Caroli's disease in children: is it commonly misdiagnosed?

AIM: Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. METHODS AND RESULTS: We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding. CONCLUSIONS: Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.

Sugiura procedure in portal hypertensive children.

Bleeding from esophageal varices is an important cause of morbidity and mortality in children with portal hypertension. The treatment protocol is planned according to the etiologic factors underlying the portal hypertension, which may be either intrahepatic or extrahepatic. Although portasystemic venous shunt operations were common previously, they are now regarded as nonphysiologic and are rarely used because of their unexpected results and complications. Today, in many centers, endoscopic procedures have become the first-step treatment modality in bleeding esophageal varices. More complicated surgical procedures, such as devascularization procedures in extrahepatic portal hypertension, and liver transplantation in patients with failing liver, should be performed when conservative measures fail. We followed up 69 patients with portal hypertension with endoscopic sclerotherapy in our department. Here we present a retrospective evaluation of the effect of the Sugiura operation on the prognosis of 12 children (6 with extrahepatic and 6 with intrahepatic portal hypertension) who were not responsive to the sclerotherapy program. No rebleeding was seen in 9 of the 12 (75%) patients after the procedure, and the mortality rate in this series was 1 of 12 (8.3%); this patient died of hepatic failure.

Albendazole therapy in the treatment of hydatid liver disease.

Hydatid disease, a common health problem in Turkey as in many countries, should be included in the differential diagnosis of all patients presenting with a cystic mass in the abdominal or thoracic cavity. Previously, surgery had been the main form of treatment. But with the introduction of preoperative medication with albendazole, treating this disease medically is now growing in popularity. We report herein the findings of ten patients with two lung and 20 liver hydatid cysts ranging from 3 to 20 cm in diameter, who were successfully treated with medical therapy. Multiple organ involvement and multiple cysts in an organ are the main indications for medical treatment with albendazole, but the indications should not be limited to certain situations such as small-sized cysts, since even huge-sized cysts responded well to the therapy in this series.

Bronchoscopic removal of tracheobroncheal foreign bodies: value of patient history and timing.

Between 1990 and 1999, 741 bronchoscopic procedures were performed in 698 children, 594 of whom were evaluated for foreign-body aspiration (FBA) (mean age 3.9 years, male:female 287/307). Based on the presenting symptoms, clinical outcome, and complications, two major groups were identified. Group 1 consisted of 438 patients with a definitive history of FBA. Most were admitted soon after the aspiration with sudden onset of symptoms such as coughing, choking, wheezing, and respiratory distress. Group 2 comprised 156 patients with chronic pulmonary infections and/or atelectasis without a definitive history of FBA. The most common radiographic finding was emphysema of one lung in group 1 (61.1%) and pneumonia in group 2 (70%). Among the patients in whom a FB was removed, the percentage of normal radiography was 17%. The FB was identified and removed in 83% of cases in group 1. The complication rate in this group was 9.8%, and all the complications were treated medically. Only 2 patients required intercostal drainage. In group 2, a FB was identified in 25% of bronchoscopic examinations and 17% of the patients developed complications. One of these patients underwent an urgent thoracotomy due to bilateral tension pneumothoraces and 2 required tracheostomies. Patients with a definitive history of FBA, even with a normal physical examination and radiographic findings, must undergo bronchoscopic investigation. Cases with late presentation and chronic pulmonary infection are at high risk. In this group care should be take in determining the indication and timing of bronchoscopy in order to prevent life-threatening complications.

Diagnostic laparoscopy in prolonged jaundice.

BACKGROUND: The early diagnosis of surgical jaundice in a neonate is an important step for the surgical success in extrahepatic biliary atresia. Diagnostic laparoscopy, as in many areas in surgery, is included in the conventional diagnostic methods of extrahepatic biliary atresia. METHODS: Since 1992, 24 infants with prolonged jaundice, in whom extrahepatic biliary atresia and neonatal hepatitis could not be differentiated with conventional diagnostic interventions, have been evaluated laparoscopically. RESULTS: A coarse, irregular, greenish-brown liver with some degree of fine angiomatous development and an atretic gallbladder are the findings of laparoscopic evaluation in an infant with extrahepatic biliary atresia. However, in neonatal hepatitis, the liver is smooth, sharp-edged, and chocolate brown in color, and simultaneously performed cholangiography should show the passage of the contrast material both into the proximal biliary tracts and the intestinal system. In this series, 10 of 24 cases were proved to be neonatal hepatitis diagnosed by laparoscopy, so unnecessary laparotomy was avoided in 42% of the cases. CONCLUSION: When the diagnostic laparoscopy, in which the liver and the gallbladder are directly visualized, is combined with the cholangiographic examination, the most accurate and earlier diagnosis in an infant with prolonged jaundice can be achieved, and the important period of time for the surgical success in extrahepatic biliary atresia will be minimally wasted.

Chylous ascites after liver transplantation with mesentero-portal jump graft.

Chylous effusions and lymphatic leaks occur after trauma, malignant disease, primary lymphatic disorders, and parasitosis, and rarely after abdominal surgery. Chylous ascites after orthotopic liver transplantation is a rare complication. We report a case of chylous ascites occurring after hepatic transplantation with a mesentero-portal venous jump graft, successfully treated with conservative management.

A case of wandering spleen causing urinary symptoms after correction of a posterolateral diaphragmatic hernia.

A 12-year-old girl who had had an operation for a posterolateral diaphragmatic hernia was admitted to the hospital because of enuresis. Physical examination and radiologic studies revealed the spleen behind the urinary bladder, left-sided hydronephrosis, and a high volume of residual urine. The hydronephrosis and residual urine resolved immediately following splenectomy.

A case of mesenteric cysts caused by abdominal tuberculosis.

We report on a 10-year old boy suffering from chronic abdominal pain. Clinical examination and imaging modalities revealed that the patient had mesenteric cysts. Exploratory laparotomy revealed two mesenteric cysts of various size and multiple enlarged mesenteric lymph nodes. M. tuberculosis was identified and histology of the specimens proved the existence of abdominal tuberculosis.

Hydatid disease of the liver in childhood.

The records of 100 children with hydatid disease were reviewed retrospectively from 1978 to 1997; 43 were girls and 57 were boys. The mean age was 9.14 years; 61 patients had 124 hepatic cysts. Presenting symptoms were asymptomatic abdominal masses, found masses incidentally during ultrasonography (US), or acute abdomen. Plain X-ray films, US, or computerized tomography (CT) are sufficient for diagnostic evaluation in endemic areas. In the differential diagnosis, laboratory investigations such as the Casoni and Weinberg tests, indirect hemagglutination, eosinophilia, and ELISA were also used. These tests may give negative results, however, in some patients with hydatid disease. The mean follow-up time was 10.5 years (range 1-18 years), the mean duration of hospitalization 7 days. The complication rate was 3.6%. Mortality was 3.27% and occurred after the administration of formaldehyde and hypertonic scolicidal agents. Hydatid disease of the liver can be treated medically in selected patients; conservative surgical approaches that save as much parenchyma as possible, such as partial cystectomy and capitonnage, are indicated in the other cases.

The effect of an H2-receptor antagonist on small-bowel colonization and bacterial translocation in newborn rats.

Bacterial translocation (BT) is defined as the passage of enteric bacteria from the gastrointestinal tract to extraintestinal tissues. Bacterial overgrowth is one of the main promoting factors of BT, which is thought to play an important role in the pathogenesis of sepsis and necrotizing enterocolitis. It is believed that small-bowel colonization is established by bacterial spread through the rectum. Gastric acid is also involved in this process. An experimental study was designed for investigating the effect of gastric acid inhibition with the use of an H2-receptor antagonist on intestinal colonization and BT in newborn rats. Animals were divided into two groups: the ranitidine group (n = 20) received ranitidine 10 mg/kg per day intramuscularly for 5 days; the control group (n = 30) received saline solution. Mesenteric lymph node, spleen, liver, stomach, small bowel/cecum, and large bowel specimens were obtained from each rat 5 days later and gram-negative and -positive aerobic bacteria identified by the use of chocolate and Endo agar. It is concluded that: (1) there was a strong correlation between gastric and small-bowel bacterial colonization in the ranitidine group; (2) no correlation between large-and small-bowel colonization could be demonstrated; and (3) BT occurred only in the ranitidine group.

Hepatic duct stone associated with chlamydia sepsis: a rare condition in childhood.

Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile stone in the hepatic duct after chlamydia sepsis.

Wandering spleen in childhood: a report of three cases.

Wandering spleen is a rare cause of abdominal pain in children, and an accurate diagnosis is seldom made preoperatively. A splenectomy is the treatment of choice in cases of splenic torsion and infarction, while in patients with chronic symptoms splenopexy may also be attempted. We herein report three patients with wandering spleen, of whom two presented with acute torsion of the splenic pedicle and one demonstrated an asymptomatic mobile abdominal mass. In the first case splenopexy was attempted, but during follow-up the spleen was found to have undergone atrophy. The presentation, diagnostic procedures, and treatment modalities in pediatric wandering spleen are reviewed.

Fetus in fetu or giant epignathus protruding from the mouth.

A huge and extremely organoid mass, protruding from the mouth of a newborn and causing respiratory embarrassment, was resected. It was difficult to make a clear distinction whether this mass was fetus in fetu or a highly organoid epignathus dealing with the findings obtained from its gross and histological examination of the mass.

[Time of disappearance of artificial pneumoperitoneum in children. Diagnostic value]. / La durée de disparition du pneumopéritoine artificiel des enfants. Intérêt diagnostique.

Postoperative pneumascos has always been interesting and provocative for surgeons because its "correct" disappearance time still remains ill-defined. Fifty-six children, for whom Golstein's intraoperative diagnostic pneumascos was carried out to demonstrate a contralateral hernia during the correction of a unilateral inguinal hernia, were followed up for disappearance of their pneumascos with abdominal radiographs taken at the 4th postoperative hour and on the 1st, 2nd, 4th and 8th days. The children were divided up into three groups: 9 children were younger than 6 months, 20 were 6 to 24 months old, and 27 were older than age 2. The intraperitoneal free air present in all subjects 4 hours after surgery has completely disappeared from the radiographs of the children younger than 6 months on the fourth day, and on the eighth day for the children aged 6 to 24 months, while a trace of subphrenic air still persisted in the 7% (2 out of 27) of children who were older than age 2. This work is aimed at establishing a correlation between the child's age and the time of disappearance of the pneumascos.

Ultrasonographic findings and management of intrahepatic biliary tract abnormalities after portoenterostomy.

Ultrasonographic examinations was made in 24 children who had undergone a portenterostomy to correct extrahepatic biliary atresia. Abnormalities were observed in six patients. These were the result of ongoing inflammatory reactions because all had been suffering from cholangitis after surgery, and the size of the biliary tract structure changed in accordance with the occurrence and subsidence of the cholangitis. Ultrasonographic examination showed dilation of the intrahepatic bile duct in one patient and cystic lesions in five patients. Treatment included percutaneous transhepatic bile drainage for dilated bile ducts, alcohol injections for intrahepatic cysts, and reoperation for cysts in the porta hepatis. Treatment was not required for cysts in controllable cholangitis. The results of these approaches were excellent, indicating that they were of benefit in treatment intrahepatic abnormalities occurring after portoenterostomy.

Congenital lobar emphysema--a report of 5 cases.

Five infants with congenital lobar emphysema whose main symptoms included dyspnea, cyanosis and recurrent respiratory infections, are presented herein. The most reliable diagnostic tool was plain chest X-ray films with antero-posterior and lateral views, while radio-isotopic investigation of the lung perfusion state took second place in the diagnosis of this disease. The affected lobes were the left upper lobe in four patients and the right middle lobe in one. Lobectomy was performed with good results in 4 patients, however, 1 infant was lost following an emergency thoracotomy.

Extensive intestinal aganglionosis.

Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprung's disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprung's disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.

[Early and late results of heterotopic spleen autotransplantation in pediatric splenic trauma]. / Früh- und Spätresultate heterotoper Milzautotransplantation beim kindlichen Milztrauma.

In this study, early and late results of heterotopic splenic autotransplantation in 18 children with type IV splenic injuries (Upadhyaya-Simpson classification) are presented. Splenic scintigrams, quantitative analysis of Howell-Jolly inclusion bodies, immunoglobulin (IgG, IgM, IgA) and complement C3 levels and T and B lymphocyte counts were analysed in the postoperative evaluation of the autotransplantation group. The total follow-up period was 7 years. According to our results, splenic implants increased complement C3 levels and improved filtration function of the splenectomized children. This autotransplantation group has two important characteristics: a) it is one of the largest series of the literature (pediatric age group), with b) longest follow-up period.

Mesenchymal hamartoma of the liver of an infant--case report.

A 10-month-old boy was found to have a huge hepatic mesenchymal hamartoma. Histologically the lesion appeared as large islands of loose mesenchymal tissue with profuse cystic bile ducts and hepatic cells. There were profuse bile duct and blood-vessel communications between the hamartoma and the right lobe of the liver. Because of benign nature of this rare neoplasm, more conservative operations are indicated rather than formal hepatic lobectomy.