The preliminary study of modified Swenson procedure in Hirschsprung disease.

PURPOSE: We have been using the Swenson procedure for more than 3 decades for Hirschsprung disease (HD). Recently, we modified this procedure, leaving the anterior wall below the peritoneal reflection undissected (mSwen). In 2000, we introduced mSwen with laparoscopic guidance (LapmSwen). We hypothesized that (1) omitting anterior wall dissection would not affect postoperative anorectal function, and (2) reduced dissection with better visualization via laparoscopy would reduce operative risks. METHOD: Charts of 89 patients with Hirschsprung disease operated on between 1990 through 2005 were retrospectively reviewed. Comparisons between Swen and mSwen, as well as between mSwen and LapmSwen, were analyzed in terms of operating time, blood loss, and complications. RESULTS: Mean operating times (minutes) were 312 for Swen, 284 for mSwen (P = .152), and 302 for LapmSwen ( mSwen, P = .218). Mean blood loss (mL) were 64.8 for Swen, 60.3 for mSwen (P = .669), and 8.7 for LapmSwen (as compared to mSwen, P = .001). We noted leakage in 7 Swen, 2 mSwen, and no LapmSwen patients. There were no significant differences between Swen and mSwen, or between mSwen and LapmSwen, in the incidence of enterocolitis, constipation, and soiling. CONCLUSION: LapmSwen appeared to be comparable to the standard and modified Swenson procedures in most measures. Operative blood loss may be reduced in the LapmSwen approach.

Management of pulmonary artery sling associated with tracheal stenosis.

BACKGROUND: Pulmonary artery sling is often associated with congenital tracheal stenosis and intracardiac anomalies. This study examines the influence of concomitant anomalies and individual surgical procedures. METHODS: Between 1984 and 2006, 31 patients underwent surgical repair of pulmonary artery sling (median, 6 months; range, 29 days to 9 years). Twenty-eight of them underwent left pulmonary artery reimplantation and tracheoplasty, whereas the remaining 3 received only left pulmonary reimplantation. The first 4 patients with long segment tracheal stenosis were treated with a costal cartilage graft and the next 19 with slide tracheoplasty. Five patients underwent tracheal resection with end-to-end anastomosis. Associated cardiac anomalies in 10 patients were repaired concomitantly with left pulmonary artery reimplantation and tracheoplasty. RESULTS: Two patients died early of low-output syndrome (n = 1) and ventricular arrhythmia (n = 1). Follow-up was complete in all patients (median, 4.6 years) with 3 late deaths arising from residual tracheal stenosis or pulmonary hypertension. Two survivors with a costal cartilage graft remain free of respiratory problems. Of 16 survivors who underwent slide tracheoplasty, 9 who did not have tracheostomy are fully active and 7 had tracheostomy to treat tracheomalacia and granulation. Among 5 survivors of tracheal resection, 4 are doing well without tracheostomy. Echocardiography showed that the left pulmonary artery was patent in all assessed patients, but 1 required balloon arterioplasty. CONCLUSIONS: Left pulmonary reimplatation with simultaneous repair of tracheal stenosis and intracardiac anomalies results in low operative mortality and satisfactory left pulmonary artery patency. However, the management of younger infants with tracheoplasty for long segment stenosis involving carina or complex cardiac anomalies remains challenging.

Development of acquired tracheal stenosis in premature infants due to prolonged endotracheal ventilation: etiological considerations and surgical management.

The aim of this study was to determine the etiology and appropriate surgical treatment for acquired tracheal stenosis that developed in patients who had undergone prolonged endotracheal mechanical ventilation as premature neonates. During the period 2000-2004, four patients aged 1-16 years were referred for tracheal stenosis characterized by stridor, choking, and recurrent pulmonary infection. All patients had undergone endotracheal mechanical ventilation for 2-5 months for respiratory distress related to prematurity (gestational age 25-29 weeks, birth weight 648-1,222 g). During this period, methicillin-resistant Staphylococcus aureus was predominantly cultured from the trachea. All patients exhibited a stenotic lesion encompassing 30-37% of the entire tracheal length on spiral CT. On palpation and inspection of the trachea during surgery, however, the stenotic segment appeared to encompass over 50% of the entire trachea. The carina was intact. Three patients underwent slide tracheoplasty with a tracheal resection and the other underwent resection and end-to-end anastomosis. Of the three patients treated by slide tracheoplasty, two are currently free of respiratory symptoms. However, one patient in this group required secondary resection of the remaining stenotic lesion with end-to-end anastomosis. This patient is currently asymptomatic. The remaining patient who underwent a resection and end-to-end anastomosis is doing well. The resected specimen showed fibrosis and degeneration of tracheal cartilage. A combination of prematurity, prolonged endotracheal mechanical ventilation and tracheal infection seem to be responsible for tracheal inflammation and stenosis. When considering surgical procedures for acquired tracheal stenosis, resection and end-to-end anastomosis are desirable. However, slide tracheoplasty with a partial tracheal resection is indicated for the treatment of stenosis involving a long tracheal segment.

A case report of glial choristoma of the tongue.

Glial choristoma of the tongue is extremely rare. The authors report the case of a 9-day-old infant with a congenital lingual glial choristoma. Complete surgical excision was performed without postoperative complications or recurrence. The authors also present a review of the literature and outcome of this benign tumor.

Composite pheochromocytoma associated with adrenal neuroblastoma in an infant: a case report.

A 5-year-old boy presented with headache and hypertension. Screening abdominal ultrasonography showed a 6-cm-sized mass in the left adrenal gland. Laboratory data included urine vanillylmandelic acid/creatinine (208.3 microg/mg), urine homovanillic acid/creatinine (114.3 microg/mg), serum epinephrine (237 pg/mL), and serum norepinephrine (1842 pg/mL). Metaiodobenzyl guanidine I 123 scintigram, abdominal computed tomography, and abdominal magnetic resonance imaging revealed only left adrenal tumor with no metastasis. Left adrenalectomy was performed transperitoneally, and histopathological diagnosis was an adrenal composite pheochromocytoma associated with neuroblastoma. Immunoreactivity for vimentin was positive in pheochromocytoma, which was negative in neuroblastoma. After operation, the symptom of hypertension disappeared. We should examine the identification of composite pheochromocytoma by immunohistochemistry method in patients with neuroblastomatous elements complicated by severe hypertension, and it is important to carefully monitor urine vanillylmandelic acid and urine homovanillic acid in the examination tests and hypertension in the clinical symptoms, and follow up this patient because of some risk of recurrence and metastasis.

Glucocorticoid receptor alpha expression in the intrahepatic biliary epithelium and adjuvant steroid therapy in infants with biliary atresia.

BACKGROUND/PURPOSE: In patients with cirrhosis, proinflammatory cytokines increase progressively in relation to the severity of liver dysfunction. Proinflammatory cytokines regulate the expression of glucocorticoid receptors (GcRs). On the other hand, GcRs mediate the effects of glucocorticoid steroids on bile excretion in the biliary epithelium. Glucocorticoid receptors have 2 isoforms: a cytoplasmic glucocorticoid receptor alpha (GcRalpha) mediates their physiological effects, whereas a nuclear localized glucocorticoid receptor beta (GcRbeta) acts as a dominant negative inhibitor of GcRalpha activity. We examined the histology features of liver biopsy and the expression of GcRalpha in the intrahepatic biliary epithelium in infants with biliary atresia. PATIENTS/METHODS: The patients were divided into 2 groups: patients in group 1 (n = 17) had a total bilirubin level below 1.0 mg/dL at least once after surgery, whereas patients in group 2 (n = 14) has never had bilirubin level below 1.0 mg/dL postoperatively. Liver biopsies taken from 31 infants with biliary atresia at the time of hepatic portoenterostomy between 1988 and 2002 were examined for immunohistochemistry and histology with H&E staining. The degree of GcRalpha expression in the biliary epithelium was semiquantitatively analyzed using staining scores. The histology features of the liver biopsy were also semiquantitatively analyzed by using the same scores to evaluate the liver injury. Intravenous prednisolone dosage was started with 4 mg/kg per day and tapered by a half dose every 2 days. The protocol was orally repeated during admission until the stool became constantly cholic. Statistical analysis was performed using Mann-Whitney U test and Spearman correlation coefficient by rank. Significance is set at a 95% confidence interval (P < .05). RESULTS: There was a significant positive correlation between the liver histology and the GcRalpha scores in all patients with biliary atresia (P = .0128; r = 0.429). In group 1, there was a significant positive correlation between the GcRalpha expression scores and the total dose of prednisolone administered (P = .0063; r = 0.767). CONCLUSIONS: The increase and degree of GcRalpha expression were associated with the severity of liver injury and may correlate with the dose of prednisolone required to sustain bile flow after successful hepatic portoenterostomy.

Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis.

A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children's Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomalous bronchi arose from the lower portion of the esophagus and connected to the lower part of the ipsilateral lung. This anomaly involved the right lung in two patients, and the left lung in one patient. Tracheobronchial stenosis extended from the inlet of the thorax to the carina in one patient, and to the contralateral main stem bronchus in two patients. Surgical treatment included division of the esophageal bronchus and anastomosis of bronchus to the trachea in one patient. In the other patient, the ipsilateral lung was resected and the stenotic tracheobronchus was stented. The remaining patient underwent pneumonectomy of the ipsilateral lung. Details of this fatal anomaly and a discussion of appropriate surgical management are described herein.

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.

Efficacy of urinary sulfated bile acids for diagnosis of bacterial cholangitis in biliary atresia.

This study evaluated the efficacy of measuring urinary sulfated bile acids (USBA) for diagnosis of bacterial cholangitis in patients with biliary atresia. Eight infants with biliary atresia were recruited. The USBA level was measured when they were admitted to hospital with a fever of unknown origin. Clinical manifestations and laboratory data were reviewed. The standard USBA value for each patient (S-USBA) was defined as the level measured when they had no fever, and USBA ratio (R-USBA) was calculated as the USBA level during the febrile episode divided by the S-USBA. Then R-USBA values were compared between febrile episodes with and without cholangitis to assess the diagnostic ability of USBA. Twenty-three febrile episodes occurred in eight patients during a 15-month period. Nine episodes were diagnosed as being due to cholangitis, five were due to non-cholangitis, and nine were of undetermined origin. The R-USBA value ranged from 1.5 to 15.4 during cholangitis episodes and from 0.4 to 1.2 during non-cholangitis febrile episodes. When fever was of undetermined origin, R-USBA was found to be increased during some episodes and not in others. USBA increased immediately in patients with cholangitis. The measurement of USBA is a useful non-invasive test for cholangitis in patients with biliary atresia who had undergone Kasai's operation.

Multistaged esophageal elongation technique for long gap esophageal atresia: experience with 7 cases at a single institution.

BACKGROUND/PURPOSE: Esophageal reconstruction for long gap esophageal atresia (LGEA) is still controversial. We successfully managed 7 cases of patients with LGEA by doing staged elongation of the native esophagus and subsequent end-to-end anastomosis. The technique and efficacy of this procedure are evaluated. METHODS: During the last 10 years, 7 patients with LGEA (Gross type A, 5; B, 1; C, 1) underwent multiple extrathoracic esophageal elongations (ETEEs) of the upper esophagus and subsequent esophagoesophagostomy. Medical records were reviewed in regard to the number of ETEE before definitive esophageal reconstruction, interval between each ETEE, operation time, time before initiation of sham feeding, duration of hospital stay, and complications. RESULTS: The definitive esophageal reconstruction was successfully achieved without major complications in all patients after 2 to 4 stages of ETEE. The interval between each ETEE was 72 days on average. The average operation time was 98 minutes. The elongation was 1 to 3.5 cm during each session. Oral sham feeding was recommenced 4.1 days after each ETEE, and the hospital stay was 9.6 days on average. Gastroesophageal reflux occurred in all patients, requiring antireflux surgery. CONCLUSIONS: We conclude from our experience (a) that effective esophageal lengthening with preservation of the native esophagus was achieved with multiple ETEE in LGEA and (b) that this procedure allows oral sham feeding at home until esophageal reconstruction.

Thoracic neuroblastoma: outcome of incomplete resection.

The prognosis for thoracic neuroblastoma has been documented as good, but the reasons have not been elucidated. We reviewed our experience of patients with thoracic neuroblastoma who were treated over the past decade. Among 102 patients treated for neuroblastoma at our hospital between December 1987 and June 1997, 20 patients had thoracic neuroblastoma (stage 1: nine, stage 2: five, stage 3: three, stage 4: three). Tumor characteristics and survival rate were compared between thoracic and nonthoracic neuroblastoma. The surgical margin was positive in 13 of the 20 patients with thoracic neuroblastoma. However, local recurrence was observed in only one patient who later underwent complete resection. All patients survived 4-14 years of follow-up. Among those over 1 year old, thoracic neuroblastoma was detected at an earlier stage than in their nonthoracic counterparts (stages 1 and 2 vs. 3 and 4: 6/3 vs. 1/17, p=0.003), and the 5-year survival rate was better than in their nonthoracic counterparts (100% vs. 44.5%, p=0.015). The incidence of ganglioneuroblastoma was significantly higher in the thoracic group at the age of >1 year ( p=0.003). In six of nine patients from the thoracic group who were >1 year old, small areas of ganglioneuroma were identified in the tumor tissue. There was a stronger tendency for the maturation of neuroblastoma into ganglioneuroma in the thoracic group. Complete resection is not required for thoracic neuroblastoma regardless of the patient's age.

Transection of the innominate artery for tracheomalacia caused by persistent opisthotonus.

Patients with cerebral palsy often develop opisthotonus. The trachea may be pinched between the innominate artery and the cervical spine. This compartmentalized thoracic inlet results in severe tracheomalacia. We successfully released tracheal compression by transection of the innominate artery. In case 1, a 4-year-old girl with cerebral palsy and opisthotonus was admitted due to respiratory distress. Bronchoscopy revealed severe tracheomalacia 2 cm above the carina. An endotracheal stent was placed through a tracheostomy. Two months later, she developed tracheal bleeding and bronchoscopy demonstrated a trachea-innominate artery fistula. Magnetic resonance brain angiography showed the presence of Willis' circle, and transection of the innominate artery was justified. This was done through a low cervical skin incision. In case 2, a 6-year-old boy with cerebral palsy and opisthotonus had long-standing respiratory distress. Ventilatory support did not resolve the symptoms. The innominate artery was transected in the same fashion as in the first case. Case 1 has been free from respiratory distress for 4 months and case 2 for 3 years. Our experience suggests that the combination of tracheomalacia, opisthotonus causes severe respiratory distress. Transection of the innominate artery is a useful therapeutic strategy to release airway obstruction in this condition.

Supraumbilical pyloromyotomy: comparison between intracorporeal and extracorporeal approaches.

We evaluated intracorporeal pyloromyotomy (ICP) as an alternative to extracorporeal pyloromyotomy (ECP) in infants with hypertrophic pyloric stenosis. From July 1994 to June 2002, 75 patients underwent ICP, and 29 patients underwent ECP through supraumbilical incisions. The medical charts were reviewed retrospectively with regard to operating time, time to return to full feeding, complications, and outcome. The average operating time in the ICP group was significantly longer than in the ECP group (56.4+/-13.6 vs. 48.7+/-16.3 min; p=.004). However, the average time to return to full feeding in the ICP group was significantly shorter than in the ECP group (2.1+/-1.6 vs. 2.9+/-1.9 days; p=.002). Extension of the skin and/or fascial incision was necessary in two of 75 patients in the ICP group, compared with 11 of 29 patients in the ECP group ( p<.0001). There were four (5.3%) wound infections and one (1.3%) mucosal perforation in the ICP group, compared with two (6.9%) wound infections in the ECP group. ICP allows patients to return to full feeding earlier than ECP does, resulting in a shorter hospital stay. ICP through a supraumbilical incision is superior to the ECP technique.

Tracheoplasty for long segment congenital tracheal stenosis: analysis of 29 patients over two decades.

PURPOSE: The authors determined the long-term outcome of patients who underwent surgical repair of long-segment congenital tracheal stenosis (LCTS) and compared the quality of cartilage graft and slide tracheoplasty techniques. METHODS: Twenty-nine patients underwent surgical repair of LCTS at 29 days to 9 years of age. In 26 patients, more than 50% of the entire length of the trachea was involved. The early 12 patients (1981 through 1992) were treated with a costal cartilage graft for tracheal reconstruction. The remaining 17 patients (1997 through 2002) were treated with slide tracheoplasty. For both procedures, a median sternotomy was preferred because of convenience when using extracorporeal circulation and for simultaneous repair of associated cardiovascular anomalies. RESULTS: Of the 12 patients who underwent a costal cartilage graft, 8 survived (66%). Six of these patients are free of disease, but 2 have persistent airway problems. Three patients died of residual stenosis and 1 of unrelated disease. Of the 17 patients who underwent slide tracheoplasty, 13 survived (76%), and 10 are free of disease. Three patients continue to require a stent because of tracheomalacia and granulation. Two patients died of cardiac failure, 1 of granulation, and 1 of aspiration pneumonia. CONCLUSIONS: The authors challenged LCTS with a unique surgical approach and succeeded in making an incurable disease curable. When the cartilage graft technique is compared with slide tracheoplasty, however, the latter is preferable because it preserves native tracheal tissue with less frequent complications.

Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction.

OBJECTIVE: A retrospective review was performed to evaluate the importance of the "triangular cord" sign in comparison with gallbladder length and contraction for the diagnosis of biliary atresia in pediatric patients. MATERIALS AND METHODS: Fifty-five fasting infants with cholestatic jaundice were examined on sonography. The examinations focused on the visualization of the triangular cord sign and assessment of gallbladder length and contraction. The diagnosis of neonatal hepatitis or of other causes of infantile cholestasis was made if symptom resolution occurred during follow-up. RESULTS: A triangular cord sign was found in 27 of 29 infants with biliary atresia and in one of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 95%, sensitivity was 93%, and specificity was 96%. The gallbladder was thought to be abnormal if it was less than 1.5 cm long, was not detectable, or was detectable but had no lumen. The gallbladder was abnormal in 21 of 29 infants with biliary atresia, whereas it was abnormal in eight of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 71%, sensitivity was 72%, and specificity was 69%. The gallbladder was detectable on sonography in 13 infants with biliary atresia and 26 infants with neonatal hepatitis or other causes of infantile cholestasis. Gallbladder contraction was not confirmed in 11 of 13 infants with biliary atresia and seven of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 77%, sensitivity was 85%, and specificity was 73%. CONCLUSION: The triangular cord sign was a more useful sonographic finding for diagnosing biliary atresia than gallbladder length and contraction.

Laryngotracheal separation for intractable aspiration pneumonia in neurologically impaired children: experience with 11 cases.

PURPOSE: Laryngotracheal separation (LTS) with or without end-to-side laryngoesophagostomy was performed as an antiaspiration procedure for intractable aspiration pneumonia in 11 children. The effectiveness of LTS for preventing aspiration was investigated. METHODS: Eleven children aged from 9 months to 16 years with intractable aspiration pneumonia underwent LTS with (n = 8) or without (n = 3) laryngoesophagostomy at our institution over the last 2 years. Of these 11 patients, 7 underwent fundoplication with or without gastrostomy for gastroesophageal reflux (GER) before LTS (n = 5) or concurrently with LTS (n = 2). The effectiveness of LTS was evaluated by chart review and follow-up phone questionnaires. RESULTS: LTS decreased the frequency of performing suction from an average of once every 30 minutes to once every 4.5 hours in all patients. In 5 patients who underwent fundoplication with gastrostomy before LTS, aspiration pneumonia remained unless they underwent LTS. Two patients who underwent LTS with or without laryngoesophagostomy tolerated oral feeding postoperatively. All parents rated LTS as excellent or good in terms of improving the quality of life. CONCLUSIONS: LTS can be recommended for neurologically impaired children with intractable aspiration as a primary surgical intervention. If patients show impaired swallowing and GER, LTS could be performed simultaneously with fundoplication and gastrostomy.

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.

Urinary sulfated bile acids: a new simple urine test for cholestasis in infants and children.

BACKGROUND/PURPOSE: Measurement of urinary sulfated bile acid (USBA) has been reported as a simple urine test that reflects the degree of cholestasis. The authors report the diagnostic value of this new laboratory test in various cholestatic conditions affecting infants and children. METHODS: A urine sample was collected from 4 surgical neonates with parenteral nutrition-induced cholestasis and 48 patients with biliary atresia (BA). USBA was measured by direct enzymatic assay. RESULTS: In 3 patients receiving parenteral nutrition, USBA increased with caloric gains. For one surgical patient, a decrease in calories because of liver dysfunction resulted in a decrease of USBA, closely reflecting the fluctuations of caloric intake. In patients with BA, a significant positive correlation was noted between serum bile acid and USBA (r = 0.85; P <.01). Ten of 14 febrile episodes in 6 patients with liver dysfunction and increased C-reactive protein showed elevated USBA, thus diagnosed as cholangitis. Four febrile episodes caused by viral infection showed no elevation of USBA. CONCLUSIONS: USBA is a simple and sensitive noninvasive test reflecting the degree of cholestasis in infants and children. USBA correlated highly with serum bile acid levels and may be helpful in diagnosis of cholestasis caused by cholangitis without blood sampling.

Surgical management for intractable cholangitis in biliary atresia.

BACKGROUND/PURPOSE: The efficacy of antireflux surgical procedures involving the Roux-en-Y jejunal limb for cholangitis was evaluated retrospectively in patients with biliary atresia (BA). METHODS: From July 1993 to December 2001, 41 patients with BA underwent hepatic portojejunostomy with Roux-en-Y reconstruction. Of these patients, 11 had intractable cholangitis that was treated by creation of a value with or without lengthening of the Roux-en-Y limb. RESULTS: Among the 11 patients, the first episode of cholangitis occurred within 6 months after portojejunostomy in 10 patients and at the age of 4 years in one patient. Cholangitis developed at various intervals from once every week to once every 2 months requiring hospitalization each time. All patients underwent valve creation at 2 months to 5 years postoperatively, whereas 2 had an additional lengthening of the limb. Cholangitis resolved completely after surgery in all cases. Two patients underwent liver transplantation, and the third patient died of an unrelated cause. The 8 survivors with native livers are doing well after 1 to 8 years of follow-up. CONCLUSION: Early surgical intervention could control intractable cholangitis in all patients, both delaying the time of liver transplantation and improving the quality of life.