Holoprosencephaly (HPE) is a congenital brain malformation resulting from incomplete prosencephalic cleavage with an incidence of approximately 1 in 8,000 live births. Alobar HPE, the most severe form, is often associated with airway-related craniofacial anomalies such as a single nostril, absent columella, and nasal septal defects, which may complicate postoperative airway management. Here, we report two pediatric cases of alobar HPE with single nostril that presented difficult postoperative airway management after cheiloplasty.
The first case involved a 22-month-old girl with a median cleft lip, a single nostril, and hypotelorism. She also had intractable epilepsy, central hypopituitarism, and thermoregulatory dysfunction and required home oxygen therapy. The anesthesia was uneventful. A nasopharyngeal airway (NPA) was inserted before extubation. Postoperatively, excessive secretion led to oxygen desaturation, which resolved after NPA removal. The remainder of the postoperative course was stable.
The second case involved a 19-month-old boy who also had a median cleft lip, a single nostril, hypotelorism, and a history of epilepsy without respiratory disorders. The anesthesia was uneventful. After extubation, retractive breathing appeared, which was improved by NPA insertion. However, the patient experienced seizure-like episodes and respiratory deterioration the following day, resulting in difficult airway management.
These cases suggest that in patients with alobar HPE and a single nostril, cheiloplasty may further narrow the nasal and oral airways, thereby increasing the risk of postoperative respiratory compromise. Careful postoperative airway evaluation, appropriate NPA positioning, and close monitoring are essential.
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