Congenital infiltrating lipomatosis of the face: A subtype of hemifacial hyperplasia

Int J Pediatr Otorhinolaryngol. 2019 Oct:125:107-112. doi: 10.1016/j.ijporl.2019.06.032. Epub 2019 Jun 30.

Abstract

Objective: To investigate the clinical, imaging and pathological features of congenital infiltrating lipomatosis of the face (CILF) and to discuss whether it is a subtype of hemifacial hyperplasia (HH).

Methods: Sixteen patients diagnosed with CILF were included in this study. All patients had undergone panoramic radiography and spiral CT examinations. Thirteen patients received biopsy, surgery treatment and pathological examination. The clinical documentation and imaging data were retrospectively reviewed.

Results: The cheeks (14/16), parotid glands (12/16), tongues (9/16), masticatory muscles (8/16) and the lips (7/16) were the most frequently affected soft tissue organs. The maxilla (14/16), zygoma (13/16), mandible (13/16) were involved among the maxillofacial bones. Dental malformations included macrodontia (8/16), poor formation of the roots (7/16), accelerated tooth germ development or premature eruption of permanent teeth (7/16) and missing of the permanent teeth (4/16). All malformations were restricted to one side of the face and did not trespass the middle line. Pathologically, CILF was featured by the diffuse infiltration of redundant mature adipose tissue into the tissue of the affected organ.

Conclusion: CILF is a congenital developmental facial malformation characterized by infiltration of nonencapsulated, mature adipose tissue, resulting in facial soft and hard tissue hypertrophy and dental malformations in hemifacial structures. CILF could be considered as a subtype of HH.

Keywords: Congenital; Dental; Face; Hyperplasia; Lipomatosis; Malformation.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Face / abnormalities*
  • Face / diagnostic imaging
  • Face / surgery*
  • Facial Asymmetry / congenital*
  • Facial Asymmetry / diagnostic imaging
  • Facial Asymmetry / etiology
  • Facial Asymmetry / surgery
  • Female
  • Humans
  • Hyperplasia / diagnostic imaging
  • Hyperplasia / etiology*
  • Hyperplasia / surgery
  • Lipomatosis / congenital*
  • Lipomatosis / diagnostic imaging*
  • Lipomatosis / surgery
  • Male
  • Radiography, Panoramic
  • Retrospective Studies
  • Tomography, Spiral Computed
  • Tooth Abnormalities / diagnostic imaging
  • Tooth Abnormalities / etiology
  • Young Adult

Supplementary concepts

  • Facial Hemihypertrophy