Gallbladder polyposis in metachromatic leukodystrophy

Gallbladder polyposis is a rare entity that can be associated with conditions such as metachromatic leukodystrophy (MLD), but the literature is sparse. We present a child with gallbladder polyposis who was diagnosed with MLD 15 months later despite normal neuroimaging and clinical examination initially.     

The use of ultrasonography in the diagnosis of biliary atresia

Preoperative upper abdominal ultrasonograms of babies with biliary atresia were reviewed in order to determine the efficacy of this technique in the differential diagnosis of biliary atresia and neonatal hepatitis. In 4 patients with neonatal hepatitis and 8 normal controls, ultrasonograms showed echoes of the gallbladder. In 14 patients with biliary atresia, echoes of the gallbladder were not apparent. It is concluded that preoperative ultrasonograms provide an efficient diagnostic method for differentiating biliary atresia from neonatal hepatitis.Offprint requests to: T. Okasora     

Gallbladder contraction in biliary atresia: a pitfall of ultrasound diagnosis

In 3 (9 %) of 34 children with biliary atresia, US revealed gallbladder contraction following an oral feed, given on admission, but not with subsequent feeds. Surgery revealed a Kasai type IIIa biliary atresia with a patent communication between the gallbladder and duodenum. We propose that the bile ducts may initially have been patent, but then gradually became obliterated secondary to inflammation. These cases may explain the development of one type of biliary atresia. Received: 28 July 1997 Accepted: 1 December 1997     

Neonatal sepsis presenting as a choledochal cyst

We report a case of a neonate who presented with Klebsiella sepsis and a fusiform dilation of the common bile duct on ultrasound suspected to be a choledochal cyst type Ia. In addition, a biliary sludge was noted in the gallbladder. All findings resolved with resolution of the sepsis. We discuss the case and review the literature regarding the association of sepsis with cholestasis and biliary sludge. CONCLUSIONS: we suggest that in any case of sepsis presenting as a choledochal cyst, it is essential to repeat the ultrasound after complete recovery before a surgery is planned.     

Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis

Twenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.     

Gallbladder hypocontractility in infantile colic

We evaluated gallbladder contractility in 58 colicky infants and 57 age-matched controls. Gallbladder size was measured before and 1 h after feeding and the contraction index of the gallbladder was calculated. The results showed decreased contractility of the gallbladder in colicky infants: mean contraction index in colicky infants was 56% (95% confidence interval, 49–63%) and in controls 67% (61–73%). No difference was found in the fasting size of the gallbladder. Postprandial contraction was decreased in colicky infants examined in the evening (n = 14) compared with those examined in the morning (n = 44). Decreased contraction of the gallbladder was found in those colicky infants who presented colicky cry compared with colicky infants without symptoms at the time of examination. No structural abnormalities were found in the general abdominal survey. In conclusion, infants with colic have hypocontractility of the gallbladder. This indicates abnormal biliary tract physiology in association with infantile colic.     

Significance of the anomalous arrangement of the pancreaticobiliary duct in the etiology of biliary atresia.

BACKGROUND/PURPOSE: The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. MATERIALS AND METHODS: Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. RESULTS: All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. CONCLUSIONS: From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.     

Hitching the gallbladder in laparoscopic-assisted cholecysto-cholangiography: a simple technique

Neonatal cholestatic disorder in the late neonatal period requires often cholangiography to differentiate between biliary atresia and other causes of prolonged neonatal jaundice. A simplified method of a laparoscopic-assisted cholecysto-cholangiography is presented. Retrospective chart review was conducted of all patients who from May 2002 to April 2012 underwent a laparoscopic-assisted cholecysto-cholangiography with routine fixation of the fundus of the gallbladder to the lateral aspect of the abdominal wall. A total of 18 infants (8 boys) aged 41–104 (median 64) days underwent laparoscopic-assisted cholecysto-cholangiography for prolonged jaundice. The technique identified ten cases of a patent bile duct system and eight biliary atresias. (Thirty-two cases of suspected biliary atresia were confirmed by laparoscopy alone.) Two cases required suturing of a bile leak at the puncture site. Hitching the gallbladder to the lateral abdominal wall is a simple method allowing an optimal radiographic assessment of the extra- and intra-hepatic bile duct anatomy.     

Serial ultrasonic examination to differentiate biliary atresia from neonatal hepatitis — special reference to changes in size of the gallbladder

We performed serial ultrasonic examinations to differentiate biliary atresia from neonatal hepatitis. The subjects studied were 144 children (100 normal neonates and infants, 31 patients with neonatal hepatitis and 13 patients with biliary atresia). They were examined by ultrasound before, during and after feeding. In 97 out of 100 normal children and all patients with neonatal hepatitis, the gallbladder was identified, and the change in size following oral feeding was observed. In four children with severe neonatal hepatitis which could not be differentiated from biliary atresia by clinical and laboratory data, we readily identified the gallbladder and observed the change in the size following oral feeding. In 8 of 13 patients with biliary atresia, we identified a small gallbladder whose size was not affected by oral feeding. In the other patients the gallbladder was not identified before, during or after oral feeding. On the basis of these results, we consider that serial ultrasonic examination with oral feeding aids in a differential diagnosis of biliary atresia and neonatal hepatits.     

Intrahepatic cholangitis associated with mucocutaneous lymph node syndrome

Although gallbladder hydrops occurs frequently in patients with mucocutaneous lymph node syndrome (MCLS), the etiology of the gallbladder lesion is unclear. Recently we performed a liver biopsy on a child with severe MCLS and demonstrated selective invasion of biliary ductular epithelial cells by polymorphonuclear leukocytes with sparing of the hepatocytes. The possible role of the selective destruction of biliary cells in the pathogenesis of biliary disease in MCLS is discussed.     

Laparoscopic excision of an extra-biliary gallbladder duplication cyst in a 9-month-old infant

Duplication of the gallbladder is a rare congenital anomaly of the biliary system. We herein present a case of a 9-month-old full-term female with a prenatally identified gallbladder duplication cyst managed via laparoscopic excision.Key words: Gallbladder duplication, paediatric laparoscopy, prenatal diagnosis     

Biliary disease in metachromatic leukodystrophy

Pediatric Radiology - This paper illustrates the previously unreported sonographic changes in the biliary tract in metachromatic leukodystrophy (MLD). Gallbladder wall thickening due to sulfatide...     

Polysplenia syndrome associated to neonatal hepatitis

OBJECTIVE: To report the unusual association between neonatal hepatitis and polysplenia syndrome.METHODS: Clinical, biochemical, histopathological, surgical and image analysis methods were used.RESULTS: The 36 days old patient presented hyperbilirubinemia with increase of direct reacting bilirubin and high alkaline phosphatase. The physical examination evidenced jaundice and hepatomegaly. The chest X-ray showed situs inversus and dextrocardia. Abdominal ultrasonography presented an increase in liver volume, mainly at the right lobe. There were two cystic lesions at the liver with absence of intrahepatic biliary tract dilatation. Absence of splenomegaly. Biliary scintigraphy didn't show any excretion of radioisotope to the duodenum while transoperative cholangiography presented contrast medium flowing to the duodenum lumen. During laparotomy it was possible to observe polysplenia, abdominal aorta to the right of the lower vena cava, gallbladder at the left lobe of the liver, appendix on the left side of the abdomen and sigmoid colon on the right side. The hepatic wedge biopsy was compatible with idiopathic neonatal hepatitis.CONCLUSION: The finding of polysplenia syndrome in patients with neonatal cholestasis doesn't necessarily indicate the recurrent presence of extrahepatic biliary atresia.     

Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates

Aim: To investigate the differences in clinical and pathological manifestations between biliary atresia with extrahepatic biliary cyst and choledochal cyst in neonates. Methods: Symptoms and clinical signs in 5 neonates with biliary atresia with extrahepatic biliary cyst (4 of type I and 1 of type III) and 17 neonates with choledochal cyst were recorded. The levels of serum alkaline phosphatase, bilirubin, direct bilirubin, transaminase, gamma-glutamyl transpeptidase were analysed. Width and length of gallbladder and choledochal cyst were measured on B-mode ultrasound before surgery. Intrahepatic or extrahepatic biliary ducts were visualized with intraoperative cholangiography. The pathologic features in specimens of the liver were studied with light- and electron transmission microscopy. Results: All malformations occurred more commonly in girls, and obstructive jaundice was the main manifestation in both groups. Laboratory tests showed similar results for all patients in this study. With regard to pathological features, no significant difference was seen in either light microscopy or transmission electron microscopy, but it was shown with ultrasound that the length and width of the cysts and the gallbladder in neonates with biliary atresia were all shorter than the measurements in patients with choledochal cyst. The intrahepatic bile ducts could not be visualized on intraoperative cholangiography in type III biliary atresia. Deformation of the biliary ducts within the liver and stricture of the portal bile duct were the predominant features in type I biliary atresia, while the bile duct within the liver was normal or dilated in neonates with choledochal cyst.

Conclusions: Cystic lesions of the extrahepatic bile duct might be a common manifestation of biliary atresia and choledochal cyst. Intraoperative cholangiography is a precise and effective technique in the differential diagnosis of those lesions and helps decide on the most rational method of treatment.     

Ultrasonographic evaluation of fatty meal stimulated gallbladder contraction in the diagnosis of biliary dyskinesia in children

AIM: Biliary dyskinesia (BD) is characterized by symptoms of biliary disease, no evidence of gallstones on ultrasonography (USG), and diminished gallbladder ejection fraction. The diagnosis is based on findings of abnormal gallbladder ejection fraction diagnosed by hepatobiliary scintigraphy before and after stimulation of gallbladder contraction with cholecystokinin (CCK). We used an easier diagnostic technique defined as ultrasonographic evaluation of fatty meal stimulated gallbladder contraction, employing USG with the diagnosis of biliary dyskinesia in children. The study was conducted by USG to investigate the volume and contractility of the gallbladder (GB) in fasting conditions and 45 min after a standardized fatty meal (SFM) in normal children and in a group of BD patients, and to compare the diagnostic value of this test with scintigraphy and to evaluate its use as a new method in the diagnostic algorithm of BD. METHODS: We assessed the volume changes and contractility of the GB in response to SFM by USG in 14 patients with BD diagnosed by cholecystokinin stimulated hepatobiliary scintigraphy (CCKs-HBS); and compared them with 14 control patients matched for age and gender before cholecystectomy. After an overnight fasting, GB volume was measured by USG then the GB volume was again measured after the SFM ingestion. Using USG, length, width and height of GB were measured, and volume of the GB was calculated using the 'Dodds' formula. These volume measurements were used to calculate the percentage of gallbladder emptying (ejection fraction). Fasting, after SFM and EF values comparisons between groups were done with student's t-test. RESULTS: No statistically significant difference of fasting GB volumes were demonstrated between BD and control groups (14.1+/-6.7 cm3 and 13.4+/-4.0 cm3 respectively). GB volumes of the BD group after SFM were significantly greater than in healthy controls (13.1+/-1.8 cm3 and 3.4+/-0.9 cm3 respectively, P<0.0005). The calculated percentage of gallbladder contraction (ejection fraction) was found to be lower in BD patients than in healthy controls (7.1%+/-1.8% and 73.8%+/-6.4%, respectively, P<0.0005). CONCLUSION: Ultrasonographic evaluation of fatty meal stimulated gallbladder contraction provides relatively reliable and reproducible results. Thus it can be used for scanning in patients with biliary symptoms as a prior modality to CCK-HBS since it is a relatively easier, safer and available method with which to make a definitive diagnosis of BD. The patients with symptoms of biliary disease and no evidence of gallstones on USG should be evaluated by the method proposed in this study before the routine laboratory and radiologic tests.     

Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length

Background. To evaluate the accuracy and utility of the triangular cord sign and gallbladder length in diagnosing biliary atresia by sonography.?Materials and methods. Sixty fasted infants with cholestatic jaundice aged 2–12 weeks were examined sonographically using a 5–10 MHz linear array transducer, focusing on the triangular cord sign (as described by Choi et al. [1]), the gallbladder, and ducts. The triangular cord is defined as a triangular or tubular echogenic density seen immediately cranial to the portal vein bifurcation; it represents the fibrotic remnant of the obliterated cord in biliary atresia. The findings were blinded to blood chemistry, ^{99 m}Tc-DISIDA hepatobiliary scintigraphy, and liver biopsy. Diagnosis of biliary atresia was confirmed at surgery and histology. Non-biliary atresia infants resolved medically. Comparative charges of the various investigations was made.?Results. Twelve infants had biliary atresia, and ten demonstrated a definite triangular cord. The two false-negatives had small or nonvisualized gallbladders. No false-positives were recorded. Gallbladder length ranged from 0–1.45 cm with a mean of 0.52 cm in biliary atresia compared to a mean of 2.39 cm in nonbiliary atresia infants. ^{99 m}Tc-DISIDA hepatobiliary scintigraphy showed no excretion (false-positive) in 23 % of nonbiliary atresia cases. Scintigraphy and liver biopsy charges were 2 and 6 times that of sonography, respectively.?Conclusion. The triangular cord sign and gallbladder length together are noninvasive, inexpensive, and very useful markers for biliary atresia. Received: 26 May 1999/Accepted: 28 July 1999     

Factors of biliary carcinogenesis in choledochal cysts.

The long-term complications of choledochal cysts include malignant degeneration of the epithelial lining of the biliary tract. Possible factors leading to malignant degeneration were examined by measuring amylase levels in the biliary tract, intraoperative biliary manometry and a histopathological study. 27 patients, 1 to 13 years of age, with choledochal cysts, were studied over a 13-year period. Amylase levels in the bile of choledochal cysts and the gallbladder were measured. The pressure profile of the biliary tract was recorded as the probe was withdrawn from the sphincter of Oddi (SO) to the distal end of the common bile duct. Hyperplasia of the gallbladder mucosa and malignancy of the biliary tract were investigated. Patients with high levels of biliary amylase had higher pressure differences between the SO and the duodenum than those with low levels of biliary amylase. The incidence of mucosal hyperplasia of the gallbladder mucosa was significantly higher in the fusiform type than in the cystic type. Adenocarcinoma in a cystic choledochal dilatation was found in a 12-year-old girl with high amylase levels. The cause of regurgitation of pancreatic juice into the biliary system might be due to a high pressure difference between the SO and the duodenum in addition to the lack of sphincter function at the abnormal junction of the pancreaticobiliary ductal system. Early diagnosis and surgical treatment to prevent the regurgitation leading to hyperplasia and malignancy of the biliary tract are important for children with choledochal cysts.     

Beware of the ventriculogallbladder shunt

We report a case of distal end malfunction in a child with ventriculoabdominal shunt. The distal placement was in the gallbladder rather than the peritoneal cavity, as is usually the case with a ventriculoperitoneal shunt. Surgeons should be alerted to the possibility of distal terminus of a shunt to be other than the peritoneal cavity, as revision surgery in patients with ventriculogallbladder shunt requires exposure of the metal connector on the gallbladder wall to prevent biliary leakage. Received: 4 May 1999/Accepted: 18 November 1999     

Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography

Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography. This approach suffers from the disadvantage that sick infants are subjected to a time-consuming and potentially negative surgical exploration. The purpose of this study was to determine if percutaneous cholecystocholangiography (PCC) prevents unnecessary laparotomy in infants whose cholestasis is caused by diseases other than biliary atresia. This study is a 10 year retrospective review of all infants with persistent direct hyperbilirubinemia and inconclusive biliary nuclear scans who underwent further evaluation for suspected biliary atresia. A gallbladder ultrasound (US) was obtained in all patients. When the gallbladder was visualized, further imaging by PCC was done under intravenous sedation; otherwise, the standard operative cholangiogram (OCG) was performed, with liver biopsy as indicated. The primary outcome was the diagnostic accuracy of PCC, especially with respect to preventing a laparotomy. There were 35 infants with suspected biliary atresia, with a mean age of 8 weeks (range 1-14 weeks). Nine infants whose gallbladder was visualized by ultrasound underwent PCC that definitively excluded biliary atresia. Of this group, the most frequent diagnosis (five patients) was total parenteral nutrition-associated cholestasis. The other 26 infants with absent or decompressed gallbladder had laparotomy and OCG, which identified biliary atresia in 16 patients (61%). Laparotomy was avoided in all 9 patients who underwent PCC, thus reducing the negative laparotomy rate by 47%. There were no complications associated with PCC. Several alternative techniques to operative cholangiogram have been described for the definitive exclusion of biliary atresia, but many of these have distinct drawbacks. Advances in interventional radiology techniques have permitted safe percutaneous contrast evaluation of the biliary tree. Identification of a normal gall bladder on sonogram is highly predictive of the absence of biliary atresia. Further confirmation can be accurately obtained by a combination of PCC and percutaneous liver biopsy.     

Adenomyomatosis of the gallbladder in childhood: A systematic review of the literature and an additional case report

AIM: To investigate the diagnostic and therapeutic assessment in children with adenomyomatosis of the gallbladder (AMG). METHODS: AMG is a degenerative disease characterized by a proliferation of the mucosal epithelium which deeply invaginates and extends into the thickened muscular layer of the gallbladder, causing intramural diverticula. Although AMG is found in up to 5% of cholecystectomy specimens in adult populations, this condition in childhood is extremely uncommon. Authors provide a detailed systematic review of the pediatric literature according to PRISMA guidelines, focusing on diagnostic and therapeutic assessment. An additional case of AMG is also presented. RESULTS: Five studies were finally enclosed, encompassing 5 children with AMG. Analysis was extended to our additional 11-year-old patient, who presented diffuse AMG and pancreatic acinar metaplasia of the gallbladder mucosa and was successfully managed with laparoscopic cholecystectomy. Mean age at presentation was 7.2 years. Unspecific abdominal pain was the commonest symptom. Abdominal ultrasound was performed on all patients, with a diagnostic accuracy of 100%. Five patients underwent cholecystectomy, and at follow-up were asymptomatic. In the remaining patient, completely asymptomatic at diagnosis, a conservative approach with monthly monitoring via ultrasonography was undertaken. CONCLUSION: Considering the remote but possible degeneration leading to cancer and the feasibility of laparoscopic cholecystectomy even in small children, evidence suggests that elective laparoscopic cholecystectomy represent the treatment of choice. Pre-operative evaluation of the extrahepatic biliary tree anatomy with cholangio-MRI is strongly recommended.