“ROLE OF IMAGING IN WUNDERLICH’S SYNDROME- A RARE CONDITION OF SPONTANEOUS NON TRAUMATIC RENAL HAEMORRHAGE.” DR. P. K. LAMGHARE DR. SHEPHALI. S. PAWAR DR. RAJESH UMAP DR. ANURAG BIJPURIYA

CASE DETAILS: 16 Years old female, came in the casualty hours with complaint of pain in upper abdomen on right side, general fatigue, nausea and vomiting on and off since 3 days. She was pale and was found to have fullness in right hypochondriac region with mild tenderness in the right flank. Her blood pressure and pulse were 100/70 mm Hg and 80/minute and body temperature was 36.5oC. Laboratory investigations were: haemoglobin 7.4 gm/dL, hematocrit 20.3%, white blood cell count 15x103/mm3 and creatinine 1 mg/dl. Urinalysis revealed red blood cell 1-2 while white blood cell 8-12 in the high power field.

Patient was examined by casualty medical officer found fullness and tenderness in right hypochondriac region. Similarly cutaneous lesions such as facial angiofibromas, hypo pigmented macules (Ash leaf macules), Shagreen patches on back, Café au lait macule on dorsum of right foot and Confetti lesions on anterior abdominal wall. On the basis of clinical examination patient was provisionally diagnosed as “ Case of tuberous sclerosis with acute abdomen” and advised radiograph abdomen and USG abdomen for further evaluation.

Radiograph Abdomen USG Abdomen USG Abdomen Radiograph abdomen standing view revealed soft tissue haziness in right hypochondriac region. USG abdomen revealed large hematoma involving upper pole of right kidney. Patient advised CT abdomen for further evaluation.

CT ABDOMEN ARTERIAL PHASE CT ABDOMEN ARTERIAL PHASE Further investigations with CT and MRI revealed large hematoma, due to aneurysmal rupture in a large angiomyolipoma in right kidney.

CT ABDOMEN DELAYED PHASE MRI ABDOMEN COR T1W IMAGE MRI ABDOMEN COR T2W IMAGE Follow up USG abdomen showed increase in size of hematoma & patient was becoming hemodynamically unstable; nephron sparing surgery i.e. partial nephrectomy was done.

Photograph of an excised specimen reveals a large circumscribed mass (black arrows) with variegated cut surface, showing predominance yellowish areas of fat admixed with intra tumour hemorrhage and necrotic foci. The normal renal parenchyma (white arrows) is compressed. Photomicrograph shows admixture of tortuous thick-walled blood vessels (black arrows), sheets of mature adipose tissue (F) and bundles of smooth muscle fibres (M) (haematoxylin and eosin stain; x40).

Follow up radiograph abdomen Follow up radiograph abdomen was within normal limits. Tip of drain tube was noted in right lumbar region. On subsequent imaging of brain with CT and MRI, revealed cortical tubers and sub ependymal nodules. Patient’s general condition was fair at the time of discharge.

DISCUSSION: Wunderlich’s syndrome is a rare condition in which there is spontaneous non traumatic renal haemorrhage into the subcapsular and perirenal spaces. Neoplasms are the most common cause. Among benign neoplasms angiomyolipoma is the commonest, while among malignancies, renal cell carcinoma is the commonest. Other non neoplastic causes are polyarteritis nodosa, renal artery aneurysms, AV malformations and fistulas, renal vein thrombosis, cystic renal diseases, calculus, nephritis and coagulation disorders. It is one of the most feared complications of renal angiomyolipoma and can be fatal if not treated promptly and aggressively. The appropriate treatment for this condition depends on the accuracy of diagnosis and the determination of its cause. Imaging modalities such as ultrasound, CT and MRI are excellent in the detection of the subcapsular and/or perinephric hemorrhage and underlying etiology and play an important role in the management.

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