Search

Liver transplantation has improved the survival of children with end-stage liver disease, and is frequently the only curative treatment for these patients. More infants are being given liver transplants because of success with older children, but many transplant centers do not accept infants, even if they have life-threatening liver disease. The outcomes of liver transplantation in 17 infants under one year of age are reported. Of the 17 patients, 5 received more than one graft and a total of 26 liver transplantations were performed. Eleven patients survived to be discharged from the hospital, and 10 survived a year or more after hospital discharge. Four of the six patients who died following transplantation had required hospitalization prior to surgery. Surgical complications included blood clots in the hepatic artery (of the liver). Bacterial infections were common; two patients died as a result of complications associated with viral infections. Eleven patients experienced 18 episodes of graft rejection. One patient with rejection required a second transplant, but nine responded successfully to medical treatment. Although several patients had impaired kidney function following surgery, it was similar to their pretransplant status. Liver function is normal in all, but two of the surviving patients. Three of the older survivors have entered primary school and have no known deficits, while a fourth is developmentally delayed. The findings suggest that liver transplantation is a feasible treatment for children under one year of age, but should only be performed in medical facilities which are capable of managing these patients. The 64.7 percent survival rate of these infants, while not out of line, is less than the 75.8 percent survival of all 139 children who received liver transplants at this facility during a 5-year period. Careful selection of candidates and preoperative nutritional boosting are highly desirable. Further studies are needed to determine the risk factors of liver transplantation, which are specific to infants. (Consumer Summary produced by Reliance Medical Information, Inc.)

A major drawback in pediatric liver transplantation is the severe shortage of appropriately sized livers. Between 25 and 30 percent of children waiting for liver transplants die before an appropriate donor organ can be found. When liver transplantation possible, it offers the hope of long-term survival. A report is presented of 141 transplantations performed on 117 children using 54 reduced-size grafts. Ten children were under one year of age; 63 were between l and 3 years old; 21 were between 3 and 6; and, 23 were between 6 and 14 years old. The most common indication for transplant was biliary atresia, a congenital absence of one or more of the biliary structures, which affected 86 patients. Reduced-size livers were used in both urgent and elective cases. There were 83 full-size livers and 54 reduced-size livers transplanted; 4 were considered other technical variants. The one-year survival rate for the entire group was 77 percent; for the full-size liver recipients it was 82 percent, and for the reduced-size liver recipients it was 68 percent. Graft loss occurred with 26 (31 percent) of the full-size livers, and 24 (44 percent) of the reduced-size livers. Of the 74 children who had functioning livers six months after transplant, 67 percent had completely normal liver function tests, and 14 percent had only slightly abnormal liver tests. Use of this technique has reduced the mortality of children on the waiting list to 14 percent. It is concluded that the technique of reduced-size liver transplantation is safe and reliable and is recommended for treatment in urgent and elective cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Modern anaesthetic techniques have resulted in the clinical presentation of malignant hyperthermia to be more often indolent and/or insidious than truly fulminant, as previously known in the anaesthetic community. We present four recently referred cases to illustrate this point: one late-onset case, two patients with slowly progressive hypercapnia as the sole sign and a fourth patient with postoperative myalgias and elevated creatine kinase. We also discuss the reasons for the shift in typical clinical presentation. The more insidious character of malignant hyperthermia is most likely due to the lower triggering potency of modern volatile anaesthetics, the mitigating effects of several intravenous drugs (neuromuscular blocking agents, alpha 2 adrenergic receptor agonists, beta-adrenergic blockade) or techniques (neuraxial anaesthesia) and the routine use of end-tidal CO2monitoring leading to the early withdrawal of triggering drugs. Awareness among anaesthetists of this change in presentation is important since the clinical diagnosis is often more doubtful and, if corroborative evidence is not sought, the diagnosis may be delayed or missed altogether.

Abstract

Abstract: 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.

In a series of 51 children presenting with an accidental caustic burn, symptoms were analysed for their predictive value of significant i.e. necrotizing oesophageal lesions (grade II or III). For the whole group, the incidence of significant oesophageal lesions was 37%. Vomiting and/or respiratory distress were associated with high incidence of significant oesophageal burn (84% and 75% respectively). The particular location of each caustic burn was analysed for its association with caustic burns at other anatomical sites. Of the 18 patients with a laryngeal burn, 72% also had a grade II or III oesophageal burn. The 19 patients with a grade II or III oesophageal burn and the 18 patients with a laryngeal burn, all had lesions at other sites. In the group of 19 patients with a caustic lesion limited to one site, only 1 patient showed mild oesophagitis (grade I) without late sequelae.

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.

Neonatal cleft lip repair is no longer an unusual procedure and several series have been published to date. We present our 3-year experience from the anesthesiologist's point of view. Preoperative evaluation, perioperative management, and postoperative problems are described. A thorough preoperative evaluation in order to exclude associated malformations (especially cardiac) and experience in neonatal anesthesia are essential for the sake of safety.

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.

Copyright of Acta Endoscopica is the property of Lavoisier and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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.

AbstractThe Brussels series ol 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.

OLT is generally associated with important cardiovascular changes occurring during the vascular clamping and at the time of revascularization of the newly transplanted liver. In our series of 60 OLT performed in 52 children, the IVC clamping is generally followed by a fall in the PAP and the PWP, the SBP remaining fairly stable in half of the cases. This good tolerance is confirmed by the fact that a veno-venous bypass was used only in 3 instances and vasopressive drugs were needed in only 6 cases. At unclamping, a severe decrease in SBP is associated with a sharp rise in PAP and PWP in most of the cases, whereas some hypovolemic patients decrease their SBP, PAP and PWP. Therefore, we believe that adequate fluid replacement under careful monitoring of the filling pressures provides a good hemodynamic stability in most of the children during the different stages of OLT.