Management of Type III fibular hemimelia continues to be controversial [15
]. Our rationale from the current study was to assess the results of using the Ilizarov technique in this group of patients and to document the complication rate and incidence of reoperation. We hoped to assess if the long period of treatment and high rate of complications associated with Ilizarov application and preservation of the limb outweigh the results of amputation and use of below-knee prostheses [2
Naudie et al. [15
] recommended clearly explaining to the families that amputation is a reconstructive procedure and should not be considered a failure of treatment. When families refuse amputation, however, we must respect their decision, but the short-term and long-term complications of repeated lengthening must be clearly explained.
The limitations of our study include that the interobserver and intraobserver reliabilities of the measured outcome were not assessed. Our assessment of the function and patient satisfaction are not validated. Also the study group is highly selected; the criteria for inclusion are variable and subjective. The indication for surgery in some patients is psychologic and not based on traditional criteria. There is concern for the families for the psychologic factor regarding the choice of treatment and for the patient during the course of treatment, and we did not precisely address that in our methods. This does not distract us from our primary conclusion that preservation of the limb outweighs the amputation.
This study showed that good results can be obtained with limb lengthening and deformity correction with the Ilizarov method. More than half of our patients were satisfied and most achieved limb-length equality, are able to walk, have minimal pain, and are quite active. With gradual distraction, good length was obtained with fewer problems. Moreover, the highly versatile system might overcome most complications. This is true even though amputation was the end result for two patients.
We studied our lengthening group to determine which preoperative factors were associated with a good result. Valgus angulations of the ankle seem to be a predictor of a poor result. Of eight patients with ankle valgus, four were not satisfied and the others had pain and/or activity restrictions.
Our results coincide with those of other authors having obtained good results with repeated lengthening. In a retrospective comparative study, Jawish and Carlioz [12
] reported good correction of the foot in 60% of cases of fibular hemimelia treated by lengthening. Miller and Bell [14
] reported no serious complications and no late amputations in 12 cases that underwent lengthening by the Ilizarov technique. Catagni et al. [6
] prefer to preserve the foot and use repeated lengthening and correction for limb-length discrepancies, angular and rotational deformities, and foot and ankle deformities. Hany [8
] achieved satisfactory results in three of four patients with Type II fibular hemimelia in his series. Walker et al. [20
] stated that patients who were treated with lengthening started out with more residual foot rays and more fibular preservation than the amputees. However, all these authors agreed that there are many potential problems, obstacles, and complications associated with repeated lengthening.
Patients who underwent amputation after several failed attempts at salvage were at high risk for emotional problems [9
]. Determining which patients may fare better with immediate amputation is important. Generally, these are patients with a nonfunctional foot or a limb-length discrepancy greater than 20 to 30 cm. For the severely affected child with a predicted limb-length discrepancy greater than 25 cm at maturity and with a poor foot and ankle, amputation generally is thought to be the best option [5
]. For patients with less severely affected limbs, particularly those with a predicted limb discrepancy of 10 cm or less and with a foot with three or more rays, which can be made plantigrade, limb reconstruction is recommended. Controversy remains regarding the best way to manage children with an intermediate deformity [5
]. We agree [16
] that amputation should be the treatment of last resort if the limb can be safely and reliably lengthened, reconstructed, and preserved. Whenever feasible, efforts should be made to involve the child in the decision-making process, especially when an ablative procedure such as amputation is being considered.
Some authors do not believe cost should play a deciding role in determining the treatment plan [13
]. Despite higher initial costs resulting from greater medical/surgical intervention, other authors have reported if the lifetime costs of prostheses are included, lengthening is less expensive than amputation [2
Despite this initial success, the long-term results of lengthening can be unpredictable. Tibiae that have been lengthened to correct a congenital deficiency subsequently can have a slowed growth rate [17
]. Also, substantial progressive angular (osseous) deformities can occur after lengthening for fibular hemimelia [7
Although lengthening in Type III fibular hemimelia is difficult and requires multiple surgeries, elongation with alignment and foot correction using the modified Ilizarov technique may offer an alternative to amputation. New studies, highly specialized centers, and professional staff are required to minimize the potential complications.