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around the lesion. Thus, long-term antifungal therapy is used as the mainstay of treatment for orbital aspergillosis. As far as the antifungal agent itself is concerned, amphotericin has a higher rate of drug-related complication, and voriconazole could be used as a substitute, recently.7 Some reports also showed comparative treatment result of voriconazole.8,9 Although Murai et al10 had reported a case of complete vision recovery after systemic antifungal medication, visual loss from aspergillosis OAS has a poorer prognosis in general. In addition, aspergillosis OAS has been associated with high mortality rates due to intracranial spread or systemic fungemia in previous reports.5,7,9 Fortunately, in the case of our patient, the symptoms responded to antifungal medication. Despite the delayed diagnosis and steroid treatment, the patient did not show deterioration of general condition or progressive meningitis. In conclusion, early diagnosis and treatment are important for aspergillosis OAS. Diagnostic examination such as biopsy and culture are necessarily in patients with risk factors such as diabetes mellitus, HIV infection, hematologic malignancy, or immunocompromised by organ transplantation.
REFERENCES 1. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol 2004;15:490Y498 2. Bray WH, Giangiacomo J, Ide CH. Orbital apex syndrome. Surv Ophthalmol 1987;32:136Y140 3. Yoon JS, Park HK, Cho NH, et al. Outcomes of three patients with intracranially invasive sino-orbital aspergillosis. Ophthal Plast Reconstr Surg 2007;23:400Y406 4. Aryasit O, Preechawai P, Aui-Aree N. Clinical presentation, aetiology and prognosis of orbital apex syndrome. Orbit 2013;32:91Y94 5. Ismail AR, Clifford L, Meacock WR. Compressive optic neuropathy in fungal hypertrophic cranial pachymeningitis. Eye (Lond) 2007;21:568Y569 6. Blitzer A, Lawson W. Fungal infections of the nose and paranasal sinuses. Part I. Otolaryngol Clin North Am 1993;26:1007Y1035 7. Pushker N, Meel R, Kashyap S, et al. Invasive aspergillosis of orbit in immunocompetent patients: treatment and outcome. Ophthalmology 2011;118:1886Y1891 8. Arakawa H, Suto C, Notani H, et al. Selection of the antifungal agent decides prognosis of invasive aspergillosis: case report of a successful outcome with voriconazole. Int Ophthalmol [published online ahead of print February 11, 2013] doi: 10.1007/s10792-013-9730-x 9. Ohlstein DH, Hooten C, Perez J, et al. Orbital aspergillosis: voriconazoleVthe new standard treatment? Case Rep Ophthalmol 2012;3:46Y53 10. Murai H, Kira J, Kobayashi T, et al. Hypertrophic cranial pachymeningitis due to Aspergillus flavus. Clin Neurol Neurosurg 1992;94:247Y250
Onlay Bone Augmentation and Bilateral Open Sinus Lifting With Simultaneous Implant Placement in a Cherubic Patient Arash Khojasteh, DMD, MS,* Seyed Jalil Sadr, DMD, MSD,Þ Aboulfazl Saboury, DMD, MSD,Þ Shireen Shidfar, DSþ Abstract: A 20-year-old edentulous woman, who was previously treated with the shave of the inferior border of the mandible and malar prominent region for aesthetic facial contouring, was selected for full
Brief Clinical Studies
mouth rehabilitation of the maxillomandibular region. The patient was treated with bilateral open sinus lifting through a lateral approach in the posterior of the maxilla and an onlay bone graft with lateral ramus as a donor site in the mandible anterior. Eight implants in the maxilla and 7 in the mandible were inserted, and implant-supported prostheses were fabricated. The 18-month follow-up showed good bone condition that suggests graft interventions and implant treatment as a good treatment modality for patients with cherubism. Key Words: Cherubism, bone fibrous dysplasia, sinus floor augmentation, bone grafting, dental implants
C
herubism is an uncommon benign fibro-osseous jaw bone disease, which is usually inherited as an autosomal dominant trait.1Y7 This clinical condition is characterized by painless, firm, and symmetrical bilateral expansion of the mandible and maxilla that causes upward turning of the eyes and a full round lower face, which creates a cherub-like appearance.3,4,8Y10 Mandibular angle, ascending ramus, retromolar region, and posterior maxilla are the most often affected sites, and sometimes the coronoid process is involved.3,11 Dental manifestations of cherubism depend on the time of onset and the severity of the disease.12 Disturbance in arrangement of primary teeth can be seen,12Y14 whereas missing and impacted teeth (mostly molars), developmental problems in molars, abnormal tooth shape, partial root resorption, malalignment, and delayed and ectopic eruption are commonly observed in permanent dentition.3,12,13,15 In most cases, the lesions tend to show varying degrees of remission and involution after puberty. Surgical interventions including partial resection, contour resection, and curettage have been suggested for aesthetic or functional considerations.3,11,12,16 In many adult patients, replacing lost or missing teeth seems challenging, because treatment with conventional removable or fixed prosthesis requires the presence of appropriate abutment teeth.3,11 Implant treatment provides significant improvement over conventional treatment in general health-related quality of life17,18; however, considering the implant treatment as an option to support restorations arises concerns about complications such as lack of osseointegration following implant placement into the porous bone of a cherub patient.3,11 Nonetheless, implant treatment has provided satisfactory results in histologically comparable bone lesions including central giant cell granuloma and fibrous dysplasia.19Y22 To the extent of our knowledge, only a few studies have evaluated dental implants as a potential treatment for cherubism. These studies indicate that cherubic patients could be treated with dental implants if treatment is performed in the stable phase of the disease.3,23 The current study presents a 20-year old patient with cherubism in whom bilateral open sinus lifting in the maxilla and onlay augmentation
From the *Department of Oral and Maxillofacial Surgery, Dental School; †Department of Prosthodontics, Dental School; and ‡Gifted and Talented Dental Students Division, Dental School, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Received July 16, 2013. Accepted for publication August 27, 2013. Address correspondence and reprint requests to Arash Khojasteh, DMD, MS, Department of Oral and Maxillofacial Surgery, Dental School, Shahid Beheshti University of Medical Sciences, Daneshjou Blvd, Evin, Tehran, Iran PO 19839; 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.0000000000000433
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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FIGURE 3. Computed tomography scan view. FIGURE 1. The patient’s frontal view, 3 years following facial contour shaving.
The maxillary implants could achieve a maximum 15 N/cm of primary
in the premolar regions of the mandible were performed to allow for full mouth rehabilitation with dental implants.
CLINICAL REPORT A 20-year-old woman, complaining aesthetic issues, was referred to the Department of Oral and Maxillofacial Surgery, Shahid Beheshti University of Medical Sciences, in December 2010 (Fig. 1). Grade IV cherubism was previously diagnosed in the patient based on clinical and radiographic findings such as lesions involving the mandible and maxilla and signs of root resorption.24,25 The preoperative diagnosis was made by carrying out panoramic radiography, combined with a computed tomography scan in whom bilateral sinus pneumatization in the maxilla and impacted teeth in a cyst and bilateral bone resorption with horizontal width reduction of the bone ridge in premolars region were seen, and the bone height to maxillary sinus was 5 to 6 mm (Figs. 2 and 3). Clinical examination revealed malposed and malformed incisors and premolars with short roots in the upper jaw, whereas only 1 premolar existed in the lower jaw (Fig. 4). Three years before referral, the inferior border and posterior body of the mandible on both sides were shaved surgically to correct the shape of the mandible and achieve acceptable aesthetic. After referral, the patient’s condition was evaluated, and the patient showed no recurrence of osseous lesions. The remaining teeth were assigned as hopeless prognosis by the prosthodontist because of the mobility and lack of bone support. The maxillomandibular implant recipient sites were developed under general anesthesia in a 1-stage procedure. Subperiosteal dissections in the anterior and posterior regions of the maxilla and mandible were performed. All remaining teeth were extracted. Additional care was given by the surgeon during the extractions to preserve facial and palatal bony walls. Sinus augmentation was achieved through lateral approaches. The lateral window need had spongious structure and was elevated with the attached Schneiderian membrane easily. Filling of the blood within the sinus cavity was more than a normal condition. The sinus cavity was then packed with natural bovine bone mineral (Cerabone; Botiss Dental GmbH, Berlin, Germany). Simultaneous to bone augmentation, 8 dental implants (Xive; DENTSPLY Friadent, Mannheim, Germany) were installed in the maxilla while drilling was accomplished at a drill speed of 400 revolutions/min.
FIGURE 4. Intraoral view showing abnormal tooth shape, spacing, and malalignment.
stability. In the lower jaw, after buccal and lingual subperiosteal dissection, the anterior bony lesion, which had a cystic lining, was enucleated, and a 10-hole miniplate was installed to decrease the risk of mandibular fracture. Seven dental implants (Xive; DENTSPLY Friadent) were installed in mandible. The posterior of the mandible in both sides had horizontal-type ridge deficiency (width G3 mm), and it resulted in dehiscence defect in the labial side of posterior implants. Thus, simultaneous onlay augmentation was planned for the patient. A 20 40-mm bone block was harvested from the lateral ramus of the right side. The bone density in this part was felt to be softer than usual (D2-D3 nature), and disengagement was easier. Harvested grafts were properly adapted to the recipient site and were fixed by fixation miniscrews (Jeil, Seoul, Republic of Korea, 10 mm in length) in the premolar region. After a 6-month healing period, all the implants had remained well integrated clinically and radiographically (Figs. 5 and 6). The patient was referred to the Division of Prosthodontics for prosthesis fabrication, and implant-supported prosthesis was delivered 6 months later (Fig. 7). Then, the patient was followed up for an additional 18 months. To date, there has been neither complication nor change in the occlusion due to further growth. The patient remained satisfied with the function of the prosthesis and aesthetic
FIGURE 5. Implants loaded in mandible (A) and maxilla (B).
FIGURE 2. Panoramic radiograph of the impacted teeth and a radiolucent lesion in the anterior part of the mandible.
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FIGURE 6. Panoramic view showing implant osseointegration.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
& Volume 25, Number 2, March 2014
Brief Clinical Studies
REFERENCES
FIGURE 7. Intraoral view of the implant-supported prosthesis.
outcome of treatment, and no sign of bone resorption or early implant failure was observed.
DISCUSSION A patient with cherubism is a challenge to clinicians because of the maxillomandibular deformities and other problems such as tooth malalignment, spacing, and missing.3,12,13,15 Moreover, there is a chance for bone resorption in the jaws due to the long-term edentulism. Treatment of cherubism has not been discussed fully in the current literature. Yilmaz et al11 described a prosthetic treatment plan for a cherubic patient by a removable partial denture in the maxilla and a mandibular overdenture using erupted teeth as abutments. The implant option was excluded from treatment because of the porous bone nature of cherubic patient, which could prevent implant osseointegration and lead to complication. In the current case, the whole remaining teeth of the patient were diagnosed as hopeless. Conventional removable denture was not considered in patient’s treatment plan because providing patient demand for aesthetic, fully functional, and comfortable artificial teeth was not feasible by removable denture. Patient’s clinical examinations and radiographs showed bilateral sinus pneumatization in posterior maxilla, cystic cavity in the anterior of the mandible, and horizontal bone deficiency in the posterior mandible. In addition, insufficient horizontal width of mandibular alveolar ridge in the premolar regions suggested the possible need for bone graft. The patient was likely to be in the stable phase of the disease, because there was no evidence of enlargement of facial bony contour in the shaved areas because of surgical shaving of the inferior border of mandible and zygomatic prominences 3 years before mouth rehabilitation. Therefore, placing dental implants was considered as a feasible option providing the optimum outcomes. Prescott et al26 reported 5 cases in which implant placement led to an implant survival rate of 79%. Despite the fact that some of the implants were placed in multilocular radiolucencies, the bone adjacent to the implants looked normal. Hachach-Haram et al23 documented a case under the title of cherubism that was complicated by neurofibromatosis type 1 in which mandibular reconstruction followed by placement of osseointegrated dental implants to restore function was performed. Dewan and Bishop3 suggested dental implant placement for cherubic patients in a stable phase. They used computed tomographyY guided, flapless surgery for the patient, because of its advantage of being minimally invasive. Considering the adequate bone level in their case, no bone graft was indicated before dental implant placement. However, because of several endo-osseous bone lesions and jaw bone loss in the patient of the current study, considering various bone augmentation techniques seemed to be indispensable. Bilateral opensinus lifting through a lateral approach was performed to achieve adequate bone in the posterior maxilla. To the extent of our knowledge, there has been no report regarding augmentation procedures in cherubic patients. Our patient showed stable results of implant-supported fixed restoration in the augmented jaw. All of the implants have survived following a 2-year follow-up.
1. Tiziani V, Reichenberger E, Buzzo CL, et al. The gene for cherubism maps to chromosome 4p16. Am J Hum Genet 1999;65:158Y166 2. Hyckel P, Berndt A, Schleier P, et al. CherubismYnew hypotheses on pathogenesis and therapeutic consequences. J Craniomaxillofac Surg 2005;33:61Y68 3. Dewan K, Bishop K. Management of a patient suffering with cherubism with dental implants. Eur J Prosthodont Restor Dent 2011;19:67 4. Tamgadge A, Modak NV, Bhalera S, et al. Cherubism: a rare case report and literature review. Int J Oral Maxillofac Pathol 2012;3:56Y60 5. Valiathan A, Prashanth V. Cherubism: presentation of a case. Angle Orthod 1997;67:237Y238 6. Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol 2008;2:177Y202 7. de Lange J, van Maarle MC, van den Akker HP, et al. A new mutation in the SH3BP2 gene showing reduced penetrance in a family affected with cherubism. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:378 8. Jones W, Gerrie J, Pritchard J. CherubismVfamilial fibrous dysplasia of the jaws. Surg Br volume 1950;32:334 9. Lima GdMG, Almeida JD, Cabral LAG. Cherubism: clinicoradiographic features and treatment. J Oral Maxillofac Res 2010;1:1Y8 10. Yamaguchi T, Dorfman HD, Eisig S. Cherubism: clinicopathologic features. Skeletal Radiol 1999;28:350Y353 11. Yilmaz B, Ozan O, Karaagaclioglu L, et al. A prosthetic treatment approach for a cherubism patient: a clinical report. J Prosthet Dent 2006;96:313Y316 12. Papadaki ME, Lietman SA, Levine MA, et al. Cherubism: best clinical practice. Orphanet J Rare Dis 2012;7:S6 13. Faircloth W, Edwards RC, Farhood VW. Cherubism involving a mother and daughter: case reports and review of the literature. J Oral Maxillofac Surg 1991;49:535Y542 14. Pontes FS, Ferreira AC, Kato AM, et al. Aggressive case of cherubism: 17-year follow-up. J Pediatr Otorhinolaryngol 2007;71:831Y835 15. Ireland A, Eveson J. Cherubism: a report of a case with an unusual post-extraction complication. Br Dent J 1988;164:116 16. Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions. Oral Maxillofac Surg Clin North Am 2005;17:415 17. Awad M, Locker D, Korner-Bitensky N, et al. Measuring the effect of intra-oral implant rehabilitation on health-related quality of life in a randomized controlled clinical trial. J Dent Res 2000;79:1659Y1663 18. Heydecke G, Locker D, Awad MA, et al. Oral and general health-related quality of life with conventional and implant dentures. Community Dent Oral Epidemiol 2003;31:161Y168 19. Bajwa MS, Ethunandan M, Flood TR. Oral rehabilitation with endosseous implants in a patient with fibrous dysplasia (McCune-Albright syndrome): a case report. J Oral Maxillofac Surg 2008;66:2605Y2608 20. Bahat O, Fontanessi R. Efficacy of implant placement after bone grafting for three-dimensional reconstruction of the posterior jaw. Int J Periodontics Restorative Dent 2001;21:220 21. Cheung L, Samman N, Pang M, et al. Titanium miniplate fixation for osteotomies in facial fibrous dysplasiaVa histologic study of the screw/bone interface. Int J Oral Maxillofac Surg 1995;24:401Y405 22. Lee H, Ercoli C, Fantuzzo JJ, et al. Oral rehabilitation of a 12-year-old patient diagnosed with a central giant cell granuloma using a fibula graft and an implant-supported prosthesis: a clinical report. J Prosthet Dent 2008;99:257Y262 23. Hachach-Haram N, Gerarchi P, Benyon SL, et al. Multidisciplinary surgical management of cherubism complicated by neurofibromatosis type 1. J Craniofac Surg 2011;22:2318Y2322 24. Motamedi MHK. Treatment of cherubism with locally aggressive behavior presenting in adulthood: report of four cases and a proposed new grading system. J Oral Maxillofac Surg 1998;56:1336Y1342
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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25. Raposo-Amaral CE, Guidi MdC, Warren SM. Two-stage surgical treatment of severe cherubism. Ann Plast Surg 2007;58:645Y651 26. Prescott T, Redfors M, Rustad CF, et al. Characterization of a Norwegian cherubism cohort; molecular genetic findings, oral manifestations and quality of life. Eur J Med Genet 2013;56:131Y137
From ‘‘Head to Heart:’’ Reconstruction of a Sternal Cleft With Split Calvarial Bone Graft in a 15-Month-Old Child Ramesh K. Sharma, MCh(Plast Surg), Diplomate Nat. Board(Plast Surg),* Sandeep S. Rana, MCh(Cardiothoracic Surg)Þ Abstract: Sternal cleft is a rare anomaly and has been variously managed by using either autogenous tissue or synthetic materials for bony reconstruction. We report the reconstruction of sternal cleft in a 15-month-old child with a single-piece outer-table cranial bone graft that was harvested by splitting in situ. The reconstructed sternum was covered with bilateral pectoralis major advancement muscle flaps. Key Words: Sternal cleft, outer-table cranial bone graft, single piece, split in situ, pectoralis major muscle flap
S
ternal cleft is a rare developmental anomaly that can vary in size and shape from a narrow linear fissure to a large defect with complete or near-complete separation of the 2 halves of the sternum.1,2 The bony defect can be isolated, associated with herniation of internal organs through the abnormal opening, or can occur as part of complex clinical syndromes consisting of abnormalities of the heart, vessels, and other organs (Cantrell pentalogy, PHACE [posterior fossa malformationsYhemangiomasYarterial anomaliesY cardiac defectsYeye abnormalitiesYsternal cleft and supraumbilical raphe] syndrome).3,4 The heart in these patients lies just under the skin and is vulnerable to even trivial trauma. It is therefore mandatory that the sternum be reconstructed at the earliest to obviate this risk. Many methods have been described for reconstruction of the sterna cleft, and these include use of autogenous tissue or synthetic materials for the closure of this defect. In children, it is desirable that autogenous tissue be used so that a proportionate growth of the reconstructed sternum is possible. Whereas in adults iliac crest and other sources such as rib have been used, these donor sites may not provide enough bone in small children. From the Departments of *Plastic Surgery and †Cardiothoracic and Vascular Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Received May 13, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Ramesh K. Sharma, MCh(Plast Surg), Diplomate Nat. Board(Plast Surg), Department of Plastic Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India; 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.0000000000000399
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We report the use of split cranial bone graft for reconstruction of congenitally missing upper half of the sternum in a 15-monthold child.
CLINICAL REPORT Simran, a 9-month-old female infant, was brought to the plastic surgery services with a pulsating mass in the chest. The sternum was missing in the upper half with the heart lying under the precordium. The mediastinal contents could be seen bulging whenever the child cried. The child also had hemangioma in the lower lip and forehead. She also had linear supraumbilical abdominal wall scarring in the midline that continued upward into the thin precordial skin (Fig. 1, left) (Parental consent was obtained to take photographs of the child). There was no divarication of recti. The child had no respiratory or cardiac distress, and it was decided in consultation with the cardiothoracic surgery colleagues that the reconstruction be undertaken at the earliest. However, the parents were not willing for surgery and were counseled for the same. The parents finally agreed for the operation, and the child was admitted for reconstruction of sternum at about age of 15 months. The computed tomography scan of the chest confirmed the clinical findings of absence of sternum (Fig. 1, right). It was decided to reconstruct the missing sternum with autogenous bone so that it could grow proportionately with the child. Because the requirement of bone would be substantial, the possibility of using cranial bone graft was kept in mind as this can provide large amount of bone even in a small child. The area of the defect was approached through the scarred skin. The pericardium was adherent to the scar; this was freed, and the pericardial sac could be closed primarily with mobilization of the available tissue. The bony defect was defined, and a template of the defect was made (Fig. 2, upper row right). The parieto-occipital area of the skull was chosen to be the site for harvesting the graft as it offered the best contour for the neosternum. The bone graft was harvested as a single piece by splitting the outer table in situ (Fig. 2, middle row). The neosternum was fixed into the defect using titanium plates and screws. The reconstructed sternum was covered with bilaterally mobilized pectoralis major muscle flaps based on the pectoral vessels. The scarred precordial skin was excised, and healthy skin was closed over the defect (Fig. 2, lower row). Postoperative healing was uneventful. Figure 3 shows appearance at the end of 6 weeks. Sternal clefts are classified as being complete or partial.5,6 The involvement of the upper sternum is commonest. Generally, the cleft extends until the fourth costal cartilage.7 There are only a few reports on repair of sternal defects. Hebra et al8 collected 72 cases worldwide up to 1995. Most authors
FIGURE 1. Left column upper row: Sternal defect showing bulge in the precordial area. The skin is thin and discolored. Left column middle row: Hemangioma lower lip and forehead. Left column lower row: Appearance at the age of 15 months. Right column: Computed tomography scan showing extent of sternal cleft.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
& Volume 25, Number 2, March 2014
around the lesion. Thus, long-term antifungal therapy is used as the mainstay of treatment for orbital aspergillosis. As far as the antifungal agent itself is concerned, amphotericin has a higher rate of drug-related complication, and voriconazole could be used as a substitute, recently.7 Some reports also showed comparative treatment result of voriconazole.8,9 Although Murai et al10 had reported a case of complete vision recovery after systemic antifungal medication, visual loss from aspergillosis OAS has a poorer prognosis in general. In addition, aspergillosis OAS has been associated with high mortality rates due to intracranial spread or systemic fungemia in previous reports.5,7,9 Fortunately, in the case of our patient, the symptoms responded to antifungal medication. Despite the delayed diagnosis and steroid treatment, the patient did not show deterioration of general condition or progressive meningitis. In conclusion, early diagnosis and treatment are important for aspergillosis OAS. Diagnostic examination such as biopsy and culture are necessarily in patients with risk factors such as diabetes mellitus, HIV infection, hematologic malignancy, or immunocompromised by organ transplantation.
REFERENCES 1. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol 2004;15:490Y498 2. Bray WH, Giangiacomo J, Ide CH. Orbital apex syndrome. Surv Ophthalmol 1987;32:136Y140 3. Yoon JS, Park HK, Cho NH, et al. Outcomes of three patients with intracranially invasive sino-orbital aspergillosis. Ophthal Plast Reconstr Surg 2007;23:400Y406 4. Aryasit O, Preechawai P, Aui-Aree N. Clinical presentation, aetiology and prognosis of orbital apex syndrome. Orbit 2013;32:91Y94 5. Ismail AR, Clifford L, Meacock WR. Compressive optic neuropathy in fungal hypertrophic cranial pachymeningitis. Eye (Lond) 2007;21:568Y569 6. Blitzer A, Lawson W. Fungal infections of the nose and paranasal sinuses. Part I. Otolaryngol Clin North Am 1993;26:1007Y1035 7. Pushker N, Meel R, Kashyap S, et al. Invasive aspergillosis of orbit in immunocompetent patients: treatment and outcome. Ophthalmology 2011;118:1886Y1891 8. Arakawa H, Suto C, Notani H, et al. Selection of the antifungal agent decides prognosis of invasive aspergillosis: case report of a successful outcome with voriconazole. Int Ophthalmol [published online ahead of print February 11, 2013] doi: 10.1007/s10792-013-9730-x 9. Ohlstein DH, Hooten C, Perez J, et al. Orbital aspergillosis: voriconazoleVthe new standard treatment? Case Rep Ophthalmol 2012;3:46Y53 10. Murai H, Kira J, Kobayashi T, et al. Hypertrophic cranial pachymeningitis due to Aspergillus flavus. Clin Neurol Neurosurg 1992;94:247Y250
Onlay Bone Augmentation and Bilateral Open Sinus Lifting With Simultaneous Implant Placement in a Cherubic Patient Arash Khojasteh, DMD, MS,* Seyed Jalil Sadr, DMD, MSD,Þ Aboulfazl Saboury, DMD, MSD,Þ Shireen Shidfar, DSþ Abstract: A 20-year-old edentulous woman, who was previously treated with the shave of the inferior border of the mandible and malar prominent region for aesthetic facial contouring, was selected for full
Brief Clinical Studies
mouth rehabilitation of the maxillomandibular region. The patient was treated with bilateral open sinus lifting through a lateral approach in the posterior of the maxilla and an onlay bone graft with lateral ramus as a donor site in the mandible anterior. Eight implants in the maxilla and 7 in the mandible were inserted, and implant-supported prostheses were fabricated. The 18-month follow-up showed good bone condition that suggests graft interventions and implant treatment as a good treatment modality for patients with cherubism. Key Words: Cherubism, bone fibrous dysplasia, sinus floor augmentation, bone grafting, dental implants
C
herubism is an uncommon benign fibro-osseous jaw bone disease, which is usually inherited as an autosomal dominant trait.1Y7 This clinical condition is characterized by painless, firm, and symmetrical bilateral expansion of the mandible and maxilla that causes upward turning of the eyes and a full round lower face, which creates a cherub-like appearance.3,4,8Y10 Mandibular angle, ascending ramus, retromolar region, and posterior maxilla are the most often affected sites, and sometimes the coronoid process is involved.3,11 Dental manifestations of cherubism depend on the time of onset and the severity of the disease.12 Disturbance in arrangement of primary teeth can be seen,12Y14 whereas missing and impacted teeth (mostly molars), developmental problems in molars, abnormal tooth shape, partial root resorption, malalignment, and delayed and ectopic eruption are commonly observed in permanent dentition.3,12,13,15 In most cases, the lesions tend to show varying degrees of remission and involution after puberty. Surgical interventions including partial resection, contour resection, and curettage have been suggested for aesthetic or functional considerations.3,11,12,16 In many adult patients, replacing lost or missing teeth seems challenging, because treatment with conventional removable or fixed prosthesis requires the presence of appropriate abutment teeth.3,11 Implant treatment provides significant improvement over conventional treatment in general health-related quality of life17,18; however, considering the implant treatment as an option to support restorations arises concerns about complications such as lack of osseointegration following implant placement into the porous bone of a cherub patient.3,11 Nonetheless, implant treatment has provided satisfactory results in histologically comparable bone lesions including central giant cell granuloma and fibrous dysplasia.19Y22 To the extent of our knowledge, only a few studies have evaluated dental implants as a potential treatment for cherubism. These studies indicate that cherubic patients could be treated with dental implants if treatment is performed in the stable phase of the disease.3,23 The current study presents a 20-year old patient with cherubism in whom bilateral open sinus lifting in the maxilla and onlay augmentation
From the *Department of Oral and Maxillofacial Surgery, Dental School; †Department of Prosthodontics, Dental School; and ‡Gifted and Talented Dental Students Division, Dental School, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Received July 16, 2013. Accepted for publication August 27, 2013. Address correspondence and reprint requests to Arash Khojasteh, DMD, MS, Department of Oral and Maxillofacial Surgery, Dental School, Shahid Beheshti University of Medical Sciences, Daneshjou Blvd, Evin, Tehran, Iran PO 19839; 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.0000000000000433
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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& Volume 25, Number 2, March 2014
FIGURE 3. Computed tomography scan view. FIGURE 1. The patient’s frontal view, 3 years following facial contour shaving.
The maxillary implants could achieve a maximum 15 N/cm of primary
in the premolar regions of the mandible were performed to allow for full mouth rehabilitation with dental implants.
CLINICAL REPORT A 20-year-old woman, complaining aesthetic issues, was referred to the Department of Oral and Maxillofacial Surgery, Shahid Beheshti University of Medical Sciences, in December 2010 (Fig. 1). Grade IV cherubism was previously diagnosed in the patient based on clinical and radiographic findings such as lesions involving the mandible and maxilla and signs of root resorption.24,25 The preoperative diagnosis was made by carrying out panoramic radiography, combined with a computed tomography scan in whom bilateral sinus pneumatization in the maxilla and impacted teeth in a cyst and bilateral bone resorption with horizontal width reduction of the bone ridge in premolars region were seen, and the bone height to maxillary sinus was 5 to 6 mm (Figs. 2 and 3). Clinical examination revealed malposed and malformed incisors and premolars with short roots in the upper jaw, whereas only 1 premolar existed in the lower jaw (Fig. 4). Three years before referral, the inferior border and posterior body of the mandible on both sides were shaved surgically to correct the shape of the mandible and achieve acceptable aesthetic. After referral, the patient’s condition was evaluated, and the patient showed no recurrence of osseous lesions. The remaining teeth were assigned as hopeless prognosis by the prosthodontist because of the mobility and lack of bone support. The maxillomandibular implant recipient sites were developed under general anesthesia in a 1-stage procedure. Subperiosteal dissections in the anterior and posterior regions of the maxilla and mandible were performed. All remaining teeth were extracted. Additional care was given by the surgeon during the extractions to preserve facial and palatal bony walls. Sinus augmentation was achieved through lateral approaches. The lateral window need had spongious structure and was elevated with the attached Schneiderian membrane easily. Filling of the blood within the sinus cavity was more than a normal condition. The sinus cavity was then packed with natural bovine bone mineral (Cerabone; Botiss Dental GmbH, Berlin, Germany). Simultaneous to bone augmentation, 8 dental implants (Xive; DENTSPLY Friadent, Mannheim, Germany) were installed in the maxilla while drilling was accomplished at a drill speed of 400 revolutions/min.
FIGURE 4. Intraoral view showing abnormal tooth shape, spacing, and malalignment.
stability. In the lower jaw, after buccal and lingual subperiosteal dissection, the anterior bony lesion, which had a cystic lining, was enucleated, and a 10-hole miniplate was installed to decrease the risk of mandibular fracture. Seven dental implants (Xive; DENTSPLY Friadent) were installed in mandible. The posterior of the mandible in both sides had horizontal-type ridge deficiency (width G3 mm), and it resulted in dehiscence defect in the labial side of posterior implants. Thus, simultaneous onlay augmentation was planned for the patient. A 20 40-mm bone block was harvested from the lateral ramus of the right side. The bone density in this part was felt to be softer than usual (D2-D3 nature), and disengagement was easier. Harvested grafts were properly adapted to the recipient site and were fixed by fixation miniscrews (Jeil, Seoul, Republic of Korea, 10 mm in length) in the premolar region. After a 6-month healing period, all the implants had remained well integrated clinically and radiographically (Figs. 5 and 6). The patient was referred to the Division of Prosthodontics for prosthesis fabrication, and implant-supported prosthesis was delivered 6 months later (Fig. 7). Then, the patient was followed up for an additional 18 months. To date, there has been neither complication nor change in the occlusion due to further growth. The patient remained satisfied with the function of the prosthesis and aesthetic
FIGURE 5. Implants loaded in mandible (A) and maxilla (B).
FIGURE 2. Panoramic radiograph of the impacted teeth and a radiolucent lesion in the anterior part of the mandible.
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FIGURE 6. Panoramic view showing implant osseointegration.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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REFERENCES
FIGURE 7. Intraoral view of the implant-supported prosthesis.
outcome of treatment, and no sign of bone resorption or early implant failure was observed.
DISCUSSION A patient with cherubism is a challenge to clinicians because of the maxillomandibular deformities and other problems such as tooth malalignment, spacing, and missing.3,12,13,15 Moreover, there is a chance for bone resorption in the jaws due to the long-term edentulism. Treatment of cherubism has not been discussed fully in the current literature. Yilmaz et al11 described a prosthetic treatment plan for a cherubic patient by a removable partial denture in the maxilla and a mandibular overdenture using erupted teeth as abutments. The implant option was excluded from treatment because of the porous bone nature of cherubic patient, which could prevent implant osseointegration and lead to complication. In the current case, the whole remaining teeth of the patient were diagnosed as hopeless. Conventional removable denture was not considered in patient’s treatment plan because providing patient demand for aesthetic, fully functional, and comfortable artificial teeth was not feasible by removable denture. Patient’s clinical examinations and radiographs showed bilateral sinus pneumatization in posterior maxilla, cystic cavity in the anterior of the mandible, and horizontal bone deficiency in the posterior mandible. In addition, insufficient horizontal width of mandibular alveolar ridge in the premolar regions suggested the possible need for bone graft. The patient was likely to be in the stable phase of the disease, because there was no evidence of enlargement of facial bony contour in the shaved areas because of surgical shaving of the inferior border of mandible and zygomatic prominences 3 years before mouth rehabilitation. Therefore, placing dental implants was considered as a feasible option providing the optimum outcomes. Prescott et al26 reported 5 cases in which implant placement led to an implant survival rate of 79%. Despite the fact that some of the implants were placed in multilocular radiolucencies, the bone adjacent to the implants looked normal. Hachach-Haram et al23 documented a case under the title of cherubism that was complicated by neurofibromatosis type 1 in which mandibular reconstruction followed by placement of osseointegrated dental implants to restore function was performed. Dewan and Bishop3 suggested dental implant placement for cherubic patients in a stable phase. They used computed tomographyY guided, flapless surgery for the patient, because of its advantage of being minimally invasive. Considering the adequate bone level in their case, no bone graft was indicated before dental implant placement. However, because of several endo-osseous bone lesions and jaw bone loss in the patient of the current study, considering various bone augmentation techniques seemed to be indispensable. Bilateral opensinus lifting through a lateral approach was performed to achieve adequate bone in the posterior maxilla. To the extent of our knowledge, there has been no report regarding augmentation procedures in cherubic patients. Our patient showed stable results of implant-supported fixed restoration in the augmented jaw. All of the implants have survived following a 2-year follow-up.
1. Tiziani V, Reichenberger E, Buzzo CL, et al. The gene for cherubism maps to chromosome 4p16. Am J Hum Genet 1999;65:158Y166 2. Hyckel P, Berndt A, Schleier P, et al. CherubismYnew hypotheses on pathogenesis and therapeutic consequences. J Craniomaxillofac Surg 2005;33:61Y68 3. Dewan K, Bishop K. Management of a patient suffering with cherubism with dental implants. Eur J Prosthodont Restor Dent 2011;19:67 4. Tamgadge A, Modak NV, Bhalera S, et al. Cherubism: a rare case report and literature review. Int J Oral Maxillofac Pathol 2012;3:56Y60 5. Valiathan A, Prashanth V. Cherubism: presentation of a case. Angle Orthod 1997;67:237Y238 6. Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol 2008;2:177Y202 7. de Lange J, van Maarle MC, van den Akker HP, et al. A new mutation in the SH3BP2 gene showing reduced penetrance in a family affected with cherubism. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:378 8. Jones W, Gerrie J, Pritchard J. CherubismVfamilial fibrous dysplasia of the jaws. Surg Br volume 1950;32:334 9. Lima GdMG, Almeida JD, Cabral LAG. Cherubism: clinicoradiographic features and treatment. J Oral Maxillofac Res 2010;1:1Y8 10. Yamaguchi T, Dorfman HD, Eisig S. Cherubism: clinicopathologic features. Skeletal Radiol 1999;28:350Y353 11. Yilmaz B, Ozan O, Karaagaclioglu L, et al. A prosthetic treatment approach for a cherubism patient: a clinical report. J Prosthet Dent 2006;96:313Y316 12. Papadaki ME, Lietman SA, Levine MA, et al. Cherubism: best clinical practice. Orphanet J Rare Dis 2012;7:S6 13. Faircloth W, Edwards RC, Farhood VW. Cherubism involving a mother and daughter: case reports and review of the literature. J Oral Maxillofac Surg 1991;49:535Y542 14. Pontes FS, Ferreira AC, Kato AM, et al. Aggressive case of cherubism: 17-year follow-up. J Pediatr Otorhinolaryngol 2007;71:831Y835 15. Ireland A, Eveson J. Cherubism: a report of a case with an unusual post-extraction complication. Br Dent J 1988;164:116 16. Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions. Oral Maxillofac Surg Clin North Am 2005;17:415 17. Awad M, Locker D, Korner-Bitensky N, et al. Measuring the effect of intra-oral implant rehabilitation on health-related quality of life in a randomized controlled clinical trial. J Dent Res 2000;79:1659Y1663 18. Heydecke G, Locker D, Awad MA, et al. Oral and general health-related quality of life with conventional and implant dentures. Community Dent Oral Epidemiol 2003;31:161Y168 19. Bajwa MS, Ethunandan M, Flood TR. Oral rehabilitation with endosseous implants in a patient with fibrous dysplasia (McCune-Albright syndrome): a case report. J Oral Maxillofac Surg 2008;66:2605Y2608 20. Bahat O, Fontanessi R. Efficacy of implant placement after bone grafting for three-dimensional reconstruction of the posterior jaw. Int J Periodontics Restorative Dent 2001;21:220 21. Cheung L, Samman N, Pang M, et al. Titanium miniplate fixation for osteotomies in facial fibrous dysplasiaVa histologic study of the screw/bone interface. Int J Oral Maxillofac Surg 1995;24:401Y405 22. Lee H, Ercoli C, Fantuzzo JJ, et al. Oral rehabilitation of a 12-year-old patient diagnosed with a central giant cell granuloma using a fibula graft and an implant-supported prosthesis: a clinical report. J Prosthet Dent 2008;99:257Y262 23. Hachach-Haram N, Gerarchi P, Benyon SL, et al. Multidisciplinary surgical management of cherubism complicated by neurofibromatosis type 1. J Craniofac Surg 2011;22:2318Y2322 24. Motamedi MHK. Treatment of cherubism with locally aggressive behavior presenting in adulthood: report of four cases and a proposed new grading system. J Oral Maxillofac Surg 1998;56:1336Y1342
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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25. Raposo-Amaral CE, Guidi MdC, Warren SM. Two-stage surgical treatment of severe cherubism. Ann Plast Surg 2007;58:645Y651 26. Prescott T, Redfors M, Rustad CF, et al. Characterization of a Norwegian cherubism cohort; molecular genetic findings, oral manifestations and quality of life. Eur J Med Genet 2013;56:131Y137
From ‘‘Head to Heart:’’ Reconstruction of a Sternal Cleft With Split Calvarial Bone Graft in a 15-Month-Old Child Ramesh K. Sharma, MCh(Plast Surg), Diplomate Nat. Board(Plast Surg),* Sandeep S. Rana, MCh(Cardiothoracic Surg)Þ Abstract: Sternal cleft is a rare anomaly and has been variously managed by using either autogenous tissue or synthetic materials for bony reconstruction. We report the reconstruction of sternal cleft in a 15-month-old child with a single-piece outer-table cranial bone graft that was harvested by splitting in situ. The reconstructed sternum was covered with bilateral pectoralis major advancement muscle flaps. Key Words: Sternal cleft, outer-table cranial bone graft, single piece, split in situ, pectoralis major muscle flap
S
ternal cleft is a rare developmental anomaly that can vary in size and shape from a narrow linear fissure to a large defect with complete or near-complete separation of the 2 halves of the sternum.1,2 The bony defect can be isolated, associated with herniation of internal organs through the abnormal opening, or can occur as part of complex clinical syndromes consisting of abnormalities of the heart, vessels, and other organs (Cantrell pentalogy, PHACE [posterior fossa malformationsYhemangiomasYarterial anomaliesY cardiac defectsYeye abnormalitiesYsternal cleft and supraumbilical raphe] syndrome).3,4 The heart in these patients lies just under the skin and is vulnerable to even trivial trauma. It is therefore mandatory that the sternum be reconstructed at the earliest to obviate this risk. Many methods have been described for reconstruction of the sterna cleft, and these include use of autogenous tissue or synthetic materials for the closure of this defect. In children, it is desirable that autogenous tissue be used so that a proportionate growth of the reconstructed sternum is possible. Whereas in adults iliac crest and other sources such as rib have been used, these donor sites may not provide enough bone in small children. From the Departments of *Plastic Surgery and †Cardiothoracic and Vascular Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Received May 13, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Ramesh K. Sharma, MCh(Plast Surg), Diplomate Nat. Board(Plast Surg), Department of Plastic Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India; 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.0000000000000399
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We report the use of split cranial bone graft for reconstruction of congenitally missing upper half of the sternum in a 15-monthold child.
CLINICAL REPORT Simran, a 9-month-old female infant, was brought to the plastic surgery services with a pulsating mass in the chest. The sternum was missing in the upper half with the heart lying under the precordium. The mediastinal contents could be seen bulging whenever the child cried. The child also had hemangioma in the lower lip and forehead. She also had linear supraumbilical abdominal wall scarring in the midline that continued upward into the thin precordial skin (Fig. 1, left) (Parental consent was obtained to take photographs of the child). There was no divarication of recti. The child had no respiratory or cardiac distress, and it was decided in consultation with the cardiothoracic surgery colleagues that the reconstruction be undertaken at the earliest. However, the parents were not willing for surgery and were counseled for the same. The parents finally agreed for the operation, and the child was admitted for reconstruction of sternum at about age of 15 months. The computed tomography scan of the chest confirmed the clinical findings of absence of sternum (Fig. 1, right). It was decided to reconstruct the missing sternum with autogenous bone so that it could grow proportionately with the child. Because the requirement of bone would be substantial, the possibility of using cranial bone graft was kept in mind as this can provide large amount of bone even in a small child. The area of the defect was approached through the scarred skin. The pericardium was adherent to the scar; this was freed, and the pericardial sac could be closed primarily with mobilization of the available tissue. The bony defect was defined, and a template of the defect was made (Fig. 2, upper row right). The parieto-occipital area of the skull was chosen to be the site for harvesting the graft as it offered the best contour for the neosternum. The bone graft was harvested as a single piece by splitting the outer table in situ (Fig. 2, middle row). The neosternum was fixed into the defect using titanium plates and screws. The reconstructed sternum was covered with bilaterally mobilized pectoralis major muscle flaps based on the pectoral vessels. The scarred precordial skin was excised, and healthy skin was closed over the defect (Fig. 2, lower row). Postoperative healing was uneventful. Figure 3 shows appearance at the end of 6 weeks. Sternal clefts are classified as being complete or partial.5,6 The involvement of the upper sternum is commonest. Generally, the cleft extends until the fourth costal cartilage.7 There are only a few reports on repair of sternal defects. Hebra et al8 collected 72 cases worldwide up to 1995. Most authors
FIGURE 1. Left column upper row: Sternal defect showing bulge in the precordial area. The skin is thin and discolored. Left column middle row: Hemangioma lower lip and forehead. Left column lower row: Appearance at the age of 15 months. Right column: Computed tomography scan showing extent of sternal cleft.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
