Paediatric aneurysmal bone cyst: not as easy as ABC

Paediatric aneurysmal bone cyst: not as easy as ABC Aneurysmal bone cysts (ABCs) are expansile cystic lesions that can affect any bone of the body. Whilst these lesions are his- tologically benign, the lesions are locally aggressive and can affect the integrity of the affected bone as well as surrounding structures. ABCs arising in the head and neck region, particularly the paranasal sinuses are rare and they are limited to case reports in the literature. Due to the proximity of critical anatomical structures and the visual apparatus, the potential com- plications can be devastating. The present article discusses both the clinical and radiological findings of an ABC arising from the ethmoid sinuses in a 6-year-old child and the potentially challenging diagnosis with its complex ensuing surgical man- agement. The identification of an ABC arising in the paranasal sinuses is both a diagnostic and surgical challenge and ideally requires complex management in a joint paediatric ENT and craniofacial unit. reflexes bilaterally but also proptosis-related ophthalmoplegia. INTRODUCTION Computed tomography (CT) imaging of the orbits and paranasal Aneurysmal bone cysts (ABCs) are osteolytic bony neoplasms sinuses demonstrated complete opacification of the right-sided found most commonly in the metaphyses of long bones and paranasal sinuses and compromise of the right frontal and vertebrae but exceptionally rarely in the paranasal sinuses. ostiomeatal unit. In addition there was an expansile abnormal- Although regarded as histologically benign, their locally expan- ity centred on the ethmoidal labyrinth with bony remodelling of sile nature poses a threat to the visual apparatus and brain the lamina papyracea and a subperiosteal collection adjacent to when identified in the paranasal sinuses. The present report the medial orbital wall (Figs 1 and 2). The most likely diagnosis illustrates one such case of a primary ABC in a 6-year-old child was felt to be an infective process with mucopyocele formation and the complex subsequent surgical challenges. complicated by a subperisoteal post-septal collection. An MRI scan with gadolinium of the orbits and sinuses confirmed the unilateral pattern of sinus opacification within the right frontal, CASE REPORT ethmoids and maxillary sinus. The lesion demonstrated multiple A 6-year-old boy with a background of asthma presented to a fluid–fluid levels and peripheral enhancement (Figs 3 and 4). The tertiary paediatric unit in May 2016 with a 3-day history of right patient was treated by endoscopic drainage of the lesion, which eye pain, proptosis and erythema under the care of the ENT revealed only blood. He initially had some improvement of his team. A provisional diagnosis of a periorbital cellulitis was made proptosis but recurred within a few days and so further, more secondary to ethmoid sinusitis and management with intraven- extensive endoscopic debridement was performed. ous antibiotics (ceftriaxone), intranasal steroids, saline douches At this point the MRI was reviewed and the possibility of an and topical oxyxlometazoline was initiated. Ophthalmological ABC was raised. This was due to the presence of fluid–fluid assessment demonstrated normal colour vision, acuity and light levels, cyst within a cyst appearance and lack of restricted Received: December 4, 2017. Accepted: January 12, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 S. Unadkat et al. Figure 3: Axial T2 weighted sequence demonstrating the presence of multiple fluid filled levels (short white arrow) within the expansile lesion and smaller Figure 1: Axial non-contrast CT demonstrating an expansile lesion centred on cyst present within the larger cyst (long white arrow). the right-sided ethmoidal labyrinth (long white arrow) with remodelling of the lamina papyracea (short white arrows). Figure 4: Axial T1 post-contrast sequence with fat suppression demonstrating peripheral enhancement (white arrows) but no internal or solid enhancing components to suggest that this is a secondary ABC. Figure 2: Axial post-contrast CT demonstrating the peripherally enhancing right-sided intra-orbital subperiosteal component. The expansile lesion centred cranial fossa floor that required a bicoronal and transcranial on the right ethmoidal labyrinth demonstrates a faint fluid–fluid level. approach with reconstruction of the medial orbital wall. Currently (November 2017), he remains well, with near total diffusion within the sinonasal contents and material drained at resolution of the proptosis. His visual acuity is normal and the the time of the first endoscopic procedure. He was discharged ophthalmoplegia has resolved. Post-operative imaging has home on oral co-amoxiclav, and topical betamethasone nose demonstrated good clearance and removal of the cyst (Figs 5 drops. and 6). Unfortunately, in June 2016 his symptoms recurred again, with evidence of soft tissue swelling medial to the medial can- DISCUSSION thus. Further imaging (CT and MRI) was requested and he was referred to both a quaternary centre for ENT surgery and a bone Aneursymal bone cysts were first described by Jaffe and tumour multidisciplinary team who agreed that the lesion was Lichstenstein [1] and are well known to affect long bones, spine most likely to represent a primary ABC. In the interim he and pelvis. The term arises from their macroscopic description: remained under close evaluation by the ophthalmologists. a cystic, blood-filled cavity within an expanded (aneurysmal) He underwent a third surgical procedure in July 2016, this bony wall [2]. Though the pathophysiology of these lesions is time a joint external and open approach. The histopathology poorly understood, ABCs tend to affect long bones, spinal verte- confirmed an ABC and his immediate post-operative was brae and pelvis. They account for 1–2% of all primary bone uneventful. His proptosis and ocular motility improved slowly. tumours [3]. Guida et al. [4] report that lesions involving the Regrettably, the ABC recurred again by October 2016 and the skull, comprise 3–6% of all ABCs. Very few have ever been imaging demonstrated substantial erosion of the anterior reported in the paranasal sinuses and are exceptionally rare in Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Paediatric aneurysmal bone cyst 3 appearance due to the presence of small cysts projected from larger cysts as in our case. Ultimately, histological evaluation is key and ABCs typically demonstrates irregular, blood-filled chambers with islands of bone and fibrous tissue [8]. Surgical resection is the treatment of choice though complete clearance is sometimes impossible at the skull base. In such cases, further surgical procedures to debulk the lesion may be needed. For refractory cases in adults, limited success with radiotherapy has been reported in cranial ABCs, albeit with an accepted risk of sarcomatous degeneration [4]. However, as there is a paucity of information for ethmoidal lesions particularly in children, there exists no clear consen- sus for radiotherapy. CONCLUSION ABCs are challenging lesions to manage in the head and neck region. Whilst they may not be truly malignant, the proximity Figure 5: Axial T2 weighted sequence following surgical intervention and med- ial orbital wall reconstruction for the most recent recurrence demonstrating no of complex anatomical structures means that potential disabil- residual abnormality. ity from untreated or inadequately treated lesions can be dev- astating. We propose that ABCs arising within the paranasal sinuses ought to be managed in a highly specialized unit, jointly with both ENT and craniofacial surgery input. CONFLICT OF INTEREST STATEMENT None. LEARNING POINTS What is already known: � Aneurysmal bone cysts (ABCs) are well known entities in long bones, the pelvis and the spine but are extremely rare in the head and neck region. � Surgical management can be curative but there is a high recurrence rate. What the article adds: Figure 6: Axial non-contrast CT at the same level demonstrating the resection of the ABC following multiple surgical procedures and a medial orbital wall � Diagnosis may be delayed due to the uncommon location of reconstruction. the lesion. � Patients ought to be managed in a multidisciplinary fash- ion with craniofacial surgery input for optimum surgical the paediatric population. Most ABCs are considered to management. represent a primary ABC, however, in up to a third of cases, � Treatment of an ABC in the paranasal sinuses is challenging ABCs are secondary to an underlying lesion (e.g. chondroblasto- due to the paucity of literature on consensus management as ma) [2]. well as its proximity to crucial anatomical structures. The diagnosis of an ABC can be challenging and clinical presentation is highly variable, largely dependent on the loca- tion of the ABC. In the present case, the patient presented with FUNDING proptosis and periorbital cellulitis likely due to the expansile This research received no specific grant from any funding nature of the lesion against the lamina papyracea. In the agency, commercial or not-for-profit sectors. review by Hnenny et al. [5], they report that lesions affecting the skull base are more likely to present with neurological defi- cits including anosmia, ataxia, otalgia, facial numbness and REFERENCES hearing loss. Imaging modalities such as CT and MRI are essential to aid 1. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with both diagnosis and for surgical planning. CT features demon- emphasis on the roentgen picture, the pathologic appear- strate the presence of an expansile, lucent bone lesion with ance and the pathogenesis. Arch Surg 1942;44:1004–25. osseous remodelling and cortical thinning [6]. Fluid levels are 2. Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, common and are more readily observed on MRI than CT [7]. controversy, clinical presentation, and imaging. AJR Am J The signal characteristics are also dependent on the age of any Roentgenol 1995;164:573–80. blood products within the lesion. Other MRI features include 3. Leithner A, Windhager R, Lang S, Haas OA, Kainberger F, the presence of multiple internal septations and a ‘soap bubble’ Kotz R. Aneurysmal bone cyst. A population based epidemiologic Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 4 S. Unadkat et al. study and literature review. Clin Orthop Relat Res 1999;363: 6. Hashemi SM, Heidarpour M, Eshaghian A, Ansari P, Hashemi 176–9. MS, Yaghoobi M, et al. A rare case of aneurysmal bone cyst 4. Guida F, Rapanà A, Conti C, Cagliari E, Civelli F, Trincia G. in the paranasal sinus. Iran J Otorhinolaryngol 2015;27:395–9. Cranial aneurysmal bone cyst: a diagnostic problem. With a 7. Senol U, Karaali K, Akyüz M, Gelen T, Tuncer R, Lüleci E. review of the literature. Childs Nerv Syst 2001;17:297–301. Aneurysmal bone cyst of the orbit. AJNR Am J Neuroradiol 5. Hnenny L, Roundy N, Zherebitskiy V, Grafe M, Mansoor A, 2002;23:319–21. Dogan A. Giant aneurysmal bone cyst of the anterior cranial 8. Mankin HJ, Hornicek FJ, Ortiz-Cruz E, Villafuerte J, Gebhardt fossa and paranasal sinuses presenting in pregnancy: case MC. Aneurysmal bone cyst: a review of 150 patients. J Clin report and literature review. J Neurol Surg Rep 2015;76:e216–21. Oncol 2005;23:6756–62. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Paediatric aneurysmal bone cyst: not as easy as ABC

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Abstract

Aneurysmal bone cysts (ABCs) are expansile cystic lesions that can affect any bone of the body. Whilst these lesions are his- tologically benign, the lesions are locally aggressive and can affect the integrity of the affected bone as well as surrounding structures. ABCs arising in the head and neck region, particularly the paranasal sinuses are rare and they are limited to case reports in the literature. Due to the proximity of critical anatomical structures and the visual apparatus, the potential com- plications can be devastating. The present article discusses both the clinical and radiological findings of an ABC arising from the ethmoid sinuses in a 6-year-old child and the potentially challenging diagnosis with its complex ensuing surgical man- agement. The identification of an ABC arising in the paranasal sinuses is both a diagnostic and surgical challenge and ideally requires complex management in a joint paediatric ENT and craniofacial unit. reflexes bilaterally but also proptosis-related ophthalmoplegia. INTRODUCTION Computed tomography (CT) imaging of the orbits and paranasal Aneurysmal bone cysts (ABCs) are osteolytic bony neoplasms sinuses demonstrated complete opacification of the right-sided found most commonly in the metaphyses of long bones and paranasal sinuses and compromise of the right frontal and vertebrae but exceptionally rarely in the paranasal sinuses. ostiomeatal unit. In addition there was an expansile abnormal- Although regarded as histologically benign, their locally expan- ity centred on the ethmoidal labyrinth with bony remodelling of sile nature poses a threat to the visual apparatus and brain the lamina papyracea and a subperiosteal collection adjacent to when identified in the paranasal sinuses. The present report the medial orbital wall (Figs 1 and 2). The most likely diagnosis illustrates one such case of a primary ABC in a 6-year-old child was felt to be an infective process with mucopyocele formation and the complex subsequent surgical challenges. complicated by a subperisoteal post-septal collection. An MRI scan with gadolinium of the orbits and sinuses confirmed the unilateral pattern of sinus opacification within the right frontal, CASE REPORT ethmoids and maxillary sinus. The lesion demonstrated multiple A 6-year-old boy with a background of asthma presented to a fluid–fluid levels and peripheral enhancement (Figs 3 and 4). The tertiary paediatric unit in May 2016 with a 3-day history of right patient was treated by endoscopic drainage of the lesion, which eye pain, proptosis and erythema under the care of the ENT revealed only blood. He initially had some improvement of his team. A provisional diagnosis of a periorbital cellulitis was made proptosis but recurred within a few days and so further, more secondary to ethmoid sinusitis and management with intraven- extensive endoscopic debridement was performed. ous antibiotics (ceftriaxone), intranasal steroids, saline douches At this point the MRI was reviewed and the possibility of an and topical oxyxlometazoline was initiated. Ophthalmological ABC was raised. This was due to the presence of fluid–fluid assessment demonstrated normal colour vision, acuity and light levels, cyst within a cyst appearance and lack of restricted Received: December 4, 2017. Accepted: January 12, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 S. Unadkat et al. Figure 3: Axial T2 weighted sequence demonstrating the presence of multiple fluid filled levels (short white arrow) within the expansile lesion and smaller Figure 1: Axial non-contrast CT demonstrating an expansile lesion centred on cyst present within the larger cyst (long white arrow). the right-sided ethmoidal labyrinth (long white arrow) with remodelling of the lamina papyracea (short white arrows). Figure 4: Axial T1 post-contrast sequence with fat suppression demonstrating peripheral enhancement (white arrows) but no internal or solid enhancing components to suggest that this is a secondary ABC. Figure 2: Axial post-contrast CT demonstrating the peripherally enhancing right-sided intra-orbital subperiosteal component. The expansile lesion centred cranial fossa floor that required a bicoronal and transcranial on the right ethmoidal labyrinth demonstrates a faint fluid–fluid level. approach with reconstruction of the medial orbital wall. Currently (November 2017), he remains well, with near total diffusion within the sinonasal contents and material drained at resolution of the proptosis. His visual acuity is normal and the the time of the first endoscopic procedure. He was discharged ophthalmoplegia has resolved. Post-operative imaging has home on oral co-amoxiclav, and topical betamethasone nose demonstrated good clearance and removal of the cyst (Figs 5 drops. and 6). Unfortunately, in June 2016 his symptoms recurred again, with evidence of soft tissue swelling medial to the medial can- DISCUSSION thus. Further imaging (CT and MRI) was requested and he was referred to both a quaternary centre for ENT surgery and a bone Aneursymal bone cysts were first described by Jaffe and tumour multidisciplinary team who agreed that the lesion was Lichstenstein [1] and are well known to affect long bones, spine most likely to represent a primary ABC. In the interim he and pelvis. The term arises from their macroscopic description: remained under close evaluation by the ophthalmologists. a cystic, blood-filled cavity within an expanded (aneurysmal) He underwent a third surgical procedure in July 2016, this bony wall [2]. Though the pathophysiology of these lesions is time a joint external and open approach. The histopathology poorly understood, ABCs tend to affect long bones, spinal verte- confirmed an ABC and his immediate post-operative was brae and pelvis. They account for 1–2% of all primary bone uneventful. His proptosis and ocular motility improved slowly. tumours [3]. Guida et al. [4] report that lesions involving the Regrettably, the ABC recurred again by October 2016 and the skull, comprise 3–6% of all ABCs. Very few have ever been imaging demonstrated substantial erosion of the anterior reported in the paranasal sinuses and are exceptionally rare in Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Paediatric aneurysmal bone cyst 3 appearance due to the presence of small cysts projected from larger cysts as in our case. Ultimately, histological evaluation is key and ABCs typically demonstrates irregular, blood-filled chambers with islands of bone and fibrous tissue [8]. Surgical resection is the treatment of choice though complete clearance is sometimes impossible at the skull base. In such cases, further surgical procedures to debulk the lesion may be needed. For refractory cases in adults, limited success with radiotherapy has been reported in cranial ABCs, albeit with an accepted risk of sarcomatous degeneration [4]. However, as there is a paucity of information for ethmoidal lesions particularly in children, there exists no clear consen- sus for radiotherapy. CONCLUSION ABCs are challenging lesions to manage in the head and neck region. Whilst they may not be truly malignant, the proximity Figure 5: Axial T2 weighted sequence following surgical intervention and med- ial orbital wall reconstruction for the most recent recurrence demonstrating no of complex anatomical structures means that potential disabil- residual abnormality. ity from untreated or inadequately treated lesions can be dev- astating. We propose that ABCs arising within the paranasal sinuses ought to be managed in a highly specialized unit, jointly with both ENT and craniofacial surgery input. CONFLICT OF INTEREST STATEMENT None. LEARNING POINTS What is already known: � Aneurysmal bone cysts (ABCs) are well known entities in long bones, the pelvis and the spine but are extremely rare in the head and neck region. � Surgical management can be curative but there is a high recurrence rate. What the article adds: Figure 6: Axial non-contrast CT at the same level demonstrating the resection of the ABC following multiple surgical procedures and a medial orbital wall � Diagnosis may be delayed due to the uncommon location of reconstruction. the lesion. � Patients ought to be managed in a multidisciplinary fash- ion with craniofacial surgery input for optimum surgical the paediatric population. Most ABCs are considered to management. represent a primary ABC, however, in up to a third of cases, � Treatment of an ABC in the paranasal sinuses is challenging ABCs are secondary to an underlying lesion (e.g. chondroblasto- due to the paucity of literature on consensus management as ma) [2]. well as its proximity to crucial anatomical structures. The diagnosis of an ABC can be challenging and clinical presentation is highly variable, largely dependent on the loca- tion of the ABC. In the present case, the patient presented with FUNDING proptosis and periorbital cellulitis likely due to the expansile This research received no specific grant from any funding nature of the lesion against the lamina papyracea. In the agency, commercial or not-for-profit sectors. review by Hnenny et al. [5], they report that lesions affecting the skull base are more likely to present with neurological defi- cits including anosmia, ataxia, otalgia, facial numbness and REFERENCES hearing loss. Imaging modalities such as CT and MRI are essential to aid 1. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with both diagnosis and for surgical planning. CT features demon- emphasis on the roentgen picture, the pathologic appear- strate the presence of an expansile, lucent bone lesion with ance and the pathogenesis. Arch Surg 1942;44:1004–25. osseous remodelling and cortical thinning [6]. Fluid levels are 2. Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, common and are more readily observed on MRI than CT [7]. controversy, clinical presentation, and imaging. AJR Am J The signal characteristics are also dependent on the age of any Roentgenol 1995;164:573–80. blood products within the lesion. Other MRI features include 3. Leithner A, Windhager R, Lang S, Haas OA, Kainberger F, the presence of multiple internal septations and a ‘soap bubble’ Kotz R. Aneurysmal bone cyst. A population based epidemiologic Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018 4 S. Unadkat et al. study and literature review. Clin Orthop Relat Res 1999;363: 6. Hashemi SM, Heidarpour M, Eshaghian A, Ansari P, Hashemi 176–9. MS, Yaghoobi M, et al. A rare case of aneurysmal bone cyst 4. Guida F, Rapanà A, Conti C, Cagliari E, Civelli F, Trincia G. in the paranasal sinus. Iran J Otorhinolaryngol 2015;27:395–9. Cranial aneurysmal bone cyst: a diagnostic problem. With a 7. Senol U, Karaali K, Akyüz M, Gelen T, Tuncer R, Lüleci E. review of the literature. Childs Nerv Syst 2001;17:297–301. Aneurysmal bone cyst of the orbit. AJNR Am J Neuroradiol 5. Hnenny L, Roundy N, Zherebitskiy V, Grafe M, Mansoor A, 2002;23:319–21. Dogan A. Giant aneurysmal bone cyst of the anterior cranial 8. Mankin HJ, Hornicek FJ, Ortiz-Cruz E, Villafuerte J, Gebhardt fossa and paranasal sinuses presenting in pregnancy: case MC. Aneurysmal bone cyst: a review of 150 patients. J Clin report and literature review. J Neurol Surg Rep 2015;76:e216–21. Oncol 2005;23:6756–62. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy008/4835616 by Ed 'DeepDyve' Gillespie user on 16 March 2018

Journal

Journal of Surgical Case ReportsOxford University Press

Published: Feb 1, 2018

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