A male infant was born at 38 weeks of gestation to a 26-year-old female. An early foetal ultrasound scan had shown a large abdominal mass suspected to be renal cystic dysplastic disease; this was accompanied by polyhydramnios. Antenatal karyotyping was normal. Labour was induced in view of this underlying abnormality, and the mother proceeded to a normal vaginal delivery. Although antenatal drainage of the renal mass was performed before induction, the delivery was complicated by abdominal dystocia requiring emergency abdominal paracentesis. The body was delivered 15 min after the head and shoulders.
At resuscitation, he immediately required very high inspiratory pressures and, despite maximal ventilatory support, including inhaled nitric oxide and high-frequency oscillation, he remained deeply cyanosed. A chest X-ray showed bilateral pneumothoraces, which were successfully drained. He continued to deteriorate despite maximal support, and intensive care was withdrawn. He died at 4 hours of age.
At autopsy, he weighed 3720 g and was of an appropriate size for gestation. There were no external dysmorphic features. The lungs had normal lobation, and their combined weight was within normal limits. The brain and spinal cord were normal. The histology of the lung distal to the bronchi was abnormal. It was composed of irregularly branching airspaces with a ciliated cuboidal lining. Capillaries in adjacent connective tissue were reduced in number. Normal alveoli were not present. Both lobules and peripheral airspaces were separated by abundant loose interstitial connective tissue. The bronchi and bronchioles showed numerous abnormal epithelial outpouchings lined by ciliated epithelium. The left kidney appeared microscopically normally formed, and the right kidney had cystic dysplasia.
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