.: OLGU SUNUMU
Yoğun Bakımda Nadir Bir Olgu: Tüberoskleroz Kompleksinin Neden Olduğu Anjiyomiyolipomdan Kaynaklanan Masif Kanama
A Rare Case in Intensive Care Unit: Massive Bleeding Due to Angiomyolipoma Caused by Tuberous Sclerosis Complex
Turkiye Klinikleri J Anest Reanim. 2021;19(3):151-4
doi: 10.5336/anesthe.2021-85048
Makale Dili: TR
Tam Metin
ÖZET
Tüberoskleroz kompleksi (TSK); mental retardasyon, epilepsi, böbrek, kemik, akciğer ve retinada görülen hamartomlarla karakterize multisistemik otozomal dominant geçişli bir hastalıktır. Böbrek lezyonları; anjiyomiyolipom (AML), basit kist, polikistik böbrek ve renal hücreli karsinom şeklinde görülebilmektedir. Renal AML, asemptomatik olabildiği gibi %10-25 olguda akut masif kanama ile şok gibi ölümcül komplikasyonlar görülebilir. Anormal damarlanmalar ve anevrizmal yapılar içermeleri nedeniyle 4 cm ve üzeri tümörler spontan kanama riski taşımakla birlikte en sık mortalite nedeni, masif spontan kanamaya bağlı hipotansiyon ve şok tablosudur. Bu olgu sunumunda, TSK hastalığı tanısı olup; Wunderlich sendromu birlikteliği nedeniyle renal AML'ye sekonder spontan masif internal kanama, hipotansiyon ve şok tablosu gelişen hastayı sunmak istedik. AML, TSK ile birlikte görülebilir ve şok gibi hayatı tehdit eden komplikasyonlara neden olabilir. Erken tanı ile mortalite ve morbidite azaltılabilir.
Anahtar Kelimeler: Tüberoskleroz kompleksi; anjiyomiyolipom; şok
Tüberoskleroz kompleksi (TSK); mental retardasyon, epilepsi, böbrek, kemik, akciğer ve retinada görülen hamartomlarla karakterize multisistemik otozomal dominant geçişli bir hastalıktır. Böbrek lezyonları; anjiyomiyolipom (AML), basit kist, polikistik böbrek ve renal hücreli karsinom şeklinde görülebilmektedir. Renal AML, asemptomatik olabildiği gibi %10-25 olguda akut masif kanama ile şok gibi ölümcül komplikasyonlar görülebilir. Anormal damarlanmalar ve anevrizmal yapılar içermeleri nedeniyle 4 cm ve üzeri tümörler spontan kanama riski taşımakla birlikte en sık mortalite nedeni, masif spontan kanamaya bağlı hipotansiyon ve şok tablosudur. Bu olgu sunumunda, TSK hastalığı tanısı olup; Wunderlich sendromu birlikteliği nedeniyle renal AML'ye sekonder spontan masif internal kanama, hipotansiyon ve şok tablosu gelişen hastayı sunmak istedik. AML, TSK ile birlikte görülebilir ve şok gibi hayatı tehdit eden komplikasyonlara neden olabilir. Erken tanı ile mortalite ve morbidite azaltılabilir.
Anahtar Kelimeler: Tüberoskleroz kompleksi; anjiyomiyolipom; şok
ABSTRACT
Tuberous sclerosis complex (TSC); it is a multisystemic autosomal dominant disease characterized by mental retardation, epilepsy, hamartomas in kidney, bone, lung and retina. Kidney lesions can be seen as angiomyolipoma (AML), simple cyst, polycystic kidney and renal cell carcinoma. Although renal AML can be asymptomatic, fatal complications such as acute massive hemorrhage and shock can be seen in 10-25% of cases. Tumors of 4 cm and above carry the risk of spontaneous bleeding because they contain abnormal vascularization and aneurysmal structures, but the most common cause of mortality is hypotension and shock due to massive spontaneous bleeding. In this case report, a patient who was diagnosed with TSC disease and developed spontaneous massive internal hemorrhage, hypotension and shock secondary to renal AML due to the association of Wunderlich syndrome. AML can be seen together with TSC and can cause lifethreatening complications such as shock. Early diagnosis can reduce mortality and morbidity.
Keywords: Tuberous sclerosis complex; angiomyolipoma; shock
Tuberous sclerosis complex (TSC); it is a multisystemic autosomal dominant disease characterized by mental retardation, epilepsy, hamartomas in kidney, bone, lung and retina. Kidney lesions can be seen as angiomyolipoma (AML), simple cyst, polycystic kidney and renal cell carcinoma. Although renal AML can be asymptomatic, fatal complications such as acute massive hemorrhage and shock can be seen in 10-25% of cases. Tumors of 4 cm and above carry the risk of spontaneous bleeding because they contain abnormal vascularization and aneurysmal structures, but the most common cause of mortality is hypotension and shock due to massive spontaneous bleeding. In this case report, a patient who was diagnosed with TSC disease and developed spontaneous massive internal hemorrhage, hypotension and shock secondary to renal AML due to the association of Wunderlich syndrome. AML can be seen together with TSC and can cause lifethreatening complications such as shock. Early diagnosis can reduce mortality and morbidity.
Keywords: Tuberous sclerosis complex; angiomyolipoma; shock
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