CHAPTER 54 Surgical rehabilitation of Graves’ orbitopathy
Introduction
Graves’ orbitopathy (GO) is one of the phenotypic appearances of Graves’ disease, a multisystem disorder which usually leads to hyperthyroidism and goiter, less frequently to GO, and rarely to pretibial myxedema and acropachy. GO is the most frequent and important extrathyroidal expression of Graves’ disease. It may also be found, although less frequently, in patients with no present or past history of hyperthyroidism (so-called euthyroid or ophthalmic Graves’ disease), or in patients who are hypothyroid due to chronic autoimmune (Hashimoto’s) thyroiditis1,2. In most affected individuals GO is mild and self-limiting, and only in 3–5% of cases is it severe and potentially sight threatening1,3.
The exact pathogenesis of GO is unknown4–7. It is, however, worth highlighting the clear-cut link between the orbit and the thyroid, because this has important clinical and therapeutic implications8. In addition to endogenous (non-preventable) determinants, such as genetics, age- and gender-related factors9, GO occurrence and progression are influenced by environmental (preventable) factors, such as cigarette smoking, thyroid dysfunction, and different treatments for hyperthyroidism3,10. This implies that control or correction of these risk factors is an integral part of GO management.
The natural history of GO is of gradual increase in severity followed by a plateau phase then gradual improvement11. These are the active phases. The inactive phase follows with no change in severity. GO is thus self-limiting, although it often does not return to baseline. Treatment is aimed at accelerating recovery, preventing serious sequelae, and eventually functional and cosmetic rehabilitation.
Therapeutic options consist of medical therapy, radiotherapy, surgery, or frequently a combination of these. Consensus as to indications and timing of these options has been reached by the EUGOGO consortium (Fig. 54.1)12,13.

Fig. 54.1 Management of Graves’ orbitopathy. Rehabilitative surgery includes orbital decompression, squint surgery, lid lengthening, and blepharoplasty/browplasty. i.v. GCs, intravenous glucocorticoids; OR, orbital radiotherapy; DON, dysthyroid optic neuropathy.
Reproduced with permission Thyroid/EJE, refs 12, 13.
Timing of surgery
Surgery may be necessary to protect visual function in the active phase or to correct the stable typical disfigurement and symptoms in the static post-inflammatory phase of the disease.
The first rehabilitative step mainly consists of orbital bony decompression. It causes reduction in exophthalmos as well as reduction in upper and lower eyelid displacement14. It may positively influence extraocular muscle restrictions, but displacement and scarring of the soft orbital tissues caused by decompression surgery may also cause strabismus. Possible squint surgery should therefore follow orbital decompressions, but considering that vertical tropias may influence eyelid position, squint surgery should precede possible eyelid corrections. Finally, when necessary, the finishing touch can be given by eyebrow lift, forehead plasty, and blepharoplasty.
In short, surgical rehabilitation needs to respect the given order since the preceding step may influence the necessity and the extent of the step that follows. When all the steps are necessary, the entire rehabilitation may require between 1.5 and 2 years. In particular cases, exceptions are possible and the rehabilitation can be favorably speeded up by carrying out more than one procedure at the same time15. The traditional management algorithm has not been respected in only a few series16–20, and it has met with vigorous criticism21.
Orbital decompression
The autoimmune process at the basis of GO induces swelling of the soft tissues contained within the boundary of the bony orbit; this causes impairment of the venous outflux towards the cavernous sinus and reverses the flux in the direction of facial circulation.
This positive feedback circle leads to an increase in the intraorbital pressure, which is first responsible for the progression of GO and later for its typical signs and symptoms5. Any surgical procedure aimed at decreasing the raised intraorbital pressure and its effects by means of enlargement of the bony orbit and/or removal of the orbital fat is defined as orbital decompression.
Orbital decompression is currently indicated for the treatment of optic neuropathy refractory to medical therapy, exposure keratopathy unresponsive to local measures and/or minor eyelid surgeries, disfiguring exophthalmos and symptoms. Eyeball subluxation (which may be a possible cause of acute optic neuropathy and exposure keratopathy) postural visual obscuration in patients with congestive inactive GO and recently onset choroidal folds due to eyeball indentation by enlarged extraocular muscles represent other functional indications for decompression surgery22.
Osteotomies can involve the medial, and lateral orbital walls and the orbital floor (Fig. 54.2); lipectomies can be performed at the level of all the orbital quadrants (Fig. 54.3). Decompression surgery can be performed through several different surgical incisions (Fig. 54.4) preferably under general anesthesia.

Fig. 54.2 Common zones of bone removal for orbital decompression. (A) Axial projection of an orbital CT scan which highlights possible lateral wall osteotomies: conservative anterior (red), deep (green), extended (red + green), total (blue). (B) Coronal projection of an orbital CT scan which highlights possible osteotomies: inferior (yellow), medial (red), inferomedial (yellow + red).
Reproduced with permission: Baldeschi L. Orbital decompression. In: Wiersinga WM, Kahaly GJ, eds. Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers, 2nd edn. Basel: Karger, 2010:171–88.

Fig. 54.3 Schematic representation of sites for orbital lipectomies reachable (yellow) or not (blue) through hidden transconjunctival incisions (red line) superimposed on a periorbital region photograph of a patient with GO (A) and on a coronal projection of an orbital magnetic resonance scan taken at the level of the middle orbit in a patient with GO (B). The fat compartment of the superior lateral quadrant (blue) can be exposed only through transcutaneous incisions; however, it is not used as an elective site for lipectomy because it hosts delicate structures and its removal gives a minimal contribution to fat decompression surgery.
Reproduced with permission: Baldeschi L. Orbital decompression. In: Wiersinga WM, Kahaly GJ, eds. Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers, 2nd edn. Basel: Karger, 2010:171–88.

Fig. 54.4 Common surgical incisions for orbital decompression: (1) coronal; (2) ‘Lynch’; (3) upper skin crease; (4) lateral canthus; (5) subciliary; (6) inferior fornix; (7) direct translower lid; (8) transcaruncular; (9) transnasal; (10) trans-oral; (4+6) swinging eyelid. Bone decompression is accomplished using one or a combination of the listed incisions; for fat decompression, incisions (3), (4), (5), (6), (8), or any combination of these is preferred.
Redrawn with permission: Baldeschi L. Orbital decompression. In: Wiersinga WM, Kahaly GJ, editors. Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers, 2nd edn. Basel: Karger, 2010:171–88.
The inferior fornix incision is an extremely popular approach to orbital decompression. It can be extended medially into a transcaruncular incision and can be associated with an incision at the lateral canthus that permits the lower lid to swing outwards, thus easing the exposure of the lateral wall. This latter association, first described by McCord in 1981, is known as ‘swinging eyelid’23. This combination of periorbital incision permits an easy approach to the orbital floor, medial, and lateral walls and lipectomies from the inferior orbital quadrants.
The coronal approach is a more invasive procedure which, however, offers the widest access to the lateral orbital wall24, and it may have distinctive advantages in a number of situations including the presence of remarkable periorbital swelling or conjunctival chemosis, the necessity of minimizing the number of periorbital incisions, or the necessity of extensive manipulation of the lateral wall (including its rim)25,26. Through a coronal incision, brow lift, and correction of frontal/glabellar rhytids, which are often necessary in patients with GO, can be performed simultaneously with orbital decompression, thus favorably speeding up the timing of rehabilitative surgery25,27.
Orbital decompression by transinferior fornix/transcaruncular/swinging eyelid: surgical technique






Fig. 54.5 Transinferior fornix decompression. Phases of surgery (A–F); for explanations see the main text.

Fig. 54.6 Orbital fat removal. Lipectomies can be performed alone or may precede or follow osteotomies in the course of the same intervention. Preventive fat removal can be sufficient to adequately reduce exophthalmos and can aid bone exposure, thus easing the possible osteotomies that may follow. Fat removal following bone decompressions aids to increase the reduction in exophthalmos. (A) Removal of fat from the inferior lateral orbital quadrant as part of a combined transinferior conjunctival fornix inferomedial bone/inferior lateral fat decompression procedure. (B) Removal of fat from the inferior medial orbital quadrant during an extensive three-wall/fat decompression procedure performed through a combined transinferior conjunctival fornix and coronal approach.
Reproduced with permission: Baldeschi L. Orbital decompression. In: Wiersinga WM, Kahaly GJ, eds. Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers, 2nd edn. Basel: Karger, 2010:171–88.
Postoperative care
At the end of surgery a pressure bandage is applied on the operated eye/s, after absence of bleeding is assessed. End-perforated wound drains are usually not required. Pupil reflexes, visual function, ductions, and versions are checked when the patient is awake postoperatively, then daily up until the morning of postoperative day 2, when the patient is discharged from the hospital. Pressure bandages are removed and reapplied daily after clinical examination up until discharge. At surgery, postoperative day 1 and 2 intravenous ceftriaxone (1 g/day) is preferably administered; patients leave the hospital without medications except for artificial tears.
Orbital decompression by upper skin crease approach: surgical technique


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