Clinical outcomes after tracheoplasty in patients with congenital tracheal stenosis in 1997-2014.

BACKGROUND: Mortality and morbidity of congenital tracheal stenosis (CTS) remain high. The aim of this study was to determine the factors predicting 12-month survival and 2-month successful extubation after tracheoplasty in patients with CTS. METHODS: Retrospective chart reviews were conducted in patients with CTS undergoing tracheoplasty at a single institution between 1997 and 2014. Patients' characteristics at disease onset and tracheoplasty were summarized. Twelve-month survival rate and 2-month extubation rate without tracheotomy after tracheoplasty were analyzed. RESULTS: We reviewed 81 patients' records. Multivariate analysis for 12-month survival revealed that older age (>2 months, hazard ratio [HR]: 0.08, 95% confidence interval [CI]: 0.02-0.36) or heavier body weight (>4.4 kg, HR: 0.13, 95% CI: 0.02-0.73) at tracheoplasty was a predictive factor for survival. Body weight at tracheoplasty (>8.2 kg, HR: 3.83, 95% CI: 1.88-7.79), preoperative balloon dilatation (HR: 0.30, 95% CI: 0.12-0.78), and carina involvement (HR: 0.36, 95% CI: 0.19-0.69) were predictive factors for successful extubation. CONCLUSIONS: Although CTS management is individualized, age or body weight at tracheoplasty needs to be considered and assessed for survival, as well as preoperative balloon dilatation, and carina involvement for successful extubation. LEVELS OF EVIDENCE: Level III.

The role of adjunctive procedures in reducing postoperative tracheobronchial obstruction in single lung patients with congenital tracheal stenosis undergoing slide tracheoplasty.

PURPOSE: Congenital tracheal stenosis (CTS) and a single right lung treated with slide tracheoplasty (ST) has relatively high rates of mortality and morbidity. We report a single institution's experience with adjunctive procedures at the time of ST to reduce postoperative tracheal obstruction in patients with a single right lung and CTS. METHODS: With IRB approval, 8 patients with a single right lung and CTS who underwent ST in our institution between 2008 and 2016 were reviewed. RESULTS: Seven of 8 patients (88%) survived, with a median follow-up period of 35 (3-89) months. The only mortality was because of complications unrelated to the airway. Six of 8 patients underwent ST anterior to the aortic arch (tracheal translocation, TT). This was combined with tracheopexy concomitantly in 3 patients and subsequently in one patient. Two patients had insufficient tracheal length for TT. Both underwent aortopexy. Of three patients undergoing TT with tracheopexy concomitantly, two have been successfully extubated, and a third has required tracheostomy for subglottic stenosis. All patients undergoing TT without tracheopexy have experienced severe tracheobronchomalacia. CONCLUSION: We conclude that adjunctive TT with tracheopexy might be associated with lower rates of tracheobronchial obstruction in patients with a single right lung undergoing ST for CTS. LEVEL OF EVIDENCE: Level IV.

Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.

PURPOSE: The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). MATERIALS AND METHODS: Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. RESULTS: Fetal hydrops was visualized using MRI in 9.2 % of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8 % of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04 ± 1.71 vs. 0.98 ± 0.50, P < 0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37 ± 1.28 to 1.14 ± 0.84 in CCAM and from 1.08 ± 0.47 to 0.46 ± 0.64 in non-CCAM). CONCLUSIONS: An estimated 8-9 % of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.

Clinical Features of Congenital Cystic Lung Diseases: A Report on a Nationwide Multicenter Study in Japan.

AIM: The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. METHODS: Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. RESULTS: In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p < 0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. CONCLUSIONS: Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

Complete transection of the left main bronchus caused by blunt thoracic trauma in a child treated by bronchoplasty and lung parenchyma preservation.

Blunt bronchial injuries can be difficult to diagnose and can present months or years after an initial incident. The present case report describes complete transection of a major airway after a potentially fatal delayed diagnosis with a successful outcome after bronchoplasty without the removal of lung parenchyma.

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.

Treatment of postoperative tracheal granulation tissue with inhaled budesonide in congenital tracheal stenosis.

PURPOSE: Tracheal obstruction by granulation tissue can compromise the postoperative course in congenital tracheal stenosis (CTS). Balloon dilatation and stenting may be required. Budesonide is a corticosteroid with topical anti-inflammatory effects. In 2008, we used inhaled budesonide for treatment of postoperative granulation tissue for the first time in CTS, resulting in significant improvement. The aim of this study was to evaluate the efficacy of inhaled budesonide for treatment of postoperative granulation tissue in CTS. METHODS: Retrospective chart review was conducted. From 2004 through 2011, we performed 39 tracheoplasties. Forced stenting ± balloon dilatation (S/B) was required when airway obstruction with tissue granulation was life-threatening. We compared the requirement for S/B between the early group without budesonide (2004-Nov. 2008, Early) and the late group with budesonide (Dec. 2008-2011, Late). Statistical analysis was performed using Fisher's Exact test. RESULTS: Eleven of 22 in Early and 8 of 17 in Late were successfully extubated, never having had life-threatening tissue granulation. The remaining patients in each group (11 in Early and 9 in Late) required tracheostomies due to postoperative complication. Ten in Early and 5 in Late with tracheostomies developed granulation tissue. Of these patients, the 10 in Early required S/B, while none of the 5 in Late required S/B (P=.0003). Bronchoscopy demonstrated significant regression of granulation tissue in all cases treated with inhaled budesonide. CONCLUSION: Inhaled budesonide is effective for treatment of tracheal granulation tissue in patients with tracheostomies after repair of CTS.

Pediatric cardiothoracic surgery in patients with unilateral pulmonary agenesis or aplasia.

BACKGROUND: Unilateral pulmonary agenesis or aplasia (UPA), a rare developmental defect of the lung, is sometimes associated with congenital heart and tracheal diseases. The purpose of this study was to assess our experience of pediatric cardiothoracic surgery in UPA patients. METHODS: Cardiothoracic surgery for congenital heart defect or tracheal stenosis performed between 1981 and 2010 in 8 UPA patients (agenesis in 5 and aplasia in 3) was reviewed retrospectively. Associated cardiac anomalies included ventricular septal defect, double outlet right ventricle with pulmonary atresia, total anomalous pulmonary venous connection, and interrupted aortic arch complex. RESULTS: For 7 patients with right UPA and 1 patient with left UPA, 12 cardiothoracic operations were performed, including 8 cardiac procedures in 4 patients and 4 tracheal procedures in 4 patients. Cardiac palliative repairs included Blalock-Park anastomosis, systemic-to-pulmonary artery shunt, and pulmonary artery banding. Cardiac definitive repairs included ventricular septal defect closure, subaortic membrane resection, modified Konno procedure, total anomalous pulmonary venous connection repair, and Rastelli-type operation. Tracheal repairs were costal cartilage tracheoplasty and slide tracheoplasty. The median age at surgery was 8 months and median body weight was 6.2 kg; the median operative time was 6.5 hours. There were 3 hospital deaths and 1 late death, with the 1-year mortality rate of 25%. Cardiopulmonary bypass-induced acute lung injury has occurred in 3 cases, 2 of which required extracorporeal membrane oxygenation support. Younger age of less than 1 month and prolonged cardiopulmonary bypass time of more than 200 minutes were related to operative risk factors for hospital mortality and morbidity. CONCLUSIONS: Most of the pediatric cardiothoracic operations in UPA patients were successfully performed through an optimal surgical approach and procedure, but they still presented surgical risks of high mortality and morbidity. Perioperative management of UPA patients should be provided with a precise understanding of anatomic configuration and a careful consideration of underlying risk factors.

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.

[Advances in pediatric thoracic surgery during the past 50 years].

Surgical strategies and treatment for pediatric airway and lung diseases have advanced significantly, especially in the fields of subglottic stenosis, congenital tracheal stenosis, congenital cystic lung lesions, and endoscopic surgery, during the past 50 years. Pediatricians, pathologists, and pediatric surgeons have engaged in continuous discussions at scientific meetings to establish standard terminology and operative indications and to refine surgical techniques such as laryngoplasty, sliding tracheoplasty, lobectomy under thoracotomy and thoracoscopy. The modified Myer-Cotton grading system for subglottic stenosis was proposed and proved to be useful in selecting reconstructive operative techniques. The addition of aortopexy to sliding tracheoplasty was also confirmed to be effective in maintaining a wide postoperative tracheal lumen. The disease entities of each type of cystic lung lesion were clarified, and the clinical and etiological importance of bronchial/bronchiolar atresia was emphasized. A classification of congenital cystic lung disease was proposed based on anatomic and embryologic considerations. In this classification, congenital pulmonary airway anomaly was introduced to replace congenital cystic adenomatoid malformation of the lung. Intralobar sequestration of the lung must be located in the lower lobe in this definition and classification.

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.

Successful tracheobronchial reconstruction of communicating bronchopulmonary foregut malformation and long segment congenital tracheal stenosis: a case report.

Communicating bronchopulmonary foregut malformation (CBPFM) and congenital tracheal stenosis (CTS) are difficult developmental disorders especially when they are presented simultaneously in a patient. The authors report a case of a newborn boy born at 37 weeks of gestation weighing 2356 g with CBPFM (right esophageal lung) and long segment CTS. Staged surgical repair (by-force endotracheal intubation for securing the airway followed by bronchotracheal anastomosis for CBPFM, tracheostomy with handmade, length-adjustable tracheostomy tube, and slide tracheoplasty) was performed. He has been healthy without tracheostomy for 25 months after slide tracheoplasty. This is the first report of a successful tracheobronchial reconstruction for a patient with a long segment CTS and CBPFM preserving the affected lung function.

Strategy for surgical treatment of congenital subglottic stenosis in children.

BACKGROUND/PURPOSE: Congenital subglottic stenosis is a rare anomaly caused by thickened cricoid cartilage. We report our surgical techniques, comprising anterior cricoid split (ACS), laryngotracheoplasty (LTP), KTP laser ablation, and application of a tracheal opening retainer (TOR) into the tracheostomy site. METHODS: Nine patients have been treated since 1988. Four patients (median age 85 days; range 5 days to 6 months) underwent ACS. Another four patients (median age, 17 months; range, 5-57 months) underwent LTP using costal cartilage grafts, although two had undergone tracheostomy before LTP. One patient underwent LTP, ablation of the projecting part of the cricoid cartilage with KTP laser (LTP + Laser) and, preservation of the tracheal opening by placement of the TOR. RESULTS: All ACS and LTP patients were successfully extubated at a median of 32 days (range 23-91 days) and 23 days (range 6-31 days) postoperatively, respectively. The LTP + Laser patient was extubated 35 days after surgery and the TOR was removed asymptomatically 20 days after extubation of the stent tube. CONCLUSIONS: Anterior cricoid split is useful for patients ≤ 6 months old and LTP is useful for patients >6 months old and/or with tracheostomy. KTP laser ablation is effective to remove thickened parts of cricoid cartilage protecting the vocal cords. The tracheal opening preserved by the TOR works as an additional channel to safeguard respiration during the extubation process.

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.

Aortopexy with tracheal reconstruction for postoperative tracheomalacia in congenital tracheal stenosis.

PURPOSE: Congenital tracheal stenosis is a rare condition and can be difficult to manage. One source of difficulty is postoperative tracheomalacia requiring long-term tracheal stenting. To prevent symptomatic postoperative tracheomalacia, we have been adding aortopexy to tracheal reconstruction since 2008. The aim of this study was to evaluate efficacy of aortopexy for preventing postoperative tracheomalacia after reconstruction of congenital tracheal stenosis. METHODS: Retrospective chart review was conducted. From October 2003 to March 2011, 24 had tracheal reconstruction without aortopexy (group A) and 8 with aortopexy (group B). Statistical analysis was performed using Fisher's Exact test. RESULTS: One had anastomotic leakage in group A, and 1, in group B (P = .44). Eleven patients required tracheostomy because of postoperative tracheomalacia confirmed by postoperative bronchoscopy in group A vs none in group B (P = .029). CONCLUSIONS: We found that aortopexy with tracheal reconstruction reduced the need for postoperative tracheostomy in this patient group. Although there is a potential risk of anastomotic leakage because of the suspension suture on the anterior tracheal wall to aorta, we did not detect an increased incidence after aortopexy. Thus, aortic suspension may be a useful adjunct to prevent symptoms of tracheomalacia in these patients.

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.