Prenatal Sonographic Findings of Extensive Low-Flow Mixed Lymphatic and Venous Malformations

Abstract

renatal sonographic diagnoses of subcutaneous soft tissue masses include hemangiomas, malformations (capillary, lymphatic, venous, arterial, or mixed), teratomas, and, rarely, sarcomas. 1-5 Prenatally depicted malformations may be seen in association with more complex fetal conditions, including Klippel-Trenaunay-Weber (angio-osteohypertrophy) and Proteus syndromes. [6][7][8][9] We present an unusual case in which extensive multiple soft tissue tumors encompassing the fetal upper chest, back, nuchal area, and bilateral axillae, considered consistent with multiple large lymphatic malformations noted at 23 weeks' gestation, decreased in size throughout the remainder of gestation. At delivery, relatively small subcutaneous masses remained. Tissue histopathologic findings obtained at biopsy and the neonate's clinical course during which the subcutaneous masses underwent a marked spontaneous decrease in size, were consistent with low-flow mixed lymphatic and venous malformations.A 29-year-old patient, gravida 3, para 2, was followed during her third pregnancy at the State University of New York Downstate Medical Center. Her medical and obstetric histories were unremarkable, and her current pregnancy was uneventful. Results of second-trimester serum screening for aneuploidy was negative.Sonography at 23 weeks' gestation depicted a singleton vertex-presenting female fetus with normal-appearing fetal anatomy other than extensive multiple semicystic, semisolid subcutaneous masses located on the entire upper anterior chest wall, both axillae, back, and nuchal areas (Figures 1-3). Minimal vascularity was depicted on color Doppler imaging. The estimated fetal weight was 700 g (80th percentile for gestational age).

Efficacy of ultrasonography in lymphatic malformations: diagnosis, treatment and follow-up: a case report

“…There may be congenital mal- formations of lymphatic vessels or some factors affecting lymphatic drainage [3]. LM's contain tissue fluid and can be macrocystic, microcystic or mixed in structure [4]. Surgery is the primary form of treatment for LM's.…”

“…Dextrose, doxycycline, bleomycin and OK-432 (picibanil) can be used for injection sclerotherapy. The most commonly accepted sclerosing agents are bleomycin and OK-432 [4]. OK-432 is a lyophilized biological product containing a mixture of the low virulence Su strain of group A Streptococcus pyogenes inactivated with Penicillin-G [8,9].…”

Imaging evaluation of fetal vascular anomalies

“…The expected internal sluggish flow usually translates to no detectable flow with color or pulsed Doppler US [7]. A prenatal report of venous-lymphatic malformation showed this low-flow pattern without detectable vascularity using color Doppler US [25]. We are not aware of previous reports on prenatal pure venous malformations with which to compare the characteristics of our case.…”

Extensive Fetal Congenital Subcutaneous Mixed Venous Lymphatic Lesion: Prenatal Diagnosis and Postnatal Management

“…1 2 The incidence is estimated to be approximately 1.2 to 1.5% and one-fourth of these lesions is completely or partially lymphatic in origin. 3 Venolymphatic malformations are more commonly located in the neck, 4 5 followed by the axilla, mediastinum, and extremities. In one series, lymphatic malformations were the most commonly diagnosed vascular anomaly and occurred more commonly in the cervicofacial region, axilla, mediastinum, and extremities (in that order).…”