Delayed Presentation of Sagittal Suture Craniosynostosis.


Journal

Annals of plastic surgery
ISSN: 1536-3708
Titre abrégé: Ann Plast Surg
Pays: United States
ID NLM: 7805336

Informations de publication

Date de publication:
01 05 2022
Historique:
medline: 25 9 2023
pubmed: 1 5 2022
entrez: 23 9 2023
Statut: ppublish

Résumé

Sagittal craniosynostosis typically presents as dolichocephaly or less frequently as clinocephaly, a "saddle-shaped" phenotype. This project aimed to characterize clinically relevant differences between sagittal synostosis phenotypes and examine the etiology of the delay in presentation. An institutional review board-approved retrospective review was performed from January 1999 to November 2018 at a single institution. Analyses examined correlations between subphenotype, time of presentation, minor suture fusion, developmental delay, and operative technique. One hundred sixty patients diagnosed with single-suture sagittal craniosynostosis were identified. A total of 30.6% had a saddle phenotype (n = 49) and 69.4% had dolichocephaly (n = 111). Patients with the saddle phenotype were more likely to present with a developmental delay and to have at least 1 minor suture fused than patients with dolichocephaly were. Patients with the saddle phenotype presented for surgery at an older age and were more likely to undergo open cranial vault repair, with increased blood loss, higher transfusion volume, and longer time. This study highlights clinical differences in sagittal craniosynostosis phenotypes and shows that developmental delay is an initial presentation of the saddle phenotype. The saddle phenotype also correlated with fusion of the minor squamous and sphenoid sutures. The link between developmental delay and minor suture fusion was notable and should be explored with a larger sample size. Patients with saddle synostosis present for surgery at an older age than patients with dolichocephaly and therefore are more likely to receive open cranial vault repair, with a taxing intraoperative experience characterized by increased blood loss, increased transfusions, and longer operation time.

Sections du résumé

BACKGROUND
Sagittal craniosynostosis typically presents as dolichocephaly or less frequently as clinocephaly, a "saddle-shaped" phenotype. This project aimed to characterize clinically relevant differences between sagittal synostosis phenotypes and examine the etiology of the delay in presentation.
METHODS
An institutional review board-approved retrospective review was performed from January 1999 to November 2018 at a single institution. Analyses examined correlations between subphenotype, time of presentation, minor suture fusion, developmental delay, and operative technique.
RESULTS
One hundred sixty patients diagnosed with single-suture sagittal craniosynostosis were identified. A total of 30.6% had a saddle phenotype (n = 49) and 69.4% had dolichocephaly (n = 111). Patients with the saddle phenotype were more likely to present with a developmental delay and to have at least 1 minor suture fused than patients with dolichocephaly were. Patients with the saddle phenotype presented for surgery at an older age and were more likely to undergo open cranial vault repair, with increased blood loss, higher transfusion volume, and longer time.
CONCLUSIONS
This study highlights clinical differences in sagittal craniosynostosis phenotypes and shows that developmental delay is an initial presentation of the saddle phenotype. The saddle phenotype also correlated with fusion of the minor squamous and sphenoid sutures. The link between developmental delay and minor suture fusion was notable and should be explored with a larger sample size. Patients with saddle synostosis present for surgery at an older age than patients with dolichocephaly and therefore are more likely to receive open cranial vault repair, with a taxing intraoperative experience characterized by increased blood loss, increased transfusions, and longer operation time.

Identifiants

pubmed: 37740467
doi: 10.1097/SAP.0000000000003137
pii: 00000637-202205004-00009
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

S351-S356

Informations de copyright

Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.

Déclaration de conflit d'intérêts

Conflicts of interest and sources of funding: none declared.

Références

Betances EM, Mendez MD, Das JM. Craniosynostosis. StatPearls. Available at: https://www.ncbi.nlm.nih.gov/pubmed/31335086. Published October 13, 2020. Accessed November 28, 2020.
Di Rocco F, Gleizal A, Szathmari A, et al. Sagittal suture craniosynostosis or craniosynostoses? The heterogeneity of the most common premature fusion of the cranial sutures. Neurochirurgie. 2019;65:232–238.
Beckett JS, Pfaff MJ, Diluna M, et al. Dolichocephaly without sagittal craniosynostosis. J Craniof Surg. 2013;24:1713–1715.
Kolar JC, Salter EM, Weinberg SM. Preoperative craniofacial dysmorphology in isolated sagittal synostosis. J Craniof Surg. 2010;21:1404–1410.
Hubli E. A functional aesthetic approach to correcting the sequelae of sagittal synostosis. Semin Plast Surg. 2014;28:130–137.
Speltz ML, Kapp-Simon KA, Cunningham M, et al. Single-suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol. 2004;29:651–668.
Tadisina KK, Lin AY. Squamosal craniosynostosis: defining the phenotype and indications for surgical management. Ann Plast Surg. 2017;79:458–466.
Yan H, Abel TJ, Alotaibi NM, et al. A systematic review and meta-analysis of endoscopic versus open treatment of craniosynostosis. Part 1: the sagittal suture. J Neurosurg Pediatr. 2018;22:352–360.
Proctor MR. Endoscopic craniosynostosis repair. Transl Pediatr. 2014;3:247–258.
McDonald L, Rennie A, Tolmie J, et al. Investigation of global developmental delay. Arch Dis Child. 2006;91:701–705.
Magge SN, Westerveld M, Pruzinsky T, et al. Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniof Surg. 2002;13:99–104.
Eley KA, Thomas GP, Sheerin F, et al. The significance of squamosal suture synostosis. J Craniofac Surg. 2016;27:1543–1549.
Fontana SC, Belinger S, Daniels D, et al. Longitudinal assessment of developmental outcomes in infants undergoing late craniosynostosis repair. J Craniofac Surg. 2018;29:25–28.
Lattanzi W, Barba M, Di Pietro L, et al. Genetic advances in craniosynostosis. Am J Med Genet A. 2017;173:1406–1429.

Auteurs

Rishi N Modi (RN)

From the UC San Diego School of Medicine.

Caitlyn C Belza (CC)

From the UC San Diego School of Medicine.

Ryan M McKee (RM)

From the UC San Diego School of Medicine.

Mary K Carbullido (MK)

Rady Children's Hospital, San Diego, CA.

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