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Fibrolipomatous hamartomas of the median nerve in infancy and early childhood—imaging hallmarks, symptomatology, and treatment

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Abstract

A fibrolipomatous hamartoma (FLH) is a rare lesion leading to an enlargement of the affected nerve and commonly manifests at the median nerve. Symptomatic patients are mostly adolescents or adults. In children below 10 years, this entity is rather unknown and likely to be misdiagnosed. We report three children with FLH, two severely and one mildly symptomatic, all below 4 years of age at the time of first presentation. Two of three children were initially misdiagnosed. We provide a review of the pertinent clinical and radiological findings of the entity. Two patients had a characteristic macrodactyly. The two symptomatic children underwent surgical carpal tunnel decompression. The intervention relived their symptoms with a long-lasting effect. Surgical reduction of the hamartoma mass is not indicated and medical treatment non-existent.

Conclusion: A symptomatic FLH of the median nerve is rare in children below the age of 5 years but has to be kept in mind as differential diagnosis in case of wrist and/or palm swelling, macrodactyly, and pain in hand or forearm. MRI is diagnostic, with very characteristic features, which can also be identified in high-resolution nerve ultrasound. This article aims to increase the knowledge about the entity including the diagnostic features and the management options.

What is Known:

• Fibrolipomatous hamartomas (FLHs) of the median nerve are rare, possibly associated with macrodactyly and tissue growth at the wrist and thenar side of the palm.

• An associated carpal tunnel syndrome typically occurs, if at all, in adulthood.

What is New:

• We describe two children below 4 years with symptomatic carpal tunnel syndrome, experiencing a long-lasting favorable outcome after carpal tunnel decompression. In this age group, only one other child undergoing surgery has been published so far.

• MRI and high-resolution ultrasound demonstrate the characteristic features of FLHs and are the diagnostic modalities of choice. Biopsy is not recommended.

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Abbreviations

FLH:

Fibrolipomatous hamartoma

NSAID:

Non-steroidal anti-inflammatory drugs

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Department of Neurosurgery, University Hospital of Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany

    Humphrey Okechi, Artemisia Dimostheni, Susanne R. Kerscher, Isabel Gugel & Martin U. Schuhmann

  2. Department of Pediatric Neurology, University Children’s Hospital of Tübingen, Tübingen, Germany

    Andrea Bevot

  3. Division of Pediatric Radiology, Department of Radiology, University Hospital of Tübingen, Tübingen, Germany

    Juergen F. Schaefer

Authors
  1. Humphrey Okechi
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  2. Artemisia Dimostheni
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  3. Susanne R. Kerscher
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  4. Isabel Gugel
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  5. Andrea Bevot
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  6. Juergen F. Schaefer
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  7. Martin U. Schuhmann
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Contributions

HO, SRK, and MUS wrote the main manuscript including literature research.

MUS performed the revision.

AD, AB, and IG supplied the case descriptions.

MUS provided the clinical photographs and the corresponding figure legends.

JFS performed MRI and ultrasound and provided the corresponding text sections and figure legends.

All authors read, edited, and approved the final manuscript.

Corresponding author

Correspondence to Martin U. Schuhmann.

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The authors declare that they have no conflict of interest.

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This article does not contain any studies with human participants performed by any of the authors.

Additional information

Communicated by Mario Bianchetti

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Cite this article

Okechi, H., Dimostheni, A., Kerscher, S.R. et al. Fibrolipomatous hamartomas of the median nerve in infancy and early childhood—imaging hallmarks, symptomatology, and treatment. Eur J Pediatr 177, 567–573 (2018). https://doi.org/10.1007/s00431-018-3100-7

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  • DOI: https://doi.org/10.1007/s00431-018-3100-7

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