Eur J Plast Surg (1998) 21:370±373
U. Beinhoff ´ H. Piza-Katzer
Double lip in a patient with Aschers syndrome
Received: 25 February 1998 / Accepted: 17 March 1998
U. Beinhoff (
) ´ H. Piza-Katzer
Abteilung für Plastische und Wiederherstellungschirurgie,
Krankenhaus der Stadt Wien - Lainz, Wolkersbergenstrasse 1,
A-1130 Wien, Austria
Tel.: +43-1-80110-2650; Fax: +43-1-80110-2719
Abstract Ascher's syndrome is a rare disease which was
first described by Ascher , an ophthalmologist from
Prague, in 1920. His careful examinations of mostly
young patients brought to light three more or less consis-
tently associated abnormalities: double upper lip, blepha-
rochalasis and enlargement of the thyroid. Of the patients
with congenital double upper lip reported in the literature
[1±15], about 50% presented with Ascher's syndrome. To
date, just over 100 cases have been described. As with
other syndromes, one or the other abnormality may pre-
dominate or be absent. A case of a patient with double
lip and blepharochalasis is presented.
Key words Double upper lip ´ Blepharochalasis ´
A 12-year-old patient complained of changes in his upper lip which
he considered cosmetically objectionable. The occurrence of double
upper lip had coincided with the eruption of his permanent dentition.
The anomaly was most obvious when the patient laughed. The fam-
ily history was not significant.
The boy had been seen by several specialists, but none had rec-
ognized the association with his prominent blepharochalasis. A
number of unnecessary examinations and interventions had been or-
dered, among them visual acuity tests, orthodontic treatment, renal
function tests and allergy tests, all of which were normal.
Full lips were a hallmark of the patient's family. With the facial
muscles at rest nothing abnormal was seen. But when the patient
opened his mouth and stretched his upper lip, i.e. when he laughed
or bared his teeth, the double lip and an enlarged transverse sulcus at
the mucosal junction bilaterally extending into the buccal mucosa
became evident. The right and left portions of the lip did not meet
in the midline (Fig. 1). The frenulum was normal without shorten-
Bilateral blepharochalasis was a prominent feature. The skin be-
tween the eyebrows and the upper lid margins drooped, was flaccid
and baggy and appeared to be thinned (Fig. 2). When the redundant
skin was picked up with the fingers and pinched, the resultant skin
fold persisted for longer than normal. The skin changes did not extend
beyond the eyelids temporally and nasally. The palpebral fold was ef-
faced and a suggested epicanthus was noticeable bilaterally. Visual
acuity was impaired and the patient wore glasses for correction.
The neck circumference was normal and the thyroid did not
show any abnormalities clinically. Serology and scintigraphy con-
firmed the normal shape, size and function of the gland.
During surgery under general anesthesia the ªaccessoryº lip was
excised as a double ellipse, the wound was closed by primary su-
tures and a light compression dressing was applied for 3 days.
Wound healing was uneventful. On histological examination, the re-
sected double lip tissue was found to contain clusters the submucous
glands with loose lymphocyte and plasma cell infiltrates. The squa-
mous epithelium was cytologically normal, but somewhat hyper-
plastic. This histology agreed well with what was reported by others.
Both the young patient and his parents were content with the out-
come of surgery, but decided to have the eyelids corrected at a later
date (Fig. 3).
In 1920 Ascher  first described the association of dou-
ble lip with blepharochalasis of both eyelids and non-tox-
ic goiter. As with other syndromes, one or the other of
these abnormalities may predominate. A review of the
pertinent literature showed a large measure of agreement
between our patient and the cases reported: as in these, the
development of the double upper lip had coincided with
the eruption of the permanent teeth and the blepharocha-
lasis had not become apparent before school age.
There is no evidence in the literature of sex predilec-
tion or heredity. But quoting the comprehensive review
by Panneton , some authors suggested a potential he-
reditary nature of the condition . There is also no evi-
dence of an association with racial features.
Double lip is a congenital anomaly although, as in
Ascher's syndrome, it does not become manifest before
childhood coinciding with the erupton of the permanent
teeth. Furrows in the upper lip mucosa are also often seen.
But these are only apparent during innervation and, in
contrast to a true double lip, disappear when the upper