Pulmonary glial heterotopia in a monoamniotic twin

Terry Morgan, Jodee Anderson, Michelle Jorden, Kathy Keller, Terry Robinson, Susan Hintz

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Brain heterotopia in the lungs is rare, but when it occurs in an otherwise healthy newborn, it presents a difficult diagnostic problem and uncertain pathophysiology. We report on a 2-week-old premature infant who presented with respiratory distress and widespread cystic lung changes identified by chest imaging studies. Autopsy demonstrated that the cyst walls were composed of well-differentiated neuroglial tissue, which was confirmed by immunohistochemistry. The cysts were partially lined by bronchial epithelium and contained keratinous debris. For the first time, we demonstrate that the debris stain for human chorionic gonadotropin, compatible with aspirated amnion. There were no other congenital abnormalities. Her monoamniotic twin was anencephalic and died at birth. Although the etiology of glial heterotopia in the lungs is unknown, the majority of cases are associated with anencephalic newborns. Some authors postulated that this heterotopia may therefore be a consequence of fetal aspiration of brain tissue. Other possibilities include glial predominant teratomas, hamartomatous malformations, and abnormal neural crest migration. Our review of the 21 cases reported over the past century suggests that in utero aspiration of glial cells, or abnormal neural crest migration, are the most likely explanations for this rare and fatal disease.

Original languageEnglish (US)
Pages (from-to)162-166
Number of pages5
JournalPediatric pulmonology
Issue number2
StatePublished - Aug 1 2003
Externally publishedYes


  • Amnion
  • Anencephaly
  • CCAM
  • Cystic lung disease
  • Glia
  • Heterotopia
  • Neural crest

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine


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