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'Z-pattern' craniosynostosis: a novel presentation of trisutural fusion.
J Craniofac Surg. 2007 May; 18(3):568-74.JC

Abstract

The majority of infants with multiple sutural craniosynostosis have identifiable syndromes, which affect both coronal sutures (Apert, Crouzon, Pfeiffer, and so on), and multiple sutural fusions not involving both these sutures are exceeding rare. We report a highly unusual pattern of trisutural fusion, which to our knowledge has not been previously described. A retrospective clinical review was performed of all cases of craniosynostosis, which had presented to the Craniofacial Center in Dallas, over a 15-year period. Two patients were identified with a unique pattern of craniosynostosis involving the left coronal, sagittal, and right lambdoid sutures creating a "Z-pattern." Both patients were treated with staged cranial vault reconstructive procedures with an initial posterior remodeling (3 to 4 months) followed by an anterior repair (7 to 8 months). Both patients developed cerebellar tonsillar herniation, and one symptomatic patient required surgical decompression. With a follow up of greater than 2 years, normocephaly has been maintained with growth, and both children appear to have normal intellectual development. A unique pattern of trisutural "Z-patterned" craniosynostosis is presented. This distinctive pattern of craniosynostosis appears to be associated with cerebellar tonsillar herniation. In this small series, a two-staged procedure successfully normalized the calvarial dysmorphism, and early growth and development appear normal.

Authors+Show Affiliations

Craniofacial Center, Medical City Dallas Hospital, Dallas, Texas, USA. reschmelzer@msn.comNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

17538319

Citation

Schmelzer, Rodney E., and Jeffrey A. Fearon. "'Z-pattern' Craniosynostosis: a Novel Presentation of Trisutural Fusion." The Journal of Craniofacial Surgery, vol. 18, no. 3, 2007, pp. 568-74.
Schmelzer RE, Fearon JA. 'Z-pattern' craniosynostosis: a novel presentation of trisutural fusion. J Craniofac Surg. 2007;18(3):568-74.
Schmelzer, R. E., & Fearon, J. A. (2007). 'Z-pattern' craniosynostosis: a novel presentation of trisutural fusion. The Journal of Craniofacial Surgery, 18(3), 568-74.
Schmelzer RE, Fearon JA. 'Z-pattern' Craniosynostosis: a Novel Presentation of Trisutural Fusion. J Craniofac Surg. 2007;18(3):568-74. PubMed PMID: 17538319.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 'Z-pattern' craniosynostosis: a novel presentation of trisutural fusion. AU - Schmelzer,Rodney E, AU - Fearon,Jeffrey A, PY - 2007/6/1/pubmed PY - 2007/8/10/medline PY - 2007/6/1/entrez SP - 568 EP - 74 JF - The Journal of craniofacial surgery JO - J Craniofac Surg VL - 18 IS - 3 N2 - The majority of infants with multiple sutural craniosynostosis have identifiable syndromes, which affect both coronal sutures (Apert, Crouzon, Pfeiffer, and so on), and multiple sutural fusions not involving both these sutures are exceeding rare. We report a highly unusual pattern of trisutural fusion, which to our knowledge has not been previously described. A retrospective clinical review was performed of all cases of craniosynostosis, which had presented to the Craniofacial Center in Dallas, over a 15-year period. Two patients were identified with a unique pattern of craniosynostosis involving the left coronal, sagittal, and right lambdoid sutures creating a "Z-pattern." Both patients were treated with staged cranial vault reconstructive procedures with an initial posterior remodeling (3 to 4 months) followed by an anterior repair (7 to 8 months). Both patients developed cerebellar tonsillar herniation, and one symptomatic patient required surgical decompression. With a follow up of greater than 2 years, normocephaly has been maintained with growth, and both children appear to have normal intellectual development. A unique pattern of trisutural "Z-patterned" craniosynostosis is presented. This distinctive pattern of craniosynostosis appears to be associated with cerebellar tonsillar herniation. In this small series, a two-staged procedure successfully normalized the calvarial dysmorphism, and early growth and development appear normal. SN - 1049-2275 UR - https://www.unboundmedicine.com/medline/citation/17538319/'Z_pattern'_craniosynostosis:_a_novel_presentation_of_trisutural_fusion_ L2 - https://doi.org/10.1097/scs.0b013e318052feee DB - PRIME DP - Unbound Medicine ER -