Craniofacial clefts are atypical congenital malformations occurring in ~1 to 5 per 100,000 live births.1,2,3,4,5,6,7
The number 5 cleft of the Tessier classification system is an extraordinarily rare facial cleft, representing ~0.25% of all facial clefts.8,9
Due to the low incidence and prevalence of this abnormality, there are a limited number of reported cases in the English-language literature, which infrequently include long-term follow-up. We hereby present a long-term follow-up report on managing late deformities associated with this craniofacial malformation 40 years following initial surgical management.
Like most craniofacial deformities, establishing a bony skeleton of appropriate dimensions and in the proper location will facilitate subsequent soft tissue reconstruction and enhance the aesthetic and functional outcomes.7
Adhering to this philosophy, we initially performed intracranial repositioning of the left orbit together with iliac crest bone graft to reconstruct the orbital rim and floor. A known disadvantage of bone grafts is the unpredictable rate of bone resorption. It is hard to pinpoint the cause (bone resorption versus displacement), but several years after the original bony reconstruction, the orbital floor needed further augmentation by rib grafts.
Successful management of ectropion depends on the underlying etiology. In general, ectropion can be attributed to either lower-lid laxity, lack of lower eyelid support, tissue deficiency, or a combination of these factors. Minor cases of ectropion can be managed using traditional techniques such as lower-lid resection or lateral tarsal strip. Additional support for the lower eyelid can be achieved by augmenting the inferior tarsal plate with spacer grafts. However, in more severe cases with deficiency of eyelid tissues, as is typical in facial clefts, tissues must be imported into the lower eyelids.10
The cheek advancement flap, first described by van der Meulen in 1985, has been advocated because of its efficacy and favorable scarring.7,8
Other potential regional flaps are the median forehead flap and the nasolabial flap.7,11
Tissue expansion has been promoted by Menard and colleagues, aiming to recruit adjacent healthy tissues.12,13
The expansion allows for good skin color and texture match and ideally a tension-free closure.12
In our patient, a lateral canthoplasty and a nasolabial flap were initially used but with limited success. It was apparent that the patient needed an aggressive removal of the scarred soft tissues and import of additional skin. This was attempted using tissue expanders, postauricular full-thickness graft, upper-eyelid flap, and cheek flaps. Multiple expansions and serial excisions were attempted but with a limited success. Free flaps have the advantage of providing unscarred skin that can be tailored to the demands of the defect, and therefore seemed to be the most logical solution to this problem.14,15
In our case, this allowed correction of the ectropion, vascularized coverage of the rib grafts, and healthy lining of the bony orbit to accommodate the future prosthesis.
We achieved correction of the ectropion and augmentation of atrophied malar soft tissue with an anterolateral thigh flap. The anterolateral thigh flap is supplied by perforating vessels arising from the descending branch of the lateral circumflex femoral artery.16
This flap is advantageous in that it can be harvested as a two-team approach, has a long vascular pedicle with suitable vessel diameter, can be sensate by including the lateral femoral cutaneous nerve, and can be tailored to suit variable defects.17
Its major disadvantages include flap bulkiness (especially if the deep fascia is included in the flap) and color mismatch.18
Our patient did require thinning and recontouring of his anterolateral thigh flap. In addition, serial excision of the skin island of the flap permitted a good aesthetic result.
The patient was counseled that because of his dissatisfaction with the appearance of his left eye, persistent ectropion, and the nonfunctioning globe, that the best aesthetic results could be obtained with an orbital implant. The primary benefit of enucleation and placement of an orbital prosthetic is enhanced cosmesis. Removal of a nonfunctioning globe can also be accomplished with evisceration where the sclera, Tenon's capsule, conjunctiva, and optic nerve are preserved. Although the benefits of evisceration include diminished orbital fat atrophy and implant migration, there is the devastating complication of sympathetic ophthalmia, which can cause bilateral loss of vision.19,20
This occurs when ocular antigens are released during evisceration, which incites an autoimmune response resulting in granulomatous uveitis in the normal eye. The autoimmune response is likely maintained because of the remaining ocular structures and antigens present in evisceration but removed in enucleation. The advantages of enucleation must be balanced against the disadvantages, which include intraoperative hemorrhage and postoperative infection or wound dehiscence.19
Different prostheses are available to camouflage as a normal eye depending on the extent of the deformity. Scleral shells are fitted thin prosthesis placed directly over the globe. These shells are managed similarly to contact lenses and include the sclera and iris. Although these cosmetic scleral shells avoid potential operative complications, attentive hygienic maintenance is required, and the possibility exists for corneal irritation and patient discomfort. Additionally, facial cleft patients represent a unique challenge to designing appropriately fitting lenses due to contour irregularities of the ocular surface and the deficient eyelids.19
An ocular prosthesis replaces the eyeball per se, and an orbital prosthesis replaces the eyeball and part of the surroundings (e.g., the eyelids). In our patient an orbital prosthesis was used. The globe is acrylic resin, and the surrounding orbital prosthesis is composed of silicone-based organic polymer custom colored to reflect the patient's skin tone and texture. The main disadvantage is low tensile strength predisposing the periphery of the prosthesis to considerable wear and tear. An orbital prosthesis is held in place with an adhesive, as opposed to an orbital implant, which is anchored into the bone. This adhesive accelerates the breakdown of the implant and should ideally be changed several times daily. In addition to hygienic maintenance, the orbital prosthesis can loosen or become detached, causing significant embarrassment to the patient. An orbital implant avoids these disadvantages but requires another surgical procedure along with sufficient bone quality to anchor the implant, which may not be present in facial cleft patients.21
Orbital implants carry a risk of extrusion, migration, infection, and bony resorption (creating a lax socket).21,22
The latter yields an improperly fitting implant that can cause chronic inflammation, which eventually results in a contracted socket requiring surgical intervention.21,23,24
In addition, because all forms of prosthetics require long-term care, many patients opt to use a black patch or no prosthesis, limiting their use of the prosthetics to social occasions or family photos.
In conclusion, we present a late presentation of a facial cleft number 5 and the patient's journey through multiple reconstructive attempts. In retrospect, it is clear that more aggressive procedures (i.e., radical excision of scarred tissues, free flaps, orbital enucleation) proved to be more effective and time efficient. This case illustrates the importance of collaboration between the craniofacial surgeon, the microsurgeon, and the prosthetist to achieve an aesthetic outcome.