Congenital partial hypoplasia of the lower lip: a rare form of 28-29
Tessier cleft?
Olina Rios (a), Virginie Lafontaine (b), Cyril Debortoli (a), Charles
Savoldelli (a), Beatrice Morand (b)
Corresponding author:
rios.o@chu-nice.fr
(a) Department of Oral and Maxillo-facial surgery, Head and Neck
Institute, University Hospital Center of Nice, 06100 Nice, France
(b) Department of Maxillo-facial and Plastic surgery, University
Hospital Center of Grenoble-Alpes, 38000 Grenoble, France
Key Clinical Message:
We aim to describe a case of congenital partial hypoplasia of the lower
lip in a 2-year-old child. As this anomaly was associated with other
oral anomalies, we explored the possibility of a modified 28-29 Tessier
cleft. However, other hypotheses were proposed as we may be dealing with
an unknown mechanism.
Keywords: labial hypoplasia, lower cleft lip, craniofacial
abnormalities, Tessier clefts.
Introduction:
First described by Couronne in 1819, cleft mandible and/or lower lip is
one of the rarest craniofacial clefts with less than 100 cases reported
in the literature 1,2. In almost all cases, the
midline location of the lip and/or mandible has been noted. According to
Tessier’s classification of 1976, cleft lip and/or mandible midline is
defined as no. 30.
The paramedian lower cleft lip is even rarer, with only 5 cases
described. However, in these cases the defect occurs in a very specific
part of the lower lip and/or mandible. In the case described below,
there was a defect of the entire lateral third of the lower lip.
Therefore, it could not be formally associated with a 28-29 Tessier
cleft and may not have the same origin.
Case history:
We aim to report the case of a 2-year-old boy who was born with
hypotrophy of the lateral third of the lower lip. To the best of our
knowledge, this type of anomaly has not been previously reported in the
literature. Written informed consent was obtained from the parents of
the child. This case report was submitted with their consent and with
the approval of the Ethics Committee of the Face and Neck Institute.
The patient was first referred to the craniofacial cleft team at the
Grenoble University Hospital Centre at the age of 5 months. He was the
first child of healthy parents with no consanguinity. The pregnancy was
spontaneous, and the mother had a previous miscarriage. There was a
family history of congenital dental anomalies: the mother’s aunt had
only milk teeth and the mother of our patient was missing two permanent
teeth (34 and 44). There was no exposure to teratogens or x-rays during
pregnancy. The mother was 30 years old at the time of delivery. The boy
was born at term by normal vaginal delivery. Birth weight was 3215 g
with a cranial circumference of 35 mm. The Apgar score was 10/10. He
didn’t have any other anomalies.
Investigations:
Cardiological examination and echocardiography were normal. Feeding was
normal, although the parents noted a tendency to drool due to
difficulties in achieving complete lip closure. Growth was normal.
Examination revealed hypoplasia of the right lateral third of the lower
lip (Figure 1A). There was no facial paralysis and the smile
appeared symmetrical. Intra-oral examination revealed a gingival notch,
a mucosal bridle and the median labial frenulum was deviated on the
right side (Figure 1B). Lip eversion was possible. There were
no abnormal findings on the tongue, oral mucosa, pharynx or tonsils, but
a kystic lesion was visible on the anterior part of the uvula. No
abnormality was seen on the rest of the body. Circumscriptions and
amputations of digits or limbs weren’t noticed. As the development was
normal, a simple follow-up with an annual visit was chosen.