The Role of Taylor Spatial Frame in the Treatment of Blount Disease.

BACKGROUND: Alteration of the posteromedial part of the proximal tibia is the main characteristic of Blount's disease and if left untreated, leg alignment and normal development of the lower limbs may be compromised. AIM: To report treatment outcomes in children with Blount's disease using the Taylor Spatial Frame (TSF). MATERIALS AND METHODS: From January 2007 to December 2014, 16 young children (24 tibia) with a mean age of 7.5 years (range of 3-14 yrs) and severe Blount's disease were treated using TSF. Preoperative long standing radiographs were performed and anatomic medial proximal tibial angle (MPTA), diaphyseal-metaphyseal tibial angle (Drennan), femoro-tibial angle and leg length discrepancy (LLD) were measured. RESULTS: Post-operative improvement of all measurements was observed. MPTA increased from a mean of 71.8° (58° - 79°) to 92.5° (90° - 95°), the Drennan decreased from 16.6° (14° - 18°) to 3.6° (0° - 6°), the F-T angle changed from 15.4° (10° - 25°) of varus to 5.9° (2° - 10°) of valgus and the LLD decreased from 208 mm (150-320) to 69 mm (0- +120). Mean follow-up was 45.6 months. According to Paley's criteria pin track infection was present in 6 tibiae, while in 5 patients software changes were necessary. Recurrence was observed in 3 patients (triplets). Complete restoration of the mechanical axis was obtained at the end of the treatment. CONCLUSIONS: In the last decades, different surgical treatments have been proposed for Blount's disease (tension band plate, staples, osteotomies using external or internal fixation). External fixation using the TSF allows gradual safe correction of multiplanar deformities and is a well-tolerated technique by patients with Blount's disease.

W/M serrated osteotomy for infantile Blount's disease in Ghana: Short-term results.

PURPOSE: The W/M serrated high tibial osteotomy is a not frequently described surgical technique for simultaneously correcting the varus and torsional deformity in patients with Blount's disease. Without the need for internal fixation, this surgical treatment is well suited for developing countries. This study describes the short-term results of the bilateral and unilateral W/M serrated osteotomy in patients with infantile Blount's disease. METHODS: Between May 2008 and January 2013, 52 patients were treated with uni- (n = 22) or bi-lateral (n = 30) W/M serrated osteotomy of the proximal tibia due to a tibial varus deformity in two district hospitals in Ghana. Other causes than infantile Blount's disease were excluded from the analysis. Pre- and post-operative clinical and radiological measurements were done, and complications were monitored up to 12 weeks after surgery. RESULTS: Seventeen patients (five males, 12 females; mean age 4.9 [standard deviation: 2.10]) were included, which underwent a total of 25 W/M serrated osteotomies. The femorotibial angle was corrected from 34.1° ([mean] range: 6-68°) to - 7.1° ([mean] range: -28-5°). Only one patient had developed a wound infection, and all reached full consolidation. CONCLUSIONS: The W/M serrated osteotomy seems a profitable alternative technique for treating the varus and torsional deformity in patients with Blount's disease in the circumstances of developing countries. The short-term outcomes are good and promising with a low complication rate and good consolidation. Long-term follow-up results of these patients are needed to observe possible complications. LEVEL OF EVIDENCE: IV, therapeutic case series.

Valgus deformity caused by dysplasia epiphysealis hemimelica in the knee.

A rare case of dysplasia epiphysealis hemimelica in the left knee which caused valgus deformity and dysfunction of the limb is presented in this article. Subtotal excision of the lesion, distal femoral medial wedge osteotomy, and reconstruction of the medial collateral ligament were performed for treatment. Cannulated screws and plaster casts were used to stabilize the ligament and distal femur. Two years after removal and reconstruction, the knee was symptom free. The left knee laxity was restored and the mechanical axis of the distal femur was realigned.

Treatment of obstructive uropathy in one of three young brothers suffering from Gorlin-Cohen syndrome: a case report.

BACKGROUND: Frontometaphyseal dysplasia, or Gorlin-Cohen syndrome, is an X-linked disorder primarily characterized by skeletal dysplasia, such as hyperostosis of the skull and abnormalities of tubular bone modeling. Some patients develop extraskeletal manifestations, such as urinary tract anomalies. CASE PRESENTATION: A 26-year-old male patient was diagnosed with frontometaphyseal dysplasia and suffered from chronic urine retention. Although the patient was primarily diagnosed with a neurogenic bladder, our work-up revealed posterior urethral valves, bladder neck stenosis, and multiple bladder stones. The patient was treated by transurethral resection of the urethral valves and bladder neck with simultaneous open cystolithotomy to remove the bladder calculi. After removal of the catheter, the patient voided normally and had no post-void residual urine. At the 1-year follow-up, he was still voiding normally; his urodynamic investigation was also normal. CONCLUSIONS: In the recent literature, there is scarce information on the diagnosis, treatment, and follow-up of patients with malformations of the urinary tract as a result of Gorlin-Cohen syndrome. The case presented here could guide urological approaches to patients suffering from this rare condition.

Fibula-related complications during bilateral tibial lengthening: 60 patients followed for mean 5 years.

BACKGROUND AND PURPOSE: Complications related to the fibula during distraction osteogenesis could cause malalignment. Most published studies have analyzed only migration of the fibula during lengthening, with few studies examining the effects of fibular complications. PATIENTS AND METHODS: We retrospectively reviewed 120 segments (in 60 patients) between 2002 and 2009. All patients underwent bilateral tibial lengthening of more than 5 cm. The mean follow-up time was 4.9 (2.5-6.9) years. RESULTS: The average lengthening percentage was 34% (21-65). The ratio of mean fibular length to tibial length was 1.05 (0.91-1.11) preoperatively and 0.83 (0.65-0.95) postoperatively. The mean proximal fibular migration (PFM) was 15 (4-31) mm and mean distal fibular migration (DFM) was 9.7 (0-24) mm. Premature consolidation occurred in 10 segments, nonunion occurred in 12, and angulation of fibula occurred in 8 segments after lengthening. Valgus deformities of the knee occurred in 10 segments. INTERPRETATION: PFM induced valgus deformity of the knee, and premature consolidation of the fibula was associated with the distal migration of the proximal fibula. These mechanical malalignments could sometimes be serious enough to warrant surgical correction. Thus, during lengthening repeated radiographic examinations of the fibula are necessary to avoid complications.

Outcome analysis of surgical treatment of Blount disease in Nigeria.

OBJECTIVE: The objective was to evaluate the results of surgery of Blount diseases using the postoperative metaphyseal-diaphyseal angle (MDA) at 2-year follow-up. BACKGROUND: The goal of surgery in Blount disease is to restore the normal configuration of the articular surface of the proximal end of the tibia in proper relationship to the mechanical axis of the limb. Our hypothesis is that patients will demonstrate significant clinical improvements following surgery and predictive models can be developed. MATERIALS AND METHODS: All patients who had surgery for Blount disease from January 2002 till December 2007 at the National Orthopaedic Hospital Lagos Nigeria and follow-up for 2 years were included. Data extracted were gender, affected limb, Blount's type, age when deformity was noted, and age at presentation, preoperative femoral tibial angle (TFA), Langenskiold score, preoperative MDA, and postoperative MDA. Linear regression was used to assess the predictive effect of selected clinical and radiographic measures on post-MDA. The model was adjusted for confounders: age deformity noted, age at presentation, affected limb, Blount's type, and gender. Variables in the adjusted model achieving significance at P < 0.05 were included in a multiple regression analysis. RESULTS: Eighty-six knees in 57 patients were included. The mean preoperative and postoperative MDA at 2 years was 34.6° ± 8.9° and 10.6° ± 4.3°, respectively. Seventy-three knees (84.9%) have correction of ≤10° with recurrence in 13 (15.1%) knees at 2 years (P < 0.001). The postoperative MDA was graded into good outcome if ≤10°. There was a significant improvement between preoperative MDA and postoperative MDA (P < 0.001). The multilinear analysis demonstrated that the preoperative MDA was a significant predictor of the postoperative MDA. The postoperative MDA was predicted with a standard error of 0.92 with the following formula: post-MDA = 1.027 + 0.404 pre-MDA. CONCLUSION: The mean postoperative MDA of 84.9% of the knees operated at 2 years was 9.4° ± 3.1° with recurrence rate of 15.1%. Postoperative MDA is a good outcome measure for surgical treatment of Blount disease and surgical correction should aim at producing post MDA ≤10°.

V-Y advancement flaps for scalp closure after acute cranial volume expansion.

Sometimes the surgeon who contemplates a coronal incision for craniofacial surgery, such as fronto-orbital advancement, faces a situation in which closure by primary suture becomes impossible because of acute cranial volume expansion. To avoid such a situation, the authors invented V-Y advancement flap method. This flap was found to be highly effective in 6 craniofacial patients who were treated between March 2007 and March 2008. The method consists of an incision line that starts from the highest spot on the ear and proceeds in the direction of the hairline at 45 degrees, and then runs up to the vertex in a sawtooth manner. This line is used when acute cranial volume expansion is expected after surgery. Another incision line is symmetrically drawn on the opposite side of the head. These 2 lines are conjoined at the vertex. The lengths of the 2 sides of a single sawtooth are both about 2 to 3 cm. When the scalp of an expanded cranium is closed, some V-shaped scalp margins under normal tension can simply be sutured using a traditional V-V advancement flap. However, the others under increased tension need to be in a Y-shaped pattern. During the follow-up period (6 months on average), application of this V-Y advancement flap method led to good functional and aesthetic outcomes in all 6 cases. The authors attribute these results to scalp closure using a mixture of V-V and V-Y flaps.

High tibial osteotomy in young adults with constitutional tibia vara.

Frontal plane malalignment of the lower extremity results in abnormal load distribution across the knee joint. Consequences of this increased stress may lead to compartmental osteoarthritis. High tibial osteotomy is well established for early osteoarthritis of the knee joint in middle-aged patients. We hypothesize that earlier realignment of the varus knee can be performed without undue risks and debilitation toward the young active patient and with good results in short-term follow-up. Open-wedge high tibia osteotomy using the Puddu plate was performed on eleven patients (19 knees) under 25 years of age for constitutional high tibia vara. The mechanical femorotibial angle (FTA) and Knee Society Knee Score (KSKS) were compared pre-operatively and 24 months post-operatively. The average FTA improved from +8.8 degrees (+5 - +16) to -0.1 (-2 - +5). The average KSKS improved from 74 (50-100) to 93 (60-100) and the function score improved from 84 (50-100) to 95 (60-100). Special concerns in this age group include unsightly cosmesis, and kneeling pain and discomfort. Open-wedge high tibia osteotomy provides a satisfactory solution for constitutional high tibia vara with minor morbidity on behalf of the patient in the short-term follow-up period.

Anterior posterior inverted-'U' osteotomy for tibia vara: technique and early results.

BACKGROUND: Blount disease often requires operative treatment including osteotomy. This study evaluated the safety and outcome of the inverted-U osteotomy in Blount disease. METHODS: We performed an anteroposterior inverted-U osteotomy without a jig or image intensifier in the treatment of a series of 31 children with the intention of achieving anterior-posterior stability at the osteotomy site without risk of major neurological and vascular injuries. RESULTS: We found no vascular or common peroneal nerve injury. There was stability at the osteotomy site without the need for internal fixation. Thirty knees in 20 patients had good outcome, while 17 knees (11 patients) had fair outcome. CONCLUSIONS: Our results suggest that this technique is safe, effective and gives satisfactory outcome in the operative correction of Blount's disease.

Femoral bifurcation with ipsilateral tibia hemimelia: early outcome of ablation and prosthetic fitting.

Femoral bifurcation and tibia hemimelia are rare anomalies. Hereby, we present a case report of a 2-year-old boy who first presented in our orthopedic clinic as a 12-day-old neonate, with a grossly deformed right lower limb from a combination of complete tibia hemimelia and ipsilateral femoral bifurcation. Excision of femoral exostosis, knee disarticulation and prosthetic fitting gives satisfactory early outcome.

Characteristics of three-dimensional acetabular morphology of patients with excellent outcome after rotational acetabular osteotomy over 20 years.

BACKGROUND: Rotational acetabular osteotomy (RAO) is a type of pelvic osteotomy performed to improve the acetabular bony coverage against the femoral head for patients with acetabular dysplasia. The acetabular bony coverage is ideally evaluated three-dimensionally; however, there is a paucity of published data regarding three-dimensional morphology in patients with long-term excellent outcome after RAO. The present study investigated the characteristics of three-dimensional acetabular morphology with long-term excellent outcome after RAO in comparison to patients with normal hip joints and those converted to total hip arthroplasty (THA) after RAO because of osteoarthritis (OA) progression. METHODS: Anteroposterior plain radiograph and computed tomography data of 57 hip joints (17 joints with excellent outcome 20 years or more after RAO, 16 normal joints, and 20 joints converted to THA after RAO) were analyzed. The two-dimensional lateral center-edge (CE) angle from plain radiographs and acetabular anteversion, anterior acetabular sector angle, and posterior sector angle from computed tomography (CT) images were calculated. RESULTS: Compared with patients converted to THA, all parameters in patients with long-term excellent outcome after RAO were similar to those in patients with normal hip joints, particularly in the three-dimensional analyses. The anterior bony coverage was excessive, whereas the posterior bony coverage was deficient in patients converted to THA after RAO. Anterior bony impingement and posterior instability may be the cause of OA progression after RAO. CONCLUSION: Caution must be taken to avoid rotating the separated fragment excessively to the anterior direction during RAO to prevent OA progression and achieve long-term excellent outcome.

Dysplasia epiphysealis hemimelica: a case report.

AIMS AND OBJECTIVES: To report the clinical presentation, course, management and associated pathology of DEH seen at the National Orthopaedic Hospital Lagos. PATIENT AND METHODS: Case review was employed to report the association of an incidental radiologic finding of asymptomatic DEH with Blount disease. RESULTS: This case is an 11-year-old girl with asymptomatic DEH of both ankles associated with right unilateral Blount disease. The incidental pathology is conservatively managed following the surgical treatment of the Blount disease which is the symptomatic pathology. CONCLUSION: DEH is an uncommon pathology usually an incidental radiologic finding which may or may not be associated with other skeletal pathologies. The management is essentially conservative but surgery is an option when symptomatic.

Is cup positioning easier in DDH patients previously treated with Bernese periacetabular osteotomy?

BACKGROUND: Acetabular orientation changes after periacetabular osteotomy (PAO) lead to technical change when performing subsequent total hip arthroplasty (THA). There is no unified consensus regarding the solution for acetabular component installation after PAO. In the current study, we performed computed tomography (CT)-based simulation of acetabular component installation and compared the acetabular defect and component position following THA after PAO and the same patient before PAO. METHODS: From January 2014 to December 2018, pelvic models of 28 patients (28 hips) underwent PAO and with the risk factors to develop secondary osteoarthritis. The acetabular reconstruction process was simulated using 3D models from CT data, and the acetabular component coverage was calculated in 3D space based on the measurement and algorithm we proposed. We evaluated the anterior, posterior, superior, inferior acetabular sector angle (ASA), the medial wall thickness (MWT), and the distance from the hip center to the plane of pubic symphysis and ossa sedentarium in the study group (post-PAO group) and control group (pre-PAO group). In addition, we investigated the changes in the acetabular component covering and size between the two groups. RESULTS: A-ASA and I-ASA values were significantly smaller in the post-PAO group than in the pre-PAO group. The S-ASA and distance values were significantly bigger in the post-PAO group. Compared to the pre-PAO group, the post-PAO group has a bone defect in the anterior and inferior medial. However, the post-PAO group has to elevate the cup to improved component coverings. CONCLUSION: Acetabular defection following simulation of cup installation after PAO was significantly changed compared to those without PAO. Elevation of hip joint centers as much as 4 mm and increase acetabular cup anteversion were therapeutic options for DDH patients following THA after PAO.

The role of external fixation in paediatric limb lengthening and deformity correction.

Limb deformity and shortening remains a common presenting complaint in paediatric orthopaedics. The ability to restore length, alignment with consistent bone formation allows standardised treatment in these difficult conditions. The use of an external fixator produces a stable construct which allows manipulation of the bone segments, with a controlled rate and rhythm. Deformity analysis permits the surgeon to plan osteotomy levels and frame application to produce accurate correction. Complications, especially in congenital conditions remain an issue and modification of the treatment is required to try and reduce their incidence. New types of fixators, the latest being hexapod frames, have increased the versatility in deformity correction. The aim of this paper is to review the use of external fixators in elective paediatric orthopaedics and highlight the latest changes in technique and application for the limb reconstruction surgeon.

Dysplasia epiphysealis hemimelica combined with contralateral accessory scaphoid bone: A case report and literature review.

RATIONALE: Dysplasia epiphysealis hemimelica (DEH), also known as Trevor disease, is a rare, developmental bone disorder of childhood. PATIENT CONCERNS: A 9-year-old girl was admitted due to pain in front of the medial malleolus of her right foot after a long walk or distance movement, in which the pain could be relieved after rest, while it was repeated and lasted for several months. DIAGNOSIS: Dysplasia epiphysealis hemimelica INTERVENTIONS:: The patient underwent an open resection surgery. After operation, the pain was totally relieved. Postoperative pathological diagnosis showed DEH. OUTCOMES: At the 6-month follow-up, pain and claudication symptoms fully disappeared, and range of motion of the right foot returned to normal level. CONCLUSIONS: Dysplasia epiphysealis hemimelica is an uncommon disease which can cause pain of foot in children. LESSONS: When the pediatric orthopedic surgeon treated the children suffered with foot pain should be aware of this rare disease, especially accessory scaphoid bone was found in another foot.

Gradual correction of proximal tibia deformity for Blount disease in adolescent and young adults.

PURPOSE: Management of Blount disease in adolescents and young adults is complex and associated with high risk of morbidities. Gradual correction with external fixator can minimize soft tissue injury and allow subsequent adjustment in degree of correction. This study investigates the surgical outcome and complication rate of gradual correction of neglected Blount disease through single-level extra-articular corticotomy. METHODS: Patients treated for Blount disease using external fixator from 2002 to 2016 were recruited for the study. We used Ilizarov and Taylor Spatial Frame (TSF) external fixator to perform simultaneous correction of all the metaphyseal deformities without elevating the tibia plateau. Surgical outcome was evaluated using mechanical axis deviation (MAD), tibial femoral angle (TFA), and femoral condyle tibial shaft angle (FCTSA). RESULTS: A total of 22 patients with 32 tibias have been recruited for the study. The mean MAD improved from 95 ± 51.4 mm to 9.0 ± 37.7 mm (medial to midpoint of the knee), mean TFA improved from 31 ± 15° varus to 2 ± 14° valgus, and mean FCTSA improved from 53 ± 14° to 86 ± 14°. Mean duration of frame application is 9.4 months. Two patients developed pathological fractures over the distracted bones, one developed delayed consolidation and other developed overcorrection. CONCLUSIONS: Correction of Blount disease can be achieved by gradual correction using Ilizarov or TSF external fixator with low risk of soft tissue complication. Longer duration of frame application should be considered to reduce the risk of pathological fracture or subsequent deformation of the corrected bone.

Staged cervical osteotomy:a new strategy for correcting ankylosing spondylitis thoracolumbar kyphotic deformity with fused cervical spine.

BACKGROUND: In patients with cervical ankylosis, the chin-brow vertical angle (CBVA) should be taken into consideration. Usually, the correction of sagittal balance is sacrificed to ensure the patient has a horizontal visual field. To our knowledge, a staged osteotomy strategy for ankylosing spondylitis kyphotic deformity with an ankylosed cervical spine has not been reported before. The aim of this study was to describe a new surgical strategy with emphasis on sagittal balance and gaze angle in correction of kyphotic deformity with a rigid cervical spine in ankylosing spondylitis thoracolumbar kyphotic deformity. METHODS: A 36-year-old man has severe thoracolumbar kyphosis accompanied with cervical hyperlordosis caused by ankylosing spondylitis. A two-stage surgery planning was managed. For the first stage, an interrupted two-level osteotomy was performed at the thoracolumbar area. After surgery, sagittal imbalance was corrected but the CBVA was - 21.7°. Cervical osteotomy was performed for the second stage. A flexion osteotomy was performed at C7, using anterior-posterior-anterior approaches. RESULTS: Both sagittal imbalance and gaze angle of the patient were improved markedly. The osteotomy sites were documented fused. Complications were not observed during and after operation. CONCLUSIONS: The aim of osteotomy for ankylosing spondylitis is to reestablish sagittal balance and improve forward gaze and the visual field. A staged cervical osteotomy is an alternative to reduce cervical lordosis to obtain a normal gaze angle. An anterior-posterior-anterior approach is recommended.

Phenotypic variability of Pai syndrome: report of two patients and review of the literature.

Pai syndrome is a rare form of frontonasal dysplasia, first described in 1987. It is a triad consisting of midline cleft of the upper lip, facial skin polyps and central nervous system lipomas. Only 14 cases have been reported in the literature. The authors describe the clinical features, diagnostic workup and treatment of two patients. A review of all cases reported in literature is presented to show the phenotypic variability of this rare syndrome.

Knee replacement for osteoarthritis secondary to chronic patellar dislocation and trochlear dysplasia.

Fifteen knees in patients suffering from symptomatic osteoarthritis secondary to chronically dislocated extensor mechanisms have been treated by knee replacement. Five knees had permanently dislocated patellae while 10 knees (four of which had undergone patellectomies) had dislocations of the extensor mechanisms with knee flexion. In fourteen instances satisfactory realignment of the extensor mechanism was obtained. In twelve knees, a substantial functional improvement was achieved. Two knees had persistent pain and one had unexpected tibio-femoral instability.