Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution.

Fetal medicine is developing rapidly and aims to improve the outcome for fetuses with congenital anomalies. Fetal endoscopic tracheal occlusion (FETO) has been developed for fetuses with congenital diaphragmatic hernia to counterbalance the compression of the lung by the abdominal viscera, preserving the pulmonary maturation. Because the perinatal morbidity and mortality of patients treated with FETO have decreased, new complications are emerging in the older survivors. Tracheomegaly has been reported to be a late complication of FETO, sometimes requiring tracheostomy. We report a case of bronchial dilatation after FETO and suggest an alternative surgical treatment.

Clinical course and determination of brainstem death in a children's hospital.

AIM: To study the aetiology and clinical course of children with brainstem death in a paediatric intensive care unit (ICU) and to determine whether current the practices that are used to declare brainstem death conform to accepted criteria. METHODS: A retrospective review chart of all patients with brainstem death (n = 31) admitted to the paediatric ICU between January 1995 and December 1998 was drawn up. RESULTS: Mean age of the patients was 51.9 +/- 54.5 mo with the main diagnoses being head trauma in 11 children, anoxic encephalopathy in 7, brain tumour in 5, drowning in 4, CNS haemorrhage in 3 and CNS infection in 1 child; 32.3% of the children were given pre-ICU admission cardiopulmonary resuscitation. The average time from insult to suspected brainstem death was 27 h and suspected brainstem death to confirmation was 25 h, with an average of 1.6 examinations performed. EEG was done in 14 patients, with electrocerebral silence in 8 after the first examination and in a further 5 after repeat testing. Cerebral blood-flow scans were done in 3 children and evoked potentials in 1 child. CONCLUSIONS: Trauma remains the most common primary diagnosis leading to brainstem death. Intensivists in this large hospital for children mainly conform to accepted guidelines for determination of brainstem death although there is a wider use of ancillary tests to aid diagnosis. The study also showed a low rate of < 10% of organ procurement for transplantation.

T cryptantigen activation is associated with advanced necrotizing enterocolitis.

BACKGROUND/PURPOSE: Thomsen-Friedenreich cryptantigen activation (TCA) exposes neonates with necrotizing enterocolitis NEC to the risk of hemolysis after transfusion of blood products. The authors aimed to determine the prevalence of TCA in neonates with NEC and to correlate TCA with severity of disease and outcome. METHODS: One hundred four neonates with NEC were tested for TCA on admission. Patients with TCA requiring transfusion were given packed red cells, low-titer anti-T fresh frozen plasma, and washed platelets to avoid hemolysis. RESULTS: Twenty-three infants had TCA, and 96% of these had stage III disease. The incidence of TCA was significantly higher in infants with stage III disease compared with those with stage II (30% v 4%; P <.01). A total of 91% of infants with TCA required laparotomy compared with 81% of those with no activation. At laparotomy, widespread disease was more common in the TCA group (71% v 55%). TCA did not significantly increase mortality rate (TCA, 39% v no TCA, 28%); this may reflect the transfusion policy of our unit. CONCLUSIONS: Twenty-two percent of neonates with NEC referred to our unit had TCA. There is an association between TCA and advanced NEC. Screening of neonates with advanced NEC for TCA is advised to identify those at risk of hematologic complications.

Persistent pulmonary hypertension of the term neonate: a strategy for management.

UNLABELLED: The management of 32 consecutive term infants referred with persistent pulmonary hypertension of the newborn were reviewed. Despite indices suggesting severe cardiorespiratory failure with a median alveolar-arterial oxygen gradient of 591 torr (inter-quartile range 432-618) and oxygenation index of 31 (18-44), all but one patient responded to conventional treatment with inhaled nitric oxide and high frequency oscillatory ventilation. CONCLUSION: Patients should be referred early to centres where maximal conventional support can be offered before consideration for extracorporeal membrane oxygenation.

Prolonged mechanical ventilation as a consequence of acute illness.

OBJECTIVE: To determine why acutely ill children become dependent upon mechanical ventilation and what happens to them. METHODS: A retrospective medical record study of all patients aged between 1 month and 16 years from 1983 to 1996 who required ventilation for more than 28 days. RESULTS: Forty children were ventilated for between 36 and 180 days before discharge or death. Before their presenting illness, 13 (33%) were normal, 15 (37%) had documented predisposing conditions such as bronchopulmonary dysplasia, and the remaining 12 (30%) had diagnoses made after admission. The cause of respiratory failure was central in four patients (10%), spinal cord in eight (20%), neuromuscular in 11 (28%), and pulmonary in 17 (42%). Severe nosocomial infection requiring treatment with intravenous antibiotics occurred in 22. To date, 16 children (40%) have died, and 10 (25%) remain ventilator dependent. Of the 24 survivors, seven (29%) have severe residual neurological deficit. CONCLUSIONS: Increasingly, children are surviving intensive care only to remain ventilator dependent and at risk of significant comorbidity. This study should inform further debate on why such children remain ventilator dependent, and how and where they are managed.

Use of tracheobronchography as a diagnostic tool in ventilator-dependent infants.

OBJECTIVE: Comparison of investigations of the airway in ventilator-dependent infants. DESIGN: Consecutive infants with suspected upper airway abnormalities were investigated using rigid bronchoscopy and tracheobronchography. SETTING: Tertiary pediatric and neonatal intensive care units. PATIENTS: Eight infants with suspected airway abnormalities. INTERVENTIONS: Rigid bronchoscopy and tracheobronchography. MEASUREMENTS AND MAIN RESULTS: Structural abnormalities, segmental narrowing of the airways and the effect of various levels of positive-end expiratory pressures on the narrowings were documented. In six of the eight cases, additional airway abnormalities were diagnosed with tracheobronchography compared with rigid bronchoscopy. CONCLUSIONS: In cases of suspected abnormalities of the upper airway in small infants unable to be weaned from ventilatory support, tracheobronchography may be a more reliable investigation method than rigid bronchoscopy. The ability to assess the structural and dynamic components of the airway accurately and safely allows a correct and long-term treatment plan to be established in this group of patients.

Tracheobronchomalacia in preterm infants with chronic lung disease.

Tracheobronchomalacia is a treatable cause of persisting ventilatory requirements in the preterm neonate, and warrants a high index of suspicion. Five preterm infants with persisting ventilatory requirements with evidence of tracheobronchomalacia are reported. Four were diagnosed by tracheobronchogram and one by flexible endoscopy. All were successfully managed by continuous positive airway pressure (CPAP) via a tracheostomy. One infant died of unrelated causes. The oldest child in this series at the age of 2 years requires no further ventilatory support. Tracheobronchial anomalies should be considered in all preterm infants with persisting ventilatory requirements.

Steroids for intubated croup masking airway haemangioma.

Recently, the beneficial role of steroids for acute laryngotracheobronchitis has been more clearly defined for both intubated and unintubated patients. However, corticosteroids also improve the clinical signs of airway haemangiomata. Two patients are described who illustrate how this can be a source of diagnostic confusion.

Negative extrathoracic pressure ventilation for phrenic nerve palsy after paediatric cardiac surgery.

OBJECTIVE: To investigate the feasibility of negative extrathoracic pressure ventilation as a respiratory support following phrenic nerve palsy after cardiac surgery. DESIGN: An uncontrolled pilot study. PATIENTS: 14 patients aged one week to 30 months (median 5.3 months) with phrenic nerve palsy diagnosed by phrenic nerve conduction tests and diaphragmatic electromyograms. Four had bilateral and 10 unilateral palsy. Before treatment all required oxygen and 10 were receiving positive pressure ventilation. One of the patients with bilateral and four of the patients with unilateral palsies had undergone a plication before negative pressure ventilation was started. INTERVENTION: Treatment was started 6-65 days (median 23) after operation with a newly designed system which included a Perspex chamber, which gave easy access to the child, and an elastic latex neck seal. Continuous negative pressure was used in conjunction with intermittent positive pressure ventilation while continuous or intermittent negative pressure ventilation was used in extubated infants. RESULTS: All four patients with bilateral palsy survived with long-term intermittent negative pressure ventilation and did not require further surgery. Of the 10 with unilateral lesions, seven required no further surgery, two underwent plication, and one had a re-plication. Three patients with unilateral palsy died of non-respiratory causes. The duration of positive pressure ventilation after starting negative pressure ranged from 0 to 23 days (median 6). Treatment with negative pressure lasted for 3-241 days (median 32) and was predominantly administered off the intensive care unit, including at home. CONCLUSIONS: Negative pressure ventilation may be an alternative to positive airway pressure ventilation in the management of phrenic nerve palsy. A multicentre randomised controlled trial is now required to assess further the role of negative pressure ventilation in phrenic nerve palsy.

Phrenic nerve injury in infants and children undergoing cardiac surgery.

Fifty infants and 50 children less than 15 years undergoing palliative or corrective cardiac surgery in the Brompton Hospital between March and October 1988 had direct percutaneous stimulation of the phrenic nerve before and after operation. Ten patients, six under 1 year of age and four over, developed unilateral phrenic nerve injury. In those aged less than 1 year recovery after operation was prolonged because their diaphragmatic palsy made it difficult to wean them from the ventilator. Older children had symptoms but their rate of recovery did not seem to be affected by the phrenic nerve injury. Phrenic nerve damage was no more frequent after a lateral thoracotomy than after a median sternotomy. There was no significant association with the type of operation performed, the experience of the surgeon, the use of bypass or topical ice, the duration of bypass, circulatory arrest or aortic cross clamping, or the age of the patient at the time of operation. In patients who had cardiopulmonary bypass the risk of injury was significantly higher in those who had undergone previous operation. The 10% frequency of phrenic nerve injury determined in this prospective study was higher than that seen in earlier retrospective reports. Direct percutaneous stimulation of the phrenic nerve can be used at the bedside in infants and children to facilitate early and accurate diagnosis of phrenic nerve palsy, and the results may influence early management.

Survival after balloon atrial septostomy for complete transposition of great arteries.

Mortality before surgery must be taken into account when comparing the surgical mortality of atrial redirection procedures (Mustard's or Senning's operation) and the arterial switch operation for patients with complete transposition. This is because the switch operation is usually performed within the neonatal period or early infancy but Mustard's or Senning's operation usually after 4 months of age. The outcome of balloon atrial septostomy was therefore assessed in all 102 infants with transposition of the great arteries (plus or minus associated anomalies) who underwent the procedure at our hospital in the 10 years from January 1975 to December 1984. We considered the procedure to have been unsuccessful if the patient died from any cause (including other surgical procedures) between the septostomy and subsequent interatrial repair (Mustard's operation) or arterial switch operation. Eighteen patients died, although in only two was this as a direct result of the septostomy. Statistical analysis showed that low weight, presence of a persistent arterial duct, and coarctation of the aorta were significant risk factors. Early survival of infants with transposition of the great arteries has been dramatically improved after the introduction of balloon atrial septostomy. Nevertheless, there is considerable attrition before definitive repair, which must be included in the prediction of overall outcome.