Orthopedics

Case Report 

Talectomy by Medial Surgical Approach for Congenital Vertical Talus in Arthrogryposis Multiplex Congenita

Edward Abraham, MD; Camille H. Quan Soon, MD; Angela Murphy, MCSP; David Toby, MD

Abstract

Arthrogryposis multiplex congenita involves stiff contracture of joints and weak atrophic muscles presenting at birth. The two most common forms are amyoplasia and distal arthrogryposis. Amyoplasia affects all 4 extremities: internally rotated shoulders, extended fixed elbows, flexed fixed wrists, extended fixed knees, clubfeet, and decreased muscle volume. Distal arthrogryposis is a group of syndromes with a genetic basis. The distal joints are contracted. Clubfeet and congenital vertical talus are the most common foot deformities. A 10-year-old boy presented with distal arthrogryposis with bilateral congenital tali. He reported having deformed and painful feet and difficulty wearing shoes. His rocker-bottom foot deformities caused him to walk with a heel to heel gait. He also had stiff extended knees. His previous foot surgeries included failed open reduction and pin fixation of the talonavicular joints with Achilles tendon lengthening and capsulotomies. The boy underwent bilateral talectomies and releases of contracted joint capsules and lengthening of multiple extrinsic tendons through separate incisions. The talectomy of each foot was performed via a novel medial surgical approach. At 2-year follow-up, he had normal-appearing plantar grade feet. He had a painless gait, could ambulate independently, and was considered to have an excellent result. This is the first detailed report of performing a talectomy via a medial approach for bilateral congenital tali in a patient with arthrogryposis multiplex congenita. [Orthopedics. 2020; 43(6):e623–e626.]

Abstract

Arthrogryposis multiplex congenita involves stiff contracture of joints and weak atrophic muscles presenting at birth. The two most common forms are amyoplasia and distal arthrogryposis. Amyoplasia affects all 4 extremities: internally rotated shoulders, extended fixed elbows, flexed fixed wrists, extended fixed knees, clubfeet, and decreased muscle volume. Distal arthrogryposis is a group of syndromes with a genetic basis. The distal joints are contracted. Clubfeet and congenital vertical talus are the most common foot deformities. A 10-year-old boy presented with distal arthrogryposis with bilateral congenital tali. He reported having deformed and painful feet and difficulty wearing shoes. His rocker-bottom foot deformities caused him to walk with a heel to heel gait. He also had stiff extended knees. His previous foot surgeries included failed open reduction and pin fixation of the talonavicular joints with Achilles tendon lengthening and capsulotomies. The boy underwent bilateral talectomies and releases of contracted joint capsules and lengthening of multiple extrinsic tendons through separate incisions. The talectomy of each foot was performed via a novel medial surgical approach. At 2-year follow-up, he had normal-appearing plantar grade feet. He had a painless gait, could ambulate independently, and was considered to have an excellent result. This is the first detailed report of performing a talectomy via a medial approach for bilateral congenital tali in a patient with arthrogryposis multiplex congenita. [Orthopedics. 2020; 43(6):e623–e626.]

Whitman1 is credited with describing the talectomy or astragalectomy that was initially used to improve foot function in polio patients with paralytic talipes calcaneus. An anterolateral surgical approach was used in all cases. With time, the indications for this salvage operation were expanded to include other childhood disorders with different rigid foot deformities.2 Arthrogryposis multiplex congenita (AMC) is one such condition.

Arthrogryposis multiplex congenita is estimated to occur in 1 of 10,000 live births.3 This is a heterogeneous group of patients who present clinically with varying degrees of multiple congenital joint contractures.4 The illness commonly leads to a disproportionate degree of muscle atrophy, muscle weakness, and early adult-onset arthritis. The foot involvement is seen in 80% to 90% of cases.5 The two important foot deformities reported in a series of 51 affected feet were congenital equinovarus clubfeet (80%) and bilateral congenital tali (CVT) (10%).6

The anterolateral exposure is the default surgical approach, but it does not address the significant differences in the pathologic anatomies of the two conditions.7,8 In this case report, a new direct medial surgical approach to performing the talectomy in AMC with recurrent and severely disabling bilateral foot deformities caused in part by CVT is described.

Case Report

A 10-year-old boy presented to the authors' clinic for a follow-up visit. He reported a progressive abnormal gait and difficulty wearing shoes for 8 years (Figure 1A). At 2 months old, he was diagnosed with AMC with the common phenotype characteristics of distal arthrogryposis. The family history was negative for AMC. On physical examination, he had bilateral vertical tali deformities of the feet and stiff extended knees. His neurologic examination yielded normal results. At 1 year old, he underwent bilateral open reduction of the talonavicular joints with Kirschner wire fixation, Achilles tendon lengthening, and posterior ankle and subtalar joint releases. At 3 years old, he had a left quadricep lengthening procedure.

Preoperative standing photograph (A) and lateral radiograph of the left foot (B). Note severe rocker-bottom deformities and externally rotated feet.

Figure 1:

Preoperative standing photograph (A) and lateral radiograph of the left foot (B). Note severe rocker-bottom deformities and externally rotated feet.

The vertical talus deformities recurred (Figure 1B). He was able to ambulate independently. He walked with externally rotated feet and a heel to heel gait caused by severe rocker-bottom deformities. In this gait pattern, the forefeet and midfeet did not touch the ground when he walked. There were painful bursae on the plantar surfaces of the feet under the talar heads. The feet actively dorsiflexed to 70° and plantarflexed to −20° bilaterally. There were bilateral dorsal bunions of the great toes with overriding second toes. He walked with straight stiff knees that extended 10° beyond the neutral normal 0° and flexed to only 15°. At 10 years, 11 months old, he underwent a left salvage talectomy with tendon releases. Four months later, he had a similar procedure on the right side.

Operative and Postoperative Procedure

Figure 2 illustrates the operative technique used to perform the talectomy on the right foot. Under general anesthesia and a thigh tourniquet, a 6-cm medial skin incision was made along the long axis of the talar bone and anterior to the medial malleolus (Figure 2A). The incision extended through the subcutaneous tissues and down to the talus head and neck. The exposed talus and periosteum were removed with the aid of a periosteal elevator and ronger instruments. Care was taken to avoid the neurovascular structures deep to the talar head and neck (Figure 2B).

Illustration of the medial surgical approach (A). Skin incision (broken line) along the long axis of the vertical talus. Note the neurovascular structures adjacent to the talar head. Removal of the talus with the aid of a towel clamp and an elevator. Insert shows a close-up of talus removal (B). Posterolateral ankle incision. Achilles 1-cm excision tenotomy (a), peroni tendons lengthening (p), and ankle joint arthrotomy (j) (C). Foot position in the ankle joint. Arrow indicates the posterior displacement of the foot to create a heel. A Steinmann pin is placed retrograde across the ankle joint to hold the neutrally positioned foot in place. Insert shows distal excision of fibula bone (D). (© Danielle Robinson. Used with permission.)

Figure 2:

Illustration of the medial surgical approach (A). Skin incision (broken line) along the long axis of the vertical talus. Note the neurovascular structures adjacent to the talar head. Removal of the talus with the aid of a towel clamp and an elevator. Insert shows a close-up of talus removal (B). Posterolateral ankle incision. Achilles 1-cm excision tenotomy (a), peroni tendons lengthening (p), and ankle joint arthrotomy (j) (C). Foot position in the ankle joint. Arrow indicates the posterior displacement of the foot to create a heel. A Steinmann pin is placed retrograde across the ankle joint to hold the neutrally positioned foot in place. Insert shows distal excision of fibula bone (D). (© Danielle Robinson. Used with permission.)

The remaining portion of the talar body was freed up with a periosteal elevator and then completely removed. The Achilles tendon was exposed in the depth of the wound. One centimeter of the full thickness of the tendon was removed. This important step allowed for better mobilization of the calcaneus bone to a more normal position.

Because the authors planned to release the lateral ankle contracture caused by the tendons of the peroneal muscles, a 4-cm posterolateral skin incision was made at and proximal to the ankle joint (Figure 2C). The peroneus longus and peroneus brevis tendons were lengthened. This incision was used to find the Achilles tendon and confirm that a full thickness of the tendon was removed. The posterior ankle joint was explored through this incision to remove any remnants of the talar bone. A separate 3-cm incision was made over the anterior distal tibia at the level of the epiphysis to address the residual anterior ankle contracture. The tibialis anterior, extensor hallucis longus, and extensor digitorium longus tendons were lengthened. The partially incised superior extensor retinaculum was repaired.

The foot was aligned within 10° of the normal 0° position. With the foot pushed backward, a large-caliber Steinmann pin was drilled percutaneously in a retrograde fashion into the calcaneus and distal tibial shaft (Figure 2D). A staple was used across the navicular bone and tibia for additional construct stability. The distal 1 cm of the fibula tip was removed. The restoration of heel alignment within 10° of normal and partial fibula excision allowed for better shoe fit. The same surgical technique including Steinmann pin and staple was used on the right side. Each of the 3 surgical wounds was closed in a routine fashion. No wound drains were necessary. Because the contractures around the foot and ankle may differ from one case to another, the secondary incisions used to release the contractures may be different in each case.

Short leg casts were used for 3 months postoperatively. During this period, the patient was kept non–weight bearing. The Steinmann pin was removed and protective full weight bearing was permitted. At his last clinic visit, which was 2 years postoperative for the left foot and 1½ years postoperative for the right foot, the patient remained able to ambulate independently, having plantigrade painless feet at neutral dorsiflexion (Figure 3). There was less than 5° of ankle motion on the left side and a range to 15° on the right side. The right side ankle motion was most likely due to a fibrous union of the ankle joint construct. The dorsal bunions of the great toes and overriding second toes were corrected. Shoe wear was improved significantly. A hinged knee brace restricted hyperextension and allowed unrestricted flexion during weight bearing. The patient was judged to have a satisfactory result based on Cooper criteria.2 The criteria for a satisfactory result include standing and walking without pain, no skin breakdown, and a stable foot.

At 2-year follow-up, standing photograph of both feet (A) and lateral radiograph of the left foot (B). Note the correction of rocker-bottom deformities, external rotation of feet forefoot deformities, and restoration of a normal lateral pitch angle from −17° to 12°. Similar observations were made on the right side.

Figure 3:

At 2-year follow-up, standing photograph of both feet (A) and lateral radiograph of the left foot (B). Note the correction of rocker-bottom deformities, external rotation of feet forefoot deformities, and restoration of a normal lateral pitch angle from −17° to 12°. Similar observations were made on the right side.

Discussion

The direct medial approach is a novel and reproducible way to perform a talectomy for CVT. Because the talar head and neck is located on the medial side of the foot, it is logical to expose the talus from a medial approach. This patient was judged to have an excellent outcome at 2-year follow-up. A review of 10 relevant articles about AMC during 40 years found 17 patients with 34 talectomies for CVT.2,5–7,9–14 Only 1 patient's clinical description and radiographs were similar to those of the current patient.10 That patient, with 26-year follow-up, could ambulate with painless feet. No information was given about the surgical foot procedure for that patient. Further, all of the other studies but 1 lacked surgical detail.5 In that study, 51 patients with AMC had foot deformities. The 4 patients with CVT all underwent talectomies via an anteromedial surgical incision. There was no additional information about the surgery.

During the past four decades, the authors have performed the talectomy sporadically. However, in the past 4 years, the procedure was successfully performed in 3 cases, which included the current patient. The other 2 children were diagnosed with lumbosacral agenesis and distal arthogryposis. They both had residual clubfeet deformities that were treated surgically via the anterolateral approach at ages 6 and 9 years, respectively. A recent study evaluated the outcomes of nonsurgical and other primary or secondary surgical methods to treat childhood CVT at different ages of presentation by using the best available evidence.15 Those authors concluded that the prognosis is worse if the treatment begins late for non-idiopathic CVT and when both the talonavicular joint and the calcaneocuboid joint are dislocated.

Conclusion

To the best of the authors' knowledge, this is the first detailed description of a medial surgical approach to perform a talectomy of the foot for CVT in AMC. The authors are not advocating this useful salvage operation for any child with CVT. Instead, the procedure should be reserved for the rare skeletally immature patient with a rigid neglected vertical talus deformity and with associated lower extremity neurovascular issues, as exemplified in the current case with AMC.

References

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Authors

The authors are from Princess Elizabeth Centre (EA, CHQS, AM, DT), Woodbrook, Port of Spain, and The University of the West Indies (CHQS), St Augustine, Trinidad and Tobago; and the Department of Orthopaedics (EA), University of Illinois at Chicago, Chicago, Illinois.

The authors have no relevant financial relationships to disclose.

Correspondence should be addressed to: Edward Abraham, MD, Department of Orthopaedics, University of Illinois at Chicago, 835 S Wolcott Ave, Rm E-270 (MC 844), Chicago, IL 60612 ( eda@uic.edu).

Received: July 09, 2019
Accepted: October 24, 2019
Posted Online: August 20, 2020

10.3928/01477447-20200812-01

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