- Email: [email protected]
Giant-Cell Granuloma in the Temporal Bone: A Case Report and Review of the Literature
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DIMITAKOPOULOS ET AL
J Oral Maxillofac Surg 64:531-536, 2006
Giant-Cell Granuloma in the Temporal Bone: A Case Report and Review of the Literature Ioannis Dimitakopoulos, MD, DDS,* Nikolaos Lazaridis, MD, DDS,† Pavlos Sakellariou, MD,‡ and Anthoula Asimaki, MD§ Giant-cell granuloma (GCG) is an uncommon benign lesion that occurs almost exclusively within the jawbones and only sporadic cases occurring in the extragnathic skeleton have been reported.1,2 Although the etiology and pathogenesis of GCG remains unclear, its histologic and clinical features as well as its clinical behavior have been studied in detail and are now well established, differentiating GCG from other giant cell lesions.3-6 Initially, after Jaffe’s7 first description of the lesion, GCG was thought to be limited to the jaws, However, isolated cases of GCG occurring in other cranial and facial bones, although rare, have been documented since then, expanding the distribution of this lesion to the rest of the skull. GCG in the temporal bone was first described by Hirsch and Katz3 in 1974. Since then, 20 additional cases have been reported in the international medical literature.1,8,9 In this article we report a new case of a large temporal bone GCG that had extended to the middle cranial and infratemporal fossa and presented as a preauricular suprazygomatic facial swelling. We also review the cases of the temporal GCG that have been reported thus far, and discuss its more interesting manifestations when this lesion arises in the temporal bone.
*Associate Professor, University Clinic of Oral and Maxillofacial Surgery, G. Papanikoulaou General Hospital, Thessaloniki, Greece. †Professor, University Clinic of Oral and Maxillofacial Surgery, General Hospital, Thessaloniki, Greece. ‡Neurosurgeon, Department of Neurosurgery, G. Papanikoulaou General Hospital, Thessaloniki, Greece. §Pathologist, Department of Histopathology, G. Papanikoulaou General Hospital, Thessaloniki, Greece. Address correspondence and reprint requests to Dr Dimitakopoulos: Pavlou Mela 28, 54622 Thessaloniki, Greece; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6403-0028$32.00/0 doi:10.1016/j.joms.2005.11.006
Report of a Case A 43-year-old man was referred to our department in April 2001 for evaluation of a slowly expanding swelling on the left side of his face that he had noticed for the past 11 months, associated with intermittent headaches. The patient’s history showed that 5 years prior to admission he had suffered a left head impact while he worked on his farm, for which he had been admitted for 2 days to a regional hospital. On examination, a painless nonfluctuant left suprazygomatic preauricular mass measuring approximately 2 ⫻ 3 cm was revealed. The lesion was firmly attached on the ipsilateral zygomatic arch without any interference with the opening of the patient’s mouth. The external auditory canal was normal. Facial nerve weakness was not detected. Palpation of the patient’s neck was negative for lymphadenopathy. Computed tomography scan as well as magnetic resonance imaging (MRI) examination revealed a 4 ⫻ 5 cm osteolytic lesion of the left temporal bone and infratemporal fossa with intracranial extension pushing upwards the dura and temporal lobe of the brain (Figs 1, 2). The mass had also eroded the root of the left zygomatic arch, leaving the structures of the left TMJ and the left external auditory canal intact (Fig l). Routine angiography revealed that the mass was only mildly vascular and that it was mainly supplied by the temporal branches of the internal maxillary artery (Fig 3). The rest of the clinical examination was normal. An incisional biopsy via a preauricular approach was performed and the microscopic examination of the specimen revealed that the lesion was a giant cell granuloma. Further hormonal and biochemical examinations revealed that serum levels, calcium, phosphorus, alkaline phosphatase, and parathyroid hormone were within normal limits, excluding brown tumor of hyperparathyroidism. A combined craniofacial surgical intervention via a left hemicoronal incision in continuity with a preauricular approach was used to access the infratemporal fossa and the lateral skull base. A red-brownish, non-encapsulated soft mass destroying the squamous temporal bone was adhered to the dura extending in the zygomatic process of the temporal bone over the left temporomandibular joint. The mass was completely excised with great care to avoid damage of the overlying dura mater. The postoperative course of the patient was uneventful. Postoperative histologic examination of the excised lesion was reported again as GCG. Microscopically, the specimen showed a delicate reticular
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FIGURE 1. Axial computed tomography scan reveals an osteolytic lesion in the left temporal bone and infratemporal fossa causing marked bone destruction and eroding the root of the left zygomatic arch in front of the left TMJ. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
and fibrillar connective tissue stroma containing large numbers of ovoid or spindle-shaped young giant cells. The giant cells resembled osteoclasts. Capillaries were numerous. Foci of hemorrhage with liberation of hemosiderin pigment and its subsequent ingestion by mononuclear phagocytes, as well as inflammatory cell infiltration, were also presented (Fig 4). There has been no evidence of recurrence 3 years after surgery.
GIANT-CELL GRANULOMA IN THE TEMPORAL BONE
FIGURE 3. Angiography of the lesion showing a mildly vascular mass supplied by branches of the internal maxillary artery. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
Discussion Giant-cell granuloma is an uncommon non-neoplastic bone lesion that consists of cellular fibrous tissue containing multiple foci of hemorrhage, aggregations of multinucleated giant cells, and occasionally trabeculae of woven bone.10 Although GCG was initially considered to be peculiar to the maxillary and man-
FIGURE 2. A, Coronal T1-weighted MRI showing a hypotense destructive mass eroding the left temporal bone, compressing the temporal lobe of the brain, and presenting in the temporal fossa. B, Axial T1-weighted MRI of the hypotense mass in the temporal lobe. C, Sagittal T1-weighted MRI with gadolinium contrast demonstrating the enhancement of the lesion that is intracranially extended pushing the temporal lobe upwards. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
DIMITAKOPOULOS ET AL
FIGURE 4. A, Osteoclast type giant cells are irregularly distributed in a fibroblast-rich stroma. Scattered hemosiderin deposits are also present. (Hematoxylin-eosin stain; magnification ⫻ 200.) B, Highpower-view of A. (Hematoxylin-eosin stain; magnification ⫻ 400.) Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
dibular bones, well documented cases of the lesion involving the cranial11-14 and the rest of facial bones15 as well as the axial skeleton and long bones,16 the hands and feet,17 have also been reported. In particular, GCG of the skull base is considered an extremely rare distinct clinicopathologic entity,2 and less than 40 cases have been reported in the medical literature since its first description by Jaffe7 in 1953. Thus, in 1969 Friedberd et al18 were the first to report 2 cases of GCG involving the ethmoid and sphenoid bones, respectively. Five years later, in 1974, Hirsch and Katz3 described the first case of a GCG originating in the temporal bone and presented a thorough review of the literature with special reference to giant cell lesions arising in this region. In the same article, the authors also discussed the diagnostic criteria and the main points of GCG differentiation, both clinically and histologically, from the giant cell tumor and other similar lesions invading the temporal region. Since then, Boedeker et al,1 reviewing the international medical literature up to 2003, found 17 other cases of GCG involving the temporal bone, including their
533 own patient. In the same year (2003) 2 new cases were published in the medical literature.8,9 Whereas, including our patient, only 21 cases of the temporal bone have been reported up to now (Table 1). From the study of the reported cases, clinically, results indicate that hearing loss, local swelling, tinnitus, pain, vertigo, and facial weakness are the most common symptoms when GCG arises in the temporal bone. A tendency of more frequent expression of the lesion in men is possible because the ratio of male to female patients is 15:6 and, despite the small number of the published cases, does not appear the female predilection that has been reported for GCG of the jaws.2,4 The age range of the reported temporal bone GCG (including the case presented by us) is 4 months to 72 years, with mean age of patients being 30.4 years, whereas adolescents and young adults (ages 10 to 25 years) have shown to be more prone in the case of jaw GCG.2,4 The etiopathogenesis of the GCG of the skull bones is unclear but it has been suggested, as in the case of jawbones, to be the result of an exacerbated reparative process related to previous trauma and intraosseous hemorrhage that triggers the reactive granulomatous process.2,3,7,13 From the 21 reported cases of the present review, including our patient, only in 5 cases was there a clear history of previous head trauma.2,3,19,20 Other causes regarding the etiopathogenesis of the lesion have also been proposed, but not one single theory has gained wide acceptance.1,3 GCG of the craniofacial region usually has a benign course. Its clinical behavior, as it results from the study of jaws, and GCG’s location in the majority of reported cases ranges from a slowly growing asymptomatic swelling to a more aggressive form which, although rarely, can cause pain, paresthesia, local destruction of bone, cortical bone perforation, and root resorption.4-6 On occasion, in the rest of the facial skeleton, GCG may show wide local slowly growing spread, as the thin maxillofacial bone form less effective barriers to lesion growth.6 In the skull base and the temporal bone, in particular, the wide local spreading of the lesion (because of the initial asymptomatic course and its proximity to vital structures) usually results in extension of the lesion to the infratemporal fossa, the structures of the ear, and up to the floor of the middle cranial fossa, behaving like an intracranial tumor.19 Histologically, GCG of the craniofacial bones should be differentiated from a number of lesions, in which giant cells are found, including benign and malignant neoplasms as well as non-neoplastic and metabolic conditions such as fibrous dysplasia, chondroblastoma, aneurysmal bone cyst, cherubism, and particularly hyperparathyroidism and giant-cell tumor.1-6,14 Differential diagnosis is usually achieved with the recognition of the specific histologic characteristics
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Table 1. SUMMARY OF AVAILABLE CLINICAL DATA OF ALL REPORTED CASES OF PATIENTS WITH TEMPORAL BONE GCG
Study
Age/Gender
Hirschl and Katz, 19743 Colclasure et al, 198122
34 yr/F 10 yr/M 22 yr/M
Pain, tinnitus, hearing loss Hearing loss Mass, hearing loss
Tesluk et al, 198927 Ciappetta et al, 199019 Cohen and GrandaRicart, 199328 Lewis et al, 199420
56 yr/M 25 yr/M 4 mo/F
Nemoto et al, 199529
Maruno et al, 199730
Initial Symptoms
Treatment
Follow-Up Well at 2 yr Well at 6 yr Well at 7 yr
Pain, mass Pain, dysphagia Mass
Biopsy and radiation Total resection Total resection and radiation Total resection Total resection Total resection
32 yr/F
Hearing loss
Total resection
36 yr/M
Hearing loss, tinnitus
Total resection
28 yr/M
Hearing loss, tinnitus
Total resection
Hearing loss, facial weakness
Total resection
Well after indefinable time Well after indefinable time Well after indefinable time Recurrence at 1 yr Recurrence at 1 yr Well at 3 yr
3 yr/F
Ung et al, 200121
NR
Hearing loss
Total resection
Lin et al, 200132
44 yr/M
Hearing loss, tinnitus
Khoduei et al, 200131 Sharma et al, 20022
72 yr/M 36 yr/M 12 yr/M
Matsui et al, 200225 Yoshimura et al, 200226 Boedeker et al, 20031
41 yr/M 38 yr/M 17 yr/F
Hearing loss Pain, tinnitus, vertigo Mass, hearing loss, facial weakness, tinnitus, vertigo Mass NR Hearing loss
Total resection and radiation Total resection Total resection Total resection
Tian et al, 20038 Montero et al, 20039 Dimitakopoulos et al*
32 yr/M 60 yr/M 43 yr/M
Mass Hearing loss Mass
Total resection Total resection Biopsy initially, total resection 3 yr after Curettage Total resection Total resection
Well at 1 yr Well at 15 yr NR
Well at 21 mo Well at 3 yr NR NR NR Well at 2 yr Well at 26 mo Well at 2 yr Well at 3 yr
Abbreviation: NR, not reported. *Present case. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
of each condition,1,5,14 whereas brown tumor of hyperparathyroidism, which is indistinguishable microscopically from GCG of bone, can be easily ruled out with biochemical tests showing normal serum calcium, phosphorous, alkaline phosphatase as well as parathyroid hormone levels. From a clinicopathologic point of view, it is more important to distinguish GCG from a giant cell tumor of the temporal bone, where both are rarely encountered, because of their different biological behavior and outcome of treatment. Giant-cell tumor is a true neoplasm with higher incidence of recurrence and potential for malignant transformation and metastatic spread.3,8,21 On the contrary, malignancy and metastasis have never been reported in GCG.1,2,21 Although several histologic features have been recommended distinguishing GCG from giant-cell tumor in borderline cases, considerable overlap between the 2 lesions may exist.1,8,22
That is why Stolovitzky et al23 introduced the term giant-cell lesion to describe these borderline cases of the craniofacial area that can be further classified as aggressive or non-aggressive on the basis of symptoms. However, it has been emphatically recommended that it is important to make every effort to separate giant-cell tumor from the skull GCG because of the much worse prognosis of the giant-cell tumor.1,3,14,22 In our case, the patient had suffered a left side head trauma during his work 5 years prior to his referral, for which he had been treated for 2 days at a regional hospital. His principal presenting symptom was a painful, slowly growing suprazygomatic preauricular swelling associated with intermittent headaches. Computed tomography scan as well as TI and T2 weighted MRI showed a large soft tissue mass arising in the left temporal bone and extending in the infra-
DIMITAKOPOULOS ET AL
temporal fossa. The lesion had also eroded the left zygomatic and the temporal squama invading in the middle cranial fossa pushing upwards the dura and temporal lobe of the brain mimicking, as the previously reported cases, an intracranial tumor.19 External carotid angiography showed a mildly vascular mass supplied by the temporal branches of the internal maxillary artery. Microscopically, the lesion, which was consistent with a GCG of the temporal bone, was composed of a delicate fibrillar connective tissue stroma containing a large number of ovoid or spindleshaped young fibroblast cells and multinucleated giant cells resembling osteoclasts. Foci of hemorrhage and deposits of hemosiderin ingested by mononuclear phagocytes as well as numerous capillaries and inflammatory cell infiltration were also present, Surgical excision is the treatment of choice for GCG of the skull base.1,21,22 The extent of tissue removal, depending on the extent, site, and progression of the lesion, has been described from aggressive curettage to en block resection.4,5,21 Although complete excision provides a unique challenge to a surgeon, because of the complexity and number of the vital structures of the skull base, it is associated with a recurrence rate of only 10% as compared with the higher recurrence rate of up to 50% when simple curettage is used.19,21,24 Where angiography has shown that GCG is highly vascular, the preoperative obliteration of the feeding arteries with super-selective intravascular embolization is helpful for total removal of the lesion.25,26 Radiotherapy has been recommended only in cases where complete surgical excision is not possible, in patients who refuse surgery, or those who cannot be subjected to surgery because of the extent and number of the involved structures of the skull base bones.3,12,14 Results from the present review of the 21 patients with GCG of the temporal bone1-3,8,9,19-22,25-32 indicate that 3 underwent radiation therapy3,22,32 which, although very effective when used in cases not amenable to surgical approach or in recurrent GCG of the temporal bone, does carry a risk for development of osteogenic sarcoma.8,13,21 Apart from radiotherapy, GCG of the temporal bone has not been reported to be treated by nonsurgical methods used in the case of the involved jaws, such as systemic administration of calcitonin,33,34 intralesional injection with corticosteroids,35 and interferon alpha-2a administration.36
References 1. Boedeker CC, Kayser G, Ridder GJ, et al: Giant-cell reparative granuloma of the temporal bone: A case report and review of the literature. Ear Nose Throat J 82:926, 2003 2. Sharma RR, Verma A, Pawar SJ, et al: Pediatric giant cell granuloma of the temporal bone: A case report and brief review of the literature. J Clin Neurosci 9:459, 2002
535 3. Hirsch S, Katz A: Giant cell reparative granuloma outside the jaw bone. Diagnostic criteria and review of the literature with the first case described in the temporal bone. Hum Pathol 5:171, 1974 4. Bataineh AB, Al-Khateed T, Rawashdeh MA: The surgical treatment of central giant cell granuloma of the mandible. J Oral Maxillofac Surg 60:756, 2002 5. Causon RA, Binnie WH, Barrett AW, et al: Oral disease, in: Clinical and Pathological Correlations. Ed 3. Edinburgh, London, New York, Mosby, 2001, pp 711-712 6. Greer RO, Rohrer MD, Young SK: Non-odontogenic tumors. Clinical evaluation and pathology, in Thawley SE, Panje WP, eds: Comprehensive Management of Head and Neck Tumors. Ed 2. Philadelphia, PA, Saunders, 1999, pp 1637-1691 7. Jaffe HL: Giant cell reparative granuloma, traumatic bone cyst and fibrous (fibroosseus) dysplasia of the jawbones. Oral Surg 6:159, 1953 8. Tian HF, Li TZ, Yu SF: Giant cell granuloma of the temporal bone: A case report with immunohistochemical, enzyme histochemical, and in vitro studies. Arch Pathol Lab Med 127: 1217, 2003 9. Montero EH, Navarro JS, Pueyo JL, et al: Giant cell reparative granuloma in the temporal bone. Am J Otolaryngol 24:191, 2003 10. Kramer RH, Pindborg J, Shear M: Histological Typing of Odontogenic Tumors. Ed 2. Berlin, Germany, Springer-Verlag, 1991, p 31 11. Rogers LF, Mickael M, Christ M, et al: A case report of 276 giant cell (reparative) granuloma of the sphenoid bone. Skeletal Radiol 12:48, 1984 12. Alappat JP, Pillai AM, Prasanna D, et al: Giant cell reparative granuloma of the craniofacial complex. Case report and review of the literature. Br J Neurosurg 6:71, 1992 13. Garza-Mercado R, Cavazos E, Hernandez-Batres F: Giant cell reparative granuloma of the cranial vault. Exceptional bone lesion. Neurosurgery 15:228, 1984 14. Santos-Briz A, Lobato RD, Ramos A, et al: Giant cell reparative granuloma of the occipital bone. Skeletal Radiol 32:151, 2002 15. Som PW, Lawson W, Cohen BA: Giant-cell lesions of the facial bones. Radiology 147:129, 1983 16. Oda Y, Tsuneyoshi M, Shinohara N: Solid variant of aneurysmal bone cyst (extragnathic giant cell reparative granuloma) in the axial skeleton and long bones. A study of its morphologic spectrum and distinction from allied giant cell lesions. Cancer 70:2642, 1992 17. Glass TA, Mills SE, Fechner RE, et al: Giant-cell reparative granuloma of the hands and feet. Radiology 149:65, 1983 18. Friedberg SA, Eisenstein R, Wallner LJ: Giant cell lesions involving the nasal accessory sinuses. Laryngoscope 79:763, 1969 19. Ciappetta P, Salvati M, Bernardi C, et al: Giant cell reparative granuloma of the skull base mimicking an intracranial tumor. Case report and review of the literature. Surg Neurol 33:52, 1990 20. Lewis ML, Weber AL, McKenna MJ: Reparative cell granuloma of the temporal bone. Ann Otol Rhinol Laryngol 103:826, 1994 21. Ung F, Li KK, Keith DA: Giant cell reparative granuloma of the temporal bone. Acta Otolaryngol 121:523, 2001 22. Colclasure JB, Shea MC, Graham SS: Giant cell lesions of the temporal bone. Am J Otol 2:188, 2001 23. Stolovitzky JP, Waldrom CA, McConnel FM: Giant cell lesions of the maxilla and paranasal sinuses. Head Neck 16:143, 1994 24. Auclair PL, Cuenin P, Kratochvil FJ, et al: A clinical and histomorphologic comparison of central giant cell granuloma and the giant cell tumor. Oral Surg Oral Med Oral Pathol 66:197, 1988 25. Matsui T, Iwamuro K, Ishikawa T, et al: Large giant cell reparative granuloma of the petrous bone-case report. Neurol Med Chir (Tokyo) 42:232, 2002 26. Yoshimura J, Onda K, Tanaka R, et al: Giant cell reparative granuloma of the temporal bone. Neuroradiological and immunohistochemical findings. Neurol Med Chir (Tokyo) 42:510, 2002 27. Tesluk H, Senders CW, Dublin AB: Case report 562: Giant cell reparative granuloma of temporal bone. Skeletal Radial 18:599, 1989
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28. Cohen D, Granda-Ricart MC: Giant cell reparative granuloma of the base of the skull in a 4-month-old infant-CT findings. Pediatr Radiol 23:319, 1993 29. Nemoto Y, Inoue Y, Tashiro T, et al: Central giant cell granuloma of the temporal bone. Am J Neuroradiol 16:982, 1995 30. Maruno M, Yoshimine T, Kubo T, et al: A case giant cell reparative granuloma of the petrous bone. Demonstration of the proliferative component. Surg Neurol 48:64, 1997 31. Knoduei I, Rowley H, Farrel M, et al: An unusual cause for tinnitus. Ir Med J 94:312, 2001 32. Lin J, Zhong DR, Liu LF, et al: Giant cell reparative granuloma of the temporal bone. Acta Otolaryngol 121:523, 2001
33. De Lange J, Rosenberg AJ, Van den Akker HP, et al: Treatment of central giant cell of the jaw with calcitonin. Int J Oral Maxillofac Surg 28:372, 1999 34. Pogrel MA, Regezi JA, Harris ST, et al: Calcitonin treatment for central giant cell granulomas of the mandible. Report of two cases. J Oral Maxillofac Surg 57:848, 1999 35. Rajeevan NS, Soumithran CS: Intralesional corticosteroid injection for central giant cell granuloma: A case report. Int J Oral Maxillofac Surg 27:1145, 1999 36. Kaban LB, Mulliken JB, Ezekowitz A: Antiangiogenic therapy of recurrent giant cell tumor of the mandible with interferon alpha-2a. Pediatrics 103:1145, 1999
J Oral Maxillofac Surg 64:536-542, 2006
A New Technique for Intraoral Maxillary Distraction: A Case Report Stuart Super, DMD,* Jonathan E. Schecter, DDS,† and Richard D. Bae, DDS‡ Distraction osteogenesis has become a major surgical technique aiding in the promotion of bone formation. The driving force behind distraction osteogenesis lies in its ability to stimulate new bone formation between 2 osteotomized segments of bone gradually separating under tension.1-3 It was not until 1992, however, that distraction osteogenesis for the craniofacial complex came into use.4 In its earliest craniofacial applications, distraction osteogenesis was limited to treating deficient mandibles in children with hemifacial microsomia. Successful advancements have been made that allowed for the treatment of the maxilla with both predictability and stability.5-7 As with the original mandibular distraction appliances, the maxillary extraoral appliances used were esthetically unappealing as well as difficult for the patient to manage.8 Recently, more inconspicuous intraoral distraction appliances have been developed and used with varying
*Chief, Department of Oral and Maxillofacial Surgery, Lenox Hill Hospital, New York, NY; Associate Professor, Department of Oral and Maxillofacial Surgery, New York University College of Dentistry, New York, NY. †Orthognathic Fellow, Department of Oral and Maxillofacial Surgery, Lenox Hill Hospital, New York, NY. ‡Chief Resident, Department of Oral and Maxillofacial Surgery, NYU/Bellevue Hospital Center, New York, NY. Address correspondence and reprint requests to Dr Super: 6 East 78th St, New York, NY 10021; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6403-0029$32.00/0 doi:10.1016/j.joms.2005.11.021
success. We present a case report using a variation of an intraoral maxillary distraction appliance.
Materials and Methods PRESURGICAL TECHNIQUE
The appliance designed was used for a 21-year-old man who presented with a surgically repaired unilateral cleft lip and palate, as well as a moderately hypoplastic maxilla. The patient had been previously treated using conventional orthognathic surgery in which a maxillary advancement was performed. In addition, the patient underwent the repair of a chronic oronasal fistula. Because of the vast amount of fibrous scar tissue created from the cleft lip and palate repair, the patient exhibited a complete relapse of the maxillary advancement within 12 months postoperatively. Therefore, it was decided that distraction osteogenesis would be used to treat the hypoplastic maxilla. At first we proposed to use the rigid external distractor system (KLS-Martin LP, Tuttlingen, Germany).9,10 However, because of the patient’s reluctance to wear the device while going to college, we instead decided to use the intraoral Antwerp transsinusoidal maxillary distractor (KLS-Martin LP)11 for treatment. Unfortunately, because of insufficient thickness and strength of the patient’s maxillary sinus wall, placement of these distractors could not be facilitated. These events propagated the development of an intraoral distraction appliance that would exhibit stability, predictability, vector control, and patient tolerance.
DIMITAKOPOULOS ET AL
J Oral Maxillofac Surg 64:531-536, 2006
Giant-Cell Granuloma in the Temporal Bone: A Case Report and Review of the Literature Ioannis Dimitakopoulos, MD, DDS,* Nikolaos Lazaridis, MD, DDS,† Pavlos Sakellariou, MD,‡ and Anthoula Asimaki, MD§ Giant-cell granuloma (GCG) is an uncommon benign lesion that occurs almost exclusively within the jawbones and only sporadic cases occurring in the extragnathic skeleton have been reported.1,2 Although the etiology and pathogenesis of GCG remains unclear, its histologic and clinical features as well as its clinical behavior have been studied in detail and are now well established, differentiating GCG from other giant cell lesions.3-6 Initially, after Jaffe’s7 first description of the lesion, GCG was thought to be limited to the jaws, However, isolated cases of GCG occurring in other cranial and facial bones, although rare, have been documented since then, expanding the distribution of this lesion to the rest of the skull. GCG in the temporal bone was first described by Hirsch and Katz3 in 1974. Since then, 20 additional cases have been reported in the international medical literature.1,8,9 In this article we report a new case of a large temporal bone GCG that had extended to the middle cranial and infratemporal fossa and presented as a preauricular suprazygomatic facial swelling. We also review the cases of the temporal GCG that have been reported thus far, and discuss its more interesting manifestations when this lesion arises in the temporal bone.
*Associate Professor, University Clinic of Oral and Maxillofacial Surgery, G. Papanikoulaou General Hospital, Thessaloniki, Greece. †Professor, University Clinic of Oral and Maxillofacial Surgery, General Hospital, Thessaloniki, Greece. ‡Neurosurgeon, Department of Neurosurgery, G. Papanikoulaou General Hospital, Thessaloniki, Greece. §Pathologist, Department of Histopathology, G. Papanikoulaou General Hospital, Thessaloniki, Greece. Address correspondence and reprint requests to Dr Dimitakopoulos: Pavlou Mela 28, 54622 Thessaloniki, Greece; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6403-0028$32.00/0 doi:10.1016/j.joms.2005.11.006
Report of a Case A 43-year-old man was referred to our department in April 2001 for evaluation of a slowly expanding swelling on the left side of his face that he had noticed for the past 11 months, associated with intermittent headaches. The patient’s history showed that 5 years prior to admission he had suffered a left head impact while he worked on his farm, for which he had been admitted for 2 days to a regional hospital. On examination, a painless nonfluctuant left suprazygomatic preauricular mass measuring approximately 2 ⫻ 3 cm was revealed. The lesion was firmly attached on the ipsilateral zygomatic arch without any interference with the opening of the patient’s mouth. The external auditory canal was normal. Facial nerve weakness was not detected. Palpation of the patient’s neck was negative for lymphadenopathy. Computed tomography scan as well as magnetic resonance imaging (MRI) examination revealed a 4 ⫻ 5 cm osteolytic lesion of the left temporal bone and infratemporal fossa with intracranial extension pushing upwards the dura and temporal lobe of the brain (Figs 1, 2). The mass had also eroded the root of the left zygomatic arch, leaving the structures of the left TMJ and the left external auditory canal intact (Fig l). Routine angiography revealed that the mass was only mildly vascular and that it was mainly supplied by the temporal branches of the internal maxillary artery (Fig 3). The rest of the clinical examination was normal. An incisional biopsy via a preauricular approach was performed and the microscopic examination of the specimen revealed that the lesion was a giant cell granuloma. Further hormonal and biochemical examinations revealed that serum levels, calcium, phosphorus, alkaline phosphatase, and parathyroid hormone were within normal limits, excluding brown tumor of hyperparathyroidism. A combined craniofacial surgical intervention via a left hemicoronal incision in continuity with a preauricular approach was used to access the infratemporal fossa and the lateral skull base. A red-brownish, non-encapsulated soft mass destroying the squamous temporal bone was adhered to the dura extending in the zygomatic process of the temporal bone over the left temporomandibular joint. The mass was completely excised with great care to avoid damage of the overlying dura mater. The postoperative course of the patient was uneventful. Postoperative histologic examination of the excised lesion was reported again as GCG. Microscopically, the specimen showed a delicate reticular
532
FIGURE 1. Axial computed tomography scan reveals an osteolytic lesion in the left temporal bone and infratemporal fossa causing marked bone destruction and eroding the root of the left zygomatic arch in front of the left TMJ. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
and fibrillar connective tissue stroma containing large numbers of ovoid or spindle-shaped young giant cells. The giant cells resembled osteoclasts. Capillaries were numerous. Foci of hemorrhage with liberation of hemosiderin pigment and its subsequent ingestion by mononuclear phagocytes, as well as inflammatory cell infiltration, were also presented (Fig 4). There has been no evidence of recurrence 3 years after surgery.
GIANT-CELL GRANULOMA IN THE TEMPORAL BONE
FIGURE 3. Angiography of the lesion showing a mildly vascular mass supplied by branches of the internal maxillary artery. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
Discussion Giant-cell granuloma is an uncommon non-neoplastic bone lesion that consists of cellular fibrous tissue containing multiple foci of hemorrhage, aggregations of multinucleated giant cells, and occasionally trabeculae of woven bone.10 Although GCG was initially considered to be peculiar to the maxillary and man-
FIGURE 2. A, Coronal T1-weighted MRI showing a hypotense destructive mass eroding the left temporal bone, compressing the temporal lobe of the brain, and presenting in the temporal fossa. B, Axial T1-weighted MRI of the hypotense mass in the temporal lobe. C, Sagittal T1-weighted MRI with gadolinium contrast demonstrating the enhancement of the lesion that is intracranially extended pushing the temporal lobe upwards. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
DIMITAKOPOULOS ET AL
FIGURE 4. A, Osteoclast type giant cells are irregularly distributed in a fibroblast-rich stroma. Scattered hemosiderin deposits are also present. (Hematoxylin-eosin stain; magnification ⫻ 200.) B, Highpower-view of A. (Hematoxylin-eosin stain; magnification ⫻ 400.) Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
dibular bones, well documented cases of the lesion involving the cranial11-14 and the rest of facial bones15 as well as the axial skeleton and long bones,16 the hands and feet,17 have also been reported. In particular, GCG of the skull base is considered an extremely rare distinct clinicopathologic entity,2 and less than 40 cases have been reported in the medical literature since its first description by Jaffe7 in 1953. Thus, in 1969 Friedberd et al18 were the first to report 2 cases of GCG involving the ethmoid and sphenoid bones, respectively. Five years later, in 1974, Hirsch and Katz3 described the first case of a GCG originating in the temporal bone and presented a thorough review of the literature with special reference to giant cell lesions arising in this region. In the same article, the authors also discussed the diagnostic criteria and the main points of GCG differentiation, both clinically and histologically, from the giant cell tumor and other similar lesions invading the temporal region. Since then, Boedeker et al,1 reviewing the international medical literature up to 2003, found 17 other cases of GCG involving the temporal bone, including their
533 own patient. In the same year (2003) 2 new cases were published in the medical literature.8,9 Whereas, including our patient, only 21 cases of the temporal bone have been reported up to now (Table 1). From the study of the reported cases, clinically, results indicate that hearing loss, local swelling, tinnitus, pain, vertigo, and facial weakness are the most common symptoms when GCG arises in the temporal bone. A tendency of more frequent expression of the lesion in men is possible because the ratio of male to female patients is 15:6 and, despite the small number of the published cases, does not appear the female predilection that has been reported for GCG of the jaws.2,4 The age range of the reported temporal bone GCG (including the case presented by us) is 4 months to 72 years, with mean age of patients being 30.4 years, whereas adolescents and young adults (ages 10 to 25 years) have shown to be more prone in the case of jaw GCG.2,4 The etiopathogenesis of the GCG of the skull bones is unclear but it has been suggested, as in the case of jawbones, to be the result of an exacerbated reparative process related to previous trauma and intraosseous hemorrhage that triggers the reactive granulomatous process.2,3,7,13 From the 21 reported cases of the present review, including our patient, only in 5 cases was there a clear history of previous head trauma.2,3,19,20 Other causes regarding the etiopathogenesis of the lesion have also been proposed, but not one single theory has gained wide acceptance.1,3 GCG of the craniofacial region usually has a benign course. Its clinical behavior, as it results from the study of jaws, and GCG’s location in the majority of reported cases ranges from a slowly growing asymptomatic swelling to a more aggressive form which, although rarely, can cause pain, paresthesia, local destruction of bone, cortical bone perforation, and root resorption.4-6 On occasion, in the rest of the facial skeleton, GCG may show wide local slowly growing spread, as the thin maxillofacial bone form less effective barriers to lesion growth.6 In the skull base and the temporal bone, in particular, the wide local spreading of the lesion (because of the initial asymptomatic course and its proximity to vital structures) usually results in extension of the lesion to the infratemporal fossa, the structures of the ear, and up to the floor of the middle cranial fossa, behaving like an intracranial tumor.19 Histologically, GCG of the craniofacial bones should be differentiated from a number of lesions, in which giant cells are found, including benign and malignant neoplasms as well as non-neoplastic and metabolic conditions such as fibrous dysplasia, chondroblastoma, aneurysmal bone cyst, cherubism, and particularly hyperparathyroidism and giant-cell tumor.1-6,14 Differential diagnosis is usually achieved with the recognition of the specific histologic characteristics
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GIANT-CELL GRANULOMA IN THE TEMPORAL BONE
Table 1. SUMMARY OF AVAILABLE CLINICAL DATA OF ALL REPORTED CASES OF PATIENTS WITH TEMPORAL BONE GCG
Study
Age/Gender
Hirschl and Katz, 19743 Colclasure et al, 198122
34 yr/F 10 yr/M 22 yr/M
Pain, tinnitus, hearing loss Hearing loss Mass, hearing loss
Tesluk et al, 198927 Ciappetta et al, 199019 Cohen and GrandaRicart, 199328 Lewis et al, 199420
56 yr/M 25 yr/M 4 mo/F
Nemoto et al, 199529
Maruno et al, 199730
Initial Symptoms
Treatment
Follow-Up Well at 2 yr Well at 6 yr Well at 7 yr
Pain, mass Pain, dysphagia Mass
Biopsy and radiation Total resection Total resection and radiation Total resection Total resection Total resection
32 yr/F
Hearing loss
Total resection
36 yr/M
Hearing loss, tinnitus
Total resection
28 yr/M
Hearing loss, tinnitus
Total resection
Hearing loss, facial weakness
Total resection
Well after indefinable time Well after indefinable time Well after indefinable time Recurrence at 1 yr Recurrence at 1 yr Well at 3 yr
3 yr/F
Ung et al, 200121
NR
Hearing loss
Total resection
Lin et al, 200132
44 yr/M
Hearing loss, tinnitus
Khoduei et al, 200131 Sharma et al, 20022
72 yr/M 36 yr/M 12 yr/M
Matsui et al, 200225 Yoshimura et al, 200226 Boedeker et al, 20031
41 yr/M 38 yr/M 17 yr/F
Hearing loss Pain, tinnitus, vertigo Mass, hearing loss, facial weakness, tinnitus, vertigo Mass NR Hearing loss
Total resection and radiation Total resection Total resection Total resection
Tian et al, 20038 Montero et al, 20039 Dimitakopoulos et al*
32 yr/M 60 yr/M 43 yr/M
Mass Hearing loss Mass
Total resection Total resection Biopsy initially, total resection 3 yr after Curettage Total resection Total resection
Well at 1 yr Well at 15 yr NR
Well at 21 mo Well at 3 yr NR NR NR Well at 2 yr Well at 26 mo Well at 2 yr Well at 3 yr
Abbreviation: NR, not reported. *Present case. Dimitakopoulos et al. Giant-Cell Granuloma in the Temporal Bone. J Oral Maxillofac Surg 2006.
of each condition,1,5,14 whereas brown tumor of hyperparathyroidism, which is indistinguishable microscopically from GCG of bone, can be easily ruled out with biochemical tests showing normal serum calcium, phosphorous, alkaline phosphatase as well as parathyroid hormone levels. From a clinicopathologic point of view, it is more important to distinguish GCG from a giant cell tumor of the temporal bone, where both are rarely encountered, because of their different biological behavior and outcome of treatment. Giant-cell tumor is a true neoplasm with higher incidence of recurrence and potential for malignant transformation and metastatic spread.3,8,21 On the contrary, malignancy and metastasis have never been reported in GCG.1,2,21 Although several histologic features have been recommended distinguishing GCG from giant-cell tumor in borderline cases, considerable overlap between the 2 lesions may exist.1,8,22
That is why Stolovitzky et al23 introduced the term giant-cell lesion to describe these borderline cases of the craniofacial area that can be further classified as aggressive or non-aggressive on the basis of symptoms. However, it has been emphatically recommended that it is important to make every effort to separate giant-cell tumor from the skull GCG because of the much worse prognosis of the giant-cell tumor.1,3,14,22 In our case, the patient had suffered a left side head trauma during his work 5 years prior to his referral, for which he had been treated for 2 days at a regional hospital. His principal presenting symptom was a painful, slowly growing suprazygomatic preauricular swelling associated with intermittent headaches. Computed tomography scan as well as TI and T2 weighted MRI showed a large soft tissue mass arising in the left temporal bone and extending in the infra-
DIMITAKOPOULOS ET AL
temporal fossa. The lesion had also eroded the left zygomatic and the temporal squama invading in the middle cranial fossa pushing upwards the dura and temporal lobe of the brain mimicking, as the previously reported cases, an intracranial tumor.19 External carotid angiography showed a mildly vascular mass supplied by the temporal branches of the internal maxillary artery. Microscopically, the lesion, which was consistent with a GCG of the temporal bone, was composed of a delicate fibrillar connective tissue stroma containing a large number of ovoid or spindleshaped young fibroblast cells and multinucleated giant cells resembling osteoclasts. Foci of hemorrhage and deposits of hemosiderin ingested by mononuclear phagocytes as well as numerous capillaries and inflammatory cell infiltration were also present, Surgical excision is the treatment of choice for GCG of the skull base.1,21,22 The extent of tissue removal, depending on the extent, site, and progression of the lesion, has been described from aggressive curettage to en block resection.4,5,21 Although complete excision provides a unique challenge to a surgeon, because of the complexity and number of the vital structures of the skull base, it is associated with a recurrence rate of only 10% as compared with the higher recurrence rate of up to 50% when simple curettage is used.19,21,24 Where angiography has shown that GCG is highly vascular, the preoperative obliteration of the feeding arteries with super-selective intravascular embolization is helpful for total removal of the lesion.25,26 Radiotherapy has been recommended only in cases where complete surgical excision is not possible, in patients who refuse surgery, or those who cannot be subjected to surgery because of the extent and number of the involved structures of the skull base bones.3,12,14 Results from the present review of the 21 patients with GCG of the temporal bone1-3,8,9,19-22,25-32 indicate that 3 underwent radiation therapy3,22,32 which, although very effective when used in cases not amenable to surgical approach or in recurrent GCG of the temporal bone, does carry a risk for development of osteogenic sarcoma.8,13,21 Apart from radiotherapy, GCG of the temporal bone has not been reported to be treated by nonsurgical methods used in the case of the involved jaws, such as systemic administration of calcitonin,33,34 intralesional injection with corticosteroids,35 and interferon alpha-2a administration.36
References 1. Boedeker CC, Kayser G, Ridder GJ, et al: Giant-cell reparative granuloma of the temporal bone: A case report and review of the literature. Ear Nose Throat J 82:926, 2003 2. Sharma RR, Verma A, Pawar SJ, et al: Pediatric giant cell granuloma of the temporal bone: A case report and brief review of the literature. J Clin Neurosci 9:459, 2002
535 3. Hirsch S, Katz A: Giant cell reparative granuloma outside the jaw bone. Diagnostic criteria and review of the literature with the first case described in the temporal bone. Hum Pathol 5:171, 1974 4. Bataineh AB, Al-Khateed T, Rawashdeh MA: The surgical treatment of central giant cell granuloma of the mandible. J Oral Maxillofac Surg 60:756, 2002 5. Causon RA, Binnie WH, Barrett AW, et al: Oral disease, in: Clinical and Pathological Correlations. Ed 3. Edinburgh, London, New York, Mosby, 2001, pp 711-712 6. Greer RO, Rohrer MD, Young SK: Non-odontogenic tumors. Clinical evaluation and pathology, in Thawley SE, Panje WP, eds: Comprehensive Management of Head and Neck Tumors. Ed 2. Philadelphia, PA, Saunders, 1999, pp 1637-1691 7. Jaffe HL: Giant cell reparative granuloma, traumatic bone cyst and fibrous (fibroosseus) dysplasia of the jawbones. Oral Surg 6:159, 1953 8. Tian HF, Li TZ, Yu SF: Giant cell granuloma of the temporal bone: A case report with immunohistochemical, enzyme histochemical, and in vitro studies. Arch Pathol Lab Med 127: 1217, 2003 9. Montero EH, Navarro JS, Pueyo JL, et al: Giant cell reparative granuloma in the temporal bone. Am J Otolaryngol 24:191, 2003 10. Kramer RH, Pindborg J, Shear M: Histological Typing of Odontogenic Tumors. Ed 2. Berlin, Germany, Springer-Verlag, 1991, p 31 11. Rogers LF, Mickael M, Christ M, et al: A case report of 276 giant cell (reparative) granuloma of the sphenoid bone. Skeletal Radiol 12:48, 1984 12. Alappat JP, Pillai AM, Prasanna D, et al: Giant cell reparative granuloma of the craniofacial complex. Case report and review of the literature. Br J Neurosurg 6:71, 1992 13. Garza-Mercado R, Cavazos E, Hernandez-Batres F: Giant cell reparative granuloma of the cranial vault. Exceptional bone lesion. Neurosurgery 15:228, 1984 14. Santos-Briz A, Lobato RD, Ramos A, et al: Giant cell reparative granuloma of the occipital bone. Skeletal Radiol 32:151, 2002 15. Som PW, Lawson W, Cohen BA: Giant-cell lesions of the facial bones. Radiology 147:129, 1983 16. Oda Y, Tsuneyoshi M, Shinohara N: Solid variant of aneurysmal bone cyst (extragnathic giant cell reparative granuloma) in the axial skeleton and long bones. A study of its morphologic spectrum and distinction from allied giant cell lesions. Cancer 70:2642, 1992 17. Glass TA, Mills SE, Fechner RE, et al: Giant-cell reparative granuloma of the hands and feet. Radiology 149:65, 1983 18. Friedberg SA, Eisenstein R, Wallner LJ: Giant cell lesions involving the nasal accessory sinuses. Laryngoscope 79:763, 1969 19. Ciappetta P, Salvati M, Bernardi C, et al: Giant cell reparative granuloma of the skull base mimicking an intracranial tumor. Case report and review of the literature. Surg Neurol 33:52, 1990 20. Lewis ML, Weber AL, McKenna MJ: Reparative cell granuloma of the temporal bone. Ann Otol Rhinol Laryngol 103:826, 1994 21. Ung F, Li KK, Keith DA: Giant cell reparative granuloma of the temporal bone. Acta Otolaryngol 121:523, 2001 22. Colclasure JB, Shea MC, Graham SS: Giant cell lesions of the temporal bone. Am J Otol 2:188, 2001 23. Stolovitzky JP, Waldrom CA, McConnel FM: Giant cell lesions of the maxilla and paranasal sinuses. Head Neck 16:143, 1994 24. Auclair PL, Cuenin P, Kratochvil FJ, et al: A clinical and histomorphologic comparison of central giant cell granuloma and the giant cell tumor. Oral Surg Oral Med Oral Pathol 66:197, 1988 25. Matsui T, Iwamuro K, Ishikawa T, et al: Large giant cell reparative granuloma of the petrous bone-case report. Neurol Med Chir (Tokyo) 42:232, 2002 26. Yoshimura J, Onda K, Tanaka R, et al: Giant cell reparative granuloma of the temporal bone. Neuroradiological and immunohistochemical findings. Neurol Med Chir (Tokyo) 42:510, 2002 27. Tesluk H, Senders CW, Dublin AB: Case report 562: Giant cell reparative granuloma of temporal bone. Skeletal Radial 18:599, 1989
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28. Cohen D, Granda-Ricart MC: Giant cell reparative granuloma of the base of the skull in a 4-month-old infant-CT findings. Pediatr Radiol 23:319, 1993 29. Nemoto Y, Inoue Y, Tashiro T, et al: Central giant cell granuloma of the temporal bone. Am J Neuroradiol 16:982, 1995 30. Maruno M, Yoshimine T, Kubo T, et al: A case giant cell reparative granuloma of the petrous bone. Demonstration of the proliferative component. Surg Neurol 48:64, 1997 31. Knoduei I, Rowley H, Farrel M, et al: An unusual cause for tinnitus. Ir Med J 94:312, 2001 32. Lin J, Zhong DR, Liu LF, et al: Giant cell reparative granuloma of the temporal bone. Acta Otolaryngol 121:523, 2001
33. De Lange J, Rosenberg AJ, Van den Akker HP, et al: Treatment of central giant cell of the jaw with calcitonin. Int J Oral Maxillofac Surg 28:372, 1999 34. Pogrel MA, Regezi JA, Harris ST, et al: Calcitonin treatment for central giant cell granulomas of the mandible. Report of two cases. J Oral Maxillofac Surg 57:848, 1999 35. Rajeevan NS, Soumithran CS: Intralesional corticosteroid injection for central giant cell granuloma: A case report. Int J Oral Maxillofac Surg 27:1145, 1999 36. Kaban LB, Mulliken JB, Ezekowitz A: Antiangiogenic therapy of recurrent giant cell tumor of the mandible with interferon alpha-2a. Pediatrics 103:1145, 1999
J Oral Maxillofac Surg 64:536-542, 2006
A New Technique for Intraoral Maxillary Distraction: A Case Report Stuart Super, DMD,* Jonathan E. Schecter, DDS,† and Richard D. Bae, DDS‡ Distraction osteogenesis has become a major surgical technique aiding in the promotion of bone formation. The driving force behind distraction osteogenesis lies in its ability to stimulate new bone formation between 2 osteotomized segments of bone gradually separating under tension.1-3 It was not until 1992, however, that distraction osteogenesis for the craniofacial complex came into use.4 In its earliest craniofacial applications, distraction osteogenesis was limited to treating deficient mandibles in children with hemifacial microsomia. Successful advancements have been made that allowed for the treatment of the maxilla with both predictability and stability.5-7 As with the original mandibular distraction appliances, the maxillary extraoral appliances used were esthetically unappealing as well as difficult for the patient to manage.8 Recently, more inconspicuous intraoral distraction appliances have been developed and used with varying
*Chief, Department of Oral and Maxillofacial Surgery, Lenox Hill Hospital, New York, NY; Associate Professor, Department of Oral and Maxillofacial Surgery, New York University College of Dentistry, New York, NY. †Orthognathic Fellow, Department of Oral and Maxillofacial Surgery, Lenox Hill Hospital, New York, NY. ‡Chief Resident, Department of Oral and Maxillofacial Surgery, NYU/Bellevue Hospital Center, New York, NY. Address correspondence and reprint requests to Dr Super: 6 East 78th St, New York, NY 10021; e-mail: [email protected] © 2006 American Association of Oral and Maxillofacial Surgeons
0278-2391/06/6403-0029$32.00/0 doi:10.1016/j.joms.2005.11.021
success. We present a case report using a variation of an intraoral maxillary distraction appliance.
Materials and Methods PRESURGICAL TECHNIQUE
The appliance designed was used for a 21-year-old man who presented with a surgically repaired unilateral cleft lip and palate, as well as a moderately hypoplastic maxilla. The patient had been previously treated using conventional orthognathic surgery in which a maxillary advancement was performed. In addition, the patient underwent the repair of a chronic oronasal fistula. Because of the vast amount of fibrous scar tissue created from the cleft lip and palate repair, the patient exhibited a complete relapse of the maxillary advancement within 12 months postoperatively. Therefore, it was decided that distraction osteogenesis would be used to treat the hypoplastic maxilla. At first we proposed to use the rigid external distractor system (KLS-Martin LP, Tuttlingen, Germany).9,10 However, because of the patient’s reluctance to wear the device while going to college, we instead decided to use the intraoral Antwerp transsinusoidal maxillary distractor (KLS-Martin LP)11 for treatment. Unfortunately, because of insufficient thickness and strength of the patient’s maxillary sinus wall, placement of these distractors could not be facilitated. These events propagated the development of an intraoral distraction appliance that would exhibit stability, predictability, vector control, and patient tolerance.