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.

Predicting Cirrhosis and Poor Outcomes of Bile Drainage Surgery for Biliary Atresia: A Multicentric Observational Study in Japan.

OBJECTIVE: To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis. BACKGROUND: Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified. METHODS: The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity. RESULTS: The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation. CONCLUSIONS: Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx.

Reappraisal of anatomical liver resection for hepatoblastoma in children.

Background: The principle of hepatoblastoma (HB) treatment is complete resection. The removal of tumor-bearing section(s) or hemiliver is widely accepted. However, neither the standardized anterior approach for right hepatectomy nor parenchymal sparing anatomical liver resection has been described for HB. Methods: We retrospectively reviewed the clinical course of two pediatric HB patients who underwent extended right hepatectomy using the anterior approach with the liver hanging maneuver and one who underwent parenchymal sparing anatomical liver resection of S4 apical+S8 ventral/dorsal+S7. The critical aspects of surgical techniques are described in detail. Results: In all three patients, R0 resection was achieved without complications and are currently alive without recurrence after an average follow-up of 23 months. Intraoperative cardiac hemodynamics were stable, even in a trisomy 18 patient with cardiac disease. Conclusions: Our findings suggest that these innovative techniques established in adults are safe and feasible for HB in children. These techniques also allow optimal anatomical liver resection to accomplish curative surgery while maintaining the functional reserve of the remnant liver.

Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions.

No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patients were collected between 1992 and 2020, including the type of enterostomy, type of revision, and length of the intestines. Ganglion cells in the myenteric plexus were enumerated using Hu C/D staining. The ratio of the length of the small intestine to its height was defined as the intestinal ratio (IR). The outcomes were assessed using the stoma output, growth parameters including the body mass index (BMI), and parenteral nutrition (PN) dependency. All patients required a diverting enterostomy. The IR ranged from 0.51 to 1.75 after multiple non-transplant surgeries. The stoma types were tube-stoma, end-stoma, Santulli-type, and Bishop-Koop (BK)-type. Patients with Santulli- or BK-type stomas had better BMIs and less PN dependency in terms of volume than those with end-stomas or tube-stomas. Two patients with BK-type stomas were off PN, and three who underwent an intestinal transplantation (Itx) achieved enteral autonomy. The management of CIHG involves a precise diagnosis using Hu C/D staining, neonatal enterostomy, and stoma revision using the adjusted IR and Itx if other treatments do not enable enteral autonomy.

Vertical Umbilical Incision Achieves Better Cosmesis Than Periumbilical Incision in Neonates and Infants.

PURPOSE:  The transumbilical approach is widely used for minimally invasive surgery in children. We compared herein the postoperative cosmesis between two types of transumbilical approach: a vertical incision versus periumbilical incision. METHODS:  Patients with a transumbilical laparotomy before age one year were prospectively enrolled between January 2018 and December 2020. A vertical incision or periumbilical incision was chosen at the surgeon's discretion. After excluding patients receiving a relaparotomy via another site, a questionnaire about the appearance of the umbilicus was completed by the patients' guardians at postoperative month 6 to assess satisfaction and determine the visual analog scale score. A photograph of the umbilicus was taken while the questionnaire was being administered for later assessment by surgeons blinded to the scar and umbilical shape. RESULTS:  Forty patients were enrolled; 24 patients received a vertical incision while 16 received a periumbilical incision. The incision length was significantly shorter in the vertical incision group (median: 2.0; range: 1.5-3.0 cm vs. median: 2.75; range: 1.5-3.6 cm) (p = 0.001). The patients' guardians reported significantly higher satisfaction (p = 0.002) and higher scores on the visual analog scale (p = 0.046) in the vertical incision group (n = 22) than in the periumbilical incision group (n = 15). The surgeons' evaluation was associated with significantly more patients with a vertical incision than with a periumbilical incision achieving a cosmetically preferable outcome, including an invisible or fine scar and a normal umbilical shape. CONCLUSION:  A vertical umbilical incision can provide better postoperative cosmesis than a periumbilical incision.

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.

Recent advances in surgical strategies and liver transplantation for hepatoblastoma.

Hepatoblastoma (HB) is the most common malignant liver tumor in children. Although the development of treatment strategies with advances in chemotherapy has greatly improved the prognosis of HB, surgical resection and liver transplantation still play a vital role in the treatment of HB. In recent years, technological innovations have led to the development of new surgical approaches for HB. In this review, we describe the latest research on the surgical management of HB, including new imaging technologies, minimally invasive approaches, and the application of associating liver partition portal vein ligation for staged hepatectomy. We also discuss the current role of liver transplantation, use of ante-situm or ex-situ liver resection with auto-transplantation, and management of metastatic HB.

Treatment Strategy for Currarino Syndrome Complicated With Anorectal Stenosis.

PURPOSE: The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies. METHODS: Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution. RESULTS: In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers. CONCLUSIONS: The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease.

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.

Successful surgical treatment of congenital tracheal stenosis combined with tracheal bronchus and left pulmonary artery sling: a 10-year single-institution experience.

PURPOSE: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. METHODS: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. RESULTS: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support. CONCLUSION: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. LEVEL OF EVIDENCE: Level IV.

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.

Efficacy of early endoscopic intervention in pediatric pancreatic duct injury management.

BACKGROUND: The optimal management method for pediatric pancreatic trauma is controversial. Moreover, the efficacy of stent placement via endoscopic retrograde pancreatography (ERP) remains poorly documented. METHODS: The present, retrospective review of pediatric patients with pancreatic trauma was conducted from 2010 to 2020 at a single institution. RESULTS: Ten, male children with the median age of 9.5 years (range 4-14 years) with a grade I (n = 2), II (n = 4) or III (n = 4) pancreatic injury were identified. Of six of these patients in whom ERP was performed, four had a pancreatic duct injury (PDI). Pancreatic stent placement was performed in all the patients with ERP at a site proximal to the injury in four patients and across the injury in two patients. A pseudocyst or pancreatic fluid collection was detected in five patients, of these, two with a grade II injury were managed successfully with conservative therapy while three with PDI required surgery. In the four patients with PDI, only one in whom the stent was placed across the PDI was able to avoid surgery. CONCLUSION: Therapeutic ERP might be effective even if a patient has a PDI, therefore, early ERP should be considered as a treatment option.

Inverse T-shaped sternotomy as novel thoracoplasty for severe chest deformation and tracheal stenosis.

BACKGROUND: Patients with severe motor and intellectual disabilities often suffer from tracheal stenosis due to chest deformation and brachiocephalic artery compression, which sometimes leads to serious complications, such as dying spell and tracheobrachiocephalic artery fistula. We herein described our experience of performing a novel and simple thoracoplastic procedure combined with brachiocephalic artery transection in two patients with severe chest deformation and tracheal stenosis. CASE PRESENTATION: The patients were a 12-year-old female with cerebral palsy due to periventricular leukomalacia and a 21-year-old male with subacute sclerosing panencephalitis stage IV in the Jabbour classification following a laryngotracheal separation. Both patients showed severe chest deformation and symptoms of airway stenosis resulting in dying spells. The sternum was laterally transected between the manubrium and the sternal body, and a manubriotomy was performed longitudinally, ending with an inverse T-shaped sternotomy. Since the clavicle and the first rib remained attached to the halves of the divided manubrium, the sternum was allowed to be left open, resulting in improvement of the mediastinal narrowing and tracheal stenosis. Postoperative computed tomography (CT) showed that the distance between the halves of the manubrium was maintained at 10-11 mm, and that the mediastinal narrowing in both patients improved; the sternocervical spine distance increased from 20 mm to 22  and 13 mm to 16 mm, respectively. The patients' tracheal stenosis below the sternal end of the clavicle and the manubrium and respiratory symptoms improved, and the patients are currently at home in a stable condition with no chest fragility and no upper limb movement disorder 1 year after surgery. CONCLUSIONS: Our observations suggested that the inverse T-shaped sternotomy combined with brachiocephalic artery transection may relieve symptoms of tracheal stenosis due to severe chest deformation in patients with severe motor and intellectual disabilities.

Current thoracoscopic approach for mediastinal neuroblastoma in Japan-results from nationwide multicenter survey.

BACKGROUND: Minimally invasive surgery (MIS) is appropriate for the treatment of some neuroblastomas (NBs); however, the indications and technical issues are unclear. This study aimed to clarify the current status of MIS for mediastinal NB in Japan. METHODS: Preliminary questionnaires requesting the numbers of neuroblastoma cases in which MIS was performed from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. Secondary questionnaires were sent to institutions with MIS cases to collect detailed data. RESULTS: One hundred thirty-four (84.2%) institutions returned the preliminary questionnaire and 83 institutions (52.2%) reported a total of 1496 operative cases. MIS was performed for 175 (11.6%) cases. Among the 175 cases, completed forms of 140 patients were returned and 40 (male, n = 28; female, n = 12) cases had mediastinal NB. Fourteen patients received thoracoscopic biopsy, none were converted to thoracotomy. Twenty-eight patients received MIS for radical resection, none were converted to thoracotomy. Perioperative complications (Horner's syndrome) were recognized after radical resection in one (2.5%) case. CONCLUSIONS: MIS was performed in a limited number of mediastinal NB cases. A thoracoscopic approach would be feasible for mediastinal NB.

Visualization of the human enteric nervous system by probe confocal laser endomicroscopy: a first real-time observation of Hirschsprung's disease and allied disorders.

BACKGROUND: Our group previously proved that the human enteric nervous system can be visualized with confocal laser endomicroscopy after topical application of cresyl violet using surgically resected intestine specimens. The present report documents the first in vivo visualization of the human enteric nervous system with confocal laser endomicroscopy using local cresyl violet staining. The aim of this study was to evaluate the technical feasibility and clinical efficiency of confocal laser endomicroscopy in patients with Hirschsprung's disease and allied disorders in vivo. METHODS: Confocal laser endomicroscopy was performed in vivo in two patients to confirm the presence of the enteric nervous system during surgery in patients with Hirschsprung's disease and allied disorders. Cresyl violet was gently injected from the serosal side into the muscular layer of the intestine, and scanning was performed within 30 min. Then, the scanned intestines were resected, and the visualized area of the specimens was pathologically evaluated. RESULTS: The ganglion cell nuclei and the enteric nervous system network were clearly visualized intraoperatively in both cases. The morphological findings were similar to the pathological findings of the enteric nervous system in both cases although the period of visibility was brief. CONCLUSION: This study demonstrated the first, real-time observation of the enteric nervous system in humans using confocal laser endomicroscopy and suggest the potential to identify the enteric nervous system intra-operatively during surgery for Hirschsprung's disease and allied disorders.

Maintenance treatment with infliximab for ulcerative ileitis after intestinal transplantation: A case report.

BACKGROUND: Evidence has been published on the successful applications of the anti-tumor necrosis factor alpha antibody infliximab, such as induction therapy, salvage treatment for acute cellular rejection, and treatment for chronic ulcerative inflammation, in intestinal transplant recipients. However, the optimal protocol for the effective use of infliximab remains largely undetermined due to scarcity of available clinical data. We report a continuative application of infliximab as maintenance therapy for recurrent chronic ulcerative ileitis in a recipient of isolated intestinal transplantation (ITx). CASE SUMMARY: The patient was a 11-year-old boy with intestinal motility disorder classified as a hypogenic type of intestinal dysganglionosis. The patient underwent living-donor related intestinal transplant. His immunosuppression regimen consisted of daclizumab, tacrolimus, and steroids. Although he did not show rejection while on tacrolimus monotherapy, routine screening endoscopy showed several ulcerative lesions in the distal end of the graft 2 years after the intestinal transplant. Endoscopic work up to evaluate the progression of anemia revealed stenosis with ulcerative inflammatory changes and multiple longitudinal ulcers in the graft. Since the endoscopic findings suggested ulcerative lesions in Crohn's disease, infliximab treatment was considered. Treatment with infliximab and a small dose of oral prednisolone afforded successful withdrawal of total parenteral nutrition and maintenance of a well-functioning graft without infectious complications for 5 years since the administration of the first dose of infliximab. CONCLUSION: Infliximab is effective as maintenance therapy for recurrent chronic ulcerative ileitis in an isolated ITx patient.

Prognosis of ultrasonographic low-grade pediatric appendicitis treated with supportive care.

BACKGROUND: A previous report proposed ultrasonography-based classification as a promising means of predicting pediatric spontaneously resolving appendicitis. The present study investigated the long-term prognosis of supportive care for low-grade appendicitis identified by ultrasonography, defined as an appendix with a smooth submucosal layer irrespective of blood flow or an appendix with an irregular layer and increased blood flow. METHODS: The present, retrospective cohort study enrolled patients under 16 years of age with acute appendicitis at a children's hospital between October 2010 and September 2016. The inclusion criteria were ultrasonography findings showing an appendix with (1) full visualization, (2) a diameter ≥6 mm, (3) a smooth submucosal layer or an irregular layer with increased blood flow, and (4) no appendiceal mass, abscess, or perforation. The exclusion criteria were: (1) a history of acute appendicitis, (2) antibiotic administration within 72 hours before diagnosis, and (3) antibiotic administration or surgery before supportive care. The primary outcome was the event-free duration, defined as a period of supportive care alone with no additional intervention or recurrence of appendicitis. RESULTS: One hundred and eighty-two patients were enrolled. The median Alvarado score was 7 (interquartile range, 6-8), and the median follow-up duration in event-free cases was 1,922 days (interquartile range, 1,347-2,614 days). The event-free rate was 75.0%, 67.0%, and 62.5%, at 1, 2, and 5 years, respectively. CONCLUSION: The long-term, event-free rate exceeded 60% in patients with low-grade appendicitis defined by ultrasonography who received neither surgery nor antibiotic treatment. Most recurrences occurred within 2 years of the initial diagnosis.