O. Heymans Æ C. Ronsmans
Congenital inﬁltrating lipomatosis of the face
Received: 16 September 2004 / Accepted: 21 December 2004 / Published online: 7 July 2005
Ó Springer-Verlag 2005
Abstract Congenital lipomatosis of the face is a very
rare syndrome deﬁned as a collection of non-encapsu-
lated mature lipocytes inﬁltrating local tissues: resulting
in cranio-facial deformities. Due to the normal psycho-
motor development of the children, aesthetic appearance
often remains the main concern. After a precise
description of a case, a complete overview of the liter-
ature data was made. New clinicopathologic data con-
cerning bone involvement will be presented and
analyzed in the light of embryonic growth.
Keywords Congenital Æ Lipomatosis Æ
Congenital lipomatosis of the face is a very rare syn-
drome, ﬁrst described by Slavin et al.  followed by
other reports, the total number of cases being 28. As
with many other birth defects, the etiopathogeny is
poorly understood. However, due to the normal psy-
chomotor development of the children, aesthetic
appearance often remains the main concern.
After a precise description of a case, a complete
overview of the literature is presented. New clinico-
pathologic data concerning bone involvement will be
discussed and analyzed in the light of embryonic growth.
A 43-month-old girl was referred to the plastic surgery
department with a right diﬀuse hemi facial deformity.
This had been noticed at birth, but was still increasing in
size. Clinical examination demonstrated an inﬁltration
of the subcutaneous tissue extending from the cheek and
the mandible to the right fronto-temporal region
(Fig. 1). The lesion was soft and painless. We noticed a
hyperaemia on the right cheek but no sign of facial nerve
compression or bony overgrowth. Although dental
occlusion was normal three masses were located on the
right side of the lower lip (Fig. 1).
The CT imaging and MRI did not show central
nervous deformity nor ocular, bony, arterial or venous
anomaly. A non-encapsulated fatty tissue was inﬁltrat-
ing the cheek, the masseteric and pterygoid region. Fatty
tissue was also noticed in the right fronto-temporal
subcutaneous layer and diploe (Fig. 2).
The parents were adamant in wishing for a surgical
approach because of the child’s appearance. We chose
the vibroliposuction technique used for subcutaneous fat
resection. A total amount of 90 cc was aspirated after
tumescent inﬁltration. Pathologic examination of the
excised fat did not reveal any particular appearance.
Although painless, the lip tumors resected were neuro-
In the short term, the child’s appearance was
improved. Fourteen months later, the frontal mass
recurred slightly as well as the mucosal neuroma leading
to another vibrolipoaspiration session and lip resection.
An MRI of the face was taken 12 months after surgery,
demonstrating improvements as shown in the illustra-
tions (Figs. 1, 2).
Slavin et al.  ﬁrst reported three cases of what they
termed as congenital inﬁltrating lipomatosis of the face
(CIL-F). It was therefore deﬁned as a distinct clinico-
pathological entity: a collection of non-encapsulated
mature lipocytes inﬁltrating local tissues. The patho-
genesis of this condition is not well known. Two cases of
CIL-F associated with congenital Cytomegalovirus
O. Heymans (&) Æ C. Ronsmans
Department of Plastic and Maxillo-Facial Surgery,
CHU Sart-Tilman, 4000 Lie
Eur J Plast Surg (2005) 28: 186–189