INTRODUCTION:The mechanisms responsible for postoperative chylothorax in Congenital Diaphragmatic Hernia (CDH) patients remain unclear. The aim of the present study was to examine the clinical features of CDH that may contribute to an association with postoperative chylothorax.MATERIAL AND METHODS:198 neonates with CDH, in whom surgical repair of a diaphragmatic defect was performed between 1981 and 2008, were retrospectively studied. The patients were divided into 2 groups; patients with postoperative chylothorax (group I, n=11) and patients without postoperative chylothorax (group II, n=187). The clinical findings were compared between group I and group II to investigate potential predictive parameters for an association with chylothorax. Moreover, the clinical findings and treatments were evaluated in patients with chylothorax.RESULTS:11 of the 198 infants (5.5%) developed a chylothorax. Although the incidence of a prenatal diagnosis was slightly higher in group I, no relationship with other clinical features was found which would indicate the severity of CDH or the occurrence of postoperative chylothorax. Treatment for chylothorax was drainage alone in 2 cases, total parenteral nutrition with drainage in 8 infants and additional intrathoracic OK-432 infusion in 1 patient. No patients required surgical intervention for chylothorax. No recurrences were observed in this patient series.CONCLUSIONS:It was concluded that postoperative chylothorax is not rare in infants after CDH repair. However, no statistically significant predictive parameters for chylothorax were identified, except for the presence of a prenatal diagnosis.

A 3-year-old boy associated with Opitz syndrome including hypospadias and anorectal anomaly was transferred to Osaka University Hospital with orotracheal intubation because of respiratory distress after respiratory infection. He had laryngeal cleft diagnosed in the neonatal period because of symptoms including stridor and aspiration. After 2 attempts at extubation, tracheostomy was performed. Fiberscopic examination demonstrated obstruction of the airway from the vocal cord to subglottic space by inspired esophageal redundant mucosa. There was no improvement observed for 3 months. He underwent anterior repair of cleft, anterior laryngotracheoplasty with costal cartilage graft, closure of tracheostomy, and endoscopic Nissen fundoplication. The postoperative course during the subsequent year was uneventful without feeding or speaking disorders. Subglottic airway obstruction caused by inspired redundant mucosa is a rare complication in laryngeal cleft. Anterior laryngotracheoplasty and Nissen fundoplication should be considered for this complication, in addition to the usual procedure for cleft repair.

BACKGROUND/PURPOSE:Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established. METHODS:Thirty-three children with ages 4.1 +/- 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy. RESULTS:No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung. CONCLUSION:These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.

PURPOSE:Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea.METHOD:Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele. CASE 1: Apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO2 below 95% occupied only 1% of the total sleep time. CASE 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI.CONCLUSIONS:These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith-Wiedemann syndrome.

The use of memory metallic stents for the urinary tract in pediatric patients has not been reported. The authors report on 2 patients with urinary tract disorders who were successfully treated with a memory metallic stent. A thermoexpandable, nickel-titanium alloy stent was placed at the urethroureteral junction of a 4-year-old boy with ureteral stenosis associated with cloacal exstrophy for 18 months and at the urethra of a 2-year-old girl with ischuria after a repair of cloacal anomaly for 6 months. Temporary insertion of a memory metallic stent is a safe and effective alternative for organic stricture or functional obstruction of the urinary tract in pediatric patients.

Background/purpose The authors experienced 3 cases of persistent pulmonary hypertension of the neonate (PPHN) associated with duodenal atresia with paradoxical dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association. Methods The medical charts of the patients were reviewed retrospectively. Results Case 1 was a 2,862-g male infant with a prenatal diagnosis of duodenal atresia. The dilated intestine was identified as the distal blind end of the duodenum during duodeno- duodenostomy. PPHN developed after surgery, and the patient died despite treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Lung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment of pulmonary vessels. Case 2 was a 2,244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in death. Autopsy results showed ACD with misalignment of pulmonary vessels and duodenal atresia associated with dilatation of the distal blind end. Case 3 was a 2,462-g female infant with a prenatal diagnosis of duodenal atresia associated with dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully under combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrasonographic findings showed enlarged echogenic fetal lungs as in the first 2 cases. Conclusions The characteristics of this novel association are enlarged echogenic fetal lungs, duodenal atresia associated with paradoxical dilatation of the distal blind end, and refractory PPHN resistant to iNO.

Background/purpose This study aimed at identifying characteristic features indicating congenital cystic adenomatoid malformation of the lung (CCAM) and evaluating the outcome predictors to identify prenatally subgroups of fetuses with significantly different probabilities of mortality or severe respiratory difficulty. Methods Twenty-eight neonates who had undergone antenatal evaluation for cystic lung disease (CLD) were reviewed retrospectively. The patients were divided into 3 groups according to the severity of their clinical course; mild (n = 7), moderate (n = 13), and severe (n = 8). Ultrasonographic findings in the fetus and their pulmonary lesion were evaluated. The normal lung to thorax transverse area ratio (L/T) was measured by ultrasonography. Results High echogenicity of the lesion throughout pregnancy and polyhydramnios were frequently seen in CCAM. All of the patients with other CLD showed isoechogenicity at the end of pregnancy. All patients in the severe group had both polyhydramnios and fetal hydrops. L/T was increased in mild and moderate groups, whereas no patient in the severe group had an increase in L/T at the final measurement. Each value of final L/T in the severe group was less than 0.25. Conclusions The subgroup of fetuses with an increased probability of mortality or severe respiratory difficulty could be predicted from the combination of polyhydramnios, fetal hydrops, and a final L/T value of less than 0.25.

The authors report a case of hepatic mesenchymal hamartoma diagnosed prenatally with ultrasound scan and magnetic resonance imaging (MRI) and confirmed histologically postdelivery. The fetus had a multicystic mass in the left upper abdomen, which showed a rapid enlargement accompanying maternal hypertension and preterm labor. The patient was delivered by cesarian section at 30 weeks and 5 days weighing 1,190 g, and, at birth, a large abdominal mass and severe anemia were noted. Surgical resection and neonatal management were successful, and the patient is alive in a good condition after 3 years follow-up. Although histologically benign, because this lesion frequently results in perinatal complications such as fetal hydrops, maternal toxemia, and preterm labor, early careful fetal ultrasonography should improve the prognosis of this lesion.

Congenital bronchial atresia is an anomaly characterized by a mucocele associated with a blindly terminating segmental or lobar bronchus. We report on a case of congenital atresia of the left superior segmental bronchus (B6) followed by fetal ultrasonography, which showed a large simple cyst with a surrounding small echogenic area. A large cyst and persistent lung fluid in the affected segment were noted in the early neonatal period. (C) 2003 Wiley-Liss, Inc.

We report 2 cases of umbilical cord ulcer associated with fetal jejunal atresia. Both of them developed a severe intrauterine hemorrhage, followed by fetal heart rate decelerations, and underwent emergency cesarean section. Bloody amniotic fluid and umbilical cord ulcers were observed in both cases. Although both cases were successfully resuscitated, neurological impairment and renal failure developed in 1 case due to prolonged asphyxia. In a review of the literature, umbilical cord ulcer was associated only with congenital duodenal atresia or jejunal atresia, but not with ileal atresia. Although the prenatal diagnosis of duodenal or upper jejunal atresia has been established, the prenatal diagnosis of this complication has not been reported. In such cases, detailed examination of the umbilical cord by ultrasonography may be useful for the prenatal diagnosis of this disease.

Although prenatal ultrasonographic (US) diagnosis has been reported in biliary atresia (BA), most cases are type I (correctable with cystic dilatation). We report three prenatal cases of type IIId BA (uncorrectable with cystic dilatation). Routine fetal US at 22 to 24 weeks of gestation showed two communicating cystic lesions 12 to 16 mm in diameter. On color Doppler US, the lesions were separate from the portal vein or hepatic artery. The size did not change during the prenatal period in any case. Choledochal cyst (CC) was considered the most likely diagnosis, although BA with cystic lesions was also considered. After birth, the patients developed acholic stools and prolonged neonatal jaundice. Hepatobiliary scintigraphy showed negative passage. Duodenal fluid showed a negative or slightly positive Gmelin test. The neonates underwent laparotomy at the age of 36, 46, and 32 days, respectively. Intraoperative cholangiography showed the gallbladder and slightly-dilated common-bile duct without entering the proximal or distal bile ducts in all cases. They were classified as type IIId BA and underwent excision of the cystic lesions and dissection of the portal bile-duct remnants, followed by hepatic portoenterostomy. Case 1 showed persistent jaundice and finally underwent liver transplantation (LTx), case 2 became anicteric. Case 3 remained jaundiced and is to undergo LTx. In conclusion, type IIId BA may be one of the differential diagnoses when a cystic lesion is detected under the hepatic hilum by fetal US. However, prenatal diagnosis of BA is still difficult with respect to differentiation from a CC or type I BA. Early postnatal diagnosis followed by immediate treatment is important, especially in type IIId BA.

Background/Purpose: Endoscopic ultrasonography (EUS) is considered a potentially useful tool to investigate structural abnormalities of the esophagus in pediatric patients, as in adults. The aim of this study was to evaluate the usefulness of EUS for the diagnosis of congenital esophageal stenosis. Methods: High-frequency catheter probe EUS was performed under general anesthesia in 2 patients who had congenital esophageal stenosis. Results: A 4-year-old boy with anorectal anomaly showed tapered narrowing in the distal esophagus, which was not ameliorated with balloon dilatation. High-frequency catheter probe EUS showed hypertrophy of the muscular layer in the esophageal wall at the narrowed portion, but no images suggested the presence of tracheobronchial remnants. The histologic diagnosis of fibromuscular hypertrophy was confirmed at esophagoplasty. A 5-month-old boy with Gross C-type esophageal atresia and symptomatic gastroesophageal reflux showed tapered narrowing in the middle esophagus on esophagography. The symptoms of stenosis were not ameliorated by balloon dilatation performed 4 times. High-frequency catheter probe EUS showed hyperechoic lesions suggesting cartilage at the esophageal narrowing. The diagnosis of tracheobronchial remnants was confirmed by the finding of 2 pieces of cartilage in the specimen obtained at the time of esophageal resection. Conclusion: EUS can be applied to show structural abnormalities of the esophageal wall even in pediatric patients with congenital esophageal stenosis and is useful for planning the therapeutic strategy. J Pediatr Surg 37:1744-1746. Copyright 2002, Elsevier Science (USA). All rights reserved.

BACKGROUND/PURPOSE:Congenital tracheal stenosis is an uncommon, life-threatening condition. Recently, tracheoplasty using costal cartilage grafts to enlarge the lumen was used successfully in such cases. In this study, we evaluated postoperative changes of costal cartilage grafts after tracheoplasty. METHODS:Costal cartilage patch tracheoplasty was used for surgical correction of long-segment congenital tracheal stenosis in 18 infants. Six patients whose tracheal specimens were obtained at autopsy are included in this study. The mean age at the time of repair was 4.8 months, and the mean period after operation was 7.5 months. Cartilage graft survival and epithelialization of inner layer was evaluated in each case. RESULTS:The mean width and length of grafts was 6.3 x 35 mm at operation and 4.0 x 22 mm at autopsy. The graft size was diminished gradually after operation and was replaced completely by mature scar tissue 2 years after operation. However, diameter of the reconstructed site was not reduced. Reepithelialization of the graft site with ciliated columnar epithelium was found in every case. Chondrocyte numbers were reduced and remaining cartilage was rather eosinophilic, suggesting that the degenerative process was ongoing in the graft. CONCLUSIONS:The costal cartilage grafts established a functional tracheal lumen, and reepithelialization with ciliated columnar epithelium was found at the graft site. The costal cartilage grafts continues to be an important option as graft material for tracheal reconstruction in infants with long segment congenital tracheal stenosis.

BACKGROUND:Pectus excavatum is sometimes associated with tracheobronchomalacia, which usually manifests left mediastinal shift, atelectasis of the left lung, and recurrent pulmonary infection. Standard repair of pectus excavatum alone usually failed to improve symptoms.METHODS:Pexis of the great vessels and pericardium combined with the support of the lower sternum, using a contralateral costal cartilage graft following the standard Ravitch's repair of pectus excavatum, has been used in 6 children during the past 5 years. In addition to respiratory symptoms, diagnosis of tracheobronchomalacia was made by bronchoscopy using an ultrathin fiberscope.RESULTS:Using the described operative technique, an excellent cosmetic and functional result was obtained in 5 of 6 children. However, atelectasis of the left lower lobe and the narrowing of the left mainstem bronchus continued postoperatively in one patient, which required the insertion of the Palmaz stent in the left mainstem bronchus.CONCLUSION:This technique may help improve tracheobronchomalacia in patients with pectus excavatum and should be tried before the insertion of an internal stent.

Background/Purpose: Sacrococcygeal teratomas (SCT) diagnosed in utero have been reported to be large and associated with high perinatal mortality rate. However, operative management including timing of operation after birth, combined abdominal approach for devascularization, and the position of the patients during resection is not well established.Methods: A retrospective review of 14 patients with SCT between 1978 and 1999 was performed. To prevent massive bleeding during surgery, the authors used an abdominoperineal resection in the supine position after devascularization. The patients' clinical and sonographic characteristics, prenatal outcome, operative management, and postnatal outcomes were examined.Results: One fetus died in utero. Two patients died within a week, but no late death and no malignant degeneration were noted. A staged operation with devascularization was performed in 2 patients, and 1 death occurred. Surgical management was analyzed between survivors without massive bleeding at surgery (n = 9) and others (n = 4). A significant difference was observed in the subgroup of tumor resection with devascularization or supine position and that of early resection with devascularization or supine position.Conclusions: Early resection using the abdominoperineal approach supported by close antenatal sonography may be preferable for a favorable outcome. Resection in the supine position after devascularization may have advantages of respiratory management, cardiac resuscitation, and bleeding prevention.

Two cases of bilateral congenital diaphragmatic hernia (CDH) followed by fetal ultrasonography were described. Although many cases of CDH are diagnosed by fetal ultrasonography, it is difficult to diagnose bilateral CDH in utero, which is a relatively rare and fatal condition. Two fetuses were diagnosed as having left CDH associated with severe anomalies. However, a retrospective review of fetal ultrasonography indicated elevation of the liver in the right posterior chest. Both patients died shortly after surgical repair for left CDH despite the use of extracorporeal membrane oxygenation. Diagnosis of bilateral CDH by fetal ultrasonography and the evaluation of its prognosis were discussed.

Journal of Pediatric Gastroenterology and NutritionVolume 33, Issue 2 p. 202-205 Case Reports Congenital Mediastinal Cystic Abnormalities Detected In Utero: Report of Two Cases Hisayoshi Kawahara, Corresponding Author Hisayoshi Kawahara [email protected] Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanAddress correspondence and reprint requests to Dr. Hisayoshi Kawahara, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka-Suita, Osaka 565-0871, Japan (e-mail: [email protected]).Search for more papers by this authorShinkichi Kamata, Shinkichi Kamata Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorKeisuke Nose, Keisuke Nose Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorTakaharu Oue, Takaharu Oue Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorAkira Okada, Akira Okada Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorKenshi Wasada, Kenshi Wasada Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorYuji Murata, Yuji Murata Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this author Hisayoshi Kawahara, Corresponding Author Hisayoshi Kawahara [email protected] Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanAddress correspondence and reprint requests to Dr. Hisayoshi Kawahara, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka-Suita, Osaka 565-0871, Japan (e-mail: [email protected]).Search for more papers by this authorShinkichi Kamata, Shinkichi Kamata Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorKeisuke Nose, Keisuke Nose Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorTakaharu Oue, Takaharu Oue Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorAkira Okada, Akira Okada Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorKenshi Wasada, Kenshi Wasada Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this authorYuji Murata, Yuji Murata Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine, Osaka, JapanSearch for more papers by this author First published: 01 August 2001 https://doi.org/10.1002/j.1536-4801.2001.tb07438.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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Purpose: Congenital diaphragmatic hernias (CDH) sometimes are associated with airway anomalies such as congenital stenosis, abnormal branching of the bronchi, and pulmonary hypoplasia. The incidence of these associated airway anomalies has not been reported previously. Methods: Bronchoscopy was performed in all neonates with CDH from 1987 to 1999. In addition to anatomic anomalies, bronchial hypoplasia was defined as narrowing and shortening of the bronchi at bronchoscopy. Results: Anatomic anomalies were identified in 7 of 39 patients with CDH: 1 had congenital tracheal stenosis with pulmonary artery sling, 1 had a defect of the right upper lobe bronchus, 2 had a tracheal bronchus, and 3 had a trifurcated trachea. Bronchial hypoplasia on the affected side was identified in 15 patients and was seen in all patients with anatomic anomalies of the tracheobronchial tree except the 2 with tracheal bronchus. After excluding 5 patients with severe associated anomalies, 6 of 14 patients with an abnormal tracheobronchial tree died, whereas 1 of 20 patients without airway abnormalities died. Conclusions: Anatomic anomalies of the tracheobronchial tree and bronchial hypoplasia on the affected side were identified in 17.9% and 38.4% of patients with CDH, respectively. CDH patients who exhibited these abnormalities showed a poor outcome. J Pediatr Surg 35:1562-1565. Copyright © 2000 by W.B. Saunders Company.

BACKGROUND/PURPOSE To define the clinical role of laparoscopic Nissen fundoplication (LNF) in children with gastroesophageal reflux (GER), an appropriate understanding of its functional effects is required. The aim of this study was to investigate the motor function of the esophageal body and the lower esophageal sphincter (LES) with special reference to the effects of caloric nutrients in children undergoing LNF. METHODS Studies were performed in 12 children with GER (age, 6 months to 13 years) before and a month after LNF. Continuous manometric examination was performed with an infusion system using a sleeve sensor for an hour each before and after the administration of apple juice (AAJ; 10 mL/kg). RESULTS AAJ increased postoperative basal LES pressure from 15 +/- 7 to 20 +/- 6 mm Hg, whereas it decreased the preoperative values from 13 +/- 5 to 10 +/- 4 mm Hg (P < .05). Significant residual pressure was noted at the nadir of swallow-induced LES relaxation after LNF, which was increased by AAJ from 7 +/- 3 to 11 +/- 4 mm Hg. A child with a high nadir LES pressure showed postoperative dysphagia. Significant changes in the patterns of esophageal contractions were not noted after LNF. CONCLUSIONS Characteristics of the effect of LNF on the LES were a postprandial increase of basal LES pressure and significant residual LES pressure at the nadir of LES relaxation. The motor function of the esophageal body was not affected by LNF.

Venous drainage to the portal vein in pulmonary sequestration is rare. A 7-month-old girl was referred to our hospital following surgery for ventricular septal defect because of a left upper abdominal mass with a large feeding artery from the abdominal aorta and venous drainage to the portal vein. She had had frequent pulmonary infections and was growth retarded. MRI demonstrated that the mass was above the left diaphragm, suggesting extralobar sequestration. An extralobar sequestered lung was resected at thoracotomy. Diagnostic problems and clinical features are presented.