Spontan Anjiomyolipom Rüptürüne Bağlı Akut

e-ISSN:2148-1547
Spontan Anjiomyolipom Rüptürüne Bağlı Akut Retroperitoneal Hematom:
Olgu Sunumuİ
Spontaneus Rupture of Renal Angiomyolipoma As a Cause of Acute
Retroperitoneal Hemorrhage: Case Report
Olgu Sunumu
Başvuru: 27.12.2014
Kabul: 01.02.2015
Yayın: 20.03.2015
Fulya Yılmaz Duran1, Özgür Duran2, Umut Öztürk2, Gizem Demir1, Zafer Kozacıoğlu3, İsmail Gülden 3,
Yasin Ceylan3
1
Bozyaka Training and Research Hospital, Anaesthesiology and Reanimation Department
2
Bozyaka Training and Research Hospital, Emergency Medicine Department
3
Bozyaka Training and Research Hospital, Urology Department
Özet
Abstract
Anjiomyolipom, yağ dokusu, düz kas hücre demetleri ve
kalın duvarlı kan damarlarının karışımından oluşan,
nadir rastlanan mezenkimnal bir tümördür. 35 yaşındaki
bayan hasta nedeniyle sol yan ağrısı, bulantı ve kusma
acil servisine başvurdu. Fizik muayenede yüzünde
adenom sebaseum gibi deri lezyonları saptandı.
Bilgisayarlı tomografide sağ alt kutupta yağ densitesinde
alanlar (35mm) ve ekzofitik kitle lezyonu saptandı. acil
laparatomi uygulandı. Renal anjiomiyolipomdrüptürüne
bağlı retroperitoneal kanama gözlendi. Bir gün sonra
yoğun bakım ünitesinden ve bir hafta sonra hastaneden
taburcu edildi. Patoloji raporu anjiomyolipom rüptürü ile
uyumlu saptandı.
Angiomyolipoma is a rare mesenchymal tumor
composed of the mixture of mature fat tissue, smooth
muscle cell bundles and thick-walled blood vessels. A
35-year-old woman was admitted to emergency
service because of left flank pain, nause and vomiting.
Physical examination revealed skin lesions on her face
like adenoma sebaceum. Computed tomography of the
abdomen revelaed areas with fat density at the lower
pole of the right kidney (35mm) and exophytic renal
mass lesions. She underwent emergency laparatomy.
Retroperitoneal
hemorrhage
due
to
renal
angiomyolipoma rupture was observed. The patient
discharged from ICU unit one day later and from
hospital one week later. Pathology report declared
angiomyolipoma.
Anahtar kelimeler: Anjiomyolipom, Spontan rüptür,
Retroperitoneal hemoraji
Keywords: Angiomyolipoma, Spontaneous rupture,
Retroperitoneal hemorrhage
Introduction
Angiomyolipoma is a rare mesenchymal tumor composed of the mixture of mature fat tissue, smooth muscle cell
bundles and thick-walled blood vessels. Although often benign, some types are considered as malign. [1-6] . Two
types were described: sporadic or associated with tuberosclerosis [4,5]. 40% of patients are symptomatic [5].
Clinically symptoms are palpable abdominal mass, hematuria, flank pain [1,3,5-7]. We report a case who underwent
emergency laparotomy due to massive retroperitoneal hemorrhage because of anjiomyolipoma rupture.
Case Report
A 35-year-old woman was admitted to emergency service because of left flank pain, nause and vomiting. She was
transferred to our emergency department from emergency room of other hospital for acute left flank pain
interpreted as a renal colic and not responsive to medical therapy. She has no feature in her and family history. On
clinical examination she was hemodynamically instable with rapid decrease in hemoglobin (Hb: 6.7mg/dL), with
Sorumlu Yazar: Fulya Yılmaz Duran, Bozyaka Training and Research Hospital, Anaesthesiology and Reanimation Department
Saim Çıkrıkçı Caddesi No. 51
[email protected]
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a persistant left flank pain and hypochondrium pain as in acute abdomen. Blood pressure:80/40 mmHg, heart
rate:135/min. Physical examination revealed skin lesions on her face like adenoma sebaceum. Abdominal
ultrasonografi showed hyperechoic lesions around the left kidney (the largest is 12cm diameter), suspected
hematoma around the left kidney, hyperechoic lesions with the same characteristics like on the left kidney on the
lower pole of the right kidney (the largest is 2cm diameter), abdominal free fluid. Subsequent computed
tomography of the abdomen revelaed areas with fat density at the lower pole of the right kidney (35mm) and
exophytic renal mass lesions. The appearance which suggested bleeding was detected in the left kidney, which
was more pronounced in the lateral segment and enlargement of the left psoas muscle was revealed. Free fluid
viewed in the pelvic area and abdomen. The patient was consulted by general surgery and urology doctors. She
underwent emergency laparatomy. Retroperitoneal hemorrhage due to renal angiomyolipoma rupture was
observed. Left radical nephrectomy was done. The patient was stabile in perioperative course. 4U of packed red
cells transfused during the operation. She was transported to intensive care unit(ICU) after the operation. The
postoperative period was uneventful. The patient discharged from ICU unit one day later and from hospital one
week later. Pathology report declared angiomyolipoma. Tuberousclerosis was not detected in the genetic analysis.
This case report was reported after the patient approvel was taken when she was discharging from the hospital.
Discussion
Spontaneous angiomyolipoma rupture was occured in our case. The patient was underwent emergency
nephrectomy with early diagnosis. Lesions at right kidney were followed up with computure tomography.
The term angiomyolipoma was first used by Morgan et al at 1951 to define the kidney tumors composed of
aberrant vascularization with varying amounts of smooth muscle and fatty elements [5] 1-2% in the general
population [7] , often frequently seen in middle-aged women [6,7]; 50-75% seen in patients with tuberous sclerosis
and at a younger age, without distinction of sex arises [7].
Anjiomyolipoms that <4 cm are often asymptomatic [1,2,6]. Sometimes classical Lenks triad occurs:mass
proportional to the diameter of lesion, flank pain and hematuria [1,3,5-7]. Massive retroperitoneal haemorrhage due
to AML (Wunderlich syndrome) was 10% in cases and the most feared complication [1,7].
In our case, at the first examination lumbar pain and non-specific symptoms such as nausea and vomiting were
present. hypotension and tachycardia were present. Hemogram revealed a decrease in hemoglobin levels during
follow-up. As a result of the tests performed emergency laparotomy was decided.
In most cases, angiomyolipoma is diagnosed with typical radiological findings [1]. CT is the most reliable
diagnostic tool [1,3]. In our case, USG detected suspicious lesions and the possibility of bleeding and the diagnosis
was confirmed by CT.
At angiomyolipoma, the walls of arteries are thicker than normal renal vessels but has less elasticity [1]. They are
willing to bleeding without trauma or minor trauma. The risk of bleeding is increasing with 4 cm or greater
diameter [1,5-8]. Spontaneous rupture is a serious medical condition.[1] In our case, there is no history of trauma.
Spontaneous rupture was considered. Sudden onset of flank pain, nausea, and hypotension was revealed.
The treatment in asymptomatic angiomyolipoma is conservative treatment and intermittant follow up
examination [1,7,8]. In patients with symptoms, the aim is to protect the nephrons and renal function [1,7]. Firstly,
selective arterial embolization, open or laparoscopic partial nephrectomy, open or laparoscopic cryoablation or
radiofrequency ablation, and total nephrectomy should be performed if necessary [2,3,7].
In our case, the result of spontaneous rupture disrupts hemodynamic parameters. We did not have time to
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implement selective uterine artery embolization. Primarily to protect the nephrons partial nephrectomy was
planned but has been forced to total nephrectomy. Active pathology in the right kidney was not observed.
In the literature, spontaneous rupture of renal anjiomyolipom at 31 year old woman during pregnancy have been
reported. It is a dangerous condition that may caause mortality in the mothr and fetus. Elective simultaneous
cesarean section and radical nephrectomy cen be performed safetly if hemodynamic status is stable [9].
Consequently, AML often accompany tuberousclerosis, but also available spontaneously. The most important
complication is massive retroperitoneal hemorrhage. While choosing treatment methods, renal functions should
be preserved maximally. In the emergency room with a diagnosis of renal colic patients are advised to check with
the ultrasonography and computure tomography.
References
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