ÖZETJüvenil hiyalin fibromatozis (JHF) yaşamın erken dönemlerinde tüm vücutta yavaş büyüyen nodüller ile karakterize otozomal resesif geçişli kalıtsal bir hastalıktır. Yumuşak dokularda anormal doku artışı, eklem hareket kısıtlılığı ve eşlik eden diğer sistem patolojileri nedeniyle hava yolu ile ilgili sorunlar yaşanabilecek bu hastalarda, operasyonda önemli kan kaybı olabilmektedir. Operasyon sonrası olası solunum yetersizliği nedeniyle yoğun bakım gereksinimi olabileceği düşünülerek gerekli hazırlıklar yapılmalıdır. Bu olgu sunumunda 7 yaşında JHF'li hastada anestezi yaklaşımlarının tartı-şılması amaçlandı.Anahtar kelimeler: Jüvenil hiyalin fibromatozis, anestezi, hava yolu kontrolü SUMMARY Anesthetic Management in Juvenile Hyaline Fibromatosis: Case ReportJuvenile hyaline fibromatosis (JHF) is an autosomal recessive congenital disorder characterized by slow-growing nodules on the body in the early stages of life. Abnormal tissue growth in soft tissues, limited joint mobility, and other system abnormalities may lead to airway problems in these patients and also massive blood loss during operation might occur. Preparations should be made in the intensive care unit due to potential respiratory failure after the operation. In this case report, we aimed to discuss the anesthetic management modalities in a 7 year-old JHF patient.
Objective The aim of this study was to compare the anaesthesia parameters, procedure duration, and the effect on hemodynamics and clinical parameters during the procedure in patients undergoing transesophageal echocardiography (TEE) with conscious sedation applied with midazolam or propofol. Methods This cross-sectional study included 401 patients (198 males, 203 females, mean age 52.9 ± 14.8 years) applied with TEE in our clinic. The demographic, clinical and laboratory parameters of the patients were recorded before the procedure. A record was made of pre-procedure ASA score and basal SaO2, the time to sedation to TEE, TEE duration, time to recovery, and during the procedure the minimum SaO2, the need for non-invasive mechanical ventilation (NIMV), the change in systolic and diastolic blood pressure (SBP and DBP), O2 saturation change, and pulse change. The data were compared between the patients in two groups according to the conscious sedation agent used; midazolam and propofol. Results The demographic, clinical, and laboratory data of the midazolam and propofol groups were found to be similar (p > 0.05 for each). The procedure duration, and time to recovery were determined to be significantly shorter in the midazolam group than in the propofol group, and the time to onset of the sedation effect was significantly longer (p < 0.05 for each). Of the respiratory parameters, the minimum SaO2 during the procedure, the absolute change in O2 saturation, and the need for NIMV were determined to be significantly lower in the midazolam group (p < 0.05 for each). The absolute pulse and change in SBP and DBP values were found to be significantly higher in the propofol group (p < 0.05 for each). Conclusions The study results demonstrated that conscious sedation applied with midazolam during TEE can be applied with a shorter procedure duration and better hemodynamic and clinical results compared to sedation with propofol.
Moyamoya disease is characterized by steno-occlusive changes of the intracranial internal carotid arteries. Cerebral blood flow and metabolism are strictly impaired. The goal in perioperative anaesthetic management is to preserve the stability between oxygen supply and demand in the brain. Peripheral nerve blockade allows excellent neurological status monitoring and maintains haemodynamic stability which is very important in this patient group. Herein, we present an axillary brachial plexus blockade in a moyamoya patient operated for radius fracture.
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