Case report: anesthesia in patients with asphyxiating thoracic dystrophy: Jeune syndrome.
OBJECTIVE
Jeune Syndrome or Asphyxiating Thoracic Dystrophy is a recessive autosomal disease. This syndrome is characterized by a bone dysplasia with varied abnormalities: thoracic, pancreatic, cardiac, hepatic, renal and retinal. Patients' age when the clinical condition is experienced correlates with the disease severity. These patients experience polychondrodystrophy with large, short, horizontal ribs and irregular costochondral junctions resulting in a rigid and reduced thoracic cage with varied respiratory injury level.


METHODS
Male patient, 4 months-old, 7kg, suffering with Asphyxiating Thoracic Dystrophy, intubated and presented with reduced thoracic cage. Echocardiogram: mild pulmonary hypertension. Chest tomography: pulmonary hypoplasia. Patient submitted to bilateral thoracoplasty and thoracotomy with general anesthesia. Anesthesia maintenance: sufentanil e sevoflurane continuous infusion. Ventilation parameters: pressure-cycled mechanical ventilation. Thorax opening provided improvement of the ventilation parameters, but after thoracic prosthesis placement, ventilation was limited. Reduction of the thoracic prosthesis was considered with consequent improvement of ventilation.


CONCLUSIONS
Diagnosis of all present abnormalities is essential for the correct anesthetic management. Observation was necessary to adequate pre- and post-thoracotomy/thoracoplasty ventilation and to maintain patient hemodynamically stable. Pressure-cycled mechanical ventilation is the most adequate type of ventilation to overcome the mechanical barrier. In the intraoperative setting, the ideal is to maintain the inspiratory pressure peak as low as possible to minimize the risk of barotrauma, venous return impairment and reduced cardiac output.
ABC Foundation and Medical School at ABC, Rua Pedro Pomponazzi 230, São Paulo, SP, Brazil.
Deise
D
Saletti
Thiago Ramos
TR
Grigio
Deoclecio
D
Tonelli
Onésimo Duarte
OD
Ribeiro Júnior
Fabríccio
F
Marini
D000768:Anesthesia, General / Q000379:methods*; D004613:Ellis-Van Creveld Syndrome; D006801:Humans; D007223:Infant; D008297:Male