Child's Nervous System

, Volume 21, Issue 5, pp 392–398 | Cite as

Surgical correction of metopic synostosis

  • Henry E. Aryan
  • Rahul Jandial
  • Burak M. Ozgur
  • Samuel A. Hughes
  • Hal S. Meltzer
  • Min S. Park
  • Michael L. Levy
Original Paper

Abstract

Background

Premature closure of the metopic suture results in deformation of the anterior portion of the calvarium, which can vary from mild to severe. In mild forms, there is only prominent ridging of the metopic suture; more severe forms result in a marked narrowing of the frontal and temporal regions that in turn affects the supraorbital rims and produces hypotelorism.

Methods

The authors retrospectively reviewed 39 consecutive cases of metopic synostosis treated over a 12-year period.

Results

The average age at referral was 5 months, with surgery performed at an average age of 7.5 months. Fifteen infants had other congenital anomalies, with eight having synostosis of other sutures. Follow-up ranged from 7 months to 6 years, with an average of 29 months. In three mild cases, burring of the metopic ridge was performed with excellent aesthetic results in all cases. The other 36 patients had significant deformity of the supraorbital ridges and temporal regions, with obvious hypotelorism for over 50% of the time. In these cases, the patients underwent craniofacial reconstruction to normalize their appearance. In addition, the lateral aspect of the sphenoid ridges, including the orbital roof and lateral orbital wall to the infraorbital fissure, was removed to free the cranial base. The average blood loss was under 400 ml and the average hospital stay was 3.6 days. Results were considered good to excellent in all except three cases, which had recurrence of a prominent metopic ridge; two required a second operation after 6 months for burring of this ridge, whereas the third was treated conservatively with an orthotic headband.

Conclusion

Mild forms of metopic synostosis can be successfully treated with burring of the metopic ridge alone. Severe forms require craniofacial reconstruction and may be associated with other congenital abnormalities, additional synostosis, and developmental delay. In all cases, the operative procedure must be tailored to the nature and severity of the deformity.

Keywords

Metopic synostosis Surgery Correction Pediatric neurosurgery Craniofacial surgery 

References

  1. 1.
    Alderman BW, Fernbach SK, Greene C, Mangione EJ, Ferguson SW (1997) Diagnostic practice and the estimated prevalence of craniosynostosis in Colorado. Arch Pediatr Adolesc Med 151:159–164Google Scholar
  2. 2.
    Anderson FM, Geiger L (1965) Craniosynostosis: a survey of 204 cases. J Neurosurg 7:22–29Google Scholar
  3. 3.
    Anderson FM, Gwinn JL, Todt JC (1962) Trigonocephaly: identity and treatment. J Neurosurg 3:19–23Google Scholar
  4. 4.
    Azimi C, Kennedy SJ, Chitayat D, Chakraborty P, Clarke JT, Forrest C, Teebi AS (2003) Clinical and genetic aspects of trigonocephaly: a study of 25 cases. Am J Med Genet 117A:127–135Google Scholar
  5. 5.
    Bird HR, Yager M, Staghezza B, Gould MS, Canino G, Rubio-Stipec M (1990) Impairment in epidemiological measurement of childhood psychopathology in the community. J Am Acad Child Adolesc Psychiatry 8:97–96Google Scholar
  6. 6.
    Bottero L, Lajeunie E, Arnaud E, Marchac D, Renier D (1998) Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg 10:952–958Google Scholar
  7. 7.
    Cohen MM (1986) Syndromes with craniosynostosis. In: Cohen MM (ed) Craniosynostosis: diagnosis, evaluation and management. Raven, New York, pp 413–590Google Scholar
  8. 8.
    Collmann H, Sorensen N, Krauss J (1996) Consensus: trigonocephaly. Childs Nerv Syst 12:664–668Google Scholar
  9. 9.
    Currarino G, Silverman FN (1960) Orbital hypotelorism, arhinencephaly and trigonocephaly. Radiology 74:206–217Google Scholar
  10. 10.
    Delashaw JB, Persing JA, Park TS, Jane JA (1986) Surgical approaches for the correction of metopic synostosis. Neurosurgery 19:228–234Google Scholar
  11. 11.
    Denis D, Genitori L, Conrath J, Lena G, Choux M (1996) Ocular findings in children operated on for plagiocephaly and trigonocephaly. Childs Nerv Syst 12:683–689Google Scholar
  12. 12.
    Dhellernrnes P, Pellerin P, Lejeune JP, Lepoutre F (1986) Surgical treatment of trigonocephaly. Experience with 30 cases. Childs Nerv Syst 2:228–232Google Scholar
  13. 13.
    DiRocco Velardi F, Ferrario A, Marchese E (1996) Metopic synostosis: in favour of a “simplified” surgical treatment. Childs Nerv Syst 12:654–663Google Scholar
  14. 14.
    Fearon J, Kolar JC, Munro JR (1996) Trigonocephaly-associated hypotelorism: is treatment necessary? Plast Reconstr Surg 97:503–509Google Scholar
  15. 15.
    Frydman M, Kauschanksy A, Elian F (1984) Trigonocephaly: a new familial syndrome. Am J Med Genet 18:55–59Google Scholar
  16. 16.
    Furuya Y, Edwards MS, Alpers CE, Tress BM, Norman D, Ousterhout DK (1984) Computerized tomography of cranial sutures. I. Comparison of suture anatomy in children and adults. J Neurosurg 61:53–58Google Scholar
  17. 17.
    Furuya Y, Edwards MS, Alpers CE, Tress BM, Norman D, Ousterhout DK (1984) Computerized tomography of cranial sutures. II. Abnormalities of sutures and skull deformity in craniosynostosis. J Neurosurg 61:59–70Google Scholar
  18. 18.
    Genitori L, Cavalheiro S, Lena G, Dollo C, Choux M (1991) Skull base in trigonocephaly. Pediatr Neurosurg 17:175–181Google Scholar
  19. 19.
    Goodrich JT, Hall GE (1995) Metopic synostosis and trigonocephaly: a spectrum of dysmorphology. In: Goodrich JT, Hall GE (eds) Craniofacial anomalies: growth and development from a surgical perspective. Thieme, New York, pp 97–106Google Scholar
  20. 20.
    Hansman CF (1966) Growth of interorbital distance and skull thickness as observed in roentgenographic measurements. Radiology 86:87–96Google Scholar
  21. 21.
    Hunter AGW, Rudd NL, Hoffman HJ (1976) Trigonocephaly and associated minor anomalies in mother and son. J Med Genet 13:77–79Google Scholar
  22. 22.
    Kapp-Simon KA, Figueroa A, Jocher CA, Schafer M (1993) Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 92:831–839Google Scholar
  23. 23.
    Kerwin WJ, Cohen SR, Burstein FD, Hudgins R, Boydston W, Simms C (1997) A longitudinal statistical study of reoperation rates in craniosynostosis. Plast Reconstr Surg 100:305–310Google Scholar
  24. 24.
    Marchac D (1978) Radical forehead remodeling for craniosynostosis. Plast Reconstr Surg 61:823–835Google Scholar
  25. 25.
    Marsh JL, Schwartz HG (1983) The surgical correction of coronal and metopic craniosynostoses. J Neurosurg 59:245–251Google Scholar
  26. 26.
    Moss MI (1975) Functional anatomy of cranial synostosis. Childs Brain 1:22–23Google Scholar
  27. 27.
    Oi S, Matsumoto S (1987) Trigonocephaly (metopic synostosis). Childs Nerv Syst 3:259–265PubMedGoogle Scholar
  28. 28.
    Paige KT, Cohen SR, Simms C, Burstein FD, Hudgins R, Boydston W (2003) Predicting the risk of reoperation in metopic synostosis: a quantitative CT scan analysis. Ann Plast Surg 51:167–172Google Scholar
  29. 29.
    Posnick JC, Lin KY, Chen P, Armstrong D (1994) Metopic synostosis: quantitative assessment of presenting deformity and surgical results based on CT scans. Plast Reconstr Surg 26:442–448Google Scholar
  30. 30.
    Preus M, Vekemans M, Kaplan P (1986) Diagnosis of chromosome 3 duplication q23-qter, deletion p25-pter in a patient with the C (trigonocephaly) syndrome. Am J Med Genet 23:935–943Google Scholar
  31. 31.
    Robin NH (1999) Molecular genetic advances in understanding craniosynostosis. Plast Reconstr Surg 103:1060–1070Google Scholar
  32. 32.
    Sadove AM, Kalsheck JE, Eppley BL, Javed I (1990) Modifications in the surgical correction of trigonocephaly. Plast Reconstr Surg 85:853–858Google Scholar
  33. 33.
    Salkind G, Sutton LN, Bruce DA, Schut L, Schut A (1986) Management of trigonocephaly. Surg Neurol 25:159–162Google Scholar
  34. 34.
    Sargent C, Burn J, Baraitser M, Pembrey ME (1985) Trigonocephaly and the Opitz C syndrome. J Med Genet 2:39–45Google Scholar
  35. 35.
    Scarfo GB, Tomaccini D (1980) Trigonocephaly. Some clinical and surgical features. Acta Neurol Belg 80:137–143Google Scholar
  36. 36.
    Schaap C, Schrander-Strumpel CT, Fryns JP (1992) Opitz-C syndrome: on the nosology of mental retardation and trigonocephaly. Genet Couns 3:209–215Google Scholar
  37. 37.
    Shillito J Jr, Matson DD (1968) Craniosynostosis: a review of 519 surgical patients. Pediatrics 41:829–853Google Scholar
  38. 38.
    Sidoti EJ Jr, Marsh JL, Marty-Grames L, Noetzel MJ (1996) Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances. Plast Reconstr Surg 97:276–281Google Scholar
  39. 39.
    Sloan GM, Wells KC, Raffel C, McComb JG (1997) Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics 100:1–9Google Scholar
  40. 40.
    Tuite GF, Chong WK, Evanson J, Narita A, Taylor D, Harkness WF, Jones BM, Hayward R (1996) The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery 38:272–278Google Scholar

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Henry E. Aryan
    • 1
    • 2
  • Rahul Jandial
    • 1
  • Burak M. Ozgur
    • 1
  • Samuel A. Hughes
    • 1
  • Hal S. Meltzer
    • 1
    • 2
  • Min S. Park
    • 1
  • Michael L. Levy
    • 1
    • 2
  1. 1.Division of NeurosurgeryUniversity of California Medical CenterSan DiegoUSA
  2. 2.Department of NeurosurgeryChildren’s HospitalSan DiegoUSA

Personalised recommendations