A rare case of peri-ocular congenital infiltrating lipomatosis with associated schizencephaly

Congenital infiltrating lipomatosis of the face (CILF) is a rare, congenital disorder, characterised by non-encapsulated overgrowth of mature adipose tissue with a propensity to infiltrate the soft tissues of the face and surrounding anatomical spaces. Although benign, the involvement of multiple soft tissue planes, muscles, and bone make complete or near complete surgical excision both challenging and disfiguring. Whilst this condition may present in the early stages of life, the hypertrophic changes are typically progressive, with more significant manifestations being reported in older age groups. Advancements in radiological testing have allowed for more accurate identification of cases with precise management through planned and staged surgical interventions. To the best of our knowledge, this is the first case in the literature to report orbital involvement of CILF with associated intracranial manifestations, in this case, schizencephaly with grey matter heterotopia.

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Publication Details

Journal
Orbit
Published
2025-12-05
DOI
https://doi.org/10.1080/01676830.2025.2594473
Primary Topic
Teratomas and Epidermoid Cysts
Type
article
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article

A rare case of peri-ocular congenital infiltrating lipomatosis with associated schizencephaly

John C. Bladen, Steve Connor, Matthew Fenech
Orbit
Teratomas and Epidermoid Cysts
article

A rare case of peri-ocular congenital infiltrating lipomatosis with associated schizencephaly

John C. Bladen, Steve Connor, Matthew Fenech
article en

Abstract

Congenital infiltrating lipomatosis of the face (CILF) is a rare, congenital disorder, characterised by non-encapsulated overgrowth of mature adipose tissue with a propensity to infiltrate the soft tissues of the face and surrounding anatomical spaces. Although benign, the involvement of multiple soft tissue planes, muscles, and bone make complete or near complete surgical excision both challenging and disfiguring. Whilst this condition may present in the early stages of life, the hypertrophic changes are typically progressive, with more significant manifestations being reported in older age groups. Advancements in radiological testing have allowed for more accurate identification of cases with precise management through planned and staged surgical interventions. To the best of our knowledge, this is the first case in the literature to report orbital involvement of CILF with associated intracranial manifestations, in this case, schizencephaly with grey matter heterotopia.

OrbitVol. 45(2)
King's College Hospital NHS Foundation Trust (GB)
Openalex Percentile: Top 42%
Teratomas and Epidermoid Cysts
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A rare case of peri-ocular congenital infiltrating lipomatosis with associated schizencephaly — John C. Bladen, Steve Connor, et al. · Orbit (2025) | TGRS Research Map | TGRS