A 35-year-old female delivered a male infant prematurely at 33 weeks. Neonate weighed 1930 grams at birth and was noted to have a keel like protrusion of the forehead, hypertelorism, narrow temples and fused metopic suture. All the other sutures were noted to be open. A detailed karyotype assessment of the neonate was normal, thus no genetic etiology could be identified. This was considered to be a case of isolated metopic synostosis or single suture craniosynostosis, also referred to as nonsyndromic craniosynostosis. We managed this case with surgical repair which is the mainstay of treatment in craniosynostosis. We present here the 3D reconstructed images from computed tomography scan of the neonate’s skull. (Figure 1) (Figure 2)

Metopic synostosis is a type of craniosynostosis where metopic suture of the fetal skull fuses prematurely leading to a skull malformation called trigonocephaly (triangular forehead) and low volume of anterior cranial fossa. This leads to impaired skull and brain growth. It is associated with advanced maternal age, male fetuses, multiple births, preterm gestation and low birth weight infants. [1] Incidence of metopic synostosis has been increasing over last few years, although incidence of other craniosynostosis including sagittal, unicoronal and multisutural has remained unchanged. [1] The increase in incidence of metopic synostosis may be attributable to increasing incidence of these risk factors. The primary modality of treatment is surgical repair, performed prior to 1 year of age, which traditionally consists of bifrontal craniotomy, skull contouring, bilateral fronto-orbital advancement and placement of a bone graft to increase the width of fronto-orbital bar. This surgical approach is safe with minimal complication rate, short length of hospital stay and great aesthetic outcome. [2] A newer endoscopic approach with all the acknowledged advantages of a minimally invasive procedure over an open surgery is also emerging. [3]

Uncorrected synostosis may cause increase in intracranial pressure and progression of craniofacial deformity.

1. Lee HQ, Hutson JM, Wray AC, et al. Changing epidemiology of nonsyndromic craniosynostosis and revisiting the risk factors. The Journal of craniofacial surgery 2012 Sep;23(5):1245-1.   [CrossRef]   [Pubmed]    
2. Engel M, Thiele OC, Mühling J, et al. Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg 2012 Jun;40(4):347-53.   [CrossRef]   [Pubmed]    
3. Hinojosa J. Endoscopic-assisted treatment of trigonocephaly. Childs Nerv Syst 2012 Sep;28(9):1381-7.   [CrossRef]   [Pubmed]