2.  1 day old neonate
 Antenatal History of anterior neck swelling
 Confirmed postnatally

3.  Maternal age 26 years
 Second gravida with one alive child
 Previous LSCS
 No other significant Medical/Surgical issues
 Regular follow up while in Antenatal period

4.  Clinical assessments normal
 Fetus has large neck mass on USG in 37th week
 Mild Polyhydramnios
 Antenatal diagnosis was cystic hygroma

5. 11.01.2011
Fetus with vertical lie
Cephalic presentation
No abnormality detected

6. 8.03.2011
Well defined hypo echoic mass
Dimensions 5.5 X 6 cm
located in cervical region
Lateral to neck vessels

7.  Guarded prognosis was explained
 Continuation of pregnancy was advised
 Mode of delivery was to be decided upon
obstetric indications
 Antenatal procedures not feasible

8.  FTLSCS at JLNHRC on 19/3/2011 12:50 pm
 Baby had massive neck mass
 Cried immediately after birth
 Had mild respiratory distress in supine position
 APGAR score at 1 min – 7
5 min - 8

9.  Birth of high risk newborn anticipated
 Instruments for securing airways were ready had the
baby deteriorated further
 Kept in thermo neutral environment
 Dried and wrapped in warm clothing's
 Oro-nasal suctioning were performed
 Distress was alleviated in right lateral position

10.  Initial stabilization assured in labour room
 Necessary equipments kept ready in NICU
 Transported with warm clothes
 Airway positioning while transporting assured
 Case was informed to pediatric surgeon

11. › Euthermic
› No other dysmorphic features
› CRT < 2 sec
› Heart rate 122/min
› Respiratory Rate 40/min
› BP 58/32 (40) mm Hg
› Anthropometry

12. › Respiratory system - B/L breath sounds distinct
equally heard
conducted sounds +
Inspiratory stridor
› CVS – S1S2 +, no murmurs
› Per Abdomen – soft, non distended, non tender
› CNS – NNR present
› Ext genital – female, normal

13. › Solitary neck mass
› Large –10x8x6 cms
› Midline, encroaching
towards right
› Non pulsatile
› Skin - normal

14.  Palpation confirmed
the findings
 Temp – normal
 Tenderness – absent
 firm to hard mass
 Not compressible
 Well encapsulated

15.  Mobile in all the
directions
 Not adherent to
underlying structures
 Not adherent to skin
 Trans illumination- absent
 Not pulsatile
 No thrills or hum

16.  Thermo neutral environment with servo control
 Airways management and positioning
 Fluid and electrolyte
 Nutrition
 Respiratory and Hemodynamic monitoring

17. › Tense cystic hygroma
› Teratoma
› Hemangioma
› Neuorblastoma
› Rhabdomyosarcoma
› Rarer conditions
 Congenital thymic cyst
 Congenital goitre
 Branchial cleft cyst

18. › Solid mass
› Well encapsulated
› Few areas of cystic degeneration
› Few stipulated calcifications
› No large calibre vessels inside the tumor
› Neck vessels pushed laterally
› Tracheal displacement +

19.  ENT consultation
› airway status
› need of elective/emergent intubation
› Intra operative help
 ENT Opinion
› No pressure effect on trachea
› No need of emergent intubation
› CT scan
› FNAC

20.  FNAC avoided due to
› possible risk of hemorrhage within the mass
leading to airway compromise.
› Non representative areas aspirated
› Limited sensitivity of FNAC

21. › Origin
› extent
› compression effects
› associated malformations.

22.  Localisation of mass
 Characterisation of nature of lesion
 Airway column assesment
 Relationship with major neck vessels

25.  3 mm section thickness plain and post
contrast
 Base of skull to diaphragm

32.  A large 6.5x6x5 cm sized heterogenous
 Mildly enhancing mass lesion wnich is well
encapsulated containing scattered nodular
calcification seen involving neck anteriorly
and on rt side
 Supreiorly upto submandibular space
 Inferiorly supraclavicular region
 Displacing airway column on left and
major vessels posteriorly possibility of
cervical teratoma.To be correlated with
clinical and histopathological findings

33.  Serum alpha feto-protein on day 2- 83,000 ng/ml
 Normal range = 100000 to 125 ng/ml from neonatal
to infancy

34. › Clinical findings - solid mass
› USG - Non vascular
› CT - Heterogenous
- Calcification
› Raised AFP
Cervical Teratoma

35. › Primary Surgical excision

36.  Airway assessment
 General anaesthesia

37. Direct larngoscopy Difficult airway

43.  Immature cervical teratoma , grade 2

44.  Specimen of 6.5x6x3.5
cm received.
 O/S- nodular with
retracted capsule.

45.  C/S- shows lobulated grey
white mass predominantly
solid with multiple small cysts .
 Cysts are of varying size from
1mm to1cm diameter filled
with mucinous material.
 Few cartilagenous
area, slimy area & bony
spicules were present in solid
part of the mass.

46. H&E STAIN 40X
Mature cartilage
H&E STAIN 10X
Immature cartilage

47. H&E STAIN 10X
RESPIRATORY EPITHLIAL
CLEFT WITH LINING

48. H&E STAIN 10X SQUAMOUS
EPITHELIUM WITH
KERATINIZATION
H&E STAIN 10X NESTS OF
IMMATURE SQUAMOUS
EPITHELIUM

49. H&E STAIN 10X IMMATURE NEURAL
EPITHELIUM

50. H&E STAIN 10X BLASTEMAL CELLS
H& E STAIN BLASTEMAL
CELLS

52. H & E STAIN 20X MUCIN
PRODUCING GLANDS

53. • Multiple sections studied from tumour shows mature as well as
immature elements derived from all 3 germ layers.
• Mature elements comprise of nests of squamous
cells, glands, mature cartilage, occasional bony tissue, neural
tissue & smooth muscle tissue.
• Immature elements include neuroepithelial
elements, occasional group of blastemal cells & immature
cartilage in myxoid stroma. Mitosis is in the range of 2/10HPF.
Normal thyroid tissue is not seen in the section studied.
• Impression:- ABOVE FEATURES FAVOUR IMMATURE CERVICAL
TERATOMA (Grade –II)

54.  0 Mature solid teratoma
 I Abundance of mature tissues, intermixed with loose
mesenchymal tissue with occasional mitoses; immature
cartilage; tooth anlage
 II Fewer mature tissues; rare foci of neuroepithelium
with common mitoses, not exceeding three 40X
fields in any one slide
 III Few or no mature tissue ; numerous neuroepithelial
elements, merging with a cellular stroma occupying
≥four 40X fields

55.  Greek word – monstrous tumour
 Derived from all three embryonic germ layers-
ectoderm, endoderm and mesoderm
 Can occur anywhere in the body
 Most common location – sacral region
 Rarer in adults since most are detected in childhood.
 Neonatal period are uncommon and virtually always benign

56.  Rare congenital tumours of neck
 Challenging in the neonatal period
 Present as massive neck swelling with airway
compression
 High perinatal mortality and morbidity rates.
 Predominantly of the mature variety

58.  Constitute 1.6 to 9.3% of pediatric teratomas, 1per
40,000 births
 Global scenario - Over 150 cases reported so far
 Indian scenario - 4 cases ,1stiiborn, 1 died soon after
birth, 2 surviving
 No apparent relationship to the mother's age
 No greater odds of occurance in males versus females
 No racial or ethnic preference.

59.  Exact cause still unknown
 Inability of totipotent cells to differentiate into
a complete body or organ
 Abnormal development of a conjoined twin
 Arises from stem cells within the thyroid gland

60.  Novel karyotypic changes on comparative
genomic hybridization
› 1p21.1 amplification
› 9p22 deletion
› 17q21.33 1-copy gain

61.  Rare
› Imperforate anus
› Chondrodystrophia fetalis,
› Hypoplastic left ventricle with pulmonary
hypoplasia,
› Cystic fibrosis,
› Absence of corpus callosum,
› Arachanoid cyst

62.  Based on birth status, age at diagnosis, and the
presence or absence of respiratory distress.
› Group I--stillborn and moribund live newborns
› Group II--newborn with respiratory distress
› Group III--newborn without respiratory distress
› Group IV--children age 1 month to 18 years
› Group V--adults

63.  Physical Examination
› Size
› Multiplicity
› Laterality
› Consistency
› Color
› Mobility
› Tenderness
› Fluctuation
› Transillumination test

71.  USG
 MRI

72.  Ultrasound – best modality
 Asymmetric, well-defined
masses
 Large and bulky.
 Calcifications
 Polyhydramnios in 20 to 40
percent cases
 Other fetal abnormalities +

73.  Shows mediastinal
involvement
 position of the
airway.
 Partial / total
Compression

74.  Ex utero intrapartum
treatment (EXIT)
procedure / OOPS
procedure
 Specifically designed to
preserve uteroplacental
gas exchange to provide
time to secure the airway

75.  provides time for:
› Neck dissection
› Clip removal
› Bronchoscopy
› Endotracheal intubation
› Surfactant administration
› Placement of umbilical arterial and venous
catheters

76.  Frequent ANCs
 Frequent ultrasound exams recommended
to monitor
› amniotic fluid volume,
› tumor size,
› growth and the general health of the fetus
 Institutional delivery encouraged
 Elective cesarean preferred
 Team approach for ex utero management

77.  Baseline hemogram and blood biochemistry
 USG
 CT scan/MRI
 FNAC and Biopsy
 Thyroid and parathyroid function test
 Serum alpha fetoprotien and beta HCG
 Transcription factors GATA-4 and GATA-6
 Genetic studies

78.  Expedient multidisciplinary approach
› Airway management
› Definitive management

79.  Primary surgical excision
 If malignancy proved then
› Chemotherapy
› Radiotherapy
› Combination therapy

80.  Risk for serious thyroid conditions
› Hypoparathyroidisim
› Hypothyroidism
 Developmental delay and mental
retardation
 Malignant transformation

81.  Recurrence
 Metastasize to regional lymph nodes
 Occurring among siblings (only one
case reported)

82.  Recommendations
› AFP levels be obtained
 at birth
 at 1month,
 three-month intervals in infancy and
 yearly thereafter, upto 3 years of life
› MRI scanning twice a year for the first three
years of life.

83.  Airway obstruction at birth
 Degree of maturity of tissues
 Completeness of resection
 Associated anomalies
 Mortality is high in untreated infants & low if
treated surgically