Original Paper Pediatr Neurosurg 2014–15;50:119–127 DOI: 10.1159/000381860
Received: September 10, 2014 Accepted after revision: March 19, 2015 Published online: May 7, 2015
The Total Calvarial Remodeling with Transsutural Distraction Osteogenesis of 21 Cases of Craniosynostosis: New, Efficient, Safe and Natural Method in Craniosynostosis Surgery Dong Ha Park b Soo Han Yoon a Departments of a Neurosurgery and b Plastic and Reconstructive Surgery, Ajou University School of Medicine, Suwon, South Korea
Abstract Introduction: The majority of the present distraction osteogenesis techniques involve local site expansion that only produces localized decompression and affords limited decompression and cosmetic results. We designed a new surgical procedure, total calvarial transsutural distraction osteogenesis (TSuDO). Methods: We performed total calvarial TSuDO surgical procedures in 21 children. The total calvarial TSuDO method consisted of suturectomy and distraction for the sagittal, bicoronal and bilambdoid sutures. Results: The mean surgery duration was 110 ± 16 min, the mean transfusion volume was 38 ± 45 ml, an average 4.1 ± 0.4 distractors were applied with a mean latency period of 3.3 ± 0.9 days. The mean activation period was 45 ± 18 days with a consolidation period of 54 ± 23 days. There were 3 complication cases of early removal of the distractors: 1 boy with transient 6th cranial nerve palsy accompanied by fever and 2 children with mild pus discharge from the distractor sites. Preoperative lumbar puncture pressures decreased significantly after distractor removal secondary to surgery (p < 0.001). The preoperative cranium size increased significantly (p < 0.001). Conclusion: Total calvarial TSuDO is a simple and safe pro-
© 2015 S. Karger AG, Basel 1016–2291/15/0503–0119$39.50/0 E-Mail [email protected] www.karger.com/pne
cedure that may produce wide generalized decompression and good cranial configurations that most closely resemble normal skulls. © 2015 S. Karger AG, Basel
Introduction
The first experimental animal trial of distraction surgery for craniosynostosis was in 1993 by Barone et al. [1] who performed cranial distraction in rabbits. This distraction osteogenesis (DO) procedure has been conducted in the mid-1990s, which although entailing disadvantages of a secondary procedure, also has advantages such as short surgery duration, less bleeding and a low risk of postoperative infection, and absence of a dead space [2]. These surgical merits have led to the development of various DO applications such as classical DO, rotating DO, anterior cranial DO, posterior cranial DO and transsutural distraction osteogenesis (TSuDO) [3–11]. However, the majority of surgical DO techniques developed to date consist of localized cranial expansion which may result in localized subdural space expansion while brain pressure is unrelieved in those portions where expansion is absent, resulting in insufficient brain development and uneven skull and brain growth that may produce developmental delay and poor cosmetic results. In other words, such a variety in the adSoo Han Yoon Department of Neurosurgery, Ajou University School of Medicine Yongtong-Gu, World Cup Road 164 Suwon 443-721 (Korea) E-Mail ee80 @ ajou.ac.kr
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Key Words Cranial expansion · Craniosynostosis · Distraction osteogenesis · Intracranial pressure · Surgical technique
Table 1. Demographic data of 21 patients with craniosynostosis
Type of craniosynostosis
1 recurrent right unilateral coronal 2 recurrent sagittal 3 recurrent bicoronal 4 recurrent bicoronal 5 recurrent right lambdoid 6 Crouzon 7 Crouzon 8 Crouzon 9 Crouzon 10 Crouzon 11 Crouzon 12 Crouzon 13 Pfeiffer 14 microcephalic sagittal 15 microcephalic sagittal 16 microcephalic sagittal 17 microcephalic sagittal bicoronal 18 sagittal bilambdoid 19 sagittal bicoronal 20 postshunt sagittal bicoronal 21 postshunt left coronal Total or Mean ± SD
Sex
Gestational age, weeks
Birth weight, g
Age, months
boy girl boy girl girl boy boy boy boy boy girl boy girl boy boy girl boy boy girl boy boy boys = 14, girls = 7
39 40 40.3 31.3 37.2 38 41.3 38.4 38 38 41.1 39.4 37 34.3 40.5 38 29 40 40 38 25.6 37.3±4.1
3,300 2,830 2,440 1,380 2,650 3,450 4,000 3,540 3,400 3,360 3,000 3,400 2,820 1,600 3,100 2,700 660 3,100 2,300 3,000 920 2,712±888
59 45 15 15 15 9 30 18 57 34 20 16 49 29 23 29 43 50 55 36 20 31.8±15.9
vances of DO surgical techniques has allowed DO surgery for simple craniosynostosis, but it still remains insufficient for patients with multiple craniosynostoses, i.e. the presence of simultaneous anterior cranial and posterior cranial suture craniosynostosis, syndromic craniosynostosis accompanied by severe intracranial pressure (ICP) elevation, or recurrent craniosynostosis with Chiari anomalies. These children would thus require standard craniotomy and remodeling or DO of the anterior cranial and posterior cranial sutures separately in a staged operation [12–14]. Here, we developed a new total calvarial TSuDO method for generalized anterior and posterior expansion so as to allow overall cranial growth that closely resembles normal growth configurations. In the present study, we conducted total calvarial TSuDO that consists of removal and expansion of all sutures in 21 craniosynostosis children who required generalized wide decompression, and report the favorable outcomes that were observed.
Materials and Methods Patient Selection Twenty-one craniosynostosis children (14 boys, 7 girls) treated with total calvarial TSuDO at the Ajou University Hospital during 19 months from April 2012 to October 2013 were selected for study. Patient selection criteria were (1) children with multiple,
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syndromic, recurrent or secondary suture craniosynostosis who required generalized expansion, (2) older microcephalic or postshunt children of more than 2 years who required generalized expansion, (3) children with recurrent or postshunt craniosynostosis and Chiari anomaly who required generalized expansion, (4) children with recurrent, microcephalic or postshunt craniosynostosis and high ICP who required generalized expansion. Among these 21 children, there were 5 with recurrent craniosynostosis (1 with sagittal craniosynostosis, 1 with unilateral coronal craniosynostosis, 2 with bicoronal craniosynostosis, 1 with lambdoid craniosynostosis), 2 with multiple craniosynostoses, 8 with syndromic craniosynostosis, 2 with postshunt craniosynostosis and 4 with microcephalic craniosynostosis. All 5 children with recurrent craniosynostosis had the first operation of distraction osteogenesis for craniosynostosis 1–2 years ago. All 4 children with microcephalic craniosynostosis did not have other accompanying anomalies. The mean gestational age was 37.3 ± 4.1 weeks, the mean birth weight was 2,712 ± 888 g, and the mean age was 31.8 ± 15.9 months. Each individual child’s characteristics are described in table 1. Cranial Expansion Surgery – Total Calvarial TSuDO Total calvarial TSuDO is identical to previous TSuDO except that the former comprises removal of all sutures, i.e. bicoronal, sagittal and bilambdoid sutures, and that all sutures are widened with the use of distractors [15]. Skin incisions are extended Tshaped incisions on the coronal suture and sagittal suture, in which the periosteum is separated from the skull. This is followed by resection of the bicoronal, sagittal and the bilambdoid sutures in 5- to 8-mm thicknesses with a burr hole and air drill saw, or an air drill without a burr hole. The coronal suture is resected up to the meeting point of the bilateral coronal suture and the temporal
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Patient No.
Color version available online
a
b
c
d
b Case 2: A 45 month old girl with recurrent sagittal craniosynostosis. c Case 6: A 9 month old boy with Crouzon syndrome. d Case
ages of 4 children showing immediate postoperative predistraction state of total cranial TSuDO that includes bicoronal, sagittal and bilambdoid suturectomy and distractor application. a Case 1: A 59 month old boy with recurrent right unicoronal craniosynostosis.
20: A 36 month old boy with postshunt sagittal and bicoronal secondary craniosynostosis.
suture, while the sagittal suture is wholly resected. The lambdoid suture is resected up to the asterion which is the meeting point of the parietomastoid and occipitomastoid sutures, or less than 1 cm (fig. 1). Before a child is 2 years old, the asterion does not close and therefore the asterion does not need to be resected, but older children may need the asterion to be removed by craniectomy. The exposed sagittal sinus and dura were protected by a covering synthetic dura, and 1 distractor was placed on one coronal suture side and one lambdoid suture side, and 2–3 distractors on the sagittal suture. One closed drainage system was inserted into the subperiosteal space of the scalp which was then closed with sutures.
A 3- to 5-day latency period was observed according to postoperative conditions including postoperative bleeding and wound swelling, and 0.15- to 0.75-mm activation was performed daily. A 3-dimensional reconstructed CT scan was conducted every 1–3 weeks to monitor if the expansion was adequate (fig. 2). The activation period lasted 1–3 months and after sufficient expansion had been confirmed, the activation was completed followed by a 1- to 3-month consolidation period, after which the distractors, fixation plates and screws were all removed. In the second surgery for distractor removal, absorbable plates were placed in cases where bone flap instability was suspected.
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Pediatr Neurosurg 2014–15;50:119–127 DOI: 10.1159/000381860
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Fig. 1. The 3 dimensional reconstructed computed tomogram im-
Color version available online
a
b
c
d
showing the postoperative postdistraction state of total cranial TSuDO that results in widely distracted bicoronal, sagittal and bilambdoid suturectomy sites. a Case 1: a 59-month-old boy with recurrent right unicoronal craniosynostosis. b Case 2: a 45-month-
Clinical Examination and Follow-Up The patients underwent preoperative and immediate postoperative EEG, MRI, 3-dimensional reconstructed CT scans and visual evoked potential. The ophthalmology department was consulted for fundus examination and intraocular pressure measurements. Chiari malformation cases included were those that demonstrated a cerebellar descent of more than 2 mm from the foramen magnum. ICP was preoperatively measured by lumbar puncture under general anesthesia, and this procedure was repeated during the secondary surgery for the distractor removal.
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old girl with recurrent sagittal craniosynostosis. c Case 6: a 9-month-old boy with Crouzon syndrome. d Case 20: a 36-monthold boy with postshunt sagittal and bicoronal secondary craniosynostosis.
Results
The mean anesthesia duration was 217 ± 42 min, the mean surgery duration was 110 ± 16 min, and the mean transfusion volume was 38 ± 45 ml. No transfusions were given in 8 of the 21 children (38%). Cranial size was –0.67 ± 2.26 SD at the time of birth, and –1.33 ± 1.57 SD before total calvarial TSuDO, which was smaller than the birth size but not statistically significant (p = 0.48). The Park/Yoon
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Fig. 2. The 3-dimensional reconstructed CT images of 4 children
Table 2. Intraoperative factors and head circumferences in 21 cases with total calvarial TSuDO surgeries
Patient No.
Anesthetic time, min
Operation time, min
Transfusion volume, ml
Head circumference at birth, SD
Preoperative head circumference, SD
Postoperative head circumference, SD
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD
215 215 170 250 200 295 195 240 210 230 215 210 350 190 230 180 170 200 190 195 210 217±42
120 105 100 135 105 135 120 115 90 135 125 115 115 95 110 80 80 110 100 110 100 110±16
50 100 0 60 0 100 30 20 0 0 0 30 160 0 30 0 0 100 30 60 30 38±45
–0.5 0.5 –1.0 –3.7 1.0 0.5 0.5 1.0 n.a. 0.5 n.a. 0.5 0.5 –3.0 –0.8 n.a. –7.0 1.0 n.a. n.a. n.a. 0.67±2.26
–1.8 0.3 –1.5 –0.3 1.0 1.5 –1.3 0.8 –1.5 –1.9 –2.3 –1.5 –3.6 –2.4 –2.5 –2.2 –2.2 –0.9 –1.8 0.9 –4.7 –1.33±1.57
–1.5 1.6 –1.0 0.8 1.0 2.7 –0.8 1.0 –0.4 –1.3 –1.5 –1.0 –3.3 –1.6 –1.7 –2.1 –1.9 –0.9 –1.9 2.0 –5.1 –0.80±1.83
n.a. = Not assessed.
cranial size increased significantly after surgery to –0.80 ± 1.83 SD (p < 0.001; table 2). An average of 4.1 ± 0.4 distractors were used per patient, the mean latency period was 3.3 ± 0.9 days, and the mean activation period was 45 ± 18 days. The mean total distraction length was sagittal 12.5 ± 2.8 mm, coronal 8.1 ± 1.9 mm and lambdoid suture 7.7 ± 2.2 mm, and the mean consolidation period was 54 ± 23 days (table 3). The mean surgery duration for distractor removal was 42 ± 15 min. Among the total of 21 patients with 10 (48%) Chiari malformations that were found by MRI before or immediately after surgery, there were 3 children who showed pale fundus, papilledema in 8 patients, demonstrating a total of 11 among 21 children (52%) with ocular abnormalities, but only 4 of 21 (19%) with abnormal visual evoked potential. The mean preoperative lumbar puncture levels were 26.7 ± 11.2 cm H2O, which showed elevated levels of >20 cm H2O in 13 of the 20 (65%) who were able to undergo the measurement, while 5 children showed slightly increased levels of 15–20 cm H2O, and the remaining 3 were below 15 cm H2O. The levels after secondary surgery for distractor removal were 15.1 ± 4.3 cm H2O, which was signifi-
cantly lower than the preoperative levels (p < 0.001; table 4). No mortalities were observed in this investigation, while a single neurological morbidity manifested as transient 6th cranial nerve palsy with fever during the consolidation period in a 57-month-old boy with Crouzon syndrome. The distractor was removed on the 16th consolidation day, the earliest among the 21 patients. He had recovered completely in the 1-month follow-up examination. Two other children experienced mild pus discharge from the distractor sites, and the distractor was removed earlier than usual on the 24th and 28th consolidation days, without presence of pus or other wound complications after distractor removal. The cranial contours of all patients were maintained 1 year after surgery with adequate bone fusion and without instability or major defects (fig. 3).
Surgical methods for the correction of craniosynostosis began as tunnel craniectomy or craniotomy in the 1890s which progressed to craniotomy and remodeling
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Discussion
Table 3. Distraction procedures in 21 cases with total calvarial TSuDO
Patient No.
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD
Distractors, n
5 4 4 4 4 4 4 4 5 4 4 4 5 4 4 4 4 4 4 4 4 4.1±0.3
Latency period, days
Activation period, days
Consolidation period, days
Total distraction length, mm sagittal
coronal
lambdoid
3 3 3 5 3 2 3 3 3 2 5 3 5 3 5 3 3 3 3 3 3 3.1±1.1
34 51 45 39 53 36 48 27 18 42 54 27 37 40 37 105 38 52 44 47 74 44±17
53 56 61 52 82 24 89 30 16 57 67 28 60 48 56 25 85 43 57 102 44 54±22
15.8 12.4 13.0 12.3 14.4 11.6 13.8 8.2 5.1 11.9 12.3 9.1 18.4 12.3 12.3 14.7 12.8 12.4 12.6 11.9 15.8 12.5±2.8
8.0 8.6 7.4 6.8 6.0 9.5 10.2 8.2 3.3 7.4 7.0 7.4 7.5 6.1 8.0 12.1 7.9 9.3 9.6 10.0 10.0 8.1±1.9
4.6 6.7 7.4 6.8 8.9 9.5 7.0 8.2 3.9 5.4 6.1 8.4 13.5 4.2 8.0 7.5 9.3 9.3 9.6 8.4 10.0 7.7±2.2
VEP
L-P, cm H2O
Table 4. Clinical findings in 21 cases with total calvarial TSuDO
Patient No.
Chiari anomaly
Papilledema
preoperative 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD t test
– – + + + + + + + + – + – – – – – + – – – 10/21 (48%)
– – unilateral – – – bilateral bilateral bilateral bilateral bilateral bilateral unilateral – bilateral – – – unilateral – bilateral 11/21 (52%)
NL prolonged NL NL NL NL NL NL NL prolonged NL NL NL NL NL NL prolonged NL NL NL no wave 4/21 (19%)
49.5 39.0 33.5 31.5 31.5 41.0 35.5 11.5 20.5 25.0 17.5 37.5 21.0 20.0 18.5 18.5 18.0 13.0 11.5 39.5 n.a. 26.7±11.2
postoperative 11.5 9.0 15.5 16.0 17.0 19.5 15.0 n.a. 11.5 24.5 24.5 15.5 n.a. 11.5 17.0 10.5 13.5 12.5 12.0 15.0 n.a. 15.1±4.3 0.0003
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VEP = Visual evoked potential; L-P = lumbar puncture pressure; n.a. = nonavailable; NL = normal.
b
c
d
Fig. 3. The 3 dimensional reconstructed computed tomogram images of 4 children showing postoperative postdistraction 1 year state of total cranial TSuDO that result in active bone growth on the suturectomy sites. a Case 1: A 74 month old boy with recurred
right unicoronal craniosynostosis. b Case 2: A 62 month old girl with recurred sagittal craniosynostosis. c Case 6: A 24 month old boy with Crouzon syndrome. d Case 20: A 50 month old boy with postshunt sagittal and bicoronal secondary craniosynostosis.
from the 1960s [16–19]. The trend further developed to the popular minimal surgery from the 1990s. From the minimal surgery concept two new minimal surgical techniques for craniosynostosis have been described simultaneously in the late 1990s which included DO and endoscopic surgery [7, 20–22]. DO had progressed to an attempt to resect only a portion of the pathological suture and several methods of DO combined with the older method of craniotomy and remodeling [3– 5, 8, 9, 11, 23–25]. With regard to the apparatus, DO
with distractors developed to DO with springs and endoscopic resection techniques without any distraction [21, 22]. Such advances in minimal surgery provide safer surgery in terms of less bleeding and subsequent transfusions and shortening of surgery duration, but its implementation is still limited in children aged more than 2–3 years who require overall generalized cranial correction and expansion due to high ICP or Chiari anomaly. Consequently, patients more than 2–3 years of age should
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Among the total of 21 children who underwent total calvarial TSuDO, we experienced a patient with transient 6th cranial nerve palsy that has not been reported in the past literature. The transient 6th cranial nerve palsy was not present during the activation phase of the treatment but manifested as fever accompanied by transient 6th cranial nerve palsy after completion of the activation period. The distractor was removed on the 16th consolidation day, and the child fully recovered from the condition. The 3-dimensional reconstructed CT scan performed immediately after onset of the 6th cranial nerve palsy did not demonstrate any changes compared to the preoperative CT with regard to fractures or other anatomical abnormalities, and no other patient in this study has shown the same condition to date. We therefore consider that the 6th cranial nerve palsy is probably not directly related to the DO but made a prudent judgment to remove the distractor earlier than planned. Distractor pin site infections have ranged from 9 to 22% in previous reports, while we observed 2/21 (9.5%) such events that is similar to the above figures [10, 15, 26, 28]. Distractor breakages that have been previously described by the present authors have decreased to less than 1% of DO patients in our recent observations due to a new strengthened structure (Cranial distractor, Jeil Medical, Seoul, South Korea), and which did not occur in the present series [10, 15, 24, 26, 29].
Conclusion
We suggest that total calvarial TSuDO is probably more efficient, safer, generalized and natural in the expansion of the intracranial volume and to correct abnormal craniofacial contours especially in older craniosynostosis children with high ICP or Chiari anomaly.
Acknowledgment We are grateful to Dr. K.H. Chang for preparing the manuscript.
Disclosure Statement The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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undergo the older extensive craniotomy and remodeling method than minimal surgery such as DO and endoscopic craniectomy. Therefore, the posterior cranial DO and rotating DO were developed in the late 2000s, which were applicable to children aged older than 2–3 years and produced more generalized cranial correction and expansion [5, 6, 10, 14, 23, 26]. However, the surgery duration, bleeding volume and transfusions in the posterior cranial DO and rotating DO was still considered to be uncomfortable although these had decreased markedly compared to craniotomy and remodeling [10, 14, 26]. Also, the above two techniques result in more generalized cranial growth than simple DO that is still dissimilar to normal growth configurations from the viewpoint of abnormal curvatures or rims on the DO borders, suggesting greater localized expansion rather than generalized cranial growth. Another limitation is that the anterior and posterior cranium requires separate surgeries so that anterior and posterior staged DO for overall cranial expansion may not be advantageous with regard to simplicity and safety compared to craniotomy and remodeling. Recent researches have proposed resection of the whole cranium in several small fragments to expand the cranium in one procedure, but this may entail too large resection areas, prolonged surgical duration and an increased volume of transfusion [27]. In addition, brain injury may be possible until the ossification between fragments has become established and sufficient to endure daily traumas, and difficulties have also been suggested for simultaneous surgery for both the anterior and posterior cranium. The total calvarial TSuDO developed by the authors of this study has overcome many of the disadvantages and limitations described above: overall cranial expansion is feasible without separate anterior and posterior surgeries; the overall expansion restricts the formation of abnormal curvatures or rims on the DO borders that follow the majority of simple DO procedures; surgical duration is markedly shortened to 1–2 h; the average bleeding is only a minimal 30 ml such that about 40% of children did not require transfusions; the procedure is versatile such that it may be applicable to children aged up to 6–7 years, and only 4–5 distractors are used contributing to cost reduction and decreased distractor pin site infection. The most important and significant superiority of total calvarial TSuDO was the ability of this technique to produce overall generalized cranial expansion, but conversely this procedure may not be applicable in the rare case requiring only localized correction.
References
Total Calvarial Transsutural Distraction Osteogenesis for Craniosynostosis
11 Uemura T, Hayashi T, Satoh K, Mitsukawa N, Yoshikawa A, Suse T, Furukawa Y: Three-dimensional cranial expansion using distraction osteogenesis for oxycephaly. J Craniofac Surg 2003;14:29–36. 12 Esparza J, Hinojosa J: Complications in the surgical treatment of craniosynostosis and craniofacial syndromes: apropos of 306 transcranial procedures. Childs Nerv Syst 2008; 24:1421–1430. 13 Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C: Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst 2012;28:1511–1523. 14 White N, Evans M, Dover MS, Noons P, Solanki G, Nishikawa H: Posterior calvarial vault expansion using distraction osteogenesis. Childs Nerv Syst 2009;25:231–236. 15 Park DH, Yoon SH: The trans-sutural distraction osteogenesis for 22 cases of craniosynostosis: a new, easy, safe, and efficient method in craniosynostosis surgery. Pediatr Neurosurg 2011;47:167–175. 16 Greene CS Jr, Winston KR: Treatment of scaphocephaly with sagittal craniectomy and biparietal morcellation. Neurosurgery 1988; 23:196–202. 17 Jane JA, Edgerton MT, Futrell JW, Park TS: Immediate correction of sagittal synostosis. J Neurosurg 1978;49:705–710. 18 Mehta VA, Bettegowda C, Jallo GI, Ahn ES: The evolution of surgical management for craniosynostosis. Neurosurg Focus 2010;29:E5. 19 Sloan GM, Wells KC, Raffel C, McComb JG: Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics 1997;100:E2. 20 Do Amaral CM, Di Domizio G, Tiziani V, Galhardi F, Buzzo CL, Rinco T, Kharmandayan P, Bueno MA, Bolzani N, Sabbatini RM, Lopes LD, Lopes PF, Paiva B, Paiva RM, Turchiari LA: Gradual bone distraction in cranio-
21 22
23
24
25
26
27
28
29
synostosis. Preliminary results in seven cases. Scand J Plast Reconstr Surg Hand Surg 1997; 31:25–37. Jimenez DF, Barone CM: Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg 1998;88:77–81. Lauritzen C, Sugawara Y, Kocabalkan O, Olsson R: Spring mediated dynamic craniofacial reshaping. Case report. Scand J Plast Reconstr Surg Hand Surg 1998;32:331–338. Kim SW, Shim KW, Plesnila N, Kim YO, Choi JU, Kim DS: Distraction versus remodeling surgery for craniosynostosis. Childs Nerv Syst 2007;23:201–206. Lao WW, Denny AD: Internal distraction osteogenesis to correct symptomatic cephalocranial disproportion. Plast Reconstr Surg 2010;126:1677–1688. Nonaka Y, Oi S, Miyawaki T, Shinoda A, Kurihara K: Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Advantages, disadvantages, and current concepts for surgical strategy in the treatment of craniosynostosis. Childs Nerv Syst 2004;20:702–709. Park DH, Chung J, Yoon SH: Rotating distraction osteogenesis in 23 cases of craniosynostosis: comparison with the classical method of craniotomy and remodeling. Pediatr Neurosurg 2010;46:89–100. Sugawara Y, Uda H, Sarukawa S, Sunaga A: Multidirectional cranial distraction osteogenesis for the treatment of craniosynostosis. Plast Reconstr Surg 2010;126:1691–1698. Yonehara Y, Hirabayashi S, Sugawara Y, Sakurai A, Harii K: Complications associated with gradual cranial vault distraction osteogenesis for the treatment of craniofacial synostosis. J Craniofac Surg 2003;14:526–528. Lee JA, Park DH, Yoon SH, Chung J: Distractor breakage in cranial distraction osteogenesis for children with craniosynostosis. Pediatr Neurosurg 2008;44:216–220.
Pediatr Neurosurg 2014–15;50:119–127 DOI: 10.1159/000381860
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1 Barone CM, Ferder M, Jimenez DF, Grossman L, Hall C, Strauch B, Argamaso RV: Distraction of the frontal bone outside the cranial plane: a rabbit model. J Craniofac Surg 1993;4:177–181. 2 Cohen SR, Rutrick R, Burstein FD: Distraction osteogenesis in the human craniofacial skeleton: initial experience with new distraction system. J Craniofac Surg 1995;6:368–374. 3 Akai T, Iizuka H, Kawakami S: Treatment of craniosynostosis by distraction osteogenesis. Pediatr Neurosurg 2006;42:288–292. 4 Cho BC, Hwang SK, Uhm KI: Distraction osteogenesis of the cranial vault for the treatment of craniofacial synostosis. J Craniofac Surg 2004;15:135–144. 5 Choi JW, Koh KS, Hong JP, Hong SH, Ra Y: One-piece frontoorbital advancement with distraction but without a supraorbital bar for coronal craniosynostosis. J Plast Reconstr Aesthet Surg 2009;62:1166–1173. 6 Chung J, Yoon SH: Rotating distraction osteogenesis in a child with secondary craniosynostosis. J Craniofac Surg 2006;17:557–560. 7 Hirabayashi S, Sugawara Y, Sakurai A, Harii K, Park S: Frontoorbital advancement by gradual distraction. J Neurosurg 1998; 89: 1058–1061. 8 Imai K, Komune H, Toda C, Nomachi T, Enoki E, Sakamoto H, Kitano S, Hatoko M, Fujimoto T: Cranial remodeling to treat craniosynostosis by gradual distraction using a new device. J Neurosurg 2002;96:654–659. 9 Komuro Y, Yanai A, Hayashi A, Nakanishi H, Miyajima M, Arai H: Cranial reshaping employing distraction and contraction in the treatment of sagittal synostosis. Br J Plast Surg 2005;58:196–201. 10 Park DH, Chung J, Yoon SH: The role of distraction osteogenesis in children with secondary craniosynostosis after shunt operation in early infancy. Pediatr Neurosurg 2009; 45: 437–445.
Received: September 10, 2014 Accepted after revision: March 19, 2015 Published online: May 7, 2015
The Total Calvarial Remodeling with Transsutural Distraction Osteogenesis of 21 Cases of Craniosynostosis: New, Efficient, Safe and Natural Method in Craniosynostosis Surgery Dong Ha Park b Soo Han Yoon a Departments of a Neurosurgery and b Plastic and Reconstructive Surgery, Ajou University School of Medicine, Suwon, South Korea
Abstract Introduction: The majority of the present distraction osteogenesis techniques involve local site expansion that only produces localized decompression and affords limited decompression and cosmetic results. We designed a new surgical procedure, total calvarial transsutural distraction osteogenesis (TSuDO). Methods: We performed total calvarial TSuDO surgical procedures in 21 children. The total calvarial TSuDO method consisted of suturectomy and distraction for the sagittal, bicoronal and bilambdoid sutures. Results: The mean surgery duration was 110 ± 16 min, the mean transfusion volume was 38 ± 45 ml, an average 4.1 ± 0.4 distractors were applied with a mean latency period of 3.3 ± 0.9 days. The mean activation period was 45 ± 18 days with a consolidation period of 54 ± 23 days. There were 3 complication cases of early removal of the distractors: 1 boy with transient 6th cranial nerve palsy accompanied by fever and 2 children with mild pus discharge from the distractor sites. Preoperative lumbar puncture pressures decreased significantly after distractor removal secondary to surgery (p < 0.001). The preoperative cranium size increased significantly (p < 0.001). Conclusion: Total calvarial TSuDO is a simple and safe pro-
© 2015 S. Karger AG, Basel 1016–2291/15/0503–0119$39.50/0 E-Mail [email protected] www.karger.com/pne
cedure that may produce wide generalized decompression and good cranial configurations that most closely resemble normal skulls. © 2015 S. Karger AG, Basel
Introduction
The first experimental animal trial of distraction surgery for craniosynostosis was in 1993 by Barone et al. [1] who performed cranial distraction in rabbits. This distraction osteogenesis (DO) procedure has been conducted in the mid-1990s, which although entailing disadvantages of a secondary procedure, also has advantages such as short surgery duration, less bleeding and a low risk of postoperative infection, and absence of a dead space [2]. These surgical merits have led to the development of various DO applications such as classical DO, rotating DO, anterior cranial DO, posterior cranial DO and transsutural distraction osteogenesis (TSuDO) [3–11]. However, the majority of surgical DO techniques developed to date consist of localized cranial expansion which may result in localized subdural space expansion while brain pressure is unrelieved in those portions where expansion is absent, resulting in insufficient brain development and uneven skull and brain growth that may produce developmental delay and poor cosmetic results. In other words, such a variety in the adSoo Han Yoon Department of Neurosurgery, Ajou University School of Medicine Yongtong-Gu, World Cup Road 164 Suwon 443-721 (Korea) E-Mail ee80 @ ajou.ac.kr
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Key Words Cranial expansion · Craniosynostosis · Distraction osteogenesis · Intracranial pressure · Surgical technique
Table 1. Demographic data of 21 patients with craniosynostosis
Type of craniosynostosis
1 recurrent right unilateral coronal 2 recurrent sagittal 3 recurrent bicoronal 4 recurrent bicoronal 5 recurrent right lambdoid 6 Crouzon 7 Crouzon 8 Crouzon 9 Crouzon 10 Crouzon 11 Crouzon 12 Crouzon 13 Pfeiffer 14 microcephalic sagittal 15 microcephalic sagittal 16 microcephalic sagittal 17 microcephalic sagittal bicoronal 18 sagittal bilambdoid 19 sagittal bicoronal 20 postshunt sagittal bicoronal 21 postshunt left coronal Total or Mean ± SD
Sex
Gestational age, weeks
Birth weight, g
Age, months
boy girl boy girl girl boy boy boy boy boy girl boy girl boy boy girl boy boy girl boy boy boys = 14, girls = 7
39 40 40.3 31.3 37.2 38 41.3 38.4 38 38 41.1 39.4 37 34.3 40.5 38 29 40 40 38 25.6 37.3±4.1
3,300 2,830 2,440 1,380 2,650 3,450 4,000 3,540 3,400 3,360 3,000 3,400 2,820 1,600 3,100 2,700 660 3,100 2,300 3,000 920 2,712±888
59 45 15 15 15 9 30 18 57 34 20 16 49 29 23 29 43 50 55 36 20 31.8±15.9
vances of DO surgical techniques has allowed DO surgery for simple craniosynostosis, but it still remains insufficient for patients with multiple craniosynostoses, i.e. the presence of simultaneous anterior cranial and posterior cranial suture craniosynostosis, syndromic craniosynostosis accompanied by severe intracranial pressure (ICP) elevation, or recurrent craniosynostosis with Chiari anomalies. These children would thus require standard craniotomy and remodeling or DO of the anterior cranial and posterior cranial sutures separately in a staged operation [12–14]. Here, we developed a new total calvarial TSuDO method for generalized anterior and posterior expansion so as to allow overall cranial growth that closely resembles normal growth configurations. In the present study, we conducted total calvarial TSuDO that consists of removal and expansion of all sutures in 21 craniosynostosis children who required generalized wide decompression, and report the favorable outcomes that were observed.
Materials and Methods Patient Selection Twenty-one craniosynostosis children (14 boys, 7 girls) treated with total calvarial TSuDO at the Ajou University Hospital during 19 months from April 2012 to October 2013 were selected for study. Patient selection criteria were (1) children with multiple,
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syndromic, recurrent or secondary suture craniosynostosis who required generalized expansion, (2) older microcephalic or postshunt children of more than 2 years who required generalized expansion, (3) children with recurrent or postshunt craniosynostosis and Chiari anomaly who required generalized expansion, (4) children with recurrent, microcephalic or postshunt craniosynostosis and high ICP who required generalized expansion. Among these 21 children, there were 5 with recurrent craniosynostosis (1 with sagittal craniosynostosis, 1 with unilateral coronal craniosynostosis, 2 with bicoronal craniosynostosis, 1 with lambdoid craniosynostosis), 2 with multiple craniosynostoses, 8 with syndromic craniosynostosis, 2 with postshunt craniosynostosis and 4 with microcephalic craniosynostosis. All 5 children with recurrent craniosynostosis had the first operation of distraction osteogenesis for craniosynostosis 1–2 years ago. All 4 children with microcephalic craniosynostosis did not have other accompanying anomalies. The mean gestational age was 37.3 ± 4.1 weeks, the mean birth weight was 2,712 ± 888 g, and the mean age was 31.8 ± 15.9 months. Each individual child’s characteristics are described in table 1. Cranial Expansion Surgery – Total Calvarial TSuDO Total calvarial TSuDO is identical to previous TSuDO except that the former comprises removal of all sutures, i.e. bicoronal, sagittal and bilambdoid sutures, and that all sutures are widened with the use of distractors [15]. Skin incisions are extended Tshaped incisions on the coronal suture and sagittal suture, in which the periosteum is separated from the skull. This is followed by resection of the bicoronal, sagittal and the bilambdoid sutures in 5- to 8-mm thicknesses with a burr hole and air drill saw, or an air drill without a burr hole. The coronal suture is resected up to the meeting point of the bilateral coronal suture and the temporal
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Patient No.
Color version available online
a
b
c
d
b Case 2: A 45 month old girl with recurrent sagittal craniosynostosis. c Case 6: A 9 month old boy with Crouzon syndrome. d Case
ages of 4 children showing immediate postoperative predistraction state of total cranial TSuDO that includes bicoronal, sagittal and bilambdoid suturectomy and distractor application. a Case 1: A 59 month old boy with recurrent right unicoronal craniosynostosis.
20: A 36 month old boy with postshunt sagittal and bicoronal secondary craniosynostosis.
suture, while the sagittal suture is wholly resected. The lambdoid suture is resected up to the asterion which is the meeting point of the parietomastoid and occipitomastoid sutures, or less than 1 cm (fig. 1). Before a child is 2 years old, the asterion does not close and therefore the asterion does not need to be resected, but older children may need the asterion to be removed by craniectomy. The exposed sagittal sinus and dura were protected by a covering synthetic dura, and 1 distractor was placed on one coronal suture side and one lambdoid suture side, and 2–3 distractors on the sagittal suture. One closed drainage system was inserted into the subperiosteal space of the scalp which was then closed with sutures.
A 3- to 5-day latency period was observed according to postoperative conditions including postoperative bleeding and wound swelling, and 0.15- to 0.75-mm activation was performed daily. A 3-dimensional reconstructed CT scan was conducted every 1–3 weeks to monitor if the expansion was adequate (fig. 2). The activation period lasted 1–3 months and after sufficient expansion had been confirmed, the activation was completed followed by a 1- to 3-month consolidation period, after which the distractors, fixation plates and screws were all removed. In the second surgery for distractor removal, absorbable plates were placed in cases where bone flap instability was suspected.
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Fig. 1. The 3 dimensional reconstructed computed tomogram im-
Color version available online
a
b
c
d
showing the postoperative postdistraction state of total cranial TSuDO that results in widely distracted bicoronal, sagittal and bilambdoid suturectomy sites. a Case 1: a 59-month-old boy with recurrent right unicoronal craniosynostosis. b Case 2: a 45-month-
Clinical Examination and Follow-Up The patients underwent preoperative and immediate postoperative EEG, MRI, 3-dimensional reconstructed CT scans and visual evoked potential. The ophthalmology department was consulted for fundus examination and intraocular pressure measurements. Chiari malformation cases included were those that demonstrated a cerebellar descent of more than 2 mm from the foramen magnum. ICP was preoperatively measured by lumbar puncture under general anesthesia, and this procedure was repeated during the secondary surgery for the distractor removal.
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old girl with recurrent sagittal craniosynostosis. c Case 6: a 9-month-old boy with Crouzon syndrome. d Case 20: a 36-monthold boy with postshunt sagittal and bicoronal secondary craniosynostosis.
Results
The mean anesthesia duration was 217 ± 42 min, the mean surgery duration was 110 ± 16 min, and the mean transfusion volume was 38 ± 45 ml. No transfusions were given in 8 of the 21 children (38%). Cranial size was –0.67 ± 2.26 SD at the time of birth, and –1.33 ± 1.57 SD before total calvarial TSuDO, which was smaller than the birth size but not statistically significant (p = 0.48). The Park/Yoon
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Fig. 2. The 3-dimensional reconstructed CT images of 4 children
Table 2. Intraoperative factors and head circumferences in 21 cases with total calvarial TSuDO surgeries
Patient No.
Anesthetic time, min
Operation time, min
Transfusion volume, ml
Head circumference at birth, SD
Preoperative head circumference, SD
Postoperative head circumference, SD
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD
215 215 170 250 200 295 195 240 210 230 215 210 350 190 230 180 170 200 190 195 210 217±42
120 105 100 135 105 135 120 115 90 135 125 115 115 95 110 80 80 110 100 110 100 110±16
50 100 0 60 0 100 30 20 0 0 0 30 160 0 30 0 0 100 30 60 30 38±45
–0.5 0.5 –1.0 –3.7 1.0 0.5 0.5 1.0 n.a. 0.5 n.a. 0.5 0.5 –3.0 –0.8 n.a. –7.0 1.0 n.a. n.a. n.a. 0.67±2.26
–1.8 0.3 –1.5 –0.3 1.0 1.5 –1.3 0.8 –1.5 –1.9 –2.3 –1.5 –3.6 –2.4 –2.5 –2.2 –2.2 –0.9 –1.8 0.9 –4.7 –1.33±1.57
–1.5 1.6 –1.0 0.8 1.0 2.7 –0.8 1.0 –0.4 –1.3 –1.5 –1.0 –3.3 –1.6 –1.7 –2.1 –1.9 –0.9 –1.9 2.0 –5.1 –0.80±1.83
n.a. = Not assessed.
cranial size increased significantly after surgery to –0.80 ± 1.83 SD (p < 0.001; table 2). An average of 4.1 ± 0.4 distractors were used per patient, the mean latency period was 3.3 ± 0.9 days, and the mean activation period was 45 ± 18 days. The mean total distraction length was sagittal 12.5 ± 2.8 mm, coronal 8.1 ± 1.9 mm and lambdoid suture 7.7 ± 2.2 mm, and the mean consolidation period was 54 ± 23 days (table 3). The mean surgery duration for distractor removal was 42 ± 15 min. Among the total of 21 patients with 10 (48%) Chiari malformations that were found by MRI before or immediately after surgery, there were 3 children who showed pale fundus, papilledema in 8 patients, demonstrating a total of 11 among 21 children (52%) with ocular abnormalities, but only 4 of 21 (19%) with abnormal visual evoked potential. The mean preoperative lumbar puncture levels were 26.7 ± 11.2 cm H2O, which showed elevated levels of >20 cm H2O in 13 of the 20 (65%) who were able to undergo the measurement, while 5 children showed slightly increased levels of 15–20 cm H2O, and the remaining 3 were below 15 cm H2O. The levels after secondary surgery for distractor removal were 15.1 ± 4.3 cm H2O, which was signifi-
cantly lower than the preoperative levels (p < 0.001; table 4). No mortalities were observed in this investigation, while a single neurological morbidity manifested as transient 6th cranial nerve palsy with fever during the consolidation period in a 57-month-old boy with Crouzon syndrome. The distractor was removed on the 16th consolidation day, the earliest among the 21 patients. He had recovered completely in the 1-month follow-up examination. Two other children experienced mild pus discharge from the distractor sites, and the distractor was removed earlier than usual on the 24th and 28th consolidation days, without presence of pus or other wound complications after distractor removal. The cranial contours of all patients were maintained 1 year after surgery with adequate bone fusion and without instability or major defects (fig. 3).
Surgical methods for the correction of craniosynostosis began as tunnel craniectomy or craniotomy in the 1890s which progressed to craniotomy and remodeling
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Discussion
Table 3. Distraction procedures in 21 cases with total calvarial TSuDO
Patient No.
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD
Distractors, n
5 4 4 4 4 4 4 4 5 4 4 4 5 4 4 4 4 4 4 4 4 4.1±0.3
Latency period, days
Activation period, days
Consolidation period, days
Total distraction length, mm sagittal
coronal
lambdoid
3 3 3 5 3 2 3 3 3 2 5 3 5 3 5 3 3 3 3 3 3 3.1±1.1
34 51 45 39 53 36 48 27 18 42 54 27 37 40 37 105 38 52 44 47 74 44±17
53 56 61 52 82 24 89 30 16 57 67 28 60 48 56 25 85 43 57 102 44 54±22
15.8 12.4 13.0 12.3 14.4 11.6 13.8 8.2 5.1 11.9 12.3 9.1 18.4 12.3 12.3 14.7 12.8 12.4 12.6 11.9 15.8 12.5±2.8
8.0 8.6 7.4 6.8 6.0 9.5 10.2 8.2 3.3 7.4 7.0 7.4 7.5 6.1 8.0 12.1 7.9 9.3 9.6 10.0 10.0 8.1±1.9
4.6 6.7 7.4 6.8 8.9 9.5 7.0 8.2 3.9 5.4 6.1 8.4 13.5 4.2 8.0 7.5 9.3 9.3 9.6 8.4 10.0 7.7±2.2
VEP
L-P, cm H2O
Table 4. Clinical findings in 21 cases with total calvarial TSuDO
Patient No.
Chiari anomaly
Papilledema
preoperative 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Mean ± SD t test
– – + + + + + + + + – + – – – – – + – – – 10/21 (48%)
– – unilateral – – – bilateral bilateral bilateral bilateral bilateral bilateral unilateral – bilateral – – – unilateral – bilateral 11/21 (52%)
NL prolonged NL NL NL NL NL NL NL prolonged NL NL NL NL NL NL prolonged NL NL NL no wave 4/21 (19%)
49.5 39.0 33.5 31.5 31.5 41.0 35.5 11.5 20.5 25.0 17.5 37.5 21.0 20.0 18.5 18.5 18.0 13.0 11.5 39.5 n.a. 26.7±11.2
postoperative 11.5 9.0 15.5 16.0 17.0 19.5 15.0 n.a. 11.5 24.5 24.5 15.5 n.a. 11.5 17.0 10.5 13.5 12.5 12.0 15.0 n.a. 15.1±4.3 0.0003
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VEP = Visual evoked potential; L-P = lumbar puncture pressure; n.a. = nonavailable; NL = normal.
b
c
d
Fig. 3. The 3 dimensional reconstructed computed tomogram images of 4 children showing postoperative postdistraction 1 year state of total cranial TSuDO that result in active bone growth on the suturectomy sites. a Case 1: A 74 month old boy with recurred
right unicoronal craniosynostosis. b Case 2: A 62 month old girl with recurred sagittal craniosynostosis. c Case 6: A 24 month old boy with Crouzon syndrome. d Case 20: A 50 month old boy with postshunt sagittal and bicoronal secondary craniosynostosis.
from the 1960s [16–19]. The trend further developed to the popular minimal surgery from the 1990s. From the minimal surgery concept two new minimal surgical techniques for craniosynostosis have been described simultaneously in the late 1990s which included DO and endoscopic surgery [7, 20–22]. DO had progressed to an attempt to resect only a portion of the pathological suture and several methods of DO combined with the older method of craniotomy and remodeling [3– 5, 8, 9, 11, 23–25]. With regard to the apparatus, DO
with distractors developed to DO with springs and endoscopic resection techniques without any distraction [21, 22]. Such advances in minimal surgery provide safer surgery in terms of less bleeding and subsequent transfusions and shortening of surgery duration, but its implementation is still limited in children aged more than 2–3 years who require overall generalized cranial correction and expansion due to high ICP or Chiari anomaly. Consequently, patients more than 2–3 years of age should
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Among the total of 21 children who underwent total calvarial TSuDO, we experienced a patient with transient 6th cranial nerve palsy that has not been reported in the past literature. The transient 6th cranial nerve palsy was not present during the activation phase of the treatment but manifested as fever accompanied by transient 6th cranial nerve palsy after completion of the activation period. The distractor was removed on the 16th consolidation day, and the child fully recovered from the condition. The 3-dimensional reconstructed CT scan performed immediately after onset of the 6th cranial nerve palsy did not demonstrate any changes compared to the preoperative CT with regard to fractures or other anatomical abnormalities, and no other patient in this study has shown the same condition to date. We therefore consider that the 6th cranial nerve palsy is probably not directly related to the DO but made a prudent judgment to remove the distractor earlier than planned. Distractor pin site infections have ranged from 9 to 22% in previous reports, while we observed 2/21 (9.5%) such events that is similar to the above figures [10, 15, 26, 28]. Distractor breakages that have been previously described by the present authors have decreased to less than 1% of DO patients in our recent observations due to a new strengthened structure (Cranial distractor, Jeil Medical, Seoul, South Korea), and which did not occur in the present series [10, 15, 24, 26, 29].
Conclusion
We suggest that total calvarial TSuDO is probably more efficient, safer, generalized and natural in the expansion of the intracranial volume and to correct abnormal craniofacial contours especially in older craniosynostosis children with high ICP or Chiari anomaly.
Acknowledgment We are grateful to Dr. K.H. Chang for preparing the manuscript.
Disclosure Statement The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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undergo the older extensive craniotomy and remodeling method than minimal surgery such as DO and endoscopic craniectomy. Therefore, the posterior cranial DO and rotating DO were developed in the late 2000s, which were applicable to children aged older than 2–3 years and produced more generalized cranial correction and expansion [5, 6, 10, 14, 23, 26]. However, the surgery duration, bleeding volume and transfusions in the posterior cranial DO and rotating DO was still considered to be uncomfortable although these had decreased markedly compared to craniotomy and remodeling [10, 14, 26]. Also, the above two techniques result in more generalized cranial growth than simple DO that is still dissimilar to normal growth configurations from the viewpoint of abnormal curvatures or rims on the DO borders, suggesting greater localized expansion rather than generalized cranial growth. Another limitation is that the anterior and posterior cranium requires separate surgeries so that anterior and posterior staged DO for overall cranial expansion may not be advantageous with regard to simplicity and safety compared to craniotomy and remodeling. Recent researches have proposed resection of the whole cranium in several small fragments to expand the cranium in one procedure, but this may entail too large resection areas, prolonged surgical duration and an increased volume of transfusion [27]. In addition, brain injury may be possible until the ossification between fragments has become established and sufficient to endure daily traumas, and difficulties have also been suggested for simultaneous surgery for both the anterior and posterior cranium. The total calvarial TSuDO developed by the authors of this study has overcome many of the disadvantages and limitations described above: overall cranial expansion is feasible without separate anterior and posterior surgeries; the overall expansion restricts the formation of abnormal curvatures or rims on the DO borders that follow the majority of simple DO procedures; surgical duration is markedly shortened to 1–2 h; the average bleeding is only a minimal 30 ml such that about 40% of children did not require transfusions; the procedure is versatile such that it may be applicable to children aged up to 6–7 years, and only 4–5 distractors are used contributing to cost reduction and decreased distractor pin site infection. The most important and significant superiority of total calvarial TSuDO was the ability of this technique to produce overall generalized cranial expansion, but conversely this procedure may not be applicable in the rare case requiring only localized correction.
References
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11 Uemura T, Hayashi T, Satoh K, Mitsukawa N, Yoshikawa A, Suse T, Furukawa Y: Three-dimensional cranial expansion using distraction osteogenesis for oxycephaly. J Craniofac Surg 2003;14:29–36. 12 Esparza J, Hinojosa J: Complications in the surgical treatment of craniosynostosis and craniofacial syndromes: apropos of 306 transcranial procedures. Childs Nerv Syst 2008; 24:1421–1430. 13 Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C: Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst 2012;28:1511–1523. 14 White N, Evans M, Dover MS, Noons P, Solanki G, Nishikawa H: Posterior calvarial vault expansion using distraction osteogenesis. Childs Nerv Syst 2009;25:231–236. 15 Park DH, Yoon SH: The trans-sutural distraction osteogenesis for 22 cases of craniosynostosis: a new, easy, safe, and efficient method in craniosynostosis surgery. Pediatr Neurosurg 2011;47:167–175. 16 Greene CS Jr, Winston KR: Treatment of scaphocephaly with sagittal craniectomy and biparietal morcellation. Neurosurgery 1988; 23:196–202. 17 Jane JA, Edgerton MT, Futrell JW, Park TS: Immediate correction of sagittal synostosis. J Neurosurg 1978;49:705–710. 18 Mehta VA, Bettegowda C, Jallo GI, Ahn ES: The evolution of surgical management for craniosynostosis. Neurosurg Focus 2010;29:E5. 19 Sloan GM, Wells KC, Raffel C, McComb JG: Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics 1997;100:E2. 20 Do Amaral CM, Di Domizio G, Tiziani V, Galhardi F, Buzzo CL, Rinco T, Kharmandayan P, Bueno MA, Bolzani N, Sabbatini RM, Lopes LD, Lopes PF, Paiva B, Paiva RM, Turchiari LA: Gradual bone distraction in cranio-
21 22
23
24
25
26
27
28
29
synostosis. Preliminary results in seven cases. Scand J Plast Reconstr Surg Hand Surg 1997; 31:25–37. Jimenez DF, Barone CM: Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg 1998;88:77–81. Lauritzen C, Sugawara Y, Kocabalkan O, Olsson R: Spring mediated dynamic craniofacial reshaping. Case report. Scand J Plast Reconstr Surg Hand Surg 1998;32:331–338. Kim SW, Shim KW, Plesnila N, Kim YO, Choi JU, Kim DS: Distraction versus remodeling surgery for craniosynostosis. Childs Nerv Syst 2007;23:201–206. Lao WW, Denny AD: Internal distraction osteogenesis to correct symptomatic cephalocranial disproportion. Plast Reconstr Surg 2010;126:1677–1688. Nonaka Y, Oi S, Miyawaki T, Shinoda A, Kurihara K: Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Advantages, disadvantages, and current concepts for surgical strategy in the treatment of craniosynostosis. Childs Nerv Syst 2004;20:702–709. Park DH, Chung J, Yoon SH: Rotating distraction osteogenesis in 23 cases of craniosynostosis: comparison with the classical method of craniotomy and remodeling. Pediatr Neurosurg 2010;46:89–100. Sugawara Y, Uda H, Sarukawa S, Sunaga A: Multidirectional cranial distraction osteogenesis for the treatment of craniosynostosis. Plast Reconstr Surg 2010;126:1691–1698. Yonehara Y, Hirabayashi S, Sugawara Y, Sakurai A, Harii K: Complications associated with gradual cranial vault distraction osteogenesis for the treatment of craniofacial synostosis. J Craniofac Surg 2003;14:526–528. Lee JA, Park DH, Yoon SH, Chung J: Distractor breakage in cranial distraction osteogenesis for children with craniosynostosis. Pediatr Neurosurg 2008;44:216–220.
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1 Barone CM, Ferder M, Jimenez DF, Grossman L, Hall C, Strauch B, Argamaso RV: Distraction of the frontal bone outside the cranial plane: a rabbit model. J Craniofac Surg 1993;4:177–181. 2 Cohen SR, Rutrick R, Burstein FD: Distraction osteogenesis in the human craniofacial skeleton: initial experience with new distraction system. J Craniofac Surg 1995;6:368–374. 3 Akai T, Iizuka H, Kawakami S: Treatment of craniosynostosis by distraction osteogenesis. Pediatr Neurosurg 2006;42:288–292. 4 Cho BC, Hwang SK, Uhm KI: Distraction osteogenesis of the cranial vault for the treatment of craniofacial synostosis. J Craniofac Surg 2004;15:135–144. 5 Choi JW, Koh KS, Hong JP, Hong SH, Ra Y: One-piece frontoorbital advancement with distraction but without a supraorbital bar for coronal craniosynostosis. J Plast Reconstr Aesthet Surg 2009;62:1166–1173. 6 Chung J, Yoon SH: Rotating distraction osteogenesis in a child with secondary craniosynostosis. J Craniofac Surg 2006;17:557–560. 7 Hirabayashi S, Sugawara Y, Sakurai A, Harii K, Park S: Frontoorbital advancement by gradual distraction. J Neurosurg 1998; 89: 1058–1061. 8 Imai K, Komune H, Toda C, Nomachi T, Enoki E, Sakamoto H, Kitano S, Hatoko M, Fujimoto T: Cranial remodeling to treat craniosynostosis by gradual distraction using a new device. J Neurosurg 2002;96:654–659. 9 Komuro Y, Yanai A, Hayashi A, Nakanishi H, Miyajima M, Arai H: Cranial reshaping employing distraction and contraction in the treatment of sagittal synostosis. Br J Plast Surg 2005;58:196–201. 10 Park DH, Chung J, Yoon SH: The role of distraction osteogenesis in children with secondary craniosynostosis after shunt operation in early infancy. Pediatr Neurosurg 2009; 45: 437–445.
