Scopus
🔓 Açık Erişim YÖKSİS Eşleşti
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
World Journal of Cardiology · Ocak 2022
YÖKSİS Kayıtları
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
World Journal of Cardiology · 2022 ESCİ
PROFESÖR MEHMET ALKILIÇ HORASANI ÖÇ →
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
World Journal of Cardiology · 2022 ESCI
PROFESÖR AHMET SERT →
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
WORLD JOURNAL OF CARDIOLOGY · 2022 ESCI
PROFESÖR OĞUZHAN ARUN →
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
World Journal of Cardiology · 2022 ESCI
PROFESÖR BAHAR ÖÇ →
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
World Journal of Cardiology · 2022 ESCI
PROFESÖR RESUL YILMAZ →
Makale Bilgileri
DergiWorld Journal of Cardiology
Yayın TarihiOcak 2022
Cilt / Sayfa14 · 54-63
Scopus ID2-s2.0-85124878622
Erişim🔓 Açık Erişim
Özet
BACKGROUND Cornelia de Lange syndrome (CdLS) is a congenital multisystemic genetic disorder. The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years. However, they still more frequently undergo cardiac surgery. There are some challenges for clinicians when faced with CdLS patients. We present the perioperative management of a child with CdLS undergoing open-heart surgery. CASE SUMMARY Severe pulmonic and subpulmonic valvular stenosis, enlargement of the right side of the heart, mild tricuspid regurgitation, atrial septal defect, and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis, developmental delay, and malnutrition. Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve, therefore it was decided to perform an open surgical repair. Following a successful and uncomplicated intraoperative course, the patient was extubated on postoperative day 5, and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10, respectively. Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed, and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32. The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems. CONCLUSION This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.
Yazarlar (6)
1
Oguzhan Arun
ORCID: 0000-0001-6101-1481
2
Bahar Oc
ORCID: 0000-0002-2253-5191
3
Esma Nur Metin
ORCID: 0000-0002-0634-2840
4
Ahmet Sert
ORCID: 0000-0002-1607-7569
5
Resul Yilmaz
6
Mehmet Oc
ORCID: 0000-0002-7832-3067
Anahtar Kelimeler
Anesthesia
Brachmann de Lange Syndrome
Cardiac surgery
Case report
Cornelia de Lange Syndrome
Pulmonary valve stenosis
Valvular heart disease
Kurumlar
Selçuk Tip Fakültesi
Konya Turkey