IntroductionThoracoscopic esophageal atresia (EA) repair was first performed in 1999, but still the technique is treated as one of the most complex pediatric surgical procedures.AimThe study presents a single-center experience and learning curve of thoracoscopic repair of esophageal atresia and tracheo-esophageal (distal) fistula.Material and methodsFrom 2012 to 2014, 10 consecutive patients with esophageal atresia and tracheo-esophageal fistula were treated thoracoscopically in our center. There were 8 girls and 2 boys. Mean gestational age was 36.5 weeks and mean weight was 2230 g. Four children had associated anomalies. The surgery was performed after stabilization of the patient between the first and fourth day after birth. Five patients required intubation before surgery for respiratory distress. Bronchoscopy was not performed before the operation.ResultsIn 8 patients, the endoscopic approach was successfully used thoracoscopically, while in 2 patients conversion to an open thoracotomy was necessary. In all patients except 1, the anastomosis was patent, with no evidence of leak. One patient demonstrated a leak, which did not resolve spontaneously, necessitating surgical repair. In long-term follow-up, 1 patient required esophageal dilatation of the anastomosis. All patients are on full oral feeding.ConclusionsThe endoscopic approach is the method of choice for the treatment of esophageal atresia in our center because of excellent visualization and precise atraumatic preparation even in neonates below a weight of 2000 g.
Myasthenia gravis is an autoimmune disorder of peripheral nervous system, leading to fluctuating muscle weakness. It is caused by circulating antibodies that block acetylcholine nicotinic postsynaptic receptors at the postsynaptic neuromuscular junction. Myasthenic crisis is a life-threatening complication, which is defined as weakness from acquired myasthenia gravis. In this paper we described a 15-year-old boy who was admitted to the Paediatric Intensive Care Unit due to myasthenic crisis. He had suffered not only from myasthenia gravis but also hypothyroidism, cerebral palsy and epilepsy. The patient required mechanical ventilation and was successfully treated with both plasmapheresis and intravenous immunoglobulins. He recovered from the crisis and then thymectomy was performed. Perioperative period and anaesthesia passed uncomplicated. Discharged home from the hospital after 2.5 month-treatment, for the last 4 years, he has only come on scheduled outpatient medical appointments. This case reveals that myasthenic crisis, albeit rare, may occur in male adolescents. In such cases multidisciplinary care followed by surgery becomes a procedure of choice. Concomitant medical problems, if well controlled, do not affect the results of outcome of the underlying disease.Key words: myasthenia gravis, children; myasthenia gravis, myasthenic crisis; myasthenia gravis, general anaesthesia; myasthenic crisis, management; myasthenic crisis, plasmapheresis Anaesthesiology Intensive Therapy 2013, vol. 45, no 2, 82-84 Myasthenia gravis (MG) is an autoimmune disease in which neuromuscular transmission is impaired due to a partial blocking of acetylcholine receptors in the postsynaptic membrane by circulating antibodies. Clinically, MG manifests as an abnormal fatigability of skeletal muscles that is relieved by rest and anticholinesterase drugs [1]. Its incidence rate is approximately 50-125: 1 000 000. The two major groups of patients include women aged 20-35 years and men aged 60-75 years. Children < 16 years of age constitute approximately 10-16% of all patients. Moreover, the prevalence of MG is three-fold higher in girls than in boys [1].When this disease progresses rapidly or the dosage of anticholinesterase drugs is too low and the susceptibility to them is consequently reduced, MG can progress to a life--threatening condition known as myasthenic crisis. CASE REPORTA 15-year-old boy (with a weight of 70 kg and a height of 165 cm) was admitted to the ICU of the Children's University Hospital from the neurology ward due to progressive hypodynamic respiratory failure and bulbar symptoms. His history revealed myasthenia of the lower limbs and articulation disorders that had been detected a year earlier. Based on clinical examinations, the patient was diagnosed with MG, and treatment with pyridostigmine at a dose of 60 mg day -1 was initiated. Moreover, a diagnosis of hypothyroidism was made, and levothyroxine was started 1 × 50 μg day -1 (euthyreosis for 3 months). The patient had a history of infantile cerebral ...
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