Severe retroperitoneal haemorrhage in the first trimester of a multiple pregnancy after spontaneous rupture of renal angiomyolipoma

Abstract

Tuberous sclerosis (TS) is a neurocutaneous hereditary disease with typical growth of benign tumours called hamartomas in different anatomical locations. Potentially, TS can affect every tissue in the body. Most frequent manifestations are hamartomas in the skin, brain, kidneys, heart, etc. [1, 2]. It is a rare disease with a prevalence in the population 1:10000 and incidence 1:6000, occurring almost identically in all races, ethnic groups and both genders. It can vary from ‘‘only’’ cosmetic skin defects to severe organ damage [3, 4]. The most important diagnostic criteria are cerebral lesions causing epileptic seizures, mental retardation, psychiatric disorders and skin lesions, cardiac rhabdomyomas and renal angiomyolipomas [5, 6]. Final diagnosis is based on DNA analysis. The life-threatening complication associated with haemodynamic changes in the pregnancy is rupture of renal angiomyolipoma [7, 8]. In our case, a 30-year-old patient, primigravida, primary observed for TS, was referred to the hospital in the 9th week of gestation. The patient complained of left lumbosacral pain. Tenderness of the abdomen was present, the colour of the patient’s skin was pale, tapotement was positive. Other clinical signs were hypotension, tachycardia and subfebrility. Ultrasonography of the pelvis described an intact multiple pregnancy—biamniotic bichorionic twins in the 9th week of gestation, the cul de sac and vesicouterine excavation were full of fluid (Fig. 1). Ultrasonography of the kidneys showed left renal mass. Laboratory tests identified severe acute anaemia. Because of haemodynamic instability of the patient, unsuccessful aggressive intravenous volumotherapy and progressive worsening of the blood count, it was not possible to extend the diagnostics to MRI or CT scan. The patient was indicated for an urgent laparotomy. By the revision of the whole peritoneal cavity sanguinolent fluid, intact peritoneum and giant violet retroperitoneal mass (30 cm) in the area of the left kidney was found. By the revision of the retroperitoneal space a giant profusely bleeding tumour surrounded with blood clots was confirmed. The total intraoperative blood loss was more than 5,000 ml, the patient was adequately substituted. Haemostasis was finally achieved by left nephrectomy with ‘‘in toto’’ extirpation of the tumour mass. The histopathological finding of the tumour (21 9 12 9 8 cm) was multiplicite angiomyolipoma (Figs. 2, 3). The subsequent ultrasound examination after the operation confirmed an intact pregnancy. Because of the severity and early onset of the complications, it was not possible to continue in the pregnancy and a therapeutic abortion was indicated. In the retrospective studies of renal tuberous sclerosis and their complications 89.1 % of cases were complicated in average gestational age at 27th week. These situations were usually described in singleton pregnancy [9]. In the report, we present this complication as a very early onset during the multiple pregnancy. Gimeno et al. refer the mode of the surgical management in case of the ruptured angiomyolipomas—total nephrectomy was indicated in 79 % of cases, polar nephrectomy, selective arterial embolisation or conservative management were performed rarely [10]. In reported V. Ferianec M. Gabor (&) P. Papcun K. Holomaň 2nd Department of Obstetrics and Gynaecology, University Hospital Bratislava, Comenius University, Ružinovska 6, Bratislava, Slovak Republic e-mail: martingabormd@gmail.com