Lambdoid Craniosynostosis in an Infant With Congenital Cystic Adenomatoid Malformation (CCAM)

Jessica D. Blum, Dillan F. Villavisanis, N. Scott Adzick, Jesse A. Taylor

Research output: Contribution to journalArticlepeer-review

Abstract

Both congenital cystic adenomatoid malformation (CCAM) and lambdoid craniosynostosis are exceedingly rare, with estimated incidences of around 1 in 30,000 live births for each. Herein, we present a case of a patient with prenatally diagnosed CCAM requiring 4 doses of prenatal steroids and ultimately thoracotomy with right lower lobectomy. The operation was uncomplicated, and genome-wide analysis of SNPs revealed no pathologic abnormalities. Development was normal until head shape changes were noted at 5 months of age at which point evaluation by a craniofacial surgeon and 3D CT imaging revealed left-sided lambdoid craniosynostosis. The patient underwent uncomplicated posterior and middle vault expansion and remodeling of his lambdoid synostosis at 11 months of age. This paper explores the etiopathogenesis of CCAM and lambdoid craniosynostosis in an attempt to identify common pathways involved in the development of these exceedingly rare diseases which, to date, have not been described in the peer-reviewed literature.

Original languageEnglish
Pages (from-to)1537-1539
Number of pages3
JournalJournal of Craniofacial Surgery
Volume33
Issue number5
DOIs
StatePublished - 1 Jul 2022
Externally publishedYes

Keywords

  • CCAM
  • CPAM
  • Craniosynostosis
  • TGFB
  • congenital cystic adenomatoid malformation
  • congenital pulmonary airway malformation
  • lambdoid
  • lambdoid craniosynostosis

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