Surgical treatment of craniosynostosis: a single institution s outcome analysis of 303 consecutive patients

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1 ARTIGO ORIGINAL Surgical treatment of craniosynostosis: a single institution s outcome analysis of 303 consecutive patients Tratamento cirúrgico das craniossinostoses: análise consecutiva de 303 pacientes operados na mesma instituição Ricardo Santos de Oliveira 1, Marcelo Volpon Santos 2, Antônio Augusto Velasco e Cruz 3, André Andó 4, João Monteiro de Pina Neto 5, Hélio Rubens Machado 6 RESUMO Objetivo: Análise retrospectiva do tratamento cirúrgico da craniossinostose ao longo de 30 anos na mesma instituição. Método: Revisão de prontuários médicos de 303 pacientes submetidos a 322 cirurgias para o tratamento de craniossinostose primária, de 1979 a Resultados: Foram estudados 216 (71,3%) pacientes do sexo masculino e 87 (28,7%) do feminino, com predomínio do sexo masculino nas trigonocefalias (86%) e escafocefalias (84,5%). Predomínio do sexo feminino foi observado no grupo braquicefalia (64,8%). Houve predominância do sexo masculino para os grupos não sindrômicos versus sindrômicos (p=0,0001). Nos casos não sindrômicos, a escafocefalia esteve presente em 134 (55,5 %), trigonocefalia em 38 (15,7%), plagiocefalia anterior em 35 (14,5%) e braquicefalia em 15 (6,2%). Em relação às formas sindrômicas, em 62 (20,5%) pacientes, a síndrome de Crouzon foi a mais frequente, ocorrendo em 21/62 casos (33,8%), Apert em 13/62 (20,9%), Pfeiffer em 9/62 (14,5%), Saethre Chotzen em 7/62 (11,2%) e outros tipos em 12/62 (19,3%). As formas de sinostoses nos casos sindrômicos mais frequentes foram: braquicefalia, crânio em trevo e sinostoses de múltiplas suturas (p=0,001). Complicações cirúrgicas foram observadas em 15 (5%) pacientes. Hemorragia intraoperatória e fístula liquórica foram as mais comuns. Houve 4 (1,3%) óbitos. O tempo de seguimento médio foi 3,7 anos. Os resultados cosméticos nos casos não sindrômicos foram classificados como tipo I em 168 (69,7%), II em 69 (28,6%) e III em 4 (1,6%) pacientes. Conclusões: Utilizando técnicas modernas de osteotomias e reconstruções ósseas, as craniossinostoses podem ser corrigidas satisfatoriamente, com bons resultados cosméticos e funcionais, e baixa morbidade e mortalidade em casos não sindrômicos. Descritores: Craniossinostose/cirurgia. Anormalidades craniofaciais. Cranio/anormalidades. 1. Professor livre-docente, Divisão de Neurocirurgia Pediátrica do Departamento de Cirurgia e Anatomia do HC-FMRPUSP. 2. Médico Assistente da Divisão de Neurocirurgia Pediátrica do HC- FMRPUSP. 3. Professor Titular, Divisão de Oculoplástica do Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço do HC-FMRPUSP. 4. Médico Assistente da Divisão de Cirurgia Plástica do HC-FMRPUSP. 5. Professor Titular do Departamento de Genética do HC-FMRPUSP. 6. Professor Titular, Divisão de Neurocirurgia Pediátrica do Departamento de Cirurgia e Anatomia do HC-FMRPUSP. Abstract Introduction: A retrospective review of a single institution s 30- year experience with surgery for primary craniosynostosis correction. Methods: The clinical data of 303 patients (322 surgeries) with primary craniosynostosis that have undergone surgery were retrospectively analyzed from 1979 to 2009 in the same institution. Results: There were 216 (71.3%) males and 87 (28.7%) females, with most of the male preponderance occurring due to the trigonocephaly (86%) and scaphocephaly (84.5%) groups. A female predominance was observed only in brachycephaly group (64.8%). There was a statistically significant male predominance between non-syndromic versus syndromic cases (p=0.0001). In non-syndromic forms, scaphocephaly was present in 134 (55.5%), trigonocephaly in 38 (15.7%), anterior plagiocephaly in 35 (14.5%), and brachycephaly in 15 (6.2%). A named syndrome was present in 62 (20.5%) patients. Crouzon syndrome was the most frequent, occurring in 21/62 (33.8%), Apert in 13/62 (20.9%), Pfeiffer in 9/62 (14.5%), Saethre Chotzen in 7/62 (11.2%), and other in 12/62 (19.3%). Genetic syndromes presented more commonly than expected with bilateral synostosis, the Kleeblattschadel deformity, and multiple suture synostosis (p=0.001). Complications were observed in 15 (5%) patients. Intraoperative hemorrhage and CSF leakage were the most common ones. There were 4 (1.3%) deaths. Morbidity was significantly associated with frontofacial advancement surgery. The mean follow-up was 3.7 years (9 months to 22 years). Morphological results in non-syndromic cases were classified in category I in 168 (69.7%), II in 69 (28.6%), and III in 4 (1.6%) cases. Conclusions: Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and relatively low morbidity and mortality, particularly for otherwise healthy, nonsyndromic infants. Key words: Craniosynostosis/surgery. Craniofacial abnormalities. Skull/abnormalities. Correspondência: Ricardo Santos de Oliveira Division of Pediatric Neurosurgery Ribeirão Preto School of Medicine, University of São Paulo - Campus Universitário - Ribeirão Preto, SP, Brazil - CEP rsoliveira@hcrp.fmrp.usp.br 206

2 Surgical treatment of craniosynostosis INTRODUction The modern era of the surgical treatment of craniosynostosis began in 1970, when plastic surgeons and neurosurgeons joined forces to form craniofacial teams and described new techniques to solve the morphological and the functional problems at the same time 1. Functional and morphological problems vary according to the type of craniosynostosis 2. Isolated craniosynostoses usually pose different problems than syndromic cases. In particular, faciocraniosynostoses carry out much more difficult problems, both in functional and morphological aspects 3. In isolated craniosynostoses, the goal of surgical correction is (i) to expand the intracranial volume, thereby allowing cerebral growth; and (ii) to improve the cosmetic appearance of the child 4,5. In this field, progress came from multidisciplinary teams involving neurosurgeons, plastic surgeons, pediatricians, geneticists, ophthalmologists, otorhinolaryngologists, psychologists and radiologists. A retrospective review of a single institution s 30 years experience with surgery for primary craniosynostosis correction was undertaken assessing multiple preoperative and operative variables. METHODS We retrospectively reviewed the cases of 303 children (322 surgeries) who were admitted consecutively to our service with primary craniosynostosis from January 1979 to December For the purposes of analyzing the patients outcome, medical charts, imaging findings, and operative notes were reviewed. This study was approved by the local ethical committee. Patients were classified based on the type of synostosis present: sagittal (scaphocephaly), metopic (trigonocephaly), unilateral coronal (anterior plagiocephaly), bilateral coronal (brachycephaly), true lambdoidal synostosis, oxicephaly, and multiple-suture synostosis (complex). We also divided the patients in non-syndromic cases and syndromic craniosynostoses (faciocraniosynostoses). Skull deformation due to positional plagiocephaly was excluded in this series. Operative Technique Operative procedures were customized for each patient with the following techniques and principles typically being followed: Scaphocephaly: A complete remodeling of the entire vault (total vertex craniectomy) was performed until 2001, and then simple craniectomies were performed along the sagittal suture and around the parietal region. Frontal and occipital barrel staving osteotomies with recontouring were typically performed in the most severe cases as well. We observed, in later follow-up, that in the total calvarectomy series there was a lack of bone in some patients due to incomplete bone reossification so we decided to change the surgical technique; Trigonocephaly: The V-shaped supraorbital bar was straightened and a new upper forehead was reconstructed using a bone flap taken from somewhere else on the vault, usually the posterior part of the forehead; Brachycephaly: These patients were treated with removal of both sides of the supraorbital bar with tenon extensions. The bulging of the temporal fossa was corrected by lifting a small bone flap and putting it back with the concavity outward. For unilateral coronal synostosis, a supraorbital bar with tenon extensions was used either unilaterally or bilaterally, depending on the attending surgeon s assessment of the degree of deformity. Lambdoidal stenosis was corrected via bilateral parietal-occipital craniotomies. The bone flaps were then reconfigured to produce the best posterior expansion. In multiple-suture synostosis, a cranial vault remodeling was performed with fronto-orbital advancement. Analysis of Operative Results For non-syndromic cases the morphological results were assessed using the following codes modified by Renier et al. 1 : 1 for excellent; 2 for incomplete, soft tissue or lesser bone contouring revisions desired; 3 for poor, necessitating surgical correction and 4 for failure (reoperation mandatory). Statistical analysis was performed using SPSS for Macintosh version 16.0 software (SPSS, Inc.). We compared categorical data using the chi-square test and the Fisher exact test, and the nonparametric Mann-Whitney U test between independent groups. A probability value was deemed significant at less than RESULTS The mean age of the 241 (79.5%) non-syndromic patients at the time of surgery was 9 months ± 1.2 years (range, 32 days 9.1 years) and in the 62 (20.5%) syndromic forms it was 1.6 years ± 2.3 years (range, 24 days-10.9 years) (p=0.034). The trigonocephaly (86%) and scaphocephaly (84.5%) groups were responsible for a male preponderance in non-syndromic cases, with a male/female proportion of 6:1 and 5:1, respectively (p=0.0001). A female predominance was observed only in the brachycephaly group (64.8%) (Figure 1). There was a statistically significant sex difference between non-syndromic versus syndromic cases (p=0.0001). Table 1 summarizes the percentages of the types of synostosis. The group was composed of sagittal (n= 136), metopic (n= 43), anterior plagiocephaly (n= 41), bilateral coronal (n= 37), oxycephaly (n= 17), lambdoidal (n= 11), complex (n= 11), and Kleeblattschadel (n= 7). Table 2 displays the various types of syndromes. The most common syndrome was Crouzon (n= 21) at 33.8%. Figure 2 represents each form of craniosynostosis in syndromic and non-syndromic group. Operative procedures were customized for each patient. For the sagittal synostosis group a total calvarectomy was performed in 81 (26.7%) patients until 2001, and then simple craniectomies were performed along the sagittal suture and around the frontal and parietal region in 39 (12.9%) patients. A complete frontocranial remodeling was performed in 145 (47.9%) cases. For the anterior plagiocephaly and 207

3 Figure 1 - Bar Graph representing syndromic and nonsyndromic cases according to gender. In non-syndromic patients a male predominance is evident, and there is a statistical correlation among groups (p=0.0001). Figure 2 - Bar Graph representing the main types of synostosis comparing syndromic (black bar) with non-syndromic group. In syndromic group bilateral synostosis, the Kleeblattschadel deformity, and multiple suture synostosis were more common than expected (p=0.001). Syndromic Gender Female Male Complex Kleeblattschadel Brachycephaly Syndromic Non-syndromic Oxycephaly Posterior Plagiocephaly Non-syndromic Anterior Plagiocephaly Trigonocephaly Scaphocephaly Number of cases Number of cases Table 1 - Distribution of syndromic and nonsyndromic craniosynostoses in the present authors series ( ). Type N % Scaphocephaly Trigonocephaly Anterior plagiocephaly Posterior plagiocephaly Oxycephaly Brachycephaly Kleeblattschädel Complex Total Table 2 - Types of syndromes (n=62). Syndrome N % Apert Crouzon Pfeiffer Saethre Chotzen Other* Total * Syndromes as nasofrontal dysplasia; Carpenter; mosaicism; Stickler syndrome brachycephaly cases, the advancement of supraorbital bar was done uni or bilaterally, respectively. Complex cases with multiple synostosis sutures were treated by total cranial vault remolding associated with fronto-orbital advancement. It is striking that the 7 cases of Kleeblattschädel or cloverleaf malformation were operated on at earlier age (median 92 days) than any other group. Due to the total sutural involvement and high risk of resultant damage to the central nervous system from increased intracranial pressure, these patients were treated quite urgently. In faciocraniosynostosis the classic management included initial anterior skull remodeling with frontoorbital advancement in 46 (74.1%) and later fronto-facial advancement (monobloc) as a second step in 6 patients (mean age at surgery was 5.7 years old). In those patients an internal device distraction were used in 5 and an external device in one patient. Two other young patients, a 4 monthold Pfeiffer type III syndrome child and a 7 month-old Apert patient were submitted to early monobloc surgery owing to severe respiratory problems associated with exorbitism with a transfacial pin and an internal device, respectively. In this series, 224 (74%) of patients received intraoperative and/or perioperative blood transfusion. The mean rate of blood transfusion was ml/kg in 98 patients (43.7%). We did not observe any major complication associated with blood transfusion. In syndromic patients progressive hydrocephalus was present in 7 patients with raised intracranial pressure treated by endoscopic third ventriculostomy (ETV) in 4 and VP-shunt in 3 patients. Eight patients were submitted to posterior fossa decompression for Chiari I malformation. 208

4 Surgical treatment of craniosynostosis Complications were observed in 15 (5%) patients. We did not observe any statistical correlation between nonsyndromic and syndromic patients (p=0.171). Intraoperative hemorrhage and CSF leakage were the most common. There were four (1.3%) deaths. In three of them the cause was severe hemorrhage and hypovolemic shock. Those cases were managed in 1985 (Scaphocephaly), 1986 (Kleeblattschädel), and 1992 (Complex synostosis). The other fatal case (Apert syndrome) of this series was due a severe larynx malformation and consequent respiratory problems in postoperative period. Recurrence as pansynostosis was observed in eight patients, who needed a second surgery. The mean follow-up was 3.7 years (9 months to 22 years). Morphological results in non-syndromic cases were classified in category I in 168 (69.7%), II in 69 (28.6%), and III in 4 (1.6%) cases. DISCUSSION The optimal timing of craniosynostosis repair depends on a multitude of factors, which include the presence of elevated intracranial hypertension, the operative technique employed, and surgeon preference 6,7. The aim of surgical intervention in craniosynostosis is to excise the prematurely fused suture and correct the associated deformities of the calvaria. Uncorrected synostosis quite frequently is associated with an increase in intracranial pressure 8. Although it is currently considered that surgery is indicated mainly for cosmetic reasons in isolated craniosynostoses, the functional aspects of the treatment must not be underestimated. Increased intracranial hypertension is more frequent in craniosynostoses affecting several sutures 9,10. It is now generally accepted that operations for craniosynostosis should be done as early as possible. However, these operations are frequently major craniofacial surgeries with unavoidable and often extensive blood loss 11. In the current series 74% of patients received blood transfusion without any problems related. In simple craniosynostosis the mean age at surgery was 9 months whilst in syndromic patients it was 1.6 years. Our preference is to perform surgery as soon as possible to prevent the effects of intracranial hypertension in the developing brain. In our series we found a striking male preponderance among the sagittal and metopic synostosis patients. That finding is not well understood. Graham et al. 12 pointed out that fetal head constraint might contribute to sagittal synostosis. The argument is that the increased occurrence of sagittal synostosis in males is related to larger fetal head size during the third trimester of pregnancy, resulting in a higher degree of physical constraint of the head in the maternal pelvis. In non-syndromic cases, the standard surgical techniques published by others was sufficient to obtain good results in late outcome 1. In our series almost 70% of patients reached an excellent morphological result with only one surgical procedure. Most craniosynostoses were treated by frontocranial remodeling, including some cases of scaphocephaly. In cases of harmonious craniosynostoses with intracranial hypertension a simple decompression was performed, using free flaps. The purpose of frontocranial remodeling was to restore the normal anatomy of the forehead and cranial vault and to allow normal growth of the brain. Traditionally, faciocraniosynostoses were managed by a two-stage strategy with a fronto-orbital advancement in the first year of life and a delayed facial advancement (LeFort III osteotomy) 13. Initially, the morbidity of the classic approach of frontofacial monobloc advancement was high. In the Necker series the rate of frontal infections was close to 30% 3,13. The use of internal distraction devices has allowed a simultaneous treatment in a single stage 14. The efficacy of the procedure has increased since then and, in the same time, its morbidity was reduced to an acceptable rate 13. Indeed, the gradual advancement obtained by distraction reduces the retrofrontal dead space and allows the occurrence of a progressive anterior expansion of the dura and brain. A significant improvement at the level of skull expansion, exorbitism correction, and breathing obstruction can thus be achieved even in very young patients 13,15. In our series, fronto-orbital advancement was performed as a first step in most of syndromic cases (74.1%). After 2003 we started our program for distraction in syndromic patients. Eight patients were treated by distraction using internal or external devices. In seven of them functional and aesthetical results were excellent. In syndromic patients a progressive hydrocephalus was present in 7 cases and Chiari I malformation in 8 patients. The timing of shunting in relation to surgery for synostosis is a matter of additional concern since the stability of the cranial reconstruction may be endangered if the dural envelope does not rapidly expand, due to the artificial depletion of cerebrospinal spaces, and the cranial content fails to support the bone flaps 16. Therefore, taking the dynamics of bone healing and brain growth into account, it is reasonable to postpone any reconstructive cranial procedure after hydrocephalus treatment until intracranial hypertension recurs. In our policy an ETV should be done if feasible because the intracranial pressure decreases more slowly than with a VP-shunt. Patients with Chiari malformation can remain asymptomatic throughout life; however, more than one third of patients become symptomatic or develop a syringomyelic cavity 17. Symptoms, even if occurring later in life, can be lifethreatening, especially if they occur in very young children. For selected cases posterior fossa decompression should be performed. Our global complication rate was 5% and recurrence as pansynostosis was observed in eight (2.6%) patients, who needed a second surgery. Pearson et al. 4 reported a total major reoperation rate in 22.9% and 19.5% in nonsyndromic rate. This number is higher than the comparative data of McCarthy et al. 18 (13%) and Williams et al. 19 (5%). These authors also point out that rigid fixation with metallic wires, plates, and screws could be associated with a high rate of complications in children and described that all other ways of fixation had an increased risk of major reoperation (n=49; 28%) when compared with resorbable plating (n=23; 17%). We disagree partially with these authors because, in our series, we used wires to fix a supra-orbital bar in the forehead in a large number of patients, and in only one patient we needed 209

5 to remove the wire. In the current series we did not use plates or screws. Resorbable plates are an excellent option to stabilize fronto-orbital advancement but their price is too high to allow widespread use in a public hospital. CONCLUSIONS In this large series the functional and the cosmetic results are better after early surgery and operative risks are not higher in infants than in older children independent of which suture is stenosed. The principles of the surgical treatment of monosutural craniosynostosis have practically remained unchanged in our center. On the other hand the management of faciocraniosynostosis has greatly changed. It was in fact greatly modified by the application of the distraction techniques to craniofacial surgery. The complexity of the theoretical and practical armamentarium nowadays necessary for treating craniosynostoses emphasizes the referral of patients to supraregional centers with multidisciplinary craniofacial teams which include geneticists, pediatric neurosurgeons, maxillofacial surgeons, head and neck specialists, intensive care pediatricians, oculoplastic surgeons and psychologists, in order to assure that the child with craniosynostosis is receiving the best available treatment. REFERENCES 1. Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Childs Nerv Syst. 2000;16(10-11): Cohen MM Jr. Craniosynostosis and syndromes with craniosynostosis: incidence, genetics, penetrance, variability, and new syndrome updating. Birth Defects Orig Artic Ser. 1979;15(5B): Di Rocco F, Arnaud E, Meyer P, Sainte-Rose C, Renier D. Focus session on the changing epidemiology of craniosynostosis (comparing two quinquennia: and ) and its impact on the daily clinical practice: a review from Necker Enfants Malades. Childs Nerv Syst. 2009;25(7): Pearson GD, Havlik RJ, Eppley B, Nykiel M, Sadove AM. Craniosynostosis: a single institution s outcome assessment from surgical reconstruction. J Craniofac Surg. 2008;19(1): Slater BJ, Lenton KA, Kwan MD, Gupta DM, Wan DC, Longaker MT. Cranial sutures: a brief review. Plast Reconstr Surg. 2008;121(4):170e-8e. 6. Persing JA. MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg. 2008;121(4 Suppl): Posnick JC. Craniofacial dysostosis. Staging of reconstruction and management of the midface deformity. Neurosurg Clin N Am. 1991;2(3): Panchal J, Uttchin V. Management of craniosynostosis. Plast Reconstr Surg. 2003;111(6): Renier D, Sainte-Rose C, Marchac D, Hirsch JF. Intracranial pressure in craniostenosis. J Neurosurg. 1982;57(3): Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C. Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review. Childs Nerv Syst. 2005;21(10): Dahmani S, Orliaguet GA, Meyer PG, Blanot S, Renier D, Carli PA. Perioperative blood salvage during surgical correction of craniosynostosis in infants. Br J Anaesth. 2000;85(4): Graham JM Jr, desaxe M, Smith DW. Sagittal craniostenosis: fetal head constraint as one possible cause. J Pediatr. 1979; 95(5 Pt 1): Arnaud E, Marchac D, Renier D. Reduction of morbidity of the frontofacial monobloc advancement in children by the use of internal distraction. Plast Reconstr Surg. 2007;120(4): Alonso N, Munhoz AM, Fogaça W, Ferreira MC. Midfacial advancement by bone distraction for treatment of craniofacial deformities. J Craniofac Surg. 1998;9(2): de Jong T, Bannink N, Bredero-Boelhouwer HH, van Veelen ML, Bartels MC, Hoeve LJ, et al. Long-term functional outcome in 167 patients with syndromic craniosynostosis; defining a syndrome-specific risk profile. J Plast Reconstr Aesthet Surg. 2010;63(10): Collmann H, Sörensen N, Krauss J. Hydrocephalus in craniosynostosis: a review. Childs Nerv Syst. 2005;21(10): Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21(10): McCarthy JG, Glasberg SB, Cutting CB, Epstein FJ, Grayson BH, Ruff G, et al. Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis: results and unsolved problems. Plast Reconstr Surg. 1995;96(2): Williams JK, Cohen SR, Burstein FD, Hudgins R, Boydston W, Simms C. A longitudinal, statistical study of reoperation rates in craniosynostosis. Plast Reconstr Surg. 1997;100(2): Paper performed at Craniofacial Unit, Hospital das Clínicas, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil. Article received: 1/7/2010 Article approved: 28/9/