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Postoperative Respiratory Failure in a Child - A Diagnostic Dilemma

Reviewed By Sleep and Respiratory Neurobiology Assembly

Submitted by

Ann C. Halbower, MD

Associate Professor of Pediatrics

Director, Pediatric Sleep Research

University of Colorado, Denver, School of Medicine and The Children's Hospital

Denver, CO

Submit your comments to the author(s).

History

A three-year-old child was admitted for adenotonsillectomy (T&A) after a sleep study demonstrated prolonged hypoxemia, obstructive sleep apnea and hypercarbia.  After the T&A she was given a narcotic for pain.  She did not arouse after withdrawal of anesthetic, was comatose and hypoxemic.  An arterial blood gas showed the following results: pH 7.0/ PaCO2 90 mm Hg/PaO2 50 mm Hg/ bicarbonate 35 mM/L.  She was placed on a ventilator and transferred to the intensive care unit where she remained comatose for 3 days.

Physical Exam

On physical exam, she was a normal-looking child with a slightly box-shaped head.  Head, neck and throat exam was normal with mild swelling of the pharynx postoperatively.  Her pupils were 4 mm with a diminished light response.  Retinas were normal.  Thyroid was soft and small.  Lungs were clear to auscultation with mechanical breaths from the ventilator, but she had no spontaneous breathing while comatose.  Heart examination demonstrated a regular rate and rhythm with no murmur.  She had a benign abdomen with no organomegaly, and there was no swelling or clubbing of her extremities.  Her neurologic exam included pupils that were equally round and reactive to light but accommodation could not be checked.  Deep tendon reflexes were normal and a babinski was not present.  She was unable to perform strength or cerebellar testing.

Lab

During her comatose state while ventilated, electroencephalogram and blood tests were normal.  An electrocardiogram demonstrated sinus bradycardia of 40 beats per minute.  She had marked sinus arrhythmia.  There was a normalization of her blood gas results to pH 7.4/ PaCO2 40 mm Hg/ PaO2 80 mm Hg during mechanical ventilation.  She woke up after 3 days and the physicians attempted to wean her to continuous positive airway pressure (CPAP).  She was placed on oxygen at 1 L/min with a CPAP of 6 cm H2O.  Oxygen saturation was 99%.  She seemed fine during the day, but the next morning became obtunded.  A blood gas demonstrated a PaCO2 of 102 mm Hg.  She underwent tracheostomy and was ventilated.  A polysomnogram during sleep was performed.

Figures

The following 4 figures are during the polysomnogram on and off a mechanical ventilator during sleep.

Figure 1

Figure 2

Figure 3

Figure 4

Question 1

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References

  1. Weese-Mayer DE, Rand CM, Berry-Kravis EM, et al. Congenital central hypoventilation syndrome from past to future: model for translational and transitional autonomic medicine. Pediatr Pulmonol 2009; 44(6):521-535.
  2. Harper RM, Macey PM, Woo MA, et al. Hypercapnic exposure in congenital central hypoventilation syndrome reveals CNS respiratory control mechanisms. J Neurophysiol 2005; 93(3):1647-1658.
  3. Gronli JO, Santucci BA, Leurgans SE, et al. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr Pulmonol 2008; 43(1):77-86.
  4. Repetto GM, Corrales RJ, Abara SG, et al. Later-onset congenital central hypoventilation syndrome due to a heterozygous 24-polyalanine repeat expansion mutation in the PHOX2B gene. Acta Paediatr 2009; 98(1):192-195.