Article
A massive sternum-to-navel varix occurring in pregnancy and ventriculoatrial shunting in childhood: Is there a connection?
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Authors
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Anna-Margarete Bohrer
- Neurochirurgische Klinik
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Hilmar Kühl
- Institut für diagnostische und interventionale Radiologie and Neuroradiologie, Universitätsklinikum Essen
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Ulrich Sure
- Neurochirurgische Klinik
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Ilonka Kreitschmann-Andermahr
- Neurochirurgische Klinik
| Published: | June 2, 2015 |
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Outline
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Objective: To clarify the pathophysiology of a massive varix reaching out from sternum to navel in a patient who underwent implantation of a ventriculoatrial (VA) shunt in childhood.
Method: A 49-year-old female patient presented to our outpatient clinic with a massive varix reaching from sternum to navel, which increased in size during contraction of the abdominal muscles. In 1971, at the age of seven, a VA shunt had been implanted due to idiopathic hydrocephalus. Seventeen years later, during her first pregnancy the patient developed the massive varix described above which remained present after delivery. Since then, swelling of the varix and hardening of the abdomen occurred during physical strain. Because of progressive discomfort due to the varix, the patient presented to our outpatient department. Coagulopathy had already been ruled out in the past.
Results: Thoracic computed tomography unfolded the strong suspicion of a filiform high-grade stenosis of the superior caval vein (SCV) with a short segment thrombosis of the SCV next to the ending of the shunt, which was positioned directly cranially of the azygos vein confluence. Subsequent MRI of the thoraco-abdominal veins and its collaterals with TWIST and dynamic contrast-enhanced confirmed this diagnosis. Additionally, there was a massive venous collateral circulation along veins of the ventral and dorsal thoracic wall through the internal thoracic veins on both sides as well as along intercostal, paravertebral and mediastinal veins, draining the blood of the upper half of the body through a dilated azygos vein into the lower SCV.
Conclusions: The distal shunt catheter in the SCV has to be postulated as the cause of the high-grade stenosis leading to thrombosis and, consequently, to the massive collateral circulation. Likely due to pregnancy-related rise of intraabdominal pressure, the impressive varix developed. Because the situation was, at time of investigation, stable for almost 25 years and a further pregnancy was not planned, the consulted thoracic surgeons saw no need for action at present. However, they cautioned that if the patient would have to undergo abdominal surgery in the future, that it would be of utmost importance to spare the collateral venous circulation with care to avoid massive bleedings or infarctions due to venous congestion. This case serves as an impressive reminder of the risk of severe, albeit infrequent thrombotic complications of VA shunts.
