The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Brief Clinical Studies
CONCLUSIONS We have presented a case where a metallic foreign body has stayed at the temporal surfaces of the sphenoid greater wing posterior to the lateral orbital wall over 20 years, the remains of which have left the patient with chronic sustained pain. Having balanced the potential surgical damage and benefit after surgical removal, we scheduled an operating procedure with the help of an endoscope and a navigation.7,9,17 When the navigation was assisted by the endoscope, extra images from other directions could provide with extensive view; therefore, we could reach the metallic bodies with a minimal damage. Although the metallic foreign body was encapsulated in the connective tissue, we managed to remove the foreign body without cutting extra surrounding tissues. This minimally invasive procedure is a good choice for removing foreign bodies; first, it increases safety, and also it maintains facial aesthetics. The combination of navigation and endoscope system can also be applied to an extensive range of foreign body removals, as well as other kinds of facial surgery.
REFERENCES 1. Liu SY, Cheng WY, Lee HT, et al. Endonasal transsphenoidal endoscopy-assisted removal of a shotgun pellet in the sphenoid sinus: a case report. Surg Neurol 2008;70(suppl 1):S1:56–59 2. Ben-David J, Fradis M, Podoshin L, et al. Plasmacytoma arising in the vicinity of a foreign body in the nasal cavity. Laryngoscope 1981;91:1150–1154 3. Shuker ST. Base-of-skull/infratemporal fossa shell fragment retrieval. J Oral Maxillofac Surg 2010;68:2668–2674 4. Freysinger W, Gunkel AR, Thumfart WF. Image-guided endoscopic ENT surgery. Eur Arch Otorhinolaryngol 1997;254:343–346 5. Brinson GM, Senior BA, Yarbrough WG. Endoscopic management of retained airgun projectiles in the paranasal sinuses. Otolaryngol Head Neck Surg 2004;130:25–30 6. Donald PJ, Gadre AK. Neuralgia-like symptoms in a patient with an airgun pellet in the ethmoid sinus: a case report. J Laryngol Otol 1995;109:646–649 7. Verhaeghe W, Schepers S, Sun Y, et al. Removal of a low-velocity projectile from the base of the sphenoid sinus using navigation-guided endoscopy. J Craniofac Surg 2012;23:472–476 8. Harris WD. Large sphenoid sinus foreign body. Arch Otolaryngol 1968;88:436–438 9. Feichtinger M, Zemann W, Karcher H. Removal of a pellet from the left orbital cavity by image-guided endoscopic navigation. Int J Oral Maxillofac Surg 2007;36:358–361 10. Strek P, Zagolski O, Skladzien J. Endoscopic removal of air gun pellet in the sphenoid sinus. B ENT 2005;1:205–207 11. Presutti L, Marchioni D, Trani M, et al. Endoscopic removal of ethmoido-sphenoidal foreign body with intracranial extension. Minim Invasive Neurosurg 2006;49:244–246 12. Felisati G, Lozza P, Chiapasco M, et al. Endoscopic removal of an unusual foreign body in the sphenoid sinus: an oral implant. Clin Oral Implants Res 2007;18:776–780 13. Heiland M, Habermann CR, Schmelzle R. Indications and limitations of intraoperative navigation in maxillofacial surgery. J Oral Maxillofac Surg 2004;62:1059–1063 14. Ewers R, Schicho K, Undt G, et al. Basic research and 12 years of clinical experience in computer-assisted navigation technology: a review. Int J Oral Maxillofac Surg 2005;34:1–8 15. Yu H, Wang X, Zhang S, et al. Navigation-guided en bloc resection and defect reconstruction of craniomaxillary bony tumours. Int J Oral Maxillofac Surg 2013;42:1409–1413 16. Li B, Wang X, Zhang S, et al. A new method of surgical navigation for orthognathic surgery: optical tracking guided free-hand repositioning of the maxillomandibular complex. J Craniofac Surg 2014;25:406–411 17. Li B, Zhang L, Sun H, et al. Minimally invasive endoscopic resection of benign tumours of the accessory parotid gland: an updated approach. Br J Oral Maxillofac Surg 2013;51:342–346
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Cholesterol Granuloma of the Orbit Jianhua Yan, PhD, MD, Yu Cai, PhD, MD, Rongjiao Liu, MD, Jing Lin, MD, Jianqun Li, MD Purpose: The aim of this study was to present image findings, clinical and histopathologic features, and surgical management of orbital cholesterol granuloma (CG), a rare orbital entity. Methods: Findings from 2 patients with orbitofrontal CG are presented along with a review of the literature on CG of the orbit. Results: Both patients were 40-year-old men. The common symptoms of this condition in the present cases were proptosis and inferomedial displacement of the globe. Computed tomography scan in patient 1 revealed the presence of a cystic lesion without bone erosion in the superolateral orbital roof. Magnetic resonance imaging in patient 2 revealed a non–contrast-enhancing lesion with moderate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. For both cases, anterior orbitotomy through subbrow incision by drainage and curettage resulted in a curative outcome. No lesion recurrence was observed by 5 years after surgery in 1 case and 3 years in the other. Histopathologic evaluation revealed numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial elements led to the diagnosis of CG. Conclusions: Orbital CG is a rare expansive cystic condition and nearly always occurs in the lateral region of the superior orbital ridge within the frontal diploic space. This condition shows a marked preponderance in middle-aged males. The findings that computed tomography scan did not reveal bone erosion in patient 1, and magnetic resonance imaging examination showed moderate signal intensity, rather than high signal intensity, on T1-weighted images in patient 2 indicated that these represented unusual presentations. Surgical excision has a high success rate with a low incidence of recurrence. Key Words: Cholesterol granuloma, orbit, surgery
C
holesterol granuloma (CG) is a rare expansive cystic condition, resulting from a foreign body reaction around cholesterol crystals. Hemorrhage, cholesterol crystals, inflammatory cells, disordered ventilation, and impaired drainage are decisive pathogenic factors in the causation of CG. While CG has been reported to be present in a variety of locations including the peritoneum, breast, lung, kidney, lymph nodes, and testis, it is most commonly associated with bony structures of the cranium.1 It is particularly common
From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, the People’s Republic of China. Received June 25, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Jianhua Yan, MD, PhD, Zhongshan Ophthalmic Center, Sun Yat-sen University, 54 Xianlie Nan Rd, Guangzhou, 510060, the People’s Republic of China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001347
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Brief Clinical Studies
in the temporal bone (in the middle ear cavity and mastoid air cell complex) but can also occur in the mandible, nasal sinuses, and cranial base.1–3 Orbital involvement by CG is extremely rare and nearly always occurs in the lateral region of the superior orbital ridge within the frontal diploic space. This condition shows a marked preponderance in middle-aged men.2,4 Given the rarity of this condition within the orbital area, the diagnosis and treatment of orbital CG remain to be elucidated. In this report, we describe the clinical, radiologic, and histopathologic features along with the diagnosis and surgical management of 2 male Chinese patients with orbital CG.
CLINICAL REPORT Patient 1 A 40-year-old man presented with left proptosis and an enlarging mass in the superolateral orbital rim with decreasing visual acuity present over the previous 2 years. Diplopia was present for about 6 months, especially when looking upward. He was otherwise in good health, with no history of eye trauma. A 2.5 1.5-cm palpable mass was present in the left superolateral orbit. The best corrected visual acuity was 25/20 in the right eye and 20/40 in the left eye. Anterior segment and fundus examination were normal in both eyes. Exophthalmometer measurements were 14 mm in the right eye and 19 mm in the left eye, with a base of 108 mm. There was no strabismus in the primary position. Upward eye movements of the left eye were moderately limited (Fig. 1A). The intraocular pressure was 16 mm Hg in the right eye and 21 mm Hg in the left eye. Computed tomography (CT) demonstrated a cystic lesion without bone erosion in the left superolateral bony orbit (Fig. 1B). B-mode ultrasonography revealed the presence of a well-defined mass, 25.3 19.6 10.0 mm, with low internal echo within the left superolateral orbit (Fig. 1C). Color Doppler ultrasonography showed no blood flow signal within the mass. An aspiration of the contents and curettage of the lining by an extraperiosteal approach through a subbrow incision were performed while the patient was under local anesthesia. At surgery, a 2.5 1.5 1.0-cm mass with an intact fibrous envelope under periosteal was excised thoroughly, and mucinous, dark green fluid intermingled with shining material was aspirated from the cyst. There was no osseous destruction near the mass. The histopathologic examination showed numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial
FIGURE 2. A, Clinical picture of patient 2, showing upper eyelid swelling and proptosis of the right eye. B and C, Magnetic resonance imaging revealing a 22 25 28-mm oval lesion of moderate T1-signal intensity and heterogeneous high T2-signal intensity at the right lacrimal fossa. D, B-mode ultrasound examination showing a 21 25 27-mm inhomogeneous low-echo mass. E, Postoperative pathology showing some cholesterol crystals, macrophages, a few multinucleated giant cell infiltrations, and tissue fibrosis, confirming the diagnosis of orbital CG.
elements led to the diagnosis of CG. No recurrence of the tumor was evident at 5 years’ follow-up.
Patient 2 A 40-year-old man presented with an 18-month history of upper eyelid swelling and proptosis of the right eye. He did not complain of ocular pain or discomfort. Medical history was negative, and systemic examination was unremarkable. On ophthalmic examination, visual acuity was 20/20 in the right eye and 25/20 in left eye. Intraocular pressure, anterior segment, and fundus were normal in both eyes. A well-defined orbital mass was palpable at the superolateral margin of the right orbit. The mass was 15 20 mm, of medium texture, and without tenderness. A proptosis of 5 mm was present in the right eye, which was pushed downward and moderately limited upward movements (Fig. 2A). Magnetic resonance imaging (MRI) showed a 22 25 28-mm oval lesion of moderate T1-signal intensity (Fig. 1B) and heterogeneous high T2-signal intensity at the right lacrimal fossa (Fig. 2C). B-mode ultrasound examination showed a 21 25 27-mm inhomogeneous lowecho mass, with no blood flow signal on color Doppler (Fig. 2D). An anterior orbitotomy via a subbrow skin incision was performed while the patient was under general anesthesia. The cystic mass consisted of a yellow-gray “wall” and contained a considerable amount of curry sauce-like fluid. The “wall” was adherent to the surrounding bone, but no bone erosion was present. Careful drilling and curettage of the affected bony surface were performed, and the mass completely removed. Postoperative pathology showed some cholesterol crystals, a few multinucleated giant cell infiltrations, and an old regional hemorrhage, which led to a diagnosis of right orbital CG (Fig. 2E). Postoperative recovery was uneventful. No tumor recurrence was observed at 3 years after the surgery, and visual acuity was 25/20 in both eyes.
DISCUSSION
FIGURE 1. A, Clinical picture of patient 1, showing proptosis and downward displacement of the left eye, with moderately limited upward eye movement. B, Computed tomography scan demonstrating a cystic lesion without bone erosion in the left superolateral bony orbit. C, B-mode ultrasonography showing a well-defined heterogeneous mass, 25.3 19.6 10.0 mm,with low internal echo within the left superolateral orbit.
Cholesterol granulomas are bone-destroying and bone-displacing lesions characterized by a foreign body reaction and granulomatous infiltration surrounding cholesterol crystals.1,2 The pathogenesis of CG is poorly understood. The most commonly accepted explanation is the presence of a nonabsorbed hematoma from an episode of bleeding (eg, mucosal bleeding, bleeding from a preexisting bony anomaly, local tissue breakdown, traumatic intradiploic hemorrhage). Hemorrhage leads to the rupture of red blood cell membranes and precipitation of cholesterol crystals, which stimulate substantial cell granulomatous reactions, resulting in the formation of a mass that continues to expand and erode adjacent bone.2,3 Histologically, CG usually contains cholesterol crystals, hemosiderin-laden
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
macrophages, multinucleated giant cells, and tissue fibrosis. Cholesterol granuloma is not associated with epithelial elements, which distinguishes this condition from cholesteatoma.5,6 The patients described here had no history of trauma of the orbit. Cholesterol granuloma of the frontal bone is a rare entity, typically involving the lateral part of the supraorbital ridge in middleaged men.2,3 McNab and Wright5 reported 27 cases of orbitofrontal CG (24 men and 3 women) whose ages ranged from 25 to 68 years (mean, 43.7 years). Right and left orbits were equally affected in these cases. Few reports indicate any recurrence as assessed years after surgery.2 The most common presenting symptom is proptosis and inferomedial displacement of the globe. Other symptoms include periorbital headache, diplopia, decreased vision, ptosis, and eyelid swelling. Computed tomography scan of the orbits typically reveals a cystic well-circumscribed homogeneous soft tissue mass (of the same density as the brain and lacking contrast-medium enhancement) in the superolateral bony orbit. An osteolytic defect, without a sclerotic bony margin, can be seen along with destruction of the outer table of the frontal bone. This slowly expanding lesion may cause gradual erosion of the bone and can eventually involve the orbit, frontal sinus, lacrimal fossa, and brain, showing a very large destructive mass with extensive intracranial and orbital extensions. A few patients had a small lytic lesion in the frontal bone without any associated soft tissue mass.3,7 On MRI, CG is characterized by a non–contrast-enhancing lesion with high signal intensity on both T1- and T2-weighted images.5,7 In the case reports presented here, however, patient 1 did not reveal bone erosion on CT scan, and patient 2 showed moderate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Both the image findings of the CT scan in patient 1 and MRI scan in patient 2 represent unusual presentations for CG. The differential diagnosis of CG includes other homogeneous lesions with bone erosion that occur in the diploic space of the frontal bone, such as lacrimal gland carcinoma, aneurysmal bone cysts, dermoid or epidermoid cysts, cystic ossifying fibroma, eosinophilic granuloma, frontal sinus mucoceles, and metastasis.1,3,5 Dermoids and epidermoids typically do not cause pain and classically have sclerotic bone margins on CT scan. Aneurysmal bone cysts are associated with hemorrhage, granulomatous inflammation, and bone destruction, but they typically occur in young children. A lacrimal gland carcinoma is usually associated with severe pain and irregular bony destruction. Cystic ossifying fibroma typically occurs in young patients and has a distinct sclerotic margin and scattered foci of calcification. Eosinophilic granuloma also usually occurs in young patients and produces an osteolytic lesion with irregular and sclerotic margins. Metastases to the frontal bone are usually painful and cause irregular bone destruction without expansion. Therefore, these typical clinical and radiologic features help to differentiate among most of these conditions to distinguish them from orbital CG prior to any surgical interventions.3 However, few patients with CG can be correctly diagnosed presurgically because of the extremely rare occurrence of this condition. Usually, the presumed diagnosis is made during surgery with the observation of a cyst containing yellowbrown viscous materials, friable yellow solid materials, and porous bone erosion. The final diagnosis in our patients was confirmed by histopathology examination of the submitted tissue. The absence of squamous- or respiratory-type epithelium of the cyst led to the diagnosis of CG. The fluid from the cyst was remarkable for numerous inflammatory cells, blood breakdown products, multinucleate giant cells, and cholesterol crystals. Orbitofrontal CG requires surgical excision. After exposure of the lesion by anterior orbitotomy through subbrow incision, treatment for most of these patients involves aspiration of the contents and curettage of the lining with an extraperiosteal approach.1–5 It is not necessary to resect a wide margin of the surrounding bone.
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Patients with large masses extending into the lateral quadrant of the orbit or anterior cranial fossa may require lateral orbitotomy or frontal craniotomy for better exposure and complete removal of the lesion.2 An endoscopic approach to orbitofrontal CG has been proposed, especially in lesions with intracranial extension.2,8 A 70-degree rigid endoscope approach was used to visualize and remove the granulomaous tissue from the inner surface of frontal bone and dura. As an alternative, a combined anterior orbital and endoscopic approach may be used as opposed to either frontal craniotomy or lateral orbitotomy in selected cases of orbitofrontal CG. Severe cases with great deformity of the skull bone involving cosmetic problems may require reconstruction after complete removal of the lesion. In our cases, the patients were subjected to anterior orbitotomy through subbrow incision. Surgical excision is successful, with a low incidence of recurrence. In order to prevent recurrence, the granulomaous lesion must be removed completely. In summary, orbital CG is a rare entity that has a predilection for men in their fourth decade and usually occurs within the frontal bone overlying the lacrimal fossa. Both ultrasonic examination and CT/MRI of the orbits reveal a cystic lesion that may erode the bony structure including orbital roof, frontal sinus, and cranial base. Computed tomography scan may reveal no bone erosion, and MRI scan may show moderate signal intensity on T1-weighted images. Anterior orbitotomy through subbrow incision by drainage and curettage is curative in all reported cases, and a low incidence of recurrence is observed. Histopathologic analysis reveals numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial elements led to the diagnosis of CG.
REFERENCES 1. Aferzon M, Millman B, O’Donnell TR, et al. Cholesterol granuloma of the frontal bone. Otolaryngol Head Neck Surg 2002;127:578–581 2. Khalatbari MR, Moharamzad Y. Recurrent orbitofrontal cholesterol granuloma in pediatric patient: case report and review of the literature. Childs Nerv Syst 2012;28:291–296 3. Arat YO, Chaudhry IA, Boniuk M. Orbitofrontal cholesterol granuloma. Ophthal Plast Reconstr Surg 2003;19:382–387 4. Chow LP, McNab AA. Orbitofrontal cholesterol granuloma. J Clin Neurosci 2005;12:206–209 5. McNab AA, Wright JE. Orbitofrontal cholesterol granuloma. Ophthalmology 1990;97:28–32 6. Fukuta K, Jackson IT. Epidermoid cyst and cholesterol granuloma of the orbit. Br J Plast Surg 1990;43:521–527 7. Hill CAR, Moseley IF. Imaging of orbitofrontal cholesterol granuloma. Clin Radiol 1992;46:237–242 8. Selva D, Lai T, Krishnan S. Orbitofrontal cholesterol granuloma: percutaneous endoscopic-assisted curettage. J Laryngol Otol 2003;117:892–894
Application of a Computer-Aided Navigation Technique in Surgery for Recurrent Malignant Infratemporal Fossa Tumors Rui Guo, MD, Yu Xing Guo, MD, Zhien Feng, PhD, MD, Chuan Bin Guo, PhD, MD Background: Because of the complexity of the local anatomy, tumors in the infratemporal fossa present a great challenge to oral © 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
CONCLUSIONS We have presented a case where a metallic foreign body has stayed at the temporal surfaces of the sphenoid greater wing posterior to the lateral orbital wall over 20 years, the remains of which have left the patient with chronic sustained pain. Having balanced the potential surgical damage and benefit after surgical removal, we scheduled an operating procedure with the help of an endoscope and a navigation.7,9,17 When the navigation was assisted by the endoscope, extra images from other directions could provide with extensive view; therefore, we could reach the metallic bodies with a minimal damage. Although the metallic foreign body was encapsulated in the connective tissue, we managed to remove the foreign body without cutting extra surrounding tissues. This minimally invasive procedure is a good choice for removing foreign bodies; first, it increases safety, and also it maintains facial aesthetics. The combination of navigation and endoscope system can also be applied to an extensive range of foreign body removals, as well as other kinds of facial surgery.
REFERENCES 1. Liu SY, Cheng WY, Lee HT, et al. Endonasal transsphenoidal endoscopy-assisted removal of a shotgun pellet in the sphenoid sinus: a case report. Surg Neurol 2008;70(suppl 1):S1:56–59 2. Ben-David J, Fradis M, Podoshin L, et al. Plasmacytoma arising in the vicinity of a foreign body in the nasal cavity. Laryngoscope 1981;91:1150–1154 3. Shuker ST. Base-of-skull/infratemporal fossa shell fragment retrieval. J Oral Maxillofac Surg 2010;68:2668–2674 4. Freysinger W, Gunkel AR, Thumfart WF. Image-guided endoscopic ENT surgery. Eur Arch Otorhinolaryngol 1997;254:343–346 5. Brinson GM, Senior BA, Yarbrough WG. Endoscopic management of retained airgun projectiles in the paranasal sinuses. Otolaryngol Head Neck Surg 2004;130:25–30 6. Donald PJ, Gadre AK. Neuralgia-like symptoms in a patient with an airgun pellet in the ethmoid sinus: a case report. J Laryngol Otol 1995;109:646–649 7. Verhaeghe W, Schepers S, Sun Y, et al. Removal of a low-velocity projectile from the base of the sphenoid sinus using navigation-guided endoscopy. J Craniofac Surg 2012;23:472–476 8. Harris WD. Large sphenoid sinus foreign body. Arch Otolaryngol 1968;88:436–438 9. Feichtinger M, Zemann W, Karcher H. Removal of a pellet from the left orbital cavity by image-guided endoscopic navigation. Int J Oral Maxillofac Surg 2007;36:358–361 10. Strek P, Zagolski O, Skladzien J. Endoscopic removal of air gun pellet in the sphenoid sinus. B ENT 2005;1:205–207 11. Presutti L, Marchioni D, Trani M, et al. Endoscopic removal of ethmoido-sphenoidal foreign body with intracranial extension. Minim Invasive Neurosurg 2006;49:244–246 12. Felisati G, Lozza P, Chiapasco M, et al. Endoscopic removal of an unusual foreign body in the sphenoid sinus: an oral implant. Clin Oral Implants Res 2007;18:776–780 13. Heiland M, Habermann CR, Schmelzle R. Indications and limitations of intraoperative navigation in maxillofacial surgery. J Oral Maxillofac Surg 2004;62:1059–1063 14. Ewers R, Schicho K, Undt G, et al. Basic research and 12 years of clinical experience in computer-assisted navigation technology: a review. Int J Oral Maxillofac Surg 2005;34:1–8 15. Yu H, Wang X, Zhang S, et al. Navigation-guided en bloc resection and defect reconstruction of craniomaxillary bony tumours. Int J Oral Maxillofac Surg 2013;42:1409–1413 16. Li B, Wang X, Zhang S, et al. A new method of surgical navigation for orthognathic surgery: optical tracking guided free-hand repositioning of the maxillomandibular complex. J Craniofac Surg 2014;25:406–411 17. Li B, Zhang L, Sun H, et al. Minimally invasive endoscopic resection of benign tumours of the accessory parotid gland: an updated approach. Br J Oral Maxillofac Surg 2013;51:342–346
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Cholesterol Granuloma of the Orbit Jianhua Yan, PhD, MD, Yu Cai, PhD, MD, Rongjiao Liu, MD, Jing Lin, MD, Jianqun Li, MD Purpose: The aim of this study was to present image findings, clinical and histopathologic features, and surgical management of orbital cholesterol granuloma (CG), a rare orbital entity. Methods: Findings from 2 patients with orbitofrontal CG are presented along with a review of the literature on CG of the orbit. Results: Both patients were 40-year-old men. The common symptoms of this condition in the present cases were proptosis and inferomedial displacement of the globe. Computed tomography scan in patient 1 revealed the presence of a cystic lesion without bone erosion in the superolateral orbital roof. Magnetic resonance imaging in patient 2 revealed a non–contrast-enhancing lesion with moderate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. For both cases, anterior orbitotomy through subbrow incision by drainage and curettage resulted in a curative outcome. No lesion recurrence was observed by 5 years after surgery in 1 case and 3 years in the other. Histopathologic evaluation revealed numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial elements led to the diagnosis of CG. Conclusions: Orbital CG is a rare expansive cystic condition and nearly always occurs in the lateral region of the superior orbital ridge within the frontal diploic space. This condition shows a marked preponderance in middle-aged males. The findings that computed tomography scan did not reveal bone erosion in patient 1, and magnetic resonance imaging examination showed moderate signal intensity, rather than high signal intensity, on T1-weighted images in patient 2 indicated that these represented unusual presentations. Surgical excision has a high success rate with a low incidence of recurrence. Key Words: Cholesterol granuloma, orbit, surgery
C
holesterol granuloma (CG) is a rare expansive cystic condition, resulting from a foreign body reaction around cholesterol crystals. Hemorrhage, cholesterol crystals, inflammatory cells, disordered ventilation, and impaired drainage are decisive pathogenic factors in the causation of CG. While CG has been reported to be present in a variety of locations including the peritoneum, breast, lung, kidney, lymph nodes, and testis, it is most commonly associated with bony structures of the cranium.1 It is particularly common
From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, the People’s Republic of China. Received June 25, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Jianhua Yan, MD, PhD, Zhongshan Ophthalmic Center, Sun Yat-sen University, 54 Xianlie Nan Rd, Guangzhou, 510060, the People’s Republic of China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001347
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Brief Clinical Studies
in the temporal bone (in the middle ear cavity and mastoid air cell complex) but can also occur in the mandible, nasal sinuses, and cranial base.1–3 Orbital involvement by CG is extremely rare and nearly always occurs in the lateral region of the superior orbital ridge within the frontal diploic space. This condition shows a marked preponderance in middle-aged men.2,4 Given the rarity of this condition within the orbital area, the diagnosis and treatment of orbital CG remain to be elucidated. In this report, we describe the clinical, radiologic, and histopathologic features along with the diagnosis and surgical management of 2 male Chinese patients with orbital CG.
CLINICAL REPORT Patient 1 A 40-year-old man presented with left proptosis and an enlarging mass in the superolateral orbital rim with decreasing visual acuity present over the previous 2 years. Diplopia was present for about 6 months, especially when looking upward. He was otherwise in good health, with no history of eye trauma. A 2.5 1.5-cm palpable mass was present in the left superolateral orbit. The best corrected visual acuity was 25/20 in the right eye and 20/40 in the left eye. Anterior segment and fundus examination were normal in both eyes. Exophthalmometer measurements were 14 mm in the right eye and 19 mm in the left eye, with a base of 108 mm. There was no strabismus in the primary position. Upward eye movements of the left eye were moderately limited (Fig. 1A). The intraocular pressure was 16 mm Hg in the right eye and 21 mm Hg in the left eye. Computed tomography (CT) demonstrated a cystic lesion without bone erosion in the left superolateral bony orbit (Fig. 1B). B-mode ultrasonography revealed the presence of a well-defined mass, 25.3 19.6 10.0 mm, with low internal echo within the left superolateral orbit (Fig. 1C). Color Doppler ultrasonography showed no blood flow signal within the mass. An aspiration of the contents and curettage of the lining by an extraperiosteal approach through a subbrow incision were performed while the patient was under local anesthesia. At surgery, a 2.5 1.5 1.0-cm mass with an intact fibrous envelope under periosteal was excised thoroughly, and mucinous, dark green fluid intermingled with shining material was aspirated from the cyst. There was no osseous destruction near the mass. The histopathologic examination showed numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial
FIGURE 2. A, Clinical picture of patient 2, showing upper eyelid swelling and proptosis of the right eye. B and C, Magnetic resonance imaging revealing a 22 25 28-mm oval lesion of moderate T1-signal intensity and heterogeneous high T2-signal intensity at the right lacrimal fossa. D, B-mode ultrasound examination showing a 21 25 27-mm inhomogeneous low-echo mass. E, Postoperative pathology showing some cholesterol crystals, macrophages, a few multinucleated giant cell infiltrations, and tissue fibrosis, confirming the diagnosis of orbital CG.
elements led to the diagnosis of CG. No recurrence of the tumor was evident at 5 years’ follow-up.
Patient 2 A 40-year-old man presented with an 18-month history of upper eyelid swelling and proptosis of the right eye. He did not complain of ocular pain or discomfort. Medical history was negative, and systemic examination was unremarkable. On ophthalmic examination, visual acuity was 20/20 in the right eye and 25/20 in left eye. Intraocular pressure, anterior segment, and fundus were normal in both eyes. A well-defined orbital mass was palpable at the superolateral margin of the right orbit. The mass was 15 20 mm, of medium texture, and without tenderness. A proptosis of 5 mm was present in the right eye, which was pushed downward and moderately limited upward movements (Fig. 2A). Magnetic resonance imaging (MRI) showed a 22 25 28-mm oval lesion of moderate T1-signal intensity (Fig. 1B) and heterogeneous high T2-signal intensity at the right lacrimal fossa (Fig. 2C). B-mode ultrasound examination showed a 21 25 27-mm inhomogeneous lowecho mass, with no blood flow signal on color Doppler (Fig. 2D). An anterior orbitotomy via a subbrow skin incision was performed while the patient was under general anesthesia. The cystic mass consisted of a yellow-gray “wall” and contained a considerable amount of curry sauce-like fluid. The “wall” was adherent to the surrounding bone, but no bone erosion was present. Careful drilling and curettage of the affected bony surface were performed, and the mass completely removed. Postoperative pathology showed some cholesterol crystals, a few multinucleated giant cell infiltrations, and an old regional hemorrhage, which led to a diagnosis of right orbital CG (Fig. 2E). Postoperative recovery was uneventful. No tumor recurrence was observed at 3 years after the surgery, and visual acuity was 25/20 in both eyes.
DISCUSSION
FIGURE 1. A, Clinical picture of patient 1, showing proptosis and downward displacement of the left eye, with moderately limited upward eye movement. B, Computed tomography scan demonstrating a cystic lesion without bone erosion in the left superolateral bony orbit. C, B-mode ultrasonography showing a well-defined heterogeneous mass, 25.3 19.6 10.0 mm,with low internal echo within the left superolateral orbit.
Cholesterol granulomas are bone-destroying and bone-displacing lesions characterized by a foreign body reaction and granulomatous infiltration surrounding cholesterol crystals.1,2 The pathogenesis of CG is poorly understood. The most commonly accepted explanation is the presence of a nonabsorbed hematoma from an episode of bleeding (eg, mucosal bleeding, bleeding from a preexisting bony anomaly, local tissue breakdown, traumatic intradiploic hemorrhage). Hemorrhage leads to the rupture of red blood cell membranes and precipitation of cholesterol crystals, which stimulate substantial cell granulomatous reactions, resulting in the formation of a mass that continues to expand and erode adjacent bone.2,3 Histologically, CG usually contains cholesterol crystals, hemosiderin-laden
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e125
Brief Clinical Studies
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
macrophages, multinucleated giant cells, and tissue fibrosis. Cholesterol granuloma is not associated with epithelial elements, which distinguishes this condition from cholesteatoma.5,6 The patients described here had no history of trauma of the orbit. Cholesterol granuloma of the frontal bone is a rare entity, typically involving the lateral part of the supraorbital ridge in middleaged men.2,3 McNab and Wright5 reported 27 cases of orbitofrontal CG (24 men and 3 women) whose ages ranged from 25 to 68 years (mean, 43.7 years). Right and left orbits were equally affected in these cases. Few reports indicate any recurrence as assessed years after surgery.2 The most common presenting symptom is proptosis and inferomedial displacement of the globe. Other symptoms include periorbital headache, diplopia, decreased vision, ptosis, and eyelid swelling. Computed tomography scan of the orbits typically reveals a cystic well-circumscribed homogeneous soft tissue mass (of the same density as the brain and lacking contrast-medium enhancement) in the superolateral bony orbit. An osteolytic defect, without a sclerotic bony margin, can be seen along with destruction of the outer table of the frontal bone. This slowly expanding lesion may cause gradual erosion of the bone and can eventually involve the orbit, frontal sinus, lacrimal fossa, and brain, showing a very large destructive mass with extensive intracranial and orbital extensions. A few patients had a small lytic lesion in the frontal bone without any associated soft tissue mass.3,7 On MRI, CG is characterized by a non–contrast-enhancing lesion with high signal intensity on both T1- and T2-weighted images.5,7 In the case reports presented here, however, patient 1 did not reveal bone erosion on CT scan, and patient 2 showed moderate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Both the image findings of the CT scan in patient 1 and MRI scan in patient 2 represent unusual presentations for CG. The differential diagnosis of CG includes other homogeneous lesions with bone erosion that occur in the diploic space of the frontal bone, such as lacrimal gland carcinoma, aneurysmal bone cysts, dermoid or epidermoid cysts, cystic ossifying fibroma, eosinophilic granuloma, frontal sinus mucoceles, and metastasis.1,3,5 Dermoids and epidermoids typically do not cause pain and classically have sclerotic bone margins on CT scan. Aneurysmal bone cysts are associated with hemorrhage, granulomatous inflammation, and bone destruction, but they typically occur in young children. A lacrimal gland carcinoma is usually associated with severe pain and irregular bony destruction. Cystic ossifying fibroma typically occurs in young patients and has a distinct sclerotic margin and scattered foci of calcification. Eosinophilic granuloma also usually occurs in young patients and produces an osteolytic lesion with irregular and sclerotic margins. Metastases to the frontal bone are usually painful and cause irregular bone destruction without expansion. Therefore, these typical clinical and radiologic features help to differentiate among most of these conditions to distinguish them from orbital CG prior to any surgical interventions.3 However, few patients with CG can be correctly diagnosed presurgically because of the extremely rare occurrence of this condition. Usually, the presumed diagnosis is made during surgery with the observation of a cyst containing yellowbrown viscous materials, friable yellow solid materials, and porous bone erosion. The final diagnosis in our patients was confirmed by histopathology examination of the submitted tissue. The absence of squamous- or respiratory-type epithelium of the cyst led to the diagnosis of CG. The fluid from the cyst was remarkable for numerous inflammatory cells, blood breakdown products, multinucleate giant cells, and cholesterol crystals. Orbitofrontal CG requires surgical excision. After exposure of the lesion by anterior orbitotomy through subbrow incision, treatment for most of these patients involves aspiration of the contents and curettage of the lining with an extraperiosteal approach.1–5 It is not necessary to resect a wide margin of the surrounding bone.
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Patients with large masses extending into the lateral quadrant of the orbit or anterior cranial fossa may require lateral orbitotomy or frontal craniotomy for better exposure and complete removal of the lesion.2 An endoscopic approach to orbitofrontal CG has been proposed, especially in lesions with intracranial extension.2,8 A 70-degree rigid endoscope approach was used to visualize and remove the granulomaous tissue from the inner surface of frontal bone and dura. As an alternative, a combined anterior orbital and endoscopic approach may be used as opposed to either frontal craniotomy or lateral orbitotomy in selected cases of orbitofrontal CG. Severe cases with great deformity of the skull bone involving cosmetic problems may require reconstruction after complete removal of the lesion. In our cases, the patients were subjected to anterior orbitotomy through subbrow incision. Surgical excision is successful, with a low incidence of recurrence. In order to prevent recurrence, the granulomaous lesion must be removed completely. In summary, orbital CG is a rare entity that has a predilection for men in their fourth decade and usually occurs within the frontal bone overlying the lacrimal fossa. Both ultrasonic examination and CT/MRI of the orbits reveal a cystic lesion that may erode the bony structure including orbital roof, frontal sinus, and cranial base. Computed tomography scan may reveal no bone erosion, and MRI scan may show moderate signal intensity on T1-weighted images. Anterior orbitotomy through subbrow incision by drainage and curettage is curative in all reported cases, and a low incidence of recurrence is observed. Histopathologic analysis reveals numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial elements led to the diagnosis of CG.
REFERENCES 1. Aferzon M, Millman B, O’Donnell TR, et al. Cholesterol granuloma of the frontal bone. Otolaryngol Head Neck Surg 2002;127:578–581 2. Khalatbari MR, Moharamzad Y. Recurrent orbitofrontal cholesterol granuloma in pediatric patient: case report and review of the literature. Childs Nerv Syst 2012;28:291–296 3. Arat YO, Chaudhry IA, Boniuk M. Orbitofrontal cholesterol granuloma. Ophthal Plast Reconstr Surg 2003;19:382–387 4. Chow LP, McNab AA. Orbitofrontal cholesterol granuloma. J Clin Neurosci 2005;12:206–209 5. McNab AA, Wright JE. Orbitofrontal cholesterol granuloma. Ophthalmology 1990;97:28–32 6. Fukuta K, Jackson IT. Epidermoid cyst and cholesterol granuloma of the orbit. Br J Plast Surg 1990;43:521–527 7. Hill CAR, Moseley IF. Imaging of orbitofrontal cholesterol granuloma. Clin Radiol 1992;46:237–242 8. Selva D, Lai T, Krishnan S. Orbitofrontal cholesterol granuloma: percutaneous endoscopic-assisted curettage. J Laryngol Otol 2003;117:892–894
Application of a Computer-Aided Navigation Technique in Surgery for Recurrent Malignant Infratemporal Fossa Tumors Rui Guo, MD, Yu Xing Guo, MD, Zhien Feng, PhD, MD, Chuan Bin Guo, PhD, MD Background: Because of the complexity of the local anatomy, tumors in the infratemporal fossa present a great challenge to oral © 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
