Management of lower limb deformities in children with osteogenesis imperfecta

Kaur, Satvinder; Kulkarni, Ketan; Kochar, Inderpal Singh; Narasimhan, Ramani

doi:10.1007/s13312-011-0103-0

Cited by 5 publications

(4 citation statements)

References 13 publications

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“…The most frequently affected body parts were the lower limbs with bowed legs followed by the vertebral spine with scoliosis. These results are similar to the reported outcomes in previous studies [14,15]. It was observed that in OI cases deformities in the lower limbs were more frequent than in upper limbs.…”

Section: Discussionsupporting

confidence: 92%

“…However, Falk [9] documents that combined therapy is very beneficial to patients with OI. This is in line with Kaur et al [15] who amplify their combined management experience in the correction of lower limb deformities in four children with OI whose medical management consisted of pre-and postoperative bisphosphonate therapy pamidronate in particular, calcium supplementation and rehabilitative care. Deformities were corrected with multiple osteotomies and intramedullary fixation by titanium elastic nails.…”

Section: Discussionsupporting

confidence: 85%

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The Prevalence and Characteristics of Patients Diagnosed with Osteogenesis Imperfecta at the Zambia Italian Orthopedics Hospital Community Based Rehabilitation Project

Kabengo¹,

Munalula-Nkandu²,

Munthali³

et al. 2015

SJPH

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Introduction: Osteogenesis Imperfecta is a rare condition that can be very disabling. It is an inherited disease of the bones, which is characterized by fragility that causes affected individuals to have recurrent fractures that subsequently lead to pain, deformity and disability. The aim of the study was to determine the prevalence of Osteogenesis Imperfecta and identify the common deformities and treatment methods used in managing affected individuals at the Zambia Italian Orthopedics Hospital. Methods: This was a retrospective cross sectional survey. A data capture sheet was used to collect data from records and descriptive trend analysis was done in excel. Results: We identified 18 patients with Osteogenesis Imperfecta out of 8250. The age range was 1 to 17 years. Eighty-three percent (n=15) of these patients had deformities which included; bowed lower limbs, scoliosis and other related complications. All the patients underwent surgery at one point in time and received physiotherapy. Conclusion: The prevalence of Osteogenesis Imperfecta at the Zambia Italian Orthopaedics Hospital Community Based Rehabilitation Project is low. Surgery is the most commonly used method of treatment coupled with physiotherapy. Though uncommon, the condition is expensive to manage for both the institution and affected individuals' families. This is as a result of repeated surgeries and prolonged physiotherapy.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 85%

The Prevalence and Characteristics of Patients Diagnosed with Osteogenesis Imperfecta at the Zambia Italian Orthopedics Hospital Community Based Rehabilitation Project

Kabengo¹,

Munalula-Nkandu²,

Munthali³

et al. 2015

SJPH

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“…[ 66 ] An interesting report by Kaur et al . [ 67 ] had highlighted the significance of combined medical management (peri- and postoperative pamidronate therapy) and surgical correction (multiple osteotomies and intramedullary fixation) of lower limb deformities in four children with OI. Oral administration of alendronate in a patient with polyostotic fibrous dysplasia was found to significantly increase the bone mineral density and result in reduction of pain and fractures.…”

Section: Resultsmentioning

confidence: 99%

A review of skeletal dysplasia research in India

Uttarilli

Shah

Shukla

et al. 2018

Journal of Postgraduate Medicine

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We aimed to review the contributions by Indian researchers to the subspecialty of skeletal dysplasias (SDs). Literature search using specific keywords in PubMed was performed to retrieve all the published literature on SDs as on July 6, 2017. All published literature on SDs wherein at least one author was from an Indian institute was included. Publications were grouped into different categories based on the major emphasis of the research paper. Five hundred and forty publications in English language were retrieved and categorized into five different groups. The publications were categorized as reports based on: (i) phenotypes (n = 437), (ii) mutations (n = 51), (iii) novel genes (n = 9), (iv) therapeutic interventions (n = 31), and (v) reviews (n = 12). Most of the publications were single-patient case reports describing the clinical and radiological features of the patients affected with SDs (n = 352). We enlisted all the significant Indian contributions. We have also highlighted the reports in which Indians have contributed to discovery of new genes and phenotypes. This review highlights the substantial Indian contributions to SD research, which is poised to reach even greater heights given the size and structure of our population, technological advances, and expanding national and international collaborations.

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“…There is wide variability in the types of intramedullary rods and their clinical results 45617181920. With bone growth, the rod becomes relatively shorter and require revisions when the patient outgrows the rod.…”

Section: Discussionmentioning

confidence: 99%

Results of a bone splint technique for the treatment of lower limb deformities in children with type I osteogenesis imperfecta

Lin

Zhai

Lian

et al. 2013

IJOO

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Background:Children with osteogenesis imperfecta (OI) can suffer from frequent fractures and limb deformities, resulting in impaired ambulation. Osteopenia and thin cortices complicate orthopedic treatment in this group. This study evaluates the clinical results of a bone splint technique for the treatment of lower limb deformities in children with type I OI. The technique consists of internal plating combined with cortical strut allograft fixation.Materials and Methods:We prospectively followed nine children (five boys, four girls) with lower limb deformities due to type I OI, who had been treated with the bone splint technique (11 femurs, four tibias) between 2003 and 2006. The fracture healing time, deformity improvement, ambulation ability and complications were recorded to evaluate treatment effects.Results:At the time of surgery the average age in our study was 7.7 years (range 5-12 years). The average length of followup was 69 months (range 60-84 months). All patients had good fracture healing with an average healing time of 14 weeks (range 12-16 weeks) and none experienced further fractures, deformity, or nonunion. The fixation remained stable throughout the procedure in all cases, with no evidence of loosening or breakage of screws and the deformity and mobility significantly improved after surgery. Of the two children confined to bed before surgery, one was able to walk on crutches and the other needed a wheelchair. The other seven patients could walk without walking aids or support like crutches.Conclusions:These findings suggest that the bone splint technique provides good mechanical support and increases the bone mass. It is an effective treatment for children with OI and lower limb deformities.

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Management of lower limb deformities in children with osteogenesis imperfecta

Cited by 5 publications

References 13 publications

The Prevalence and Characteristics of Patients Diagnosed with Osteogenesis Imperfecta at the Zambia Italian Orthopedics Hospital Community Based Rehabilitation Project

The Prevalence and Characteristics of Patients Diagnosed with Osteogenesis Imperfecta at the Zambia Italian Orthopedics Hospital Community Based Rehabilitation Project

A review of skeletal dysplasia research in India

Results of a bone splint technique for the treatment of lower limb deformities in children with type I osteogenesis imperfecta

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