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Living-related liver transplantation was developed in the context of deceased donor organ shortage, which is particularly acute for pediatric recipients. This retrospective study analyzes the surgical technique and complications in the first 100 pediatric liver transplantation using left segmental liver grafts from living donors, performed at Saint-Luc University Clinics between July 1993 and April 2002. Pre-operative evaluation in donors and recipients, analysis of the surgical technique, and postoperative complications were reviewed. After a median follow-up period of 2526 days, no donor mortality was encountered, with a minimal morbidity and no long-term sequelae. At one and five yr post-transplantation, the actuarial patient survival rates were 94% and 92%, the corresponding figures being 92% and 89% for graft survival. The incidences of portal vein and hepatic artery thromboses, and of biliary complications were 14%, 1%, and 27%, respectively. Living-related liver transplantation in children constitutes an efficient therapy for liver failure to face the increased demand for liver grafts. Donor morbidity was kept to acceptable incidence, and surgical technique in the recipient needs to be tailored to minimize postoperative complications.

The Paediatric Liver Transplant Program at Saint-Luc University Clinics constitutes a substantial single centre experience, including 667 transplantations performed between March 1984 and April 2003, and the history of this program reflects the tremendous progress in this field since twenty years. Liver transplantation in children constitutes a considerable undertaking and its results depend on multiple, intermingled risk factors. An analysis of the respective impact of several surgical and immunological parameters on patient/graft outcome and allograft rejection after paediatric liver transplantation showed a significant learning curve effect as well as the respective impact of pre-transplant diagnosis on survival and of primary immunosuppression on the rejection incidence. The introduction of living related liver transplantation in 1993 not only permitted to provide access to liver replacement in as many as 74% more candidate recipients, but also resulted in better graft survival and reduced retransplantation rate. The results of a recent pilot study suggest that steroid avoidance is not harmful, and could even be beneficial for paediatric liver recipients, particularly regarding growth, and that combining tacrolimus with basiliximab (anti-CD25 chimeric monoclonal antibody) for steroid substitution appears to constitute a safe alternative in this context. The long-term issues represent the main future challenges in the field, including the possibility of a full rehabilitation through immunosuppression withdrawal and tolerance induction, the development of adolescence transplant medicine, and the risk of early atherogenesis in the adulthood.

The authors present the results of a single centre study of 587 liver transplants performed in 522 adults during the period 1984-2002. Results have improved significantly over time due to better pre-, peri- and post-transplant care. One, five, ten and fifteen year actuarial survivals for the whole patient group are 81.2; 69.8; 58.9 and 51.2%. The high incidence of de novo tumors (12.3%), of cardiovascular diseases (7.5%) and of end-stage renal function (3.6%) should be further incentives to tailor the immunosuppression to the individual patient and to direct the attention of the transplant physician to the long-term quality of life of the liver recipient.

We report a case of respiratory distress in the post anesthesia care unit following general anaesthesia for a dilatation and curettage related to miscarriage in a 32-year-old woman. The preoperative physical examination showed no abnormalities except for the presence of dry cough during the preceding two or three days. A few minutes after her arrival in the PACU, the patient developed hyperthermia till 40.6 degrees C, cough, polypnea and oxygen desaturation (SpO(2): 82% on FiO(2): 40%). A thoraco-abdominal angioscanner showed pulmonary basal condensations and a thrombosis of the right ovarian vein. The patient had to be transferred to the intensive care unit where she remained intubated and ventilated during 13 days because of a Haemophilus influenzae pneumonia.

Preparing a child for a short stay in the hospital involves the evaluation of its health status and its psychological preparation but also providing clear instructions for perioperative care to its parents. The success depends on the professionalism, flexibility and teaching skills of a dedicated team.

We report the anaesthetic management of a patient suffering from an ischaemic arteriopathy of the CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy) type. The anaesthetic implications of this pathology are discussed. By analogy with other cerebral arteriopathies, the aim of our management was to keep mean arterial blood pressure and end-tidal carbon dioxide so as to prevent any cerebral ischaemic or vasospastic phenomenon. We preserved the cerebral venous return by avoiding excessive head-down position. We used a balanced anaesthetic technique because it allows easier titration of the depth of anaesthesia with regard to mean arterial pressure. There is no contraindication to the use of loco-regional anaesthesia.

Purpose: To describe the modification of the ropivacaine (R) pharmacokinetics produced by the addition of epinephrine (E)., Methods: After Institutional Review Board approval, 18 ASA I boys received a caudal block (1 mL x kg(-1)) with either plain 0.2% R (Group E-) or with 0.2% R containing E (5 microg x mL(-1); Group E+). Venous blood samples were taken at zero, 15, 30, 60, 90, 120, 180, 240, 420, 720, 1440 min after caudal injection. Total R concentration in plasma was determined by high pressure liquid chromatography. Maximal concentration (C(max)) and time to peak concentration (T(max)) were obtained from the data, terminal half-life (T(1/2z)), clearance (Cl) and volume of distribution (Vd) were estimated by a non-compartmental approach. Subsequently, in order to determine the absorption rate (Ka) and to reduce to number of blood samples, 25 other children, receiving plain R and another group of 25 receiving the E solution were studied using a population approach (NONMEM). A one compartment model with first order absorption was used. The effect of weight, age and E on Cl, Vd and Ka was estimated., Results: C(max) was significantly lower in Group E+ (0.93 mg x L(-1) +/- 0.29 vs 0.61 mg x L(-1) +/- 0.28, P = 0.05) and T(max) occurred later (124 min +/- 53 vs 47 min +/- 16, P = 0.003). Weight was a significant covariate for Cl and Vd while E significantly slowed R Ka [Group I Ka 0.025 min(-1) [coefficient of variation (CV) 21%] vs 0.078 min(-1) (CV 25%) in Group II]., Conclusion: The addition of E significantly modifies the pharmacokinetics of R injected caudally.

Background: The original method of liver transplantation (LT) included recipient inferior vena cava (IVC) resection and the use of extracorporeal venovenous bypass (VVB). Refinements in technique permit transplantation to be done with IVC preservation and without VVB use., Material and Methods: Between November 1993 and November 2000, 202 consecutive grafts were performed in 188 adults (>/=16 years of age). Twelve patients (6.4%) received two and three retransplants (re-LT). Split grafting was performed 19 times (19 of 202 grafts, 9.4%). Risk factors included United Network of Organ Sharing status I (n=30, 16%), previous right upper abdominal surgery (n=32, 17.1%), caudate lobe encirclement of IVC (n=65, 32.2%), IVC (n=24, 11.9%), and splanchnic venous modification (n=58, 30.9%), transjugular intrahepatic portosystemic stent shunt (n=34, 16.8%), giant (>5 kg) liver tumor (n=6, 3%), septic necrosis of the caudate lobe (n=1, 0.5%), and previous cavocaval (n=13, 6.4%) or classical LT (n=5, 2.5%)., Results: IVC preservation, avoidance of IVC cross clamping and of VVB use were possible in 98.9%, 93%, and 99.5% of 183 primary LT and in 89.5%, 84.2%, and 89.5% of 19 re-LT. Temporary portocaval shunting was never applied. Perioperative mortality was 1.2%. There was no allotransfusion in 73 (36%) grafts and 45 (22%) patients were immediately extubated. Permanent hepatic vein and caval problems were encountered in three (1.5%) grafts. One patient needed stent placement to treat IVC stenosis. Actual 3- and 12-month patient survival for whole, re-LT, and right-lobe split LT groups were 94.7%, 94.1%, 94.7%, 88.2%, 94.1%, and 89%. Three-month graft survival rates for these groups were 92.6%, 94.7%, and 84.2%., Conclusions: LT with IVC preservation and without VVB use and portocaval shunting is possible in nearly all primary transplants and in the majority of re-LT.

Due to its hemodynamic properties and ease of administration (quick induction, rare cough or laryngospasm), Sevoflurane has rapidly become the agent of induction of choice in pediatric patients. However, it can induce troublesome excitation phenomena during induction and awakening and it could have an epileptogenic effect. The mechanisms and strategies to reduce those drawbacks are reviewed.

Simple techniques of local analgesia applicable in children either in the medical office or in the emergency room by non-specialist practitioners are described: infiltration of the skin, topical anaesthesia of the skin, whether intact or damaged, topical anaesthesia of the mucosae.... In addition safety rules, early signs and treatment of local anaesthetics intoxication are underlined.

The advantages of rapid induction of and emergence from sevoflurane anaesthesia may be more than offset by the frequent occurrence of agitation during induction and recovery, and a possible epileptogenic effect. The mechanisms and possible strategies to prevent these drawbacks are reviewed, on the basis of the most recent literature.

Purpose: To show the use of the short acting opioid remifentanil for labour analgesia when epidural analgesia is considered to be contraindicated., Clinical Features: After Ethics Committee approval and informed consent, six patients (36-40 wk gestation), in whom epidural analgesia was considered contraindicated (women refusing regional analgesia, presenting with coagulation or platelet abnormalities or sepsis) benefited from patient-controlled intravenous analgesia (PCIA) with remifentanil. The Abbott Lifecare patient-controlled analgesia (PCA) pump with remifentanil 50 microg x ml(-1) was set to deliver remifentanil continuous background infusion of 0.05 microg x kg(-1) x min(-1) and 25 microg boluses with a five minutes lockout period. The PCIA was started when the parturients experienced regular painful contractions (cervical dilatation of at least 4 cm) and stopped just before delivery (cervix fully dilated). Maternal monitoring included non-invasive blood pressure measurements, heart rate, percutaneous arterial oxyhemoglobin saturation and respiratory rate. Percutaneous fetal heart rate was continuously monitored. All patients remained alert or sleepy but easily arousable and were satisfied with their analgesia. No particular side effects have been noticed. Apgar scores were between 6 and 10., Conclusion: Remifentanil PCIA combining low continuous background infusion and small bolus doses is an alternative when epidural analgesia in labour is contraindicated. Under careful anesthesia monitoring, the technique seems to be safe for both mother and baby, at least when delivery occurs at or near the normal term of pregnancy.

We evaluated the use of remifentanil administered as a component of an inhalation or of a Target Controlled Infusion (TCI) anesthetic technique during outpatient oral surgery. Sixty-three unpremedicated patients undergoing removal of four impacted third molars participated to this prospective, randomized study. Anesthesia was induced with Propofol and Rocuronium. Remifentanil 1 microgram.kg-1 i.v. was given over 30 s followed by a continuous infusion reduced from 25% each time a tooth was removed (0.25-->0.0625 microgram.kg-1 min-1). Anesthesia was maintained with Desflurane (group D, n = 31) (end-tidal concentration 4-6%) or Propofol (group P, n = 32) (initial infusion TCI 8 micrograms.ml-1 reduced to 2-3 micrograms.ml-1 after intubation). Corticosteroids, a non-steroidal anti-inflammatory drug (NSAID) (Diclofenac) and a partial mu agonist drug (Tramadol) were administered i.v. during the procedure to prevent early postoperative pain. Recovery time, postoperative pain, recovery of cognition and nausea or vomiting were also evaluated during the first six postoperative hours. Overall mean systolic blood pressures and heart rate were similar in the two groups during surgery. Mean times to extubation and to recall of birth-date and room number were also similar. The quality of awakening was good in the two groups. Most patients complained of moderate pain or had no pain during the first six postoperative hours. The incidence of nausea and vomiting was similar in both groups. No other side effect was observed. These data suggest that the association of Remifentanil, Methylprednisolone, Diclofenac and Tramadol is an useful technique in ambulatory oral surgery in two comparable anesthetic regimens.

The Brussels series of living related liver transplantation (LRLT) in 77 children (< 15 years) is reviewed. Median (range) recipient age at liver transplantation was 1.1 year (0.4-13.1). The main indication for LT was biliary atresia in 55/77 cases (71%). The living-related donor was one of the parents in 74 instances. Hepatic segments 2-3 (n = 67) or 2-3-4 (n = 10) were implanted orthotopically, with a median (range) graft weight to recipient body weight ratio of 3.17% (0.91-8.08). No severe complications or significant long-term sequelae were encountered in the living donors. One and five year survival rates were 92% and 89% for the patients, and 90% and 86% for the grafts, respectively. The retransplantation rate was 2/77 (2.6%), the indication being chronic rejection in both instances. In conclusion, LRLT is now a validated procedure in the living donors as well as in pediatric recipients with chronic or acute liver diseases. In the current context of organ shortage, it provides a valuable alternative to cadaveric LT.

Ehlers-Danlos syndrome type VIIc is characterized by altered tensile strength of connective tissue. Several severe complications exist but skin fragility is the origin of perioperative morbidity during routine procedures. We describe the difficulties encountered during the anaesthetic management of a child suffering from the disease, and suggest special care advices to avoid any skin injury.

Fifty children (2-9 years) scheduled for tonsillectomy were enrolled in a double-blind randomized prospective study to compare postoperative analgesia provided with propacetamol/paracetamol (acetaminophen) or tramadol. A standard anaesthetic technique was used; each patient received sufentanil 0.25 microg kg(-1) intravenously followed with either i.v. propacetamol 30 mg kg(-1) or tramadol 3 mg kg(-1) before surgical incision. For postoperative analgesia, each child received either tramadol drops (2.5 mg kg(-1)) or paracetamol (acetaminophen) suppositories (15 mg kg(-1)), 6 and 12 h after surgery the first day and three times a day during postoperative days 2 and 3. This dosage of paracetamol is lower than the current recommended dosage, which is 40 mg kg(-1) loading dose followed by 20 mg kg(-1) 8 h(-1). Rescue medication consisted of i.v. diclofenac (1 mg kg(-1)) during the first six postoperative hours and oral ibuprofen (6-9 mg kg(-1)) afterwards. Postoperative pain scores (Children's Hospital of Eastern Ontario Pain Scale) in recovery, numerical pain scale in the ward and at home, and rescue analgesic use were significantly lower in the tramadol group. No serious adverse effects were observed.

Purpose: To report the perioperative management of anesthesia and analgesia in a child presenting with the association of multiminicore myopathy (MMM) and anhydrotic ectodermic dysplasia (AED)., Clinical Features: An eight-year-old girl was admitted for elective orthopedic surgery of the lower limbs. AED is a congenital dermatosis characterized by the absence of sweating and subsequent problems with thermoregulation; in addition, maxillary hypoplasia and abnormal teeth can render intubation difficult. MMM is a rare congenital myopathy characterized by proximal muscle weakness, stable in time or with a slow and progressive evolution. It can involve respiratory muscles and be associated with severe cardiomyopathy. Moreover, MMM shares some characteristics with Central Core Disease which is known to be associated with malignant hyperthermia. Since depolarizing muscle relaxants and halogenated agents could not be used, a combined propofol-based intravenous anesthesia with lumbar epidural analgesia was chosen. This combination provided stable anesthesia, smooth recovery and excellent analgesia during and after the operation, without complications. Temperature was monitored closely during surgery and in the postoperative period., Conclusions: The association of MMM and AED requires rapid distinction between hyperthermia secondary to anhydrosis and malignant hyperthermia. The management should provide a "trigger-free" anesthetic and optimal postoperative analgesia without sedation. If appropriate for the surgical procedure, a combination of general with regional anesthesia is particularly attractive in achieving these objectives.

Background: Sevoflurane is metabolized by cytochrome P450 and produces inorganic fluoride. The anhepatic phase of liver transplantation provides a useful tool to study the extrahepatic metabolism of drugs. The authors therefore studied the extrahepatic metabolism of sevoflurane by measuring the fluoride production in children receiving sevoflurane solely during the anhepatic phase of orthotopic liver transplantation., Methods: Children with end-stage liver disease undergoing orthotopic liver transplantation were studied. Anesthesia was provided with isoflurane, sufentanil, and pancuronium. In one group, isoflurane was replaced by sevoflurane as soon as the liver was removed from the patient and maintained until reperfusion of the new liver. Arterial blood samples were drawn at induction, before removal of the liver, 15 min and 30 min after the beginning of the anhepatic phase, at the unclamping of the new liver, and finally 60 and 120 min after the unclamping. Plasma fluoride concentrations were determined by ion-selective electrode., Results: No differences between the two groups (n = 10) regarding age, weight, duration of the anhepatic phase, or basal level of inorganic fluoride were found. The fluoride concentration increased significantly as soon as sevoflurane was introduced; it remained stable in the group receiving isoflurane. The peak fluoride concentration was also significantly higher in the first group (mean +/- SD: 5.5 +/- 0.8 microM (sevoflurane group) versus 1.4 +/- 0.5 microM (isoflurane group) P < 0.05)., Conclusions: These results demonstrate the existence of an extrahepatic metabolism of sevoflurane at least in children with end-stage liver disease.

Unlabelled: Children with Alagille's syndrome are at increased perioperative risk during orthotopic liver transplantation due to the cardiopulmonary abnormalities and the hemodynamic changes associated with this procedure. We studied 16 children with Alagille's syndrome who underwent 21 orthotopic liver transplantations. Peripheral pulmonary stenosis was present in all subjects. Right ventricular pressures were increased in 15 cases. Caval clamping resulted in a mean decrease of 15 +/-9 mm Hg in systolic blood pressure, 5 +/- 3 mm Hg in mean pulmonary artery pressure, and 4 +/- 3 mm Hg in central venous pressure. Systolic blood pressure decreased by 16 +/- 13 mm Hg, whereas mean pulmonary artery pressure and central venous pressure increased by 3 +/- 4 mm Hg and 1 +/- 4 mm Hg, respectively, at portal vein unclamping. There was no correlation between severity of pulmonary artery stenosis and hemodynamic changes. Veno-venous bypass used in four cases resulted in smaller hemodynamic changes. Time to extubation and duration of intensive care unit stay were unrelated to severity of pulmonary artery stenosis., Implications: Some children with Alagille's syndrome require liver transplantation. In our study, associated pulmonary artery stenosis did not dramatically increase perioperative risk. Veno-venous bypass decreased intraoperative hemodynamic changes in these patients.

Objective: To evaluate the impact of standardized operative and peri-operative care on the outcome of liver transplantation in a single center series of 395 adult patients., Method and Material: Between February 1984 and December 31, 1998, 451 orthotopic liver transplantations were performed in 395 adult patients (> or = 15 years) at the University Hospitals St-Luc in Brussels. Morbidity and mortality of the periods 1984-1990 (Gr I--174 pat.) and 1991-1998 were compared (Gr II--221 pat.). During the second period anti-infectious chemotherapy and perioperative care were standardized and surgical technique changed from classical orthotopic liver transplantation with recipients' vena cava resection (and use of veno-venous bypass) towards liver implantation with preservation of the vena cava (without use of bypass). Immunosuppression was cyclosporine based from 1984 up to 1996 and tacrolimus based during the years 1997 and 1998. Immunosuppression was alleviated during the second period due to change from quadruple to triple and even double therapy and due to the introduction of low steroid dosing and of steroid withdrawal, once stable graft function was obtained. Indications for liver grafting were chronic liver disease (284 pat--71.9%), hepatobiliary tumor (52 pat--13.2%), acute liver failure (40 pat--10.1%) and metabolic disease (19 pat--4.8%). Regrafting was necessary because of graft dysfunction (21 pat), technical failure (12 pat), immunological failure (18 pat) and recurrent viral allograft disease (5 pat); three of these patients were regrafted at another institution. Follow-up was complete for all patients with a minimum of 9 months., Results: Actuarial 1, 5 and 10 years survival rates for the whole group were 77.9%, 65.7% and 58.3%. These survival rates were respectively 77.3%, 69.7%, 62.5% and 73.2%, 59.6% 51.4% for benign chronic liver disease and acute liver failure; those for malignant liver disease were 80.6%, 44.3% and 36.7%. Early (< 3 months) and late (> 3 months) posttransplant mortalities were. 14.4% (57 pat) and 21.2% (84 pat). Early mortality lowered from 20% in Gr I to 9.4% in Gr II (p < 0.02); this was due to a significant reduction during the second period of bacterial (99/174 pat.--56.9% vs 82/221 pat.--37.1%), fungal (14 pat.--8% vs 7 pat.--3.2%) and viral (87 pat.--50% vs 49 pat.--22.2%) infections (p < 0.05) as well as of perioperative bleeding (92 pat.--52.9% vs 39 pat.--17.6%--p < 0.001). Late mortality remained almost identical throughout the two periods as lethal outcome was mainly caused by recurrent allograft diseases, cardiovascular and tumor problems. Morbidity in these series was important considering that almost, half of the patients had a technical complication, mostly related to bleeding (131 pat--33.2%) and biliary problems (66 pat--16.7%). Retransplantation index was 1.1 (54 pat.--14%). Early retransplantation mortality was 24%; it lowered, although not yet significantly, during the second period (8/25 pat.--32% vs. 5/29 pat.--17.2%)., Conclusion: Despite a marked improvement of results, liver transplantation remains a major medical and surgical undertaking. Standardization of operative and perioperative care, less haemorraghic surgery and less aggressive immunosuppression are the keys for further improvement.

The use of a cuffed endotracheal tube should no longer be limited by the age of the child but by his or her clinical condition (e.g. poor lung compliance). To prevent pharyngeal damage, overinflation of the cuff of the laryngeal mask airway should be avoided by inflating it with the minimum volume required to maintain an effective seal and by monitoring intracuff pressure if nitrous oxide is used. Percutaneous tracheostomy in children is still in the experimental stage. New and older devices to perform one-lung ventilation in children are also described.

Study Objectives: To compare the clinical and management aspects of tracheobronchial aspirated foreign body (AFB) removal in children and adults; to assess the influence of the operator's experience on the outcome of the procedure., Design: A retrospective review of a 20-year experience (from 1976 to 1996)., Setting: A 900-bed university hospital., Patients: Eighty-four children up to 8 years old (the child group) and 28 adult patients (the adult group)., Results: The peak incidence of foreign body aspiration occurred during the second year of life in the child group and during the sixth decade in the adult group. The symptoms at presentation were similar in both age groups, but the diagnosis was significantly delayed in the adults. The AFBs were lodged preferentially in the right bronchial tree only in the adults; a central location was predominant (but not at all exclusive) in the children. Atelectasis was more common in the adults, and air trapping was more common in the children. The most frequent procedure was rigid bronchoscopy; when a flexible bronchoscope was used, it was always in the adult patients. When the operator was less experienced, a failed first attempt at bronchoscopy and the need for a second procedure were significantly more frequent., Conclusions: At presentation, the symptoms seen with AFBs do not differ according to the age of the patient; however, the delay to diagnosis, the location of the AFBs, and the radiographic images differ between child and adult populations. The removal of AFBs in patients of all ages can be performed by the same operators. Because the outcome associated with these procedures improves when the operator is experienced, the removal of AFBs should be performed in medical centers that are capable of acquiring and maintaining the necessary expertise.

Between 1984 and 1996, the authors performed 499 liver transplants in 416 children less than 15 years old. The overall patient survival at 10 years was 76.5%. It was 71.3% for the 209 children grafted in 1984-1990; 78.5% for biliary atresia (n = 286), 87.3% for metabolic diseases (n = 59), and 72.7% for acute liver failure (n = 22). The 5-year survival was 73.6% for the 209 children grafted in 1984-1990 and 85% for the 206 grafted in 1991-1996. Scarcity of size-matched donors led to the development of innovative techniques: 174 children who electively received a reduced liver as a first graft in our center had a 5-year survival of 76% while 168 who received a full-size graft had a survival of 85% (NS). Results of the European Split Liver Registry showed 6-month graft survival similar to results obtained with full-size grafts collected by the European Liver Transplant Registry. Extensive use of these techniques allowed the mortality while waiting to be reduced from 16.5% in 1984-1990 to 10% in 1991-1992. It rose again to 17% in 1993, leading the authors to develop a program of living related liver transplantation (LRLT). The legal and ethical aspects are analyzed. Between July 1993 and October 1997, the authors performed 53 LRLTs with 90% survival. In elective cases, a detailed analysis was made of the 45 children listed for LRLT between July 1993 and March 1997 and the 79 registered on the cadaveric waiting list during the same period. Mortality while waiting was 2% and 14.5% for the LRLT and cadaveric lists, respectively. The retransplantation rate was 4.6% and 16.1% for LRLT and cadaveric transplants, respectively. Overall post-transplant survival was 88% and 82% for children who received a LRLT or a cadaveric graft, respectively. Overall survival from the date of registration was 86% and 70% (P < 0.05) for LRLT or cadaveric LT respectively. The 2-year post-transplant survival in children less than 1 year of age at transplantation was 88.8% and 80. 3% with a LRLT or cadaveric graft, respectively; patient survival after 3 months post-transplant was 95.8% and 91.9% for stable children waiting at home, 93.7% and 93.7% in children hospitalized for complications of their disease, and 89.5% and 77.7% for children hospitalized in an intensive care unit at the time of transplantation for children who received a LRLT or cadaveric graft, respectively. It is concluded that LRLT seems to be justified for multidisciplinary teams having a large experience with reduced and split liver grafting.

Purpose: The Marshall-Smith Syndrome (MSS) is a rare disease characterized by orofacial dysmorphism, failure to thrive, accelerated osseous maturation and mental retardation. It has anaesthetic implications due to upper airway problems and possible atlanto-axial instability. We present the perioperative problems (difficult intubation, airway obstruction) encountered in a child with MSS who underwent several anaesthetics during his first two years of life., Clinical Features: At birth, the child presented with asphyxia due to obstructive apnoea. His trachea was, therefore, intubated immediately. The morphological diagnosis of MSS was confirmed by the pathognomonic radiological appearance of the bones (bone age was eight months at the age of four days). Upper airway difficulty was caused by functional problems at the level of the hypopharynx (inspiratory collapse at the level of the velum palatinum), and was solved by the use of a nasopharyngeal airway (NPA) during the induction of anaesthesia and early postoperative period., Conclusion: The use of an NPA during both induction and recovery of anaesthesia may be particularly useful to prevent upper airway problems in children with MSS.

Seventy-seven hypospadias, selected from a series of 504 treated in our institution, were operated using the pediculated transverse "onlay" preputial graft. We try to demonstrate that this technique produces excellent functional and cosmetic results. Chordee can be corrected using extensive dissection of the urethral plate with association, if necessary, of a Nesbit type of dorsal plication. Complications rate, in our hands, is lower in comparison to the technique described by John Duckett, where the urethral plate is divided and a complete urethral tube is performed. We report 4 fistulas, one distal end breakdown of the repair and one postoperative hematoma that had to be revised. Because of this low complication rate, in many cases, the "onlay" pediculated graft replaces advantageously the Duckett type of repair.

Until recently, hypoxaemia was considered as a relative contraindication for liver transplantation. The hepatopulmonary syndrome associated with a right to left shunt of blood through the lungs is reversible in adults and children after correction of the cirrhosis by liver transplantation. However, concerns have been raised regarding the risks of anaesthesia in such hypoxaemic patients. Since the peroperative management of children undergoing liver transplantation and suffering from hepatopulmonary syndrome and severe hypoxemia has never been described, we report here our experience in seven children. Despite the fact that severe arterial desaturation was recorded throughout the procedure, no major complications were recorded peroperatively. The postoperative intubation time was 58 +/- 21 h, five children being extubated while still hypoxaemic. All seven patients reversed their hepatopulmonary syndrome after a mean postoperative period of 24 +/- 10 weeks. This shows that liver transplantation can be successfully achieved in severely hypoxaemic children and that postoperative correction of the right to left shunt is then obtained.

Two cases of gastroduodenal outlet obstruction caused by arteriomesenteric compression in children who have cerebral palsy are reported. Clinical symptoms of gastrointestinal obstruction include recurrent postprandial nausea and vomiting, upper abdominal distension, and pain. In such patients, multiple predisposing factors can contribute to the development of arteriomesenteric compression, including marked weight loss, supine position, and severe scoliosis. Upper gastrointestinal x-rays using barium contrast allow diagnostic confirmation. In our experience, this cause of acute gastroduodenal outlet obstruction may usually resolve after conservative treatment using a jejunal feeding tube passed beyond the compression, left lateral positioning, and renutrition.

Numerous hypospadias correction techniques were described in the literature. The technique varies according to the position of the meatus and the importance of the chordee. These new techniques and the care taken in the manipulation of the tissues tend to decrease complications like stenoses and fistulas, frequent in the long urethroplasty. The correction of hypospadias should conform to aesthetic and plastic surgery. More than 300 corrections were carried out in our service. Surgical technique varied in the long run. Currently, we choose as often as possible a correction in one time: release of the chordae and urethroplasty. Straightening of the penis is obtained on one hand by release of the cutaneous chordae and wide dissection of the hypoplastic urethral plate and one or more dorsal plications according to Nesbit are carried out if it proves necessary. In the distal, glandular and coronal forms, the correction is of type M.A.G.P.I. (meatal advancement, glanduloplasty). The complication rate is extremely low. The aesthetic and functional result is very satisfactory. In the proximal forms, Duckett technique is used only in the obligatory cases given the large number of complications. It is generally replaced by Onlay technique. The urethral plate is left in continuity and serves as support to the pedicled and vascularized flap. In the intermediate situations with a middle shaft hypospadias, Mathieu technique is again of application. The aesthetic and functional result of this type of surgery requires good knowledge and careful application of the technique adapted to each case.

Progressive familial intrahepatic cholestasis (PFIC) or Byler's disease is one of the most common forms of intrahepatic cholestasis of metabolic and genetic origin. Affected children progress to terminal cirrhosis before adulthood and at present the only curative treatment of PFIC is orthotopic liver transplantation (OLT). We present a retrospective review of 40 general anaesthetics administered in our hospital to 22 patients with PFIC undergoing various procedures. The clinical features of PFIC and the anaesthetic implications of chronic cholestasis in children (malnutrition, cirrhosis, portal hypertension, chronic hypoxaemia) are reviewed.

Varicocele is a benign pathology, for which the less invasive treatment is percutaneous embolisation. Literature reports results from 70 to 85% (1,2). Laparoscopic treatment seems to be a good alternative for patients suffering from a recurrence, or patients who can not be embolised. We report our experience concerning the first 13 patients treated with laparoscopic technic. In all 13 cases it was possible to practice the technic, and patients were cured.

Urethral duplication is a less frequent malformation. The Williams classification, which is currently admit, classifies them in sagital, hypospadias or complex and collateral duplications. They can be complete, incomplete or reduced to an incomplete sinus. The most severe cases are often accompanied by multi-abnormality syndromes. Clinical manifestations are various: asymptomatic, urinary infection, incontinence, double urinary stream,... We will report two new cases of incomplete epispadias duplication. The first case presents a purulent flow from the fistula, the second a frank epispadiac status. In both cases, corrective surgical treatment was performed, after a complete balance-sheet. The embryological, diagnostical and therapeutical aspects of the different forms of urethral duplication will be studied based on data from literature.

Five cases of acute allergic reaction to latex are reported. Four of those were diagnosed during surgery and presented anaphylactic shock. One was suspected before surgery by systematic screening and dramatic presentation was avoided. Increasing frequency of latex allergy is reported in the literature and is usually seen in patients with extensive neurosurgical or urological histories, or by peoples currently exposed to natural rubber products (medical staff). Systematic screening in history before surgery is important so that exposure to latex should be avoided in suspected cases.