Colonic diverticulitis in children: A retrospective study of 16 patients.

BACKGROUND: Pediatric colonic diverticulitis (CD) is a rare entity. This study aimed to investigate the clinical features of CD in children. METHODS: We performed a retrospective chart review of children aged ≤15 years who were diagnosed with CD in our institution from May 2006 to November 2016. RESULTS: Sixteen patients were diagnosed with CD. All CD cases were observed to be solitary cecal diverticulitis; 14 cases were detected using ultrasound and the other two cases were diagnosed by computed tomography. Five patients were male (31.3%), and the median age was 12 years (range, 8-15 years). Initial symptoms were fever (temperature >38°C) in six (37.5%) patients, right lower quadrant abdominal pain in 16 (100%), anorexia in eight (50%), and nausea / vomiting in five (31.3%). A patient experienced persistent constipation; however, diarrhea was not observed as a clinical symptom in any patient. The median duration from symptom onset to admission was 1 day (range, 0-4 days), and the median length of hospital stay was 6 days (range, 4-10 days). All CD cases were treated with intravenous antibiotics. The median follow-up period was 90 months (range, 37-163 months), and during this period, recurrence of CD was observed in three (18.8%) patients. At recurrence, antibiotics were administered in all cases. CONCLUSIONS: In this study, all cases of CD were solitary cecal diverticulitis, and ultrasound was useful for the diagnosis of cecal diverticulitis in children. Non-operative treatment should be recommended as an initial treatment for CD in children.

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.

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.

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.

Mitochondrial respiratory chain complex IV deficiency complicated with chronic intestinal pseudo-obstruction in a neonate.

A female infant born at 36 weeks gestational age with birthweight 2135 g, and who developed respiratory disorder, hyperlactacidemia and hypertrophic cardiomyopathy after birth, was admitted to hospital at 3 days of age. After admission, bilious emesis, abdominal distention, and passage disorder of the gastrointestinal tract were resistant to various drugs. Exploratory laparotomy was performed at 93 days of age, but no organic lesions were identified and normal Meissner/Auerbach nerve plexus was confirmed, which led to a clinical diagnosis of chronic intestinal pseudo-obstruction (CIPO). She was diagnosed with mitochondrial respiratory chain complex IV deficiency on histopathology of the abdominal rectus muscle and enzyme activity measurement. This is the first report of a neonate with mitochondrial respiratory chain complex deficiency with intractable CIPO. CIPO can occur in neonates with mitochondrial respiratory chain disorder, necessitating differential diagnosis from Hirschsprung disease.

A novel technique of catheter placement with fibrin glue to prevent pericatheter leakage and to enable no break-in period in peritoneal dialysis.

OBJECTIVE: Pericatheter leakage is a catheter-related complication of peritoneal dialysis (PD). To prevent pericatheter leakage, a modified technique of PD catheter insertion with fibrin glue was performed in 19 children. METHODS: At the time of PD catheter insertion, as much fibrin glue as possible was injected into the subcutaneous tissue along the tunneled segment of the catheter and then the skin was compressed. RESULTS: There was no occurrence of pericatheter leakage and full PD could be initiated 1 day (median) after implantation. CONCLUSIONS: This technique prevented pericatheter leakage completely even in smaller-weight infants and will enable initiation of full PD with no break-in period.

Intraventricular hemorrhage as a comorbid factor for intestinal obstruction in very low-birthweight infants.

BACKGROUND: In very low-birthweight infants (VLBWI), intestinal obstruction (IO) tends to increase in severity due to the immaturity of the intestine and perinatal events. We examined the clinical comorbid factors related to IO in VLBWI. METHODS: Clinical data of 160 VLBWI admitted to the neonatal intensive care unit in 2006-2011 were retrospectively reviewed. Patients were divided into two groups: IO group (n = 62) and non-IO group (n = 98). IO was defined as bile excretion via the mouth or nasogastric tube within 30 days after birth. The relationship between clinical factors and the incidence of IO was analyzed. RESULTS: On univariate analysis, gestational age, birthweight, and the incidence of chronic lung disease, patent ductus arteriosus, intraventricular hemorrhage (IVH), retinopathy of prematurity, and the postnatal use of mechanical ventilation, catecholamines, steroids, and sedatives were associated with IO. On multivariate analysis, only IVH was strongly associated with IO (OR, 4.74; P < 0.01). CONCLUSIONS: IVH is a significant comorbid factor of IO in VLBWI.

Efficacy and histopathological esophageal wall damage of biodegradable esophageal stents for treatment of severe refractory esophageal anastomotic stricture in a child with long gap esophageal atresia.

A case in which a self-expandable biodegradable (BD) esophageal stent was used for a refractory esophageal anastomotic stricture (EAS) in a 5-year-old female is presented. The patient underwent closure of a tracheoesophageal fistula and gastrostomy in the neonatal period. Esophagoesophagostomy was performed at 18 months of age after a multistaged extrathoracic esophageal elongation procedure. The patient developed refractory EAS and required repeated esophageal balloon dilation. Four sessions of esophageal BD stenting were performed from the age of 5-8 years. Each BD stenting allowed her to eat chopped food, but the anastomotic stricture recurred 4-7 months after the procedure. No major complications were observed, though transient chest pain and dysphagia were observed after each stenting. Finally, at 8 years of age, EAS resection and esophagoesophageal anastomosis were performed. The resected specimens showed thickened scar formation at the EAS lesion, while the degree of esophageal wall damage, both at the proximal and distal ends of the stricture, was slight. To the best of our knowledge, this is the first case report of this kind of treatment and assessment of damage to the esophageal wall microscopically. The advantages and problems of the use of BD stents in children are discussed.

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.