adolescent with bilateral femoral and tibial rotational ... · fig. 3 preoperative photograph and...

Adolescent with Bilateral Femoral and Tibial Rotational Deformity(Miserable Malalignment Syndrome) Combined with Proximal TibialVarus and Recurvatum

Haim Shtarkera* and Mikhail SamchukovbaWestern Galilee Hospital, Nahariya, IsraelbCenter for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas,TX, USA

Abstract

A fourteen-year-old female with significant knee pain due to bilateral rotational malalignmentsyndrome, varus-recurvatum deformity of the proximal tibiae, excessive joint laxity, and noprevious treatment underwent external left femoral de-rotation osteotomy with lockingintramedullary nail fixation and tibial/fibular osteotomy followed by gradual correction of multi-planar deformity using hexapod-type TL-Hex circular external fixation.

1 Brief Clinical History

The patient is a 14-year-old female who suffered from bilateral chronic knee pain and was not treatedpreviously. She was diagnosed with the miserable malalignment syndrome (MMS) characterized bysubstantial bilateral internal femoral torsion and external tibial torsion associated with 15� of genuvarum, 20� of genu recurvatum, and significant joint laxity (Figs. 1, 2, 3, and 4). Her computedtomography rotational malalignment test (CTRMT) revealed 37� and 38� of internal femoral torsionand 47� and 49� of external tibial torsion on the left and right lower extremities, respectively (Fig. 5).She had normal hip joint and ankle joint range of motion and was otherwise healthy andneurovascularly intact.

2 Preoperative Clinical Photos and Radiographs

See Figs. 1, 2, 3, 4 and 5.

3 Preoperative Problem List

• Bilateral internal femoral torsion (38� on the right and 37� on the left)• Bilateral external tibial torsion (49� on the right and 47� on the left)

*Email: [email protected]

*Email: [email protected]

Limb Lengthening and Reconstruction Surgery Case AtlasDOI 10.1007/978-3-319-02767-8_70-1# Springer International Publishing Switzerland 2014

of 13

• Bilateral proximal tibial varus (15�)• Bilateral proximal tibial recurvatum (10�)• Bilateral significant joint laxity (20�) in the sagittal plane• Bilateral knee joint pain amplified during the gait

4 Treatment Strategy

Because of bilateral nature of complex multi-segmental deformity, the treatment strategy was basedon staged ipsilateral realignment of lower extremities providing at least one leg for weight bearingduring the entire duration of treatment. The management of the rotational malalignment deformityon each lower extremity included proximal femoral osteotomy followed by acute correction ofexcessive internal torsion with internal fixation using rigid intramedullary locking nail. In addition,osteotomy of the proximal tibia and midshaft fibula was performed followed by gradual correctionof excessive external tibial torsion, varus, and recurvatum using hexapod-type TL-Hex externalfixation system. This method provides stable fixation of bone fragments and permits full weightbearing while allowing for simultaneous correction of multi-planar deformities gradually withoutoverstretching of the peroneal nerve. The gradual correction continues until the patient is satisfied

Fig. 1 Preoperative front view photograph and AP radiograph with feet pointing forward demonstrating bilateralinternal femoral torsion. Note internal orientation of both patellae, which is further amplified during the gait


of 13

Fig. 3 Preoperative photograph and LAT radiograph of the left tibia demonstrating substantial genu recurvatum(HE = 20�, PPTA = 85�)

Fig. 2 Preoperative front view photograph and AP radiograph with patellae pointing forward demonstrating bilateralexternal tibial torsion associated with 15� of genu varum (MPTA = 78�, JLCA = 5� varus). Note external orientationof both feet significantly affecting her gait


of 13

with the cosmetic appearance of the lower extremities. Due to more severe knee pain, the left leg waschosen for the first stage of treatment.

5 Basic Principles

Rotational alignment of lower extremities is equally important as an alignment in the coronal andsagittal planes. Normal anatomical relationship between the different limb segments providesnormal function of the adjacent joints (Yagi and Sasaki 1986). The femur is normally positionedat 10–20� of internal torsion, which reflects the equivalent normal femoral neck anteversion. At thesame time, normal rotational alignment of the tibia is approximately 5–20� of external rotation withan average of 16� (Delgado-Martinez et al. 2000; Merchant et al. 1974). The combination ofexcessive internal femoral torsion (anteversion) with excessive external tibial torsion resulting ininternal rotation of the knee joints is called the MMS (Bruce and Stevens 2004; Delgado et al. 1996;Moussa 1994). Due to significant biomechanical abnormalities, chronic stress will be developed atthe knee joint, thereby producing pain and resulting in early degenerative changes. It is notuncommon to find varus deformity of the proximal tibia associated with the MMS as well as othercombinations of rotational malalignment and deformities in coronal and sagittal planes. Duringanalysis of femoral-tibial rotational malalignment, it is very important to exclude patellarmalalignment, which appears due to incongruence of the patellar joint surface relative to patellargroove of the femur. Position of the tibial tuberosity may also seriously influence patellar tracking(Yoshioka et al. 1989). In addition, foot deformity may imitate rotational malalignment and shouldbe excluded in the examination of torsional limb deformities.

Fig. 4 Preoperative photograph of the patient in the sitting position illustrating bilateral rotational malalignment


of 13

6 Images During Treatment

See Figs. 6, 7, 8, 9, 10, 11, 12, and 13.

7 Technical Pearls

Preoperative measurement of the femoral and tibial torsion in patients with MMS is very importantfor successful realignment of the limb. Because of the association between external tibial torsion andproximal tibial varus deformity, measurement of tibial torsion when the patient is in the supineposition with a flexed femur can be misleading. Therefore, evaluation of the tibial torsion should be

Fig. 5 Preoperative CTRMT revealing bilateral internal femoral torsion and external tibial torsion. Note representativeCTcuts through the femoral head and neck (a), femoral condyles and femoro-patellar joint (b), proximal tibia and upperborder of tibial tuberosity (c), malleoli (d), and talus and calcaneus (e) followed by rotational malalignment test for rightand left lower extremities (R/L): FVA femoral version angle = 18/20�, KTA knee torsion angle = 20/17�, PCA patellacongruency angle = 22/17�, TTA tibial tuberosity angle = 24/27�, TRA tibial rotation angle = 29/30�, and FRA footrotation angle = 20/19�


of 13

done in the prone position with a fully extended femur. For more precise preoperative assessment ofthe rotational component of combined deformities, CTRMTwas introduced (Shtarker et al. 2002).Briefly, CTRMT includes five CT cuts taken through the: (1) femoral head and neck, (2) femoralcondyles and patellofemoral joint, (3) proximal tibia at the upper border of the tibial tuberosity,(4) malleoli, and (5) talus and calcaneus. This is followed by the measurement of femoral versionangle (cut 1) and knee torsion angle (cut 2) relative to coronal plane, patellar congruency angle (cut2) relative to the line bisecting the patellar groove angle, tibial tuberosity angle (cut 3), tibial rotation

Fig. 6 Intraoperative photograph during proximal femoral osteotomy. Note two parallel half pins inserted above andbelow the osteotomy and used for monitoring of femoral de-rotation

Fig. 7 Intraoperative AP radiographs after femoral osteotomy before (left) and after (right) acute correction of internalfemoral torsion and internal fixation using 8.5 mm TrigenTM intramedullary locking nail


of 13

Fig. 9 Intraoperative photograph and AP radiograph of the tibia after insertion of the Gigli saw blade at the intendedosteotomy level

Fig. 8 Photograph of preoperative planning illustrating frame assembly diagrams for simultaneous correction ofproximal tibial varus (left), recurvatum (center), and external torsion (right) using TL-Hex software. Note frameparameters (top), anticipated mounting parameters (center), and computer-calculated strut lengths (bottom) used forpreoperative assembly of external fixator


of 13

Fig. 10 Intraoperative photograph of the tibia demonstrating applied TL-Hex external fixator consisting of proximalring and distal double-ring block interconnected with six struts. Note two anterior struts disconnected from the frameproximally to the tibia during Gigli saw osteotomy

Fig. 11 Postoperative AP and LAT radiographs of the tibia after proximal tibial and midshaft fibular osteotomies beforegradual multi-planar deformity correction


of 13

angle (cut 4), and foot rotation angle (cut 5) relative to the sagittal plane (Fig. 5). In patients withunilateral deformities, these parameters are compared with those on the opposite side. In patientswith bilateral deformities, the measurements are compared with known normal data. Correction ofthe rotational deformity can be achieved either acutely or gradually using special rotational modulesattached to the Ilizarov type of external circular fixation frame (Herzenberg et al. 1991; Ilizarov1992). To enhance the precision of the rotational deformity correction and to avoid time-consumingframe manipulations with this type of external fixator, the hexapod-type circular external fixators areutilized in association with web-based software.

8 Outcome Clinical Photos and Radiographs

See Figs. 14 and 15.

Fig. 12 Postoperative AP and LAT radiographs of the tibia during consolidation period after completion of gradualdeformity correction


of 13

9 Avoiding and Managing Problems

In order to restore a normal knee joint axis and improve its function, correction of rotationalmalalignment should be combined with correction of angular deformities in the coronal and sagittalplanes. Therefore, all rotational and associated deformities should be documented and included intothe preoperative planning. In this case, correction of the proximal tibial varus deformity combinedwith re-creation of the tibial slope in the sagittal plane (to eliminate severe proximal tibialrecurvatum and laxity) was required to supplement the correction of the femoral-tibial rotationaldeformities. Utilization of the hexapod type of external fixator in this situation allows simultaneous,precise correction of all tibial deformities to be achieved. Correction of the femoral torsion is usuallyperformed acutely using intramedullary rigid nail fixation. Therefore, intraoperative control of thefemoral de-rotation is critically important due to its influence on the subsequent gradual de-rotationof the tibia. Steinmann pins inserted above and below the femoral osteotomy are not always a precisemethod for evaluation of the amount of rotation. The excessive soft tissue stretching that occurs in

Fig. 13 Front view photograph of lower extremities with feet pointing forward after completion of deformity correction.Patient was walking during the treatment with full weight bearing without additional supports and felt very comfortablein the frame. Note the rotational malalignment of the right tibia and the restored alignment of the left tibia with properpatella orientation


of 13

the de-rotation can result in backing up of the pins. This complication occurred in our case during theinitial surgery, resulting in excessive external de-rotation. An additional surgery for correction of therotational deformity and relocking the nail was required. We prefer now to use 5 or 6 mm rigid halfpins inserted at the posterior cortex of the bone instead of the more flexible Steinmann pins. Inaddition, the anatomical/mechanical axis of the femur should be considered when performingde-rotation around the femoral shaft to prevent undesirable femoral head rotation. Due to the spiralpath of the peroneal nerve from posterior to anterior and from lateral to medial at the level of theproximal tibia, correction of excessive tibial external torsion may result in overstretching on thenerve causing peroneal nerve palsy. Therefore, the rate of internal de-rotation should be carefullycontrolled. We prefer to limit the rate of internal de-rotation on the tibia to 5–8� per day under verycareful control of foot neurovascular function and sensitivity at the dorsal aspect of the first webspace.

Fig. 14 Front view photograph and AP radiograph of lower extremities 1 year after frame removal. Note completeconsolidation of both osteotomies with restored alignment of the left lower extremity (LDFA = 82�, MPTA = 87�)


of 13

10 Cross-References

▶Correction of Valgus-Torsion Deformity of the Femur and Varus-Torsion Deformity of the LowerLeg Accompanied by LLD

▶Correction of Windswept Rotational Deformity with Fixator Assisted Plating Technique▶ Femoral and Tibial Rotational Deformity Treated with Fixator-Assisted Nailing and GradualCorrection with the Taylor Spatial Frame

References and Suggested Reading

Bruce WD, Stevens PM (2004) Surgical correction of miserable malalignment syndrome. J PediatrOrthop 24(4):392–396

Delgado WD, Schoenecker PL, Rich MM, Capelli RN (1996) Treatment of severe malalignmentsyndrome. J Pediatr Orthop 16:484–488

Delgado-Martinez AD, Rodriguez-Merchant EC, Ballesteros R, Luna JD (2000) Reproducibility ofpatellofemoral CT scan measurements. Int Orthop (SIOT) 24:5–8

Fig. 15 Lateral view photograph and LAT radiograph of the lower extremities 1 year after frame removal. Note no kneejoint laxity and restored alignment of the left lower extremity (PPTA = 78�). Patient has no complaints of knee pain,noticed improved knee joint function, and is satisfied with cosmetic appearance of her left leg. She continues physicaltherapy for restoring her knee function in preparation for the second stage of treatment


of 13

http://link.springer.com/SpringerLink:ChapterTarget





Herzenberg JE, Smith JD, Paley D (1991) Correcting torsion deformities with Ilizarov’s apparatus.Clin Orthop 302:36–41

Ilizarov GA (1992) Transosseous osteosynthesis. Theoretical and clinical aspects of the regenerationand growth of tissues. Springer, New York, pp 63–136

Merchant AC, Mercer RL, Jacobsen RH, Cool CR (1974) Roentgenographic analysis ofpatellofemoral congruence. J Bone Joint Surg Am 56:1391–1396

Moussa M (1994) Rotational malalignment and femoral torsion in osteoarthritic knees withpatellofemoral joint involvement. Clin Orthop 304:176–183

Shtarker H, Volpin G, Stolero J, Kaushansky A, Samchukov M (2002) Correction of combinedangular and rotational deformities by the Ilizarov method. Clin Orthop 402:184–195

Yagi T, Sasaki T (1986) Tibial torsion in patients with medial type osteoarthritis knee. Clin Orthop213:177–182

Yoshioka Y, Siu DW, Scudamore RA, Cooke TD (1989) Tibial anatomy and functional axes.J Orthop Res 7:132–137


of 13

adolescent with bilateral femoral and tibial rotational ... · fig. 3 preoperative photograph and...

Documents