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.

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.

[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.

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.

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.

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.

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.

[The study of spinal abnormalities associated with cloacal exstrophy].

The treatment of spinal abnormalities with cloacal exstrophy is controversial. Ten cases of this complex treated at Hyogo Prefectural Kobe Children's Hospital between 1991 and 2010 are presented. In our series, all 10 patients had tethered spinal cords. In addition, there were 3 terminal myelocystoceles, 2 meningoceles, 7 lipomas, 5 thickened filums and 3 syrinxes. Eight of 10 patients underwent surgery, and no patient deteriorated. All 3 patients with terminal myelocystocele had lower extremity weakness, but motor functions in two patients improved after surgery. All 4 patients with lipoma and/or thickened filum were asymptomatic. Two of them were conservatively treated, and they remain asymptomatic. Terminal myelocystocele and symptomatic syrinx should be surgically treated.

[Multidisciplinary treatment for severe syndromic craniosynostosis].

We describe the treatment of patients having syndromic craniosynostosis with severe craniofacial abnormality. One patient had Cruzon's syndrome, one had Beare-Stevenson cutis gyrata syndrome, and four had Pfeiffers syndrome. Anterior cranial deformity in all patients was treated using fronto-orbital advancement (FOA) by gradual distraction. Initially, the frontal and supraorbital bones were removed and remodeled. Next, the frontal bones were fixed loosely to the supraorbital bones with absorbable threads. Then, the supraorbital and temporal bones were connected using distraction devices on both sides. The temporal bones were thereafter reinforced with titanium plates. Distraction was started one week postoperation, and the mean amount of elongation was 28.9 mm. Distraction devices were removed one to five months after the operation. One case required FOA by the traditional method ten months after the initial operation. Local infection was observed in three cases, but there were no majors complications. Posterior cranial remodelings were performed in five cases, with one requiring a second operation. We chose the appropriate procedure according to the degree of cranial deformity and operative findings. We performed decompression of the foramen magnum in five cases and laminectomy of the atlas in two cases. Ventriculoperitoneal shunt for hydrocephalus and tracheotomy for airway obstruction were performed in all cases. Cranial remodeling for treating severe craniofacial abnormality requires careful inspection of abnormalities, proper timing, close attention to the procedure and adequate perioperative care. Multidisciplinary therapy is essential for treating severe syndromic craniosynostosis due to systematic osseocartilaginous aplasia.

[Long-term complications of esophageal atresia].

Esophageal atresia (EA) is a rather common neonatal anomaly frequently associated with other congenital anomalies such as cardiac, genitourinary, and anorectal malformations and chromosomal disorders. It is suggested that the abnormal pattern of expression of the gene Sonic hedgehog is associated with failure of tracheoesophageal separation resulting in EA in an animal model. EA with distal tracheoesophageal fistula (TEF) is the most common type of EA and is usually treated successfully with division of the TEF and primary anastomosis. However, the optimal surgical treatment for long-gap EA remains controversial. Even though the results of delayed anastomosis with the Lividitis method or Collis-Nissen method are mainly reported to be satisfactory, long-term results show a high incidence of gastroesophageal reflux (GER) and esophageal dysmotility. Replacement with the gastric tube, stomach, colon, or jejunum has been performed when the gap is too long to use the native esophagus. Anastomotic stricture, obstruction due to redundancy, and adhesion of substitutes were reported in long-term follow-up studies. Moreover, the risk of carcinogenesis in Barrett's metaplasia, which is associated with GER, must be taken into consideration. Thus, long-term follow-up into adulthood is warranted in patients with EA.

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.

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.

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.

Laparoscopic fenestration of a neonatally detected hepatic mesenchymal hamartoma.

We report a case of hepatic mesenchymal hamartoma that was detected neonatally and successfully treated by laparoscopic fenestration. A baby girl was referred to our hospital 9 days after birth with a cystic lesion in her liver. Screening abdominal ultrasonography revealed a sonolucent lesion measuring 65x43x40 mm in the right lobe of the liver with a small solid area. Magnetic resonance imaging depicted the lesion as hyperintense on both T1- and T2-weighted images with no enhancement. A laparoscopic biopsy and fenestration of the lesion was performed. Histologic examination of the cyst wall led to a diagnosis of hepatic mesenchymal hamartoma. The postoperative course was uneventful and the patient is doing well at 4 years' follow-up, with no recurrence.

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.

Vascular ring in an extremely low birth weight infant.

Vascular rings due to aortic arch anomalies can be a major cause of tracheoesophageal obstruction in infants. But there is no report of vascular ring in an extremely low birth weight (ELBW) infant. This is a report of an infant with birth weight of 560 g, who has not diagnosed vascular ring until 6 months of age because of asymptomatic process by prolonged tracheal intubation. The patient was treated for double aortic arch with tracheomalacia surgically by simple division of the atretic left arch and the ligamentum arteriosus, aortopexy and tracheostomy before reaching full recovery at 3 years of age. We reported our management of vascular rings in the ELBW infant. This case suggested that the difficulty of extubation in ELBW infants in spite of the improvement of respiratory condition should raise a suspicion of the tracheal compression by other lesions such as vascular rings.