Zekeriya Tosun Æ Adem Ozkan Æ Sadık Senturk
A rare involvement of Romberg’s disease: frontal region
Received: 29 March 2004 / Accepted: 28 December 2004 / Published online: 28 April 2005
Ó Springer-Verlag 2005
Abstract Progressive hemifacial atrophy is characterized
by slow progressive atrophy of the subcutaneous tissue.
Although, in the majority of suﬀerers, half of the face
and the chin are the most severely-aﬀected areas, we
present the case of a patient with a 2-year history of local
atrophy in the forehead, a very rare involvement.
Reconstruction was done using bilateral subcutaneous
ﬂaps. There was no recurrence of the atrophy at the
12th-month postoperative check.
Keywords Romberg’s disease Æ Face Æ Atrophy
Progressive hemifacial atrophy, known as Romberg’s
disease, begins in the 1st or 2nd decade of life and aﬀects
the skin, fat, muscle, cartilage and bone . All patients
were previously healthy. The etiology of Romberg’s
disease is unknown . However, some theories have
been proposed, such as encephalitis, complicated mi-
graine, slow virus infection, sympathetic nervous system
hyperactivity, trigeminal peripheral neuritis and sclero-
Several conventional reconstructive procedures have
been used to correct facial asymmetry, and soft-tissue
augmentation is adequate in most cases . However,
there is no deﬁnitive treatment for cases involving
the forehead region. We present an unusual case of
Romberg’s disease and its treatment in this paper.
Case report and surgical technique
A 21-year-old male patient presented at our clinic with a
vertical depression in the forehead with a 2-year history.
It was an atrophy measuring 8 cm·2 cm which was lo-
cated in the middle of the forehead, from the eyebrow
to the hairline (Fig. 1a, b). The patient had no other
systemic disorders, and had no family history for
Romberg’s or any immune disease. Computed tomog-
raphy of the brain was normal. Three-dimensional
computed tomography showed atrophy of the frontal
bone (Fig. 2). Antinuclear antibody was investigated.
The atrophic area was de-epithelialised under local
anesthesia. In order to reconstruct the tissue three-
dimensionally, the skin, subcutaneous tissue and frontal
muscle were prepared as an advancement ﬂap. The
location of the patient’s atrophy was restricted to the
forehead region only, so big ﬂap or graft operations
were not necessary, as subcutaneous advancement ﬂaps
were adequate for reconstruction.
Both lateral subcutaneous tissues were approximated
as an advancement ﬂap and then sutured with 4/0 po-
lyglactin absorbable suture material. The two cutaneous
tissues were sutured with 4/0 polypropylene. Histo-
pathologic examination of excised skin revealed atrophy
of skin adnexies, and chronic inﬂammation with pres-
ervation of elastic tissue.
The patient was followed up for 1 year, and there was
no recurrence of the atrophy at the 12-month postop-
erative check. The patient exhibited his satisfaction with
the good cosmetic result (Fig. 3a–c).
Romberg’s disease was ﬁrst described by Romberg and
later by Parry in 1825 and 1946 respectively . It is a
rare condition characterized by progressive hemifacial
atrophy. To date, the etiology of the disease is unknown.
The atrophic process typically has its onset in the ﬁrst
or second decade of life; it continues for a few years
Z. Tosun (&) Æ A. Ozkan Æ S. Senturk Æ N. Savaci
Department of Plastic and Reconstructive Surgery,
Selc¸ uk University Meram Medical Faculty,
42080 Meram Konya, Turkey
Eur J Plast Surg (2005) 28: 38–41