Placental abruption and spontaneous rupture of renal angiomyolipoma in a pregnant woman with tuberous sclerosis

PLACENTAL

ABRUPTION

SPONTANEOUS

RUPTURE

ANGIOMYOLIPOMA WOMAN

WITH

AND OF RENAL

IN

A PREGNANT

TUBEROUS

Euan V. Forsnes, MD, Maurice and Mark Burtman, MD

SCLEROSIS K. Egyleston, MD,

The renal angiomyolipoma is a benign tumor composed of smooth muscle, blood vessels, and adipose tissue. Renal angiomyolipomas occur in up to 80% of tuberous sclerosis patients.’ In pregnant women with known tuberous sclerosis, spontaneous rupture of renal angiomyolipomas has been reported three times.2,” We present the first reported case of a pregnant woman with tuberous sclerosis who experienced spontaneous rupture of a renal angiomyolipoma in association with placental abruption.

Case A 22-year-old primigravida with tuberous sclerosis presented with flank pain and hematuria at 37 weeks’ gestation. Prevously, she had been diagnosed with tuberous sclerosis based on bilateral renal angiomyolipomas, cortical brain hamartomas, a hypopigmented skin lesion, and a history of seizures. On admission, a urinary catheter returned frank blood. Severe decelerations were noted on fetal heart rate monitoring in association with the acute onset of abdominal pain and palpable uterine tetany. Emergency cesarean produced a male infant with Apgar scores of 6 and 9 at 1 and 5 minutes, respectively. On entry into the uterus, grossly bloody fluid was noted and there were several large blood clots behind the placenta. Intraoperative cystoscopy showed active bleeding from the right ureter. Retroperitoneal exploration revealed an enlarged right kidney with active bleeding. Because of evidence of bleeding from a ruptured renal angiomyolipoma, a right nephrectomy was performed. Histologic examination confirmed a diagnosis of multiccntric angiomyolipoma, with the additional finding of focal acute pyelonephritis. The largest angiomyolipoma was 12 X 6 X 4 cm. The patient experienced no complications and was discharged on the fifth postoperative day with her child.

Comment Tuberous sclerosis is an autosomal disorder of cellular differentiation and proliferation that can affect the brain, skin, kidneys, heart, and other organs.” Renal angiomyolipomas are a common manifestation of tuberous sclerosis. In patients with tuberous sclerosis, renal angiomyolipomas tend to be bilateral, multiple, and of various sizes, whereas those found in patients without tuberous sclerosis tend to be unilateral and solitary. Renal angiomyolipomas are differentiated from other renal neoplasms by intratumor fat on computed tomography.’ Of the previously reported cases of ruptured renal angiomyolipomas in pregnancy, all presented with decreasing hematocrit, with orthostatic hypotension reported in one case. One case reported left flank pain as a presenting symptom, and another reported right lower quadrant pain. Nephrectomy was performed in two of these patients, whereas tumor removal was performed in one patient. No neonatal or maternal deaths were reported. No maternal sequelae were noted, although one patient’ required dialysis for 6 days. Angiomyolipoma rupture is a potentially serious complication in the pregnant woman with tuberous sclerosis. The presenting symptoms of ruptured renal angiomyolipoma include decreasing hematocrit and abdominal pain, making it difficult to differentiate it from placental abruption. Renal evaluation should be performed as part of the preconceptual counseling and perinatal care in patients with tuberous sclerosis. Genetic counseling is mandatory for this autosomal dominant

disorder.

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