Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles

Fetal Pediatr Pathol. 2020 Jun;39(3):194-245. doi: 10.1080/15513815.2019.1641859. Epub 2019 Jul 25.

Abstract

Background: Lumbosacral spinal lipomas and lipomyeloceles are usually identified in early childhood. Terminology, histopathology, and diagnosis for these malformations can be confusing. Materials and Methods: This is a PubMed review with comparison of embryology, gross, and histopathology, and reporting requisites for these and related closed spinal malformations. Results: The spinal lipoma group (congenital spinal lipomatous malformations) includes subcutaneous, transdural, intradural, and noncontiguous malformations stretching through the entire lower spinal region. This lipomyelocele trajectory overlaps the embryonic tail's caudal eminence. Histopathologically, the lipomyelocele spectrum is a heterogeneous, stereotypical set of findings encountered from dermis to spinal cord. Diagnosis requires detailed correlation of images, intraoperative inspection, and histopathology. Conclusions: Appropriate terminology and clinicopathologic correlation to arrive at a diagnosis is a critical activity shared by pathologist and clinician. Prognostic and management differences depend on specific diagnoses. Familial and genetic influences play little if any role in patient management in closed spinal malformations.

Keywords: Spinal lipoma; birth defects; human tail; lipomyelocele; lipomyelomeningocele; medullary spinal cord; myelocystocele; spina bifida.

Publication types

  • Review

MeSH terms

  • Female
  • Humans
  • Lipoma / congenital*
  • Lipoma / diagnosis
  • Lipoma / pathology*
  • Male
  • Meningomyelocele / diagnosis
  • Meningomyelocele / pathology*
  • Spinal Cord / abnormalities*

Supplementary concepts

  • Lipomyelomeningocele