Abstract
Objective: The current management of fibrous dysplasia (FD)
involving the paranasal sinuses and the adjacent skull base is
controversial. This study is to present our experience in the management
strategy of FD involving the paranasal sinuses and the adjacent skull
base.
Design/setting: 23 patients from 2006 to 2019 with monostotic
fibrous dysplasia (MFD), polyostotic fibrous
dysplasia (PFD), or
McCune-Albright Syndrome (MAS) involving the paranasal sinuses and the
adjacent skull base were retrospectively reviewed. This study series was
divided into 3 groups based on the management strategies: the
observation group, the surgery group, and the optic nerve decompression
group.
Main outcome/results: The observation group included 9
asymptomatic MFD patients with a stable condition during the follow-up
period of 15 to 164 months. The surgery group included 10 symptomatic
MFD patients having personalized endoscopic endonasal surgery. The
patients’ symptoms were relieved after surgery. The optic nerve
decompression group included 4 patients with visual loss, who underwent
endonasal endoscopic optic nerve decompression with the aid of
image-guided navigation. Their vision improved after surgery.
Conclusion: The clinical
observation and periodic computed tomography (CT) scan is adopted for
asymptomatic patients. Surgery is indicated in symptomatic patients.
Optic nerve decompression is recommended as soon as possible if the
patient has visual loss, whereas prophylactic decompression is not
recommended if the optic nerve is encroached upon by FD without visual
loss. Navigation plays an important role in endoscopic surgery involving
the paranasal sinuses and the adjacent skull base, especially in FD
resection and optic nerve decompression.
Keywords: fibrous dysplasia (FD), paranasal sinus, skull base,
functional endoscopic sinus surgery (FESS), navigation-assisted surgery
Key Points :The clinical observation is adopted for
asymptomatic patients. Surgery is indicated in symptomatic patients.
Navigation plays an important role in endoscopic surgery in FD resection
and optic nerve decompression.
Introduction
Fibrous dysplasia (FD) of bone, also referred to as fibrous hyperplasia
of bone, is a group of non-neoplastic diseases with
fibro-osseous
bone lesions. FD is a developmental disorder induced by abnormal
proliferation of fibroblasts resulting in replacement of normal
cancellous bone by structurally immature osseous tissue1-3. FD may involve any bone of the body and affect
one or more bones. It is divided into three types: monostotic FD (MFD),
polyostotic FD (PFD), and McCune-Albright syndrome (MAS). When the
lesion involves the
craniofacial
skeleton, the skull base is the most common site of involvement1-3.
Because of the low incidence rate,
diverse natural history and complicated surgical procedure, at present,
the management of FD involving the paranasal sinuses and the adjacent
skull base is controversial. Therefore, many scholars have called for
development of a global consensus on improving patient management4-7. In this study, we showed our experience and
treatment strategies for the management of 23 FD cases which involved
the paranasal sinuses and the adjacent skull base.
Material and Methods