- Email: [email protected]
Early surgical treatment of nonsyndromic craniosynostosis
Surgical Neurology 65 (2006) S1:22 – S1:26 www.surgicalneurology-online.com
Technique
Early surgical treatment of nonsyndromic craniosynostosis Marcelo Paglioli Ferreira, MDa,b,c,*, Marcus Vinı´cius Martins Collares, MD, PhDc,d, Nelson Pires Ferreira, MD, PhDa,b,c,e, Jorge Luiz Kraemer, MD, PhDa, Arthur De Azambuja Pereira Filho, MDa,e, Gustavo De Azambuja Pereira Filho, MSe a
Neurosurgery Department, Hospital Sa˜o Jose´/Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre 90020-060, Brazil b Neurosurgery Department, Hospital de Clı´nicas de Porto Alegre, Porto Alegre 90035-000, Brazil c Medical School, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-000, Brazil d Craniomaxillofacial Surgery Unit, Hospital de Clı´nicas de Porto Alegre, Porto Alegre 90035-000, Brazil e Medical School, Fundac¸a˜o Faculdade Federal de Cieˆncias Me´dicas de Porto Alegre, Porto Alegre 90050-170, Brazil Received 31 October 2005; accepted 17 November 2005
Abstract
Background: Craniosynostosis, a premature fusion of cranial sutures, can happen as an isolated defect (nonsyndromic) or as part of a syndrome. It may lead to raised intracranial pressure and deformity of both the cranial and facial skeletons. Early surgery is usually the best treatment choice. The aim of this study was to review the experience of a multidisciplinary staff with the surgical treatment of nonsyndromic craniosynostosis. Methods: All the nonsyndromic craniosynostosis cases treated surgically from 1991 to 2005 at 2 neurosurgical centers were reviewed. The variables analyzed were sex, age, weight, type of deformity, estimated volemia, surgical technique, surgical time, blood loss, PO hospitalization time, and complications. All data were stored in a database and analyzed with Microsoft Access (Microsoft Corp, Seattle, WA) and SPSS V11.0 (SPSS, Inc, Chicago, IL). Results: The sample consisted of 120 patients (70 males, 50 females). Six patients were excluded because of their atypical timing of treatment (average age, 72 months). The final average age was 7.08 months (variation, 1-18), and the average weight was 7.8 kg. The types of deformities found were scaphocephaly (n = 54, 45%), plagiocephaly (n = 27, 22%), trigonocephaly (n = 19, 16%), brachycephaly (n = 16, 13%), and oxycephaly (n = 4, 3%). The mean surgical time was 186 minutes and the mean PO hospitalization time was 6.8 days. The mortality on this series was 2.6% (3 patients). In the last operated cases, a significant improvement in morbid-mortality was observed. Conclusion: A multidisciplinary approach, including neurosurgeons, neurologists, and pediatricians, and appropriate training of the clinical surgical staff can minimize the risks and decrease the complications in the treatment of craniosynostosis, leading to a satisfactory outcome. D 2006 Published by Elsevier Inc.
Keywords:
Craniosynostosis; Craniostenosis; Craniofacial deformity
1. Introduction Craniosynostosis is defined as the premature closure of 1 or more of the sutures that normally separate the bony plates of the infant’s skull [15]. Abnormal head shapes are therefore produced because of the restricted growth,
Abbreviations: PO, postoperative. 4 Corresponding author. Tel.: +55 51 3395 4182/+55 51 9978 1151; fax: +55 51 3222 2562. E-mail address: [email protected] (M.P. Ferreira). 0090-3019/$ – see front matter D 2006 Published by Elsevier Inc. doi:10.1016/j.surneu.2005.11.038
which occurs perpendicular to the fused sutures and to the compensatory growth in the skull’s unfused bony plates [28]. Craniosynostosis can happen as an isolated defect (nonsyndromic) or as part of a syndrome. It is called simple when only 1 suture is involved and compound when 2 or more sutures are involved [3,30]. Craniosynostosis occurs within the population with a frequency of 1:2000 to 1:4000 [14,29]. Most cases involve a single suture and are nonsyndromic. The sagittal suture is affected in 40% to 60% of cases, the coronal suture in 20% to 30% of cases, and the metopic suture in less than
M.P. Ferreira et al. / Surgical Neurology 65 (2006) S1:22 – S1:26
10% of cases; true lambdoid synostosis is rare [5,30]. Syndromic craniosynostosis is less common (20%), although more than 150 syndromes with craniosynostosis have been identified. The etiology of nonsyndromic craniosynostosis is still unknown, and the condition is sporadic in most instances [13,29]. Potential risk factors identified from previous studies include white maternal race [1], advanced maternal age [1], male infant sex [1], maternal smoking [11], maternal residence at high altitudes [2], use of nitrosatable drugs (eg, nitrofurantoin, chlordiazepoxide, chlorpheniramine) [8], certain paternal occupations (eg, agriculture and forestry, mechanics, repairman) [4], and fertility treatments [22]. Familial nonsyndromic craniosynostosis, which affects 2% to 6% of infants with sagittal synostosis and 8% to 14% of infants with coronal synostosis, is transmitted as an autosomal dominant disorder [30]. Craniosynostosis may lead to 2 main groups of problems. A raised intracranial pressure with or without hydrocephalus may be seen, although this is more common where multiple sutures are involved [5,15,23,24]. Also, deformity of both the cranial and facial skeletons may also be found. To correct aesthetics or to reduce intracranial pressure, early corrective surgery may be indicated because 50% of skull growth is achieved by 6 months of age [9,16,21,31,32].
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The aim of this study was to review the experience of a multidisciplinary staff with the surgical treatment of nonsyndromic craniosynostosis. 2. Patients and methods All the nonsyndromic craniosynostosis cases treated surgically from 1991 to 2005 at Hospital Sa˜o Jose´/ Complexo Hospitalar Santa Casa de Porto Alegre (a hospital complex in the city of Porto Alegre, Brazil [southern]) and Hospital de Clı´nicas de Porto Alegre (a hospital in the city of Porto Alegre, Brazil [southern]) were reviewed. The following variables were analyzed: sex, age, weight, type of deformity, estimated volemia, surgical technique, surgical time, blood loss, PO admission time, and PO complications. All data were stored in a database and analyzed with Microsoft Access (Microsoft Corp, Seattle, WA) and SPSS V11.0 (SPSS, Inc, Chicago, IL). Diagnosis was confirmed after a history and general examination and plain radiography of the skull. All patients underwent computed tomographic scanning to exclude hydrocephalus and other cranial or cerebral abnormalities [3]. Preoperative assessment was carried out immediately before surgery and included pediatric clinical evaluation, blood coagulation tests, hemogram, urea and electrolyte estimations, and blood crossmatching.
Fig. 1. Craniostenosis due to premature closure of the unilateral coronal suture (plagiocephaly). A: Preoperative. B: Six-year PO result. C: Immediate preoperative, frontal deformity. D: Transoperative, fronto-orbital remodeling.
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Operations were carried out under endotracheal anesthesia. Intravenous antibiotics (cefazolin) were administered at the time of induction of anesthesia and continued for 48 hours postoperatively. The surgical techniques used were variations of the Persing technique: total cranial remodeling (Fig. 1) and orbital-cranial remodeling (Fig. 2) [7,19,18,26,30]. Postoperative management was routinely carried out on a pediatric intensive care unit.
3. Results The sample consisted of 120 patients (70 males and 50 females). Six patients were excluded because of their atypical timing of treatment (average age, 72 months; variation, 24-144). The final average age was 7.08 months (variation, 1-18). The minimum weight found was 2.6 kg and the maximum was 20 kg (average, 7.8 kg). The types of deformities found on the series were scaphocephaly (n = 54, 45%), plagiocephaly (n = 27, 22%; Fig. 1), trigonocephaly (n = 19, 16%), brachycephaly (n = 16, 13%), and oxycephaly (n = 4, 3%; Table 1). Among the cases of multiple deformities, posterior scapho-
Table 1 Types of nonsyndromic craniosynostosis (N = 120) Type of deformity
n (%)
Scaphocephaly Plagiocephaly Trigonocephaly Brachycephaly Oxycephaly
54 27 19 16 4
(45) (22) (16) (13) (3)
plagiocephaly (2 cases, Fig. 2) and anterior plus posterior brachycephaly (2 cases) were reported. The surgical technique used was total cranial remodeling in 58 patients and orbital-cranial remodeling in 62 cases. Surgical time ranged from 120 to 780 minutes (average, 186 minutes). Postoperative hospitalization time ranged from 4 to 21 days, with an average of 6.8 days. There was an estimated volemia loss of 32% during the transoperative period and of 27% 2 days after surgery (suction drain). No surgical complications were observed in 103 patients (90.3%). In 6 patients, there were surgical anesthetic complications: cardiorespiratory arrest in the transoperative period, hypovolemic shock, seizure, cerebrospinal fluid fistula, extradural hematoma, and partial dehiscence in the scalp. Clinical complications, observed in 7 patients,
Fig. 2. Craniostenosis due to premature closure of the sagittal and unilateral lambdoid sutures (scaphocephaly and posterior plagiocephaly). A: Preoperative. B: Five-year PO result. C: Computer tomographic scans, frontoparieto-occipital deformity. D: Transoperative, total cranial remodeling.
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included pulmonary edema, respiratory obstruction, urinary tract infection, orbital cellulitis, and septic shock. Overall mortality on this series was 2.6% (n = 3). The causes of death were hypovolemic shock (2 cases), respiratory complications, and septic shock (1 case). In the last operated cases, an improvement in surgical time, blood loss (transoperative and PO periods), PO complications, hospitalization time, and mortality rates was observed. 4. Discussion Craniosynostosis remains primarily a surgical disease. The goals of therapy are to provide adequate intracranial volume to allow space for brain expansion and to minimize cognitive sequelae and to create an aesthetically normal skull shape [13,28]. The optimal timing for the surgical treatment of craniosynostosis is still controversial [9,16,20,24,33]. Many surgeons prefer to operate early to capitalize on the ameliorating effects of brain growth on skull shape. If there is any evidence of increased intracranial pressure (eg, bulging fontanelles, progressive optic atrophy, seizures, or multiple-suture synostosis), most surgeons operate at the earliest opportunity [29]. In this series of nonsyndromic craniosynostosis, the chosen surgical treatment was performed preferably around 6 months of age because approximately 50% of skull growth is achieved by this period of life [9,16,21,31,32]. Surgical treatment dates from the late 19th century, when the first techniques were aimed solely at correcting the functional aspects of this deformity. The earliest technique, linear craniectomy and fragmentation of the cranial vault, is still occasionally used today by some surgeons for particularly profound deformities. This method provides temporary brain and eye protection until a more definitive craniofacial procedure can be undertaken. The next step, simple craniectomy, was unfortunately accompanied by a high rate of reossification and gave only modest results, unless mobilization of the orbits, midface, and cranium was performed concurrently. Advances in craniofacial surgery have progressed with the implementation of different surgical techniques [6,25]. Today’s surgical interventions can be divided into 3 procedures: (1) suture release, cranial vault decompression, and upper orbital reshaping, and advancement in infancy (6-12 months); (2) operations to correct midface deformities in childhood (6-12 years); and (3) orthognathic surgery in adolescence (14-18 years). The exact timing and sequence of each of the aforementioned surgical procedures is dependent on both the functional and the psychologic needs [29]. In this series of nonsyndromic craniosynostosis, the chosen treatment was surgical correction of the anomaly in only one time, preferably around 6 months of age. The techniques used were variations of the Persing technique: total cranial remodeling or orbitalcranial remodeling [7,18,19,26,30]. This technique is accepted worldwide, and it is the favorite of the authors.
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It facilitates global shaping through radial sections in the bone and also permits large reconstructions. Several craniosynostosis series have been reported in literature. Harrop et al [9], in a series of 40 consecutive craniosynostosis cases in babies, reported a mean age for intervention of 9.2 months. Kadri and Mawla [10], in a study of 116 cases of Syrian children with craniosynostosis, related that surgical intervention was performed in children between 3 and 12 months old. Nonaka et al [17] reported a series of 25 children surgically treated for craniosynostosis between the ages of 1 year 5 months and 12 years 6 months, with a mean age of 4 years 11 months. In this series of nonsyndromic craniosynostosis, the average age of treatment was 7.08 months. Kadri and Mawla [10] reported in his study the following sex distribution: 52% male and 48% female. Alderman et al [1] and Singer et al [27] achieved similar sex results. In the present series, the sex distribution was 70 males and 50 females. These findings are therefore in agreement with the literature [1,10,27]. Kane [12], in a craniosynostosis review article, reported that sagittal synostosis represents 64% of all cases, followed by 28% for coronal, 5% for metopic, and 3% for lambdoid synostosis. Harrop et al [9] and Kadri and Mawla [10] found similar results in their series. In the present study, the distribution of deformities was also in agreement with the literature [9,10,12]. Harrop et al [9] reported a morbidity of 0.02% and no mortality in 40 consecutive craniosynostosis operated cases. Kadri and Mawla [10] referred a mortality of 3 patients (2.58%) in a study of 116 children with craniosynostosis. Nonaka et al [17], in a series of 25 patients with craniosynostosis, had a morbidity of 12% (3 patients). In the present series, morbidity was 9.7% and mortality was 2.6%. This finding is in agreement with the literature [9,10,17]. In the last cases of this series, it was observed that surgical time, blood loss (in the transoperative and PO periods), PO complications, hospitalization time, and mortality rates showed a significant reduction. These findings may reflect an improvement in the relationship among surgical (craniomaxillofacial and neurosurgical) team members in dealing with this complex and interesting disease. 5. Conclusion A multidisciplinary approach and appropriate training of the clinical surgical staff can minimize the risks and decrease the complications in the treatment of craniosynostosis, leading to a satisfactory outcome.
References [1] Alderman BW, Lammer EJ, Joshua SC, et al. An epidemiologic study of craniosynostosis: risk indicators for the occurrence of craniosynostosis in Colorado. Am J Epidemiol 1988;128:431 - 8.
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[2] Alderman BW, Zamudio S, Baron AE, et al. Increased risk of craniosynostosis with higher antenatal maternal altitude. Int J Epidemiol 1995;24:420 - 6. [3] Aviv RI, Rodger E, Hall CM. Craniosynostosis. Clin Radiol 2002;57:93 - 102. [4] Bradley CM, Alderman BW, Williams MA, et al. Parental occupations as risk factors for craniosynostosis in offspring. Epidemiol 1995;6:306 - 10. [5] Bristol RE, Lekovic GP, Rekate HL. The effects of craniosynostosis on the brain with respect to the intracranial pressure. Semin Pediatr Neurol 2004;11:262 - 7. [6] David L, Proffer P, Hurst W, et al. Spring-mediated cranial reshaping for craniosynostosis. J Craniofac Surg 2004;15(5):810 - 6. [7] Delashaw JB, Persing JA, Jane JA. Cranial deformation in craniosynostosis. A new explanation. Neurosurg Clin N Am 1991;2(3):611 - 20. [8] Gardner JS, Guyard-Boileau B, Alderman BW, et al. Maternal exposure to prescription and non-prescription pharmaceuticals or drugs of abuse and risk of craniosynostosis. Int J Epidemiol 1998;27:64 - 7. [9] Harrop CW, Avery BW, Marks SM, Putnam GW. Craniosynostosis in babies: complications and management of 40 cases. Br J Oral Maxillofac Surg 1996;34:158 - 61. [10] Kadri H, Mawla AA. Incidences of craniosynostosis in Syria. J Craniofac Surg 2004;15(4):703 - 4. [11] Kallen K. Maternal smoking and craniosynostosis. Teratology 1999;60:146 - 50. [12] Kane AA. An overview of craniosynostosis. J Prosth Orthot 2004;4:S50 – 5. [13] Lekovic GP, Bristol RE, Rekate HL. Cognitive impact of craniosynostosis. Semin Pediatr Neurol 2004;11:305 - 10. [14] Lima D. The management of deformational plagiocephaly: a review of the literature. J Prosth Orthot 2004;16(4):S9 – S14. [15] Marchac D, Renier D. Textbook of craniofacial surgery for craniosynostosis. Boston7 Little and Brown; 1982. [16] Marchac D, Renier D. Treatment of craniosynostosis in infancy. Clin Plast Surg 1987;14:61 - 72. [17] Nonaka Y, Oi S, Miyawaki T, et al. Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Childs Nerv Syst 2004;20:702 - 9. [18] Persing JA, Luce C. Remodeling techniques for immature and mature cranial vault bone: technical note. J Craniofac Surg 1990;1(3):147 - 9. [19] Persing JA, Jane JA. Treatment of syndromic and nonsyndromic bilateral coronal synostosis in infancy and childhood. Neurosurg Clin N Am 1991;2(3):655 - 63. [20] Polley J, Charbel F. Nonsyndromal craniosynostosis: longitudinal outcome following cranio-orbital reconstruction in infancy. Clin Plast Surg 1998;102:619. [21] Rannan-Eliya SV, Middleton JA, Wall SA. Functional implications of single suture craniosynostosis. Curr Paediatr 2002;12:199 - 205. [22] Reefhuis J, Honein MA, Shaw GM. Fertility treatments and craniosynostosis. California, Georgia, and Iowa, 1993-1997. Pediatrics 2003;111:1163 - 6. [23] Renier D, Brunet L, Marchac D. IQ and craniostenosis: evolution in treated and untreated cases. In: Marchac D, editor. Craniofacial surgery. Berlin7 Springer-Verlag; 1987. p. 114 - 17. [24] Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostosis. Childs Nerv Syst 2000;16:645 - 58. [25] Ririe D, David L, Glazier S, et al. Surgical advancement influences perioperative care: a comparison of two surgical techniques for sagittal craniosynostosis repair. Anesth Analg 2003;97:699 - 703. [26] Shaffrey ME, Persing JA, Delashaw JB, et al. Surgical treatment of metopic synostosis. Neurosurg Clin N Am 1991;2(3):621 - 7.
[27] Singer S, Bower C, Southall P, et al. Craniosynostosis in Western Australia, 1980-1994: a population-based study. Am J Med Genet 1999;83:382 - 7. [28] Speltz ML, Kapp-Simon KA, Cunningham M, Marsh J, Dawson G. Single suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol 2004;29(8):651 - 68. [29] Stephen MW. The pathogenesis of craniosynostosis in the fetus. Yonsei Med J 2001;42:646 - 59. [30] Sun PP, Persing JA. Craniosynostosis. In: Albright AL, Pollack IF, Adelson PD, editors. Principles and practice of pediatric neurosurgery. New York7 Thieme Medical; 1999. p. 219 - 42. [31] Whitacker LA, Bartlett SP, Schut L. Craniosynostosis: an analysis of the timing, treatment and complications in 164 consecutive patients. Plast Reconstr Surg 1987;80:195 - 212. [32] Whitacker LA, Munro IR, Salyer KE, et al. Combined report of problems and complications in 793 craniofacial operations. Plast Reconstr Surg 1987;64:198 - 203. [33] Williams K, Cohen S, et al. A longitudinal, statistical study of reoperation rates in craniosynostosis. Clin Plast Surg 1997;100:305.
Commentary This article by Ferreira et al summarizes a series over a 14-year period. From 1991 to 2005, 120 patients with nonsyndromic craniosynostosis were treated surgically and the variables sex, age, weight, deformity, surgical technique, volemia, blood loss, hospitalization time, and complications were analyzed. The morbidity and mortality seem to be similar to the literature, and there is a decrease of complications with multidisciplinary approach and appropriate training of the clinical surgical staff. The actual consensus is that the surgical treatment is to be done anytime between 3 and 6 months old. At this period of life, it is easier to proceed with the surgery because the bone is thinner, and even when there is a great PO skull bone absence, it will be appropriate with spontaneous coverage on growth. The main problem is intraoperative bleeding and the appropriate clinical management. As we saw in this series, 2 deaths happened because of hypovolemic shock. We emphasize that the anesthesiologist must have knowledge/ experience with the different nonsyndromic craniosynostosis intraoperative behavior and must also have knowledge/ experience in volume replacement in this kind of procedure to avoid hypotension and hypovolemic shock to the point of being able to perform, in advance, transfusion before having a hemoglobin level of less than 10.
Jose´ Marcus Rotta, MD, PhD Hospital do Servidor Pu´blico de Sa˜o Paulo Sa˜o Paulo, Brasil
Technique
Early surgical treatment of nonsyndromic craniosynostosis Marcelo Paglioli Ferreira, MDa,b,c,*, Marcus Vinı´cius Martins Collares, MD, PhDc,d, Nelson Pires Ferreira, MD, PhDa,b,c,e, Jorge Luiz Kraemer, MD, PhDa, Arthur De Azambuja Pereira Filho, MDa,e, Gustavo De Azambuja Pereira Filho, MSe a
Neurosurgery Department, Hospital Sa˜o Jose´/Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre 90020-060, Brazil b Neurosurgery Department, Hospital de Clı´nicas de Porto Alegre, Porto Alegre 90035-000, Brazil c Medical School, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-000, Brazil d Craniomaxillofacial Surgery Unit, Hospital de Clı´nicas de Porto Alegre, Porto Alegre 90035-000, Brazil e Medical School, Fundac¸a˜o Faculdade Federal de Cieˆncias Me´dicas de Porto Alegre, Porto Alegre 90050-170, Brazil Received 31 October 2005; accepted 17 November 2005
Abstract
Background: Craniosynostosis, a premature fusion of cranial sutures, can happen as an isolated defect (nonsyndromic) or as part of a syndrome. It may lead to raised intracranial pressure and deformity of both the cranial and facial skeletons. Early surgery is usually the best treatment choice. The aim of this study was to review the experience of a multidisciplinary staff with the surgical treatment of nonsyndromic craniosynostosis. Methods: All the nonsyndromic craniosynostosis cases treated surgically from 1991 to 2005 at 2 neurosurgical centers were reviewed. The variables analyzed were sex, age, weight, type of deformity, estimated volemia, surgical technique, surgical time, blood loss, PO hospitalization time, and complications. All data were stored in a database and analyzed with Microsoft Access (Microsoft Corp, Seattle, WA) and SPSS V11.0 (SPSS, Inc, Chicago, IL). Results: The sample consisted of 120 patients (70 males, 50 females). Six patients were excluded because of their atypical timing of treatment (average age, 72 months). The final average age was 7.08 months (variation, 1-18), and the average weight was 7.8 kg. The types of deformities found were scaphocephaly (n = 54, 45%), plagiocephaly (n = 27, 22%), trigonocephaly (n = 19, 16%), brachycephaly (n = 16, 13%), and oxycephaly (n = 4, 3%). The mean surgical time was 186 minutes and the mean PO hospitalization time was 6.8 days. The mortality on this series was 2.6% (3 patients). In the last operated cases, a significant improvement in morbid-mortality was observed. Conclusion: A multidisciplinary approach, including neurosurgeons, neurologists, and pediatricians, and appropriate training of the clinical surgical staff can minimize the risks and decrease the complications in the treatment of craniosynostosis, leading to a satisfactory outcome. D 2006 Published by Elsevier Inc.
Keywords:
Craniosynostosis; Craniostenosis; Craniofacial deformity
1. Introduction Craniosynostosis is defined as the premature closure of 1 or more of the sutures that normally separate the bony plates of the infant’s skull [15]. Abnormal head shapes are therefore produced because of the restricted growth,
Abbreviations: PO, postoperative. 4 Corresponding author. Tel.: +55 51 3395 4182/+55 51 9978 1151; fax: +55 51 3222 2562. E-mail address: [email protected] (M.P. Ferreira). 0090-3019/$ – see front matter D 2006 Published by Elsevier Inc. doi:10.1016/j.surneu.2005.11.038
which occurs perpendicular to the fused sutures and to the compensatory growth in the skull’s unfused bony plates [28]. Craniosynostosis can happen as an isolated defect (nonsyndromic) or as part of a syndrome. It is called simple when only 1 suture is involved and compound when 2 or more sutures are involved [3,30]. Craniosynostosis occurs within the population with a frequency of 1:2000 to 1:4000 [14,29]. Most cases involve a single suture and are nonsyndromic. The sagittal suture is affected in 40% to 60% of cases, the coronal suture in 20% to 30% of cases, and the metopic suture in less than
M.P. Ferreira et al. / Surgical Neurology 65 (2006) S1:22 – S1:26
10% of cases; true lambdoid synostosis is rare [5,30]. Syndromic craniosynostosis is less common (20%), although more than 150 syndromes with craniosynostosis have been identified. The etiology of nonsyndromic craniosynostosis is still unknown, and the condition is sporadic in most instances [13,29]. Potential risk factors identified from previous studies include white maternal race [1], advanced maternal age [1], male infant sex [1], maternal smoking [11], maternal residence at high altitudes [2], use of nitrosatable drugs (eg, nitrofurantoin, chlordiazepoxide, chlorpheniramine) [8], certain paternal occupations (eg, agriculture and forestry, mechanics, repairman) [4], and fertility treatments [22]. Familial nonsyndromic craniosynostosis, which affects 2% to 6% of infants with sagittal synostosis and 8% to 14% of infants with coronal synostosis, is transmitted as an autosomal dominant disorder [30]. Craniosynostosis may lead to 2 main groups of problems. A raised intracranial pressure with or without hydrocephalus may be seen, although this is more common where multiple sutures are involved [5,15,23,24]. Also, deformity of both the cranial and facial skeletons may also be found. To correct aesthetics or to reduce intracranial pressure, early corrective surgery may be indicated because 50% of skull growth is achieved by 6 months of age [9,16,21,31,32].
S1:23
The aim of this study was to review the experience of a multidisciplinary staff with the surgical treatment of nonsyndromic craniosynostosis. 2. Patients and methods All the nonsyndromic craniosynostosis cases treated surgically from 1991 to 2005 at Hospital Sa˜o Jose´/ Complexo Hospitalar Santa Casa de Porto Alegre (a hospital complex in the city of Porto Alegre, Brazil [southern]) and Hospital de Clı´nicas de Porto Alegre (a hospital in the city of Porto Alegre, Brazil [southern]) were reviewed. The following variables were analyzed: sex, age, weight, type of deformity, estimated volemia, surgical technique, surgical time, blood loss, PO admission time, and PO complications. All data were stored in a database and analyzed with Microsoft Access (Microsoft Corp, Seattle, WA) and SPSS V11.0 (SPSS, Inc, Chicago, IL). Diagnosis was confirmed after a history and general examination and plain radiography of the skull. All patients underwent computed tomographic scanning to exclude hydrocephalus and other cranial or cerebral abnormalities [3]. Preoperative assessment was carried out immediately before surgery and included pediatric clinical evaluation, blood coagulation tests, hemogram, urea and electrolyte estimations, and blood crossmatching.
Fig. 1. Craniostenosis due to premature closure of the unilateral coronal suture (plagiocephaly). A: Preoperative. B: Six-year PO result. C: Immediate preoperative, frontal deformity. D: Transoperative, fronto-orbital remodeling.
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Operations were carried out under endotracheal anesthesia. Intravenous antibiotics (cefazolin) were administered at the time of induction of anesthesia and continued for 48 hours postoperatively. The surgical techniques used were variations of the Persing technique: total cranial remodeling (Fig. 1) and orbital-cranial remodeling (Fig. 2) [7,19,18,26,30]. Postoperative management was routinely carried out on a pediatric intensive care unit.
3. Results The sample consisted of 120 patients (70 males and 50 females). Six patients were excluded because of their atypical timing of treatment (average age, 72 months; variation, 24-144). The final average age was 7.08 months (variation, 1-18). The minimum weight found was 2.6 kg and the maximum was 20 kg (average, 7.8 kg). The types of deformities found on the series were scaphocephaly (n = 54, 45%), plagiocephaly (n = 27, 22%; Fig. 1), trigonocephaly (n = 19, 16%), brachycephaly (n = 16, 13%), and oxycephaly (n = 4, 3%; Table 1). Among the cases of multiple deformities, posterior scapho-
Table 1 Types of nonsyndromic craniosynostosis (N = 120) Type of deformity
n (%)
Scaphocephaly Plagiocephaly Trigonocephaly Brachycephaly Oxycephaly
54 27 19 16 4
(45) (22) (16) (13) (3)
plagiocephaly (2 cases, Fig. 2) and anterior plus posterior brachycephaly (2 cases) were reported. The surgical technique used was total cranial remodeling in 58 patients and orbital-cranial remodeling in 62 cases. Surgical time ranged from 120 to 780 minutes (average, 186 minutes). Postoperative hospitalization time ranged from 4 to 21 days, with an average of 6.8 days. There was an estimated volemia loss of 32% during the transoperative period and of 27% 2 days after surgery (suction drain). No surgical complications were observed in 103 patients (90.3%). In 6 patients, there were surgical anesthetic complications: cardiorespiratory arrest in the transoperative period, hypovolemic shock, seizure, cerebrospinal fluid fistula, extradural hematoma, and partial dehiscence in the scalp. Clinical complications, observed in 7 patients,
Fig. 2. Craniostenosis due to premature closure of the sagittal and unilateral lambdoid sutures (scaphocephaly and posterior plagiocephaly). A: Preoperative. B: Five-year PO result. C: Computer tomographic scans, frontoparieto-occipital deformity. D: Transoperative, total cranial remodeling.
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included pulmonary edema, respiratory obstruction, urinary tract infection, orbital cellulitis, and septic shock. Overall mortality on this series was 2.6% (n = 3). The causes of death were hypovolemic shock (2 cases), respiratory complications, and septic shock (1 case). In the last operated cases, an improvement in surgical time, blood loss (transoperative and PO periods), PO complications, hospitalization time, and mortality rates was observed. 4. Discussion Craniosynostosis remains primarily a surgical disease. The goals of therapy are to provide adequate intracranial volume to allow space for brain expansion and to minimize cognitive sequelae and to create an aesthetically normal skull shape [13,28]. The optimal timing for the surgical treatment of craniosynostosis is still controversial [9,16,20,24,33]. Many surgeons prefer to operate early to capitalize on the ameliorating effects of brain growth on skull shape. If there is any evidence of increased intracranial pressure (eg, bulging fontanelles, progressive optic atrophy, seizures, or multiple-suture synostosis), most surgeons operate at the earliest opportunity [29]. In this series of nonsyndromic craniosynostosis, the chosen surgical treatment was performed preferably around 6 months of age because approximately 50% of skull growth is achieved by this period of life [9,16,21,31,32]. Surgical treatment dates from the late 19th century, when the first techniques were aimed solely at correcting the functional aspects of this deformity. The earliest technique, linear craniectomy and fragmentation of the cranial vault, is still occasionally used today by some surgeons for particularly profound deformities. This method provides temporary brain and eye protection until a more definitive craniofacial procedure can be undertaken. The next step, simple craniectomy, was unfortunately accompanied by a high rate of reossification and gave only modest results, unless mobilization of the orbits, midface, and cranium was performed concurrently. Advances in craniofacial surgery have progressed with the implementation of different surgical techniques [6,25]. Today’s surgical interventions can be divided into 3 procedures: (1) suture release, cranial vault decompression, and upper orbital reshaping, and advancement in infancy (6-12 months); (2) operations to correct midface deformities in childhood (6-12 years); and (3) orthognathic surgery in adolescence (14-18 years). The exact timing and sequence of each of the aforementioned surgical procedures is dependent on both the functional and the psychologic needs [29]. In this series of nonsyndromic craniosynostosis, the chosen treatment was surgical correction of the anomaly in only one time, preferably around 6 months of age. The techniques used were variations of the Persing technique: total cranial remodeling or orbitalcranial remodeling [7,18,19,26,30]. This technique is accepted worldwide, and it is the favorite of the authors.
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It facilitates global shaping through radial sections in the bone and also permits large reconstructions. Several craniosynostosis series have been reported in literature. Harrop et al [9], in a series of 40 consecutive craniosynostosis cases in babies, reported a mean age for intervention of 9.2 months. Kadri and Mawla [10], in a study of 116 cases of Syrian children with craniosynostosis, related that surgical intervention was performed in children between 3 and 12 months old. Nonaka et al [17] reported a series of 25 children surgically treated for craniosynostosis between the ages of 1 year 5 months and 12 years 6 months, with a mean age of 4 years 11 months. In this series of nonsyndromic craniosynostosis, the average age of treatment was 7.08 months. Kadri and Mawla [10] reported in his study the following sex distribution: 52% male and 48% female. Alderman et al [1] and Singer et al [27] achieved similar sex results. In the present series, the sex distribution was 70 males and 50 females. These findings are therefore in agreement with the literature [1,10,27]. Kane [12], in a craniosynostosis review article, reported that sagittal synostosis represents 64% of all cases, followed by 28% for coronal, 5% for metopic, and 3% for lambdoid synostosis. Harrop et al [9] and Kadri and Mawla [10] found similar results in their series. In the present study, the distribution of deformities was also in agreement with the literature [9,10,12]. Harrop et al [9] reported a morbidity of 0.02% and no mortality in 40 consecutive craniosynostosis operated cases. Kadri and Mawla [10] referred a mortality of 3 patients (2.58%) in a study of 116 children with craniosynostosis. Nonaka et al [17], in a series of 25 patients with craniosynostosis, had a morbidity of 12% (3 patients). In the present series, morbidity was 9.7% and mortality was 2.6%. This finding is in agreement with the literature [9,10,17]. In the last cases of this series, it was observed that surgical time, blood loss (in the transoperative and PO periods), PO complications, hospitalization time, and mortality rates showed a significant reduction. These findings may reflect an improvement in the relationship among surgical (craniomaxillofacial and neurosurgical) team members in dealing with this complex and interesting disease. 5. Conclusion A multidisciplinary approach and appropriate training of the clinical surgical staff can minimize the risks and decrease the complications in the treatment of craniosynostosis, leading to a satisfactory outcome.
References [1] Alderman BW, Lammer EJ, Joshua SC, et al. An epidemiologic study of craniosynostosis: risk indicators for the occurrence of craniosynostosis in Colorado. Am J Epidemiol 1988;128:431 - 8.
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[2] Alderman BW, Zamudio S, Baron AE, et al. Increased risk of craniosynostosis with higher antenatal maternal altitude. Int J Epidemiol 1995;24:420 - 6. [3] Aviv RI, Rodger E, Hall CM. Craniosynostosis. Clin Radiol 2002;57:93 - 102. [4] Bradley CM, Alderman BW, Williams MA, et al. Parental occupations as risk factors for craniosynostosis in offspring. Epidemiol 1995;6:306 - 10. [5] Bristol RE, Lekovic GP, Rekate HL. The effects of craniosynostosis on the brain with respect to the intracranial pressure. Semin Pediatr Neurol 2004;11:262 - 7. [6] David L, Proffer P, Hurst W, et al. Spring-mediated cranial reshaping for craniosynostosis. J Craniofac Surg 2004;15(5):810 - 6. [7] Delashaw JB, Persing JA, Jane JA. Cranial deformation in craniosynostosis. A new explanation. Neurosurg Clin N Am 1991;2(3):611 - 20. [8] Gardner JS, Guyard-Boileau B, Alderman BW, et al. Maternal exposure to prescription and non-prescription pharmaceuticals or drugs of abuse and risk of craniosynostosis. Int J Epidemiol 1998;27:64 - 7. [9] Harrop CW, Avery BW, Marks SM, Putnam GW. Craniosynostosis in babies: complications and management of 40 cases. Br J Oral Maxillofac Surg 1996;34:158 - 61. [10] Kadri H, Mawla AA. Incidences of craniosynostosis in Syria. J Craniofac Surg 2004;15(4):703 - 4. [11] Kallen K. Maternal smoking and craniosynostosis. Teratology 1999;60:146 - 50. [12] Kane AA. An overview of craniosynostosis. J Prosth Orthot 2004;4:S50 – 5. [13] Lekovic GP, Bristol RE, Rekate HL. Cognitive impact of craniosynostosis. Semin Pediatr Neurol 2004;11:305 - 10. [14] Lima D. The management of deformational plagiocephaly: a review of the literature. J Prosth Orthot 2004;16(4):S9 – S14. [15] Marchac D, Renier D. Textbook of craniofacial surgery for craniosynostosis. Boston7 Little and Brown; 1982. [16] Marchac D, Renier D. Treatment of craniosynostosis in infancy. Clin Plast Surg 1987;14:61 - 72. [17] Nonaka Y, Oi S, Miyawaki T, et al. Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Childs Nerv Syst 2004;20:702 - 9. [18] Persing JA, Luce C. Remodeling techniques for immature and mature cranial vault bone: technical note. J Craniofac Surg 1990;1(3):147 - 9. [19] Persing JA, Jane JA. Treatment of syndromic and nonsyndromic bilateral coronal synostosis in infancy and childhood. Neurosurg Clin N Am 1991;2(3):655 - 63. [20] Polley J, Charbel F. Nonsyndromal craniosynostosis: longitudinal outcome following cranio-orbital reconstruction in infancy. Clin Plast Surg 1998;102:619. [21] Rannan-Eliya SV, Middleton JA, Wall SA. Functional implications of single suture craniosynostosis. Curr Paediatr 2002;12:199 - 205. [22] Reefhuis J, Honein MA, Shaw GM. Fertility treatments and craniosynostosis. California, Georgia, and Iowa, 1993-1997. Pediatrics 2003;111:1163 - 6. [23] Renier D, Brunet L, Marchac D. IQ and craniostenosis: evolution in treated and untreated cases. In: Marchac D, editor. Craniofacial surgery. Berlin7 Springer-Verlag; 1987. p. 114 - 17. [24] Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostosis. Childs Nerv Syst 2000;16:645 - 58. [25] Ririe D, David L, Glazier S, et al. Surgical advancement influences perioperative care: a comparison of two surgical techniques for sagittal craniosynostosis repair. Anesth Analg 2003;97:699 - 703. [26] Shaffrey ME, Persing JA, Delashaw JB, et al. Surgical treatment of metopic synostosis. Neurosurg Clin N Am 1991;2(3):621 - 7.
[27] Singer S, Bower C, Southall P, et al. Craniosynostosis in Western Australia, 1980-1994: a population-based study. Am J Med Genet 1999;83:382 - 7. [28] Speltz ML, Kapp-Simon KA, Cunningham M, Marsh J, Dawson G. Single suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol 2004;29(8):651 - 68. [29] Stephen MW. The pathogenesis of craniosynostosis in the fetus. Yonsei Med J 2001;42:646 - 59. [30] Sun PP, Persing JA. Craniosynostosis. In: Albright AL, Pollack IF, Adelson PD, editors. Principles and practice of pediatric neurosurgery. New York7 Thieme Medical; 1999. p. 219 - 42. [31] Whitacker LA, Bartlett SP, Schut L. Craniosynostosis: an analysis of the timing, treatment and complications in 164 consecutive patients. Plast Reconstr Surg 1987;80:195 - 212. [32] Whitacker LA, Munro IR, Salyer KE, et al. Combined report of problems and complications in 793 craniofacial operations. Plast Reconstr Surg 1987;64:198 - 203. [33] Williams K, Cohen S, et al. A longitudinal, statistical study of reoperation rates in craniosynostosis. Clin Plast Surg 1997;100:305.
Commentary This article by Ferreira et al summarizes a series over a 14-year period. From 1991 to 2005, 120 patients with nonsyndromic craniosynostosis were treated surgically and the variables sex, age, weight, deformity, surgical technique, volemia, blood loss, hospitalization time, and complications were analyzed. The morbidity and mortality seem to be similar to the literature, and there is a decrease of complications with multidisciplinary approach and appropriate training of the clinical surgical staff. The actual consensus is that the surgical treatment is to be done anytime between 3 and 6 months old. At this period of life, it is easier to proceed with the surgery because the bone is thinner, and even when there is a great PO skull bone absence, it will be appropriate with spontaneous coverage on growth. The main problem is intraoperative bleeding and the appropriate clinical management. As we saw in this series, 2 deaths happened because of hypovolemic shock. We emphasize that the anesthesiologist must have knowledge/ experience with the different nonsyndromic craniosynostosis intraoperative behavior and must also have knowledge/ experience in volume replacement in this kind of procedure to avoid hypotension and hypovolemic shock to the point of being able to perform, in advance, transfusion before having a hemoglobin level of less than 10.
Jose´ Marcus Rotta, MD, PhD Hospital do Servidor Pu´blico de Sa˜o Paulo Sa˜o Paulo, Brasil