Progressive Hypoglobus in a patient with a history of remote facial fracture and repair.
Authors: Marisa Palumbo, O.D.
1 , James Aylward, O.D.
1 , Lisa Fanciullo O.D 1
1 Boston VA HCS and the New England College of Optometry
ABSTRACT: Case report of hypoglobus secondary to recurrent frontal sinus mucocele. Mucoceles are mucous or fluid filled cysts which can displace the globe. The clinical features present and factors predisposing to recurrent mucocele formation will be discussed.
Case Report Outline:
I Case History:
Patient demographics: 59 year old Caucasian Male
Chief Complaint: Patient and his wife are concerned with puffiness superior nasally to left globe and more dramatic inferior displacement of left eye over past month. Patient denies diplopia, flashes, floaters, and eye pain.
Ocular, medical history: o Medical history is notable for hypertension, hyperlipidemia, and type II diabetes
Mellitus. o Patient is s/p facial trauma in 1966 with facial fracture and no surgical repair. o S/p left orbital floor fracture with trauma to bilateral frontal sinuses in 1969 secondary to high impact mine explosion. Pt then suffered subsequent CSF leak, which was managed with a dural patch. o 3/2005 Patient found to have a left frontal sinus mass expanding into frontal anterior cranial fossa epidural space and left superior orbit. o 5/2005 s/p left frontal mucocele excision and osteoplastic flap reconstruction with cranialization of left frontal sinus and temporalis pericranial flap obliteration.
Surgery was performed by ENT in collaboration with neurosurgery and oculoplastics. o Ocular history : Pertinent findings
 Noted facial asymmetry over past several years.
 Patient has never reported diplopia in primary gaze
 Excellent visual acuity with correction over past several years. Patient is a hyperope and presbyope with a RX of OD +3.25-.25x 90 OS +5.00 -1.25x 165
 Patient has had a history of choroidal folds OS. OD has a subtle small choroidal rupture inferior temporally to the optic nerve head.
Medications: Patient is not on any systemic medications at time of presentation. Patient is currently using ketotifen .025% ophthalmic solution 1 gtt BID OU for allergy symptoms.

Other salient information: o Patient has been prescribed systemic medication for his Diabetes, hypertension, and hyperlipidemia but stopped taking them without his primary care physician’s knowledge.
II Pertinent findings:
Clinical: o Distance visual acuity is 20/20 OD and OS. o Cover test without correction at near reveals a left hypo exophoria which is greater in right gaze. o Slight anisocoria, pupils are 4 mm in the right eye and 3 mm in the left eye with no afferent pupillary defect. o Ocular motilities are full in the right eye and severely restricted in up gazes OS. No diplopia in primary gaze but patient reports diplopia in left, right, and superior gaze. o Confrontation fields are full in each eye. o Exophthalmometry by Hertel with a base of 108 is 22 mm in the right eye and 26mm in the left eye. o Slit lamp bimicroscopy is unremarkable with clear lids, lashes, conjunctiva, and lens.
Patient has arcus OD and OS and angles are open 1:1 by Van Herrick estimation. The anterior chamber is deep. o Intraocular pressures are 14 mmHg in the right eye and 15mmHg in the left eye. o Color vision is normal in each eye with Ishihara testing, 11/11 plates OD and OS. o Dilated fundus exam reveals a cup to disc ration of .10 for the right eye with distinct disc margins with evidence of an old choroidal rupture inferior temporal to the optic nerve head. The left eye has no visible cup and is congested, edematous and hyperemic. The macula OD is clear. There are horizontal linear pigment streaking
OS representing resolved choroidal folds and prominent choroidal folds radiating superior temporally from the optic nerve head which appear more dramatic than in past examinations. The vessels OS are 1+ tortuous compared to the OD. Peripheral retinal examination is unremarkable OD and OS.
Physical: o No rhinorrhea present, unilateral or otherwise. o Patient is afebrile with a normal white blood cell count.

o Facial asymmetry which has been noted in prior exams appears more dramatic.
Patient has hypoglobus of the left eye. o There is non tender fullness to upper nasal area of left upper lid which has a rubbery feel upon palpation not like orbital fat. There is no crepitus, erythema, or edema present. o The following results were found on cranial nerve VII testing. Patient has a loss of forehead furrows on the left side but the frontalis muscle is intact allowing him to raise the left brow. Possible slight mild weakness left lower face, visible when patient smiles. The orbicularis muscle strength is good and symmetrical on right and left side.
Laboratory studies: Normal white blood cell count.
Radiology studies: o CT with and without contrast scan was done to image the head and orbits, patient is unable to have MRI secondary to known shrapnel from previous injury. o CT reveals a 2.7cm x 3.5cm hypodense mass in the left frontal region with associated expansile appearance of bone shown in medial aspect of lesion. There is also an extension to orbit as well as epidural space. Focally prominent meninges are also noted in the left frontal region with possible enhancement. Ct also shows that the globe of the left eye is displaced caudally and laterally as are the optic nerves and sheath.
Others: o B-scan OS reveals a large cystic lesion superior to globe but not impacting the optic nerve but may be causing a mild flattening of superior globe. Cystic lesion appears larger than globe in size. o Humphrey visual field 30-2. Reliable testing revealed no visual field defect in either eye. o Fundus photos were taken as well as external photos of face.
III Differential diagnosis:
Primary/leading: o Recurrent mucocele formation.
Others: o Muscle herniation (muscle that was used to obliterate frontal sinus in first mucocele removal). o Silent Sinus syndrome

IV Diagnosis and Discussion:
Elaborate on the condition: o Mucoceles are epithelial lined mucous containing sac filling a paranasal sinus. In this patients’ case it is in the frontal sinus. The patient had experienced the same signs and symptoms approximately thirteen years ago with the same diagnosis of mucocele. o The most common causes of mucocele formation are trauma to the sinuses and any injury to the sinus mucosa including prior surgery to the sinuses. The most common causes of frontal sinus fractures are motor vehicle accidents or any high force impact injury. Our patient did have a remote history of a frontal sinus fracture from a mine explosion. Our patient also had surgery to repair the frontal sinus with cranialization and obliteration of the sinus o Recurrence of a mucocele results if pieces of the sinus mucosa are inadvertently left behind after surgical repair/removal of the first mucocele. o Usually, mucoceles are benign but there are times when it can become infected creating a dangerous situation when in close proximity to the anterior cranial fossa.
If the mucocele is a pyomucocele (pus filled) there is a greater risk of the patient developing meningitis, or brain abscess due to the proximity to the dura. o This patient did not have a pyomucocele which was determined by a white blood cell count and due to the fact that the patient is currently afebrile. o The patient first presented with ocular complaints typical of a frontal sinus mucocele. All clinical findings and testing including CT and B scan ultrasound confirmed the patients’ diagnosis. o Ocular Clinical findings consistent with a sinus mucocele are: diplopia, proptosis, deviation of the globe, and loss of vision. o A critical part of diagnosing the extent of this patient’s condition was to determine if he had a watery rhinorrhea. This finding or lack there of is significant in patient’s with facial fractures because it may be an indication of CSF leak. CSF leak may occur because mucoceles have the ability to expand and may be erosive by virtue of a process of bone resorption and new bone formation. The posterior table of the frontal sinus, if still intact at time of mucocele formation can be eroded by the mucocele, leading to risk of a dural tear and CSF leak. o The Watery rhinorrhea can be tested for the enzyme beta-2-transferrin to determine if the fluid is CSF. Beta-2- transferring is only found in CSF, perilymph, and ocular aqueous humor but not in sinonasal mucous secretions and tears.
Expound on unique features:

o The patients’ past history of trauma and surgical repair of the left frontal sinus along with his presenting signs and symptoms lead us directly to his diagnosis. This patient has already had 3 craniotomies in his lifetime, and treatment of this most recent incident will be his fourth. o Patient does not have diplopia in primary gaze which leads to question of him suppressing the left eye. The patient has also adapted in other ways, including having his bifocals made so that the seg height on the left eye is lower than the right eye in order to avoid having the segment line bisect his pupil.
V Treatment, management:
Treatment and response to treatment: o Patient is schedule for a bifrontal craniotomy removal of mucocele and reconstruction of orbital roof. o Patients often have cranialization and sinus obliteration as treatment for sinus mucocele. During the procedure the posterior table or wall of the frontal sinus is completely removed and the sinus mucosa is drilled away. The nasofrontal duct is plugged with temporoparietal fascia or muscle and secured with glue so that there is no longer any communication with the sinonasal cavity. Then there are two options. One is to let the brain and dura rest against the anterior wall of the frontal sinus. The second option is to fill the space with adipose tissue. o Cranialization is performed to prevent any future mucocele/pyomucocele formation, cosmetic deformity, and CSF leak. o Risks of the procedures are CSF leak and infection.
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VI Conclusion:
Clinical Pearls, take away points: o Without proper diagnosis mucocele formation can lead to CSF leak and a risk of meningitis. o Long term ophthalmological monitoring of patients who have had sinus surgery is critical. o Clinicians should be aware of key clinical and physical features of mucoceles when examining a patient with a history of facial fractures, including: proptosis, diplopia, deviation of the globe, and watery unilateral rhinorrhea. o With proper surgical remediation there should never be a recurrence of a mucocele.