Peng Li, Qiangyi Zhou, Zhijun Yang, Zhenmin Wang, Shiwei Li, Xingchao Wang, Bo Wang, Fu Zhao, Pinan Liu,

1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China

2Department 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

1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China

2Department of Neural Reconstruction, Beijing Neurosurgery Institute, Capital Medical University, Beijing 100050, China

ARTICLE INFO

Received: 20 July 2016

Revised: 20 July 2016

Accepted: 15 August 2016

? The authors 2016. This article is published with open access at www.TNCjournal.com

craniofacial fibrous dysplasia;

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.

1 Introduction

Fibrous dysplasia (FD), with an incidence of 1:4000–1:10,000, is an unusual developmental abnormality of the skeleton. Four clinical conditions of FD have been reported to date[1]: monostotic, polyostotic, McCune-Albright syndrome (MAS), and Mazabraud’s syndrome. The monostotic form occurs most frequently andrepresents approximately 75% of FD cases. MAS, accounting for about 3% of polyostotic FD, is characterized by polyostotic FD, café-au-lait cutaneous spots, and various endocrinopathies[2]. Mazabraud’s syndrome is a clinical type characterized by soft tissue mass (intramuscular myxoma) concomitant to polyostotic FD[3].

The involvement of facial and cranial bones in FD, also termed craniofacial fibrous dysplasia (CFD), occurs in nearly 50% of polyostotic FD and in 10%–27% of monostotic FD cases[4]. Depending on the type and location of CFD, the signs and symptoms vary and include facial deformity and asymmetry, vision changes, hearing impairment, nasal congestion and/or obstruction, pain, paresthesia, and malocclusion[5]. We present the first case of post-traumatic cerebrospinal fluid (CSF) rhinorrhea associated with CFD.

2 Case study

2.1 History and examination

A 30-year-old man presented with a 20-day history of clear discharge from his right nostril. He suffered from a mild head trauma 40 days before counseling at our institution. Laboratory tests of the discharge showed a glucose level of 3.73 mmol/L. This high glucose level suggested that the discharge was CSF. Physical examination showed that the patient’s left eye and left frontal part were more prominent than the equivalent right side. According to the patient’s recall, these symptoms first appeared at the age of twenty and had changed slowly since then. Computed tomography (CT) cisternogram located the site of the defect in the posterior wall of the right frontal sinus and demonstrated the evident expansion of multiple skull bones (Figure 1). Lumbar puncture was done and intracranial pressure was 150 mm H2O.

Preoperative routine X-ray chest analysis demonstrated evident scoliosis and multiple local enlargements of the ribs (Figure 2). Because of a lack of any symptoms of the limbs and cavitas pelvis, X-ray analysis of these regions was not performed. No caféau-lait cutaneous spots were found on the patient. The patient did not have any symptoms related to endocrine abnormalities. Levels of related serum hormones were not tested. Without café-au-lait cutaneous spots and endocrine abnormalities, polyostotic FD was diagnosed instead of MAS.

2.2 Operation and postoperative course

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”, “l(fā)ytic”, 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).

Figure 2X-ray chest analysis demonstrating scoliosis (black arrow) and multiple local enlargements of the ribs (white arrow).

The patient underwent craniotomy fistula repair surgery. A coronal incision was made and a pericraniumflap was prepared for cranial base repair. The bone window was 7 cm × 15 cm. We explored the entire anterior cranial base and located the fistula to the posterior wall of the right frontal sinus as demonstrated by the preoperative CT cisternogram. The pericranium was folded to cover the defect and then sutured to the cranial base dura. The bone flap was repositioned and fixed using standard techniques. To increase the cranial cavity and decrease the intracranial pressure, the excised bone flap was contoured to be thinner before it was placed back (Figure 3). As the patient’s sight was good in both eyes, optic nerve decompression was not performed although the left optic nerve was evidently compressed by expanded bones (Figure 1).

CSF rhinorrhea stopped after the surgery. Twelve days after the fistula repair surgery, enhanced magnetic resonance imaging (MRI) was performed to evaluate the effect of the operation and to obtain more information about the FD lesions (Figure 4). A nineteenmonth follow up was made. The patient recovered well and CSF leakage did not recur. However, the sight of the patient’s left eye had decreased during the last month. A repeat CT scan was performed, which showed that the compression of the left optic nerve seemed to be more severe than nineteen months ago (Figure 5). MAS was suspected. Decompression surgery of the left optic nerve canal and laboratory tests of serum hormones were proposed, but the patient refused them. No other new neurological deficits appeared during the follow-up period.

3 Discussion

FD represents about 2.5% of all primary bone masses and 7% of benign bone masses[6]. Three main histological types have been identified in FD: the“Chinese letters” model, the “pagetoid” model, and the“hypercellular” model. Each of these is differentiated on the basis of the architecture and cellularity of the osseous tissue[7]. CT is recommended to define the anatomy of individual lesions and to establish the extent of disease in CFD[5,8]. The natural radiographic progression may vary from a “ground-glass” or homogenous appearance to a mixed radio-dense/ radio-lucent lesion as the patient ages. The radiographic density of the lesions depends on the proportion of fibrous and osseous elements[7]. MRI is a complementary method to CT, and it is used to examine cranial nerve involvement and obtain more information about the lesion[9]. The signal intensities of FD are usually low on T1-weighted images, but often variable on T2-weighted images, ranging from low-to-high signals[10]. Enhanced MRI often indicates the involved bones contain numerous small vessels and fibrous elements[3].

There were two factors associated with CFD that together might have contributed to the CSF rhinorrhea risk increase in this particular patient.

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.

The first was pathological changes in the skull bone. FD is characterized by the excessive formation of fibrous tissue in the bone marrow and the destruction of normal bone in conjunction with abnormal bone formation and increased osteoclast activity[11]. Normal bone tissue is replaced by the gradual abnormal proliferation of fibrous tissue[9]. These pathological changes weaken the structural integrity of the involved bones[12]. Under slight stress, these bones tend to deform and might even fracture[13]. As the limbs, especially both lower limbs, usually bear most of the mechanical stressduring daily life, pathologic fractures often occur in these bones with FD. Without significant stress, skull bones with FD rarely fracture. However, when a CFD patient suffers from head trauma, these skull bones might fracture more easily than normal bones. In our patient, CFD was involved in multiple skull bones, including the right frontal bone. A CT scan indicated a low density of the involved right frontal bone (Figure 1), and enhanced MRI showed evident enhancement of the right frontal sinus wall (Figure 4). This suggested that the bone in right frontal sinus wall contained numerous small vessels and fibrous elements[3], which would decrease the strength of the bone. These pathological changes increased the risk of fractures after head trauma. As the enhanced MRI was performed after surgery, the enhancement of right frontal sinus posterior wall might also be caused by a healing reaction. However, because the anterior wall of right frontal sinus and surrounding involved bones were also enhanced, it was reasonable to presume that the enhanced effect was mainly caused by pathological changes due to FD.

The second factor was increased intracranial pressure associated with CFD that might contribute to CSF rhinorrhea. In this case, a CT scan demonstrated significant thickening and expansion of multiple skull bones, decreasing the cranial cavity (Figure 1). However, before the head trauma, this patient did not have symptoms associated with increased intracranial pressure, including headache, nausea and vomiting. This suggested that before the trauma, the patient’s intracranial pressure was well compensated. However, as the cranial cavity was decreased after trauma, this compensation for a higher intracranial pressure was constrained. During the period of trauma, both the swelling of the brain and pneumocephalus might have contributed to the increased intracranial pressure. Without good compensation ability, the patient’s intracranial pressure increased. Although the intracranial pressure measured by lumber puncture before the fistula repair surgery was normal, this might have been associated with an alleviation of the swellingbrain after a forty-day recovery and the discharge of CSF through the fistula. To increase the cranial cavity, we contoured the excised bone to be thinner before it was placed back during the fistula repair surgery. This treatment effect was satisfactory (Figure 3).

Figure 4Axial 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).

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).

In this case, CSF rhinorrhea began about twenty days after the head trauma. Initially, the fistula might be temporarily blocked by tissues such as the brain, granulation tissue, and sinus mucosa[14]. Sometimes, CSF leakage can occur more than 3 months after head trauma, termed delayed onset of CSF rhinorrhea[15]. Spontaneous CSF rhinorrhea has also been reported in various anterior cranial base pathologies, such as osteoma, pituitary adenoma, primary empty sella, and chordoma[16–19]. Spontaneous CSF rhinorrhea may be caused by pathological invasion of the cranial base or intracranial hypertension[20,21]. Although it was reported that CFD may have a tendency for spontaneous CSF leakage[22], there have been no related case reports published to the best of our knowledge. Our case report may provide support to the idea that CFD may have a tendency for spontaneous CSF leakage. However, in some situations, CFD may decrease the risk of CSF rhinorrhea. For example, if the cavity of the paranasal sinus is filled with expanded bone tissue as in the left frontal sinus of our patient (Figure 1), CSF cannot drain out through the paranasal sinus. Furthermore, if the wall of the paranasal sinus contains more osseous elements, the bone strength is stronger and less likely to fracture. Therefore, whether CFD increases the risk of CSF rhinorrhea in different situations needs to be evaluated.

Because the sight of the patient’s left eye had decreased during follow up, we discuss the treatment strategy of CFD.

FD commonly behaves as a slow and indolent growing mass lesion, which is often present in the first 3 decades of life and usually stabilizes when the patient reaches skeletal maturity[6]. There have also been reports of persistence at later periods underlining their variable clinical behavior[22]. In CFD, the most common presenting symptom is a gradual, painless enlargement of the involved bone. If cranial foramina or bony cavities are involved, symptoms such as visual disturbances, orbital dystopia, proptosis, epiphora, facial paralysis, hearing loss, and nasal obstruction may occur.

The surgical treatment of CFD aims to correct the facial deformity in most cases and restore the obliterated foramina, when it the cause of problems. However, this can result in recurrence in 15%–20% of all cases, especially during the growth period[7,23]. The most devastating consequence of CFD is the loss of vision due to optic nerve compression (ONC). There has been significant controversy regarding the management of FD of the sphenoid bones that encase the optic nerve, particularly in patients whose vision is normal. A meta-analysis[24]showed that most patients with CFD remain asymptomatic during long-term follow-up, and therefore expectant management is recommended in asymptomatic patients even in the presence of radiological evidence of ONC. Lee et al.[5]recommended that FD in the skull base around vital structures, including the optic nerve, should be managed according to the clinical examination and regular diagnostic imaging and observation is appropriate in asymptomatic patients. When visual change or vision loss occurs due to ONC, decompression surgery can be performed. In this case, the patient’s age was over 30 years and a CT scan of the involved skull bones showed a mixed appearance (Figure 1). This indicated that the involved bones had entered a stable period. However, nineteen months later, sight in the patient’s left eye had decreased. A CT scan showed that compression of the left optic nerve was more severe than at nineteen months previously (Figure 5). MAS was suspected. Unfortunately, the patient refused to undergo the proposed decompression surgery and laboratory tests of serum hormones.

MAS is characterized by the triad of polyostotic FD, café-au-lait cutaneous spots, and various endocrinopathies. Patients may have only 2 of the 3 characteristics, sometimes lacking the café-au-lait spots, and may have one or more of the numerous endocrine symptoms[12]. In MAS, the overall degree of bony enlargement and deformity is often more severe and disfiguring than in patients with polyostotic FD. The frequency of malignant change is also increased in MAS[25]. Furthermore, endocrine abnormalities can worsen the situation. For example, excessive growth hormone production might cause severe deformities and ahigher risk of vision loss related to optic nerve compression[26,27]. Chronic thyroid hormone excess can cause clinically significant bone mineral loss through the direct stimulation of bone resorption[28]. Therefore, MAS should be precluded before polyostotic FD is diagnosed.

This case suggests that CFD, especially in cases where walls of the paranasal sinuses are involved and contain numerous fibrous elements, may increase the risk of CSF rhinorrhea after head trauma. If we can recognize the high risk of CSF rhinorrhea in similar situations, immediate treatment or close observation may improve the prognosis of patients.

Conflict of interests

The authors have no financial interest to disclose regarding the article.

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Li P, Zhou QY, Yang ZJ, Wang ZM, Li SW, Wang XC, Wang B, Zhao F, Liu PN. Post-traumatic cerebrospinal fluid rhinorrhea associated with craniofacial fibrous dysplasia: Case report and literature review. Transl. Neurosci. Clin. 2016, 2(3): 188–194.

* Corresponding author: Pinan Liu, E-mail: pinanliu@ccmu.edu.cn

Supported by the National Science and Technology Support Program of the 12th Five-Year of China (grant number: 2012BAI12B03) and Natural Science Foundation of Beijing (grant number: 7112049).

cerebrospinal fluid rhinorrhea;

post-traumatic;

literature review

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.