomes of the groups with or without simulated STP. The models were useful not only for surgical planning but also for sharing important information among the multidisciplinary team and the patients' family. CONCLUSION: Our experience using 3D tracheal models demonstrated several features enabling improvement in the surgical treatment of CTS.

Outcomes of slide tracheoplasty for congenital tracheal stenosis in 80 children: A 22-year single-center experience.

PURPOSE: The application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes. METHOD: Patients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records. RESULTS: Eighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival. CONCLUSION: Although STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention.

Post-esophageal atresia repair double acquired tracheoesophageal fistulas treated successfully by gastric transposition: a case report.

BACKGROUND: Postoperative recurrence of tracheoesophageal fistula (TEF) is a frequent complication in the repair of esophageal atresia (EA). Based on the recent etiologic classification, a TEF that develops in a different new pathway from the original one is categorized as an acquired TEF. The TEFs that fall into this category have been reported to be refractory and their mechanisms have not been fully understood. Here, we report the complicated case of an acquired TEF derived from mediastinitis after the original TEF repair developed an anastomotic stricture. The TEF contained double fistulas, both towards the right lobe bronchi, and was repaired by gastric transposition through a retrosternal route. CASE PRESENTATION: The patient was diagnosed with Gross C esophageal atresia after birth and underwent tracheoesophageal fistula banding during the neonatal period. He experienced an intractable anastomotic stenosis after surgery which was treated with repeated balloon dilation therapy. By the age of 11 months, he developed a mediastinal abscess that improved with conservative treatment. At 18 months old, a fistula from the esophagus to the right superior lobe bronchus was identified. The patient underwent a right upper lobectomy to resect the fistula. However, at 21 months old, another fistula to the right lower lobe was revealed. An esophageal banding was done to relieve the respiratory symptoms. This was followed by esophagectomy and gastric transposition through the retrosternal route at 26 months old. The patient started rehabilitation and oral intake gradually after surgery. By 3 years after gastric transposition, he could already take blended food orally with the support of small amounts of enteral feeding. CONCLUSION: Cases of TEF derived from severe inflammation have the potential to form a complicated network and lead to recurrence. Surgeons should consider the possibility of multiple tiny fistulas in cases of severe acquired TEF. These may be repaired successfully by gastric transposition through the retrosternal route.

Living Donor Liver Re-Transplantation for Recurrent Hepatoblastoma in the Liver Graft following Complete Eradication of Peritoneal Metastases under Indocyanine Green Fluorescence Imaging.

The curability of chemotherapy-resistant hepatoblastoma (HB) largely depends on the achievement of radical surgical resection. Navigation techniques utilizing indocyanine green (ICG) are a powerful tool for detecting small metastatic lesions. We herein report a patient who underwent a second living donor liver transplantation (LDLTx) for multiple recurrent HBs in the liver graft following metastasectomy for peritoneal dissemination with ICG navigation. The patient initially presented with ruptured HB at 6 years of age and underwent 3 liver resections followed by the first LDLTx with multiple sessions of chemotherapy at 11 years of age. His alpha-fetoprotein (AFP) level increased above the normal limit, and metastases were noted in the transplanted liver and peritoneum four years after the first LDLTx. The patient underwent metastasectomy of the peritoneally disseminated HBs with ICG navigation followed by the second LDLTx for multiple metastases in the transplanted liver. The patient has been recurrence-free with a normal AFP for 30 months since the second LDLTx. To our knowledge, this report is the first successful case of re-LDLTx for recurrent HBs. Re-LDLTx for recurrent HB can be performed in highly select patients, and ICG navigation is a powerful surgical tool for achieving tumor clearance.

Laparoscopic Partial Closure for Congenital Portosystemic Shunt-Indications, Postoperative Management, and Subsequent Complete Closure.

Extrahepatic congenital portosystemic shunts (CPSSs) can be occluded by surgical or endovascular approaches. However, when the estimated portal vein (PV) pressure after the closure is high enough to induce symptoms associated with portal hypertension, partial closure is recommended to avoid life-threatening events. In this study, we attempted laparoscopic partial closure of a CPSS in two patients. Along with intraoperative real-time measuring of the PV pressure and angiography, laparoscopic partial closure was performed to achieve a PV pressure of ≤25 mmHg. Subsequently, the intrahepatic portal system grew in both patients. The partially ligated CPSS closed spontaneously in the first patient. In the second patient, laparoscopic complete closure was performed for the residual CPSS 6 months after the first operation. To our knowledge, this is the first report of laparoscopic partial closure for CPSS. Minimally invasive laparoscopic partial ligation of CPSS is technically feasible and useful when the estimated PV pressure is too high to tolerate one-step complete closure.

Metastasectomy of Hepatoblastoma Utilizing a Novel Overlay Fluorescence Imaging System.

BACKGROUND: The curability of hepatoblastoma (HB) largely depends on the achievement of radical surgical resection, even for metastatic tumors. However, the extension of the metastatic tumor when viewed through an endoscope with the conventional white-light mode is often unclear. Advancements in imaging technology utilizing indocyanine green (ICG) have facilitated precise resection of metastatic HBs, owing to the longer retention of ICG in such lesions than in other normal tissues. CASE: We utilized an endoscope loaded with the PINPOINT system (NOVADAQ Technologies, Inc., Ontario, Canada), which allows for real-time overlay visualization with the same focal range between the white-light mode and near-infrared mode. Metastatic HBs that have taken up ICG are visualized as an area of green color superimposed on a high-definition white-light image. A 19-year-old female who underwent liver transplantation for an unresectable HB 2 years earlier was noted to have metastases on the diaphragm and the pleura. Preoperative magnetic resonance imaging showed metastatic HBs on the right pleura extending from the ribs and the diaphragm. The margin of the metastatic tumor was more sharply demarcated by the PINPOINT system than that detected in the normal white-light mode. The tumor was successfully resected en bloc with real-time guidance utilizing the overlay image. The alphafetoprotein levels were normalized and have remained within normal limits in the 12 months since the operation. CONCLUSION: Novel overlay imaging technology with ICG makes it possible to achieve real-time precise resection of metastatic HBs.

Successful living donor liver retransplantation for graft failure within 7 days due to acute de novo donor-specific anti-human leukocyte antigen antibody-mediated rejection.

Growing evidence suggests a relationship between antibody-mediated rejection (AMR) and early graft failure due to a previously unknown etiology in liver transplantation (LTx). We herein report a 3-year-old boy who developed rapid graft failure due to de novo donor-specific antibody (DSA)-driven AMR a week after living donor LTx, requiring a second transplant on the 10th day after the first LTx. The pathology of the first graft showed massive necrosis in zone 3 along with positive C4d and inflammatory cell infiltrates in portal areas. The mean fluorescence intensity against human leukocyte antigen (HLA)-DR15, which was possessed by both the first and the second donor, peaked at 12 945 on the day before the second LTx. Antithymocyte globulin, plasma exchange along with i.v. immunoglobulin, rituximab, and the local infusion of prostaglandin E1, steroids, and Mesilate gabexate through a portal catheter were provided to save the second graft. To our knowledge, this is the first report to show a clear association between de novo DSA and acute AMR within 7 days of a LTx. Furthermore, we successfully rescued the recipient with a second graft despite possessing the same targeted HLA. The rapid decision to carry out retransplantation and specific strategies overcoming AMR were crucial to achieving success in this case of immunologically high-risk LTx.