The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Brief Clinical Studies
FIGURE 5. Complete epithelialization after 4 weeks postoperatively.
Neder5 presented 2 clinical cases using free graft of the BFP to portray surgical, pathologic, and cosmetic problems, showing good results without loss of the fat tissue transplanted. The surgical technique recommended in this study uses the free graft of the BFP in oronasal fistula remaining after cleft lip and palate surgery. Closure of the fistula was performed in 3 planes: the deep plane was obtained with the own nasal fistula tissue; the intermediate plane was made by apposition of the free graft of the BFP; and the superficial plane was composed by the buccal surrounding mucosa, promoting obliteration of “dead space” between the oral and nasal cavity. The BPF proved to be an excellent autogenous tissue, representing a reliable alternative procedure for cleft palate surgery.13,16 It is a simple and quick procedure that can be performed under local anesthesia, presenting low morbidity and low incidence of failure, without loss of depth of the vestibular sulcus.4,15 However, some complications have been reported in the literature using the BFP to repair these defects: hematoma, infection, facial nerve trauma, arterial bleeding, morbidity at the donor site, trismus, wound dehiscence, and excess of granulomatous tissue.1,4,17 In the current study, only mild pain and discreet swelling in the cheek region were observed with spontaneous regression after 5 days. Previous studies7,17 reported a complete epithelialization period of 4 to 6 weeks. Clinically, the surface of the orally exposed yellowish-white fat exhibited 3 days after surgery gradually became red within a week, which is most likely due to the formation of young granulation tissue. After 3 weeks, there is epithelialization of the fat tissue and a quick retraction of the wound.18 Histologically, at the fourth week, the BFP presents a surface completely covered by parakeratinized stratified squamous epithelium, with the subepithelial tissue already showing dense fibrous connective tissue, with no further signals of the presence of fat, suggesting tissue metaplasia.10 The current case corroborates clinically with the findings described in the literature. One of the blood supplies that ensure the vasculature of the maxilla is the portion of the gingiva and the buccal alveolar mucosa19; therefore, the blood supply from the contralateral soft tissue, along with the surgical technique, was sufficient to prevent the resorption and to aid in the fat tissue metaplasia, leading to the successful use of the BFP as free graft. The current study represents the first case, in the English literature, reporting the use of a free graft of the BFP for closure of oronasal fistula in a cleft palate patient, providing new features and advantages for its use in reconstructive surgery for cleft lip and palate.
REFERENCES 1. Asthiani AK, Fatemi MJ, Pooli AHM, et al. Closure of palatal fistula with buccal fat pad flap. Int J Oral Maxillofac Surg 2011;40:250–254 2. Egyedi P. Utilization of the buccal fat pad for closure of oro-antral and/or oronasal communications. J Maxillofac Surg 1977;5:241–244 3. Abuabara A, Cortez ALV, Passeri LA, et al. Evaluation of different treatments for oroantral/oronasal communications: experience of 112 cases. Int J Oral Maxillofac Surg 2006;35:155–158 4. Jain MK, Ramesh C, Sankar K, et al. Pedicled buccal fat pad in the management of oroantral fistula: a clinical study of 15 cases. Int J Oral Maxillofac Surg 2012;41:1025–1029 5. Neder A. Use of buccal fat pad. Oral Surg Oral Med Oral Pathol 1983;55:349–350
e16
6. Zhang HM, Yan YP, Qi KM, et al. Anatomical structure of the buccal fat pad and its clinical adaptations. Plast Reconstr Surg 2002;109:2509–2518 7. Tideman H, Bosanquet A, Scott J. Use of the buccal fat pad as a pedicled graft. J Oral Maxillofac Surg 1986;44:435–440 8. Amaratunga NA. Occurrence of oronasal fistulas in operated cleft palate patients. J Oral Maxillofac Surg 1988;46:834–838 9. Smith DM, Vecchione L, Jiang S, et al. The Pittsburgh Fistula Classification System: a standardized scheme for the description of palatal fistulas. Cleft Palate Craniofac J 2007;44:590–594 10. Samman N, Cheung LK, Tideman H. The buccal fat pad in oral reconstruction. Int J Oral Maxillofac Surg 1993;22:2–6 11. Ashtiani AK, Emami SA, Rasti M. Closure of complicated palatal fistula with facial artery musculomucosal flap. Plast Reconstr Surg 2005;116:381–386 12. Schwabegger AH, Hubli E, Rieger M, et al. Role of free-tissue transfer in the treatment of recalcitrante palatal fistulae among patients with cleft palates. Plast Reconstr Surg 2004;113:1131–1139 13. Gröbe A, Eichhorn W, Hanken H, et al. The use of buccal fat pad (BFP) as a pedicled graft in cleft palate surgery. Int J Oral Maxillofac Surg 2011;40:685–689 14. Toshihiro Y, Nariai Y, Takamura Y, et al. Applicability of buccal fat pad grafting for oral reconstruction. Int J Oral Maxillofac Surg 2013;42:604–610 15. Singh J, Prasad K, Lalitha RM, et al. Buccal pad of fat and its applications in oral and maxillofacial surgery: a review of published literature (February) 2004 to (July) 2009. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:698–705 16. Hudson JW, Anderson G, Russell RM Jr, et al. Use of pedicled fat pad as an adjunct in the reconstruction of palatal cleft defects. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:24–27 17. Rapidis AD, Alexandridis CA, Eleftheriadis E, et al. The use of the buccal fat pad for reconstruction of oral defects: review of the literature and report of 15 cases. J Oral Maxillofac Surg 2000;58:158–163 18. Hanazawa Y, Itoh K, Mabashi T, et al. Closure of oroantral communications using a pedicled buccal fat pad graft. J Oral Maxillofac Surg 1995;53;771–775 19. Jensen OT, Bell W, Cottam J. Osteoperiosteal flaps and local osteotomies for alveolar reconstruction. Oral Maxillofac Surg Clin North Am 2010;22:331–346
Mandibular Lengthening by Distraction Osteogenesis in the Setting of Osteogenesis Imperfecta Jonathan S. Black, MD,*† Arlen D. Denny, MD, FACS*† Abstract: Osteogenesis imperfecta (OI) is an inherited disorder characterized by bone fragility and deformity. The craniofacial skeleton may be involved either primarily or by result of a concomitant From the *Department of Plastic Surgery, and †Center for Craniofacial Disorders, Medical College of Wisconsin, Milwaukee, WI. Received December 9, 2013. Accepted for publication July 23, 2014. Address correspondence and reprint requests to Jonathan S. Black, MD, Center for Craniofacial Disorders, PO Box 1997, MS C340, Milwaukee, WI 53201-1997; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001232
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
diagnosis. Distraction osteogenesis has emerged as a versatile reconstructive option for many craniofacial deformities. Mandibular lengthening by distraction has not been reported in a patient with OI. We present a patient in whom mandibular lengthening was successfully performed twice for hemifacial microsomia. Bilateral lengthening was initially performed with successful airway improvement. This was followed by transport distraction on the more severely affected side for condylar reconstruction. Successful mandibular lengthening by distraction is possible in the setting of OI. Key Words: Osteogenesis imperfecta, distraction, hemifacial microsomia
O
steogenesis imperfecta (OI) is an inheritable connective tissue disorder with an incidence of approximately 1 in 20,000 births.1 Most patients carry an autosomal dominant mutation on either the COL1A1 or COL1A2 genes.2 This affects the structure of type 1 collagen α chains, resulting in either decreased collagen production or weakened collagen. The variety of mutations described accounts for the heterogeneity of clinical severity seen. The disease has been classified into 3 clinical categories with 9 major subtypes on the basis of clinical manifestation. Category I is a mild disease with a variable fracture rate, normal stature, and minimal deformity. Category II is lethal, representing the most severe form. Those affected often die in utero or early infancy. Category III encompasses the intermediate disease subtypes of III to IX, with subtype III being the most severe. These patients experience frequent fractures, bony deformities, short stature, kyphoscoliosis, and dentofacial malformations with malocclusion. Distraction osteogenesis has been successfully reported in correcting limb length discrepancies in those with OI.3,4 Craniofacial applications in this setting have been scarce, including only case reports. Mandibular lengthening by distraction osteogenesis has not been reported in patients with OI. The authors describe their experience in a case of type IV OI associated with bilateral hemifacial microsomia.
CLINICAL REPORT A 10-month-old male infant was referred to the Center of Craniofacial Disorders at the Children's Hospital of Wisconsin for micrognathia and associated airway obstruction. He was born with bilateral hemifacial microsomia with asymmetric vertical shortening of both mandibular rami, which was worse on the left (Pruzansky type 2). His left zygomatic arch was absent and he had bilateral microtia. He required tube feedings and demonstrated intermittent signs of airway obstruction prompting referral. In addition to his craniofacial anomalies, this patient was also diagnosed with OI type IV. He repeatedly presented to another facility with fractures at multiple sites prompting further evaluation and confirmation of the diagnosis near the age of 1 year. He began treatment with Fosamax and continued this therapy throughout his treatment at our center. Polysomnography demonstrated 93 obstructive apneas and 10 obstructive hypopneas for an index of 14. This was worse in rapid eye
FIGURE 1. Preoperative computed tomographic (CT) scans (bilateral distraction). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
Brief Clinical Studies
FIGURE 2. Preoperative CT scans (unilateral distraction, left). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
movement (REM) sleep with an REM index of 22. He spent 1.8% of the study with oxygen saturation below 80% and was diagnosed as having moderately severe obstructive sleep apnea. A bronchoscopy was performed, confirming normal laryngeal airway anatomy and obstruction at the base of the tongue. He underwent bilateral osteotomies of the mandibular ramus with external distractor device placement at 12 months of age. His micrognathia was confirmed on preoperative imaging as primarily deficient vertically (Fig. 1). The devices were oriented parallel to the posterior borders of the rami for vertical lengthening. The external distractors were placed with 2 pins on each side of the osteotomies. After a latency period of 5 days, the devices were activated at a rate of 1 mm/d for a total distraction length of 10.5 mm. This brought the mandible into a class 1 incisor relationship with the maxilla. After a 6-week consolidation period, the patient was taken to the operating room where an examination under anesthesia confirmed bony stability and the distractors were removed. His mother reported significant improvement in feeding with the ability to now eat pureed foods without difficulty. He no longer required tube feedings or had any obstructive airway events. A postoperative polysomnogram demonstrated significant improvement with a decrease in the apnea hypopnea index to 1.8 and the REM index to 2.9. Oxygen saturation was maintained above 90% throughout the study. This study was repeated 1 year after the surgery and had normal results. At the age of 4 years, he was examined for worsening leftward and vertical deviation of the mandible. Preoperative imaging showed hypoplasia of the left condyle and condylar neck consistent with his known congenital deformity on that side (Pruzansky type 2). (Fig. 2) This was consistent with poor growth on the left side compared with the less affected right side. He underwent left mandibular condyle lengthening using transport distraction. The left mandibular ramus and condyle were exposed through an external approach. A C-shaped osteotomy was made from the apex of the sigmoid notch inferiorly to the posterior border of the mandible. An external distractor was placed with 2 pins on each side of the osteotomy. After a latency period of 5 days, the device was activated at a rate of 1 mm/d for a total distraction length of 8 mm. After a 6-week consolidation period, the patient was taken to the operating room where examination under anesthesia confirmed bony stability and the distractor was removed. Postoperative imaging at 1 year after the surgery demonstrated excellent ossification and maintenance of distraction length (Fig. 3). Now, 8 years after his unilateral distraction, he has maintained a satisfactory occlusal relationship and symmetry (Fig. 4). He has not required further orthognathic surgery or distraction. Despite his
FIGURE 3. Postoperative CT scans (3 y after unilateral distraction, 6 y after bilateral distraction). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Brief Clinical Studies
FIGURE 4. Postoperative photographs (age, 6 y). A, Anteroposterior view. B and C, Lateral views.
diagnosis of OI, he has not sustained a fracture of the mandible including the regenerated sites of either distraction procedure.
DISCUSSION Distraction osteogenesis is a frequently used technique for broad application throughout the craniofacial skeleton. In particular, mandibular lengthening in the setting of micrognathia or hemifacial microsomia has been described with great success.5 Its many advantages include large advancements without the need for bone grafting, less surgical morbidity, and accompanying soft tissue changes and diminished relapse. Scattered reports on the successful use of distraction techniques in the setting of OI exist. Craniofacial application has been limited to 2 reports, both in adults. The first detailed Le Fort I maxillary advancement by distraction for hypoplasia attributed to the patient's OI.6 The second is a detailed treatment of a segmental defect of the mandibular body after tumor resection with transport distraction.7 The authors decreased their distraction rate to 0.5 mm/d out of caution. Distraction osteogenesis has not been previously reported for mandibular lengthening or condylar reconstruction by transport in the setting of OI. The patient presented is an illustration as to the outcomes possible with distraction of the mandible. Initially, his micrognathia was corrected, resolving his issues with both breathing and feeding. This was followed by transport distraction for condylar lengthening in correction of left mandibular deformity relating to his diagnosis of hemifacial microsomia. Both treatments were successful without fracture of the regenerated or hardware complications. He has demonstrated durability of the reconstruction now 8 years from his condylar transport without fracture of the mandible. Many concerns regarding the distraction technique applied to inherently fragile bone exist. The abnormal collagen risks compromise of the osteotomy, hardware fixation, and fracture of the regenerated complication. Despite bony fragility, the management and healing of fractures for patients with OI are similar to those with normal bone. This is evidenced by the ability of patients with OI to successfully heal their many fractures with standard techniques. Distraction is fundamentally a method to alter fracture healing and allow for greater amounts of bony deposition. Therefore, the physiology of distraction osteogenesis parallels fracture healing and is feasible in patients with OI. Mandibular lengthening and condylar reconstruction are possible using distraction osteogenesis in a patient with OI. No complications relating to hardware fixation or regenerate fracturing were observed. This is the first report to document the use of either of these distraction techniques in a patient with OI.
REFERENCES 1. Marini JC. Osteogenesis imperfecta: comprehensive management. Adv Pediatr 1988;35:391 2. Gajko-Galicka A. Mutations in type I collagen genes resulting in osteogenesis imperfecta in humans. Acta Biochim Pol 2002;49:433 3. Saldanha KA, Saleh M, Bell MJ, et al. Limb lengthening and correction of deformity in the lower limbs of children with osteogenesis imperfecta. J Bone Joint Surg Br 2004;86:259–265
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4. Sulko J, Radlo W. Limb lengthening in children with osteogenesis imperfecta. Chir Narzadow Ruchu Ortop Pol 2005;70:243–247 5. Yu J, Fearon J, Havlik R, et al. Distraction osteogenesis of the craniofacial skeleton. Plast Reconstr Surg 2004;114:1e–20e 6. Binger T, Rucker M, Spitzer WJ. Dentofacial rehabilitation by osteodistraction, augmentation and implantation despite osteogenesis imperfecta. Int J Oral Maxillofac Surg 2006;35:559–562 7. Muraki Y, Tominaga K, Yoshioka I, et al. Mandibular reconstruction with bone transport in a patient with osteogenesis imperfecta. Int J Oral Maxillofac Surg 2008;37:870–873
The Use of a Computed Tomographic Application for Mobile Devices in the Diagnosis of Oral and Maxillofacial Surgery Eduardo Massaharu Aoki, DDS, Arthur Rodriguez Gonzalez Cortes, DDS, PhD, Emiko Saito Arita, DDS, PhD Abstract: The aim of the current technical report was to introduce a computed tomographic (CT) application for mobile devices as a diagnostic tool for analyzing CT images. An iPad and an iPhone (Apple, Cuppertino, CA) were used to navigate through multiplanar reconstructions of cone beam CT scans, using an application derived from the OsiriX CT software. Tools and advantages of this method were recorded. In addition, images rendered in the iPad were manipulated during dental implant placement and grafting procedures to follow up and confirm the implant digital planning in real time. The study population consisted of 10 patients. In all cases, it was possible to use image manipulation tools, such as changing contrast and brightness, zooming, rotating, panning, performing both linear and area measurements, and analyzing gray-scale values of a region of interest. Furthermore, it was possible to use the OsiriX application in the dental clinic where the study was conducted, to follow-up the analyzed implant placement and grafting procedures at the chairside. The current findings suggest that technological and practical methods to visualize radiographic images are invaluable resources to improve training, teaching, networking, and the performance of real-time follow-up of oral and maxillofacial surgical procedures. This article discusses the advantages and disadvantages of introducing this new technology in the clinical routine. Key Words: Cone beam computed tomography, mobile devices, maxillofacial surgery, implant placement, networking From the Department of Oral Radiology, School of Dentistry, University of Sao Paulo, Sao Paulo, Brazil. Received May 14, 2014. Accepted for publication July 23, 2014. Address correspondence and reprint requests to Eduardo Massaharu Aoki, DDS, Department of Oral Radiology, School of Dentistry, University of Sao Paulo, Av. Prof. Lineu Prestes, 2227, Sao Paulo, SP, 05508-000, Brazil; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001249
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
FIGURE 5. Complete epithelialization after 4 weeks postoperatively.
Neder5 presented 2 clinical cases using free graft of the BFP to portray surgical, pathologic, and cosmetic problems, showing good results without loss of the fat tissue transplanted. The surgical technique recommended in this study uses the free graft of the BFP in oronasal fistula remaining after cleft lip and palate surgery. Closure of the fistula was performed in 3 planes: the deep plane was obtained with the own nasal fistula tissue; the intermediate plane was made by apposition of the free graft of the BFP; and the superficial plane was composed by the buccal surrounding mucosa, promoting obliteration of “dead space” between the oral and nasal cavity. The BPF proved to be an excellent autogenous tissue, representing a reliable alternative procedure for cleft palate surgery.13,16 It is a simple and quick procedure that can be performed under local anesthesia, presenting low morbidity and low incidence of failure, without loss of depth of the vestibular sulcus.4,15 However, some complications have been reported in the literature using the BFP to repair these defects: hematoma, infection, facial nerve trauma, arterial bleeding, morbidity at the donor site, trismus, wound dehiscence, and excess of granulomatous tissue.1,4,17 In the current study, only mild pain and discreet swelling in the cheek region were observed with spontaneous regression after 5 days. Previous studies7,17 reported a complete epithelialization period of 4 to 6 weeks. Clinically, the surface of the orally exposed yellowish-white fat exhibited 3 days after surgery gradually became red within a week, which is most likely due to the formation of young granulation tissue. After 3 weeks, there is epithelialization of the fat tissue and a quick retraction of the wound.18 Histologically, at the fourth week, the BFP presents a surface completely covered by parakeratinized stratified squamous epithelium, with the subepithelial tissue already showing dense fibrous connective tissue, with no further signals of the presence of fat, suggesting tissue metaplasia.10 The current case corroborates clinically with the findings described in the literature. One of the blood supplies that ensure the vasculature of the maxilla is the portion of the gingiva and the buccal alveolar mucosa19; therefore, the blood supply from the contralateral soft tissue, along with the surgical technique, was sufficient to prevent the resorption and to aid in the fat tissue metaplasia, leading to the successful use of the BFP as free graft. The current study represents the first case, in the English literature, reporting the use of a free graft of the BFP for closure of oronasal fistula in a cleft palate patient, providing new features and advantages for its use in reconstructive surgery for cleft lip and palate.
REFERENCES 1. Asthiani AK, Fatemi MJ, Pooli AHM, et al. Closure of palatal fistula with buccal fat pad flap. Int J Oral Maxillofac Surg 2011;40:250–254 2. Egyedi P. Utilization of the buccal fat pad for closure of oro-antral and/or oronasal communications. J Maxillofac Surg 1977;5:241–244 3. Abuabara A, Cortez ALV, Passeri LA, et al. Evaluation of different treatments for oroantral/oronasal communications: experience of 112 cases. Int J Oral Maxillofac Surg 2006;35:155–158 4. Jain MK, Ramesh C, Sankar K, et al. Pedicled buccal fat pad in the management of oroantral fistula: a clinical study of 15 cases. Int J Oral Maxillofac Surg 2012;41:1025–1029 5. Neder A. Use of buccal fat pad. Oral Surg Oral Med Oral Pathol 1983;55:349–350
e16
6. Zhang HM, Yan YP, Qi KM, et al. Anatomical structure of the buccal fat pad and its clinical adaptations. Plast Reconstr Surg 2002;109:2509–2518 7. Tideman H, Bosanquet A, Scott J. Use of the buccal fat pad as a pedicled graft. J Oral Maxillofac Surg 1986;44:435–440 8. Amaratunga NA. Occurrence of oronasal fistulas in operated cleft palate patients. J Oral Maxillofac Surg 1988;46:834–838 9. Smith DM, Vecchione L, Jiang S, et al. The Pittsburgh Fistula Classification System: a standardized scheme for the description of palatal fistulas. Cleft Palate Craniofac J 2007;44:590–594 10. Samman N, Cheung LK, Tideman H. The buccal fat pad in oral reconstruction. Int J Oral Maxillofac Surg 1993;22:2–6 11. Ashtiani AK, Emami SA, Rasti M. Closure of complicated palatal fistula with facial artery musculomucosal flap. Plast Reconstr Surg 2005;116:381–386 12. Schwabegger AH, Hubli E, Rieger M, et al. Role of free-tissue transfer in the treatment of recalcitrante palatal fistulae among patients with cleft palates. Plast Reconstr Surg 2004;113:1131–1139 13. Gröbe A, Eichhorn W, Hanken H, et al. The use of buccal fat pad (BFP) as a pedicled graft in cleft palate surgery. Int J Oral Maxillofac Surg 2011;40:685–689 14. Toshihiro Y, Nariai Y, Takamura Y, et al. Applicability of buccal fat pad grafting for oral reconstruction. Int J Oral Maxillofac Surg 2013;42:604–610 15. Singh J, Prasad K, Lalitha RM, et al. Buccal pad of fat and its applications in oral and maxillofacial surgery: a review of published literature (February) 2004 to (July) 2009. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:698–705 16. Hudson JW, Anderson G, Russell RM Jr, et al. Use of pedicled fat pad as an adjunct in the reconstruction of palatal cleft defects. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:24–27 17. Rapidis AD, Alexandridis CA, Eleftheriadis E, et al. The use of the buccal fat pad for reconstruction of oral defects: review of the literature and report of 15 cases. J Oral Maxillofac Surg 2000;58:158–163 18. Hanazawa Y, Itoh K, Mabashi T, et al. Closure of oroantral communications using a pedicled buccal fat pad graft. J Oral Maxillofac Surg 1995;53;771–775 19. Jensen OT, Bell W, Cottam J. Osteoperiosteal flaps and local osteotomies for alveolar reconstruction. Oral Maxillofac Surg Clin North Am 2010;22:331–346
Mandibular Lengthening by Distraction Osteogenesis in the Setting of Osteogenesis Imperfecta Jonathan S. Black, MD,*† Arlen D. Denny, MD, FACS*† Abstract: Osteogenesis imperfecta (OI) is an inherited disorder characterized by bone fragility and deformity. The craniofacial skeleton may be involved either primarily or by result of a concomitant From the *Department of Plastic Surgery, and †Center for Craniofacial Disorders, Medical College of Wisconsin, Milwaukee, WI. Received December 9, 2013. Accepted for publication July 23, 2014. Address correspondence and reprint requests to Jonathan S. Black, MD, Center for Craniofacial Disorders, PO Box 1997, MS C340, Milwaukee, WI 53201-1997; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001232
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
diagnosis. Distraction osteogenesis has emerged as a versatile reconstructive option for many craniofacial deformities. Mandibular lengthening by distraction has not been reported in a patient with OI. We present a patient in whom mandibular lengthening was successfully performed twice for hemifacial microsomia. Bilateral lengthening was initially performed with successful airway improvement. This was followed by transport distraction on the more severely affected side for condylar reconstruction. Successful mandibular lengthening by distraction is possible in the setting of OI. Key Words: Osteogenesis imperfecta, distraction, hemifacial microsomia
O
steogenesis imperfecta (OI) is an inheritable connective tissue disorder with an incidence of approximately 1 in 20,000 births.1 Most patients carry an autosomal dominant mutation on either the COL1A1 or COL1A2 genes.2 This affects the structure of type 1 collagen α chains, resulting in either decreased collagen production or weakened collagen. The variety of mutations described accounts for the heterogeneity of clinical severity seen. The disease has been classified into 3 clinical categories with 9 major subtypes on the basis of clinical manifestation. Category I is a mild disease with a variable fracture rate, normal stature, and minimal deformity. Category II is lethal, representing the most severe form. Those affected often die in utero or early infancy. Category III encompasses the intermediate disease subtypes of III to IX, with subtype III being the most severe. These patients experience frequent fractures, bony deformities, short stature, kyphoscoliosis, and dentofacial malformations with malocclusion. Distraction osteogenesis has been successfully reported in correcting limb length discrepancies in those with OI.3,4 Craniofacial applications in this setting have been scarce, including only case reports. Mandibular lengthening by distraction osteogenesis has not been reported in patients with OI. The authors describe their experience in a case of type IV OI associated with bilateral hemifacial microsomia.
CLINICAL REPORT A 10-month-old male infant was referred to the Center of Craniofacial Disorders at the Children's Hospital of Wisconsin for micrognathia and associated airway obstruction. He was born with bilateral hemifacial microsomia with asymmetric vertical shortening of both mandibular rami, which was worse on the left (Pruzansky type 2). His left zygomatic arch was absent and he had bilateral microtia. He required tube feedings and demonstrated intermittent signs of airway obstruction prompting referral. In addition to his craniofacial anomalies, this patient was also diagnosed with OI type IV. He repeatedly presented to another facility with fractures at multiple sites prompting further evaluation and confirmation of the diagnosis near the age of 1 year. He began treatment with Fosamax and continued this therapy throughout his treatment at our center. Polysomnography demonstrated 93 obstructive apneas and 10 obstructive hypopneas for an index of 14. This was worse in rapid eye
FIGURE 1. Preoperative computed tomographic (CT) scans (bilateral distraction). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
Brief Clinical Studies
FIGURE 2. Preoperative CT scans (unilateral distraction, left). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
movement (REM) sleep with an REM index of 22. He spent 1.8% of the study with oxygen saturation below 80% and was diagnosed as having moderately severe obstructive sleep apnea. A bronchoscopy was performed, confirming normal laryngeal airway anatomy and obstruction at the base of the tongue. He underwent bilateral osteotomies of the mandibular ramus with external distractor device placement at 12 months of age. His micrognathia was confirmed on preoperative imaging as primarily deficient vertically (Fig. 1). The devices were oriented parallel to the posterior borders of the rami for vertical lengthening. The external distractors were placed with 2 pins on each side of the osteotomies. After a latency period of 5 days, the devices were activated at a rate of 1 mm/d for a total distraction length of 10.5 mm. This brought the mandible into a class 1 incisor relationship with the maxilla. After a 6-week consolidation period, the patient was taken to the operating room where an examination under anesthesia confirmed bony stability and the distractors were removed. His mother reported significant improvement in feeding with the ability to now eat pureed foods without difficulty. He no longer required tube feedings or had any obstructive airway events. A postoperative polysomnogram demonstrated significant improvement with a decrease in the apnea hypopnea index to 1.8 and the REM index to 2.9. Oxygen saturation was maintained above 90% throughout the study. This study was repeated 1 year after the surgery and had normal results. At the age of 4 years, he was examined for worsening leftward and vertical deviation of the mandible. Preoperative imaging showed hypoplasia of the left condyle and condylar neck consistent with his known congenital deformity on that side (Pruzansky type 2). (Fig. 2) This was consistent with poor growth on the left side compared with the less affected right side. He underwent left mandibular condyle lengthening using transport distraction. The left mandibular ramus and condyle were exposed through an external approach. A C-shaped osteotomy was made from the apex of the sigmoid notch inferiorly to the posterior border of the mandible. An external distractor was placed with 2 pins on each side of the osteotomy. After a latency period of 5 days, the device was activated at a rate of 1 mm/d for a total distraction length of 8 mm. After a 6-week consolidation period, the patient was taken to the operating room where examination under anesthesia confirmed bony stability and the distractor was removed. Postoperative imaging at 1 year after the surgery demonstrated excellent ossification and maintenance of distraction length (Fig. 3). Now, 8 years after his unilateral distraction, he has maintained a satisfactory occlusal relationship and symmetry (Fig. 4). He has not required further orthognathic surgery or distraction. Despite his
FIGURE 3. Postoperative CT scans (3 y after unilateral distraction, 6 y after bilateral distraction). A, Anteroposterior view. B, Right lateral view. C, Left lateral view.
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
Brief Clinical Studies
FIGURE 4. Postoperative photographs (age, 6 y). A, Anteroposterior view. B and C, Lateral views.
diagnosis of OI, he has not sustained a fracture of the mandible including the regenerated sites of either distraction procedure.
DISCUSSION Distraction osteogenesis is a frequently used technique for broad application throughout the craniofacial skeleton. In particular, mandibular lengthening in the setting of micrognathia or hemifacial microsomia has been described with great success.5 Its many advantages include large advancements without the need for bone grafting, less surgical morbidity, and accompanying soft tissue changes and diminished relapse. Scattered reports on the successful use of distraction techniques in the setting of OI exist. Craniofacial application has been limited to 2 reports, both in adults. The first detailed Le Fort I maxillary advancement by distraction for hypoplasia attributed to the patient's OI.6 The second is a detailed treatment of a segmental defect of the mandibular body after tumor resection with transport distraction.7 The authors decreased their distraction rate to 0.5 mm/d out of caution. Distraction osteogenesis has not been previously reported for mandibular lengthening or condylar reconstruction by transport in the setting of OI. The patient presented is an illustration as to the outcomes possible with distraction of the mandible. Initially, his micrognathia was corrected, resolving his issues with both breathing and feeding. This was followed by transport distraction for condylar lengthening in correction of left mandibular deformity relating to his diagnosis of hemifacial microsomia. Both treatments were successful without fracture of the regenerated or hardware complications. He has demonstrated durability of the reconstruction now 8 years from his condylar transport without fracture of the mandible. Many concerns regarding the distraction technique applied to inherently fragile bone exist. The abnormal collagen risks compromise of the osteotomy, hardware fixation, and fracture of the regenerated complication. Despite bony fragility, the management and healing of fractures for patients with OI are similar to those with normal bone. This is evidenced by the ability of patients with OI to successfully heal their many fractures with standard techniques. Distraction is fundamentally a method to alter fracture healing and allow for greater amounts of bony deposition. Therefore, the physiology of distraction osteogenesis parallels fracture healing and is feasible in patients with OI. Mandibular lengthening and condylar reconstruction are possible using distraction osteogenesis in a patient with OI. No complications relating to hardware fixation or regenerate fracturing were observed. This is the first report to document the use of either of these distraction techniques in a patient with OI.
REFERENCES 1. Marini JC. Osteogenesis imperfecta: comprehensive management. Adv Pediatr 1988;35:391 2. Gajko-Galicka A. Mutations in type I collagen genes resulting in osteogenesis imperfecta in humans. Acta Biochim Pol 2002;49:433 3. Saldanha KA, Saleh M, Bell MJ, et al. Limb lengthening and correction of deformity in the lower limbs of children with osteogenesis imperfecta. J Bone Joint Surg Br 2004;86:259–265
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4. Sulko J, Radlo W. Limb lengthening in children with osteogenesis imperfecta. Chir Narzadow Ruchu Ortop Pol 2005;70:243–247 5. Yu J, Fearon J, Havlik R, et al. Distraction osteogenesis of the craniofacial skeleton. Plast Reconstr Surg 2004;114:1e–20e 6. Binger T, Rucker M, Spitzer WJ. Dentofacial rehabilitation by osteodistraction, augmentation and implantation despite osteogenesis imperfecta. Int J Oral Maxillofac Surg 2006;35:559–562 7. Muraki Y, Tominaga K, Yoshioka I, et al. Mandibular reconstruction with bone transport in a patient with osteogenesis imperfecta. Int J Oral Maxillofac Surg 2008;37:870–873
The Use of a Computed Tomographic Application for Mobile Devices in the Diagnosis of Oral and Maxillofacial Surgery Eduardo Massaharu Aoki, DDS, Arthur Rodriguez Gonzalez Cortes, DDS, PhD, Emiko Saito Arita, DDS, PhD Abstract: The aim of the current technical report was to introduce a computed tomographic (CT) application for mobile devices as a diagnostic tool for analyzing CT images. An iPad and an iPhone (Apple, Cuppertino, CA) were used to navigate through multiplanar reconstructions of cone beam CT scans, using an application derived from the OsiriX CT software. Tools and advantages of this method were recorded. In addition, images rendered in the iPad were manipulated during dental implant placement and grafting procedures to follow up and confirm the implant digital planning in real time. The study population consisted of 10 patients. In all cases, it was possible to use image manipulation tools, such as changing contrast and brightness, zooming, rotating, panning, performing both linear and area measurements, and analyzing gray-scale values of a region of interest. Furthermore, it was possible to use the OsiriX application in the dental clinic where the study was conducted, to follow-up the analyzed implant placement and grafting procedures at the chairside. The current findings suggest that technological and practical methods to visualize radiographic images are invaluable resources to improve training, teaching, networking, and the performance of real-time follow-up of oral and maxillofacial surgical procedures. This article discusses the advantages and disadvantages of introducing this new technology in the clinical routine. Key Words: Cone beam computed tomography, mobile devices, maxillofacial surgery, implant placement, networking From the Department of Oral Radiology, School of Dentistry, University of Sao Paulo, Sao Paulo, Brazil. Received May 14, 2014. Accepted for publication July 23, 2014. Address correspondence and reprint requests to Eduardo Massaharu Aoki, DDS, Department of Oral Radiology, School of Dentistry, University of Sao Paulo, Av. Prof. Lineu Prestes, 2227, Sao Paulo, SP, 05508-000, Brazil; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001249
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
