Esophageal duplication cyst is a rare congenital anomaly resulting from a foregut budding error during the fourth to sixth week of embryonic development. Cervical esophageal duplication cysts are very rare and may cause respiratory distress in infancy. A full-term newborn girl who was born by normal delivery was transferred to our hospital because of swelling of the right anterior neck since birth. Cervical ultrasonography showed a 40 × 24 × 33 mm simple cyst on the right neck. Tracheal intubation was required at 2 weeks of age because of worsening external compression of the trachea. Fine-needle aspiration cytology revealed the existence of ciliated epithelium. At 1 month of age, exploration was performed through a transverse neck incision. The cyst had a layer of muscle connected to the lateral wall of the esophagus. Histopathological diagnosis was a cervical esophageal duplication cyst. We describe the clinical features of infantile cervical esophageal duplication cysts based on our experience of this rare disease in a neonate, along with a review of 19 cases previously reported in literature.

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BITI was measured in healthy infants. Further evaluation is needed to determine the significance and usefulness of BITI.

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.