Clefting in trisomy 9p patients: genotype-phenotype correlation using microarray comparative genomic hybridization

J Craniofac Surg. 2010 Sep;21(5):1376-9. doi: 10.1097/SCS.0b013e3181ef2bbf.

Abstract

Duplication 9p syndrome (partial trisomy 9p) is characterized by craniofacial anomalies, mental retardation, and distal phalangeal hypoplasia. Here, we present a female patient with microcephaly and incomplete bilateral cleft lip and palate, whose initial cytogenetic analysis revealed a de novo trisomy 9p. The patient, now 21 years old, has persistent microcephaly, craniofacial and hand anomalies, history of a seizure disorder, and global mental retardation. Oligonucleotide-based array comparative genomic hybridization was performed and revealed partial trisomy 9p21.1->9pter and a deletion of 9p12.1 to 9p11.2. Our case supports the utility of array comparative genomic hybridization for the precise characterization of chromosomal anomalies and for the ascertainment of genotype-phenotype correlation in patients with partial trisomy 9p.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / surgery
  • Chromosome Aberrations
  • Chromosomes, Human, Pair 9*
  • Cleft Lip / genetics*
  • Cleft Lip / surgery
  • Cleft Palate / genetics*
  • Cleft Palate / surgery
  • Comparative Genomic Hybridization*
  • Female
  • Genotype
  • Humans
  • Microarray Analysis
  • Microcephaly / genetics
  • Phenotype
  • Young Adult