[Endoscopic transnasal surgery for giant fibrous dysplasia of the skull base, spreading to the right orbital cavity and nasopharynx (a case report and literature review)].

BACKGROUND An extended endoscopic endonasal approach is increasingly used in surgical treatment of space-occupying skull base lesions. The international literature reports only 20 cases of surgical treatment for fibrous dysplasia (PD) of the skull base using the endoscopic endonasal approach. We present our experience with the endoscopic endonasal approach in surgical treatment for giant fibrous dysplasia of the skull base, spreading to the right orbital cavity and nasopharynx. CLINICAL CASE A 26-year-old male patient presented with cranial pain, Vth nerve dysfunction on the right, right keratopathy. OD=0.2 (near acuity - 0.3), OS=1.0, OD - incomplete eyelid closure of 2 mm, conjunctival injection, mucous discharge, corneal opacity in the lower pole and paracentrally, OS - normal appearance. Severe right-sided exophthalmos (more than 15 mm), impaired nasal breathing on the right, nasal (hemorrhagic) discharge. Magnetic resonance imaging and spiral computed tomography scans revealed a bone density lesion located in the area of the orbit, nasal cavity, maxillary sinus on the right, and labyrinth of the ethmoid bone. The patient underwent endonasal endoscopic resection of the lesion. RESULTS The lesion was resected totally, which was confirmed by control SCT. Right-sided exophthalmos partially regressed (on the right: exophthalmos of 8 mm; protrusion: OD=23 mm, OS=15 mm; the eyeball was displaced downward and outward). The visual and oculomotor functions did not change. The neurological status remained at the preoperative level. CONCLUSION Fibrous dysplasia of the skull base is an extremely rare disease. Modern techniques expand the indications for surgery of giant tumors of the skull base using minimally invasive approaches, in particular the endoscopic endonasal approach.

[1]  L. Doyle,et al.  Refinements in Sarcoma Classification in the Current 2013 World Health Organization Classification of Tumours of Soft Tissue and Bone. , 2016, Surgical oncology clinics of North America.

[2]  А С Артюшкевич,et al.  Клиника, диагностика и лечение невропатий лицевого нерва , 2015 .

[3]  A. Stamm,et al.  The endoscopic endonasal approach for extradural and intradural clivus lesions. , 2014, World neurosurgery.

[4]  Ignacio Mínguez-Martínez,et al.  Management of fibro-osseous lesions of the craniofacial area. Presentation of 19 cases and review of the literature , 2013, Medicina oral, patologia oral y cirugia bucal.

[5]  M. Farhadi,et al.  Gigantism Treated by Pure Endoscopic Endonasal Approach in a Case of McCune-Albright Syndrome with Sphenoid Fibrous Dysplasia: A Case Report , 2013, Journal of Neurological Surgery—Part A.

[6]  C. Fletcher,et al.  WHO classification of tumours of soft tissue and bone , 2013 .

[7]  F. Más-Estellés,et al.  [Transpterygoid expanded endonasal approach in fibrous dysplasia of the skull base: case report and technical note]. , 2012, Neurocirugia.

[8]  S. Ceylan,et al.  Endoscopic transphenoidal approach for fibrous dysplasia of clivus, tuberculum sellae and sphenoid sinus; report of three cases. , 2012, Turkish neurosurgery.

[9]  M. Amit,et al.  Fibrous dysplasia of the sphenoid and skull base. , 2011, Otolaryngologic clinics of North America.

[10]  S. Yin,et al.  [Surgical management of sinonasal and adjacent skull base benign fibro-osseous lesions]. , 2011, Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology, head, and neck surgery.

[11]  J. Landeiro,et al.  Monostotic fibrous dysplasia of the clivus: image-guided endoscopic endonasal transsphenoidal surgery. , 2010, Minimally invasive neurosurgery : MIN.

[12]  Rijuneeta,et al.  Fibrous Dysplasia of Skull Base: Is There a Role of Endonasal Endoscopic Approach? , 2010 .

[13]  M. Atalar,et al.  Monostotic fibrous dysplasia of the clivus: imaging findings. , 2010, Turkish neurosurgery.

[14]  N. Wood,et al.  The nature of fibrous dysplasia , 2009, Head & face medicine.

[15]  Qi Liu,et al.  Surgical treatment of skull fibrous dysplasia. , 2009, Surgical neurology.

[16]  G. Wang,et al.  [Endoscopic surgery in occupation lesions of the skull base]. , 2007, Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery.

[17]  R. Chapurlat Medical Therapy in Adults With Fibrous Dysplasia of Bone , 2006, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

[18]  P. Dabirmoghaddam,et al.  Endoscopic endonasal surgery for resection of benign sinonasal tumors: experience with 105 patients. , 2006, Archives of Iranian medicine.

[19]  P. Dabirmoghaddam,et al.  Endoscopic Endonasal Surgery for Resection of Benign Sinunasal Tumors , 2004 .

[20]  J. Casselman,et al.  MRI in craniofacial fibrous dysplasia , 2004, Neuroradiology.

[21]  J. DelGaudio,et al.  Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. , 2003, American journal of otolaryngology.

[22]  C. Duran,et al.  Monostotic fibrous dysplasia of the clivus: MRI and CT findings , 2002, Neuroradiology.

[23]  E. McCarthy,et al.  Fibrous dysplasia involving the skull base and temporal bone. , 2001, Archives of otolaryngology--head & neck surgery.

[24]  J. Devogelaer Treatment of bone diseases with bisphosphonates, excluding osteoporosis , 2000, Current opinion in rheumatology.

[25]  J. Tehranzadeh,et al.  Computed tomography of Paget disease of the skull versus fibrous dysplasia , 1998, Skeletal Radiology.

[26]  Pietro Ruggieri,et al.  Malignancies in fibrous dysplasia , 1994, Cancer.

[27]  P. Parizel,et al.  CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone. , 1990, European journal of radiology.

[28]  H. Sissons,et al.  Malignant transformation of fibrous dysplasia. A case report and review of the literature. , 1988, Clinical orthopaedics and related research.

[29]  A. Huvos,et al.  Bone sarcomas arising in fibrous dysplasia. , 1972, The Journal of bone and joint surgery. American volume.

[30]  J. Sassin,et al.  Neurological complications of fibrous dysplasia of the skull. , 1968, Archives of neurology.

[31]  D. T. Schwartz,et al.  THE MALIGNANT TRANSFORMATION OF FIBROUS DYSPLASIA , 1964, The American journal of the medical sciences.