Pulmonary glial heterotopia in a monoamniotic twin

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

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

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
Volume36
Issue number2
DOIs
StatePublished - Aug 1 2003
Externally publishedYes

Fingerprint

Neuroglia
Neural Crest
Lung
Cysts
Newborn Infant
Amnion
Teratoma
Brain
Chorionic Gonadotropin
Rare Diseases
Premature Infants
Autopsy
Coloring Agents
Thorax
Epithelium
Immunohistochemistry
Parturition

Keywords

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

ASJC Scopus subject areas

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

Cite this

Morgan, T., Anderson, J., Jorden, M., Keller, K., Robinson, T., & Hintz, S. (2003). Pulmonary glial heterotopia in a monoamniotic twin. Pediatric Pulmonology, 36(2), 162-166. https://doi.org/10.1002/ppul.10292

Pulmonary glial heterotopia in a monoamniotic twin. / Morgan, Terry; Anderson, Jodee; Jorden, Michelle; Keller, Kathy; Robinson, Terry; Hintz, Susan.

In: Pediatric Pulmonology, Vol. 36, No. 2, 01.08.2003, p. 162-166.

Research output: Contribution to journalArticle

Morgan, T, Anderson, J, Jorden, M, Keller, K, Robinson, T & Hintz, S 2003, 'Pulmonary glial heterotopia in a monoamniotic twin', Pediatric Pulmonology, vol. 36, no. 2, pp. 162-166. https://doi.org/10.1002/ppul.10292
Morgan T, Anderson J, Jorden M, Keller K, Robinson T, Hintz S. Pulmonary glial heterotopia in a monoamniotic twin. Pediatric Pulmonology. 2003 Aug 1;36(2):162-166. https://doi.org/10.1002/ppul.10292
Morgan, Terry ; Anderson, Jodee ; Jorden, Michelle ; Keller, Kathy ; Robinson, Terry ; Hintz, Susan. / Pulmonary glial heterotopia in a monoamniotic twin. In: Pediatric Pulmonology. 2003 ; Vol. 36, No. 2. pp. 162-166.
@article{8827d1d6e211463fa70cb58f9c03fe32,
title = "Pulmonary glial heterotopia in a monoamniotic twin",
abstract = "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.",
keywords = "Amnion, Anencephaly, CCAM, Cystic lung disease, Glia, Heterotopia, Neural crest",
author = "Terry Morgan and Jodee Anderson and Michelle Jorden and Kathy Keller and Terry Robinson and Susan Hintz",
year = "2003",
month = "8",
day = "1",
doi = "10.1002/ppul.10292",
language = "English (US)",
volume = "36",
pages = "162--166",
journal = "Pediatric Pulmonology",
issn = "8755-6863",
publisher = "Wiley-Liss Inc.",
number = "2",

}

TY - JOUR

T1 - Pulmonary glial heterotopia in a monoamniotic twin

AU - Morgan, Terry

AU - Anderson, Jodee

AU - Jorden, Michelle

AU - Keller, Kathy

AU - Robinson, Terry

AU - Hintz, Susan

PY - 2003/8/1

Y1 - 2003/8/1

N2 - 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.

AB - 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.

KW - Amnion

KW - Anencephaly

KW - CCAM

KW - Cystic lung disease

KW - Glia

KW - Heterotopia

KW - Neural crest

UR - http://www.scopus.com/inward/record.url?scp=0041695199&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0041695199&partnerID=8YFLogxK

U2 - 10.1002/ppul.10292

DO - 10.1002/ppul.10292

M3 - Article

C2 - 12833496

AN - SCOPUS:0041695199

VL - 36

SP - 162

EP - 166

JO - Pediatric Pulmonology

JF - Pediatric Pulmonology

SN - 8755-6863

IS - 2

ER -