A child with unresectable biliary rhabdomyosarcoma: 48-month disease-free survival after liver transplantation.

We describe here a two-yr-old boy with biliary RMS successfully treated by chemotherapy and LT. The child presented with obstructive jaundice at 20 months of age. A mildly vascularized, non-calcified, partially cystic lesion was visualized in the left hepatic lobe. Solid infiltration of the common bile duct and of both left and right hepatic ducts was suspected. Liver biopsy suggested a botryoid-type embryonal RMS originating from the biliary tract. After extrahepatic spread of the tumor was excluded, a biliary drain was applied and neoadjuvant chemotherapy was started. After the treatment, although reduced in volume, the mass was still unresectable without aggressive surgery and gross residual disease. LT with a reduced segment II/III graft was performed four months after diagnosis. The patient received six cycles of adjuvant chemotherapy, and he is alive and recurrence-free 48 months post-transplantation. A posteriori, the transplant might have possibly been avoided with an aggressive resection with biliary reconstruction. Nevertheless, although the risk of the transplant has to be balanced against the chemoresponsiveness of the tumor, the four-yr disease-free survival of this patient suggests that, when coupled with effective chemotherapy, transplantation might be considered a potential treatment for unresectable biliary RMS.

What bilateral damage of the superior parietal lobes tells us about visual attention disorders in developmental dyslexia.

Neuroimaging studies have identified the superior parietal lobules bilaterally as the neural substrates of reduced visual attention (VA) span in developmental dyslexia. It remains however unclear whether the VA span deficit and the deficits in temporal and spatial attention shifting also reported in dyslexic children reflect a unitary spatio-temporal deficit of attention - probably linked to general posterior parietal dysfunction- or the dysfunction of distinct attentional systems that relate to different neural substrates. We explored this issue by testing an adult patient, IG, with a specific damage of the bilateral superior parietal lobules after stroke, on tasks assessing the VA span as well as temporal and spatial attention shifting. IG demonstrated a very severe VA span deficit, but preserved temporal attention shifting. Exogenous spatial orientation shifting was spared but her performance was impaired in endogenous attention. The overall findings show that distinct sub-systems of visual attention can be dissociated within the parietal lobe, suggesting that different attentional systems associated with specific neural networks can be selectively impaired in developmental dyslexia.

Implication of unfavorable histology, MYCN amplification and diploidy for stage I and II neuroblastomas.

BACKGROUND: Surgery is the first line treatment for low-grade neuroblastomas. In stage I tumors, the presence of MYCN amplification is rarely detected and the Shimada histology is not always taken into consideration when deciding on the treatment. This study concerns the significance of these two factors in the evolution of children with low-grade neuroblastomas. METHODS: We analyzed the assessment and follow-up of children with low-grade neuroblastomas (stages I and II) with or without MYCN amplification, with either a favorable or unfavorable histology and with or without tumor cell diploidy. Favorable histology was defined as stroma-poor tumors with more than 5 % differentiating neuroblasts and a mitosis karyorrhexis index (MKI) of less than 100/5000 cells. RESULTS: From 1995 to 2006, out of 114 neuroblastomas, nine (7.9 %) were stage I and 21 (18.4 %) stage II. Of these 30 patients, 27 underwent surgery alone and three received chemotherapy after surgery. The combination of MYCN amplification, unfavorable histology and diploidy was noted in one patient who developed metastases within two months. MYCN amplification alone was noted in two cases who are still tumor-free after two years. Unfavorable histology alone was noted in four patients, of whom one suffered a recurrence of the tumor (previously stage I) and three are tumor-free after six years. Tumor cell diploidy alone was present in 11 patients whose evolution is satisfactory. CONCLUSION: Because MYCN amplification and unfavorable histology are rare in early stage neuroblastomas, these tumors may be misclassified if they are not investigated further. It seems that no single clinical or biological feature can be considered a significant factor in establishing a prognosis or determining whether additional treatment is required.

Harmonization of strategies for the validation of quantitative analytical procedures. A SFSTP proposal--part II.

As reported in a previous paper, the main objective of the new commission of the Société Française des Sciences et Techniques Pharmaceutiques (SFSTP) was the harmonisation of approaches for the validation of quantitative analytical procedures. In a series of meetings, members of this Commission have first tried to review the objectives of analytical methods and the objectives of validation methods and to recommend the use of two-sided beta-expectation tolerance intervals for total error of validation samples (accuracy profile) in the acceptance/rejection of analytical method in validation phase. In the context of the harmonization, the other objectives were: (i) to propose a consensus on the norms usually recognized, while widely incorporating the ISO terminology; (ii) to recommend to validate the analytical procedure accordingly to the way it will be used in routine; (iii) to elaborate a rational, practical and statistically reliable strategy to assure the quality of the analytical results generated. This strategy has been formalised in a guide and the three latter objectives made by the Commission are summarised in the present paper which is the second part of summary report of the SFSTP commission. The SFSTP guide has been produced to help analysts to validate their analytical methods. It is the result of a consensus between professionals having expertise in analytical and/or statistical fields. The suggestions presented in this paper should therefore help the analyst to design and perform the minimum number validation experiments needed to obtain all the required information to establish and demonstrate the reliability of its analytical procedure.

Enhancing reading performance through action video games: the role of visual attention span.

Recent studies reported that Action Video Game-AVG training improves not only certain attentional components, but also reading fluency in children with dyslexia. We aimed to investigate the shared attentional components of AVG playing and reading, by studying whether the Visual Attention (VA) span, a component of visual attention that has previously been linked to both reading development and dyslexia, is improved in frequent players of AVGs. Thirty-six French fluent adult readers, matched on chronological age and text reading proficiency, composed two groups: frequent AVG players and non-players. Participants performed behavioural tasks measuring the VA span, and a challenging reading task (reading of briefly presented pseudo-words). AVG players performed better on both tasks and performance on these tasks was correlated. These results further support the transfer of the attentional benefits of playing AVGs to reading, and indicate that the VA span could be a core component mediating this transfer. The correlation between VA span and pseudo-word reading also supports the involvement of VA span even in adult reading. Future studies could combine VA span training with defining features of AVGs, in order to build a new generation of remediation software.

Liver transplantation for cirrhosis in cystic fibrosis.

BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure. AIMS: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population. PATIENTS: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation. RESULTS: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure. CONCLUSION: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.

Harmonization of strategies for the validation of quantitative analytical procedures. A SFSTP proposal--Part I.

This paper is the first part of a summary report of a new commission of the Société Française des Sciences et Techniques Pharmaceutiques (SFSTP). The main objective of this commission was the harmonization of approaches for the validation of quantitative analytical procedures. Indeed, the principle of the validation of theses procedures is today widely spread in all the domains of activities where measurements are made. Nevertheless, this simple question of acceptability or not of an analytical procedure for a given application, remains incompletely determined in several cases despite the various regulations relating to the good practices (GLP, GMP, ...) and other documents of normative character (ISO, ICH, FDA, ...). There are many official documents describing the criteria of validation to be tested, but they do not propose any experimental protocol and limit themselves most often to the general concepts. For those reasons, two previous SFSTP commissions elaborated validation guides to concretely help the industrial scientists in charge of drug development to apply those regulatory recommendations. If these two first guides widely contributed to the use and progress of analytical validations, they present, nevertheless, weaknesses regarding the conclusions of the performed statistical tests and the decisions to be made with respect to the acceptance limits defined by the use of an analytical procedure. The present paper proposes to review even the bases of the analytical validation for developing harmonized approach, by distinguishing notably the diagnosis rules and the decision rules. This latter rule is based on the use of the accuracy profile, uses the notion of total error and allows to simplify the approach of the validation of an analytical procedure while checking the associated risk to its usage. Thanks to this novel validation approach, it is possible to unambiguously demonstrate the fitness for purpose of a new method as stated in all regulatory documents.

Total cholesterol correlates with cyclosporine C2 levels in kidney transplant recipients under maintenance immunosuppression.

The aim of this study was to assess the relationship between cyclosporine (CyA) trough level (C0) and 2-hour postdose (C2) and total cholesterol (TC) in kidney transplant (KT) recipients on Neoral maintenance immunosuppression. In KT recipients who had more than 5 years of follow-up, stable graft function, and stable Neoral dose, we measured C2 and C0 blood levels, serum creatinine, mean total cholesterol (TC) over the last 5 years, prednisone dose, use of beta-blockers and thiazides. Correlations between C0 and C2 levels and TC were performed with the Pearson coefficient. Receiver operating characteristics (ROCs) were used to define the threshold with greater accuracy for significant variables at the correlation test. Statistical tests were performed with SPSS 9.5 The C2 correlated with TC (0.31; P=.008) whereas C0 did not. The C2 level was an independent predictor for TC after adjusting for recipient age, gender, dose of prednisone, creatinine clearance, and use of beta-blockers and thiazides (B coefficient=1.124(E-3); P=.009). A threshold C2 value of 700 microg/L yielded to a TC level of 5.2 mmol/L. This is the first study to report a correlation between C2 levels and TC. Although C2 explained a small fraction of TC variability, it is an independent predictor of TC in KT recipients on Neoral maintenance immunosuppression. A long-term C2 value under 700 microg correlates with better control of hypercholesterolemia.

Perineal hemangioma, anorectal malformation, and genital anomaly: a new association?

Two patients presented as full-term baby girls with anorectal and genital malformations with extensive perineal hemangiomas. The first patient had a vestibular anus with a perineal hemangioma involving the bladder, rectal, and vaginal walls. Skin ulcerations required a transverse loop colostomy for wound care. The vulva, urethral opening, and clitoris were deviated to the left, labia minora were absent, and the labia majora were abnormal. The second patient had an anus displaced anteriorly and deviated to the right. The external anal sphincter was hypertrophic on the left and atrophic on the right. Rectal examination showed agenesis of the right levator ani and a dentate line located at the skin level. She had a large perineal, sacral, vaginal, pararectal and retroperitoneal hemangioma and developed extensive skin ulcerations. She had only a hemiclitoris located to the left of the midline, near absence of labia minora, and hypertrophied labia majora. The urethra was displaced to the left and opened in the vestibule. Both patients had a spinal malformation (one with tethered cord and one with spina bifida) and a normal karyotype. Steroids and interferon allowed near-complete resolution of hemangiomas in both patients. The authors were impressed by the similarity of these two cases and could not find any previous description of this association.

The otolaryngologic manifestations of gastroesophageal reflux: when is a pH study indicated?

PURPOSE: The aim of this study was to evaluate the pertinence of pH studies for persistent ear, nose, and throat (ENT) symptoms and their eventual relationship to gastroesophageal reflux (GER). METHODS: Retrospective analysis was performed of age, reason for referral, pH study, treatment, and follow-up of patients with ENT symptoms suspected to have GER. RESULTS: Of 3,000 esophageal pH studies performed over 16 years, 105 children were referred for ENT symptoms by an otorhinolaryngologist to rule out GER. Mean age was 33 months; 65% were boys. Reasons for referral included (number and mean age): stridor (n = 31, 8 months), laryngomalacia (n = 18, 13 months), recurrent otitis (n = 12, 42 months), laryngitis (n = 16, 50 months), dysphonia (n = 14, 59 months), laryngeal papillomatosis (n = 8, 62 months), sinusitis (n = 5, 56 months), and dysphagia (n = 1). Overall, 41% of study results were positive: stridor (58%), laryngomalacia (61%), laryngitis (56%) and sinusitis (40%). Patients with otitis, dysphonia and laryngeal papillomatosis had GER in 1%, 14%, and 25%, respectively. Follow-up in the three larger groups of patients showed resolution of the ENT symptoms after medical treatment of the reflux in 83% of patients with stridor and reflux, 86% with laryngitis and reflux, and 80% with laryngomalacia and reflux. Four fundoplications were performed: one neurologically impaired patient, and four nonresponders. CONCLUSIONS: The authors recommend that a pH study be performed in children with stridor, laryngomalacia, laryngitis, and sinusitis when faced with failure of the usual treatment. However, a pH study does not seem as beneficial for recurrent otitis, dysphonia, or laryngeal papillomatosis.

The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: a single institution experience.

BACKGROUND/PURPOSE: The antenatal diagnosis of sacrococcygeal teratoma (SCT) is increasingly being made with fetal sonography. However, the natural history of SCT detected on routine obstetric sonogram is not well defined. METHODS: A retrospective chart review of 21 fetal SCT diagnosed on routine sonography at Hospital Sainte-Justine between 1980 and 1997 were performed. The patients' clinical and sonographic characteristics, prenatal, and perinatal outcomes were examined. Prognostic criteria were identified by correlating patients' characteristics with outcome. RESULTS: In utero mortality rate was 19% and perinatal mortality rate was 14%. The incidence of premature labor was 50%. Of the various criteria examined as an independent variable, the presence of a solid tumor was found to be a important negative prognostic factor with a 67% incidence of death in utero and an overall mortality rate of 100%. Patients with new onset polyhydramnios were at significant risks for premature labor (100%). All of the perinatal deaths were attributable to tumor rupture. CONCLUSIONS: The course of sacrococcygeal teratoma diagnosed on routine sonograms is associated with a higher-than-expected incidence of prenatal and perinatal complications. Close antenatal follow-up for new onset polyhydramnios and the presence of a completely solid tumor will help optimize patient counseling and treatment.

Falls from heights among children: a retrospective review.

BACKGROUND: Falls are a major cause of emergency room visits and admissions in pediatric hospitals. METHODS: To better understand the epidemiology of falls from height and develop prevention strategies, the authors reviewed all admissions after a fall at a single institution from 1994 to 1997. Inclusion criteria are falls from a minimum height of 10 feet. RESULTS: Of 1,410 patients admitted after a fall, 64 patients including 45 boys and 19 girls with a mean age of 7.4 years (range, 1 to 18) are included in this study. Fifty (78%) children fell from 20 feet or less (two stories) and 14 (22%) from height greater than 20 feet. Patients mainly fell from balconies (n = 15), windows (n = 13), trees (n = 9), roofs (n = 6), stairs (n = 6), diving board (n = 3) and miscellaneous (n = 12). Over 60% of falls occurred in private houses and during the summer months. Fifty-five patients (86%) sustained only one system injury, two patients had no significant injury, and seven patients had multisystem injury. Major injuries included head trauma (39%), musculoskeletal (34%), abdominal (12%), maxillofacial (8%), and spine (6%). A surgical intervention was required for 43% of intracranial trauma, 39% of musculoskeletal injuries, 60% of facial trauma, and 50% of spine fractures. Mean length of stay in hospital varied according to the injured system. The overall survival rate is 98% with only one death after a fall greater than 50 feet. CONCLUSIONS: Although rarely mortal, falls from height carry a significant morbidity and are costly to the health care system. To decrease the occurrence of injuries caused by falls, strategies should include awareness campaigns, parent's education about the mechanisms of falls, increase parenteral supervision during playing activities, and legislative measures to ensure the safety of windows and balconies before the onset of summer.

Abdominal injuries associated with thoraco-lumbar fractures after motor vehicle collision.

PURPOSE: The goal of the current study was to evaluate the risk of intraabdominal injury in children who sustained spinal fractures in a motor vehicle collision (MVC). METHODS: Between 1980 and 1999, 48 patients, 24 girls and 24 boys, with a mean age of 12.8 years (range, 4 to 17) were reviewed. Twenty-nine were rear seat passengers, 12 front seat, and 7 unknown. Fifty-eight percent wore a seat belt. Thirty fractures involved the lumbar spine, 12 the thoracic, and 6 combined. Computed tomography (CT) scan, abdominal ultrasound, and peritoneal lavage were used to screen for abdominal injuries. RESULTS: Twenty-two of 48 patients had an intraabdominal injury. Eighteen (38%) required an early (<24 hours; n = 12) or delayed (n = 6) therapeutic laparotomy. Fourteen patients were rear seat passengers, 15 wore a seat belt, and 13 had an abdominal wall ecchymoses (AWE). They were 17 lumbar fractures (13 Chance) and one thoracic. The most common findings at laparotomy were hollow viscus injury (n = 12), mesenteric tear (n = 9), and solid organ injury (n = 8). Seventy-two percent of patients presenting with a lumbar fracture and AWE needed a therapeutic laparotomy. The overall survival rate was 98% with only 1 death. The mean hospital stay was 22.4 days. In this study, 38% of patients presenting with a spinal fracture required laparotomy, 68% of whom had simultaneous lumbar fracture and AWE. CONCLUSION: In light of these results, the authors propose that laparoscopy or laparotomy should be strongly considered in patients sustaining lumbar fracture and AWE after MVC. J Pediatr Surg 36:760-762.

Total anomalous pulmonary venous return complicating portoenterostomy for biliary atresia.

Cardiovascular anomalies such as absent inferior vena cava and preduodenal portal vein are reported in cases of biliary atresia and make hepatic portoenterostomy a technical challenge. The authors present the case of a severe cardiac anomaly that significantly altered the functional outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic portoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibiotic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (> 90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transplantation is contemplated if the patient survived. Within 48 hours postoperatively, his hepatic function had improved drastically. He became afebrile, had an improved appetite and weight gain, and was finally discharged 203 days after admission. One year later, he is thriving and remains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TAPVR had a significant effect on the functional outcome of the portoenterostomy.

Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers of MEN2 syndrome.

BACKGROUND/PURPOSE: Although medullary thyroid carcinoma (MTC) can occur sporadically, in the pediatric population it is most often associated with the multiple endocrine neoplasia syndrome (MEN type 2). Traditional screening was based on evaluation of basal and stimulated serum calcitonin levels. The recent cloning of the MEN2 gene on the RET proto-oncogene of chromosome 10 now allows for testing of gene carrier status in individuals at risk who could benefit from prophylactic treatment. The current study was undertaken to determine the appropriate age for safe total prophylactic thyroidectomy. METHODS: Over a 16-year period, 12 patients with a family history of MEN2A and one with a MEN2B underwent total thyroidectomy and central neck dissection without parathyroid autotransplantation. Four patients (31%) were treated previously for Hirschsprung's disease. RESULTS: In seven patients (mean age, 11.8 years) undergoing biochemical screening for diagnosis, multifocal MTC and C cell hyperplasia (CCH) were found in all the resected specimens. Of six patients identified with genetic screening (mean age, 9.1 years), two had elevated stimulated calcitonin levels, one (age 14) had evidence of MTC, and one (age 6) had CCH. Four patients with normal calcitonin levels had no evidence of MTC (ages 6, 8, 10) but there was one occurrence of CCH (age 11). No permanent postoperative hypoparathyroidism or recurrent laryngeal nerve damage occurred in this series. With a mean follow-up of 4 years (range, 1 to 14 years), the overall disease-free survival is 100%. CONCLUSIONS: From this study the authors conclude that total thyroidectomy can be performed safely in children and should be the treatment of choice in patients with a family history of MEN2A carrying a germinal RET mutation even if the serum basal or stimulated serum calcitonin level is normal. Total thyroidectomy should be performed as early as 5 years of age before the occurrence of CCH or MTC.

Pleuropulmonary blastoma: a rare pathology with an even rarer presentation.

BACKGROUND: Pleuropulmonary blastoma is among the rarest tumors of childhood. Three types have been described: cystic, solid, and mixed. To date, bilateral disease has not been documented. METHODS AND RESULTS: A 5-week-old girl presented with a history of fever. Chest x-ray showed bilateral diffuse cystic lesions. Bowel obstruction developed that required laparotomy. Multiple small bowel polyps were resected. The patient was readmitted 4 months later with deteriorating respiratory status. She underwent sequential thoracotomies for resection of multiple bullae under high-frequency oscillatory ventilation. Small bowel polypectomies were again required because of obstruction. Lung lesions were compatible with pulmonary blastoma but could not be correlated with intestinal polyposis. Bilateral cystic renal lesions were seen on ultrasound scan. Her disease progressed, despite chemotherapy, with the appearance of metastatic iris lesions. She again underwent laparotomies for multiple recurrent generalized small bowel polyps that were causing obstruction. Expanding renal cysts affected kidney function, and she died at 14 months of age. CONCLUSIONS: The rare association between pleuropulmonary blastoma and Wilms' tumor or nephroblastomatosis is known but rarely reported. Lacking pathological evidence, we can only speculate that this was the case. We have been unable to demonstrate any histological association between the renopulmonary and digestive lesions. Despite many unanswered questions, we are likely dealing with a "syndrome" of sorts with a dire outcome, despite aggressive treatments.

Vascular complications after pediatric liver transplantation.

From February 1986 to July 1994, 81 hepatic transplantations were performed in 73 children, with an overall patient survival rate of 83%. Forty-two patients received whole-liver grafts (WLG) and 39 had reduced-size grafts (RSG). The mean patient weight was 19.7 kg, with 29 patients weighing less than 10 kg. Seventeen vascular complications (21%) occurred in 13 children: 8 (10%) had hepatic artery thrombosis (HAT), 5 (6%) had portal vein thrombosis (PVT), 1 had both HAT and PVT (1%), and 3 (4%) had aortic conduit perforation (ACP). There was no significant difference in the incidence of HAT between RSG (5%) and WLG (14%) or between children weighing less than 10 kg (10%) and those weighing more than 10 kg (10%). The site of arterial reconstruction, end-to-end to the recipient common hepatic artery or end-to-side to the infrarenal aorta, had no significant effect on the occurrence of HAT (7% v 8%), but HAT occurred in 2 of 6 cases (33%) in which an aortic conduit was used. PVT documented in 5 cases (6%) was associated with technical complications (2), preduodenal portal vein (2), and a circulating cardiolipid antibody (1), and required thrombectomy, with no graft loss. Combined HAT and PVT was found in one patient 2 years postretransplantation for HAT. Although graft function is normal, portal hypertension persists. The aortic conduit, used in six patients, led to arterial perforation (3), HAT (2), and death (2). Of the 8 cases of HAT, 1 was diagnosed during autopsy and 7 occurred within 30 days and required retransplantation (6) or thrombectomy with rearterialization (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Chromosomal anomalies in newborns with omphalocele.

Omphalocele is the most common congenital abdominal wall defect; its reported incidence is 1 in 4,000 to 5,000 live births. With large defects, the liver is a median organ and lies within the sac (extracorporeal liver [ECL]). With small defects, only bowel or stomach is found outside the abdominal cavity (intracorporeal liver [ICL]). The goal of this study was to determine whether a relationship exists between the sac contents or the timing of diagnosis and the incidence of chromosomal abnormalities or survival among fetuses and newborns with omphalocele. From 1985 to 1995, 83 cases of omphalocele were managed at the authors' institution. In 50 cases the diagnosis was made using prenatal ultrasonography. All patients underwent fetal cardiac echography and amniocentesis. Twenty-four pregnancies were terminated electively because of severe associated anomalies. Of the 59 live births, 41 patients (69%) survived. The incidences of cardiac, chromosomal, and other anomalies were 24% (14), 10% (6), and 21% (16), respectively. Omphalocele with ICL is associated with a better survival rate than omphalocele with ECL (82% v 48%; P < .01) despite the significantly higher rate of karyotype abnormalities (16% v 0%; P < .05). The prognosis was poorer for patients with prenatally diagnosed omphalocele than for those with a postnatal diagnosis (mortality rate, 42% v 21%) because the former group had a higher percentage (70% v 9%) of ECL. Although the incidence of cardiac anomalies was similar for the ECL and ICL groups (33% v 18%), the former had more complex malformations. Death usually occurred in newborns who had neonatal respiratory distress owing to prematurity, or in those with chromosomal or cardiac anomalies. Chromosomal anomalies occurred mainly in cases of small omphaloceles that contained gut only, and it was the major cause of death among this group. In ECL cases, survival was primarily affected by the associated complex cardiac anomalies.

Biliary tract complications in pediatric orthotopic liver transplantation.

Biliary tract complications are reported in 15% to 30% of orthotopic liver transplantations (OLTs). Since 1986, 53 OLTs were done in 48 children with a mean age and weight of 5.3 years and 18.9 kg, respectively. Twenty-seven transplantations (51%) were reduced liver grafts (RLG) and 26 (49%) were whole liver grafts (WLG). Since 1988, 70% of transplantations have been RLG. Choledochocholedochostomy (mean weight, 25 kg) with a T-tube (CC) or choledochojejunostomy (CJ) (mean weight, 14.5 kg) were done in 24 (45%) and 29 (55%) cases, respectively. The overall mortality was 19% but none of the deaths were related to biliary problems. There were 13 biliary tract complications (24.5%) in 11 patients including 7 leaks, 5 obstructions, and 1 intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4), to the Roux-en-Y loop (2), and to the transection margin of a RLG (1). Obstruction was documented in 5 cases with none associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (2) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct after a left lateral segment graft and an anastomotic stricture 46 months after OLT. The last patient developed a vanishing bile duct syndrome 4 months posttransplant and is awaiting retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. Neither the type of grafts (RLG 25.9% v WLG 23.1%) nor the type of biliary reconstruction (CC 25% v CJ 24%) influenced the rate of biliary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

[Biliary complications in pediatric liver transplantation]. / Complications biliaires en transplantation hépatique pédiatrique.

Biliary tract complications are reported in 15% to 20% of orthotopic liver transplantations (OLT). Since 1986, 55 OLT were done in 50 children with a mean age and weight of 5.6 years and 18.8 kg respectively. There were 28 (51%) reduced liver grafts (RLG) and 27 (49%) whole liver grafts (WLG). Since starting using RLG in 1988, 70% of transplantations have been RLG. Choledochocholedochostomy with a T-tube (CC) or choledochojejunostomy (CJ) were done in 25 (45%) and 30 (55%) cases, respectively. The overall mortality was 19% with one death related to biliary problems. There were 14 biliary tract complications (25%) in 12 patients including 7 leaks, 6 obstructions and one intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4, to the Roux-en-Y loop (2) and the transection margin of a RLG (1). Obstruction was documented in 6 cases, none of which were associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (3) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct and an anastomotic stricture 46 months after OLT. One patient developed a vanishing bile duct syndrome 4 months post-transplant and died while waiting for retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. The rate of biliary complications was not influenced by neither the type of graft (RLG : 25% vs WLG : 25,9%) nor the type of biliary reconstruction(CC : 28% vs CJ : 23%).(ABSTRACT TRUNCATED AT 250 WORDS)