A 27-year-old girl and 22 weeks gestation was referred with presumptive ultrasound diagnosis of cystic pulmonary adenomatoid (CMA).
Ultrasound reassessment revealed a massive increase in echogenicity, with marked increase in lung size, decrease and inversion of both diaphragms and dilatation of the intrathoracic airway without fetal ascites.
The presumptive diagnosis was CHAOS.
1.
a.
Coronal section of the fetal neck and chest.
The upper airway was markedly dilated trachea; b.
The transversal section of the chest shows a large increase in the size of both lung fields.
Figure 1.
Fetal ultrasound
Underlying ultrasound showed adequate fetal growth, with higher lung volume and amniotic fluid and rectal dilatation.
Fetal echocardiography showed congenital malformations.
To corroborate the diagnosis, a fetal magnetic resonance imaging was performed at 35 weeks, confirming: laryngeal stenosis, dilation of the airway distal to the obstruction and increased lung size.
A fetal karyotype was suggested, to which the parents opposed.
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Fetal magnetic resonance imaging (coronal chordae) showed narrowing of the fetal larynx (cloud plate), tracheal dilatation (large foil) and pulmonary enlargement (asteris).
Figure 2.
Fetal magnetic resonance imaging
At 36 weeks, the increase in polyhydramnios was marked.
It was decided to perform fetal lung maturation with glucocorticoids without amniocentesis, in order not to increase the risk of triggering delivery.
A multidisciplinary team of obstetricians, anesthesiologists, neonatologists and endoscopists was formed to inform the patient and her partner about prenatal diagnosis and available maternal-fetal therapeutic options, with risks and benefits.
Bibliography was provided to them and, after reading the informed consent, they agreed to undergo a scheduled cesarean section with EXIT.
After propofol induction, sevofluorane and remifentanil were used as anesthetics and becuronium as uterine inhibitor.
To maintain the intrauterine volume, a minimal hysterotomy was performed to expose the head and neck, avoiding amnioinfusion.
With protection of the umbilical cord, the fetal head was exposed and a videolaryngoscopy was performed, which confirmed a laryngeal atresia that would prevent natural ventilation and conventional endotracheal intubation, so it was decided to perform a ventilation procedure.
During the procedure, which lasted 25 minutes, normal fetal arterial oxygen saturation (60%) was verified with sensors in the earlobe.
After securing the airway, we proceeded to the complete extraction of a live male fetus of 2800 g, whose acid-base status (umbilical cord) was: pH= 7.45; pCO2= 96%; pCO2= 35;
A central venous line was placed and maintained under assisted ventilation in intensive care for 48 h.
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Head extraction to perform videolaryngoscopy and determine laryngeal injury
Figure 3.
Fetal extraction
After clamping the cord and performing hysterorrhaphy, oxytocin was administered, which reversed uterine hypotonia.
The neonatal chest X-ray showed air attraction, costal horizontalization and tracheostomy cannula.
The child had laryngeal atresia, splenic agenesis, anal imperforation and significant communication.
Twenty-four hours after surgery, the patient developed congenital heart disease and three weeks after surgery.
Laryngeal reconstruction (laryngotracheofissure) was performed one year later and, after two years, the fistula was closed (total resection).
Currently, at the age of 4 years, the growth and weight development, and the timbre and vocal volume are normal and attend the garden of children without difficulties.
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Postnatal radiography showed increased lung volume, costal horizontalization and tracheostomy cannula.
Figure 4.
Postnatal radiography
