We report a case of a very low birth weight infant treated successfully with tracheoplasty using the thymus against tracheo-esophageal fistula due to necrotizing tracheobronchitis. A baby boy was born at 31 weeks gestation with a birth body weight of 1,230 g. Suddenly on Day 19, his respiration deteriorated, and a flexible bronchofiberscopy showed tracheostenosis, tracheomalacia, and tracheal diverticula. On Day 21, his abdomen became significantly distended, and a flexible bronchofiberscopy showed a tracheo-esophageal fistula due to a rupture of the diverticula. On Day 105, during surgery, we confirmed necrosis of the trachea. Because the lesion was widespread, we used the thymus for tracheoplasty and closure of the tracheo-esophageal fistula. His post-operative course went well, and was discharged at 1 year 9 months of age. Now, 8 years after the operation, although he still needs a T-tube airway management through tracheostomy, he has achieved speech, normal growth and development, and takes regular classes at school. Tracheoplasty using the thymus is thought to be a treatment worth considering when there are no other effective alternative treatments for tracheo-esophageal fistula due to necrotizing tracheobronchitis.
Background
A definitive diagnosis of congenital central hypoventilation syndrome (CCHS) is made by genetic testing. However, there are only a few examinations that warrant genetic testing. Electrical activity of the diaphragm (Edi) reflects neural respiratory drive from respiratory center to diaphragm. We evaluated the function of the respiratory center in CCHS by Edi monitoring.
Methods
Monitoring of Edi was performed in six CCHS cases without mechanical ventilation. The monitoring time was 30 consecutive minutes from wakefulness to sleep. The TcPCO2 or EtCO2 and SpO2 were recorded simultaneously.
Results
The Edi peak during wakefulness was 14.0 (10.3–21.0) µV and the Edi peak during sleep was 6.7 (3.8–8.0) µV. The Edi peak during sleep was significantly lower than the Edi peak during wakefulness, and patients were in a state of hypoventilation. Although TcPCO2 or EtCO2 increased due to hypoventilation, an increase in the Edi peak that reflects central respiratory drive was not observed. ΔEdi/ΔCO2 was −0.06μV/mmHg. Maximum EtCO2 or TcPco2 was 51 mmHg, and the average SpO2 was 91.5% during monitoring.
Conclusions
We confirmed that Edi monitoring could evaluate the function of the respiratory center and reproduce the hypoventilation of CCHS. The present study suggested that Edi monitoring is a useful examination in deciding whether to perform genetic testing or not and it may lead to an early diagnosis of CCHS.
Background Methods of evaluating the ventilatory response to CO2 (VRCO2)
of the respiratory center include the steady-state and the rebreathing
method. Although the rebreathing method can evaluate the respiratory
center more in detail, the steady-state method has been mainly performed
in infants. The aim of this study was to investigate whether we could
perform the VRCO2 with the rebreathing method in normal infants. Methods
The subjects were 80 normal infants. The gestational age was
39.9(39.3-40.3)weeks, and the birth body weight was 3,142
(2,851-3,451) grams. We performed the VRCO2 with Read’s rebreathing
method, measuring the increase in minute volume (MV) in response to the
increase in EtCO2 by rebreathing a closed circuit. The value of VRCO2
was calculated as follow: VRCO2 (mL/min/mmHg/kg) = ΔMV / ΔEtCO2 / Body
weight. Results We performed the examination without adverse events. The
age in days at examination was 3 (2-4), and the examination time was
150±38 seconds. The maximum EtCO2 was 51.1 (50.5-51.9) mmHg. The value
of VRCO2 was 34.6 (29.3-42.8). Tidal volume had a greater effect on the
increase in MV than respiratory rate (5.4 to 14.3 mL/kg, 44.1 to 55.9
/min, respectively). Conclusion This study suggests that the rebreathing
method can evaluate the ventilatory response to high blood CO2 in a
short examination time. We conclude that the rebreathing method is
useful even in infants. In the future, we plan to measure the VRCO2 of
preterm infants, and evaluate the respiratory center of infants in more
detail.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.