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2008-11-15-10 Answer to the case of the week #234 © Racanska

Answer to the case of the week #234

December 19, 2008 - January 15, 2009

Eva Racanska, MD; Romana Gerychova, MD; Petr Janku, MD.

Department of Obstetrics and Gynecology, University Hospital Brno, Czech Republic.

Case report

A 33-year-old primigravida with negative previous history presented to our department at 25 weeks of her pregnancy. She underwent an amniocentesis at 16 weeks, due to positive biochemical screening, with the result of normal male karyotype (46 XY). A large fetal thoracic hygroma with maximum on the left side was found on ultrasound scan. No other abnormalities were seen. Our suspected diagnoses were congenital lymphangioma or hemangioma.

The fetus was delivered via uncomplicated caesarean section at 39 weeks for fetopelvic disproportion. Our oncologists suggested a diagnosis of Kaposiform hemangioendothelioma, Kasabach-Merritt syndrome and considered surgical resection. The surgery was performed 7 days after delivery with total resection of the tumor on the right and subtotal on the left side of the thorax and the final histological diagnosis was diffuse neonatal hemangiomatosis. The newborn was released from the hospital 35 days after delivery and is followed up by the plastic surgeon and dermatologist. Laser therapy aimed at scar tissue during 8 months after delivery was indicated and so far no other surgical intervention was done.

The right side of the thorax is healed completely and there is a residue of the hemangioma and hypertrophic scar tissue on the left side, planned to be resected after 2 years of age.

Images 1, 2, 3, and 4: Transverse and oblique scans of the fetal thorax and abdomen showing subcutaneous hypoechoic septate lesion representing the diffuse hemangiomatosis. 



Images 5, 6: MRI images showing large subcutaneous para-thoracal lesions of the diffuse hemangiomatosis.


Image 7: X ray image of the thorax and para-thoracal subcutaneous lesion.

Images 8, 9: Postnatal appearance of the newborn.



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