Multifocal lymphangioendotheliomatosis with devastating intracranial hemorrhage

J Neurosurg Pediatr. 2013 Nov;12(5):517-20. doi: 10.3171/2013.8.PEDS12604. Epub 2013 Sep 20.


An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

MeSH terms

  • Child
  • Dandy-Walker Syndrome / complications*
  • Female
  • Gastrointestinal Tract / pathology
  • Humans
  • Hydrocephalus / complications
  • Hydrocephalus / pathology
  • Hydrocephalus / surgery
  • Intracranial Hemorrhages / etiology*
  • Lymphangioma / complications*
  • Lymphangioma / pathology
  • Magnetic Resonance Imaging
  • Skin / pathology*
  • Skin Neoplasms / complications
  • Skin Neoplasms / pathology
  • Thrombocytopenia / complications
  • Thrombocytopenia / pathology

Supplementary concepts

  • Dandy Walker variant