Rapid development of life-threatening complete atrioventricular block in Kearns-Sayre syndrome.

Kearns-Sayre syndrome is a rare mitochondrial disorder with defined diagnostic criteria. Knowledge of these diagnostic criteria and early diagnosis are important to ensure periodic electrocardiograms for identification of cardiac conduction disorders, which are the most important prognostic factor of the disease. We report on a 9-year-old girl with rapid development of a life-threatening complete atrioventricular block within 10 months and discuss the importance and time interval of regular electrocardiograms. Our patient survived by placing a temporary transvenous pacemaker lead followed by permanent pacemaker implantation a few days later.

Remifentanil for INSURE in preterm infants: a pilot study for evaluation of efficacy and safety aspects.

AIM: To evaluate intubating conditions, extubation times and outcome in preterm infants receiving remifentanil as induction agent for the INSURE procedure. METHODS: In twenty-one preterm infants of 29 to 32 weeks gestation and signs of respiratory distress, we utilized remifentanil as induction agent for the INSURE procedure. Following intubation and surfactant application, the infants were mechanically ventilated until respiratory drive was judged to be satisfactory for continuing CPAP therapy. Intubating conditions were classified by our own scoring system by rating limb movements, coughing and breathing. Heart rate, blood pressure and oxygen saturation were recorded during the entire INSURE procedure. RESULTS: Remifentanil provided excellent or good intubating conditions in all patients. We observed no serious side effects after remifentanil infusion, in particular, no thorax rigidity, clinically significant bradycardia or arterial hypotension. Average extubation time after surfactant administration was 16.9 min (1-45 min); none of the infants had to be reintubated. Following extubation, the infants required only 3.3 days (1-8 days) of CPAP therapy. None exhibited serious complications of prematurity like periventricular leucomalacia, intraventricular haemorrhage >I degree, necrotizing enterocolitis or retinopathy. CONCLUSION: In this pilot study, INSURE with remifentanil was associated with good intubating conditions and early extubation resulting in an excellent neonatal outcome.

Primary respiratory support in preterm infants with cleft lip and palate.

OBJECTIVE: Preterm infants with respiratory distress are routinely treated by application of nasal mask continuous positive airway pressure. In preterm infants with cleft lip and palate, nasal mask attachment is not feasible due to air leakage through the cleft defect. Here, we describe a modified continuous positive airway pressure application method that overcomes this problem. DESIGN AND SUBJECTS: Observation study, university neonatal intensive care unit. The neonates (n = 4) were between 28 and 33 weeks' gestation and weighed 1160 to 1680 g at birth. Immediately after birth, infants with unilateral cleft lip and palate (n = 3) were respiratory stabilised by a Medijet generator using a nasal tube. To minimise the pressure cap, hydrocolloid bandages were adhered over the total cleft defect. Immediately after orthodontic passive palatal plates insertion (within 27 hours of life), the nasal tube was removed and continuous positive airway pressure was applied through a nasal mask covering the complete nose of the infant. RESULTS: The system proved suitable for patients with unilateral cleft lip and palate for whom the generated nasal mask continuous positive airway pressure remained constant between 5 to 7 cm of water but failed in the patient with bilateral cleft lip and palate. None of the patients had to be ventilated due to respiratory failure, and all survived to discharge. CONCLUSION: Preterm infants with unilateral, but not bilateral cleft lip and palate, can be successfully stabilised using the described nasal mask continuous positive airway pressure system, thereby avoiding primary intubation and its associated risk of complications.

Aerosolized iloprost in the treatment of pulmonary hypertension in extremely preterm infants: a pilot study.

BACKGROUND: In premature infants with preterm prolonged rupture of membranes, death after birth is often due to persistent pulmonary hyper-tension. PATIENTS: Aerosolized iloprost was used to treat pulmonary hypertension due to prolonged preterm rupture of fetal membranes (7-56 days) in four extremely low-birthweight neonates (23-25 weeks' gestation, weight 448-645 g) under spontaneous breathing supported by nasal continuous positive airway pressure. METHOD: Inhalation dose was 2 microg/kg b.w. and between 44 and 65 inhalations were performed in each patient starting within the first hour of life over a total of several days. Single inhalations lasted 5 min and were not repeated until 60 min had elapsed. RESULTS: After the first inhalation, the PaO2/FiO2 mean ratio increased from 65 (range 35-114) to 194 (148-250) mmHg and oxygenation requirements decreased within the next 7 days. Echocardiography similarly showed reduction in pulmonary resistance. We observed no severe side effects on blood pressure or prolonged bleeding time during inhalation. CONCLUSIONS: Iloprost inhalation might therefore be an additional treatment for improving oxygenation in cases of persistent pulmonary hypertension in extremely low-birthweight infants under spontaneous breathing. Further randomized clinical studies are required to establish the role of iloprost in this setting.