Scanning electron microscopy studies of the human lens capsule after capsulorhexis

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.     

Neonatal cholestasis syndrome: an appraisal at a tertiary center

OBJECTIVE: To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS). DESIGN: Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years. SETTING: Tertiary level referral gastroenterology center in North India. METHODS: Liver function tests, urine examination, serum antibodies against Cytomegalovirus (CMV), Rubella and Toxoplasma; abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy were done. In appropriate setting, laparotomy and surgical corrections were done for biliary tract disorders. RESULTS: NCS constituted 19% of pediatric liver diseases. Considerable delay in presentation was observed [mean delay, extrahepatic biliary atresia (EHBA) = 4 +/- 2.0 months, neonatal hepatitis (NH) = 2.2 +/- 1.3 months]. Thirty three (55%) infants had EHBA, 14 (23%) NH (4 CMV, 2 galactosemia, 1 urinary tract infection and 7 idiopathic), 2 (3%) paucity of intralobular bile ducts and 11 (18%) were of indeterminate etiology. Liver biopsy was the most accurate (96.4%) investigation in discriminating between EHBA and NH. Of the 18 operated infants with EHBA (portoenterostomy-15 and hepatico-jejunostomy-3), 10 were alive (mean follow up = 22.8 +/- 8.6 months) of which 4 were completely asymptomatic. CONCLUSIONS: (i) NCS is an important cause of liver disease in Indian children. (ii) It requires prompt referral, quick investigative approach and targeted management. (iii) Liver biopsy is highly accurate in differentiating EHBA and NH. (iv) infants with EHBA and compensated status of liver should undergo corrective surgery irrespective of age at presentation.     

A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis

BACKGROUND/PURPOSE: The authors evaluated prospectively the utility of ultrasonography, Tc-99m-DISIDA hepatobiliary scintigraphy, and liver needle biopsy in differentiating biliary atresia (BA) from intrahepatic cholestasis in 73 consecutive infants who had cholestasis. METHODS: Sixty three ultrasonographic examinations of 61 infants with 7.0-MHz transducer were carried out, focusing on the fibrous tissue at the porta hepatis. The authors defined the triangular cord (TC) as visualization of a triangular or tubular shaped echogenic density just cranial to the portal vein bifurcation on a transverse or longitudinal scan. RESULTS: Although 17 of 20 ultrasonographic examinations from infants who had BA denoted TC, 43 ultrasonographic examinations from infants with either neonatal hepatitis (NH) or other causes of cholestasis denoted no TC, showing a diagnostic accuracy of 95% with 85% sensitivity and 100% specificity. Investigation with Tc-99m-DISIDA hepatobiliary scintigraphy showed that 24 of 25 infants who had BA had no gut excretion, and 16 of 46 infants who had either NH or other causes of cholestasis had gut excretion, showing a diagnostic accuracy of 56% with 96% sensitivity and 35% specificity. Therefore, gut excretion of tracer excluded BA, but no gut excretion of tracer needed further investigations as liver needle biopsy. Forty-four liver needle biopsies were carried out in 19 infants who had BA and 24 infants who had either NH or other causes of cholestasis. Although 18 of 20 biopsy findings in infants who had BA were correctly interpreted as having BA, 23 of 24 biopsy results in infants who had either NH or other causes of cholestasis were correctly diagnosed, showing a diagnostic accuracy of 93% with 90% sensitivity and 96% specificity. CONCLUSIONS: Since the introduction of ultrasonographic TC sign in the diagnosis of BA by our institution, we have found that it seemed to be a simple, time-saving, highly reliable, and non-invasive tool in the diagnosis of BA from other causes of cholestasis. The authors propose a new diagnostic strategy in the evaluation of infantile cholestasis with emphasis on ultrasonographic TC sign as first priority of investigations. When the TC is visualized, prompt exploratory laparotomy is mandatory without further investigations. When the TC is not visualized, hepatobiliary scintigraphy is the next step. Excretion of tracer into the small bowel actually rules out BA. Liver needle biopsy is reserved only for the infants with no excretion of tracer. The authors believe that a correct decision regarding the need for surgery can be made in almost all cases with infantile cholestasis by this multidisciplinary approach.     

Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.     

Biliary atresia and biliary cysts

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

Three-dimensional magnetic resonance cholangiopancreatography for evaluation of obstructive jaundice

The purpose of this study was to assess the diagnostic value of magnetic resonance cholangiopancreatography (MRCP) in studying the anatomy, sites, and causes of obstructive jaundice. From September 1994 to May 1996 three-dimensional MRCP was performed on 31 patients with abdominal pain and obstructive jaundice with a fast spin-echo T2-weighted pulse sequence. The images were reconstructed using maximal intensity projection, AVERAGE and SURFACE algorithm processing techniques at a graphics workstation. All the reconstructed images were compared with those obtained using conventional cholangiographic techniques, such as endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography and drainage, and intraoperative cholangiography. The patients' diagnoses included choledochal cyst (13), cholangiocarcinoma (five), choledocholithiasis (four), pancreatic head carcinoma (three), rhabdomyosarcoma (one), papillary Vater carcinoma (one), recurrent gastric carcinoma (one), ascaris (one), and biliary atresia (two). Extrahepatic biliary dilatation was present in all 13 patients with choledochal cyst; the pancreatic ducts and their entrance level to the common bile duct were observed in eight of these patients. The level of obstruction in patients with cholangiocarcinoma was well documented but the biliary tract of one patient with biliary atresia was not identified by MRCP. In one patient with biliary rhabdomyosarcoma, MRCP clearly delineated the extrabiliary extension of the tumor. In a patient with ascaris in the common bile duct an increase in signal intensity inside the digestive tract of the worm denoted fluid in its gut. Lithiasis was shown in all of the four patients with choledocholithiasis. Thus, MRCP is a useful tool in the assessment of biliary tract obstruction and its causes, and is a valuable addition to ultrasonography.     

Anatomic anomalies in neonatal cholestatic jaundice

Disorders of the biliary tree are an important cause of cholestatic jaundice in infancy. For the most frequent diseases in this group, biliary atresia and choledochal cyst, prognosis is strongly dependent on timely diagnosis and treatment. In biliary atresia the bile flow is obstructed due to obliteration of the extrahepatic bile ducts. Construction of an hepatic portoenterostomy before 60 days of age will result in restoration of bile flow in the vast majority of patients. When failed, the disease is progressive and ultimately fatal, unless a liver transplantation is performed. For those patients in which restoration of the bile flow succeeds, the subsequent course is strongly dependent on the occurrence of cholangitis. For all patients fat-soluble vitamins should be supplemented and caloric intake should be carefully monitored. Presentation of a choledochal cyst can be either before or after the first year of life. It is mostly characterized by jaundice, with or without abdominal pain. Therapy consists of resection of the cyst, followed by a hepatico-jejunostomy. Paucity of bile ducts is an intrahepatic disorder, in which--almost--no bile ducts can be found in the portal tracts. This anomaly is frequently found in combination with a typical facies, a pulmonary stenosis and vertebral anomalies, a combination which is called Alagille syndrome. Prognosis is generally good.     

Is it possible to differentiate between choledochal cyst and congenital biliary atresia (type I cyst) by antenatal ultrasonography?

Both choledochal cyst and congenital biliary atresia (type I cyst) may share the same ultrasonographic pattern. We report 2 cases which were shown to have cystic structures on the upper abdomen by antenatal ultrasonography. The size of the choledochal cyst found at 24 weeks' gestation increased steadily as gestational age advanced. But the size of the cyst in congenital biliary atresia found at 29 weeks' gestation remained unchanged throughout the remaining pregnancy. Cyst enlargement, therefore, may suggest the possibility of a choledochal cyst.     

Prenatal diagnosis of biliary atresia associated with choledochal cyst

We report a case of biliary atresia promptly detected by its association with a choledochal cyst, prenatally diagnosed at 20 weeks' gestation. The baby was operated at 14 days of life. A choledochal cyst was found and fibrosis of the extrahepatic bile ducts was also noted. So, excision of the choledochal cyst and a Roux-en-Y porto-jejunostomy was done. Nine months later, despite an appropriate biliary drainage, echographic and histological changes compatible with liver cirrhosis have been detected. Including this one, six cases have been reported.     

Usefulness of liver biopsy in the study of the pediatric patient. Review of 145 cases

There were reviewed 145 cases of children in which hepatic biopsy was done at the Hospital Infantil del Estado de Sonora, from 1978 to 1990. The larger age group were infants and preschool children (74.3 percent) males being predominant; signs and symptoms were related with hepatic illness, as well as the admission diagnoses. The indication of biopsy was for confirmation of liver disease in more than 50 percent, 37.1 percent for unknown diagnoses and 20.6 percent to look for liver disease by a systemic illness. The most usual procedure was percutaneous biopsy with Vim-Silverman needle in 111 cases (76.5 percent), in 23 percent, the biopsy was done by major surgical method. Nine percent of the children needed open surgical method after percutaneous biopsy. The time from the admission to biopsy performance in patients with neonatal hepatitis vs biliary atresia was 14 days. In other type of illness the time was 25 days. The morbidity was 1 percent. There was no mortality. The histopathologic diagnosis of liver diseases was done in 96 cases (66.7 percent) by this method in 31 children (21.3 percent) with investigation of jaundice (neonatal hepatitis vs biliary atresia). The diagnostic mistake in tissues obtained by percutaneous needle, was statistically significant (p < 0.05). Average hospitalization stay was less than two months in 70 percent of the cases.     

Hyaline cartilage at porta hepatis in extrahepatic biliary atresia

Hyaline cartilage was found on microscopy of sections of the extrahepatic biliary tree in two infants with extrahepatic biliary atresia (EHBA). Respiratory epithelium was not present, and the cartilage did not seem to block the bile duct lumen. Hyperbilirubinemia was manifest in one infant on the second postnatal day, but clinical courses were otherwise unremarkable. In neither infant was the ductal plate malformation found on light microscopy of liver biopsy specimens, and in neither infant was visceral topography abnormal. Hyaline cartilage at the porta hepatis appears to be a novel finding in EHBA. Its significance remains to be defined.     

Mitchell osteotomy for adolescent hallux valgus

BACKGROUND: Complications often follow if a choledochal cyst is treated simply by drainage, either internal or external. This study reviews 17 patients who had had previous cystoenterostomy (n = 9) or external drainage (n = 8) and who required reoperation and cyst excision. METHODS: The study was a retrospective review including ten women and seven men managed over 9 years. The indications for reoperation were stone formation (10 patients), pancreatitis (three), portal hypertension (two) and hepatic abscess (one); two patients were asymptomatic. RESULTS: Definitive surgery with cyst excision was possible in all patients who had previously had external cyst drainage and in seven of nine who had had previous cystoenterostomy. There were no deaths. Two postoperative biliary leaks and two duodenal fistulas resolved spontaneously. CONCLUSION: Excision of a choledochal cyst is possible and desirable even after a previous drainage operation. In severely ill patients with a complication of choledochal cyst, external drainage may be a preferable initial manoeuvre.     

Direct enzymatic assay of urinary sulfated bile acids to replace serum bilirubin testing for selective screening of neonatal cholestasis

Direct enzymatic assay of urinary sulfated bile acids is a sensitive, rapid, minimally invasive, and convenient method of detecting cholestasis in young infants. It may replace measurement of serum direct bilirubin for selective screening for biliary atresia and neonatal hepatitis syndrome at 1 month of age.     

Gastrointestinal immune responses in HIV infected subjects

Biliary atresia (BA) is characterized by luminal obstruction of the extrahepatic bile duct with fibrous remnants. The authors reviewed ultrasonographic examinations of the fibrous tissue in the bifurcation of the portal vein at the porta hepatis and identified the triangular- or tubular-shaped echogenic density, the so-called "triangular cord" (TC), in the vicinity of the portal vein on a transverse or longitudinal scan. In this prospective study, the authors investigated whether TC was useful in the noninvasive diagnosis of biliary atresia in 18 infants who had persistent neonatal jaundice. This was done by comparing the ultrasonographic examination with the histopathologic examination (HPE) of liver specimens obtained from a needle biopsy. The TC was identified in nine patients, all of whom were confirmed to have BA by HPE. The TC was not observed in the other nine patients, who had neonatal hepatitis (NH). The mean size of the TC was 13 mm (wide) x 6 mm (thick) (width range, 5 to 21 mm; thickness range, 4 to 12 mm). The diagnosis of BA was confirmed at the time of Kasai hepatoportojejunostomy in eight of the nine patients whose TC was noted by ultrasonography (US). The other patient was discharged because his parents refused the operation; he died of liver failure at 15 months of age. The nine patients with absent TC were treated medically for NH. Eight of them improved clinically. The other, diagnosed to have NH by needle and wedge liver biopsies, was reexamined 40 days after the initial examination because of worsening jaundice. A 18 x 12-mm TC was visualized ultrasonographically. Additionally, a percutaneous liver biopsy specimen showed BA with severe portal fibrosis and ductal proliferation. The patient underwent a Kasai hepatoportoenterostomy. On the basis of these results, the authors conclude that TC is a very specific ultrasonographic finding, representing the fibrous cone at the porta hepatis, and is a useful tool in the noninvasive diagnosis of BA. However, early exploration or close US follow-up is recommended for any patient suspected of having BA clinically, even if a liver biopsy confirms the NH.     

Hypermethioninemia in the differential diagnosis of infantile obstructive jaundice (author's transl)

In 7 infants suffering from obstructive jaundice we found transient high levels of methionine in serum. All cases had only intrahepatic cholestasis, especially with intrahepatic biliary hypoplasia, whereas other patients with extrahepatic biliary atresia and/or combination of extra- and intrahepatic obstructive jaundice always showed normal levels of methionine. Therefore hypermethioninemia seems to be helpful in differentiating the various causes of infantile obstructive jaundice.     

Paediatric liver transplantation: a 10 year experience in Taiwan

Between March 1984 and August 1994, 13 orthotopic liver transplantations were performed in 13 patients < or = 25 years of age. The indications included Wilson's disease (n = 7), biliary atresia (n = 4), choledochal cyst (n = 1) and hepatitis C cirrhosis (n = 1). Technical variants included full-size (n = 11), reduced-size (n = 1) and living-related (n = 1) liver transplantation. These recent technical innovations have offered an expanded donor pool for earlier transplantation, shorter waiting times and excellent quality grafts. Surgical complications occurred in six patients; all required additional surgery. Biliary complications were encountered more commonly in our earlier patients. Our actuarial patient and graft survival rate is 92% at 2 years. The long-term follow-up of our liver-transplanted Wilson's disease patients provides confirmatory evidence that orthotopic liver transplantation cures the underlying metabolic defect with complete normalization of biochemical abnormalities of copper metabolism, reversal of neurological impairments and the disappearance of Kayser-Fleischer corneal rings. The high rate of patient survival and excellent rehabilitation indicate that with prudent clinical judgement, liver transplantation can be achieved with an acceptable rate of morbidity, mortality and cost in a setting where manpower and donor organs are very limited.     

Evidence of peripheral axonal neuropathy in primary restless legs syndrome

The aim of this study was to assess the risk and prognostic factors of gut perforation after orthotopic liver transplantation in children with biliary, atresia using univariate and stepwise regression analysis. Among 51 pediatric recipients who underwent transplantation because of biliary atresia after failure of portoenterostomy, 10 patients (20%) had 19 episodes of gut perforations after 14 transplantations. The median delay between transplantation and perforation was 13 days. These perforations were treated either by suture (n = 21) or ostomy (n = 11). The study of preoperative and perioperative variables showed that children with gut perforation were in surgery for a significantly longer period of time including a longer period of receiving hepatectomy and undergoing portal venous clamp. These children also needed large amounts of blood transfused during hepatectomy. After transplantation there was no difference regarding total steroid doses and early occurrence of cytomegalovirus disease between the two groups. Stepwise regression analysis identified three factors associated with the occurrence of gut perforation: duration of transplant operation, posttransplant intra-abdominal bleeding requiring reoperation, and early portal vein thrombosis. During the postoperative course, severe fungal infections were significantly more frequent in the gut perforation group. The 3-year patient survival rate was 70% in the group with gut perforation and was not different from the group without perforation (80%). This study shows that children with previous portoenterostomy carry a high risk of developing gut perforation after liver transplantation. This is especially true for those patients with the most difficult hepatectomies, which are responsible for the iatrogenic injury of the bowel. Other risk factors pointed out in this study were splanchnic congestion in case of prolonged portal venous clamp time or early portal vein thrombosis and repeated trauma of the bowel caused by reoperations. On the other hand, other well known risk factors, such as steroid therapy and viral diseases, were not involved in the occurrence of gut perforations in this study. Besides emergent surgical treatment, this type of complication requires aggressive therapy against fungal infections.     

Successful management of ruptured choledochal cyst by primary cyst excision and biliary reconstruction

One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.     

Choledochal cyst: a review of 19 cases

Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.