Evaluation and Management of Nonsyndromic Craniosynostosis

Abstract

Nonsyndromic craniosynostosis (NSC) is more common than syndromic craniosynostosis and predominantly involves single suture. It affects sagittal, coronal, metopic, and lambdoid sutures in the decreasing order of frequency. A surgery for NSC is generally recommended to avoid potential neurodevelopmental delays and sequelae of raised intracranial pressure. Open calvarial vault reconstruction, strip craniectomy with/without the use of a postoperative molding helmet, strip craniectomy with spring implantations, endoscopic suture release, and cranial distraction osteogenesis are various surgical options used for NSC cases. The ideal age for intervention is 6-12 months for open procedures and 3-4 months for endoscopic approaches. The management is directed toward minimizing operative trauma and improving the neurocognitive outcome. The role of nonsurgical intervention by the use of genetic manipulation is still not a reality because of the nature of disease and time of presentation.

Keywords: Barrel stave craniotomy; endoscopic-assisted; nonsyndromic craniosynostosis; strip craniectomy; suturectomy.

Figure 1

A case of 6-month-old female child with complex nonsyndromic craniosynostosis (bicoronal, metopic, and partial sagittal synostosis)

Figure 2

Abnormally shaped head as a presentation of craniosynostosis in a child of trigonocephaly, A; brachycephaly, B; scaphocephaly, C

Figure 3

Schematic diagrams showing how to measure cephalic index, A, and different canthal distances, B, as a part of anthropometric parameters in craniosynostotic child. Head breadth = biparietal diameter, head length = occipitofrontal diameter, ICD = intercanthal distance, IOCD = outer canthal distance, IPD = interpupillary distance

Figure 4

A case of complex NSC with MRI brain showing Chiari malformation and MR venogram showing hypoplastic sagittal and B/L transverse sinus with multiple collateral venous channels with prominent straight sinus

Figure 5

A 5-month-old male child has undergone endoscopic suturectomy for sagittal synostosis, and postoperative molding helmet therapy, D, is offered. Pre- and postoperative CT films, A–C, showing the extent of suturectomy

Figure 6

Modified pi technique for sagittal synostosis (3D CT head showing isolated sagittal synostosis), A. After placing a patient in a prone position, adequate calvaria is exposed, B; craniotomy markings being done, indicating midline strip craniotomy involving sagittal suture and bilateral parietal bone craniotomy for barrel staving, C; complete exposure after craniotomy, D; midline strip craniotomy with barrel staving of bilateral parietal bone and fixed with miniplates and sutures, E

Figure 7

3D CT head showing bicoronal synostosis, A, and unilateral coronal synostosis, B

Figure 8

Di Rocco et al. (1988) classified anterior plagiocephaly into three types (schematic representation)

Figure 9

A 9-month-old child with the right anterior plagiocephaly undergone frontoorbital advancement and anterior 2/3 cranial vault remodeling (classical zigzag bicoronal incision extending behind the ear), A; the exposure of cranium anteriorly up to frontozygomatic and frontonasal suture and posteriorly up to lambdoid suture, B; marking frontoorbital bandeau (20–25 mm) involving temporal squama after retracting both periorbita, C; single-piece bifrontal craniotomy above bandeau to switch by 360°, D; newly formed forehead with orbital bandeau with biparietal bone, E; frontoorbital bandeau fixed in an advanced manner (in case of unilateral coronal synostosis more advancement done on affected side), F; and barrel staving of bifrontal bone switched posteriorly and fixed loosely with PDS sutures hanging freely, pre- and postoperative images of head and 3D CT head showing newly formed frontoorbital contour, G

Figure 10

A 1-year-old child with trigonocephaly managed with frontoorbital advancement and anterior 2/3 cranial vault remodeling and after 8-month follow-up

Figure 11

Endoscopic-assisted approach for metopic craniosynostosis in a 4-month-old male child, A, and molding helmet therapy, B, was offered postoperatively and being followed up

Figure 12

A case of oxycephaly with severe copper beaten skull appearance in CT head

Developmental milestone chart for the assessment of pediatric development