Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients.

OBJECTIVE Surgery for craniosynostosis has evolved rapidly over the past two decades, with increased emphasis on early, extensive operations. Older published series may not accurately reflect more recent experience. Our study was designed to analyze outcome in a large series of consecutive patients treated recently at a single center. METHODS We reviewed 250 consecutive patients who underwent surgical treatment of craniosynostosis between January 1, 1987 and December 31, 1992. They were divided into nine groups by suture involvement: sagittal, unilateral coronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid, metopic, multiple suture, the Klee-blattschädel deformity (cloverleaf skull), and acquired craniosynostosis. Outcome was analyzed in terms of residual deformities and irregularities, complications, mortality, as well as the need for additional surgery. RESULTS There were 157 males (62. 8%) and 93 females (37.2%), with most of the male preponderance accounted for by the large sagittal synostosis group, which consisted of 82 males and 25 females. Median age at first operation was 147 days. A named syndrome was present in 23 patients (9.2%) and was more common than expected with bilateral and unilateral coronal synostosis, the Kleeblattschädel deformity, and multiple suture synostosis. There were two deaths (0.8%), both with Klee-blattschädel patients, and 17 other complications (6.8%). Morbidity and mortality were significantly associated with secondary vs primary operations and syndromic vs nonsyndromic patients. Outcome analysis revealed the best surgical results with metopic synostosis and significantly less good results with the Kleeblattschädel deformity, multiple suture synostosis, and bilateral coronal synostosis. CONCLUSIONS Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and relatively low morbidity and mortality, particularly for otherwise healthy, nonsyndromic infants.

[1]  D. Rénier,et al.  Experience with the "floating forehead". , 1988, British journal of plastic surgery.

[2]  S. Bartlett,et al.  Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. , 1987, Plastic and reconstructive surgery.

[3]  J. Jane,et al.  Variants of sagittal synostosis: strategies for surgical correction. , 1984, Journal of neurosurgery.

[4]  American Academy of Pediatrics AAP Task Force on Infant Positioning and SIDS: Positioning and SIDS. , 1992, Pediatrics.

[5]  B H Grayson,et al.  Twenty‐Year Experience with Early Surgery for Craniosynostosis: II. The Craniofacial Synostosis Syndromes and Pansynostosis—Results and Unsolved Problems , 1995, Plastic and reconstructive surgery.

[6]  J L Marsh,et al.  Observations on a recent increase in plagiocephaly without synostosis. , 1996, Pediatrics.

[7]  Phillip K. T. Chen,et al.  Metopic Synostosis: Quantitative Assessment of Presenting Deformity and Surgical Results Based on CT Scans , 1994, Plastic and reconstructive surgery.

[8]  A. Sokol Advancement of the orbits and the midface in one piece, combined with frontal repositioning, for the correction of Crouzon's deformities: F. Ortiz-Monasterio, A. Fuente del Campo, and A. Carrillo. Plast and Reconstr Surg 61:507–516 (April), 1978 , 1978 .

[9]  J. McCarthy,et al.  Early Surgery for Craniofacial Synostosis: An 8‐Year Experience , 1984, Plastic and reconstructive surgery.

[10]  Aap Task Force on Infant Positioning and Sids Positioning and SIDS , 1992, Pediatrics.

[11]  J. McComb Occipital reduction-biparietal widening technique for correction of sagittal synostosis. , 1994, Pediatric neurosurgery.

[12]  J. Graham,et al.  Sagittal craniostenosis: fetal head constraint as one possible cause. , 1979, The Journal of pediatrics.

[13]  L. Shuer,et al.  Multiple-suture synostosis subsequent to ventricular shunting. , 1994, Plastic and reconstructive surgery.

[14]  S. Berkowitz,et al.  The Monobloc Frontofacial Advancement: Do the Pluses Outweigh the Minuses? , 1993, Plastic and reconstructive surgery.

[15]  M. Edgerton,et al.  External Cranial Vault Molding after Craniofacial Surgery , 1986, Annals of plastic surgery.

[16]  H. Hoffman,et al.  Delayed and progressive multiple suture craniosynostosis. , 1990, Neurosurgery.

[17]  J. Posnick,et al.  Sagittal synostosis: quantitative assessment of presenting deformity and surgical results based on CT scans. , 1993, Plastic and reconstructive surgery.

[18]  L. Whitaker,et al.  EARLY SURGERY FOR ISOLATED CRANIOFACIAL DYSOSTOSIS: Improvement and Possible Prevention of Increasing Deformity , 1977, Plastic and reconstructive surgery.

[19]  H. Hoffman,et al.  Progressive cranial suture stenosis in craniosynostosis. , 1991, Neurosurgery clinics of North America.

[20]  D. Marchac Radical forehead remodeling for craniostenosis. , 1978, Plastic and reconstructive surgery.

[21]  A. F. del Campo,et al.  ADVANCEMENT OF THE ORBITS AND THE MIDFACE IN ONE PIECE, COMBINED WITH FRONTAL REPOSITIONING, FOR THE CORRECTION OF CROUZON'S DEFORMITIES , 1978, Plastic and reconstructive surgery.

[22]  B H Grayson,et al.  Twenty‐Year Experience with Early Surgery for Craniosynostosis: I. Isolated Craniofacial Synostosis—Results and Unsolved Problems , 1995, Plastic and reconstructive surgery.

[23]  D. Rénier,et al.  Craniofacial Surgery for Craniosynostosis Improves Facial Growth: A Personal Case Review , 1985, Annals of plastic surgery.

[24]  D. D. Matson,et al.  Craniosynostosis: a review of 519 surgical patients. , 1968, Pediatrics.

[25]  L. Whitaker,et al.  Complications with Facial Advancement: A Comparison Between the Le Fort III and Monobloc Advancements , 1993, Plastic and reconstructive surgery.