Rare lymphatic malformation in an extreme premature infant: question

Veronica Mugarab Samedi, Adel Elsharkawy


Our patient was born to a healthy 35-year-old Asian mother.

The pregnancy was uneventful until 25 weeks of gestational age (GA), when she had preterm prolonged rupture of membranes (PPROM). Ultrasound at that point showed a new finding (confirmed by MRI) of 4.7 x 4.1 cm heterogenous cystic skin-covered mass with areas of echogenicity on the posterior aspect of the fetus with blood flow to the mass and minimal vascularity overall.

The male infant was delivered at 26 + 4 weeks by emergency caesarian section for fetal distress with birth weight of 720 grams. A significant finding was a massive swelling over the upper back with a firm central mass. Total size of swelling was 10.2 x 12.2 cm, central cystic mass was smaller (5.0 x 4.2 cm), firm in consistency, slightly mobile, non-pulsatile, non-compressible and non-translucent. Examination of mass did not cause changes in vital signs, thus the mass was considered as a painless malformation.

  1. What is your diagnosis?
  2. Which further investigations would you suggest?
  3. Which subspecialties need to be involved in the management of this case?


lymphatic malformation; cystic lymphangioma; extreme prematurity; congenital mass

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