Case Reports Spontaneous Decompression Fracture in Thyroid Eye Disease Marc A. Richardson, M.D.* and Kyle T. Lewis, M.D.† Abstract: This is a case of a 44-year-old female with a history of Graves’ orbitopathy presented to the emergency department after waking from a nap with sudden onset of left facial and periorbital swelling, ecchymosis, and subconjunctival hemorrhage. A CT scan obtained in the emergency department revealed a left blowout fracture and enlarged extraocular muscles. The patient lives with her mother and both adamantly denied any trauma. The patient had sustained a spontaneous orbital fracture; a process reported but few times in the medical literature.
the emergency department. The patient also denied fever, chills, discharge, or recent illness. Upon initial examination, she was found to have 20/20 visual acuity in both eyes and full ocular motility. The intraocular pressure was mildly elevated in the left eye and there was significant subconjunctival hemorrhage and chemosis. The remainder of the eye exam was within normal limits without signs of ocular trauma. A CT of the face and orbits revealed a left orbital blowout fracture of the medial wall and floor with enlarged extraocular muscles and a subjective increase in orbital fat consistent with GO (Fig.)
G
raves’ orbitopathy (GO) causes an increase in orbital volume and pressure due to inflammatory cellular infiltration, accumulation of glycosaminoglycans, and retention of fluid. The increased pressure and volume can lead to compressive optic neuropathy, and surgical decompression of the orbit is needed in some cases to prevent or limit visual impairment. Autodecompression also may occur through proptosis, as well as a variety of recently proposed mechanisms, including medial orbital wall remodeling,1 and spontaneous orbital floor fractures.2,3 This is a case of auto decompression by spontaneous orbital floor fracture in a patient with GO. To our knowledge this is only the third case reported describing such a phenomenon. In accordance with the Health Insurance Portability and Accessibility Act guidelines, no patient identifiers have been used in this case report.
CASE REPORT The patient is a 44-year-old female who was diagnosed with Graves’ hyperthyroidism in 1997 and treated with radioactive iodine at that time. She had no signs of GO until 2009 when she noticed that her right eye was “bulging” and had sensation of pressure behind it. She presented to and was treated by another oculoplastic surgeon for dry eye and exposure keratopathy due to eyelid retraction, which was worse in the right eye than the left. She later required levator recession to correct eyelid retraction of the right eye in April 2011 and was happy with the outcome of the procedure. The patient never required or received systemic or local steroids. The patient was presented to the emergency department in September 2013 after waking from an afternoon nap with a sudden onset of left-sided facial pain, significant facial and periorbital swelling, ecchymosis, and subconjunctival hemorrhage. The patient was then seen by our ophthalmology consult service. Upon questioning, the patient adamantly denied any trauma or falls prior to or during her nap, an assertion which was confirmed by the patient’s mother, who was present in the home at the onset of symptoms and accompanied the patient to *Department of Ophthalmology and †Ophthalmic Plastic and Reconstructive Surgery, University of Mississippi Medical Center, Jackson, Mississippi, U.S.A. Accepted for publication October 7, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Kyle T. Lewis, m.d., f.a.c.s., Ophthalmic Plastic and Reconstructive Surgery, University of Mississippi Medical Center, 2500 N. State St., Jackson, MS 39216. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000354
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014
CT scan, coronal view, showing fractures of the orbital floor and medial orbital wall with herniation of orbital fat into the fracture site and enlargement of the extraocular muscles consistent with Graves’ orbitopathy.
The patient has been followed by the oculoplastic surgery service since discharge from the hospital. As a result of the decompression fracture, the patient developed relative enophthalmos with exophthalmometry measurements of 24 OD and 21 OS. Fracture repair was subsequently performed due to the fact that she was unhappy with her appearance. The risk of increased intraocular pressure after fracture repair was of some concern, though this was not seen, and the patient experienced no postoperative complications. A right-sided, medial orbital wall endoscopic decompression was later performed because the right eye remained slightly more exophthalmic than the left. Ultimately, good globe symmetry was achieved postoperatively.
DISCUSSION GO causes an increase in intraorbital pressure due to volumetric increases in orbital contents secondary to inflammatory cellular infiltration, accumulation of glycosaminoglycans, and retention of fluid. A recent study by Berthout4 demonstrated this fact by measuring intraorbital pressures before, during, and after decompression surgery in patients with GO. The study showed significant increases in intraorbital pressure in patients with GO over that of the general
e1
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014
Case Reports
population, and pressures were even higher in those patients presenting with optic neuropathy. As expected, pressures decreased after decompression surgery. Of note, reduction of pressure was found to be greatest after collapse of the first wall in multiwall decompression surgery. Proptosis is commonly seen in GO, and is a form of autodecompression of the orbit. Enlargement of the extraocular muscles and orbital fat causes the eye to be forced forward, resulting in protrusion of the globe. Laxity of the orbital septum, as occurs with increased age, allows for more pronounced proptosis. A recent article by Detorakis1 describes another hypothesized method of autodecompression. He describes the medial orbital wall remodeling/displacement caused by enlargement of an adjacent medial rectus muscle in a patient with GO. A review of the medical literature revealed two reported cases of bony autodecompression. Bhermi2 reported a case of a patient who, like our patient, presented with sudden symptomatic orbital floor fracture upon awaking without a history of trauma. The authors proposed a possible mechanism for the bony decompression; local pressure leading to slow erosion of orbital floor, which can be exacerbated by use of high-dose steroids. Kashkouli3 reported a case of asymptomatic orbital fracture in GO. They believed that intraorbital pressure may have exceeded the bone tolerance after gradual remodeling of the floor, and that minor trauma (eye rubbing) increased the pressure enough
e2
to cause a fracture. They also believe that steroid use as well as previous hyperthyroid state had a deleterious effect on the bone mass, increasing susceptibility to fracture. Spontaneous decompression fracture in GO is an extremely rare entity, with few cases previously reported in the medical literature. In this case, the fracture seems to have occurred during sleep, without apparent history of trauma or manipulation. We share the belief that hydraulic forces, caused by increased intraorbital pressures commonly seen in GO, were responsible for the blowout fracture sustained by our patient. This blowout greatly reduced intraorbital pressures, resulting in reversal of proptosis and subsequent enophthalmos, which was later surgically corrected. Thus, spontaneous bony decompression must be in the differential in any GO patient with suggestive history and signs.
REFERENCES 1. Detorakis ET. Spontaneous medial orbital decompression associated with medial wall remodeling in Graves’ orbitopathy. Ophthal Plast Reconstr Surg 2014;30:79–80. 2. Bhermi GS, Gauba V, Brittain P. Spontaneous bony orbital autodecompression in thyroid ophthalmopathy. Orbit 2006;25:123–5. 3. Kashkouli MB, Pakdel F. Spontaneous orbital floor fracture in thyroid eye disease. Ophthal Plast Reconstr Surg 2010;26:301–2. 4. Berthout A, Vignal C, Jacomet PV, et al. [Intraorbital pressure measured before, during, and after surgical decompression in Graves’ orbitopathy]. J Fr Ophtalmol 2010;33:623–9.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
the emergency department. The patient also denied fever, chills, discharge, or recent illness. Upon initial examination, she was found to have 20/20 visual acuity in both eyes and full ocular motility. The intraocular pressure was mildly elevated in the left eye and there was significant subconjunctival hemorrhage and chemosis. The remainder of the eye exam was within normal limits without signs of ocular trauma. A CT of the face and orbits revealed a left orbital blowout fracture of the medial wall and floor with enlarged extraocular muscles and a subjective increase in orbital fat consistent with GO (Fig.)
G
raves’ orbitopathy (GO) causes an increase in orbital volume and pressure due to inflammatory cellular infiltration, accumulation of glycosaminoglycans, and retention of fluid. The increased pressure and volume can lead to compressive optic neuropathy, and surgical decompression of the orbit is needed in some cases to prevent or limit visual impairment. Autodecompression also may occur through proptosis, as well as a variety of recently proposed mechanisms, including medial orbital wall remodeling,1 and spontaneous orbital floor fractures.2,3 This is a case of auto decompression by spontaneous orbital floor fracture in a patient with GO. To our knowledge this is only the third case reported describing such a phenomenon. In accordance with the Health Insurance Portability and Accessibility Act guidelines, no patient identifiers have been used in this case report.
CASE REPORT The patient is a 44-year-old female who was diagnosed with Graves’ hyperthyroidism in 1997 and treated with radioactive iodine at that time. She had no signs of GO until 2009 when she noticed that her right eye was “bulging” and had sensation of pressure behind it. She presented to and was treated by another oculoplastic surgeon for dry eye and exposure keratopathy due to eyelid retraction, which was worse in the right eye than the left. She later required levator recession to correct eyelid retraction of the right eye in April 2011 and was happy with the outcome of the procedure. The patient never required or received systemic or local steroids. The patient was presented to the emergency department in September 2013 after waking from an afternoon nap with a sudden onset of left-sided facial pain, significant facial and periorbital swelling, ecchymosis, and subconjunctival hemorrhage. The patient was then seen by our ophthalmology consult service. Upon questioning, the patient adamantly denied any trauma or falls prior to or during her nap, an assertion which was confirmed by the patient’s mother, who was present in the home at the onset of symptoms and accompanied the patient to *Department of Ophthalmology and †Ophthalmic Plastic and Reconstructive Surgery, University of Mississippi Medical Center, Jackson, Mississippi, U.S.A. Accepted for publication October 7, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Kyle T. Lewis, m.d., f.a.c.s., Ophthalmic Plastic and Reconstructive Surgery, University of Mississippi Medical Center, 2500 N. State St., Jackson, MS 39216. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000354
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014
CT scan, coronal view, showing fractures of the orbital floor and medial orbital wall with herniation of orbital fat into the fracture site and enlargement of the extraocular muscles consistent with Graves’ orbitopathy.
The patient has been followed by the oculoplastic surgery service since discharge from the hospital. As a result of the decompression fracture, the patient developed relative enophthalmos with exophthalmometry measurements of 24 OD and 21 OS. Fracture repair was subsequently performed due to the fact that she was unhappy with her appearance. The risk of increased intraocular pressure after fracture repair was of some concern, though this was not seen, and the patient experienced no postoperative complications. A right-sided, medial orbital wall endoscopic decompression was later performed because the right eye remained slightly more exophthalmic than the left. Ultimately, good globe symmetry was achieved postoperatively.
DISCUSSION GO causes an increase in intraorbital pressure due to volumetric increases in orbital contents secondary to inflammatory cellular infiltration, accumulation of glycosaminoglycans, and retention of fluid. A recent study by Berthout4 demonstrated this fact by measuring intraorbital pressures before, during, and after decompression surgery in patients with GO. The study showed significant increases in intraorbital pressure in patients with GO over that of the general
e1
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014
Case Reports
population, and pressures were even higher in those patients presenting with optic neuropathy. As expected, pressures decreased after decompression surgery. Of note, reduction of pressure was found to be greatest after collapse of the first wall in multiwall decompression surgery. Proptosis is commonly seen in GO, and is a form of autodecompression of the orbit. Enlargement of the extraocular muscles and orbital fat causes the eye to be forced forward, resulting in protrusion of the globe. Laxity of the orbital septum, as occurs with increased age, allows for more pronounced proptosis. A recent article by Detorakis1 describes another hypothesized method of autodecompression. He describes the medial orbital wall remodeling/displacement caused by enlargement of an adjacent medial rectus muscle in a patient with GO. A review of the medical literature revealed two reported cases of bony autodecompression. Bhermi2 reported a case of a patient who, like our patient, presented with sudden symptomatic orbital floor fracture upon awaking without a history of trauma. The authors proposed a possible mechanism for the bony decompression; local pressure leading to slow erosion of orbital floor, which can be exacerbated by use of high-dose steroids. Kashkouli3 reported a case of asymptomatic orbital fracture in GO. They believed that intraorbital pressure may have exceeded the bone tolerance after gradual remodeling of the floor, and that minor trauma (eye rubbing) increased the pressure enough
e2
to cause a fracture. They also believe that steroid use as well as previous hyperthyroid state had a deleterious effect on the bone mass, increasing susceptibility to fracture. Spontaneous decompression fracture in GO is an extremely rare entity, with few cases previously reported in the medical literature. In this case, the fracture seems to have occurred during sleep, without apparent history of trauma or manipulation. We share the belief that hydraulic forces, caused by increased intraorbital pressures commonly seen in GO, were responsible for the blowout fracture sustained by our patient. This blowout greatly reduced intraorbital pressures, resulting in reversal of proptosis and subsequent enophthalmos, which was later surgically corrected. Thus, spontaneous bony decompression must be in the differential in any GO patient with suggestive history and signs.
REFERENCES 1. Detorakis ET. Spontaneous medial orbital decompression associated with medial wall remodeling in Graves’ orbitopathy. Ophthal Plast Reconstr Surg 2014;30:79–80. 2. Bhermi GS, Gauba V, Brittain P. Spontaneous bony orbital autodecompression in thyroid ophthalmopathy. Orbit 2006;25:123–5. 3. Kashkouli MB, Pakdel F. Spontaneous orbital floor fracture in thyroid eye disease. Ophthal Plast Reconstr Surg 2010;26:301–2. 4. Berthout A, Vignal C, Jacomet PV, et al. [Intraorbital pressure measured before, during, and after surgical decompression in Graves’ orbitopathy]. J Fr Ophtalmol 2010;33:623–9.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
