A 3-year-old boy with trisomy 18 developed a fatal tracheoarterial fistula involving an anomalous left common carotid artery originating from the brachiocephalic trunk, highlighting the clinical importance of preoperative vascular mapping in patients with chromosomal abnormalities.
Key Findings
Results
A pediatric patient with trisomy 18 developed a tracheoarterial fistula (TAF) involving the left common carotid artery (LCCA) following emergency tracheostomy.
The patient was a 3-year-old Japanese boy with trisomy 18.
Emergency tracheostomy was performed for respiratory failure secondary to pneumonia.
Massive hemorrhage from the tracheostoma occurred on postoperative day 31.
The hemorrhage led to the patient's death.
Results
Autopsy revealed that the LCCA had an anomalous origin from the brachiocephalic trunk and coursed medially, forming a fistulous connection with the trachea.
Normally, the LCCA originates directly from the aortic arch; in this case it arose from the brachiocephalic trunk.
The anomalous medial course of the LCCA placed it in proximity to the trachea, predisposing fistula formation.
The anatomical anomaly was identified post-mortem via autopsy.
This vascular anomaly was not identified prior to the surgical procedure.
Background
The long-term survival of individuals with trisomy 18 has improved, increasing the need for tracheostomy in this population.
The authors note that improved long-term survival in trisomy 18 has led to increased use of tracheostomy in this population.
Trisomy 18 is associated with chromosomal abnormalities that may include vascular anomalies.
This trend increases the clinical relevance of understanding procedure-related risks specific to this population.
Conclusions
The authors recommend preoperative vascular mapping before tracheostomy in patients with chromosomal abnormalities such as trisomy 18.
The case highlights the clinical importance of preoperative vascular mapping to identify anomalous vessels near the surgical field.
Vascular anomalies in chromosomal abnormality patients may not be apparent without dedicated imaging.
Failure to identify the anomalous LCCA course preoperatively contributed to the fatal outcome in this case.
What This Means
This research describes the case of a 3-year-old boy with trisomy 18 (a chromosomal condition) who required emergency surgery to create a breathing hole in his throat (a tracheostomy) after developing severe pneumonia. About a month after the surgery, he experienced a sudden, massive, fatal bleeding episode. An autopsy revealed the cause: one of the major arteries in his neck (the left common carotid artery) was positioned unusually close to the trachea due to an abnormal anatomical route, and the tracheostomy tube had worn through the wall between the artery and the airway, creating a dangerous connection called a fistula.
Normally, the left common carotid artery branches directly off the main artery from the heart (the aorta), but in this boy it branched off a different vessel and took an unusual path that brought it dangerously close to the trachea. This abnormal positioning was not detected before surgery. The authors note that as medical care has improved, more children with trisomy 18 are surviving longer and may require tracheostomies, making awareness of this risk more important.
This research suggests that doctors should consider performing detailed imaging of the blood vessels near the neck and throat (vascular mapping) before performing a tracheostomy in patients with chromosomal abnormalities like trisomy 18, who may be more likely to have unusual vascular anatomy. Identifying such anomalies beforehand could allow surgeons to adjust their approach and potentially prevent life-threatening complications.
Shiina T, Watanabe H, Yoshimoto J, Sato T, Morimoto D, Okamura T, et al.. (2026). Post-Tracheostomy Fistula Formation Between the Trachea and Left Common Carotid Artery in a Pediatric Patient with Trisomy 18.. Acta medica Okayama. https://doi.org/10.18926/AMO/71068