Post-traumatic cerebrospinal fluid rhinorrhea associated with craniofacial fibrous dysplasia: Case report and literature review
Peng Li1, Qiangyi Zhou1, Zhijun Yang1, Zhenmin Wang1, Shiwei Li1, Xingchao Wang1, Bo Wang1, Fu Zhao2, Pinan Liu1,2
1 Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China;
2 Department of Neural Reconstruction, Beijing Neurosurgery Institute, Capital Medical University, Beijing 100050, China
Post-traumatic cerebrospinal fluid rhinorrhea associated with craniofacial fibrous dysplasia: Case report and literature review
Peng Li1, Qiangyi Zhou1, Zhijun Yang1, Zhenmin Wang1, Shiwei Li1, Xingchao Wang1, Bo Wang1, Fu Zhao2, Pinan Liu1,2
1 Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China;
2 Department of Neural Reconstruction, Beijing Neurosurgery Institute, Capital Medical University, Beijing 100050, China
摘要 Objective: Fibrous dysplasia (FD) is an unusual developmental abnormality of the skeleton. When facial and cranial bones are involved in FD, it is termed craniofacial fibrous dysplasia (CFD). Although several reports have reported that CFD has a tendency for spontaneous cerebrospinal fluid (CSF) leakage, there have been no related English-language case reports. We present the first case of post-traumatic CSF rhinorrhea associated with CFD. Methods: A 30-year-old man presented with CSF rhinorrhea after a mild head trauma. Computed tomography cisternogram located a defect in the posterior wall of the right frontal sinus. Imaging examination also showed the evident expansion of multiple skull bones, spinal scoliosis, and multiple local enlargements of ribs. Without café-au-lait cutaneous spots and endocrine abnormalities, polyostotic FD was diagnosed instead of McCune-Albright syndrome (MAS). The patient underwent craniotomy fistula repair surgery. The excised bone was contoured to be thinner to increase the cranial cavity. The patient recovered well and CSF leakage did not recur. But during a nineteen-month follow up, sight in the patient's left eye was decreased. MAS was suspected. Unfortunately the patient refused to take the proposed decompression surgery and laboratory tests of serum hormones. Conclusions: CFD, if the wall of the paranasal sinus is involved and the cranial cavity is decreased, may increase the risk of CSF rhinorrhea after head trauma. Expectant management is recommended in asymptomatic CFD patients even in the presence of optic nerve compression. As MAS may cause more problems, it should be precluded before polyostotic FD is diagnosed.
Abstract: Objective: Fibrous dysplasia (FD) is an unusual developmental abnormality of the skeleton. When facial and cranial bones are involved in FD, it is termed craniofacial fibrous dysplasia (CFD). Although several reports have reported that CFD has a tendency for spontaneous cerebrospinal fluid (CSF) leakage, there have been no related English-language case reports. We present the first case of post-traumatic CSF rhinorrhea associated with CFD. Methods: A 30-year-old man presented with CSF rhinorrhea after a mild head trauma. Computed tomography cisternogram located a defect in the posterior wall of the right frontal sinus. Imaging examination also showed the evident expansion of multiple skull bones, spinal scoliosis, and multiple local enlargements of ribs. Without café-au-lait cutaneous spots and endocrine abnormalities, polyostotic FD was diagnosed instead of McCune-Albright syndrome (MAS). The patient underwent craniotomy fistula repair surgery. The excised bone was contoured to be thinner to increase the cranial cavity. The patient recovered well and CSF leakage did not recur. But during a nineteen-month follow up, sight in the patient's left eye was decreased. MAS was suspected. Unfortunately the patient refused to take the proposed decompression surgery and laboratory tests of serum hormones. Conclusions: CFD, if the wall of the paranasal sinus is involved and the cranial cavity is decreased, may increase the risk of CSF rhinorrhea after head trauma. Expectant management is recommended in asymptomatic CFD patients even in the presence of optic nerve compression. As MAS may cause more problems, it should be precluded before polyostotic FD is diagnosed.
20161117200932 Figure 1 (a, b) A computed tomography (CT) scan demonstrating the expansion of multiple skull bones. The involved bones present with various appearances of mixed radio-dense/radio-lucent lesions, which can be described as “ground-glass”, “lytic”, and “cystic”. Left: Axial CT cisternography demonstrates a fistula at the posterior wall of the right frontal sinus (white arrow). The density of both frontal involved bones is slightly lower than for other skull bones. Right: A coronal CT scan demonstrates pneumocephalus in the right frontal lobe (black arrow).
20161117200955 Figure 2 X-ray chest analysis demonstrating scoliosis (black arrow) and multiple local enlargements of the ribs (white arrow).
20161117201031 Figure 3 A CT scan demonstrating a contoured bone and enlarged cranial cavity. (a, b) Before the fistula repair surgery. (c, d) After the fistula repair surgery.
20161117201119 Figure 4 Axial MRI demonstrating abnormal thickening of the bones in a patient with FD. (a) T2-weighted images show the involved skull bones are hypointense. (b, c) T1-weighted images show the involved skull bones are hypointense (b) with varying enhancement (c). Left frontal bone and the wall of the right frontal sinus showed evident enhancement (white arrows) while the left posterior skull bones did not (black arrows).
20161117201145 Figure 5 (a, b) CT showing the left optic canal before and nineteen months after surgery (arrows). The compression of the left optic nerve was more severe at nineteen months after surgery (right) than before surgery (left).
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2016, Vol. 2 
