Stelnicki EJ. Endoscopic treatment of craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am 2002;10:57-72.
We are entering a new era of craniosynostosis repair. When detected early, endoscopic skull remodeling, combined with a postoperative external skull-molding device, gives an excellent long-standing reconstruction of the cranial skeleton. This technique diminishes the morbidity of the operation and decreases the overall cost. It does not replace classic plate and screw cranial vault reconstruction in the older patient but is a useful weapon in the armamentarium of the craniofacial surgeon for the treatment of craniosynostosis in the neonatal period.


Flores-Sarnat L. New insights into craniosynostosis. Semin Pediatr Neurol 2002;9:274-91.
Craniosynostosis is a congenital developmental disorder involving premature fusion of cranial sutures, often associated with multiple neurological manifestations. The perspective of this group of disorders has changed dramatically in the new era of molecular genetics. In the last decade a large literature with new concepts in craniosynostosis has appeared. More than 100 syndromes associated with craniosynostosis have been described, and in about a dozen, the molecular defect has been identified. Pediatric neurologists are less aware than geneticists, neurosurgeons, and craniofacial surgeons of these changes. General concepts about craniosynostosis are here presented with updates of clinical and genetic aspects of well-defined syndromes such as Apert, Crouzon, Pfeiffer, Saethre-Chotzen. Evidence of their relationship with fibroblast growth factor receptors (FGFRs) 1, 2, and 3, and with causative genes such as TWIST has been documented. New and other less common syndromes also are discussed. The differences between positional and synostotic plagiocephaly are important, as well as the cause of nonsyndromic craniosynostosis. The prognosis and neurological outcome of patients, including “benign” forms of craniosynostosis, are other important aspects. Major advances have occurred in understanding pathogenesis, diagnosis, and treatment of craniosynostosis. The role of local dura mater and apoptosis; modalities of imaging such as prenatal ultrasound and three-dimensional and spiral CT have improved the accuracy in diagnosis, and the new approaches in surgical treatment involving efficient and less invasive methods, are evidence of these advances.


Levi D, Rampa F, Barbieri C, et al. True 3D reconstruction for planning of surgery on malformed skulls. Childs Nerv Syst 2002;18:705-6.
OBJECTIVES. Since the difficulty associated with surgical planning of craniosynostosis is mostly due to the inadequate possibilities for simulation of surgery, a new technique for constructing a precise reproduction of a patient’s malformed skull has been developed. MATERIALS AND METHODS. CT scans of ten malformed skulls on a scale of 1:1 and with 1-mm slices have been used to reconstruct the skulls in a special resin using a special lathe used in the automobile construction industry for formula one engines. This lathe works in the opposite way to other lathes: by apposition of material instead of subtraction. RESULTS. The anatomical detail of the phantom is of a very high degree. The surgical planning it allows has proved highly consistent with reality in all cases in which it has been used in the planning before the operation. DISCUSSION AND CONCLUSIONS. This technique has made it possible to plan the surgical treatment of all patients with craniosynostosis in a highly satisfactory way. It has reduced operating times, in addition to which it provides information on materials needed, avoiding waste, and is also an excellent teaching method for residents.


Tantipasawasin S, Sinn DP, Ghali GE. Management of syndromic craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am 2002;10:101-30.
Normalization of craniofacial malformation in syndromic craniosynostosis patients is an extended process. The care starts at birth and ends in adolescence. Multiple surgeries may be required for each deformity. The relationship among patient, family, and doctor team is important because they must understand the complete processes of management. Psychologists provide an important support mechanism.


Ghali GE, Sinn DP, Tantipasawasin S. Management of nonsyndromic craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am 2002;10:1-41.
In approximately 1 in 1000 live births in the United States, an infant has some variant of a facial, skeletal, or craniofacial deformity. If cleft lip and palate deformities are included, the incidence is greater. Timing of the surgical management of these patients has been advocated from the first few weeks after birth until well into the second decade. Many of these patients require multiple, staged procedures that involve movements of the bone and soft tissue from the intracranial and extracranial approaches. The surgical approach to most of these congenital deformities was radically changed by techniques introduced to the United States by Paul Tessier of France in 1967. From his imaginative intracranial and extracranial approaches, numerous advances have been made that facilitate the care of most of these children. More recently, additional advances in pediatric anesthesia and biodegradable plating systems have improved the management of these complex craniomaxillofacial deformities.


Imai K, Komune H, Toda C, et al. Cranial remodeling to treat craniosynostosis by gradual distraction using a new device. J Neurosurg 2002;96:654-9.
OBJECT: The authors describe their experience in remodeling the shape of the cranium in children with craniosynostosis by using gradual distraction. In half of the cases, a new distraction device developed by the authors was used. METHODS: Twenty children with craniosynostosis underwent frontoorbital advancement and cranial remodeling assisted by gradual distraction. There were five children with brachycephaly, two with oxycephaly, five with scaphocephaly, two with plagiocephaly, one with combined scaphocephaly and plagiocephaly, and five with trigonocephaly. Four cases were syndromic and 16 were nonsyndromic. The patients ranged in age from 3 to 50 months (mean 14.5 months) at the first surgery. Simulated surgery was first performed on a three-dimensional solid model made of polyurethane, which accurately represented cranial flexibility, to determine the most favorable osteotomy line. Distraction was initiated 1 week postoperatively. The speed and extent of advancement (maximum extension 45 mm) were predetermined on the basis of previously reported criteria and the results of simulated surgery. Postoperatively, the cranial configuration was favorable in all cases. Spontaneous remodeling of irregularities and/or gaps apparent after distraction was found to occur 2 to 5 months after removal of the distraction devices, especially in patients with trigonocephaly or scaphocephaly. No major perioperative complication was observed in any patient. There were minor complications in six of the first 10 cases, including exposure of the device, shaft slippage, and fluid discharge. A new device was developed and used on the last 10 patients treated; it successfully eliminated device exposure and shaft slippage. A 3-year follow-up review confirmed that there was no relapse of advanced bones. CONCLUSIONS: Highly satisfactory results were achieved in cases of both syndromic and nonsyndromic craniosynostosis when gradual distraction was performed.


Aviv RI, Rodger E, Hall CM. Craniosynostosis. Clin Radiol 2002;57:93-102.
Craniosynostosis, caused by premature fusion of the sutures, may be syndromic or non-syndromic. Radiology has an important role to play in the assessment, management and follow-up of these patients. Initial investigations may often be undertaken within general radiology departments. An understanding of the terminology and recognition of the predictable patterns of presentation are essential to enable appropriate referral to a craniofacial unit where further investigation and management are undertaken. We present the radiological features of several craniosynostoses with an emphasis on the role of plain radiography. We demonstrate the complementary role of computed tomography in their evaluation within the setting of a specialist centre.


Dhellemmes P, Pellerin P, Vinchon M, Capon N. [Surgery for craniosynostosis: timing and technique.][Article in French.] Ann Fr Anesth Reanim 2002;21:103-10.
Craniosynostoses are a group of diseases, the presentation of which differs markedly on account of the cranial suture involved. Their impact is cosmetic, cerebral, and ophthalmologic. Syndromic craniosynostoses associate a more or less pronounced faciostenosis, which requires surgical correction as well, because of cosmetic, ophthalmologic or airway problems. Surgical treatment depends on the type of the craniosynostosis and the patient’s age; ideally, the child should be operated between 3 and 12 months. This surgery requires a perfect collaboration between neurosurgeon, plastic surgeon, and anaesthesiologist. Surgical correction allows in large measures the preservation of intellect, sight, and body image.


Magge SN, Westerveld M, Pruzinsky T, Persing JA. Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniofac Surg 2002;13:99-104.
The link between cranial deformity and functional disability is not obvious in single-suture sagittal craniosynostosis. Physicians have anecdotally reported that children with simple craniosynostosis often seem to have a higher proportion of learning disabilities and cognitive problems than their nonafflicted peers. These problems have not been systematically studied, however. This study examined the long-term neuropsychological effects of single-suture sagittal craniosynostosis on selected aspects of neurological development. It did so by going beyond global measures of mental function (intelligence quotient) in an attempt to assess the incidence of subtle neuropsychological sequelae. Retrospective inspection of the Yale Department of Neurosurgery records between 1980 and 1990 was used to identify study subjects born with nonsyndromic sagittal suture craniosynostosis who were between 6 and 16 years of age at the time of the study. Of the 16 study subjects born with sagittal synostosis, which is thought to be among the most benign of the single-suture craniosynostoses, this study found that 50% had a reading and/or spelling learning disability. Although children with single-suture sagittal craniosynostosis fall within the normal range for intelligence, there is a significantly higher incidence of learning disabilities in this group than in the general population.


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.
OBJECTIVE: To assess the safety, efficacy, and results of the early treatment of infants with craniosynostosis using minimally invasive endoscopic strip craniectomies and postoperative helmet molding therapy. METHODS: A total of 100 patients with documented diagnosis of craniosynostosis were prospectively studied and treated with endoscopic strip craniectomies. A total of 106 stenosed sutures were operated on with the following distribution: 61 sagittal, 23 coronal, 18 metopic, and 4 lambdoid sutures. Sixty-three patients were treated under 16 weeks of age. After surgery, all patients were treated with custom-made molding helmets for up to 7 months. Follow-up ranged between 4 months and 50 months. RESULTS: All patients underwent the surgical procedures successfully and without complications. The mean surgical operative time was 52.7 minutes. The mean estimated blood loss was 26.2 mL; only 1 patient underwent intraoperative blood transfusion, and 10 patients had a non- life-threatening postoperative blood transfusion. All but 3 patients were discharged on the first postoperative day. There were no infections, dural sinus tears, cerebrospinal fluid leaks, or neurologic injuries, and there were no significant complications related to the use of helmet therapy. Most patients have achieved or are in the process of reaching normalization of their craniofacial deformities. CONCLUSIONS: The results indicate that the early treatment of craniosynostosis with minimally invasive endoscopic strip craniectomies is a safe, efficacious, and valuable therapeutic alternative to the current extensive surgical treatment modalities. The significantly less blood loss, need for blood transfusions, and length of stay and decreased costs make this procedure an excellent early option for treating infants who present with craniosynostosis.


Zeiger JS, Beaty TH, Hetmanski JB, et al. Genetic and environmental risk factors for sagittal craniosynostosis. J Craniofac Surg 2002;13:602-6.
The authors investigated whether genetic and environmental factors influence risk for sagittal craniosynostosis. Cases were ascertained from craniofacial clinics in the Baltimore-Washington metropolitan region. Controls were recruited from the Johns Hopkins newborn nursery and a large pediatric practice in Baltimore County. Forty-two probands with isolated, nonsyndromic sagittal craniosynostosis born in the mid-Atlantic region were included in this analysis. Controls are infants born in Maryland without any known birth defects (n = 182). Odds ratios (OR) and corresponding 95% confidence intervals (CI) were calculated. Cases were genotyped at several loci implicated in malformation syndromes including craniosynostosis. There were no elevated risks for craniosynostosis related to maternal or paternal smoking or maternal vitamin usage. Case mothers consumed less alcohol (OR = 0.38, 95% CI = 0.17-0.85) and had less education than control mothers ( < 0.001). All cases that were sequenced were negative for mutations at the following genes: exon IIIa 755C->G, (exons IIIa and IIIc,), exon IIIa, and exon 1. These findings suggest that whereas TWIST and the genes are important for syndromic craniosynostosis, they are unlikely to be involved in isolated sagittal craniosynostosis. Parental education and alcohol consumption were associated with sagittal craniosynostosis in this study.


Weaver CE, Duncan JA. Management of pediatric head shape abnormalities: Craniosynostosis and positional posterior plagiocephaly. Med Health R I 2001;84:395-8. No abstract available.


Huber Z. [Surgery of multi-sutured craniosynostosis in childhood][Article in Polish]. Neurol Neurochir Pol 2001;35:633-47.
According to own critical experience with the treatment of craniosynostoses (1954-81), using various methods from partial morcellement to total ablation of the cranial vault, the author applied (1982-2000) compound surgical decompression in 40 cases of turricephalic craniostenosis due to multisutural craniosynostosis. The surgical approach to the problem was supported by pre- and postoperative observations of computed three-dimensional bone reconstruction, furthermore by investigation of alterations of SPECT detecting the critical areas of most pronounced intracranial tightness of the skull leading to localized and general cerebral blood flow impairment of the developing brain. The application of newer diagnostic methods contributed to deeper understanding of mechanisms concerning neurodevelopmental retardation and behaviour or defect, disturbances as epilepsy, cranial nerve palsy, spastic paresis, hypothalamic-pituitary insufficiency or poor intelectual performance. This made the possible treatment of preoperative clinical symptoms more efficient. By an early operation we solve the disproportion between the too small skull volume limited by premature ossification of sutures which resulted previously in compression of the growing in size, of the maturing brain. In cases of more complex pathology orbitotomies, with a reconstructive advancement of the orbital rim and maxillas should be performed with multidisciplinary cooperation.


Warren SM, Longaker MT. The pathogenesis of craniosynostosis in the fetus. Yonsei Med J 2001;42:646-59.
Craniosynostosis occurs in approximately 1:2000 live births. It may affect the coronal, sagittal, metopic and lambdoid sutures in isolation or in combination. Although non-syndromic synostoses are more common, over 150 genetic syndromes have been identified. Recent advances in genetic mapping have linked chromosomal mutations with craniosynostotic syndromes. Despite the identification of these genetic mutations, the fundamental biomolecular mechanisms mediating cranial suture biology remain unknown. Today, many laboratories are investigating murine cranial suture biology as a model for human cranial suture development and fusion. Normal murine cranial suture biology is very complex, but evidence suggests that the dura mater provides the biomolecular blueprints (e.g. the soluble growth factors), which guide the fate of the pleuripotent osteogenic fronts. While our knowledge of these dura-derived signals has increased dramatically in the last decade, we have barely begun to understand the fundamental mechanisms that mediate cranial suture fusion or patency. Interestingly, recent advances in both premature human and programmed murine suture fusion have revealed unexpected results, and have generated more questions than answers.


Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Childs Nerv Syst 2000;16(10-11):645-58.
Although it is currently thought that surgery is indicated mainly for cosmetic reasons in isolated craniosynostoses, the functional aspects of the treatment must not be underestimated. Prospective studies on intracranial pressure and mental evolution of these children have shown that there were functional consequences in a significant proportion of cases even of single suture fusion. The frequency of increased intracranial hypertension and the risk of mental impairment depend on the age of the child and the type of craniosynostosis. In nonsyndromic cases, the higher risks are observed in multisutural craniosynostoses (brachycephaly, oxycephaly). In syndromic cases, the risk of intracranial hypertension is higher in Crouzon syndrome, and Apert syndrome carries the higher risk of mental retardation. The study of a personal series of 2,137 craniosynostoses shows that the functional and the cosmetic results are better after early surgery, and that the operative risks are not higher in infants than in older children.

 


Dahmani S, Orliaguet GA, Meyer PG, et al. Perioperative blood salvage during surgical correction of craniosynostosis in infants. Br J Anaesth 2000;85(4):550-5.
Surgical correction of craniosynostosis in infants is a very haemorrhagic procedure. The aim of this study was to determine whether the perioperative use of the continuous autotransfusion system (CATS) would reduce homologous transfusion during repair of craniosynostosis. Two groups of patients were studied according to the availability of the CATS in our hospital. The control group had surgery before the system was introduced and the study group had operations subsequently. Use of CATS was associated with a significant decrease in the median (95% confidence interval) volume of homologous blood transfused [413 (250-540) ml in the control group versus 317 (150-410) ml in the CATS group, P = 0.02] and in the median (95% confidence interval) number of packed red cell units transfused [2 (1-2) in the control group versus 1 (1-2) in the CATS group, P = 0.04] in the perioperative period. Use of CATS is associated with a reduction in homologous transfusion during the surgical correction of craniosynostosis in infants.
Honein MA, Rasmussen SA. Further evidence for an association between maternal smoking and craniosynostosis. Teratology 2000;62(3):145-6. No abstract available.


Barone CM, Jimenez DF. Endoscopic craniectomy for early correction of craniosynostosis. Plast Reconstr Surg 1999;104(7):1965-73; discussion 1974-5.
Twelve patients between 0.4 and 7.8 months of age were treated by an endoscopic approach to strip craniectomy. Nine patients had sagittal suture involvement. Two patients had a single unilateral lambdoid suture synostosis, and one patient had a combination of a right coronal synostosis and a metopic synostosis. Postoperatively, all patients were placed in cranial remodeling helmets and the results showed that the estimated blood loss ranged from 5 cc to 150 cc, with blood transfusion required in only one patient. All patients were discharged from the hospital by day 2, and all patients had an improvement in their cranial head shape. The specific technique of using the endoscope to aid in performing a strip craniectomy will be discussed. Nine endoscopically treated patients with the diagnosis of sagittal suture synostosis were compared with nine patients treated by using the Marchac remodeling techniques. The mean operative time (1.6 hours versus 3.5 hours), estimated blood loss (43 cc versus 168 cc), hospital costs ($11,671 versus $36,685), and length of stay (1.16 days versus 5.1 days) were less by using the endoscopic technique. All nine patients treated by using the Marchac technique required a blood transfusion, whereas only one patient was transfused in the endoscopically treated group.


Lajeunie E, Catala M, Renier D. Craniosynostosis: From a clinical description to an understanding of bone formation of the skull. Childs Nerv Syst 1999;15(11-12):676-80.
The genetic studies of syndromic craniosynostoses lead to the characterisation of genes that regulate the correct development of the bones of the skull. From these studies, it appears that FGF/FGFR signalling has a crucial role in this problem. Numerous mutations affecting the genes coding for FGFR1, 2 or 3 are responsible for these syndromes. It is interesting to note that some identical mutations produced various different phenotypes, suggesting that other genes modulate the phenotypic expressivity. The other involved genes in these syndromes code for such proteins as Msx2 or Twist that interact in the cellular pathways responsible for FGF action. From these genetic studies, it is now important to establish the role of these proteins during the development of the skull. Msx2 plays a repressive role in osteogenesis, whereas FGFRs act as promoting proteins. In the near future, it will be very important to improve our understanding of these phenomena in order to test specific treatments to prevent the development of such syndromes.


Alden TD, Lin KY, Jane JA. Mechanisms of premature closure of cranial sutures. Childs Nerv Syst 1999;15(11-12):670-5.
Craniosynostosis is defined as premature closure of the sutures of the skull, resulting in cranial deformity. Since Virchow’s original paper describing the relationship between premature suture closure and skull morphology, we have learned much about the underlying mechanisms and consequences of premature suture closure. In this paper we will describe the biology of suture closure, the rules governing the resulting phenotypes seen clinically, and a prospective study of skull growth during the 1st year of life.


Williams JK, Ellenbogen RG, Gruss JS. State of the art in craniofacial surgery: Nonsyndromic craniosynostosis. Cleft Palate Craniofac J 1999;36(6):471-85.
Craniosynostosis refers to the premature fusion of one of the six major sutures of the cranial vault. Functionally, craniosynostosis may be defined as the premature conversion of the dynamic region of growth and resorption between two adjacent bones of the cranium into a static region of bony union. Molecular analysis has blurred the traditional categories of nonsyndromic and syndromic synostosis to some extent, but, in general, the distinctions between the two groups still hold true. The complexity of the congenital anomalies may be limited with the former, whereas the latter usually requires reoperations and correction of the facial skeleton. This article briefly outlines the characteristic deformities produced from nonsyndromic craniosynostosis. Various approaches to surgical correction of the deformities are described. Finally, new biomaterials that are used in the correction of nonsyndromic craniosynostosis are reviewed.


Hehr U, Muenke M. Craniosynostosis syndromes: From genes to premature fusion of skull bones. Mol Genet Metab 1999;68(2):139-51. Review. No abstract available.


Sgouros S, Hockley AD, Goldin JH, et al. Intracranial volume change in craniosynostosis. J Neurosurg 1999;91(4):617-25.
OBJECT: There is still controversy regarding the optimum time to perform surgery for craniosynostosis. Some recommend surgery soon after birth and others delay until the age of 12 months. Intracranial pressure has been measured in an attempt to provide a scientific rationale, but many questions remain unanswered. To date, little attention has been given to intracranial volume and its changes during the first few years of life in children with craniosynostosis. The authors’ goal was to focus on intracranial volume during this period and to compare measurements obtained in patients with craniosynostosis with measurements obtained in healthy individuals. METHODS: Using the technique of segmentation, the intracranial volume of 84 children with various forms of craniosynostosis was measured on preoperative computerized tomography scans. The change in average volume that occurs with increasing age was calculated and compared with a model of normal intracranial volume growth. The age at presentation for children with craniosynostosis was 1 to 39 months; 76% of the patients were younger than 12 months. In eight patients in whom only one cranial expansion procedure was performed, postoperative intracranial volumes were measured as well. Several interesting observations emerged. 1) There was little difference in head growth between boys and girls with craniosynostosis during the first few months of life. After the age of 12 months, however, the difference in intracranial volume normally seen between the two genders was observed in the craniosynostosis group as well. 2) Excluding children with complex pansynostosis, who have smaller heads, children with all other types of craniosynostosis have similar head growth after the 1st year of life, with no difference between the number of and type of suture affected. Children with Apert’s syndrome develop greater than normal intracranial volumes after the 1st year of life. 3) Although children with craniosynostosis are born with a smaller intracranial volume, by the age of 6 months volume has reached normal levels, and from that point on volume follows the pattern of normal head growth. 4) Children who presented after the age of 6 months and later developed recurrent craniosynostosis after initial successful treatment had a small intracranial volume at their initial presentation. 5) Of the patients whose postoperative intracranial volumes were measured, all but one had preoperative volumes at or above normal values, and their postoperative volumes were considerably higher than normal for their age. These children all followed a growth curve parallel to that of healthy children but at higher volume value. One patient with a smaller-than-normal initial intracranial volume was surgically treated at a very young age and, despite cranial expansion surgery, postoperative volume did not reach normal levels. It is postulated that this was due to the fact that the operation was performed at a time when craniosynostosis was still active. CONCLUSIONS: The results of this study indicate that the underlying mechanism leading to craniosynostosis and constriction of head volume “exhausts” its effect during the first few months of life. Measurement of intracranial volume in clinical practice could be used to “fine tune” the optimum time for surgery. In late-presenting children, this may be useful in predicting possible recurrence.


Kapp-Simon KA. Mental development and learning disorders in children with single suture craniosynostosis. Cleft Palate Craniofac J 1998;35(3):197-203.
OBJECTIVE: This study examined the global intellectual development and presence/absence of learning disorders in children with nonsyndromic metopic, sagittal, and unilateral coronal synostosis who had early surgery (under 1 year of age), late surgery (over 1 year of age), or no surgery to correct the synostosis across three time periods. DESIGN: The design was longitudinal. Children were assessed at initial team evaluation, 1 year after surgery or initial evaluation if surgery was not performed, and annually thereafter. PARTICIPANTS: Participants included 84 consecutively evaluated patients. Seventy-two children were evaluated at Time 1 (T1), 8 months; 57 at Time 2 (T2), 21 months; and 39 at Time 3 (T3), 50 months. Data on learning disorders or mental retardation were available on 34 children who were school age. MAIN OUTCOME MEASURES: The Mental Development Index from the Bayley Scales of Infant Development was the main outcome measure for T1 and T2. The General Cognitive Index from the McCarthy Scales of Children’s Abilities was the main outcome measure for T3. Learning disorders were determined from school and clinical records. RESULTS: Repeated measures analyses of variance found no statistical differences in the development of children based on diagnosis or surgical status across time. Base rate of mental retardation at T1 was consistent with normative data; however, the incidence of retardation was two to three times the expected rate at T2 and T3. Learning disorders were present in 47% of school-age children who were not retarded. Chi-square analysis showed no significant differences between rates of retardation or learning disorders based on surgical status. CONCLUSIONS: Most children with nonsyndromic craniosynostosis obtain developmental quotients within the normal range in infancy. Rates of retardation may increase relative to normative expectations as children mature. A high rate of learning disorders was identified. Results are preliminary due to sample size.


Mouradian WE. Controversies in the diagnosis and management of craniosynostosis: A panel discussion. Cleft Palate Craniofac J 1998;35(3):190-3.
This introductory article summarizes and comments on a group of four papers based in part on a panel discussion of craniosynostosis (CS) held at the 53rd annual meeting of the American Cleft Palate-Craniofacial Association. The purpose of this panel was to review the differential diagnosis of CS and the evidence for increased intracranial pressure (ICP) and developmental problems in CS patients. First, a correct diagnosis must be made, with true synostosis being differentiated from positional deformities and other normal variants. Second, medical indications must be balanced against the risks of operating on CS patients. Release of fused sutures is commonly undertaken to avoid increased ICP, although studies of ICP in infants and children with CS are hampered by a lack of normative data and by difficulties with measurement techniques. A subgroup of children with isolated craniosynostosis may have increased ICP, which can be deleterious to brain function. Longitudinal studies of children with isolated CS suggest an increased risk of mental retardation and learning disorders; surgical release of the suture may not diminish this risk. Patients with metopic suture fusion appear to be particularly at risk. These findings must be confirmed with a larger sample size. These uncertainties raise ethical issues and complicate medical decision-making for the infant with CS. A trusting and truthful relationship between the parents and the professional is necessary for a balanced discussion of the best interests of the child. All patients with confirmed synostosis should be followed for evidence of progressive deformity, intracranial hypertension, and neurodevelopmental problems.


Jimenez DF, Barone CM. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg 1998;88(1):77-81.
OBJECT: The authors sought to minimize scalp incisions, blood loss, and operative time by using endoscopically assisted strip craniectomies and barrel-stave osteotomies to treat infants with sagittal suture synostosis. METHODS: Four patients, aged 2, 4, 9, and 12 weeks, who presented with scaphocephaly underwent endoscopic midline craniectomies through small midline scalp incisions. The mean operative time for the procedure was 1.68 hours (range 1.15-2.8 hours); the mean blood loss was 54.2 ml (range 12-150 ml). Three patients did not require blood transfusions and were discharged within 24 hours. Postoperatively, all patients were fitted with custom cranial molding helmets. Follow-up evaluation ranged between 8 and 15 months. All patients had successful correction of their scaphocephaly with no mortalities, morbidities, or complications. CONCLUSIONS: The use of endoscopic techniques for early correction of sagittal synostosis is safe; decreases blood loss, operative time, and hospitalization costs; and provides excellent early surgical results.


Speltz ML, Endriga MC, Mouradian WE. Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis. Cleft Palate Craniofac J 1997;34(5):374-9.
OBJECTIVE: The current study compared the mental and psychomotor development of infants with nonsyndromic sagittal synostosis (SS) with a demographically matched comparison group without congenital defects. Within the SS group, we tested the hypothesis that age of cranial release would be inversely correlated with mental development. DESIGN: The design was prospective and longitudinal. Participants were assessed at 4, 12, and 24 months of age. SETTING: The study was conducted in a craniofacial clinic at an urban children’s hospital. PARTICIPANTS: Participants were 19 infants with SS (consecutive craniofacial program referrals) and 19 demographically matched comparison infants recruited from the community. One infant with SS did not attend the 24-month assessment. MAIN OUTCOME MEASURES: Mental and Psychomotor Indices from the Bayley Scales of Infant Development were the primary outcome measures. Subdomains of development were created using Kohen-Raz scoring procedures. All measures were determined a priori. RESULTS: Repeated-measures MANOVAs revealed no statistically significant differences in the developmental trajectories of the two groups. None of the SS group infants received Mental Development Index (MDI) scores in the mentally retarded or borderline range of intellectual functioning (i.e., below 78). An inverse correlation (r = -.30) was found between the age at surgery and Bayley growth curve coefficients; however, this association was not statistically significant (p = .10, one-tailed). CONCLUSIONS: Results are consistent with previous studies of the mental and psychomotor development of infants with nonsyndromic craniosynostoses in relation to normative test data. The relation between surgery age and developmental outcome merits further study in a larger sample with a greater range of surgery ages.


Keating RF. Craniosynostosis: Diagnosis and management in the new millennium. Pediatr Ann 1997;26(1O):600-12. Review. No abstract available.


Williams JK, Cohen SR, Burstein FD, et al. A longitudinal, statistical study of reoperation rates in craniosynostosis. Plast Reconstr Surg 1997;100(2):305-10.
A prospective, statistical study of reoperation rates was done in the treatment of 167 consecutive children with nonsyndromic and syndromic craniosynostosis over a 6-year period at Scottish Rite Children’s Medical Center in Atlanta, Georgia. Mean length of follow-up was 2.8 years, with a range of 3 months to 6 years. Reoperation equal to or exceeding the magnitude of the original procedure occurred in 7 percent of cases. Multiple regression analysis revealed several factors associated with reoperation: Females and children with syndromic synostoses were more likely to require reoperation. Total reoperation rates for syndromic and nonsyndromic synostoses were 27.3 and 5.9 percent, respectively. Age at initial surgery, length of operation, and estimated blood loss did not predict a higher reoperation rate.


Sloan GM, Wells KC, Raffel C, McComb JG. Surgical treatment of craniosynostosis: Outcome analysis of 250 consecutive patients. Pediatrics 1997;100(1):E2.
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.


Cavadas PC. Reattachment of the temporalis muscle to methacrylate in secondary surgery for craniosynostosis. Plast Reconstr Surg 1996;98:573-4. No abstract.


Harrop CW, Avery BS, Marks SM, Putnam GD. Craniosynostosis in babies: Complications and management of 40 cases. Br J Oral Maxillofac Surg 1996;34(2):158-61.
Forty consecutive craniofacial cases in babies operated on in a district general hospital by a craniofacial team consisting of maxillofacial and neurosurgeons are reviewed with regard to diagnosis, surgical treatment, complications and outcome. Surgery achieved the release of craniosynostosis and the treatment protocol, and perioperative complications are discussed. Dural breaches occurred on four occasions with no postoperative sequelae. Blood transfusion was required in all cases with an average replacement of 36 percent estimated blood volume (EBV). No central nervous system complications occurred but in one case a brachial plexus haematoma resulted in a temporary neuropraxia to the shoulder. No major infections or deaths occurred in this series.


Posnick JC. Upper facial asymmetries resulting from unilateral coronal synostosis. Diagnosis and surgical reconstruction. Atlas Oral Maxillofac Surg Clin North Am 1996;4(1):53-66. Review. No abstract available.


Bradley JP, Levine JP, Blewett C, et al. Studies in cranial suture biology: In vitro cranial suture fusion. Cleft Palate Craniofac J 1996;33(2):150-6.
The biology underlying craniosynostosis remains unknown. Previous studies have shown that the underlying dura mater, not the suture itself, signals a suture to fuse. The purpose of this study was to develop an in vitro model for cranial-suture fusion that would still allow for suture-dura interaction, but without the influence of tensional forces transmitted from the cranial base. This was accomplished by demonstrating that the posterior frontal mouse cranial suture, known to be the only cranial suture that fuses in vivo, fuses when plated with its dura in an organ-culture system. In such an organ-culture system, the sutures are free from both the influence of dural forces transmitted from the cranial base and from hormonal influences only available in a perfused system. For the cranial-suture fusion in vitro model study, the sagittal sutures (controls that remain patent in vivo) and posterior frontal sutures (that fuse in vivo) with the underlying dura were excised from 24-day-old euthanized mice, cut into 5 x 4 x 2-mm specimens, and cultured in a chemically defined, serum-free media. One hundred sutures were harvested at the day of sacrifice, then every 2 days thereafter until 30 days in culture, stained with H & E, and analyzed. A subsequent cranial-suture without dura in vitro study was performed in a similar fashion to the first study, but only the calvariae with the posterior frontal or sagittal sutures (without the underlying dura) were cultured. Results from the cranial-suture fusion in vitro model study showed that all sagittal sutures placed in organ culture with the underlying dura remained patent. More importantly, the posterior frontal sutures with the underlying dura, which were plated-down as patent at 24 days of age, demonstrated fusion after various growth periods in organ culture. In vitro posterior frontal mouse-suture fusion occurred in an anterior-to-posterior direction but in a delayed fashion, 4 to 7 days later than in vivo posterior frontal mouse-suture fusion. In contrast, the subsequent cranial-suture without dura in vitro study showed patency of all sutures, including the posterior frontal suture. These data from in vitro experiments indicate that: (1) mouse calvariae, sutures, and the underlying dura survive and grow in organ-culture systems for 30 days; (2) the local dura, free from external influences transmitted from the cranial base and hormones from distant sites, influences the cells of its overlying suture to cause fusion; and (3) without dura influence, all in vitro cranial sutures remained patent. By first identifying the factors involved in dural-suture signaling and then regulating these factors and their receptors, the biologic basis of suture fusion and craniosynostosis may be unraveled and used in the future to manipulate pathologic (premature) suture fusion.


Sidoti EJ, Marsh JL, Marty-Grames L, Noetzel MJ. Long-term studies of metopic synostosis: Frequency of cognitive impairment and behavioral disturbances. Plast Reconstr Surg 1996;97(2):276-81.
Although the occurrence of cognitive impairment and behavioral disturbances in patients with metopic synostosis has been described, the incidence of this dysfunction has not been established. The records of 36 consecutive children with metopic synostosis followed at one craniofacial center from 1978 to 1993 were reviewed and parental questionnaires were completed to establish the frequency of mental retardation, learning disabilities, and behavioral problems associated with this synostosis. Documentation of syndromes, abnormal karyotype, and central nervous system anomalies also was done. The study group consisted of 27 males and 9 females. The average age at most recent follow-up was 7 years and 1 month (range 6 months to 22 years). Two patients had chromosomal abnormalities (9p syndrome and trisomy 21). On the basis of CT and MRI scans, intracranial anomalies were identified for only one patient having an absent corpus callosum. Thirty-two of the study patients had adequate information for longitudinal assessment. Twenty patients have normal development without apparent disability. Of these, those of school age are at appropriate grade level. Eight patients have mild to moderate learning disabilities or behavioral problems, including attention deficit/hyperactivity disorder and impaired language development. Four patients have significant mental impairment. Impaired cognitive development was not limited to children with abnormal karyotype or central nervous system anomaly. Cognitive and behavioral abnormalities occur in at least a third of patients with metopic synostosis. The, at times, subtle nature of these abnormalities mandates longitudinal developmental and neurologic evaluation for infants with metopic synostosis.



McCarthy JG, Glasberg SB, Cutting CB, et al. Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis--results and unsolved problems. Plast Reconstr Surg 1995;96(2):272-83.
Early surgery for isolated craniosynostosis is designed to improve morphology, to prevent functional disturbances, and equally important, to enhance the psychosocial development of the child. As the first of a two-part series, 104 patients with isolated craniofacial synostosis were retrospectively analyzed. Diagnoses included bilateral coronal (10), unilateral coronal (57), metopic (29), and sagittal synostosis (8). All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 8.1 months). Thirteen percent of patients (14) required a secondary cranial vault operation (mean age 22.6 months) to address residual deficits in craniofacial form. Perioperative complications were minimal (5.0 percent), and there was no mortality. Average length of postoperative follow-up was 46.0 months. By the classification of Whitaker et al., which assesses surgical results, 87.5 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. Overall rates of hydrocephalus, shunt placement, and seizures (3.8, 1.0, and 2.9 percent, respectively) were low. Among the isolated craniosynostoses, unilateral coronal synostosis/plagiocephaly poses the most complex problems, including vertical orbital dystopia, nasal tip deviation, and residual craniofacial asymmetry; there is also a wide spectrum of findings and growth patterns in this subgroup.


McCarthy JG, Glasberg SB, Cutting CB, et al. Twenty-year experience with early surgery for craniosynostosis: II. The craniofacial synostosis syndromes and pansynostosis—results and unsolved problems. Plast Reconstr Surg 1995;96(2):284-95; discussion 296-8.
As the second of a two-part series, 76 patients with pansynostosis and craniofacial synostosis syndromes were retrospectively analyzed. Diagnoses included pansynostosis (7), craniofrontonasal dysplasia (8), and Apert (24), Crouzon (15), and Pfeiffer (15) syndromes. All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-eight patients (36.8 percent) required a secondary cranial vault operation (mean age 28.4 months). Additionally, a major tertiary procedure was necessary in 5 patients to deal with persistent unacceptable craniofacial form. To address the associated finding of midface hypoplasia, 64.8 percent (n = 35) of patients underwent Le Fort III midface advancement or had that procedure recommended for them. The remainder were awaiting appropriate age for this reconstruction. The more extensive pathologic involvement of the pansynostosis and craniofacial syndrome group is illustrated. As compared with the isolated craniofacial synostosis group previously reported, the incidence of major secondary procedures (36.8 versus 13.5 percent), perioperative complications (11.3 versus 5.0 percent), follow-up complications (44.7 versus 7.7 percent), hydrocephalus (42.1 versus 3.9 percent), shunt placement (22.4 versus 1.0 percent), and seizures (11.8 versus 2.9 percent) was significantly increased. Complex problems including those of increased intracranial pressure, airway obstruction, and recurrent turricephaly or cranial vault maldevelopment are repeatedly encountered. In addition, that early fronto-orbital advancement-cranial vault remodeling failed to promote midface development and hypoplasia of this region is almost a consistent finding in the craniofacial syndromic group. The average length of postoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. An algorithmic approach to the treatment of all patients with craniosynostosis is presented utilizing early surgical intervention as the key element.



Persing JA, Mayer PL, Spinelli HM, Miller L, Criscuolo GR. Prevention of “temporal hollowing” after fronto-orbital advancement for craniosynostosis. J Craniofac Surg 1994;5:271-4.
Depression of profile in the temporal region is commonly seen after orbital rim advancement procedures. A newly developed temporalis musculoosseous flap has been designed with the intent to prevent this postoperative occurrence.


Kakitsuba N, Sadaoka T, Motoyama S, et al. Sleep apnea and sleep-related breathing disorders in patients with craniofacial synostosis. Acta Otolaryngol Suppl 1994;517:6-10.

Seventeen patients with craniofacial synostosis (CFS) have been treated at the Department of Plastic and Reconstructive Surgery at Osaka Medical College during the past 10 years. Six patients were thoroughly evaluated at the Department of Otolaryngology by polysomnography (PSG), cephalometric X-ray and nasopharyngoscopy during sleep. In 4 of the patients PSG showed obstructive sleep apnea syndromes (OSAS). Heavy snoring without apnea and paradoxical respiration were noted in the other 2 patients. Thus, all of the patients had sleep-related breathing disorders (SRBD). It is assumed that the incidence of SRBD in CFS is high. Cephalometric analysis and profilogram showed maxillomandibular hypoplasia in each patient, and it was assumed that the main cause of SRBD in CFS was stenosis of the upper airway tract caused by maxillo-mandibular hypoplasia. Nasopharyngoscopy was performed during sleep in 2 patients with OSAS secondary to CFS. One patient with adenotonsillar hypertrophy had nasopharyngeal obstruction and another patient whose posterior airway space (PAS) on cephalometric radiograph was 3 mm (normal value: 11 mm) had obstruction at the tongue base.


Cheng H, Burdon MA, Shun-Shin GA, Czypionka S. Dissociated eye movements in craniosynostosis: A hypothesis revived. Br J Ophthalmol 1993;77:563-8.
A characteristic pattern of dissociated eye movements was observed in a large proportion of our patients with a variety of craniosynostosis syndromes. These anomalies simulate overaction of the inferior oblique and underaction of the superior oblique muscles which, however, cannot fully explain the abnormalities. In a number of cases, excyclorotation of the muscle cone was observed, with the upper pole of the eye tilted away from the midline. It is postulated that such excyclorotation of the eyes will lead to dissociated eye movements which can be explained on physiological grounds according to Hering’s law. This paper presents a review of our patients and evidence to support this hypothesis.


Meyer P, Renier D, Arnaud E, et al. Blood loss during repair of craniosynostosis. Br J Anaesth 1993;71(6):854-7.
Surgical repair of craniosynostosis carries a high risk with large blood losses. Over a 2-yr period, we have managed 115 patients undergoing craniosynostosis repair with peroperative haemodilution to achieve a final PCV of 0.28-0.35. Measurements of PCV allowed calculation of estimated blood losses and transfused volumes in terms of red blood cell mass. Total estimated red cell volume lost was 91 +/- 66% of patient’s estimated red blood cell volume during the peroperative period. The type of skull deformation and surgical procedure determined the extent of peroperative bleeding. Peroperative transfusion was satisfactory in 48% of patients and slight overtransfusion was noted in 32%. During the postoperative period, liberal administration of blood led to overtransfusion and possibly unnecessary transfusion in 74% of patients. Because of the well known risks of transmission of infectious disease, strict volume compensation with development of haemodilution and autotransfusion procedures should be used to limit these risks.


Posnick JC, Lin KY, Chen P, Armstrong D. Sagittal synostosis: Quantitative assessment of presenting deformity and surgical results based on CT scans. Plast Reconstr Surg 1993;92(6):1015-24; discussion 1225-6.
We reviewed our experience with nine consecutive patients with untreated isolated nonsyndromic sagittal synostosis. Using a method of 14 clinically relevant measurements taken from preoperative and postoperative CT scan images of these patients, we documented their presenting skeletal dysmorphology and the results of surgical correction at least 1 year after operation. Significant preoperative findings included an elongated cranial vault length that averaged 103 percent of normal and a narrowed cranial vault width both anteriorly at 92 percent and posteriorly at 86 percent of normal. Results of surgical correction, as documented by CT scan measurements, included normalization of the cranial length to 100 percent and of the anterior width to 101 percent of normal and improvement (but undercorrection) of the posterior width to 94 percent of normal. Quantitative measurement of CT scan images confirmed clinically observed findings in these patients before suture release and reconstruction and proved useful in assessing the surgical results achieved.


Kapp-Simon KA, Figueroa A, Jocher CA, Schafer M. Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 1993;92(5):831-9; discussion 840-1.
The effect of cranial release and reconstruction on the mental development of infants with nonsyndromic craniosynostosis was evaluated. Longitudinal assessment of mental development for infants before and after cranial release and reconstruction and for infants not undergoing surgical treatment was obtained by using the mental scale of the Bayley Scales of Infant Development. Severity of anatomic craniofacial deformity, perinatal medical risk factors, and age at time of surgery also were investigated. None of the infants displayed mental retardation [Mental Development Index (MDI) score < 70] before or after cranial release and reconstruction. Scores ranged from borderline retardation to very superior following a normal distribution. Severity of anatomic craniofacial deformity and perinatal risk factors were unrelated to mental development. Cranial release and reconstruction did not affect mental development positively or negatively but did result in improvement of the original craniofacial deformity.


Cohen MM. Sutural biology and the correlates of craniosynostosis. Am J Med Genet 1993;47(5):581-616.
The purpose of this paper is to provide a new perspective on craniosynostosis by correlating what is known about sutural biology with the events of craniosynostosis per se. A number of key points emerge from this analysis: 1) Sutural initiation may take place by overlapping, which results in beveled sutures, or by end-to-end approximation, which produces nonbeveled, end-to-end sutures. All end-to-end sutures occur in the midline (e.g., sagittal and metopic) probably because embryonic biomechanical forces on either side of the initiating suture tend to be equal in magnitude. A correlate appears to be that only synostosed sutures of the midline have pronounced bony ridging. 2) Long-term histologic observations of the sutural life cycle call into question the number of layers within sutures. The structure varies not only in different sutures, but also within the same suture over time. 3) Few, if any, of the many elegant experimental research studies in the field of sutural biology have increased our understanding of craniosynostosis per se. An understanding of the pathogenesis of craniosynostosis requires a genetic animal model with primary craniosynostosis and molecular techniques to understand the gene defect. This may allow insight into pathogenetic mechanisms involved in primary craniosynostosis. It may prove to be quite heterogeneous at the basic level. 4) The relationship between suture closure, cessation of growth, and functional demands across sutures poses questions about various biological relationships. Two conclusions are provocative. First, cessation of growth does not necessarily, or always lead to fusion of sutures. Second, although patent sutures aid in the growth process, some growth can take place after suture closure. 5) In an affected suture, craniosynostosis usually begins at a single point and then spreads along the suture. This has been shown by serial sectioning and calls into question results of studies in which the affected sutures are only histologically sampled. 6) Craniosynostosis is etiologically and pathogenetically heterogeneous. Known human causes are reviewed. Is craniosynostosis simply normal suture closure commencing too early?


Prevot M, Renier D, Marchac D. Lack of ossification after cranioplasty for craniosynostosis: A review of relevant factors in 592 consecutive patients. J Craniofac Surg. 1993 Oct;4(4):247-54; discussion 255-6.
The Center for Craniofacial Anomalies of Necker-Enfants-Malades Hospital presents a retrospective study of the outcome of 592 patients who were operated on for craniosynostosis between 1976 and 1991. The quality of ossification 1 year after operation is reported, with a focus on influencing factors. The lack of ossification rate was 5% (30 of 592). Three parameters are identified as increasing the risk of poor osseous wound healing: local postoperative infection; forehead advancement, especially when accomplished with resorbable osteosynthesis; and brachycephaly. In contrast, repaired tears of the dura mater do not seem to pose a risk. Seventy-five percent of patients with local infection and 12.4% with forehead advancement presented a lack of ossification, which is statistically significant (p < 0.001). Lack of ossification can compromise aesthetic and functional results. Decreasing postoperative infection and stable fixation may help promote ossification.


Fehlow P. Craniosynostosis as a risk factor. Childs Nerv Syst 1993;9(6):325-7.
Craniosynostosis is a little known organic factor in sociopathy. This factor should be among those taken into consideration in selecting patients to undergo craniotomy. Among 22,000 skulls of neuropsychiatric patients, there were 100 with premature coronal synostosis, compared with 57 with dolichocephaly. Thirty-seven of the 100 patients with coronal synostosis exhibited disorders of social adaptation; frontal cortex functions are assumed to be involved. There were 34 cases of mental deficiency, 21 cases of psychosis, 13 of cerebral vascular disease, 10 cases of epilepsy, 4 of acrocephalosyndactyly, 3 of decompensation by slight craniocerebral trauma, and 1 case of ependymoma of the IV ventricle. Dolichocephalic patients exhibited a stronger tendency towards depressive states and cerebral vascular disease. The risks of cosmetic impairment and resulting psychosocial problems are discussed; especially in girls with oxy- and scaphocephaly craniofacial correction, is indicated, as it is also in patients with Saethre-Chotzen syndrome. In cases of premature synostosis of the coronal suture or synostosis of several sutures for carrying out a craniotomy, it is advisable to employ a combination of orbito-frontosphenoidal osteotomy for extension of the anterior cranial fossa. Craniosynostosis is a risk factor which, depending on the individual case and the sex and age of the patient, can impair central nervous functions, social adaption, and the blood supply of the brain.


Turtas S, Tondi M, Tola G, et al. Neuropsychological evaluation in a series of 17 cases operated on for craniosynostosis. Acta Neurochir (Wien) 1993;120(3-4):123-5.
In a study of patients suffering from craniosynostosis various pre- and post-operative parameters were identified that may affect subsequent intellectual capacity. In particular, facial malformations appeared to be closely correlated with impaired intellectual development. Other parameters included measurement of cranial circumference and volume; their subaverage values correlated with slight mental insufficiency. However, in the pre-operative period they cannot be used as prognostic indices because these relations were statistically not significant. The remaining parameters (neurological and electroencephalographic findings, post-operative re-ossification of the skull, pre-operative digital impressions, CT findings) did not seem to be related to intellectual development.


Colak A, Tahta K, Bertan V, et al. Craniosynostosis: A review of 143 surgically-treated cases. Turk J Pediatr 1992;34(4):231-8.
In this study, 143 cases of craniosynostosis are presented. There were 109 males and 34 females. The major complaints were skull deformity (92 patients), proptosis (38 patients) and microcephalus (32 patients). Neurological examination revealed the presence of optic atrophy in 24 patients and papilledema in 20 patients. Seventy-four patients (53%) had three or more suture closures, with the sagittal suture being the most commonly involved (20% of patients). All patients underwent surgery. Suture removal was performed in 131 patients (91.7%), suture removal plus orbital decompression in 34 (23.8%), and linear craniectomy plus wrapping in 12 (8.3%). The reoperation rate was 6.2 percent. During the follow-up period, preoperative papilledema and proptosis improved in 88.2 and 78.9 percent of patients, respectively. Skull deformity disappeared in 46.9 percent of patients, but remained unchanged in 16.6 percent.


Gault DT, Renier D, Marchac D, Jones BM. Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 1992;90(3):377-81.
Intracranial volume and intracranial pressure have been measured in 66 children with craniosynostosis, 48 boys and 18 girls. The premature fusion of skull sutures is assumed to restrict skull growth and predispose to elevated intracranial pressure. Thirteen children (20 percent) had raised intracranial pressure and demonstrated a significant restriction of skull growth. In this series, volume measurement alone, however, did not serve as a reliable predictor that the intracranial pressure was raised.


di Rocco C, Marchese E, Velardi F. Craniosynostosis: Surgical treatment during the first year of life. J Neurosurg Sci 1992;36(3):129-37.
Out of 276 children with craniosynostosis operated on in a 9 year period, 231 were operated on under 1 year of age. According to the sutures involved in the process of early fusion, two groups of infants were individuated. Patients belonging to the first group were characterized by the involvement of the sagittal suture, while infants of group II presented with a synostosis of the anterior or posterior sutural complex. Two different techniques were required for the 2 groups of subjects, the first of them being mainly based on linear craniectomies, the second requiring more complex reconstructive surgical procedures. The morbidity, mortality and recurrence rates of the series were low, demonstrating that infants can tolerate the procedure as well as older children. The advantage of the early treatment consists of a better cosmetic result with a more physiological growth of faciocranial skeleton.


Shillito J. A plea for early operation for craniosynostosis. Surg Neurol 1992;37(3):182-8.
In times when much is written of complex craniofacial operations for synostosis, it is important to reassess the efficacy of simpler operations on the cranium when carried out in the first few weeks of life. Examples of sagittal, coronal, and lambdoid synostosis are shown, as well as one patient with multiple suture involvement.


Hayward R, Harkness W, Kendall B, Jones B. Magnetic resonance imaging in the assessment of craniosynostosis. Scand J Plast Reconstr Surg Hand Surg 1992;26(3):293-9.
Modern interest in craniosynostosis began when clinicians, regardless of their individual specialty but united in their interest in the problems raised by patients with this condition, first realised that if treatment was confined simply to opening up the fused sutures of the skull vault, success in terms of both function and the restoration of a more normal appearance was likely to be limited. Indeed, the grouping together of such specialists into formal craniofacial units was tacit recognition of this, as was the acceptance that many affected children had evidence of clinical problems that could not be explained on simple mechanical grounds alone. It is not surprising, therefore, that the advent of any new method of investigation has been welcomed by craniofacial surgeons eager to learn anything that might lead to an improvement in management, particularly of the more complex syndromes.


Goscinski I, Partyk A, Betlej M, Kusmiderski J. [Fate of patients after surgical treatment of premature closure of cranial sutures.] [Article in Polish] Neurol Neurochir Pol 1991;25(5):618-25.
From 1954 to 1971, 69 operations in patients with crs, resulting in relieving the intracranial hypertension symptoms, were performed. The patients were aged 1-34 years. In 1989, i.e. 20-29 years after the operation (mean 22.8 years), 14 patients were submitted again for a neurological, neuropsychological, EEG and brain CT check-up. The patients were divided into 3 groups: I gr. (3 patients)--with negligible disorders of attention and memory, without neurological changes in the EEG and CT--in a good social and occupational status. II gr. (4 patients)--with slight headaches, with discrete neurological and neuropsychological symptoms, slight generalized changes with the moderate burst activity in EEG, signs of hydrocephalus in CT scan. III. gr (7 patients)--with seizures, deficit symptoms, some with symptoms of mental impairment, generalized epileptic changes in EEG, signs of cortical and subcortical atrophy in CT scan. In this group some patients did not work and had no families. We have found that the frequency of epileptic seizures in the crs patients is higher, and their social and occupational status is worse.


Newman SA. Ophthalmic features of craniosynostosis. Neurosurg Clin N Am 1991;2(3):587-610.
With the possible exception of aesthetic considerations, the visual system is more involved in craniosynostosis than any other. Changes may be an inherent feature of the pathologic process or occur as a secondary complication. Several of these are potentially devastating, resulting in substantial permanent impairment in visual function. Because these are avoidable if recognized and managed early, prompt involvement of an ophthalmologist in the care of children with craniosynostosis is essential. Improvement in the appearance of these cases cannot obscure the importance of functional problems. In particular, disc edema, optic atrophy, and progressive optic nerve dysfunction may accompany increased intracranial pressure even without evidence of hydrocephalus and even with apparently open fontanelles. Uncorrected refractive error (particularly anisometropia), strabismus, ptosis, and corneal exposure problems are an invitation to the development of amblyopia. If not reversed, this can lead to permanent visual disability. Proptosis and corneal exposure problems are a third potentially treatable cause of functional blindness. Early diagnosis and prompt care will hopefully continue to improve the prognosis for these increasingly treatable children.


Vargervik K, Miller AJ. Observations on the temporal muscle in craniosynostosis. Birth Defects Orig Artic Ser 1982;18:45-51. No abstract.


 

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