RESUMEN

BACKGROUND: Choledochal cyst is usually diagnosed in childhood. Early treatment can prevent further complication. We report on our series of patients over the past 30 years. METHODS: A retrospective study was performed on all pediatric patients who presented with choledochal cyst from January 1978 to December 2008. The main outcome measures recorded were the clinical presentation, management, and long-term outcome of the patients. RESULTS: Eighty-three patients presented to us during the caption period with a mean age at diagnosis of 45 months (0 month to 16 years). Diagnoses were made antenatally in 15 patients. The most common symptoms were abdominal pain (n = 39) and jaundice (n = 35). Seventy-five patients had surgery, in which 72 patients had resection of the cyst and Roux-en-Y hepaticojejunostomy. Ten were performed by laparoscopic means. We categorized the cysts based on the Todani classification. There was no mortality. No malignant change was documented. For those 4 who had Caroli disease, 2 underwent liver transplantation and 2 had hepatectomy. Overall early complication rate was 5.3% (4/75). CONCLUSIONS: Complete excision of cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice, and the late result is good. Laparoscopic surgery is feasible. Long-term follow-up is necessary. There is no evidence to suggest that some type IV cysts are the result of disease progression from type I cysts.

Asunto(s)

Asunto(s)

RESUMEN

PURPOSE: The aim of this study was to analyze the early and late results of pediatric liver transplantation, with particular reference to complications that required surgical or radiologic intervention. METHODS: The records and code sheets of children who underwent liver transplantation in the authors' institution between September 1993 and December 2001 were reviewed. RESULTS: Twenty-nine children (16 boys and 13 girls) underwent 31 liver transplantations (23 living donor, 8 cadaveric donor) during the study period. The ages of the children ranged from 4 months to 132 months (median, 16 months). Eighteen children had complications that required surgical or radiologic interventional procedures. Complications included, among others, hepatic vein thrombosis (n = 1, 3%), hepatic vein stenosis (n = 2, 7%), portal vein thrombosis (n = 2, 7%), biliary stricture (n = 3, 10%), bile leakage (n = 2, 7%), hepatic artery pseudoaneurysm (n = 1, 3%), jejuno-jejunostomy leakage (n = 1, 3%), graft hepatitis (n = 1, 3%), and posttransplant lymphoproliferative disorder (n = 2, 7%). In addition, 6 children (21%) suffered from intraabdominal bleeding from a variety of causes. After appropriate interventions, at a median follow-up of 38 months (range, 1 to 96 months), patient and graft survival rates were 79% and 74%, respectively. The retransplantation rate was only 7%. There was no incidence of hepatic artery thrombosis. All living donors remain alive and well. CONCLUSIONS: Complications are inevitable in pediatric liver transplantation. However, with timely recognition and active intervention, a good outcome can be achieved.

Asunto(s)

RESUMEN

BACKGROUND/PURPOSE: Liver transplantation is the only therapeutic option for biliary atresia (BA) patients with failed Kasai portoenterostomy (PE). The indication for and timing of liver transplantation in these patients are important management issues. The aims of this study are to evaluate the relationship between portal venous velocity (PVV) and clinical and biochemical indicators of liver function in BA, and to examine the role of PVV in the postoperative surveillance of these patients. METHODS: Twenty-nine children (mean age, 8 years 4 months) who had PE for BA underwent Doppler ultrasonography to evaluate PVV. Using regression analysis, these findings were correlated with biochemical indicators of liver function. The Mann-Whitney test was used to detect any significant differences in PVV between the Child-Pugh A group and combined Child-Pugh B and C group patients. RESULTS: The mean +/- SD PVV for the Child-Pugh A group and the combined Child-Pugh B and C group was 17.1 cm/s +/- 8.9 cm/s and 10.2 cm/s +/- 3.3 cm/s, respectively, and this difference was statistically significant (P =.037). CONCLUSIONS: Doppler ultrasonography measurement of PVV provides adjunctive information to the clinical and biochemical assessment of the liver function status in BA. Its use in the postoperative surveillance of these patients is helpful in identifying the need for and in timing of liver transplantation.

Asunto(s)

RESUMEN

Oesophageal atresia (OA) is often associated with anomalies of other systems. The genetic contribution to the formation of the VACTERL association is not clear. The objective of this study was to evaluate the incidence of associated anomalies in two different racial populations. The associated anomalies in neonates with OA managed in an Asian and a European paediatric surgical centre from 1982 to 1998 were reviewed. Non-Asian and non-European patients were excluded from the respective centres. The incidence of anomalies was compared using Fisher's exact test, taking #E5/E5# below 0.05 as statistically significant. Forty-eight consecutive Asian (25 boys and 23 girls) and 34 consecutive European patients (20 boys and 14 girls) were included in the analysis. The percentage of patients with at least one associated anomaly was 50% and 74% in the Asian and European populations, respectively, which was significantly different (#E5/E5#=0. 04). There was no statistically significant difference in the incidence of associated cardiovascular (29% vs 39%), anorectal (11% vs 18%), and musculoskeletal (16% vs 22%) anomalies, duodenal atresia (4% vs 3%), or Down's syndrome (3% vs 6%) between the two populations. However, the European patients had a significantly higher incidence of urogenital (UG) anomalies (26% vs 4%, #E5/E5#=0.006), the most common being agenesis (n=4) and dysplasia (n=3) of one or both kidneys. Hereditary factors may influence the incidence of associated anomalies in children with OA, particularly of the UG system. However, environmental factors cannot be excluded.

Asunto(s)

RESUMEN

PURPOSE: The aim of this study was to review the efficacy and safety of using a Fogarty balloon catheter to ablate posterior urethral valves (PUV) in the newborn in a developing country. METHODS: Five newborn babies weighing 1.4 to 3.9 kg (mean, 2.08 kg; with 4 weighing less than 2.0 kg) who had severe obstructive uropathy caused by PUV were subjected to valve ablation using a size 6F Fogarty balloon catheter in the radiology department without anesthesia. The ages ranged from 10 to 61 days (mean, 26.4 days) at the time of valve ablation. RESULTS: Successful ablation of PUV was achieved in 4 of 5 (80%) patients after one attempt at ablation. There was failure to ablate the PUV in 1 patient despite 2 attempts to do so. All 4 patients with successful ablation of PUV had grade IV vesicoureteric reflux (VUR) before valve ablation. Postablation micturating cystourethrogram (MCU) showed complete disappearance of VUR in one child. In 2, VUR improved to grade III and II immediately after valve ablation, and further improved to grade I and 0, respectively, at MCU repeated 3 months later. In one child, grade IV VUR persisted during the postablation VUR and remained at that grade 3 months later. CONCLUSIONS: Fogarty balloon catheter ablation of PUV is an effective, economic, and simple alternative to endoscopic valve fulguration in very sick and small neonates. It is particularly suitable for use in developing countries in which expensive endoscopes may not be readily available.

Asunto(s)

RESUMEN

BACKGROUND/PURPOSE: Scar formation after scald injury in children is managed commonly by application of pressure garment. The duration of the treatment depends on clinical assessment. The objective of the study was to evaluate the efficacy of the pressure garment therapy and to correlate the clinical assessment scar thickness with ultrasound measurement. METHODS: Prospective study of children with hypertrophic scars receiving pressure garment therapy was carried out between 1993 and 1998. The scars were mapped with paper cutting. Representative scar sites were scored clinically according to Vancouver General Hospital Burn Assessment Scale (pigmentation, vascularity, pliability, height, pain, itchiness). Blind to the clinical results, 2 radiologists measured the scar thickness of the same sites with ultrasonography (5 to 10 MHz transducer, General Electric LOGIQ 500). The correlation between the clinical scores and ultrasound measurement of scar thickness were analysed using analysis of variance (ANOVA). RESULTS: The median age of 58 children with scald injury (M:F = 36:22) was 2.8 years (0.5 to 15.8 years). The mean percentage of scald was 8.8% (24% had scald area >10%). In addition, there were 5 children with burn from naked flame and 9 children with keloid secondary to surgical incisions. With pressure garment therapy, the scar thickness reached a plateau 1 (1/2) years after the injury and began to decline gradually thereafter. The clinical estimation of scar thickness correlated well with the ultrasound measurement (ANOVA, P =.003, Adjusted R square = 0.99). The accuracy rate of clinical thickness estimation was 67%. Overestimation and underestimation accounted for 15% and 18%, respectively. There also was good correlation between total clinical score and the ultrasound measurement (ANOVA, P <.001). CONCLUSIONS: In children with scald injury, the pressure garment needs to be continued for a minimum of 1 year. Ultrasound measurement correlates well with overall score of the Vancouver General Hospital Burn Assessment Scale but not individual components of the scoring system except wound height.

Asunto(s)

Asunto(s)

Asunto(s)

RESUMEN

PURPOSE: The purpose of this report is to obtain an overview of pediatric surgery training in Asia and to have a glimpse of its delivery. METHODS: A questionnaire survey was conducted, and the returns from 41 (67%) pediatric surgeons from 14 (88%) Asian countries were reviewed. RESULTS: The number of pediatric surgeons per million population is lowest in Indonesia (0.1) and highest in Japan (25). Two cities in China have 100 or more pediatric surgeons. The number of staff surgeons per center ranges from 1 to 36. Centers with 5 or fewer neonatal surgical operations per staff surgeon per year are located in Cambodia, China, and Japan, and centers with more than 40 neonatal surgical operations per staff surgeon per year are found in India, South Korea, Myanmar, and Thailand. There are centers in which more than 400 postneonatal pediatric surgical operations are performed per staff surgeon each year in Cambodia, India, Indonesia, South Korea, and Myanmar. After general surgery training, pediatric surgery training commonly lasts 2 to 3 years (71%). The shortest training period is 1 year in a center in 1 country to 5 or more years in a few countries. At the end of the training, a variety of exit examinations or assessments (clinicals, orals, written) are conducted in most countries. CONCLUSIONS: Pediatric surgery training programs in Asia are diverse. Clinical cases per trainee surgeon vary greatly. Although some countries have an adequate number of pediatric surgeons to deliver a high quality service, others are severely short staffed, and have huge caseloads, delivering pediatric surgical services under extremely difficult conditions. Governments subsidize the cost of surgical care in most countries (93%): self-payment is common (86%) and insurance is least practiced (64%).

Asunto(s)

RESUMEN

The authors review the technique of ultrasound-guided hydrostatic reduction of childhood intussusception and illustrate, in real-time fashion, the treatment of three cases with this technique. Two cases of successful reduction of ileocolic intussusception are demonstrated. The third case is an example of the complex fronded appearance of ileo-ileocolic intussusception and failed reduction. This technique is recommended as an alternative method for the treatment of childhood intussusception, as it does not involve ionizing radiation and is a simple and safe procedure.

Asunto(s)

RESUMEN

PURPOSE: In view of the earlier reports that children below 1 year of age constitute a high-risk group for liver transplantation, the authors reviewed their experience in performing orthotopic liver transplantation in this age group. METHODS: The records of 9 children aged less than 1 year who underwent 6 living-related liver transplants and 3 reduced-size liver transplants between December 1993 and June 1997 were reviewed. RESULTS: Five reexplorations were required for 3 children who had 1 or more of the following early complications: bleeding from hepatic vein to inferior vena cava anastomosis (n = 1), right hepatic vein stump bleeding (n = 1), intraabdominal hematoma (n = 2), jejuno-jejunostomy leakage (n = 1), and colonic perforation (n = 1). Late complications include stricture at the biliary-enteric anastomosis requiring percutaneous balloon dilatation (n = 3) and hepatitis of undetermined etiology requiring retransplantation (n = 1). There was no hepatic artery thrombosis despite the small arteries available for anastomosis. Follow-up ranged from 19 to 61 months (mean, 40 months). Patient survival rate was 100%, and graft survival with good liver function was 89%. All living donors, 2 fathers and 4 mothers, are well. CONCLUSIONS: Liver transplantation in infants less than 1 year of age is technically demanding but feasible and still can be performed with a good outcome. Age alone (under 1 year) should not be considered as a contraindication for liver transplantation.

Asunto(s)

Asunto(s)

RESUMEN

OBJECTIVE: We report our experience using MR cholangiography and CT cholangiography in pediatric patients with choledochal cysts. SUBJECTS AND METHODS: Fourteen patients (two boys, 12 girls; mean age, 7.8 years) with either a preoperative diagnosis of choledochal cyst or a surgical finding of choledochal cyst underwent non-breath-hold MR cholangiography using T2-weighted fat-suppressed fast spin-echo sequences with a 1.5-T magnet, and CT cholangiography with IV infusion of meglumine iodoxamic acid. Radiologic findings were correlated with findings from surgery, operative cholangiography, or percutaneous transhepatic cholangiography. RESULTS: The biliary tree was visualized in all 14 patients with MR cholangiography and in 13 patients (92.9%) with CT cholangiography. In the 11 preoperative cases of choledochal cyst, MR cholangiography correctly showed all cysts and CT cholangiography showed 10 cysts (90.9%). The quality of images on CT cholangiography and MR cholangiography was comparable. The sensitivity of CT cholangiography and MR cholangiography in revealing intrahepatic stones was 83.3% and 66.7%, respectively; the specificity for both techniques was 100%. The rate of detecting the pancreatic duct and the common channel by CT cholangiography and MR cholangiography was 63.6% and 45.5% respectively. After surgery, CT cholangiography was superior to MR cholangiography in revealing the location of biliary-enteric anastomosis and the extent of anastomotic narrowing. CONCLUSION: Because non-breath-hold MR cholangiography is not invasive and does not use ionizing radiation and potentially toxic contrast agents, it is recommended as the imaging technique of choice in children with choledochal cysts. CT cholangiography can be considered as an adjunct after surgery and in patients in whom MR cholangiography is unsatisfactory.

Asunto(s)

RESUMEN

The MRI and CT features of confluent hepatic fibrosis (CHF) are reported in two 6-year-old twins. This is the first case report in children of this little known entity, which may mimic mass lesions, and the first to describe an association of CHF with anti-tuberculous drugs.

Asunto(s)

RESUMEN

PURPOSE: The aim of this study was to examine the influence of associated anomalies in babies born with esophageal atresia (EA). METHODS: A retrospective review of the records of 41 consecutive cases of esophageal atresia managed over an 11-year period was undertaken. RESULTS: A higher incidence of associated anomalies was seen in those babies with lower birth weights. Although all five (100%) babies with EA who weighed less than 1,800 g had associated anomalies, those who weighed 1,800 to 2,500 g and more than 2,500 g were associated with 67% (10 of 15) and 43% (9 of 21) anomalies, respectively. The most common system in which anomalies occurred was the cardiovascular system (37%) followed by gastrointestinal (24%), musculoskeletal (17%), genitourinary (7%), chromosomal (5%), and others (12%). All 17 (41%) babies with no associated anomalies survived. Four of the 10 babies who had two or more systems involvement died, whereas only one of 31 babies with less than two systems involvement died; the difference between these two groups was highly significant (Fisher's Exact test, P = .009). The overall mortality rate was 12%. Three of the deaths were associated with severe anomalies that were incompatible with life such as bilateral renal agenesis, trisomy 18, and complex cardiac anomalies. CONCLUSION: The association of two or more system anomalies and the severity of associated anomalies influence mortality in esophageal atresia.

Asunto(s)

RESUMEN

A 12-year-old girl who suffered from cholangitis was treated successfully with appropriate antibiotics. She had undergone an operation in mainland China, the exact nature of which was unknown. After an ultrasound study, she underwent a helical computed tomography (CT) cholangiogram using intravenous meglumine iodoxamine. The volume data were transferred to a workstation and virtual endoscopy rendering reconstruction of bile ducts was performed. Findings of this study showed that she had cyst excision with hepaticojejunostomy (HJ), and her intrahepatic ducts were packed with stones. Virtual endoscopy gave the impression that the examiner was flying inside the lumen and showed the stricture at the HJ anastomosis, the inner surface of the bile ducts, areas of dilated intrahepatic ducts, and the intrahepatic stones. The study overcame the need for an invasive study such as a percutaneous transhepatic cholangiography (PTC) and also facilitated appropriate surgical treatment in a timely fashion. It is believed that virtual intraluminal endoscopy (VIE) is helpful in the evaluation and management of selected cases of choledochal cyst.

Asunto(s)

Asunto(s)

RESUMEN

OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation.

Asunto(s)

RESUMEN

BACKGROUND/PURPOSE: Reports on the late results of choledochal cyst excision with hepaticojejunostomy in children are relatively few. METHODS: Of the 84 patients who had choledochal cyst who came under our care, 79 have had definitive surgery, three are awaiting surgery, one is being observed with Caroli's disease, and the parents of one child have refused surgery. Thirty-eight patients treated decades ago had internal drainage procedures. Since 1972, 41 patients have had cyst excision with hepaticojejunostomy using a 40-cm Roux loop without an antireflux procedure. Early complications in those who underwent cyst excision with hepaticojejunostomy included anastomotic leak in three patients who required reoperation, cholangitis in two, and fluid collection in the gall-bladder bed that required no intervention in one. RESULTS: During a follow-up period ranging from 4 months to 17 years (mean, 8.5 years), anastomotic stricture, cholangitis, and intrahepatic stone formation developed in two children after being well for 8 years and over 11 years. These children required additional surgical procedures to overcome their problems. Asymptomatic intrahepatic stones 2 years after cyst excision with hepaticojejunostomy developed in a third child. There was no mortality in the entire group that underwent cyst excision and they are all enjoying a good quality of life. CONCLUSIONS: Careful, long-term follow-up is important in children who have choledochal cyst excision with hepaticojejunostomy.

Asunto(s)