Osteogenesis Imperfecta

Franzone, Jeanne M.; Shah, Suken A.; Wallace, Maegen; Kruse, Richard W.

doi:10.1016/j.ocl.2018.10.003

Cited by 42 publications

(24 citation statements)

References 58 publications

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“…The most common foot deformity in patients with OI reported in the literature is pes planovalgus (flatfoot) 58,60,61 , while skew foot and clubfoot have also been reported by patients with OI 58,62 . Conservative management includes foot orthoses and bracing to optimize alignment, prevention, and protection of the foot and ankle 60,64 . Surgical corrections involving the osseous and soft tissue may be warranted in severe cases or patients who fail conservative management 60 .…”

Section: Discussionmentioning

confidence: 99%

“…Patients with OI and a mild degree of foot deformity may initially benefit from foot orthoses, like those previously referenced, or AFOs to prevent equinus contractures, support musculoskeletal integrity and articular collapse 60,64 . Surgical correction involving the osseous and soft tissue may be warranted in severe cases or patients who fail conservative management 60 .…”

Section: Osteogenesis Imperfectamentioning

confidence: 99%

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Developmental Foot Deformities in Patients with Connective Tissue Disorders

et al. 2023

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Foot deformities make up a large percentage of all orthopaedic complaints in patients with Down syndrome, Marfan syndrome, Ehlers-Danlos syndrome, Larsen syndrome, and osteogenesis imperfecta.» Some common causes of foot deformities in these conditions include increased ligament laxity, hypotonia, and hypermobility of the joints.» Treatment options for syndromic foot deformities include the use of foot orthoses, physical therapy, bracing, and various surgical procedures.» There is limited evidence supporting the use of surgical intervention to correct foot deformities associated with Down syndrome, Marfan syndrome, Ehlers-Danlos syndrome, Larsen syndrome, and osteogenesis imperfecta. Therefore, further research is needed to determine the short-term and long-term outcomes of these procedures.

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Section: Discussionmentioning

confidence: 99%

Section: Osteogenesis Imperfectamentioning

confidence: 99%

Developmental Foot Deformities in Patients with Connective Tissue Disorders

et al. 2023

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“…Autosomal dominant mutations in these genes were first associated with OI in the late 1970s. (3) Decreased quantity of normal type I collagen is associated with less severe OI, whereas the abnormal quality of type I collagen is associated with more severe OI. (4) Longtime standard classification of OI types I-IV was proposed by Sillence and colleagues in 1979 based on clinical and radiographic features and inheritance patterns.…”

Section: Introductionmentioning

confidence: 99%

Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

et al. 2023

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Intravenous (iv) bisphosphonates are widely used to treat the skeletal manifestations of osteogenesis imperfecta (OI). Obtaining peripheral iv access in pediatric patients with OI is often difficult and traumatic. Although this may be mitigated with surgically placed iv ports (port-a-caths), surgeons may be hesitant to perform this procedure on these children because of the lack of safety data. This study aims to gain better insight into the safety and efficacy of port-a-cath use in this population and identify risk factors for port-acath complications. In the present study, we conducted a retrospective cohort analysis of patient characteristics and the incidence of port-a-cath-related complications in children with OI. Fifty-three port-a-caths were placed in 29 children (21 males and 8 females). Of the 29 patients, most are OI type III (n = 18), followed by type I (n = 4), type IV (n = 4), and type V (n = 3). At the time of initial port-acath placement, the median age was 52 months (10-191 months), and the median weight was 7.9 kg (5.1-41.1 kg). Most patients (n = 20) weighed less than 10 kg during initial placement. Weight correlated significantly with OI type (p = 0.048), sex (p = 0.03), and vessel used (p = 0.02). Median initial port-a-cath longevity was 43 months (1-113 months), and we found no significant difference in port-a-cath longevity between sexes, OI types, or vessels used. Most patients (n = 19) required multiple port-a-cath placements. There is a significant difference (p = 0.02) between the number of placements and OI type, with type IV having more than type III. Port-a-cath removal was almost always due to mechanical complications (n = 19) but also for infection (n = 1) and malposition (n = 1). Eight patients still had their initial port-a-caths in place at the conclusion of this study. These findings indicate that complications associated with port-a-cath placement are mild and can be used to safely deliver iv bisphosphonates to pediatric OI patients.

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“…Osteogenesis imperfecta (OI) is a genetic disorder characterized by poor-quality bone, with a propensity to fracture, as well as extraskeletal manifestations, including dentinogenesis imperfecta, hearing loss, and growth deficiency 1,2. Children with moderate and severe OI develop long bone bowing, which can predispose them to fracture, alter the mechanical axis, and impair ambulation.…”

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confidence: 99%

The Fate of the Bent Rod in Children With Osteogenesis Imperfecta

Fralinger

Kraft

Rogers

et al. 2023

Journal of Pediatric Orthopaedics

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Background: Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones and long bone deformity. Realignment and intramedullary rodding with telescopic rods are indicated for progressive deformity and can help prevent fractures. Rod bending is a reported complication of telescopic rods and a common indication for revision; however, the fate of bent lower extremity telescopic rods in the setting of OI has not been reported. Methods: Patients with OI at a single institution who underwent lower extremity telescopic rod placement with at least 1-year follow-up were identified. Bent rods were identified, and for these bone segments, we collected the location and angle of bend, subsequent telescoping, refracture, increasing angulation of bend, and date of revision. Results: One hundred sixty-eight telescopic rods in 43 patients were identified. Forty-six rods (27.4%) bent during follow-up, with an average angulation of 7.3 (range: 1 to 24) degrees. In patients with severe OI, 15.7% of rods bent compared with 35.7% in nonsevere OI (P = 0.003). The proportion of bent rods was different between independent and nonindependent ambulators (34.1% and 20.5%; P = 0.035). Twenty-seven bent rods (58.7%) were revised, with 12 rods (26.0%) revised early (within 90 d). The angulation of rods that were revised early was significantly higher than rods not (14.6 and 4.3 degrees, P < 0.001). Of the 34 bent rods not revised early, the average time to revision or final follow-up was 29.1 months. Twenty-five rods (73.5%) continued to telescope, 14 (41.2%) increased in angulation (average 3.2 degrees), and 10 bones (29.4%) refractured. None of the refractures required immediate rod revision. Two bones had multiple refractures. Conclusions: Bending is a common complication of telescopic rods in the lower extremities of patients with OI. It is more common in independent ambulators and patients with nonsevere OI, possibly because of the increased demand placed on the rods. Rods with a small bend and maintained fixation can telescope and need not be an indication for immediate revision.

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Osteogenesis Imperfecta

Cited by 42 publications

References 58 publications

Developmental Foot Deformities in Patients with Connective Tissue Disorders

Developmental Foot Deformities in Patients with Connective Tissue Disorders

Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

The Fate of the Bent Rod in Children With Osteogenesis Imperfecta

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