title: Central hypoventilation with cerebellar cortical dystrophy and hypoplasia of arcuate nuclei of brainstem.
abstract: A baby boy was emergently delivered through normal spontaneous delivery due to meconium stain of amniotic fluid; his gestational age was 39 weeks, birth body weight 2935 g, head circumference 33 cm, Apgar scores were 6 and 9 at 1 min and 5 min, respectively. After birth, initially the baby behaved vigorously; however, nasal flaring and subcostal retractions occurred later. A chest x ray revealed a ground glass appearance. A nasal prong with intermittent mandatory ventilation was applied initially and was shifted to endotracheal intubation 1 day after birth due to persistent tachypnoea and subcostal retraction. Extubation was performed at the age of 3 days; however, frequent apnoea and arterial oxygen saturation (SaO(2)) down to 40% to 50% during sleep were noted and reintubation was performed. Apnoea and desaturation still happened if the ventilator rate was slowed down. A pulmonary function test revealed feeble chest wall motion and small tidal volume (2-3 ml/kg). It was attempted to wean the patient from the ventilator again at the age of 11 days. Unfortunately, the patient's condition deteriorated suddenly. The baby survived for only 12 days.
authors.affiliation: Veterans General Hospital, No. 201, Section 2, Shih-Pai Road, Taipei, Taiwan.
authors.forename: Pei-Chen
authors.initials: PC
authors.lastname: Tsao
authors.forename: Shujen
authors.initials: S
authors.lastname: Chen
authors.forename: Jen-Her
authors.initials: JH
authors.lastname: Lu
authors.forename: Soong
authors.initials: S
authors.lastname: Wen-Jue
authors.forename: Yeh
authors.initials: Y
authors.lastname: Yi-Chen
authors.forename: Pan
authors.initials: P
authors.lastname: Chin-Chen
authors.forename: Renbing
authors.initials: R
authors.lastname: Tang