The Journal of Craniofacial Surgery
Brief Clinical Studies
eruptions on the face, scar on the left side of the upper lip caused by repair of the cleft, and bridging of the sella turcica. Squamous cell carcinoma occurred few months after diagnosing BCNS. A partial maxillectomy was performed after a 37.5-Gy dose of irradiation. Therapeutic protocol has been completed by 40 Gy of telecobalt gamma-ray irradiation in combination with continuous intra-arterial infusion of 5-fluorouracil and 2-stage of surgical reduction. Five months after discharge, they discovered that the patient had lymph node metastasis in the left mandibular region without recurrence in the primary area. A radical neck dissection was then performed. Six years later, the patient was considered cured. In 2010, Habibi and Jafarzadeh6 reported the second patient, a 29-year-old male patient who has developed an SCC of the maxillary sinus 10 years after diagnosing a BCNS. All his immediate family except for his mother has been affected by severe BCNS. Clinical features were several OKCs, calcification of the falx cerebri, bifid ribs, and first-degree relative with BCNS. He also presented a pseudoprognathism of the mandible, mild hypertelorism, and mental retardation. Squamous cell carcinoma occurred 21 years after the first cystic mandibular lesion. A hemimaxillectomy was performed in combination with radiotherapy and chemotherapy. The patient was stable at the last follow-up. The early diagnosis of BCNS is essential for detection of severe manifestations in young patients and for provision of advice concerning sunlight protection. Severe complications may affect patient with a Gorlin syndrome and justify a regular follow-up. The management of these patients must include an interdisciplinary cooperation.8 Despite her medical history, no major criterion suggestive of a BCNS had been previously identified. No maxillofacial or dermatological follow-up had been established.
ACKNOWLEDGMENT The authors thank Dr. Delattre C., Department of Anatomopatholgy, CHU, Lille, France. REFERENCES 1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 1960;262:908Y912 2. Anderson DE, Cook WA. Jaw cysts and the basal cell nevus syndrome. J oral Surg 1966;24:15Y26 3. Dalati T, Zhou H. Gorlin syndrome with ameloblastoma: a case report and review of literature. Cancer Invest 2008;26:975Y976 4. Moos KF, Rennie JS. Squamous cell carcinoma arising in a mandibular keratocyst in a patient with Gorlin’s syndrome. Br J Oral Maxillofac Surg 1987;25:280Y284 5. Hasegawa K, Amagasa T, Shioda S, et al. Basal cell nevus syndrome with squamous cell carcinoma of the maxilla: report of a case. J Oral Maxillofac Surg 1989;47:629Y633 6. Habibi A, Jafarzadeh H. Squamous cell carcinoma of the maxillary sinus associated with nevoid basal cell carcinoma syndrome: report of a case with 21-year evaluation. J Oral Maxillofac Surg 2010;68: 1982Y1986 7. Kimonis VE, Goldstein AM, Pastakia B, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299Y308 8. Bree AF, Shah MR, for the BCNS Colloquium Group. Consensus statement from the first international colloquium on basal cell nevus syndrome (BCNS). Am J Med Genet A 2011;155:2091Y2097 9. Haring JI, van Dis ML. Odontogenic keratocysts: a clinical, radiographic, and histopathologic study. Oral Surg Oral Med Oral Pathol 1988;66:145Y153 10. Brannon RB. The Odontogenic keratocyst. A clinicopathologic study of 312 cases. Part I. Clinical features. Oral Surg Oral Med Oral Pathol 1976;42:54Y72
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11. Voorsmit RA, Stoelinga PJ, van Haelst UJ. The management of keratocysts. J Maxillofac Surg 1981;9:228Y236 12. Toller PA. Origin and growth of cysts of the jaws. Ann R Coll Surg Engl 1967;40:306Y336 13. Woolgar JA, Rippin JW, Browne RM. A comparative histological study of odontogenic keratocysts in basal cell naevus syndrome and control patients. J Oral Pathol 1987;16:75 14. Barnes L, Eveson JW, Reichart P, et al. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon, France: IARC Press; 2005 15. Dabbs DJ, Schweitzer RJ, Schweitzer LE, et al. Squamous cell carcinoma arising in recurrent odontogenic keratocyst: case report and literature review. Head Neck 1994;16:375Y378
Extracranial Orbital Meningioma Extending Into the Nasosinus in Neurofibromatosis Type 2 Jianhua Yan, PhD, MD, Yongping Li, MD Purpose: The purpose of this study was to report a rare case of neurofibromatosis type 2 (NF2) with extracranial orbital meningioma extending into the frontal and ethmoidal sinuses. Methods: This is clinical report of a 17-year-old male patient with NF2. Results of an ocular examination, computed tomography scan, and pathological findings of space-occupied orbital and nasosinus lesions are presented. Results: Computed tomography scan revealed a high-density soft tissue mass occupying the right superionasal orbit extending into the frontal and ethmoidal sinuses and bilateral acoustic schwannomas in the brain. Biopsy of the right orbital lesion demonstrated a typical meningioma as indicated with routine hematoxylin-eosin staining and immunohistochemical evaluation. Conclusions: In this patient, an extracranial orbital meningioma, rather than optic nerve sheath meningioma extending into nasosinus, designates a diagnosis of NF2. Ophthalmologists should be aware of the possibility that patients with an extracranial orbital meningioma may also have NF2. Key Words: Orbit, sinus, meningioma, neurofibromatosis type 2
N
eurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder that predisposes the affected patient to bilateral vestibular schwannomas and other central nervous system tumors. The NF2 gene has been identified on chromosome band 22q12 and is characterized as a putative tumor suppressive gene.1Y5 Many ocular presentations are associated with NF2, with cataracts being the most frequent ocular abnormality. Other ocular findings include combined pigment epithelial and retinal hamartomas, optic disc From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou, China. Received August 27, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Dr. Jianhua Yan, Zhongshan Ophthalmic Center, Sun Yat-Sen University, 54 Xieli Nan Rd, Guangzhou 510060, China; 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.0000000000000466
* 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
gliomas, epiretinal membranes, conjunctival neurofibroma, retinal hemangiomas, and choroidal hamartomas.1Y5 Optic nerve sheath meningiomas have been frequently described in NF2.6Y9 However, an association of NF2 with extracranial orbital meningioma, rather than optic nerve sheath meningioma extending into the frontal and ethmoidal sinuses, together with presumed lung schwannoma, has not been previously reported.
CLINICAL REPORT A 17-year-old male (Chinese) patient was referred to our clinic on December 28, 1999, with the primary symptom being a gradually enlarged right orbital and eyelid mass that was present for 9 years. A biopsy performed at a local hospital 5 years ago indicated no definitive pathological diagnosis. At that time, a chest radiograph revealed a round mass in the apex of the right lung, which was not treated. The patient developed an obvious proptosis, poor eyelid closure, and decreased visual acuity in the right eye, which were present for more than 1 year. Overall, the patient reported that his past general health was good. Family history revealed that his father had neurofibromatosis with numerous cutaneous nodules throughout the body. At our clinic, general physical examination was unremarkable. A chest radiograph indicated a round, homogenous, and well-defined 5 5-cm soft tissue mass without bone erosion at the apex of the right lung (Fig. 1A). Several sporadic subcutaneous ill-defined masses with moderate consistency ranging from 0.5 to 1.0 cm in diameter were present on the skull, calf, and back. On ocular examination, the patient’s best corrected visual acuity was 20/400 in the right eye and 20/20 in the left eye. Intraocular pressure was normal in both eyes. The anterior segment and fundus of the left eye were normal. A 3 3-cm mass was present in the right superionasal orbital region. The margins of this mass were ill defined and fixed to underlying structures but not tender to touch (Fig. 1B). There was a 6-cm surgical skin scar just below the right eyebrow. The patient had a remarkable hyperemia and chemosis of the bulbar conjunctiva. There was an exposed corneal ulcer of about 6 4 mm. A Hertel exophthalmometer displayed an 11-mm proptosis with an inferior and lateral displacement of the globe. Ocular motility revealed a complete lateral rectus muscle palsy and a moderate restriction in the cardinal directions of the superior and inferior rectus muscles of the right eye. A computed tomography (CT) scan showed a high-density soft tissue mass lesion in the upper nasal quadrant of the right orbit with a maximal
Brief Clinical Studies
diameter of 6 cm. The mass infiltrated the adjacent medial orbital wall causing osseous hypergenesis of the right frontal and ethmoidal sinuses. There were also 2 round, homogenous, and well-defined vestibular masses as observed with CT scan (Fig. 1C). An anterior orbital biopsy and partial debulking of the mass were performed on January 3, 2000. A 2 2 2-cm tumor mass with a profuse blood supply, gray-pink color, firm in consistency, poorly defined, and strongly adherent to nearby orbital structures was resected without destroying any critical adjacent anatomical elements. A small portion of the gross hyperostosis present in the affected bone of the medial orbital wall was also excised. Pathological examination demonstrated that lobules and whorls of epithelial-like cells were separated by thin, vascularized, fibrous septae, and infiltrated orbital fat (Fig. 1D). The removed thickened bone was associated with clumps of tumor cells. Immunohistochemical assay indicated that the tumor cells were positive for S100 and negative for keratin. Diagnosis of this pathology was orbital and sinus meningioma. Final diagnosis was an extracranial orbital meningioma extending into the nasosinus, together with presumed lung schwannoma, associated with NF2. The patient refused any further treatment after anterior orbitotomy and was lost to follow-up thereafter.
DISCUSSION Neurofibromatosis type 2 is characterized by central nervous system tumors, mainly bilateral vestibular schwannomas and meningiomas. Cutaneous changes occur rarely in NF2. The diagnostic criteria for NF2 are the presence of bilateral eighth nerve masses or a unilateral eighth nerve mass and a first-degree relative with bilateral vestibular schwannomas or 2 of the following: meningioma, glioma, schwannoma, or posterior subcapsular lens opacity at a young age. As this patient had bilateral eighth nerve masses and his father also had neurofibromatosis, he met the clinical criteria for the diagnosis of NF2. A well-defined spectrum of ocular abnormalities, such as cataract, epiretinal membranes, retinal glial hamartoma, hyperplasia of the retinal pigment epithelium, disk gliomas, strabismus, and amblyopia, has been shown to be associated with NF2.1Y5 Bosch et al6,7 emphasized the close association between NF2 and optic nerve sheath meningioma, as 27% of their patients with NF2 harbored optic nerve sheath meningioma. Our patient with NF2 had a characteristic finding of orbital meningioma involving the frontal and ethmoidal sinuses associated with a presumed schwannoma of the lung. Such a compilation had not been described previously in the literature. Therefore, the presence of an orbital meningioma as observed at any region, rather than optic nerve sheath meningioma, should alert ophthalmologists to consider the diagnosis of NF2.
REFERENCES
FIGURE 1. A, Chest radiograph revealed a round, homogenous, and well-defined 5 5-cm soft tissue mass without bone erosion at the apex of the right lung (presumed schwannoma). B, Clinical figure showing a 3 3-cm mass in the right superionasal orbital region. The margins of the mass were ill defined and fixed to the underlying structures but were not tender to touch. C, A CT tomography scan showed a soft tissue mass lesion in the superionasal quadrant of the right orbit with a maximal diameter of 6 cm. The mass infiltrated the adjacent medial orbital wall causing osseous hypergenesis of the right frontal and ethmoidal sinuses. There were also 2 round, homogenous, and well-defined vestibular masses on the scan. D, Pathological examination demonstrated that lobules and whorls of epithelial-like cells were separated by thin, vascularized, fibrous septae and infiltrated orbital fat, with a diagnosis of orbital and sinus meningioma (hematoxylin-eosin, original magnification 200).
1. Ragge NK, Baser ME, Klein J, et al. Ocular abnormalities in neurofibromatosis 2. Am J Ophthalmol 1995;120:634Y641 2. Ragge NK, Baser ME, Riccardi VM, Falk RE. The ocular presentation of neurofibromatosis 2. Eye 1997;11:12Y18 3. Kaye LD, Rothner D, Beauchamp GR, et al. Ocular findings associated with neurofibromatosis type. Ophthalmology 1992;99:1424Y1429 4. Landau K, Yasargil GM. Ocular fundus in neurofibromatosis type 2. Br J Ophthalmol 1993;77:646Y649 5. Lueder GT, Doll JT. Pseudopapilledema in neurofibromatosis type 2. Am J Ophthalmol 2000;129:405Y407 6. Bosch MM, Boltshauser E, Harpes P, et al. Ophthalmologic findings and long-term course in patients with neurofibromatosis type 2. Am J Ophthalmol 2006;141:1068Y1077 7. Bosch MM, Wichmann WW, Boltshauser E, et al. Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol 2006;124:379Y385
* 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
8. Jain D, Ebrahimi KB, Miller NR, et al. Intraorbital meningiomas: a pathologic review using current World Health Organization criteria. Arch Pathol Lab Med 2010;134:766Y770 9. Kiratli H, Yildiz S, Soylemezo?lu F. Neurofibromatosis type 2: optic nerve sheath meningioma in one orbit, intramuscular schwannoma in the other. Orbit 2008;27:451Y454
An Underestimated ‘‘Ghost’’ Foreign Body as an Unusual Cause of Mandibular Osseocutaneous Fistula Formation: Impacted Spike of a Soccer Cleat C¸a?ri C¸akmako?lu, MD,* Nebil Yez ilo?lu, MD,* Emre Gu¨vercin, MD,* Ismail Mithat Akan, MDÞ Abstract: In this article, the case of a patient with osteocutaneous fistula at the left malar region secondary to impacted spike of a soccer cleat to the mandible is presented. Both the clinical and radiologic diagnoses failed because of an obscure anamnesis of the patient and the unavailability of viewing the spike in orthopantomogram and computed tomography. Surgical extirpation was performed to the 41-year-old man who was injured in a football match 3 months before the presentation and had a swooning history after an accidental booting. There were no early or late complications after the surgery at the end of 9 months. This study shows the importance of both obtaining a definite history of patients and sequential radiologic imaging to make a differential diagnosis between the foreign bodies and cystic or noncystic tumors and inflammatory lesions of the mandible. Key Words: Mandibular fistula, soccer cleat, sports injuries, mandibular fractures, football injuries
I
njuries of facial structures in football matches are usually caused by the impact of elbow, knee, or head-to-head contacts in our experience. Rarely, accidental booting to the face causes fractures and soft tissue lacerations. Although designs of clothing and shoes have evolved in the recent decades to provide more security in sport activities,1,2 they may still cause serious maxillofacial injuries. In this article, the authors present a consequence of such an injury with
From the *Department of Plastic Reconstructive and Aesthetic Surgery, Dr. Lu¨tfi KNrdar Kartal Training and Research Hospital; and †Division of Plastic Reconstructive and Aesthetic Surgery, Medipol University Medical Faculty, Istanbul, Turkey. Received August 27, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Nebil Yezilo?lu, MD, Department of Plastic Reconstructive and Aesthetic Surgery, Dr. Lu¨tfi KNrdar Kartal Training and Research Hospital, yemsi Denizer Cad. E-5 Karayolu Cevizli Mevkii 34890 Kartal/Istanbul, Turkey (Office); Acibadem Mh. Go¨me0 Sk. Sabanci-1 Sitesi 8/16 34718 Kadiko¨y, Istanbul, Turkey (Home); 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.0000000000000389
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FIGURE 1. The orifice of the osteocutaneous fistula tract at the left malar region.
an unexpected diagnosis of spike of a soccer cleat that was impacted to the mandible as a rare cause of osteocutaneous fistula.
CLINICAL PRESENTATION The patient was a 41-year-old man who presented with edema and a purulent discharge from an opening on the left malar skin. He was injured in a football match 3 months before the presentation and had a swooning history after an accidental booting. After the injury, he underestimated the small laceration at the inferior jaw line, which was healed with dressing changes. In the physical examination, a 3-cmYwide area of edema and induration was palpated. An orifice of the fistula tract was detected (Fig. 1). There were no signs of an intraoral communication of the fistula, such as odor, discharge, or inflammatory changes. In the orthopantomogram (Fig. 2), there was a slight erosion of the mandibular bone, whereas maxillofacial computed tomographic (CT) views revealed a ‘‘bitelike’’ erosion on the left ramus of the mandible that gives an impression of a fistula formation secondary to a mandibular cyst (Fig. 3). Initial symptoms and an obscure trauma history made us to take an excisional biopsy from the area including the skin island around the fistula, soft tissue, and bone. The patient underwent a surgery for both accurate diagnosis and treatment under general anesthesia. After the resection of the skin orifice of the fistula tract combined to an underlying soft tissue, a 2-cmYlong plastic cleat spike was detected in the area and extracted (Fig. 4). After deep tissue culture sampling, bony curettage and soft tissue debridement and irrigation with saline solution were performed. Some of the curettage materials were sent to the pathology department for assessment. During the exploration, the marginal mandibular branch of the facial nerve was intact. After the soft tissue and skin repair, empirical antibiotic therapy including cefazolin (1 g, intravenous)
FIGURE 2. Orthopantopogram of the patient with a minimal destruction of the left mandibular ramus.
FIGURE 3. Computed tomographic scan of the same patient.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
eruptions on the face, scar on the left side of the upper lip caused by repair of the cleft, and bridging of the sella turcica. Squamous cell carcinoma occurred few months after diagnosing BCNS. A partial maxillectomy was performed after a 37.5-Gy dose of irradiation. Therapeutic protocol has been completed by 40 Gy of telecobalt gamma-ray irradiation in combination with continuous intra-arterial infusion of 5-fluorouracil and 2-stage of surgical reduction. Five months after discharge, they discovered that the patient had lymph node metastasis in the left mandibular region without recurrence in the primary area. A radical neck dissection was then performed. Six years later, the patient was considered cured. In 2010, Habibi and Jafarzadeh6 reported the second patient, a 29-year-old male patient who has developed an SCC of the maxillary sinus 10 years after diagnosing a BCNS. All his immediate family except for his mother has been affected by severe BCNS. Clinical features were several OKCs, calcification of the falx cerebri, bifid ribs, and first-degree relative with BCNS. He also presented a pseudoprognathism of the mandible, mild hypertelorism, and mental retardation. Squamous cell carcinoma occurred 21 years after the first cystic mandibular lesion. A hemimaxillectomy was performed in combination with radiotherapy and chemotherapy. The patient was stable at the last follow-up. The early diagnosis of BCNS is essential for detection of severe manifestations in young patients and for provision of advice concerning sunlight protection. Severe complications may affect patient with a Gorlin syndrome and justify a regular follow-up. The management of these patients must include an interdisciplinary cooperation.8 Despite her medical history, no major criterion suggestive of a BCNS had been previously identified. No maxillofacial or dermatological follow-up had been established.
ACKNOWLEDGMENT The authors thank Dr. Delattre C., Department of Anatomopatholgy, CHU, Lille, France. REFERENCES 1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 1960;262:908Y912 2. Anderson DE, Cook WA. Jaw cysts and the basal cell nevus syndrome. J oral Surg 1966;24:15Y26 3. Dalati T, Zhou H. Gorlin syndrome with ameloblastoma: a case report and review of literature. Cancer Invest 2008;26:975Y976 4. Moos KF, Rennie JS. Squamous cell carcinoma arising in a mandibular keratocyst in a patient with Gorlin’s syndrome. Br J Oral Maxillofac Surg 1987;25:280Y284 5. Hasegawa K, Amagasa T, Shioda S, et al. Basal cell nevus syndrome with squamous cell carcinoma of the maxilla: report of a case. J Oral Maxillofac Surg 1989;47:629Y633 6. Habibi A, Jafarzadeh H. Squamous cell carcinoma of the maxillary sinus associated with nevoid basal cell carcinoma syndrome: report of a case with 21-year evaluation. J Oral Maxillofac Surg 2010;68: 1982Y1986 7. Kimonis VE, Goldstein AM, Pastakia B, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299Y308 8. Bree AF, Shah MR, for the BCNS Colloquium Group. Consensus statement from the first international colloquium on basal cell nevus syndrome (BCNS). Am J Med Genet A 2011;155:2091Y2097 9. Haring JI, van Dis ML. Odontogenic keratocysts: a clinical, radiographic, and histopathologic study. Oral Surg Oral Med Oral Pathol 1988;66:145Y153 10. Brannon RB. The Odontogenic keratocyst. A clinicopathologic study of 312 cases. Part I. Clinical features. Oral Surg Oral Med Oral Pathol 1976;42:54Y72
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& Volume 25, Number 2, March 2014
11. Voorsmit RA, Stoelinga PJ, van Haelst UJ. The management of keratocysts. J Maxillofac Surg 1981;9:228Y236 12. Toller PA. Origin and growth of cysts of the jaws. Ann R Coll Surg Engl 1967;40:306Y336 13. Woolgar JA, Rippin JW, Browne RM. A comparative histological study of odontogenic keratocysts in basal cell naevus syndrome and control patients. J Oral Pathol 1987;16:75 14. Barnes L, Eveson JW, Reichart P, et al. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon, France: IARC Press; 2005 15. Dabbs DJ, Schweitzer RJ, Schweitzer LE, et al. Squamous cell carcinoma arising in recurrent odontogenic keratocyst: case report and literature review. Head Neck 1994;16:375Y378
Extracranial Orbital Meningioma Extending Into the Nasosinus in Neurofibromatosis Type 2 Jianhua Yan, PhD, MD, Yongping Li, MD Purpose: The purpose of this study was to report a rare case of neurofibromatosis type 2 (NF2) with extracranial orbital meningioma extending into the frontal and ethmoidal sinuses. Methods: This is clinical report of a 17-year-old male patient with NF2. Results of an ocular examination, computed tomography scan, and pathological findings of space-occupied orbital and nasosinus lesions are presented. Results: Computed tomography scan revealed a high-density soft tissue mass occupying the right superionasal orbit extending into the frontal and ethmoidal sinuses and bilateral acoustic schwannomas in the brain. Biopsy of the right orbital lesion demonstrated a typical meningioma as indicated with routine hematoxylin-eosin staining and immunohistochemical evaluation. Conclusions: In this patient, an extracranial orbital meningioma, rather than optic nerve sheath meningioma extending into nasosinus, designates a diagnosis of NF2. Ophthalmologists should be aware of the possibility that patients with an extracranial orbital meningioma may also have NF2. Key Words: Orbit, sinus, meningioma, neurofibromatosis type 2
N
eurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder that predisposes the affected patient to bilateral vestibular schwannomas and other central nervous system tumors. The NF2 gene has been identified on chromosome band 22q12 and is characterized as a putative tumor suppressive gene.1Y5 Many ocular presentations are associated with NF2, with cataracts being the most frequent ocular abnormality. Other ocular findings include combined pigment epithelial and retinal hamartomas, optic disc From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou, China. Received August 27, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Dr. Jianhua Yan, Zhongshan Ophthalmic Center, Sun Yat-Sen University, 54 Xieli Nan Rd, Guangzhou 510060, China; 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.0000000000000466
* 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
gliomas, epiretinal membranes, conjunctival neurofibroma, retinal hemangiomas, and choroidal hamartomas.1Y5 Optic nerve sheath meningiomas have been frequently described in NF2.6Y9 However, an association of NF2 with extracranial orbital meningioma, rather than optic nerve sheath meningioma extending into the frontal and ethmoidal sinuses, together with presumed lung schwannoma, has not been previously reported.
CLINICAL REPORT A 17-year-old male (Chinese) patient was referred to our clinic on December 28, 1999, with the primary symptom being a gradually enlarged right orbital and eyelid mass that was present for 9 years. A biopsy performed at a local hospital 5 years ago indicated no definitive pathological diagnosis. At that time, a chest radiograph revealed a round mass in the apex of the right lung, which was not treated. The patient developed an obvious proptosis, poor eyelid closure, and decreased visual acuity in the right eye, which were present for more than 1 year. Overall, the patient reported that his past general health was good. Family history revealed that his father had neurofibromatosis with numerous cutaneous nodules throughout the body. At our clinic, general physical examination was unremarkable. A chest radiograph indicated a round, homogenous, and well-defined 5 5-cm soft tissue mass without bone erosion at the apex of the right lung (Fig. 1A). Several sporadic subcutaneous ill-defined masses with moderate consistency ranging from 0.5 to 1.0 cm in diameter were present on the skull, calf, and back. On ocular examination, the patient’s best corrected visual acuity was 20/400 in the right eye and 20/20 in the left eye. Intraocular pressure was normal in both eyes. The anterior segment and fundus of the left eye were normal. A 3 3-cm mass was present in the right superionasal orbital region. The margins of this mass were ill defined and fixed to underlying structures but not tender to touch (Fig. 1B). There was a 6-cm surgical skin scar just below the right eyebrow. The patient had a remarkable hyperemia and chemosis of the bulbar conjunctiva. There was an exposed corneal ulcer of about 6 4 mm. A Hertel exophthalmometer displayed an 11-mm proptosis with an inferior and lateral displacement of the globe. Ocular motility revealed a complete lateral rectus muscle palsy and a moderate restriction in the cardinal directions of the superior and inferior rectus muscles of the right eye. A computed tomography (CT) scan showed a high-density soft tissue mass lesion in the upper nasal quadrant of the right orbit with a maximal
Brief Clinical Studies
diameter of 6 cm. The mass infiltrated the adjacent medial orbital wall causing osseous hypergenesis of the right frontal and ethmoidal sinuses. There were also 2 round, homogenous, and well-defined vestibular masses as observed with CT scan (Fig. 1C). An anterior orbital biopsy and partial debulking of the mass were performed on January 3, 2000. A 2 2 2-cm tumor mass with a profuse blood supply, gray-pink color, firm in consistency, poorly defined, and strongly adherent to nearby orbital structures was resected without destroying any critical adjacent anatomical elements. A small portion of the gross hyperostosis present in the affected bone of the medial orbital wall was also excised. Pathological examination demonstrated that lobules and whorls of epithelial-like cells were separated by thin, vascularized, fibrous septae, and infiltrated orbital fat (Fig. 1D). The removed thickened bone was associated with clumps of tumor cells. Immunohistochemical assay indicated that the tumor cells were positive for S100 and negative for keratin. Diagnosis of this pathology was orbital and sinus meningioma. Final diagnosis was an extracranial orbital meningioma extending into the nasosinus, together with presumed lung schwannoma, associated with NF2. The patient refused any further treatment after anterior orbitotomy and was lost to follow-up thereafter.
DISCUSSION Neurofibromatosis type 2 is characterized by central nervous system tumors, mainly bilateral vestibular schwannomas and meningiomas. Cutaneous changes occur rarely in NF2. The diagnostic criteria for NF2 are the presence of bilateral eighth nerve masses or a unilateral eighth nerve mass and a first-degree relative with bilateral vestibular schwannomas or 2 of the following: meningioma, glioma, schwannoma, or posterior subcapsular lens opacity at a young age. As this patient had bilateral eighth nerve masses and his father also had neurofibromatosis, he met the clinical criteria for the diagnosis of NF2. A well-defined spectrum of ocular abnormalities, such as cataract, epiretinal membranes, retinal glial hamartoma, hyperplasia of the retinal pigment epithelium, disk gliomas, strabismus, and amblyopia, has been shown to be associated with NF2.1Y5 Bosch et al6,7 emphasized the close association between NF2 and optic nerve sheath meningioma, as 27% of their patients with NF2 harbored optic nerve sheath meningioma. Our patient with NF2 had a characteristic finding of orbital meningioma involving the frontal and ethmoidal sinuses associated with a presumed schwannoma of the lung. Such a compilation had not been described previously in the literature. Therefore, the presence of an orbital meningioma as observed at any region, rather than optic nerve sheath meningioma, should alert ophthalmologists to consider the diagnosis of NF2.
REFERENCES
FIGURE 1. A, Chest radiograph revealed a round, homogenous, and well-defined 5 5-cm soft tissue mass without bone erosion at the apex of the right lung (presumed schwannoma). B, Clinical figure showing a 3 3-cm mass in the right superionasal orbital region. The margins of the mass were ill defined and fixed to the underlying structures but were not tender to touch. C, A CT tomography scan showed a soft tissue mass lesion in the superionasal quadrant of the right orbit with a maximal diameter of 6 cm. The mass infiltrated the adjacent medial orbital wall causing osseous hypergenesis of the right frontal and ethmoidal sinuses. There were also 2 round, homogenous, and well-defined vestibular masses on the scan. D, Pathological examination demonstrated that lobules and whorls of epithelial-like cells were separated by thin, vascularized, fibrous septae and infiltrated orbital fat, with a diagnosis of orbital and sinus meningioma (hematoxylin-eosin, original magnification 200).
1. Ragge NK, Baser ME, Klein J, et al. Ocular abnormalities in neurofibromatosis 2. Am J Ophthalmol 1995;120:634Y641 2. Ragge NK, Baser ME, Riccardi VM, Falk RE. The ocular presentation of neurofibromatosis 2. Eye 1997;11:12Y18 3. Kaye LD, Rothner D, Beauchamp GR, et al. Ocular findings associated with neurofibromatosis type. Ophthalmology 1992;99:1424Y1429 4. Landau K, Yasargil GM. Ocular fundus in neurofibromatosis type 2. Br J Ophthalmol 1993;77:646Y649 5. Lueder GT, Doll JT. Pseudopapilledema in neurofibromatosis type 2. Am J Ophthalmol 2000;129:405Y407 6. Bosch MM, Boltshauser E, Harpes P, et al. Ophthalmologic findings and long-term course in patients with neurofibromatosis type 2. Am J Ophthalmol 2006;141:1068Y1077 7. Bosch MM, Wichmann WW, Boltshauser E, et al. Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol 2006;124:379Y385
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
665
The Journal of Craniofacial Surgery
Brief Clinical Studies
& Volume 25, Number 2, March 2014
8. Jain D, Ebrahimi KB, Miller NR, et al. Intraorbital meningiomas: a pathologic review using current World Health Organization criteria. Arch Pathol Lab Med 2010;134:766Y770 9. Kiratli H, Yildiz S, Soylemezo?lu F. Neurofibromatosis type 2: optic nerve sheath meningioma in one orbit, intramuscular schwannoma in the other. Orbit 2008;27:451Y454
An Underestimated ‘‘Ghost’’ Foreign Body as an Unusual Cause of Mandibular Osseocutaneous Fistula Formation: Impacted Spike of a Soccer Cleat C¸a?ri C¸akmako?lu, MD,* Nebil Yez ilo?lu, MD,* Emre Gu¨vercin, MD,* Ismail Mithat Akan, MDÞ Abstract: In this article, the case of a patient with osteocutaneous fistula at the left malar region secondary to impacted spike of a soccer cleat to the mandible is presented. Both the clinical and radiologic diagnoses failed because of an obscure anamnesis of the patient and the unavailability of viewing the spike in orthopantomogram and computed tomography. Surgical extirpation was performed to the 41-year-old man who was injured in a football match 3 months before the presentation and had a swooning history after an accidental booting. There were no early or late complications after the surgery at the end of 9 months. This study shows the importance of both obtaining a definite history of patients and sequential radiologic imaging to make a differential diagnosis between the foreign bodies and cystic or noncystic tumors and inflammatory lesions of the mandible. Key Words: Mandibular fistula, soccer cleat, sports injuries, mandibular fractures, football injuries
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njuries of facial structures in football matches are usually caused by the impact of elbow, knee, or head-to-head contacts in our experience. Rarely, accidental booting to the face causes fractures and soft tissue lacerations. Although designs of clothing and shoes have evolved in the recent decades to provide more security in sport activities,1,2 they may still cause serious maxillofacial injuries. In this article, the authors present a consequence of such an injury with
From the *Department of Plastic Reconstructive and Aesthetic Surgery, Dr. Lu¨tfi KNrdar Kartal Training and Research Hospital; and †Division of Plastic Reconstructive and Aesthetic Surgery, Medipol University Medical Faculty, Istanbul, Turkey. Received August 27, 2013. Accepted for publication September 16, 2013. Address correspondence and reprint requests to Nebil Yezilo?lu, MD, Department of Plastic Reconstructive and Aesthetic Surgery, Dr. Lu¨tfi KNrdar Kartal Training and Research Hospital, yemsi Denizer Cad. E-5 Karayolu Cevizli Mevkii 34890 Kartal/Istanbul, Turkey (Office); Acibadem Mh. Go¨me0 Sk. Sabanci-1 Sitesi 8/16 34718 Kadiko¨y, Istanbul, Turkey (Home); 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.0000000000000389
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FIGURE 1. The orifice of the osteocutaneous fistula tract at the left malar region.
an unexpected diagnosis of spike of a soccer cleat that was impacted to the mandible as a rare cause of osteocutaneous fistula.
CLINICAL PRESENTATION The patient was a 41-year-old man who presented with edema and a purulent discharge from an opening on the left malar skin. He was injured in a football match 3 months before the presentation and had a swooning history after an accidental booting. After the injury, he underestimated the small laceration at the inferior jaw line, which was healed with dressing changes. In the physical examination, a 3-cmYwide area of edema and induration was palpated. An orifice of the fistula tract was detected (Fig. 1). There were no signs of an intraoral communication of the fistula, such as odor, discharge, or inflammatory changes. In the orthopantomogram (Fig. 2), there was a slight erosion of the mandibular bone, whereas maxillofacial computed tomographic (CT) views revealed a ‘‘bitelike’’ erosion on the left ramus of the mandible that gives an impression of a fistula formation secondary to a mandibular cyst (Fig. 3). Initial symptoms and an obscure trauma history made us to take an excisional biopsy from the area including the skin island around the fistula, soft tissue, and bone. The patient underwent a surgery for both accurate diagnosis and treatment under general anesthesia. After the resection of the skin orifice of the fistula tract combined to an underlying soft tissue, a 2-cmYlong plastic cleat spike was detected in the area and extracted (Fig. 4). After deep tissue culture sampling, bony curettage and soft tissue debridement and irrigation with saline solution were performed. Some of the curettage materials were sent to the pathology department for assessment. During the exploration, the marginal mandibular branch of the facial nerve was intact. After the soft tissue and skin repair, empirical antibiotic therapy including cefazolin (1 g, intravenous)
FIGURE 2. Orthopantopogram of the patient with a minimal destruction of the left mandibular ramus.
FIGURE 3. Computed tomographic scan of the same patient.
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
