Fibular Lengthening for the Management of Translational Talus Instability in Hereditary Multiple Exostoses Patients

Lee, Dong Yeon; Kim, Joong Il; Song, Mi Hyun; Choi, Eun Seok; Park, Moon Seok; Yoo, Won Joon; Chung, Chin Youb; Choi, In Ho; Cho, Tae Joon

doi:10.1097/bpo.0000000000000181

Cited by 10 publications

(6 citation statements)

References 17 publications

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“…The femoral neck-shaft angle was defined as the angle between the axis of the femoral neck passing through the center of the femoral head and the axis of the femoral shaft [ 13 ], and an increased femoral neck-shaft angle was defined as an increase of ≥10° in the femoral neck-shaft angle of the affected limb compared with the contralateral limb. The tibiofemoral angle was defined as the angle formed by the axis of the femur and the axis of the tibia [ 14 ], whereas the ankle valgus angle was regarded as the angle between the axis of the tibial shaft and the tibial plafond line [ 15 ]. We also examined the effect of an increased femoral neck-shaft angle on FO.…”

Section: Methodsmentioning

confidence: 99%

Femoral overgrowth in children with congenital pseudarthrosis of the Tibia

Song

Park

Yoo

et al. 2016

BMC Musculoskelet Disord

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BackgroundHaving observed a tendency towards femoral overgrowth (FO) of the affected limb in children with atrophic-type congenital pseudarthrosis of the tibia (CPT), we aimed to identify the incidence of, contributors to, and patterns of FO among such children.MethodsWe retrospectively evaluated 55 children with CPT, 22 with prepseudarthrosis and 33 with atrophic-type CPT from 1989 to 2012. FO was defined as an affected femoral segment ≥10 mm longer than the contralateral segment. We investigated FO incidences in prepseudarthrosis versus atrophic-type CPT. Sex, laterality, coexistence of neurofibromatosis type 1, development of frank pseudarthrosis, extent of tibial shortening, shortening in foot height, deformity severity, distraction osteogenesis (DO) treatment, refracture, increased femoral neck-shaft angle, tibiofemoral angle, and ankle valgus angle were investigated to identify potential contributors to FO. Patterns of FO were also determined.ResultsAt initial presentation, 11 patients exhibited a mean of 13 mm (10–23) of FO. However, the nature of FO changed over time during the follow-up period (mean, 10.8 years; range, 4.3–19.3). At the last follow-up, 14 patients presented with a mean of 12 mm (10–18) of FO. With the exception of one patient, all patients with FO presented with atrophic-type CPT. Frank pseudarthrosis, DO treatment, and increased femoral neck-shaft angle were significantly associated with FO (p = 0.016, p = 0.001, and p = 0.005, respectively). Diverse patterns of FO were observed.ConclusionsFO of the affected limb is frequently encountered in patients with atrophic-type CPT. A compensatory response to frank pseudarthrosis, DO treatment, and neurofibromatosis may play a role in the diverse patterns of FO.

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Section: Methodsmentioning

confidence: 99%

Femoral overgrowth in children with congenital pseudarthrosis of the Tibia

Song

Park

Yoo

et al. 2016

BMC Musculoskelet Disord

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“…The first reports have promising results 79,80. Fibular lengthening has also been evaluated in the prevention and initial correction of ankle valgus deformities and talus instability with optimal results 81. Valgus deformities of the proximal femur could be corrected by several approaches with variable results 82.…”

Section: Treatmentmentioning

confidence: 99%

<p>Hereditary Multiple Exostoses: Current Insights</p>

D’Arienzo¹,

Andreani²,

Sacchetti³

et al. 2019

ORR

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Hereditary multiple exostoses (HME), also called hereditary multiple osteochondromas, is a rare genetic disorder characterized by multiple osteochondromas that grow near the growth plates of bones such as the ribs, pelvis, vertebrae and especially long bones. The disease presents with various clinical manifestations including chronic pain syndromes, restricted range of motion, limb deformity, short stature, scoliosis and neurovascular alteration. Malignant transformation of exostosis is rarely seen. The disease has no medical treatment and surgery is only recommended in symptomatic exostoses or in cases where a malignant transformation is suspected. HME is mainly caused by mutations and functional loss of the EXT1 and EXT2 genes which encode glycosyltransferases, an enzyme family involved in heparan sulfate (HS) synthesis. However, the peculiar molecular mechanism that leads to the structural changes of the cartilage and to osteochondroma formation is still being studied. Basic science studies have recently shown new insights about altering the molecular and cellular mechanism caused by HS deficiency. Pediatricians, geneticists and orthopedic surgeons play an important role in the study and treatment of this severe pathology. Despite the recent significant advances, we still need novel insights to better specify the role of HS in signal transduction. The purpose of this review was to analyze the most relevant aspects of HME from the literature review, give readers an important tool to understand its clinical features and metabolic-pathogenetic mechanism, and to identify an effective treatment method. We focused on the aspects of the disease related to clinical management and surgical treatment in order to give up-to-date information that could be useful for following best clinical practice.

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“…These interventions are often performed to prevent severe deformities, arthrosis and dysmetria. [17][18][19][20][21][22] In 2012 Goud et al evaluated pain and quality of life in a large cohort of patients with HME. The author found that 57 children (58%) participated in sports at the time of evaluation.…”

Section: Discussionmentioning

confidence: 99%