Central hypoventilation with cerebellar cortical dystrophy and hypoplasia of arcuate nuclei of brainstem

Pei Chen Tsao, Shu Jen Chen, Jen Her Lu, Wen Jue Soong, Yi Chen Yeh, Chin Chen Pan, Renbing Tang

Research output: Contribution to journalArticlepeer-review

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 (SaO2) 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.

Original languageEnglish
JournalBMJ Case Reports
DOIs
Publication statusPublished - Sep 20 2009
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)

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