I thought these articles, all of which were obtained from the National Library of Medicine (you can order copies directly from them for a fee, or look for copies at any university or medical library near you), were interesting and/or helpful in learning more about craniosynostosis. Some of them reflect my own experiences with post-surgical problems such as exotropia, a damaged temporalis muscle, and temporal hallowing.


Cunningham ML, Seto ML, Ratisoontorn C, et al. Syndromic craniosynostosis: From history to hydrogen bonds. Orthod Craniofac Res 2007;10(2):67-81.
The syndromic craniosynostoses, usually involving multiple sutures, are hereditary forms of craniosynostosis associated with extracranial phenotypes such as limb, cardiac, CNS and tracheal malformations. The genetic etiology of syndromic craniosynostosis in humans is only partially understood. Syndromic synostosis has been found to be associated with mutations of the fibroblast growth factor receptor family (FGFR1, -R2, -R3), TWIST1, MSX2, and EFNB1. Apert, Pfeiffer, Crouzon, and Jackson-Weiss syndromes are due to gain-of-function mutations of FGFR2 in either the Ig II-III linker region (Apert) or Ig III domain. Loss of function mutations of TWIST1 and gain-of-function mutations of MSX2 lead to Saethre-Chotzen and Boston-type syndromes, respectively. The mutations in Pfeiffer (FGFR1), Muenke (FGFR3), and Apert syndrome (FGFR2) are caused by the same amino acid substitution in a highly conserved region of the Ig II-III linker region of these proteins, which suggests that these receptor tyrosine kinases have an overlapping function in suture biology. In this review we will discuss the historical descriptions, current phenotypes and molecular causes of the more common forms of syndromic craniosynostosis.

Church MW, Parent-Jenkins L, Rozzelle AA, et al. Auditory brainstem response abnormalities and hearing loss in children with craniosynostosis. Pediatrics 2007;119(6):e1351-60.
OBJECTIVES: Craniosynostosis is a devastating disorder characterized by premature closure of the cranial plates before or shortly after birth. This results in an abnormally shaped skull, face, and brain. Little is known about hearing disorders in such patients, and nothing has been published about their auditory brainstem responses. Our objective was to evaluate such patients for auditory brainstem response and hearing disorders with the long-term goal of improving patient evaluation and management. PATIENTS AND METHODS: We evaluated the auditory brainstem responses, hearing, and brain images of children with fibroblast growth factor receptor 2 craniosynostosis (n = 11). RESULTS: Prolongation of the auditory brainstem response I-to-III interpeak latency was a frequent characteristic of fibroblast growth factor receptor 2 craniosynostosis, occurring in 91% of our patients. Prolongation of the III-to-V interpeak latency was an occasional characteristic, occurring in 27% of our patients. Whenever the I-to-III interpeak latency was prolonged, wave II was always abnormal. Associated morbidities included sensorineural hearing loss (27%), recurrent otitis media (100%), and Arnold-Chiari malformation (27%). Cranial decompression improved the interpeak latencies of 2 children. CONCLUSIONS: These previously undocumented auditory brainstem response abnormalities reflect abnormal neural transmission, which could cause peripheral and central auditory processing disorders. We speculate that the major pathogenic basis of the I-to-III interpeak latency and wave II abnormalities is compression of the auditory nerve as it passes through the internal auditory meatus and posterior fossa, which would explain the auditory nerve hearing loss, tinnitus, and vertigo that affect these children. Awareness of these abnormalities could lead to important advancements in the auditory and neurosurgical assessment and management of this overlooked patient group. We provide recommendations for the improved assessment and management of these patients. In particular, we recommend that auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression.

Morawiec HZ, Lekston ZH, Kobus KF, et al. Superelastic NiTi springs for corrective skull operations in children with craniosynostosis. J Mater Sci Mater Med 2007 May 5.
This paper concerns the formation and characterization of superelastic springs and rings of NiTi alloys for long-term skull correction. Superelastic properties of the rings were induced in the process of ageing of the already formed rings which cause hardening of parent phase by the precipitation of coherent Ni(4)Ti(3) particles. The efficacy of the worked out springs and rings were successfully proved in several clinical applications.

Clayman MA, Murad GJ, Steele MH, et al. History of craniosynostosis surgery and the evolution of minimally invasive endoscopic techniques: The University of Florida experience. Ann Plast Surg 2007;58(3):285-7.
Craniosynostosis is the premature and abnormal fusion of 1 of the 6 suture lines that form the living skull and can occur as part of a syndrome or as an isolated defect (nonsyndromic). The first reported surgical procedure for correction of craniosynostosis was performed in 1890 by Lannelongue who advocated releasing, but not resecting, the fused suture. Craniofacial surgery has developed its own identity in the last 3 decades, with the Frenchman Tessier seen as the founding father. There have been many new developments such as distraction osteogenesis, biodegradable miniplate fixation, and the development of minimally invasive endoscopic techniques. Through the pioneering work of Jimenez and Barone, minimally invasive approaches to the surgical correction of craniosynostosis are now gaining wider acceptance. Here the authors review the history of craniosynostosis, the current literature and technique for endoscopic repair of craniosynostosis, as well as their own experience at the University of Florida, with minimally invasive endoscope-assisted techniques.

Speltz ML, Kapp-Simon K, Collett B, et al. Neurodevelopment of infants with single-suture craniosynostosis: Presurgery comparisons with case-matched controls. Plast Reconstr Surg 2007;119(6):1874-81.
BACKGROUND: The hypothesized association between single-suture craniosynostosis and neurodevelopment remains unclear, given the methodologic limitations of previous studies, most notably the absence of control groups. METHODS: Standardized measures were used to assess the neurodevelopment of 125 matched case-control pairs shortly after cases were first diagnosed with isolated fusions of the sagittal, metopic, lambdoid, or right or left coronal sutures. Participants varied in age from 2 to 24 months. RESULTS: Cases had significantly lower mean standardized scores than controls on measures of cognitive ability and motor functioning (p < 0.02). These differences were unaffected by the location of synostosis, age of diagnosis, infant sex, and maternal IQ. Measures of early language functions revealed no group differences. CONCLUSIONS: Before cranioplasty, single-suture craniosynostosis is associated with modest but reliable neurodevelopmental delays that cannot be attributed to maternal intelligence and family sociodemographic variables. Follow-up of this sample will determine the predictive significance of these delays. In the meantime, routine neurodevelopmental screening of infants with isolated craniosynostosis is recommended.

Smyth MD, Tenenbaum MJ, Kaufman CB, Kane AA. The “clamshell” craniotomy technique in treating sagittal craniosynostosis in older children. J Neurosurg 2006;105(4 Suppl):245-51.
OBJECT: Although most patients with sagittal craniosynostosis are recognized and treated in infancy, some children are not referred to craniofacial centers until later in childhood. In this paper the authors describe a novel operative technique for calvarial reconstruction in older children with previously untreated sagittal craniosynostosis. METHODS: The authors report a clinical series of eight patients who were treated using novel single-stage calvarial reconstruction, and they assess the complications and outcomes. The patient is placed supine for the procedure, which consists of a coronal incision, bifrontal craniotomy without orbital osteotomy, and multiple interlocking midline parietooccipital osteotomies and recontouring. Fixation is achieved using a bioabsorbable plate system. Cranial indices were calculated from measurements obtained before and after the reconstructive procedures. Preoperative, intraoperative, and postoperative photographs and three-dimensional computed tomography scans are presented for review. Between November 2003 and April 2005, the authors treated seven boys (age range approximately 1-10 years, mean age 4.2 years) with uncorrected sagittal craniosynostosis and one with bicoronal and sagittal synostosis. The mean operating time was 5.13 hours (range 4.3-8 hours), with a mean blood loss of 425 ml (range 200-800 ml). As a percentage of the estimated circulating blood volume, the mean operative blood loss was 33.5% (range 17-57%). The mean hospital stay was 4.9 days. The cranial index significantly improved from a mean of 65.6 to 71.3% (p = 0.001). No acute or delayed complications have been noted. Follow-up examinations performed at an average of 12 months (range 1-17 months) have confirmed early patient and family satisfaction. CONCLUSIONS: An approach of aggressive calvarial reconstruction with multiple interleaving osteotomies crossing the midline achieves improvements in biparietal narrowing. Combined with a bifrontal reconstruction, early outcomes are excellent, with an acceptable amount of intraoperative blood loss and no significant complications.

Agrawal D, Steinbok P, Cochrane DD. Long-term anthropometric outcomes following surgery for isolated sagittal craniosynostosis. J Neurosurg 2006;105(5 Suppl):357-60.
OBJECT: A number of studies have shown good short-term cosmetic outcomes following surgery for isolated sagittal craniosynostosis. Whether the improvement in head shape persists in the longer- term is less clear. The aim of this study was to investigate the long-term anthropometric outcomes following surgery for isolated sagittal craniosynostosis. METHODS: Records were retrospectively reviewed for children with isolated sagittal synostosis who underwent surgical revision between 1987 and 2000. Only children who underwent surgery before 8 months of age and for whom serial anthropometric data (skull width, skull length, and cephalic index) were available were included in the study. The operative procedure consisted of vertex and parietal craniectomies involving removal of the sagittal suture and a 1.5- to 2.5-cm piece of adjacent parietal bone on each side as well as bilateral parietal barrel-stave osteotomies. Ninety cases satisfied the eligibility criteria. The mean age of the patients at surgery was 5 months (range 1.9-7.5 months). The mean preoperative cephalic index was 66.78. The follow-up period ranged from 1.8 to 167 months (mean 39.6 months). In 24 cases, the follow-up period was longer than 36 months. Eighteen (75%) and five (20.8%) of these 24 cases were followed up for longer than 5 and 10 years, respectively. The mean increase in cephalic index at the last follow up was 8.69% (p < 0.0001). The maximum improvement in the cephalic index occurred within 6 months of surgery, at which point it had improved by a mean of 11.1% (p < 0.0001). The cephalic index remained increased throughout the follow-up period, with a mean change of -1.84% (standard deviation, 4.28%; 99% confidence interval -3.33 to -0.37%) from the first postoperative to the final measurement. CONCLUSIONS: Surgery for isolated sagittal craniosynostosis leads to a significant improvement in the cephalic index, which is most marked in the early postoperative period. Improvement in the cephalic index is still present after prolonged follow up.


page 2

page 3