Perioperative management in Rothmund–Thomson syndrome: A case report

Autor: Ahmed Uslu, Nedim Çekmen
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Bali Journal of Anesthesiology, Vol 7, Iss 1, Pp 43-46 (2023)
Druh dokumentu: article
ISSN: 2549-2276
DOI: 10.4103/bjoa.bjoa_244_22
Popis: Rothmund-Thomson Syndrome (RTS) is a rare, multisystem disease accompanied by many anomalies that require careful attention from preoperative evaluation to discharge regarding anesthesia preparation and management. Due to craniofacial deformities accompanying facial abnormalities, maintaining the airway becomes a complete struggle with a race against time for survival. This case report presents the preoperative preparation and perioperative management, the risks that may be encountered, and the detailed preparation for a 7-year-old patient diagnosed with RTS and multiple system involvement. General anesthesia was administered to this patient, who had intrauterine growth retardation, nail streaking, redness of the bullae and legs, and a history of somatotropin treatment and antinuclear antibody (ANA) positivity under optimum conditions. Thanks to successful and detailed preoperative preparation and perioperative management, the patient was followed up and discharged without any complications. Although RTS is rare, the need for general anesthesia often arises due to juvenile cataracts, dental anomalies, syndactyly and other extremity anomalies in these patients whose airway management is complex. Furthermore, this increases the current risk in the population of these patients. Managing these patients with a multidisciplinary approach will reduce complications, morbidity, mortality, and length of stay.
Databáze: Directory of Open Access Journals
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