Congenital infiltrating lipomatosis of the face is a rare lesion that comprises a subgroup of lipomatous tumor-like lesions of infancy and childhood. It is characterized by (1) no encapsulation, (2) diffuse infiltration of mature adipose tissue over normal muscle fiber and surrounding structures of face, (3) osseous hyperplasia of subjacent bone, and (4) a high recurrence rate. We report a case of a nine-month-old infant who presented with swelling over right face since birth. Early diagnosis of this lesion provides better surgical approach to control the infiltrative nature of its growth with recurrence and aesthetic appearance.
Congenital infiltrating lipomatosis of the face (CIL-F) is a distinct clinicopathological rare entity, first described by Slavin et al. in 1983. It comprises a subgroup of lipomatous tumor-like lesions and is characterized by collection of nonencapsulated, mature adipocytes that infiltrate local tissues, leading to craniofacial deformities and tend to recur after surgery. The tumor-like lesion is congenital in origin and occurs in infancy or early childhood as unilateral facial asymmetry [
A 9-month-old male infant presented with swelling over the right face since birth that had been progressively increasing in size and early eruption of deciduous teeth. Mother gives history of uneventful pregnancy and birth. Developmental mile stones were normal. Local examination revealed a soft nontender, noncompressible, ill-defined, and diffuse swelling over the right side of the face causing unilateral facial hypertrophy (Figure
Right diffuse facial swelling.
Grossly the specimen measured
Yellowish white adipose tissue with grey-white areas.
Nonencapsulated, mature adipose tissue infiltrating salivary gland tissue. (H and E, 100x).
High-power view showing mature adipose tissue infiltrating muscle, nerve fibers, and thickened blood vessels. (H and E, 400x).
CIL-F is a diffuse overgrowth of fatty tissue of the face, infiltrating local tissues resulting in cranio facial deformity [
The facial asymmetry at birth was the common clinical presentation of CIL-F. The other associated clinical features that have been described in the recent studies include ipsilateral hypertrophy of the underlying facial skeleton, increased density of the facial hair on affected side, ipsilateral hemimacroglossia and ptosis, ipsilateral faint cutaneous capillary stain, early eruption of deciduous, permanent teeth and missing teeth associated with dentigerous cyst [
Associations with various intracranial abnormalities like hemimegalencephaly, arachnoid cyst [
Microscopically, this lesion has to be differentiated from various lipomatous lesions. CIL-F is termed lipomatosis because it is more diffusely infiltrative in nature than a lipoma, which is well defined and encapsulated. Lipoblastomatosis is composed of immature lipoblasts and most occur on the extremities, trunk, and neck. Liposarcomas differs from CIL-F by the presence of pleomorphism, mitotic figures with capillary network and are usually located on the trunk and extremities, and rarely occur in infantile or congenital forms [
The treatment modalities available are excision or liposuction mainly for cosmetic reasons [
CIL-F of the face is a rare benign disorder of lipomatous tissue in infancy or childhood. It should be considered when evaluating the cause of facial asymmetry. Clinical examination, imaging studies, and histopathological findings help in early diagnosis. The main purpose of surgery is to improve the cosmetic appearance of the face rather than to eradicate the tumour.