Ullrich Sendromlu Bir Hasta ve Akut Batın Cerrahisinde Anestezi Yönetimimiz: Olgu Sunumu / A Patient with Ullrich Syndrome and Anesthetic Management at Acute Abdominal Surgery: Case Report

Başak Altıparmak, Ali İhsan Uysal, Eylem Yaşar, Semra Demirbilek

Özet


Özet

Ullrich sendromu kollajen tip-VI ilişkili bozukluklar içerisinde yer alan bir konjenital musküler distrofidir. Entübasyon çoğu zaman mikrognati, çene eklemlerinde sertlik ve büyük dil sebebiyle güçleşmiştir. Bu vaka takdiminde, nadir görülen bir sendromdaki genel anestezi deneyimimizi sunmayı amaçladık. 9 yaşında kız hasta sağ alt kadran ağrısı sebebiyle acile başvurdu. Hastanın genel durumu iyiydi. Mikrognatisi olan hastanın boyun hareketleri sınırlıydı. Her iki alt ektremitede kontraktürleri olan hastanın kalça çıkığı ve skolyozu da mevcuttu. Hemogramda beyaz küre sayısının yüksek olduğu görüldü, akut apandist ön tanısıyla ameliyata alındı. Propofol+ketamin+fentanil karışımı ile uyutuldu ve roküronyum ile kas gevşekliği sağlandı. Hasta, zor entübasyon ihtimaline karşı videolaringoskop eşliğinde entübe edildi. İdamede propofol ve remifentanil infüzyonu uygulandı. Vaka sonunda hastaya sugammadex verildi. Hasta sorunsuz şekilde uyandı ve ameliyat odasında ekstübe edildi. Ullrich sendromu anestezik açıdan çok ciddi riskler taşımaktadır. Bu tarz hastalara yaklaşımda karşılaşılabilecek tüm komplikasyonlar anestezist tarafından bilinmeli ve preoperatif dönemde önemler alınmalıdır.

Anahtar Kelimeler: Abdominal Cerrahi, Distrofi, Ullrich

Abstract

Ullrich syndrome is a congenital muscular distrophia which is associated with collagen type-VI disorders. Intubation is usually difficult due to micrognathia, rigidity at mandibular joint and big tongue. In this case report, we aim to represent our general anesthetic experience at a rare syndrome. A nine year old female applied to emergency service with a pain in her right lower abdomen. Her general condition was good. She had micrognathia and limitation at her neck movements. The patient had contractures in her lower extremities, hip dislocation and scoliosis. White blood cells were found to be high, she underwent surgery for acute apendicitis. Porofol+ketamin+fentanil combination was used for induction and muscle relaxation was achieved with rokuronium. The patient was intubated with videolaringoscopy in case of difficult intubation. Propofol and remfentayl were used for maintenance. Sugammadex was administered at the end of the surgery. The patient woke up without any problems and was extubated in the operating room. Ullrich syndrome carries very serious risks for anesthesia. All possible complications should be known by the anesthesiologist and all precautionary measures should be held pre-operatively.

Keywords: Abdominal Surgery, Distrophia, Ullrich


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Referanslar


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