Long-term T-tube stenting as definitive treatment of severe acquired subglottic stenosis in children.

PURPOSE: We analyzed our results of long-term T-tube stenting for severe acquired subglottic stenosis secondary to prolonged endotracheal intubation in a neonatal period. MATERIALS AND METHODS: Twenty children treated with T-tube stenting since 1999 were retrospectively analyzed. T-tube stenting consisted of anterior cricoid split and placing silicon T-tube as a stent for expanded subglottic lumen. RESULTS: Fourteen of 20 patients were treated as an initial operation and 6 patients as an additional operation after other failed procedures. Decannulation was achieved in 8 (57.1%) of 14 children in the initial operation group and in 4 (66.7%) of 6 patients in the additional operation group. The average duration of T-tube stenting was 16.1 months in the initial group and 65.8 months in the additional group. Four of 20 patients continued to require T-tube stenting. In the other 4 patients, T-tubes were switched to tracheostomy tubes. The voice quality improved gradually after decannulation. CONCLUSION: T-tube stenting for severe acquired subglottic stenosis is recommended as a best available treatment in postoperative quality of life. T-tube stenting should be performed as the initial treatment because the time to decannulation was shorter than as an additional operation.

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.

Congenital tracheal stenosis: the prognostic significance of associated cardiovascular anomalies and the optimal timing of surgical treatment.

BACKGROUND/PURPOSE: Cardiovascular anomalies (CA) are frequently associated with congenital tracheal stenosis (CTS), but their prognostic impact on CTS and the optimal timing of surgical treatment remain uncertain. The aim of this study was to explore the prognostic factors and the optimal timing of surgical treatment in CTS patients with CA. METHODS: After obtaining institutional review board approval, a retrospective review of 42 patients who underwent surgical repair of CTS between 1996 and 2006 was conducted. The patients were divided into 3 groups: CTS without CA (n = 10, group A), CTS with CA repaired simultaneously (n = 27, group B), and CTS with CA repaired in stages (n = 5, group C). Seven clinical characteristics, including gestational week and weight at birth, the age and body weight at operation, the length of tracheal stenosis (%), the duration of cardiopulmonary bypass (CPB) during surgery, and operation time were compared among the groups using analysis of variance, Fisher's Exact test, and Student's t test. RESULTS: Although no operative mortalities occurred in groups A and C, there were 3 early deaths and 1 late death in group B. The deaths occurred in cases with associated complex CA (critical pulmonary stenosis, tetralogy of Fallot with an absent pulmonary valve, right ventricular outflow block, and cor triatrium). The duration of CPB was significantly different between groups A and B (P = .017), and furthermore, CPB time was significantly longer in early death cases than in surviving cases in group B (318.3 +/- .71.1 vs 204.0 +/- 67.8 minutes; P = .012). CONCLUSIONS: Complex CA and long CPB duration would be prognostic factors for the outcome of surgical management for CTS and CA. Simultaneous reconstruction of CTS and simple CA appears to be a reasonable method of surgical intervention, but patients with long segment CTS with complex CA may still be difficult to cure using this strategy, and staged correction may be considered.

[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.

Completely isolated alimentary tract duplication in a neonate.

A rare case of a completely isolated, alimentary tract duplication cyst in a 27-day-old neonate is reported. The duplication cyst was detected on antenatal fetal ultrasound and magnetic resonance (MR) imaging at 27 weeks' gestational age. At surgery, the duplication cyst was in a retroperitoneal site with no apparent communication between the cyst and any portion of the alimentary tract. On histopathological examination, the diagnosis was a gastric duplication cyst. The patient's postoperative course was uneventful. There have been eight cases of completely isolated duplication reported in the literature, of which seven were detected during the prenatal or neonatal period. No previous report in the English literature has described the fetal MR imaging findings of this type of duplication cyst.

Pretransplant risk factors and optimal timing for living-related liver transplantation in biliary atresia: experience of one Japanese children's hospital and transplantation center.

BACKGROUND/PURPOSE: Although living-related liver transplantation (LRLT) is effective for patients with biliary atresia (BA) after a failed Kasai operation, the pretransplant factors affecting post-LRLT mortality and the optimal timing of the procedure remain unclear. METHOD: A retrospective review of 27 patients with BA after a failed Kasai operation (median age, 22 months; range, 6-237 months) who received LRLT from 1994 to 2005 was done. The clinical characteristics at the time of the pre-LRLT assessment of those who did and did not survive were compared. A simple regression analysis and receiver operating characteristic analysis were done to correlate the clinical data. RESULTS: Among the 27 patients, 4 patients died within 1 year post-LRLT. The significant factors affecting posttransplant death were hepatopulmonary syndrome (HPS), age at LRLT, and graft-to-recipient weight ratio. The arterial blood gas analysis of HPS patients showed that there was a significant negative correlation between the SaO(2) value on room air and the intrapulmonary shunt ratio. The receiver operating characteristic analysis of age at LRLT showed that the optimal cutoff point was 103 months of age. CONCLUSION: Older children with HPS or a lower graft-to-recipient weight ratio are not ideal candidates for LRLT. The correlation between the shunt ratio and SaO(2) suggests that HPS could be detected early using pulse oximetry.

Can biliary atresia be diagnosed by ultrasonography alone?

BACKGROUND/PURPOSE: The triangular cord sign (TCS) is a specific ultrasonographic finding, reflecting a fibrotic mass at the porta hepatis in biliary atresia (BA). We evaluated whether BA can be diagnosed by ultrasonography alone using 3 findings: TCS, gallbladder length (GBL), and gallbladder contractility (GBC). METHODS: Subjects comprised 85 infants (median age, 47 days; range, 4-144 days) with cholestatic jaundice who underwent ultrasonographic examination for diagnosis between May 1996 and June 2006. Medical records were reviewed with regard to TCS, GBL, and GBC. Positive findings for BA included TCS >or=3 mm, GBL <15 mm, and GBC <68% (for < 12 weeks) or <25% (for >or=12 weeks). RESULTS: Biliary atresia was diagnosed in 48 patients surgically, with other cholestatic diseases diagnosed in the remaining 37 patients. Triangular cord sign was positive in 41 of 48 infants with BA and negative in 35 of 37 infants without BA. The 7 patients with BA who displayed negative results for TCS displayed positive findings for GBL or GBC. Positive predictive value in the diagnosis of BA was 98% if positive TCS was combined with abnormal gallbladder findings, whereas negative predictive value in diagnosis of BA was 100% if negative TCS was combined with findings of a normal gallbladder. CONCLUSIONS: Biliary atresia can be accurately diagnosed by ultrasonography using the findings of TCS combined with GBL and GBC.

MCRA of an anastomotic stenosis after esophagoesophagostomy for long gap esophageal atresia: a case report.

A 25-month-old boy with long gap esophageal atresia developed severe esophageal stenosis refractory to balloon dilatations after definitive esophagoesophagostomy. At 33 months of age, the patient had a magnetic compression revision anastomosis, in which a pair of 2 cylindrical Samarium-cobalt rare-earth 320 mT (3200 G) magnets, 15 x 5 mm (diameter x thickness), were placed in the esophagus to compress the scar tissue of the anastomosis. The magnets were retrieved 34 days after the magnetic compression revision anastomosis procedure. For 3 months, the patient subsequently had balloon dilatations to prevent restenosis, and he has been able to eat normally by mouth without further balloon dilatations since then.

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.

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.

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.

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.

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.