Lower respiratory tract foreign bodies: a retrospective review of morbidity, mortality and first aid management.

OBJECTIVES: The authors describe 28 pediatric cases of foreign body inhalation requiring treatment in intensive care units between 1987 and 1999. The purpose of this study was: (1) to analyze the circumstances, diagnostic difficulties and initial treatment of serious foreign bodies and (2) to compare our series with other literature descriptions and define principles for optimal prevention and initial treatment. METHODS: Twenty-one children presented a penetration syndrome, which was responsible in 13 cases for asphyxia with cardiorespiratory arrest. All these children died, regardless of the initial treatment. Seven children were hospitalized for apparent asthmatic symptoms that did not respond to traditional treatment. RESULTS: The inefficiency of external extraction methods like the Heimlich maneuver and the mean delay between clinical signs and initial treatment lead us to propose a new strategy for the emergency treatment of foreign bodies with asphyxia. CONCLUSIONS: We recommend that emergency teams promote the use of a laryngoscope and Magill forceps. Flexible endoscopy is still recommended as the appropriate diagnostic tool to eliminate doubt in the case of a first severe asthma attack.

[Effects of nasal continuous positive airway pressure ventilation in infants with severe acute bronchiolitis]. / Place de la ventilation non invasive nasale dans la prise en charge des broncho-alvéolites sévères.

OBJECTIVE: Usefulness of nasal continuous positive airway pressure (NCPAP) in severe acute bronchiolitis has been checked. The objective of this descriptive study was to evaluate the feasibility, safety and risk factors of NCPAP failure. POPULATION AND METHODS: One hundred and forty-five infants were hospitalised in our intensive care unit during the 2 last epidemics (2003-2004, 2004-2005). Among them, 121 needed a respiratory support, either invasive ventilation (N=68) or NCPAP (N=53). RESULTS: General characteristics were similar during the 2 periods. Percentage of NCPAP failure, defined by tracheal intubation requirement during the stay in paediatric intensive care unit, was quite similar during the 2 periods (25%), but number of NCPAP increased twofold. Whatever the evolution was in the NCPAP group, we observed a significant decrease in respiratory rate (60+/-16 vs 47.5+/-13.7 cycle/min., P<0.001) and PaCO2 (64.3+/-13.8 vs 52.6+/-11.7 mmHg, P=0.001) during NCPAP. Only PRISM calculated at day 1 and initial reduction of PaCO2 were predictive of NCPAP failure. Percentage of ventilator associated pneumonia was similar (22%) between the invasive ventilation group and infants who where intubated because of failure of NCPAP. Duration of respiratory support and stay were reduced in the NCPAP group (P<0.002). CONCLUSION: NCPAP appears to be a safe alternative to immediate intubation in infants with severe bronchiolitis.

[Bronchiolitis treated with mechanical ventilation: prognosis factors and outcome in a series of 135 children]. / Bronchiolites en unité de réanimation pédiatrique: facteurs pronostiques et devenir respiratoire des nourrissons ventilés.

UNLABELLED: Viral bronchiolitis is usually associated with favorable outcome as regard to mortality. Only few studies reported severe bronchiolitis requiring mechanical ventilation, and respiratory outcome is not well described. METHODS: Therefore, we conducted a retrospective study in a series of 135 children admitted in a single Pediatric Intensive Care Unit (PICU) over a four year period (1994-1998). All of them were admitted for viral bronchiolitis requiring mechanical ventilation. RESULTS: At admission, 83% of them were less than three months old. Prematurity at birth was present in 33,3%. Mortality was observed in four cases (2,9%), all premature babies with mechanical ventilation at birth. Univariate analysis showed as main factors associated to mortality: prematurity (P =0,056) and acute respiratory distress syndrome (P =0,017). Childhood asthma was observed in 40,4% of children without any associated factor wether at birth or in PICU related to such outcome. CONCLUSION: Bronchiolitis associated with mechanical ventilation is particularly observed in very young babies and prematurity is the main factor associated to mortality. Mechanical ventilation seems not to be associated with unfavorable respiratory outcome. Considering physiology and population, non invasive ventilation could be an effective alternative of mechanical ventilation.

Orthotopic liver transplantation in children with chronic liver disease and severe hypoxemia.

Liver transplantation has been considered until recently as an absolute contraindication in hypoxemic patients. We report our experience in nine patients who had orthotopic liver transplantation between June 1986 and June 1992. These patients had cirrhosis-related hypoxemia with intrapulmonary shunting (IPS). The arterial oxygen pressure (PaO2) on room air ranged from 47 to 78 mmHg. OLT resulted in resolution of hypoxemia and closure of IPS in five patients whose hypoxemia was higher than 60 mmHg, and in death for the remaining four patients who had severe hypoxemia (PaO2 < 60 mmHg). We conclude that hypoxemia is no longer a contraindication to liver transplantation. Patients having PaO2 levels higher than 60 mmHg should have OLT as soon as possible before reaching lower levels of PaO2, and combined lung-liver transplantation or heart-lung-liver transplantation should be discussed in patients with severe hypoxemia (PaO2 < 60 mmHg).

Orthotopic liver transplantation for Byler disease.

Byler disease is a rare form of familial intrahepatic cholestasis that is fatal before puberty. This retrospective study reviewed the results of orthotopic liver transplantation in 14 children with Byler disease using 12 whole-liver grafts and 2 reduced-size grafts. One post-operative death occurred after retransplantation for arterial thrombosis. In the other patients, infectious problems and rejection episodes were the most frequent complications during the postoperative period. In the 13 patients alive, graft function, growth, and quality of life were good after an average follow-up of 17 months without any sign of disease recurrence.

Reversal of hypoxemia by inhaled nitric oxide in children with severe hepatopulmonary syndrome, type 1, during and after liver transplantation.

BACKGROUND: The hepatopulmonary syndrome with profound hypoxemia is a rare but severe complication for children with liver cirrhosis. It can be reversed by liver transplantation (LT), which is now regarded as a good indication. However, previous reports have described cases of transient or fatal deteriorations of intrapulmonary shunting after pediatric liver transplantation with dramatically worsening hypoxemia. METHODS AND RESULTS: A similar case during and after LT in a 4-year-old girl with severe hepatopulmonary syndrome is described with prompt reversal of hypoxemia by inhaled nitric oxide, which was discontinued definitely until day 14 after LT. CONCLUSIONS: During or after LT, worsening hypoxemia may be improved by using inhaled nitric oxide in pediatric patients undergoing liver transplantation for liver cirrhosis and hepatopulmonary syndrome. The mechanisms are unclear, but may involve mismatching lung ventilation-perfusion. However, additional clinical reports are necessary before accepting these results.

Postshunt encephalopathy in liver transplanted children with portal vein thrombosis.

BACKGROUND: Surgical portosystemic shunting has been reported to alleviate successfully portal hypertension in liver transplanted recipients with portal vein thrombosis. METHODS: We report two liver transplanted children with portal vein thrombosis who developed post-shunt acute encephalopathy. In one child, a mesocaval H-type shunt was created surgically because of bleeding related to Roux-en-Y loop varices at 3 months posttransplantation; in the other, a large spontaneous splenorenal shunt was discovered at the time of diagnosis of portal vein thrombosis on day 34 posttransplantation and was preserved. RESULTS: Post-shunt encephalopathy developed 6 months and 2.7 years after transplantation, causing death in one child. CONCLUSIONS: This report illustrates the risk and the possible dismal outcome of post-shunt encephalopathy in liver transplanted children. Therapeutic procedures other than portosystemic shunting that will restore an hepatopetal portal flow to the liver graft should be considered in liver-transplanted children with portal vein thrombosis.

Liver transplantation in children with Alagille syndrome--a study of twelve cases.

Cholestasis associated with Alagille syndrome may, in a few cases, be extremely severe and result in major impairment in the quality of life during early childhood and end up in cirrhosis eventually. We report the results of liver transplantation in 12 children with a severe hepatic form of Alagille syndrome. All children presented with cholestatic jaundice from birth, peculiar facies, stenosis of the peripheral pulmonary artery, and posterior embryotoxon; butterfly-like vertebrae were present in 9 children. At the time of transplantation (mean age 7 years 10 months) refractory pruritus was present in 9 children, xanthoma in 11, and height and weight retardation in 11. Total serum bilirubin ranged from 116 to 322 mumol/L and total serum cholesterol from 3.5 to 29 mmol/L. Systolic right ventricular pressure was moderately raised (36 to 48 mmHg) in 5 children; mean creatinine clearance was 99 ml/min/1.73 m2. Histologic examination of the removed livers showed cirrhosis, severe annular fibrosis, and moderate portal fibrosis in 4 children each. Follow-up in the 11 survivors has ranged from 14 months to 5 1/2 years. All lead normal lives. Pruritus and xanthomas disappeared. Increase in height was observed in 8 of the 10 survivors who had growth retardation prior to transplantation. School level is normal in 4 (median age at LT: 5 yr 9 mo) and below normal in 6 (median age at OLT: 9 yr 9 mo). Liver function tests are normal in 10 children. Mean creatinine clearance is 101 ml/min/1.73 m2. These results indicate that the quality of life can be considerably improved after liver transplantation in children with a severe hepatic form of Alagille syndrome and suggest that it could be carried out before these children attend elementary school.

Orthotopic liver transplantation for mitochondrial respiratory chain disorders: a study of 5 children.

BACKGROUND: Liver involvement in mitochondrial respiratory chain disorders (MRCD) frequently ends in liver failure and death. Because of the high risk of extrahepatic, particularly neuromuscular, manifestations of the disease, the indication of orthotopic liver transplantation (OLT) in these patients remains controversial. We report on 5 such children in whom OLT was carried out, in an attempt to help clarify the matter. PATIENTS: Patients 1 and 2 presented with fulminant liver failure at ages 7 and 6 months respectively. Emergency liver transplantation was performed before etiological investigations were completed. Retrospective examination of the explanted livers showed defects in complexes I, III and IV. In patient 1, severe neurological deterioration occurred 2 months after OLT with fatal outcome 9 months later. Patient 2 is alive 22 months after OLT with moderate motor impairment. Patients 3, 4 and 5 presented with progressive liver failure before 6 months of age. Surgical liver biopsies displayed a 50% defect in complex IV (patient 3), a defect in complexes I, IV (patient 4) and in complexes I, III, IV (patient 5). Because there was no clinical extrahepatic involvement on investigations, OLT was carried out in these patients. Patient 3 died of multiple organ failure soon after OLT, patients 4 and 5 are alive respectively 21 months and 12 months after OLT with normal neurological examination. CONCLUSION: OLT may be a valid therapeutic option in infants with delayed liver cell failure due to MRCD, only after performing in emergency a thorough inves tigation to exclude clinically significant extrahepatic, especially neuromuscular, involvement.

Incidence of Reye's syndrome in France: a hospital-based survey.

At the time of the study no information was available in France about the incidence of Reye's Syndrome (RS) and no warnings about RS and aspirin. The objective was to evaluate the incidence of RS in France by a hospital-based study. For a period of 1 year from November 1995 to November 1996, all French paediatric departments were required to report any child under 15 years with unexplained noninflammatory encephalopathy (i.e., CDC consciousness level stage I or deeper with normal CSF) and a threefold (or greater) increase in serum aminotransferase and/or ammonia. All suspected cases were classified by a panel of experts as probable RS or excluded RS. In 10% of randomly selected paediatric departments we checked that every suspected case had been reported. Forty-six suspected cases were reported during the year of the survey, of which 14 were classified as RS. Five of these 14 cases had a metabolic disorder. Nine children were definitively diagnosed as having RS (i.e., an estimated incidence of RS of 0.79/1,000,000 children, i.e., below 15/year). Eight children had been exposed to aspirin, four to aspirin alone and four to aspirin and acetaminophen. On the basis of these results the incidence of RS in France in 1996-1997 was not substantially different from that of countries where warning labels were already in use, but it was higher than in the US after 1994. This was probably due to the reduction in aspirin prescription in France because of warnings in Europe and the US and also because many cases of RS are now identified as metabolic disease. On the basis of these results and because the relationship between aspirin and RS has already been proved, public and professional warnings concerning RS on aspirin-containing products in cases of varicella and viral febrile illness have been adopted by the French Drugs Agency.

Severe cardiac failure in newborns with VGAM. Prognosis significance of hemodynamic parameters in neonates presenting with severe heart failure owing to vein of Galen arteriovenous malformation.

OBJECTIVE: Neonatal vein of Galen malformation complicated by severe cardiac failure is a rare disease. The purpose was to assess the outcome of this life-threatening malformation and identify hemodynamic prognostic factors. DESIGN: Retrospective study. PATIENTS: Twenty-four newborns with cardiac failure requiring mechanical ventilation were consecutively admitted from 1986 to 2000. INTERVENTIONS: Cardiovascular evaluation including echocardiogram was performed in all cases. Eighteen transarterial shunt occlusions with glue were applied by the same team of three physicians. MEASUREMENTS AND RESULTS: Twelve babies survived and underwent one endovascular session at least (median age 20 days) with a mean 63 months follow-up. Embolization was not performed in 6 of the 12 nonsurvivors because of severe brain damage or profound hypotension Cardiogenic shock occurred in all nonsurvivors, but also in one long-term survivor (p<0.0001). Echocardiogram showed signs of right ventricular failure, most often in the dead babies (p=0.005). The pulmonary systemic arterial pressure ratio was significantly higher in the nonsurvivor group (p=0.031), and it decreased significantly after the first embolization only in patients who survived (p=0.01). Patent ductus arteriosus and a diastolic aortic reversed-flow were present in all nonsurvivors in contrast to 30% of the long-term survivors (p=0.003 ). There was no difference in the left ventricular contractility and mean cardiac output between the two groups. CONCLUSIONS: The outcome of vein of Galen malformation complicated by severe cardiac failure requiring mechanical ventilation remains poor. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension.

Orthotopic liver transplantation with a reduced-size graft: an ideal compromise in pediatrics?

This study retrospectively reviewed the results of 81 orthotopic liver transplantations in 72 pediatric patients with either a full-size graft (n = 41) or a reduced-size graft (n = 40) during a 4-year period. Two types of liver reduction were used to obtain a left lobe graft (n = 30) or a left lateral segment graft (n = 10). The choice of the reduction technique was based on two criteria: the donor/recipient body weight ratio and the transverse size of the recipient's abdomen. The patients who underwent transplantation with a reduced-size graft were younger and smaller than those who received a full-size graft. The mortality rate of the candidates on the pediatric transplantation waiting list was 2.7% during the same period. The 3-year survival rate after elective transplantation was 85% and 75% in the full-size graft group and the reduced-size graft group, respectively (difference not significant). The use of reduced-size grafts was associated with higher amounts of red cell and fresh-frozen plasma transfusions after graft revascularization. The incidence of postoperative bleeding and bile leakage from the transected surface was low, whereas hepatic artery and biliary complications were less frequent in the reduced-size graft group than in the full-size graft group. At the time of follow-up, liver biochemical test results and quality of life were not different between the two groups. Despite the constraining technique and the increase in intraoperative blood loss, orthotopic liver transplantation with a reduced-size graft allows us to overcome the shortage of pediatric donors and improve the overall survival rate of children with end-stage liver disease.

Flumazenil in the treatment of hepatic encephalopathy in children with fulminant liver failure.

OBJECTIVE: To evaluate the effect of flumazenil on hepatic encephalopathy complicating fulminant liver failure in children. DESIGN: Uncontrolled prospective study. SETTING: Pediatric Intensive Care Unit, tertiary care center. PATIENTS: 9 children with fulminant liver failure and hepatic encephalopathy awaiting emergency liver transplantation. INTERVENTIONS: Changes in hepatic encephalopathy grade and in electroencephalogram were recorded during the injection of a bolus of flumazenil (0.01 mg/kg/i.v. bolus) followed by a continuous infusion of flumazenil (0.01 mg/kg/h). MEASUREMENTS AND MAIN RESULTS: Before flumazenil, 7 children had grade 2 and 2 had grade 3 hepatic encephalopathy. Flumazenile injection mediated an arousal effect in 1 child in whom encephalopathy improved from grade 3 to grade 2. This effect lasted 30 min. No clinical response was observed in other children. An improvement of EEG anomalies was observed lasting 3 min in one child. Despite continuous infusion of flumazenil, encephalopathy worsened in all children. CONCLUSION: The effect of intravenous administration of flumazenil on hepatic encephalopathy in children with fulminant liver failure is inconsistent. Its efficacy is transient. The therapeutic value of flumazenil in children with fulminant liver failure awaiting a liver graft is minimal at this dosage.

Haemorrhagic shock and encephalopathy syndrome: neurological course and predictors of outcome.

UNLABELLED: The haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease. The aetiology of this syndrome is unknown, and, despite intensive treatment, the outcome is often fatal or associated with severe neurological sequelae. OBJECTIVE: To assess the neurological features and potential prognostic markers of the disease. DESIGN: Retrospective study. SETTING: Division of Neuropaediatrics in a children's university hospital. PATIENTS AND METHODS: Fourteen patients fulfilling the HSES criteria out of 42 children admitted with fever and shock to the Paediatric Intensive Care Unit between 1986 and 1994, were analysed for clinical, biological, neuroradiological, EEG and neuropathological findings. RESULTS: The patients (age range from 2 to 33 months) were found at night or in the morning either comatous (n = 3) or convulsing (n = 11). All but one were healthy before admission, although eight had had a brief prodromal infectious disease. All were febrile (mean body temperature 39.9 degrees C +/-0.9 degrees). Seasonal clustering during the winter months was observed. Coma and seizures with frequent status epilepticus were the main neurological manifestations. All children recovered from their multiple organ failure within a few days. Seven died (50%); four survivors had neurological sequelae (29%) with a developmental quotient (DQ) of 50% or less in three and a DQ of 75% in one and three infants (21%) had normal outcomes. Computed tomography (CT) displayed a diffuse area of low density mainly in the cerebral cortex and intraventricular and parenchymal haemorrhages. Magnetic resonance imaging (MRI) showed haemorrhagic cortical lesions. Postmortem examination of the brain conducted in three patients showed necrotic and haemorrhagic lesions, mainly in cortical areas. Comparison of the children with adverse outcome (death or neurological sequelae) with those with normal outcome revealed that predictors of poor outcome were status epilepticus (p = 0.003) and coma for more than 24 h (p = 0.01). Infants without disseminated intravascular coagulation, without a biphasic course and without brain hypodensities or haemorrhages on CT scans performed at least 4 days after onset had a normal neurodevelopmental outcome. CONCLUSION: The central nervous system appeared to be the main target of the HSES lesions. The most common outcome was brain death or severe brain damage. Further studies with a larger sample are necessary to determine whether the prognostic indicators we identified are reliable.

Prognostic factors of severe infectious purpura in children.

The French Club of Pediatric Intensive Care has prospectively studied 90 cases of infectious purpura which were hospitalized in 1981; the purpose of this study was to determine prognostic factors. The statistical study (X2 test) of all these cases is in agreement with data in the literature and shows that the mortality is significantly higher when there is: shock (p less than 0.001), coma (p less than 0.05), ecchymotic or necrotic purpura (p less than 0.01), temperature less than 36 degrees C (p less than 0.05), no clinical meningism (p less than 0.001), white cell count less than 10,000/mm3 (p less than 0.05), thrombocytopenia less than 100,000 (p less than 0.01), fibrinogen less than 1.5 g/l (p less than 0.001), kalemia greater than 5 mEq/l (p less than 0.01), spinal fluid cell count less than 20/mm3 (p less than 0.01). Because shock is one of the main prognostic factors (23 deaths in 55 shocked patients, versus 2 in 35 non-shocked) we have performed another statistical study (with the Benzecri method) to determine a prognostic index for patients in shock. For its determination, five initial parameters are used: age, kalemia, white cell count, clinical meningism, platelet count. The predictive value for survival is 91%. The predictive value for death is 87%. The score was applied on the patients hospitalized in shock in 1982: the predictive value for survival is 75%, the predictive value for death is 61%.

[Serious acute hepatocellular insufficiency in children: EEG prognostic features]. / Insuffisance hépato-cellulaire aiguë grave de l'enfant: aspects EEG pronostiques.

Fulminant Hepatic Failure (FHF) in children leads to 60 to 90% of mortality without age influence, depending on the studies. Twenty-two children with this disease had daily EEG, 15 died, seven survived. The neurological electroclinical study evidenced a stereotyped evolution of the EEG anomalies which were not age-related, but correlated to clinical stages of coma and to severe coagulation disorders; 5/22 children with clinical and/or EEG convulsive disorders died. One child exhibited triphasic slow waves and died from paracetamol intoxication. A prospective study of a larger population could help to recognize factors of decision for hepatic transplantation.

[[Emergency pediatric EEG in mental confusion, behavioral disorders and vigilance disorders: a retrospective study]. / L'EEG d'urgence en pédiatrie dans les troubles de la vigilance, la confusion mentale et les troubles du comportement: étude rétrospective.

Recording of electroencephalogram (EEG) is of value to estimate vigilance states in children as in adults. In order to determine the diagnostic and prognostic value of emergency EEG in case of mental confusion, behavioral disorders and vigilance disorders in childhood, we conducted a retrospective study in 397 children (aged 2 months to 16 years). EEG was recorded less than 24 hours after an emergency consultation for acute confusion or acute behavioral disorder (n = 106) or after admission to the intensive care unit for comatose stage (n = 291). EEG gave diagnostic indications mainly in case of convulsive pathology or hepatic encephalopathy. In comatose children, we established a 4-stage EEG scale of increasing severity. This classification was compared to EEG scales already published in the literature and appeared very similar to that from Pampiglione and Harden, established in 150 children after cardiac arrest. A highly poor prognostic value was associated with burst-suppression post-anoxic patterns and with isoelectric records signaling brain death. Our classification of emergency EEG patterns is mainly helpful in these two situations, but does not exclude strict and repeated clinical and EEG follow-up in other cases, as a relatively preserved initial EEG may later deteriorate.

Fatal hemobilia after liver graft biopsy in a transplanted child.

Severe hemobilia after liver transplantation is a rare complication. We report one case of fatal hemobilia resulting from arterio-biliary fistula after a liver graft needle biopsy in a 7-year-old girl transplanted with a reduced-size graft. Arterial reconstruction was performed by an iliac conduit implanted in the aorta. The diagnosis of hemobilia was made by angiography but the hemorrhage could not be controlled by selective embolization of the bleeding arterial branch. The patient died from early and massive recurrent bleeding before surgery. The prevention of iatrogenic hemobilia and current therapeutic strategies are discussed.

[Cerebral complications of incontinentia pigmenti. A clinicopathological study of a case]. / Complications cérébrales de l'incontinentia pigmenti. Etude clinique et pathologique d'un cas.

A baby born with the typical features of incontinentia pigmenti, developed convulsions from the 5th day onward. CSF was normal. CT scan showed diffuse hypodensities in the left hemisphere. The baby died at 2 months of age of primary pulmonary hypertension. The neuropathological examination showed a normal morpho-histogenesis of the brain. There were necrotic, non inflammatory changes in the cortex and white matter, which did not affect a vascular topography. The lesions appeared to have occurred in the neonatal period and were markedly predominating in the left hemisphere.

[Treatment of acute liver failure in children]. / Traitement de l'insuffisance hépatique aiguë de l'enfant.

Among the main causes of acute liver failure (ALF) in children, metabolic diseases (especially in infants), viral and toxin or drug induced hepatitis are the most frequent. The cause remains, however, undetermined in about 30% of the cases. Management must be conducted in a pediatric hepatology unit or intensive care unit in relation with a pediatric transplant team in order: 1) to perform urgent etiological diagnosis; 2) to initiate specific therapy and symptomatic treatment; 3) to evaluate the severity and prognosis of liver disease for selection of children for emergency liver transplantation; 4) to evaluate contraindications to liver transplantation. The overall survival of post-emergency liver transplantation for ALF in children averages 65%.