n

Case Report

Management of ACL Elongation in the Surgical

Treatment of Congenital Knee Dislocation
Kevin E. Klingele, MD; Scott Stephens, MD

abstract
Full article available online at Healio.com/Orthopedics. Search: 20120621-29

Congenital knee dislocation is a rare anomaly associated with a variety of neuromus-

cular diseases and deformities. The etiology of this condition remains unclear, but it is
usually associated with a variety of disorders, such as Larsen’s syndrome, arthrogryposis,
spondyloepiphyseal dysplasia, Ehlers-Danlos syndrome, Down syndrome, and Streeter’s
dysplasia. It is rarely an isolated entity, and 60% of patients with congenital knee dis-
location had additional congenital anomalies, most commonly hip dysplasia. The ideal
method of treatment is debated. No current treatment algorithms address anterior cruci-
ate ligament (ACL) elongation and its role in recurrent deformity or hyperextension.

This article describes 2 patients who underwent open reduction of the knee for recur-
Figure: Anterior-view illustration status post ante-
rent and neglected congenital knee dislocations. An ACL shortening and reinforce-
rior cruciate ligament (ACL) shortening and rein-
ment technique is described. Both patients’ treatment consisted of V-Y advancement forcement using an autogenous iliotibial band.
of the extensor mechanism, soft tissue release, anterior capsulotomy, and posterior
capsulorrhaphy. Anterior cruciate ligament shortening and reinforcement using an
iliotibial band physeal-sparing technique was performed. The technique improved
maintenance of reduction and prevented hyperextension of the knee. Anterior cruci-
ate ligament elongation is an underemphasized anatomical feature associated with
congenital knee dislocation. Due to its role in the prevention of anterior subluxation of
the tibia and its effect on knee stability, incompetence should be addressed at the time
of open reduction. The presence of an intact ACL with a congenital knee dislocation
does not preclude management of anterior instability. Competence of the intact ACL
should be addressed following reduction.

Dr Klingele is from the Department of Orthopaedics, Nationwide Children’s Hospital, and the
Department of Orthopaedics, The Ohio State University, and Dr Stephens is from Mount Carmel West
Medical Center, Columbus, Ohio.
Drs Klingele and Stephens have no relevant financial relationships to disclose.
The authors thank Anthony Baker from The Ohio State University, who created the illustrations for
this article.
Correspondence should be addressed to: Kevin E. Klingele, MD, Department of Orthopaedics,
Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH 43205 (kevin.klingele@nationwide
childrens.org).
doi: 10.3928/01477447-20120621-29

e1094 ORTHOPEDICS | Healio.com/Orthopedics


C
ongenital dislocation of the knee is
a rare disorder initially described
by Chatelaine in 18221 and Bord
in 1834.2 It is seen in approximately 1 of
100,000 newborns and is less common
than congenital hip dislocations.3 The eti-
ology of this condition remains unclear,
but it is usually associated with a variety
of disorders, such as Larsen’s syndrome,
arthrogryposis, spondyloepiphyseal dys-
plasia, Ehlers-Danlos syndrome, Down
syndrome, and Streeter’s dysplasia. It is
rarely an isolated entity, and 60% of pa-
tients with congenital knee dislocation
were found to have additional congenital
anomalies, most commonly hip dysplasia.2
Although no clear pathogenesis has
been found, a variety of anatomical pat-
terns are consistently found in congenital-
ly dislocated knees. The lateral quadriceps
and fascia lata are typically fibrosed, with
a scarred suprapatellar pouch.4 The ham-
strings and collateral ligaments are dis-
placed anteriorly to their normal position.
The patella may be absent or hypoplastic.4
Hypoplasia of the intercondylar notch and
tibial spines, increased posterior slope and
proximal tibial bowing, and valgus align-
ment at the knee may exist.4 Posterior
capsular redundancy is significant. The
anterior cruciate ligament (ACL) may be
absent, elongated, or hypoplastic, and the
posterior cruciate ligament may be short-
ened and tight.5-8 Variable involvement of
the menisci may include discoid menisci
or hypoplastic menisci.9
Treatment modalities range from se-
rial stretching or closed manipulations to
open reduction. Outcomes vary depend-
ing on the severity and type of treatment
required, but several residual problems
have been identified, including extensor
mechanism weakness, recurrent hyperex-
tension, and progressive valgus instability
and angulation.7,10 The cause of the defor-
mity is not well understood and may be
related to failure to address posterior cap-
sular redundancy at the index operation.8
Operative techniques now emphasize
extensor advancement with or without
JULY 2012 | Volume 35 • Number 7
Congenital Knee Dislocation | Klingele et al
1A
Figure 1: Preoperative photograph (A), lateral radiograph in forced flexion (B), and magnetic resonance
image (C) showing anterior tibial subluxation and elongation of the anterior cruciate ligament.
femoral shortening, associated soft tissue
release and transposition to obtain reduc-
tion, and the addition of a posterior cap-
sulorrhaphy. Few descriptions address the
competence of an often elongated ACL
and the role this pathology may have in
preventing or treating recurrent or chronic
deformity.
This article presents 2 cases of con-
genital knee dislocation associated with
significant ACL elongation. Both patients
underwent open reduction in addition to
ACL shortening and physeal-sparing re-
inforcement. This as an important vari-
able during the management of congeni-
tal knee dislocation. The parents of both
patients were advised that information
regarding their cases would be submitted
for publication and provided consent.
Case Reports
Patient 1
A 2-year, 3-month-old girl presented
following international adoption with
an isolated left knee hyperextension de-
formity. No history of treatment prior to
adoption was found. On physical exami-
nation, the patient had 60° of hyperexten-
sion and was unable to flex beyond neutral
(Figure 1A). Anteroposterior and lateral
radiographs revealed anterior subluxation
of the tibia with hyperextension and with
1B 1C
forced flexion to neutral (Figure 1B).
Preoperative magnetic resonance imaging
(MRI) confirmed the anterior tibial sub-
luxation and identified significant elonga-
tion of an intact ACL (Figure 1C).
The patient underwent open reduction
of her left knee consisting of anterior ar-
throtomy, extensive lateral release, V-Y
quadriceplasty, posterior transposition of
the pes anserinus attachment, and poste-
rior capsulorrhaphy via posteromedial and
posterolateral capsular imbrication. On
reduction of the anterior subluxation, sig-
nificant laxity of the elongated ACL was
noted. This allowed for continued anterior
subluxation and residual 30° of hyperex-
tension despite posterior capsulorrhaphy.
Using the technique discussed below, an
acute shortening of the native ACL was
augmented with a physeal-sparing ACL
reconstruction using an autogenous il-
iotibial band, which prevented sublux-
ation and hyperextension. At the time of
arthrotomy, the patient was also noted to
have a complete, stable discoid lateral
meniscus that was managed with an open
1-piece saucerization.
Postoperatively, the patient was placed
in a long-leg cast in 70° of flexion for 4
weeks. On cast removal, she was transi-
tioned to a hinged knee–ankle–foot or-
thosis with a neutral extension stop for 3
e1095
n Case Report
2A 2B
Figure 2: Nine-month postoperative anteroposte-
rior (A) and lateral (B) radiographs.
months. At 1-year follow-up, the patient
exhibited full and symmetric passive knee
flexion and passive extension to neutral
(Figure 2). She demonstrated no anterior
subluxation of her tibia throughout pas-
sive and active range of motion and had
no active extension lag.
Patient 2
A 2-year, 6-month-old boy presented
with an undetermined genetic disorder
resulting in multiple joint contractures.
He had undergone bilateral open reduc-
tions of his knees at age 5 months due to
failure of nonoperative treatment. Surgery
consisted of acute femoral shortening,
V-Y quadriceplasty, transposition of the
pes anserinus attachment, and posterome-
dial and lateral capsulorrhaphy. He had
3A
Figure 3: Preoperative lateral radiograph in forced flexion (A) and magnetic
resonance image (B) demonstrating tibial subluxation and anterior cruciate amount of laxity
ligament elongation.
e1096
an intact ACL at the index procedures. At
2-year follow-up, the patient’s left knee
showed a recurrent hyperextension de-
formity of approximately 45° with forced
flexion only to neutral. Radiographs con-
firmed anterior subluxation of the tibia,
and follow-up MRI confirmed an elon-
gated ACL (Figure 3).
The patient underwent revision open
reduction with ACL shortening, rein-
forced with a physeal-sparing ACL recon-
struction. Repeat quadriceplasty required
the use of autogenous semitendinosis and
gracilis grafts to reinforce the extensor
mechanism. This resulted in a knee range
of motion of 0° to 90° intraoperatively. He
was placed in a long-leg cast for 4 weeks
and was then transitioned into a hinged
knee–ankle–foot orthosis for 3 months. At
1-year follow-up, the patient exhibited 0°
to 90° of flexion without hyperextension
and minimal quad lag.
Surgical Technique
After wide exposure via a longitudinal
anterior incision, the anterior and central
portion of the iliotibial band is harvested
with maximum length and left attached to
Gerdy’s tubercle. The graft is tubularized
with a heavy suture and the open reduc-
tion is performed in the usual fashion. The
graft harvest site can be used as a lateral
release. Following anterior arthrotomy, a
V-Y quadriceplasty with or without femo-
ral shortening and
posterior transposi-
tion of the pes anser-
ine structures is per-
formed. Posterior
capsulorrhaphy fol-
lowing tibial reduc-
tion is achieved via
posteromedial and
posterolateral cap-
sular imbrication.
If intact, the length
3B of the ACL is then
examined and the
determined. If ante-
rior tibial subluxation is seen with exten-
sion to or beyond 10° to 15° of neutral,
the redundant ligament is excised and the
shortened ligament repaired to the tibial
epiphysis footprint (Figure 4). The ilio-
tibial graft is brought around the posterior
lateral condyle and over the top of the
native ACL.11 The graft is placed under
the intermeniscal ligament and sewn un-
derneath the periosteum of the proximal
medial tibia. Tenodesis to the posterolat-
eral femur and intermuscular septum is
reinforced with suture attachment to the
native ACL (Figures 5, 6). The graft is ten-
sioned to prevent more than 1 to 2 mm of
anterior tibial translation at full extension
of the knee. Adequate tensioning prevents
hyperextension.
Discussion
Congenital knee dislocation is a com-
plex disorder associated with a variety of
syndromes and additional comorbidities
that can complicate treatment and func-
tion. Because of its variable presentation,
ranging from flexible hyperextension to
frank dislocation, no consensus exists
on the ideal treatment of this condition.
Although the pathogenesis of this dis-
order is unclear, typical patterns of ana-
tomic deformity exist that surgeons must
consider when attempting to gain reduc-
tion of the knee. Surgical management
includes extensor mechanism lengthening
with or without femoral shortening, soft
tissue releases, posterior transposition of
the pes anserinus, and posterior capsulor-
rhaphy. The elongation and incompetence
of the ACL has limited discussion in the
literature and is not incorporated in cur-
rent surgical algorithms.
Mayer,5 McFarland,6 and Odell and
Holt12 described the ACL as being attenu-
ated, elongated, and poorly developed.
Katz et al2 reported that the cruciate liga-
ments of 5 patients with congenitally dis-
located knees were either absent or hypo-
plastic. The ACL was reconstructed with
the use of a retinaculum and a portion of
the patellar tendon. They reported that the
ORTHOPEDICS | Healio.com/Orthopedics

4A
Figure 4: Illustration of anterior tibial subluxation and anterior cruciate ligament (ACL) elongation (A). On
reduction, redundancy of the ACL and posterior capsule are noted (B).
primary cause of the congenital disloca-
tion was the absence or hypoplasia of the
cruciate ligaments.5
Sud et al7 reported 17 knees in 10 pa-
tients who underwent V-Y lengthening of
the quadriceps tendon and anterior capsu-
lotomy and reported that no patients had
clinically apparent sagittal plane instabil-
ity and that the ACL was intact visually
but was long and atrophic.4
Soyuncu et al13 reported on 2 adoles-
cents with congenital knee dislocation.
One patient had an elongated ACL after
reduction and was tightened with the use
of a Bunnell suture technique at the base
of the tibia and suture tunnels to properly
tension the ACL. They recommended ad-
vancement of the ACL to prevent recur-
rences, especially in delayed cases, and
noted decreased anterior laxity on knees
that were tightened.7
Oetgen et al10 performed a retrospec-
tive review of 7 patients treated surgically
and compared quadriceplasty and femoral
shortening. Patients were followed for an
average of 12 years, and each patient un-
derwent a clinical examination, functional
evaluation, and gait evaluation. Seven
(78%) of 9 knees had some degree of in-
stability on examination, but no patient
wore a brace. Although good functional
results were demonstrated with both sur-
gical approaches, they scored significant-
ly lower in the sports and physical func-
tioning aspects, demonstrating an under-
lying instability during more demanding
activities. This instability was noted even
JULY 2012 | Volume 35 • Number 7
Congenital Knee Dislocation | Klingele et al
4B 5
Figure 5: Illustration showing that following tibial
reduction, anterior cruciate ligament shortening is
performed, as well as posteromedial and postero-
lateral capsulorrhaphy.
in patients who achieved early successful
knee reduction, indicating that ligamen-
tous structures may be abnormal.
On open reduction of the knee in the
current cases, the ACL was intact but
functionally incompetent due to elonga-
tion. The authors identified residual an-
terior instability and a tendency toward
hyperextension following open reduction,
quadriceps lengthening, lateral release,
posterior transposition of the pes anser-
ine, and posterior capsulorrhaphy. The de-
scribed technique of ACL shortening and
reinforcement helped maintain reduction
and prevented anterior tibial subluxation 6
and residual hyperextension at surgery Figure 6: Anterior-view illustration status post
anterior cruciate ligament (ACL) shortening and
and at last follow-up.
reinforcement using an autogenous iliotibial band.
Long-term follow-up studies of con-
genitally deficient ACL knees and congen-
ital knee dislocations describe progressive surgical treatment of congenital knee dis-
valgus angulation. Much of this angulation location. In addition to assisting in main-
may be related to the anterior instablility tenance of reduction, stabilizing and rein-
and subsequent rotatory instability seen forcing this ligament may prevent long-
with cruciate deficiency. Treatment of an- term anterior instability, hyperextension
terior instability may prevent progressive of the knee, and recurrent deformity.
valgus angulation. Nonetheless, the pres-
ence of an ACL in management of congen- References
ital knee dislocation does not equate to a 1. Shattock SG. Genu recurvatum in a foetus at
stable knee. The surgeon must account for term. Trans Pathol Soc Lond. 1891; 42:280-
292.
ACL elongation following reduction.
2. Katz MP, Grogono BJ, Soper KC. The etiol-
ogy and treatment of congenital dislocation
Conclusion of the knee. J Bone Joint Surg Br. 1967;
49(1):112-120.
Although good results have been re-
ported with a variety of treatment tech- 3. Curtis BH, Fisher RL. Congenital hyper-
extension with anterior subluxation of the
niques, ACL incompetence due to elonga- knee. Surgical treatment and long-term ob-
tion is an underreported anatomic feature servations. J Bone Joint Surg Am. 1969;
51(2):255-269.
that warrants consideration during initial
e1097
n Case Report
4. Klingele KE. Congenital knee dislocation.
In: Micheli L, Kocher M, eds. The Pediatric
and Adolesccent Knee. Philadelphia, PA: El-
sevier; 2006:421-435.
5. Mayer L. Congential anterior subluxation of
the knee. Am J Orthop Surg. 1913; 10:411.
6. McFarland BL. Congenital discloation of the
knee. J Bone Joint Surg Am. 1929; 11(2):281-
285.
7. Sud A, Chaudhry A, Mehtani A, Tiwari A,
Sharma D. Functional outcome following
quadriceps tendon lengthening in congenital
dislocation of the knee, with special refer-
e1098
ence to extensor weakness. Strategies Trau-
ma Limb Reconstr. 2009; 24:24.
8. Insall JN, Scott WN. Surgery of the Knee.
3rd ed. New York, NY: Churchill-Livingston;
2001.
9. Ooishi T, Sugioka Y, Matsumoto S, Fujii T.
Congenital dislocation of the knee. Its patho-
logic features and treatment. Clin Orthop
Relat Res. 1993; 287(287):187-192.
10. Oetgen ME, Walick KS, Tulchin K, Karol LA,
Johnston CE. Functional results after surgical
treatment for congenital knee dislocation. J
Pediatr Orthop. 2010; 30(3):216-223.
11. Kocher MS, Garg S, Micheli LJ. Physeal
sparing reconstruction of the anterior cruciate
ligament in skeletally immature prepubes-
cent children and adolescents. J Bone Joint
Surg Am. 2005; 87(11):2371-2379.
12. Odell RT, Holt EP Jr. Congenital anterior
dislocation of the knee. South Med J. 1954;
47(11):1056-1062.
13. Soyuncu Y, Mihci E, Ozcanli H, Ozenci M,
Akyildiz F, Aydin AT. Reconstruction of
quadriceps tendon with Achilles tendon al-
lograft in older children with congenital dis-
location of the knee. Knee Surg Sports Trau-
matol Arthrosc. 2006; 14(11):1171-1175.
ORTHOPEDICS | Healio.com/Orthopedics