S.V. Berestenko, V.I. Alamaha, S.A. Konareva, I.V. Zhurilo-Nikonenko, V.V. Plechun, E.L. Vazhenina, N.V. Berestenko, A.V. Panchenko, Y.Y. Cepelev

Neurofibromatosis. Spontaneous rupture of the right kidney of the 24 year old girl (case from practice)

We present the clinical case of the treatment of patients 24 years old with neurofibromatosis complicated with spontaneous rupture of the right kidney.

The history: the age of three patient operated on for a brain astrocytoma, in the age of 17 - the operation - commissurotomy of the aortic valve in the age of 18 - Ross procedure (replacement of a damaged aortic valve own pulmonary valve). In July 2012 the Regional Hospital. Mechnikova diagnosed - phakomatoses, angiomyolipomas both kidneys, liver cyst. Epilepsy since childhood.

Against the background of complete well-being of the patient 01.04.2013 there abdominal pain, weakness, dizziness, dark eyes. On admission to the hospital in a serious condition, pale skin, dry skin. Respiration rate - 18 per minute, heart rate - 120 per minute, the blood pressure - 60/40 mm Hg. Art. Clinic of internal bleeding - severe anemia (hemoglobin 81 g/L), white blood cells in the blood 40,6h109 / l urine diastase - 128 IU, creatinine of blood - 120 mg/dL. The patient was urgently operated on - discovered during laparotomy retroperitoneal hematoma with rupture of the right kidney that was removed.

Macropreparations: kidney size 14,5x9x4,5 cm, the surface is light brown, with small yellow formations with a diameter of 0.3 to 2.0 cm, with a large tumor formation 8,0x5,5x5,5 see, with the collapse, the cut fabric bud light brown with yellow rounded tumor. Tumor formation on the cut yellow-brown with patches of dark red color.

Slides: in pieces of a tumor from a kidney built of cell-fiber complexes located randomly, forming eddies and somewhere mushy structure polymorphism of cells, including a large number of tumor vessels with thick hyaline walls. Atrophic renal parenchyma. Conclusion: Angionevrinoma - retroperitoneal tumor.