Selahattin AYAS, Gülçin BENBİR ŞENEL, Derya KARADENİZ

Arnold Chiari malformasyonu nedeniyle vokal kord paralizisi gelişen ve trakeostomi takılan hastada apnöstik solunuma tedavi yaklaşımı

Arnold Chiari malformasyonu (ACM) serebellar tonsiller başta olmak üzerebeyin sapı yapılarının foramen magnumdan servikal spinal kanal içerisineherniasyonu ile şekillenir. Uyku ile ilişkili solunum bozuklukları bu hasta grubunda genel nüfustan daha yüksek oranda, yaklaşık %60-75 düzeyinde bildirilmektedir. Kırk üç yaşında erkek hastanın, 2009 yılında ekstremitelerdegüçsüzlük, konuşmada bozukluk, öksürme ile tetiklenen senkop şikayetleri ilebaşvurduğu merkezde ACM tip 1.5 tanısı aldığı ve posterior fossa dekompresyonu (PFD) uygulandığı öğrenildi. 2013 yılında sık tekrarlayan aspirasyonpnömonisi ve dispne şikayetleri üzerine bilateral vokal kord paralizisi saptanan hastaya kalıcı trakeostomi uygulandı. 2018 yılından itibaren her gece,gecede 6-7 kez olabilen, 30 saniye süren, gözler açık hareketsiz kalma vemorarma şeklinde apne atakları başlayan hastanın tanı amaçlı yapılan tümgece polisomnografi (PSG) tetkikinde uyku ve uyanıklıkta sürekli, santral vemikst tipte apneler ile şekillenen apnöstik solunumu tespit edildi. İnvazivolmayan mekanik ventilasyon tedavisi amacıyla yapılan PSG’de trakeostomiüzerine yerleştirilen kanül ile farklı modlar denendi (CPAP, BPAP-S/T, ASV)ancak tek başına hiçbirisi etkin olamadı. IVAPS/AVAPS modu denenen hastada, basınç desteği, solunum sayısı ve tidal hacim desteği ile tüm anormalsolunum olayları, düzensiz apnöstik solunum paterni ve oksijen desatürasyonları tüm pozisyonlarda, uyanıklıkta ve tüm uyku evrelerinde etkin birşekilde kontrol altına alındı. ACM’de görülen obstrüktif veya santral tipteapnelerin tedavisinde ilk basamak tedavi olarak PFD önerilmektedir; invazivolmayan mekanik ventilasyon tedavisi ile ilgili bilgiler yetersizdir. Bu olgu kapsamında ACM tanısı alan, PFD uygulanan, ancak bilateral vokal kord paralizi nedeniyle kalıcı trakoestomi uygulanan bir hastadaki uyku ile ilişkili solunum bozukluklarının tanı ve tedavisindeki güçlüklerin sunulması amaçlanmıştır.

Treatment approach to apnestic breathing in a patient with vocal cord paralysis due to Arnold Chiari malformation and tracheostomy

Arnold Chiari malformation (ACM) is herniation of brain stem structures, especially cerebellar tonsils, from the foramen magnum into the cervical spinal canal. Sleep-related respiratory disorders are reported at a higher rate in this patient group than the general population, at about 60-75%. A 43-year-old male patient was diagnosed with ACM type 1.5 at the center where he applied with complaints of weakness in the extremities, speech disorder and syncope triggered by coughing, and a decompression of posterior fossa (PFD) was applied to the patient. Since 2018, the apnea attacks, which were able to be 6-7 times a night every night, last in 30 seconds, with eyes open, inactivity and bruising, have started in the patient, so in all night-time polysomnography (PSG) examination for diagnostic purpose, apnestic breathing including continuous, central and mixed type apneas in sleep and wakefulness was detected. Different modes (CPAP, BPAP-S/T, ASV) were tried with the cannula placed on the tracheostomy in the PSG performed for non-invasive mechanical ventilation therapy, but none alone was effective. In the patient, whom IVAPS/AVAPS mode was tried, all abnormal breathing events, irregular apnetic breathing pattern and oxygen desaturations were effectively controlled in all positions, wakefulness and all sleep stages with pressure support, respiratory rate and tidal volume support. PFD is recommended as the first-line treatment for the treatment of obstructive or central type apneas in ACM; Information on non-invasive mechanical ventilation therapy is insufficient. In this case, we aimed to present the difficulties in the diagnosis and treatment of sleep-related respiratory disorders in a patient whom ACM diagnosed, PFD was applied but underwent permanent trachoestomy due to bilateral vocal cord paralysis.

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