CHAPTER 56 Management of post-enucleation socket syndrome
Introduction
The purpose of this chapter is to familiarize the reader with the problems associated with a post-enucleation socket syndrome (PESS) and an acquired socket contraction and to introduce current concepts of their surgical management.
Socket surgery includes a variety of surgical procedures, which are mainly implemented to either substitute orbital volume or augment socket conjunctival lining. This includes the replacement of orbital volume by insertion of secondary orbital implants, which can be either alloplastic or autologous. Alloplastic implant volume is stable; however, with any aggressive dissection and increased intraorbital pressure, existing orbital soft tissue, i.e. orbital fat, might be damaged, resulting in fat atrophy. Alternatively autologous dermofat grafting can be considered instead of alloplastic implants. Any residual volume deficit might then be corrected with an orbital floor implant, if necessary combined with fornix deepening procedures. Finally, in rare cases a dermis-fat graft can be placed in the superior sulcus.
In severely contracted sockets mucous membrane grafting into the fornices will reconstitute socket architecture and improve prosthesis fitting. Finally, lid malpositions like upper and lower eyelid entropion, lower eyelid laxity, or acquired ptosis can be corrected by means of classical eyelid procedures, including Jones entropion repair, lateral tarsal sling procedure or anterior levator resection ptosis repair. Eventually an upper lid blepharoplasty on the contralateral eyelid is helpful to camouflage some asymmetry. However, for good esthetic and functional results, a close cooperation with the ocularist is not only desirable but also mandatory.
Terminology and pathophysiology
An enucleation of the eyeball causes orbital volume deficiency and changes in the orbital soft tissue architecture leading to the clinical picture of a post-enucleation socket syndrome (PESS). The term PESS was introduced by Tyers and Collin with the clinical features of an enophthalmos of the artificial eye, a deep upper eyelid sulcus, lower lid laxity, and eyelid malpositions such as ptosis or lid retraction1. Smit2 then added the feature of a tilting of the prosthesis associated with an anterior-to-posterior and superior-to-inferior rotation of orbital tissues. This reallocation of orbital soft tissues in combination with a lack of eyeball volume – and not orbital fat atrophy – is responsible for the development of a PESS3. This undesirable condition of PESS can be prevented best by a primary insertion of an adequate and safe orbital implant (see Chapter 55).
A contracted socket is defined as the shrinkage and/or shortening of (soft) tissues in the anophthalmic orbit, which makes the fitting of a satisfactory cosmetic prosthesis impossible4. Pathophysiologically this is a consequence of conjunctival and subconjunctival scar formation involving fibroblasts and their contraction after wound healing or inflammation. Causes are mechanical and surgical trauma, thermal and chemical burn, irradiation and – probably most commonly – chronic inflammation. The latter is mainly due to prosthesis problems with poorly fitted, too large, and not well-maintained artificial eyes with rough surfaces and sharp edges. At early stages therefore it is promising to administer conservative treatment using lubricants, anti-inflammatory therapy (i.e. topical steroids), and prosthesis modification or polish.
Clinical features
With PESS, patients often are suffering from an undesirable appearance with an enophthalmos of the artificial eye, a deep upper eyelid sulcus, which can be very marked, lower eyelid laxity, and eyelid malpositions such as ptosis or lid retraction. However, a prosthesis can always be fitted and worn without spontaneous loss. This might be different in contracted sockets with its various degrees of severity. Mild socket contraction is associated with lagophthalmos, entropion, and lower eyelid retraction due to the contraction of the conjunctiva and underlying orbital connective soft tissue compartments causing shallow fornices, where an artificial eye still can be retained. More advanced cases show a loss of fornices, provoking spontaneous loss of the artificial eye, to severely contracted sockets with a complete loss of the conjunctival lining and the formation of symblephara or even ankyloblepharon. This makes wearing of an artificial eye impossible. Often secretion and chronic discharge are associated. Features of both conditions, PESS and a contracted socket, might be present in any combination.
Indications and goal of surgery
The aim of socket surgery in anophthalmic patients is to minimize this disfigurement, related to either volume or lining deficiency in the socket. In consideration of the significant psychological strain of patients showing features of a post-enucleation socket syndrome due to an unsatisfactory and unsightly appearance, any primary surgery for eyeball removal plays a crucial role. Choosing the optimal surgical procedure with the best and safest orbital implant available can prevent the patient from wasting long hours of unsuccessful prosthesis fitting and life long suffering.
Fundamental principles
In the presence of PESS, however – optimal prosthesis fitting provided, ideally in close cooperation with the ocularist – the patient can be offered a range of surgical interventions in order to maximize the wearing comfort of the artificial eye and to minimize discomfort and disfigurement. Depending on patients’ clinical features and appearance, and their complaints and ambitions, different defects can be addressed. This includes orbital volume deficiency, eyelid malpositions like lid laxity, entropion, ptosis or retraction, and socket contraction. Often more than one procedure might be necessary to improve or even solve the problem. In order to obtain the best possible result with a minimum of effort, it might then be mandatory to keep to a certain sequence of procedures. However, surgical rehabilitation in severe cases of the volume deficiency or socket contraction can be very challenging for both the patient and the surgeon.
Preoperative assessment
Before any surgical correction, it is mandatory to evaluate the patient’s concerns. What bothers the patient most? Is it pain and discomfort? Is it mainly the appearance, or does he or she even suffer from a spontaneous loss of the artificial eye? Is it a long ongoing history? For the past medical history, matters of interest are previous surgeries, orbital implants, irradiation and fittings of an artificial eye. During clinical examination, the following aspects are assessed: overall impression, the position and the stability of the artificial eye (spontaneous loss?) or, and whether the patient is able to wear prosthesis at all? On gross examination and slit-lamp exam, one should look for an enophthalmos of the prosthesis and the configuration and depth of the upper eyelid sulcus. Inside the socket, it is of interest to examine the fundus and the fornices, the type of lining (conjunctiva, mucous membrane, skin?), and whether conjunctival/subconjunctival scars or symblephara are detectable. Is there a volume deficit? Is an orbital implant palpable? The situation of the eyelids should be respected. Do they close properly, or are lagophthalmos and retraction present? Is there any horizontal laxity detectable, and how is the eyelid margin configured? Finally, the condition of the artificial eye should be appreciated. This includes size and configuration, the surface with possible deposits, and the edges of the prosthesis.
Post-enucleation socket syndrome
Surgical principles (volume augmentation)
In patients with a classical post-enucleation socket syndrome with volume deficiency only, volume replacement is the first measure. Such a volume augmentation is promising if the soft tissue components in the socket are still soft and malleable and not constricted due to significant fibrosis. Pushing the orbital content back into the socket with a finger pressing onto the lower eyelid can easily check this: if the upper sulcus is then filled by any existing orbital fat, an alloplastic orbital implant will have a good chance of improving the problem.
If no orbital implant is present, secondary implantation using alloplastic implants or autologous transplants is used. However, extensive dissection in the deep orbital soft tissues in order to locate and visualize the extraocular muscles should be avoided. It usually causes tissue alteration and persistent damage. It has been shown that repeated and extensive surgical dissection will cause a significant shrinkage of orbital fat tissue with a loss of orbital volume, undoing any volume augmentation initially intended3. For this reason it is advisable to avoid any gratuitous dissection in the soft tissue planes of the orbit, including the exposure of the extraocular muscles. An alloplastic implant should be positioned intraconally in the depth of the orbit, possibly behind the remnants of the posterior Tenon’s capsule.
Technique (secondary alloplastic orbital implant insertion)
Before the surgery, which usually is performed under general anesthesia, the motility of the socket without an artificial eye is checked during slit-lamp examination. The virtual sagittal axis, along which the anterior surface of the socket is moving, is marked on the conjunctival surface with a marker pen. At the beginning of the procedure, the conjunctiva is opened horizontally at this mark. Scar tissue, if present, is dissected and excised. This is followed by mainly blunt dissection into the depth of the orbit to create a space to accommodate the orbital implant. This should be performed in as atraumatic a manner as possible to avoid damage to the soft tissues and bleeding, with only minimal use of cauterization. A solid or porous orbital implant – according the preferences of the surgeon – is wrapped with donor sclera or Vicryl™ mesh. The size of the implant depends on the amount of volume deficiency and the space available, and sphere diameters may vary from 18 to 22 mm. However, undue pressure in the socket caused by a too large an implant increases the risk of extrusion. It can be helpful to use a sizer. Also the correct insertion technique of the secondary orbital implant is mandatory to prevent later exposure. To avoid gradual tissue restitution after forced ball implantation (‘cactus syndrome’) as described by Rose5, a polythene glide can be used – comparable to an injector in intraocular lens implantation. The best position for the implant is deep into the apex of the orbit, behind the posterior Tenon’s capsule.
Closure of Tenon’s capsule over a baseball implant may result in shortened fornices exacerbating a pre-existent mild form of socket contraction. To avoid this, the conjunctiva is dissected far into the fornices. However, in cases with shallow fornices baseball implantation should be avoided and secondary dermis-fat grafting is preferred as a safe alternative6.
Surgical principles (dermofat graft [DFG])
A dermis-fat graft consists of de-epithelialized skin (dermis) with adjacent fatty tissue, preferably harvested from the gluteal area. It is transplanted into the socket, where it is attached to the orbital soft tissue components (Fig. 56.1
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