Scaphocephaly orsagittal craniosynostosis is a type ofcephalic disorder which occurs when there is a prematurefusion of thesagittal suture. Premature closure results in limited lateral expansion of theskull, resulting in a characteristic long, narrow head.[1] Theskull base is typically spared.[2] The word comes from Ancient Greekσκᾰ́φη (skáphē)'boat' and κεφαλή (kephalḗ)'head'.
Scaphocephaly is the most common of thecraniosynostosis conditions and accounts for approximately 50% of all craniosynostosis.[3] It is most commonlyidiopathic (non-syndromic).
The underlying cause of the non-syndromic form is unknown. Over 100 mutations have been associated, including mutations in theFGFR genes.[4] Several potential risk factors have been identified for craniosynostosis include:[5]
Advanced maternal age
White maternal race
Maternal smoking
Male infant
Certain paternal occupations (e.g. agriculture, forestry, repairmen)[6]
Syndromic
Sagittal craniosynostosis is seen in many conditions and syndromes:[7]
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 associatedhydrocephalus, which has been found to be present in 44% of cases in one study.[9] A measure ofcephalic index may also be reduced, however the reliability of measurements may not be a reliable measure.[10]
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 correctintracranial 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 primarycraniofacial surgeon, team members may includeaudiologists,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]
Endoscopic strip craniectomy: Minimally invasive removal of the fused suture. Following surgery, patients typically wear a helmet to help shape the head. Helmets are usually worn for 3–12 months.[20]
Open cranial vault remodeling: Open surgical removal of the fused sagittal suture and re-shaping of the skull, generally with resorbable plates.[21]
Spring cranioplasty: Combination of an endoscopic strip craniectomy with placement of springs which provide continuous force for re-shaping the skull.[22]
The term, from Greekskaphe meaning 'light boat or skiff' andkephale meaning 'head', describes a specific shape of a long narrow head[23] that resembles a boat.
^abcSandoval, Jose I.; De Jesus, Orlando (2022),"Scaphocephaly",StatPearls, Treasure Island (FL): StatPearls Publishing,PMID33620830, retrieved2023-01-03
^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.ISSN1529-4242.PMID28654600.S2CID31253312.
.jpg)
.jpg)