Anaesthetic Management of Case With Lowe Syndrome

Year 2005, Volume: 14 Issue: 3, 205 - 208, 01.12.2005

Berrin Işık Mustafa Arslan Mehmet Akçabay

Abstract

Oculocerebrorenal Syndrome of Lowe (OCRL) or Lowe Syndrome (LS), is a rare X chromosome-linked disorder that is characterized by severe mental retardation, congenital cataracts, and Renal Fanconi Syndrome. The Renal Fanconi Syndrome develops in the neonatal period with impaired renal proximal tubular function including acidosis, aminoaciduria, phosphaturia, and proteinuria. Patients with OCRL may also candidate to general anaesthesia by reason of cataract and glaucoma. However musculo-skeletal abnormalities, dental cysts and metabolic acidosis and hypokalemia are the most important aspects and should be noticed preoperative or intraoperatively. In this case report; By monitoring peroperative blood gases and plasma electrolyt levels and being avoided from applications which can influence acidose and affects of acidose, the case mentioned hereby, who has OCRL Syndrome and has entubation difficulties with the need of sodium bicarbonate that has to be given during operation, didn’t show any complication caused by anesthetics and the post operative period could be maintened

Keywords

Lowe Syndrome, anaesthesia, acidosis

LOWE SENDROMLU OLGUDA ANESTEZİK YAKLAŞIM

Abstract

Okülo-Serebro-Renal Lowe (OCRL) Sendromu ya da Lowe Sendromu (LS); konjenital katarakt, mental retardasyon ve Renal Fanconi Sendromuyla seyreden, X kromozomuna bağlı, resesif geçişli, nadir görülen bir sendromdur. Renal Fanconi Sendromu proksimal tubulus fonksiyon yetersizliği nedeniyle yenidoğan döneminde asidoz, aminoasitüri, fosfatüri ve proteinüriye neden olur. OCRL Sendromu seyrinde izlenen katarakt ve glokom nedeniyle bu olgular genel anestezi uygulanmasına adaydırlar. Ancak kas iskelet sistemine ait anomaliler, dental kistler ve metabolik problemler nedeniyle anestezi uygulaması öncesi veya anestezi sırasında dikkat edilmelidir. Sunulan; OCRL Sendromu tanılı, entübasyon güçlüğü yaşanan ve operasyon sırasında sodyum bikarbonat verilmesi gereken olguda peroperatif kan gazları ve plazma elektrolit düzeyleri izlenerek asidoz ve asidozun etkilerini artıracak uygulamalardan kaçınıldığında anesteziden kaynaklanabilecek komplikasyonların oluşmadığı postoperatif dönemin sağlanabildiği gözlenmiştir

Keywords

Lowe sendromu, anestezi, asidoz

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