Presentation
Presented at the Consensus Conference on Craniosynostoses, Rome 1995 Google Scholar 28. Posnick JC, Lin KY, Chen P, Armstrong D (1994) Metopic synostosis: quantitative assessment of presenting deformity and surgical results based on CT scans. [doi.org]
A review of 22 patients with Opitz-C syndrome from the literature is presented. Most of the typical facial dysmorphism can be regarded as part of a trigonocephaly "sequence" rather than presenting characteristic features of a syndrome. [ncbi.nlm.nih.gov]
Entire Body System
- Disability
Moreover PTPRD is known to recruit multiple postsynaptic partners such as IL1RAPL1 which gene alterations lead to non syndromic intellectual disability (ID). [hal-amu.archives-ouvertes.fr]
Twenty patients have normal development without apparent disability. Of these, those of school age are at appropriate grade level. [doi.org]
Both daughters have glabellar capillary hemangiomas, a congenital heart defect and mild developmental disabilities. Review of the literature failed to disclose any syndrome with similar findings. [ncbi.nlm.nih.gov]
Developing as well as discovering disabilities prevail. [valleyfamilymedicineurgentcarecenter.com]
- Surgical Procedure
procedure after a minimum of 1 year. [doi.org]
For this reason, our surgical procedure includes turning the orbital ridge away from the midline, frontal vault remodeling, and a wide ostectomy of the pterional bone. [ncbi.nlm.nih.gov]
Our technique allows shortening the entire surgical procedure, especially in the departments where blood saving devices are not available. 1. Baird L.C., Proctor M.R.: Craniosynostosis, Albright A.L., Pollack I.F., Adelson P.D. [degruyter.com]
If, after evaluation, your child is determined to need treatment for his metopic synostosis, members of his Craniofacial Anomalies Program care team may include: neurosurgeons, who perform surgical procedures on a child’s brain, spinal cord or nerves [childrenshospital.org]
- Swelling
Results: The postoperative period was uneventful except for the expected forehead swelling. The babies were discharged from the hospital on average at the 8 th postoperative day. [degruyter.com]
Other benefits of minimally invasive techniques would be avoidance of anaesthetic surgical scarring, decrease in postoperative swelling and discomfort, and lower rate of complications such as duramater tears, postoperative hyperthermia, or infection. [ncbi.nlm.nih.gov]
Our son recovered so well that 3 weeks after surgery he was looking almost normal (still had some swelling) and starting to move arund and play as any 7 month old would. I can not express how important it was for DS to have the surgery. [huggies.com.au]
Psychiatrical
Neurologic
- Stroke
[…] port-wine stain involving the first division of the trigeminal nerve with the diagnosis of a craniosynostosis justifies the indication of a prophylactic magnetic resonance imaging acquisition before craniofacial surgeries, in order to prevent seizures and stroke-like [ncbi.nlm.nih.gov]
Treatment
CONCLUSIONS: Early detection and treatment of metopic suture synostosis has a significant, favorable influence on the outcomes. [ncbi.nlm.nih.gov]
Anderson FM (1981) Treatment of coronal and metopic synostosis: 107 cases. Neurosurgery 8:143–149 Google Scholar 2. Anderson FM, Gwinn JL, Todt JC (1962) Trigonocephaly: identity and surgical treatment. J Neurosurg 19:723–730 Google Scholar 3. [doi.org]
Some children with just a ridge or mild metopic synostosis don’t need any medical treatment. For those who do, surgery has proven to be a successful approach. [childrenshospital.org]
Prognosis
The importance of the minor anomalies in its recognition and its good prognosis are emphasized. [ncbi.nlm.nih.gov]
Prognosis In general, a single operation is all that is necessary to treat this condition and the cosmetic and clinical outcomes are usually excellent especially for otherwise healthy, nonsyndromic infants. [skullbaseinstitute.com]
Prognosis The clinical outcome for patients having undergone surgery is usually satisfactory with a low requirement for additional interventions, especially in the mild forms. Recurrence of a prominent metopic ridge may occur in some rare cases. [orpha.net]
Etiology
Etiology The underlying genetic cause of isolated trigonocephaly remains to be delineated. [orpha.net]
Sinn, Classification, Diagnosis, and Etiology of Craniofacial Deformities, Maxillofacial Surgery, 10.1016/B978-0-7020-6056-4.00059-9, (803-834), (2017). [doi.org]
However, the concordance rate of isolated trigonocephaly in monozygotic twins is 43%, suggesting that both genetic and environmental factors are involved in the etiology of this disorder. [panafrican-med-journal.com]
Epidemiology
The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. [books.google.de]
Summary Epidemiology Incidence is estimated at 1/15,000 births. Males are more frequently affected than females (sex ratio of 2:1) and the frequency of trigonocephalic twins is unexpectedly high. [orpha.net]
Bartlett, The Changing Epidemiologic Spectrum of Single-Suture Synostoses, Plastic and Reconstructive Surgery, 10.1097/PRS.0b013e31817d548c, 122, 2, (527-533), (2008). [doi.org]
Pathophysiology
However, because the exact pathophysiology remains unknown, we examined the pathophysiologic mechanisms behind cognitive dysfunction in patients with trigonocephaly, with an aim of providing a model for cognitive dysfunction based on routinely available [ncbi.nlm.nih.gov]
Okkerse, Insight into the Pathophysiologic Mechanisms behind Cognitive Dysfunction in Trigonocephaly, Plastic and Reconstructive Surgery, 10.1097/PRS.0000000000003179, 139, 4, (954e-964e), (2017). David M. Yates and Douglas P. [doi.org]
Prevention
The possibility of coincidence should not prevent continued surveillance of OTS patients in the future for the occurrence of malignancy. [ncbi.nlm.nih.gov]
Conclusion: The early recognition of these anomalies including all craniosynostoses, the deformities of the newborn and infant’s head and the preventive operative reconstruction would prevent abnormal disturbance of the psychomotor development during [degruyter.com]