ORIGINAL ARTICLE
Complications in 54 Frontofacial Distraction Procedures in Patients With Syndromic Craniosynostosis Jesse A. Goldstein, MD,* James Thomas Paliga, BA,† Jesse A. Taylor, MD,† and Scott P. Bartlett, MD†
Abstract: Patients with syndromic craniosynostosis manifest midfacial hypoplasia often treated by midfacial advancement. Benefits of midfacial advancement by distraction osteogenesis have been well studied; little is known about the perioperative morbidity of these procedures, specifically relating to device selection. This study compares the perioperative complications between semiburied- and halo-type distraction osteogenesis of the midface. A retrospective review was performed on all patients with syndromic craniosynostosis who underwent midface distraction with semiburied- or halo-type external distractors. Demographic information and operative/postoperative course were reviewed. Complications were categorized as hardwarerelated, infectious, and either as major (requiring additional intervention) or minor (requiring medication only). Chi-squared and Fisher exact test were used to compare variables. From 1999 to 2012, a total of 54 patients underwent midface distraction osteogenesis, including 23 patients with Apert syndrome, 19 patients with Crouzon syndrome, 10 patients with Pfeiffer syndrome, and 2 patients with other craniofacial syndromes. Thirtythree patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures.
The mean age during surgery was 8.0 (range, 4.0–17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. Thirty procedures were performed with external halotype distractors (18 Le Fort III and 12 monobloc distractions), whereas 25 were performed with buried midface distractors (16 Le Fort III and 9 monobloc distractions). There were no significant differences in diagnoses or interventions between the distraction devices. Of the 19 distractor-related complications, there were a total of 10 (18.2%) in the halo group including 5 (9.1%) requiring separate operative intervention as well as 9 (16.4%) in the buried distractor group including 6 (10.1%) requiring separate operative intervention. Major infections were more common in the buried distractor group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). There were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration related to postoperative positioning or falls and required operative repositioning. Frontofacial distraction is an important technique in patients with syndromic craniosynostosis. Higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with buried distractors. Key Words: Midface distraction osteogenesis, complications, external, buried, distractors
From the *Department of Plastic Surgery, The University of Pittsburgh Medical School, Children's Hospital of Pittsburgh, Pittsburgh; †Division of Plastic Surgery, The Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Received April 10, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Scott P. Bartlett, MD, Division of Plastic Surgery, The Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia, Colket Translational Research Bldg, 3501 Civic Center Blvd 9th Floor, Philadelphia, PA; E-mail: [email protected] Supported by the Department of Surgery of The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania. The authors report no conflicts of interest. Authorship Participation and Contribution Jesse A. Goldstein, MD: data analysis, data interpretation, and manuscript preparation J. Thomas Paliga, BA: data analysis, data interpretation, and manuscript preparation Jesse A. Taylor, MD: data interpretation and manuscript preparation Scott P. Bartlett, MD: study conception, data analysis, data interpretation, and manuscript preparation Presented at the 15th Congress of the International Society of Craniofacial Surgeons, September 10–14, 2013, Jackson Hole, WY. This study was reviewed and approved by the institutional review board of the Children's Hospital of Philadelphia. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001320
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BACKGROUND Patients with syndromic forms of craniosynostosis often manifest severe midfacial hypoplasia with characteristic exophthalmos and class 3 dental relations. Conventional frontofacial and midface advancement procedures such as the monobloc, facial bipartition, and the Le Fort III have been performed for more than 30 years to address these malformations by advancing the retrusive supraorbital bandeau, increasing projection of the infraorbital rim, zygoma, and maxilla as well as improving malocclusion.1–3 Introduced over the last 2 decades, distraction osteogenesis has been increasingly used. This technique allows for greater advancement paired with significantly lower complication rates compared with conventional methods.4–7 Today, many craniofacial surgeons prefer distraction osteogenesis to treat brow and midface deficiencies in patients with syndromic craniosynostosis.8,9 However, considerable variation exists among centers with respect to timing, protocol, and distraction device selection. Early distractors used in frontofacial procedures were modified from internal mandibular distractors being exclusively internal or semiburied in design.10–12 Recently, external- or halo-type distractors have gained popularity among craniofacial surgeons.13,14 There are currently many options when choosing a device, and each distractor has its own advantages and disadvantages (Table 1). Although semiburied distractors are well hidden
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Complications in Midface Distraction
infections). The validated Clavien-Dindo surgical classification system based on severity of complication was also applied (Table 2).15
TABLE 1. Device Comparison Semiburied Device
Halo-Type Device
Total distraction limited
Larger distraction lengths achievable Increased facial scarring Multivector, push/pull Vectors can be changed, molding easy Larger moment arm (pins/splints); requires better bone stock Increased social stigma Easier to remove, useful during infection
Minimal facial scarring One vector, push only No change in vector Small moment arm (plate to bone); requires less bone stock Less conspicuous Removal more difficult
and simple to operate, they allow for no postoperative vector adjustment. This limitation can result in overprojection of the lateral face compared with the central face, accentuating a “dish-faced” appearance. In contrast, external- or halo-type distractors allow for continuous postoperative vector adjustment and greater central facial expansion. The disadvantages, however, include being unsightly and prone to dislodgement. Although the benefits of frontofacial and midfacial advancement by distraction osteogenesis have been well studied, little has been written about the perioperative morbidity of these procedures, especially as they relate to device selection. This study was designed to (1) review distractor-associated complications in patients with syndromic forms of craniosynostosis undergoing frontofacial or midfacial advancement with distraction osteogenesis and (2) compare perioperative morbidity between the semiburied- and halo-type devices.
Surgical Procedures and Device Selection Patients found to have both retrusive brow and midface deformities were selected for monobloc or facial bipartition osteotomies. These procedures have been previously described.7,16 Briefly, after a coronal approach as well as periorbital and infratemporal dissection, a frontal craniotomy is performed by a neurosurgeon. Orbital and nasal osteotomies are performed, followed by pterygomaxillary disjunction and downfracture. After mobilization, the frontal bone is replated to the bandeau. For internal distractor application, 2 to 4 midface semiburied distractors are placed bilaterally at the level of the bandeau and the zygoma. For external halo-type distractor application, frontal and maxillary footplates and percutaneous wire fixation screws are placed and attached to the halo device with 24-gauge wire. Patients with isolated midface retrusion and acceptable brow position were selected for Le Fort III osteotomy. This procedure has been previously described.11,17,18 Briefly, after a coronal approach as well as periorbital and infratemporal dissection, orbital and nasal osteotomies are performed, followed by pterygomaxillary disjunction and downfracture. For internal distractor application, 2 midface semiburied distractors are placed bilaterally at the level of the zygoma. For external halo-type distractor application, maxillary footplates and percutaneous wire fixation screws are placed and attached to the halo device with 24-gauge wire. The distraction protocol was standardized regardless of osteotomy or device choice and included a 5-day latency period, followed by a 1-mm/d activation rate until correction was completed. A consolidation period of 6 to 8 weeks was used in all patients.
Statistical Analysis
METHODS
Demographic and operative characteristics were assessed with descriptive statistics. Fisher exact test was used to compare complication types between the distractor groups. An α of less than 0.05 was considered significant.
An institutional review board–approved retrospective review was performed on all patients with syndromic forms of craniosynostosis who underwent frontofacial or midface advancement using distraction osteogenesis for a 13-year period. Patients were included if they underwent monobloc or Le Fort III distraction with either external (halo-type) distractors or internal (semiburied) distractors. Patients with incomplete medical records and those who had conventional Le Fort III or monobloc osteotomies with acute advancements were excluded from this analysis. Demographic information, perioperative details, and postoperative complication data were collected. Complications were evaluated on the basis of type and etiology as surgical (inappropriate vector), hardware-related (hardware failure or dislodgement), or infectious (pin site, superficial, or deep
Between 1999 and 2013, a total of 54 patients with syndromic craniosynostosis underwent a total of 55 frontofacial and midfacial distraction procedures. Forty-three percent (n = 23) had diagnoses of Apert syndrome, 35% (n = 19) had diagnoses of Crouzon syndrome, and 19% (n = 10) were diagnosed with Pfeiffer syndrome. An additional 2 patients (4%) had undetermined syndromes associated with craniosynostosis and midface hypoplasia. The mean age at the time of distraction was 7.8 (range, 4.4–18.7) years. Thirty-three patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures (Fig. 1). Thirty procedures were performed using external halo-type distractors, including 18 Le Fort III and 12 monobloc osteotomies. Twenty-five procedures were performed
Study Design
RESULTS
TABLE 2. Clavien-Dindo Surgical Complication Classification Grading
Definition
Minor complications
Grade Grade Grade Grade Grade Grade Grade
Major complications
Mortality
I II IIIa IIIb IVa IVb V
Adverse event that alters the standard postoperative course without requiring a specific treatment Pharmacologic treatment or minor intervention required Surgical, radiologic, endoscopic treatment, or multitherapy required without general anesthesia Surgical, radiologic, endoscopic treatment, or multitherapy required with general anesthesia Intensive care unit treatment for single-organ dysfunction required Intensive care unit treatment for multiple-organ dysfunction required Adverse event that leads to death
© 2014 Mutaz B. Habal, MD
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TABLE 3. Distraction Complication Severity Clavien-Dindo Score, %
Halo (n = 30) Buried (n = 25)
FIGURE 1. Surgery characteristics.
1
2
3a
3b
4a
4b
5
Total
1 (3.3) 0
3 (10) 2 (8)
0 0
6 (20) 7 (28)
0 0
0 0
0 0
10 (33.3) 9 (36)
common in the buried distraction group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). In addition, there were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration. This was related to postoperative positioning or falls and required operative repositioning. During the course of the study period, complications remained stable while a trend of increasing cases over time was noted (Fig. 3).
DISCUSSION using semiburied distractors (16 Le Fort III and 9 monobloc osteotomies). The mean age at surgery was 8.0 (range, 4.0–17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. There were no significant differences in diagnoses or operation type between the distraction techniques. The trends in device selection were examined during the study period (Fig. 2). There was a significant difference in the use of the 2 distraction techniques. A steady decline in the use of semiburied distractors was noted from 1999 to 2010 when the last device was used. At the same time, there was a steady increase in the number of halo-type devices during the study period with the first halo-type used in 2004 (P = 0.001). There were 19 (35.6%) distractor-related complications in both groups during the study period. Table 3 demonstrates the severity stratification of these distractor-related complications. In those patients who underwent external halo-type distraction, there were a total of 10 (33.3%) complications, including 4 (13.3%) minor complications (Clavien-Dindo score of 1–2) and 6 (20%) moderate complications requiring a return to the operating room (Clavien-Dindo score of 3b). In the semiburied distractor group, there were a total of 9 (36%) complications, including 2 (8%) minor complications and 7 (28%) moderate complications. In neither the halo-type nor the semiburied distractor group were there any major complications (single- or multiple-organ system failure) or mortalities. Although the semiburied distractor group was weighted toward more severe complications as compared with the halo-type group, differences in the distribution were not statistically significant. Major infections requiring operative intervention were more
The largest study, to date, critically analyzing distractorrelated complications in patients with syndromic craniosynostosis undergoing frontofacial or midfacial surgery is presented. The overall complication rate of 35.6% is similar or less than those in previously published studies.19,20 However, this rate remains surprisingly high, indicating a need to further refine our devices and techniques. Thirteen patients (23.6%) in our series required an additional operative intervention to manage their distractor-related complications, mostly for device-related soft tissue infections. Four of the patients in the halo-type distractor group had pin migration and/or malposition after falling or due to head positioning. This represents close to one third of all complications requiring operative intervention in both groups. In 3 patients, the halo simply had to be repositioned in a different plane. In 2 patients, computed tomographically proven transcranial pin migration instigated a return to the operating room. In the first of these 2 patients, the migration occurred after a fall 1 month into consolidation and was treated by removal of the halo completely with conversion to orthodontic bone anchors to maintain the advancement. In the second patient, transcranial migration occurred without a fall while the patient was still in the hospital (Fig. 4). In this instance, the temporal bone was found to be thin and a titanium mesh was placed in the subgaleal plane to reinforce the weak bone and act as an “internal washer” for the pins. The patients displayed neither neurologic sequelae nor cerebrospinal fluid leak as a result of these complications, and both patients successfully completed therapy without further incident. In addition to the 2 cases reported here, several authors have published individual case reports of intracranial pin migration in the setting of halo-type distractor use in conjunction with midface
FIGURE 2. Trends in device selection.
FIGURE 3. Trends in complications over time.
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© 2014 Mutaz B. Habal, MD
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The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Complications in Midface Distraction
safety and efficacy; thus, there are differences in procedures over time, further confounding the results of our study. Despite these limitations, the results of this analysis demonstrate that external distraction offers a decreased infection rate with potential for improved outcomes.
CONCLUSIONS
FIGURE 4. Computed tomographic scan showing transcranial pin migration.
osteotomies.20–24 In these instances, no acute or ongoing neurologic sequelae were reported. While the dura was violated in 2 cases, treatment consisted of dural repair at the time of device removal in 1 case and intravenous antibiotics for subclinical infection in the other. To avoid these complications, several authors have published methods to reinforce the temporal bone and provide secure and safe halo anchorage.25,26 Below are some important conclusions from our experience: 1. Use at least 4 pins on each side of the halo distractor to distribute forces more evenly. In addition, pins may loosen during consolidation, oftentimes necessitating removal. Placing extra pins ensures enough persistence to safely finish consolidation. 2. Avoid using halo-type distractors shortly after fronto-orbital advancements where pterional osteotomies undermine the quality of temporal bone stock. Similarly, in patients with thin bone, primary reinforcement with titanium mesh may be necessary. 3. Consider placing resorbable “washers” in the subgaleal plane to reinforce the pin/bone interface. 4. Limit patient activity as much as possible during activation and consolidation phases to minimize risk for traumatic halo displacement. 5. Counsel families to be aware of asymmetric pin depth while at home. Early recognition of transcranial pin migration is a key to preventing significant sequelae. When evaluating the complications presented here, it is important to note the trends in device selection over time at our institution. As described in Figure 2, the use of semiburied distractors predominated early in the study period. Halfway through the study period, however, the use of semiburied distractors waned. External distractors are now the device of choice at our institution. In addition, we report consistent complication numbers with increasing numbers of frontofacial distractions performed over time (Fig. 3). This fact cannot simply be attributed to device selection but may also be a consequence of increasing proficiency with the procedure. As stated in Table 1, external distractors offer significant advantages compared with semiburied devices. These include the ability to “orthodontically” adjust the distracted segment in multiple vectors instead of being fixed in 1 vector at the time of surgery. In addition, halo-type distractors provide a central “pull” rather than a peripheral “push,” which serves to further unfurl facial concavity often present in syndromic patients. The ability to minimize buried hardware, especially in the region of the bony regenerate, helps to minimize infectious complications and maximize bone formation. Importantly, this study is not without limitations. The study is retrospective in design and reports on procedures that are relatively rare. Therefore, statistically significant conclusions are limited from these data. In addition, the trends in device selection prohibit a truly objective comparison of complication rates. As surgeons, we are constantly altering treatment protocols to maximize
Frontofacial distraction is an important technique in patients with syndromic craniosynostosis and midface hypoplasia. Although higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with semiburied distractors, the increased vector choice and beneficial force conveyance make careful use of external distractors advantageous.
REFERENCES 1. Tessier P. The definitive plastic surgical treatment of the severe facial deformities of craniofacial dysostosis. Crouzon's and Apert's diseases. Plast Reconstr Surg 1971;48:419–442 2. Ortiz-Monasterio F, del Campo AF, Carrillo A. Advancement of the orbits and the midface in one piece, combined with frontal repositioning, for the correction of Crouzon's deformities. Plast Reconstr Surg 1978;61:507–516 3. Wolfe SA, Morrison G, Page LK, et al. The monobloc frontofacial advancement: do the pluses outweigh the minuses? Plast Reconstr Surg 1993;91:977–987 4. McCarthy JG, Schreiber J, Karp N, et al. Lengthening the human mandible by gradual distraction. Plast Reconstr Surg 1992;89:1 5. Mofid MM, Manson PN, Robertson BC, et al. Craniofacial distraction osteogenesis: a review of 3278 cases. Plast Reconstr Surg 2001;108:1103 6. Gosain AK. Distraction osteogenesis of the craniofacial skeleton. Plast Reconstr Surg 2001;107:278 7. Bradley JP, Gabbay JS, Taub PJ, et al. Monobloc advancement by distraction osteogenesis decreases morbidity and relapse. Plast Reconstr Surg 2006: 1181585–1181597 8. Nout E, Cesteleyn LLM, van der Wal KGH, et al. Advancement of the midface, from conventional Le Fort III osteotomy to Le Fort III distraction: review of the literature. Int J Oral Maxillofac Surg 2008;37:781–789 9. Shetye PR, Grayson BH, McCarthy JG. Le Fort III distraction: controlling position and path of the osteotomized midface segment on a rigid platform. J Craniofac Surg 2010;21:1118–1121 10. Chin M, Toth BA. Distraction osteogenesis in maxillofacial surgery using internal devices: review of five cases. J Oral Maxillofac Surg 1996;54:45–53 11. Chin M, Toth BA. Le Fort III advancement with gradual distraction using internal devices. Plast Reconstr Surg 1997;100:819–830 12. Alonso N, Munhoz AM, Fogaça W, et al. Midfacial advancement by bone distraction for treatment of craniofacial deformities. J Craniofac Surg 1998;9:114–118 13. Polley JW, Figueroa AA. Management of severe maxillary deficiency in childhood and adolescence through distraction osteogenesis with an external, adjustable, rigid distraction device. J Craniofac Surg 1997;8:181–185 14. Polley JW, Figueroa AA. Rigid external distraction: its application in cleft maxillary deformities. Plast Reconstr Surg 1998;102:1360–1372 15. Dindo D, Demartines N, Clavien P-A. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004;240:205–213 16. 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;120:1009–1026 17. Fearon JA. Halo distraction of the Le Fort III in syndromic craniosynostosis: a long-term assessment. Plast Reconstr Surg 2005;115:1524–1536
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18. Satoh K, Mitsukawa N, Tosa Y, et al. Le Fort III midfacial distraction using an internal distraction device for syndromic craniosynostosis: device selection, problems, indications, and a proposal for use of a parallel bar for device-setting. J Craniofac Surg 2006; 17:1050–1058 19. Holmes AD, Wright GW, Meara JG, et al. LeFort III internal distraction in syndromic craniosynostosis. J Craniofac Surg 2002;13:262–272 20. Nout E, Wolvius EB, van Adrichem LNA, et al. Complications in maxillary distraction using the RED II device: a retrospective analysis of 21 patients. Int J Oral Maxillofac Surg 2006;35:897–902 21. Le BT, Eyre JM, Wehby MC, et al. Intracranial migration of halo fixation pins: a complication of using an extraoral distraction device. Cleft Palate Craniofac J 2001;38:401–404
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22. Brown R, Higuera S, Boyd V, et al. Intracranial migration of a halo pin during distraction osteogenesis for maxillary hypoplasia: case report and literature review. J Oral Maxillofac Surg 2006;64:130–135 23. Breugem CC, Bush K, Fitzpatrick DF. Le Fort III rigid external distraction complicated by intracranial movement of halo fixation pins. Cleft Palate Craniofac J 2008;45:332–336 24. Cai M, Shen G, Wang X, et al. Intracranial fixation pin migration: a complication of external Le Fort III distraction osteogenesis in Apert syndrome. J Craniofac Surg 2010;21:1557–1559 25. Mavili ME, Vargel I, Tunçbilek G. Stoppers in RED II distraction device: is it possible to prevent pin migration? J Craniofac Surg 2004;15:377–383 26. Nemeth JA, Mattingly LG. Six-pin halo fixation and the resulting prevalence of pin-site complications. J Bone Joint Surg Am 2001;83-A:377–382
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Complications in 54 Frontofacial Distraction Procedures in Patients With Syndromic Craniosynostosis Jesse A. Goldstein, MD,* James Thomas Paliga, BA,† Jesse A. Taylor, MD,† and Scott P. Bartlett, MD†
Abstract: Patients with syndromic craniosynostosis manifest midfacial hypoplasia often treated by midfacial advancement. Benefits of midfacial advancement by distraction osteogenesis have been well studied; little is known about the perioperative morbidity of these procedures, specifically relating to device selection. This study compares the perioperative complications between semiburied- and halo-type distraction osteogenesis of the midface. A retrospective review was performed on all patients with syndromic craniosynostosis who underwent midface distraction with semiburied- or halo-type external distractors. Demographic information and operative/postoperative course were reviewed. Complications were categorized as hardwarerelated, infectious, and either as major (requiring additional intervention) or minor (requiring medication only). Chi-squared and Fisher exact test were used to compare variables. From 1999 to 2012, a total of 54 patients underwent midface distraction osteogenesis, including 23 patients with Apert syndrome, 19 patients with Crouzon syndrome, 10 patients with Pfeiffer syndrome, and 2 patients with other craniofacial syndromes. Thirtythree patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures.
The mean age during surgery was 8.0 (range, 4.0–17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. Thirty procedures were performed with external halotype distractors (18 Le Fort III and 12 monobloc distractions), whereas 25 were performed with buried midface distractors (16 Le Fort III and 9 monobloc distractions). There were no significant differences in diagnoses or interventions between the distraction devices. Of the 19 distractor-related complications, there were a total of 10 (18.2%) in the halo group including 5 (9.1%) requiring separate operative intervention as well as 9 (16.4%) in the buried distractor group including 6 (10.1%) requiring separate operative intervention. Major infections were more common in the buried distractor group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). There were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration related to postoperative positioning or falls and required operative repositioning. Frontofacial distraction is an important technique in patients with syndromic craniosynostosis. Higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with buried distractors. Key Words: Midface distraction osteogenesis, complications, external, buried, distractors
From the *Department of Plastic Surgery, The University of Pittsburgh Medical School, Children's Hospital of Pittsburgh, Pittsburgh; †Division of Plastic Surgery, The Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Received April 10, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Scott P. Bartlett, MD, Division of Plastic Surgery, The Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia, Colket Translational Research Bldg, 3501 Civic Center Blvd 9th Floor, Philadelphia, PA; E-mail: [email protected] Supported by the Department of Surgery of The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania. The authors report no conflicts of interest. Authorship Participation and Contribution Jesse A. Goldstein, MD: data analysis, data interpretation, and manuscript preparation J. Thomas Paliga, BA: data analysis, data interpretation, and manuscript preparation Jesse A. Taylor, MD: data interpretation and manuscript preparation Scott P. Bartlett, MD: study conception, data analysis, data interpretation, and manuscript preparation Presented at the 15th Congress of the International Society of Craniofacial Surgeons, September 10–14, 2013, Jackson Hole, WY. This study was reviewed and approved by the institutional review board of the Children's Hospital of Philadelphia. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001320
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BACKGROUND Patients with syndromic forms of craniosynostosis often manifest severe midfacial hypoplasia with characteristic exophthalmos and class 3 dental relations. Conventional frontofacial and midface advancement procedures such as the monobloc, facial bipartition, and the Le Fort III have been performed for more than 30 years to address these malformations by advancing the retrusive supraorbital bandeau, increasing projection of the infraorbital rim, zygoma, and maxilla as well as improving malocclusion.1–3 Introduced over the last 2 decades, distraction osteogenesis has been increasingly used. This technique allows for greater advancement paired with significantly lower complication rates compared with conventional methods.4–7 Today, many craniofacial surgeons prefer distraction osteogenesis to treat brow and midface deficiencies in patients with syndromic craniosynostosis.8,9 However, considerable variation exists among centers with respect to timing, protocol, and distraction device selection. Early distractors used in frontofacial procedures were modified from internal mandibular distractors being exclusively internal or semiburied in design.10–12 Recently, external- or halo-type distractors have gained popularity among craniofacial surgeons.13,14 There are currently many options when choosing a device, and each distractor has its own advantages and disadvantages (Table 1). Although semiburied distractors are well hidden
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Complications in Midface Distraction
infections). The validated Clavien-Dindo surgical classification system based on severity of complication was also applied (Table 2).15
TABLE 1. Device Comparison Semiburied Device
Halo-Type Device
Total distraction limited
Larger distraction lengths achievable Increased facial scarring Multivector, push/pull Vectors can be changed, molding easy Larger moment arm (pins/splints); requires better bone stock Increased social stigma Easier to remove, useful during infection
Minimal facial scarring One vector, push only No change in vector Small moment arm (plate to bone); requires less bone stock Less conspicuous Removal more difficult
and simple to operate, they allow for no postoperative vector adjustment. This limitation can result in overprojection of the lateral face compared with the central face, accentuating a “dish-faced” appearance. In contrast, external- or halo-type distractors allow for continuous postoperative vector adjustment and greater central facial expansion. The disadvantages, however, include being unsightly and prone to dislodgement. Although the benefits of frontofacial and midfacial advancement by distraction osteogenesis have been well studied, little has been written about the perioperative morbidity of these procedures, especially as they relate to device selection. This study was designed to (1) review distractor-associated complications in patients with syndromic forms of craniosynostosis undergoing frontofacial or midfacial advancement with distraction osteogenesis and (2) compare perioperative morbidity between the semiburied- and halo-type devices.
Surgical Procedures and Device Selection Patients found to have both retrusive brow and midface deformities were selected for monobloc or facial bipartition osteotomies. These procedures have been previously described.7,16 Briefly, after a coronal approach as well as periorbital and infratemporal dissection, a frontal craniotomy is performed by a neurosurgeon. Orbital and nasal osteotomies are performed, followed by pterygomaxillary disjunction and downfracture. After mobilization, the frontal bone is replated to the bandeau. For internal distractor application, 2 to 4 midface semiburied distractors are placed bilaterally at the level of the bandeau and the zygoma. For external halo-type distractor application, frontal and maxillary footplates and percutaneous wire fixation screws are placed and attached to the halo device with 24-gauge wire. Patients with isolated midface retrusion and acceptable brow position were selected for Le Fort III osteotomy. This procedure has been previously described.11,17,18 Briefly, after a coronal approach as well as periorbital and infratemporal dissection, orbital and nasal osteotomies are performed, followed by pterygomaxillary disjunction and downfracture. For internal distractor application, 2 midface semiburied distractors are placed bilaterally at the level of the zygoma. For external halo-type distractor application, maxillary footplates and percutaneous wire fixation screws are placed and attached to the halo device with 24-gauge wire. The distraction protocol was standardized regardless of osteotomy or device choice and included a 5-day latency period, followed by a 1-mm/d activation rate until correction was completed. A consolidation period of 6 to 8 weeks was used in all patients.
Statistical Analysis
METHODS
Demographic and operative characteristics were assessed with descriptive statistics. Fisher exact test was used to compare complication types between the distractor groups. An α of less than 0.05 was considered significant.
An institutional review board–approved retrospective review was performed on all patients with syndromic forms of craniosynostosis who underwent frontofacial or midface advancement using distraction osteogenesis for a 13-year period. Patients were included if they underwent monobloc or Le Fort III distraction with either external (halo-type) distractors or internal (semiburied) distractors. Patients with incomplete medical records and those who had conventional Le Fort III or monobloc osteotomies with acute advancements were excluded from this analysis. Demographic information, perioperative details, and postoperative complication data were collected. Complications were evaluated on the basis of type and etiology as surgical (inappropriate vector), hardware-related (hardware failure or dislodgement), or infectious (pin site, superficial, or deep
Between 1999 and 2013, a total of 54 patients with syndromic craniosynostosis underwent a total of 55 frontofacial and midfacial distraction procedures. Forty-three percent (n = 23) had diagnoses of Apert syndrome, 35% (n = 19) had diagnoses of Crouzon syndrome, and 19% (n = 10) were diagnosed with Pfeiffer syndrome. An additional 2 patients (4%) had undetermined syndromes associated with craniosynostosis and midface hypoplasia. The mean age at the time of distraction was 7.8 (range, 4.4–18.7) years. Thirty-three patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures (Fig. 1). Thirty procedures were performed using external halo-type distractors, including 18 Le Fort III and 12 monobloc osteotomies. Twenty-five procedures were performed
Study Design
RESULTS
TABLE 2. Clavien-Dindo Surgical Complication Classification Grading
Definition
Minor complications
Grade Grade Grade Grade Grade Grade Grade
Major complications
Mortality
I II IIIa IIIb IVa IVb V
Adverse event that alters the standard postoperative course without requiring a specific treatment Pharmacologic treatment or minor intervention required Surgical, radiologic, endoscopic treatment, or multitherapy required without general anesthesia Surgical, radiologic, endoscopic treatment, or multitherapy required with general anesthesia Intensive care unit treatment for single-organ dysfunction required Intensive care unit treatment for multiple-organ dysfunction required Adverse event that leads to death
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TABLE 3. Distraction Complication Severity Clavien-Dindo Score, %
Halo (n = 30) Buried (n = 25)
FIGURE 1. Surgery characteristics.
1
2
3a
3b
4a
4b
5
Total
1 (3.3) 0
3 (10) 2 (8)
0 0
6 (20) 7 (28)
0 0
0 0
0 0
10 (33.3) 9 (36)
common in the buried distraction group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). In addition, there were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration. This was related to postoperative positioning or falls and required operative repositioning. During the course of the study period, complications remained stable while a trend of increasing cases over time was noted (Fig. 3).
DISCUSSION using semiburied distractors (16 Le Fort III and 9 monobloc osteotomies). The mean age at surgery was 8.0 (range, 4.0–17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. There were no significant differences in diagnoses or operation type between the distraction techniques. The trends in device selection were examined during the study period (Fig. 2). There was a significant difference in the use of the 2 distraction techniques. A steady decline in the use of semiburied distractors was noted from 1999 to 2010 when the last device was used. At the same time, there was a steady increase in the number of halo-type devices during the study period with the first halo-type used in 2004 (P = 0.001). There were 19 (35.6%) distractor-related complications in both groups during the study period. Table 3 demonstrates the severity stratification of these distractor-related complications. In those patients who underwent external halo-type distraction, there were a total of 10 (33.3%) complications, including 4 (13.3%) minor complications (Clavien-Dindo score of 1–2) and 6 (20%) moderate complications requiring a return to the operating room (Clavien-Dindo score of 3b). In the semiburied distractor group, there were a total of 9 (36%) complications, including 2 (8%) minor complications and 7 (28%) moderate complications. In neither the halo-type nor the semiburied distractor group were there any major complications (single- or multiple-organ system failure) or mortalities. Although the semiburied distractor group was weighted toward more severe complications as compared with the halo-type group, differences in the distribution were not statistically significant. Major infections requiring operative intervention were more
The largest study, to date, critically analyzing distractorrelated complications in patients with syndromic craniosynostosis undergoing frontofacial or midfacial surgery is presented. The overall complication rate of 35.6% is similar or less than those in previously published studies.19,20 However, this rate remains surprisingly high, indicating a need to further refine our devices and techniques. Thirteen patients (23.6%) in our series required an additional operative intervention to manage their distractor-related complications, mostly for device-related soft tissue infections. Four of the patients in the halo-type distractor group had pin migration and/or malposition after falling or due to head positioning. This represents close to one third of all complications requiring operative intervention in both groups. In 3 patients, the halo simply had to be repositioned in a different plane. In 2 patients, computed tomographically proven transcranial pin migration instigated a return to the operating room. In the first of these 2 patients, the migration occurred after a fall 1 month into consolidation and was treated by removal of the halo completely with conversion to orthodontic bone anchors to maintain the advancement. In the second patient, transcranial migration occurred without a fall while the patient was still in the hospital (Fig. 4). In this instance, the temporal bone was found to be thin and a titanium mesh was placed in the subgaleal plane to reinforce the weak bone and act as an “internal washer” for the pins. The patients displayed neither neurologic sequelae nor cerebrospinal fluid leak as a result of these complications, and both patients successfully completed therapy without further incident. In addition to the 2 cases reported here, several authors have published individual case reports of intracranial pin migration in the setting of halo-type distractor use in conjunction with midface
FIGURE 2. Trends in device selection.
FIGURE 3. Trends in complications over time.
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The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Complications in Midface Distraction
safety and efficacy; thus, there are differences in procedures over time, further confounding the results of our study. Despite these limitations, the results of this analysis demonstrate that external distraction offers a decreased infection rate with potential for improved outcomes.
CONCLUSIONS
FIGURE 4. Computed tomographic scan showing transcranial pin migration.
osteotomies.20–24 In these instances, no acute or ongoing neurologic sequelae were reported. While the dura was violated in 2 cases, treatment consisted of dural repair at the time of device removal in 1 case and intravenous antibiotics for subclinical infection in the other. To avoid these complications, several authors have published methods to reinforce the temporal bone and provide secure and safe halo anchorage.25,26 Below are some important conclusions from our experience: 1. Use at least 4 pins on each side of the halo distractor to distribute forces more evenly. In addition, pins may loosen during consolidation, oftentimes necessitating removal. Placing extra pins ensures enough persistence to safely finish consolidation. 2. Avoid using halo-type distractors shortly after fronto-orbital advancements where pterional osteotomies undermine the quality of temporal bone stock. Similarly, in patients with thin bone, primary reinforcement with titanium mesh may be necessary. 3. Consider placing resorbable “washers” in the subgaleal plane to reinforce the pin/bone interface. 4. Limit patient activity as much as possible during activation and consolidation phases to minimize risk for traumatic halo displacement. 5. Counsel families to be aware of asymmetric pin depth while at home. Early recognition of transcranial pin migration is a key to preventing significant sequelae. When evaluating the complications presented here, it is important to note the trends in device selection over time at our institution. As described in Figure 2, the use of semiburied distractors predominated early in the study period. Halfway through the study period, however, the use of semiburied distractors waned. External distractors are now the device of choice at our institution. In addition, we report consistent complication numbers with increasing numbers of frontofacial distractions performed over time (Fig. 3). This fact cannot simply be attributed to device selection but may also be a consequence of increasing proficiency with the procedure. As stated in Table 1, external distractors offer significant advantages compared with semiburied devices. These include the ability to “orthodontically” adjust the distracted segment in multiple vectors instead of being fixed in 1 vector at the time of surgery. In addition, halo-type distractors provide a central “pull” rather than a peripheral “push,” which serves to further unfurl facial concavity often present in syndromic patients. The ability to minimize buried hardware, especially in the region of the bony regenerate, helps to minimize infectious complications and maximize bone formation. Importantly, this study is not without limitations. The study is retrospective in design and reports on procedures that are relatively rare. Therefore, statistically significant conclusions are limited from these data. In addition, the trends in device selection prohibit a truly objective comparison of complication rates. As surgeons, we are constantly altering treatment protocols to maximize
Frontofacial distraction is an important technique in patients with syndromic craniosynostosis and midface hypoplasia. Although higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with semiburied distractors, the increased vector choice and beneficial force conveyance make careful use of external distractors advantageous.
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