Severe Equinovarus Deformity Research Articles

Case Report: Tibial Hemimelia

Unilateral tibial hemimelia is an anomaly characterized by the absence of the tibia. Tibial hemimelia is the rarest form of congenital longitudinal deficiency of the tibia with an incidence of 1 in 1,000,000 live births. It is characterized by deficiency of tibia with relatively intact fibula. It causes marked shortening of the limb with a severe equinovarus deformity. It may occur in isolated form or combined with other congenital anomalies. Some cases of tibia hemimelia are genetically transmitted as autosomal dominant or recessive, whereas others are sporadic. The treatment is always difficult and challenging. Amputation was the preferred treatment option specially in complete tibial absence; however, a conservative management is used in other types of the deformity.

Tibiotalocalcaneal Arthrodesis With the Hindfoot Arthrodesis Nail: A Prospective Consecutive Series From a Single Institution

Tibiotalocalcaneal arthrodesis (TTCA) is a salvage procedure. We report a series of 20 patients who underwent TTCA using an intramedullary nail. Of the 20 patients, 7 (35%) had diabetes mellitus. The patient experiences and outcomes were analyzed. Their mean age was 61.1 (range 39 to 78) years. The minimum follow-up period was 13 (mean 28, range 13 to 49) months. Surgical indications included diabetic Charcot arthropathy in 7 (35%), hindfoot osteoarthritis in 10 (50%), and severe equinovarus deformity in 3 (15%). A calcaneal spiral blade was used in 2 patients (10%). Significant improvements (p < .05) were observed in 5 of 8 Short-Form 36-item Health Survey components, the American Orthopaedic Foot and Ankle Society Ankle-Hindfoot scale (p < .001), and visual analog scale for pain (p < .001). The mean length of the hospital stay was 6.7 (range 1 to 27) days. Of the 20 patients, 76.9% had improvement in their activity postoperatively. Also, 81.8% were able to resume their preoperative work after a mean of 7.89 (range 3 to 24) months. Overall, 19 patients (95%) reported favorable outcomes. Superficial wound infection (n = 4; 20%) and deep wound infection (n = 3; 15%) were the most common complications (35%), with 1 case (5%) culminating in a below-the-knee amputation. Radiographic union was achieved in 16 of the tibiotalar joints (80%), 16 subtalar joints (80%), and 4 tibiocalcaneal fusions (20%). In a subgroup analysis of 7 patients with diabetes mellitus (35%), the incidence of wound complications and fusion was comparable to that of the primary cohort. TTCA performed with an intramedullary nail appears to offer a reliable and safe alternative for patients with severe ankle and hindfoot pathologic entities, including those with diabetes mellitus.

P09.08: Rare case of unilateral tibial hemimelia in the first trimester

Unilateral tibial hemimelia is an anomaly characterised by the absence of the tibia. Tibial hemimelia is a very rare disease with an incidence of 1 in 1,000,000 live births. It is characterised by deficiency of tibia with relatively intact fibula. It causes marked shortening of the involved extremity with a severe equinovarus deformity. It may be isolated or associated with other anomalies. We report a case showing the absence of tibia in a 25-year-old primigravida diagnosed at 13 weeks of gestation who was referred to our centre for a routine first trimester scan. She had no notable history of intake of any medication or maternal infection. Ultrasound examination showed a single viable fetus of 13 weeks gestation which revealed foreshortening of the right lower limb with associated flexion at the knee and with talipes equino varus deformity of the right foot. Careful examination of right lower leg showed the absence of the tibia and the flexion at the knee. This was due to the atrophy of the quadriceps muscle. The left leg and the upper limbs were unremarkable. The aneuploidy markers on ultrasound were also normal. In view of the future disability of the baby, the couple opted for the termination of pregnancy. The abortus showed the same findings as seen on ultrasound and the autopsy also confirmed the diagnosis of unilateral tibial hemimelia.

Tibiocalcaneal arthrodesis using intramedullary nail for severe rigid equinovarus deformity in adults

Aim: To evaluate the results of tibiocalcaneal arthrodesis using intramedullary nails in adults with severe rigid equinovarus deformity of foot. Background: Foot is an amazing structure of support, balance, locomotion and sensibility. Impairment of one or more of these functions can lead to catastrophic sequelae. Equinovarus deformities of foot can be very disabling by marring the ambulation, daily activities, shoe wear and hygiene. In this paper we tend to share our experience of treating patients with posttraumatic severe rigid equinovarus deformities via tibiocalcaneal arthrodesis with retrograde intramedullary nailing. Settings and Design: Prospective follow up study. Materials and Methods: We selected 10 patients (3 females, 7 males) aged 18–60 years, suffering from disabling non-braceable posttraumatic severe rigid equinovarus deformity of foot. The patients were treated via tibiocalcaneal arthrodesis with retrograde intramedullary nailing and functional outcome was evaluated by comparing the preoperative and postoperative Kitaoka Patzer ankle arthrodesis scores and Mazur ankle arthrodesis scores. Statistical Analysis Used: SPSS program for Windows, version 17.0 (SPSS, Chicago, Illinois). Result: The comparison of the preoperative and postoperative scores showed significant improvement from poor to good after tibiocalcaneal arthrodesis was done in such patients. Conclusion: We can conclude that tibiocalcaneal arthrodesis using intramedullary nails is a good option to provide a stable plantigrade foot to the patient though further improvements in implant designs may bring better options.

Talectomy and tibiocalcaneal arthrodesis with intramedullary nail fixation for treatment of equinus deformity in adults.

Severe equinovarus foot deformity in adults is a challenging problem. Conservative treatment rarely is effective, and operative options are limited. The aim of this study was to evaluate the results of talectomy and tibiocalcaneal arthrodesis with intramedullary nail fixation for the treatment of severe equinovarus deformity in adults. Twelve patients (average age 39 years, range 15-70 years) with severe equinovarus deformities of the foot were treated with talectomy and tibiocalcaneal arthrodesis with intramedullary nail fixation between March 2010 and February 2013. Average follow-up was 20 months (range 10-37 months). Tibiocalcaneal fusion was achieved in all patients at an average of 12 weeks (range 8-17 weeks). Preoperatively, all patients had severe, irreducible equinovarus deformities; at last follow-up, almost all feet had mild residual deformity, but were plantigrade and did not require a brace or orthosis. The average AOFAS ankle score improved from 41.1 (range 8-66) preoperatively to 78.4 (range 67-86) postoperatively (P = .02). There was a similar improvement in the average VAS score from 6.3 (range 2-10) preoperatively to 0.8 (range 0-4) postoperatively (P = .02). The combination of talectomy and tibiocalcaneal arthrodesis was effective in correcting severe rigid equinovarus deformity in adults. Removal of the talus resulted in laxity of the soft tissues, making correction of the deformity easier. Tibiocalcaneal arthrodesis achieved a stable foot without the problems associated with talectomy alone. Level IV, case series.

Role of talectomy in management of severe equinovarus deformity in adults

Role of talectomy in management of severe equinovarus deformity in adults

Staged correction of an equinovarus deformity due to pyoderma gangrenosum using a Taylor spatial frame and tibiotalar calcaneal fusion with an intramedullary device

Pyoderma gangrenosum is a rare autoinflammatory syndrome manifested by skin lesions eventually creating ulcers. Surgical management can lead to scarring and contracture at the site of the lesion due to the pathergy phenomenon. A 43-year-old woman presented with a 5-year history of severe equinovarus deformity due to chronic pyoderma gangrenosum on her posteromedial ankle. She underwent a staged fusion. A gradual “closed” correction was performed in a Taylor spatial frame for 8 weeks in order to obviate the need for a surgical release in the area of the ulcer. She was ambulatory and full weight-bearing within 4 weeks of her frame removal. She maintained her correction with an accommodative foot orthosis until she had an uneventful tibiotalar calcaneal fusion with an intramedullary device. This case represents the success of using a Taylor spatial frame for staged fusion involving soft-tissue correction of severe, rigid equinovarus deformity due to pyoderma gangrenosum.

Freeman-Sheldon syndrome-prenatal and postnatal diagnosis

A six-day-old girl, born to normal non-consanguineous parents presented with mask like facies with a small mouth giving a 'whistling' appearance. Other dysmorphic features include deep set eyes, broad nasal bridge, long philtrum and 'H' shaped cutaneous dimple on the chin. There was congenital windmill vane hand position and severe talipes equinovarus deformity. The above features are characteristic of Freeman-Sheldon syndrome also known as Whistling Face syndrome. Ultrasound scanning during 8(th) month of the pregnancy showed the fetus to have facial abnormality and bilateral clenched hand and talipes with extension contractures of knees. Provisional diagnosis of FSS was made which was confirmed after the birth. Thus all cases of Arthrogryposis during prenatal scan should be carefully looked for the facial abnormality in the fetus.

Arthrogryposis multiplex congenital (AMC) in a three year old boy: differential diagnosis with distal arthrogryposis: a case report

IntroductionArthrogryposis multiplex congenital (AMC) is characterized by contractions of multiple joints present at birth. The involved muscles are partially or totally replaced by fat or fibrous tissue. Talipes equinovarus and scoliosis are also frequently reported.Case presentationThis 2 year was born after uneventful pregnancy, with normal birth weight and length. The parents are unrelated, young and healthy. No malformations or mental retardation have been reported in the family. Since his birth a specific posture was noted: internal rotation at the shoulders, extension at the elbows, and flexion at the wrists. In addition, the child has a severe equinovarus deformity of the feet. Syndactily between II and III finger was also noted. His face is round with a frontal midline capillary hemangioma, while his jaw appears to be small. Mental development is normal. The karyotype is: 46, XY.ConclusionsAbout 150 syndromes have arthrogryphosis as a presenting sign. AMC is a distinct entity and distinction with the distal forms of arthrogryphosis can be difficult, since there is a considerable clinical and genetic heterogeneity. A comprehensive musculoskeletal evaluation and genetic consultation is necessary.

Primary Shortening with Secondary Limb Lengthening for Gustilo IIIB Open Tibial Fractures: A Report of Six Cases

The treatment of type IIIB open tibial fractures remains a challenge for orthopedic surgeons, particularly with respect to the soft-tissue and subsequent bony reconstruction. The primary shortening and limb lengthening (PSLL) simplifies wound closure for severe open injuries without requiring microsurgical procedures as a main advantage. This method is thought to be also useful for type IIIB patients with polytrauma and other life-threatening injuries because it helps to control both wound sepsis and their general state. In the present study, we attempted to assess the problems, long-term functional outcome, and quality of life (QOL) of patients who were treated by PSLL for Gustilo type IIIB open tibial fractures in our facility. Six patients with type IIIB open tibial fractures treated with PSLL were retrospectively reviewed. The mean shortening length was 7.4 cm (range, 4.5-10.3 cm). The mean percent shortening of the entire bone was 18.7% (range, 12.3-29.7%). Limb lengthening started at a mean interval of 10.3 months (range, 3-18 months) after the original injury. The mean healing index was 56.5 days/cm (range, 31.3-86.7 days/cm). The complications, functional outcome, and quality of life were evaluated for all cases. One superficial infection at the initial corticotomy, one deep infection around the shortening site, one refracture at the healed docking site, several wire breaks in external frames in two cases, and two severe equinovarus deformities occurred as complications of these procedures. Regarding functional outcome, three patients showed good outcome, two showed fair outcome, and one showed poor outcome. The percent shortening of the entire bone in the two fair cases were more than 25%. The median scale of physical health summary, mental health summary, and total general health summary in Short Form-36 (QOL) were lower than the standard scale in age-matched individuals. This PSLL treatment was thought to be a useful option for severe open fracture of the tibia, which had bony defect in more than 4.5 cm in length after serial debridement, although several complications occurred in this regimen. However, it is difficult to achieve an excellent function and QOL using these techniques. In addition, it is difficult for patients who underwent limb lengthening after shortening more than 25% of the total length of bone to gain good function.

Partial wound closure after surgical correction of equinovarus foot deformity using a synthetic skin substitute

We retrospectively reviewed the results of partial wound closure after surgical release of severe equinovarus deformity in forty-eight (48) feet in thirty-two (32) patients. Epigard®, a commercially manufactured synthetic skin substitute was used as a temporary coverage medium. The decision to perform synthetic skin substituted partial closure was made intraoperatively, if primary closure with the foot in the corrected position threatened circulatory compromise, or loss of correction. We did not find it necessary to deviate from our usual protocol of 6 weeks of postoperative casting, with one cast change at 3 weeks for hardware removal. There were no infections. No feet required grafting or other secondary procedures, including dressing changes. We conclude that synthetic skin substituted partial wound closure is a viable alternative after surgical correction of the severe equinovarus deformity. This technique avoids the inconvenience of frequent cast changes or uncovered partial wound closure, and the expense and increased surgical risk of skin grafting and rotational flap techniques.

Tendon transfer of antagonist muscles in patients with severe equinovarus deformity after adolescence

Tendon transfer of antagonist muscles in patients with severe equinovarus deformity after adolescence

Poster 16: Use of open phenol blocks in the treatment of lower-extremity spasticity in an adult with severe traumatic brain injury: a case report

Setting: Free-standing acute rehabilitation facility. Patient: A 37-year-old man with severe traumatic brain injury. Case Description: Physical examination revealed spasticity of bilateral lower extremities (rated as a 4 on the Ashworth Scale) resulting in severe equinovarus deformities. This resulted in the patient’s inability to stand, transfer, and ambulate. Treatments with positioning, splinting, serial casting, dantrolene, transcutaneous phenol blocks, and botulinum toxin type A injections showed no improvements. Further investigation of spasticity management revealed another treatment option. Open phenol blocks to the bilateral tibial nerves at the popliteal fossa were performed. The patient tolerated the procedure well. Assessment/Results: 2 weeks after bilateral open phenol blocks, the patient’s ankle range of motion improved. He was able to maintain both feet in a neutral position and that led to an increased ability to aid in transfers and standing. With physical therapy and casting, the patient was able to ambulate using a walker. Discussion: Although open phenol blocks were once well-known procedures, they are no longer considered current treatment options. Conclusion: This case demonstrates the effectiveness of injecting phenol directly into the nerve to decrease spasticity. An open phenol block is an effective treatment of severe spasticity and should be considered.

Eight year's experience with soft tissue release and Alkhooly external fixator in the treatment of resistant clubfoot

Summary From Aug 1992 to Feb 2001, thirty-two operations on the foot and ankle were performed in the Department of Orthopedics and Traumatology of El-Minia University Hospital in 24 children, 16 boys and 8 girls suffering from severe clubfoot deformity. There were eight cases with bilateral and 16 with unilateral affection. Their ages ranged from three to three and a half years. One of the unilateral deformed patients was aged 13 years and had severe equinovarus deformity due to burn contracture. One case aged 14 years had bilateral deformity that was treated when she was 2 years old and upon recurrence, it was neglected until that age. They were treated by soft tissue release and skin flaps with either rotation or multiple Z-plasty supplemented with the Alkhooly external fixator. The followup period ranged between 4 and 8 years. The results according to Mittal (1987) were excellent in 23 feet (71.9%), good in 8 feet (25%) and poor in one foot (3.1%).

Foot Lengthening Using the Ilizarov Device: The Transverse Tarsal Joint Resection Versus Osteotomy

The aim of the study was the evaluation of both the foot correction and foot lengthening obtained using the distraction method with osteotomies versus distraction after the transverse tarsal joint resection. Ten patients (10 feet) aged from 5 years to 24 years (average, 10.5 years) were analyzed. Seven of them were treated for severe equinovarus deformity: six of congenital and one of posttraumatic etiology. In three patients, the indication for treatment was foot shortening due to hypoplasia with tibial shortening, combined with foot deformity. Preoperative shortening of the foot ranged from 1.5 cm to 10 cm (average, 4.5 cm). In four patients, osteotomy between the tarsometatarsal and transverse tarsal joints was carried out. In two cases, 'V-shaped' osteotomy through the hindfoot and midfoot was performed. In the remaining four patients, wedge resection of the transverse tarsal joint was performed. The follow-up was a mean of 32 months (range, 12-55 months). It was observed that foot lengthening after transverse osteotomy of the midfoot is difficult and unpredictable, because of distraction at the adjacent joints level instead of osteotomy site. The greatest lengthening of the foot (mean, 4 cm) was observed in the patients with the transverse tarsal joint resection. It was concluded that the transverse tarsal joint resection following callus distraction in the place of the resected joint is the effective method for foot lengthening, which can be combined with deformity correction.

Single-stage talectomy and tibiocalcaneal arthrodesis as a salvage of severe, rigid equinovarus deformity.

Seven adult males with rigid, severe equinovarus deformities underwent single-stage corrective surgery. The procedure included extensive soft tissue release, talectomy and tibiocalcaneal, calcaneocuboid, and tibionavicular fusion. All seven patients fused successfully at an average of 3.5 months with a plantigrade foot. All feet were stiff and had an average of 3.5 cm limb length discrepancy. All of the patients were satisfied with the surgery and would choose to have the procedure again. This small series represents one method of achieving a plantigrade foot in patients with severe, rigid equinovarus deformities.

Complex foot deformities associated with soft-tissue scarring in children

Two cases of deformities in scarred feet are presented. One case had an old, well healed forefoot amputation with severe equinovarus deformity, and the other had an equinus deformity following a burn injury 10 months prior. Both the cases were managed by primary release of the contracted joint capsules. The correction of the soft-tissue contractures was achieved by gradual distraction using the Ilizarov apparatus. The clinical presentation and surgical treatment of complex foot deformities, complicated by the presence of scar tissue, are presented. These cases illustrate the benefits of combining soft-tissue release with the Ilizarov technique of distraction histogenesis in the treatment of complicated foot deformities associated with scarring in pediatric patients.

神経筋疾患に伴う高度両尖足の治療経験

We report a case of severe equinovarus deformity successfully treated only by soft tissue release in a 18-year-old male patient who was suspected of some type of neuromuscular disease such as spinal muscular atrophy. He began to walk at 1 year and 9 months of age, but showed Gower's sign and wadding gait at 2 year of age and could not walk independently at 8 years of age. Equinovarus deformities of both his feet appeared and increasingly worsened. At 18 years of age, he could stand only on his toes with pain but could hardly walk even with the aid of braces or crutches. We, therefore, performed posterior release of both ankles including Achilles tendon lengthening. The equinus contracture improved from 60 degrees to 20 degrees and when toe pain was found to almost diminish when standing, with improved stability.

The role of magnetic resonance imaging in the investigation of spinal dysraphism in the child with lower limb abnormality.

A review of magnetic resonance imaging (MRI) performed to exclude the presence of spinal dysraphism in children presenting with lower limb pathology is unrewarding in the absence of abnormal neurology. Over a 5-year period, 29 children ages 2 weeks to 15 years with a mean age of 6 years presenting with lower limb abnormalities were referred for MRI of the spine to exclude an occult neurologic cause for the deformities. More than one limb abnormality, for example pes cavus and limb length discrepancy, was present in 93% of the children, and 11 children had severe or recurrent talipes equinovarus deformity. Only two children (7%), both of whom had abnormal limb neurology, had abnormal MRI scans. In the absence of a demonstrable neurologic deficit in the lower limb, there appears to be no advantage in requesting MRI of the spine in children presenting with lower limb abnormality.

Talectomy in the treatment of resistant talipes equinovarus deformity in myelomeningocele and arthrogryposis.

Eighteen patients (28 feet) with spina bifida and arthrogryposis had talectomy for correction of severe equinovarus deformity. In 26 feet, posteromedial release had been performed previously. The average age at surgery was 4 1/2 years, with a range of 1-9 years of age. The length of follow-up averaged 4 years and ranged from 12 months to 8 years. Twenty-three feet were rated good, and five were poor. Six feet, because of forefoot adduction, required further surgery (metatarsal osteotomy). Talectomy is an effective procedure for correction of hindfoot deformity. Forefoot problems must be treated as a separate entity.