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Fibrolipomatous hamartomas of the median nerve in infancy and early childhood—imaging hallmarks, symptomatology, and treatment
- Source :
- European Journal of Pediatrics. 177:567-573
- Publication Year :
- 2018
- Publisher :
- Springer Science and Business Media LLC, 2018.
-
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.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.
- Subjects :
- Male
medicine.medical_specialty
Macrodactyly
Hamartoma
Wrist
030218 nuclear medicine & medical imaging
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
Forearm
medicine
Humans
Child
Carpal tunnel syndrome
Ultrasonography
030222 orthopedics
medicine.diagnostic_test
business.industry
Infant
Magnetic resonance imaging
Decompression, Surgical
medicine.disease
Carpal Tunnel Syndrome
Magnetic Resonance Imaging
Median nerve
Median Nerve
medicine.anatomical_structure
Child, Preschool
Pediatrics, Perinatology and Child Health
Female
Radiology
Differential diagnosis
business
Subjects
Details
- ISSN :
- 14321076 and 03406199
- Volume :
- 177
- Database :
- OpenAIRE
- Journal :
- European Journal of Pediatrics
- Accession number :
- edsair.doi.dedup.....2214d2861e9e74bcddb173e50e30f955