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Case presentation on congenital

midline oral mass


Presenter: Dr Aditya Gupta
Moderator: Dr Anumodan Gupta
B/O Shaista/39 weeks GA/2550 gms
born by NVD .

Demography
Presented at 6 hrs of age with C/C of:
Inability to
Palatal mass , Cleft palate.
feed

Currently this baby is discharged from


our hospital after staying in for 32
days.
Born to a 34 years old women
(G3P2L2 ) at 39 weeks of gestation
age out of non-consanginous
marriage

ANTENATAL First trimester: Uneventful


HISTORY

2nd trimester: received iron and


calcium supplementation and two
doses of tetanus toxoid.
No history of gestation HTN, diabetes,
hypothyroidism , smoking and intake of any
teratogenic drug .

No h/o of decreased fetal movements, excess or


decreased amniotic fluid .

No h/o s/c in other sibling or in family ,


No h/o intake of diet rich in polyunsaturated fats .

The other investigations done during pregnancy like


HIV, hepatitis B, VRDL were reported as normal.
Baby was delivered at kishtwar
hospital via normal vaginal delivery
and referred to SMGS hospital in
V/O congenital palatal mass .
DELIVERY
DETAILS Amniotic fluid was clear & fetal well
being was monitored by
intermittent fetal heart
auscultation. No history of fetal
distress present .
The baby then brought in NICU ,
kept NPO ,iv fluids started along
with antibiotics in V/0 leaking for
greater than 1 day, ENT call sent
for further management .
Post-natal
period
After ENT consultation baby was
planned for surgery ( excision of
palatal mass ) and all baseline
investigations done as advised .
Baby was lying supine comfortably under warmer , kept
NPO .
Vitals of the baby were :

HR 134 /min .

RR 34 /min .

TEMP 36.6 c .

SPO2 95 % on room air .

BSR 98 mg dl .
General examination : stable
General Appearance ..not sick looking
Vitals : with in normal limits
Anthropometry : within normal limits
length 50 cms , weight 2.5 kgs, OFC .35cm
No abnormal facial features ..
EYES , EARS , NOSE , MOUTH , NECK..nrml
SKIN ..no jaundice , cyanosis ,haemangioma
UMBLICUS norml
Genitalia ..testes descended , no ambiguous
genitalia
Back and Spine …no MMC or Spina bifida
Respiratory ..no tachypnea , scr ,

EXAMIN no added sounds


CVS ..S1, S2 present , no murmur
Abdomen soft , nt , nd ,
no organomegaly
CNS .. Active ,

.
• No evidence of turner syndrome
local examination
HISTORY related to mass
Site ..oral cavity ( palatal mass )
Duration ….since birth
No progression with time
Secondary changes …ulceration etc
not present
No family history

EXAMINATION:
1. INSPECTION :

A. Location ..oral cavity ..( hanging from palate both


hard and soft palate ) mass is peduculated .
B Size 2x1.5x1.5 cm mass
C. Shape oval shaped
D. Surface smooth
E. no secondary changes , no visible
vessels/pulsations , no ulceration , no
haemorrhage , no necrosis.
F. Extension ..Till dorsum of tongue .
G. Association ..with cleft palate .
H. Colour ..pink

2 PALPATION
A. Temperature ..normal
B. Tenderness ..non tender
C. Consistency ..soft but non cystic ..
D . Non fluctuant and non translucent .
E. Non compressible
F . Non pulsatile
G . Fixity .. To palate only

3. PERCUSSION ..dull note

4 . AUSCULTATION ..no bruit ..


INVESTIGATIONS
• TLC..7.8 k
• DLC ..N42L45M8E3
• HB 17.5
• P.C 1.6 L
• RFT ..within normal range
• S. NA ..136
• S.K 4.7
• CRP neg
• PT , PTI , INR ..NORMAL
• USG HEAD AND ABDOMEN ..NORMAL
• CECT Face and Neck :Defect in hard palate with
well defined soft tissue density lession with
internal Fat attenuation areas and a densely
calcified focus .
• Likely midline oral teratoma
• AFP Levels …720 ng/ml
• Biopsy ..show lobules of mature adipose tissue
admixed with fasciles of skeletal muscle , serous
and mucinous acini of salivary glands and covered
by keratinized squamous epithelium containing
hair follicles associated with sebaceous glands .
• Features consistent with mature teratoma
Baby was kept NPO initially ,
antibiotics started along with I/V
fluids ,ENT call sent .

ENT call matured ,Tube feed started ,


course baby was planned for surgery and all
necessary investigations done .

CECT Face and Neck done :Defect in


hard palatewith well defined soft
tissue density lession with internal
Fat attenuation areas and a densely
calcified focus .
Likely midline oral teratoma

Further Subsequently surgery was done ( excision


of mass under G.A) . mass freed from
palate and removed en block and sample
course sent for histopathology .

Post op kept NPO for day , antibiotics


upgraded to 2nd line , I/V fluids started ,
FFP and PRBC transfusion given .
Further course in NICU

Oral hygiene
maintained using
Tube feed started on
liquid betadine swab
post op day 2
Inj t stat given to and spoon feed

control local bleeding started on post op


day 4 .
Further course

Attendants were advised to


come up for follow up with
Antibiotics course completed histopathology report.
Futher course of treatement
and baby was discharged . to be decided as per
histopathology findings and
AFP levels .
SUMMARY
• 39 weeks gest age baby born to non consg
marriage via NVD at kishtwar hospital referred to
us with c/c palatal mass , cleft palate and inability
to feed. Baby received antibiotics, I/V fluids and
operated for palatal mass under G.A during her
stay in hospital. Now baby is successfully
discharged frm hospital after a stay of 32 days on
oral feeds and parents are advised to come back
for follow up with histopathology reports .
probable
diagnosis

congenital
Palatal
teratomas
Objectives of this
presentation

DISCUSS DIFFERENTIAL DISCUSS APPROACH TO DISCUSS THE


OF CONGENITAL PALATAL MASS EVALUATION AND
MIDLINE PALATAL MASS MANAGEMENT OF ORAL
MASS /TERATOMAS

UNDERSTAND THE
TYPICAL OUTCOMES FOR
PATIENTS WITH
TERATOMAS
Differential diagnosis of congenital midline oral
masses

Vascular 1.Congenital haemangiomas


2.AV malformations

Non vascular 1.Teratomas( M/C)


2. Epignathus
3.Oral hamartomas
4.Oral lipomas
5.Dermoid cyst
6.Oral papilloma
7.Oral neurofibromas
ORAL TERATOMAS
• Points in favour
1. Female sex 2. congenital mass 3.solid tumor
4. Calcified areas in tumor with fat attenuation (admixture
of tissue) 5. midline structure
6. No causative factor 7. foreign elements ( calcification )
8.biopsy findings
EPIGNATHUS
Rare teratoma High AFP levels
Teratoma of oropharynx palate is M/c site in
Immature and malignant oral cavity
High mortality due to airway obstruction
After surgery image
Antenatally detection possible by usg
Poor prognosis

Points in favour
1.site ..oral cavity (palate ) 2 .a/w cleft palate

3.solid mass 4.calcification present 5.midline .

6 female sex .

points against
1. antenatally not diagnosed 2. no polyhydraminos

3.very large size 4.cause resp distress mostly

5.normal AFP 6. with extension to nasopharynx and brain .


DERMOID TUMOR

• Cystic teratomas
• contain mature tissue and
benign .
• Manly contain skin , hairs ,
PRteeth , bone , sweat glands .
• Grow slowly .
• Points in favour
1.congenital 2.midline . 3
female
• Points against
1. cystic lesion 2 .contain hair
and skin
Oral hamartomas
• Benign tumor like malformation made up of an
abnormal mixture of cells found in area of body
where it grows .( teratoma tissue is foreign to the
area of growth ) .
• Points against
1 calcification not present 2.rare in head and neck
3 . Histology ..normal tissue
AV malformations and congenital haemangiomas

• Points in favour
1 . Congenital 2. in oral cavity M/c site is palate

• Points against
1 . Non cystic 2. non compressible 3. non
blanchable 4. non vascular
TERATOMAS

Germ cell tumor. As a result abnormal development


of pluripotent cells : germ cells and embryonal cells .

Teratomas derived from germ cells occur in teticles


or ovaries .Teraomas from embryonic cells occur on
the subject midline ..like brain ,neck, oral
cavity ,mediastinum and coccyx.
Teratomas continue
Contain elements of all three germ layers like bone , teeth , muscle , hairs .
Contain tissue foreign to the anatomical site of origin .
Oral teratomas are 2% of all teratomas .
Oral teratomas present as midline masses

Causes airway obstruction or difficuly in breathing .


Usually congenital .
Associated anomalies ..cleft palate
Sacrococcygeal 40 %
Ovary 25%
Testicles 12%
Brain 5%
Others 18 %

Types ..mature /benign more common( females)


Immature /malignant less common ( males )
Gonzalez –Crussi grading
system

Grade 0
mature( benign)
Grade 1
immature , probably benign
Grade 2
immature , possibly malignant (cancerous )
Grade 3
frankly malignant
PRENATALLY via prenatal
ultrasound or MRI

POST NATALLY
DIAGNOSIS 1. AFP levels ..usually
raised( return to normal
after excision )
2. X ray …calcification
3. CECT OR MRI .. Calcification
and for extension of mass
4. Histopathology ..for
definite diagnosis
• Principles of management of
teratomas
• 1.Secure airway
• 2.Early surgical excision (en block )
• 3.If histopathology showed malignant tumor then adjuvant
chemotherapy (bleomycin , etoposide and cisplatin ) and
radiotherapy .
• 4 Monitor AFP levels for recurrence and treatment efficacy.
• 5. repair cleft palate .
• Prognosis
5 year survival rate for grade 1 is greater than
90 %
While advanced stage survival drops to about
50 %with grade 2 and 25% or less with grade 3
In general mature teratomas have good
prognosis in comparison with immature one.
Epignathus type teratomas have poor prognosis
while dermoid cyst have best prognosis
THANKS

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