BAHATTİN TUNCALI, Pınar ZEYNELOĞLU, Tuğbahan YILMAZ

PRADER WİLLİ SENDROMLU MORBİD OBEZ BİR OLGUDA ANESTEZİ UYGULAMASI

Giriş: Prader Willi Sendromu nadir görülen iki evreli genetik bir bozukluktur. Klinik seyir hipotonik infantil evre ve onu takip eden hiperfaji ve obez evreden oluşur. Morbid obeziteden kaçınıldığında beklenen yaşam süresi ellili yaşlara ulaşır. Olgu Sunumu: Onüç yaşında morbid obez (vücut kitle indeksi 74.4 kg m-2) Prader Willi Sendromlu ciddi restrikti f akciğer hastalığı olan erkek çocukta laparoskopik tüp mide operasyonu uygulandı. İntraoperatif mekanik ventilasyon sorunluydu. Pnömoperitonyumdan hemen sonra oksijenasyon ve ventilasyonda ani bozulma ile birlikte hemodinamik instabilite oluştu. Bu yazıda, olası nedenleri tartışmak ve recruitment manevraları ve poziti f ekspirasyon sonu basıncı ile cerrahi işlemin farklı aşamalarında yapılan ventilatör ayarlamaları dahil olmak üzere anestezi yönetimimizi sunmayı planladık. Hasta başarıyla ekstübe edildi ve yoğun bakım ünitesi gereksinimi olmadı. Sonuç: Hiperfaji ve obez evredeki Prader Willi Sendromu hastaları morbid obezite nedeniyle solunum problemleri ve ardından ölüme eğilimlidir. Bu hastalarda, sorunsuz bir perioperati f dönem için hastalığa ve cerrahi işleme ait özelliklerin dikkate alınması esastır.

ANESTHETIC MANAGEMENT OF A MORBIDLY OBESE PATIENT WITH PRADER WILLI SYNDROME

Introduction: Prader Willi Syndrome is a rare, biphasic genetic disorder. The clinical course includes hypotonic infantile phase followed by hyperphagia and obese phase. Li fe expectancy reach the fi fth decade i f morbid obesity is avoided. Case Report: A 13-year-old morbidly obese (body mass index of 74.4 kg m-2) boy with Prader Willi Syndrome and severe restrictive pulmonary disease underwent a laparoscopic sleeve gastrectomy. Intraoperative mechanical ventilation was a challenge. Abrupt impairment in oxygenation and ventilation occurred with hemodynamic instability immediately after pneumoperitoneum. Herein, we planned to discuss the possible causes, describe our anesthetic management, including alveolar recruitment maneuvers and positive end-expiratory pressure with adjustments in ventilator settings in di fferent parts of the surgical procedure. The patient was succesfully extubated with no need for intensive care unit. Conclusion: Prader Willi Syndrome patients with hyperphagia and obese phase are prone to respiratory problems and subsequent death due to morbid obesity. In these patients, it is essential to consider the unique features of disease and surgical procedure for an unevent ful perioperative period.

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