A case of biliary atresia with pancreaticobiliary maljunction.

BACKGROUND: The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings. CASE PRESENTATION: The patient was born at 40 weeks of gestation. His body weight at birth was 2850 g. At the age of 4 days, he had an acholic stool and was referred to our hospital. Abdominal ultrasonography showed that triangular cord sign was negative. The gallbladder was isolated with a diameter of 19 mm, and it contracted in response to oral feeding. His ultrasonographic findings were atypical for BA, but his jaundice did not improve. Therefore, we performed an operation at the age of 56 days. Intraoperative cholangiography showed a common bile duct and pancreatic duct and a common channel patent, while the common hepatic duct or intrahepatic duct was not visualized. Bile in the gallbladder contained colorless fluid, which showed elevated lipase level (34,100 IU/L). We performed Kasai portoenterostomy under the diagnosis of Kasai type IIIa BA with PBM. The patient's postoperative course was uneventful, and he was discharged on day 30 after the operation. Histopathological evaluation showed that the lumens of the common bile duct and cystic duct were patent. However, the common hepatic duct was closed, and only bile ductules with diameters of less than 50 µm were isolated. Infiltration of lymphocytes was detected in the porta hepatis. No apparent inflammation was observed around the cystic duct, which was constantly exposed to pancreatic juice because of reflux through PBM. CONCLUSIONS: Reflux of pancreatic juice through PBM might not be an etiological factor for BA, but might be associated with patency of the common and cystic bile ducts in Kasai type IIIa BA.

Predictive factor for intraoperative tumor rupture of Wilms tumor.

PURPOSE: For Wilms tumor, intraoperative tumor rupture with wide tumor spillage during surgical manipulation raises the classification to stage 3. Then, postoperative chemotherapy must be more intensive, and abdominal radiotherapy is added. Therefore, intraoperative tumor rupture should be avoided if possible. However, predictive factors for intraoperative tumor rupture have not been sufficiently described. Here we examined the risk factors for intraoperative tumor rupture. METHODS: Patients with Wilms tumor who underwent treatment according to the National Wilms Tumor Study or the Japanese Wilms Tumor Study protocol at our institution were reviewed retrospectively. Collected cases were categorized into two groups: the ruptured group and the non-ruptured group. Risk factors for intraoperative tumor rupture, including the ratio of the tumor area to the abdominal area in a preoperative single horizontal computed tomography slice (T/A ratio), were investigated in both groups. RESULTS: The two groups were not different in age, body weight, tumor laterality, sex, or histological distribution. The T/A ratio in the ruptured group was significantly higher than that in the non-ruptured group. Receiver operating characteristic curve analysis identified a discriminative value for a T/A ratio >0.5. CONCLUSION: The T/A ratio can be a predictive factor for intraoperative tumor rupture of Wilms tumor.

Evaluation of the intraoperative risk factors for esophageal anastomotic complications after primary repair of esophageal atresia with tracheoesophageal fistula.

PURPOSE: The aim of this study is to identify the risk factors for esophageal anastomotic stricture (EAS) and/or anastomotic leakage (EAL) after primary repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) in infants. METHODS: A retrospective chart review of 52 patients with congenital EA/TEF between January 2000 and December 2015 was conducted. Univariate and multivariate analyses were performed to identify the risk factors for anastomotic complications. RESULTS: Twenty-four patients were excluded from the analysis because they had insufficient data, trisomy 18 syndrome, delayed anastomosis, or multi-staged operations; the remaining 28 were included. Twelve patients (42.9 %) had anastomotic complications. EAS occurred in 12 patients (42.9 %), and one of them had EAL (3.57 %). There was no correlation between anastomotic complications and birth weight, gestational weeks, sex, the presence of an associated anomaly, age at the time of repair, gap between the upper pouch and lower pouch of the esophagus, number of sutures, blood loss, and gastroesophageal reflux. Anastomosis under tension and tracheomalacia were identified as risk factors for anastomotic complications (odds ratio 15, 95 % confidence interval (CI) 1.53-390.0 and odds ratio 8, 95 % CI 1.33-71.2, respectively). CONCLUSION: Surgeons should carefully perform anastomosis under less tension to prevent anastomotic complications in the primary repair of EA/TEF.

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.

PURPOSE: The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis. METHODS: The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed. RESULTS: Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior-anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior-anterior slide tracheoplasty and was successfully extubated. Two patients with left-right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated. CONCLUSION: Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior-anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

Innominate artery compression of the trachea in patients with neurological or neuromuscular disorders.

OBJECTIVES: The purpose of this study was to review and assess our surgical management of innominate artery compression of the trachea (IACT) in patients with neurological or neuromuscular disorders (NMDs). METHODS: Thirty patients with NMD who underwent surgical treatment for IACT at Kobe Children's Hospital and Kobe University Hospital from 2002 to 2012, were enrolled in this retrospective study. The clinical outcomes of preventive elective surgery for IACT (Group A, n=20) were assessed and compared with those of emergent surgery (Group B, n=10). RESULTS: A total of 27 patients underwent innominate artery transection (17 in Group A and 10 in Group B), and 3 patients in Group A underwent innominate artery reimplantation using prosthetic graft interposition. No operative or early death occurred. There were no cases of postoperative mediastinitis or neurological complications. The operative benefits in Group A included a smaller skin incision, more limited sternotomy, less blood loss, shorter operative time and shorter hospital stay, compared with Group B. No blood transfusion was required in Group A. The number of patients in whom cerebral circulation was assessed before surgery in Group A was significantly higher than those in Group B. CONCLUSIONS: Preventive elective surgery for IACT provides many advantages, including minimally invasive procedures and successful postoperative outcomes without neurological complications in patients with NMD. Because this surgical management can prevent the tragic occurrence of a tracheo-innominate artery fistula or an exacerbation of tracheomalacia, it would be an optimal surgical treatment for IACT to improve the quality of life in patients with NMD.

Severely high serum unbound bilirubin level after abdominal surgery in a newborn.

We report a newborn with intestinal malrotation who developed a severely high serum unbound bilirubin level and a low serum albumin level without a marked increase in serum total bilirubin level after abdominal surgery, which required exchange transfusion and albumin supplementation. The serum unbound bilirubin level may be highly relative to the serum total bilirubin level in newborns who have undergone abdominal surgery soon after birth and are hypoalbuminemic after surgery.

Innominate artery transection for patients with severe chest deformity: optimal indication and timing.

PURPOSE: The innominate artery sometimes compresses the trachea, leading to tracheomalacia and highly fatal tracheoinnominate fistula in patients with severe chest deformity. This study is focused on the indication of innominate artery transection for the definitive treatment of these complications. PATIENTS AND METHODS: We retrospectively analyzed the medical records of eight patients who underwent transection of innominate artery. RESULTS: All patients had developed severe chest deformity and their symptoms were life-threatening anoxic spell or endotracheal hemorrhage. Bronchoscopy showed tracheomalacia with or without pulsatile granulations on the anterior wall of the trachea underlying the innominate artery. In six cases who had previously undergone tracheostomy or laryngotracheal separation, the tracheal tube tip made granulations or tracheoinnominate fistulas. In addition to transection of innominate artery, the tracheoinnominate fistula was closed in two cases and the artery was transposed in one. All patients survived without neurologic complications and airway symptoms postoperatively. CONCLUSIONS: For patients with severe chest deformity, innominate artery transection is indicated when they have tracheal compression by the artery and need to be intubated through the compressed part of trachea to secure the airway. This would be the best timing to schedule the prophylactic operation.

A very low-birth-weight infant with spontaneous perforation of a choledochal cyst and adjacent pseudocyst formation.

Spontaneous perforation of a choledochal cyst with ensuing pseudocyst formation is a very rare complication. We report the development of a pseudocyst adjacent to a choledochal cyst in a very low-birth-weight infant at 2 months of age. Elective excision of the choledochal cyst and biliary tract reconstruction were successfully performed 2 months later when the infant weighed 3 kg. Delayed primary repair may be a viable alternative treatment for low-birth-weight infants with choledochal cysts.

Topical application of basic fibroblast growth factor reduces esophageal stricture and esophageal neural damage after sodium hydroxide-induced esophagitis in rats.

PURPOSE: To investigate the effect of topical application of basic fibroblast growth factor (bFGF) after caustic esophageal injury in rats. METHODS: Thirty-six male rats were randomly divided into three groups. Corrosive esophageal injury was produced by internal application of 30% sodium hydroxide (NaOH) solution to the distal esophagus. Group A rats were uninjured. Group B rats were injured and untreated. Group C rats were injured and received topical bFGF (10 µg/ml). Surviving rats were killed at 28 days. The survival rate, body weight gain, symptoms and histopathological changes that included tissue damage score, ratio of esophageal luminal area/total esophageal area (LA/TA) and the proportion of a neural marker PGP 9.5-positive area were assessed. RESULTS: The survival rate and the prevalence of symptoms were not significantly different between Groups B and C. Although the tissue damage score did not differ in Groups B and C, LA/TA was significantly higher in Group C than in Group B. The proportion of the PGP 9.5-positive area was significantly lower in Groups B and C than in Group A; however, it was higher in Group C than in Group B. CONCLUSION: Topical application of bFGF was effective in preventing stricture after NaOH-induced esophagitis.

The efficacy of slide tracheoplasty in a growing rabbit model.

PURPOSE: Slide tracheoplasty for congenital tracheal stenosis was reported by Tsang. This procedure provides good results, but there are few studies about the tracheal growth of post-surgical condition. Recently, not only traditional posterior to anterior slide plasty but also right side to left side slide plasty was reported. We evaluated the tracheal growth after slide tracheoplasty in growing Japanese white rabbit model. METHODS: The Japanese white rabbits, weighing 1,600-2,400 g, were used for this study. One-third of the estimated length of the trachea was slantingly resected about an appropriate angle. We performed a slant, side-to-side tracheal anastomosis in two ways (slanted from the front to the back in four animals, and slanted from right to left in four animals) on growing rabbits as an animal model of slide tracheoplasty. RESULTS: All the rabbits were alive until the 10th week after surgery. There was no evidence of differential growth in any part of the circumference of the normal and anastomotic tracheas. Histologically, all tracheal lumina were completely lined with normal respiratory epithelium and normal surrounding cartilage. CONCLUSION: The results presented that these two methods did not prevent the tracheal growth and trachea did not buckle macroscopically. Both slide tracheoplasties did not interrupt the growth of trachea.

Hypoalbuminemia following abdominal surgery leads to high serum unbound bilirubin concentrations in newborns soon after birth.

BACKGROUND: The serum concentration of unbound bilirubin (UB), which is bilirubin not bound to albumin (Alb), is a better index than total bilirubin concentration (TB) for identifying infants at risk for developing bilirubin neurotoxicity. The degree to which the hypoalbuminemia following abdominal surgery in jaundiced newborns affects bilirubin binding is unknown. OBJECTIVE: To determine whether lower Alb occurring in newborns undergoing abdominal surgery shortly after birth results in significantly higher UB in serum versus nonsurgical patients at comparable serum TB. METHODS: A matched case-control study was conducted with term and late-preterm newborns. The surgery group included 15 newborns who underwent abdominal operation within 3 days after birth. Clinical and laboratory data (serum UB, TB, and Alb concentrations, UB/TB ratio, and binding constant) in the surgery group were collected and compared with those of 30 control newborns who did not undergo abdominal surgery (control group). RESULTS: Serum UB and the UB/TB ratio in the surgery group were significantly higher than those in the control group (p < 0.02, p < 0.001, respectively), whereas there were no significant differences in serum TB and binding constant between the groups. Serum Alb concentrations in the surgery group were significantly lower than those in the control group (p < 0.001). When pre- and postoperative serum Alb concentrations were compared, there was a significant decrease from 3.4 to 2.7 g/dl (p < 0.001). CONCLUSIONS: Our study suggests that hypoalbuminemia following abdominal surgery causes a higher serum UB at comparable serum TB in newborns.

Development of a model of benign esophageal stricture in rats: the optimal concentration of sodium hydroxide for stricture formation.

PURPOSE: To investigate the optimal concentration of sodium hydroxide (NaOH) on esophageal stricture formation in rats to establish an animal model of benign esophageal stricture (BES). METHODS: Corrosive esophageal burn was produced by internal application of different concentrations of NaOH to the distal esophagus in rats. As much as 66 male rats were randomly divided into eight groups: Group A (control, n = 6), Group B (sham-operated group, n = 6), Group C (5% NaOH, n = 8), Group D (10% NaOH, n = 8), Group E (20% NaOH, n = 8), Group F (30% NaOH, n = 10), Group G (40% NaOH, n = 14), and Group H (50% NaOH, n = 6). Surviving rats were killed at 28 days. The survival rate, body weight gain, symptoms, and histopathological changes were assessed. RESULTS: The mortality rate was high in Groups G and H (73 and 67%). The prevalence of symptoms of BES was 43% in Groups D and E, 50% in Group F, 75% in Group G, and 100% in Group H. Statistically significant stricture formation of the esophagus was observed in Groups F and G. The degree of tissue damage was significantly higher in Groups E, F, and G. CONCLUSION: A high concentration of NaOH of 30% was required to establish a survivable BES model in rats.

Minimally invasive innominate artery transection for tracheomalacia using 3-dimensional multidetector-row computed tomographic angiography: report of a case.

We successfully performed transection of the innominate artery in a patient with a neuromuscular disorder through minimally invasive access after confirming the anatomical relationships of the vessel using 3-dimensional multidetector-row computed tomographic angiography. A 16-year-old girl with spinal muscular atrophy type 1 had been on long-term mechanical ventilation with a tracheostomy. She had scoliosis and tracheomalacia. Bronchoscopy showed a flattened and narrow lower trachea and an anterior pulsatile compression by the innominate artery. She underwent transection of the innominate artery to prevent tracheoinnominate artery fistula formation. Based on preoperative 3-dimensional multidetector-row computed tomographic angiography images, the innominate artery was transected through a small transverse curvilinear skin incision just below the suprasternal notch and an oblique partial manubriotomy from the suprasternal notch to the first left intercostal space.

Efficacy and safety of balloon-occluded retrograde transvenous obliteration for gastric fundal varices in children.

Balloon-occluded retrograde transvenous obliteration (B-RTO) is an interventional radiologic technique that obliterates gastric fundal varices (GFV) from draining veins under balloon occlusion. Few reports have described the use of B-RTO in children. Here, we report a case of B-RTO in a 2-year-old female patient with isolated huge varices in the gastric fundus associated with portal hypertension after surgery for biliary atresia. There was no complication and thrombosis of the varices was achieved. Our results demonstrate that B-RTO is an efficacious and safe treatment for children with isolated GFV.

Transplantation of the cryopreserved tracheal allograft in growing rabbits: effect of immunosuppressant.

Transplantation of a cryopreserved tracheal allograft is considered to be a useful strategy in treating congenital tracheal stenosis. Recent reports of tracheal transplant experiments have shown that the antigenicity of the trachea is decreased by cryopreservation, making transplantation of the trachea possible. However, we reported that cryopreserved tracheal allografts exhibited favorable patency, but no significant growth in an animal model. In this study, we hypothesize that an immune reaction may play a role in the failure of an allograft's growth. Each allograft was harvested from 90- to 120-day-old Japanese rabbits, immersed in preservation solution, stored in a programmable freezer until it reached -80 C, and then kept for 1 month. Orthotopic tracheal transplantation of four tracheal rings in an end-to-end fashion was performed in age-matched young rabbits. Ten recipients were classified into three groups: a group provided with tacrolimus (FK-506) as an immunosuppressant (n = 6), a group receiving a graft irradiated before transplantation (n = 2), and a control group (n = 2). All grafts were evaluated 4-8 weeks after transplantation (tacrolimus group: 3-13 weeks). Body weight gain of the tacrolimus group was less than that of the other two groups. All grafts were well incorporated with the recipients macroscopically, but the grafts showed no growth in diameter. Microscopic examination showed inflammation in the tacrolimus and control groups temporarily. The irradiated allografts had marked fibrosis in the subepithelium. Although calcification of the tracheal cartilage was present in all transplanted allografts, the difference between groups was not significant. Patency of the cryopreserved tracheal allografts was favorable but no growth occurred even with an immunosuppressant. Further studies are required to address the growth of the tracheal allograft.

Circular myectomy for the treatment of congenital esophageal stenosis owing to tracheobronchial remnant.

The management of the congenital esophageal stenosis is not well established. The authors present an infant with critical esophageal stenosis owing to tracheobronchial remnant. This lesion was managed successfully by circular myectomy of the esophageal wall without resection. An 1-year-old girl was referred for recurrent vomiting and dysphagia. An esophagogram showed an abrupt stenosis, and endoscopic ultrasonography showed cartilaginous tracheobronchial remnants within the esophageal wall. The extirpation of muscular layer, which contained cartilage, was attempted. Circular muscular layer was resected in 1 cm width leaving mucosal layer intact. Muscular layer was closed horizontally with absorbable sutures. The patient is free from the symptoms and eats normally 1 year after surgery. In case of short segmental stenosis owing to tracheobronchial remnant, this may be the preferred approach.