Scaphocephaly

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Scaphocephaly
Scaphocephalic cranium.jpg
Boy with Scaphocephaly
Pronunciation
  • skaf-o-SEF-aly
Specialty Medical genetics   OOjs UI icon edit-ltr-progressive.svg

Scaphocephaly or sagittal craniosynostosis is a type of cephalic disorder which occurs when there is a premature fusion of the sagittal suture. Premature closure results in limited lateral expansion of the skull, resulting in a characteristic long, narrow head. [1] The skull base is typically spared. [2] The word comes from Ancient Greek σκᾰ́φη (skáphē) 'boat'and κεφαλή (kephalḗ) 'head'.

Contents

Scaphocephaly is the most common of the craniosynostosis conditions and accounts for approximately 50% of all craniosynostosis. [3] It is most commonly idiopathic (non-syndromic).

Etiology

Non-syndromic

The underlying cause of the non-syndromic form is unknown. Over 100 mutations have been associated, including mutations in the FGFR genes. [4] Several potential risk factors have been identified for craniosynostosis include: [5]

Syndromic

Sagittal craniosynostosis is seen in many conditions and syndromes: [7]

Diagnosis and evaluation

Diagnosis of scaphocephaly is with physical exam, which may show characteristic features such as an elongated head in the anterior-posterior dimension, narrow head in the lateral dimension, and bony ridge at the vertex. [8]

Further evaluation with imaging may also be performed. Ultrasound may be used to detect fusion of the suture. CT scans may also be used to help with surgical planning and to diagnose associated hydrocephalus, which has been found to be present in 44% of cases in one study. [9] A measure of cephalic index may also be reduced, however the reliability of measurements may not be a reliable measure. [10]

Classification

Scaphocephaly can be classified into specific types, depending on morphology, position, and suture closure: [11]

Treatment

This condition can be corrected by surgery if the child is young enough, typically within the first 3–6 months. [8] The goal of treatment is to correct intracranial pressure and repair bony deformities. [18] The decision to treat is multifactorial and should be performed at a center with an experienced craniofacial team. [19] In addition to the primary craniofacial surgeon, team members may include audiologists, dentists, otolaryngologists, neurosurgeons, plastic surgeons, and other supporting members. [19]

Surgery is generally aimed at removal of the fused sagittal suture to allow for lateral expansion of the skull. Surgical options include: [8]

Terminology

The term, from Greek skaphe meaning 'light boat or skiff' and kephale meaning 'head', describes a specific shape of a long narrow head [23] that resembles a boat.

See also

References

    1. Sandoval, Jose I.; De Jesus, Orlando (2022), "Scaphocephaly", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID   33620830 , retrieved 2023-01-11
    2. Márquez, Juan Camilo; Herazo Bustos, Cherina; Wagner, Matthias W. (2021-03-01). "Craniosynostosis: Understanding the Misshaped Head" . RadioGraphics. 41 (2): E45 –E46. doi:10.1148/rg.2021200127. ISSN   0271-5333. PMID   33646908. S2CID   232090523.
    3. "Pediatric Sagittal Synostosis | Doernbecher Children's Hospital | OHSU". www.ohsu.edu. Retrieved 2023-01-11.
    4. Ye, Xiaoqian; Guilmatre, Audrey; Reva, Boris; Peter, Inga; Heuzé, Yann; Richtsmeier, Joan T.; Fox, Deborah J.; Goedken, Rhinda J.; Jabs, Ethylin Wang; Romitti, Paul A. (2016-03-01). "Mutation Screening of Candidate Genes in Patients with Nonsyndromic Sagittal Craniosynostosis". Plastic and Reconstructive Surgery. 137 (3): 952–961. doi:10.1097/01.prs.0000479978.75545.ee. ISSN   1529-4242. PMC   4770826 . PMID   26910679.
    5. Kabbani, Haidar; Raghuveer, Talkad S. (2004-06-15). "Craniosynostosis". American Family Physician. 69 (12): 2863–2870. PMID   15222651.
    6. Bradley, C. M.; Alderman, B. W.; Williams, M. A.; Checkoway, H.; Fernbach, S. K.; Greene, C.; Bigelow, P. L.; Reif, J. S. (1995-05-01). "Parental occupations as risk factors for craniosynostosis in offspring". Epidemiology. 6 (3): 306–310. doi: 10.1097/00001648-199505000-00020 . ISSN   1044-3983. PMID   7619941. S2CID   24625014.
    7. "Sagittal craniosynostosis (Concept ID: C0432123)". MedGen. NCBI. Retrieved March 17, 2023.
    8. 1 2 3 Sandoval, Jose I.; De Jesus, Orlando (2022), "Scaphocephaly", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID   33620830 , retrieved 2023-01-03
    9. Wall, Steven A.; Thomas, Gregory P. L.; Johnson, David; Byren, Jo C.; Jayamohan, Jayaratnam; Magdum, Shailendra A.; McAuley, David J.; Richards, Peter G. (2014-12-01). "The preoperative incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis is underestimated in the literature". Journal of Neurosurgery. Pediatrics. 14 (6): 674–681. doi: 10.3171/2014.8.PEDS1425 . ISSN   1933-0715. PMID   25259602.
    10. Fearon, Jeffrey A.; Ditthakasem, Kanlaya; Herbert, Morley; Kolar, John (2017-07-01). "An Appraisal of the Cephalic Index in Sagittal Craniosynostosis, and the Unseen Third Dimension". Plastic and Reconstructive Surgery. 140 (1): 138–145. doi:10.1097/PRS.0000000000003422. ISSN   1529-4242. PMID   28654600. S2CID   31253312.
    11. Skadorwa, Tymon; Wierzbieniec, Olga (August 5, 2022). "The foramen magnum in scaphocephaly". Child's Nervous System. 38 (11): 2163–2170. doi:10.1007/s00381-022-05624-2. PMC   9617951 . PMID   35931858.
    12. Neto, Antonio Rodrigues de Aguiar. "Bathrocephaly | Radiology Reference Article | Radiopaedia.org". Radiopaedia. Retrieved 2023-04-06.
    13. 1 2 Kim, Hyun; Roh, Hong; Lee, Il (May 2016). "Craniosynostosis : Updates in Radiologic Diagnosis - PMC". Journal of Korean Neurosurgical Society. 59 (3): 219–226. doi:10.3340/jkns.2016.59.3.219. PMC   4877543 . PMID   27226852.
    14. Pfaff, Miles; Fenton, Regina; Goldstein, Jesse (2023). "The Clinical Significance of Clinocephaly in Late-Presentation Sagittal Craniosynostosis" . The Cleft Palate Craniofacial Journal. 60 (5): 521–525. doi:10.1177/10556656211064484. PMID   35538850. S2CID   248695057 . Retrieved April 5, 2023.
    15. midwifeshawn (2014-04-01). "Dolichocephaly - understanding 'breech head' molding". Breech Birth Network. Retrieved 2023-04-06.
    16. Vinchon, Matthieu; Pellerin, Philippe; Guerreschi, Pierre; Baroncini, Marc; Dhellemmes, Patrick (2012-09-01). "Atypical scaphocephaly: a review" . Child's Nervous System. 28 (9): 1319–1325. doi:10.1007/s00381-012-1807-8. ISSN   1433-0350. PMID   22872243. S2CID   24212027.
    17. "sphenocephaly", The Free Dictionary, retrieved 2023-04-06
    18. Ciurea, AV; Toader, C; Mihalache, C (2011-11-14). "Actual concepts in scaphocephaly". Journal of Medicine and Life. 4 (4): 424–431. ISSN   1844-122X. PMC   3227159 . PMID   22514579.
    19. 1 2 Buchanan, Edward P; Xue, Yunfeng; Xue, Amy S; Olshinka, Asaf; Lam, Sandi (2017-07-06). "Multidisciplinary care of craniosynostosis". Journal of Multidisciplinary Healthcare. 10: 263–270. doi: 10.2147/JMDH.S100248 . ISSN   1178-2390. PMC   5505551 . PMID   28740400.
    20. "Endoscopic Strip Craniectomy - Seattle Children's". Seattle Children's Hospital. Retrieved 2023-01-04.
    21. Panchal, Jayesh; Uttchin, Venus (2003-05-01). "Management of craniosynostosis". Plastic and Reconstructive Surgery. 111 (6): 2032–2048, quiz 2049. doi:10.1097/01.PRS.0000056839.94034.47. ISSN   0032-1052. PMID   12711969.
    22. "Spring Assisted Cranioplasty | UPMC Children's Hospital". Children's Hospital of Pittsburgh. Retrieved 2023-01-04.
    23. "scaphocephaly" at Dorland's Medical Dictionary

    References