Outcomes of Limb Lengthening With Computer-assisted Hexapod External Fixators in the Treatment of Fibular Hemimelia.

BACKGROUND: Fibular hemimelia (FH) represents the most common deficiency of the long bones and is associated with multiple deformities. Reconstructive treatment with external fixators in FH restores normal lower extremity alignment and length with plantigrade feet for a balanced and effective gait. The aim of this study is to evaluate the outcomes of lower limb lengthening and simultaneous tri-plane deformity correction with a computer-assisted hexagonal external fixator in children with FH. METHODS: A retrospective review was performed for FH cases treated with a computer-assisted hexagonal external fixator in a tertiary referral center. Leg length discrepancy (LLD), interphyseal angles, tibiocalcaneal distances, healing index (HI), and callus shapes were analyzed for radiologic evaluation, and the Pediatric Quality of Life Inventory (PedsQL) was used for functional assessment. Limbs with HI <50 days/cm, PedsQL >75, and without regenerate fractures were considered successful lengthenings. RESULTS: Twenty-four limbs of 23 patients were included. The limbs were lengthened for a mean of 7.24 cm (range, 4.7 to 15.6). The initial LLD of 5.6 cm (range, 0.5 to 19 cm) increased to 1.7 cm (range, 0.1 to 6 cm), and the mean interphyseal angle was 12.7 degree (range, 1.5 to 54.2 degree), tibiocalcaneal distance was 0.85 cm (range, 0.1 to 1.7) at final follow-up. The most common regenerated bone morphology was cylindrical, as seen in 11 limbs (45.8%). The average PedsQL score was 83.5 (range, 69.5 to 96.7). Sixteen limbs (66.7%) had successful lengthening at their first, and 4 limbs (80%) had successful lengthening at their second surgeries. Seven limbs had complications requiring surgical intervention (29.1%), with 3 (12.5%) regenerate fractures after external fixators removal. CONCLUSIONS: Limb reconstruction with computer-assisted hexapod fixators is a successful and reliable option for the treatment of LLD in FH, and patients demonstrate good functional outcomes. Surgeons should be aware of potential complications and should utilize prophylactic measures when necessary. LEVELS OF EVIDENCE: Level III, retrospective comparative study.

Anesthetic management in an infant with tetra-amelia syndrome with congenital maxillomandibular fusion: A case report.

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.

Amputation Versus Limb Reconstruction for Fibula Hemimelia: A Meta-analysis.

PURPOSE: Fibula hemimelia is the most common congenital deficiency of long bones. Primary treatment options include amputation with prosthetic fitting or limb reconstruction. The aim of our study was to conduct a systematic review comparing amputation with limb reconstruction for fibula hemimelia. METHODS: MEDLINE, EMBASE, Web of Science, Elsevier Scopus, and the Cochrane Registry of Clinical Trials were searched from 1951 to 2019 for studies that evaluated amputation versus limb reconstruction for fibula hemimelia. Random effect models were utilized for the meta-analytic comparisons of amputation versus limb reconstruction for patient satisfaction and surgical complications. Descriptive, quantitative, and qualitative data were extracted. RESULTS: Seven retrospective cohort studies were eligible for the meta-analysis, with a total of 169 fibula hemimelia cases. Amputation resulted in an odds ratio of 6.8 (95% confidence interval: 2.4, 19.2) when compared with limb reconstruction in terms of patient satisfaction. Furthermore, limb reconstruction was found to have an odds ratio of 28 (95% confidence interval: 7.8, 100.3) for complications. The total surgical complication rates in the amputation and limb reconstruction groups were 0.2 and 1.2 complications per limb. The rate of surgical procedures per patient was 1.5 and 4.2 for amputation and limb reconstruction, respectively. CONCLUSIONS: The cumulative evidence at present indicates better patient satisfaction with less surgical complications and less number of procedures with amputation for fibula hemimelia when compared with limb reconstruction. Absence of uniform protocols make it difficult to compare results accurately. LEVEL OF EVIDENCE: Level III-therapeutic.

Fronto-Orbital Advance in a Patient With Roberts Syndrome.

Roberts Syndrome is an extremely rare syndrome reporting about 150 cases in the literature, with a very low survival rate. The authors present a case of a female patient with Roberts Syndrome who also had a coronal craniosynostosis. The aim of this case report is to present a case of a patient with Roberts Syndrome with a brachycephaly that required management of fronto-orbital advancement. In conclusion Roberts Syndrome is a rare disease, which can have different skeletal variations. This syndrome can manifest itself with craniosynostosis, with the requirement of a comprehensive management to correct it and avoid compression of the brain with endocranial hypertension.

Three-dimensional correction of fibular hemimelia using a computer-assisted planning : technical report and literature review.

The purpose of this survey in Belgium and the Netherlands was to assess treatment variation in glenohumeral osteoarthritis between experienced and less experienced orthopedic surgeons, and to investigate perioperative treatment after shoulder arthroplasty in a large group of orthopedic surgeons. Orthopedic surgeons specialized in shoulder surgery were invited to complete a survey between November 2013 and February 2015. Seventy-one percent of the approached surgeons com-pleted the survey. Less experienced surgeons (< 6 years) and surgeons from the Netherlands find patient characteristics (e.g. smoking p=0.01) more relevant than more experienced surgeons (≥ 6 years) and surgeons from Belgium. Less experienced surgeons will less likely (p=0.001) perform resurfacing arthroplasty compare to experienced surgeons. The less and the experienced surgeons use similar indications for a reverse shoulder arthroplasty regarding age limit and cuff arthropathy without osteoarthritis. Less experienced surgeon will more likely (p=0.003) prescribe a low molecular weight heparin during the hospital stay after a shoulder arthroplasty. In this survey, we found a decrease in the use of resurfacing arthroplasty and a strong increase in the use of reverse shoulder arthroplasty. Besides, there is little consensus concerning pre-operative planning, patient characteristics, surgical technique, and patient reported outcome measures. Level of evidence: IV.

Toe-to-Hand Transfer in an Ulnar Hemimelia Patient: A Case Report.

Ulnar hemimelia is a rare congenital deformity. The severity of the deformity increases with the number of fingers that are absent. Clearly, grip function is impossible with a mono-finger hand. Here, we present a case report of a patient with bilateral ulnar hemimelia that included the absence of radial fingers and also deformity in the toes of his left foot. A toe-to-hand transfer was performed successfully to help the patient gain grip function. Both the patient and the parents were very pleased with the result. To our knowledge, this is the first case report of a patient with ulnar hemimelia and absent radial fingers who was treated with a toe-to-hand transfer.The most important factors in a successful surgery are careful preoperative planning and microsurgical expertise. Angiography is the key to careful planning, and physiotherapy should also be done to increase the patient's functions. The psychological well-being of patients and parents will be positively affected after a successful surgery.

Use of Paley Classification and SUPERankle Procedure in the Management of Fibular Hemimelia.

BACKGROUND: Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities. METHODS: We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014. Among the 29 patients, 27 were treated with reconstructive procedures. Type 1 (8 patients) cases were treated with only limb lengthening, and correction of tibial deformities. Type 2 (7 patients) cases were treated by distal tibial medial hemiepiphysiodesis or supramalleolar varus osteotomy. In type 3 (10 patients) cases, the foot deformity was corrected using the SUPERankle procedure. Type 4 (2 patients) cases were treated with supramalleolar osteotomy along with posteromedial release and lateral column shortening. In a second stage, limb lengthening was performed, using the Ilizarov technique. In the remaining 2 patients (type 3A and type 3C), amputation was performed using Syme technique as a first choice of treatment. RESULTS: The results were evaluated using Association for the Study and Application of Methods of Ilizarov scoring. Excellent results were obtained in 15 of 27 (55%) patients. Six (22%) patients had good results, 4 (14.8%) had fair results, and 2 (7%) had poor results. Mean limb length discrepancy at initial presentation was 3.55 cm (range: 2 to 5.5 cm) which significantly improved to 1.01 cm (range: 0 to 3 cm) after treatment (P=0.015). CONCLUSIONS: Our results and a review of the literature clearly suggest that limb reconstruction according to Paley classification, is an excellent option in the management of fibular hemimelia. Our 2-staged procedure (SUPERankle procedure followed by limb lengthening) helps in reducing the complications of limb lengthening and incidence of ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities. LEVEL OF EVIDENCE: Level IV.

Functional Assessment in Tibial Hemimelia (Can We Also Save the Foot in Reconstruction?).

BACKGROUND: The congenital absence of the tibia is a rare disease, and an orthopaedic surgeon may not encounter such cases during the course of his/her career. This is the largest report to date of the management of such cases by a single surgeon. The foot and leg were persevered in the majority of the cases, and a functional evaluation system was used to report outcomes. METHODS: Thirty-six patients with tibial hemimelia, who had been under the direct care of the authors since infancy, were evaluated clinically and radiographically. The patients or their parents filled out the Pediatric Quality of Life and the parents' satisfaction forms. The surgical interventions performed, and their effects on school attendance and, and also the shoe type they wore were documented. RESULTS: Thirty-six patients (19 girls and 17 boys) with 48 tibial-deficient limbs (19 right, 5 left, and 12 both right and left sides) were studied. The patients were assessed at 12 years (2.5 to 32.5 y), with a mean follow-up of 9 years (2 to 23 y). The 48 limbs included 14 type I, 16 type II, 11 type IV, and 7 unclassified by using the Jones classification; and 6 type I, 11 type II, 16 type III, 1 type IV, and 14 type VII by using the Weber classification. Primary amputation was performed in 8 patients (10 limbs) and limb preservation surgeries on 38 legs (28 patients). Tibiofibular synostosis, centralization of the ankle, and Ilizarov lengthening were the most common procedures. Nonunion of tibiofibular synostosis (2 cases) and knee stiffness (6 cases) were the main complications. Among the reconstructed limbs, 12 were in regular and 18 in modified shoes. The Pediatric Quality of Life of 68 points in the reconstructed group was a significant achievement, and it was also better than the score of patients who had undergone amputation. CONCLUSION: Reconstruction of tibial hemimelia with foot preservation provides good functional outcome in the majority of cases. LEVEL OF EVIDENCE: Level IV.

Prevention of recurrence of tibia and ankle deformities after bone lengthening in children with type II fibular hemimelia.

PURPOSE: This study aimed to evaluate development of the tibia after Ilizarov lengthening and deformity correction depending on whether or not the simultaneous resection of fibular anlage was performed in children with fibular aplasia type II, who did not undergo early surgery. METHODS: The study analyses results of reconstructive treatment in 38 children at the age of over four years. Two groups of children are compared: bifocal tibial lengthening with the Ilizarov device (group I) and bifocal lengthening associated with resection of the fibular anlage (group II). The results were estimated at 12 months and in the long-term exceeding three years. RESULTS: Radiological data of measurement of the anatomical lateral distal tibial angle (aLDTA) show surgical correction of deformities achieved in both groups. During the further limb growth a tendency to normalisation of the aLDTA was observed only in the group II. Quick relapse of the angular deformities of the tibial shaft in the first group occurred mainly during further growth of the limb regardless of complete correction at the time of treatment. On the other hand, there were no recurrences of diaphyseal deformities in the group II. CONCLUSIONS: In children with congenital fibular deficiency of type II at the age of four years, the bone lengthening and deformity correction should be associated with fibular anlage resection. That approach improves conditions for distal tibia development and prevents or decreases significantly the recurrence of deformities of the tibia and ankle joint in long-term follow-up.

Patient Satisfaction After Limb Lengthening With Internal and External Devices.

External fixation has long been used for limb lengthening but can result in many complications, such as tethering of the soft tissues, pain, decreased joint motion, scarring, and nerve injury. Recently, a controllable, telescopic, internal lengthening nail was developed to address many of these issues and hopefully improve the overall experience for the patient. The satisfaction rates of internal and external fixation for limb lengthening were compared in 16 patients, all of whom have experienced both methods. Thirteen out of 16 patients responded to a limb-lengthening questionnaire, developed by the authors for this patient population. Patients preferred the internal device with respect to overall satisfaction, reduced pain, ease of physical therapy, and better cosmetic appearance. When asked which device they would prefer if another surgery was required, all patients chose the internal device. From the patients' perspective, the internal lengthening device is an improvement over the traditional external fixator.

A child with split-hand/foot associated with tibial hemimelia (SHFLD syndrome) and thrombocytopenia maps to chromosome region 17p13.3.

We describe a-2-year-old boy who presented with a neonatal history of thrombocytopenia associated with a constellation of limb malformations mimicking split hand/foot malformation with long bone deficiency (SHFLD) syndrome. Limb malformations consisted of unilateral monodactyly with radial aplasia, unilateral split foot and bilateral club foot. Tibial aplasia of one limb and tibial hypoplasia of the other limb were notable. Partial agenesis of the sacrum was additional skeletal malformation. Craniofacial features included dense thick scalp hair, narrow frontal area, thick eye-brows, deep-set eyes, depressed nasal bridge, and small overhanging nasal tip, full-cheeks, and large ears. Array-CGH showed duplication of the short arm of chromosome 17p13.3 in the boy and his father, respectively. The father was free from any skeletal abnormalities, though he shares similar craniofacial dysmorphic features like his son. In addition, a paternal sib (uncle of the proband) manifested a phenotype similar to that of the proband. To the best of our knowledge the overall phenotypic and genotypic characterizations were consistent but not completely compatible with the traditional type of TAR syndrome or with SHFLD syndrome. We report on what might be a novel variant of SHFLD associated with transient thrombocytopenia, dysmorphic facial features, and a constellation of bone malformations.

Internal lengthening device for congenital femoral deficiency and fibular hemimelia.

BACKGROUND: Patients with congenital limb shortening can present with joint instability, soft tissue contractures, and significant leg length discrepancy. Classically, lengthening is done with external fixation, which can result in scarring, pin site infection, loss of motion, and pain. We therefore developed an alternative to this approach, a new, controllable, internal lengthening device for patients with congenital limb shortening. QUESTIONS/PURPOSES: We evaluated this device in terms of (1) healing index, (2) complications, (3) accuracy of the device's external controller, and (4) adjacent-joint ROM. METHODS: Between January 2012 and May 2013, we treated 66 patients for congenital limb shortening, of whom 21 were treated using this device. During this period, general indications for using the device were patients with leg length discrepancies of 2 cm or more, with intramedullary canals able to withstand rods of at least 12.5-mm diameter and 230-mm length, without active infection in the affected bone, able to comply with the need for frequent lengthening, and without metal allergies or an implanted pacemaker. We included only those patients who had completed their course of treatment and were currently fully weightbearing, leaving 18 patients (21 bone segments) available for followup at a minimum of 6 months after limb lengthening (mean, 14 months; range, 6-22 months). Mean age was 19 years (range, 9-49 years). Sixteen femurs and five tibias were lengthened a mean of 4.4 cm (range, 2.1-6.5 cm). Mean distraction index was 1.0 mm/day (range, 0.5-1.8 mm/day). Healing index, complications, device accuracy, and ROM were recorded. To date, 10 of the 21 devices have been removed. This was typically done 12-24 months after insertion when the bone was solidly healed on all four cortices. RESULTS: Mean healing index was 0.91 months/cm (range, 0.2-2.0 months/cm). There were seven complications requiring an additional unplanned surgery, including one hip flexion contracture, three femurs with delayed healing, one tibia with delayed healing, one hip subluxation/dislocation, and one knee subluxation. The external controller was accurate as programmed and actual lengthening amounts were consistent. ROMs of the hip, knee, and ankle were essentially maintained. CONCLUSIONS: This device is completely internal, allowing for satisfactory joint motion during treatment in most patients. Lengthening was achieved in an accurate, controlled manner, and all patients reached their goal length. Complications remain a concern, as is the case with all approaches to this complex patient population. Both future comparative studies and longer-term followup are needed. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Instructions for Authors for a complete description of levels of evidence.

[Congenital tibial hemimelia]. / Die kongenitale Tibiahemimelie.

BACKGROUND: The congenital tibia hemimelia, also called tibial deficiency, is a rare disorder with unknown cause, showing many associated abnormalities or varying syndromes. METHODS: The correct diagnosis can be easily established using radiographs and/or magnetic resonance imaging in the postpartum setting. However, treatment may be difficult and needs to take into consideration the given anatomic situation in the knee and ankle joint. CONCLUSION: Prosthetic fitting may be possible in mild cases. Nevertheless, the majority of patients need to undergo surgical reconstruction in order to restore a functional, mobile, and stable knee and ankle joint.

Management of fibular hemimelia using the Ilizarov method at Siriraj Hospital in Thailand.

BACKGROUND: Fibular hemimelia is one of the most common congenital longitudinal bone deficiencies. Previous treatment protocols called for amputation of the deficient limb; while others made attempts to save the limb. The objective of treatment is to restore function and achieve patient satisfaction. The authors evaluated the outcomes of the Ilizarov technique for the treatment of leg-length discrepancy and bone associated deformities in patients with fibular hemimelia. The present study also evaluated and assessed complications, knee and ankle function, and patient satisfaction with the treatment. MATERIAL AND METHOD: Nine patients with fibular hemimelia who underwent tibial lengthening using the Ilizarov method were reviewed in the present study. Initial condition data, including age, gender type offibular hemimelia, initial limb-length discrepancy, predicted limb-length discrepancy, and the data were collected and analyzed. Activity level, patient satisfaction, complications, and residual leg-length discrepancy were assessed at the end of treatment. RESULTS: According to Achterman and Kalamchi classification, there were 4 patients with Type IA, 3 patients with Type IB, and 2 patients with Type II. In Type IA, the affected leg-length discrepancy and mean age at the initial treatment were 3.25 cm and 7.75 years, respectively. In type IB, the affected leg-length discrepancy and mean age at the initial treatment were 5.83 cm and 4.3 years, respectively. In Type II, the affected leg-length discrepancy and mean age at the initial treatment were 5.5 cm and 5 years, respectively. The mean follow-up was 5 years (range: 7-10). The mean lengthening was 7.52 cm (range: 4-13). The lengthening index was 1.28 mo/cm. The mean residual leg-length discrepancy was 0.94 cm. There was ankle joint stiffness and mild equinous foot in type II cases, but patients could walk well without gait aid. No patients were experiencing pain by the end of treatment. All patients expressed satisfaction with this technique. CONCLUSION: The Ilizarov technique for bone lengthening of the tibia has shown satisfactory results in the treatment of all types of congenital fibular hemimelia and should be considered an attractive alternative to amputation, as measureable functional improvement can be expected.

The role of Taylor Spatial Frame for the treatment of acquired and congenital tibial deformities in children.

This study evaluates the use of the Taylor Spatial Frame (TSF) for the correction of acquired and congenital tibial deformities in children. The purpose is to underline problems, obstacles and complications that can be observed during treatment to reveal the learning curve and potential risk factors and to propose solutions to avoid difficulties during its use 86 tibia deformities were corrected in 66 children during a period of 7 years and were classified according to anatomical and dominant type of deformity. Follow up was 54.2 months. Gradual correction was performed according to the individualized time schedule. We faced 42 difficulties: 29 problems, 10 obstacles and 3 complications, distributed across all years. Significant correlation was found between patient's age and number of difficulties. The incidence of the difficulties was equally spread over the different etiologies, but it was statistically significant across the years. Proximal tibia and complex multi-plane deformities seem to be related to an increased incidence of postoperative difficulties. TSF can yield accurate results, is easy to handle and provides an excellent concomitant 3-direction correction.

Novel prosthesis with proximity sensor for upper extremity phocomelia.

We herein report on the application of a novel motorized prosthetic hand in a child with upper extremity phocomelia.

Outcomes of Simultaneous Lengthening and ACL Reconstruction in Fibular Hemimelia: A Retrospective Case Series.

Background: Fibular hemimelia is the most common congenital long bone deficiency. It is often associated with femoral and tibial deficiencies which result in a clinically evident leg length discrepancy. The primary soft tissue concern is ACL/PCL deficiency. If treatment includes bony lengthening, joint stability is imperative to avoid complications. In this study, we detail a novel technique for long bone lengthening and ACL reconstruction in a single, cohesive surgery. This consolidates the need for multiple procedures and offers improved limb length symmetry and knee stability for this patient population. Clinical outcomes of pediatric patients with hemimelia who underwent either femoral or tibial lengthening with PRECICE® nail and concomitant ACL reconstruction are presented. Methods: After IRB approval, we identified five patients with complex fibular hemimelia who underwent ACL reconstruction and concomitant lengthening with at least two years of follow-up. Two patients (40%) presented with congenital short femur, and three (60%) with congenital short tibia. In each case, ACL reconstruction and either femoral or tibial guided growth via PRECICE® nail were performed. Operative techniques involving both soft tissue and bony methodology are described in detail. Results: All patients had objective improvement in knee stability as assessed both intra and post operatively, as well as successful intermedullary lengthening without complications related to joint stability. Three patients had minor complications unrelated to joint stability that did not interfere with overall result. Conclusion: Fibular hemimelia associated with hypoplasia of bony and soft tissue structures can be successfully addressed with concomitant ligamentous reconstruction at the time of implantation of lengthening devices. This addresses knee instability and reduces both number of operative procedures and potential complications related to joint instability while pursuing bony lengthening. Level of Evidence: V.

Fibular hemimelia and risk of patellar dislocations: A case series.

The likelihood of patellar instability and consequently, risk of patellar dislocations is higher in those with anatomical abnormalities. Fibular hemimelia is a congenital disorder resulting in partial or full absence of the fibula, often with absence of the lateral and cruciate ligaments, although this patient group rarely undergoes ligament reconstruction. There is potential for adverse outcomes, in the longer term, including, possible increased risk of patellar dislocation and pain in the knee and hip. We aim to investigate the potential risk of spontaneous, unprovoked patellar dislocation among patients with fibular hemimelia, through a review of medical records and radiological investigations. All patients with a diagnosis of fibular hemimelia were included (n = 25), regardless of ultimate approach to management. Tibiofemoral angle measurement and Caton-Deschamps indices were calculated where suitable radiology was available, to better establish extent of potential patellar instability. All the patients with normal Caton-Deschamps indices had only partial fibular absence, although this does not detract from absence or hypoplasia of the anterior cruciate ligament, as a risk factor for patellar dislocation by predisposing to anterior tibial translation. Notably, of the three patients with increased Caton-Deschamps indices, two had complete fibular absence and underwent definitive amputation surgery at age 18 months and 3 years, respectively. Ultimately, this was a young patient group and on-going follow-up might yield better understanding of knee stability. Maintaining a well-aligned lower limb throughout growth might be protective even in the presence of anatomical abnormalities. This article mainly aims to raise awareness among prosthetic and orthotic professionals regarding the increased risk of patella dislocations.

Case report: vertebral body tethering for idiopathic scoliosis in a patient with bilateral phocomelia.

PURPOSE: The purpose of this study is to present a case report of a patient with bilateral upper extremity phocomelia with progressive scoliosis, who underwent vertebral body tethering (VBT). METHODS: This is a case report on the use of VBT in a patient with scoliosis and bilateral congenital phocomelia, with 5 year follow-up. RESULTS: A male patient with bilateral phocomelia had early onset scoliosis that progressed to 45° at age 10. Surgical options were discussed, including traditional VBT, posterior spinal fusion, growing rods, magnetically controlled growing rods, and vertical expandible prosthetic titanium ribs. These options would limit the flexibility of the spine. Given these pitfalls, VBT was chosen, as it would address the scoliosis while maintaining trunk flexibility. Preoperatively, he had 45° right main thoracic curve, bending to 22°; he was Risser 0 with open triradiate cartilage. He underwent T6-T11 thoracoscopic VBT, with postoperative correction to 37°. Postoperatively, the patient was able to continue to use his lower extremities for writing, feeding, and personal grooming. He had no postoperative complications. At 3 years, his curve was 21°, and at 5 years was 19°. CONCLUSION: This case describes a novel technique for treating scoliosis in patients with bilateral phocomelia. Other forms of scoliosis surgical treatment limit motion of the spine. Due to this, we present VBT as an option for this unique set of patients for correcting scoliosis, while also preserving trunk flexibility for its role in feeding and self-care.

Simultaneous femoral and tibial lengthening for severe limb length discrepancy in fibular hemimelia.

BACKGROUND: Fibular Hemimelia (FH) is the most common longitudinal limb deficiency. Significant limb length discrepancy (LLD) will necessitate long treatment times and multiple settings to compensate for LLD when associated with femoral shortening. This study evaluates the outcome of simultaneous femoral and tibial lengthening using the Ilizarov frame. METHODS: This retrospective study included the cases of 12 children with severe limb length discrepancy caused by combined FH and ipsilateral femoral shortening from May 2015 to August 2022. The total LLD ranged from 7 to 14.5 cm. All patients underwent single-session femoral and tibial lengthening using the Ilizarov ring external fixator technique. Additional procedures were performed in the same setting, including Achilles tendon lengthening, fibular anlage excision, peroneal tendons lengthening, and iliotibial band release. Follow-up ranged from 2 to 4 years. RESULTS: The planned limb lengthening was achieved in ten cases (83%). No cases of joint subluxation or dislocation were encountered. No neurovascular injury has occurred during the treatment course. In all cases, the bone healing index was better on the femoral side than on the tibia. Poor regeneration and deformity of the tibia occurred in two cases (16.6%). CONCLUSION: Simultaneous femoral and tibial lengthening using the Ilizarov fixator is a relatively safe procedure with the result of correction of total LLD in one session in a shorter time and less morbidity.