and ϩ100, complete shrinkage).Descriptive statistics and the observer intraclass correlation were performed. Results. TheTable lists patient characteristics and response data.Clinically, 3 patients had IHs still in the proliferative phase; 2 had IHs that were stable; and 1 had IHs in regression.The difference in appearance of IHs at the various stages of treatment is shown inFigure 1.The mean change in VAS over time is presented in Figure 2. None of the patients experienced any local or systemic adverse events.Comment.This proof of concept study shows that timolol maleate, 0.5%, gel, a nonselective ␤-blocker in topical formulation, is effective and safe for the treatment of IHs.Patients with superficial IHs and those treated for longer periods showed better response to timolol.Early intervention during the rapid proliferative phase (age 1-6 months) may result in better and faster resolution of IHs.This preliminary work suggests that topical timolol maleate, 0.5%, gel is an alternative to systemic propranolol for treatment of superficial IHs.Further prospective studies are required to substantiate the safety and efficacy of timolol maleate, 0.5%, gel in the treatment of IHs.

Here we describe the clinical, histopathological and molecular studies of a female proband that died at 2 months of age in the context of a syndromic polymicrogyria. There was no significant family history. Clinical and radiological features included poor contact, cleft palate, facial dysmorphic features with frontal bossing, down-slanting and small palpebral fissures, inferior epicanthic folds, low-set and malformed ears, flat nose, retrognathism and short neck, minor limb anomalies, polymicrogyria that appear more severe in the perisylvian regions and cerebellar vermis hypoplasia. Autopsy findings revealed a patent foramen ovale with persistent left superior vena cava, left renal hypoplasia and microphthalmia. This description does not fit with any of the known syndromes with polymicrogyria and cytogenetic analyses including standard and high resolution chromosome analyses, telomeric FISH studies and array-CGH were normal. Consequently, the reported features in this child are unique and are likely to represent a new syndrome.

The phenotype of mosaic variegated aneuploidy (MVA) syndrome is characterized by severe microcephaly, growth deficiency, mental retardation, and mild physical anomalies. The MVA syndrome is associated with mosaicism for several different aneuploidies involving many different chromosomes with or without premature centromere division (PCD). To date 28 cases of MVA syndrome have been reported. We report the first case of MVA syndrome without microcephaly. The clinical features in our patient included craniofacial dysmorphic features, growth retardation, and developmental delay. Cytogenetics analyses and FISH studies showed multiple aneuploidy with trisomy 18, 19, and 8, respectively in blood lymphocyte and fibroblasts without PCD. This case is compared with the other of MVA syndrome previously reported in literature. From this case report, we suggest that microcephaly is not mandatory for the diagnosis of MVA syndrome.

BACKGROUND:Continuous hemodiafiltration is potentially more efficient than hemofiltration for removing low molecular metabolites.CASE REPORT:A newborn (2100 g) was admitted suffering from acute distress with statuts epilepticus, liver failure with generalized hyperaminoacidemia, hypoglycemia and oligo-anuria. Peritoneal dialysis, performed on the 17th day of life, was ineffective and the patient was treated by continuous hemofiltration alternating with hemodiafiltration. Clearance of amino-acids was studied for 15 minutes under each technique. The amino-acid concentrations were measured in the infused fluid and in the ultrafiltrate. The baby died on the 20th day despite this treatment.RESULTS:Amino acid clearance by hemodiafiltration was 181 +/- 176% greater than by hemofiltration. The mean improvement for all amino acids was 148%, with extremes of +43% for citrulline and 941% for glutamic acid.CONCLUSION:Continuous hemodiafiltration is an efficient method of removing amino acids; it could be used to treat severe inborn errors of metabolism such as leucinosis.

Background. - Aortic thrombosis is more frequent since the use of umbilical artery catheters in neonatal intensive care units. Some drugs or surgery are proposed to prevent complications; experience with tissue plasminogen activator (tPA) is still limited.Case report no 1. - A neonate, weighing 2400 g, developed respiratory distress requiring insertion of a catheter into her umbilical artery at H12. Ultrasonography on day 3 showed aortic thrombosis extending to the right renal artery which was confirmed by angiography. tPA 0.1 mg/kg was administered through the catheter, followed by 0.3 mg/kg/h for 3 hours and heparin, 100 IU/kg/hour for 54 hours. Angiography, performed 18 hours later, showed complete disappearance of the thrombosis.Case report no 2. - A neonate, weighing 2520 g suffered at 12 hours of life from seizures, apnea and bradycardia which required insertion of a catheter into her umbilical artery. Cyanosis of the right leg with weakening of femoral pulsations, 14 hours later, lead to the diagnosis of aortic thrombosis which was confirmed by aortography. The patient was given tPA 0.1 mg/kg followed by 0.3 mg/kg/h for 3 hours and heparin 100 IU/kg/hour for 6 hours. Amplitude of femoral pulsations strikingly increased within 6 hours with the disappearance of cyanosis.Conclusion. - These results suggest that tPA can be useful in neonates presenting with aortic thrombosis.

Six cases of bacterial laryngotracheitis were seen over a three-year period in patients aged 23 months to 11 years 7 months. Evidence of severe infection with obstructive dyspnea worsening markedly during coughing were the clinical features. Emergency rigid catheter endoscopy under general halothane anesthesia in the operating room confirmed the diagnosis and ruled out epiglottitis and subglottic edematous laryngitis. The causative bacterial agent was identified in five of the six cases (Staphylococcus aureus : 2 ; Haemophilus influenzae : 2, Streptococcus pneumoniae : 1). Outcome was favorable in every case after endoscopic relief of the obstruction followed by tracheal intubation, humidification, frequent aspiration, antimicrobial therapy, and corticosteroids. There were no severe complications such as asphyxia-induced cardiorespiratory arrest, intrathoracic air leaks, or Staphylococcus toxic shock syndrome.

BACKGROUND--Aortic thrombosis is more frequent since the use of umbilical artery catheters in neonatal intensive care units. Some drugs or surgery are proposed to prevent complications; experience with tissue plasminogen activator (tPA) is still limited. CASE REPORT NO 1--A neonate, weighing 2400 g, developed respiratory distress requiring insertion of a catheter into her umbilical artery at H12. Ultrasonography on day 3 showed aortic thrombosis extending to the right renal artery which was confirmed by angiography. tPA 0.1 mg/kg was administered through the catheter, followed by 0.3 mg/kg/h for 3 hours and heparin, 100 IU/kg/hour for 54 hours. Angiography, performed 18 hours later, showed complete disappearance of the thrombosis. CASE REPORT NO 2--A neonate, weighing 2520 g suffered at 12 hours of life from seizures, apnea and bradycardia which required insertion of a catheter into her umbilical artery. Cyanosis of the right leg with weakening of femoral pulsations, 14 hours later, lead to the diagnosis of aortic thrombosis which was confirmed by aortography. The patient was given tPA 0.1 mg/kg followed by 0.3 mg/kg/h for 3 hours and heparin 100 IU/kg/hour for 6 hours. Amplitude of femoral pulsations strikingly increased within 6 hours with the disappearance of cyanosis. CONCLUSION--These results suggest that tPA can be useful in neonates presenting with aortic thrombosis.

Gouyon, J. B. M.D.; Benoit, A. M.D.; Bétremieux, P. M.D.; Sandre, D. M.D.; Sgro, C. M.D.; Bavoux, F. M.D.; Beneton, C. M.D.; Badoual, J. M.D. Author Information

Background . — Continuous hémodiafiltration is potentially more efficient than hemofiltration for removing low molecular metabolites. Case report — A newborn (2100 g) was admitted suffering from acute distress with statuts epilepticus, liver failure with generalized hyperaminoacidemia, hypoglycemia and oligo-anuria. Peritoneal dialysis, performed on the 17 th day of life, was ineffective and the patient was treated by continuous hemofiltration alterning with hemodiafiltraticn. Clearance of amino-acids was studied for 15 minutes under each technique. The amino-acid concentrations were measured in the infused fluid and in the ultrafiltrate. The baby died on the 20 th day despite this treatment. Results . — Aminoacid clearance by hemodiafiltration was 181 ± 176% greater than by hemofiltration. The mean improvement for all aminoacids was 148%, with extremes of +43% for citrulline and 941% for glutamic acid. Conclusion . — Continuous hemodiafiltration is an efficient method of removing aminoacids; it could be used to treat severe inborn errors of metabolism such as leucinosis.

Extracorporeal shock wave lithotripsy has become the standard method of treating renal stone disease. The appropriate choice of anaesthetic technique depends on the shock wave technic mode. Recent modifications of lithotriptors have reduced the physiological consequences of the procedure and most often these treatments can be performed without local or general anaesthesia. However, some sedation/analgesia may be necessary and a good knowledge of anaesthesia for lithotripsy remains most useful so as to meet any particular requirements.

Background. — Necrotizing tracheobronchitis is a severe complication observed in some mechanically ventilated neonates. Case report 1. — A twin premature (GA = 31 weeks), weighing 1500 g required oral endotracheal intubation for mechanical ventilation because he suffered from respiratory distress syndrome. He was given indomethacin on day 4 for patent ductus arteriosus. Progressive weaning of ventilation on day 9 was dramatically complicated by hypoxia, respiratory acidosis and right pneumothorax. Immediate endoscopy showed total obstruction of trachea by necrotic secretions the suction of which was followed by rapid improvement of the respiratory condition. Bacterial examination of secretions showed coagulase-negative staphylococcus. The patient was given steroids + antibiotics. Prolonged ventilation resulted in bronchopulmonary dysplasia and the patient was only extubated at week 12 after a normal endoscopic control. Case report 2. — A premature girl (GA = 32 weeks), weighing 1800 g required oral endotracheal intubation for mechanical ventilation because she suffered from respiratory distress syndrome. The respiratory condition worsened on day 3, requiring tracheography which showed distal tracheal obstruction. Immediate endoscopy showed thin, adherent and necrotic membranes which were removed by suction. The patient was given steroids + antibiotics and was extubated on day 14 after a normal endoscopic control. Conclusions. — This iatrogeneous complication must be recognized in a ventilated infant when the respiratory condition dramatically worsens. Emergency bronchoscopy permits endotracheal suction of necrotic secretions.

Background. - Necrotizing tracheobronchitis is a severe complication observed in some mechanically ventilated neonates.Case report 1. - A twin premature (GA = 31 weeks), weighing 1 500 g required oral endotracheal intubation for mechanical ventilation because he suffered from respiratory distress syndrome. He was given indomethacin on day 4 for patent ductus arteriosus. Progressive weaning of ventilation on day 9 was dramatically complicated by hypoxia, respiratory acidosis and right pneumothorax. Immediate endoscopy showed total obstruction of trachea by necrotic secretions the suction of which was followed by rapid improvement of the respiratory condition. Bacterial examination of secretions showed coagulase-negative staphylococcus. The patient was given steroids + antibiotics. Prolonged ventilation resulted in bronchopulmonary dysplasia and the patient was only extubated at week 12 after a normal endoscopic control.Case report 2. - A premature girl (GA = 32 weeks), weighing 1 800 g required oral endotracheal intubation for mechanical ventilation because she suffered from respiratory distress syndrome. The respiratory condition worsened on day 3, requiring tracheography which showed distal tracheal obstruction. Immediate endoscopy showed thin, adherent and necrotic membranes which were removed by suction. The patient was given steroids + antibiotics and was extubated on day 14 after a normal endoscopic control.Conclusions. - This iatrogeneous complication must be recognized in a ventilated infant when the respiratory condition dramatically worsens. Emergency bronchoscopy permits endotracheal suction of necrotic secretions.

BACKGROUND:Necrotizing tracheobronchitis is a severe complication observed in some mechanically ventilated neonates.CASE REPORT:A twin premature (GA = 31 weeks), weighing 1,500 g required oral endotracheal intubation for mechanical ventilation because he suffered from respiratory distress syndrome. He was given indomethacin on day 4 for patent ductus arteriosus. Progressive weaning of ventilation on day 9 was dramatically complicated by hypoxia, respiratory acidosis and right pneumothorax. Immediate endoscopy showed total obstruction of trachea by necrotic secretions the suction of which was followed by rapid improvement of the respiratory condition. Bacterial examination of secretions showed coagulase-negative staphylococcus. The patient was given steroids + antibiotics. Prolonged ventilation resulted in bronchopulmonary dysplasia and the patient was only extubated at week 12 after a normal endoscopic control.CASE REPORT:A premature girl (GA = 32 weeks), weighing 1,800 g required oral endotracheal intubation for mechanical ventilation because she suffered from respiratory distress syndrome. The respiratory condition worsened on day 3, requiring tracheography which showed distal tracheal obstruction. Immediate endoscopy showed thin, adherent and necrotic membranes which were removed by suction. The patient was given steroids +antibiotics and was extubated on day 14 after a normal endoscopic control.CONCLUSIONS:This iatrogeneous complication must be recognized in a ventilated infant when the respiratory condition dramatically worsens. Emergency bronchoscopy permits endotracheal suction of necrotic secretions.

A five month-old infant presented with gastro-intestinal symptoms followed by a multiple organ failure with: shock, status epilepticus, disseminated intravascular coagulation, hepatic and renal failure. The infant survived with major neurological sequelae. The diagnosis and the actuality of the so-called <<hemorrhagic shock and encephalopathy syndrome>> are discussed.

A five month-old infant presented with gastro-intestinal symptoms followed by a multiple organ failure with: shock, status epilepticus, disseminated intravascular coagulation, hepatic and renal failure. The infant survived with major neurological sequelae. The diagnosis and the actuality of the so-called "hemorrhagic shock and encephalopathy syndrome" are discussed.

An acute kidney failure (AKF) was present at birth in a 28 week-old premature infant exposed in utero to ketoprofen during the last 4 days of pregnancy. Usual causes of AKF were not identified. Thus, AKF might be due to a ketoprofen-induced impairment in renal prostaglandin synthesis.

An acute kidney failure (AKF) was present at birth in a 28 week-old premature infant exposed in utero to ketoprofen during the last 4 days of pregnancy. Usual causes of AKF were not identified. Thus, AKF might be due to a ketoprofen-induced impairment in renal prostaglandin synthesis.

The authors report 10 cases of newborns with neonatal hypoxic encephalopathy and presence of spikes in the vertex area on EEG. Evolution was characterized by the onset of a spastic paraparesy with frontal atrophy on CT scan examination and disappearance of the spikes after 2 months of age. This graphic pattern is usually absent in neonatal anoxic encephalopathy. Vertex spikes thus appear to have a prognostic value in neonates with brain anoxia.