Problems during and after pregnancy in female patients with biliary atresia.

BACKGROUND/PURPOSE: Advances in the management for biliary atresia (BA) have improved the prognosis and has greatly increased the number of long-term survivors. Even the long-term survivors, however, still face some problems. This retrospective review was performed to assess pregnancy-associated issues in long-term survivors after surgery for BA. MATERIALS AND METHODS: Of 55 patients with BA surviving for 16 years or more without liver transplantation, 9 patients have experienced pregnancy and delivery. Clinical courses, the outcome of pregnancy and delivery, and current statuses were retrospectively evaluated from their clinical records. RESULTS: The study revealed 14 pregnancies and 11 deliveries. Before pregnancy, no patient showed visible jaundice, but 6 patients had some complications such as episodes of cholangitis and portal hypertension. Two of the patients had both conditions and 2 others developed visible jaundice after pregnancy. One intrauterine fetal death occurred. CONCLUSIONS: Our retrospective review suggests that the previously mentioned conditions can be risk factor for cholangitis and gastrointestinal bleeding during or after pregnancy but are not considered to be contraindications for pregnancy and delivery. Complications can occur with pregnancy even during the normal course. Thus, careful observation is recommended.

Long-term outcome in type I biliary atresia.

PURPOSE: This retrospective study reviews the long-term outcome of type I biliary atresia (BA). METHODS: Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated. RESULTS: The overall survival rate of the nontransplant type I patients was better than that of the type II/III patients (52% vs 33%, P = .0009). Cholangiograms of 32 patients were classified into 3 types: cloudy (48%), treelike (13%), and mixed (39%). Of 26 patients who underwent corrective surgery in 1972 or later, 7 (50%), 7 (78%), and 3 (100%) patients of the cloudy type, mixed type, and treelike type, respectively, have survived without liver transplantation (LTx). Of 18 type I patients who survived more than 20 years without LTx, 7 developed severe late complications. Two of them eventually required LTx after 20 years old. CONCLUSIONS: Use of cholangiograms during corrective surgery might have a long-term prognostic value. The overall survival rate of type I BA was better than that of type II/III. The incidence of late complications was, however, considerably high in the type I survivors. All patients required careful long-term follow-up.

Concentrations of copper and zinc in liver and serum samples in biliary atresia patients at different stages of traditional surgeries.

Many patients with biliary atresia (BA) have impaired metabolism of copper (Cu) and zinc (Zn) because of the obstruction of bile ducts. An excessive Cu accumulation is cytotoxic and results in fibrosis in hepatic tissues. Since Zn works antagonistically to Cu, lower Zn concentrations may deteriorate liver damage. In the 1980's, we performed a series of surgeries on BA patients for the construction and alteration of the bile flow route, which is the major excretion route for Cu. We obtained liver and serum samples at each surgery, and measured Cu and Zn concentrations by inductively coupled plasma atomic emission spectrometry. Hepatic Cu concentration decreased with the improvement of cholestasis after the establishment of bile excretion. Conversely, when cholestasis persisted or recurred, increases in hepatic and serum Cu concentrations were noted. Hepatic Zn concentration was lower than previously reported normal values. High hepatic and serum Cu concentrations due to persistent or recurrent cholestasis and low hepatic Zn concentration may deteriorate hepatic fibrosis and liver cirrhosis.

Primary tracheal schwannoma (neurilemoma) in a 9-year-old girl.

The authors report a case of primary tracheal schwannoma (neurilemoma) causing symptoms of airway obstruction in a 9-year-old girl. Computerized tomography scan showed a polypoid intratracheal mass obstructing 73% of the lumen. Partial tracheal resection with primary anastomosis was performed. Histologic analysis showed a benign neurogenic tumor of Schwann cell origin.

Cholangitis as a late complication in long-term survivors after surgery for biliary atresia.

BACKGROUND/PURPOSE: A retrospective review was performed to assess the utility of diagnostic imaging (DI), efficacy of treatment, and outcome of late cholangitis in long-term survivors after surgery for biliary atresia. METHODS: Sixty-one patients surviving without liver transplantation (LTx) for more than 20 years were divided into 2 groups depending on whether cholangitis developed after age 20. Clinical factors including the type of obstruction, the age at the initial operation, and the early complication with cholangitis were compared between the 2 groups. DI such as computed tomography scan and magnetic resonance imaging, clinical courses after treatment of cholangitis, and current status of the patients were also evaluated. RESULTS: Thirteen patients had cholangitis after age 20. There was no statistical difference in the clinical factors studied between the 2 groups. Abnormal DI findings including dilatation of intrahepatic bile ducts and hepatic fibrosis were seen in 10 patients with late cholangitis. One patient died, and 2 ultimately underwent LTx. The remaining 10 patients including 4 with normal or near-normal liver function have survived without LTx. CONCLUSIONS: Although the majority of the patients had potential predisposing factors for cholangitis such as dilatation of intrahepatic bile ducts, a few patients unexpectedly had cholangitis without such abnormal findings after an excellent, long-term postoperative course.

Postoperative corticosteroid therapy for bile drainage in biliary atresia--a nationwide survey.

PURPOSE: Members of the Japanese Biliary Atresia Society were surveyed to determine their current practice regarding early use of corticosteroids after Kasai's operation. METHODS: Questions included the patient's background data, dosage, timing, complications, and outcome. Anicteric survival with the native liver was statistically compared between groups categorized by steroid dosage using Kaplan-Meier analysis. RESULTS: Among 54 institutions surveyed, a total of 222 patients with uncorrectable BA were collected from 34 responders, including 208 patients who received steroid therapy and 14 without it. Prednisolone was started during the first postoperative week in 31% and during the second week in 64%. Perforation and peritonitis occurred in 1 patient given 3 mg/kg/d of prednisolone on postoperative day 1. The survival rates of the steroid and nonsteroid groups were 58.0% and 35.7%, respectively (P = .052). The initial dose of prednisolone was or=4.0 mg/kg/d in 108 patients. The survival rates of the group receiving >or=4.0 mg/kg/d and the nonsteroid group were 58.9% and 35.7%, respectively (P = .0494). CONCLUSIONS: (1) Most surgeons use steroids. (2) Although the anicteric survival rate was higher in the steroid group, the number of patients in the nonsteroid group was too small to draw conclusions. (3) The recommended initial steroid dose is >or=4.0 mg/kg/d. (4) Complications are uncommon if administration is not started too early.

Biliary atresia with associated complicated anorectal and urogenital malformations.

We report on a girl with biliary atresia (BA) who also suffered with anorectal agenesis without fistula and complicated urogenital malformation. The outcome of patients with these severe anomalies is poor, but she has survived without liver and/or renal transplantation for more than 3 years. A careful treatment plan for each anomaly in addition to prevention of cholangitis and urinary tract infection is indispensable for managing these complicated anomalies.

Development of an implantable artificial anal sphincter using a shape memory alloy.

BACKGROUND/PURPOSE: The authors created a new artificial anal sphincter using a shape memory alloy (AS-SMA) to treat fecal incontinence and evaluated its validity. METHODS: AS-SMA consists of 2 Ti-Ni plates to sandwich the intestine and generates a pressure of 55 mm Hg at its resting position. With the electric power supply, the 2 metals bend to form an almondlike shape making a maximum gap of 33 mm between each other at the temperature of 55 degrees C. The device was attached to the colostomy in a piglet and was operated several times a day for 1 month. Fecal continence, bowel movements, and general condition of the piglet were recorded. After the experiment, tissue damage around the device was investigated. RESULTS: Fecal continence was obtained while the device was in the resting position. When it was operated, bowel movements were observed. The bowel movements to operations ratio was 82:105 (78%). During the experiment, the animal had neither abdominal distension nor vomiting. At the autopsy, there was mild inflammation and shallow burns around the device. No compression injury was detected. CONCLUSIONS: AS-SMA achieved fecal continence of the colostomy. With reduction of the associated side effects, it would be a potential substitute for an impaired anal sphincter.

Long-term efficacy of partial splenic embolization in children.

PURPOSE: To elucidate the role of partial splenic embolization (PSE) procedures, long-term outcome was assessed in terms of the recurrence of thrombocytopenia. METHODS: A retrospective study was performed after 41 PSE procedures in 36 patients for hypersplenism owing to portal hypertension. The underlying disease was biliary atresia in 32 patients, extrahepatic portal obstruction in 3, and idiopathic cirrhosis in 1. RESULTS: The average volume embolized was 70.1%. The patients were followed up from 20 days to 182 months (average, 70.8 months). Five patients subsequently died, and 6 underwent liver transplantation. The causes of death or the reasons for liver transplantation were not related to hypersplenism. Eleven patients (30.6%) had recurrence of thrombocytopenia (<100,000/mm3). There was no significant difference in the volume embolized or platelet count before PSE between the patients with and without recurrence of thrombocytopenia. The peak value of platelet count after PSE was significantly lower in the patients with recurrence of thrombocytopenia (P =.0091). In 17 of 24 survivors without liver transplantation, platelet counts remained normal throughout the follow-up period. CONCLUSIONS: PSE is a safe and effective procedure. Hematologic indices improved in all 36 patients after PSE, and its long-term efficacy was shown in 70% of the survivors.

Clinical significance of 99mTc-DTPA-galactosyl human serum albumin liver scintigraphy in follow-up patients with biliary atresia.

PURPOSE: Technetium 99m DTPA-galactosyl human serum albumin (GSA) liver scintigraphy was performed in follow-up patients with biliary atresia, and its clinical significance was investigated. METHODS: Between 1994 and 2001, GSA liver scintigraphy was performed 153 times in 57 follow-up patients. HH15, LHL15, and H/L15 (HH15/LHL15) were obtained. Patients were divided into 3 groups according to the clinical status (good, n = 17; fair, n = 24; poor, n = 16). The correlation between these parameters and liver function tests was examined. Twenty-six patients of the 57 underwent 3 serial GSA scintigraphies and also were divided into 3 groups (good, n = 13; fair, n = 8; poor, n = 5). (3rd/1st)H/L15 (3rd H/L15/1st H/L15) was obtained and compared. RESULTS: H/L15 had a correlation with serum albumin and serum cholinesterase. H/L15 was statistically different among 3 groups (good, 0.97 +/- 0.15; fair, 0.94 +/- 0.09; poor, 1.12 +/- 0.21; P <.05). Although most patients in the good (10 patients; 76.9%) and fair (7 patients; 87.5%) groups showed (3rd/1st)H/L15 of less than 1.1, 3 patients (60%) in the poor group showed (3rd/1st)H/L15 of more than 1.1. (3rd/1st)H/L15 in the poor group was significantly higher than those in good and fair groups (P <.05). CONCLUSIONS: Technetium 99m-GSA liver scintigraphy is useful to assess the functional hepatic reserve in follow-up patients with biliary atresia. Serial assessment with GSA scintigraphy can provide the trend of the patient's liver condition and can estimate the prognosis of the liver.

Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry.

PURPOSE: The aim of this study was to elucidate the epidemiology and short- and long-term results of biliary atresia in Japan analyzing the data of the Japanese Biliary Atresia Registry (JBAR). METHODS: In 1989, the Japanese Biliary Atresia Society started a nationwide registry, JBAR, to investigate all aspects of biliary atresia. A total of 1,381 patients, 863 girls, 507 boys, and 11 unknown, were registered between 1989 and 1999. JBAR includes an initial and follow-up questionnaires. Using these patients' data, the incidence, sex distribution, associated anomalies, the type of obstruction, the type of operation, and the surgical results were evaluated. The 5- and the 10-year results of 735 patients who were registered initially in or before 1994 also were analyzed. RESULTS: The incidence of biliary atresia was 1 in 9,640 live births. One hundred sixty-four patients (11.9%) had type I atresia of the common bile duct, 34 (2.5%) had type II atresia of the hepatic ducts, and 1,162 (84.1%) had type III atresia at the porta hepatis. Congenital associated anomalies were found in 19.6% of the patients including 33 cases associated with polysplenia. Impact of the age at operation on bile flow was not clear until 90 days of age, and after 90 days the bile flow rate worsened. The original Roux-en-Y procedure had been used in more than 50% of the patients since 1995. In 1999, 96% of the patients underwent the original Roux-en-Y procedure or the Roux-en-Y with an intestinal valve, and only 3 patients (3.5%) underwent other modifications. There were no significant differences in either the rate of disappearance of jaundice or the incidence of cholangitis among these 3 procedures. Of the 735 patients registered in or before 1994, 19 patients (2.6%) were lost to follow-up. The 5-year survival rates of patients registered in 1989, 1990, 1991, 1992, 1993, and 1994 were 62%, 64.5%, 61.3%, 59.0%, 58.7%, and 52.7% without liver transplantation (LTx), and 69.4%, 74.2%, 75.2%, 79.5%, 78%, and 78.3% with LTx, respectively. Although the overall 5-year survival rate changed from 69.4% to 78.3%, the difference was not statistically significant. According to the 10-year follow-up results of the 108 patients initially registered in 1989, 72 (66.7%) and 57 (52.8%) survived with and without the aid of LTx, respectively. CONCLUSIONS: The overall 5- and 10-year survival rates were 75.3% (553 of 734) and 66.7% (72 of 108), respectively. In spite of the increasing number of survivors after LTx, there was no significant improvement in the 5-year survival rate. It was shown that the JBAR system was functioning well with only 19 patients lost to follow-up among the 743 patients registered from 1989 to 1994.

A newly developed small-caliber videoduodenoscope for endoscopic retrograde cholangiopancreatography in children.

BACKGROUND: In neonates and younger children, the procedure of endoscopic retrograde cholangiopancreatography (ERCP) is now optimally performed using a small-caliber fiberoptic duodenoscope. However, there have been no reports on a specially designed videoduodenoscope for pediatric ERCP. GOAL: To evaluate performance and clinical usefulness of a newly developed small-caliber videoduodenoscope for ERCP in children. STUDY: We have developed a side-viewing videoduodenoscope with a 8.0-mm outside diameter of the tip and a 2.0-mm diameter instrument channel (Olympus PJF-240). In a total of 10 pediatric patients aged 11 months to 15 years (mean age, 6.1 years) who underwent ERCP using this videoduodenoscope, its feasibility was evaluated. Clinical diagnoses included pancreatitis (n = 4), choledochal cyst (n = 3), sclerosing cholangitis (n = 1), cholecystitis (n = 1), and choledocholithiasis (n = 1). In all patients, ERCP was done in the standard fashion under general anesthesia. RESULTS: ERCP was successful in 9 of 10 patients (90%). In the procedure, entering the descending duodenum was smooth and positioning the papilla on the endoscopic image was proper. In an 11-month-old infant, extraction of biliary stones by endoscopic balloon dilation was successfully done. Post-ERCP complications, including a significant increase of serum amylase levels, were not reported in any patients. CONCLUSION: This new videoduodenoscope has a potential for ERCP in all age groups of children.

The functional roles of porcine CD80 molecule and its ability to stimulate and regulate human anti-pig cellular response.

BACKGROUND: The pig is currently considered to be the most likely candidate for a xenogenic-organ source. Anti-pig human T-cell response via co-stimulatory molecules has been studied with great interest. The soluble form of porcine CD80 has recently been cloned and characterized, but the sequence of the transmembrane form has not been determined. The purpose of this study was to investigate the functional interaction between porcine CD80 and human T cells using the full-length clone of porcine CD80. MATERIALS AND METHODS: Specific complementary DNA (cDNA) clones encoding porcine CD80 were isolated and sequenced using rapid amplification of cDNA ends-polymerase chain reaction. Polymerase chain reaction-amplified cDNA coding for the open reading frame of the porcine CD80 transmembrane form was subcloned into an expression vector and then transfected into Chinese hamster ovary (CHO) cells. CHO cells transfected with porcine CD80 (CHO-pCD80) were co-cultured with human CD4+ T cells and then interleukin-2 secretion was measured and transferred pCD80 expression in these human T cells was detected by flow cytometry. RESULTS: We cloned and determined the complete nucleotide sequence for the transmembrane form of porcine CD80. Results from our T-cell co-stimulatory assay showed significant interleukin-2 production when co-stimulated with CHO-pCD80. Human naïve CD4+ T cells acquired xenogenic pCD80 molecules in the process of T-cell activation. CONCLUSIONS: Findings from this study seem to suggest that pCD80 has the functional ability to regulate human anti-pig cellular response. In addition, genetic manipulation of porcine co-stimulatory molecules offers a potentially new therapeutic strategy to prevent xenogeneic rejection across species.

[Gastroesophageal reflux in neurologically impaired children].

We present here the indication, procedure and results of surgical treatment of gastroesophageal reflux (GER) for neurologically impaired children. We decide its indication based on clinical symptoms and findings of upper GI series, esophageal pH monitoring and GI fiberscopy, respecting the QOL of the patient and family. Laparoscopic fundoplication has become the first choice in surgical treatment of GER because of its good results. However, an anti-reflux procedure is not effective in patients with intractable aspiration. A laryngotracheal separation procedure should be applied for these cases.

Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases.

PURPOSE: The aim of this study was to review 14 patients with congenital esophageal stenosis (CES) from diagnostic and therapeutic points of view. METHODS: From 1976 to 1999, 14 patients with CES were treated at the authors' hospital. In each diagnostic examination, an esophagogram, 24-hour pH monitoring, a manometric study, and an endoscopy were performed. The medical, radiologic, and operative reports of these patients were reviewed. RESULTS: Esophagogram results showed an abrupt stenosis in 5 patients and a tapered stenosis in 9. After 24-hour pH monitoring and an endoscopy, we were able to rule out the possibility of gastroesophageal reflux with esophagitis in most of the patients. Of the 11 patients who initially underwent bougienage or balloon dilatation, 8 of them had recurrent stenosis. Eleven patients required surgical treatment, including the 8 who had recurrent stenosis after undergoing dilatation. Nine of the patients underwent a resection of the stenotic segment and esophago-esophagostomy. An antireflux procedure was performed on 2 patients. Another patient had an esophageal rupture, which required a jejunal interposition. A histopathologic study showed that tracheobronchial remnants were present in 6 patients, and fibromuscular thickening was found in 4 patients. CONCLUSIONS: The efficacy of dilatation seems to be limited and may even result in severe complications such as an esophageal rupture. In this study, most patients ultimately required surgical treatment. Surgical repair for CES is the authors' preferred treatment, although initial dilatation may be effective for some patients.

Serum insulinlike growth factor-I in biliary atresia.

BACKGROUND/PURPOSE: Low level of Insulinlike growth factor-I (IGF-I) has been reported in children with chronic liver disease like biliary atresia (BA) awaiting liver transplantation. However, there has been no report on IGF-I in BA managed without liver transplantation. METHODS: The authors measured IGF-I and growth hormone (GH) in 21 postoperative BA, and 17 choledochal cysts (CC) as a control with normal liver function. To avoid an influence of aging, IGF-I was analyzed after converting them into a newly defined index "IGF%." IGF% is proportional to the lower limit of the value of IGF-I in gender- and age-matched normal control previously reported in literature. RESULTS: IGF% in BA was significantly lower than that in CC. IGF% tended to be lower in Kasai's type III (atresia at the porta hepatis) and higher in the jaundice-free group. IGF% in patients with esophageal varices was significantly lower. The correlation between choline esterase and IGF% was positive and that for TTT and IGF% was negative. CONCLUSIONS: Low level of IGF-I is a characteristic finding in BA, especially in patients without need of liver transplantation. And it may reflect the severity of pathologic changes (ie, hepatic fibrosis and reduced volume of normally functioning liver) in BA liver.

[Problems and reformation of the board of medical specialties in Japan].

The Board of Medical Specialties, which is closely related to postgraduate training in Japan, including the Board of Surgery, has serious defects in its system because it has developed on its own initiative and has been administered by a medical society. Many of the public are not aware of its existence, and its workings are difficult for them to understand. In the past 20 years, the Organization for the Board of Medical Societies (tentative English translation) has aimed to ensure sound development of the Board of Medical Specialties. Along with the activities of this organization, the Japan Surgical Society also decided to improve and reform its Board of Surgery and the boards of its subspecialties. The management, examination, and evaluation of the board system should be under the control of a third party separate from the medical societies. The ideal Board of Medical Specialties should not only foster gifted physicians but also benefit the public, communities, and medical societies.