Anesthesia Management in a Rare Case: Wolf-Hirschhorn Syndrome

Year 2025, Volume: 78 Issue: 2, 164 - 166, 01.07.2025

Özgün Ömer Asiller , Ezgi Yıldırım , Barış Sakul Menekşe Özçelik

Abstract

Wolf-Hirschhorn syndrome is a rare disease and may cause difficulties in airway management. In addition, intraoperative complications may occur.
We will share with you our anesthesia management experience in a patient who is scheduled to undergo repair due to a cleft lip.

Keywords

Wolf-Hirschhorn syndrome , pediatric anesthesia , rare diseases

Ethical Statement

Informed Consent: Informed consent was obtained both orally and in writing from the patient’s legal representative.

Supporting Institution

-

Project Number

03779

Thanks

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Nadir Bir Olguda Anestezi Yönetimi: Wolf-Hirschhorn Sendromu

Abstract

Wolf-Hirschhorn sendromu nadir görülen bir hastalıktır ve hava yolu yönetiminde zorluklara neden olabilir. Ayrıca, intraoperatif komplikasyonlar
meydana gelebilir. Dudak yarığı nedeniyle onarım planlanan bir hastada anestezi yönetimi deneyimimizi sizinle paylaşacağız.

Keywords

Wolf-Hirschhorn sendromu , pediatrik anestezi , nadir hastalıklar

Ethical Statement

-

Supporting Institution

-

Project Number

03779

Thanks

-

References

• 1. Hirschhorn K, Cooper HL. Chromosomal aberrations in human disease. A review of the status of cytogenetics in medicine. Am J Med. 1961;31:442- 470.
• 2. Wolf U, Reinwein H, Porsch R, Schröter R, Baitsch H. [Deficiency on the short arms of a chromosome No. 4]. Humangenetik. 1965;1:397-413.
• 3. Battaglia A, Carey JC, South ST. Wolf-Hirschhorn syndrome: A review and update. Am J Med Genet C Semin Med Genet. 2015;169:216-223.
• 4. Battaglia A, Filippi T, Carey JC. Update on the clinical features and natural history of Wolf–Hirschhorn (4p-) syndrome: Experience with 87 patients and recommendations for routine health supervision. Am J Med Genet C Semin Med Genet. 2008;148C:246-251.
• 5. Gamble JF, Kurian DJ, Udani AG, Greene NH. Airway management in a patient with Wolf-Hirschhorn syndrome. Case Rep Pediatr. 2016;2016:7070125.
• 6. Choi JH, Kim JH, Park YC, Kim WY, Lee Y-S. Anesthetic experience using total intra-venous anesthesia for a patient with Wolf-Hirschhorn syndrome-A case report. Korean J Anesthesiol. 2011;60:119-123.
• 7. Tsukamoto M, Yamanaka H, Yokoyama T. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report. J Dent Anesth Pain Med. 2017;17:231.
• 8. Kim HJ, You JA, Park S, Kim EJ, Park SJ, Kim HY. Anesthetic considerations for an adult with Wolf-Hirschhorn syndrome - A case report. Anesth Pain Med (Seoul). 2020;15:120-123.
• 9. Bloor M, Nandi R, Thomas M. Antiepileptic drugs and anesthesia. Paediatr Anaesth. 2017;27:248-250.
• 10. Abdallah C. Considerations in perioperative assessment of valproic acid coagulopathy. J Anaesthesiol Clin Pharmacol. 2014;30:7.
• 11. Sandler SG, Mallory D, Malamut D, Eckrich R. IgA anaphylactic transfusion reactions. Transfus Med Rev. 1995;9:1-8.
• 12. Bosenberg A. Anaesthesia and Wolf-Hirschhorn syndrome. SAJAA. 2014;13:31-34.
• 13. Ginsburg R, Purcell-Jones G. Malignant hyperthermia in the Wolf-Hirschhorn syndrome. Anaesthesia. 1988;43:386-388.
• 14. Chen JC, Jen RK, Hsu YW, et al. [4P- syndrome (Wolf-Hirschhorn syndrome) complicated with delay onset of malignant hyperthermia: a case report]. Acta Anaesthesiol Sin. 1994;32:275-278.
• 15. Hulin J, Veyckemans F. [Anaesthetic management of the Wolf-Hirschhorn syndrome: a report of two cases]. Ann Fr Anesth Reanim. 2012;31:89-90.
• 16. Humston C, Bernard R, Khan S, Tobias JD. Perioperative care of an infant with wolf-hirschhorn syndrome: is there a risk of malignant hyperthermia. J Med Cases. 2016;7:126-129.