Advances in Clinical and Experimental Medicine
2019, vol. 28, nr 5, May, p. 625–635
Publication type: original article
Trigonocephaly: Long-term results after surgical correction of metopic suture synostosis
1 Department of Plastic Surgery, Wroclaw Medical University, Polanica-Zdrój, Poland
Background. Premature closure and ossification of the metopic suture results in a triangular head shape called trigonocephaly and is characterized by a wedge-shaped forehead and frontotemporal narrowing. Untreated craniosynostosis may lead to increased intracranial pressure (ICP) and, thereby, impaired neurodevelopment. Over the last decades, its incidence has been increasing, currently making it the 2nd most common type of isolated craniosynostosis. Treatment consist of cranioplasty, which should be performed before the age of 1 year.
Objectives. The aim of this study was to evaluate the long-term surgical outcomes in children operated on for trigonocephaly.
Material and Methods. The authors reviewed 30 consecutive cases of metopic synostosis treated over a 14-year period in the Plastic Surgery Department in Polanica-Zdrój, Poland. The data was evaluated using the patients’ clinical records, and preoperative and postoperative photographs. The patients showed up on a follow-up visit at a median age of 9 years and were examined by an ophthalmologist and a neurologist. The surgical outcomes were evaluated according to the Whitaker classification. In 23 patients, remodeling and the advancement of fronto-orbital skull segments was performed at a median age of 18 months and in 7 milder cases, simple suturectomy or burring of the metopic ridge was sufficient.
Results. According to the Whitaker classification, results were considered good to excellent (category I and II). Only 1 patient was included into category III. None of the examined cases were included into category IV, which would require a major craniofacial procedure, duplicating or exceeding the original operation. Neurological abnormalities were found in 12 cases and vision defects in 15 cases.
Conclusion. Trigonocephaly is currently the 2nd most common type of isolated synostosis. Surgical treatment based on Tessier’s and Marchac’s modified methods provides good results in patients at the age of about 12 months and prevents the consequences of ICP increase. Primary neurological and behavioral disorders may occur, despite corrective surgery.
craniosynostosis, trigonocephaly, metopic synostosis
- Vu HL, Panchal J, Parker EE, Levine NS, Francel P. The timing of physiologic closure of the metopic suture: A review of 159 patients using reconstructed 3D CT scans of the craniofacial region. J Craniofac Surg. 2001;12(6):527–532.
- Persing JA, Jane JA, Shaffrey M. Virchow and the pathogenesis of craniosynostosis: A translation of his original work. Plast Reconstr Surg. 1989;83(4):738–742.
- van der Meulen J. Metopic synostosis. Child Nerv Syst. 2012;28(9):1359–1367.
- Lajeunie E, Le Merrer M, Marchac D, Renier D. Syndromal and nonsyndromal primary trigonocephaly: Analysis of a series of 237 patients. Am J Med Genet. 1998;75(2):211–215.
- Engel M, Thiele OC, Muhling J, et al. Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg. 2012;40(4):347–353.
- Lajeunie E, Crimmins DW, Arnaud E, Renier D. Genetic considerations in nonsyndromic midline craniosynostoses: A study of twins and their families. J Neurosurg. 2005;103(4 Suppl):353–356.
- van der Vlugt JJB, van der Meulen J, Creemers HE, Verhulst FC, Hovius SER, Okkerse JME. Cognitive and behavioral functioning in 82 patients with trigonocephaly. Plast Reconstr Surg. 2012;130(4):885–893.
- van der Meulen J, van der Hulst R, van Adrichem L, et al. The increase of metopic synostosis: A pan-European observation. J Craniofac Surg. 2009;20(2):283–286.
- Di Rocco F, Arnaud E, Renier D. Evolution in the frequency of nonsyndromic craniosynostosis. J Neurosurg Pediatr. 2009;4(1):21–25.
- Kweldam CF, van der Vlugt JJ, van der Meulen JJ. The incidence of craniosynostosis in the Netherlands, 1997–2007. J Plast Reconstr Aesthet Surg. 2011;64(5):583–588.
- Cohen SR, Persing JA. Intracranial pressure in single-suture craniosynostosis. Cleft Palate Craniofac J. 1998;35(3):194–196.
- Selber J, Reid RR, Gershman B, et al. Evolution of operative techniques for the treatment of single-suture metopic synostosis. Ann Plast Surg. 2007;59(1):6–13.
- Tessier P. Definitive plastic surgical treatment of severe facial deformities of craniofacial dysostosis: Crouzons and Aperts diseases. Plast Reconstr Surg. 1971;48(5):419–442.
- Marchac D. Radical forehead remodeling for craniostenosis. Plast Reconstr Surg. 1978;61(6):823–835.
- Patel A, Chang CC, Terner JS, Tuggle CT, Persing JA. Improved correction of supraorbital rim deformity in craniosynostosis by the “tilt” pro-cedure. J Craniofac Surg. 2012;23(2):370–373.
- Di Rocco C, Frassanito P, Tamburrini G. The shell technique: Bilateral fronto-orbital reshaping in trigonocephaly. Childs Nerv Syst. 2013;29(12):2189–2194.
- Marchac D, Renier D, Broumand S. Timing of treatment for craniosynostosis and faciocraniosynostosis: A 20-year experience. Br J Plast Surg. 1994;47(4):211–222.
- Whitaker LA, Bartlett SP, Schut L, Bruce D. Craniosynostosis: An analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg. 1987;80(2):195–206.
- Lajeunie E, Le Merrer M, Arnaud E, Marchac D, Renier D. Primary trigonocephaly: Isolated, associated and syndromic forms. Genetic study of a series of 278 patients. Arch Pediatr. 1998;5(8):873–879.
- Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C. Intracranial pressure monitoring in children with single suture and complex craniosynostosis: A review. Childs Nerv Syst. 2005;21(10):913–921.
- Walsh TJ, Garden JW, Gallagher B. Obliteration of retinal venous pulsations during elevation of cerebrospinal-fluid pressure. Am J Ophthalmol. 1969;67(6):954–956.
- Tuite GF, Chong WK, Evanson J, et al. The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery. 1996;38(2):272–278.
- Campbell JW, Albright AL, Losken HW, Biglan AW. Intracranial hypertension after cranial vault decompression for craniosynostosis. Pediatr Neurosurg. 1995;22(5):270–273.
- Denis D, Genitori L, Bardot J, Saracco JB, Choux M, Maumenee IH. Ocular findings in trigonocephaly. Graefes Arch Clin Exp Ophthalmol. 1994;232(12):728–733.
- Bottero L, Lajeunie E, Arnaud E, Marchac D, Renier D. Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg. 1998;102(4):952–958; discussion 959–960.
- Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Childs Nerv Syst. 2000;16(10–11):645–658.
- Ozlen F, Kafadar AM, Abuzayed B, et al. Surgical treatment of trigonocephaly: Technique and long-term results in 48 cases. J Neurosurg Pediatr. 2011;7(5):565–565.
- van der Meulen J, Willemsen J, van der Vlugt J, et al; Dutch Craniofacial Unit. On the origin of bitemporal hollowing. J Craniofac Surg. 2009;20(3):752–756.
- Oh AK, Greene AK, Mulliken JB, Rogers GF. Prevention of temporal depression that follows fronto-orbital advancement for craniosynostosis. J Craniofac Surg. 2006;17(5):980–985.
- Wes AM, Paliga JT, Goldstein JA, Whitaker LA, Bartlett SP, Taylor JA. An evaluation of complications, revisions, and long-term aesthetic outcomes in nonsyndromic metopic craniosynostosis. Plast Reconstr Surg. 2014;133(6):1453–1464.
- Hormozi AK, Shahverdiani R, Mohammadi HR, Zali A, Mofrad HRH. Surgical treatment of metopic synostosis. J Craniofac Surg. 2011;22(1):261–265.
- Esparza J, Hinojosa J. Complications in the surgical treatment of craniosynostosis and craniofacial syndromes: Apropos of 306 transcranial procedures. Childs Nerv Syst. 2008;24(12):1421–1430.
- Jimenez DF, Barone CM, Cartwright CC, Baker L. Early management of craniosynostosis using endoscopic-assisted strip craniectomies and cranial orthotic molding therapy. Pediatrics. 2002;110(1 Pt 1):97–104.
- Metzler P, Ezaldein HH, Persing JA, Steinbacher DM. Comparing two fronto-orbital advancement strategies to treat trigonocephaly in metopic synostosis. J Craniomaxillofac Surg. 2014;42(7):1437–1441.
- Tokumaru AM, Barkovich AJ, Ciricillo SF, Michael SB. Skull base and calvarial deformities: Association with intracranial changes in craniofacial syndromes. Am J Neuroradiol. 1996;17(4):619–630.
- Raybaud C, Di Rocco C. Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: A review. Childs Nerv Syst. 2007;23(12):1379–1388.
- Speltz ML, Kapp-Simon KA, Cunningham M, Marsh J, Dawson G. Single-suture craniosynostosis: A review of neurobehavioral research and theory. J Pediatr Psychol. 2004;29(8):651–668.
- Kapp-Simon KA, Speltz ML, Cunningham ML, Patel PK, Tomita T. Neurodevelopment of children with single suture craniosynostosis: A review. Childs Nerv Syst. 2007;23(3):269–281.