Otolaryngology Case Reports 13 (2019) 100131
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Reconstructive septorhinoplasty in radiation-induced reactive bone dysplasia
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Nkechi N. Nwabuezea,∗, Justin R. Shinnb, Scott Owenc,d, W. Russell Riesb,e, Scott J. Stephanb,e a
Vanderbilt University School of Medicine, Nashville, TN, USA Department of Otolaryngology, Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, TN, USA c Department of Otolaryngology, Head and Neck Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA d Division of Facial Plastics and Reconstructive Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA e Division of Facial Plastics and Reconstructive Surgery, Vanderbilt University Medical Center, Nashville, TN, USA b
A R T I C LE I N FO
A B S T R A C T
Justin Shinn, Scott Owen, and Scott Stephan. “Reconstructive Septorhinoplasty in RadiationInduced Reactive Bone Dysplasia.” Combined Otolaryngology Spring Meeting. Poster presentation. San Diego, California. April 2017.
Objective: To describe a complicated external septorhinoplasty in a case of nasal adenocarcinoma radiationinduced reactive bone dysplasia. Case description: We describe a 40-year-old female with high-grade sinonasal adenocarcinoma who was treated with induction chemotherapy and radiation. The patient eventually presented with findings consistent with radiation-induced osseous changes that mimicked fibrous dysplasia. She was successfully treated with external septorhinoplasty for restoration of function and improved cosmesis. To our knowledge, this is one of few reported cases of craniofacial fibrous dysplasia caused by radiation therapy. Conclusions: Fibrous dysplasia is a disease that can lead to significant debilitation in patients, however radiationinduced fibrous dysplasia is a rarely reported disease with no clear consensus guidelines. Select patients can be treated surgically to provide symptomatic relief and improved cosmesis.
Keywords: Septorhinoplasty Fibrodysplasia Fibrous dysplasia Saddle nose
Introduction Fibrous dysplasia (FD) is a non-neoplastic condition most often presenting in early life with hamartomatous aberrant formation of immature bony and fibrous remodeling [1,2]. FD is categorized as monostotic and polyostotic based on the involved bones, however, craniofacial FD is considered unique in that it is often isolated to the face and skull while involving contiguous bony regions [2]. In craniofacial FD, patients most often present with painless and slowly expanding deformities. However, the bony involvement can lead to mass effect and nerve impingement depending on the bones involved. Patients frequently present complaints related to this process such as visual changes, proptosis, nasal obstruction, epiphora, hearing loss and facial nerve dysfunction. Surgical intervention is typically indicated when symptoms arise, and is indicated for slowing progression, restoration of function, and improvement of cosmesis [2]. FD most commonly affects children and young adults and occurs in approximately one to two per 30,000 people [1]. In the head and neck region, FD most often effects the skull at the skull base [3,4]; which varies in clinical behavior and progression, however, there are limited established clinical guidelines to help guide operative indications.
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Computed tomography (CT) findings are distinctive and can confirm the diagnosis of FD. These typically show a thin bony cortex and ground glass hyperostotic remodeling [2,3,5]. Similarly, radiation can cause changes with comparable imaging characteristics that appear identical to the classical FD findings on imaging. We describe a case of radiationinduced fibrodysplasia, with typical imaging findings, isolated to the nasal cavity and nasal pyramid that required complex functional septorhinoplasty for the restoration of nasal breathing. Case report A 40 year-old-female with high-grade sinonasal adenocarcinoma treated with induction chemotherapy followed by concurrent chemoradiation in 2016, presented with bilateral nasal obstruction and saddlenose deformity. Figs. 1 and 2 show her pre-operative facial analysis and imaging findings, respectively. She elected to undergo functional external septorhinoplasty for reconstruction of her bony nasal pyramid and restoration of nasal airflow. During her operative case, she was noted to have thickened, reactive, and brittle bone of the entire naso-orbital-ethmoid region and perpendicular plate of the ethmoid and vomer, weakened caudal and
Corresponding author. Vanderbilt University School of Medicine, 303 Light Hall, Nashville, TN, 37232-8605, USA. E-mail address:
[email protected] (N.N. Nwabueze).
https://doi.org/10.1016/j.xocr.2019.100131 Received 18 May 2019; Accepted 24 October 2019 Available online 31 October 2019 2468-5488/ © 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Otolaryngology Case Reports 13 (2019) 100131
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Fig. 1. Preoperative Photos displaying frontal, inferior, and lateral views of the patient's saddle nose deformity.
Fig. 2. (A) Prior to chemoradiation treatment; (B) After chemoradiation treatment showing relative thickening of the naso-orbital-ethmoid area following radiation therapy.
dorsal cartilaginous septal struts with saddle nose deformity, and a severely compromised keystone attachment. Using an otology drill, a #5 cutting burr was used to remove excess reactive bone on the
external surface of the pyramid with subsequent placement of bilateral spreader grafts tailored from cadaveric rib cartilage that were secured through bone holes to the bony pyramid to reconstruct the keystone. A 2
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Fig. 3. Intraoperative Photos: Left: View of nasal bones after supraperichondrial elevation; Center: After drilling nasal pyramid with 5 mm otologic drill for contour; Right: After middle vault reconstruction with extended spreader graft placement.
reactive osseous dysplasia. Radiation is an effective and recommended treatment for many diseases, both benign and malignant, but is known to subsequently trigger additional hypertrophic and even malignant processes. One such well-known example is that of radiation induced craniofacial osteosarcoma [8,9]. Many cases are due to the treatment of pre-existing benign conditions. Radiation leads to genomic instability and damage to intracellular repair mechanisms, however, cells at the periphery of the radiation field may become violatile [10]. Similarly, and perhaps as a clue to the etiology of nonhereditary FD, chronic inflammation and inflammatory regulators have been shown to be involved in osteosarcomagenesis [11]. It is reasonable, that similar to radiation-induced osteosarcoma, genomic instability from radiation leads to hamartomatous formation of immature bone and fibrosis as is seen in FD. While radiation is known to increase the likelihood of malignant transformation of FD into oseteosarcoma, fibrosarcoma, and chondrosarcoma [12–15], particularly in polyostotic disease, there is little information regarding the impact of radiation therapy leading to fibrotic and immature bony remodeling. Clinical guidelines are also unclear. In the case of radiation-induced osteosarcoma, there are clear guidelines for diagnosis, namely the Cahan criteria which were later modified by Arlen et al. [16,17] While there have been criteria for other radiation-induced disease; there is no
caudal septal extension graft was then placed to support a short and weak caudal septum and lateral osteotomies were performed to narrow the excessive external width of the upper nasal dorsum. Finally, cadaveric rib cartilage was used for a dorsal onlay graft and supratip cap graft. Fig. 3 illustrates intraoperative details. Final pathology revealed reactive bone formation and atypical stromal cells. Post-operatively, the patient had to undergo endoscopic nasal debridement for nasal crusting and developed left sided periorbital pain, post nasal drip, and dizziness exacerbated by movement, all of which eventually subsided without further intervention. Fig. 4 shows the patient six weeks following her surgery. Discussion FD is a complicated disease that can lead to significant functional debilitation. While the literature extensively describes genetic and familial causes [6,7], less knowledge exists on etiologies due to secondary insults, such as post-radiation changes that mimic FD. Although these secondary changes are not well described within the literature, the imaging characteristics and clinical presentation closely resemble hereditary craniofacial FD. We report a unique case of fibrous dysplasia of the nasal bones and septum that required detailed sculpting of hypertrophic and dysplastic nasal bones in a patient with radiation-induced
Fig. 4. Postoperative photos following septorhinoplasty showing inferior, frontal, and lateral views. There was significant improvement of patient's saddle nose deformity as well as a decrease in nasal obstruction. 3
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Declaration of competing interest
similar clear consensus for radiation-induced FD. Operative guidelines also do not exist. The major goals of treatment include prevention of pathologic fractures, pain control, and reduction of deformities [2]. Treatment includes observation, medical therapy, surgical remodeling, and radical excision and reconstruction. Medical treatment most commonly employs bisphosphonates to reduce osteoabsorption. Additionally, Tocilizumab, an IL-6 inhibitor, is being evaluated as a possible treatment choice [18]. However, surgery remains the mainstay of treatment and is aimed at controlling progression of symptoms such as proptosis, orbital dystopia, vision changes, and hearing loss [2]. In general, surgical treatment of craniofacial FD is based on four zones of involvement, but is left to the surgeon's discretion [19]. Zone one, the most appropriate category for our patient, involves the frontoorbito-malar region and radical excision and reconstruction is usually recommended. Zone two includes the hair-bearing scalp, and intervention is optional. Zone three is the central skull base, which encompasses the mastoid, temporal bone, pterygoid, and the sphenoid. Observation is typically recommended for these lesions. Zone four contains the maxilla and the mandible, and conservative management is recommended [2,19]. Typically, patients with complete excision experiences no recurrence of disease [20]. Our patient presented with changes consistent with FD following chemoradiation for adenocarcinoma. Her changes were relatively confined to the fronto-orbito-malar region and she subsequently received an appropriate, though complex, functional external septorhinoplasty. While the patient did have some side effects, these eventually spontaneously resolved and we were able to provide her with long-term functional relief, improved cosmesis, and relief of her nasal obstruction. In this case report we are limited by the relatively few cases of craniofacial fibrous dysplasia and the even fewer cases of craniofacial radiation-induced reactive bone dysplasia and results should be thought of accordingly.
None. References [1] Frisch CD, Carlson ML, Kahue CN, et al. Fibrous dysplasia of the temporal bone: a review of 66 cases. The Laryngoscope 2015. https://doi.org/10.1002/lary.25078. [2] Menon S, Venkatswamy S, Ramu V, Banu K, Ehtaih S, Kashyap V. Craniofacial fibrous dysplasia: surgery and literature review. Ann Maxillofac Surg 2013. https:// doi.org/10.4103/2231-0746.110088. [3] Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001. https://doi. org/10.1001/archotol.127.10.1239. [4] Perera AP, Mehta GU, Pratt D, Quezado MM, Gilbert MR, Heiss JD. Diagnosis of a growing radiation-induced skull lesion in a patient: an unusual scar. doi:10.3171/ 2015.7.JNS15989. [5] Lee JS, Fitzgibbon EJ, Chen YR, et al. Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis 2012. https://doi.org/10.1186/ 1750-1172-7-S1-S2. [6] Rahman AMA, Madge SN, Billing K, et al. Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye 2009. https://doi.org/10.1038/eye. 2009.6. [7] Weinstein LS. Gsα Mutations in fibrous dysplasia and McCune-Albright syndrome. J Bone Miner Res 2006. https://doi.org/10.1359/jbmr.06s223. [8] Valentí V, López-Pousa A, Gonzalez Y, Farré N. Radiation-induced mandibular osteogenic sarcoma: report of a case and review of the literature. J Craniofac Surg 2005;16(3)https://journals.lww.com/jcraniofacialsurgery/Fulltext/2005/05000/ Radiation_Induced_Mandibular_Osteogenic_Sarcoma_.23.aspx. [9] Liao LQ, Yan HH, Mai JH, et al. Radiation-induced osteosarcoma of the maxilla and mandible after radiotherapy for nasopharyngeal carcinoma. Chin J Canc 2016. https://doi.org/10.1186/s40880-016-0153-8. [10] Chan L-L, Czerniak BA, Ginsberg LE. Radiation-induced osteosarcoma after bilateral childhood retinoblastoma. Am J Roentgenol 2000;174(5):1288. https://doi.org/10. 2214/ajr.174.5.1741288. [11] Radons J. The role of inflammation in sarcoma. In: Aggarwal Bharat B, Sung B, GSCeditors. Inflammation and cancer Basel: Springer Basel; 2014. p. 259–313. https://doi.org/10.1007/978-3-0348-0837-8_11. [12] Hansen MR, Moffat JC. Osteosarcoma of the skull base after radiation therapy in a patient with McCune-albright syndrome: case report vol 13. 2003. [13] Qu N, Yao W, Cui X, Zhang H. Malignant transformation in monostotic fibrous dysplasia: clinical features, imaging features, outcomes in 10 patients, and review. 2015. https://doi.org/10.1097/MD.0000000000000369. Med (United States). [14] Mardekian SK, Tuluc M. Malignant sarcomatous transformation of fibrous dysplasia. Head Neck Pathol 2015. https://doi.org/10.1007/s12105-014-0567-z. [15] Ruggieri P, Sim FH, Bond JR, et al. Malignancies in fibrous dysplasia. Cancer 1994;73:1411–24. [16] Cahan WG, Woodward H. Sarcoma arising in irradiated bone; report of 11 cases. Cancer 1948;1(1):3–29. [17] Arlen M, Higinbotham NL, Huvos AG, Marcove RC, Miller T, Shah IC. Radiationinduced sarcoma of bone. Cancer 1971;28(5):1087–99. [18] de Boysson H, Johnson A, Hablani N, Hajlaoui W, Auzary C, Geffray L. Tocilizumab in the treatment of a polyostotic variant of fibrous dysplasia of bone. Rheumatol (United Kingdom); 2015. https://doi.org/10.1093/rheumatology/kev221. [19] Chen YR1 NM. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1990;86(5):835–42. [20] Valentini V, Cassoni A, Marianetti TM, Terenzi V, Fadda MT, Iannetti G. Craniomaxillofacial fibrous dysplasia: conservative treatment or radical surgery? a retrospective study on 68 patients. Plast Reconstr Surg 2009;123(2):653–60. https://doi.org/10.1097/PRS.0b013e318196bbbe.
Conclusion Non-hereditary fibrous dysplasia of the head and neck is rarely reported within the literature and consensus guidelines for operative intervention do not currently exist. Therefore, clinicians must intervene based on clinical expertise and patient symptoms. Fibrous dysplasia can result after secondary insults, such as radiation therapy, and involve the skull base or nasal cavity. In select patients, this can have functional and cosmetic outcomes, yet it can be treated surgically to relieve symptoms and provide a superior cosmetic outcome. Funding None.
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