Abstract
Crouzon syndrome (CS) is a rare autosomal dominant inherited disorder caused by mutations in the fibroblast growth factor receptor 2 (FGFR2) gene. The disease is characterized by premature fusion of the coronal and sagittal sutures of the skull, resulting in clinical manifestations of midfacial hypoplasia, shallow orbit, maxillary dysplasia, and occasional upper respiratory obstruction. This article presents the case of a child aged 2 years and 7 months with CS scheduled for bilateral tonsillectomy and adenoidectomy. The patient had a difficult procedure of extubation and was reintubated and the tracheal intubation was removed 2 days after surgery. The CS is a rare condition with physical characteristics that can result in difficult airway manipulation. It is important for anesthesiologists to recognize and avoid potential airway complications in the management of such patients through detailed preoperative evaluation and careful observation after surgery to reduce perioperative risks.
Zusammenfassung
Das Crouzon-Syndrom (CS) ist eine seltene autosomal-dominant vererbte Krankheit, die durch Mutationen in dem Gen des Fibroblasten-Wachstumsfaktor-Rezeptors 2 (FGFR2) verursacht wird. Die Erkrankung ist durch eine vorzeitige Fusion der koronalen und sagittalen Nähte des Schädels gekennzeichnet, was zu klinischen Manifestationen wie Mittelgesichtshypoplasie, flacher Augenhöhle, maxillarer Dysplasie und gelegentlicher Obstruktion der oberen Atemwege führt. Dieser Beitrag stellt den Fall eines Kindes im Alter von 2 Jahren und 7 Monaten mit CS vor, bei dem eine bilaterale Tonsillektomie und Adenoidektomie geplant war. Der Patient hatte einen schwierigen Extubationseingriff und wurde reintubiert. Die Trachealintubation wurde 2 Tage nach der Operation entfernt. Das CS ist ein seltener Zustand mit körperlichen Merkmalen, die zu einer schwierigen Manipulation der Atemwege führen können. Für Anästhesisten ist es wichtig, potenzielle Atemwegskomplikationen bei der Behandlung solcher Patienten zu erkennen und zu vermeiden, und zwar durch eine detaillierte präoperative Beurteilung und sorgfältige Beobachtung nach der Operation, um die perioperativen Risiken zu reduzieren.
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Acknowledgements
We thank the patient and relatives for permission to share with the readers the diagnosis and treatment of difficult ventilation in CS. Yang Xiao helped to revise and translate the report.
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X. Wang, Z. Xu, Y. Xiao and G. Chen declare that they have no competing interests.
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For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case. Additional written informed consent was obtained from the legal representatives of the patient for whom identifying information is included in this article.
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Wang, X., Xu, Z., Xiao, Y. et al. Successful management of anesthesia complications in a child with Crouzon syndrome. Anaesthesist 69, 432–435 (2020). https://doi.org/10.1007/s00101-020-00778-7
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DOI: https://doi.org/10.1007/s00101-020-00778-7