Wolf-Hirschhorn Sendromlu pediatrik bir hastanın anestezi yönetimi: Olgu sunumu

Yıl 2025, Cilt: 30 Sayı: 1, 136 - 139, 29.01.2025

Erol Karaaslan

https://doi.org/10.21673/anadoluklin.1491983

Öz

Wolf-Hirschhorn sendromu (WHS) 4. kromozomun kısa kolunun silinmesiyle ortaya çıkan nadir görülen bir kromozomal anomalidir. Önde gelen ölüm nedenleri aspirasyon pnomonisi, epilepsi ve enfeksiyondur. Bu çalışmamızda WHS’ lu yarık damak cerrahisi planlanan Total intravenöz anestezi (TİVA) uyguladığımız bir olguda anestezi deneyimimizi sunmayı amaçladık.
WHS tanısıyla takip edilen, yarık damak cerrahisi planlanan 8 yaşında, 13 kg olan olgumuzun klinik muayenesinde, gelişme geriliği, yarık damak, mikrognati, epikantus ve hiptoni mevcuttu. Anestezi indüksiyonunda 1mg/kg i.v lidokain, 2mg/kg i.v propofol ve 1mcg/kg i.v fentanil, 0.5 mg/kg i.v rocuronium bromide uygulandı. Anestezi idamesinde TİVA (0.5 µg/kg/dk remifentanil ve 75 µg/kg/dk propofol i.v) kullanıldı. Perioperatif dönemde nabız kısa süreli 170/atım/dk oldu. Cerrahi bitiminden 13 dk sonra ekstübasyon sağlandı.
WHS sendromlu çocukların anestezi yönetiminde TİVA (Propofol ,remifentanil)’ nın ve entübasyon cihazı olarak C-MAC videolaringoskopun güvenilir bir yöntem olduğunu düşünüyoruz.

Anahtar Kelimeler

Anestezi, havayolu yönetimi, laringoskop, Wolf-Hirschhorn sendromu

Anesthesia management of a pediatric patient with Wolf-Hirschhorn Syndrome: A case report

Öz

Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal anomaly caused by the deletion of the short arm of chromosome 4. The leading causes of death are aspiration pneumonia, epilepsy, and infection. In this study, we aimed to present our anesthesia experience in a patient with WHS who was scheduled for cleft palate surgery and in whom we performed total intravenous anesthesia (TIVA).
In the clinical examination of our 8-year-old patient with WHS, weighing 13 kg, who was planned to undergo cleft palate surgery. Growth retardation, cleft palate, micrognathia, epicanthus and hyptonia were present. Anesthesia induction was performed with 1 mg/kg i.v lidocaine, 2 mg/kg i.v propofol and 1 mg/kg i.v fentanyl, 0.5 mg/kg i.v rocuronium bromide. TIVA (0.5 µg/kg/min remifentanil and 75 µg/kg/min propofol i.v) was used for maintenance of anesthesia. In the perioperative period, the pulse rate was 170 beats/min for a short time. Extubation was achieved 13 minutes after the end of surgery.
We think that TIVA (Propofol, remifentanil) and C-MAC videolaryngoscope as intubation device is a reliable method in the anesthetic management of children with WHS syndrome.

Anahtar Kelimeler

Airway management, anesthesia, laryngoscope, Wolf-Hirschhorn syndrome

Etik Beyan

Financial Resource No financial resources were used for this article. Conflict of Interest There is no conflict of interest regarding this article.

Destekleyen Kurum

yok

Teşekkür

yok

Kaynakça

• Rutherford EL, Lowery LA. Exploring the developmental mechanisms underlying Wolf-Hirschhorn Syndrome: Evidence for defects in neural crest cell migration. Dev Biol. 2016;420(1):1-10.
• Paradowska-Stolarz AM. Wolf-Hirschhorn syndrome (WHS) - literature review on the features of the syndrome. Adv Clin Exp Med. 2014;23(3):485-9.
• Bösenberg AT. Anaesthesia and Wolf-Hirschhorn syn-drome. SAJAA. 2007;13(3):31-4.
• Shannon NL, Maltby EL, Rigby AS, Quarrell OW. An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality. J Med Genet. 2001;38(10):674-9.
• Aldrete JA, Kroulik D. A postanesthetic recovery score. Anesth Analg. 1970;49(6):924-34.
• Ginsburg R, Purcell-Jones G. Malignant hyperthermia in the Wolf-Hirschhorn syndrome. Anaesthesia. 1988;43(5):386-8.
• Choi JH, Kim JH, Park YC, Kim WY, Lee YS. Anesthetic experience using total intra-venous anesthesia for a patient with Wolf-Hirschhorn syndrome -A case report-. Korean J Anesthesiol. 2011;60(2):119-23.
• Gamble JF, Kurian DJ, Udani AG, Greene NH. Airway Management in a Patient with Wolf-Hirschhorn Syndrome. Case Rep Pediatr. 2016;2016:7070125.
• Muldoon S, Sambuughin N, Voelkel M, Bunger R, Grocott HP, Sulzer C. Malignant hyperthermia, thermoregulation, and perioperative hypothermia. In: Anesthesiology. Edited by Longnecker DE, Brown DL, Newman MF, Zapol WM: The United States, The McGraw-Hill companies. 2008, pp 1964-98.
• Shannon NL, Maltby EL, Rigby AS, Quarrell OW. An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality. J Med Genet. 2001;38(10):674-9.
• 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(4):126-9.
• Gerald AG, Isaac NP, Heila MM, Timothy JT. Malignant Hyperthermia. In: Miller’s Anaesthesia. 6th ed. Edited by Miller RD: Philadelphia, Elsevier Churchill Livingstone. 2005;1169-90.
• Azar I. The response of patients with neuromuscular disorders to muscle relaxants: a review. Anesthesiology. 1984;61(2):173-87.
• Bala M, Rashmi, Kaur R, Ajithkumar, Jangra V.Anaesthetic Considerations for General Anaesthesia in an Adult Patient with Wolf-Hirschhorn Syndrome and Kyphoscoliosis: A Case Report. J Clin of Diagn Res. 2023;17(9):4-5.