Trigonocephaly associated with myelomeningocele in infant: Case report

Context: Trigonocephaly is a type of craniostenosis due to the early closure of the metopic suture. His diagnosis is eminently clinical, but imaging tests are essential. The recommended treatment is surgical and should preferably be performed at the age of 3 to 9 months. Association between craniosynostosis and neural tube defect (NTDs) is rare and was sometimes considered as mere coincidence. However, Martinez-Lage et al. hypothesize that there is a causal relationship between these malformations since myelomeningocele reduces intracranial pulse pressure, which stimulates the early closure of cranial sutures. For Graham et al. the restriction of fetal movements in NTD carriers predisposes to craniosynostosis, as they are likely to keep the skull fixed against the mother’s pelvic bones. Case report: Male patient, 11 months, with West Syndrome. He congenitally presented myelomeningocele, and this deformity was corrected on his first day of life. He subsequently developed a keel-shaped forehead, protrusion of metopic suture, and hypertelorism. The diagnosis of trigonocephaly was ratified by computed tomography of the skull with three-dimensional reconstruction. Surgery was performed at 11 months. The access chosen was coronal and there was a wide exposure of the calvarium. Then all the metopic suture was removed through a bifrontal craniotomy, which allowed the remodeling of the frontal bone. Conclusions: It is necessary to make a diagnosis and early treatment of both comorbidities since delays in the conduct can result in disastrous consequences. In addition, there is a need to conduct more research to elucidate the interrelationship between craniosynostosis and NTD.