CLINICAL STUDY
Simultaneous Unicoronal and Sagittal Distraction Osteogenesis for the Treatment of Nonsyndromic Multisutural Craniosynostosis Kaitlyn Marie Paine, BM, Youssef Tahiri, MD, MSC, J. Thomas Paliga, BA, and Jesse A. Taylor, MD Abstract: We present a case of multiplanar distraction osteogenesis for the simultaneous treatment of sagittal and unicoronal craniosynostosis in a nonsyndromic 2-month-old boy. Unilateral frontoorbital advancement and sagittal suturectomy were performed. Distracters were fixed orthogonally in the sagittal and coronal positions to distract the affected coronal and sagittal sutures. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations. A total intracranial volume increase of 62% was noted at 6 months’ follow-up. This preliminary report demonstrates the procedure’s short-term safety; future investigation is needed over the long term to determine its efficacy. Key Words: Distraction osteogenesis, craniosynostosis, multisutural (J Craniofac Surg 2015;26: 214–216)
C
raniosynostosis has an incidence of roughly 3.1 to 5.06 per 10,000 live births1–3 and is classified according to the suture or sutures that are prematurely fused. Most cases involve a single suture, often without an identifiable genetic marker.1–3 Multisutural craniosynostosis without an identifiable syndrome or genetic anomaly, also called complex craniosynostosis, is less common and occurs in approximately 8% to 13% of nonsyndromic craniosynostotic cases.2,3 Bicoronal suture fusion is the most common multisutural craniosynostosis.2–4 A smaller subset of cases is classified as syndromic craniosynostosis and is associated by a greater pattern of multisutural fusion, along with potential limb and other deformities. Genetic anomalies in the fibroblast growth factor receptor (FGFR)1 FGFR2, FGFR3, TWIST, and MSX have all been associated with a variety of craniosynostotic syndromes.5 Presentation of complex craniosynostosis is variable, and dependent on the sutures involved. Multisutural fusion leads to From the Department of Plastic Surgery, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Received May 23, 2014. Accepted for publication July 31, 2014. Address correspondence and reprint requests to Kaitlyn Marie Paine, BM, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Colket Translational Research Building, 9th Floor, 3501 Civic Center Blvd, Philadelphia, PA 19104; E-mail: [email protected] Disclaimers: None. The Department of Surgery of The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania funded this work. The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001255
214
complex growth-pattern restriction, and management must be tailored to each individual’s phenotype. Treatment strategies center on preventing increased intracranial pressure (ICP), which has been documented in as many as 41% to 47% of children with complex craniosynostosis,4,6 as opposed to 14% involving a single suture.6 Normalizing head shape and other aesthetic concerns are also primary objectives of treatment. In 1992, Remmler et al7–10 attempted the first true cranial distraction osteogenesis in a rabbit model, although others had attempted spring-based expansion previously.8–10 Today, the indications for distraction osteogenesis in the craniofacial skeleton continue to expand, and include posterior cranial distraction, anterior cranial distraction, monoblock craniofacial distraction, and LeFort III midface distraction.11 In this manuscript, we present a unique case of simultaneous sagittal and unilateral fronto-orbital distraction in an infant with severe and progressive sagittal and right unicoronal craniosynostosis.
MATERIALS AND METHODS The patient is a male diagnosed at delivery with sagittal and right unicoronal craniosynostosis after an uncomplicated pregnancy. His phenotypic presentation included synostosis of the sagittal and right unicoronal sutures with ridging along the right coronal suture and left frontal bulging as well as a dolichocephalic head shape. There was associated bilateral orbital deformity including a shallow left orbit and elongated right orbit. The diagnosis of unicoronal and sagittal synostosis was confirmed by computed tomography (CT) (Fig. 1). The father also noted intermittent lack of eye tracking and, on evaluation by an ophthalmologist, the patient’s condition was diagnosed as strabismic amblyopia. Genetic testing for FGFR1, FGFR2, FGFR3, and TWIST was negative. On his subsequent visit, at 8 weeks old, his parents claimed that he was becoming more irritable and his head shape was worsening. A decision to surgically intervene was taken along with the neurosurgery team. To address the concerns for increased ICP, achieve significant volumetric expansion, produce an improved head shape, and to limit the operative time and potential complications, simultaneous distraction of the coronal and sagittal suture was performed. Craniometric comparison of 3-dimensional CT (3D CT) was performed preoperatively and after completion of distraction and removal of devices evaluating cranial volume. Volumetric data were analyzed using Mimics (Materialise; Leuven, Belgium).
Surgical Technique With the patient in the supine position, a curvilinear coronal incision provided access to the anterior and posterior cranial vault. Suturectomies along the sagittal and right coronal suture were performed using the Sonopet device (Stryker Instruments, Kalamazoo, MI). The coronal craniotomy was then extended to release the ipsilateral temporal region, sphenoid wing, superior half of the orbit,
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
Unicoronal and Sagittal Osteogenesis
FIGURE 1. Preoperative clinical anteroposterior (A/P) 3D CT and lateral 3D CT photos. Cranial volume was 673,765.27 mm3.
FIGURE 3. Six-month follow-up clinical A/P 3D CT and lateral 3D CT photos. Cranial volume was 1,092,272.08 mm3.
and nasofrontal region. Care was taken to leave most of the frontal bone and bandeau vascularized by the underlying dura. Once the osteotomies were completed, bilateral, semiburied 40-mm distractors (KLS Martin, Jacksonville, FL), were rigidly affixed with 3.5-mm self-drilling screws perpendicular to the involved sutures. Both distractors were activated to verify proper movements and completion of the osteotomies and then deactivated. Particulate bone graft was used to fill the suturectomy sites. A layered closure was performed, and the patient was extubated in the operating room and thereafter taken to the intensive care unit in good condition.
The procedure lasted 147 minutes, with an estimated blood loss of 305 mL. He received 305 mL of whole blood during the operation and required no further transfusion after surgery. His 3-day
inpatient stay was uneventful, and the parents were instructed daily on how to perform activation of the device. Distraction was initiated, on both devices, on postoperative day 2 at a rate of 1 mm/d, and was carried out for 20 and 22 days. Removal of the distractors was performed after 8 weeks of consolidation. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations, respectively. Using 3D CT scans, volumetric data were acquired both preoperatively and postoperatively, and these data are presented in Figures 1, 2, and 3. Preoperative cranial volume 2½ weeks before surgery was 673,765.27 mm3. Postoperative day 1 and 6-month follow-up cranial vault volumes were 736,128.98 mm3 and 1,092,272.08 mm3, respectively. The change in the cranial vault volume between the preoperative and immediate post was 9.26% and between the immediate post and follow-up was 48.38%. The total volume change from preoperative to 6-month follow-up was 62.11%.
FIGURE 2. Postoperative day 1 clinical A/P 3D CT and lateral 3D CT photos. Cranial volume was 736,128.98 mm3.
In this case study, we report a relatively rare instance of simultaneous sagittal and unicoronal distraction osteogenesis in a 2-month-old boy who presented with severe and progressive sagittal and right coronal synostosis. The decision to use distraction instead of partial craniectomy at such a young age was largely due to the desire to preserve and maximize the amount of vascularized virgin bone for future operations. The sagittal distractors created forces in a transverse vector, while the coronal distractors brought the forehead and orbit anteriorly and inferiorly. We were able to perform this procedure without perioperative or postoperative complications, achieving adequate increase in intracranial volume and observing improved head shape. Distraction osteogenesis potentially offers several advantages over cranial vault remodeling in cases like ours. There is less dural dissection, improved maintenance of bone vascularity, and increased amounts of cranial volume gain.12 Reduced operative time and blood loss have also been reported.13,14 Importantly, the gradual expansion of the intracranial contents commensurate with the bone eliminates dead space, thereby minimizing the risk of infection. Cranial distraction may also have improved growth implications, as well as growth trajectory relative to conventional cranial vault remodeling. In a study by Kim et al15 examining the growth
RESULTS
DISCUSSION
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
215
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Paine et al
of involved bones after distraction, they found continued growth of the osteotomized segments. Another study by Choi et al16 demonstrated cranial base remodeling after cranial distraction that is not seen with cranial vault remodeling. Since its introduction to the craniofacial skeleton, distraction osteogenesis has been seen as a technique that potentially limits the major complications associated with more invasive procedures. In a review performed by Pelo et al (n = 138), complications of distraction were mostly minor and included local infection, device fracture, strabismus, trimus, zygomatic-maxillary fracture, and a palatal midline fracture.17 Other studies have also demonstrated stable outcomes, low relapse rates, and low complication rates consisting mostly of minor superficial infections that were selflimited.13–15 Even with these advantages, the main concerns of distractor osteogenesis continue to be the increased length of treatment due to the need for latency, distraction, and consolidation periods; the need for a second surgery for distractor removal; and the risk of an infection as a result of having an exposed device. In addition, not all studies have demonstrated low morbidity rates. Esparza’s case series demonstrated a higher major complication rate in his distraction group including 8 CSF leaks, 3 dural tears, 8 local wound infections, and 2 basal encephaloceles. That stated, Esparza’s distraction cases were all redos, only performed after relapse or poor results after conventional frontal-orbital advancement. More research is needed to truly understand the advantages of distraction over cranial vault remodeling in reducing complications.18 Thus far, only a few studies have examined volume changes using 3D imaging after posterior vault, total cranial vault, and anterior cranial vault distraction.19–21 In particular, the study by Yamaguchi et al found a postoperative period where growth was suppressed by examining the cranial growth gap between their patients and healthy persons using the Abbott curve. In the study by Deschamps-Braly et al on cranial vault distraction in the 3 patients who had ICP monitoring, a volume change of 4.7% led to a mean of 73.6% decrease in ICP. Our initial volume change is slightly larger at 9.3%, but it is difficult to compare values across methodologies, and we cannot make any definitive statements about ICP in our patient. Indirectly, he had disc bulging preoperatively on fundoscopic examination and no disc bulging on follow-up examinations. Certainly, an increase in volume of more than 60% would seem like enough.
CONCLUSIONS We present an alternative treatment, orthogonal multivector distraction, for the early treatment of severe and progressive multisuture craniosynostosis. This initial report demonstrates the procedure’s safety; future investigation is needed over the long term to determine its efficacy.
216
REFERENCES 1. Singer S, Bower C, Southall P, et al. Craniosynostosis in Western Australia, 1980–1994: a population-based study. Am J Med Genet 1999;83:382–387 2. Boulet SL, Rasmussen SA, Honein MA. A population-based study of craniosynostosis in metropolitan Atlanta, 1989–2003. Am J Med Genet A 2008;146A:984–991 3. Lee HQ, Hutson JM, Wray AC, et al. Changing epidemiology of nonsyndromic craniosynostosis and revisiting the risk factors. J Craniofac Surg 2012;23:1245–1251 4. Shillito J Jr, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics 1968;41:829–853 5. Müller U, Steinberger D, Kunze S. Molecular genetics of craniosynostotic syndromes. Graefes Arch Clin Exp Ophthalmol 1997;235:545–550. Review 6. Renier D, Sainte-Rose C, Marchac D, et al. Intracranial pressure in craniostenosis. J Neurosurg 1982;57:370–377 7. Remmler D, McCoy FJ, O’Neil D, et al. Osseous expansion of the cranial vault by craniotasis. Plast Reconstr Surg 1992;89:787–797 8. Ten Cate AR, Freeman E, Dickinson JB. Sutural development: structure and its response to rapid expansion. Am J Orthod 1977;71:622–636 9. Persing JA, Babler WJ, Nagorsky MJ, et al. Skull expansion in experimental craniosynostosis. Plast Reconstr Surg 1986;78:594–603 10. Persing JA, Morgan EP, Cronin AJ, et al. Skull base expansion: craniofacial effects. Plast Reconstr Surg 1991;87:1028–1033 11. Hopper RA. New trends in cranio-orbital and midface distraction for craniofacial dysostosis. Curr Opin Otolaryngol Head Neck Surg 2012;20:298–303 12. Uemura T, Hayashi T, Satoh K, et al. Three-dimensional cranial expansion using distraction osteogenesis for oxycephaly. J Craniofac Surg 2003;14:29–36 13. Kim SW, Shim KW, Plesnila N, et al. Distraction vs remodeling surgery for craniosynostosis. Childs Nerv Syst 2007;23:201–206 14. Akai T, Iizuka H, Kawakami S. Treatment of craniosynostosis by distraction osteogenesis. Pediatr Neurosurg 2006;42:288–292 15. Kim YO, Choi JW, Kim DS, et al. Cranial growth after distraction osteogenesis of the craniosynostosis. J Craniofac Surg 2008;19:45–55 16. Choi JW, Ra YS, Hong SH, et al. Use of distraction osteogenesis to change endocranial morphology in unilateral coronal craniosynostosis patients. Plast Reconstr Surg 2010;126:995–1004 17. Pelo S, Gasparini G, Di Petrillo A, et al. Distraction osteogenesis in the surgical treatment of craniostenosis: a comparison of internal and external craniofacial distractor devices. Child’s Nerv Syst 2007;23:1447–1453 18. Esparza J, Hinojosa J, García-Recuero I, et al. Surgical treatment of isolated and syndromic craniosynostosis. Results and complications in 283 consecutive cases. Neurocirugia (Astur) 2008;19:509–529 19. Deschamps-Braly J, Hettinger P, el Amm C, et al. Volumetric analysis of cranial vault distraction for cephalocranial disproportion. Pediatr Neurosurg 2011;47:396–405 20. Goldstein JA, Paliga JT, Bailey RL, et al. Posterior vault distraction with midface distraction without osteotomy as a first stage for syndromic craniosynostosis. J Craniofac Surg 2013;24:1263–1267 21. Yamaguchi K, Imai K, Fujimoto T, et al. Cranial distraction osteogenesis for syndromic craniosynostosis: long-term follow-up and effect on postoperative cranial growth. J Plast Reconstr Aesthet Surg 2014;67:e35–41
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Simultaneous Unicoronal and Sagittal Distraction Osteogenesis for the Treatment of Nonsyndromic Multisutural Craniosynostosis Kaitlyn Marie Paine, BM, Youssef Tahiri, MD, MSC, J. Thomas Paliga, BA, and Jesse A. Taylor, MD Abstract: We present a case of multiplanar distraction osteogenesis for the simultaneous treatment of sagittal and unicoronal craniosynostosis in a nonsyndromic 2-month-old boy. Unilateral frontoorbital advancement and sagittal suturectomy were performed. Distracters were fixed orthogonally in the sagittal and coronal positions to distract the affected coronal and sagittal sutures. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations. A total intracranial volume increase of 62% was noted at 6 months’ follow-up. This preliminary report demonstrates the procedure’s short-term safety; future investigation is needed over the long term to determine its efficacy. Key Words: Distraction osteogenesis, craniosynostosis, multisutural (J Craniofac Surg 2015;26: 214–216)
C
raniosynostosis has an incidence of roughly 3.1 to 5.06 per 10,000 live births1–3 and is classified according to the suture or sutures that are prematurely fused. Most cases involve a single suture, often without an identifiable genetic marker.1–3 Multisutural craniosynostosis without an identifiable syndrome or genetic anomaly, also called complex craniosynostosis, is less common and occurs in approximately 8% to 13% of nonsyndromic craniosynostotic cases.2,3 Bicoronal suture fusion is the most common multisutural craniosynostosis.2–4 A smaller subset of cases is classified as syndromic craniosynostosis and is associated by a greater pattern of multisutural fusion, along with potential limb and other deformities. Genetic anomalies in the fibroblast growth factor receptor (FGFR)1 FGFR2, FGFR3, TWIST, and MSX have all been associated with a variety of craniosynostotic syndromes.5 Presentation of complex craniosynostosis is variable, and dependent on the sutures involved. Multisutural fusion leads to From the Department of Plastic Surgery, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Received May 23, 2014. Accepted for publication July 31, 2014. Address correspondence and reprint requests to Kaitlyn Marie Paine, BM, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Colket Translational Research Building, 9th Floor, 3501 Civic Center Blvd, Philadelphia, PA 19104; E-mail: [email protected] Disclaimers: None. The Department of Surgery of The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania funded this work. The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001255
214
complex growth-pattern restriction, and management must be tailored to each individual’s phenotype. Treatment strategies center on preventing increased intracranial pressure (ICP), which has been documented in as many as 41% to 47% of children with complex craniosynostosis,4,6 as opposed to 14% involving a single suture.6 Normalizing head shape and other aesthetic concerns are also primary objectives of treatment. In 1992, Remmler et al7–10 attempted the first true cranial distraction osteogenesis in a rabbit model, although others had attempted spring-based expansion previously.8–10 Today, the indications for distraction osteogenesis in the craniofacial skeleton continue to expand, and include posterior cranial distraction, anterior cranial distraction, monoblock craniofacial distraction, and LeFort III midface distraction.11 In this manuscript, we present a unique case of simultaneous sagittal and unilateral fronto-orbital distraction in an infant with severe and progressive sagittal and right unicoronal craniosynostosis.
MATERIALS AND METHODS The patient is a male diagnosed at delivery with sagittal and right unicoronal craniosynostosis after an uncomplicated pregnancy. His phenotypic presentation included synostosis of the sagittal and right unicoronal sutures with ridging along the right coronal suture and left frontal bulging as well as a dolichocephalic head shape. There was associated bilateral orbital deformity including a shallow left orbit and elongated right orbit. The diagnosis of unicoronal and sagittal synostosis was confirmed by computed tomography (CT) (Fig. 1). The father also noted intermittent lack of eye tracking and, on evaluation by an ophthalmologist, the patient’s condition was diagnosed as strabismic amblyopia. Genetic testing for FGFR1, FGFR2, FGFR3, and TWIST was negative. On his subsequent visit, at 8 weeks old, his parents claimed that he was becoming more irritable and his head shape was worsening. A decision to surgically intervene was taken along with the neurosurgery team. To address the concerns for increased ICP, achieve significant volumetric expansion, produce an improved head shape, and to limit the operative time and potential complications, simultaneous distraction of the coronal and sagittal suture was performed. Craniometric comparison of 3-dimensional CT (3D CT) was performed preoperatively and after completion of distraction and removal of devices evaluating cranial volume. Volumetric data were analyzed using Mimics (Materialise; Leuven, Belgium).
Surgical Technique With the patient in the supine position, a curvilinear coronal incision provided access to the anterior and posterior cranial vault. Suturectomies along the sagittal and right coronal suture were performed using the Sonopet device (Stryker Instruments, Kalamazoo, MI). The coronal craniotomy was then extended to release the ipsilateral temporal region, sphenoid wing, superior half of the orbit,
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
Unicoronal and Sagittal Osteogenesis
FIGURE 1. Preoperative clinical anteroposterior (A/P) 3D CT and lateral 3D CT photos. Cranial volume was 673,765.27 mm3.
FIGURE 3. Six-month follow-up clinical A/P 3D CT and lateral 3D CT photos. Cranial volume was 1,092,272.08 mm3.
and nasofrontal region. Care was taken to leave most of the frontal bone and bandeau vascularized by the underlying dura. Once the osteotomies were completed, bilateral, semiburied 40-mm distractors (KLS Martin, Jacksonville, FL), were rigidly affixed with 3.5-mm self-drilling screws perpendicular to the involved sutures. Both distractors were activated to verify proper movements and completion of the osteotomies and then deactivated. Particulate bone graft was used to fill the suturectomy sites. A layered closure was performed, and the patient was extubated in the operating room and thereafter taken to the intensive care unit in good condition.
The procedure lasted 147 minutes, with an estimated blood loss of 305 mL. He received 305 mL of whole blood during the operation and required no further transfusion after surgery. His 3-day
inpatient stay was uneventful, and the parents were instructed daily on how to perform activation of the device. Distraction was initiated, on both devices, on postoperative day 2 at a rate of 1 mm/d, and was carried out for 20 and 22 days. Removal of the distractors was performed after 8 weeks of consolidation. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations, respectively. Using 3D CT scans, volumetric data were acquired both preoperatively and postoperatively, and these data are presented in Figures 1, 2, and 3. Preoperative cranial volume 2½ weeks before surgery was 673,765.27 mm3. Postoperative day 1 and 6-month follow-up cranial vault volumes were 736,128.98 mm3 and 1,092,272.08 mm3, respectively. The change in the cranial vault volume between the preoperative and immediate post was 9.26% and between the immediate post and follow-up was 48.38%. The total volume change from preoperative to 6-month follow-up was 62.11%.
FIGURE 2. Postoperative day 1 clinical A/P 3D CT and lateral 3D CT photos. Cranial volume was 736,128.98 mm3.
In this case study, we report a relatively rare instance of simultaneous sagittal and unicoronal distraction osteogenesis in a 2-month-old boy who presented with severe and progressive sagittal and right coronal synostosis. The decision to use distraction instead of partial craniectomy at such a young age was largely due to the desire to preserve and maximize the amount of vascularized virgin bone for future operations. The sagittal distractors created forces in a transverse vector, while the coronal distractors brought the forehead and orbit anteriorly and inferiorly. We were able to perform this procedure without perioperative or postoperative complications, achieving adequate increase in intracranial volume and observing improved head shape. Distraction osteogenesis potentially offers several advantages over cranial vault remodeling in cases like ours. There is less dural dissection, improved maintenance of bone vascularity, and increased amounts of cranial volume gain.12 Reduced operative time and blood loss have also been reported.13,14 Importantly, the gradual expansion of the intracranial contents commensurate with the bone eliminates dead space, thereby minimizing the risk of infection. Cranial distraction may also have improved growth implications, as well as growth trajectory relative to conventional cranial vault remodeling. In a study by Kim et al15 examining the growth
RESULTS
DISCUSSION
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
215
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Paine et al
of involved bones after distraction, they found continued growth of the osteotomized segments. Another study by Choi et al16 demonstrated cranial base remodeling after cranial distraction that is not seen with cranial vault remodeling. Since its introduction to the craniofacial skeleton, distraction osteogenesis has been seen as a technique that potentially limits the major complications associated with more invasive procedures. In a review performed by Pelo et al (n = 138), complications of distraction were mostly minor and included local infection, device fracture, strabismus, trimus, zygomatic-maxillary fracture, and a palatal midline fracture.17 Other studies have also demonstrated stable outcomes, low relapse rates, and low complication rates consisting mostly of minor superficial infections that were selflimited.13–15 Even with these advantages, the main concerns of distractor osteogenesis continue to be the increased length of treatment due to the need for latency, distraction, and consolidation periods; the need for a second surgery for distractor removal; and the risk of an infection as a result of having an exposed device. In addition, not all studies have demonstrated low morbidity rates. Esparza’s case series demonstrated a higher major complication rate in his distraction group including 8 CSF leaks, 3 dural tears, 8 local wound infections, and 2 basal encephaloceles. That stated, Esparza’s distraction cases were all redos, only performed after relapse or poor results after conventional frontal-orbital advancement. More research is needed to truly understand the advantages of distraction over cranial vault remodeling in reducing complications.18 Thus far, only a few studies have examined volume changes using 3D imaging after posterior vault, total cranial vault, and anterior cranial vault distraction.19–21 In particular, the study by Yamaguchi et al found a postoperative period where growth was suppressed by examining the cranial growth gap between their patients and healthy persons using the Abbott curve. In the study by Deschamps-Braly et al on cranial vault distraction in the 3 patients who had ICP monitoring, a volume change of 4.7% led to a mean of 73.6% decrease in ICP. Our initial volume change is slightly larger at 9.3%, but it is difficult to compare values across methodologies, and we cannot make any definitive statements about ICP in our patient. Indirectly, he had disc bulging preoperatively on fundoscopic examination and no disc bulging on follow-up examinations. Certainly, an increase in volume of more than 60% would seem like enough.
CONCLUSIONS We present an alternative treatment, orthogonal multivector distraction, for the early treatment of severe and progressive multisuture craniosynostosis. This initial report demonstrates the procedure’s safety; future investigation is needed over the long term to determine its efficacy.
216
REFERENCES 1. Singer S, Bower C, Southall P, et al. Craniosynostosis in Western Australia, 1980–1994: a population-based study. Am J Med Genet 1999;83:382–387 2. Boulet SL, Rasmussen SA, Honein MA. A population-based study of craniosynostosis in metropolitan Atlanta, 1989–2003. Am J Med Genet A 2008;146A:984–991 3. Lee HQ, Hutson JM, Wray AC, et al. Changing epidemiology of nonsyndromic craniosynostosis and revisiting the risk factors. J Craniofac Surg 2012;23:1245–1251 4. Shillito J Jr, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics 1968;41:829–853 5. Müller U, Steinberger D, Kunze S. Molecular genetics of craniosynostotic syndromes. Graefes Arch Clin Exp Ophthalmol 1997;235:545–550. Review 6. Renier D, Sainte-Rose C, Marchac D, et al. Intracranial pressure in craniostenosis. J Neurosurg 1982;57:370–377 7. Remmler D, McCoy FJ, O’Neil D, et al. Osseous expansion of the cranial vault by craniotasis. Plast Reconstr Surg 1992;89:787–797 8. Ten Cate AR, Freeman E, Dickinson JB. Sutural development: structure and its response to rapid expansion. Am J Orthod 1977;71:622–636 9. Persing JA, Babler WJ, Nagorsky MJ, et al. Skull expansion in experimental craniosynostosis. Plast Reconstr Surg 1986;78:594–603 10. Persing JA, Morgan EP, Cronin AJ, et al. Skull base expansion: craniofacial effects. Plast Reconstr Surg 1991;87:1028–1033 11. Hopper RA. New trends in cranio-orbital and midface distraction for craniofacial dysostosis. Curr Opin Otolaryngol Head Neck Surg 2012;20:298–303 12. Uemura T, Hayashi T, Satoh K, et al. Three-dimensional cranial expansion using distraction osteogenesis for oxycephaly. J Craniofac Surg 2003;14:29–36 13. Kim SW, Shim KW, Plesnila N, et al. Distraction vs remodeling surgery for craniosynostosis. Childs Nerv Syst 2007;23:201–206 14. Akai T, Iizuka H, Kawakami S. Treatment of craniosynostosis by distraction osteogenesis. Pediatr Neurosurg 2006;42:288–292 15. Kim YO, Choi JW, Kim DS, et al. Cranial growth after distraction osteogenesis of the craniosynostosis. J Craniofac Surg 2008;19:45–55 16. Choi JW, Ra YS, Hong SH, et al. Use of distraction osteogenesis to change endocranial morphology in unilateral coronal craniosynostosis patients. Plast Reconstr Surg 2010;126:995–1004 17. Pelo S, Gasparini G, Di Petrillo A, et al. Distraction osteogenesis in the surgical treatment of craniostenosis: a comparison of internal and external craniofacial distractor devices. Child’s Nerv Syst 2007;23:1447–1453 18. Esparza J, Hinojosa J, García-Recuero I, et al. Surgical treatment of isolated and syndromic craniosynostosis. Results and complications in 283 consecutive cases. Neurocirugia (Astur) 2008;19:509–529 19. Deschamps-Braly J, Hettinger P, el Amm C, et al. Volumetric analysis of cranial vault distraction for cephalocranial disproportion. Pediatr Neurosurg 2011;47:396–405 20. Goldstein JA, Paliga JT, Bailey RL, et al. Posterior vault distraction with midface distraction without osteotomy as a first stage for syndromic craniosynostosis. J Craniofac Surg 2013;24:1263–1267 21. Yamaguchi K, Imai K, Fujimoto T, et al. Cranial distraction osteogenesis for syndromic craniosynostosis: long-term follow-up and effect on postoperative cranial growth. J Plast Reconstr Aesthet Surg 2014;67:e35–41
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
