Intra-lesional steriod for the management of symptomatic Infantile Digital Fibromatosis

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Summary

Background

Infantile Digital Fibromatosis (IDF) is a benign, often asymptomatic nodular proliferation of fibrous tissue occurring almost exclusively on the extremities. Conventional treatment has included radical surgery but this is associated with a high level of recurrence. Whilst some authors suggest a strictly conservative approach, this is unacceptable when lesions become symptomatic from pain, contracture formation or functional deformity

Methods

We present a retrospective analysis of 12 symptomatic lesions of which 7 were treated with a novel technique of intra-lesional steroid.

From 2004–2009, a total of ten patients received treatment for symptomatic IDFs. Patients were followed-up for an average of 5 years 9 months (range 8–131 months).

Results

Corticosteroid was well tolerated with no significant complications and was associated with lower morbidity that compared with surgery. There was no significance difference between rate of recurrence (1/7 vs. 5/10) for those treated with corticosteroid than compared to those patients who underwent surgery (p = 0.3) but the study is underpowered.

Conclusions

This is the first ever study to look at the role of intra-lesional steroid in the management of IDF. Whilst the majority of asymptomatic Infantile Digital Fibromatoses can be safely observed until natural resolution, intra-lesional corticosteroid is a safe and well-tolerated alternative to surgery for all symptomatic digital fibromatoses of infancy. We suggest it replaces surgery as first-line treatment but look forward to a large multicentre trial to allow comparison.

Introduction

Infantile Digital Fibromatosis (IDF) is a rare, distinct, fibrous tumour of infancy consisting of solitary or multiple lesions on the fingers and toes. Ever since Reye classified Infantile Digital Fibromatosis in 1956, much debate has surrounded the management of this condition.1 Over the last decades, management has ranged from extensive, often mutilating surgery2, 3 to a strictly conservative approach.2, 3, 4, 5 Whilst the majority of hand surgeons have adopted a conservative approach for asymptomatic lesions, surgery still remains first choice when these lesions become symptomatic. With surgery associated with a recurrence rate of 60% new treatment modalities are needed for symptomatic Infantile Digital Fibromas.6

Our study’s aim was to firstly assess the efficacy of intra-lesional corticosteroid on symptomatic IDFs and secondly, attempt to compare recurrence rate between those lesions treated with surgery and those treated with injection of corticosteroid.

Section snippets

Methods

We retrospectively analysed all patients with symptomatic Infantile Digital Fibromatosis that were treated at a tertiary children’s hand unit UK between 2004 and 2009. Symptomatic IDF was defined as either pain at the site of the lesion, contracture deformity or a doubling in size over a four week period consistent with a high risk for development of functional deformity. During this period, patients were treated with either surgery or steroid as first line treatment. Subsequent recurrence

Results

A total of 10 patients were included in our study with a total of 12 lesions. All patient were of Caucasian ethnicity. Sex was equally distributed and average age of onset was four years and nine months (range 2–107 months) (see Figure 1). Average duration of lesion before treatment was 14 months (range 2–72 months). Of the twelve lesions, 58% (7/12) were causing pain and 16% (2/12) contractures. The remaining three lesions 25% (3/12) were rapidly growing, defined as doubling in size over a

Discussion

Infantile Digital Fibromatosis (IDF) is a relatively benign childhood tumour that occurs almost exclusively on the digits. Classically lesion appear de novo as a small raised firm skin-coloured nodule on the digital extremities in the first three years of life.1 They can be single or multiple. In the majority of cases, these small dome-shaped lesions are asymptomatic but symptoms can develop if located over points of flexion or from an increase in size. Classically these lesions slowly increase

Conclusion

Out study is the first study to look at the role of corticosteroid in the treatment of symptomatic IDF. Whilst our study has not demonstrated a significant difference in recurrence rate between surgery and steroid treatment, we have shown for the first time results of using intra-lesional steroid for this condition and have provided power calculations to allow a multicentre trial to be conducted.

For small asymptomatic lesions a period of observation is appropriate. In the case of symptomatic or

Acknowledgment

Special thanks to Dr Kerry Dwan, Department of Biostatistics, University of Liverpool, for her help with statistical analysis.

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    8th World Symposium on Congenital Malformations of the Hand and Upper limb. Hamburg, Germany. September 2009.

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    BAPRAS, Royal College of Surgeons England. London UK. December 2009.

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