Knee Reconstruction in Syndromes With Congenital Absence of the Anterior Cruciate Ligament

Abstract

The authors review their experience with four patients with congenital deficiency of the anterior cruciate ligament (ACL) who underwent surgical treatment of symptomatic knee instability at a mean age of 15.8 years (range 14-17 years). Associated syndromes included fibular hemimelia, congenital short femur, and an unspecified skeletal dysplasia. All patients had undergone multiple previous realignment and leg lengthening procedures and were skeletally mature at the time of the reconstruction. All four patients underwent ACL reconstruction, and one patient underwent concomitant posterolateral corner reconstruction. One patient required an osteochondral autograft transplant procedure in addition to ACL reconstruction. Hypertrophy of the meniscofemoral ligament of Humphrey was a consistent anatomic finding at surgery. The patients were followed for a mean of 38 months (range 26-58 months) after the reconstruction. The mean preoperative Lysholm II score was 38 (range 28-56); the score had improved to a mean of 81 (range 78-93) at the latest follow-up. The authors conclude that reconstructive surgery is a viable option for restoration of knee stability and function in appropriately selected patients with congenital ACL deficiency.

Ligament Reconstruction in Congenital Absence of the Anterior Cruciate Ligament

“…Good results have been reported with ACL reconstruction surgery in these patients. For example, Sonn [15] reported two reconstructions in monozygotic twins with good results at 32-month follow-up and Gabos [6] reported four ACL reconstructions with 31-month follow-up with excellent results.…”

“…Furthermore, there are some reports of grossly hypertrophied and anteriorly positioned meniscofemoral ligament of Humphrey which could play a role in diminishing instability symptoms in the setting of PCL deficiency [6].…”

“…Given the rarity of the condition, there is no consensus regarding the optimal therapeutic approach, although most studies conclude that non-surgical options are the appropriate management despite early degenerative joint disease. Anterior cruciate ligament reconstruction is indicated when symptoms of instability appear [6]. This is a report on two cases of congenital absence of the ACL, one with a femoral length discrepancy and another with a lateral femoral condyle hypoplasia and posterior cruciate ligament (PCL) dysplasia.…”

Congenital absence of the cruciate ligaments

“…Other authors report favourable results with cruciate ligament reconstruction so far, but the long-term outcome of these knees is unknown [8,28]. Literature teaches us that the need for a knee replacement seems to be inevitable before or after and during the life of a patient without cruciate ligaments [16].…”

The Knee: MR Imaging of Uniquely Pediatric Disorders

“…Most cases are sporadic, bilateral, and combined with other malformations such as fibular hemimelia, or a congenital short femur, or occur in association with a skeletal dysplasia. 50 The most common presentation is knee laxity and pain. MR imaging demonstrates an abnormal shape of the tibial spines and dysplastic femoral condyles, absent (Fig.…”