The exact pathogenesis of GO is unknown ⁴^–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 hyperthyroidism^3,¹⁰. This implies that control or correction of these risk factors is an integral part of GO management.

Independently of the complex association with thyroid dysfunction and its treatment, management of GO is difficult: decisions need to be made regarding the need for specific treatment or whether spontaneous regression is likely.

The natural history of GO is of gradual increase in severity followed by a plateau phase then gradual improvement ¹¹. 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 displacement ¹⁴. 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 time ¹⁵. 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 symptoms ⁵. 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 surgery ²².

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.

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.

As an alternative, an upper skin crease incision can be used separately or in combination with the pure inferior fornix or with the swinging eyelid approach in order to address the lateral orbital wall through a wider exposure, and to have access to the upper fat compartments of the medial and lateral quadrants.

The coronal approach is a more invasive procedure which, however, offers the widest access to the lateral orbital wall ²⁴, 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 surgery^25,²⁷.

Orbital decompression by transinferior fornix/transcaruncular/swinging eyelid: surgical technique

Step 1. After the exposure of the inferior fornix by means of a Desmarres retractor and a malleable orbital retractor, the conjunctiva and lower lid retractor complex are transected en bloc with a Colorado needle and the periorbit of the inferior orbital rim is exposed (Fig. 54.5A). During this step, and in all those which imply the use of a Colorado needle, the part of the retractors in contact with tissues should be rubber coated or isolated by means of sterile plasters in order to avoid unwanted burn to the eye, eyelids, and extraocular muscles.

Step 2. The exposed periorbit is incised and the orbital floor and the medial and orbital wall exposed by developing a subperiorbital plane (Fig. 54.5B,C). In order to obtain the best possible exposure of the medial wall, the bony insertion of the inferior oblique muscle may be detached without consequences, and if required the conjunctival incision extended upwards, laterally to the caruncle. A separate incision lateral to the caruncle (transcaruncular approach)²⁸ can possibly be used to address the medial orbital wall when the floor is not to be removed.

Step 3. After this a Frazier suction tip is used to fracture the delicate bone of the medial orbital wall and the floor and Blakesley forceps no. 1 and no. 2 are used to remove bony fragments and mucosa of the sinuses (Fig. 54.5D). The inferomedial osteotomy extends between the frontoethmoidal suture (orbital landmark of the inferior limit of the anterior cranial fossa) and the inferior orbital fissure. The bulla ethmoidalis is opened towards the orbit from the posterior lacrimal crest up to the orbital apex, and then the orbital floor medial to the infraorbital canal is removed from about 1 cm behind the inferior orbital rim up to the posterior wall of the maxillary sinus. The bony infraorbital canal, the floor lateral to it (Fig. 54.5E), and the posterior two-thirds of the maxillary ethmoidal strut can also be removed. The anterior one-third of the strut should be left intact in order to prevent globe displacement and the possibility of medial entropion.

Step 4. In order to implement the effect of decompression fat can be removed from the medial and lateral inferior quadrants (Fig. 54.6). An adequate exposure attained by the assistant, by means of electrically insulated Desmarres retractor and different sized malleable orbital spatulas, aids the surgeon to perform the lipectomy by exerting gentle traction on the fat with fine toothed forceps simultaneously dissecting it with a Colorado needle. Fat can be removed up to the limit of the intraconal space (see Fig. 54.3). Lipectomies can also precede step 2 in order to aid subsequent bone exposure, and can be alone sufficient to attain an adequate decompression effect.

Step 5. If necessary the lateral wall can be removed, starting the osteotomy from the anterior portion of the inferior orbital fissure by means of bone-nibbling rongeurs and a surgical high speed drill equipped with a cutting-burr or a diamond-burr tip. In order to aid the removal of the upper part of the lateral orbital wall, and in particular its anterior–superior portion, Stevens scissors can be used to perform a lateral canthotomy, detaching the inferior crus of the lateral canthal tendon from its bony insertion (swinging eyelid approach).

Step 6. At the end of the procedure the periorbit is usually incised or removed in order to allow the required prolapse of fat and muscles into the newly created spaces. On occasion, if a conservative reduction of exophthalmos is required, the periorbit can also be left intact.

Step 7. The pure transinferior fornix incision is not sutured; tissues are simply repositioned (Fig. 54.5F). Where the conjunctival incision is extended upwards, laterally to the caruncle, such an extension may require two to three interrupted 8.0 absorbable sutures to adequately reapproximate the conjunctiva. Where a lateral canthotomy and inferior cantholysis is performed, the lateral canthal tendon should be reapproximated to the periosteum of the lateral orbital rim with a 5.0 long acting absorbable suture. Orbicularis muscle and skin are closed in layers respectively with 6.0 long acting absorbable and 6.0 non-absorbable sutures, or at the same time by means of 6.0 vertical mattress non-absorbable sutures. Skin sutures can be removed 5–7 days after surgery; vertical mattress sutures are removed later, approximately at postoperative day 10.

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.

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

Step 1. After having marked the upper skin crease, an incision through skin and orbicularis is performed with a no. 15 Bard–Parker blade. It should overcome the lateral orbital rim for not more than 5–10 mm, and can be limited to the lateral

of the upper skin crease if the upper medial fat compartment is not to be addressed. On occasion it can be joined to the lateral canthotomy of a possible swinging eyelid approach to implement the exposure of the surgical site (Fig. 54.7A).

Step 2. A preseptal plane is developed and the superior lateral arcus marginalis exposed. The periosteum is incised along the superior lateral orbital rim, and the periorbit elevated in order to expose the superior and lateral orbital walls (Fig. 54.7B,C).

Step 3. The bony edge of the superior and lateral orbital rim are removed with a surgical high speed drill equipped with a cutting burr in order to implement exposure of the superior and lateral orbital walls (Fig. 54.7D).

Step 4. Zygomatic temporal and facial neurovascular bundles are coagulated by means of diathermy forceps and the lateral orbital osteotomy initiated with a surgical high speed drill equipped with a cutting-burr or a diamond-burr tip. A first sulcus is carved from just inside the lateral orbital rim, to the head of the superior orbital fissure (see Fig. 54.7D). It should run parallel to the plane of the orbital roof. A second sulcus is then carved from the head of the superior to the head of the inferior orbital fissure. The bone interposed between the two sulci and the lateral orbital rim is then removed leaving a small frame of bone at the level of the orbital rim. Finally the osteotomy is carefully extended superiorly and deeply by removing the spongious bone of the greater wing of the sphenoid up to its inner table or small spots of dura mater encephali are exposed.

Step 5. In order to implement the effect of decompression, fat can be removed from the medial and to a lesser extent from the lateral superior quadrant. After the orbital septum has been incised with the Colorado needle or with fine pointed scissors, the removal of the fat is achieved as described above for the transinferior fornix, transcaruncular, swinging eyelid approaches (Fig. 54.8). The lipectomy can also precede step 2, and can be alone sufficient to attain an adequate decompression effect.

Step 6. At the end of the procedure the periorbita can be incised, removed or left intact.

Step 7. The skin only is closed with a continuous spiral running suture in 6.0 non-absorbable material, which can be removed 5–7 days after surgery. Where the removal of the lateral wall is not associated with other osteotomies which may favor drainage towards sinuses and nose, a 2.8 mm diameter end-perforated wound drain is positioned into the orbit at the site of surgery. Unless there are complications, it is removed after 12–18 hours. If the upper skin crease incision was associated with a swinging eyelid approach, lateral canthotomy inferior cantholysis are closed as described above for such a procedure (see previous technique, step 7), and the inferior fornix incision is left unsutured.

Fig. 54.7 (A–D) Lateral wall decompression through an upper skin crease approach: for details see the main text.

Fig. 54.8 Transcutaneous septal superomedial orbital fat decompression, surgical site. The skin and the orbicularis oculi muscle are retracted. An aperture through the orbital septum (white arrow heads), which is kept open with a cotton tip applicator following partial removal of the superomedial extraconal fat, shows (from left to right): medial margin of the levator aponeurosis (blue arrow), anterior margin of the tendon of the superior oblique (white arrow), intraconal fat (yellow arrow), and belly of the medial rectus muscle (red arrow).

Postoperative care

Except for the handling of the possible wound drains, it is similar to that described for orbital decompression by the transinferior fornix, transcaruncular, swinging eyelid approach.

Orbital decompression by coronal approach: surgical technique

Step 1. After razoring the hair and marking, a coronal incision is made with a No. 10 blade from ear to ear, 4–5 cm behind the hairline (Fig. 54.9A). In the central portion of the skull a subperiosteal plane is created by blunt dissection and laterally a surgical plane is bluntly developed between the deep and the superficial temporalis fascia (Fig. 54.9B). Laterally and inferiorly, where the deep temporalis fascia divides into a deeper and a more superficial layer to enclose Yasargil’s superficial temporal fat pad (which contain the branches of facial nerve), the surgical dissection is carried out alongside the medial surface of the deeper division of the fascia (Fig. 54.9C). The forehead flap thus created is then turned down in order to expose the superior and lateral orbital rims. Bleeding from the wound edges is controlled with Raney scalp clips (see Fig. 54.9B,C).

Step 2. The supraorbital nerve (Fig. 54.9D) is set free by chiselling its bony foramen when present (Fig. 54.9E) and the periorbita, including the trochlea, is dissected off the orbital bones.

Step 3. The temporalis muscle is dissected from its anterior origin with a No. 10 blade and periosteal elevators, leaving sufficient tissue for suturing at the end of surgery (Fig. 54.9F). In this way the lateral orbital wall is exposed.

Step 4. A small osteotomy is chiselled behind the lateral orbital rim, then it is extended inferiorly up to the inferior orbital fissure, and superiorly and posteriorly up to the inner cortical bone of the sphenoid, by means of bone-nibbling rongeurs and a surgical high speed drill equipped with a cutting-burr or a diamond-burr tip. Bone removal is terminated when small spots of dura mater of the anterior and middle cranial fossa are exposed through the thin inner cortical bone of the greater wing of the sphenoid. During these surgical maneuvers, the soft orbital tissues and the temporalis muscle are retracted and protected with different sized malleable orbital retractors (Fig. 54.9G).

Step 5. A Frazier suction tip is used to fracture the delicate bone of the medial orbital wall and the floor, and Blakesley forceps Nos. 1 and 2 are used to remove bony fragments and mucosa of the sinuses. The bulla ethmoidalis beneath the frontoethmoidal suture is opened toward the orbit from the posterior lacrimal crest up to the orbital apex, and then the orbital floor medial to the infraorbital canal is removed from 0.5–1 cm behind the inferior orbital rim up to the posterior wall of the maxillary sinus. The posterior two-thirds of the maxillary ethmoidal strut are removed creating a wide antrostomy, whereas the anterior one-third of the strut is left intact to prevent globe displacement and medial entropion. Bone nibbling and different sized Blakesley forceps are used for these maneuvers.

Step 6. The periorbita is addressed as for the above described techniques.

Step 7. The temporalis muscle is sutured back into position with four to five interrupted 2-0 non-absorbable sutures and, after the insertion of a 3.3 mm diameter end-perforated wound drain into each temporalis fossa, the scalp incision is closed with iron staples (Fig. 54.9H). They are removed 10–14 days after surgery.

Fig. 54.9 Orbital decompression by coronal approach. Surgical phases (A–H); the explanation is in the text.

Postoperative care

At the end of surgery a pressure bandage is applied to the forehead and on the operated eyes. Pupil reflexes, visual function, ductions, and versions are checked when the patient is awake postoperatively, then daily. Forehead pressure bandage and end-perforated wound drain are removed 12–18 hours after surgery unless there are complications. Eye pressure bandages are removed and reapplied daily after clinical examination up until postoperative day 2. Patients are discharged between postoperative days 2 and 5. At surgery, and postoperative days 1 and 2 intravenous ceftriaxone (1 g/day) is preferably administered; intravenous glucocorticoids (metilprednisolone 250–500 mg) can also be administered at surgery to reduce postoperative face swelling. Patients leave the hospital without medications except for artificial tears.

Possible complications of decompression surgery

Despite their invasiveness, bone and fat orbital decompressions are safe procedures. Common complications of this surgical approach are consecutive strabismus, infraorbital hypoesthesia and sinusitis, lower lid entropion, and eyeball dystopia. Leakage of cerebrospinal fluid, infections involving the central nervous system, damage to the eye and optic nerve or their vasculature, cerebral vasospasm, ischemia, and infarction are severe but rare events. Reactivation of GO after rehabilitative bony orbital decompression and infraorbital pain are other rare complications ²².

In addition to complications common to orbital decompressions in general, different surgical approaches may carry the risk of specific complications. The coronal approach leaving the eyelid undisturbed is less likely than periorbital incisions to create complications, which may potentially be harmful to the eye. Periorbital scarring with iatrogenic lid retraction and cicatrical lagophthalmos, eyelid margin malpositions, and ptosis (although rare) are more likely to occur with periorbital incisions. On the other hand, temporal bossing, damage to the frontalis nerve, scarring and alopecia at the site of the scalp incision, or effects upon ischemic areas of the frontal flap after healing by secondary intention, may complicate the coronal approach ²⁵.

Most of the possible complications cannot be predicted and their prevention is based on recommendations which are not specific in nature, and which include careful manipulation of the orbital content, accurate dissection of the orbital fat, and avoidance of expandable hemostatic agents and/or extensive use of diathermy within the orbit ²².

Complications with known pathogenesis such as sinusitis can be simply prevented by taking care to create adequate sinus aeration as a part of the surgical procedure at the time of bone decompression ²².

The occurrence of other complications, namely sinuses atelectasis, infraorbital hypoesthesia or pain, eyeball dystopia leakage of cerebrospinal fluid, and possible consecutive infectious involvement of the central nervous system, can be reduced by means of accurate evaluation of preoperative imaging, adequate planning of surgical intervention, and the use of prophylactic antibiotics ²².

Correction of lid retraction

In GO, upper and lower lid retraction are due to a combination of inflammation, fibrosis, adrenergic stimulation, and restriction of the vertical rectus muscles. Exophthalmos also contributes in increasing the eyelid aperture by displacing either the upper or the lower lid ¹⁴. Correction of upper or lower lid retraction implies recession of the lid retractors. Spacers are not essential for upper lid-lengthening procedures, but are necessary to provide height and the necessary stiffness to support the lower lid against gravity when it is severely retracted. A number of autologous, homologous, xenogenic, and synthetic materials have been used including ethanol-preserved donor sclera, upper lid tarsus, cartilage grafts, porous polyethylene, polytetrafluoroethylene or polyester meshes. Autogenous hard palate mucosal graft is relatively easy to obtain, is similar to lower lid tarsus in terms of thickness and stiffness, has a mucosal surface, has no risk of rejection, and undergoes minimal shrinkage following grafting. Ophthalmic complications of hard palate mucosal grafting are uncommon and morbidity at the donor site is rare²⁹. Hard palate mucosal graft providing structural and epithelial elements represents an elective material for posterior lamella augmentation in lower lid lengthening. Homologous acellular dermal matrix with an appropriate consistency for posterior lamella augmentation is an optimal alternative to hard palate mucosa; unfortunately it is not readily available throughout Europe.

The treatment of persistent upper eyelid retraction is mainly surgical and far less predictable than that of lower lid. The medical therapy with topical alpha-blocker eye drops or with post-ganglial adrenergic blocker drugs is scarcely effective or connected to side effects. Botulinum toxin is an effective option, but its effect is limited in time and repeated injections are necessary. Temporary under- or over-correction, deficit of elevation, and paralysis of the orbicularis muscle can occur. The latter two being highly undesirable side effects in GO patients who are at risk for corneal exposure.

Upper eyelid lengthening is one of the last steps of the long-lasting surgical rehabilitation of patients with GO and, despite several surgical techniques being currently in use, an ideal method is lacking. For this it can be worth considering the use of the simplest and less time consuming technique. Sutureless Müllerectomy, transconjunctival or transcutaneous (blepharotomy) free en-bloc recession of levator complex and conjunctiva fulfil these criteria and can be used systematically for mild to moderate and for moderate to severe degrees of upper lid retraction respectively.

Lower eyelid lengthening

Hard palate mucosal graft harvesting: surgical technique

Selection of candidates prior to surgery is mandatory. Any lesion suspected for malignancy should be biopsied; oral candidiasis in immunocompromised patients should be cured in order to avoid delayed granulation at the donor site; the presence of exostosis such as torus palatinus or prominent palatal roots of teeth should be considered since the thin overlying mucosa of these areas may lead to unwanted periosteal or root damage at surgery. The presence of a bifid uvula or a muscular diastases of the soft palate can be an important clue to an underlying bony palatal cleft that is not otherwise evident, but which represents an absolute contraindication for mucosal harvesting. The presence of small isolated clefts of the bony palate are asymptomatic, and may escape clinical detection. Besides infectious causes and, iatrogenic damage to the bone and periosteum, oral–nasal fistulas may also develop from such malformations. Alcoholic and immunocompromised patients can also be at risk for delayed granulation at the surgical site and fistulas.

Local anesthesia is possible, but general anesthesia is preferred for the patient’s comfort.

Step 1. The mouth is opened with a Johnson mouth spreader, the hard palate is dried, and a donor area of approximately 10 × 20–25 mm is marked between the median raphe and the alveolar process. It should not extend posteriorly behind the first molar. At this level the submucosa is well-defined and partial thickness mucosal dissection can be easily carried out anteriorly to the neurovascular bundle emerging from the greater palatine foramen. The latter can in fact be located medially to the third or to the second molar. Areas close to the gingival border or to the median raphe are not suitable as donor sites since the risk of bone necrosis secondary to periosteal damage is much higher here: at these levels the mucosa is in fact directly attached to the periosteum without interposition of the submucosa (Fig. 54.10A). Also in the case of general anesthesia, the donor site is injected with lidocaine 2% with epinephrine 1 : 80 000 to favor hemostasis, and to elevate the mucosa from the underlying fatty tissues thus aiding dissection. Local anesthesia contributes to reducing immediate postoperative discomfort.

Step 2. Harvesting is carried out first using a No. 15 Bard–Parker blade to incise the marked area, then with a disposable corneal slit knife and fine toothed surgical forceps to dissect the submucosal plane and elevate the graft. The use of suction and a tongue depressor by an assistant will greatly aid in this phase. The maneuver is also facilitated if the surgeon stands on the side opposite to the site of surgery. Periosteum should not be bared and excessive diathermy should be avoided. In the case of persistent bleeding, injection of saline, possibly with epinephrine can be useful to induce hemostasis and vasoconstriction. In the case of bleeding from major vessels, ligation can be considered.

Step 3. At the end of surgery a rolled petroleum jelly or hemostatic gauze inserted into the mucosal defect and kept in place with a previously prepared palatal shell can be used to control the oozing at the donor site. In cases of more plentiful bleeding, digital pressure on the shell can be applied for some time and fibrin glue used if necessary.

Step 4. The mucosa of the graft, consisting of epithelium and thick collagenous lamina propria, is then prepared for the recipient site by removing the fatty submucosa.

Fig. 54.10 (A) Location and aspect of the donor sites of the hard palate mucosal grafts 3 weeks after surgery; the defects at the site of harvesting have almost completed the healing process by granulation. (B) A custom-made acrylic palatal stent.

Reproduced with permission. Baldeschi L. Correction of lid retraction and exophthalmos. In: Brewitt H, Geerling G, eds. Scientific Evidence and Guidelines for the Clinical Management of Dry Eye Associated Ocular Surface Disease. Basel: Karger, 2007:103–26.

Postoperative care

Broad-spectrum systemic antibiotics and antiseptic mouthwashes are recommended for 1 week after surgery. Soreness at the donor site for a few days after surgery is common especially during eating; custom-made acrylic palatal stents, which aid hemostasis and facilitate granulation, can increase the patient’s comfort (Fig. 54.10B).

Lower lid lengthening: surgical technique

Step 1. Topical oxybuprocaine HCl 0.4% drops are applied to the eye, then 0.5–1 ml of lidocaine 2% solution with epinephrine 1 : 80 000 is administered subconjunctivally along the inferior margin of the tarsal plate; 0.25 ml of the same anesthetic is administrated subcutaneously into the center of the lower lid.

Step 2. A 5-0 traction suture is placed in the center of the lower lid along the eyelid margin and the eyelid is everted over a Desmarres retractor. An infratarsal incision is done for the whole width of the tarsal plate with a No. 15 Bard–Parker blade and the conjunctiva lower lid retractor complex is transsected en bloc (Fig. 54.11A).

Step 3. A preseptal plane is then bluntly developed in order to adequately recess the lower lid retractors and conjunctiva. Bleeding is minimal and easily controllable with bipolar diathermy (see Fig. 54.11A).

Step 4. A continuous interlocked suture in 6.0 absorbable material is used to suture the hard palate mucosal graft into place, between the border of the conjunctiva lower lid retractors complex and the inferior border of the tarsal plate, the mucosal surface of the graft facing the eyeball (Fig. 54.11B).

Fig. 54.11 (A) A lower lid everted over a Desmarres retractor in the course of a lower lid-lengthening procedure: the conjunctiva lower lid retractor complex (star) had been recessed from the inferior border of the tarsal plate (arrow). (B) Aspect of a hard palate mucosal graft a few months after a lower lid-lengthening procedure: the graft is integrated between the conjunctiva lower lid retractor complex and the inferior border of the tarsal plate.

Postoperative care

Multiple Steri-strips are applied to the lid skin in order to immobilize the lid and graft in the correct position for a few days after surgery. A bandage is not applied and artificial tears are prescribed to implement the patient’s comfort.

Upper lid lengthening

Sutureless transconjunctival müllerectomy: surgical technique

Sutureless transconjunctival müllerectomy is an effective procedure for the cure of mild degrees of upper eyelid retractions up to 2–3 mm. It does not interfere with the position of the skin crease since surgical dissection is carried out under the levator aponeurosis. It is a fast procedure that takes about 10 minutes per eyelid. Reintervention for under- or over-correction is rare and usually due to surgical mistakes like an incomplete excision of Müller’s muscle or damage to the aponeurosis of the levator muscle. Tear secretion may be reduced after müllerectomy; nevertheless, the risk of dry eye is low, and counterbalanced by the usually good functional and cosmetic results of the procedure.

Step 1. Topical oxybuprocaine HCl 0.4% drops are applied to the eye, then 0.25 ml of lidocaine 2% solution with epinephrine 1 : 80 000 is administered subcutaneously into the center of the upper eyelid. A 5-0 silk traction suture is placed in the center of the upper eyelid along the eyelid margin and the eyelid is everted over a Desmarres retractor. Then 0.5 ml of lidocaine 2% with epinephrine 1 : 80 000 is injected close to the superior tarsal border subconjunctivally and between Müller’s muscle and the levator aponeurosis over the whole width of the eyelid (Fig. 54.12A).

Step 2. A No. 15 Bard–Parker blade or a high temperature battery-powered cautery is used to incise the conjunctiva along and just above the superior tarsal border (Fig. 54.12B).

Step 3. The conjunctiva is separated from Müller’s muscle, first with sharp dissection by means of fine toothed forceps and pointed spring scissors, then bluntly with cotton tip applicators. In this way the superior fornix is approached (Fig. 54.12C). Injections of saline beneath the conjunctiva aid this passage.

Step 4. The tension exerted with the Desmarres retractor is relieved; the Müller’s muscle is grasped with fine toothed forceps at its lateral aspect; a blunt pointed straight scissors is used to buttonhole the Müller’s muscle at this level and to develop a plane between its anterior surface and the posterior surface of the levator’s aponeurosis from the proximal to the distal insertion of the Müller’s muscle.

Step 5. The distal insertion of the Müller’s muscle is gently cauterized, and afterwards severed with scissors. The isolated Müller’s muscle (Fig. 54.12D) is then removed by similarly severing its proximal insertion. At the end of the procedure the conjunctiva is not sutured. This prevents possible suture-related corneal erosions and leaves a natural drainage with reduction of postoperative ecchymosis.

Step 6. If required, additional lengthening can be achieved by weakening the levator aponeurosis. This can be attained by means of several small horizontal incisions which transform the aponeurosis in a kind of network, increasing its vertical length.

Step 7. At the end of the procedure the traction suture which was placed in the center of the eyelid is removed.

Fig. 54.12 Sutureless transconjunctival Müllerectomy. Surgical phases A–D, for explanations see the text. Symbols: upper border of the tarsal plate (arrow), conjunctiva (star), levator aponeurosis (arrow head), Müller’s muscle (dot).

Postoperative care

No bandage is applied, but ice compresses are recommended for a few hours after surgery. Elevation of the head during sleep is advisable in the immediate postoperative period. Artificial tears are prescribed for the patient’s comfort.

Free en bloc recession of conjunctiva–levator complex by anterior (blepharotomy) or sutureless posterior approach

Upper eyelid lengthening by means of blepharotomy was developed by Leo Koornneef, but because of his untimely death he was unable to publish his idea; a large series published by one of his fellows contributed to popularize the technique ³⁰.

Free en bloc resection of conjunctiva–levator complex performed via an anterior or a sutureless posterior approach is an effective technique for the treatment of medium to severe degrees of upper lid retraction. The results are, however, not always predictable and reintervention may be necessary.

Success can be implemented by controlling intraoperative factors with potential effect on the final result. Intravenous sedatives interfere with the alertness of the patient and with his collaboration in setting eyelid position; thus these drugs should be used only when it is strictly necessary. Povidone iodine is an adrenergic-blocking agent. Preoperative irrigation of the conjunctival sac with this solution can potentially result in a paralysis of Müller’s muscle and should therefore be avoided. Paralysis of the levator and orbicularis muscle induced by local anesthetic and the contraction of Müller’s muscle induced by local anesthetics containing epinephrine are variables that may interfere with the position of the upper eyelid. These variables can be partially controlled if the dose of anesthetic, its epinephrine concentration, the sites, and the pressure of injection are maintained constant.

In order to account for the effect of gravity on the eyelid height, this is assessed with the patient in an upright position. The anterior approach gives good exposure; however, meticulous skin suturing is required to avoid formations of fistulas. The posterior approach is unsuitable in severe retraction, which does not allow an eversion of the eyelid, but it is the first choice in dark-skinned patients in whom unpleasant scars may result from transcutaneous approaches.

A full-thickness incision of the conjunctiva–levator complex may easily create a flat eyelid contour, if dissection is carried out too medially. In order to prevent a flat contour a central bridge of conjunctiva can be left at the level of the pupil. Careful medial dissection also prevents possible nasal droops that are often difficult to correct.

In the event of early retraction after surgery, the patient should be instructed to massage the lid downward while looking upward in order to maintain the eyelid at the desired position.

Blepharotomy: surgical technique

Step 1. Topical oxybuprocaine HCl 0.4% drops are applied to the eye, the skin crease is lined with a surgical skin marker. Then 0.5–1 ml of lidocaine 2% with epinephrine 1 : 80 000 is injected close to the superior tarsal border subcutaneously and between the orbicularis muscle and levator aponeurosis over the whole length of the eyelid.

Step 2. An incision is made along the line previously drawn at the level of the upper skin crease through the skin-orbicularis layer using a No. 15 Bard–Parker blade (Fig. 54.13A,B).

Step 3. The wound is kept open by gentle traction by means of fine toothed forceps. The assistant pulls the lower edge downward while the surgeon pulls the upper edge upward. Further dissection is carried out with spring scissors in order to expose the levator aponeurosis and the orbital septum. Bleeding is controlled with bipolar diathermy. The orbital septum is opened along the whole length of the eyelid (Fig. 54.13C).

Step 4. An en bloc, full-thickness levator aponeurosis–Müller’s–conjunctiva incision is carried out just above the upper border of the tarsal plate: the levator–conjunctiva complex is grasped with fine-toothed forceps in the central portion of the upper lid, and, after a gentle localized cauterization, spring scissors are used to start the incision as a buttonhole (Fig. 54.13D,E). From this step to step 7, the assistant keeps the surgical site open by means of a toothed forceps and a Desmarres retractor.

Step 5. Gentle cauterization of the levator–conjunctival complex with diathermy forceps precedes any extension of the first incision (Fig. 54.13F).

Step 6. The buttonhole incision is extended laterally and medially with spring scissors until the desired eyelid level and contour are reached (Fig. 54.13G,H).

Step 7. If a lateral flare is present the deformity is corrected by means of a graded vertical incision through the levator–conjunctiva complex at the level of the lateral horn of the aponeurosis. It should be performed with blunt straight scissors, and should be parallel to the ductules of the lacrimal gland to avoid any damage to these structures.

Step 8. The desired degree of lengthening is checked, against gravity, by closing the skin with a temporary suture positioned in the center of the lid and bringing the patient to a sitting position. At surgery, two to three millimeters of over-correction are advisable in order to compensate for postoperative retraction because of wound healing.

Step 9. When the desired level is obtained, a careful inspection of the wound is carried out and any bleeding point cauterized.

Step 10. The blepharotomy is closed by carefully suturing the skin only, with a running interlocked 6-0 non-absorbable suture, which should stay in place for 1 week (Fig. 54.13I). As for müllerectomy, the conjunctiva is not sutured. This leaves a natural drainage, preventing possible ecchymosis, suture-related corneal erosions, and compensates for possible conjunctival shortening due to long-lasting lid retraction.

Fig. 54.13 (A–I) Transcutaneous blepharotomy, for explanation see the text.

Squint surgery

Double vision due to dysthyroid strabismus is the factor which more than any other adversely affects the quality of life of patients with GO.

Under the definition of dysthyroid strabismus, there is restrictive strabismus due to GO (primary strabismus), and that induced by rehabilitative or functional orbital decompression (secondary strabismus). Primary strabismus can be worsened, or ameliorated by functional or rehabilitative decompression.

Small angle strabismus may benefit from prismatic lenses, while stable, larger angle strabismus requires surgical treatment.

Surgery aims at restoring fusion in the primary position of gaze and then at correcting residual incapacities. In order to achieve these goals, muscle recessions followed by retroequatorial myopexias are usually used. Oblique muscle surgery is rarely necessary. Complications are infrequent.

Surgical techniques are similar to those described in Chapter 57; in the case of dysthyroid strabismus muscle recession is often generous. In such cases, in order to maintain the contact arch, extraocular muscle tendon elongation should be done. It can be attained by means of autologous or donor fascia grafts sutured between the recessed muscle tendon and the eye globe, or more simply by recessing the muscle by means of hang-back loops attained with sutures in 5.0 absorbable material (Fig. 54.15A).

Fig. 54.15 (A) A large recession of the medial rectus muscle by means of hang-back loops attained with a 5.0 absorbable suture in a patient with Graves’ orbitopathy. (B) After a large recession of the inferior rectus muscle, the limbar edge of the base fornix conjunctival incision is recessed to the level of the original muscle insertion.

Long-lasting dysthyroid restrictions are associated with conjunctival scarring; for this reason muscle recessions should preferably be done through a base fornix conjunctival incision, which permits conjunctival lengthening by means of its recession at the end of surgery (Fig. 54.15B).

Because of the enlarged GO muscles, retroequatorial miopexias can be difficult to perform, unless the ‘faden’ is passed through the sclera after having detached the muscle to be treated from its eye globe insertion. In many case this maneuver does not increase the surgical time as often in dysthyroid strabismus retroequatorial miopexias need to be associated with muscle recessions. A double passage of the ‘faden’ into the sclera is advisable in order to increase the strength of the suture the mechanical action of the oversized muscle (Fig. 54.16).

Fig. 54.16 Retro equatorial miopexia. The left inferior rectus muscle is pulled nasally and a 5.0 non-absorbable suture is passed twice through the sclera 14 mm behind the sclero-corneal limbus.

Cosmetic eyelid surgery

Cosmetic periorbital surgery in GO is substantially similar to that for the aging face (see Chapter 51). Skin removal should, however be more conservative in GO, as skin excess is often more apparent than real in the affected patients. Another difference concerns the treatment of the sub eye brow fat pad. Up until recently, in the aging patient, it was sculptured or resected with the unfortunate effect of taking away the natural fullness of the upper eyelid fold. Nowadays, sub-eyebrow fat repositioning is in general preferred in cosmetic surgery, while it continues to be sculptured or resected when, as in GO, it is pathologically enlarged (Fig. 54.17A–C). In light of the anti-vitamin-K action of commonly used thionamides such as methimazole in patients with GO, sculpturing or excision of the sub-brow fat pad can be assisted by the use of a CO₂ laser (Fig. 54.18A–C).

Fig. 54.17 A patient with Graves’ orbitopathy after three-wall orbital decompression by coronal approach. (A, B) The patient just before upper lid blepharoplasty. Enlarged pre-aponeurotic and sub-brow fat pad are present. (C) The patient immediately after upper lid blepharoplasty which included removal of pre-aponeurotic and sub-brow fat pads, and sculpturing of the sub-brow fat pad with a CO₂ laser.

Fig. 54.18 Surgery on patient in Figure 54.17. (A) The pre-aponeurotic fat pad is exposed through the incised septum and (B) excised. (C) The sub-brow fat pad is excised. (A–C) The procedure is carried out bloodess by means of a CO₂ laser.

References

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