Didier Moulin

Size reduction of the donor liver is a safe way to alleviate the shortage of size-matched organs in pediatric liver transplantation.

The development of pediatric liver transplantation is considerably hampered by the dire shortage of small donor organs. This is a very sad situation because in most experienced centers, liver replacement can offer a long-term hope of survival of more than 70% in a growing variety of pediatric liver disorders. The reported experience with 54 reduced-size grafts on a total of 141 transplants performed in 117 children between 1984 and 1988 demonstrates that the technique of reduced-size liver transplantation not only allows long-term survival but, in fact, offers the same survival hope with the same quality of liver function, regardless of the childs age and clinical condition. The prominent feature of our experience with the reduced liver concerns its deliberate use for elective cases. Seventy-seven per cent of the 30 children who electively received a reduced liver were alive 1 year after transplantation, as were 85% of the 62 children who received a full-size graft. There is no difference in the long-term survival rate of patients who received elective grafts, which is in the range of 75% with both techniques.

Liver transplantation in children less than 1 year of age.

Of 139 children who received an orthotopic liver transplant in our center between March 1984 and July 1989, a total of 17 patients (12%) had transplants before their first birthday (mean age 10.3 months; range 8 to 11). The mean weight was 7.3 kg (range 5.2 to 13). Nine retransplantations were performed in five children because of primary nonfunction (three children), hepatic artery thrombosis (four), or rejection (two). A reduced donor liver was used for 11 of 26 transplants. Baseline immunosuppression included cyclosporine, prednisone, and azathioprine with OKT3 or anti-thymocyte globulin for steroid-resistant rejection episodes. Survivors were discharged after a mean hospital stay of 47 days (range 22 to 87), and nonsurvivors died within a mean of 40 days (range 0 to 120). The 1 year actuarial survival rate was 64.7%, in comparison with 75.8% in the whole series. One patient died perioperatively, two died from primary nonfunction, one from adenovirus infection, two from rejection, and one from bone marrow aplasia. Eighteen rejection episodes, of which 11 were steroid resistant, occurred in 11 patients. Our series shows that liver transplantation can be successful in this age group.

Prevalence and treatment of silent gastro-oesophageal reflux in children with recurrent respiratory disorders.

Thirty-six infants and children presenting with recurrent respiratory disorders (RRD) as the sole clinical symptom including bronchial asthma (6), recurrent obstructive bronchitis with or without wheezing (18), chronic nocturnal cough (3), recurrent episodes of pneumonia (3), recurrent pharyngitis (3) and recurrent laryngitis (3) were investigated for associated gastro-oesophageal reflux (GER) by oesophagram, endoscopy and continuous 24 h pH monitoring of the distal oesophagus.The pH monitoring criteria were selected on the basis of a preliminary study comparing statistically measurements of 32 variables recorded in 15 patients who all had clinical, radiological and endoscopic evidence of GER and in 8 asymptomatic controls. Although patients with symptomatic GER differed significantly from the asymptomatic ones for 27 variables examined, 6 variables emerged as having the highest value for discrimination (overlap score 0–1). Among these, the Euler-Byrne index (number of reflux pH<4+4 times the number of reflux episodes of more than 5 min), the percentage of total reflux time and the number of reflux episodes 1 h post-cibal scored 0 (no overlap). GER was considered to be present when at least five of these six parameters were abnormal.The overeall incidence of GER in children with RRD was 41% (15) when detected by oesophagram and 61% (22) when diagnosed by pH monitoring criteria. In the children with bronchial asthma or with recurrent laryngitis, the percentage of reflux time during sleep was about 40 times higher than in asymptomatic controls and 2 times higher than in those with symptomatic GER. Of the 22 patients with RRD and GER, 9 were elected to have fundoplication because of poor response to medical antireflux management. All of these, showed complete (6) or partial (3) resolution of respiratory symptoms. Of the 13 patients in whom medical therapy was continued, 9 showed satisfactory improvement with a mean follow-up time of 1 year. Silent GER might be an important contributing factor to the severity of some common chronic respiratory disorders in children.

Diverticulum formation after circular myotomy for esophageal atresia

Out of our last 70 cases of esophageal atresia, a circular myotomy of the upper pouch (Livaditis technique) was required in six patients to achieve an end-to-end anastomosis: all had a lower tracheoesophageal fistula. They were operated on between 15 and 96 hours after birth, without any preoperative attempt of elongation of the upper pouch. Follow-up ranged from 14 to 77 months. Three patients had an uneventful postoperative course and late outcome, despite a moderate ballooning at the myotomy site in one. Three babies developed a mild stricture which responded easily to a few dilatations. Asymptomatic ballooning at the myotomy site was observed in one baby; the other two developed a diverticulum responsible for acute respiratory distress in one case and severe dysphagia in the other. both required esophageal replacement. These observations call attention to the fact that patients treated by myotomy should be followed very carefully early and late, especially if an anastomotic stricture develops or in case of associated tracheomalacia.

AIDS in two African children--one with fibrosarcoma of the liver.

We report here on two black African girls who developed an acquired immune deficiency syndrome (AIDS). The first patient was a premature girl born to healthy parents. She suffered from interstitial pneumonitis during the first week of life and died of it at the age of 6 months. Her mother, although asymptomatic, had polyclonal hypergammaglobulinaemia, a reversed T-helper/T-suppressor ratio and a decreased lymphocyte response to mitogens. The second patient had the first symptoms at the age of 6 years, developed a primitive malignant fibrosarcoma of the liver at 8 years old and died 1 year later. AIDS can affect black African children who have not been transfused and whose family members are not considered as at a high risk for this disease. In children, AIDS and cancer can be associated. In the second patient, cytotoxic suppressor lymphocytes (OKT8 positive cells) were shown to behave in vitro as precursors of T-killer cells.

Early hepatocyte, endothelial, and bile duct cell injury after pediatric liver transplantation from cadaveric or living-related donors.

BACKGROUND When compared with cadaveric grafts (Cad), the potential advantages of pediatric orthotopic liver transplantation (OLT) from living-related (LR) donors may include better graft quality, shorter ischemic time, appropriate preparation of the recipient, and better immunologic compatibility. METHODS The aim of this study was to analyze early hepatocyte, endothelial, and bile duct cell injury following pediatric OLT using LR (n=15) or uncomplicated Cad reduced-size (n=10) grafts. Median (range) total ischemic times were 190 min (105-261) versus 760 min (418-948) in LR and Cad groups, respectively (P<0.001). RESULTS The post-OLT cytolytic profile, assessed daily during the first 7 days using both plasma glutamate-pyruvate transaminase and serum alpha-glutathione S-transferase, showed significantly higher levels of both parameters for the 10 uncomplicated Cad cases when compared with the 15 LR grafts (P<0.001). The evaluation of hepatic endothelial cell function during the first week after OLT, using serum hyaluronic acid levels, suggested lower endothelial injury in the LR grafts, when compared with the Cad grafts (P=0.059). Bile duct cell injury, as assessed using plasma gamma-glutamyl transferase levels, was similar in both groups, with a progressive increase at the end of the first week after OLT, which was correlated with a similar incidence of early acute rejection in both groups (80% in the LR group vs. 62% in the Cad group, NS). CONCLUSION (1) The hepatocellular and endothelial cell damage was reduced after OLT with LR grafts, which may be related to shorter ischemic time when compared with Cad grafts; (2) the putative immunologic advantage for LR grafts was not confirmed in terms of incidence of acute rejection.

Upper airway lesions in children after accidental ingestion of caustic substances.

Of 33 children admitted within 24 hours after accidental ingestion of a caustic substance, 14 (42.5%) had evidence of upper airway lesions on direct laryngoscopy. Three patients, 10 to 12 months old, needed endotracheal intubation for acute respiratory obstruction; four patients younger than 2 years had severe dyspnea without obstruction; seven patients had mild or no respiratory symptoms. All were discharged without respiratory sequelae, although esophageal stenosis developed in six patients. Nine of 13 patients younger than 2 years, compared to five of 20 patients older than 2 years, had upper airway lesions (P less than 0.01). The frequency of respiratory tract lesions was higher in patients with severe esophagitis. Eleven of 17 patients with severe esophagitis, compared to three of 16 with mild inflammation, had respiratory tract lesions (P less than 0.025). No specific caustic substance predisposed to upper airway lesions.

Hepatopulmonary syndrome and liver transplantation: a review of the peroperative management of seven paediatric cases.

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.

Platelet aggregates in small lung vessels and death during liver transplantation

10 children who died suddenly during liver transplantation were found at necropsy to have extensive obstruction of small lung vessels by platelet aggregates. In 7 of these patients pulmonary artery pressure changes before death were consistent with acute obstruction of the pulmonary vascular bed. Platelet aggregates were not strikingly increased in blood vessels in other tissues. No single obvious cause for these unusual histological findings could be identified, although the presence of intravascular catheters, perioperative blood and platelet concentrate transfusions, and cellular debris from the liver forced into the circulation during surgery might predispose to platelet aggregation.

Management of intra-abdominal organ injury following blunt abdominal trauma in children.

ObjectiveTo evaluate the strategy of a combined diagnostic and therapeutic approach in children with intra-abdominal organ injury following blunt abdominal trauma.DesignRetrospective clinical study.SettingPediatric intensive care unit of an university hospital.Patients38 children with documented intra-abdominal injury.InterventionInitial non-surgical treatment by a team of pediatric intensivists, radiologists and surgeons.Measurements and resultsPhysical examination, oriented blood and urine tests, plain abdominal film, abdominal ultrasound (US) and computed tomography (CT) with contrast. US documented intra-abdominal fluid in 30 and initial organ lesion in 14 out of 31 patients evaluated. Abdominal CT demonstrated the precise organ lesion in 34 out of 36 patients examined with solid organ lesion. Early laparotomy was needed in 7 because of severe shock, pneumoperitoneum and ruptured diaphragm, and delayed surgery in 6 patients. All 38 patients regained a normal life.ConclusionsThe stepped diagnostic approach combined with initial non-surgical treatment by a team provided accurate diagnosis and appropriate treatment. Abdominal US, by demonstrating free intra-abdominal fluid is very sensitive to detect patients with intra-abdominal organ injury, CT scan with contrast is needed to give precise information of specific organ lesions.