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Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment.
J Craniofac Surg. 2016 Jun; 27(4):871-3.JC

Abstract

INTRODUCTION

Due to the rarity of isolated frontosphenoidal suture synostosis clinical diagnosis can be challenging. This study of 6 patients aims to review the clinical, radiological findings, and operative techniques used to correct the underlying pathology.

METHODS

Patients with isolated frontosphenoidal suture craniosynostosis were selected from a retrospective review of 88 patients with unicoronal synostosis treated during a 3-year period. Two-dimensional photography of patients' soft tissue morphology from the vertex view allowed assessment of the following morphology: frontal bossing, brow depression, nasal tip deviation, and ear position. Quantitative measure of the extent of bony deformity was measured using various angles measured from two-dimensional axial views of computerized tomography scans. Last, technical variations in correction of isolated frontosphenoidal craniosynostosis were collected from operative notes.

RESULTS

On the side of isolated frontosphenoidal craniosynostosis, contralateral bossing and ipsilateral brow depression was present in all 6 patients. Ipsilateral nasal tip deviation was seen in 3 out of the 6 patients. Ear position was symmetrical in the cranial-caudal and anterior-posterior axes. No radiological evidence of harlequin deformity was seen on skull X-ray in all 6 patients, but computerized tomography scans demonstrated isolated frontosphenoidal suture craniosynostosis. The angle drawn between the foramen magnum, sella turcica, and anterior cribriform plate in 3 of 6 patients showed deflection of the anterior cranial fossa opposite to the side of isolated frontosphenoidal suture craniosysnotosis. There was no difference in the angle between the petrosal pyramid and the midline. In all patients, operative technique involved taking a deeper fronto-orbital bandeau to capture and reshape the pathological suture.

CONCLUSIONS

In isolated frontosphenoidal suture craniosynostosis, contralateral bossing and ipsilateral flattening of the forehead were the most consistent clinical features with nasal tip deviation away from the side of pathology less consistent. Ear position is unaffected. Measurements of various angles of the skull base were not consistent. A deeper vertical osteotomy at the site of isolated frontosphenoidal suture craniosysnotosis on removing the fronto-orbital bandeau was 1 operative technical variation.

Authors+Show Affiliations

Department of Craniofacial Surgery, Birmingham Children's Hospital, Birmingham, UK.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

27152571

Citation

Lloyd, Mark Sheldon, et al. "Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment." The Journal of Craniofacial Surgery, vol. 27, no. 4, 2016, pp. 871-3.
Lloyd MS, Rodrigues D, Nishikawa H, et al. Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment. J Craniofac Surg. 2016;27(4):871-3.
Lloyd, M. S., Rodrigues, D., Nishikawa, H., White, N., Solanki, G., Noons, P., Evans, M., & Dover, S. (2016). Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment. The Journal of Craniofacial Surgery, 27(4), 871-3. https://doi.org/10.1097/SCS.0000000000002559
Lloyd MS, et al. Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment. J Craniofac Surg. 2016;27(4):871-3. PubMed PMID: 27152571.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment. AU - Lloyd,Mark Sheldon, AU - Rodrigues,Desi, AU - Nishikawa,Hiroshi, AU - White,Nicholas, AU - Solanki,Gurish, AU - Noons,Peter, AU - Evans,Martin, AU - Dover,Stephen, PY - 2016/5/7/entrez PY - 2016/5/7/pubmed PY - 2017/6/27/medline SP - 871 EP - 3 JF - The Journal of craniofacial surgery JO - J Craniofac Surg VL - 27 IS - 4 N2 - INTRODUCTION: Due to the rarity of isolated frontosphenoidal suture synostosis clinical diagnosis can be challenging. This study of 6 patients aims to review the clinical, radiological findings, and operative techniques used to correct the underlying pathology. METHODS: Patients with isolated frontosphenoidal suture craniosynostosis were selected from a retrospective review of 88 patients with unicoronal synostosis treated during a 3-year period. Two-dimensional photography of patients' soft tissue morphology from the vertex view allowed assessment of the following morphology: frontal bossing, brow depression, nasal tip deviation, and ear position. Quantitative measure of the extent of bony deformity was measured using various angles measured from two-dimensional axial views of computerized tomography scans. Last, technical variations in correction of isolated frontosphenoidal craniosynostosis were collected from operative notes. RESULTS: On the side of isolated frontosphenoidal craniosynostosis, contralateral bossing and ipsilateral brow depression was present in all 6 patients. Ipsilateral nasal tip deviation was seen in 3 out of the 6 patients. Ear position was symmetrical in the cranial-caudal and anterior-posterior axes. No radiological evidence of harlequin deformity was seen on skull X-ray in all 6 patients, but computerized tomography scans demonstrated isolated frontosphenoidal suture craniosynostosis. The angle drawn between the foramen magnum, sella turcica, and anterior cribriform plate in 3 of 6 patients showed deflection of the anterior cranial fossa opposite to the side of isolated frontosphenoidal suture craniosysnotosis. There was no difference in the angle between the petrosal pyramid and the midline. In all patients, operative technique involved taking a deeper fronto-orbital bandeau to capture and reshape the pathological suture. CONCLUSIONS: In isolated frontosphenoidal suture craniosynostosis, contralateral bossing and ipsilateral flattening of the forehead were the most consistent clinical features with nasal tip deviation away from the side of pathology less consistent. Ear position is unaffected. Measurements of various angles of the skull base were not consistent. A deeper vertical osteotomy at the site of isolated frontosphenoidal suture craniosysnotosis on removing the fronto-orbital bandeau was 1 operative technical variation. SN - 1536-3732 UR - https://www.unboundmedicine.com/medline/citation/27152571/Isolated_Unilateral_Frontosphenoidal_Suture_Synostosis_in_Six_Patients:_Lessons_Learned_in_Diagnosis_and_Treatment_ L2 - https://doi.org/10.1097/SCS.0000000000002559 DB - PRIME DP - Unbound Medicine ER -