Madison Lampkin, University of Arkansas for Medical Sciences

Suzanne Smart, MD, Arkansas Children's Hospital

Byron Wilkes, MD, McFarland Eye Care

Intro- Graves’ disease is the leading cause of hyperthyroidism in the developed world. Some of the signs and symptoms of Graves’ disease include goiter, tremor, elevated heart rate, hyperreflexia, eye disease, and pretibial myxedema.¹ The ocular findings of Graves’ disease are due to antithyroglobulin immune complexes which bind to extraocular muscle membranes triggering an inflammatory response leading to the fibrosis and edema.^3,5 The acute phase is managed medically with steroids and immunomodulatory agents. However, surgical intervention may be necessary if the patient becomes unresponsive or intolerant of steroids, or reports changes in vision.^3–5 The goal of orbital decompression is to reduce pressure within the orbit to allow for recession of the globe.³

Materials/methods– A fifteen-year-old female patient was referred to the otolaryngology (ENT) clinic for chronic refractory Graves’ disease. Approximately 1 year after undergoing total thyroidectomy, the patient was scheduled for bilateral orbital decompression due to worsening proptosis of both eyes, significant corneal exposure, and chronic eye irritation. Under the care of an oculoplastic surgeon, the patient underwent bilateral orbital decompressions via the transconjunctival approach with bilateral external ethmoidectomies.

Results– Immediately post-operatively the patient had improved proptosis. The patient was sent home the same day of surgery. The periorbital edema resolved and the incisions healed well. She showed improvement in the inferior scleral show bilaterally as well as improvement in the anterior/posterior position of both globes.

Graves’ disease is the leading cause of hyperthyroidism in the developed world. It is an autoimmune disease caused by the production of auto-antibodies that bind to and activate thyroid stimulating hormone receptors, resulting in thyroid hyperplasia and the production of excess thyroid hormone.¹ Some of the signs and symptoms of Graves’ disease include goiter, tremor, elevated heart rate, hyperreflexia, eye disease, and pretibial myxedema. Girgis et al (2011) reports that 95% of patients with Graves’ disease have diffuse goiter and symptoms of hyperthyroidism such as weight loss, heat intolerance, insomnia, and palpitations¹; and 50% have ocular manifestations including proptosis, lid lag, lid retraction, corneal exposure, restrictive ocular myopathy, and optic neuropathy.^2–4

The ocular findings of Graves’ disease are due to antithyroglobulin immune complexes which bind to extraocular muscle membranes triggering an inflammatory response leading to the fibrosis and edema.^3,5 Fibroblasts deposit mucopolysaccharides in the periorbital tissues leading to reduced ocular motility, anterior protrusion of the globe, and eventually compression of the optic nerve and blindness.^3,5 Graves’ ophthalmopathy can be divided into the initial acute phase of inflammation and then the stable chronic phase of fibrosis.³ Symptoms of corneal and conjunctival irritation can be managed with lubricants and taping eyelids closed at night.⁴ The acute phase is managed medically with steroids and immunomodulatory agents. However, surgical intervention may be necessary if the patient becomes unresponsive or intolerant of steroids, or reports changes in vision.^3–5

The goal of orbital decompression is to reduce pressure within the orbit to allow for recession of the globe.³ Indications for surgical orbital decompression include failure of medical therapy, optic neuropathy, corneal exposure keratitis, and to improve cosmesis.³ Complications range from severe—such as reduction in visual acuity, cerebrospinal fluid leak with cerebral complications, intra- and post-operative hemorrhage—to  less serious complications such as hypesthesia or anestheia of the face, post-operative sinusitis or mucocele, and palpebral lesions.⁶

A fifteen-year-old female patient was referred to the otolaryngology (ENT) clinic for chronic refractory Graves’ disease. At this point, she had been on multiple medications to control the “storm of hyperthyroidism.” Upon exam, she had thyromegaly and evidence of orbital proptosis. The patient also complained of dysphagia and neck pain which was tender to palpation. Due to the patient’s chronic recalcitrant thyroid disorder, Graves’ syndrome, and orbital proptosis, the ENT physician recommended total thyroidectomy following appropriate preoperative work-up (i.e. repeat thyroid function levels and calcium, magnesium, vitamin D levels, ultrasound of the neck). One month later, the patient underwent total thyroidectomy and was started on thyroid hormone replacement therapy. She was discharged from the hospital without any complications. She returned one month later for her postoperative follow-up visit in clinic. At that time, she denied numbness, tingling, and thyroid symptoms; however, she was still proptotic.

During her endocrinology follow-up visits over the following months, the patient continued to complain of headaches due to eye pain. Approximately 1 year after undergoing total thyroidectomy, the patient was scheduled for bilateral orbital decompression due to worsening proptosis of both eyes, significant corneal exposure, and chronic eye irritation. Under the care of an oculoplastic surgeon, the patient underwent bilateral orbital decompressions via the transconjunctival approach with bilateral external ethmoidectomies.

The risks, benefits, alternatives of the procedure were discussed with the patient and family and informed consent was obtained before the procedure. The patient was taken back to the operating room where general anesthesia was established without complications. Lateral canthi, medial canthi, and lower eyelids were injected with 1% lidocaine with epinephrine. The patient was then prepped and draped in the normal sterile fashion for facial surgery.  Attention was first directed to the right side where a lateral canthotomy and cantholysis incision was made. The inferior ramus of the canthal tendon was then incised using scissors to free the tendon from the bone. The lid was then placed on stretch and the conjunctivae and lower eyelid retractors were then incised laterally, then extended medially. A preseptal plane was then identified down to the inferior orbital rim and Bovie cautery was used to dissect down to the level of the periosteum in this region. A caudal elevator was then used to elevate the periosteum along the floor of the eye socket. The infraorbital nerve was visualized and an opening in the floor of the orbit was made medial to this landmark. A Kerrison was then used to remove segments of the orbital floor medial to the infraorbital nerve and along the posterior aspect of the floor.

Once this was complete, attention was then directed to the medial canthus. A medial canthal incision was made at the base of the nose, just above the medial canthal tendon. This was carried out down to the nasal bone periosteum using Bovie cautery. A caudal elevator was then used to establish a subperiosteal dissection along the medial wall of the orbit. Orbital retractors were used to retract the orbit and the anterior and posterior and ethmoid arteries were visualized. The lacrimal fossa was then entered using a Kerrison and large segments of the medial orbital wall were removed. Care was taken to make sure that the dissection was made inferior to the posterior and anterior ethmoid arteries. Once an adequate dissection of bone had taken place and the external ethmoidectomy was complete, the periosteum of the orbit was opened along both the medial walls and the floor and gentle pressure was applied to the eye to allow for movement of the fat and orbital tissues into these newly opened spaces.

At this point, the conjunctiva and lower eyelid retractors were closed using a 6-0 plain gut suture in interrupted fashion. The lateral canthus was then closed using a 5-0 Monocryl suture to close the tendon, followed by 6-0 plain gut suture for the skin. The medial canthal incision was closed first using a 5-0 vicryl suture, closing the deep portions of the wound and re-establishing the canthal tendon position. The remaining skin incision was closed using 6-0 plain gut suture. Attention was then directed to the left side, where the identical procedure was performed.

Immediately post-operatively the patient had improved proptosis, as seen in the video. She had incisions near the medial canthi bilaterally and some periorbital edema due to surgical manipulation of the tissues surrounding the orbit (see Image 3). Post-operative care includes checking pupillary light reflexes, visual acuity, and elevation of the head of the bed to 45 degrees.⁸ A stool softener is recommended to avoid Valsalva maneuver. Post-operative antibiotics may be used up to 7 days post-operatively.⁸ The patient was sent home the same day of surgery. The periorbital edema resolved, and the incisions healed well (Images 4 and 5). She showed improvement in the inferior scleral show bilaterally as well as improvement in the anterior/posterior position of both globes.

There are several different surgical approaches to orbital decompression: such as the endoscopic transnasal approach, transantral approach, and an external orbital approach to ethmoidectomy with reported mean globe reductions of 4.7,⁵ 4.7,⁷ and 4.2 mm⁷ respectively. Thus each approach yields comparable globe reductions. The approach to decompression is based on the experience of the surgeon, the individual needs of the patient, the severity of the ophthalmopathy, and anatomical characteristics of the patient.

Some important factors to consider before performing orbital decompression surgery including the oculocardiac reflex, nasolacrimal duct, ethmoid arteries, and the infraorbital nerve. Traction on the extraocular muscles and the globe can induce the oculocardiac reflex, which triggers cardiac dysrhythmias most commonly bradycardia. Thus, frequent communication with the anesthesiologist regarding the patient’s heart rate and rhythm is encouraged. Releasing tension/pressure on the eye should resolve this cardiac reflex.⁹ When performing the ethmoidectomy, some important nearby structures to consider include the nasolacrimal duct and the ethmoid arteries. Damage to the ethmoid arteries, specifically the anterior ethmoid artery, can lead to the formation of an orbital hematoma which can lead to loss of vision.⁸ It is also important to avoid damaging the infraorbital nerve (maxillary branch of cranial nerve V or trigeminal nerve) which supplies sensation to the face inferior to the eye, including the upper lip and inferior aspect of the nose, superior to the lower lip. This nerve runs beneath the floor of the orbit and exits the maxilla inferior to the orbit from the infraorbital canal. The infraorbital groove is an important bony landmark for locating this nerve.

Some limitations to this technique include the skill and experience of the surgeon and intrinsic factors of the patient (i.e. wound healing, compliance with post-operative follow-up exams and treatment plan). Thus, this patient’s outcomes may not be generalizable. This approach to surgical decompression of the orbit is at the discretion of the surgeon and the specific needs of the individual patient. Furthermore, there may be some selection bias with regard to demonstrating this surgical technique, for example, this case was not emergent and the patient was otherwise young and healthy. These qualities were taken into consideration to effectively demonstrate a surgical procedure for educational purposes.

There are no funding sources or conflicts of interest to disclose.

Arkansas Children's Hospital Pediatric Otolaryngology Department

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