Paediatrics and International Child Health

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Cleft palate caused by congenital teratoma

Alexis Veyssière, Libor Streit, Hamady Traoré & Hervé Bénateau

To cite this article: Alexis Veyssière, Libor Streit, Hamady Traoré & Hervé Bénateau (2017) Cleft
palate caused by congenital teratoma, Paediatrics and International Child Health, 37:1, 66-69, DOI:
10.1179/2046905515Y.0000000057

To link to this article: http://dx.doi.org/10.1179/2046905515Y.0000000057

Published online: 06 Mar 2017.

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Download by: [FU Berlin] Date: 10 March 2017, At: 11:24

 Case Report
Cleft palate caused by congenital teratoma
Alexis Veyssière1, Libor Streit2, Hamady Traoré1, Hervé Bénateau1
1
Department of Maxillofacial and Plastic Surgery, Caen University Hospital, France, 2Department of Plastic and
Aesthetic Surgery, St Anne University Hospital, Brno, Czech Republic

A cleft palate results from incomplete fusion of the lateral palatine processes, the median nasal septum
and the median palatine process. This case report describes a rare case of congenital teratoma originating
from the nasal septum that may have interfered with the fusion of the palatal shelves during embryonic
development, resulting in a cleft palate. An infant girl was born at 40 weeks of gestation weighing 3020
g with a complete cleft palate associated with a large central nasopharyngeal tumour. Computed tomogra-
phy (CT) of the head showed a well defined mass of mixed density. The tumour was attached to the nasal
septum in direct contact with the cleft palate. A biopsy confirmed the teratoma. Tumour resection was
performed at 5 months, soft palate reconstruction at 7 months and hard palate closure at 14 months.
There was no sign of local recurrence 1 year later. Most teratomas are benign and the prognosis is usually
good. However, recurrence is not rare if germ cell carcinomatous foci are present within the teratoma. For
these reasons, we advocate the use of a two-stage procedure in which closure of the cleft palate is post-
poned until histological examination confirms complete excision of the teratoma.
Keywords: Benign teratoma, Cleft palate

Introduction Case Report

A cleft palate results from the incomplete fusion of
the palatal shelves with each other, and with the
median nasal septum and the median palatine pro-
cess. Any factor that leads to incorrect palate
fusion, which occurs most frequently between the
7th and 11th week of gestation, can result in the
development of a cleft palate.1
A teratoma is a true neoplasm consisting of differ-
ent types of tissue arising from all three embryonic
germ layers. Occurrence in the head and neck is
rare, but predominantly involves the cervical region
and the nasopharynx (1/20,000–40,000 births).
Head and neck teratomas most commonly emerge
during the neonatal period between the 7th and 9th
week of gestation and are associated with airway
obstruction and a high rate of mortality in untreated
patients. If tumours occur before birth, they are
usually benign, whereas malignant teratomas are
more common in the post-neonatal period.2
The aim of this case report is to report a rare case
of congenital teratoma originating from the nasal
septum that may have prevented the palatal shelves
from fusing during embryonic development, resulting
in a cleft palate.
Correspondence to: A Veyssière, Department of Maxillofacial and Plastic
Surgery, Caen University Hospital, Avenue Côte de Nacre, 14000 Caen,
France. Email: alexis.veyssiere@hotmail.fr
A full-term infant girl was born by normal vaginal
delivery. The pregnancy was uneventful with no
sign of cleft palate or teratoma on prenatal ultra-
sound screening and there was no relevant medical
history within the family. Her birthweight was 3020
g and her Apgar score was 8 and 10 at 1 and 5 min-
utes, respectively. She showed signs of respiratory
distress and was monitored in the neonatal intensive
care unit. Some desaturation (down to a PaO2 of
90%) was observed for a few days while the infant
was sleeping in the supine position, but bradycardia
was not observed.
Clinical examination demonstrated a complete
cleft palate associated with a large central nasophar-
yngeal tumour (Fig. 1). Moderate retrognathism was
also noted. A head computed tomography (CT) scan
showed a well defined mass measuring 1.7|1.5|3.8
cm. The tumour was attached to the nasal septum
and was of mixed density with solid and cystic com-
ponents. The mass was in direct contact with the clef
palate laterally (Fig. 2). Transfontanelle, cardiac and
abdominal ultrasound scans were normal. The dur-
ation of postnatal hospital stay was 14 days. Home
apnoea and bradycardia monitoring was undertaken
for the 3 months after birth.
The first surgical procedure was a simple biopsy of
the tumour performed at 5 weeks of age under gen-
eral anaesthesia. Histopathological examination
demonstrated a benign, mature teratoma which was

ß W. S. Maney & Son Ltd 2016

66 DOI 10.1179/2046905515Y.0000000057 Paediatrics and International Child Health 2017 VOL . 37 NO . 1

Figure 1 Complete cleft palate associated with a large
central nasopharyngeal tumour arising centrally from the
nasal septum
Figure 2 Pre-operative head CT scan demonstrating a
tumour between the two palate shelves attached to the
nasal septum
surgically excised at the age of 5 months along with a
margin of healthy tissue surrounding the mass, which
revealed a cleavage plane on the nasal septum (Fig.
3). The cartilaginous and bony nasal septum was pro-
tected by suturing the two sides of the mucosa
Figure 3 The nasopharynx after ablation of the teratoma at
5 months; the cartilaginous and bony nasal septum is cov-
ered by the mucosal membrane
2 Paediatrics and International Child Health
Veyssière et al. Teratoma and cleft palate
together. Post-operative healing was normal. The
patient needed 3 days to resume bottle-feeding with-
out vomiting or regurgitation. She was discharged 1
week after surgery. The final histological examin-
ation confirmed a mature benign teratoma which
was completely excised.
Soft palate reconstruction was performed by the
Sommerlad technique at the age of 7 months. The
post-operative period was uneventful and the infant
was discharged 4 days after surgery. Hard palate closure
was performed at the age of 14 months and there was no
sign of local recurrence of the teratoma at this time.
Figures 4 and 5 demonstrate the hard palate before
and after surgery. The procedure was undertaken in
our Cleft Center without relaxing incisions and bone
exposure. The cleft closure was successful and there
was no sign of recurrence within a 12-month follow-
up period after removal of the tumour. Today, the girl
is 26 months old and the palate is well closed.
Discussion
Cleft lip and palate is a common congenital anomaly.
In Europe, about 1/500–700 children are born with a
cleft lip or palate or both. Risk factors can be genetic
or environmental (e.g. alcohol, radiation, drugs).
Figure 4 The nasopharynx at 13 months before hard palate
closure
Figure 5 The nasopharynx at 14 months after hard palate
closure
2017 VOL . 37 NO . 1 67
Veyssière et al. Teratoma and cleft palate
The development of the mammalian palate can be
divided into four stages: migration of the neural
crest cells at days 17–20 of gestation; growth of pala-
tal shelves at the end of the 6th week; elevation of the
palatal shelves from the 7th to the 10th week; and
differentiation of the palatal epithelium and fusion
of the palatal shelves and nasal septum during the
12th week. Any interference during these stages
may result in failure of fusion and the formation of
a cleft palate.1 Thus, any tumour growing between
the palatal shelves before or at these stages may
lead to a cleft palate because the mass mechanically
interferes with fusion of the palatal shelves.3 In
theory, the growth of this abnormal mass at a later
time point may also lead to a cleft palate by mechan-
ical extrusion and destruction of an already existing
palate, although such events are very rare. It is poss-
ible that this pathological mass arose on an existing
cleft palate in the later stages of gestation, but the
coincidental growth of a pathological mass and the
formation of a cleft palate seems unlikely.
Teratomas are the most frequently occurring
pathological masses in the head and neck region of
neonates and usually involve the central neural
axis. It is still unclear what causes them. The most
widely accepted theory is that they arise from
embryonic pluripotent cells that are somehow dis-
placed during embryogenesis.4,5 According to
Arnold’s frequently used classification, there are
four mains types of teratoma which are distinguished
by the degree of cellular differentiation: dermoids,
the most common type, also called hair polyps, are
composed of ectoderm and mesoderm; teratoids,
which are poorly differentiated lesions consisting of
three germinal layers (ectoderm, mesoderm, endo-
derm); true teratomas, which also contain all three
germ layers like teratoids but are well differentiated
and sometimes organised into a recognisable early
organ (cartilage, teeth, etc.); and epignathus, also
known as ‘fetus in fetu’, which are highly differen-
tiated teratomas that often arise from the jaw and
protrude from the mouth.6 Epignathuses are very
rare and have a high mortality rate.3,5–7
Oropharyngeal teratomas are a rare neoplasm
usually arising from the base of the skull in the pos-
terior region of the nasopharynx. They often appear
in the midline (from the brain to the sacral area).
Teratomas in the mouth are rare but may involve
the hard palate and sphenoid bone.3,5,8 Teratomas
usually develop during weeks 7–9 of gestation. The
growth of a teratoma during this critical period
may interfere with the narrowing and fusion of the
palatal shelves and nasal septum.3
Some teratomas may be associated with other mal-
formations. The most frequently associated malfor-
mation is a cleft palate and there are 21 cases in
68 Paediatrics and International Child Health 2017 VOL .
the literature of midline teratomas arising from the
skull base associated with a cleft palate7. However,
other malformations may also be reported. These
include cardiac abnormalities, microcephaly, atresia
of the left common carotid artery, micrognatia, ingu-
inal and umbilical hernias and hydrocele.7 Our infant
also had an inguinal hernia.
Ideally, nasopharyngeal teratoma should be diag-
nosed in utero. Approximately 18% of mothers carry-
ing infants with a nasopharyngeal teratoma have
polyhydramnios on fetal ultrasound screening because
the fetus is unable to swallow amniotic fluid.9 In some
cases, the teratoma can be detected directly on prena-
tal ultrasound but false negative results are commonly
reported for the palatal region. High alpha-fetoprotein
(AFP) levels in amniotic fluid have also been
reported.9 Prenatal diagnosis of a teratoma should
facilitate the planning of childbirth.
The management of intra-oral teratomas is chal-
lenging and airway control usually takes priority.
In cases of respiratory or feeding problems, immedi-
ate surgery is required to remove the tumour. Other-
wise, surgery can be delayed, as in this patient.
However, regardless of timing, complete surgical
excision is the main treatment. We believe that
early surgical excision may prevent further destruc-
tion of the palate by a teratoma growing in a patient
with a cleft palate. Most teratomas are benign and
the prognosis is usually good. However, recurrence
is not rare if germ cell carcinomatous foci are present
within the teratoma.3,9 When histological examin-
ation confirms complete excision of the teratoma,
the cleft palate can be repaired. If there is doubt
about complete excision, re-excision should be under-
taken. Our Cleft Center uses the Sommerlad tech-
nique for soft palate reconstruction at 6 months of
age with hard palate closure between 12 and 18
months. In our experience, this results in closure of
almost all types of cleft palate without lateral relax-
ing incisions and bone exposure.
Regular multi-discipline clinical follow-up of patients
with a nasopharyngeal teratoma does not differ signifi-
cantly from that of other cleft palate patients. However,
care must be taken to verify that the teratoma does not
recur. Computer tomography or magnetic resonance
examination and monitoring of serum levels of human
chorionic gonadotropin (HCG) and AFP may be
useful.10 HCG and AFP levels in our patient were
within the normal range before excision and were there-
fore not useful during follow-up.
Teratomas are embryonic tumours which develop
between the 7th and 9th week of gestation and can
be malignant or benign.
Management of a cleft palate on a nasopharyngeal
teratoma commences with extirpation of the tera-
toma and then classical management of the cleft
37 NO . 1
 Veyssière et al. Teratoma and cleft palate

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1870;50:482–512.
Funding Yes. 7 Cay A, Bektas D, Imamoglu M, Bahadir O, Cobanoglu U,
Sarihan H. Oral teratoma: a case report and literature review.
Pediatr Surg Int. 2004;20:304–8.
Conflict of interest None. 8 Makki FM, Al-Mazrou KA. Nasopharyngeal teratoma associ-
ated with cleft palate in a newborn. Eur Arch Otorhinolaryn-
Ethics approval Not required. gol. 2008;265:1413–15.
9 Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P,
Nuyts JP, Cuisset JM, et al. Epignathus teratoma: report of
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